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What is ICD-10? ICD-10 is the International Classification of Diseases, Tenth Edition. It is a diagnostic code system that is used in nearly every country in the world, except the United States. Developed by the World Health Organization, ICD-10 was implemented in 1993 in order to replace the ninth edition. This is a strictly diagnostic coding system, though sometimes it is inaccurately intended as ICD-10-CM/PCS. The official guidelines for coding and reporting are available in PDF format at the CDC website. What is ICD-10-CM? Often, ICD-10 refers to the clinical modification of the code, which is abbreviated as ICD-10-CM. The clinical modification system was developed by the Center for Disease Control and Prevention, which already uses the code for mortality reporting. ICD-10-CM will replace the currently used ICD-9-CM. The official transition to ICD-10-CM will occur on October 1, 2013. This date is not flexible, and there is no plan for extension; it must be implemented on time. What is ICD-10-PCS? ICD-10-PCS is a separate designation for the procedural coding system. It was developed for documenting the procedural services of hospitals. Developed for the Centers for Medicare & Medicaid Services (CMS) by 3M Health Information Management, this system will replace ICD-9-CM Volume 3. Hospital inpatient procedures involving operating rooms, nursing services and the like will use ICD-10-PCS. Other common procedures that are not limited to inpatient services such as laboratory tests or educational seminars are not included. Physician services will continue to utilize CPT® (Current Procedural Terminology) codes for outpatient procedures. Though it was created by 3M Health Information Management and not WHO, it is meant to operate in conjunction with ICD-10-CM. Why is the United States making the transition to ICD-10-CM/PCS? ICD-9-CM has reached its limit. The scientific organization of ICD-9-CM means that there are only ten subcategories available for each three-digit category. Most of these have already been used to assign diagnoses. In effect, this leaves no room for the inclusion of medical discoveries. Furthermore, the limitations of ICD-9-CM results in codes being inappropriately placed throughout the book, lowering specificity and contributing to deterioration in its structure. The need for new codes that are easily accessible is great. The CMS offers a collection of project slides that will help entities with the transition from ICD-9-CM to ICD-10-CM more clearly. Issues covered include the ICD-10 MS-DRG Conversion Project, ICD-9 code conflicts, process efficiencies and enhancements to GEMs. Why are there now two systems (CM and PCS) instead of one? Both systems were developed by different organizations under different timeframes. Previously, ICD-9-CM included procedural as well as clinical information. Despite being created by CMS and NCHS respectively they were referred to as one system. Similarly, ICD-10-CM and ICD-10-PCS may be referred to jointly as ICD-10-CM when implemented. What is GEM? GEM stands for General Equivalence Mappings. They were developed by prominent institutions in health care such as the CMS, CDC, AHIMA and the AHA. GEMs assist in converting ICD-9-CM codes to ICD-10-CM codes and vice versa. They are often referred to as crosswalks. Who is behind the transition to ICD-10-CM/PCS? The U.S. Department of Health and Human Services (HHS) released a final rule (HIPAA Administrative Simplification: Modifications to Medical Data Code Set Standards to Adopt ICD-10-CM and ICD-10-PCS Final Rule) requiring all entities covered by the Health Insurance Portability and Accessibility Act (HIPAA) to implement ICD-10-CM/PCS medical coding by October 1, 2013. Entities that use ICD-9 codes in claims after this date will not be paid. How is ICD-10-CM similar to ICD-9-CM? ICD-10-CM is based on similar guidelines and formal conventions as ICD-9-CM. It is also organized in a similar way. This means that those who are familiar with ICD-9-CM will be able to learn ICD-10-CM relatively quickly. Many chapters used in ICD-9-CM are also found in the newer edition, though sometimes they are found in a different order. How is ICD-10-CM different from ICD-9-CM? ICD-10-CM is an entirely new structure for diagnosis classification. The most significant structural differences are longer code length, the inclusion of more alphanumeric codes and narrative descriptions. The ICD-10-CM allows for codes to be anywhere from three to seven digits of mixed numbers and letters (beginning with a letter), while the ninth edition only allows for three to five digit codes that are mostly numeric. Another difference is that ICD-10-CM uses computer-readable tables that no longer have section header or chapter codes. While the ninth edition has approximately 13,000 codes, the tenth has approximately 69,000. The concurrent addition of ICD-10-PCS raises this number of codes to 140,000. Part of the reason for this dramatic increase is due to the emphasis on laterality. Systems that are highly lateral see an increased number of chapters in ICD-10-CM. In fact, twenty-seven percent of the codes include “right,” while another twenty-seven percent includes “left.” These descriptions will allow for greater specification and efficiency. How will the ICD-10-CM/PCS differences be beneficial to treatment? Despite the daunting increase in number, ICD-10-CM can transmit greater amounts of information. One code can inform a health care worker of: a disease and its manifestation, stage of treatment (initial, follow-up, or late effects), or in obstetric care, trimester of procedure. Further, while ICD-9-CM only specifies condition, ICD-10-CM specifies location. In the lungs, for instance, the ICD-9-CM would only be able to indicate a condition of the lungs, while ICD-10-CM indicates left lung, right lung, both lungs, or unspecified. This will improve speed of diagnosis as well as treatment. How else are the ICD-10-CM/PCS differences beneficial to healthcare providers and patients? These more detailed codes will result in a better understanding of disease patterns as well as treatment outcomes. Such valuable information is important to the advancement of medical care. Additionally, ICD-10-CM offers several logistic improvements. For instance, the detailed information transmitted through the new code will make operational processes such as claim submissions and reimbursement more efficient. This additional detail will make initial claims clearer for payers. Furthermore, this more precise system will result in fewer unnecessary tests and ultimately will reduce administrative overhead. How is ICD-10-PCS different from ICD-9-CM? ICD-10-PCS is substantially different from Volume 3 of ICD-9-CM that it is replacing. It is also noticeably different from CPT ® codes that are used. It is an entirely new system that is meant to better address the quickly changing medical field and its medical procedures. Utilizing the same multi-axial design as ICD-10-CM, it also results in greater granularity. Similarly to ICD-10-CM, the code emphasizes laterality, dissimilarly; ICD-10-PCS requires it as “unspecified” is not anatomically possible. Those using ICD-10-PCS will be required to have greater anatomical and physiological knowledge than under ICD-9-CM. This difference may require the coder to gain additional knowledge outside of their specialty areas. How does ICD-10-PCS code work? The first characters indicate what, where and how, while the fifth indicates approach, the sixth indicates device and the seventh includes a qualifier. Anatomy and procedure are well covered by this coding system. For instance, medical and surgical (0) action on the endocrine system (G) in the form of excision (B)of the left adrenal gland (2) via a percutaneous approach (3) carried out with no device (Z) or qualifier (Z) results in the code 0GB23ZZ. Clearly, ICD-10-PCS transmits a great deal of information through a relatively small code. A PDF that includes some examples of ICD-10 coding tables can be found here. These useful examples demonstrate the way in which coding will be organized. Steven J. Steindel, PhD, FACMI, further explains learning and using ICD-10-PCS in an entry on the Journal of AHIMA’s website. He provides greater insight into how coding in ICD-10-PCS works as well as its use. What challenges will be presented by the transition to ICD-10-CM/PCS? Equating the documentation in medical records as they correlate to ICD-10-CM/PCS definitions will be a significant challenge in transitioning to the new format. Healthcare professionals, who are already working under tight time constraints, will be asked to use an unfamiliar coding system. Many will be reluctant to make the switch in their documentation procedures unless there is a strong urge towards following through with ICD-10-CM/PCS notation. This is especially true in cases of complex procedures that may require multiple codes. In order to ensure clinical clarity and precision, healthcare providers must adhere to the new coding standard. Will ICD-10-CM/PCS help build an electronic medical record? Yes, both systems are tailored to be used in electronic health record systems. These more modern codes will take advantage of clinical reference terminology and are more conducive to computer-assisted coding. How much will ICD-10-CM/PCS cost to implement? The RAND Science and Technology Policy Institute conducted a survey on the costs and benefits of introducing the ICD-10 systems. Their study found that the new systems could save up to seven billion dollars over the next ten years. What happens when ICD-11 is created? Why not just wait for ICD-11? Currently, WHO is drafting an updated ICD edition for endorsement by 2015 at its World Health Assembly. The alpha draft was made available online in July 2011. Unlike previous developments, the WHO is utilizing Web 2.0 principles to construct the document via a multi-author drafting platform. There is no set implementation date, or clear timeframe. This means waiting many extra years and further additional modifications will likely be need for implementation in the United States. Furthermore, ICD-10 will be more similar to ICD-11 than ICD-9. Many of the costs that would go into upgrading directly to ICD-11 will be surpassed by first implementing ICD-10-CM/PCS. What is HIPAA 5010? HIPAA (the Health Insurance Portability and Accountability Act) 5010 is a replacement for the current X12 submission standard that is used for determining what entities must use for conducting electronic transactions. Such transactions are responsible for transmitting sensitive health information between specific parties. For instance, a health care provider sending medical claims to a health plan for payment via an electronic exchange must do so under a specified cryptographic standard. Other transactions include: - remittance advice - claims status - enrollment and disenrollment The standard is intended to protect the sensitive patient information that is transmitted. Currently, version 4010 is being used under HIPAA standards. HIPAA 5010 is equally important to the realization of ICD-10-CM/PCS, despite its lack of notoriety. When will HIPAA 5010 be implemented? Covered entities must have met level I compliance by January 1, 2011. This level of compliance entails the ability to process 5010 transactions for use in testing as well as transition with partners that are able. Level II compliance is required by January 1, 2012. Covered entities must use HIPAA 5010 for all transactions at this time. Why is HIPAA 5010 necessary and how is it different from HIPAA 4010? HIPAA 5010 will account for the increased complexity of ICD-10-CM/PCS codes, ensuring the cryptographic protection of sensitive information sent electronically. It also operates in a distinctly different way. For instance, it includes more clearly defined situational rules that will help with specific transactions. HIPAA 500 does not necessarily require ICD-10-CM/PCS codes. It is able to differentiate between ICD-9 and ICD-10-CM/PCS, potentially clarifying issues surrounding billing due to two sets of code. You can read more about the eleventh edition of the WHO ICD and its development online at their website.	4	5	0	0	0	1	0.287436	1	2,782
What is HIPAA? Created in 1996, HIPAA is an act of Congress that protects the health insurance of workers and their families if they lose their jobs. HIPAA also protects the privacy of children 12 to 18 years of age and establishes a number of regulations for the electronic transfer of healthcare data. This last point is where we’ll spend the most time in our summary, but let’s look at the whole Act in brief first. HIPAA is divided into five sections, or Titles. For the sake of this course, we’ll focus only on the first two Titles, which are the largest and most far-reaching. Title I establishes rules for how group health organizations (like managed care organizations) interact with patients. Title I limits the restrictions a group health organization can put into place based solely on a pre-existing condition. Title I also limits the amount of time it takes to get coverage for that pre-existing condition. Specifically, once a person has coverage under a group health organization, that person must receive coverage for their pre-existing condition within 12 months (or 18 months in certain circumstances). Title I also provides protection to individuals and their families when that individual changes or loses their job. If an individual has health insurance under their old job, they are allowed to keep that insurance until the point when their new health care coverage kicks in. There are a few caveats to this, of course, and HIPAA does not provide permanent health insurance. It does, however, ensure that persons out of work can continue their health care coverage while in between jobs. As you may be able to tell, this Title of HIPAA affects insurance companies and their interactions with patients much more than it does medical billers. Title II is where we’ll see HIPAA affect medical billing more directly. When HIPAA was passed, an increasing number of medical transactions were being performed electronically. While electronic transactions (like claims) were faster, more cost-efficient, and less error-prone, they also caused some patients and regulators to worry about the privacy of the personal medical records. Title II addresses theses concerns and establishes standards and guidelines for these types of transactions. Privacy and Security Title II lays out a set of security guidelines that ensure the safety of both physical and electronic records. These regulations limits who can view medical information, and also dictates how this information is transferred. Title II also established a set of rules limiting who can distribute your medical information, and when. These rules give patients more control over their medical records, including who can access them and at what times. These rules prevent anyone—including providers, payers, or government agencies—from viewing or distributing a patient’s medical information for anything not related to treatment for the patient. For instance, on a worker’s compensation claim for a broken finger, a biller would not include the patient’s history of heart disease. Title II and Medical Billing The passage of HIPAA added an “Administrative Simplification” (AS) to a portion of the Social Security Act. With the AS, Title II established a set of regulations and guidelines for the electronic transmission of healthcare data, and sets up guidelines for the code sets used in medical billing and coding. The goal of the AS was to establish a regular, uniform method of communication for any party involved in healthcare, such as insurance payers, providers, clearinghouses, and government agencies. All bodies covered by HIPAA (and this includes most providers and payers, including Medicare and Medicaid) must adhere to these standards of transactions. Under Title II, all electronic transactions must be performed as a type of Electronic Data Interchange (EDI). (An EDI is a standardized form of electronic transaction. It’s widely used in all types of commerce. An ATM withdrawal, for example, uses an EDI). For healthcare transactions, providers and payers must use the EDI approved by the Accredited Standards Committee X12 (ASC X12). Under Title II, each medical transaction has to adhere to a certain format. HIPAA dictates that those electronic transactions follow the format laid out by the ASC X12. This form is the ASC X12 005010. Included in that form are various subforms, each of which corresponds to a certain type of medical transaction. Again, we’ll discuss more of how HIPAA Title II affects medical billing in the next course. Let’s close out this section with a brief overview of what else Title II does. Title II establishes the mandatory use of National Provider Identifier (NPI) numbers. You should remember NPIs from our discussion on creating medical claims. These NPIs are ten characters long, may be alphanumeric, and are never re-used (except in very particular situations). Like ICD or CPT codes, NPIs provide an efficient universal shorthand for identifying a crucial part of the healthcare process. In addition to establishing the above regulations and rules, Title II also outlines a number of offenses related to healthcare and prescribes civil and criminal punishments for these fraudulent offenses.	3	2	0	0	0	3	0.662248	3	1,050
The improved absolute survival rates may be obscured or downplayed in some patients by a detriment to quality of life. As would be expected, many patients who have an ICD feel less healthy, have emotional and physical issues, and have an overall lower level of psychological well-being. This is most prominent in patients who have received prior shocks. The psychological downsides of an ICD, particularly post-shock, include anxiety, panic disorder, or overt agoraphobia. Explaining the benefits of an ICD shock, along with reassurance and psychological intervention, are crucial to improving the quality of life in any patient with an ICD. Psychiatric medications may be necessary, but they are not the total answer. Probably the most effective way to decrease psychological problems with an ICD is to decrease inappropriate discharges. This is best accomplished by continuing to monitor the device and reprogramming it once an inappropriate ICD shock has been identified. Because antitachycardic pacing is effective in almost 95 percent of cases of spontaneous slow VT (160–180 beats/min), some devices will attempt overdrive pacing up to three times before delivering even a low-energy shock. Rapid VT can be successfully terminated less often with antitachycardic pacing, but it's still often effective. Obviously, overdrive pacing is preferred to shock, and patient acceptance of the device is greatly enhanced if they are not delivered an electrical shock. That shock, however, is designed to save a life if it is appropriately used. Sophisticated programming allows the ICD to distinguish between sinus tachycardia and VT, allow the device to observe the rhythm for a short period of time, and undertake other sophisticated diagnostic analysis and interventions. The ICD can usually distinguish VT from AF because of the stability of the QRS complexes. Even the morphology of the QRS complex can be evaluated. Some devices have atrial sensing capabilities to increase discrimination of VT from SVT. The device can be programmed to wait for 16 to 24 beats of VT before responding, reducing shocks for short runs of VT that spontaneously convert. Non-life threatening arrhythmias are essentially ignored by sophisticated ICDs. Some patients are empirically placed on prophylactic antiarrhythmics, but most have medication added once the ICD fires. More than half of ICD patients are eventually prescribed such medications. Drugs to control inappropriate shocks due to ICD misinterpretation of atrial dysrhythmias (AF, SVT, sinus tachycardia) are commonly used. A variety of cardiac medications can reduce ICD shocks by decreasing the overall frequency of VT or produce a slower ventricular response, rendering more episodes amenable to overdrive pacing while avoiding a shock. Sotalol (Betapace) is a beta blocker that has reasonable efficacy in this situation. A recent new class of antiarrhythmics, including azimilide, shows some promise to reduce ICD discharges. The most effective regimen so far, however, appears to be a combination of a beta blocker and amiodarone. This combination decreases the inappropriate shock rate to about 10 percent versus 20 percent. Both drugs do have their limitations and side effects, however. Interestingly, the lipid-lowering statins also may have an antiarrhythmic effect to reduce the occurrence of ICD shocks. With drug therapy also comes the chance that the threshold for defibrillation will be increased, making an ICD shock less likely to terminate VF successfully. Amiodarone can increase the defibrillation threshold, but this seems variable, and it can be corrected by reprogramming the device. Amiodarone also has pulmonary and thyroid toxicity, and sotalol has been associated with Torsades de pointes. These authors suggest that all patients first be treated with a beta blocker unless contraindicated. If the ICD has already discharged, sotalol and amiodarone are suggested as a first option combination. The occurrence of three or more episodes of sustained VT requiring a shock within a 24-hour period has been termed electrical storm. This often produces repetitive shocks, and this distressing event can occur in up to 20 percent of patients with an ICD. The ICD discharge can be appropriate or inappropriate, and electrical storm for appropriate discharges indicates serious life-threatening dysrhythmias. There are limited data on how to manage this phenomenon acutely. Some obvious precipitants, such as electrolyte imbalance, drug overdose, worsening CHF, and myocardial ischemia, may be treated. The first antiarrhythmic choice for electrical storm appears to be amiodarone. In addition, sympathetic blockade with beta blockers can be added. Sedation is also important in controlling electrical storm to decrease sympathetic outflow and the tachycardia associated with it. Ablation therapy also is an option if drug therapy fails, but this cannot be accomplished rapidly. These authors reiterate the prior adage that a single ICD shock is not necessarily a requirement for an ED or office visit. The authors note that “occasional shocks are to be expected.” Evaluation within one week seems to be the suggested norm for a single shock without associated symptoms. Syncope, shortness of breath, palpitations, or chest pain prior to the shock should prompt an ED visit. Following a shock in a now-asymptomatic patient requires evaluating ICD-stored EKGs via device interrogation to determine the exact event. The authors note that multiple shocks for sinus tachycardia can be made worse by anxiety, pain, or fever. The ICD can be disabled by placing a magnet over the chest wall pocket. This would be counterproductive in the presence of VT. Comment: The ability of the ICD to detect and treat cardiac dysrhythmias is mind-boggling. These devices appear to be better than some physicians at differentiating AF from VT, and are even smart enough to wait to observe patients further before shocking them again. Instituting antitachycardic pacing rather than delivering a shock at the first sign of VT also is an amazing ability. Low-energy shocks are used first, reserving high-energy shocks for non-responders. Despite the admonition by our cardiology colleagues that a single shock without associated symptoms is “no big deal,” very few patients can sit at home and follow their doctor's advice that an occasional shock is nothing to worry about. When a patient rolls into the ED, usually by ambulance in a terrified or agitated state, the emergency physician must be prepared not to act promptly. Once the shock and awe subsides, the clinician can then try to figure out what happened and what to do next. I recently treated one patient with electrical storm who experienced an amazing 51 shocks for bona fide VT, all delivered over a few hours. It was not a pleasant experience for me and certainly not for the patient. Imagine getting a 30 joules shock every few minutes, often sensing the VT and knowing that the jolt will come soon after. I cannot overemphasize that need for rapid and appropriate sedation and analgesia under such circumstances. The authors of this paper suggest propofol, stating that it has some “antiarrhythmic properties” (news to me, but interesting). Most emergency physicians would likely opt first for a benzodiazepine and fentanyl, an effective combination that is familiar to physicians for a variety of ED procedures, including cardioversion. Most patients with electrical storm cannot wait for a cardiologist in the ED, so some ED game plan needs to be in place. After sedation and analgesia, the cause of the discharge should be determined. It's easy if the underlying problem is VT. Those patients should be treated with IV amiodarone (in a dose equal enough to be effective), beta blockers, and possibly lidocaine. The exact dose and sequence has yet to be determined, but don't forget beta blockers as an early intervention. It seems most reasonable to first administer amiodarone (150 mg IV) and then metoprolol (Lopressor in 2–3 mg aliquots). This approach slows the heart rate, treats the underlying VT, and hopefully also negates the next shock. If the patient is in rapid atrial fibrillation, IV beta blockers and calcium channel blockers, in standard regimens familiar to most emergency physicians, are indicated. Other ICD-triggering atrial arrhythmias are treated as necessary, including adenosine. If the ICD continues to fire in the presence of AF, placing a magnet over the device will inactivate it, and stop the inappropriate discharge. The key is to determine whether this is AF with aberrant conduction (wide QRS) or VT, not easy with a very fast rate (hint: try carotid massage). Theoretically, helpful adjuncts are diuretics for worsening CHF, acetaminophen for fever, IV nitrates for hypertension, and high-flow oxygen. Once the initial electrical event has been addressed, interrogation of the device is important to gather historic information. All device companies have 24-hour assistance with this process, although some cardiologists can interrogate the device themselves. In my experience, the company rep is always called. Amazing information can be obtained by interrogation, and it seems reasonable to do it almost every time a true emergency exists. A leisurely stop by the ED for a checkup when the device discharged two days earlier, without symptoms, could probably await an office visit for interrogation. Of course, one should always consider reversible causes such as electrolyte abnormalities. Drug toxicity presents a more puzzling scenario; hopefully the patient did not overdose on a tricyclic antidepressant or digoxin. It seems reasonable also to take a chest x-ray to look for lead fracture or displacement, but such findings are subtle and actually quite rare. Some antiarrhythmics are pro-arrhythmic by nature. Sticking with amiodarone and beta blockers for ventricular arrhythmias seems like a safer bet to me. If the patient is already maxed out on medication, there are few options left. Such patients are probably near the end of their lives, and have end-stage heart failure or intractable arrhythmias, and this is indeed a dire situation. Ablation procedures, cardiac assist devices, or some of the newer experimental drugs may be helpful, but this is a sad state of affairs for the patient. Finally, the psychological impact of an ICD can probably only be appreciated by the recipient of the shock. I can only imagine what it is like to walk around with a bulge in your chest wall that can shock the daylights out of you at any time. That can't be good for your psyche or love life, and certainly doesn't prompt you to make reservations for an exotic cruise. So much for the golden years when you have an ICD. The ICD is a remarkable device that can keep some patients productively alive for many years. The data are not as sanguine as the theories behind them. The number needed to implant with an ICD to save one life is about 15, probably attesting to the seriousness of end-stage CHF, which has a prognosis worse than many cancers, particularly in the presence of prior MI and cardiomyopathy. Interventions and Options for ICD Programming Suggested Initial ED Approach to ICD Electrical Storm ▪ IV, oxygen, monitor, 12-lead EKG, vital signs (including temperature) ▪ Immediate sedation/analgesia if active discharge ▪ STAT electrolyte determination (including magnesium)1 ▪ Atrial arrhythmia (ST, AF, SVT): Diltiazem/metoprolol/adenosine2 ▪ Ventricular arrhythmia: Amiodarone/metoprolol3 ▪ Cardiology consultation ▪ Address CHF, thyroid status, hypertension, hypotension, hypoxia ▪ Evaluate for AMI, reversible ischemia ▪ Post-therapy: Interrogation of device, admission ▪ Consider diuretics, statins, BP control, nitrates ▪ Evaluate for drug overdose/toxicity 1. Value not known/more likely if patient is on diuretics. 2. Place magnet over ICD if truly inappropriate discharges. 3. Consider also lidocaine, magnesium, procainamide, others. Episode Lists Report Discharges/Shocks from an ICD ▪ Monomorphic ventricular tachycardia ▪ Polymorphic ventricular tachycardia ▪ Torsades de pointes ▪ Ventricular fibrillation ▪ Atrial fibrillation ▪ Atrial flutter ▪ Atrial tachycardia ▪ Supraventricular tachycardia ▪ Junctional tachycardia ▪ Sinus tachycardia ▪ Multiple premature ventricular contractions ▪ Oversensing T waves ▪ Double counting of QRS complex ▪ Oversensing due to lead failure or insulation break ▪ Oversensing of diaphragmatic myopotentials ▪ Electromagnetic interference Adapted from JAMA 2006;296:2839. CME Participation Instructions To earn CME credit, you must read the article in Emergency Medicine News, and complete the quiz, answering at least 80 percent of the questions correctly. Mail the completed quiz with your check for $10 payable to Lippincott Continuing Medical Education Institute, Inc., 770 Township Line Road, Suite 300, Yardley, PA 19067. Only the first entry will be considered for credit, and must be received by Lippincott Continuing Medical Education Institute, Inc., by September 30, 2009. Acknowledgment will be sent to you within six to eight weeks of participation. Lippincott Continuing Medical Education Institute, Inc. is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. Lippincott Continuing Medical Education Institute, Inc. designates this educational activity for a maximum of 1 AMA PRA Category 1 Credit™. Physicians should only claim credit commensurate with the extent of their participation in the activity. © 2008 Lippincott Williams & Wilkins, Inc.	2	11	1	0	0	0	0.792959	1	2,978
Coverage Indications, Limitations, and/or Medical Necessity Human Leukocyte Antigen (HLA) typing is performed to assess compatibility of recipients and potential donors as a part of solid organ and hematopoietic stem cell/ bone marrow pre-transplant testing. HLA testing is also performed to identify HLA alleles and allele groups (antigen equivalents) associated with specific diseases and individualized responses to drug therapy (e.g., HLA-B27 and ankylosing spondylitis and HLA-B57:01 and abacavir hypersensitivity), as well as other clinical uses. One or more HLA genes may be tested in specific clinical situations (e.g., HLA A, B, C,-DRB1, and DQB1 for kidney transplantation). Each HLA gene typically has multiple variant alleles or allele groups that can be identified by typing. HLA antigens are divided into types: Class I (A, B, C) and Class II (DR, DP, DQ). The primary use for HLA testing is to match organ and tissue transplant recipients with compatible donors. Different kinds of transplants necessitate different levels of matching between donor and intended recipient. This may determine which HLA tests are performed and which HLA genes are tested for. HLA typing identifies the unique constellation of HLA antigens for an individual. HLA typing using newer DNA technologies provides tests that are more robust, accurate and reliable in resolving allele-level differences in HLA genes that cannot be detected by serology. DNA tests can be performed using a variety of source materials (lymphocytes, whole blood, buccal swabs, biopsy samples, frozen tissue) and are less affected by viability and sample age. Several approaches to HLA typing are used, offering a range of typing resolution levels from low (antigen-level) to high (allele-level). Examples include, tests used to identify HLA types that rely on amplification of limited stretches of genomic DNA within the HLA genes. The genetic polymorphisms associated with the different HLA alleles are identified through hybridization with specific amplification primers: sequence-specific primer (SSP) or sequence specific oligonucleotide probes (SSO) or by direct sequencing-based typing (SBT). Reverse SSO hybridization is used to determine HLA-A, -B, -C, -DR, -DQ and -DP locus types at an intermediate level of resolution, somewhat higher than serological testing. Tests of this type are used when low or intermediate resolution typing is required or as a screening test to identify potential donors or individuals who may later require higher resolution testing. This technology is used for high volume testing and allows for relatively low-cost typing for bone marrow donor drives or other applications involving large sample numbers. PCR-SSP is also used to determine HLA-DP and to determine, at a resolution similar to serological testing, HLA-A, -B, -C, -DR and DQ locus types. PCR-SSP is a very rapid test that can be performed in 3-4 hours from the time a sample is received. PCR-SSP is used for typing deceased organ donors when speed is an important consideration. PCR-SSP can also be used to provide higher resolution testing and may be employed to resolve alleles. In this technique, PCR primers are designed to anneal only to a specific set of alleles or to a single allele. SBT provides the highest resolution HLA typing for HLA-A, -B, -C, -DR, -DQ and -DP locus alleles. SBT is used when the highest resolution typing is important as in donors and recipients of stem cell transplants or in examining disease associations. The commercial availability does not ensure that a molecular diagnostic test is indicated for clinical application. Molecular diagnostic testing is a rapidly evolving science in which the significance of detecting specific mutations has yet to be clarified in many circumstances. Analytical and clinical validity as well as clinical utility are the responsibility of the provider, and all testing must meet standards of care. For the purpose of this LCD, the Molecular Pathology Procedures for HLA typing will be considered medically and reasonable necessary when the following apply: Standard of care determination of HLA matching for solid organ transplant (donor/recipient). Solid organ transplant registries include both serological HLA testing (e.g. crossmatch) and genomic molecular DNA typing. Family members, or unrelated living donors or cadaveric donors who donate bone marrow or a solid organ are HLA tested pretransplant to determine compatibility with the potential recipients. Standard of care identification of determination of HLA matching for hematopoietic stem cell/bone marrow transplantation. Allele-level typing will provide clinical guidance for the HLA-A,B,C Class I and DRB1, DQB1,DPB1, and DQA1 Class II loci in the average transplant program because it is well established that mismatches at certain HLA loci between donor-recipients are closely linked to the risk of graft versus host disease. Potential marrow donors may enroll with a national registry such as the United States National Marrow Donor Program or the Canadian Blood Services registry. Standard of care testing to diagnose certain HLA related diseases/conditions when the testing is supported by the clinical literature and is informative for the direct management of a patient bearing a certain allele(s). It is not expected that more than one test would be required in a given beneficiary’s lifetime. Possible covered indications when standard laboratory testing (tissue typing) is not adequate: HLA-B27 for the diagnosis of certain cases of symptomatic patients with presumed ankylosing spondylitis or related inflammatory disease. HLA-B27 is covered for ankylosing spondylitis in cases where other methods of diagnosis would not be appropriate or have yielded inconclusive results (National Coverage Determination 190.1). In the work-up of certain patients with an unclear diagnosis of celiac disease and gluten hypersensitivity usually related to ambiguous standard laboratory results and/or inconsistent biopsy results (e.g., HLA-DQ2 by HLA-DQB102 and of DQ8 by HLA-DQB10302). Standard of care testing to diagnose certain HLA related drug hypersensitivity reactions when the testing is supported by the clinical literature and is informative for the direct management of a patient bearing a certain allele(s) associated to fatal skin drug reactions (Stevens-Johnson syndrome and toxic epidermal necrolysis). It is not expected that more than one test would be required in a given beneficiary’s lifetime. Possible covered indications: HLA –B5701 when testing performed prior to the initiation of an abacavir-containing regime in the treatment of HIV Infection. HLA-B1502 when genotyping may be useful for risk stratification when the testing is performed prior to the initiation of carbamazepine therapy in the treatment of patients at high risk of having this allele. HLA-B1502 occurs almost exclusively in patients with ancestry across broad areas of Asia, including South Asian Indians. Identification of HLA compatible platelets for transfusion. The following will be considered noncovered as applicable due to statutory exclusion, lack of Medicare benefit, not reasonable and necessary, or not separately billable (a component of the service per NCCI regulations). Tests considered screening in the absence of clinical signs and symptoms of disease (e.g., HLA-DQB106:02P as a positive/negative predictor for narcolepsy). Tests that do not provide the clinician with actionable data (information that will improve patient outcomes and/or change physician care and treatment of the patient). Tests that confirm a known diagnosis or known information (and no new data for decision making). Tests to determine risk for developing a disease or condition. Tests without diagnosis specific indications. Tests performed to measure the quality of a process. Tests for Quality Control/Quality Assurance (QC/QA), i.e., tests performed to ensure a tissue specimen matches the patient. Tests assessing the risk of allopurinol hypersensitivity reactions (HLA-B58:01P). Group 1 Paragraph: N/A Group 1 Codes: 81370 Hla i & ii typing lr 81371 Hla i & ii type verify lr 81372 Hla i typing complete lr 81373 Hla i typing 1 locus lr 81374 Hla i typing 1 antigen lr 81375 Hla ii typing ag equiv lr 81376 Hla ii typing 1 locus lr 81377 Hla ii type 1 ag equiv lr 81378 Hla i & ii typing hr 81379 Hla i typing complete hr 81380 Hla i typing 1 locus hr 81381 Hla i typing 1 allele hr 81382 Hla ii typing 1 loc hr 81383 Hla ii typing 1 allele hr ICD-10 Codes that Support Medical Necessity ICD-10 CODE DESCRIPTION T86.00 – T86.819 – Opens in a new window Unspecified complication of bone marrow transplant – Unspecified complication of lung transplant T86.830 – T86.839 – Opens in a new window Bone graft rejection – Unspecified complication of bone graft T86.850 – T86.99 – Opens in a new window Intestine transplant rejection – Other complications of unspecified transplanted organ and tissue Z48.21 – Z48.298 – Opens in a new window Encounter for aftercare following heart transplant – Encounter for aftercare following other organ transplant Z94.0 – Z94.9 – Opens in a new window Kidney transplant status – Transplanted organ and tissue status, unspecified Z95.3 – Z95.4 – Opens in a new window Presence of xenogenic heart valve – Presence of other heart-valve replacement Group 2 Paragraph: CPT Code 81374 for HLA-B*27 Testing ICD-10 CODE DESCRIPTION M08.1 Juvenile ankylosing spondylitis M45.0 – M45.9 – Opens in a new window Ankylosing spondylitis of multiple sites in spine – Ankylosing spondylitis of unspecified sites in spine M48.8X1 – M48.8X9 – Opens in a new window Other specified spondylopathies, occipito-atlanto-axial region – Other specified spondylopathies, site unspecified	4	16	2	0	0	3	0.712242	5	2,299
DiGeorge syndrome, also known as 22q11.2 deletion syndrome, is a syndrome caused by the deletion of a small segment of chromosome 22. While the symptoms can vary, they often include congenital heart problems, specific facial features, frequent infections, developmental delay, learning problems and cleft palate. Associated conditions include kidney problems, hearing loss and autoimmune disorders such as rheumatoid arthritis or Graves' disease. DiGeorge syndrome is typically due to the deletion of 30 to 40 genes in the middle of chromosome 22 at a location known as 22q11.2. About 90% of cases occur due to a new mutation during early development, while 10% are inherited from a person's parents. It is autosomal dominant, meaning that only one affected chromosome is needed for the condition to occur. Diagnosis is suspected based on the symptoms and confirmed by genetic testing. Although there is no cure, treatment can improve symptoms. This often includes a multidisciplinary approach with efforts to improve the function of the potentially many organ systems involved. Long-term outcomes depend on the symptoms present and the severity of the heart and immune system problems. With treatment, life expectancy may be normal. DiGeorge syndrome occurs in about 1 in 4,000 people. The syndrome was first described in 1968 by American physician Angelo DiGeorge. In late 1981, the underlying genetics were determined. Signs and symptoms The features of this syndrome vary widely, even among members of the same family, and affect many parts of the body. Characteristic signs and symptoms may include birth defects such as congenital heart disease, defects in the palate, most commonly related to neuromuscular problems with closure (velopharyngeal insufficiency), learning disabilities, mild differences in facial features, and recurrent infections. Infections are common in children due to problems with the immune system's T cell-mediated response that in some patients is due to an absent or hypoplastic thymus. DiGeorge syndrome may be first spotted when an affected newborn has heart defects or convulsions from hypocalcemia due to malfunctioning parathyroid glands and low levels of parathyroid hormone (parathormone). Affected individuals may also have other kinds of birth defects including kidney abnormalities and significant feeding difficulties as babies. Gastrointestinal issues are also very common in this patient population. Digestive motility issues may result in constipation. Disorders such as hypothyroidism and hypoparathyroidism or thrombocytopenia (low platelet levels), and psychiatric illnesses are common late-occurring features. Microdeletions in chromosomal region 22q11.2 are associated with a 20 to 30-fold increased risk of schizophrenia. Studies provide various rates of 22q11.2DS in schizophrenia, ranging from 0.5 to 2.0% and averaging about 1.0%, compared with the overall estimated 0.025% risk of the 22q11.2DS in the general population. Salient features can be summarized using the mnemonic CATCH-22 to describe 22q11.2DS, with the 22 signifying the chromosomal abnormality is found on the 22nd chromosome, as below: - Cardiac abnormality (commonly interrupted aortic arch, truncus arteriosus and tetralogy of Fallot) - Abnormal facies - Thymic aplasia - Cleft palate Individuals can have many possible features, ranging in number of associated features and from the mild to the very serious. Symptoms shown to be common include: - Congenital heart disease (40% of individuals), particularly conotruncal malformations (interrupted aortic arch (50%), persistent truncus arteriosus (34%), tetralogy of Fallot, and ventricular septal defect) - Cyanosis (bluish skin due to poor circulation of oxygen-rich blood) - Palatal abnormalities (50%), particularly velopharyngeal incompetence, submucosal cleft palate, and cleft palate; characteristic facial features (present in the majority of Caucasian individuals) including hypertelorism - Learning difficulties (90%), including cognitive deficits, attention deficit disorders - Hypocalcemia (50%)(due to hypoparathyroidism) - Significant feeding problems (30%) - Renal anomalies (37%) - Hearing loss (both conductive and sensorineural) (hearing loss with craniofacial syndromes) - Laryngotracheoesophageal anomalies - Growth hormone deficiency - Autoimmune disorders - Immune disorders due to reduced T cell numbers - Seizures (with or without hypocalcemia) - Skeletal abnormalities - Psychiatric disorders This syndrome is characterized by incomplete penetrance. Therefore, there is a marked variability in clinical expression between the different patients. This often makes early diagnosis difficult. Children with DiGeorge syndrome have a specific profile in neuropsychological tests. They usually have a below-borderline normal IQ, with most individuals having higher scores in the verbal than the nonverbal domains. Some are able to attend normal schools, while others are home-schooled or in special classes. The severity of hypocalcemia early in childhood is associated with autism-like behavioral difficulties. Adults with DiGeorge syndrome are a specifically high-risk group for developing schizophrenia. About 30% have at least one incident of psychosis and about a quarter develop actual schizophrenia. Individuals with DiGeorge syndrome also have a higher risk of developing early onset Parkinson's disease (PD). Diagnosis of Parkinson's can be delayed by up to 10 years due to the use of antipsychotics, which can cause parkinsonian symptoms. Speech and language Current research demonstrates a unique profile of speech and language impairments is associated with 22q11.2DS. Children often perform lower on speech and language evaluations in comparison to their nonverbal IQ scores. Common problems include hypernasality, language delays, and speech sound errors. Hypernasality occurs when air escapes through the nose during the production of oral speech sounds, resulting in reduced intelligibility. This is a common characteristic in the speech and language profile because 69% of children have palatal abnormalities. If the structure of the soft palate velum is such that it does not stop the flow of air from going up to the nasal cavity, it will cause hypernasal speech. This phenomenon is referred as velopharyngeal inadequacy (VPI). Hearing loss can also contribute to increased hypernasality because children with hearing impairments can have difficulty self monitoring their oral speech output. The treatment options available for VPI include prosthesis and surgery. Difficulties acquiring vocabulary and formulating spoken language (expressive language deficits) at the onset of language development are also part of the speech and language profile associated with the 22q11.2 deletion. Vocabulary acquisition is often severely delayed for preschool-age children. In some recent studies, children had a severely limited vocabulary or were still not verbal at 2–3 years of age. School-age children do make progress with expressive language as they mature, but many continue to have delays and demonstrate difficulty when presented with language tasks such as verbally recalling narratives and producing longer and more complex sentences. Receptive language, which is the ability to comprehend, retain, or process spoken language, can also be impaired, although not usually with the same severity as expressive language impairments. Articulation errors are commonly present in children with DiGeorge syndrome. These errors include a limited phonemic (speech sound) inventory and the use of compensatory articulation strategies resulting in reduced intelligibility. The phonemic inventory typically produced consists of sounds made in the front or back of the oral cavity such as: /p/, /w/, /m/, /n/, and glottal stops. Sound made in the middle of the mouth are completely absent. Compensatory articulation errors made by this population of children include: glottal stops, nasal substitutions, pharyngeal fricatives, linguapalatal sibilants, reduced pressure on consonant sounds, or a combination of these symptoms. Of these errors, glottal stops have the highest frequency of occurrence. It is reasoned that a limited phonemic inventory and the use of compensatory articulation strategies is present due to the structural abnormalities of the palate. The speech impairments exhibited by this population are more severe during the younger ages and show a trend of gradual improvement as the child matures. DiGeorge syndrome is caused by a heterozygous deletion of part of the long arm (q) of chromosome 22, region 1, band 1, sub-band 2 (22q11.2). Approximately 80-90% of patients have a deletion of 3 Mb and 8% have a deletion of 1.5Mb. The number of genes affected by the deletion has been cited as approximately 30 to 50. Very rarely, patients with somewhat similar clinical features may have deletions on the short arm of chromosome 10. The disorder has an autosomal dominant inheritance pattern. A French study of 749 people diagnosed between 1995 and 2013 found that the mutation was inherited in 15% of patients, of which 85.5% was from the mother. Other studies have found inheritance rates of 6-10%. The majority cases are a result of a de novo (new to the family) deletion. This is because the 22q11 region has a structure that makes it highly prone to rearrangements during sperm or egg formation. The exact mechanism that causes all of the associated features of the syndrome is unknown. Of the 30–50 genes in the deleted region, a number have been identified as possibly playing a role in the development of some of the signs and symptoms. Haploinsufficiency of the TBX1 gene (T-box transcription factor TBX1) is thought to be the cause of some of the symptoms observed. Point mutations in this gene have also been observed in individuals with DiGeorge syndrome. TBX1 is part of the T-box family of genes which have an important role in tissue and organ formation during embryonic development and it may have a role in the regulation of differentiation of post migration neural crest cells. The neural crest forms many of the structures affected in DiGeorge syndrome, including the skull bones, mesenchyme of the face and palate, the outflow tract of the heart, and the thymus and parathyroid stroma. When there is a loss of expression of FGF18 during the development of the pharyngeal arches, neural crest cell death is seen. Although neither FGF18 or TBX1 are expressed in the neural crest cells, TBX1 might have a role in the regulation of FGF18 expression, ensuring that the differentiation of these cells in the pharyngeal region is correct. Therefore, dysfunction of TBX1 may be responsible for some of the symptoms in DiGeorge syndrome. Research in mouse models has shown that deletion of Tbx1 leads to several defects similar to those seen in humans, mainly affecting development of the great arteries and the thymus. The abnormalities seen in the great arteries of mice deficient of Tbx1 are a consequence of abnormal formation and remodelling of the aortic arches during early development. The role of Tbx1 for correct formation and remodelling of the aortic arches has been extensively studied in various mouse models suggesting the key role of Tbx1 for cardiovascular development and the phenotypes seen in DiGeorge syndrome. In mice, haploinsufficiency of the DGCR8 gene has been linked to improper regulation of the microRNA miR-338 and 22q11.2 deletion phenotypes. Transport and golgi organization 2 homolog (TANGO2) also known as chromosome 22 open reading frame 25 (C22orf25) is a protein that in humans is encoded by the TANGO2 gene. The gene coding for C22orf25 is located on chromosome 22 and the location q11.21, so it is often associated with 22q11.2 deletion syndrome. But with TANGO2 disorder being autosomal recessive, will not occur in all cases. Mutations in the TANGO2 gene may cause defects in mitochondrial β-oxidation and increased endoplasmic reticulum stress and a reduction in Golgi volume density. These mutations results in early onset hypoglycemia, hyperammonemia, rhabdomyolysis, cardiac arrhythmias, and encephalopathy that later develops into cognitive impairment. Parkinson's disease genes 22q11.2DS has been associated with a higher risk of early onset Parkinson's disease (PD). The neuropathology seen is similar to LRRK2-associated PD. None of the genes affected in individuals with 22q11.2DS have previously been linked to PD but there are a number that are likely candidates. These include DGCR8 which is important for biogenesis of brain microDNA, SRPT5 which encodes a protein that interacts with the PARK2 protein, COMT which is involved in regulating dopamine levels, and microRNA miR-185 which is thought to target known PD loci LRRK2. Diagnosis of DiGeorge syndrome can be difficult due to the number of potential symptoms and the variation in phenotypes between individuals. It is suspected in patients with one or more signs of the deletion. In these cases a diagnosis of 22q11.2DS is confirmed by observation of a deletion of part of the long arm (q) of chromosome 22, region 1, band 1, sub-band 2. Genetic analysis is normally performed using fluorescence in situ hybridization (FISH), which is able to detect microdeletions that standard karyotyping (e.g. G-banding) miss. Newer methods of analysis include Multiplex ligation-dependent probe amplification assay (MLPA) and quantitative polymerase chain reaction (qPCR), both of which can detect atypical deletions in 22q11.2 that are not detected by FISH. qPCR analysis is also quicker than FISH, which can have a turn around of 3 to 14 days. A 2008 study of a new high-definition MLPA probe developed to detect copy number variation at 37 points on chromosome 22q found it to be as reliable as FISH in detecting normal 22q11.2 deletions. It was also able to detect smaller atypical deletions that are easily missed using FISH. These factors, along with the lower expense and easier testing mean that this MLPA probe could replace FISH in clinical testing. Genetic testing using BACs-on-Beads has been successful in detecting deletions consistent with 22q11.2DS during prenatal testing. Array-comparative genomic hybridization (array-CGH) uses a large number of probes embossed in a chip to screen the entire genome for deletions or duplications. It can be used in post and pre-natal diagnosis of 22q11.2. Fewer than 5% of individuals with symptoms of DiGeorge syndrome have normal routine cytogenetic studies and negative FISH testing. In these cases, atypical deletions are the cause. Some cases of 22q11.2 deletion syndrome have defects in other chromosomes, notably a deletion in chromosome region 10p14. No cure is known for DiGeorge syndrome. Certain individual features are treatable using standard treatments. The key is to identify each of the associated features and manage each using the best available treatments. For example, in children, it is important that the immune problems are identified early, as special precautions are required regarding blood transfusion and immunization with live vaccines. Thymus transplantation can be used to address absence of the thymus in the rare, so-called "complete" DiGeorge syndrome. Bacterial infections are treated with antibiotics. Cardiac surgery is often required for congenital heart abnormalities. Hypoparathyroidism causing hypocalcaemia often requires lifelong vitamin D and calcium supplements. Specialty clinics that provide multi-system care allow for individuals with DiGeorge syndrome to be evaluated for all of their health needs and allow for careful monitoring of the patients. An example of this type of system is the 22q Deletion Clinic at SickKids Hospital in Toronto, Canada, which provides children with 22q11 deletion syndrome ongoing support, medical care and information from a team of health care workers. DiGeorge syndrome is estimated to affect between one in 2000 and one in 4000 live births. This estimate is based on major birth defects and may be an underestimate, because some individuals with the deletion have few symptoms and may not have been formally diagnosed. It is one of the most common causes of intellectual disability due to a genetic deletion syndrome. The number of people affected has been expected to rise because of multiple reasons: (1) surgical and medical advances, an increasing number of people are surviving heart defects associated with the syndrome. These individuals are in turn having children. The chances of a person with DiGeorge syndrome having an affected child is 50% for each pregnancy; (2) Parents who have affected children, but who were unaware of their own genetic conditions, are now being diagnosed as genetic testing become available; (3) Molecular genetics techniques such as FISH (fluorescence in situ hybridization) have limitations and have not been able to detect all 22q11.2 deletions. Newer technologies have been able to detect these atypical deletions. The signs and symptoms of DiGeorge syndrome are so varied that different groupings of its features were once regarded as separate conditions. These original classifications included velocardiofacial syndrome, Shprintzen syndrome, DiGeorge sequence/syndrome, Sedlackova syndrome, and conotruncal anomaly face syndrome. All are now understood to be presentations of a single syndrome. ICD-10 2015 version mentions DiGeorge syndrome using two codes: D82.1 (Di George syndrome) and Q93.81 (Velo-cardio-facial syndrome). The ICD-11 Beta Draft discusses the syndrome under “LD50.P1 CATCH 22 phenotype". However, since this syndrome is caused by the deletion of a small piece of chromosome 22, some recommend that the name "22q11.2 deletion syndrome (22q11.2DS)" be used. Some experts support changing the name of both DiGeorge and velocardiofacial syndromes to CATCH-22. The International 22q11.2 Foundation, through its "Same Name Campaign", advocates for the name 22q11.2 deletion syndrome. - 22q11.2 duplication syndrome - Asymmetric crying facies - Contiguous gene syndrome - List of radiographic findings associated with cutaneous conditions - Genetic counseling - Zellweger syndrome - CHARGE syndrome	2	16	0	0	0	2	0.922766	2	3,925
Key differences between ICD-9 and ICD-10? Many, many more codes. With a nearly fivefold increase from 14,000 diagnosis codes to over 69,000, and a nearly 19-fold increase from almost 4,000 procedure codes to almost 72,000, the transition from ICD-9 to ICD-10 can seem overwhelming. Specialties affected differently While many of the ICD-10 codes are built upon existing ICD-9 codes, some codes are significantly different than ICD-9. Similar diagnoses may have completely different codes. Specialties such as obstetrics, psychiatry, and emergency medicine, along with specialties that deal with musculoskeletal disease and injuries, will encounter disproportionately significant changes. ICD-10 codes will be longer to allow for greater specificity and for more flexibility to add new codes. ICD-10 has alphanumeric categories instead of numeric ones. The order of some chapters have changed, some titles have been renamed, and conditions have been grouped differently. \|Comparison of Diagnosis Code Sets\| \|3-5 Characters in length\|\|3-7 Characters in length\| \|First character may be alpha or numeric, characters 2-5 are numeric\|\|Character 1 is alpha; Characters 2 and 3 are numeric; characters 4-7 are alpha or numeric\| \|Less specificity\|\|Greater specificity\| \|Laterality not specified\|\|Laterality specified (e.g. left versus right)\| \|Limited space for new codes\|\|Flexibility to add new codes\| \|ICD-10-CM Code Structure\| \|Characters 1 through 3 – Category\| \|Characters 4 through 6 – Etiology, anatomic site, severity, or other clinical detail\| \|Character 7 – Extension\| \|ICD-10-CM Code Detail\| \|S52 Fracture of the forearm\| \|S52.3 Fracture of the shaft of the radius\| \|S52.32 Transverse fracture of the shaft of the radius\| \|S52.321 Displaced transverse fracture of the shaft of the right radius\| \|S52.321A Displaced transverse fracture of the shaft of the right radius,initial encounter for closed fracture\| How an EHR helps you The biggest worry providers have is that they or their coders will need to learn so many new ICD-10 codes and standards that they will make mistakes, resulting in claim denials. Incorrect coding can mean the difference between full reimbursement and no reimbursement. Cloud-based EHRs will fare better Medical professionals who use cloud-based EHRs and billing services should fare better during this transition. Since mappings from ICD-9 to ICD-10 are not one-to-one, electronic tools like EHRs help guide users to the appropriate ICD-10 diagnosis and, in some cases, automate the conversion from one code-system to another. This helps ensure that users are selecting the most specific, billable, ICD-10 code for a particular diagnosis category. Practice Fusion has the tools you need To this end, Practice Fusion aims to fully support your practice through this transition with tools that provide ICD-10 translation support, directly within your EHR workflows. We plan to release additional tools, well in advance of the ICD-10 deadline on October 1, 2015, to help you eliminate the need to memorize the differences or to hire a new billing service to handle the work of cross-walking your codes. These tools will walk you through the new ICD-10 coding process, so you can easily learn the codes as you work while avoiding disruption to your workflow. Think of it as an ICD-9/SNOMED to ICD-10 crosswalk. You will also need a billing partner who is also prepared for the ICD-10 transition. This will ensure that you get the proper notification for codes that may not be valid for billing. All of our preferred billing partners should be ICD-10 ready and prepared to provide you the notifications you need to ensure success in your EHR and in the billing software of your choice. Once this functionality is released, you will have the opportunity to test out your existing ICD-9 codes to make sure that you are identifying the correct ICD-10 code.	4	6	0	0	0	2	0.465506	2	900
Classification and external resources Guillain-Barré syndrome (GBS) (in French pronounced [ɡilɛ̃ baˈʁe], in English pronounced /ˈɡiːlæn ˈbɑreɪ/, /ɡiːˈæn bəˈreɪ/, etc. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings G00-G99 - Diseases of the Nervous system (G00-G09 Inflammatory diseases of the Central nervous system ( Bacterial meningitis The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books ) is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. Neuropathy is a medical term usually short for Peripheral neuropathy. The peripheral nervous system ( PNS) resides or extends outside the Central nervous system (CNS which consists of the Brain and Spinal cord. It is included in the wider group of peripheral neuropathies. Peripheral neuropathy is the term for damage to Nerves of the Peripheral nervous system, which may be caused either by diseases of the Nerve or from the There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, acute inflammatory demyelinating polyneuropathy (AIDP). It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, the majority of patients will regain full functional capacity. Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily However, death may occur if severe pulmonary complications and dysautonomia are present. Dysautonomia is any disease or malfunction of the Autonomic nervous system. All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents or vaccines) but mistargeted to host nerve tissues instead (a form of antigenic mimicry). Molecular mimicry is defined as the theoretical possibility that sequence similarities between foreign and self-peptides are sufficient enough to result in the cross-activation of autoreactive The targets of such immune attack are thought to be gangliosides, which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the nodes of Ranvier. Ganglioside is a compound composed of a Glycosphingolipid ( Ceramide and Oligosaccharide) with one or more Sialic acids (AKA N-acetylneuraminic Nodes of Ranvier are known as the gaps (about 1 micrometer in diameter formed between myelin sheath cells along axons or nerve fibers An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those preceded by Campylobacter jejuni infections. Campylobacter jejuni is a Species of curved Rod-shaped, non-spore forming Gram-negative Microaerophilic, Bacteria Another example is the GQ1b ganglioside, which is the target in the Miller Fisher syndrome variant (see below). The end result of such autoimmune attack on the peripheral nerves is inflammation of myelin and conduction block, leading to a muscle paralysis that may be accompanied by sensory or autonomic disturbances. Autoimmunity is the failure of an organism to recognize its own constituent parts as self, which results in an immune response against its own cells and tissues Myelin is an electrically-insulating Dielectric Phospholipid layer that surrounds only the Axons of many Neurons It is an outgrowth Paralysed redirects here For other uses see xx Paralysed (disambiguation Paralysis is the complete loss of Muscle function However, in mild cases, axonal function remains intact and recovery can be rapid if remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see below), axonal degeneration occurs, and recovery depends on axonal regeneration. Recovery becomes much slower, and there is a greater degree of residual damage. Recent studies on the disease have demonstrated that approximately 80% of the patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of the disease is indeed axon loss. Myelin is an electrically-insulating Dielectric Phospholipid layer that surrounds only the Axons of many Neurons It is an outgrowth An axon or nerve fiber is a long slender projectionof a nerve cell or Neuron, that conducts electrical impulses away from the neuron's Cell The disease is characterized by weakness which affects the lower limbs first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without dysesthesias (numbness or tingling). Dysesthesia is defined as an unpleasant abnormal sensation It is caused by lesions of the nervous system peripheral or central and it involves abnormal sensations whether spontaneous As the weakness progresses upward, usually over periods of hours to days, the arms and facial muscles also become affected. Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, (oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or maintaining an open airway) and respiratory difficulties. Cranial nerves are Nerves that emerge directly from the Brain stem in contrast to Spinal nerves which emerge from segments of the Spinal cord. Most patients require hospitalization and about 30% require ventilatory assistance. Facial weakness is also commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant (see below. ) Sensory loss, if present, usually takes the form of loss of proprioception (position sense) and areflexia (complete loss of deep tendon reflexes), an important feature of GBS. Proprioception (ˌproʊpriːəˈsɛpʃən PRO -pree-o-SEP-shun from Latin proprius, meaning "one's own" and perception is the Sense Loss of pain and temperature sensation is usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain usually in the weakened muscles, which patients compare to the pain from overexercising. These pains are self-limited and should be treated with standard analgesics. Bladder dysfunction may occur in severe cases but should be transient. If severe, spinal cord disease should be suspected. Fever should not be present, and if it is, another cause should be suspected. In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension, and cardiac arrhythmias. Orthostatic hypotension (also known as postural hypotension, and colloquially as head rush or a dizzy spell) is a form of Hypotension in which The symptoms are similar to those for progressive inflammatory neuropathy. Progressive inflammatory neuropathy ( PIN) is a Disease that was identified in a report released on January 31 2008 by the Centers for Disease Control and Prevention Although ascending paralysis is the most common form of spread in GBS, other variants also exist. The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. CSF and ECD is used almost every time to verify symptoms, but because of the acute nature of the disease, they may not become abnormal until after the first week of onset of signs and symptoms. Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Botulism ( Latin, botulus, "sausage" is a rare but serious Paralytic illness caused by Botulin Toxin. Diphtheria ( Greek διφθερα ( diphthera)—“pair of leather scrolls" is an upper respiratory tract illness characterized by sore Lyme disease, or borreliosis, is an Emerging infectious disease caused by at least three Species of Bacteria belonging to the Genus Porphyrias are a group of inherited or acquired disorders of certain Enzymes in the Heme biosynthetic pathway (also called Porphyrin pathway Poliomyelitis, often called polio or infantile paralysis, is an acute viral Infectious disease spread from person to person primarily via Myasthenia gravis (literally "serious muscle-weakness" from Greek μύς "muscle" "weakness" and Latin gravis An organophosphate (sometimes abbreviated OP) is the general name for Esters of Phosphoric acid. Poison hemlock redirects here For other uses of "hemlock" see Hemlock. Thallium (ˈθæliəm is a Chemical element with the symbol Tl and Atomic number 81 Arsenic (ˈɑrsənɪk is a Chemical element that has the symbol As and Atomic number of 33 Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early intubation should be considered in any patient with a vital capacity (VC) <20 ml/kg, a Negative Inspiratory Force (NIF) <-25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disease, or autonomic instability. In Medicine, intubation refers to the placement of a tube into an external or internal orifice of the body Vital capacity is the maximum volume of air that a person can exhale after maximum Inhalation. Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose intravenous immunoglobulins (IVIg) at 400mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone. Antibodies (also known as immunoglobulins, abbreviated Ig) are Gamma globulin Proteins that are found in Blood or other Bodily Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood Therapy is no longer effective after 2 weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (. 4kg each day). The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Glucocorticoids have NOT been found to be effective in GBS. Glucocorticoids (GC are a class of Steroid hormones characterised by an ability to bind with the glucocorticoid receptor ( GR) and trigger similar effects If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) is administered four times over a week. Following the acute phase, the patient may also need rehabilitation to regain lost functions. This treatment will focus on improving ADL (activities of daily living) functions such as brushing teeth, washing and getting dressed. Activities of daily living ( ADLs) are "the things we normally do in daily living including any daily activity we perform for self-care (such as feeding ourselves bathing Depending on the local structuring on health care, there will be established a team of different therapists and nurses according to patient needs. An occupational therapist can offer equipment (such as wheel chair and cutlery) to help the patient achieve ADL independence. A physiotherapist would plan a progressive training programme, and guide the patient to correct, functional movement, avoiding harmful compensations which might have a negative effect in the long run. A Speech and Language Therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a tracheostomy. The Speech and Language Therapist would also offer advice to the medical team regarding the swallowing abilities of the patient and would help the patient regain their communication ability pre- Dysarthria. There would also be a doctor,nurse and other team members involved depending on the needs of the patient. This team contribute with their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient's own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed. Most of the time recovery starts after 4th week from the onset of the disease. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5-10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disease and despite all improvements in treatment and supportive care, the death rate among patients with this disease is still about 2-3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting 4 to 6 weeks, and in some cases up to 1 year, when a ventilator is needed in the worst cases. About 5-10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP). Chronic Inflammatory Demyelinating Polyneuropathy (CIDP is an acquired immune-mediated inflammatory disorder of the Peripheral nervous system but often can have The quickest known recovery from GBS was Robert Bernatz of Newport Beach, CA who in 1998 was released from a Hoag Memorial Hospital in just ten days from the onset of his symptoms and was running six miles at the end of one month. His primary treatment was the relatively new process of plasmapheresis. Plasmapheresis (from the Greek plasma, something molded and apheresis, taking away is the removal treatment and return of (components of Blood The disease was first described by the French physician Jean Landry in 1859. This article is about the country For a topic outline on this subject see List of basic France topics. Jean Baptiste Octave Landry de Thézillat (b October 10, 1826 &ndash d Year 1859 ( MDCCCLIX) was a Common year starting on Saturday (link will display the full calendar of the Gregorian calendar (or a Common In 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count. Year 1916 ( MCMXVI) was a Leap year starting on Saturday (link will display the full calendar of the Gregorian calendar (or a Leap year Georges Charles Guillain ( March 3, 1876 - June 29, 1961) was a French Neurologist. Jean Alexandre Barré ( May 25, 1880 – April 26, 1967) French Neurologist, worked in 1916 on the identification of the André Strohl ( March 20, 1887 - March 10, 1977) was a French Physiologist who was a native of Poitiers. GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio and Landry's ascending paralysis. The National Institute of Neurological Disorders and Stroke is a part of the U GPnotebook is a British medical database for General practitioners (GPs	2	15	0	0	0	2	0.767556	2	3,607
Socioculturalism and Health Throughout the ICDs that can be evaluated are those of a sociocultural nature. Any study of socioculturalism and disease is complex due to the subjectivity of how this field was initially developed and since then has been subsequently re-defined and re-adapted several times. One of the major outcomes of this controversy are questions regarding lack of objectivity. The development of DSM and ICD systems has been somewhat culturally-based. This has prevented these methods of coding diseases to be effective when it comes to relying upon ICD-9 codes to identify culturally-bound syndromes. Like the nosologies of medicine during the 19th century, the current taxonomy (a more detailed and binary logic-based nosology) has relied upon an ever changing flowchart to define each and every diagnosis. A physician familiar with this system for coding disease is familiar more with the global or western philosophy of disease, not always any true cause for certain diseases. For this reason, the new ICD-10 about to be implemented partially corrects for many of these failures produced by the ICD-9. As multiculturalism becomes more respected socially, so too does the need for better understanding any other cultural traditions and beliefs that make a disease develop. Some diseases are purely cultural in that they are psychosomatic in nature and a consequence of the belief system the patient has towards his/her disease. This may even result in new labels given to the disease, as well as new names, new expectations, and even new responsibilities. In one social setting, a child with Tourette’s Syndrome is considered to have some sort of age-link tic-related disorder, and yet in another considered to be chanelling with extracoporeal or past spirits, and in another a victim of loss of soul followed by replacement of a past soul. The old ICD-9 system has just a few ways to define this condition, noting it with neurological and psychiatric and perhaps psychological, psychosomatic ICDs. The newer system has a more detailed cultural definition for the original state and its various components or axes, ranging from direct to indirect physiological and non-physiological linkages to other conditions or behaviors. The newer ICD system manages to provide a way to identify certain cultural differences that exist in disease progression. But it doesn’t necessarily change the interpretation of disease at all. Instead, it provides us with a way to define why some ICDs which have been assigned to a medical condition fail to meet the age-gender distribution features of that particular psychiatric, mental, psychosomatic, or physical disease. This review of population pyramids demonstrates how the current ICD system still misses the boat often when it comes to exactness in details. The new ICD assignments for culturally bound syndromes still lack in demonstrating any direct age-gender link to the traditional ICD and the actual appearance of a particular medical condition. In addition, this new system still lacks some of the associations being made betwen particular conditions and the possible application of the terms “culturally-bound” to these Western Medicine, Developed Country related medical conditions or diseases. A disease like anorexia nervosa is in some ways just as culturally bound as a possession of soul believed in by Hmong parents with children who demonstrate a history of epilepsy. For this reason, we have to dissect “culturally-bound” diseases further. These sociocultural-linked diseases are of several forms. Some are completely physical in nature, meaning they are physical changes and manifestations of a health condition in the body that is due completely to heredity. There are also physical diseases that develop due to cultural behaviors, and are often related to morees and taboos such as restrictions in diet, restrictions in physical social engagements (sports, groups meeting behaviors, religious practices involving interperson contact and the like), restrictions in how we must behave in terms of selecting spouses and marriages. Thirdly, there are behavioral psychological and biopsychological (even biological and psychiatric related) conditions that at times have an important physical science component, for example schizophrenia is an inherited trait common to the German speaking Amish communities. Finally, there are those primarily sociocultural or simply socially defined diseases, which are behavioral and psychological problems defined as such by the traditional culture, such as the Amok suicidal problem experienced by South Asian communities, or the unique stress syndromes many cultures have due to moving to a new setting. Culturally-linked diseases excluded from this review are those with an ecological component to their social, regional or even ethnic history. Examples of conditions excluded from this review include some very biological sociocultural-linked conditions also common to Western Society, but with different incidence rates, such as - numerous zoonotic pathogens, for example - intestinal organisms like the African tapeworm (Taenia solis) , - the Mexican-borne version of Syphilis strain resulting in pinta - the unique occupational disease Chiclero’s Ear. - “Old World” versus “New World” STDs, due to the controversy over the true origins of certain STDs during the colonial era - Recreational or religious natural products use, including cocaine leaf chewing, the Kat of Somalia, Betel Leaf consumption, India’s Hashish, Island Community nutmeg- or salap-related consumption, etc. Those most interesting diseases, conditions, or states to review are those which have distinct cultural definitions. In many cases these are defined as “diseases” or maladies only due to the way in which the Western Physician making use of these ICD systems defines a lack of normality, and subsequently identifies someone as “not normal”. These conditions are atypical to Western Culture, and at times could be likened to someone claiming a pattient is sick due to some misbehaved chakra or acupuncture point, reasoning not at all different from the “hot spots” or sensitive points philosophy used to explain something as western medicine-defined as fibromyalgia. The result of this philosophical approach used in western medicine by way of certain portions of the ICD system is the creation of certain conditions or diseases that are assumed to be proper and in need of such identification. The most commonly known results of such subjectivity and individual beliefs in medicine are those ICDs used, once used, or considered for the explanation in such human conditions and behaviors as the “Psychosomatically-induced” Irritable Bowel Syndrome identified as such by the 1950s, or the reason an individual becomes a criminal, or engages in unique sexual acts. Each of these has past and present pathological definitions attached to them, some considered solely psychiatrc, others considered biological, still others behavioral and as a result of “social detachment.” Culturally-bound syndromes change in definition over time. Most of the currently accepted syndromes for this category have some unique traits that define them as not a consequence of what western culture defines to be illness-inducing conditions or behaviors. The first list of these syndromes which follows are for ICD defined states that are very much subjective in their representation, causology and health-related outcomes. A certain number of these ICD-defined states or conditions are cultural in nature due to the rarity of what they represent, and the decision made by the medical field in general to categorize these features to just a particular group. The ICD 10 or DSM-IV-TR recognizes the following examples of these syndromes which are most closely linked to the contemporary definition for a “culturally bound” syndrome. - Amok. Malaysia. Periods of “homicidal frenzy”, brooding, and finally somnolence and amnesia. Afflicts mostly young to middle-aged males living away from home (college students). Often ends in suicide. ICD 10 code = F68.8 Other personality disorders of adult personality and behavior. Associated with Lin-Keh-Ming and hwa-byung of Korea. Other similar culture bound syndromes are perhaps: colerina-Andes Bolivia, Columbia, Ecuador, Peru, iich’aa Southwestern US Indigena, berserkergang-Scandinavia (Saami), cafard-Polynesia, benzi mazarazura- south African Shona, and ahad idze be-New Guinea. - Ataque de Nervios. Puerto Ricans and certain other Latinos/as. Uncontrollable shouting, trembling, heart palpitations, sense of heat rushing to the head, fainting and seizure like activities, temporary loss of consciousness, purported amnesia. Afflicts socially disadvantages women > 45 yo with no high school education. See “Nerves.” - Brain Fag. West Africa. Sometimes equated with Chinese equivalent neurasthenia, Korean hwa-byung, or Latino susta. A form of depression, anxiety or somatoform condition. - Dhat. India, China. Also called dhatu, jiryan, shen k’uei, and shen-kui. Characterized by anxiety, fatigue, somatic pains, “loss of energy”. Philosophically felt to be linked to semen loss, and so due to excess semen loss, leading to imbalances in the body’s five elements/four humours, made worse by bad diet. According to Chinese medical philosophy, the traditional mechanism for developing this condition is excess coitus, with chi loss incurred and loss of the spiritual “energy” or parts involved with making chi. The shen is akin to the essence used to produce the ‘vital force’ of late 18th and early 19th century western medical philosophy. Shen is “the sign of life in someone’s eyes”; loss of shen preceding death results in a very dark appearance of the inner orbit as seen through the pupil. As the Chinese names imply, disturbance and removal of this shen ensues (shen-kui). ICD 10 assignment: F48.8 Other specific Neurotic Disorder, or F45.34 Somatoform autonomic dysfunction of the genitourinary system (this may include autonomic anxiety symptomatology). Western medical collquial terms identify this as a sexual neurosis. - Hwa-byung. Korea. “Firey illness”. Anger or rage manifesting with pressure on the chest, movement of heat about the body, indigestion, fatigue, headache, and a variety of mood changes. Women are perhaps afflicted more than men. Philosophically this syndrome blamed on the oppression of Korean culture. - Koro. Hainan island of China, India, Singapore, Thailand. From “koro” for turtle. Anxiety and panic resulting in or are caused by a fear that the penis is retracting (like a tortoise head) into the abdomen. For women, breasts and genitalia are thought to contract. Excess coitus, exposure to cold, illness induce the signs (related appearances). Devices are used to prevent the retraction. Cantonese refer to this as suk-yeong, Mandarins as suo-yang, Assams as jinjinia bamar, Thai as rok-joo, Eastern Indians as rabt. ICD 10 assignment: F48.8 Other specific Neurotic Disorder, or F45.34 Somatoform autonomic dysfunction of the genitourinary system (this may include autonomic anxiety symptomatology). ICD9 - any combination of: Psychosexual dysfunction (302.7), Impotence/Frigidity (302.72), Anorgasmia (302.73, 302.74) - Latah. Malays. Startle or fright reflex responses characterized by exageration, such as overesponse to stimuli (recall autonomics and the fight or flight responses in relation to fright). Echopraxia, echolalia, and command obedience behaviors can ensue. Patient seems trancelike or even dissociative. Most often associated with women of low economic status. Similar syndromes in other cultures include amurakh (Siberia), iekunii, ikota, okan, myriachit, menkeiti, Lapp panic (Lapps), bachtschhi (Thailand), imu (Ainu indigena of Japan), mali-mali (Philippines), silok, susto (Mexico), yuan (Myanmar, formerly Burma), jumping (French Canada). ICD 10 – F48.8 Other Specified Neurotic Disorders, F48.88 Other Specified Dissociative [Conversion] disorders. - “Nerves.” Nerfiza (Egypt), Nevra (Greece), nervios (Mexico, Central and South America), nerves (northern Europe). Sorrow or anxiety, leading to head and muscle pain, reactivity, nausea, appetite loss, insomnia, fatigue, agitation. Linked to stress and low self-esteem. Women experience this more than men. ICD 10 F32.11 Moderate Depressive Episode with somatic syndrome, F48.0 Neurasthenia, F 45.1 Undifferentiated Somatoform Disorder. Related syndromes include: anfechtung (Hutterites), brain fag (Nigeria), colerina, pension and bilis (Mexico, Central America, South America), hsieh-bing or xie -bing (China), hwa-byung (Korean peninsula), qissaatuq (Inuits in Arctic territories), narahati-e a sa or maraz-e a-sab (Islamic Republic of Iran). - Pa-leng. China, esp. Taiwan, parts of southeast Asia. A frigiphobia, or fear of the cold or winds, due to association of these natural features with fatigue, impotence and death. Belief in the humoral theory for illness is an important part of these cultures. Fear is accompanied by onset of anxiety and obsessiveness. ICD 10- F40.2 Specific Phobias. Mexico and Central and South America have the related disorders agua frio, aire frio and frio. - Pibloktoq. Arctic and Subarctic Inuit. Arctic hysteria. Primarily associated with women. Preceded by tiredness, lack of facial expression, social extreme quietness, confusions. Events begin as “extreme excitement”, followed by convulsions and pseudo-coma like states. Peak attacks demonstrate adrenalin-like effects including significant strength and abberant physical and verbal behaviors. Indicative actions include removal of clothing, fleeing, jumping in water, rolling in snow, mimetic acts, choreiform movement, glossolalia, and coprophagia. Events end with fatigue, sleep and return to typical states following reawakening (see ref1., ref2; ICD 10 possibly: 1) F44.7 Mixed Dissociative (Conversion) Disorder or 2) F44.88 Other specific dissociative (conversion) disorder. Related to amok (Indonesia, Malaysia), banga or misala (Congo, Malawi, formerly Nyasaland), ebenzi (southern Africa, esp., Shona and affiliates), grisi siknis (Miskito, Honduran Indigena), imu (Ainu, indigena of Japan), latah (Indonesia, Malaysia), mali-mali (Philippines), nangiarpok, kayak angst, quajimaillituq (Inuit), ufufuyane (southern Africa, esp. Bantu, Zulu and affilated groups). Blood and physiological biochemical findings have led to numerous theories, including Vitamin A overdose. Episodes last for several minutes, and may have some relation to hypocalcemia-induced tetany cases. Interpersonal anxieities and cultural stressors play an important role, along with the meaning and purpose of this event. - Shenjing Shuairuo. China. Sense of mental and physical exhaustion, memory loss, inability to concentrate, irritability, headaches, weakening of nervous activities or nervous system (“neurasthenia”). - Susto. Also called Espanto. Mexico, Central and South America. “Soul loss” induced by the supernatural. Chief emotional result is fright, with minimal traumatic events physically experienced. Symptoms include agitation, anorexia, insomnia, fever, diarrhea, mental confusion, apathy, depression, introversion. Hypoglycemic states related to some events. Anxiety due to social stressors is a major contributor. Related to grief-loss events in life. ICD 10 is F45.1 Undifferentiated somatoform disorders, or F48.8 Other Specified Neurotic Disorders. Related to lanti (Philippines), latah (Indonesia, Malaysia), malgri (Australian aborigines), mogo laya (New Guinea), narahati (Islamic Republic of Iran), and saladera (Amazonian region). - Taijin Kyofusho. Japan. Other names: shinkeishitshu, or anthropophobia (fear of people, people interactions, and contracting disease). Social phobia about how one appears of presents himself/herself, behaviorally and somatically. Avoidance of close contacts may result. Feelings of embarassment, blushing; concerns that appearance, facial expressions, smell, words stated, might cause social unrest. Mostly afflicts younger people. Perfectionism may be a behavioral feature that results, with cultural influences upon younger populations resulting in “over-socilization” accompanied by perfectionism and then followed by breakage of social bonds due to feelings of inferiority and anxiety about personal presentation. ICD 10 – F40 Social Phobias, or F40.8 Other Phobic Anxiety Disorders. Other conditions with similar presentation: anfechtung (Hutterites), and itiju (Nigeria). - Ufufuyane. South Africa, esp. Bantu, Zulu and allies, and Kenya. Also referred to as saka. Anxiety state attributed to magic potions administered by rejected lovers, or due to spirit possession. Akin to voodoo (voodu) beliefs in part. Shouting, sobbing, paralysis, convulsions, trance-like stupor and loss of consciouness is associated with these spells. Young unmarried women are most often afflicted. Can relate to nightmares and night horrors with sexual themes, somnambulism, temporary blindness. Attacks last from days to weeks, and have a psychosomatic onset triggered by the sight of certain men or foreigners. ICD 10 codes are F44.3 Trance and possession disorders, or F44.7 Mixed dissociative [conversion] disorders. Other syndromes related to this include aluro (Nigeria), phii pob (Thailand), and zar (Egypt, Ethiopia and Sudan). - Uqamairineq. Inuits in the Arctic region. Paralysis of sudden onset with borderline sleep states; anxiety, agitation and hallucinations are related. Prodromal indicators are noted such as transient sound or smell (taste as well?). Chronic disease, resulting in attacks that last for several minutes at a time. Syndrome is related to “soul loss” experiences, “soul wandering” or spiritual possession. Dissociative hysteria cases have been realted to this, along with narcolepsy-catalepsy syndromes. ICD 10 is F44.88 Other Specific Dissociative [Conversion] Disorders and G47.4 Narcolepsy and Catalepsy (including sleep paralysis). Related to aluro (Nigeria), old hag (Newfoundland), and phii pob (Thailand). - Windigo. Northeastern Indigena of North America. Traditionally considered a form of psychosis. Rare cases have cannibalistic obsession. Traditional beliefs blame this on possession, usually of males, turning them into “cannibal monsters.” Depression, homicidal or suicidal thinking, and delusions are common, The ‘wish to eat human flesh’ is an indicator. Typical of people ostracized by their immediate peers and/or society. Chronic food shortages and cultural myths support the belief system leading to these states. Some studies suggest this medical illness is a made up syndrome meant to ostracise or execute governmental changes. ICD 10 suggested is F68.8 Other Specific disorders of adult personality and behavior. Related to amok (Malaysia), hseih-ping (China, esp. Taiwan), and zar (Egypt, Ethiopia and Sudan). The new ICD 10s for the above states are from the WHO ICD-10 classification of mental and behavioural disorders (see also Hirsch and Weinberger’s (eds.) Schizophrenia (2003)); a partial list of possible ICD9s appear in brackets. These diagnoses are as follows. - F32.11 Moderate Depressive Episode with somatic syndrome [296.2-3?] – Ataque de Nervios, Nerves - F40 Social Phobias [300.23] – Taijin Kyofusho - F40.2 Specific Phobias [300.2] – Pa-leng - F40.8 Other Phobic Anxiety Disorders [300.2, 300.20] – Taijin Kyofusho - F44.3 Trance and possession disorders – Possession Disorder, Ufufuyane - F44.7 Mixed dissociative [conversion] disorders [ICD9 300.1, 300.11] – Pibloktoq, Possession Disorder, Ufufuyane - F48.8 Other specific Neurotic Disorder – Dhat, Koro - F44.88 Other specific dissociative (conversion) disorder [ICD9 300.1, 300.11] – Latah, Pibloktoq, Uqamairineq - F45.1 Undifferentiated somatoform disorders [308.?] – Nerves, Susto - F45.34 Somatoform autonomic dysfunction of the genitourinary system (this may include autonomic anxiety symptomatology) [306?] – Dhat, Koro - F48.0 Neurasthenia – Nerves - F48.8 Other Specified Neurotic Disorders – Susto - F68.8 Other Specific disorders of adult personality and behavior [301.5?, 301.50?] – Amok, Windigo - G47.4 Narcolepsy and Catalepsy (including sleep paralysis) [306.0] – Uqamairineq Some of the ICD graphs for these syndromes are as follows. Dissociative States, Bipolar Disorders, Delusional Behavior, Narcolepsy/Catalepsy, and Social Phobia are several ICDs related to Sociocultural Syndromes in recent decades. Mixed Cultural and Multicultural Perspectives Cultural bound syndromes represent a set of conditions in which socioculturalism and western medical philosophy come face to face as people and doctors, both traditional and modern, try to understand the cause for an “illness” and the most effective remedial process. ”Modern” medicine also bears beliefs about diseases, some which are centered on its own culture. How people with specific illnesses are treated plays a very important role in their long term progress. In the early 1800s, people with specific conditions such as epilepsy were treated like criminals and housed in jails or prisons. A few decades into the 19th century these legal practices were diminished or ceased due to the work of various humanitarian groups. In spite of these successes, however, public and professional views about certain diseases continue to exist thereby influencing greatly the life and future of people with such diagnoses. When western medical thought prevails over alternative philosophies for a disease, and as a result reduces the quality of life for these people, we have to question sometimes the values of such practices. Western societies and western medicine for example treat elders much differently than many developing countries. - Epilepsy. Multicultural. For example “Spirit Captures you and you fall down” of the Hmong. (see 1997 book with same title). ICD 9 = 345.* - Schizophrenia. For example Amish. - Possession Disorder. Multicultural. Individual sense his/her body is taken over by another entity. Disturbance of consciousness, identity or memory, changes in voice patterns or speech, loss of awareness of surroundings, with a high tolerance for pain and other environmental stimuli. ICD 10 codes are F44.3 Trance and possession disorders, or F44.7 Mixed dissociative [conversion] disorders. Relates closely to fuufuyane or saka. “Modern” medicine isn’t exact. In fact it is filled with medical conditions and ailments that could more accurately be defined as the “best fit” for the present time. This inherent trait of disease classification systems begins with the premise that the individual who defines the diseases in such a way is correct, even if his philosophy differs from others in the field. Nosology of disease is a specialty that was developed in the late 18th century, modified in the first half of the 19th century, modified and slightly modernized following the discovery of the bacterium and pharmaceutical means for disposing of pathologies during the early 1900s, and finally developed into a system of classification much like that of today some time in the 1960s or 1970s. This late 20th century nosology is not at all “perfected”. Even with this method of classifying disease, we are left with the culturally accepted assumption that the bifurcating system used to produce this methodology is very approporiate and most correct, and that the links drawn are “correct”. This subjectivity of human classification methods is what adds ‘cultural bias errors’ back into the classification system each time we cannot define the complete and total reasons for a particular medical condition existing. Examples of how these types of self-created, self or ethno-centered ideologies played out in the past are demonstrated by the histories of past diseases, such as the following: - The “Divine Somnambulism” of 1800 - The “Mania” demonstrated by Jesus, a diagnosis published by French physicians ca. 1830-1840 - Agua frio, or Aire frio, an illness still experienced by some Mexican nationals today - Iich’aa (southwestern US) and the native American philosophy - Hypoglycemia and fasting, to MDs a concerning health condition, to others a religious experience and an important part of shamanics - Temporary Blindness - a reason for the miracle cure of blindness? - Nightmares/Night Horrors and “having visions” - Tics and Tourette’s Syndrome, in the past a manifestation of nerves and nervous energy; today the consequence of personal growth and psychology, or to some, adolescent/teenage neurochemicals One of the more interesting issues is cultural medicine today pertains to these “health practices”, reactions to stress and change, and “illnesses” experienced by patients of the United States medical care system. Equally interesting is the attraction we have to certain historical tales about these illnesses and very strange activities. Porphyrism for example was used to claims possible truths for and assign meaning to the 14th century story of vampires of Eastern Europe. Bread tainted with ergot is used to explain why towns might have had a history of being possessed by evil spirits. Epilepsy is used to define the signs of demonic possession. The following culturally bound syndromes or behaviors important to US culture are reviewed in detail. These are examples of physicians in the current US paradigm trying to make other culturally defined interpretations of illness fit into their perspectives on health, behavior and disease. African Infibulation [ICD9 729.2] Demonic Possession and the like Genetic Blood Dyscrasias African Infibulation [ICD9 729.2] When specific cultures migrate into the United States, there are medical beliefs and syndromes that accompany these migrations. In the case of in-migration of certain parts of the African tradition, a commonly accepted practice in parts of Africa is female genital surgical manipulation or change in the form of one or more of the following surgical procedures: clitorectomy, labiectomy, and infibulation. The end result is the removal of female genital parts and closure of the vaginal passage, symbolizing a young female’s passage into her menstruation stage in life. These surgeries, according to cultural beliefs, “prepare her” for later marriage in a virginal state; it symbolized her “promise” of not engaging in any premarital promiscuity. These cultural practices are not accepted as humane practices by most “Western” and United States practitioners. In recent years, this sensitive sociocultural rite of passage related “health care method” has undergone intense professional scrutiny, legally as well as medically. The procedure and end result of this surgical practice are a part of ICD9 classification system, enabling this review of age-gender patterns to be completed. The ICD9s for these processes are as follows: - 729.2 – all of the below combined - 729.20 – unspecified mutilation process - 729.21 – involving a clitorectomy only - 729.22 - clitoris and labia removal or modification - 729.23 - clitoris and labia modification along with an infibulation process - 729.29 – all others The surgical processes typical of certain African societies includes a clitorectomy, labial removal and closure process (infibulation). Different ICDs are provided based on the degree to which this surgical procedures completes all three of these tasks. Although infibulation and its precursors are extremely rare events, rarely noted in the medical records along with use of the appropriate ICDs, these processes are found in exceptionally large population studies (>50M). They therefore provide us with some insight into the very poorly studied age-specific sociocultural behavior problem. The above age-gender curves of two of these processes demonstrate the possibility of 3 age ranges of distribution for this diagnosis. These three peaks are defined as follows, based upon the history and location for the performance of this procedure: - a) 70-100 yo-primarily individual who immigrated into this country with a history of experiencing this procedure, - b) individuals between 25 and 50 years of age, with peaks at 25-30 and 40-50 yo, with the possibility that the older age group received this procedure in their native lands before immigrating to the United States, or as a result of social pressure after their arrival into this country, and - c) individuals < 15 years of age, who are most likely experiencing this process either just before a recent migration into this country, or who have received this process as a result of cultural pressure to perform this act while residing in the United States. The following symbol is a link to more on this topic. Psychiatric or Culturally-bound? Demons and Physicians Demonic possession, hyperreligiosity, group hysteria, and pibloktoq or arctic hysteria are all psychological changes in individuals and people which physicians have historically tried to associate with personality disorders, and sometimes even biochemical disorders. This “branding” behavior (“the scarlet letter”) in medicine is especially true with certain disease types. History has allowed us to imagine new diagnoses for people who never seemed to fit in. This is because society forms its definitions of medical conditions or psychological states and then tries to place that person in some sort of category that is assigned based opn the most socially accepted profiles of these individuals. During the Salem witchcraft trials for example, young women with schizophrenia, dissociative disorder, certain forms of epilepsy, tics or tourette’s syndrome, epilepsy, or even some sort of ergot poisoning had the potential of ending up in the next farm pond dead from total immersion in water. A half century later the same events recurring briefly in the Hudson Valley around 1720 led a local elderly women to be accused of practicing the same sort of dissociative behaviors accompanied due to her poor social practices and her non-traditional religious upbringing. Likewise several decades later, around the turn of the century, an old woman near North Salem was accused of many things due to her loner style of living along the mountain face facing New York; but because she attended church on a regular basis she never received the punishment that society could have given her had she been labelled a witch by the local children. In the lower part of the Hudson Valley another young girl was suffering from a unique form of mental illness, one that made her pseudo-hallucinate whilst laying in bed, as if she were asleep. During her “events” she would recite bible passages and appear to be communicating with spirits from above. This behavior was an offshoot of an increasingly popular behavior Emanuel Swedenborg demonstrate just a could of decades earlier–whilst talking with angles visiting each of his body parts. Swedenborg’s schizophrenia was socially defined to be a consequence of his skills as a seer, and was supported more as time passed due to peoples’ curiosities about the metaphysical world. What Swedenborg then introduced to the United States by the end of the 18th century, a spirit seeker like Rachel brought into the next stage of social behaviors with metaphysics. About the same time, when the spoons started flying in Thorn house down in Fishkill, the daughter of this family was considered to be communicating with another spirit floating about in the vicinity, someone whose spirit was later found to be locked within the Thorn’s home attack in the form of an old stone that was finally discarded much later, as the daughter’s demonic possession also decided to leave this home behind. Further north in New York State, we see this same story re-juvenated such a following when the daughters of a farming family began spirit rapping with past spirits, thus making popular once again the possibility of channeling with old time family members, making your own necessary personal connections with the matron or patron of several generations back. All of these practices require some sort of imagination and belief to become active and effective in influencing how society looked upon such conditions as diseases or disasters for someone’s spirit, mind and personality. Many of the illnesses of this form are mostly define by cultural means. It is the beliefs of people that define whether or not a particular practice is good or bad, or can be assigned some name indiciating it is a curse or a gift. Even today, culture defines not only the type of condition a person has–good or bad–by which ICDs are assigned to a particular behavior or condition, but modern medicine is also guilty of mislabelling and misclassifying some of these conditions. Medicine in general limits its ability to categorize someone along a certain pre-approved dischotomous path of diagnostics when various unique culturally defined syndromes have to be classified using the ICD system. It is possible that the majority of the diagnoses developed using this method might even be misdiagnoses, or conditions redefined and erroneously rewritten in other words in order to best fit the category the diagnostician wishes to place someone’s behavior in. How does a regular physician define a shaman for example? Is he simply a delusional very old man? a child who had hyperreligiosity brought about due to temporal lobe epilepsy? someone with so much grandiosity and incongruent argumentativeness that he must be a schizophrenic? Regular medicine cannot answer these questions effectively, in the past, now or even in the future. Cultural beliefs and practice such as those associated with “demons” and religion are not terms that easily found in ICD books. It is even much less possible to find mention of the words like shaman, pibloktoq, chi, vital force, or the like in the medical books. These terms help to define a set of conditions referred to colloquially in the anthropological writings as cultural syndromes. They require the culture that pays close attention to them, believe in them and support this philosophy by way on engaging in expected behavioral responses. When this “illness” is identified in accordance with sociocultural expectations, the individual experiencing these events might even have a better chance of recovery, assuming traditional treatments are properly applied. In Native American tradition, the individual going through a shamanic initiation process might be recommended to spend much of that fasting period under appropriate medical control, by a medically trained individual who is a member of that tribe or cultural group. It is typically the case that regular physicians spend little time trying to understand these socioculturally-defined prognostical medical conditions. Doctors assign very little value to the alternative views of the world and disease related to these syndromes, and almost always resort to their standards–the use of ICDs to document the social beliefs, practices, behaviors and medical outcomes that result. This suggests that physicians in the western world can be very ethnocentric at times when it comes to understanding people and health. When an individual belief system or faith becomes an offshoot of traditional philosophies, and their practices seem to work for inexplicable reasons, the western physician lacks the understanding and ability to understand what is taking place when a person changes or gets better. A regular physician might even have to resort to his/her unofficial fallbacks for explaining such events, such as spontaneous recovery, or mind-body effects, in turn falling upon whatever pseudo-scientific theory is out there for the time being used to explain the body-to-mind events and abilities (autonomics, psychosomatically induced hormonal change, prostaglandins effect, interferons and enkephalins “natural opiates” responses, psychoneuroimmunological or neuroendocrine causality, etc). The following are the ICDs closely linked to psychoneuroimmunological and self-induced psychobehavioral reactions to illnesses that otherwise a physician is unable to explain or define (see figure above). Takotsubo (ICD9 429.83) “Worrying or crying yourself to death”, or apical balooning of the ventricular walls in Takotsubo cardiomyopathy (ICD9=429.83). The primary theory proposed for this is weakening of the muscle wall due to: a) changes in arterial flow (left anterior descending coronary artery), b) vasospasms of this and/or other coronary arteries, and/or c) microvasculature failure within the cardiac tissue — any or all of these must be accompanied by a period of severe emotional stress resulting in autonomic effects upon the coronary arteries system. http://en.wikipedia.org/wiki/Takotsubo_cardiomyopathy; http://en.wikipedia.org/wiki/File:TakoTsubo_scheme.png. A major problem with this theory is that the age distribution of Takotsubo patients is nearly opposite of the the age gender features for traditional cardiomyopathy. Takotsubo is a condition associated primarily with Japanese culture, and has its greatest impacts on older populations, with female patients far outnumbering male patients (see population pyramids below). A similar psychosomatic like disorder does exist in the Western World/WHO ICD system — Factitious Disorder [300.16]. By definition and description Factitious disorder is of the 300 series and is therefore a pyschologically induced condition, which does not exactly match the ICD provided for Takutshobo [429.83]. The 400 series ICDs are typically assocaited with cardiological disturbances. It is interesting to note several similarities between these two ICDs in terms of Age-Gender distributions: Incidence Peaks at ages traditionally related to socially important “elders”, with ages > 65 or even 70 years of age. Female incidence for both ICDs for these older age patients is much greater than incidence/prevalence for males In the above images, the culturally-linked disease that is the focus of this query is on the left. Which of the other 3 best matches it’s form and distribution by both age and gender? There is a formula that can be used to quantify this overlap, but the picture makes it fairly clear which one it is in this case. Takotsubo ~ Factitious Disorder, in that both afflict older populations and are attached to some sort of emotional trigger that impacts women more than men in their older years. The anatomical-physiological theory often posed in the literature has a distribution that is the most equally distributed for the above, in terms of men versus women. Overall age-gender distributions for these first and last ICDs displayed are remarkable similar, suggesting there is either a psychoneurological-cardiological (hypothalmic-autonomic) feature involved in both processes, or that one system affect is somehow related to the other through a third mechanism, such as stress-induced (endocrine, neurtransmitter, releasing factor?) cardioelectric response for Takotsubo, with this cardiac reaction missing from non Japanese elders. For this reason, the proposed cardiomyopathy based theory does not quite fit in statistical distribution of this disease one the age-gender population pyramid. It is possible that the 439.83 pyramid is a result of late diagnosis, with early cases misidentified as other forms of myopathies. But the gender difference in takotshubo cannot be explained using this line of reasoning. More importantly, the more specific and unique forms of cardiomyopathy such as Obscure African Cardiomyopathy (425.2), Alcohol Cardiomyopathy (425.5), and Nutritional Cardiomyopathy (425.7) have male age peaks at either mid-life statge (45-55 yo) or the elderly stage (>70 yo). The other common cardiomyopathy–hypertrophic subaortic stenosis (425.1)–has a distribution mimicking the overall 425.* series. The same gender distribution is seen for the various cardiomyopathies, with male onset peaking in the 20-30 age group for both the Acute myocarditis (422.) and Acute Pericarditis (420.) series. The subacute endocarditis is not male gender weighted (421., this gender equality is also incidentally found for other endocarditis ICDs as well, in particualr valve-related disorders and therapeutic events). In sum, based on age distribution alone, cardiomyopathy cannot explain how and why takotshubo’s syndrome prevails primarily in older women. The similarity of form of the population pyramid with 300.16 Factitious Disorder suggests this is very much a pscyhologically based syndrome or condition manifesting itself (psychosomatically- and/or autonomically-induced?) with regards to the cardiac musculature and perhaps conduction system. This synopsis of course is based primarily on just the 1-year age-gender distribution pyramids (and therefore could be totally incorrect). One of the most classical lessons in biology classes focused on genetics is the Sickle Cell disease and the impact it has on the potential for malaria. Due to a genetic condition, hemoglobin becomes capable of sickle-shpaed (curved) or forming a chain like structure which in turn forces the red blood cell to change its morphology into an elongated, bent sickle-linke form. This in turn changes the 3-dimenstionality of the red blood cell and prevents it from passing through the narrow capillary beds. This failure to pass in turn results in reduced oxygenation and ultimately the death of many solid organ structures and tissues immediately surrounding richly vascularized capillary bed regions. The effects of malaria on blood include causing the red blood cell to sickle, thereby resulting in the destruction of cells that bear the organism responsible fo this disease. One result of this is the natural selection of sickle cell hybrids, people who carry but do not fully present with sickle cell sickling. These individuals have the chance to live longer lives in spite of the sickle cell gene, and so “select for” those who are hybrids with this condition or bear a version of the sickle cell trait that only partially presents itself. This sickle cell behavior is different from the living and survival biologies noted for thalassemia and hemophilia victims. Thalassemics live into later years of a normal life, due to lack of organ damage induced by sickling red blood cells. Hemophiliacs have a genetic condition influencing a completely different part of the metabolic path and result in survival curves not at all similar to the other two types of conditions. They bear the greatest longevity of these 3 conditions, four conditions if we separate sickle cell carriers from those actively presenting their sickle cell trait. We can constrast the Sickle Cell curves with those of Thalassemia and Hemophilia (all are above). There is an inveitably high mortality rate by the age of 65 years for people with Sickle Cell, be it in its active state or partially presenting itself within the bodies of carriers. The latter have a slightly longer longevity rate, and demonstrate male prevalence peaks earlier than female peaks, with females living longer and in greater numbers during their entire reproductive years period. The numerous reasons for socile cell mortality pertain to the effects of sickling cells on the microvasculature of solid organs, the splenomegaly these effects can result in at the organ systems level, and a variety of long term well localized anoxic states. Contrast these sickle cell disease and carrier curves with those of thalassemia and hemophilia. The latter two lack a demonstration of early mortality. Thalassemia is diagnosed very early on in life (beginning in the neonatal stage). Hemophilia tends to be diagnosed a few years later in life. Which is the above best represents SUNDS (Sudden Unexplained Nocturnal Death Syndrome) Cultural Shock and SUNDS After Saigon was attacked during the Vietnam War, a large number of Vietnamese upper class political leaders migrated to the United States. This migration was followed by requests for their assistance submitted by United States military leaders who assisted them in their need for asylum. The immigrants settled in just several parts of the country, usually in close association with the American military leaders who befriended them and made recommendations regarding places to settle. Following the immigration of these families was the migration of relatives and other immediate family friends and associates. By 1979, a number of Vietnamese, Cambodian and Laotian refugees communities were established, which have ever since remained their own unique sociocultural entities. Some of the more sizeable establishments were placed in unsuspected parts of the country such as Colorado, Iowa and Oregon. Immediately, the most important disease and public health issue during this time became tuberculosis. A number of the elders in these populations were found to be carriers. Another common malady experienced by this population was schizophrenia, a diagnosis which remains popular to this day within these southeast Asian American communities, possibly as a result of some familial genetic pattern common to this psychiatric condition. Several culturally-bound syndromes are also linked to this cultural group. The best known of these southeast asian syndromes is the suicidal state that acculturation and problematic adaptation processes often result in. Very young children are more capable of adapting than older children and young adults, with child bearing adults aged 35 and greater the most difficult group to provide assistance for. As these adults reach 55 years of age and greater, cultural traditions become a stumbling block for families trying to take advantage of government-based assistance. One of the chief problems these governmental programs had over the years was its ability to meet the needs of three or more cultural groups, all lumped into the same category for assistance. Aside from Laotian, versus Cambodian, versus Vietnamese, there were the Hmong emigrated from China who lived in the upper-eastern parts of this territory, a number of southeast asian cultural groups residing in wilderness settings, and the more urban adapted and often Christianized southeast asians residing close to and within the more urban settings of these countries. Such social diversity not only sets the stage for large amounts of political instability to ensue, but also makes it more likely that a number of psychological states and psychiatric and, in western society, criminal-related or developmental and academic related problems from developing in eacho of these populations. Cultural differences only add to these public health concers in that there are some basic statistics that may differ considereably between these groups. Education is found to be higher in social sociocultural groups than others. The likelihood of developing som familiarity with ESL standards is another, and even fertility rates are impacted by these southeast asian subpopulation differences. The Hmong for example have a fertility rate that greatly suprasses the same for nearly all other sociocultural groups in this country, save the Mennonites of the Pacific Northwest perhaps. This condition was typically experienced by the precollege and college age groups, and is characterized bywhat might first appear to be anxiety or fugue-like states or a sense of social detachment and panic; these occur immediately before a suicidal act like jumping through a window is engaged in. Recent studies tend to link the immigrant illness experience to well defined psychiatric and psychological states common to western society, such as agoraphobia, social phobia, depression, avoidant disorder, and anxiety. But like many studies of sociocultural disease, the Laotian-Cambodian-Vietnamese illness can have its own distinct causes and symptomatology. For this reason, one disease common to elders, manifesting itself primarily at night, was soon identified and linked to the oldest of the old immigrants, and given the name Sudden Unexected Nocturnal Death Syndrome (SUNDS, for which see http://www.deathreference.com/Sh-Sy/Sudden-Unexpected-Nocturnal-Death-Syndrome.html). SUNDS was identified by several authors focused on potential biological causes as a heart-realted death induced by mental anguish, mostly during the nighttime hours and during specific stages of the sleep cycle. Important contributors to the possiblity for SUNDS were sleeping during the dream state, with highly stressful behaviors acted out possibly due to the content of the nightmare or nigth horrors being experienced. These problems in turn resulted in cardia dysarhythmia according to the physicians researching this condition, thus resulting in nocturnally-induced cardiac dysarrhythmias and death. (See http://www.mja.com.au/public/issues/177_08_211002/mck10856_fm.html) Can SUNDS be detected within an ICD data pull process? The first problem with pulling SUNDS related conditions is the variability in potential ICD realtionships developed for this culturally-bound illness. To date, the following ICDs already assessed may have some relationship with how these cases get coded in the medical records. No SUNDS ICD exists for ICD9, although it is possible that ICD 446.7 Takotsubo’s disease is incredibly close to the Hmong-Laotian syndrome, including the cardiac physiological events related to Takotsubo’s disease. The strong tendency for Tak0tsubo’s syndrome to influence women over men suggests a sociocultural/psychological component, which if this is true may help to explain the apparent gender difference the other cultural groups with SUNDS may be demonstrate (lesser female, more male elder related events were noted clinically). - 290. Dementias - 300.* Anxiety states - 300.02 Generalized Anxiety Disorder - 300.1 Dissociative Disorders - 300.11 Conversion Disorder - 300.13 Fugue - 300.14 Identity - 300.16 Factitious Disorder - 300.21-.22 Agoraphobia - 300.23 Social Phobia (301.7 Antisocial) - 306.2 Acute Physiological Malfunctions of the Heart due to Mental Factors (306.8 bruxism, 306.6 endocrine) - 307 Sleep Disorders (307.45 Circadian, 307.46 Arousal) - 307.46 Sudden Affective Disorder (SAD) - 308 Acute Reaction to Stress - 309. 4 Emotion and Conduct Adjustment Disorders - 309.21 Separation Anxiety - 309.29 Culture Shock - 309.81 PTSD - 309.* Adjustment Disorders - 309.1 Prolonged Depression - 310.1 Personality Change due to . . . - 427.5 Cardiac Arrest - 446.7 Takotsubo’s Disease (see http://en.wikipedia.org/wiki/Takotsubo_cardiomyopathy) - 452.2 Obscure Cardiomyopathy of Africa - Brugada Syndrome or Asian Pseudo-RBBB (http://cvgenetics.med.nyu.edu/genetic-conditions/brugada-syndrome; http://www.cigna.com/customer_care/healthcare_professional/coverage_positions/medical/mm_0052_coveragepositioncriteria_genetic_testing.pdf) SUNDS is most often linked or related to the above 3 ICDs. This study (and my 2000-2003 field studies for a Quantitative/Qualitative Analysis Certification program) again demonstrate that, like Takotsubo, SUNDS has a tendency to behave most like Factitious Disorder, but with a more equally distributed gender-related pattern for elderly populations. There is a mindbody relationship in cardiomyopathy that is worth noting. The following schematic was produced by a researcher attempting to define the causology of cardiomyopathies. Barry J. Maron, MD, Chair; Jeffrey A. Towbin, MD, FAHA; Gaetano Thiene, MD; Charles Antzelevitch, PhD, FAHA; Domenico Corrado, MD, PhD; Donna Arnett, PhD, FAHA; Arthur J. Moss, MD, FAHA; Christine E. Seidman, MD, FAHA; James B. Young, MD, FAHA. AHA Scientific Statement. Contemporary Definitions and Classification of the Cardiomyopathies. An American Heart Association Scientific Statement From the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation. 2006; 113: 1807-1816. Even though some of the conclusions these authors present have already been questioned, their overall research methodology is worth noting due to its similarity with the Health Belief Model utilized by many health education/health promotion researchers and employees. The above flowchart for cardiomyopathies can be integrated with the Health Belief Model. For African cardiomyopathy(425.2) , the age-gender distribution most closely resembles nutritional deficiency cardiomyopathy (425.7). Given the age-gender distribution of Takatsubo versus cardiomyopathy, it appears tak0tsubo is primarily a psychosomatic condition induced primarily through personal perceptions, beliefs and knowledge, more so than a result of a biological phenomenon related mostly to some histological or cellular defect in cardiac tissue. There is no other way to explain why women within their older years manifest this condition so much more than men. [To be Continued]	4	15	0	0	0	35	0.551158	35	11,795
Unless you've been living in a cave, you are probably aware that come Oct. 1, 2015, the old ICD-9 coding system will be completely replaced with a "newer version": ICD-10. The purpose of this newer system is to modernize coding -- recognizing that since the last update in 1979, the number and precision of diagnoses has increased dramatically. Unfortunately, with only a maximum of five characters to use, ICD-9 is currently limited to around 13,000 codes. By expanding this to a maximum of seven characters, at least 68,000 diagnoses are being considered under ICD-10. Also, the newer coding system will allow for greater specificity and laterality (left/right). Under the newer system, the first character will always be a letter (alpha character), which will specify the organ system involved. For many, but not all, rheumatologic diagnoses, this will be "M". The next two characters can be either a number or alpha (#2 will always be a number, #3 can be either a number or alpha), and will define a category of diseases. For example, chronic gout: M1A; inflammatory arthropathies: M05-M14; OA: M15-M19; Systemic Connective Tissue Diseases: M30-36; and Spondyloarthropathies: M45-M49. These three characters are always followed by "." (period) before the next characters, if needed, are to be added. Character 4 will describe features like etiology or organ system (RA lung: M05.1, RA other: M05.8....by the way, there is a different code for seroposivite RA (M05) and seronegative RA(M06). Character 5 will be location (wrist=3, knee=6, multiple sites=9) and character 6 will be laterality (right=1, left=2, bilateral=3). The last character will be for additional features, such as tophi (chronic idiopathic gout of the right wrist with tophi=M1A.0911; no tophi=M1A.0910). Confused yet? Wait, there's more. Sometimes there is no laterality, so a placeholder "X" is added (chronic idiopathic gout, multiple sites with tophi=M1A.09X1). Some codes can exist by themselves (gout: M1A,xxxx, xxxx, seropositive RA: M05.8xxx, PMR: M35.3). Others can be reported as combinations with another disease (IBD-related right knee arthritis due to Crohn's disease=M07.661 and K50.918). Still, others need to be reported in combination with other diagnoses, but in a specific order (Reactive Arthritis of the Left ankle due to Yersinia infection=A04.6 (which must be listed first) and M02.172). My recommendations for implementation of ICD-10 The first recommendation I have is to start NOW, if not sooner. Summer is rapidly coming to an end and October will soon be upon us. Once this date happens, anything you code using the old system WILL BE REJECTED!! (RheumNow editor's note: Dr. Bergman's advice is spot on. Note that CMS and the AMA have stated that they will be "lenient" in the first year of implementation. What that means remains to be seen – JC) Do you bill using a computer? (Hopefully, a rhetorical question.) Make sure NOW that your software is able to handle ICD-10. Using a billing service? Make sure NOW that they are able to handle ICD-10. Buy a coding book. Let's face it; while there is logic to this system, it is far from intuitive. Having a comprehensive code book, like the ones provided by the AMA (disclosure, I am a member, but not otherwise affiliated) and learn to use it. This is not the time or place to save money. Remember what I wrote above...failure to use these codes will lead to your claims being REJECTED!! Start small, but get to work. The good news is: for the most part we use the same codes over and over again. We are not FPs who must have every code in the book entered into our computers. More likely we will need 100-200. While that sounds like a large number, it is far from daunting. I'm starting with the most common codes (RA, Gout, Joint Pain, and Joint Swelling). A list of the most commonly used codes in rheumatology is available on the ACR website. (Editor's note: this link was updated on 8/12/15) Once those are in the system, I'm going through my entire list of frequent codes, A-Z, and changing the codes, one at a time. If I do 10-20 per day, which is not unrealistic, I should be through this in under a month. Educate your staff, especially your billing folks, about the new codes. Did I mention that if you don't use the new codes, your billing WILL BE REJECTED. Once you have a list of effective codes, re-create your super-bill (if you are still using them) or let your computer system generate the bills, after you have added them. Anticipate problems and have ready cash available during the transition period. Even in the best of circumstances, problems are bound to occur. This is a nationwide roll-out, glitches are bound to occur. Being ready is always the best option. If all of this sends you into panic mode, the other option is to pay a service to do this for you. There are many out there who are more than willing to assist you in this transition, for a fee. I'm not making enough in clinical practice to have me want to share it with others, but that is my decision. You may feel differently. Regardless, the time to act is NOW, if not yesterday. October will be here, soon enough. Do you know where your reimbursements will be? Martin J. Bergman, MD, is clinical associate professor of medicine at Drexel University College of Medicine in Philadelphia and is chief of the section of rheumatology at Taylor Hospital in Ridley Park, Pa. A version of this article first appeared on RheumNow. RheumNow is a news, information, and commentary site dedicated to the field of rheumatology. Register to receive their free rheumatology newsletter. The author has no conflicts of interest to disclose related to this subject.	4	3	0	0	0	6	0.607652	6	1,413
Although benign tumors will not metastasize or locally invade tissues, some types may still produce negative health effects. The growth of benign tumors produce a "mass effect" that can compress tissues and may cause nerve damage, reduction of blood to an area of the body (ischaemia), tissue death (necrosis) and organ damage. The mass effect of tumors are more prominent if the tumor is within an enclosed space such as the cranium, respiratory tract, sinus or inside bones. Tumors of endocrine tissues may overproduce certain hormones, especially when the cells are well differentiated. Examples include thyroid adenomas and adrenocortical adenomas. Although most benign tumors are not life-threatening, many types of benign tumors have the potential to become cancerous (malignant) through a process known as tumour progression. For this reason and other possible negative health effects, some benign tumors are removed by surgery. \|Cell Origin\|\|Cell type\|\|Tumor\| \|Liver\|\|Liver cell adenoma\| \|Renal\|\|Renal tubular adenoma\| \|Squamous\|\|Squamous cell papilloma\| Benign neoplasms are typically but not always composed of cells which bear a strong resemblance to a normal cell type in their organ of origin. These tumors are named for the cell or tissue type from which they originate, followed by the suffix "-oma" (but not -carcinoma, -sarcoma, or -blastoma, which are generally cancers). For example, a lipoma is a common benign tumor of fat cells (lipocytes), and a chondroma is a benign tumor of cartilage-forming cells (chondrocytes). Adenomas are benign tumors of gland-forming cells, and are usually specified further by their cell or organ of origin, as in hepatic adenoma (a benign tumor of hepatocytes, or liver cells). Teratomas contain many cell types such as skin, nerve, brain and thyroid, among others, because they are derived from germ cells. Hamartomas are a group of benign tumors that have relatively normal cellular differentiation but the architecture of the tissue is disorganised. There are a few cancers with 'benign-sounding' names which have been retained for historical reasons, including melanoma (a cancer of pigmented skin cells, or melanocytes) and seminoma (a cancer of male reproductive cells). Skin tags, vocal chord polyps and hyperplastic polyps of the colon are often referred to as benign but they are actually overgrowths of normal tissue rather than neoplasms. Benign tumors are very diverse, and may be asymptomatic or may cause specific symptoms depending on their anatomic location and tissue type. They grow outwards, producing large rounded masses, which can cause what is known as a "mass effect". This growth can cause compression of local tissues or organs, which can cause many effects such as blockage of ducts, reduced blood flow (ischaemia), tissue death (necrosis) and nerve pain or damage. Some tumors also produce hormones that can lead to life-threatening situations. Insulinomas can produce large amounts of insulin leading to hypoglycemia. Pituitary adenomas can cause elevated levels of hormones such as growth hormone and insulin-like growth factor-1, which cause acromegaly; prolactin; ACTH and cortisol, which cause Cushings disease; TSH, which causes hyperthyroidism; and FSH and LH. Bowel intussusception can occur with various benign colonic tumors. Cosmetic effects can be caused by tumors, especially those of the skin, possibly causing psychological effects on the person with the tumor. Vascular tumors can bleed, which in some cases can be substantial, leading to anemia. Some benign tumors need no treatment; others may be removed if they cause problems such as seizures, discomfort or cosmetic concerns. Surgery is usually the most effective approach and is used to treat most benign tumors. In some case other treatments may be of use. Adenomas of the rectum may be treated with sclerotherapy, a treatment in which chemicals are used to shrink blood vessels in order to cut off the blood supply. Most benign tumors do not respond to chemotherapy or radiation therapy, although there are exceptions; benign intercranial tumors are sometimes treated with radiation therapy and chemotherapy under certain circumstances. Radiation can also be used to treat hemangiomas in the rectum. Benign skin tumors are usually surgically ressected but other treatments such as cryotherapy, curettage, electrodesiccation, laser therapy, dermabrasion, chemical peels and topical medication are used. Benign vs malignant One of the most important factors in classifying a tumor as benign or malignant is its invasive potential. If a tumor lacks the ability to invade adjacent tissues or spread to distant sites by metastasizing then it is benign, whereas invasive or metastatic tumours are malignant. For this reason, benign tumours are not classed as cancer. Benign tumours will grow in a contained area usually encapsulated in a fibrous connective tissue capsule. The growth rates of benign and malignant tumors also differ; benign tumors generally grow more slowly than malignant tumors. Although benign tumors pose a lower health risk than malignant tumors, they can both be life-threatening in certain situations. There are many general characteristics which apply to either benign or malignant tumors, but sometimes one type may show characteristics of the other. For example, benign tumors are mostly well differentiated and malignant tumors are often undifferentiated. However, undifferentiated benign tumors and differentiated malignant tumors can occur. Although benign tumors generally grow slowly, cases of fast growing benign tumors have also been documented. Some malignant tumors are mostly non-metastatic such as in the case of basal cell carcinoma. Tumours are formed by carcinogenesis, a process in which cellular alterations lead to the formation of cancer. Multistage carcinogenesis involves the sequential genetic or epigenetic changes to a cell's DNA, where each step produces a more advanced tumour. It is often broken down into three stages; initiation, promotion and progression, and several mutations may occur at each stage. Initiation is where the first genetic mutation occurs in a cell. Promotion is the clonal expansion (repeated division) of this transformed cell into a visible tumour that is usually benign. Following promotion, progression may take place where more genetic mutations are acquired in a sub-population of tumor cells. Progression changes the benign tumor into a malignant tumor. A prominent and well studied example of this phenomenon is the tubular adenoma, a common type of colon polyp which is an important precursor to colon cancer. The cells in tubular adenomas, like most tumors which frequently progress to cancer, show certain abnormalities of cell maturation and appearance collectively known as dysplasia. These cellular abnormalities are not seen in benign tumors that rarely or never turn cancerous, but are seen in other pre-cancerous tissue abnormalities which do not form discrete masses, such as pre-cancerous lesions of the uterine cervix. Some authorities[who?] prefer to refer to dysplastic tumors as "pre-malignant", and reserve the term "benign" for tumors which rarely or never give rise to cancer. PTEN hamartoma syndrome PTEN hamartoma syndrome comprises four distinct hamartomatous disorders characterised by genetic mutations in the PTEN gene; Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome and Proteus-like syndrome. Although they all have distinct clinical features, the formation of hamartomas is present in all four syndromes. PTEN is a tumor suppressor gene that is involved in cellular signalling. Absent or dysfunctional PTEN protein allows cells to over-proliferate, causing hamartomas. Cowden syndrome is an autosomal dominant genetic disorder characterised by multiple benign hamartomas (trichilemmomas and mucocutaneous papillomatous papules) as well as a predisposition for cancers of multiple organs including the breast and thyroid. Bannayan-Riley-Ruvalcaba syndrome is a congenital disorder characterised by hamartomatous intestinal polyposis, macrocephaly, lipomatosis, hemangiomatosis and glans penis macules. Proteus syndrome is characterised by nevi, asymmetric overgrowth of various body parts, adipose tissue dysregulation, cystadenomas, adenomas, vascular malformation. Familial adenomatous polyposis Familial adenomatous polyposis (FAP) is a familial cancer syndrome caused by mutations in the APC gene. In this disorder adenomatous polyps are present in the colon that invariably progress into colon cancer. The APC gene is a tumor suppressor and its product is involved in many cellular processes. Inactivation of the APC gene leads to a buildup of a protein called β-catenin, which activates two transcription factors; T-cell factor (TCF) and lymphoid enhancer factor (LEF). These cause the upregulation of many genes involved in cell proliferation, differentiation, migration and apoptosis (programmed cell death), causing the growth of benign tumors. Tuberous sclerosis complex Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder caused by mutations in the genesTSC1 and TSC2, which produce the proteins hamartin and tuberin, respectively. This disorder presents with many benign hamartomatous tumors including angiofibromas, renal angiomyolipomas, pulmonary lymphangiomyomatosis. Tuberin and hamartin inhibit the mTOR protein in normal cellular physiology and the inactivation of the TSC tumour suppressors causes an increase in mTOR activity. This leads to the activation of genes and the production of proteins that increase cell growth. Von Hippel-Lindau disease Von Hippel-Lindau disease is a dominantly inherited cancer syndrome that massively increases the risk of various tumors including benign hemangioblastomas and malignant pheochromocytomas, renal cell carcinomas, pancreatic endocrine tumors and endolymphatic sac tumors. It is caused by genetic mutations in the Von Hippel–Lindau tumor suppressor gene. The VHL protein (pVHL) is involved in cellular signalling in oxygen starved (hypoxic) cells. One role of pVHL is to cause the cellular degradation of another protein, HIF1α. Dysfunctional pVHL leads to accumulation of HIF1α, which in turn activates the production of several genes involved in cell growth and blood vessel production (VEGF, PDGFβ, TGFα and erythropoietin). - Wilson, Kathleen Atkins; Waugh, Anne; Chambers, Graeme; Grant, Allison; Ross, Janet (2006). Ross and Wilson anatomy and physiology in health and illness. Edinburgh: Churchill Livingstone. pp. 53–54. ISBN 0-443-10101-9. - Nunn, Laura Silverstein; Silverstein, Alvin; Silverstein, Virginia B. (2006). Cancer. Brookfield, Conn: Twenty-First Century Books. pp. 11–12. ISBN 0-7613-2833-5. - David Lowell Strayer; Raphael Rubin; Rubin, Emanuel (2008). Rubin's pathology: clinicopathologic foundations of medicine. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins. pp. 138–139. 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"Radiotherapy for benign brain tumours coming of age; example of vestibular schwannoma". Radiother Oncol 106 (2): 157–60. PMID 23462704. doi:10.1016/j.radonc.2013.01.009. - Sioka C, Kyritsis AP (March 2009). "Chemotherapy, hormonal therapy, and immunotherapy for recurrent meningiomas". J. Neurooncol. 92 (1): 1–6. PMID 19023520. doi:10.1007/s11060-008-9734-y. - Luba MC, Bangs SA, Mohler AM, Stulberg DL (February 2003). "Common benign skin tumors". Am Fam Physician 67 (4): 729–38. PMID 12613727. - Marghoob AA, Borrego JP, Halpern AC (December 2007). "Congenital melanocytic nevi: treatment modalities and management options". Semin Cutan Med Surg 26 (4): 231–40. PMID 18395671. doi:10.1016/j.sder.2008.03.007. - Skorić T, Korsić M, Zarković K et al. (June 1999). "Clinical and morphological features of undifferentiated monomorphous GH/TSH-secreting pituitary adenoma". Eur. J. Endocrinol. 140 (6): 528–37. PMID 10366409. doi:10.1530/eje.0.1400528. - Song HJ, Xue YL, Qiu ZL, Luo QY (2012). 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Please note that these scenarios, medical reports, and coding examples are all theoretical. Some of them may have been simplified for space. They are presented solely as teaching examples. Let’s start off with a relatively straightforward visit to the doctor’s office. Here’s some context: our patient is in his late 20s and has not seen this doctor before. He’s had a fever, a cough, some slight chest pain, and difficulty breathing. Here’s the hypothetical medical report. Patient is a 27-year-old white male. Not an established patient. Height is 74 inches, weight 220 lbs. Patient states he is allergic to penicillin, but has no other outstanding medical history. Does not smoke, exercises moderately. Patient presents with chills, headache, cough, fever (101 degrees), difficulty breathing. Examination via stethoscope yields heavy rales. Percussion test on thorax suggests buildup in lungs. Streptococcal pneumoniae suspected. Obtained blood sample for Antistreptolysin O titer. Results yield level of ASO above 200. Diagnosis of streptococcal pneumoniae confirmed. Prescribed patient two weeks of 500mg azithromycin (Zithromax), and scheduled follow-up for next week. Let’s start abstracting the information from this medical report. We can start right away with the most straightforward code: the diagnosis. The doctor has diagnosed streptococcal pneumoniae in this patient. (Remember, we always code the definitive diagnosis, meaning we wouldn’t code the cough, fever, or any of the other symptoms unless the doctor could come to a definitive diagnosis). So, in order to get the proper diagnosis code, we turn to the ICD-10-CM manual. We’d look to the range J00– J99, for Diseases of the Respiratory System. In that section we’d find “J13 – pneumonia due to Streptococcus pneumoniae,” which is the code we’re looking for: pneumonia caused by streptococcal bacteria. In this case, there are no subcategories or subclassifications. J13 is our diagnosis code. (Bear in mind that you could also just look up Streptococcus Pneumoniae in the alphabetic index of ICD-10-CM. We’re taking the long route for the sake of instruction). Now we get into the procedure codes. There are a couple here. Firstly, since a new patient is coming into the doctor’s office with an as-yet unspecified condition, we know it’s an Evaluation and Management (E&M) procedure. Secondly, we see that the doctor performed a test on the patient to confirm the diagnosis. That’s a pathology and laboratory test. Let’s start with the E&M codes. You may think that the prescription of medication by the doctor falls into yet another category of procedure, but prescribing a medication is actually part of the E&M process. So too are the basic examinations the doctor performed (the thoracic percussion and the use of the stethoscope). Those are each part of the History, Exam, and Medical Decision-Making portions of E&M codes, often abbreviated to HEM. The doctor asked the patient about his history, he performed various exams, and he made a medical decision to prescribe antibiotics. When you’re selecting E&M codes, you look at the intensity of the evaluation and patient management. We can do this by the time spent with the patient, or we can do it by evaluating the amount of work that goes into the HEM. E&M codes will list the level of intensity (and the time) of the procedure in their description. In our example, since the patient is new, there will be a more involved ‘history’ section of HEM. This would go with a thorough examination. The medical decision-making of prescribing antibiotics by oral administration is relatively low-risk, so this E&M procedure would probably be of low to moderate complexity. Since the patient is a new patient, we’d look for codes in that subsection of E&M. We’d find code 99203, which includes a “detailed history; a detailed examination; medical decision making of low complexity.” That’s our E&M code. But the doctor also performed a test that confirmed his diagnosis. How do we code that? Since the doctor took a sample from the patient to test, we know it’s in the Pathology Section of the CPT Manual. A little medical background here: the test in the medical report was an Antistreptolysin O, or ASO, titer. A titer is a type of test. ASOs are antibodies the immune system creates to combat streptococcal bacteria. The human body typically has around 200 units of ASO as a baseline. If a test confirms a heightened level of ASO, that’s usually a definitive sign of the presence of strep bacteria. We can find the test for ASO in the immunology subsection of Pathology. We could also look up antistreptolysin in the index at the back of the CPT manual. Both methods would lead us to the CPT code 86060, “antistreptolysin; titer.” That’s our Pathology code. Now what about the medication the doctor’s prescribed to our patient? We’d include that as part of our medical decision making process—the prescription of antibiotics is the decision the doctor made for the patient. However, if we wanted to add it to the E&M code to give the payer more information, we can do that pretty easily. Previously, we talked about Category II CPT codes as useful for administrative and performance management purposes, but they can also be used in informational settings. These Category II codes are optional, and are added to Category I codes with a hyphen. We’d look up the proper Category II code in the index under “antibiotic administration.” That would point us to 4120F, “antibiotics prescribed or dispensed.” We’d flip there in Category II to confirm. So we’d add the Category II CPT code 4120F to our extant E&M code 99203 to get 99203-4120F, which is a new patient visit of low complexity, with the prescription or dispensation of antibiotics. So our codes would be… We’d enter these into a superbill that would include… - The patient’s information - The provider information - Procedure information - Diagnostic information We’d then send that superbill on to the medical biller so that they could create the medical claim. Let’s look at another hypothetical situation, this time involving surgery. We’ll jump right in with our medical report. This one, you’ll note, is different in format from the previous one. There’s no uniform style of medical report, so coders should be prepared to abstract information from a whole variety of reports. CHIEF COMPLAINT: Pain and swelling in abdomen HISTORY OF PRESENT ILLNESS: Patient is a 67-year-old female presents to the emergency room with sharp, shooting pain in her lower abdomen and pronounced swelling. Patient is nauseous, has vomited, and has a fever. Abdomen is firm and slightly distended. Patient states she has no history of abdominal problems, disease, or hernia. PAST MEDICAL HISTORY: Patient is on a program of anti-depressants, but is otherwise physically sound. States she has never been admitted for any abdominal problems. CURRENT MEDICATIONS: Wellbutrin, 5 mg daily SOCIAL HISTORY: Used to smoke (using Wellbutrin to quit) and does not drink REVIEW OF SYMPTOMS: As above Vital Signs: Blood pressure is 150/88, with a fever of 102 degrees. Skin: Warm and dry and normal, except in lower abdomen, where it is swollen and tight Chest: no respiratory problems detected Cardiac: regular rhythm Back: No abnormalities detected Abdomen: is firm above the pelvic bones. Patient experiences pain upon palpation. Blumberg sign elicits painful response, as does a forced cough. Abdomen is tight and swollen. INTERVENTION: Physical examination suggests appendicitis. Ultrasound test ordered, and diagnosis of appendicitis is confirmed. Patient is rushed to surgery and is prepped for general anesthesia. Once anesthetized, patient receives appendectomy via laparoscopy. Exploration during surgery reveals no signs of peritonitis, local or general. Following surgery, patient is taken to observation room, and then to hospital room. Patient responds well to surgery, and is discharged later that night with a prescription for pain medication. Diagnosis: Acute appendicitis As you can tell, this medical report is significantly more involved than our first one. Thankfully, it’s a pretty direct procedure, so we can dive right in. Let’s start with the diagnosis. As listed above, the diagnosis is acute appendicitis. To find this in ICD-10-CM, we’d look for Diseases of the Digestive System. In that section we’d find the code for acute appendicitis: K35. Under that code we’d find multiple subcategories. As we saw in the medical report, there was no sign of any peritoneal complications, so we’d choose K35.80, unspecified acute appendicitis. That’s our diagnosis code. Let’s move on to procedure codes. We’ll start again with E&M. This is an emergency department services code. Emergency codes, like outpatient or office codes, are coded depending on the intensity/risk of the procedure. When our patient checks in, she’s not in directly life-threatening danger, but if the appendicitis goes septic, it could endanger her life. With that in mind we’d pick the E&M code 99284, a visit for a condition that “requires urgent evaluation by the physician…. but [does] not pose an immediate significant threat to life or physiological function.” So, 99284 is our E&M code. Of course, our procedure coding doesn’t stop there. As we saw from the medical report, the emergency department performed an ultrasound to confirm their diagnosis, and performed a surgical procedure with general anesthesia. This means we need three more codes: a Radiology code (for the ultrasound), a Surgery code (for the appendectomy), and an Anesthesia code (for the general anesthesia). Let’s start with the ultrasound. Diagnostic ultrasounds are found after diagnostic radiology (e.g. x-rays) in the Radiology Section. We’d look for a diagnostic ultrasound for the abdomen and find 76700, for “ This means it’s an ultrasound for the entire abdomen. That’s close, but not exactly what we want. Below 76700 you’ll see code 76705, for a limited abdominal ultrasound of a single organ or quadrant. That’s what we want. So 76705 is our Radiology code. Let’s move to Surgery. The Surgery Section is relatively straightforward. We find the procedure we want by the area of the body it’s performed on. In our case, we’d turn to the digestive system (recall that the appendix is attached to the large intestine). There we’d find codes for the appendix, including incision, excision, and laparoscopy. We’re looking for an appendectomy done via laparoscopy, so we’d look at laparoscopy codes, and we’d select 44970, laparoscopy, surgical, appendectomy. That’s our Surgery code. With every surgery we’ll need some sort of Anesthesia code. Per the medical report, the patient received general anesthesia, but we select Anesthesia codes based on the location of the procedure performed under the anesthetic. So the Anesthesia code would be located under “Lower Abdomen” in the Anesthesia Section. Specifically, we’d choose 00840, Anesthesia for intraperitoneal procedures in lower abdomen including laparoscopy; not otherwise specified.” Remember also that with every Anesthesia code, you need to add a physical status modifier. These are found in the Anesthesia section, and are added to the Anesthesia code to describe the condition of the patient. In our example, the patient is a P3—the patient has a severe systemic disease (since appendicitis can go septic, and thus potentially life-threatening, it’s regarded as severe). So our Anesthesia code is 00840-P3. Here’s what we’d end up with: with 00840-P3 (Anesthesia) K35.80 – Unspecified acute appendicitis We’d include that information in a superbill, along with the provider information (in this case it’d be a hospital), patient information, and prices. We’d send those on to the medical biller, whose work we’ll explore in the next Course.	4	10	14	0	0	9	0.526025	23	2,866
Category:Symptoms and signs of mental disorders Wikimedia Commons has media related to Symptoms and signs: Cognition, perception, emotional state and behaviour. \|This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision.\| Generally, diseases outlined within the ICD-10 codes R40-R46 within Chapter XVIII: Symptoms, signs and abnormal clinical and laboratory findings should be included in this category. This category has the following 8 subcategories, out of 8 total. - Psychopathological syndromes (1 C, 55 P) - Amnesia (3 C, 17 P) - Coma (2 C, 17 P) - Hallucinations (3 C, 53 P) - Psychosis (7 C, 68 P) - Self-harm (1 C, 34 P, 1 F) - Thought disorders (1 C, 8 P) Pages in category "Symptoms and signs of mental disorders" The following 128 pages are in this category, out of 128 total. This list may not reflect recent changes. - Alcohol withdrawal syndrome - Alice in Wonderland syndrome - Altered level of consciousness - Angor animi - Athymhormic syndrome - Auditory hallucination - Aura (symptom) - Automatic behavior - Automatic negative thoughts - Avoidance coping	3	8	0	0	0	2	0.833606	2	290
Cough ICD 10 In this topic we shall discuss on types of Cough, Cough ICD 10 guidelines along with examples. Coughing is a common reflex action when we want to release any irritants from our throat or airway. It is normal to cough sometimes. But we need to visit doctor if continuous cough for more than 3 weeks or coughing with any other symptoms like fever, shortness of breath, chest pain, wheezing, yellow or green mucus or blood because these are due to an underlying disease. Cough is not a disease, it is a symptom of some other condition. Acute cough is the one which cures within 3 weeks, but chronic lasts for more than 8 weeks in adult. Do not get confuse with the term choking. A person coughs with sound. On the other hand when airway is completely blocked there will be no airflow which makes the person to choke without any sound. This needs urgent attention. Types of cough: The main two types of cough are dry cough and wet cough. Dry cough :- Dry cough is one of the common symptom of Covid-19. It does not produce sputum. Some other diseases like asthma and GERD also can produce a dry cough. Wet cough :- It produces mucus from lungs or sinuses. This occurs in pneumonia, flu, COPD, asthma or bronchitis. Other few types of cough which can be found in medical record. Whooping cough :- When coughing it sounds like ‘whoop’, hence it is known as whooping cough. It has another name “pertussis”. This occurs due to bacterial infection. Croup cough :- Patient will have a barking cough due to viral infection. Smoker’s cough :- Occurs in long term smokers which is characterized by persistent cough for more than 3 weeks. Below are few conditions in which cough is a routine symptom. - Postnasal drip - Upper respiratory infections – cold, flu - Lung cancer Depending on the type of cough and other symptoms, physician will do further testing such as chest X-ray, CT, angiogram, bronchoscopy, Covid-19 PCR etc to find out the definitive diagnosis. Cough ICD 10 codes and guidelines - Cough with haemorrhage (bleeding) should be coded as haemoptysis – R04.2 - Choking should be coded as R09.89 (choking sensation) - Croup cough should be coded as “Croup” J05.0 - Avoid coding unspecified cough R05 when a definitive diagnosis is coded in which cough is a routine symptom - Do not code J41.0 without diagnosed by physician. Just because patient is a smoker coming with cough can not be assumed and coded as smoker’s cough. \|Cough ICD 10 Code\|\|Description\| -Laryngeal spasmodic cough \|B49\|\|Tea taster’s Cough\| \|A37.90\|\|Whooping cough without pneumonia, unspecified species\| Few scenarios on cough Cough ICD 10 Scenario 1 40 year old Jennifer visits clinic with dry cough, shortness of breath and feverish from past 4 days. She had a business related travel to Australia 2 weeks back. She states she had followed all the precautions of Covid-19 and not had contact with any Covid positive persons. On exam she does not have fever, BP was 125/85 mm Hg. She is alert and oriented with laboured breathing. Physician ordered Covid-19 PCR test. Chest X-ray performed today showed no abnormalities. Physician mentioned in the final assessment – suspected exposure to Covid-19 ICD-10 codes to be coded: Z20.828 – Contact with or suspected exposure to someone who has positive Covid-19 R05 – Dry cough R06.02 – SOB Note : Dry cough is a common symptom of Covid-19. But R05 is coded here as there is no definitive diagnosis of Covid-19. Cough ICD 10 Scenario 2 A 67 year old man presented to the emergency department with coughing blood from last 2 weeks. He stated he had small streaks of blood in sputum, but today just half an hour before he coughed up a cup of blood. He has no history of pneumonia, kidney disease or any autoimmune disease. He use tobacco. He continued coughing small amount of blood during physical exam. Vitals noted as BP 100/60 mm Hg, heart rate of 112 beats per minute, respiratory rate of 30-35 breaths per minute, oxygen saturation of 93% on a 100% oxygen via high flow face mask. Head and neck exam showed presence of blood in oropharynx and nares. Physician ordered for labs and chest X-ray and admitted the patient for further work up. ICD-10 codes to be coded: R04.2 – Hemoptysis Z72.0 – Tobacco use Note : Coughing blood is coded as Hemoptysis as per guideline.	4	9	0	0	0	10	0.724076	10	1,083
Historic Update to ICD-10 Endometriosis Diagnosis Codes With ever-increasing medical knowledge, the classification of diseases is important to provide a common language for reporting and monitoring. This is where the International Classification of Diseases (ICD) comes in. The World Health Organization (WHO) and 10 international centers create, maintain, and revise the ICD. The latest version, ICD-10, contains important updates regarding endometriosis. How have ICD codes evolved over time? Coding for medical conditions has existed since the 19th century. The International Statistical Institute adopted the first “International List of Causes of Death” in 1893. The sixth edition of this list was published in 1948. The WHO adopted this in the form of ICD coding. The WHO released ICD-7 in 1957, ICD-8 in 1968, and ICD-9 in 1979. The U.S. modified ICD-9 into its own version and created ICD-9-CM (clinical modification). The introduction of ICD-9-CM was revolutionary because it enhanced the scope and utility of these codes by incorporating additional information such as surgical procedures. ICM-9-CM was available in three volumes: the first two volumes contained diagnosis codes while the third included inpatient procedures. However, with the fast pace of medical advancements, ICD-9-CM soon started becoming inadequate. The WHO started working on ICD-10 even before ICD-9 was complete. ICD-10 was published in 1990 with more than 155,000 codes. Based on input from the National Center for Health Statistics (NCHS), the U.S. created ICD-10-CM. This includes the creation of diagnosis-symptom combinations to reduce the number of codes, information about managed care, and ambulatory encounters, and greater specificity. NCHS updates ICD-10-CM each year. In the U.S., ICD-10 is in two forms. ICD-10-CM for all healthcare settings and ICD-10-PCS (procedure coding system) for inpatient settings only. The latest ICD revision is ICD-11. The 72nd World Health Assembly adopted this in 2019, which came into effect on January 1, 2022. What is the use of ICD coding? ICD codes are important in collecting mortality data and enabling clinicians, healthcare providers, and financial providers to make evidence-based decisions. Coding helps compare health statistics across WHO member countries in a standardized manner. All WHO member countries must use the most current ICD version for reporting disease and death statistics. As mandated by the U.S. Department of Health and Human Services (HHS), all Health Insurance Portability and Accountability Act (HIPAA)-covered entities must follow the most up to date ICD codes. What were the traditional endometriosis codes in ICD-10-CM? Despite affecting up to 1 in 10 women of reproductive age, doctors still don’t understand endometriosis well. They therefore often misdiagnose the disease as symptoms overlap with those of other diseases of the abdomen and pelvic region. Moreover, noninvasive diagnoses are often not conclusive. There were no ICD codes for endometriosis for over 40 years. Since the 1970s, all endometriosis diagnoses were under the ICD-10 code N80.9 – “Endometriosis, unspecified”. Similarly, all endometriosis surgery appeared under the Current Procedural Terminology (CPT) code 58662 – “laparoscopy, surgical; with fulguration or excision of lesions of the ovary, pelvic viscera, or peritoneal surface by any method“. We currently know there are many stages and manifestations of this disease that include bladder, bowel, and thoracic endometriosis, among others. These codes put severe limits on the manner in which various manifestations of the disease are detailed and treated. It ultimately created disparities in diagnosis and posed challenges in providing appropriate health coverage. More importantly, the previous ICD-10 endometriosis coding did not validate the myriad of debilitating endometriosis symptoms that patients experience and the work that clinicians and researchers have dedicated their time to. Who was behind the initiative to update ICD-10-CM endometriosis codes? Dr. Ted T. M. Lee is currently the Director of Minimally Invasive Gynecologic Surgery at the University of Pittsburgh Medical Center at Magee Women’s Hospital and President of the American Association of Gynecologic Laparoscopists (AAGL). In 2016, the AAGL commissioned him to liaison with the American College of Obstetricians and Gynecologists (ACOG) Coding Committee to understand and improve the coding problem for endometriosis. Dr. Lee made a presentation to the Centers for Disease Control and Prevention (CDC) in 2020. Here, he detailed various aspects of endometriosis and the need to revise the ICD-10-CM coding for the disease. He and his colleagues went on to develop 100 new codes for endometriosis. These codes serve to represent the various multi-organ manifestations of the disease. They also lay the foundation for future procedure code changes. What is the new ICD-10-CM update for endometriosis? The new ICD-10-CM coding continues to group endometriosis under N80. However, this now includes codes from N80.0 to N80.5 that cover endometriosis of the uterus (N80.0), ovaries (N80.1), fallopian tubes (N80.2), pelvic peritoneum (N80.3), rectovaginal septum and vagina (N80.4), and intestines (N80.5). The new ICD-10-CM update also provides codes for various pelvic and extra-pelvic endometriosis forms. These include bladder and ureters (N80.A), cardiothoracic space (N80.B), abdomen (N80.C), pelvic nerves (N80.D), and endometriosis at other sites (N80.8). A unique case study of isthmocele endometriosis, which Dr. Tamer Seckin recently presented also gets a mention under N85.A. Each section offers many sub-section codes that further detail the diagnosis. For example, N80.3 denotes endometriosis of the pelvic peritoneum. Under this, the N80.31 sub-section indicates endometriosis of the anterior cul-de-sac. N80.31 further includes: - N80.311: superficial endometriosis of the anterior cul-de-sac - N80.312: deep endometriosis of the anterior cul-de-sac - N80.319: endometriosis of the anterior cul-de-sac, unspecified depth, endometriosis of the anterior cul-de-sac NOS Responses to these new changes to ICD-10-CM have been overwhelmingly positive. Dr. Karli Provost Goldstein said, “We look forward to many changes to come for patients finally with a comprehensive way to define their disease and eventually be able to better define and bill for the complex surgery required. As an endometriosis surgeon and previous patient myself, this feels like a great stride forward.” Dr. Tamer Seckin said, “We’re thrilled about this long overdue but monumental step forward to legitimize the complex, multi-organ nature of endometriosis surgery. This will open doors for the patient community, improving patient treatment and providing critical data for research advancements. A great thank you to Dr. Lee and everyone else who worked on this”. The inclusion of 100 new endometriosis codes into ICD-10-CM is a historical step that lays the foundation for further changes in endometriosis CPT surgical codes and corresponding billing systems, thus giving proper validation and legitimacy to both patients and healthcare providers. Please do not hesitate to share your thought about this historic event below our post on Facebook or Instagram. Ready for a Consultation?Our endometriosis specialists are dedicated to providing patients with expert care. Whether you have been diagnosed or are looking to find a doctor, they are ready to help. Our office is located on 872 Fifth Avenue New York, NY 10065. You may call us at (646) 960-3080 or have your case reviewed by clicking here.	4	6	3	0	0	14	0.589975	17	1,816
Textbook of Psychiatry/Personality Disorders - 1 Introduction - 2 Each of the Personality Disorders - 2.1 Cluster A - 2.2 Cluster B - 2.3 Cluster C - 3 References Treating psychopathology requires an understanding of personality. Research on the DSM and ICD disorders is making it increasingly clear that: 1. anxiety, depression, eating disorders, substance abuse, sexual disorders, and other DSM Axis I Clinical Syndromes occur more often in the context of Personality Disorders (PDs) (Shea, Widiger, & Klein, 1992); 2. patients with multiple clinical syndrome diagnoses often have PDs (Newman, Moffitt, Caspi,& Silva, 1998); and 3. even those patients who lack personality disturbances severe enough to warrant a DSM or ICD personality diagnosis often have clinically significant pathology, such as difficulties with intimacy, management of aggression or self-assertion, rejection-sensitivity, etc (Westen, 1997). There is little question that inclusion of a PD axis in the DSM and ICD, and its refinement through two decades of research, has been a crucial step in the evolution of more clinically and empirically useful diagnostic manuals. Knowing that a patient has major depression is certainly important, but adding the "qualifier" that the patient also has borderline PD is equally important because it has significant implications for prognosis and treatment. PDs have historically been in a tangential position among diagnostic syndromes, never having achieved a significant measure of recognition in the literature of either clinical psychiatry or abnormal psychology. Prior to the DSM-III and ICD-8, they were categorized in the official nomenclature with a mélange of other miscellaneous and essentially secondary syndromes. Today, PDs occupy a place of diagnostic prominence, having been accorded a contextual role in the multiaxial schema of the DSM. Personality pathologies comprise one of two required "mental disorder" axes in the DSM. Henceforth, clinicians must not only assess the patient's current symptomatology, indicated on Axis I, but also evaluate those pervasive features which characterize the patient's enduring personality pattern, recorded on Axis II. In effect, the revised American multiaxial format requires that symptom states no longer be assessed as clinical entities isolated from the broader context of the patient's lifelong style of relating, coping, behaving, thinking, and feeling - that is, his or her personality. Personality and its disorders are regarded as a potential diathesis (Tyrer, 2007). There are clinical theorists who assert that it is the patient’s personality that should be evaluated first; only secondarily should the patient’s clinical state be considered. There are substantive reasons for attending to the PDs first, beyond the pragmatics of adhering to official nosological requirements. Lifelong personality traits appear to serve as a substrate, as well as a context for understanding more florid and distinct forms of psychopathology. Since the early 1960s, most societies have been increasingly committed to the early identification and prevention of mental disorders. This emphasis has led clinicians to attend to both premorbid behavioural signs and the less severe variants of emotional disturbance. Ordinary anxieties, minor personal conflicts, and social inadequacies are now seen by many clinicians as the forerunners of more serious problems. A significant impetus to this movement is the emergence of community health centres whose attentions are directed to the needs of the less seriously disturbed. As a result of these developments, the scope of clinical psychopathology was broadened far beyond its historical province of "Hospital Psychiatry." As a field, it now encompasses the full spectrum of mild to severe mental disorders. With personality as a contextual foundation, diagnosticians have become more proficient in understanding personality dynamics and can more clearly trace the sequences through which both subtle and dramatic clinical symptoms unfold. PDs have been estimated to affect at least 10% of the population, and constitute a large percentage of the patients seen by psychiatrists. Yet unlike other diagnoses, PDs may or may not be associated with subjective symptoms. While some categories show high comorbidity with symptomatic diagnoses such as anxiety and depression, some PDs produce distress in other people rather than in the patient. But in either case, the overall functioning of patients with PDs is often marginally social, comparable in many cases to levels seen in patients with chronic conditions such as schizophrenia. Numerous studies suggest that PDs are underappreciated causes of social cost, morbidity, and mortality. PDs are associated with crime, substance abuse, disability, increased need for medical care, suicide attempts, self-injurious behaviour, assaults, delayed recovery from Axis I and medical illness, institutionalization, underachievement, underemployment, family disruption, child abuse and neglect, homelessness, illegitimacy, poverty, STDs, misdiagnosis and mistreatment of medical and psychiatric disorders, malpractice suits, medical and judicial recidivism, disruption of psychiatric treatment settings, and dependency on public support. The amount of social cost and disruption caused by the PDs is disproportionate to the amount of attention it gets in the public consciousness, in government research funding, in medical school education or even in psychiatric residency training And no less important than dealing with the social costs of personality disorders is the potential value inherent in preventive programs designed to enhance personality resilience and adaptive capacities. Personality is seen today as a complex pattern of deeply embedded psychological characteristics that are largely nonconscious and not easily altered, expressing themselves automatically in almost every facet of functioning. Intrinsic and pervasive, these traits emerge from a complicated matrix of biological dispositions and experiential learnings, and ultimately comprise the individual's distinctive pattern of perceiving, feeling, thinking, coping, and behaving. Personality is the patterning of characteristics across the entire matrix of the person. Rather than being limited to a single trait, personality regards the total configuration of the person’s characteristics: interpersonal, cognitive, psychodynamic, and biological. Each trait reinforces the others in perpetuating the stability and behavioural consistency of the total personality structure. For the personality disorders, then, causality is literally everywhere. Each domain interacts to influence the others, and together, they maintain the integrity of the whole structure. Personality disorders are not diseases or disorders in the usual medical disease sense. Rather, PDs are theoretical constructs employed to represent varied styles or patterns in which the personality system functions maladaptively in relation to its environment. When the alternative strategies employed to achieve goals, relate to others, and cope with stress are few in number and rigidly practiced (adaptive inflexibility), when habitual perceptions, needs, and behaviours perpetuate and intensify pre-existing difficulties (vicious circles), and when the person tends to lack resilience under conditions of stress (tenuous stability), we speak of a clinically maladaptive personality pattern, that is, a PD. Differentiating Normality and Abnormality Distinctions between normality and pathology are largely social constructions or cultural artefacts. Normality and pathology must be viewed as relative concepts; they represent arbitrary points on a continuum or gradient - no sharp line divides normal from pathological behaviour. Among diverse and ostensibly content- and culture-free criteria used to signify normality are a capacity to function autonomously and competently, a tendency to adjust to one’s social milieu effectively and efficiently, a subjective sense of contentment and satisfaction, and the ability to self-actualize or to fulfil one’s potentials throughout the life span into one’s later years. PDs were noted either by deficits among the preceding or by the presence of characteristics that actively undermine these capacities. Perhaps these criteria are too westernized or Eurocentric to be universal. In some Asian cultures, for example, where the individual is expected to subordinate individual ambitions to group consensus, the capacity to function autonomously might be praiseworthy, but the desire to do so is not. The traits which compose a number of personality styles are likely in certain historical periods or cultures, such as contemporary Western societies, to promote healthy functioning (e.g., Histrionic, Compulsive, Narcissistic traits). Similarly, in this society, there are personality styles and traits that are highly conducive to pathological functioning (e.g., Avoidant, Dependent, Masochistic). There are other personality patterns (e.g., Schizotypal, Borderline, Paranoid) which have a very small probability of falling at the normal end of the continuum in almost all cultures. The interest in the description of individual differences is very old. In Theophrastus’ Characters, written in the 3rd century BC, 32 different types of human beings are described, some of them familiar to clinicians nowadays (Theophrastus, 1998). In the fourth century B.C. Hippocrates concluded that all disease stemmed from an excess of or imbalance among four bodily humours: yellow bile, black bile, blood, and phlegm. Hippocrates identified four basic temperaments, the choleric, melancholic, sanguine, and phlegmatic; these corresponded, respectively, to excesses in yellow bile, black bile, blood, and phlegm. Although the doctrine of humours has been abandoned, giving way to scientific studies on topics such as neurohormone chemistry, its terminology and connotations still persist in such contemporary expressions as being sanguine or good humoured. Along the 19th century the concept of pathological personality was forged. Pinel in 1809 described his manie sans délire, that is to say, mental illness without symptoms of illness, to which he later on also referred as folie raisonnante, that is to say, madness without insanity. J.A. Koch who proposed, replacing the established label moral insanity, with the term psychopathic inferiority Koch used the term psychopathic, a generic label employed to characterize all personality diagnoses until recent decades, to signify his belief that a physical basis existed for these character impairments. The prime psychiatric nosologist at the turn of the century, Emil Kraepelin, did not systematize his thinking on PDs, but in his efforts to trace the early course of these syndromes, Kraepelin "uncovered" two premorbid types: the "cyclothymic disposition," exhibited in four variants, each inclined to maniacal-depressive insanity; and the "autistic temperament," notably disposed to dementia praecox. The best-known European classification of disordered personalities was proposed by Kurt Schneider. Schneider differed from many of his contemporaries, most notably the prime modern constitutionalist The best-known and perhaps most fully conceptualized of PDs are those formulated by psychoanalytic theorists. Their work was crucial to the development of an understanding of the causal agents and progressions that typify the background of these disorders. It was Sigmund Freud and his younger associates, Karl Abraham and Wilhelm Reich, who laid the foundation of the psychoanalytic character typology h. Although numerous analytic theorists have continued to contribute to the study of character, the contemporary work of Otto Kernberg deserves special note. Taking steps to develop a new psychoanalytic characterology, Kernberg constructed a framework for organizing personality types in terms of their level of severity to speak of "higher, intermediate and lower levels" of character pathology; both intermediate and lower levels are referred to as "borderline" personality organizations. Note should be made of another productive personologist who utilized a mathematical/factorial approach to construct personality dimensions, namely Raymond Cattell (Cattell, RB (1965)). His research has led him to identify 16 primary traits, which he then arranged in sets of bipolar dimensions that would undergird personality types. Other contemporary quantitative contributors include Peter Tyrer (Tyrer, 1988) and W. John Livesley (Livesley, 1987). In a model which seeks to draw on genetic and neurobiologic substrates, Robert Cloninger has proposed a complex theory based on the interrelationship of several trait dispositions. Another biosocial model using three pairs of evolutionary polarities as a basis is one developed by Theodore Millon. Here, he derived a PD taxonomy that subsumed the dependent, independent, ambivalent, and detached coping styles with an activity-passivity dimension. Notably, in their recent work, numerous theorists have begun to turn their attention to positive mental health, speaking of personality resilience and adaptive capacities. The Current Official Systems, ICD-10 and DSM-IV TR Two classificatory systems of mental disorders are recognized internationally today, namely, the Diagnostic and Statistical Manual of Mental Disorders - 4th Edition-Text Revised (DSM-IV-TR)19 and the International Classification of Mental and Behavioural Disorders (ICD-10)20. Personality disorders are given important weight in both classifications. The DSM-IV-TR places them in its separate Axis II (this classification comprises five such axes). The personality disorders in the DSM are grouped into three clusters, based essentially on empirical descriptive similarities; this cluster grouping has not (and maybe never will be) been satisfactorily validated but its widespread use indicates a frequent wish to reduce the number of categories. Cluster A includes paranoid, schizoid and schizotypal personality disorders (the so-called odd or eccentric individuals), Cluster B comprises antisocial, borderline, histrionic and narcissistic PDs (the ostensible dramatic, emotional or erratic individuals), and Cluster C includes avoidant, dependent and obsessive-compulsive PDs (anxious/fearful individuals). A last category, "PD not otherwise specified," comprises disorders of personality that do not fulfil the specific criteria for any of the above individual PDs. The ICD-10 Classification includes a single section covering all personality abnormalities and persistent behavioural disturbances. This is separated into specific named personality disorders, mixed and other personality disorders, and enduring personality changes. The individual personality disorders are paranoid, schizoid, dissocial, emotionally unstable (impulsive and borderline types), histrionic, anxious (avoidant), anankastic and dependent ones. Two more categories are "other specific PDs" and "PD, unspecified." The ICD classification is similar to that of DSM-IV, although differences are noteworthy. For example, the borderline PD of the DSM-IV is subsumed as one of the two emotionally unstable disorders in ICD-10, the obsessive-compulsive adjective in DSM-IV is retained as "anankastic" in ICD-10, and avoidant personality disorder is only a partial equivalent of the ICD-10 anxious personality disorder. Two more disorders included in the official section of the DSM-IV are excluded from ICD-10; schizotypal disorder is a variant within the schizophrenia spectrum of conditions in ICD-10 and narcissistic personality disorder is only mentioned in the section on "other specific PDs" in ICD-10, without any specific criteria noted for this diagnosis. The ICD-10 contains other general categories that refer to PDs that have no counterpart in the DSM-IV, such as "mixed disorders" and "other disorders of adult personality and behaviour." Five broad sources of information are available to help describe the clinical problem; each has its own advantages and limitations. The first comprise clinical interviews and observations; the clinician observes and asks the questions and the subject responds verbally, often in a free-form style. The clinician is free to follow any particular line of questioning desired and usually mixes standard questions with those specific to the current problem. The second is structured or semi-structured interviews. Open ended, free form style clinical interviews may provide insufficient information to assess the different personality disorders. Interviewer- administered interviews, structured or semi-structured, systematically address and assess each personality disorder criteria with standard questions or probes. The most often used are International Personality Disorders Examination (IPDE), Structured Clinical Interview for DSM-IV Axis II Personality Disorders (SCID-II) and The third second are formal rating scales and checklists; a person familiar with the subject completes those forms in order to provide an objective perspective. Rating scales and checklists often serve as a memory aid, ensuring that everything relevant to the disorder is included in developing a treatment plan. Rating scales usually have more items than the diagnostic criteria for the same syndrome and are usually held to a higher standard of scientific rigor. Because they have more items, they provide more fine-grained measurements, but they also take more time to complete. For example, the revised Psychopathy Checklist (PCL-R) consists of 20 items, whereas the DSM-IV offers only seven criteria for the diagnosis of antisocial PD. Although the PCL-R is widely used in the study of psychopathy, few rating scales exist for use with other PDs. The third source is the self-report inventory; subjects literally report on themselves by completing a standard list of items. Because self-reports represent the subject’s own responses, they can be especially valuable in quickly identifying clinical symptoms. Unless the individual is violent or psychotic, a self-report inventory can be given at any point during the clinical process, often with minimal supervision. A profile obtained at the beginning of therapy, for example, can be used as a baseline to evaluate future progress. A number of other self-report instruments are available. The Tridimensional Personality Questionnaire (TPQ),, Millon Clinical Multiaxial Inventory (MCMI-III), Neuroticism-Extroversion-Openness- Personality Inventory Revised (NEO-PI-R), The Dimensional Assessment of Personality Pathology-Basic Questionnaire (DAPP), The Schedule of Nonadaptive and Adaptive Personality (SNAP) are the most often used self-report assessment instruments. The fourth source of information is projective techniques, an attempt to access unconscious structures and processes that would not ordinarily be available to the subject at the level of verbal report. These techniques seek to draw out internal, and frequently unconscious, influences on behaviour by presenting the subject with inherently unstructured, vague, or ambiguous situations. The Rorschach Inkblot Test is the classic example. The subject is presented with a series of 10 blots in turn and asked to report what he or she sees. The Thematic Apperception Test uses pictures of various interpersonal situations. The subject constructs a story to explain what is happening in the picture, what led up to these events, and how matters will end. Because projective instruments are time-consuming and not widely regarded as being as psychometrically sound as self-report inventories, their use has waned in recent years, especially with the economic constraints of managed care. Finally the use of inmates (informants) of the subject, perhaps a spouse, teacher, parent, or good friend, someone who can provide perspective on the problem, might also be considered another important source of information. Problems in the current classification The official classification systems reflect a variety of personality related issues that are likely to be solved in the near future with the revision of both systems. First, there is the question of the retention of personality disorders on a different axis (Axis II) from that of clinical syndromes (Axis I) in the DSM-IV. The division between Axis I and Axis II seems to some to be arbitrary and not justified adequately. A second persistent problem is the classificatory status of the individual categories of personality disorder. There is great overlap between the criteria for diagnosing personality disorders in both DSM-IV and ICD-10 and this seriously compromises their validity as separate disorders. Clear differentiation between the disorders is often difficult and many individuals diagnosed with a personality disorder have several other personality disorders that do not always appear to be fundamentally different. A third issue is the overlap of some personality disorders with disorders in Axis I. An example is the relationship between avoidant PD and generalized social phobia, both of which address the same group of symptoms without a clear distinction between them. Although still included in the Appendix B of DSM-IV, there seems to be a similar problem between depressive personality disorder (Axis II) and dysthymia (Axis I). A fourth question is how many personality disorders deserve separate description in the two classification systems? It is also uncertain what type of criteria should constitute the building blocks of personality disorder and how many of them are needed for each diagnosis. Both classifications rest mainly on historical traditions and committee consensus rather than on empirical data or well-constructed theoretical grounds. Many of the assumptions of each classification are implicit or covert and need to be exposed so that diagnosis can be made consistently and subjected to systematic testing. Fifth, there are also many questions about the division between "normal" personality and personality disorders that need answering and whether it is wise to have a division at all. Sixth, another major controversy in the field is the categorical/dimensional/prototypical controversy, to which we will turn shortly. A further issue is the polythetic criterion lists used in current classification systems; these produce considerable intragroup variability such that two people with the same diagnosed PD may display very different features because they score for different sections. Finally seventh , as already mentioned, PDs are tied to cultural variables to a much greater extent than the clinical disorders in Axis I, creating difficulties when diagnosing this kind of disorders across different cultures, a topic we will also address in a later section. Given the need for a clear unambiguous official classificatory system for personality disorders and the dissatisfaction with the current two systems, there are likely to be important changes in the classification of personality disorders in DSM-V and ICD-11. Perhaps the most important question is "how do we improve the clinical utility of the classification of personality disorders so that it is recognised to be helpful in decision-making at all levels?" Each of the Personality Disorders The Personality Disorders are grouped into three clusters based on descriptive similarities. Cluster A includes paranoid, schizoid and schizotypal personality disorders (the so-called odd or eccentric individuals), Cluster B comprises antisocial, borderline, histrionic and narcissistic PDs (the ostensible dramatic, emotional or erratic individuals), and Cluster C includes avoidant, dependent and obsessive-compulsive PDs (anxious/fearful individuals). A general definition of personality disorders are provided in DSM-IV-R and in ICD-10. It can be useful to psychiatrists and clinical psychologists, because the most common diagnosis in clinical practice is the diagnosis "not other specified" (Clark et al. 1995) General criteria diagnostic criteria for a Personality Disorder (ICD-10) A specific personality disorder is a severe disturbance in the characterological constitution and behavioural tendencies of the individual, usually involving several areas of the personality, and nearly always associated with considerable personal and social disruption. Personality disorder tends to appear in late childhood or adolescence and continues to be manifest into adulthood. It is therefore unlikely that the diagnosis of personality disorder will be appropriate before the age of 16 or 17 years. General diagnostic guidelines applying to all personality disorders are presented below; supplementary descriptions are provided with each of the subtypes. Conditions not directly attributable to gross brain damage or disease, or to another psychiatric disorder, meeting the following criteria: (a) markedly dysharmonious attitudes and behaviour, involving usually several areas of functioning, e.g., affectivity, arousal, impulse control, ways of perceiving and thinking, and style of relating to others; (b) the abnormal behaviour pattern is enduring, of long standing and not limited to episodes of mental illness; (c) the abnormal behaviour pattern is pervasive and clearly maladaptive to a broad range of personal and social situations; (d) the above manifestations always appear during childhood or adolescence and continue into adulthood; (e) the disorder leads to considerable personal distress but this may only become apparent late in its course; (f) the disorder is usually, but not invariably, associated with significant problems in occupational and social performance. For different cultures it may be necessary to develop specific sets of criteria with regard to social norms, rules and obligations. For diagnosing most of the subtypes listed below, clear evidence is usually required of the presence of at least three of the traits or behaviours given in the clinical description. For additional information and references see Module II in Simonsen E, Ronningstam E, Millon T (Eds). (2007). WPA ISSPD Educational Program on Personality Disorders. www.wpanet.org/education/education.shtml: Henning Sass & Reinhild Schwarte: Schizoid Personality Disorder (pp. 129-133) Schizoid Personality Disorder. Svenn Torgersen: Schizotypal Personality Disorder (pp. 134-141). Elizabeth Iskander & Larry J. Siever. Paranoid Personality Disorder (pp. 110-116) Schizoid Personality Disorder (partly adopted from Henning Sass & Reinhild Schwarte ) Jacob is a 26 years old man. Despite extraordinary intelligence John was not able to complete or participate in any educational program. He wanted to have a normal life with a family and friends, but thought that he was rootless and he felt that other people thought that he was peculiar or odd. He felt that he was outside. As a child he went to various schools because his parents moved around. He was thought of as a lonely wolf and did not participate in the social life or games of sports with his peers. During school class he was often absent minded being absorbed in his own thoughts and fantasies. From around the age of thirteen he became interested in computers and was quite advanced in his understanding of mathematics. He became exceedingly isolated with his computer as his sole companion. This vignette schizoid personality illustrates the difficulties how to establish a stable relationship to significant others like peers and family. Often it is regarded as unusual that a person with schizoid personality disorders complains by himself or herself to be isolated. Many schizoid patients, in the contrary, claim to be quite satisfied with their loneliness and it is quite unusual that he wish to have a family. Also schizoid persons usually accept their situation or even deny any desire for closer relationships. Diagnostic Criteria ICD-10 F60.1 Schizoid personality disorder Personality disorder meeting the following description: (a) few, if any, activities, provide pleasure; (b) emotional coldness, detachment or flattened affectivity; (c) limited capacity to express either warm, tender feelings or anger towards others; (d) apparent indifference to either praise or criticism; (e) little interest in having sexual experiences with another person (taking into account age); (f) almost invariable preference for solitary activities; (g) excessive preoccupation with fantasy and introspection; (h) lack of close friends or confiding relationships (or having only one) and of desire for such relationships; (i) marked insensitivity to prevailing social norms and conventions. Excludes: Asperger's syndrome (F84.5) delusional disorder (F22.0) schizoid disorder of childhood (F84.5) schizophrenia (F20. - ) schizotypal disorder (F21) The central feature of Schizoid Personality Disorder (SPD) is a pattern of pervasive social detachment and a narrow range of emotional expression in social settings. The DSM-IV criteria for SPD differ in detail in three criteria from the ICD-10 diagnostic criteria. The both describe the SPD by seven criteria, of which at least three must be applicable. The SPD is most clearly defined within relationships. Individuals with this disorder are characterized by a profound defect in their ability to form personal relationships or to respond to others in an emotionally meaningful way and appear to lack a desire for intimacy. They are introverted, aloof, and seclusive, and select activities that do not include much interaction with others. This style of life easily results in social isolation. The differential diagnosis of SPD includes: 1. a normal preference for solitary pursuits that does not meet the criteria for schizoid personality disorder; 2. schizophrenia (in which further characteristic negative or positive symptoms occur); The SPD appears to characterize the negative symptoms of schizophrenia, e.g., social, interpersonal, and affective deficits like little affect, low energy, anhedonia, diffidence about, shyness in, or detachment from relationships. 3. schizotypal personality disorder (in which there are cognitive and perceptual distortions); In contrast to the schizotypal personality disorder the SPD does not include psychotic-like cognitive/perceptual distortions. 4. paranoid personality disorder (in which the patient displays suspiciousness and paranoid ideations); 5. avoidant personality disorder (in which the patient has a fear of being embarrassed or inadequate, with excessive anticipation of rejection); 6. obsessive-compulsive personality disorder (in which there may be apparent social detachment that arises from devotion to work and discomfort with emotions; capacity for intimacy is usually preserved); 7. disorders of more severely impaired social interaction, stereotyped behaviours and limited interests (e.g., autistic disorder, Asperger’s disorder); 8. personality change caused by a general medical condition (e.g., temporal lobe epilepsy); personality symptoms derived from chronic substance use (Sass, 2007). The most frequent co-occurring personality disorders with SPD are schizotypal and avoidant personality disorders and to a lesser degree paranoid, antisocial and borderline personality disorders (Kalus et al. in Livesley, 1995, p.65). The highest co-occurrences may perhaps be because of the high overlap between the two criteria sets. The SPD and the schizotypal personality disorder, for example, share the important criteria of social isolation and restricted affect. Also the avoidant personality disorder may seek isolation, but individuals with SPD will tolerate the separation with comfort, while individuals with avoidant personality disorder will be distressed and lonely. SPD can an antecedent disorder to schizophrenia, major depression, dysthymia or a delusional disorder. Further it shows high comorbidity with social phobia and agoraphobia. If people with SPD are detached from a supportive family they often become involved with drugs and alcohol. SPD is uncommon in clinical treatment settings. SPD is diagnosed more frequently in males who seem to be more impaired than females with SPD. The etiology of SPD has not been established. A close genetic relationship to schizophrenia has been proposed but is doubtful. Conversely, introversion has been shown to be a highly heritable personality trait. Psychological theories suggest sociocultural factors in the genesis of the disorder: In the psychodynamic approach, the SPD emerges from inadequacies in earliest relationships with parental figures. The cognitive approach suggests that the most important source of dysfunctional behaviour and affects lie in incorrect attributions that people make. As patients with SPD have few complaints and do not seek an interpersonal context for solving their problems, they rarely seek therapy. The disorder is most likely to come to medical attention in the course of intervention for another condition, in response to acute stressors or because of family influence. Others who come into treatment are forced to do so by family or even the legal system. Acutely stressful situations often require crisis intervention. Aims of long-term psychotherapeutic interventions are to maintain stability and support, to improve social skills and comfort, to help maximize quality of an isolated lifestyle. In treatment, clients with SPD challenge service providers with the absence of response. As they do not response to emotional leverage, therapists easily feel frustrated and ineffective. The contact between therapist and patient should be an important element of the therapy. An important step of the therapy should be to open possibilities to make new experiences and changes (Saß and Jünemann, 2001). The therapist should be aware that major changes and modifications of character structure are unlikely. The therapy should be aimed at achieving modest reductions in social isolation and in prompting more effective adjustment to new circumstances (Kalus et al. in Livesley, 1995). Behavioural psychotherapy can be helpful for some patients including, for example, methods such as problem solving, social skills training or role plays. Educational strategies may be effective in working with individuals with SPD to identify (1) their own emotions; (2) the emotions they elicit in others; and (3) possible feeling states of people with whom they relate. Intervention with individuals with SPD may include methods of cognitive therapy, e.g., exploring their self-concept and sense of where they belong in the world. Confrontation should clarify the relation of emotions to thinking and encourage these clients to be present with reality. Individual psychoanalytically oriented psychotherapies are less likely to succeed (Kalus et al. in Livesley, 1995, p.66). Most psychopharmacological interventions apply to comorbid disorders such as depression or anxiousness. Schizotypal Personality Disorder (partly adopted from Svenn Torgersen) A 37 year old, unemployed man claimed of recurrent irrational thoughts, compulsive behaviour, and social isolation. Since his childhood he had always been eccentric, withdrawn with no real friends anxiously fearing closer relationships, preoccupied with reading stories about Dracula and other myths. He didn’t share his inner thoughts or feeling with anybody, including his parents. He never finished an education, but worked in factories, often at night. Some years earlier he started doubting if his work was accurate enough. Although he recognized these thoughts as irrational, he started spending a lot of time controlling his work over and over again. Soon these compulsive controls took so much time that he could not finish his work, was continuously annoyed by intrusive vivid homosexual images, was preoccupied with doubts concerning almost everything at home and also he had to look persistently at people in order to be sure to maintain their images in his memory. He started fearing that people could notice his behaviour, and he felt that unknown people were staring at him and that they secretly were making fun of him. He complained of being unable to reveal his feelings and thoughts to other people and felt isolated. He started drinking alcohol to control his increasing anxiety. He adopted different peculiar strategies, which ended in new vicious circles of obsessive symptoms and suspiciousness. This case is diagnosed with obsessive-compulsive disorder (OCD), Alcohol abuse and Schizotypal Personality Disorder. He had long lasting personality difficulties like suspiciousness, odd behaviour and social anxiety prior to the OCD symptoms. Comorbidity is often seen in Schizotypal Disorder, and it is the axis I disorders that usually brings the patients to treatment. The historical roots of schizotypal personality disorder (STPD) are the non-psychotic personality syndromes within the spectrum of schizophrenia. The definition of schizotypal personality disorder has remained more or less the same during the revisions of DSM and consists in DSM-IV of the following criteria: (1) ideas of reference (excluding delusions of reference), (2) odd beliefs and magical thinking that influences behaviour and is inconsistent with subcultural norms (e.g., superstitiousness, belief in clairvoyance, telepathy, or "sixth sense;" in children and adolescents, bizarre fantasies or preoccupations), (3) unusual perceptual experience, including bodily illusions, (4) odd thinking and speech (e.g., vague, circumstantial, metaphorical, over elaborate, or stereotyped), (5) suspiciousness or paranoid ideation, (6) inappropriate or constricted affects, (7) behaviour or appearance that is odd, eccentric, or peculiar, (8) lack of close friends or confidants other than first-degree relatives, (9) excessive social anxiety that does not diminish with familiarity and tends to be associated with paranoid fears rather than negative judgment about self. ICD-10 included schizotypal disorder among the psychoses and defined it partly similarly, partly differently from DSM. The only difference between DSM-IV and ICD-10 is that DSM-IV includes "ideas of reference" and "excessive social anxiety," while ICD-10 includes obsessive rumination and micropsychoses. However, ideas of reference are close to suspiciousness, and micropsychoses are close to unusual perceptual experiences, so the only real difference is social anxiety and obsessive ruminations. As evolving from the spectrum of schizophrenia, the boundaries between schizotypal personality disorder and schizophrenia are not easy to define. The prodromal symptoms of schizophrenia are similar to the schizotypal personality disorder. Thus, retrospectively, "premorbid" may be added to STPD, according to DSM-IV. When fully developed hallucinations and delusions are presented during a one -month period, the diagnosis is schizophrenia. However, a person with delusions or hallucinations plus negative symptoms may show a clinical picture similar to STPD; even so schizophrenia is the correct diagnosis, if the duration requirements are fulfilled. Even more difficult is the differentiation between simple schizophrenia and STPD in ICD-10. In practice, the differentiation is impossible, as the criteria for simple schizophrenia, personality changes, negative symptoms: and reduced social function is indistinguishable from the early developmental phase of STPD. However, simple schizophrenia requires change, while STPD implies no clear starting point. The possible early start of STPD, however, may make it difficult to distinguish STPD from milder forms of pervasive developmental disorders (autism). As to other psychotic disorders, the manifestation of full-blown delusions (not only ideas of reference and suspiciousness) and hallucinations (not only illusions) preclude any diagnosis of STPD. The boundaries between STPD and borderline personality disorder are of course difficult to draw, as both personality disorders emerged from the same borderline psychoses concept. They share the pseudo-psychotic and paranoid features, and quite a few people may live an unstable and turbulent life similar to those with borderline personality disorder. Even so, the impulsivity and affective intensity and variability in the borderline personality disorder are not part of the STPD criteria set. Furthermore, those with borderline personality disorder are not expected to display the socially inept and chronically withdrawn pattern of STPD. Instead, some people with borderline personality disorder may withdraw when they get older, as a consequence of using up the patience of their acquaintances and having experienced a brimful of disappointments in their partnerships and relationships. STPD is close to schizoid personality disorders. The two disorders share the social isolation and the constricted affects. However, STPD has the oddness and the pseudo-psychotic features in addition. In the same vein, those with paranoid personality disorder share the paranoid features with STPD, but not the withdrawal, oddness and pseudo-psychotic features. STPD shares the social anxiety and the tendency to withdrawal with avoidant personality disorder, but not the eccentricity, paranoid features and illusions. Avoidant, paranoid and borderline personality disorders were especially highly correlated to STPD. STPD is associated with psychotic disorders including schizophrenia . Furthermore, there seems to be an association with obsessive compulsive and phobic disorders. There may also be an association with dysthymic disorder, panic disorder, somatoform disorders and eating disorders. Relatively few studies of the prevalence in the general population have been performed. The samples are seldom quite representative, and differently structured interviews are applied, based on different editions of DSM. The most representative studies show a prevalence of 0.7 (Maier et al. 1992) and 0.6 (Torgersen et al. 2001). Those with a higher number of schizotypal traits have less education and more often live alone in the city centre compared with those with a lower number. STPD seems thus more prevalent among men in clinical samples. A meta-analysis of the so-called "Big-Five" and personality disorders showed that what characterized those with STPD were first and foremost Neuroticism, second Introversion and third Non-Agreeableness. The pattern was similar to paranoid and borderline personality disorders in Neuroticism and Non-agreeableness, and similar to avoidant personality disorder in Neuroticism and Introversion. Furthermore, STPD was similar to schizoid personality disorder in Introversion, to antisocial and narcissistic personality disorders in Non-agreeableness, and to dependent personality disorder in Neuroticism. There were no similarities to histrionic and obsessive-compulsive personality disorders. Together with borderline personality disorder, those with STPD were extreme on most personality disorders, three out of five dimensions. The results of the studies of the relationships between STPD and personality dimensions fit in with the large overlap between STPD and paranoid, avoidant and borderline personality disorders. A study of the relationships between personality disorders and Cloninger’s temperament and character scales suggests that STPD is negatively correlated to Self-directedness and Cooperation, and positively correlated to Self-transcendence. The results illustrate the vulnerable, withdrawn and psychotic-like aspects of STPD. Even if STPD is correlated to common personality dimensions one cannot jump to the conclusion that STPD is a construct based on these dimensions. It may be that those with schizotypal traits simply answer in an extreme way when these dimensions are measured by the questionnaires. Then we approach the question about the categorical or dimensional nature of schizotypal features. Some statistical analyses suggest that that a latent discontinuity underlies the variation in schizotypal traits (Lenzenweger & Korfine, 1995). Others believe more in a dimensional model of schizotypy, with poorly functional individuals at one end of the dimensions, and well-functioning individuals among those with somewhat lower scores on schizotypal inventories (Goulding, 2004). Those more poorly functioning are more anhedonic and with more cognitive disturbance, while those well-functioning are more characterized by unreal experiences. STPD is genetically influenced as are other personality disorders (Torgersen 1986; Torgersen et al. 2000; Kendler & Hewitt, 1992). This is also the case for schizotypal traits in children (Coolidge et al. 2001). However, what is especially important is the genetic relationship to other mental disorders. Some studies suggest a familial relationship between STPD and the whole realm of psychoses (Squires-Wheeler et al. 1989; Kendler et al. 1995). As STPD evolved out of the familial schizophrenic spectrum, the genetic relationship to schizophrenia is of particular interest. Studies of co-twins of schizophrenic patients (Torgersen, 1992), and biological relatives of adopted-away schizophrenics (Kendler & Gruenberg, 1984) confirmed the specific familial and genetic relationship between STPD and schizophrenia. No other personality disorders seem to be consistently related to schizophrenia. However, STPD as defined by DSM does not seem to cover adequately the schizophrenia-related STPD. Those adopted-away offspring of schizophrenics who develop personality disorders seem to experience frequent somatoform complaints and poor social function in addition to withdrawal and emotional constriction (Gunderson et al. 1983). In fact, STPD consists of two syndromes that may be independently inherited (Siever, 1995; Kendler and Hewitt, 1992), a constricted/eccentric syndrome that is characterized by odd and eccentric appearance and behaviour, thoughts and communication, and a psychotic-like syndrome that is characterized by ideas of reference, magical thinking, illusions and depersonalization/derealization. While the former syndrome seems to be genetically related to schizophrenia (Torgersen, 1993), the latter is not. Even if there might exist a familial relationship between STPD and affective disorder, a genetic relationship to major depression is not confirmed. We do not know what environmental factors influence the development of STPD. A retrospective study showed that those with STPD more often reported neglectful parenting from both parents, which means little love and also little control (Torgersen & Alnæs, 1992). Those with borderline personality disorder more often reported affectionless control, meaning little love and much control. Those with other personality disorders more often experienced affectionate constraint; much love and much control, while those without personality disorder reported optimal parenting; much love and little control. Course and prognosis A Norwegian twin study showed that those with STPD had poor social as well as occupational adjustment (Torgersen, 1986). Skodol et al. (2002) found dysfunction in relation to parents, sibs, and friends, occupational dysfunction, and dysfunction in relation to more distant family members among those with schizotypal personality disorder. Quality of life is also reduced among those with STPD (Cramer et al. 2003). They have a poor subjective well-being, poor self-realization, less contact with friends and family, less social support, a lot of negative life events, poor neighbourhood quality, and generally a poorer global quality than those without STPD in the general population. Among the personality disorders, nobody displayed poorer quality of life than those with STPD. The neuropsychological and biological fundamentals of STPD are far from settled. Even so, some results are forthcoming. There seems to be a difference between the constricted/ eccentric and the psychotic-like STPD syndromes. Neuro-psychological tests measuring attention and information processing observe impairment among those with constricted/eccentric traits (Siever, 1995). Indication of a low dopamine level are found among those with the constricted/eccentric syndrome, for instance by a low concentration of homovannilic acid (HVA). An adequate dopamine activity is necessary for maintenance of working memory, a function necessary for social engagement as well as other executive functions. The deficient information processing may contribute to the social withdrawal, emotional constriction and eccentricity among those with STPD. On the other hand, those with the psychotic-like syndrome seem to have an exceptionally high level of dopamine-activity, as also demonstrated in a high concentration of HVA. The increased dopaminergic activity may explain the psychotic-like traits such as illusions, paranoid ideations etc. Usually psychotherapeutic approaches are applied for patients with STPD. No controlled results are published. However, from clinical experience there are some precautions that are important to take into account. Some less experienced clinicians may be fascinated with all the grotesque and symbol-rich material patients with STPD may produce. They show interest, ask for details and encourage the patient to tell more. This can be great for the clinician, but hardly helpful for the patient. The patient may slide even more into the disturbing inner fantasies. A better approach is to dedramatize the strange thoughts and pictures, not reject, if the patient is active in telling, not refrain from showing a strong interest in the material. Instead, it is important for the patient to learn social skills, to discuss what went wrong in interpersonal situations, what behaviour is common and appropriate. As to pharmacotherapy, the best approach is to treat the axis-I disorder in cases where those with STPD have it in addition. If the clinical picture is dominated by psychotic-like features, neuroleptic may be the treatment of choice. There are some indications that blocking of dopaminergic activation may help those with psychotic-like traits. On the other hand, those with constricted/eccentric features may be helped by drugs that functions like amphetamine - releasing dopamine and blocking its reuptake. Paranoid Personality Disorder (partly adopted from Elizabeth Iskander & Larry J. Siever) A 36 year old divorced worker developed a severe depression after he was fired from his job and subsequently had severe alcohol problems. He presented himself to the general practitioner with somatic complaints, anxiety, compulsively washing his hands, fatigue, disturbing inner feelings of hatred towards other people. His troubles started in his childhood. He reported that he was very aggressive towards other children and he was involved in recurrent conflicts. At home he was constantly on guard. In his work relations he was involved in severe interpersonal conflicts, reacting with aggressive attacks at the slightest offences. The last years he spent working, he was continuously involved in conflicts with his colleagues. After a short contact with a female colleague who terminated the relationship with him. The only person he stayed friends with was his brother-in-law who lived a hundred kilometres away. This vignette illustrates important issues and characteristic features of the paranoid personality. First, they do not seek treatment unless they are in a crisis (fired from job) or because of additional pathology (depression). Second, when decompensated they most often get depression, panic attacks, OCD, somatoform disorder as in this case or in other cases an additional alcohol abuse. Third, the vignette may support a psychodynamic formulation of key elements in his personality functioning. His personality pathology is excessive aggression and mistrust. Clinical Description Diagnostic Criteria ICD-10 F60.0 Paranoid personality disorder Personality disorder characterized by: (a) excessive sensitiveness to setbacks and rebuffs; (b) tendency to bear grudges persistently, i.e., refusal to forgive insults and injuries or slights; (c) suspiciousness and a pervasive tendency to distort experience by misconstruing the neutral or friendly actions of others as hostile or contemptuous; (d) a combative and tenacious sense of personal right s out of keeping with the actual situation; (e) recurrent suspicions, without justification, regarding sexual fidelity of spouse or sexual partner; (f) tendency to experience excessive self-importance, manifest in a persistent self-referential attitude; (g) preoccupation with unsubstantiated "conspiratorial" explanations of events both immediate to the patient and in the world at large. lncludes: expansive paranoid, fanatic, querulant and sensitive paranoid personality (disorder) Excludes: delusional disorder (F22. - ) schizophrenia (F20. - ) Paranoid personality disorder is a clinically well-recognized disorder that has not, however, been the object of a great deal of investigation. Although noted in the writings of psychiatrists since the late 1800's, the condition was first called "paranoid personality" by Kraepelin in 1921 (Akhtar, 1990). The hallmark criteria regarding paranoid personality disorder (PPD) are distrust and suspiciousness of others such that others are seen as purposefully attempting to harm one in some way without any evidence to suggest this is the case. Those with paranoid personality disorder also may be very critical of others, argumentative and rigid in beliefs, again stemming from harbouring unwarranted suspicions about people around them. This often leads to problems with relationships, both personal and in the work place. The ICD-10 lists seven criteria (see above) of which only three must be met. The current criteria for diagnosing paranoid personality disorder in DSM IV-TR includes seven symptoms of which at least four must be met. Most are essentially the same as the ICD criteria. These include suspicion that others are harming or deceiving one in some way, preoccupation with doubts about the loyalty of friends, reluctance to confide in others out of fear that information may be used against them, reading threatening meaning into benign events, bearing grudges over insults or slights, hasty and angry reaction to perceived attacks on character, and unjustified suspicion regarding the fidelity of a spouse or partner. There is one criterion that does not exist in the DSM IV and that is "tendency to experience excessive self-importance, manifest in a persistent self-referential attitude." This item, basically implying a level of grandiosity, also did not exist in the DSM III or III-R versions. Paranoid personality disorder must be diagnosed to the exclusion of schizophrenia, or any other psychotic disorder including psychosis in the context of a mood disorder. Paranoid personality disorder is considered "premorbid" if it is present prior to an Axis I psychotic disorder. There is substantial comorbidity of Axis I disorders; individuals with paranoid personality disorder appear to have an increased likelihood of developing depression, agoraphobia, obsessive compulsive disorder and alcohol or substance abuse or dependence. With regard to comorbid personality disorders, there is some variation in the literature. Generally though, it has been suggested that in clinically based samples, over 75% of patients who met paranoid personality disorder criteria also met criteria for other personality disorders, the most common were found to be schizotypal and narcissistic. One area of research is the possible relationship of PTSD with paranoid personality disorder. When 180 outpatients were analyzed using the DSM III-R, subjects with paranoid personality disorder had a higher rate of comorbid PTSD than subjects without the disorder (29% compared with 12%) (Golier et al. 2003). In addition, they had elevated rates of physical abuse and assault in childhood and adulthood (54% compared with 35%). This suggests a possible link between trauma during early events in life and subsequent paranoid behaviour and mistrust. Another area that has received some attention is the relationship of violence to paranoid personality disorder. Paranoid cognitive personality style was found to increase the risk of violence in subjects with personality disorders, particularly schizophrenia spectrum disorders (Nestor, 2002). According to the DSM-IV, the prevalence of paranoid personality disorder was 0.5 to 2.5% in the general public, and more common in males. Interestingly, the 1997 National Survey of Mental Health and Wellbeing (conducted in Australia) using the ICD-10 to assess personality disorders found a 1.34 % prevalence of paranoid personality disorder and no sex difference, despite the similarities in criteria between the DSM and the ICD. There is some current evidence that paranoid personality disorder may be more difficult to diagnose than other personality disorders. A study of interrater reliability using DSM IV achieved good agreement. However, in the same study, when analyzing test-retest reliability based on how consistent a patient’s report is from one clinician to another, and how information is interpreted and scored, paranoid personality disorder had the lowest reliability of all the personality disorders (Zanarini et al. 2000). It has been suggested that paranoid personality disorder may be related to certain Axis I disorders, including schizophrenia and delusional disorder. Kendler found a much higher risk of paranoid personality disorder in first degree relatives of those with delusional disorder as opposed to relatives of those with schizophrenia, 4.8% compared to 0.8% (Kendler et al. 1985). On the other hand, paranoid personality disorder was significantly more common in the biologic relatives of patients with schizophrenia when compared with relatives of controls (Kendler et al. 1982). Using data from the Roscommon family study, an epidemiologic study conducted in Ireland, it was discovered that biological relatives of those with schizophrenia had a significantly higher amount of paranoid personality disorder compared with relatives of controls (Kendler et al. 1993). As with other disorders, cultural factors must be taken into account in diagnosing this disorder. There are some groups that might, for reasons of maltreatment, language barriers, and unfamiliarity to this society, display what could be labelled paranoid traits. Those groups include: minority groups, immigrants and refugees. In an epidemiologic study recently completed on personality disorders, minorities such as blacks, Hispanics and Native Americans were at greater risk for having paranoid personality disorder than whites (Grant et al. 2004). Also according to the same study, paranoid personality disorder was more common among younger people (18-29), those with lower incomes, and those who were divorced or never married. Some of these findings are not surprising, taking into account the nature of paranoid personality disorder. However, this does bring up the question of which came first: Are some paranoid traits the result of maltreatment by others due to socioeconomic status, race, etc., or does the disorder contribute to, for example, inability to succeed professionally or remain in a relationship? There appears to be a combination of both, which can contribute to complications in diagnosing the disorder. - Course and Prognosis Paranoid personality disorder can be noted first in childhood; symptoms observed include solitariness, social anxiety and odd thoughts and language. There is not a lot of data regarding the course and prognosis of the disorder. This is likely due to the fact that as it is a personality disorder, it tends to be stable over adult life and although it can cause interpersonal problems, does not often require treatment. It has been observed that the course of the disorder rarely worsens or goes into remission (Akhtar, 1990). There is no specific treatment or medication for paranoid personality disorder. When existing in conjunction with other personality disorders, i.e., borderline personality disorder, treatment may be sought but that is primarily due to symptoms experienced in other personality disorders. There is some data on the effectiveness of day treatments for patients with personality disorders in general (Karterud et al. 2003). Treatment results, although effective for some personality disorders (i.e., borderline), were the poorest for those with paranoid, schizoid, and schizotypal personality disorders. Cluster B includes four personality disorders: Antisocial (ASPD), Borderline (BPD), Histrionic (HPD) and Narcissistic (NPD). According to DSM IV-TR individuals with these disorders appear dramatic, emotional or erratic. For additional information and references see Module II in Simonsen E, Ronningstam E, Millon T (Eds). (2007). WPA ISSPD Educational Program on Personality Disorders. www.wpanet.org/education/education.shtml: Hart S., Cooke D. Antisocial Personality Disorder (pp. 60-66); Bateman A., Fonagy P. Borderline Personality Disorder (pp. 74-83); Pfohl B. Histrionic Personality Disorder (pp. 90-94) and Ronningstam E: Narcissistic Personality Disorder (pp.95-103 ). (adopted from Stephen Hart & David Cooke) - Case vignette This is a 27 year old male who committed murder at age 17. He stayed in a high-security hospital for 10 years and started individual treatment after being released. He was an intelligent boy who did well in school until his peers began to tease him. This made him feel helpless and unable to defend himself. At home, however, he felt strong and supportive of his mother. His father lived with another woman. He experienced him self as a looser among his pears but as a winner with his mother. At the end of primary school his father, who then had accumulated substantial wealth, returned home, and the parents resumed their marriage and intimacy. His situation at school changed as he became popular and the teasing stopped, but he still felt insecure and uneasy. He decided to attend karate school to gain a sense of power. A peer introduced him to the criminal milieu where he felt accepted and appreciated. During a robbery he became incredible angry and physically violent without really understanding why. The victim died as a consequence of his attack. He was send to prison for 2 years, followed by a high security hospital for treatment. While he accepted his prison sentence he protested treatment in psychiatric hospital. He was suspicious, remained non-relative and was often restrained due to anger outbursts. A therapist confronted him with the fact that his behaviour could lead to prolonged hospital stay and pointed to his choice of future inside or outside the hospital. This was turning point that made him focus on goals and training for a future out in real life. After discharge he continued to work on self-esteem and trustworthiness, shame and guilt and how to understand, control and come to terms with his anger. Two years later he was married with a son, and pursued a career as a teacher. - Clinical description Diagnostic Criteria ICD-10 F60.2 Dissocial personality disorder Personality disorder, usually coming to attention because of a gross disparity between behaviour and the prevailing social norms, and characterized by: (a) callous unconcern for the feelings of others; (b) gross and persistent attitude of irresponsibility and disregard for social norms, rules and obligations; (c) incapacity to maintain enduring relationships, though having no difficulty in establishing them; (d) very low tolerance to frustration and a low threshold for discharge of aggression, including violence; (e) incapacity to experience guilt or to profit from experience, particularly punishment; (f) marked proneness to blame others, or to offer plausible rationalizations, for the behaviour that has brought the patient into conflict with society. There may also be persistent irritability as an associated feature. Conduct disorder during childhood and adolescence, though not invariably present, may further support the diagnosis. Includes: amoral, antisocial, asocial, psychopathic, and sociopathic personality (disorder) Excludes: conduct disorders (F91. - ) emotionally unstable personality disorder (F60.3) People with ASPD (Dissocial in ICD 10) show unreliability, recklessness, restlessness, disruptiveness, and aggressiveness. According to DSM IV-TR (2000) they have a pervasive pattern of disregard for, and violation of, the rights of others. Negative symptoms include lack of anxiety and remorse, and lack of emotional depth and stability. They are interpersonally detached, suspicious, and exploitative, and they lack commitment to and concern for others. Antagonism, deceitfulness, manipulativeness, dishonesty, and glibness are typical interpersonal features. Some come across as self-aggrandizing and self-justifying with a sense of entitlement and invulnerability. Cognitive deficits include inflexibility, and lack of concentration. The lifetime prevalence of ASPD is about 2-3 % in the general population. The rate in the community and psychiatric population is relatively low (1-2%), but among correctional offenders, forensic psychiatric patients, and substance users it is high (< 50%). Theoretical models for the etiology of ASPD suggest a mental abnormality with social and biological causal factors, and have excluded child rearing experiences, familial dysfunctions, or adverse life experiences. Sociocultural and neurological factors are associated with symptoms of ASPD, but not clearly pathognomonic. Other theories consider ASPD as an extreme variant of personality traits found in all people, or as an adaptation. Early manifestations of ASPD are evident in children (age 6-10 years), and it is common that adults with ASPD in their childhood or adolescence were diagnosed with conduct disorder, oppositional defiant disorder, or attention deficit hyperactivity disorder. Symptoms of ASPD can persist into middle or late adulthood. ASPD has been associated with increased rate of morbidity and mortality. Antisocial personality disorder is often comorbid with substance-use disorders, but also with other personality disorders, such as the Cluster B borderline, narcissistic, and histrionic in DSM-IV or emotionally unstable and histrionic in ICD-10. There is no good evidence that ASPD can be successfully treated. Most treatment studies have aimed at reducing criminal behaviour in mixed groups of patients or offenders, including some with ASPD, rather than attempting to alleviate symptoms of ASPD. Nevertheless, structured psychosocial treatments that focus on the acquisition of important life skills, such as communication, assertiveness, and anger management skills are useful (Hemphill & Hart, 2002). Pharmacological treatments that target treatment-interfering symptoms, such as extreme hostility or impulsivity, may play a useful adjunctive role in certain cases. Borderline personality disorder (adopted from Anthony Bateman & Peter Fonagy) - Case vignette A 23 years old woman reacted with depressive symptoms and suicidal thoughts to the death of her grandfather. She was treated with antidepressant medication without addressing the loss. Three years later after a suicidal attempt, she was admitted to hospital where she first presented with depressed mood and suicidal thoughts, but quickly engaged in vivid conversations with the others patients. She was discharged with the diagnosis of personality disorder, but soon re-admitted because of suicidal thoughts, and referred to an outpatient program specialized on treatment of personality disorder. Since childhood she had unstable mood, aggressive temperament and self-destructive behaviour (head banging). At the age of 10 she was sexually abused by an older man. Suicidal thoughts and urges to kill herself was first experienced at age 11. Since age 13 she has had multiple sexual partners but also one 7 year long relationship which was quite unstable with frequent conflicts and impulsive acts. She dropped out of school and has been living on sickness benefits, interrupted by short periods of unskilled employment. In a two year psychoanalytic treatment program with one individual session and one group session a week in addition to psycho education, she worked together with other patients on identifying and understanding the characteristic features of BPD, and the dynamics of borderline pathology with a special focus on self-destructive behaviour. Her self-destructive behaviour tapered off after 3 months as she began to process her feelings of aggression and sadness. The pharmacological treatment terminated after 6 months and she quickly became less sedated and anxious. She resumed school towards the end of the first year of treatment, with the intention of taking a degree in teaching. The relationship with her boyfriend stabilized. Contacts with class became more satisfying, and conflicts with her teachers stopped. Her ability to begin to contain feelings increased dramatically. - Clinical Description Diagnostic Criteria ICD-10 F60.3 Emotionally unstable personality disorder A personality disorder in which there is a marked tendency to act impulsively without consideration of the consequences, together with affective instability. The ability to plan ahead may be minimal, and outbursts of intense anger may often lead to violence or "behavioural explosions"; these are easily precipitated when impulsive acts are criticized or thwarted by others. Two variants of this personality disorder are specified, and both share this general theme of impulsiveness and lack of self-control. - F60.30 Impulsive type The predominant characteristics are emotional instability and lack of impulse control. Outbursts of violence or threatening behaviour are common, particularly in response to criticism by others. lncludes: explosive and aggressive personality (disorder) Excludes: dissocial personality disorder (F60.2) - F60.31 Borderline type Several of the characteristics of emotional instability are present; in addition, the patient's own self-image, aims, and internal preferences (including sexual) are often unclear or disturbed. There are usually chronic feelings of emptiness. A liability to become involved in intense and unstable relations hip s may cause repeated emotional crises and may be associated with excessive efforts to avoid abandonment and a series of suicidal threats or acts of self-harm (although these may occur without obvious precipitants) lncludes: borderline personality (disorder) Individuals with BPD (Emotionally unstable in ICD 10) have according to DSM IV-TR (2000) a pervasive pattern of instability in interpersonal relationships, self-image and affects, and marked impulsivity. They show frantic efforts to avoid real or imagined abandonment, a pattern of unstable and intense interpersonal relationships and identity disturbance. They also present with impulsivity, recurrent suicidal gestures, affective instability, chronic feelings of emptiness, inappropriate intense anger. In severe cases transient stress-related paranoid ideation or severe dissociative symptoms are noticeable. BPD is relatively rare in the general population (0.2%- 1.8%) while prevalence rate among psychiatric inpatient and outpatient is higher (15% – 25%). Early separations and losses, disturbed parental involvement with conflictual relationships, childhood history of physical and/or sexual abuse, and high prevalence of affective disorder in first-degree relatives of borderline probands are specific developmental and psychosocial factors for BPD (Zanarini & Frankenburg, 1997). Low level of serotonin, stress sensitivity and a tendency for impulsive aggression can, when combined with psychosocial factors, contribute to adult BPD. Although borderline patients improve over time they still can remain functionally impaired. Especially those who experienced sexual abuse or incest in childhood have a poor prognosis. Emotional instability, impulsivity and aggressive relationships worsen prognosis as do co-morbid substance abuse, and schizotypal, antisocial or paranoid features. Around 60% of patients with BPD have major depressive disorder, 30% panic disorder with agoraphobia, 12% substance use disorder, 10% bipolar-I, and 4% bipolar-II disorder. Comorbid BPD tends to interfere with treatment of Axis I. Multimodal treatment and a combination of psychotherapy and psychopharmacological treatment offer the best chance of a good outcome (Oldham, Phillips, Gabbard, et al. 2001). Psychodynamic treatment is preferable while long in-patient treatment has proved ineffective. Evidence based manualized treatment modalities, i.e., Mentalization Based Treatment (Bateman & Fonagy, 1999; Bateman & Fonagy, 2001), Cognitive therapy (Ryle, 1997), Dialectic Behaviour Therapy (DBT) (Lenihan 1993; Linehan Heard, Armstrong, 1993) and Transference Focused Psychotherapy (TFP) (Clarkin, Foelsch, Levy, et al., 2001), have all proved beneficial and effective in changing borderline symptoms and character functioning. Although no specific psychotropic drug is effective for BPD, some can help reducing disabling symptoms; i.e., typical and atypical antipsychotic drugs, tricyclic antidepressants (TCA’s) and selective serotonin reuptake inhibitors (SSRI’s), monoamine oxidase inhibitors (MAOI’s), and mood stabilisers. Narcissistic personality disorder - Case vignette A 42-year-old male professional in public office, was forced to resign after being arrested when visiting a brothel. In the aftermath he suffered from depression and considerable alcohol consumption, and was admitted for a three months treatment. He stopped drinking, but his depression remained nonresponsive to anti-depressant medication. Still without meaningful activities he felt empty and restless, and he was referred to psychotherapy. Developmental history indicates that at age 5 his father left the family, and they did not meet until he was in law school. He was always ahead of his age and went through school without difficulty. In law school he got high marks without hard work. He had many acquaintances but no friends, and he felt like an outsider. He got married and had two children. Reaching mid-thirties he felt bored. He had everything: house, career, and family. He was respected and accomplished, but felt he didn’t belong. He started drinking heavily and visiting brothels. The psychotherapist found him self-assured, easily irritated, and quick to make devaluating remarks, and felt a mixture of irritation, compassion and powerlessness. Interactions during weekly appointments were extremely difficult. Unwilling to explore his situation or his feelings, he blamed the therapist for the impasse and told him that he will not change and that the therapist could not help. The therapist dreaded the appointments, while the patient despite finding the sessions unhelpful, always showed up. When the therapist announced a three weeks break his patient suggested the treatment to end and did not return. Nine months later he informed the therapist that he moved to another city, had a leading position working with international trade, and was greeted as a king. He said nothing about his wife and children. Nor did he indicate how he felt about the treatment. - Clinical Description People with NPD (not included in the ICD 10) have a grandiose sense of self-importance and accompanying grandiose fantasies. According to DSM-IV TR (2000) they present a pervasive pattern of grandiosity, need for admiration and lack of empathy. In addition they have a sense of entitlement and tendencies to be exploitive, and take advantage of other people. They can come across as arrogant and haughty or boastful and self-centered. However, they also have vulnerable and fluctuating self-esteem, feelings of shame, intense reactions to criticism or defeat, and vocational irregularities. Some may appear more sensitive, inhibited, vulnerable, shame-ridden and socially withdrawn, and others can present with psychopathic or antisocial characteristics. Variable prevalence rate of NPD has been found both in the general community (1% - 6%) (Stinson, Dawson Goldstein et al 2008) and in the clinical population (1.3% - 17%). Studies have suggested a genetic influence on the development of NPD, including hypersensitivity, strong aggressive drive, low anxiety or frustration tolerance, and defects in affect regulation (Torgersen et al 2000; Schore, 1994). Inconsistent attunement and insufficient attachment in the early parent-child interaction can lead to failure in the development of self-esteem and affect regulation. - Course and prognosis Although narcissistic traits can be frequent in adolescence, NPD develop in adulthood and can persist into old age. Severe disability has been indicated especially among those with comorbid Axis I disorder. NPD patients with ability for object relations actually improve over time and may have better prognosis (Ronningstam, Gunderson, Lyons 1995). NPD is considered to have one of the highest rates of diagnostic overlap among the Axis II disorders, especially with ASPD (25%). Major depression and dysthymia are the most common concomitant Axis I disorders (42 - 50%), followed by substance use disorder (24 – 50%) and bipolar disorder (5 – 11 %). Co-occurring narcissistic features can worsen course and prognosis for Axis I disorders. Psychoanalysis and psychoanalytically oriented psychotherapy are the most common treatment for NPD (Kernberg, 1975; Kohut, 1968; Fiscalini, 1994). Additional modalities include the Schema Focused Therapy (Young & Flanagan, 1998), and couples or family therapy (Solomon, 1998; Kirshner, 2001). Potentially beneficial psychopharmacological treatment focused on mood, anger or anxiety, is often challenged by the patients’ reluctance to adhere to such modality. Histrionic Personality Disorder (adopted from Bruce Pfohl) - Case vignette A 25-year-old female university student sought psychoanalytic treatment as she suffered from depression, difficulties in interpersonal relationships, and vocational dissatisfaction. Her first panic attack occurred during the last year in high school when her boyfriend was treated for panic attacks. She believed she was "influenced" by him. In psychotherapy she overcame family difficulties, especially in relation to her mother, but continued to feel insecure and pessimistic, blaming it all on her boyfriend. Their conflictual relationship ended when she had an episode of depression. She felt she wanted to die and sought consultation for psychoanalytic treatment saying that she was wasting her life, and lacked motivation for studies or career. She dreamt about her former boyfriend, and after breaking up with two other men she felt extremely lonely. She is the third of seven children. The father was hard-working, affectionate and caring, but also irritable and depressive. The mother was impulsive and sarcastic. Mother and daughter had a close but conflictive relationship as the mother could be intrusive, opinionated and idealizing. At age three the parents moved abroad for one year and left her to live with relatives. Upon their return she was presented to a baby brother. Significant sensitivity during her school years led her to break up friendships and feel extremely lonely. She did well at university, formed friendships but noticed that she often felt rejected without knowing why. In psychoanalysis four times per week she presented several contradictions, i.e., pursuing treatment and lapsing, or describing her mother as unsupportive, cold and envious but nevertheless readily resorting to her when facing difficulties. As the psychoanalysis progressed she presented infantile histrionic features; a precarious identity, strong affective dependence, dissociation, infantilization and self destructive work related behaviour. She brought multiple dreams to the sessions and gave vivid images of conflicts that worried her. Despite efforts to interpret, the analyst noticed no progress. Paradoxically, her presentation of dreams and associations indicated in-depth psychological work, but her persistent tardiness and absenteeism reflected the opposite. After eight months of psychoanalysis, the analyst suggested 3 sessions per week of face to face psychotherapy and referred her to a colleague. - Clinical description Diagnostic Criteria DSM-10 F60.4 Histrionic personality disorder Personality disorder characterized by: (a) self-dramatization, theatricality, exaggerated expression of emotions; (b) suggestibility, easily influenced by others or by circumstances; (c) shallow and labile affectivity; (d) continual seeking for excitement and activities in which the patient is the centre of attention; (e) inappropriate seductiveness in appearance or behaviour; (f) over-concern with physical attractiveness Associated features may include egocentricity, self-indulgence, continuous longing for appreciation, feelings that are easily hurt, and persistent manipulative behaviour to achieve own needs. lncludes: hysterical and psychoinfantile personality (disorder) In DSM-IV-TR (2000) HPD is described as a pervasive pattern of excessive emotionality and attention-seeking behaviour. People with HPD show seductive inappropriate behaviour, shallow emotional expressions, impressionistic speech, suggestibility, and a belief that relationships are more intimate than they really are. They have strong need for attention, pursued by a sensational physical appearance, or by being emotionally dramatic and expressive, or inappropriately sexually provocative or seductive. Individuals with HPD range from high level classical hysterical character neuroses to more primitive character functioning presenting with dissociative language, vivid fantasy life and infantile dependence. DSM-IV-TR (2000) suggests that about 2% - 3% of the general population and 5% – 10% of the clinical population meet criteria for HPD. Repression and somatisation of strong emotions are considered the main etiological factors in hysteria. - Course and prognosis The course and prognosis of HPD depends upon comorbidity and level of severity of the disorder. Intense and chronic anger and stormy close relationships are indicators of poorer prognosis. Ability to reflect and tolerate regularity can prevent treatment failure (Stone, 2005). Major depressive disorder, Somatization disorder and Conversion disorder are the most common comorbid Axis I disorder with HPD. Association between the other Cluster B personality disorders have also been found. Individuals with HPD can also present with increased attention driven risk for suicidal gestures and threats. Histrionic personality traits are usually requiring long-tem treatment and psychodynamic psychotherapy is the most common modality. Higher functioning neurotically organized individuals can be treated with psychoanalysis, while people with more primitive functioning may benefit from supportive or cognitive therapy which focuses on the patient's automatic thoughts and beliefs and on modifying emotional and interpersonal reactivity (Gabbard & Allison, 2007). Cluster C includes the Avoidant, Dependent, and Obsessive-Compulsive Personality Disorders. Individuals with these disorders often appear anxious or fearful. Avoidant Personality Disorders exhibit a pattern of social inhibition, feelings of inadequacy, and hypersensitivity to negative evaluation. Dependent Personality Disorders show a pattern of submissive and clinging behavior that evidence an excessive need to be taken care of. Lastly, Obsessive-Compulsive Personality Disorders manifest a preoccupation with orderliness, perfectionism, and control. For additional information and references see Module II in Simonsen E, Ronningstam E, Millon T (Eds). (2007). WPA ISSPD Educational Program on Personality Disorders. www.wpanet.org/education/education.shtml: Avoidant Personality Disorder - Case Vignette The patient was a 35 year old, unmarried data technician referred to a specialized treatment program for personality disorders from an out-patient drug addiction service. His personality pathology was considered more devastating than his substance abuse. Presenting complaints included low self esteem, loneliness, sense of emptiness, suicidal ideation, social isolation, substance abuse, general dissatisfaction with life. Present complaints had been chronic in nature, dating back to childhood. He recalled having daily suicidal thoughts for several years in his early youth. On axis I he fulfilled the criteria for dysthymic disorder and drug abuse in partial remission, but not panic disorder or social phobia. His avoidant behavior was more prominent than his level of experienced anxiety. On axis II he fulfilled all seven criteria for avoidant personality disorder and an additional seven criteria spread across other personality disorders. The most prominent feature was a pervasive fear of being ridiculed when interacting with others. In a group-based treatment program lasting for 20 weeks, he was a regular, but somewhat detached participant. The therapists encountered a series of problems related to passivity: He postponed most of his obligations, resisted sorting out practical affairs, did not pay his bills and avoided contacting people who could be helpful. - Clinical description Diagnostic Criteria ICD-10 F60.6 Anxious [avoidant] personality disorder Personality disorder characterized by: (a) persistent and pervasive feelings of tension and apprehension; (b) belief that one is socially inept, personally unappealing, or inferior to others; (c) excessive preoccupation with being criticized or rejected in social situations; (d) unwillingness to become involved with people unless certain of being liked; (e) restrictions in lifestyle because of need to have physical security; (f) avoidance of social or occupational activities that involve significant interpersonal contact because of fear of criticism, disapproval, or rejection. Associated features may include hypersensitivity to rejection and criticism Avoidant personality disorder is a pervasive pattern of social inhibition, feelings of inadequacy or inferiority, and hypersensitivity to negative evaluation, according the definition of American Psychiatric Association DSM-IV-TR (American Psychiatric Association, 2000). The term of avoidant personality disorder has been used in DSM, while anxious personality disorder is used in ICD-10 (World Health Organization, 1993). Although the term avoidant personality disorder was first used by Millon, (1969) these patients have been described as sensitive character (Kretschmer, 1921), introvert (Jung, 1936), interpersonally avoidant (Horney, 1945)), insecure psychopath (Schneider, 1950), phobic personalities (Fenichel, 1945), or active-detached personalities (Millon, 1973). People with this disorder are timid, extremely self-conscious and fearful of criticism, humiliation, and rejection. Clinical literature has reported that Cluster C personality disorders including avoidant personality disorder often co-occur with mood and anxiety disorders. Avoidant and dependent personality disorders were strongly related to mood disorders, especially major depression, dysthymia, and mania. Avoidant personality disorders were reported to be strongly related to anxiety disorders, especially panic disorder with agoraphobia and social phobia. In addition to mood and anxiety disorders, eating disorders tend to be comorbid with avoidant personality disorder (Oldham et al. 1995). Avoidant personality disorder often co-occurs with other Cluster C personality disorders. This disorder is especially strongly correlated with dependent personality disorder. Although there was concern that the prevalence of avoidant personality disorder might be low when it was first included in the DSMIII classification system, it became clear that this is one of most common personality disorders. However, this disorder appears to be more prevalent according to the recent national studies with a large sample size e.g., 2-2.5%. It is quite prevalent within clinical settings and reported to present 5% -35% in psychiatric populations (Mattia et al. 2001). The odds of avoidant personality disorder are greater for the lower income group, people with less than a high school education, the widowed/divorced /separated and never married, and residents in the most urbanized areas (Grant et al., 2004). Although the etiology of avoidant personality disorder is not known, a few models are proposed. The biological learning theory hypothesizes that the interaction of a biologically determined sensitivity to interpersonal relationships and social experiences affects the development of the disorder. It is also postulated to be an extreme variant of the personality traits of introversion and neuroticism which have heritability. According to the interpersonal etiology model, the disorder is explained based on a conflict between seeking closeness and fearing it. Cognitive theory hypothesizes negative schema which originate in early childhood, and which lead to social avoidance behavior. When the disorder begins in childhood, the symptoms could worsen in adolescence due to the complex and demanding social relationships of this time. It is essential to establish a good therapeutic relationship which is, however, very difficult because of the patients’ low self-esteem and hypersensitivity to rejection. Cognitive individual or group format is effective for these types of patients. Social skills training, systematic desensitization, and graded hierarchy of in vivo exposure to feared social situations could be useful (Beck & Freeman, 1990). Both short-term dynamic psychotherapy and cognitive therapy have a place in the treatment of patients with cluster C personality disorders (Svartberg et al. 2004). Exploratory and supportive group therapy may be helpful for these patients by providing a holding environment in which they can share their insecure feelings. Dependent Personality Disorder - Case Vignette The patient was a 27 year old white female administrative assistant whose work required much use of the computer and data entry. She gradually began to develop pain in her wrists. Physicians diagnosed a potential carpal tunnel syndrome. The damage to her wrists was not reparable by surgery and Sally was left in significant daily pain. The patient demonstrates the key aspect of Dependent personality, the need to please others even at the expense to herself. The degree to which her self-destructive passivity and compliance at work stemmed from her early experiences within the family are unclear, but her parents’ overprotectiveness likely played some role in the etiology of her personality pathology. Research confirms that overprotective and authoritarian parenting, alone or in combination, often lead to excessive interpersonal dependency in offspring. - Clinical description Diagnostic Criteria ICD-10 F60.7 Dependent personality disorder Personality disorder characterized by: (a) encouraging or allowing others to make most of one's important life decisions; (b) subordination of one's own needs to those of others on whom one is dependent, and undue compliance with their wishes; (c) unwillingness to make even reasonable demands on the people one depends on; (d) feeling uncomfortable or helpless when alone, because of exaggerated fears of inability to care for oneself; (e) preoccupation with fears of being abandoned by a person with whom one has a close relationship, and of being left to care for oneself; (f) limited capacity to make everyday decisions without an excessive amount of advice and reassurance from others. Associated features may include perceiving oneself as helpless, incompetent, and lacking stamina lncludes: asthenic, inadequate, passive, and self-defeating personality (disorder) Although early diagnosticians discussed at length the clinical implications of exaggerated dependency needs, it was not until publication of the third edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-III) that dependent personality disorder (DPD) became a full-fledged diagnostic category. DPD is defined as "a pervasive and excessive need to be taken care of that leads to submissive and clinging behavior and fears of separation" (APA, 1994, p. 668). The person must show several of the following symptoms to receive a DPD diagnosis: difficulty making everyday decisions without excessive advice and reassurance; needing others to assume responsibility for most major areas of life; difficulty initiating projects or doing things on one’s own; going to excessive lengths to obtain nurturance and support; feeling uncomfortable and helpless when alone, being unrealistically preoccupied with fears of being left to care for oneself. The DSM-IV-TR indicates that three Axis I diagnoses - mood disorders, anxiety disorders, and adjustment disorder - show substantial comorbidity with DPD. Evidence supports continued inclusion of these three categories in future versions of the DSM, but also suggests that eating disorders and somatisation disorder co-occur with DPD at higher-than-expected rates (Piper et al. 2001). Problematic dependency is widespread in the community as well as in clinical populations, and is associated with an array of psychological disorders. Studies typically report Dependent Personality Disorder prevalence rates of between 15 % and 25% in hospital and rehabilitation settings (Oldham et al. 1995). Bornstein’s (1993, 1997) meta-analyses of epidemiological findings indicated that gender moderates DPD prevalence rates. When data from extant studies were combined, the overall base rate of DPD was 11% in women and 8% in men. Although this difference seems modest, it is highly significant. Several theoretical frameworks have been particularly influential in conceptualizing the etiology of the Dependent Personality. Research does not support the early psychodynamic hypothesis that variations in infantile feeding and weaning behaviors play a role in the development of dependent personality traits (Bornstein, 1996). Many psychodynamic researchers (e.g., Luborsky & Crits-Christoph, 1990) now conceptualize problematic dependency as resulting from unconscious conflicts. Cognitive models of DPD focus on the ways in which a person’s manner of thinking helps foster dependent behavior. As Freeman and Leaf (1989) noted, dependency-related automatic thoughts (i.e., reflexive self-statements that reflect the person’s perceived lack of competence) are central in this process. Automatic thoughts are accompanied by negative self statements, which combine to create a persistent attributional bias that reinforces the person’s view of himself as vulnerable and weak. A vicious cycle ensues. No studies have documented the long-term course of DPD in inpatients, outpatients, or community adults. In the short term, research confirms that dependent patients exhibit behaviors that both facilitate and undermine treatment. For example, dependent psychotherapy patients are cooperative and conscientious, but also make more requests for after-hours contact. Dependent patients delay less long than nondependent patients when psychological symptoms appear, but they also have difficulty terminating treatment after symptoms remit (Bornstein, 1993). Over the years clinicians have provided recommendations for intervention strategies based on cognitive (Young, 1994), psychodynamic (Luborsky & Crits-Christoph, 1990), behavioral (Turkat, 1990), and experiential (Schneider & May, 1995) treatment models. However, only two studies assessed changes in DPD symptoms during the course of psychotherapy, and these investigations produced conflicting results. Obsessive-Compulsive Personality Disorder - Case Vignette The patient was a 42-year-old single male, who lives with his parents. He has been unemployed for some time. He presented to the anxiety disorders clinic at a major teaching hospital, because of concerns regarding his long-term unemployment. He tended to procrastinate when making decisions or carrying out plans. On weekends, when the family planned to visit the grandparents he would start packing on Friday afternoon, but on many occasions did not finish the packing until Sunday, by which time it was too late to go. He spent long periods of time in the bathroom, would take half an hour to wash his hands-first washing the tap, then his hands, then the tap again. This routine also made it difficult for him to go out and look for job. In fact, it totally prevented him from doing so. After leaving school, he has had 30 or 40 jobs, mostly factory work. The longest he has lasted in a job has been one week, often only one day. He was very punctual in treatment and never missed a session; he talked freely, and in great detail. The initial part of therapy mainly dealt with family relationships. When the time came to leave the sessions he would often continue talking and delaying even when the therapist was standing at the door. - Clinical Descriptions Diagnostic Criteria ICD-10 F60.5 Anankastic personality disorder Personality disorder characterized by: (a) feelings of excessive doubt and caution; (b) preoccupation with details, rules, lists , order, organization or schedule; (c) perfectionism that interferes with task completion; (d) excessive conscientiousness, scrupulousness, and undue preoccupation with productivity to the exclusion of pleasure and interpersonal relationships; (e) excessive pedantry and adherence to social conventions; (f) rigidity and stubbornness; (g) unreasonable insistence by the patient that others submit to exactly his or her way of doing things, or unreas6nable reluctance to allow others to do things; (h) intrusion of insistent and unwelcome thoughts or impulses. lncludes: compulsive and obsessional personality (disorder) obsessive - compulsive personality disorder Excludes: obsessive - compulsive disorder (F42. - ) Diagnostic criteria of ICD-10 (WHO, 1992) and DSM-IV-TR (APA, 2000) for the OCPD (or anankastic personality disorder, following the ICD-10) are quite similar. Both nosological systems describe a syndrome characterized by symptoms such as excessive perfectionism, stubbornness, rigidity, and lack of decision. For the DSM-IV-TR, the OCPD is a pervasive pattern of preoccupation with orderliness, perfectionism, and mental and interpersonal control, at the expense of flexibility, openness, and efficiency, beginning by early adulthood and present in a variety of contexts. Most research shows that most individuals with Axis I Obsessive-Compulsive Disorder do not fulfill the criteria of OCPD. Furthermore, it has been found that patients with OCD and with a personality disorder show similar or more frequent relationships with the avoidant or dependent personality disorder than with OCPD. Comorbidity with other personality disorders has varied markedly depending on the specific study. (Maier et al. (1992) found that the Obsessive-Compulsive Personality Disorder was the second most frequent personality disorder (among the 11 included in the DSM-III-R) in his study sample (individuals without psychiatric disorders), showing a range from 1.6% to 6.4%, while the prevalence found by Widiger & Sanderson (1997) ranged from 1% to 3%. The etiology of Obsessive-Compulsive Personality Disorder is unknown. There are not data regarding the influence of biological factors in the onset and development of this disorder, but it is believed that environmental factors play an important role in its etiology. Millon (1996) proposes some of these tentative variables: parental over-control is a method of restrictive child-rearing in which punitive processes are used to set distinct limits on children’s behavior. As long as they operate within the parental approved boundaries, children are safe from parental punishment. 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Washington, DC, American Psychiatric Press, pp 269-293. - Young J. Flanagan C. (1998). Schema-Focused Therapy for Narcissistic Patients. In: E Ronningstam (Ed.): Diagnostic, Clinical, and Empirical Implications. Washington, DC, American Psychiatric Press, pp 239-267. - Bateman A, Fonagy P. (1999) The effectiveness of partial hospitalization in the treatment of borderline personality disorder - a randomised controlled trial. American Journal of Psychiatry, 156, 1563-1569. - Clarkin J F, Foelsch P, Levy K., et al. (2001) the development of a psychodynamic treatment for patients with borderline personality disorder: a preliminary study of behavioural change. Journal of Personality Disorders, 15, 487-495. - Linehan MM. (1993) The skills training manual for treating borderline personality disorder. New York: Guilford Press. - Linehan MM, Heard HL, Armstrong HE. (1993) Naturalistic follow-up of a behavioural treatment for chronically parasuicidal borderline patients. Archives of General Psychiatry, 50, 971-974. - Oldham J, Phillips K, Gabbard G, et al. (2001). Practice Guideline for the Treatment of Patients with Borderline Personality Disorder. American Psychiatric Association. American Journal of Psychiatry, 158, 1-52. - Ryle A. (1997) Cognitive Analytic Therapy and Borderline Personality Disorder: The Model and the Method. Chichester, UK: John Wiley & Sons. - Schore, A (1994). Affect Regulation and the Origin of the Self. Hillsdale, NJ. Erlbaum - Stinson FS, Dawson DA, Goldstein RB, Chou PS, Huang B, Smith SM, Ruan WJ, Pulay AJ, Saha TD, - Torgersen, S., Lygren, S., Oien, P., et al. (2000) A twin study of personality disorders. Comprehensive Psychiatry, 41, 416-425. - Zanarini MC, Frankenburg FR. (1997) Pathways to the development of borderline personality disorder. Journal of Personality Disorders, 11, 93-104. The following general questions are suggested to be discussed in classrooms: - Why are PDs useful for mental health workers (psychiatrists, psychologists, social workers) to understand as a key component of their clinical activities? - Discuss the social costs of the PDs, their widespread prevalence and their associated civic and public health consequences and disruptions. - Why is the traditional concept of "disease" not suitable when discussing the nature of the PDs? Why do some thinkers consider PDs to be best considered as similar to the biological immune system? - How can normality and abnormality best be differentiated? Is there a sharp line separating them or are they on a continuum? - The history of ideas about personality goes back to the early Greeks. Discuss some of these interesting ideas and major thinkers from the past to the present. - What are some of the issues, as well as the similarities and differences between the ICD-10 and DSM-IV in their formulation of the PDs. - Do personality disorders really exist or are they just convenient fictions of theory, clinical observation or research investigations? - What are the issues in the categorical vs. dimensional PD debate, and does the prototypical idea help solve them? - Discuss the role of biogenic, psychogenic and sociogenic influences in PD development pathogenesis? Describe some of the research evidence for their respective contributions. - Describe the several modes and specific tools of diagnosing the PDs, and discuss their respective strengths and weaknesses. - Go into considerable detail in specifying the strengths of either the cognitive or the psychodynamic approach to therapy for the PDs. - What are the comparative advantages and disadvantages of adhering to one specific school of therapy versus several combined schools, e.g., behavioural, pharmacologic in treating the PDs. - Curriculum Suggestions – Module II The following questions for each personality disorder are suggested to be discussed in classrooms: - What are the most outstanding and significant features for each personality disorder that best identifies and differentiates them from other personality disorders? - In what way does each personality disorder resemble or overlap with other disorders, including both Axis I and Axis II disorders? - What are the major commonalities in the etiology of the personality disorders? Which personality disorders have primarily developmental origin, and which have a strong potential genetic origin? - Identify specific cultural factors in your country/cultural environment that influence the understanding and treatment of certain personality disorder features. - What are the most striking gender differences among personality disorders – i.e., which disorders are, according to the text, most common among men, and among woman? How does that compare to your cultural experiences? Discuss reasons for observed differences - How does the prevalence of each personality disorder vary in your country/culture compare to those prevalence rates mentioned in the Module II text? - Discuss and compare the differences between treating personality disorders and Axis I disorders. How do co-occurring Axis I disorders influence treatment of a personality disorder, and vice versa, how can the presence of a personality disorder affect the course and treatment of an Axis I disorder such as Bipolar disorder or Major Depression or Eating Disorder. Give examples. - Compare the major contemporary controversies of each personality disorder and discuss future changes in diagnostic classifications and important areas for research. - Andrew E. Skodol & John G. Gunderson. Personality Disorders. In: The Textbook of Psychiatry (eds. Robert E. Hales, Stuart C. Yudifsky & Glen O. Gabbard). 5th edition. American Psychiatric Publishing, Washington, 2008. - Personality Disorders. WPA Series Evidence and experience in Psychiatry. Volume 8. John Wiley & Sons: Chichester, 2005. - Personality Disorders. Chapter 27. In: Kaplan & Saddock’s Synopsis of Psychiatry, Behavioral Sciences/Clinical Psychiatry. 10th Edition. Lippencott, Williams & Wilkins: Philadelphia, 2007. - Personality Disorders. Chapter 62. In: Essential of Psychiatry. Jerald Kay & Allan Tasman (eds.). John Wiley & Sons: Chicester, 2006. - Livesley, W. John (2003). Practical management of Personality Disorder. New York: Guilford Press. - Millon, Theodore & Davis, Rodger (1996). Disorders of Personality DSM-IV and Beyond. New York: John Wiley & Sons. - John G. Gunderson: Personality Disorders. Chapter 15. In: The Harvard Guide to Psychiatry. Armand M. Nicholi (ed.) 3rd Edition, 1999.	2	27	1	0	0	30	0.750566	31	23,843
A clinical coder – also known as clinical coding officer, diagnostic coder, medical coder or medical records technician – is a health care professional whose main duties are to analyse clinical statements and assign standard codes using a classification system. The data produced are an integral part of health information management, and are used by local and national governments, private healthcare organizations and international agencies for various purposes, including medical and health services research, epidemiological studies, health resource allocation, case mix management, public health programming, medical billing, and public education. For example, a clinical coder may use a set of published codes on medical diagnoses and procedures, such as the International Classification of Diseases (ICD) or the Common Coding System for Healthcare Procedures (HCPCS), for reporting to the health insurance provider of the recipient of the care. The use of standard codes allows insurance providers to map equivalencies across different service providers who may use different terminologies or abbreviations in their written claims forms, and be used to justify reimbursement of fees and expenses. The codes may cover topics related to diagnoses, procedures, pharmaceuticals or topography. The medical notes may also be divided into specialities for example cardiology, gastroenterology, nephrology, neurology or orthopedic care. A clinical coder therefore requires a good knowledge of medical terminology, anatomy and physiology, a basic knowledge of clinical procedures and diseases and injuries and other conditions, medical illustrations, clinical documentation (such as medical or surgical reports and patient charts), legal and ethical aspects of health information, health data standards, classification conventions, and computer- or paper-based data management, usually as obtained through formal education and/or on-the-job training. - 1 In practice - 2 Competency levels - 3 Education and professional qualification - 4 Classification types - 5 Professional associations - 6 See also - 7 References - 8 External links The basic task of a clinical coder is to classify medical and health care concepts using a standardised classification. Most clinical coders are employed in coding inpatient episodes of care. However, mortality events, outpatient episodes, general practitioner visits and population health studies can all be coded. Clinical coding has three key phases: a) Abstraction; b) Assignment; and c) Review. The abstraction phase involves reading the entire record of the health encounter and analysing the information to determine what condition(s) the patient had, what caused it and how it was treated. The information comes from a variety of sources within the medical record, such as clinical notes, laboratory and radiology results, and operation notes. The assignment phase has two parts: finding the appropriate code(s) from the classification for the abstraction; and entering the code into the system being used to collect the coded data. Reviewing the code set produced from the assignment phase is very important. Clinical coder must ask themselves, "does this code set fairly represent what happened to this patient in this health encounter at this facility?" By doing this, clinical coders are checking that they have covered everything that they must, but not used extraneous codes. For health encounters that are funded through a case mix mechanism, the clinical coder will also review the diagnosis-related group (DRG) to ensure that it does fairly represent the health encounter. Clinical coders may have different competency levels depending on the specific tasks and employment setting. Entry-level / trainee coder An entry level coder has completed (or nearly completed) an introductory training program in using clinical classifications. Depending on the country; this program may be in the form of a certificate, or even a degree; which has to be earned before the trainee is allowed to start coding. All trainee coders will have some form of continuous, on-the-job training; often being overseen by a more senior coder. Intermediate level coder An intermediate level coder has acquired the skills necessary to code many cases independently. Coders at this level are also able to code cases with incomplete information. They have a good understanding of anatomy and physiology along with disease processes. Intermediate level coders have their work audited periodically by an Advanced coder. Advanced level / senior coder Advanced level and senior coders are authorized to code all cases including the most complex. Advanced coders will usually be credentialed and will have several years of experience. An advanced coder is also able to train entry-level coders. A nosologist understands how the classification is underpinned. Nosologists consult nationally and internationally to resolve issues in the classification and are viewed as experts who can not only code, but design and deliver education, assist in the development of the classification and the rules for using it. Nosologists are usually expert in more than one classification, including morbidity, mortality and casemix. In some countries the term "nosologist" is used as a catch-all term for all levels. Education and professional qualification In some countries, clinical coders may seek voluntary certification or accreditation through assessments conducted by professional associations, health authorities or, in some instances, universities. The options available to the coder will depend on the country, and, occasionally, even between states within a country. As of 2016; the typical qualification for an entry-level medical coder in the United States is completion of a diploma or certificate, or, where they are offered, an associate degree. The diploma, certificate, or degree will usually always include an Internet-based and/or in-person internship, at some form of a medical office or facility, at the conclusion. Some form of on-the-job training, or at least oversight, is also usually provided in the first months on the job, until the coder can earn an intermediate or advanced level of certification and accumulate time on the job. For further academic training, a baccalaureate or master's degree in medical information technology, or a related field, can be earned by those who wish to advance to a supervisory or academic role. That option would be recommended for those wishing to teach medical billing or coding at a college or university, community college, or technical or vocational institute, or who wish to become heads of medical billing and coding departments, especially if the doctor's office or clinic, or other facility (among other working options, a medical school or hospital, a skilled nursing facility or other nursing home, a psychiatric facility, an assisted or independent living facility, a rehabilitation facility, a rest home or domiciliary or boarding house, etc.) is very large and receives complex cases, such as a referral facility or a Level I trauma teaching hospital center. A nosologist (medical coding expert) in the U.S. will usually be certified by either AHIMA or the AAPC (often both) at their highest level of certification and specialty inpatient and/or outpatient certification (pediatrics, obstetrics/gynecology, gerontology, oncology are among those offered by AHIMA and/or the AAPC), have at least 3-5 years of intermediate experience beyond entry-level certification and employment, and often holds an associate, bachelor's, or graduate degree. The AAPC offers the following entry-level certifications in the U.S.: Certified Professional Coder (CPC); which tests on most areas of medical coding, and also the Certified Inpatient Coder (CIC) and Certified Outpatient Coder (COC). Also in the American Health Information Management Association (AHIMA) offers the entry-level Certified Coding Associate (CCA); which is, like the AAPC's CPC, a wide-ranging introductory test. Some U.S. states, though decidedly not the majority, as it is a very recent trend, now mandate or at least strongly encourage certification or a degree from a college- or at the minimum, some evidence of competency beyond the record of on the job training- and/or from either the AAPC or AHIMA, to be employed. Some states have registries of medical coders, though these can be voluntary listings- which is, for those few who do, most often the case- and so not mandatory. This trend was accelerated in part by the passage of HIPAA (which enforces among other things, patient privacy and access to and the form of medical records) and the Affordable Care Act (U.S. President Barack Obama's health care reform law); and similar changes in other developed and developing countries, many of which, especially in the Western developed countries, and beyond, use the ICD-10 for diagnostic medical coding, which is a quite complex system of codes. The change to more regulation and training has also been driven by the need to create accurate, detailed, and secure medical records- especially patient charts, bills, and claim form submissions, that can be recorded efficiently in an electronic era of medical records where they need to be carefully shared between different providers or institutions of care, which was encouraged and later required by legislation and institutional policy. Clinical coders may use many different classifications, which fall into two main groupings: statistical classifications and nomenclatures. A statistical classification, such as ICD-10 or DSM-5, will bring together similar clinical concepts, and group them into one category. This allows the number of categories to be limited so that the classification does not become too big, but still allows statistical analysis. An example of this is in ICD-10 at code I47.1. The code title (or rubric) is Supraventricular tachycardia. However, there are several other clinical concepts that are also classified here. Amongst them are paroxysmal atrial tachycardia, paroxysmal junctional tachycardia, auricular tachycardia and nodal tachycardia. With a nomenclature, for example SNOMED CT, there is a separate listing and code for every clinical concept. So, in the tachycardia example above, each type and clinical term for tachycardia would have its own code listed. This makes nomenclatures unwieldy for compiling health statistics. In many countries clinical coders are accommodated for by both professional bodies specific to coding, and organisations who represent the health information management profession as a whole. - Clinical Coders' Society of Australia (CCSA) - Health Information Management Association of Australia (HIMAA) - Institute of Health Records and Information Management (IHRIM) - Professional Association of Clinical Coders UK (PACC-UK) There are several associations that medical coders in the United States may join, including: - American Health Information Management Association (AHIMA) - AAPC (formerly American Academy of Professional Coders) The AHIMA and AAPC societies' accredited programs will generally train medical coders at a sufficient level to work in their respective states. Some medical coders elect to be certified by both societies. AHIMA maintains a list of accredited medical coding certificate (and health information management associate, bachelor's, and graduate programs, through a link on the AHIMA accredited programs page, to CAHIIM) here. - Clinical medicine - Current Procedural Terminology - Diagnosis-related group - Diagnostic and Statistical Manual of Mental Disorders (DSM) - Health informatics - International Classification of Diseases (ICD) / ICD-11 (in development) / ICD-10 / ICD-9-CM - Medical diagnosis - Pathology Messaging Implementation Project - WHO Family of International Classifications - Marie A. Moisio (2000). A Guide to Health Insurance Billing. Thomson Delmar Learning. ISBN 0-7668-1207-3. - Michelle A. Green and JoAnn C. Rowell (2011). Understanding Health Insurance, A Guide to Billing and Reimbursement, 10e. Cengage Learning. ISBN 978-1-111-03518-1. - World Health Organization. Classifying health workers: Medical records and health information technicians. Geneva, 2010. - Department of Human Services, Victoria, Australia. Clinical Coders Creed. Health Data Standards and Systems Bulletin, Issue 13, 19 July 2000. - Wooding A (2004). "Clinical coders and decision making". HIM J. 33 (3): 79–83. PMID 18490784. - Walker S (2006). "Capturing health information—a perspectives paper". HIM J. 35 (3): 13–22. PMID 18195424. - Nosologist. Popular Science, posted 11.11.2004. - Bramley, M; Reid, B. "Evaluation standards for clinical coder training programs" (PDF). Health Information Management Journal. 36 (3): 2007. - "3. How to use the ICD". International statistical classification of diseases and related health problems (10th revision, 2010 ed.). Geneva: World Health Organization. 2011. p. 19. ISBN 9789241548342. In the context of the ICD, "rubric" denotes either a three-character category or a four-character subcategory. - "Clinical Coders' Society of Australia". Retrieved 16 March 2015. - "Health Information Management Association of Australia Limited". Retrieved 16 March 2015. - "CHIMA: The Canadian Health Information Management Association". Retrieved 16 March 2015. - "IHRIM - Institute of Health Records and Information Management (IHRIM)". Retrieved 16 March 2015. - "PACC-UK - Home". Retrieved 16 March 2015. - "AHIMA Home - American Health Information Management Association". Retrieved 16 March 2015.	3	14	1	0	0	2	0.23926	3	2,853
@ Diabetes Nursing Diagnosis ★★ How To Treat Diabetic Pain In Feet The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11. Diabetic Neuropathy. 11. Diabetic foot is the main consequence of neuropathy and peripheral vascular diseases, including lower limb infection, formation of ulcers, and (or) destruction of deep tissues . 1. Photos of four typical cases were as following, showing the initial condition and the situation of healing after systemic nursing interventions. How To Treat Peripheral Neuropathy? What Is Peripheral Neuropathy, And What Are Its Symptoms? ★★ Diagnosis Code For Diabetic Neuropathy ★★ ::The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days.[ DIAGNOSIS CODE FOR DIABETIC. @ Nursing Diagnosis For Diabetes ★★ Current Diabetes Recommendations The 3 Step Trick that Reverses Diabetes Permanently in As Little as. Diabetic Neuropathy. 11. Diabetic neuropathy information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. Prevention, or early diagnosis and good diabetic control are essential to reduce the burden of diabetes for patients, and for health and social care. Symptoms of sensory neuropathy include tingling and numbness, loss of pain or temperature sensation, loss of coordination or burning/shooting pains. Autonomic neuropathy. Our Mission is to prevent and cure diabetes and to improve the lives of all people affected by diabetes. 2017 Wrap Up Report. Don’t try anything before you read. Keep your diabetes and blood pressure under control to lower the chance of getting kidney disease. Estimated 7% of US population is diabetic; Twice that many have prediabetes; 21 % of those over 60 have diabetes; 45% of new diagnoses are being made in children and. Related to autonomic neuropathy; Will not experience the sympathetic surge—with sweating, shakiness, HA, etc. Nursing Management of HHNS. Nursing Care Plans: Examples, NANDA Plans, Plans for Diabetes, Characteristics Peripheral neuropathy is a nervous system problem. Patients with peripheral neuropathy experience a lost of function of their sensory nerves (nerves that. Nurses' role in diabetic foot prevention and care; a review. M Aalaa,; O Tabatabaei Malazy,; M Sanjari,; M Peimani and; MR Mohajeri-TehraniEmail author. Journal of Diabetes & Metabolic Disorders201211:24. https://doi.org/ 10.1186/2251-6581-11-24. © Aalaa et al.; licensee BioMed Central Ltd. 2012. Received: 25 October. @ Icd 9 Diabetes With Neuropathy ★★ Nursing Diagnosis For Diabetes The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days.[ ICD 9 DIABETES. Diabetes is ranked second behind congestive heart failure as the primary diagnosis at entry into home care. The role of home health care nurses is increasingly important as patients are discharged from hospitals and rehabilitation centers early in the course of illness and require more sophisticated nursing management. Laser Therapy And Diabetic Neuropathy Neuropathy, Fibromyalgia or Chronic Pain? The #1 Place for Chronic Pain Management including Laser Therapy. Welcome To NeuropathyDR.com Oct 14, 2016. Can low-level laser therapy relieve your Neuropathy symptoms? Can it stop the numbing, the burning, tingling and excruciating pain – without any side effects? The answer is yes. Page Contents [hide]. What is Infrared Assessment and Management of Patients with Diabetes Mellitus By Linda Self Growing problem Estimated 7% of US population is diabetic Twice that many have. Overview of kidney disease, including most common causes, and associated tests It is important to remember that once a problem develops, appropriate diagnosis and treatment is essential if the foot is to be returned to full function. Without. In the diabetic patient, foot infection is the result of several factors, which include neuropathy, vascular disease, and decreased resistance to infection. If trauma is. @ Nursing Diagnosis Diabetes ★★ Icd 9 Code For Diabetic Peripheral Neuropathy The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days. 20-40% of people with diabetes experience NeP (painful diabetic neuropathy) ( Serpell, 2004);. – 25-50% of patients aged over. A detailed pain history is necessary to enable nurses to make a diagnosis and help inform the multidisciplinary team of treatment strategies that are available for NeP. A clear diagnosis includes. ★ Diabetes Diagnosis And Treatment ★★ How To Prevent Diabetes In Teens ::The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days. Modern Love Modern Love Podcast: Richard Jenkins Reads ‘In My Fantasy, I Caught Up to Reality’ On this week’s podcast, the “Shape of. May 6, 2013. The pathogenesis of diabetic peripheral neuropathy (DPN) is not completely understood. Evidence suggests that. However, there is accumulating evidence suggestive of subclinical changes of nerve function shortly after diagnosis even in children. In addition to. The British Journal of Nursing. 2006; 15:. @ Nursing Diagnosis For Gestational Diabetes ★★ Meds For Diabetic Neuropathy The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days.[ NURSING. Evidence-based recommendations on the diagnosis and management of type 1 diabetes in adults Vitamin B12 Peripheral Neuropathy Pain. Tingling. Numbness. If you have a type of nerve damage from diabetes called diabetic peripheral neuropathy, chances are you’ve experienced these. Learn about peripheral neuropathy causes such as diabetes, alcohol, medication, shingles, vitamin deficiency, autoimmune diseases, and inherited diseases. Jul 11, 2017. Vitamin B12 deficiency can cause peripheral neuropathy among a number of different In this post are 10+ diabetes mellitus nursing care plans (NCP). Diabetes is a chronic disease, which occurs when the pancreas does not produce enough insulin. May 22, 2012. This nursing care plan is for patients who have diabetes. Diabetes is where the body is unable to control blood sugar levels due to either the. Oct 13. Because of the increased numbers of individuals affected with diabetes, diabetic PN (DPN)has become one of the most common causes of neuropathy. It is important to note that combined use of both modalities significantly increases sensitivity and specificity, allowing NPsto be more confident with the diagnosis.25. There are plenty of factors that should be considered when creating a nursing care plan for diabetes. Diabetes is one of the top causes of morbidity nowadays and in. Aug 13, 2015. Diabetic Peripheral Neuropathy information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. Find support with our neuropathy community- support group meetings, Tai Chi classes, online resources, and a network of healthcare providers you can. Have questions about what’s causing your bellyache? Take a look at this information on stomach disorders. Find information on kids and adults. I have a 23 year old friend who has gastric cancer that was found to have mestatised to the liver, adrenal glands, ovaries & bones in the spinal region on. Surgery is used to treat most stages of bladder cancer. Urinary diversion is surgery that makes a new way for your body to store urine. This can be done. Shoes For Peripheral Neuropathy Uk WebMD experts and contributors provide answers to your health questions. 09.09.2017 · Has anybody regained the sensation in their feet after being diagnosed with neuropathy?? I can’t detect temperature and feel like my feet are dead. Carpal Tunnel Syndrome And Diabetic Neuropathy DiabetesMine 411 info series investigates how diabetes and carpel tunnel syndrome go hand-in-hand, and A patient with a longstanding diagnosis of type 1 diabetes has a history of poor glycemic control. The nurse recognizes the need to assess the patient for signs and symptoms of peripheral neuropathy. Peripheral neuropathy constitutes a risk for what nursing diagnosis? A) Infection B) Acute pain C) Acute confusion D). @ Herbal Treatments For Diabetic Neuropathy ★★ Nursing Diagnosis For Type 2 Diabetes The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days. Poor diabetes control commonly leads to damage to the nerves and blood vessels, and are part of the metabolic syndrome resulting in neuropathy, peripheral arterial disease and atherosclerosis. Included in this are foot checks by HCPs, which must be undertaken on diagnosis, and at least at their annual review. Sep 25, 2013. Peripheral neuropathy r/t diabetes type 2AGE (s)64 year-oldfemale (non- compliant) DIAGNOSIS:Peripheral neuropathy r/t diabetesmellitustype 2. Report should include diagnosis definition, Etiology, diagnostic tests, clinical manifestations or S & S, complications, prognosis, nursing interventions, and. Read about diabetic foot care. Causes of foot problems in people with diabetes include footwear, nerve damage, poor circulation, trauma, infections, and. As Saunders (1995) suggested: 'Nurses are in a good position to. anxiety generated by the diagnosis. 3Traditionally, newly diagnosed type 2 diabetes is managed initially by doctors in primary or secondary care. 4There is often little systematic education. l Nurse-led care. Journal of Diabetes Nursing Vol 2 No 1 1998. 12. I would like some help in coming up with the nursing diagnoses in the patient with peripheral neuropathy. She is not a diabetic or oncology patient. What I have so. Neuropathies are characterized by a progressive loss of nerve fiber function. A widely accepted definition of diabetic peripheral neuropathy is Best practice guidelines (BPG)are systematically developed statements to assist nurses and patients in decision-making about appropriate healthcare (Field & Lohr, 1990). This guideline has been developed to address the question of how to assess and manage patients with established diagnosis of diabetic foot ulcers. ★★ Nursing Diagnosis For Diabetes ★★ ::The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days.[ NURSING DIAGNOSIS FOR DIABETES ] The REAL. Natural Home Remedies for Kidney Infection • Herbs like uva ursi, cornsilk, parsley, juniper berry, alfalfa, echinacea, goldenseal, Oregon graperoot etc. 4 contact hour diabetes CEU course on type 1 diabetes mellitus (T1DM). Identify development, diagnosis, treatment regimens, management, and interventions to avoid acute and chronic complications. Applicable for nursing, case management , occupational therapy, physical therapy, and EMS personnel. @ Nursing Diagnosis For Diabetes ★★ Type 2 Diabetes Cure 2016 The 3 Step Trick that Reverses Diabetes Permanently in As Little as 11 Days.[ NURSING DIAGNOSIS FOR. Bachelor's thesis. Degree program in Nursing. Nursing. 2015. Isaac Tireito, Johnpaul Ronoh & Margaret Maiyo. DIABETIC NEUROPATHY, SELF. CARE AND PAIN. KEYWORDS: Pain, diabetes, diabetes neuropathy, self- management, foot. that 7.5% of patients suffered neuropathy already after diabetes diagnosis was. Aug 27, 2012. The aim of this study was to evaluate the effectiveness of SNCP implementation, based on North American Nursing Diagnosis Association (NANDA) and Nursing Interventions Classification (NIC), in the improvement of metabolic, weight, and blood pressure control of Type 2 Diabetes Mellitus (T2DM). More Neuropathy Articles ... - Chemical Causes Of Peripheral Neuropathy: Detailed analysis of 13 causes of Upper arm burning sensation symptom, alternative diagnoses and related symptoms. Peripheral neuropathy is a disorder that occurs when your peripheral nerves malfunction because they're damaged. Your Trusted Sour... - How To Cure Diabetic Neuropathy: Herbal Treatments. St. John’s wort is the most effective herbal remedy for neuropathy. It particularly helps to cure injuries afflicted on nerves of the. May 31, 2015. Neuropathy, or damage to the peripheral or autonomic nerves, is a common complicat... - Effects Of Alcohol On Diabetic Neuropathy: 1456 n engl j med 374;15 nejm.orgApril 14, 2016 The new england journal of medicine peripheral artery disease. The lifetime risk of a foot lesion. Keep your blood glucose levels in target range. If you drink alcohol, keep within the recommended guide... - Diabetic Painful And Insensate Neuropathy Pathogenesis And Potential Treatments: . likely contribute to the pathogenesis of painful diabetic neuropathy. Diabetic painful and insensate neuropathy: pathogenesis and potential treatments. Diabetic Painful and Insensate Neuropathy: Pathogenesis. and developing potential pathogenetic t... - Cayenne Pepper For Peripheral Neuropathy: How To Make A Cayenne Pain Relief Salve. master cayenne pepper. from diabetic neuropathy, shingles, Read an interesting article that I would like to share Cayenne Pepper & Peripheral Neuropathy Overview In some cases, cayenne products may ease a ... - Epstein Barr Virus Peripheral Neuropathy: At least ten percent of those infected with Human Immunodeficiency Virus (HIV) develop neuropathy, some because HIV medications can be toxic to nerves. Other infections associated with neuropathy include hepatitis C, Herpes varicella- zoster (shingle...	2	14	0	0	0	2	0.669798	2	2,939
What does ALOC stand for in medical terms? What does ALOC mean in medical terms? In the previous article, we learned about the SLP medical abbreviation. Now let’s talk about the meaning of the ALOC medical abbreviation! Table of Contents ALOC medical abbreviation meaning The meaning of the ALOC acronym can vary based on the context in which it is employed. For example: - Altered Level Of Consciousness - Acute Loss of Consciousness - Alternate Level Of Care ALOC medical abbreviation – Altered Level Of Consciousness What is an altered level of consciousness? An altered Level of Consciousness (ALOC) is a medical condition that disrupts an individual’s normal mental state. It ranges from mild confusion to loss of consciousness. ALOC is common in healthcare settings, resulting from various underlying medical conditions. The severity of the ALOC depends on the underlying medical condition, which can range from head injuries, strokes, seizures, infections, hypoglycemia, and drug intoxication to metabolic disturbances. Recognizing ALOC early is crucial for proper management. When assessing a patient with ALOC, healthcare providers prioritize stabilizing the patient’s vital signs like breathing, heart rate, and blood pressure. Afterward, they systematically evaluate the patient’s mental status, including assessing consciousness, cognitive function, and response to stimuli. Healthcare providers must consider the patient’s medical history, medication use, drug or alcohol use, and past medical conditions. A physical exam, including a neurological examination, must also identify any focal deficits that suggest an underlying cause. Treatment of ALOC depends on the underlying cause. This may involve administering medication to control seizures, antibiotics for infections, or surgery for head injuries’ intracranial pressure. Supportive care like oxygen therapy and intravenous fluids may also be necessary. Early recognition and treatment of ALOC are vital to prevent further complications. Delayed recognition or treatment of ALOC can lead to irreversible brain damage or death. Therefore, prompt identification and treatment of ALOC are critical in ensuring the best possible outcome for the patient. In conclusion, an Altered Level of Consciousness is a medical emergency requiring prompt evaluation and treatment. Healthcare providers should recognize the signs and symptoms of ALOC, identify the underlying cause, and intervene early. With early intervention and appropriate treatment, healthcare providers can help prevent the devastating consequences of ALOC. 5 levels of consciousness medical Consciousness is a critical aspect of medical evaluation, and medical professionals use five levels of consciousness to assess patients’ awareness. These levels provide essential information about a patient’s neurological function and overall health. The first level is full consciousness, where patients are alert, aware of their surroundings, and can communicate effectively. This level is considered normal; most individuals spend most of their waking hours in this state. The second level is a mildly altered state, characterized by minor cognitive impairment leading to slowed reaction times or memory lapses. Medication side effects, sleep deprivation, or alcohol consumption can cause this level of consciousness. The third level is a moderately altered state, where patients have significant cognitive impairment leading to confusion, disorientation, and difficulty understanding or responding to stimuli. Head injuries, strokes, or seizures can cause this level of consciousness. The fourth level is a deeply altered state, where patients are unresponsive and do not respond to stimuli. This level can be caused by severe injuries, infections, or illnesses that affect the brain and may result in patients being in a coma or having a decreased level of consciousness. The fifth and final level is the absent state, where patients have no detectable brain activity and are considered brain dead. This level of consciousness is irreversible and typically results from severe brain injuries sustained in accidents or falls. Medical professionals use various tools and assessments, such as the Glasgow Coma Scale, to determine a patient’s level of consciousness. Evaluating a patient’s level of consciousness is critical in providing appropriate treatment. Mild alterations in consciousness may require rest or medication changes, while more severe levels may require hospitalization and intensive treatments such as surgery or medications to reduce brain swelling. ALOC medical abbreviation ICD-10 ICD 10 code for aloc – The complex realm of medical diagnostics requires precise tools and assessments to accurately evaluate a patient’s condition. One assessment uses the International Classification of Diseases, Tenth Revision (ICD-10) coding system. When identifying a patient’s level of consciousness, healthcare professionals may utilize the code R40.24, which denotes an altered level of consciousness (ALOC). This code includes symptoms and signs involving cognition, perception, emotional state, and behavior. However, it’s crucial to note that the R40.24 code is a broad code that does not specify the underlying cause of the ALOC. Thus, healthcare professionals must thoroughly evaluate the patient to accurately identify the root cause of their altered level of consciousness. Factors such as head injury, medication side effects, sleep deprivation, or alcohol consumption can all result in ALOC. The severity of the patient’s ALOC can also vary, ranging from minor cognitive impairment to a deeply altered state where patients are unresponsive and do not respond to stimuli. Medical professionals must carefully document the patient’s condition to ensure accurate coding and appropriate treatment. Depending on the underlying cause, treatment may range from medication changes or rest to hospitalization and intensive treatments such as surgery or medications to reduce brain swelling. Evaluating a patient’s level of consciousness is critical in providing appropriate care. The ICD-10 coding system is useful for healthcare professionals in accurately diagnosing and treating patients with ALOC. ALOC medical term – Acute Loss of Consciousness ALOC (Acute Loss of Consciousness) is an alarming and bewildering medical condition that can threaten an individual’s well-being. Various factors, including neurological and non-neurological conditions, can trigger ALOC. The neurological causes of ALOC encompass diverse conditions like epilepsy, stroke, traumatic brain injury, and brain tumors. In contrast, non-neurological causes encompass heart disorders, low blood sugar, dehydration, and drug overdose. ALOC can unleash an array of perplexing symptoms, which can vary depending on the underlying cause. Common symptoms of ALOC comprise sudden loss of consciousness, confusion, dizziness, and disorientation. Also, ALOC can be accompanied by other bewildering symptoms, such as seizures, muscle cramps, or bladder and bowel control loss. To diagnose ALOC, a comprehensive medical examination is imperative, which includes a physical examination, neurological examination, and several diagnostic tests like an electroencephalogram (EEG), computed tomography (CT) scan, or magnetic resonance imaging (MRI). The treatment of ALOC hinges on the underlying cause. For neurological causes, medication is the primary treatment option, while for non-neurological causes, treatment may encompass medication, surgery, or lifestyle changes, such as diet and exercise. Furthermore, ALOC can be prevented by managing health conditions, staying hydrated, avoiding excessive alcohol consumption, and getting adequate sleep. Immediate medical attention is critical if someone experiences ALOC. Delayed treatment can lead to severe complications, such as brain damage or death. Prompt medical attention can help identify the underlying cause and provide appropriate treatment. ALOC medical terminology – Allyloxycarbonyl Allyloxycarbonyl, commonly known as Alloc, is a critical chemical group in medicinal chemistry. It is a preferred protecting group for amino acids during peptide synthesis because it can prevent unwanted reactions and ensure successful peptide formation. Alloc offers stability under harsh chemical conditions and is easy to remove under mild conditions, making it a valuable tool in synthesizing various compounds, including anticancer drugs, antibiotics, and antiviral agents. Besides its use in peptide synthesis, Alloc has also gained attention for its potential in drug delivery systems. Researchers have used Alloc as a linker to attach drugs to carrier molecules, such as nanoparticles, liposomes, and polymers, to enhance drug solubility, stability, and bioavailability. Moreover, drug conjugates using Alloc can provide targeted drug delivery to specific cells or tissues, thereby improving drug efficacy while reducing side effects. Despite the numerous benefits of Alloc, it has its limitations. Alloc can be toxic and may not react well with certain amino acids, limiting its application in some peptide synthesis procedures. Therefore, medicinal chemists are continually searching for new protecting groups with improved properties that can overcome the limitations of Alloc. Medical abbreviation ALOC – Alternate Level Of Care ALOC or Alternate Level of Care refers to a complex healthcare phenomenon where individuals who have been discharged from acute care hospital services are yet to be placed in another healthcare setting. These individuals are usually elderly with intricate medical conditions that require ongoing care and support. However, they occupy hospital beds that could be put to use for patients requiring acute care. ALOC patients can take up to 15% of hospital beds and spend an average of 18.7 days there. The root cause of the ALOC issue is the lack of long-term care beds and home care services. The lack of viable options in the community leaves elderly patients stranded in hospitals, which results in high healthcare costs and detrimental effects on the patient’s well-being. Hospitals are not designed for long-term care; patients must be placed in appropriate care options. To tackle this predicament, one solution is to invest in and prioritize community-based care. This entails expanding the capacity of long-term care facilities, increasing the availability of home care services, and providing more support for caregivers. By offering patients appropriate care options in the community, hospitals can reduce the number of ALOC patients, thereby improving patient flow. Transitional care programs are another viable solution that provides patients with the necessary care and support to move from the hospital to a community-based care setting. These programs include various services such as medical management, rehabilitation, and social support, ensuring patients receive the appropriate care and support they need after leaving the hospital. Hospitals can implement strategies to improve patient flow by optimizing bed management processes, establishing discharge planning teams, and ensuring timely access to home care services. By streamlining the patient discharge process, hospitals can reduce the number of ALOC patients, freeing up hospital beds for patients requiring acute care. Alright, I think that’s all for today. I believe that the meaning behind the ALOC medical abbreviation is now clear to you.	3	2	0	0	0	3	0.97188	3	2,183
Meningioma Classification and external resources A contrast enhanced CT scan of the brain, demonstrating the appearance of a Meningioma. ICD-10 C70, D32 ICD-9 225.2 ICD-O: M9530/0 OMIM 607174 DiseasesDB 8008 eMedicine neuro/209 radio/439 MeSH D008579 The word meningioma was first used by Harvey Cushing in 1922 to describe a tumor originating from the meninges, the membranous layers surrounding the CNS . Meningiomas are the second most common primary neoplasm of the central nervous system, arising from the arachnoid "cap" cells of the arachnoid villi in the meninges. These tumors are usually benign in nature; however, they can be malignant. Most cases are sporadic while some are familial. Persons who have undergone radiation to the scalp are more at risk for developing meningiomas. The most frequent genetic mutations involved in meningiomas are inactivation mutations in the neurofibromatosis 2 gene (merlin) on chromosome 22q. Other possible genes/loci include: Signs and symptomsMain article: brain tumor Small tumors (e.g., < 2.0 cm) are usually incidental findings at autopsy without having caused symptoms. Larger tumors can cause symptoms depending on the size and location. - Focal seizures may be caused by meningiomas that overlie the cerebrum. - Progressive spastic weakness in legs and incontinence may be caused by tumors that overlie the parasagittal frontoparietal region. - Sylvian tumors may cause myriad motor, sensory, aphasic, and seizure symptoms, depending on the location. - Increased intracranial pressure eventually occurs, but is less frequent than in gliomas. Meningiomas arise from arachnoidal cells, most of which are near the vicinity of the venous sinuses, and this is the site of greatest prevalence for meningioma formation. They are most frequently attached to the dura over the superior parasagittal surface of frontal and parietal lobes, along the sphenoid ridge, in the olfactory grooves, the sylvian region, superior cerebellum along the falx cerebri, cerebellopontine angle, and the spinal cord. The tumor is usually gray, well-circumscribed, and takes on the form of space it occupies. They are usually dome-shaped, with the base lying on the dura. Histologically, the cells are relatively uniform, with a tendency to encircle one another, forming whorls and psammoma bodies (laminated calcific concretions). They have a tendency to calcify and are highly vascularized. Meningiomas are often considered benign tumors curable by surgery, but most recurrent meningiomas correspond to histologic benign tumors. The metabolic phenotype of these benign recurrent meningiomas indicated an aggressive metabolism resembling that observed for atypical meningioma . Meningiomas are readily visualized with contrast CT, MRI with gadolinium, and arteriography, all attributed to the fact that meningiomas are extra-axial and vascularized. CSF protein is usually elevated if lumbar puncture is attempted. Though the majority of meningiomas are benign, they can have malignant presentations. Classification of meningiomas are based upon the WHO classification system. - Benign (Grade I) - (90%) - meningothelial, fibrous, transitional, psammomatous, angioblastic (most aggressive) - Atypical (Grade II) - (7%) - chordoid, clear cell, atypical (includes brain invasion) - Anaplastic/malignant (Grade III) - (2%) - papillary, rhabdoid, anaplastic In a recent retrospective review of atypical and anaplastic meningioma cases, the mean overall survival for atypical meningiomas was found to be 11.9 years vs. 3.3 years for anaplastic meningiomas. Mean relapse free survival for atypical meningiomas was 11.5 years vs. 2.7 years for anaplastic meningiomas. Malignant Anaplastic meningioma is an especially malignant tumor with aggressive behavior. Even if - by general rule - neoplasms of the nervous systems (brain tumors) cannot metastasize into the body (because of the blood-brain barrier) Anaplastic Meningioma can. Although they are inside the cerebral cavity, they are located on the bloodside of the BBB, because meningioma tend to "connect" themselves to bloodvessels to "feed". Cancerized cells can escape into the bloodstream. This is why meningioma when they metastasize often turn up around the lungs. It should be noted that Anaplastic meningioma and hemangiopericytoma are difficult to distinguish (even by pathological means), as they look similar, especially if the first occurrence is a meningeal tumor, and both tumors occur in the same places (same type of tissue) Observation with close imaging follow-up can be used in select cases if a meningioma is small and asymptomatic. In a retrospective study on 43 patients, 63% of patients were found to have no growth on follow-up, and the 37% found to have growth grew at an average of 4 mm / year. In this study, younger patients were found to have tumors that were more likely to grow on repeat imaging, thus are poorer candidates for observation. In another study, clinical outcomes were compared for 213 patients undergoing surgery vs. 351 patient under watchful observation. Only 6% of the conservatively treated patients later developed symptoms, while among the surgically treated patients, 5.6% developed persistent morbid condition, and 9.4% developed surgery-related morbid condition. Observation is not recommended in tumors already causing symptoms. Furthermore, close follow-up with imaging is required with an observation strategy to rule out an enlarging tumor. Meningiomas can usually be surgically resected with permanent cure if the tumor is superficial on the dural surface and easily accessible. Transarterial embolization has become a standard preoperative procedure in the preoperative management. If invasion of the adjacent bone occurs, total removal is nearly impossible. Malignant transformation is rare. The probability of tumor recurrence or growth after surgical resection can be estimated by the tumor's WHO Grade and by the extent of surgery by the Simpson Criteria. Simpson Grade Completeness of Resection 10-year Recurrence Grade I complete removal including resection of underlying bone and associated dura 9% Grade II complete removal + coagulation of dural attachment 19% Grade III complete removal w/o resection of dura or coagulation 29% Grade IV subtotal resection 40% Radiation therapy may include photon beam or proton beam treatment, or fractionated external beam radiation. Radiosurgery can be used in lieu of surgery in small tumors located away from critical structures. Fractionated external beam radiation can also be used as primary treatment for tumors that are surgically unresectable, or for patients who are inoperable for medical reasons. Radiation therapy is often considered for WHO Grade I meningiomas after subtotal (incomplete) tumor resections. The clinical decision to irradiate after a subtotal resection is somewhat controversial, as no class I randomized controlled trials exist on the subject. Numerous retrospective studies, however, have strongly suggested the addition of postoperative radiation to incomplete resections improves both progression free survival (i.e. prevents tumor recurrence) and improves overall survival. In the case of a Grade III meningioma, the current standard of care involves postoperative radiation treatment regardless of the degree of surgical resection. This is due to the proportionally higher rate of local recurrence for these higher grade tumors. Grade II tumors can behave variably and there is no standard of whether to give radiotherapy following a gross total resection. Subtotally resected grade II tumors should be radiated. Current chemotherapies are likely not effective. Antiprogestin agents have been used, but with variable results. Recent evidence that hydroxyurea has the capacity to shrink unresectable or recurrent meningiomas is being further evaluated. - ^ Cushing H. (1922). "The meningiomas (dural endotheliomas): their source, and favoured seats of origin". Brain (45): 282–316. http://www.medmerits.com/index.php/article/meningiomas/P1. - ^ Buetow MP, Buetow PC, Smirniotopoulos JG (November 1991). "Typical, atypical, and misleading features in meningioma". Radiographics 11 (6): 1087–106. PMID 1749851. http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=1749851. Retrieved 2008-12-01. - ^ Goldsmith BJ, Wara WM, Wilson CB, Larson DA (February 1994). "Postoperative irradiation for subtotally resected meningiomas. A retrospective analysis of 140 patients treated from 1967 to 1990". J. Neurosurg. 80 (2): 195–201. doi:10.3171/jns.1994.80.2.0195. PMID 8283256. - ^ Rabin BM; Meyer, JR; Berlin, JW; Marymount, MH; Palka, PS; Russell, EJ (September 1996). "Radiation-induced changes in the central nervous system and head and neck". Radiographics 16 (5): 1055–72. PMID 8888390. http://radiographics.rsnajnls.org/cgi/reprint/16/5/1055. - ^ Lekanne Deprez RH, Riegman PH, Groen NA, et al. (April 1995). "Cloning and characterization of MN1, a gene from chromosome 22q11, which is disrupted by a balanced translocation in a meningioma". Oncogene 10 (8): 1521–8. PMID 7731706. - ^ Staal FJ, van der Luijt RB, Baert MR, et al. (May 2002). "A novel germline mutation of PTEN associated with brain tumours of multiple lineages". Br. J. Cancer 86 (10): 1586–91. doi:10.1038/sj.bjc.6600206. PMC 2746590. PMID 12085208. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2746590. - ^ Zattara-Cannoni H, Roll P, Figarella-Branger D, et al. (April 2001). "Cytogenetic study of six cases of radiation-induced meningiomas". Cancer Genet. Cytogenet. 126 (2): 81–4. doi:10.1016/S0165-4608(00)00398-8. PMID 11376799. http://linkinghub.elsevier.com/retrieve/pii/S0165-4608(00)00398-8. - ^ "moon.ouhsc.edu". http://moon.ouhsc.edu/kfung/jty1/Com/Com306-1-Diss.htm. Retrieved 2008-11-30. - ^ "Neuropathology For Medical Students". http://www.pathology.vcu.edu/WirSelfInst/neuro_medStudents/tumor-1.html. Retrieved 2008-11-30. - ^ Monleón D, Morales JM, Gonzalez-Segura A, Gonzalez-Darder JM, Gil-Benso R, Cerdá-Nicolás M, López-Ginés C (November 2010). "Metabolic aggressiveness in benign meningiomas with chromosomal instabilities". Cancer Research 70 (21): 8426–8434. doi:10.1158/0008-5472.CAN-10-1498. PMID 20861191. http://cancerres.aacrjournals.org/content/70/21/8426.long. - ^ Meningioma from Radiopaedia.org. - ^ Wrobel G, Roerig P, Kokocinski F, et al. (March 2005). "Microarray-based gene expression profiling of benign, atypical and anaplastic meningiomas identifies novel genes associated with meningioma progression". Int. J. Cancer 114 (2): 249–56. doi:10.1002/ijc.20733. PMID 15540215. - ^ Yang SY et al.: Atypical and anaplastic meningiomas: prognostic implications of clinicopathological features. J Neurol Neurosurg Psychiatry. 2008 May;79(5):574-80. Epub 2007 Aug 31. - ^ Herscovici Z, et al.: Natural history of conservatively treated meningiomas." Neurology. 2004 Sep 28;63(6):1133-4. - ^ Yano S, Kuratsu J.: Indications for surgery in patients with asymptomatic meningiomas based on an extensive experience. J Neurosurgery. 2006 Oct 105(4):538-43. - ^ Olivero WC et al.: "The natural history and growth rate of asymptomatic meningiomas: a review of 60 patients." J Neurosurg. 1995 Aug;83(2):222-4 - ^ http://www.aans.org/education/journal/neurosurgical/july03/15-1-10.pdf - ^ Simpson D. "The recurrence of intracranial meningiomas after surgical treatment." J Neurol Neurosurg Psychiatry. 1957 Feb;20(1):22-39. - ^ Kullova A et al.: Radiosurgery for benign meningioma. J Neurosurg. 2007 Aug;107(2):325-36. - ^ Taylor BW et al.: The meningioma controversy: postoperative radiation therapy. Int J Radiat Oncol Biol Phys. 1988 Aug;15(2):299-304. - ^ Goldsmith BJ et al.: Postoperative irradiation for subtotally resected meningiomas. A retrospective analysis of 140 patients treated from 1967 to 1990. J Neurosurg. 1994 Feb;80(2):195-201. - ^ Goyal LK et al. "Local control and overall survival in atypical meningioma: a retrospective study," Int J Radiat Oncol Biol Phys. 2000 Jan 1;46(1):57-61. - ^ Wahab M et al.: Meningioma and hormonal influences. Climacteric. 2003 Dec;6(4):285-92. - ^ Newton HB. Hydroxyurea chemotherapy in the treatment of meningiomas. Neurosurg Focus. 2007;23(4):E11. - Meningioma Information from the American Brain Tumor Association - Meningioma information from the Mayo Clinic - MR/CT scans of meningioma from MedPix - MR/CT scans of pneumosinus dilatans from MedPix Nervous tissue tumors/NS neoplasm/Neuroectodermal tumor (ICD-O 9350–9589) (C70–C72, D32–D33, 191–192/225) Endocrine/ sellar (9350–9379)sellar: Craniopharyngioma · Pituicytomaother: Pinealoma spinal tumors)Astrocytoma (Pilocytic astrocytoma, Pleomorphic xanthoastrocytoma, Fibrillary (also diffuse or lowgrade) astrocytomas, Anaplastic astrocytoma, Glioblastoma multiforme)Ependymoma · SubependymomaMultiple/unknownMature neuronNeuroblastoma (Esthesioneuroblastoma, Ganglioneuroblastoma) · Medulloblastoma · Atypical teratoid rhabdoid tumorPrimitiveMeningiomas (9540–9579)cranial and paraspinal nerves: Neurofibroma (Neurofibrosarcoma, Neurofibromatosis) · Neurilemmoma/Schwannoma (Acoustic neuroma) · Malignant peripheral nerve sheath tumornote: not all brain tumors are of nervous tissue, and not all nervous tissue tumors are in the brain (see brain metastases) - Nervous system neoplasia - Brain tumor Wikimedia Foundation. 2010.	2	24	3	0	4	6	0.40558	13	3,673
Can artificial intelligence and machine learning, using an artificial intelligence–enhanced, 12-lead electrocardiogram (AI-ECG), distinguish patients with long QT syndrome from those who do not have the syndrome? In a diagnostic study using a deep neural network, the AI-ECG successfully distinguished patients with long QT syndrome (n = 967) from those who were evaluated for long QT syndrome but discharged without this diagnosis (n = 1092) presenting to a specialized arrhythmia clinic. The model performed better than the corrected QT alone, even in the setting of a normal QT interval. The AI-ECG model evaluated was able to distinguish patients with electrocardiographically concealed long QT syndrome from those without the syndrome and could potentially provide a simple and inexpensive method for early detection of congenital long QT syndrome. Long QT syndrome (LQTS) is characterized by prolongation of the QT interval and is associated with an increased risk of sudden cardiac death. However, although QT interval prolongation is the hallmark feature of LQTS, approximately 40% of patients with genetically confirmed LQTS have a normal corrected QT (QTc) at rest. Distinguishing patients with LQTS from those with a normal QTc is important to correctly diagnose disease, implement simple LQTS preventive measures, and initiate prophylactic therapy if necessary. To determine whether artificial intelligence (AI) using deep neural networks is better than the QTc alone in distinguishing patients with concealed LQTS from those with a normal QTc using a 12-lead electrocardiogram (ECG). Design, Setting, and Participants A diagnostic case-control study was performed using all available 12-lead ECGs from 2059 patients presenting to a specialized genetic heart rhythm clinic. Patients were included if they had a definitive clinical and/or genetic diagnosis of type 1, 2, or 3 LQTS (LQT1, 2, or 3) or were seen because of an initial suspicion for LQTS but were discharged without this diagnosis. A multilayer convolutional neural network was used to classify patients based on a 10-second, 12-lead ECG, AI-enhanced ECG (AI-ECG). The convolutional neural network was trained using 60% of the patients, validated in 10% of the patients, and tested on the remaining patients (30%). The study was conducted from January 1, 1999, to December 31, 2018. Main Outcomes and Measures The goal of the study was to test the ability of the convolutional neural network to distinguish patients with LQTS from those who were evaluated for LQTS but discharged without this diagnosis, especially among patients with genetically confirmed LQTS but a normal QTc value at rest (referred to as genotype positive/phenotype negative LQTS, normal QT interval LQTS, or concealed LQTS). Of the 2059 patients included, 1180 were men (57%); mean (SD) age at first ECG was 21.6 (15.6) years. All 12-lead ECGs from 967 patients with LQTS and 1092 who were evaluated for LQTS but discharged without this diagnosis were included for AI-ECG analysis. Based on the ECG-derived QTc alone, patients were classified with an area under the curve (AUC) value of 0.824 (95% CI, 0.79-0.858); using AI-ECG, the AUC was 0.900 (95% CI, 0.876-0.925). Furthermore, in the subset of patients who had a normal resting QTc (<450 milliseconds), the QTc alone distinguished those with LQTS from those without LQTS with an AUC of 0.741 (95% CI, 0.689-0.794), whereas the AI-ECG increased this discrimination to an AUC of 0.863 (95% CI, 0.824-0.903). In addition, the AI-ECG was able to distinguish the 3 main genotypic subgroups (LQT1, LQT2, and LQT3) with an AUC of 0.921 (95% CI, 0.890-0.951) for LQT1 compared with LQT2 and 3, 0.944 (95% CI, 0.918-0.970) for LQT2 compared with LQT1 and 3, and 0.863 (95% CI, 0.792-0.934) for LQT3 compared with LQT1 and 2. Conclusions and Relevance In this study, the AI-ECG was found to distinguish patients with electrocardiographically concealed LQTS from those discharged without a diagnosis of LQTS and provide a nearly 80% accurate pregenetic test anticipation of LQTS genotype status. This model may aid in the detection of LQTS in patients presenting to an arrhythmia clinic and, with validation, may be the stepping stone to similar tools to be developed for use in the general population. Congenital long QT syndrome (LQTS) is characterized by prolongation of the QT interval on a standard 12-lead surface electrocardiogram (ECG).1 Clinically, patients are often asymptomatic but can present with syncope, seizures, or sudden cardiac death.1 Although corrected QT (QTc) values exceeding the 99th percentile in men (≥470 milliseconds) or women (≥480 milliseconds) are the thresholds for further evaluation for LQTS as an otherwise incidental finding in ECG-based screening programs, individuals with a QTc greater than or equal to 500 milliseconds are at increased risk of LQTS-associated events. Over the years, in addition to the QTc, several ECG features, such as specific T wave shapes for the LQTS genetic subtypes,2 QT prolongation on treadmill exercise testing,3 following epinephrine challenge,4 or upon brisk standing,5 as well as novel ECG- or echocardiogram-derived risk factors, such as T wave morphologic characteristics and the electromechanical window,6-8 have improved diagnosis and risk stratification for these patients. However, although QT prolongation is the disease’s hallmark feature, many patients with clinically and/or genetically confirmed LQTS have an otherwise normal-looking ECG.9 In fact, approximately 40% of patients with LQTS have a normal QTc at rest (concealed LQTS).9 Especially for those patients, distinguishing the normal ECG from one in patients with LQTS is important for early diagnosis and possible treatment, particularly enabling the implementation of simple preventive measures in terms of avoiding exposure to QT–prolonging medications whenever possible. Herein, we sought to determine whether artificial intelligence (AI) using deep neural networks is better than the ECG-derived QTc alone at identifying patients with LQTS presenting to a specialized arrhythmia clinic, especially for patients with a normal QTc on their resting 12-lead ECG. For this diagnostic case-control study, we performed a retrospective review of all patients presenting to the Mayo Clinic Genetic Windland Smith Rice Genetic Heart Rhythm Clinic between January 1, 1999, and December 31, 2018. This study followed the Standards for Reporting of Diagnostic Accuracy (STARD) reporting guideline for case-control studies. This study was approved by the Mayo Clinic Institutional Review Board with waiver of consent because of minimal risk. Patients were included if they had a definitive diagnosis of LQTS that included a positive genetic test with the identification of a LQT1-, LQT2-, or LQT3-causative variant or were evaluated because of an initial suspicion for LQTS, but subsequently were discharged without that diagnosis in the absence of sufficient evidence (including a negative LQTS genetic test) to establish this diagnosis (dismissed normal) (Figure 1), providing a carefully evaluated control group for our study. For all patients, demographic data, clinical history pertaining to LQTS, and genetic information were collected. In addition, for all patients, every ECG performed at Mayo Clinic was obtained for our AI-enhanced ECG (AI-ECG) analysis. A QTc calculated by the ECG machine’s algorithm (GE MAC 5500 HD; GE Healthcare) was obtained for each ECG and full-waveform data were obtained for model development and subsequent analyses. Each QTc was validated by a genetic cardiologist (M.J.A.) specializing in LQTS and reannotated where appropriate. To compare our neural network predictions with a clinically validated tool, we then used the Schwartz score to assign a probability of LQTS.10 A score of 3.5 is associated with a high probability of LQTS, a score between 1.5 and 3.0 indicates an intermediate probability, and a score of 1.0 or below is associated with a low probability. ECG Waveform Data, Convolutional Neural Network Development, and Application For the development of the AI-ECG, we used a convolutional neural network (CNN) model as shown in the diagram in eFigure 1 in the Supplement, which was similar in development as described before.11 The model was trained using 60% of the patients, validated in 10% of the patients, and tested on the remaining patients (30%). A detailed description of ECG waveform abstraction, CNN architecture, hyperparameters, and parameters used for training the binary and multiclass model, and details on splitting of the data set (both random and temporal) can be found in the eMethods in the Supplement. Because the QTc is currently the most important ECG value in the diagnosis of LQTS, the goal of our study was to see whether we could develop an ECG-focused model that would be superior to this single measurement. We used the model to evaluate whether the AI-ECG could distinguish LQTS from a normal QTc (LQTS detection) and distinguish the most common genotypic subtypes of LQTS, classified as KCNQ1-encoded LQT1, KCNH2-encoded LQT2, and SCN5A-encoded LQT3 (LQTS genotype classification). To be able to classify these, we used the developed QT model as described for each one, but a softmax function for categorical classification was added as the final activation, with 2 outputs for the LQTS detection network and 3 outputs for the LQTS genotype classification network. The QTc (defined herein as QTc alone) was used as the current standard ECG parameter for the diagnosis of LQTS to evaluate the performance of the new AI model. For subsequent analyses to test the model’s capability to detect concealed LQTS, only patients with a QTc less than 450 milliseconds were included. In addition, to account for the substantially different number of ECGs available per patient (Table) and the expected variability over time, we performed our comparisons using QTc and model output for a patient’s first ECG as well as the mean QTc and deep neural network output score from all of a patient’s ECGs. Demographic and basic ECG data (continuous or categorical) were expressed as mean (SD). Differences between groups were calculated using a t test (if normally distributed) or Wilcoxon log rank test for continuous data, or a χ2 test for categorical data. All tests were unpaired, and a 2-tailed P value <.05 was considered statistically significant. All calculations were performed using JMP Pro, version 14 (SAS Institute Inc). The model was evaluated at a single threshold selected from the internal validation data set. The selected threshold was subsequently applied to the hold-out data set and sensitivity, specificity, and F1 were calculated. The CI for the area under the curve (AUC) was determined based on the DeLong method. All analyses were computed using Python, version 3.6 (Python Software Foundation). The process of how the cohort was obtained is detailed in Figure 1. Overall, 2984 patients were seen in the Windland Smith Rice Genetic Heart Rhythm Clinic between January 1, 1999, and December 31, 2018. One or more digitally recorded ECGs with a verified QT/QTc were available for 2059 of these patients who were either diagnosed with LQTS or who were evaluated for the suspicion of LQTS but were ultimately discharged from the clinic without this diagnosis (Table). For this study, this group of fully evaluated patients served as our control group. The 2059 patients in these 2 cohorts accounted for a total of 9085 ECGs. Of the 2059 patients, 1180 were men (57%) and 879 were women (43%); mean (SD) age at the time of the first ECG was 21.6 (15.6) years. Demographics of both cohorts are summarized in the Table, demonstrating an equal distribution of sex and mean age at first Mayo Clinic ECG for both study cohorts. In addition, as expected, the mean (SD) Schwartz scores were significantly higher for patients eventually diagnosed with LQTS compared with those considered to not have LQTS (3.3 [1.7] vs 1.8 [1.0]; P < .001) (Table). For these cohorts, we tested the model’s ability to differentiate patients with LQTS from those dismissed as normal (LQTS detection). Subsequently, to test the model’s ability to distinguish specific LQTS genetic subtypes (LQT1, 2, and 3 LQTS genotype classification) (Figure 1). Overall, based on a patient’s first Mayo Clinic ECG—the potentially diagnostic one—patients with LQTS had a significantly higher mean (SD) QTc compared with normal (LQTS: 467 milliseconds vs normal: 429 milliseconds; P < .001) (Table). However, the AI-ECG outperformed the QTc determined on the ECG alone in distinguishing patients with LQTS from those who were dismissed as normal with an AUC of 0.900 (95% CI, 0.876-0.925; accuracy, 82.5%; sensitivity, 83.7%; specificity, 80.6%; positive predictive value [PPV], 83.2%; negative predictive value [NPV], 81.3%; and F1, 83.6%) compared with 0.824 for the QTc alone (95% CI, 0.79-0.858; accuracy, 76.0%; sensitivity, 74.1%; specificity, 77.7%; PPV, 79.2%; NPV, 72.4%; and F1, 76.7%) (Figure 2A). Furthermore, testing the model’s ability to distinguish persistent QT interval prolongation from a potentially incidental finding, our model slightly improved when using the mean of all available ECGs for a patient (AUC, 0.914; 95% CI, 0.892-0.937; accuracy, 84.7%; sensitivity, 83.7%; specificity, 85.4%; PPV, 86.8%; NPV, 82.1%; and F1, 85.2%) compared with an AUC of 0.847 when using the QTc alone (95% CI, 0.817-0.877; accuracy, 76.1%; sensitivity, 75.4%; specificity, 77.0%; PPV, 78.9; NPV, 73.3%; and F1, 77.1%) (Figure 2B). The CNN output modestly correlated with a patient's Schwartz score showing that a higher Schwartz score correlated with a higher neural network output value (r2 = 0.28; P < .001) (eFigure 2 in the Supplement). A similar correlation was observed when independently analyzing patients with LQTS (r2 = 0.13; P < .001). Nevertheless, approximately 40% of patients with genetically confirmed LQTS had a normal QTc on their resting 12-lead ECG. We therefore set out to subsequently test our CNN on just patients with LQTS (concealed LQTS) or individuals dismissed as normal whose QTc on a 12-lead ECG was less than 450 milliseconds. In this analysis, again, the AI-ECG successfully distinguished patients with concealed LQTS on their first Mayo Clinic 12-lead ECG (AUC, 0.863; 95% CI, 0.824-0.903; accuracy, 78.7%; sensitivity, 80.6%; specificity, 77.7%; PPV, 66.2%; NPV, 88.1%; and F1, 72.7%) compared with the QTc alone (AUC, 0.741; 95% CI, 0.689-0.794; accuracy, 67.6%; sensitivity, 72.9%; specificity, 64.7%; PPV, 52.8%; NPV, 81.5%; and F1, 61.2%) (Figure 3). LQTS Genotype Classification Early identification of a patient’s genetic subtype can facilitate risk stratification and disease management. We therefore tested whether the AI-ECG was able to differentiate the 3 major genetic subtypes (LQT1, 2, and 3) and demonstrated that our model was able to do so. To do this we used the same model but changed the softmax outputs from 2 to 3 parameters classifying the 3 LQTS subtypes. In the test samples for each of the genotypic subsets, the CNN was best able to distinguish LQT2 (n = 109) from LQT1 (n = 149) and LQT3 (n = 32) (AUC, 0.944; 95% CI, 0.918-0.970; accuracy, 89.0%; sensitivity, 88.1%; specificity, 89.5%; PPV, 83.5%; NPV, 92.6%; and F1, 85.7%), followed by distinguishing patients with LQT1 from those with LQT2 or LQT3 (AUC, 0.921; 95% CI, 0.890-0.951; accuracy, 85.9%; sensitivity, 84.6%; specificity, 87.2%; PPV, 87.5%; NPV, 84.2%; and F1, 86.0%) (Figure 4A). Likely owing to the small numbers, the model’s performance in the identification of patients with LQT3 was lower (AUC, 0.863; 95% CI, 0.792-0.934; accuracy, 80.0%; sensitivity; 78.1%; specificity, 80.2%; PPV, 32.9%; NPV, 96.7%; and F1, 46.3%) (Figure 4B). Internal Validation Using Temporal Splitting In the absence of an external validation cohort, we performed an internal validation of our model by using a temporal- rather than a random-splitting approach. In this newly developed temporal-splitting model, the neural network—like our original model—again outperformed use of the QTc alone in distinguishing patients with LQTS from those who were dismissed as normal. Although the AUC for the LQTS detection was slightly lower than when using the randomly split model (0.900) vs the temporally split model (0.862), the overall performance metrics for the neural network between these 2 methods were indistinguishable as evidenced by overlapping 95% CIs: AUC for random sampling, 0.900 (95% CI, 0.876-0.925; accuracy, 82.5%; sensitivity, 83.7%; specificity, 80.6%; PPV, 83.2%; NPV, 81.3%; and F1, 83.6%) vs AUC for temporal splitting, 0.862 (95% CI, 0.812-0.912; accuracy, 78.9%; sensitivity, 79.0%; specificity, 78.7%; PPV, 87.2%; NPV, 67.0%; and F1, 82.9%). Similarly, no significant differences were observed for the concealed QT detection (AUC for random splitting, 0.863; 95% CI, 0.824-0.903 vs AUC for temporal splitting, 0.785; 95% CI, 0.703-0.867) and the LQTS genotype classification model. The receiver operating characteristic curves for performance of the model following temporal splitting are shown in eFigure 3 in the Supplement, and eTable 1 in the Supplement compares the AUCs. Because prediction of performance in rare diseases can be difficult, we also performed repeated analyses on a synthetic data set with different prevalences created by down-sampling the positive examples. As expected, the AUC, sensitivity, and specificity were not affected, but the PPV decreased with increasing prevalence (overall PPV, 86.8%; NPV, 82.4%). The PPV as a function of the prevalence is presented in eTable 2 in the Supplement. Characterized by QT prolongation on 12-lead ECGs, patients with LQTS can experience syncope, seizures, and sudden cardiac death as sequelae of its hallmark arrhythmia of torsades de pointes. Once the patient is fully evaluated and optimally treated, the risk of cardiac events is low; nevertheless, a small group of patients might experience potentially lethal, LQTS-triggered cardiac events.12 Although QT prolongation is LQTS’ pathognomonic feature, approximately 40% of patients with proven (genotype-positive) LQTS have a normal QT,9 highlighting a need for additional tools to identify at-risk patients. To this end, we developed and herein present a deep neural network that is able to distinguish the ECG of a patient with LQTS from the ECG of a patient who was evaluated for LQTS but discharged without this diagnosis (AUC, 0.900). Furthermore, this model was able to distinguish these 2 populations even when the QTc was normal (450 milliseconds) in both groups and differentiate the 3 most common LQTS subtypes (LQT1-3). In addition, the deep neural network correlated significantly with the patient’s clinical probability score for the diagnosis of LQTS (Schwartz score13; r2 = 0.28; P < .001). One of the main findings of our study is that our AI model outperformed evaluation of the ECG based on the QTc alone. This finding demonstrates the network’s ability to identify additional features in an ECG waveform of a patient with LQTS, despite its generally normal appearance to the expert cardiologist.9 In addition, although previous studies have identified unique ECG features associated with LQTS or its specific genetic subtypes, these features have mostly involved specific, human-selected features of the ECG, such as shape, slope, and overall morphologic characteristics of the T wave.2,6,7,14 In a step to involve AI, Hermans and colleagues15 built upon these T wave morphologic studies by developing a machine learning, support-vector model showing that vectorcardiographic parameters from the T wave can improve diagnosis of LQTS with the capacity to distinguish patients with LQTS from genotype-negative family members with an AUC up to 0.901 based on the model used. In contrast, our AI-ECG used unsupervised feature extraction in which an agnostic approach of the complete ECG waveform across all 12 leads of the ECG, as compared with the T wave alone, was analyzed during training, with network feature selection based on minimizing an error function. This unsupervised trained model was subsequently used to distinguish between our selected (thus supervised) subgroups. Given the nature of CNNs, we were unable to determine which ECG features were important for patient classification. Nonetheless, it is not surprising that substantial diagnostic information is discarded when a complex, 3-dimensional, nonlinear biological signal (ie, the ECG) is distilled to a single number (ie, the QTc) for the diagnosis of LQTS. Thus, use of the CNN added significant diagnostic power to the ECG, permitting it to distinguish patients with LQTS from those without the disease and classify the main genotypic subtypes. This approach could significantly aid both experienced and inexperienced electrophysiologists when presented with a patient with a suspicion of LQTS in establishing this diagnosis and guiding genetic test prediction and initial treatment strategies as a bridge to genetic testing by giving an a priori designation of a possible genetic subtype. These findings could be combined with the currently used clinical score for probability of LQTS: the Schwartz score. This score was developed in 1985 by Schwartz to provide a clinical, diagnostic score to better estimate the clinical likelihood of congenital LQTS and guide treatment, family screening, and phenotype-directed genetic testing, and the test has been used and revised with updated knowledge of the disease.13,16 With a range of points assigned from 0.5 to 3 for each of the LQTS-attributable ECG findings and clinical markers, a score greater than or equal to 3.5 is associated with a high likelihood of LQTS.10,17 In this scenario, a QTc greater than or equal to 480 milliseconds already assigns 3 points, demonstrating the importance and weight of the QTc in this disease and, in reverse, downgrades its likelihood for patients with concealed LQTS. It is here that our model’s performance in patients with concealed LQTS may play an important role. In addition to functioning in providing an a priori likelihood of LQTS score based solely on the ECG, our model and deep neural network output could potentially be implemented into the Schwartz score, especially to better identify and risk stratify a patient with true LQTS with a low QTc value that would be assigned 2 points or only even 1 point based on the ECG findings alone. Identifying At-Risk Patients Our initial model was run on all patients with LQTS, many of whom had QT prolongation on their ECGs, permitting diagnosis by the Schwartz criteria. However, the subset of patients with a definite (genetic) diagnosis of LQTS in the setting of a normal QTc (<450 milliseconds) are at high risk of underdetection and consequent clinical events. The AI-ECG was able to distinguish patients with LQTS from those dismissed as normal with an AUC of 0.863 and overall accuracy of 78.7%. Thus, the AI-ECG may help identify healthy-appearing individuals at risk for potentially lethal, LQTS-associated arrhythmias. These individuals do not meet the QTc cutoff values recommended by international screening criteria to warrant further evaluation.18,19 Although individuals with QTc values within the normal limits are at significantly lower risk of such arrhythmias, identification of these patients is still important to, at minimum, implement the potential life-saving measure of avoiding drugs that prolong the QT interval and perhaps institute prophylactic β-blocker therapy (class IIa recommendation) and help to identify family members who might be at (possibly higher) risk. Moreover, a previous study demonstrated the use of AI-ECG to detect depressed ventricular function using smartphone-based ECG electrodes.11 If confirmed to function with fewer leads, screening for prolonged QT using this approach could be offered as a point-of-care test at pharmacies and clinics to avoid administering QT-prolonging medications to individuals with concealed LQTS with an otherwise unsuspected high risk for cardiovascular events. A study on a rare disease, such as LQTS, has challenges and limitations. Studies such as these demonstrate that AI can be a useful tool and, when applied to the ECG, could provide a low-cost, easily accessible replacement for more expensive tests, such as the echocardiogram or magnetic resonance imaging. However, before these kinds of AI models can be implemented into clinical practice or incorporated into mobile technology, several limitations have to be recognized and steps have to be taken to increase its validity and generalizability. First, although the AI-ECG may facilitate diagnosis, it must be recognized that this model in its current form was built on patients presenting to a dedicated LQTS specialty center with control patients evaluated under the same suspicion (ie, patients referred under the suspicion of possible LQTS, but discharged without this diagnosis). Herein, although useful for the type of patients presenting to such a clinic to determine a clear diagnosis and treatment for their disease, both the relative uniqueness of the setting as well as the selection of patients with suspicion of possible LQTS but discharged without that diagnosis may limit its generalizability. Although we have provided an internal validation of our findings by applying an additional approach to temporal splitting of the data set, further internal validation awaits prospective enrollment of future patients seen and evaluated in our Windland Smith Rice Genetic Heart Rhythm Clinic. Second, the study lacks external validation and calibration from a cohort of similar patients evaluated at a different genetic heart rhythm clinic. Third, the performance of this neural network model compared with either healthy controls derived from an unselected cohort or the general population of ECGs is not known. To this end, to extend generalizability and potentially offer a model that could lead to a neural network that functions as a true QT screening tool, our model will need to be trained and validated in a larger, unselected population of patients to be able to develop an AI-ECG model for detection of LQTS and/or malignant QT prolongation when juxtaposed to an unselected background. However, because QT prolongation on an ECG is not unique to patients with LQTS, development of this model is dependent on recognition and differentiation of patients with other causes of acquired QT prolongation, such as medications or electrolyte abnormalities, which are seen in approximately 10% of patients who undergo ECG screening in the outpatient setting.20 In an AI model derived from cases and controls evaluated in a specialized genetic heart rhythm clinic, the AI-ECG was able to distinguish patients with electrocardiographically concealed LQTS from those discharged without the diagnosis and provide a 78.7% accurate pregenetic test anticipation of LQTS genotype status. Accepted for Publication: November 22, 2020. Published Online: February 10, 2021. doi:10.1001/jamacardio.2020.7422 Corresponding Author: Michael J. Ackerman, MD, PhD, Mayo Clinic Windland Smith Rice Genetic Heart Rhythm Clinic, Windland Smith Rice Sudden Death Genomics Laboratory, Mayo Clinic, 200 First St SW, Guggenheim 501, Rochester, MN 55905 ([email protected]). Author Contributions: Drs Bos and Attia had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: Bos, Noseworthy, Friedman, Ackerman. Acquisition, analysis, or interpretation of data: Bos, Attia, Albert, Noseworthy, Friedman. Drafting of the manuscript: Bos, Attia. Critical revision of the manuscript for important intellectual content: All authors. Statistical analysis: Bos, Attia. Obtained funding: Ackerman. Administrative, technical, or material support: Albert. Supervision: Noseworthy, Friedman, Ackerman. Conflict of Interest Disclosures: Dr Albert reported having a patent to artificial intelligence (AI) for QT assessment pending. Dr Friedman reported having a licensed agreement with AliveCor related to LongQT know-how and AI tools. Dr Ackerman is a consultant for Abbott, Audentes Therapeutics, Biotronik, Boston Scientific, Daiichi Sankyo, Invitae, LQT Therapeutic, Medtronic, MyoKardia, and UpToDate. Drs Bos, Attia, Noseworthy, Friedman, and Ackerman, and Mayo Clinic have an equity/royalty relationship with AliveCor Inc but without remuneration thus far. Dr Albert is an equity partner and employee of AliveCor Inc. No other disclosures were reported. Funding/Support: This work was supported by the Mayo Clinic Windland Smith Rice Comprehensive Sudden Cardiac Death Program and Mayo Clinic Center for Clinical and Translational Research through grant UL1TR002377 from the National Center for Advancing Translational Sciences, a component of the National Institutes of Health. Role of the Funder/Sponsor: The funding organizations had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. Additional Information: Screening criteria available at CredibleMeds (http://www.crediblemeds.org). et al. Architectural T-wave analysis and identification of on-therapy breakthrough arrhythmic risk in type 1 and type 2 long-QT syndrome. Circ Arrhythm Electrophysiol . 2017;10(11):e005648. doi:10.1161/CIRCEP.117.005648 et al. Automated T-wave analysis can differentiate acquired QT prolongation from congenital long QT syndrome. Ann Noninvasive Electrocardiol . 2017;22(6):e12455. doi:10.1111/anec.12455 MJ. Beyond the length and look of repolarization: defining the non-QTc electrocardiographic profiles of patients with congenital long QT syndrome. Heart Rhythm . 2018;15(9):1413-1419. doi:10.1016/j.hrthm.2018.04.033 et al; American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; American College of Cardiology Foundation; Heart Rhythm Society. AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part IV: the ST segment, T and U waves, and the QT interval: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology. Circulation . 2009;119(10):e241-e250. doi:10.1161/CIRCULATIONAHA.108.191096PubMedGoogle ScholarCrossref et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA). Heart Rhythm . 2011;8(8):1308-1339. doi:10.1016/j.hrthm.2011.05.020PubMedGoogle ScholarCrossref	2	14	2	0	0	0	0.709297	2	7,473
What is an ICD? An ICD (implantable cardioverter defibrillator) is an electronic device that constantly monitors your heart rhythm. When it detects a very fast, abnormal heart rhythm, it delivers energy to the heart muscle. This causes the heart to beat in a normal rhythm again. Why is an ICD needed? Ventricular tachycardia and ventricular fibrillation are two life-threatening heart rhythms that cause the heart to beat very fast. These conditions can be fatal if not treated immediately. Your doctor has recommended that you receive an ICD because you have had at least one episode of these heart rhythms or are at high risk of developing these types of heart rhythms. Who needs an ICD? An ICD may be recommended for people who: - Had a prior episode of sudden cardiac arrest - Had a prior episode of ventricular fibrillation - Had at least one episode of ventricular tachycardia - Had a prior heart attack and have an increased risk for sudden cardiac arrest or sudden cardiac death - Have hypertrophic cardiomyopathy Together, you and your doctor will determine if an ICD is the right treatment for you. How does an ICD work? The ICD monitors the heart rhythm, identifies abnormal heart rhythms and determines the appropriate therapy to return your heartbeat to a normal heart rhythm. Your doctor programs the ICD to include one or all of the following functions: - Anti-tachycardia pacing (ATP) – When your heart beats too fast, a series of small electrical impulses are delivered to the heart muscle to restore a normal heart rate and rhythm. - Cardioversion – A low energy shock is delivered at the same time as your heartbeat to restore a normal heart rhythm. - Defibrillation – When the heart is beating dangerously fast, a high-energy shock is delivered to the heart muscle to restore a normal rhythm. - Bradycardia pacing – When the heart beats too slow, small electrical impulses are sent to stimulate the heart muscle to maintain a suitable heart rate. Types of ICDs Is the ICD implant procedure safe? A device implant is generally a very safe procedure. However, as with any invasive procedure, there are risks. Special precautions are taken to decrease your risks. Please discuss your specific concerns about the risks and benefits of the procedure with your doctor. Where is the implant procedure performed? In most cases, the implant procedure takes place in a special room in the Electrophysiology Lab. When the epicardial implant approach is used, the procedure takes place in a surgical suite. What time should I report for my procedure? The day before your scheduled procedure, please call 800.223.2273 and ask for Pager Number 21215, the EP scheduling nurse. Please call between 3:00 p.m. and 5:00 p.m. to find out what time you should arrive. If your procedure is scheduled for a Monday, please call the Friday before. If your procedure is scheduled after a holiday, please call the last business day before the holiday. Please do not call the Cleveland Clinic Operator or the Admitting Office for information about your procedure. We will notify you if there are any changes to your schedule. Should I take my medications? If you take Coumadin, the results of your INR test (a blood test to evaluate the blood clotting) must be within a suitable range before the implant procedure can be performed. You will likely need to stop taking anticoagulant medications, including aspirin or warfarin (Coumadin), a few days before the procedure. Your doctor may also ask you to stop taking other medications, such as those that control your heart rate. Do not stop taking any of your medications without first consulting with your healthcare provider. Ask your doctor which medications you should stop taking and when to stop taking them. If you have diabetes, ask the nurse how to adjust your diabetes medications or insulin. Can I eat before the procedure? Eat a normal meal the evening before your procedure. However, DO NOT eat, drink or chew anything after midnight the night before your procedure. This includes gum, mints, water, etc. If you must take medications, take them with small sips of water. When brushing your teeth, do not swallow any water. What should I wear? - When getting ready, please do not wear makeup and remove nail polish. - Wear comfortable clothes when you come to the hospital. You will change into a hospital gown for the procedure. - Please leave all jewelry (including wedding rings), watches and valuables at home. - The clothing you are wearing that morning will be returned to the person who accompanies you. What should I bring? You will not need a robe or toiletries when you first arrive. You may pack these items and have a family member keep your bag until after the procedure. Bring a one-day supply of your prescription medications. Do not take these medications without first asking your doctor or nurse. You may bring guided imagery tapes or music and the appropriate player. What happens before the procedure? Before the procedure begins, a nurse will help you get ready. You will be given a hospital gown to change into. You may keep your clothes in a locker or you may give them to a family member. You will lie on a bed and the nurse will start an intravenous (IV) line in a vein in your arm or hand. The IV is used to deliver medications and fluids during the procedure. To prevent infection and to keep the device insertion site sterile: - You will receive an antibiotic through the IV at the beginning of the procedure. - One side of your chest will be shaved. - The area will be cleansed. - You will be covered from your neck to feet with sterile drapes. - A soft strap may be placed across your waist and arms to keep your hands from touching the sterile area. Will I be awake? A medication will be given through your IV to relax you and make you feel drowsy, but you will not be asleep during the procedure. Will I be monitored? Several monitors will be used during the procedure to constantly check things like your heart rhythm and blood pressure. (See box below). Monitors During the Procedure Defibrillator/pacemaker/cardioverter: Attached to one sticky patch placed on the center of your back and one on your chest. This allows the doctor and nurse to pace your heart rate if it is too slow, or deliver energy to your heart if the rate is too fast. Electrocardiogram or EKG: Attached to several sticky electrode patches placed on your chest, as well as inside your heart. Provides a picture on the monitors of the electrical impulses traveling through the heart. Blood pressure monitor: Connected to a blood pressure cuff on your arm. Checks your blood pressure throughout the procedure. Oximeter monitor: Attached to a small clip placed on your finger. Checks the oxygen level of your blood. Fluoroscopy: A large X-ray machine will be positioned above you to help the doctors see the leads on an X-ray screen during the procedure. How is the device implanted? The ICD is usually implanted using the endocardial (transvenous) approach. During the procedure, a local anesthetic (pain-relieving medication) is injected to numb the area. Small incisions are made in the chest where the lead(s) and device are inserted. The lead is inserted through the incision and into a vein, then guided to the heart with the aid of the fluoroscopy machine. The tip of the lead is attached to the heart muscle, while the other end is attached to the pulse generator. The generator is placed in a pocket created under the skin in the upper chest. When the endocardial approach is used, the hospital recovery time is generally 24 hours. Endocardial (transvenous) approach Epicardial (surgical) approach In rare cases when the endocardial approach cannot be used, the epicardial (surgical) approach is used. With this approach, you are put to sleep with general anesthesia. The generator is placed in a pocket created under the skin in the lower abdomen. The hospital recovery time for this approach is generally 3 to 5 days, although minimally invasive techniques may be used that can result in a shorter hospital stay and quicker recovery time. Your doctor will determine which is the best implant procedure approach for you. How are the leads tested? After the leads are in place, they are tested to make sure they are in the right place and working properly. This lead function test is called “pacing.” During the test, small amounts of energy are sent through the leads into the heart muscle. This energy causes the heart to contract. You will be asleep for several minutes during the lead function test. Once the leads have been tested, the doctor will connect them to the device. The rate and settings of your device are determined by your doctor. After the implant procedure, the doctor uses an external device (programmer) to make the final device settings. What will I feel? With the endocardial approach: You will feel a burning or pinching sensation when the doctor injects the local numbing medication. Then, the area will become numb. You may feel a pulling sensation as the doctor makes a pocket in the tissue under your skin for the device. Please tell your doctor what symptoms you are feeling. You should not feel pain. If you do, tell your nurse right away. How long does the procedure last? The device implant procedure may last from 2 to 4 hours. Will I have to stay in the hospital? Yes. You will be admitted to the hospital and stay overnight after the procedure. You will probably be able to go home the day after your device was implanted, unless the epicardial approach was used during the procedure. What should I expect during my recovery? A special monitor, called a telemetry monitor, will continually monitor your heart rhythm. The telemetry monitor consists of a small box connected by wires to your chest with sticky electrode patches. The nurses can see information about your heart’s rhythm on several monitors in the nursing unit. You will also have a holter monitor, which is a small recorder, attached to your chest with sticky electrode patches. The holter monitor records your heart rhythm for 12 hours to make sure the device is working properly. What tests will be done after the procedure? A chest X-ray will be done after the device implant procedure to check your lungs as well as the position of the device and lead(s). The monitor will be removed before you leave the hospital and your doctor will get the results. You will then go to the Device Clinic. What happens at the Device Clinic? You will sit in a reclining chair. Electrode patches will be placed on your chest and connected via wires to a computer. A nurse will place a small device called a programmer directly over the device. The programmer allows the nurse to change the device settings and to check the device and lead function. You may feel your heart beat faster or slower. Although this is normal, please tell the nurse what symptoms you are experiencing. The results of the device check are reported to your doctor, who then determines the appropriate settings for the device. The holter monitor results are also reviewed. You’ll also review your home-going instructions, including incision care, activity guidelines and follow-up schedule. An echocardiogram may be performed as part of the Device Clinic evaluation or may be performed at your next follow-up appointment. How will I feel? You may feel discomfort at the device implant site during the first 48 hours after the procedure. The doctor will tell you what medications you can take for pain relief. Please tell your doctor or nurse if your symptoms are prolonged or severe. What instructions will I receive before I leave the hospital? Your doctor will discuss the results of the procedure and answer any questions you have. Be sure to ask the doctor if you should continue taking your previous medications. You will receive specific instructions about how to care for yourself after the procedure, including medication guidelines, wound care, activity guidelines, device care and maintenance and a follow-up schedule. Also refer to the handout, “ICD Discharge Instructions.” You will receive a temporary ID card that tells you what type of device and leads you have, the device manufacturer, the date of the device implant and the doctor’s name who implanted the device. Within 3 months, you will receive a permanent ID card from the device company. It is important to carry this card at all times in case you need medical attention.. Will I be able to drive myself home after the procedure? No. For your safety, a responsible adult must drive you home. We request that your ride be ready to take you home by 10:00 a.m. on the morning of your discharge day. Ask your doctor when you can start driving again. When should I follow-up? A follow-up appointment to check your ICD will be scheduled within 6 weeks after the device implant procedure. The appointment will be scheduled automatically and you will receive an appointment notice in the mail. The ICD check is performed at the Device Clinic and takes about 15 to 30 minutes. The programmer will be placed over the device to retrieve device settings, measure battery voltage and collect information from the device about any fast heart rhythms and/or therapies you received. The leads will also be tested. This first follow-up appointment is critical because adjustments will be made that will prolong the life of your device. This appointment is for a device check with the electrophysiology nurse. If you need to see your doctor for follow-up care, you will need to schedule a separate appointment. How long does the ICD last? Your ICD can last 3 to 6 years. By keeping your follow-up appointments in the Device Clinic, your healthcare team can monitor the function of your device and anticipate when it needs to be changed. Managing your condition ICD therapy is only one part of a comprehensive treatment program. It is also important for you to take your medications, make changes to your diet, live a healthy lifestyle, keep your follow-up appointments and be an active member of your treatment team. Before your procedure: - Call the scheduling nurse between 3 p.m. and 5 p.m. the day before the procedure to get your procedure time. Call 800.223.2273 and ask for Pager 21215. - Do not eat or drink anything after midnight the night before the procedure. - Talk to your doctor about the medications you should take before the procedure. - Do not bring valuables. After your procedure: - You will be admitted to the hospital overnight. - Your doctor will discuss your medications, homegoing instructions and follow-up schedule. - Ask your doctor which medications you should be taking after your procedure. You may not need to take everything you needed before the implant. - Be sure to keep your follow-up appointments and make lifestyle changes as needed.	3	8	2	0	0	0	0.95136	2	3,197
\|This category reflects the organization of International Statistical Classification of Diseases and Related Health Problems, 10th Revision. Generally, diseases outlined within the ICD-10 codes F30-F39 should be included in this category. Mood disorder is the term given for a group of diagnoses in the DSM IV TR classification system where a disturbance in the person's emotional mood is hypothesised to be the main underlying feature. The classification is known as mood (affective) disorders in ICD 10. English psychiatrist Henry Maudsley proposed an overarching category of affective disorder. The term was then replaced by mood disorder, as the latter term refers to the underlying or longitudinal emotional state, whereas the former refers to the external expression observed by others. Two groups of mood disorders are broadly recognized; the division is based on whether the person has ever had a manic or hypomanic episode. Thus, there are depressive disorders, of which the best known and most researched is major depressive disorder commonly called clinical depression or major depression, and bipolar disorder, formerly known as "manic depression" and described by intermittent periods of manic and depressed episodes. \|Wikimedia Commons has media related to Mood disorders.\| This category has the following 7 subcategories, out of 7 total. Pages in category "Mood disorders" The following 62 pages are in this category, out of 62 total. This list may not reflect recent changes (learn more).	3	2	0	0	0	2	0.946293	2	294
I. "Telehealth” versus “Telemedicine": The Health Resources Services Administration (HRSA), an agency of the U.S. Department of Health and Human Services (HHS), has defined the term "telehealth" as the: "[U]se of electronic communication and information technologies to provide or support long-distance clinical health care, patient professional health-related education, public health, and health administration." The most common technologies used to provide telehealth services include videoconferencing, the internet, store-and-forward imaging, streaming media, and terrestrial and wireless communications. Telehealth is different from telemedicine because it refers to a broader scope of remote healthcare services than telemedicine. While telemedicine refers specifically to remote clinical services, telehealth can refer to remote non-clinical services, such as provider training, administrative meetings, and continuing medical education, in addition to clinical services. II. Background of Medicare Telehealth Coverage Requirements: The Balanced Budget ACT of 1997 (BBA) mandated that Medicare cover certain Fee-For-Service (FFS) payments for “telehealth” services beginning in 1999. The BBA also provided for the funding of telehealth demonstration projects. Initially, covered telehealth services were limited to only those provided to Medicare beneficiaries in health professional shortage areas (HPSAs). Additionally, the legislation mandated that Medicare practitioners be with the patient (and function as a “telepresenter”) when the telehealth consultation was conducted. Not surprisingly, these restrictions were viewed as practically unworkable by health care providers and patients alike. Subsequent legislation under the Benefits Improvement and Protection Act of 2000 (BIPA) amended portions of the BBA and expanded Medicare‘s telehealth payment rules. The list of telehealth services that may qualify for coverage under Medicare are released each year through the annual physician fee schedule rulemaking process. As a general rule, Medicare Part B will pay for covered telehealth services when they are furnished by an interactive telecommunications system, as long as the following requirements are met: - The physician or practitioner at the distant site must be licensed to furnish the service under State law. The physician or practitioner at the distant site who is licensed under State law to furnish a covered telehealth services described in this section may bill, and receive payment for, the service when it is delivered via a telecommunications system. - Under 42 C.F.R. § 410.78(b)(2), the following providers qualify as a “practitioner at the distant site:” - A physician as described in 42 C.F.R. § 410.20. - A physician assistant as described in 42 C.F.R. § 410.74. - A nurse practitioner as described in 42 C.F.R. § 410.75. - A clinical nurse specialist as described in 42 C.F.R. § 410.76. - A nurse-midwife as described in 42 C.F.R. § 410.77. - A clinical psychologist as described in 42 C.F.R. § 410.72. - A clinical social worker as described in 42 C.F.R. § 410.73. - A registered dietitian or nutrition professional as described in 42 C.F.R. § 410.134. - A certified nurse anesthetist as described in 42 C.F.R. § 410.69. Please note, as set out under 42 C.F.R. § 410.78(e)(1), clinical psychologists and clinical social workers cannot bill Medicare for medical evaluation and management services. They may only bill and receive payment for individual psychotherapy via a telecommunications system. Additionally, as provided under 42 C.F.R. § 410.78(e)(2), physician visits required under 42 C.F.R. § 483.40(c) may not be furnished as telehealth services. - The telehealth services are furnished to a beneficiary at an “originating site.” Under 42 C.F.R. § 410.78(b)(3), an originating site includes one of the following: - The office of a physician or practitioner - A critical access hospital (as described in section 1861(mm)(1) of the Social Security Act). - A rural health clinic (as described in section 1861(aa)(2) of the Social Security Act). - A Federally qualified health center (as defined in section 1861(aa)(4) of the Social Security Act). - A hospital (as defined in section 1861(e) of the Social Security Act). - A hospital-based or critical access hospital-based renal dialysis center (including satellites). - A skilled nursing facility (as defined in section 1819(a) of the Social Security Act). - A community mental health center (as defined in section 1861(ff)(3)(B) of the Social Security Act). - As required out under 42 C.F.R. § 410.78(b)(3), to qualify as an originating site, the location must also be: - Located in a health professional shortage area (HPSA), as defined under section 332(a)(1)(A) of the Public Health Service Act (42 U.S.C. 254e(a)(1)(A)), that is either outside of a Metropolitan Statistical Area (MSA) as of December 31st of the preceding calendar year or within a rural census tract of an MSA as determined by the Office of Rural Health Policy of the Health Resources and Services Administration as of December 31st of the preceding calendar year, or - Located in a county that is not included in a Metropolitan Statistical Area as defined in section 1886(d)(2)(D) of the Act as of December 31st of the preceding year, or - An entity participating in a Federal telemedicine demonstration project that has been approved by, or receive funding from, the Secretary as of December 31, 2000, regardless of its geographic location. The medical examination of the patient is under the control of the physician or practitioner at the distant site. Additionally, as set out under 42 C.F.R. § 410.78(c), a telepresenter is not required as a condition of payment unless it is determined to be medically necessary by the physician or practitioner at the distant site. Simply put, to qualify under Medicare as covered telehealth services, providers must take ensure that each element of the telehealth services comply fit within the restrictions set out above. III. Medicaid Telehealth Services: As with the case of Medicare, state Medicaid telehealth guidelines require that all qualified practitioners providing telehealth services meet the requirements of their State Practice Act. With respect to the coverage and payment of telehealth services, the Medicaid programs of 49 states and the District of Columbia currently pay for live video telehealth services as long as jurisdiction specific requirements have been met. For example, as long as all applicable requirements are met, Texas Medicaid will pay for live video and for store-and-forward telehealth services (in certain circumstances). In Texas, Medicaid also covers home telemonitoring as long as certain conditions are met. It is important that you conduct a careful review of your state’s requirements and restrictions before assuming that your state’s Medicaid program will cover telehealth services. IV. Private Payor Coverage of Telehealth Services: Over the last decade, considerable progress has been made with respect to the coverage and payment of telehealth services by private payors. This is due, in large part, to the passage of various private payor parity laws. At last count, 39 states and the District of Columbia have enacted such laws. Private payor parity laws vary from one jurisdiction to another. While some states laws focus on the types of specialty services qualify for telehealth coverage, other states have also enacted laws that require payers to treat telehealth-delivered care the same way as in-person care with respect to reimbursement. V. OIG Telehealth Report Findings: As the telehealth services industry has grown, regulatory compliance concerns have also increased. Both governmental and private payors have identified a number of program integrity risks that require ongoing monitoring and assessment. In recent years, the OIG has included a number of telehealth-related projects as part of their Work Plan. Most recently, the OIG identified the use of unauthorized telehealth originating site as areas of deficiency in its recent report titled “CMS Paid Practitioners for Telehealth Services that Did Not Meet Medicare Requirements.” As reflected in the OIG’s report, after reviewing a sample of 100 claims: - 24 claims were unallowable because the beneficiaries received services at nonrural originating sites, - 7 claims were billed by ineligible institutional providers, - 3 claims were for services provided to beneficiaries at unauthorized originating sites, - 2 claims were for services provided by an unallowable means of communication, - 1 claim was for a noncovered service, and - 1 claim was for services provided by a physician located outside the United States. Collectively, 31 out of 100 claims (31%) of the telehealth claims examined by the OIG did not meet Medicare’s requirements for coverage and payment. VI. Telehealth Fraud Cases: The following cases are illustrative of the various types of telehealth fraud cases that are being identified, investigated and prosecuted around the country: - (2019): New Jersey. In this case, a New Jersey physician plead guilty to working for two telemedicine companies for which he wrote medically unnecessary orders for orthotic braces for Medicare beneficiaries. His conduct resulted in $13 million in losses to Medicare. - (2019): New York. The government recently charged an anesthesiologist fir her role in telemedicine fraud. The physician allegedly ordered and prescribed durable medical equipment (DME) and prescription drugs in connection with purported telemedicine services. The government claims that the physician and other providers signed prescriptions and order forms for DME and drugs that were not medically necessary and that were induced by kickbacks, and provided for beneficiaries whom Steiner and others had not examined and evaluated. - (2019): Medellin, Colombia. In this case, the owner of a telemedicine company admitted that he and others agreed to solicit and receive illegal kickbacks and bribes from patient recruiters, pharmacies, brace suppliers and others in exchange for arranging for doctors to order medically unnecessary orthotic braces (braces) for beneficiaries of Medicare and other insurance carriers. The beneficiaries were contacted through an international telemarketing network that lured hundreds of thousands of elderly and/or disabled patients into a criminal scheme that crossed borders, involving call centers in the Philippines and throughout Latin America. - (2018): Tennessee. The government recently charged four individuals and seven companies with fraud for their involvement in an alleged fraud scheme that resulting in almost $1 billion in claims being submitted to insurance payors. The government has alleged that a defendant telemedicine company had their employees call and impersonate medical professionals in order to obtain information from patients. The telemedicine company then contacted local physicians and falsely advised them that an electronic consultation was ready for their review. The prescribing physicians were led to believe that a licensed health professional (not merely a telemedicine company employee) had examined the patient’s medical history and that the patients had requested the drug at issue. The case is ongoing. - (2018): California. In this case, seven defendants have been charged with fraud for their roles in a massive “sham” telemedicine scheme that defrauded TRICARE out of an estimated $65 million. The defendants included a nurse practitioner, a physician and two chiropractors, among others. In this case, it was alleged that one or more individuals recruited and paid Marines and the defendants to fill prescriptions for specialty medications (filled by co-conspirator pharmacies) that resulted in fraudulent and / or tainted claims being submitted to TRICARE for payment. This case remains ongoing. - (2018): Florida. In this case, a Florida compounding pharmacy paid the government $350,000 to settle allegations that it violated the federal False Claims Act when it filled prescriptions that did not meet TRICARE’s telemedicine coverage requirements. When investigating the case, agents for the Defense Criminal Investigative Service (DCIS) found that pharmacy representatives made “unsolicited calls to TRICARE beneficiaries, provided medically unnecessary compound medications to beneficiaries, and knowingly filled prescriptions from doctors who did not meet or properly consult with TRICARE beneficiaries.” - (2017): Florida. In this case, a Florida compounding pharmacy and its owner agreed to pay the government $170,000 for violations of the federal False Claims Act. The government alleged that the defendants knowingly submitted claims to TRICARE for compounded medications that did not qualify for coverage and payment because they the prescriptions were not issued pursuant to a valid physician-patient relationship. Instead, the prescriptions were issued after brief calls were conducted between the prescribing physician and the TRICARE beneficiary. The telephone calls made did not meet applicable telehealth coverage requirements. Notably, prosecutors also alleged that the prescriptions for compounded medications were not medically necessary AND were tainted because of kickbacks paid to marketers. - (2016): Connecticut. In this case, a Connecticut psychiatrist and his practice were sued by a whistleblower under the federal Civil Claims Act for improper submitting telehealth claims to Medicare for payment. As the government found, the psychiatrist provided psychiatric services over the phone to Medicare beneficiaries that did not meet Medicare’s coverage requirements. More specifically, treating a patient over the phone does not meet Medicare’s requirements that the services be provided using an interactive audio and video communications system that permits real-time communications between the physician and the Medicare beneficiary. Additionally, the Medicare patients receiving the telehealth services did not live in a HPSA nor meet any of the other requirements under 42 C.F.R. § 410.78(b)(3) to properly qualify as an originating site. To resolve the false claims allegations, the defendants paid the government $36,704. - (2016): Florida. In this complex case, multiple defendants (including a physician, a physician’s assistant, a pharmacist, individuals working for a telemarketing company and related corporate entities) were convicted of various charges arising out of their improper submission of fraudulent, tainted prescription claims to TRICARE for payment and resulting in approximately $5.7 million in payments. The government alleged that the clinicians with prescribing authority were paid kickbacks by the defendant pharmacist to sign prescriptions made out to TRICARE beneficiaries. In addition to the kickback-tainted prescriptions, it is worth noting that there was no valid provider-patient relationship. Moreover, the prescriptions did not meet TRICARE’s telemedicine coverage requirements. A number of the defendants have plead guilty to the charges and are awaiting sentencing. As the Medicare requirements above reflect, telehealth services are subject to a number of restrictions. First and foremost, not all services qualify for payments as telehealth services under Medicare’s coverage rules. Assuming that the telehealth services to be provided do, in fact, qualify for coverage by Medicare, several additional requirements must be met. For example, the originating site where the Medicare beneficiary is located must qualify as an authorized geographic location. Is the region being served a rural area that has been designated as a HPSA? The specific location where the beneficiary will be receiving the services (such as a physician’s office) must also fit within Medicare’s narrow list of sites where a beneficiary can receive qualifying telehealth services. Additionally, the type of practitioner providing the services at the distant site qualify as an eligible provider type under Medicare’s rules. Eligible providers must also meet applicable State Practice Act requirements to provide telehealth services. For instance, several state legislatures have enacted rules that require practitioners providing telehealth services across state lines to also hold a valid state license where the patient is located. Finally, the telehealth services must be provided through the use of an appropriate interactive telecommunications system. Are you providing telehealth services? If so, it is essential that you conduct a comprehensive review of the logistical, documentation, medical necessity, billing and coding requirements that must be met in order for the services to qualify for coverage and payment. Our attorneys can assist you with these efforts and / or represent you if your telehealth services are audited by Medicare, Medicaid or a private payor. - Telehealth in Rural America, Policy Brief March 2015. National Advisory Committee on Rural Health and Human Services. - During Calendar Year (CY) 2018, services that may qualify for coverage as telehealth services under Medicare include: Service HCPCS / CPT Code Telehealth consultations, emergency department or initial inpatient HCPCS codes G0425–G0427 Follow-up inpatient telehealth consultations furnished to beneficiaries in hospitals or SNFs HCPCS codes G0406–G0408 Office or other outpatient visits CPT codes 99201–99215 Subsequent hospital care services, with the limitation of 1 telehealth visit every 3 days CPT codes 99231–99233 Subsequent nursing facility care services, with the limitation of 1 telehealth visit every 30 days CPT codes 99307–99310 Individual and group kidney disease education services HCPCS codes G0420 and G0421 Individual and group diabetes self-management training services, with a minimum of 1 hour of in-person instruction to be furnished in the initial year training period to ensure effective injection training HCPCS codes G0108 and G0109 Individual and group health and behavior assessment and intervention CPT codes 96150–96154 Individual psychotherapy CPT codes 90832–90834 and 90836–90838 Telehealth Pharmacologic Management HCPCS code G0459 Psychiatric diagnostic interview examination CPT codes 90791 and 90792 End-Stage Renal Disease (ESRD)-related services included in the monthly capitation payment CPT codes 90951, 90952, 90954, 90955, 90957, 90958, 90960, and 90961 End-Stage Renal Disease (ESRD)-related services for home dialysis per full month, for patients younger than 2 years of age to include monitoring for the adequacy of nutrition, assessment of growth and development, and counseling of parents CPT code 90963 End-Stage Renal Disease (ESRD)-related services for home dialysis per full month, for patients 2-11 years of age to include monitoring for the adequacy of nutrition, assessment of growth and development, and counseling of parents CPT code 90964 End-Stage Renal Disease (ESRD)-related services for home dialysis per full month, for patients 12-19 years of age to include monitoring for the adequacy of nutrition, assessment of growth and development, and counseling of parents CPT code 90965 End-Stage Renal Disease (ESRD)-related services for home dialysis per full month, for patients 20 years of age and older CPT code 90966 End-Stage Renal Disease (ESRD)-related services for dialysis less than a full month of service, per day; for patients younger than 2 years of age (effective for services furnished on and after January 1, 2017) CPT code 90967 End-Stage Renal Disease (ESRD)-related services for dialysis less than a full month of service, per day; for patients 2-11 years of age (effective for services furnished on and after January 1, 2017) CPT code 90968 End-Stage Renal Disease (ESRD)-related services for dialysis less than a full month of service, per day; for patients 12-19 years of age (effective for services furnished on and after January 1, 2017) CPT code 90969 End-Stage Renal Disease (ESRD)-related services for dialysis less than a full month of service, per day; for patients 20 years of age and older (effective for services furnished on and after January 1, 2017) CPT code 90970 Individual and group medical nutrition therapy HCPCS code G0270 and CPT codes 97802–97804 Neurobehavioral status examination CPT code 96116 Smoking cessation services HCPCS codes G0436 and G0437 and CPT codes 99406 and 99407 Alcohol and/or substance (other than tobacco) abuse structured assessment and intervention services HCPCS codes G0396 and G0397 Annual alcohol misuse screening, 15 minutes HCPCS code G0442 Brief face-to-face behavioral counseling for alcohol misuse, 15 minutes HCPCS code G0443 Annual depression screening, 15 minutes HCPCS code G0444 High-intensity behavioral counseling to prevent sexually transmitted infection; face-to-face, individual, includes: education, skills training and guidance on how to change sexual behavior; performed semi-annually, 30 minutes HCPCS code G0445 Annual, face-to-face intensive behavioral therapy for cardiovascular disease, individual, 15 minutes HCPCS code G0446 Face-to-face behavioral counseling for obesity, 15 minutes HCPCS code G0447 Transitional care management services with moderate medical decision complexity (face-to-face visit within 14 days of discharge) CPT code 99495 Transitional care management services with high medical decision complexity (face-to-face visit within 7 days of discharge) CPT code 99496 Advance Care Planning, 30 minutes (effective for services furnished on and after January 1, 2017) CPT code 99497 Advance Care Planning, additional 30 minutes (effective for services furnished on and after January 1, 2017) CPT code 99498 Psychoanalysis CPT code 90845 Family psychotherapy (without the patient present) CPT code 90846 Family psychotherapy (conjoint psychotherapy) (with patient present) CPT code 90847 Prolonged service in the office or other outpatient setting requiring direct patient contact beyond the usual service; first hour CPT code 99354 Prolonged service in the office or other outpatient setting requiring direct patient contact beyond the usual service; each additional 30 minutes CPT code 99355 Prolonged service in the inpatient or observation setting requiring unit/floor time beyond the usual service; first hour (list separately in addition to code for inpatient evaluation and management service) CPT code 99356 Prolonged service in the inpatient or observation setting requiring unit/floor time beyond the usual service; each additional 30 minutes (list separately in addition to code for prolonged service) CPT code 99357 Annual Wellness Visit, includes a personalized prevention plan of service (PPPS) first visit HCPCS code G0438 Annual Wellness Visit, includes a personalized prevention plan of service (PPPS) subsequent visit HCPCS code G0439 Telehealth Consultation, Critical Care, initial, physicians typically spend 60 minutes communicating with the patient and providers via telehealth (effective for services furnished on and after January 1, 2017) HCPCS code G0508 Telehealth Consultation, Critical Care, subsequent, physicians typically spend 50 minutes communicating with the patient and providers via telehealth (effective for services furnished on and after January 1, 2017) HCPCS code G0509 Counseling visit to discuss need for lung cancer screening using low dose CT scan (LDCT) (service is for eligibility determination and shared decision making (effective for services furnished on and after January 1, 2018) HCPCS code G0296 Interactive Complexity Psychiatry Services and Procedures (effective for services furnished on and after January 1, 2018) CPT code 90785 Health Risk Assessment (effective for services furnished on and after January 1, 2018) CPT codes 96160 and 96161 Comprehensive assessment of and care planning for patients requiring chronic care management (effective for services furnished on and after January 1, 2018) HCPCS code G0506 Psychotherapy for crisis (effective for services furnished on and after January 1, 2018) CPT codes 90839 and 90840 For ESRD-related services, a physician, NP, PA, or CNS must furnish at least one “hands on” visit (not telehealth) each month to examine the vascular access site. - Under 42 C.F.R. § 410.78(a)(3), the term “interactive telecommunications system” means:"[M]ultimedia communications equipment that includes, at a minimum, audio and video equipment permitting two-way, real-time interactive communication between the patient and distant site physician or practitioner. Telephones, facsimile machines, and electronic mail systems do not meet the definition of an interactive telecommunications system." (emphasis added). Although the technologies described above do not qualify as interactive telecommunications systems, they still qualify as telehealth services (albeit non-covered telehealth services under Medicare). - 42 C.F.R. § 483.40(c) services that may not be furnished by a physician as telehealth services include behavioral health related rehabilitation services such as physical therapy, speech-language pathology, occupational therapy, and rehabilitative services for mental disorders and intellectual disability, as required in a resident’s comprehensive plan of care. - An originating site is the location of the qualified eligible Medicare patient when the telehealth service is furnished by a physician or practitioner at the distant site. Originating sites are paid a fee for hosting the telehealth service. In 2018, the fee paid to an originating site was $25.76. Please note, under the Bipartisan Budget Act of 2018, the originating site fee will be paid in certain circumstances. - The definition of “store-and-forward” telehealth services varies from state to state. Generally, store-and-forward telehealth services are those where information, images, sound, video, etc., are stored or recorded and later forwarded to another site for clinical evaluation. In other words, it is not a real-time, live evaluation of a patient by a distant provider. - Center for Connected Health Policy, “State Telehealth Laws & Reimbursement Policies.” (Fall 2018). - A-05-16-00058 (April 2018). - Ibid, page 5. - Of the nearly $1 billion in claims submitted to payors for payment, only $174 million was ultimately paid by the payors. Nevertheless, federal prosecutors will likely argue that the face value of the claims billed (not necessary paid) is the amount that should be used when calculating the defendants’ potential period of imprisonment under the Sentencing Guidelines.	4	67	3	0	2	0	0.242921	5	5,551
About this course: This course reviews the anatomy and physiology of the heart, including blood flow and electrical activity. The course also examines the principles for assessing a telemetry strip and the variations in telemetry monitoring. Finally, this course reviews the pathophysiology, risk factors, signs and symptoms, telemetry features, and essential treatment and management for various arrhythmias. This course reviews the anatomy and physiology of the heart, including blood flow and electrical activity. The course also examines the principles for assessing a telemetry strip and the variations in telemetry monitoring. Finally, this course reviews the pathophysiology, risk factors, signs and symptoms, telemetry features, and essential treatment and management for various arrhythmias. After this activity, learners will be prepared to: define the terms and recall the anatomy and physiology of the heart briefly discuss the variations of telemetry monitoring and how to assess a telemetry strip identify the characteristics of normal sinus rhythm and its variations, including sinus arrhythmia, sinus bradycardia, sinus tachycardia, sinus arrest, and sick sinus syndrome identify supraventricular arrhythmias on telemetry, including premature atrial contractions, atrial tachycardia, atrial flutter, atrial fibrillation, and wandering pacemaker, and illustrate their pathophysiology, risk factors, protective factors, signs and symptoms, telemetry features, other associated diagnostic tests, nursing care, and basic treatment/management identify junctional arrhythmias on telemetry, including Wolff-Parkinson-White, premature junctional contractions, junctional escape rhythm, accelerated junctional rhythm, and junctional tachycardia, and illustrate their pathophysiology, risk factors, protective factors, signs and symptoms, telemetry features, other associated diagnostic tests, nursing care, and basic treatment/management identify ventricular arrhythmias on telemetry, including premature ventricular contractions, idioventricular rhythms, ventricular tachycardia, Torsades de Pointes, ventricular fibrillation, asystole, and pulseless electrical activity, and illustrate their pathophysiology, risk factors, protective factors, signs and symptoms, telemetry features, other associated diagnostic tests, nursing care, and basic treatment/management identify heart blocks on telemetry, including first-degree, second-degree type I, second-degree type II, or third-degree SA and AV blocks, as well as bundle branch blocks and illustrate their pathophysiology, risk factors, protective factors, signs and symptoms, telemetry features, other associated diagnostic tests, nursing care, and basic treatment/management Healthcare providers (HCPs) across various clinical environments will care for patients with known cardiac arrhythmias or risk factors for future arrhythmias. Arrhythmias encompass a broad spectrum of cardiac abnormalities related to heart rate (HR) and rhythm. The most common way to categorize arrhythmias is based on the rate of conduction, including bradyarrhythmia and tachyarrhythmia. Arrhythmias are further categorized based on their origin, means of transmission, and associated clinical syndromes. The prevalence of arrhythmias within the general population is 1.5% to 5%, with atrial fibrillation the most commonly occurring. However, these statistics underrepresent the true prevalence of arrhythmias since many are paroxysmal (i.e., sudden, and short in duration) and asymptomatic. Patients experiencing arrhythmias may exhibit significant variations in clinical presentation, ranging from asymptomatic to cardiac arrest. Given that arrhythmias are associated with higher morbidity and mortality, HCPs must be adequately prepared to recognize signs and symptoms of various arrhythmias, including the principles of telemetry monitoring and arrhythmia identification. In addition, HCPs must understand evaluation and treatment/management strategies for the various arrhythmias (Desai, 2021). Anatomy and Physiology The heart is a strong, muscular pump that circulates blood throughout the body. Each day, the heart beats approximately 100,000 times and pumps 2,000 gallons of blood. The heart consists of four chambers: the right atrium, which accepts deoxygenated blood from the body via the veins; the right ventricle, which pumps that deoxygenated blood to the lungs via the pulmonary artery; the left atrium, which accepts newly oxygenated blood from the pulmonary veins; and the left ventricle, which pumps oxygenated blood to the rest of the body via the aorta. These four chambers are separated with four ...purchase below to continue the course The contraction of the heart muscle is a highly coordinated and precisely timed series of events. During diastole, the atria fill from the venous system (i.e., the superior vena cava and left pulmonary veins), and passive filling of the ventricles occurs. The atria contract simultaneously, filling the ventricles with the remainder of the blood via the open mitral and tricuspid valves, also known as the atrioventricular or AV valves. During systole, the full ventricles contract, closing the mitral and tricuspid valves (creating the S1 sound) and opening the aortic and pulmonary valves (or semilunar valves) to allow the blood into the pulmonary arteries and aorta. Once empty, decreased pressure inside the ventricles causes the semilunar valves to close again while the atria fill. This valve closure causes the second heart sound, or S2, which marks the start of diastole. Ventricular filling may be heard as S3, or if the ventricles are resistant and filling is slow, S4 (see Figure 2; AHA, 2016a; Hinkle and Cheever, 2018; Jackson Health System, 2018). An electrical impulse initiates the sinoatrial (SA) node contraction, also known as nature’s pacemaker. The impulse is transmitted from the SA node through the atria via Bachman’s bundle and the internodal pathways to the atrioventricular (AV) node. The impulse passes through the AV node down through the ventricles via the bundle of His, the right and left bundle branches, and the Purkinje fibers. A healthy, properly functioning SA node will keep the heart pumping between 60 and 100 beats per minute (bpm). If the SA node becomes damaged and is unable to send an impulse, the AV node, the heart’s first backup system, will create its own electrical impulse of 40-60 bpm. Finally, the Purkinje fibers can also act as a tertiary pacemaker, but only at 20-40 bpm. The ability of cardiac cells to contract after receiving a signal is called contractility. This electrical signal has a depolarizing effect on the cardiac cells. The ability of a cardiac cell to initiate an impulse is called automaticity, while the ability to react to the impulse is called excitability, and the ability of a cell to transmit an impulse is called conductivity. The signal from the SA node triggers rapid depolarization, which consists of sodium (Na+) quickly entering the cells and calcium (Ca+) moving slowly into the cells. Repolarization can be broken down further into three stages: early repolarization, which consists of Na+ channels closing; the plateau phase, which consists of Ca+ moving into the cell and potassium (K+) moving out of the cell; and rapid repolarization, which consists of the Ca+ channels closing and K+ moving out of the cell rapidly. The resting phase between impulses actively pumps K+ in and Na+ out of the cell. The cell is impermeable to Na+ diffusion during the resting phase, but K+ can slowly diffuse out of the cell (see Figure 3; AHA, 2016a; Hinkle & Cheever, 2018; Jackson Health System, 2018). Risk Factors for Cardiac Arrhythmias The term arrhythmia refers to abnormalities in HR or rhythm caused by changes in heart tissue and activity or in the electrical signals that control the heartbeat. Typically, arrhythmias are set off by a trigger, such as exertion or stress, and can persist if there is a problem with the heart (National Heart, Lung, and Blood Institute [NHLBI], n.d.) The NHLBI lists various causes of arrhythmias, including: - changes to the heart - anatomical changes - reduction in blood flow to the heart - damage to the heart’s electrical system - restoring blood flow during treatment for a heart attack - stiffening (i.e., fibrosis or scarring) of the heart tissue - stress or exertion - pain, anger, emotional stress, or startling can increase the workload of the heart, raise blood pressure, and cause the body to release stress hormones - vomiting or coughing can trigger arrhythmias - physical activity in individuals with heart disease can cause arrhythmias due to excess adrenaline - imbalances in the blood - excessive thyroid hormone can cause tachycardia, while thyroid hormone deficiency can cause bradycardia - dehydration can cause tachycardia - hypoglycemia can cause bradycardia or extra heartbeats - hypokalemia, hypomagnesemia, and hypocalcemia can cause arrhythmias - blood pressure medication, antidepressants, antipsychotics, antibiotics, and allergy or cold medicines can cause arrhythmias - conduction disorders - electrical signals can be delayed or blocked - another part of the heart produces electrical signals, disrupting the normal heartbeat (NHLBI, n.d.) Although signs and symptoms vary depending on the type of arrhythmia, general signs and symptoms include anxiety, blurry vision, chest pain, difficulty breathing, syncope, confusion or foggy thinking, fatigue, excessive sweating, weakness, dizziness, and lightheadedness (AHA, 2016d). The NHLBI and AHA list several risk factors for developing/experiencing an arrhythmia, including: - increased age - air pollutants such as particulates and gases - family history or genetics: there is an increased risk for arrhythmia if a patient’s parent or another immediate relative has been diagnosed with an arrhythmia and an increased risk with certain inherited cardiac conditions and genetic mutations (e.g., malfunctioning ion channels) - alcohol abuse - tobacco or illicit drug use (e.g., cocaine and amphetamines) - certain medical conditions such as aneurysm, rheumatoid arthritis (RA), lupus, diabetes mellitus (DM), cardiomyopathy, eating disorders, myocardial infarctions (MIs), congestive heart failure (CHF), hypertension (HTN), hypoglycemia, chronic obstructive pulmonary disease (COPD), obesity, or obstructive sleep apnea - race/ethnicity: atrial fibrillation (AFib) is more common in Caucasians; the risk for other arrhythmias is increased in African Americans - sex: AFib is more common in men, while supraventricular tachycardias (SVT) are more common in women - following surgery: there is an increased risk of atrial flutter in the weeks following significant heart, lung, or esophageal surgery (AHA, 2016d; NHLBI, n.d. This learning activity will cover additional risk factors for specific arrhythmias later. The NHLBI also lists protective factors, including a heart-healthy lifestyle of regular exercise, a diet high in fruits, vegetables, and fiber and low in saturated fats and salt, avoiding illicit drugs, tobacco, and excessive alcohol, and managing stress on a regular daily basis (NHLBI, n.d.). HCPs should counsel patients regarding diet and lifestyle choices to limit their risk for arrhythmias, and caffeine consumption is often a recommended area in which patients can make beneficial changes. Many providers will recommend reducing caffeine intake. Although excessive caffeine may impact cardiac function, moderate intake is likely acceptable. A recent study of 51 adult patients with CHF and moderate to severe systolic dysfunction (as defined by left ventricular ejection fraction [EF] of < 45% and a New York Heart Association [NYHA] functional class rating of I-III) were given either 500 mg of caffeine or a placebo with no significant increase in arrhythmias seen in the intervention group (Zuchinali et al., 2016). The HCP should pay close attention to the auscultation of heart sounds, palpating peripheral pulses, and auscultating the apical pulse if necessary for patients with a known or suspected arrhythmia. While auscultating, count the pulse rate and note any irregularity in the heart rhythm and any audible murmurs. The HCP should check for lower extremity swelling, indicating CHF or fluid overload. The lungs should be auscultated, noting any crackles or other adventitious sounds. The nurse should ask the patient for a full history of present illness and all medications they are taking, including prescribed, over-the-counter (OTC), and herbal medications. In addition, the HCP should also gather information on signs or symptoms of arrhythmias or risk factors for arrhythmias (i.e., coughing may indicate exposure to pollutants or tobacco smoke). When completing the health history, physical activity, eating habits, and family history are also important to gather (AHA, 2016b; NHLBI, n.d.). Bloodwork may help determine the cause or risk factors for arrhythmias, including thyroid-stimulating hormone (TSH), a metabolic profile with electrolytes, a toxicity screen, and any therapeutic medication levels, as these are common reversible causes of many arrhythmias. A chest x-ray may be ordered to assess for various lung abnormalities and determine the presence of cardiomegaly. An electrocardiogram (EKG) is the primary screening test for detecting arrhythmias. More invasive testing may be done if abnormalities are seen in labs, imaging, or electrocardiographic (ECG) monitoring. This may include an echocardiogram (echo) to assess the heart’s size, shape, and function, a cardiac catheterization to assess for complications of heart disease, or an electrophysiology study (EPS) to assess the heart’s electrical activity. Cardiac catheterization carries an increased risk of bleeding, infection, vessel or heart damage, blood clot formation/embolization, or the development of a new arrhythmia. In an EPS, a wire is inserted to stimulate the heart and trigger an arrhythmia to assess risk and test potential treatments. Finally, a stress test may be ordered to assess for arrhythmias when the heart is working hard. For patients who report transient palpitations, a Holter or event monitor can be used to record the heart’s electrical activity over a longer period. Another option is an implantable loop recorder placed under the skin to continuously record the heart’s electrical activity. The recordings can be transmitted directly to the HCP (AHA, 2016b; NHLBI, n.d.). Telemetry Monitoring Basics The use of telemetry or ECG monitoring has become more widely used in hospital settings. The need for more ECG monitoring has moved beyond simple rhythm determination to include the diagnosis of complex arrhythmias, detection of MIs, and identification of drug-induced prolonged QT intervals. The AHA published guidelines in 2004 regarding which patients are appropriate candidates for ECG monitoring. Since then, the AHA has updated these guidelines to address the overuse of ECG monitoring more comprehensively in some populations, the underuse of ST-segment and QT-interval monitoring in select populations, alarm fatigue, and documentation in the electronic health record. These guidelines exclude patients admitted to the intensive care unit (ICU), as these patients are significantly more complicated and often require continuous monitoring. On a medical-surgical floor, the decision for telemetry monitoring is more nuanced (Sandau et al., 2017). The AHA outlines four primary rationales for monitoring a patient with telemetry on a medical or surgical floor in a hospital: - to detect cardiac arrest sooner and therefore reduce the time to defibrillation - to recognize deteriorating conditions (i.e., early afterdepolarizations or non-sustained arrhythmias) - to facilitate the management of arrhythmias (even if not life-threatening) - to facilitate the diagnosis of an arrhythmia or a cause for specific signs/symptoms (Sandau et al., 2017) In addition to these general goals of telemetry monitoring, the AHA guidelines recommend monitoring in specific discrete patient populations. Cardiac arrest Is the leading cause of death and the most common cause of death after an MI in adults in the US. There is a substantial risk of death in the early hours to days after an MI, highlighting the importance of early recognition, evaluation, and ECG monitoring for patients with acute ischemic events. The most recent AHA (Sandau et al., 2017) recommendations for ECG monitoring for arrhythmias include: - immediately for all patients at immediate or high risk of ACS and those with documented ST-segment elevated myocardial infarction (STEMI; monitoring should continue uninterrupted for 24 to 48 hours or until negative biomarkers [i.e., troponin] or successful revascularization) - immediately on presentation of an MI and continued for 12 to 24 hours after reperfusion - immediately after an MI with no reperfusion or revascularization and continued for 24 to 48 hours until there is no evidence of ongoing modifiable ischemia or hemodynamic or electric instability - patients treated with therapeutic hypothermia after cardiac arrest - patients with vasospastic angina until symptoms resolve - patients with left ventricular apical ballooning until symptoms resolve - patients with a newly recognized critical left coronary artery stenosis while awaiting revascularization - after nonurgent PCI with suboptimal results or complications, beginning immediately and continuing for over 24 hours until the complication is resolved - after routine angiography is not recommended in low-risk patients (beyond the immediate post-procedure area) - monitoring is not indicated for patients with ACS symptoms determined to be low risk or noncardiac in origin - patients with uncomplicated open-heart surgery for a minimum of 48 to 72 hours postoperatively; for patients at high risk for AFib, monitoring should be done for the duration of the hospital stay - patients who are hemodynamically unstable with an immediate need for mechanical circulatory support - patients in the postoperative period after ventricular assist device (VAD) implantation; monitoring is not indicated for patients with VADs in rehabilitation facilities or when admitted with noncardiac problems - patients with a transcatheter aortic valve replacement (TAVR) should be monitored at least 3 days post-procedure and longer in patients with periprocedural conduction abnormalities - patients undergoing transcatheter intervention should be monitored after the procedure (duration depends on procedure and patient factors) - patients with life-threatening ventricular arrhythmias, resuscitated from cardiac arrest, or unstable ventricular tachycardia should be monitored until ICD implantation or reversible cause is corrected - in patients with premature ventricular contractions (PVCs) and nonsustained ventricular tachycardia, monitoring is not required unless there are other indicators present - patients with new-onset or recurrent atrial tachyarrhythmias should be monitored throughout the evaluation; patients with symptomatic or hemodynamically unstable atrial arrhythmias should be monitored until stable; monitoring should also be done if rate control is deemed necessary - patients with known AFib and who have adequate rate control, ECG monitoring is not indicated - patient with symptomatic bradycardia (e.g., syncope) but is not recommended for asymptomatic or hemodynamically stable bradycardia; may be beneficial for initiating chronotropic medications, where worsening baseline sinus bradycardia may be a concern - inpatient initiation of the following antiarrhythmic medications: dofetilide (Tikosyn), sotalol (Betapace), flecainide (Tambocor), propafenone (Rythmol); monitoring may be considered for amiodarone (Cordarone) and dronedarone (Multaq) initiation - patients with symptomatic second-degree AV block, asymptomatic second-degree AV block caused by distal conduction system disease, and third-degree AV block; patients with Wenckebach or transient AV block of any degree determined to be of vagal origin do not need monitoring - patients with congenital or genetic arrhythmia syndromes who are hemodynamically unstable until appropriate therapy is delivered - patients with Wolff-Parkinson-White (WPW) syndrome with rapid conduction via an accessory pathway until antiarrhythmic or ablation therapy is delivered - patients with a congenital long QT interval who are unstable should be monitored until stabilization of ventricular arrhythmias, reversal of exacerbating metabolic or medical conditions, and return of QTc to baseline - patients who are admitted for syncope with suspected cardiac origin should be monitored for at least 24 hours - patients with uncomplicated supraventricular tachycardia ablation may be discharged home after a short observation period - patients with complex ablations (e.g., pulmonary vein isolation for AFib) or serious comorbidities (e.g., heart failure) should be monitored for 12 to 24 hours after the procedure; this includes patients receiving atrioventricular nodal ablation who experience prolonged tachycardia and those with chronic AFib with concomitant pacemaker implantation - patients who receive transcutaneous pacing or standard temporary transvenous wires should be monitored until it is no longer required, or the device is replaced with a permanent device - patients receiving semipermanent transvenous pacing should be monitored for 24 hours - patients receiving a permanent pacemaker or ICD should be monitored for 12 to 24 hours after implantation - patients admitted after an ICD shock should be monitored for the duration of the hospital stay; monitoring is not recommended for patients with an ICD admitted for noncardiac conditions who do not meet another indication - monitoring is not recommended for patients with a wearable cardiac defibrillator who are admitted for noncardiac reasons - patients with acute decompensated heart failure should be monitored until the precipitating event is resolved - patients with infective endocarditis until clinically stable - patients undergoing conscious sedation until awake, alert, and hemodynamically stable - monitoring is not recommended after noncardiac surgery for asymptomatic patients; exceptions include major thoracic surgery (e.g., pulmonary resection) who should be monitored for 2 to 3 days after surgery and patients with risk factors for AFib - patients admitted with stroke should be monitored for 24 to 48 hours - patients with moderate to severe potassium or magnesium imbalance - patients who sustained a drug overdose should be monitored until they are clinically stable and free from the influence of the drug (i.e., psychotropic drugs, opiates, inhalants, cocaine, stimulants, and other drugs) - efficacy of monitoring patients admitted to the hospital who receive chronic hemodialysis is not well established (Sandau et al., 2017) Despite the comprehensive list of indications for ECG monitoring, a recent study in hospitalized patients conducted by Dai and colleagues (2016) found that the initial ECG was prompted by the patient’s report of signs or symptoms in over half of the cases. In less than 20% of cases, it was prompted by a change in vital signs. Therefore, HCPs must pay close attention when taking a patient history to recognize and respond to signs and symptoms of arrhythmias. These researchers also found that patient outcomes improved significantly across the entire hospital system when nurses and other staff were adequately and regularly educated about the various signs and symptoms of an MI or arrhythmia (Dai et al., 2016). The AHA revised the 2004 guideline recommendations for ST-segment ECG monitoring. The 2004 guidelines recommended continuous ST-segment monitoring for four patient populations: - during the early phase of acute coronary syndrome [ACS], - patients presenting to the emergency department with chest pain, - after nonurgent percutaneous coronary intervention [PCI] with suboptimal results, - possible variant angina caused by coronary vasospasm. The benefit of continuous ST-segment monitoring includes early identification of ischemic events, leading to prompt revascularization and prevention of myocardial tissue damage. However, the AHA updated guidelines for continuous ST-segment monitoring changed the class of recommendation (COR) from an I to IIa, because of false (i.e., no valid triggering event) and nonactionable (i.e., correctly sound but for an event with no clinical relevance) alarm signals. These false and nonactionable signals have led to alarm fatigue and sentinel events (Sandau et al., 2017). A hospital in Delaware published findings in 2015 summarizing the effects of instituting the AHA guidelines regarding the monitoring of adult patients. In a similar period before implementing the AHA guidelines, they saw a decrease in the total number of alarms in a month from 4,106 to 3,094. Of these alarms, 78 were emergencies, 29 were clinically relevant, 14 led to a change in treatment, and one alarm was life-threatening in the 2 months examined. They concluded that life-threatening events are rare and that reducing unnecessary telemetry use is not likely to miss life-threatening events of any clinical importance (Kansara et al., 2015). The AHA guidelines call for technology and protocol improvement for continuous ST-segment monitoring while addressing and exploring remedies for alarm fatigue (Sandau et al., 2017). Similarly, Dressler and colleagues (2014) found that when the ECG monitoring orders in the electronic health record (EHR) were embedded with the list of approved AHA indications for ECG monitoring and the appropriate associated predetermined durations for those orders, they saw a 43% reduction in their number of monitored patients per week. They saw a 47% reduction in the average length of telemetry monitoring on a single patient and a reduction in cost for their telemetry monitoring services from an average of over $18,000 per day to less than $6,000 per day. They also instituted an order for a complete nursing assessment after ECG monitoring to determine if it was safe to discontinue monitoring. If it was not safe to stop ECG monitoring, the nurses were instructed to contact the ordering provider for a new order. During this time, they saw no significant change in the hospital census, the number of rapid responses called, the number of code blues called, or the number of mortalities (Dressler et al., 2014). A 2016 study by Cantillon and colleagues looked at the effect of standardized ECG monitoring inclusion criteria being established and an off-site centralized monitoring unit, or CMU. This CMU allows the ECG to be monitored remotely. In their study, the institution of standardized criteria for ECG monitoring reduced the ECG monitoring census by over 15%. They saw no significant change in cardiopulmonary arrests after instituting the changes. During the study period, the researchers found that 979 patients with rhythm or rate changes within one hour of emergency response team activation, with 772 (79%) of these changes being CMU detected, and 105 were provided discretionary direct emergency response team notification. The CMU also provided advanced warning of 27 cardiopulmonary arrests, leading to 25 cases of successful cardiopulmonary resuscitation (Cantillon et al., 2016). To better identify an abnormal rate or rhythm, it is essential to understand regular cardiac rate and rhythm. As previously mentioned, a standard rate generated from the SA node is 60-100 bpm. Rhythm is more complicated to define but not impossible. Because the electrical impulses in the heart trigger contraction, the heart's rhythm can easily and non-invasively be determined and monitored electronically via electrodes placed on the skin's surface in specific locations. These electrodes can sense and record the electrical currents from the heart and use that information to graph a heart rhythm. The classic example of this is the 12-lead ECG, which uses 12 different views to electronically create a complete picture of the heart. An ECG is done using six limb leads and six precordial (V) leads. The limb leads provide information about the heart’s frontal (vertical) plane using three bipolar leads (I-III) and three unipolar (aVR, aVL, and aVF). All six limb leads produce a positive deflection on ECG apart from aVR, which creates a negative deflection (the waves are upside down). The precordial leads (V1-6) are unipolar and provide information about the heart’s horizontal plane. A modified chest lead (MCL1-6) may be used instead of a precordial lead. They give the same information about the horizontal plane of the heart but are bipolar. A rhythm strip is a detailed picture of the electrical information gathered simultaneously from one or more lead(s). The transmission of the electrical activity to a computer that generates the pictograph can be hardwired, as is commonly seen in EDs and ICUs, or transmitted from a small portable box to a main computer for monitoring, as is seen in telemetry (Hinkle & Cheever, 2018; Shank Coviello, 2020). The 12 leads of an ECG are characterized as follows (Hinkle & Cheever, 2018; Shank Coviello, 2020): - Lead I: a bipolar limb lead with a current that runs from right to left, with a negative electrode on the right arm (RA) and a positive electrode on the left arm (LA) or chest. It helps detect atrial arrhythmias and hemiblocks. - Lead II: a bipolar limb lead with a current that runs from the negative electrode on the RA or below the right clavicle to a positive electrode down on the left lower extremity (LLE) or below the lowest palpable rib on the left midclavicular line. It produces a high-voltage deflection on ECG with tall P, R, and T waves. It is helpful for routine monitoring or to detect sinus or atrial arrhythmias. - Lead III: the final bipolar limb lead with a current that runs down from the negative electrode on the LA to the positive electrode on the LLE. It is beneficial in detecting inferior wall MIs. - aVR: an augmented unipolar limb lead with a negative deflection (the only limb lead with negative deflection). The positive electrode should be placed on the RA. - aVL: an augmented unipolar limb lead with a positive deflection, the positive electrode on the LA, and useful in detecting lateral wall infarctions. - aVF: an augmented unipolar limb lead with a positive deflection, the positive electrode on the LLE (or “foot”), and useful in detecting inferior wall infarctions. - V1: the first precordial (or chest) lead, unipolar, biphasic, with the electrode placed to the right of the sternum at the 4th intercostal space. It provides views of the P wave, QRS complex, and ST-segment and detects ventricular arrhythmias, ST-segment changes, a bundle branch block (BBB), and ectopic beats. - V2: the second precordial (or chest) lead, unipolar, biphasic, with the electrode placed to the left of the sternum at the 4th intercostal space. It identifies ST-segment elevation. - V3: the third precordial (or chest) lead, unipolar, biphasic, with the electrode placed on the left half of the chest, between the sternum and the midclavicular line at the 4th/5th intercostal space. It detects ST-segment elevation. - V4: the fourth precordial (or chest) lead, unipolar, biphasic, with the electrode placed on the left half of the chest, at the midclavicular line, and the 5th intercostal space. It is useful in detecting ST-segment and/or T wave changes. - V5: the fifth precordial (or chest) lead, unipolar, positive deflection, with the electrode placed on the left half of the chest, at the anterior axillary line, and the 5th intercostal space. It is useful in detecting ST-segment and/or T wave changes. - V6: the sixth precordial (or chest) lead, unipolar, positive deflection, with the electrode placed on the left half of the chest, at the midaxillary line, and the 5th intercostal space. As previously mentioned, the MCLs may be used in conjunction with the limb leads instead of the precordial leads. The MCL1 is like V, but the negative electrode is placed on the left upper chest, the positive electrode is placed just to the right of the sternum at the 4th intercostal space, and a ground electrode is placed on the right upper chest just below the clavicle. This placement produces a negative deflection waveform and helps detect premature ventricular contractions (PVCs), ventricular tachycardia, SVT, bundle branch defects, P wave changes, or confirm pacemaker wire placement. MCL6 is another alternative to MCL1 to monitor ventricular conduction. The negative lead is placed below the left shoulder. The positive electrode is placed on the left half of the chest at the midaxillary line and the 5th intercostal space (same location as V6). A ground electrode is placed just below the right shoulder. As previously mentioned, in most cases, only one lead will be monitored at a time, and proper selection of the most appropriate lead for a particular patient is key to successful cardiac monitoring. The AHA recommends the V1 lead when attempting to distinguish between ventricular tachycardia and aberrancy in adult patients. The AHA recommends that lead II be used in pediatric patients as supraventricular arrhythmias are more common in children than ventricular arrhythmias, and P waves are best visible in inferior leads (Hinkle & Cheever, 2018; Sandau et al., 2017; Shank Coviello, 2020). Before applying electrodes, the skin should be prepped. It should be washed, dried, and hair trimmed, and if needed, the skin may be rubbed briskly with a dry, clean washcloth to remove dead skin cells and improve electrode contact. If the electrode is a snap design, the lead wire should be attached to the electrode before applying the electrode to the patient. The electrode can be applied to the patient first, and the lead wire attached second if a clip design. Ten electrodes are placed in a traditional 12-lead ECG: on the RA, RLE, LA, LLE, and V1-6 positions. While this is a complete picture of the heart, this system is not conducive to ambulatory patients that may need monitoring for hours or even days (see Figure 4 below). The Mason-Likar system is a variation in which the four limb leads are moved to the torso to reduce motion artifact (see Figure 5). The RA/LA electrodes are placed medial to the deltoid muscle in the infraclavicular fossa about 2 cm below the clavicle, while the RLE/LLE electrodes are placed along the anterior axillary line halfway between the ribs and the iliac crest. The AHA guidelines recommend against leads on the limbs and advocate placing all leads on the patient’s torso to allow increased mobility and reduced artifact (Hinkle & Cheever, 2018; Sandau et al., 2017; Shank Coviello, 2020). There are three streamlined options for placing lead wires on ambulatory patients, depending on the ECG monitoring system used in an individual facility. A three-lead wire system (see Figure 6 below), which is the simplest system, includes a positive, a negative, and either a left lower (LL)or a ground electrode that can all be placed and adjusted depending on the desired lead to be monitored (Jackson Health System, 2018; Shank Coviello, 2020). A third alternative is the five-lead wire EASI system (see Figure 8), which uses just five lead wires to project a complete three-dimensional view of the heart’s conduction, just as a 12-lead ECG would do (Shank Coviello, 2020). This reduced lead continuous 12-lead ECG has: - the E lead in the center of the sternum at the 5th intercostal space - the A lead on the left midaxillary line at the 5th intercostal space - the S lead in the center of the upper sternum at about the first intercostal space - the I lead at the right midaxillary line at the 5th intercostal space - a ground lead placed anywhere on the torso (Jackson Health System, 2018; Shank Coviello, 2020). Once a readout has been produced, it should be printed and labeled with the patient’s name and ID number, date, and time if not already included. The HCP’s interpretation of the patient’s cardiac rhythm may also be noted on the printout and any current medications, signs/symptoms, or activities affecting the rhythm. For many healthcare institutions, this documentation will be electronically in the EHR. The measurements are standardized when reading an ECG printout or telemetry strip (see Figure 6 below). The horizontal axis represents time in seconds (s), while the vertical axis represents amplitude in millimeters (mm) or voltage in millivolts (mV). A small box or square represents 0.04s and 1 mm or 0.1 mV. Five small boxes make up one large box, representing 0.2s and 5 mm or 0.5 mV. Based on these numbers, 15 large boxes make up a 3-second strip, and 1500 small squares make up one full minute (see Figure 9; Jackson Health System, 2018; Shank Coviellom, 2020). A normal heart rhythm (see figure 10) has a P wave, a QRS complex, a T wave, and occasionally a U wave. Other measurements that should be considered include the PR interval, the ST segment, and the QT interval. Understanding the typical appearance of all these items helps HCPs identify abnormal characteristics. The P wave is the first component and represents atrial depolarization. The normal amplitude is 2-3 mm, and the expected duration is 0.06-0.12s. The P wave should be smooth, rounded, and upright in leads I, II, aVF, and V2-6, negative in lead aVR, and variable in leads III, aVL, and V1. If the P wave meets all these characteristics and precedes each QRS complex, the impulse originated in the SA node as is expected (Jackson Health System, 2018; Shank Coviello, 2020). Some variables might include a P wave that is: - peaked, notched, or enlarged, which may indicate atrial hypertrophy secondary to COPD, pulmonary embolism (PE), valvular disease, or CHF - inverted, which may indicate retrograde or reverse conduction up from the AV node - variable, which may indicate impulses that are coming from various sites within the heart, such as with wandering pacemaker, irritable atrial tissue, or damage near the SA node - absent, indicating conduction by a route other than the SA node, such as a junctional rhythm, AFib, or ventricular arrhythmia (Jackson Health System, 2018; Shank Coviello, 2020) The PR interval is measured from the beginning of the P wave to the beginning of the QRS complex. It represents the time for the conduction impulse to travel from the atria to the AV node and through the bundle of His and right and left bundle branches. The expected duration for the PR interval is 0.12-0.2s (twice the QRS complex). A lengthened PR interval may indicate a conduction delay such as an AV block or digoxin (Lanoxin) toxicity. On the other hand, a PR interval that is too short may indicate the impulse did not originate in the SA node, such as with a junctional arrhythmia or pre-excitation syndrome (Jackson Health System, 2018; Shank Coviello, 2020). The QRS complex represents the depolarization of the ventricles. The average amplitude is 5-30 mm, and the expected duration is 0.06-0.1s (half of the PR interval) measured from the beginning of the Q wave to the end of the S wave. The Q and S waves will have a negative deflection, while the R wave will have a positive deflection in leads I-III, aVL, aVF, and V4-6. The deflections will be reversed in leads aVR and V1-3 (Jackson Health System, 2018; Shank Coviello, 2020). Some possible variations in the QRS complex include: - a deep Q wave (> 25% amplitude of the R wave) or a wide Q wave (>0.04s), which may indicate a possible MI - a notched R wave, which may indicate a bundle branch block (BBB) - a widened QRS complex (> 0.12s), which may mean a ventricular conduction delay - an absent QRS complex, which may indicate an AV block or ventricular standstill (Jackson Health System, 2018; Shank Coviello, 2020) The ST segment represents the end of ventricular conduction and depolarization to the beginning of recovery and repolarization. It is measured from the end of the S wave (called the J point) to the beginning of the T wave. It should be isoelectric (at the established baseline, not above or below) with a positive amplitude of no more than 1mm and a negative amplitude no less than -0.5mm below the baseline. An elevated ST segment (amplitude > 1mm) may indicate myocardial injury. In contrast, an ST segment that is depressed (< -0.5mm below baseline) may predict myocardial ischemia or digoxin (Lanoxin) toxicity. The AHA guidelines suggest that hospitals develop clear interdisciplinary protocols regarding which patients would benefit from ST-segment monitoring, as changes in the ST segment or the adjoining T wave are often the first indicators of myocardial ischemia. Ideally, the AHA recommends computer software that allows for the simultaneous monitoring of the ST segment on all 12 leads in these at-risk patients. They also comment that ST-segment changes may also be caused by hyperkalemia, hypothermia, electrolyte abnormalities, pericarditis, BBB, or an expected reaction post-defibrillation. See above for specific indications for ST-segment monitoring recommendations (Jackson Health System, 2018; Sandau et al., 2017; Shank Coviello, 2020). - The T wave represents ventricular recovery and repolarization. It should appear round and smooth with an amplitude of 0.5 mm in leads I-III. Its deflection should be upright in leads I, II, and V3-6, negative in lead aVR, and variable in leads III, aVF, aVL, and V1-2. If the T wave is: - taller, peaked, or tented may indicate myocardial injury or hyperkalemia - inverted T wave in leads I, II, or V3-6 may indicate myocardial ischemia - notched or pointed may indicate pericarditis, which is inflammation of the pericardium, the double layer of connective tissue that covers the heart (Jackson Health System, 2018; Shank Coviello, 2020) The QT interval is measured from the beginning of the Q wave to the end of the T wave and represents ventricular depolarization and repolarization. The typical duration is 0.36-0.44s but can vary with age, sex, and HR. It should be less than half the time between consecutive R waves in any patient. If a patient’s QT interval is shortened, this may indicate digoxin (Lanoxin) toxicity or hypercalcemia. On the other hand, a prolonged QT interval puts the patient at increased risk for a serious arrhythmia called Torsades de Pointes (TdP; Jackson Health System, 2018; Shank Coviello, 2020). TdP may be caused by medications that prolong the QT interval, including: - antiarrhythmics (e.g., amiodarone [Cordarone, Pacerone], disopyramide [Norpace], dofetilide [Tikosyn], ibutilide [Corvert], procainamide [Procan], quinidine [Quinora], sotalol [Betapace]) - antidepressants (e.g., amitriptyline [Elavil], desipramine [Norpramin], fluoxetine [Prozac], sertraline [Zoloft]) - antipsychotics (e.g., chlorpromazine [Thorazine], haloperidol [Haldol], thioridazine [Mellaril]) - antibiotics (e.g., clarithromycin [Biaxin], erythromycin [Erythrocin], levofloxacin [Levaquin]) - antiemetics (e.g., dolasetron [Anzemet], droperidol [Inapsine]) - antifungals (e.g., ketoconazole [Nizoral]) - antimigraine medications (e.g., sumatriptan [Imitrex]) - opioid agonists (e.g., methadone [Methadose, Dolophine]; Jackson Health System, 2018; Shank Coviello, 2020) In addition to the above list, the AHA also cautions about the risk of QT prolongation with azithromycin (Zithromax), ciprofloxacin (Cipro), moxifloxacin (Avelox), citalopram (Celexa), escitalopram (Lexapro), ondansetron (Zofran), and fluconazole (Diflucan). The AHA also recommends monitoring and recording the corrected QT (QTc) by correcting the QT interval for HR. Regular ECG monitoring is recommended in patients who have recently started antiarrhythmics such as dofetilide (Tikosyn), ibutilide (Corvert), sotalol (Betapace), disopyramide (Norpace), procainamide (Procan), quinidine (Quinora), amiodarone (Cordarone), dronedarone (Multaq), and flecainide (Tambocor); patients with a history of prolonged QTc or risk factors for TdP; patients undergoing targeted temperature management (therapeutic hypothermia); patients with inherited prolonged QT with unstable ventricular arrhythmias; patients with medically or metabolically induced prolonged QTc; patients with drug overdose or toxicity; and patients with moderate to severe hypokalemia or hypomagnesemia (Sandau et al., 2017). The U wave is not consistently seen, but when present, it represents the recovery and repolarization of the Purkinje fibers. It should be upright and rounded. If it is prominent, it may indicate hypercalcemia, hypokalemia, or digoxin (Lanoxin) toxicity (Jackson Health System, 2018; Shank Coviello, 2020). Interpreting a telemetry strip will become faster and easier with repetition, but initially, following a stepwise approach can be helpful. This stepwise process is one such approach (Jackson Health System, 2018; Shank Coviello, 2020): - Determine the rhythm: measure the distance between consecutive P and R waves using paper and pencil or a caliper. A variation of less than 0.04s is OK. Are they regular (consistently spaced)? If not regular, is there a pattern? - Determine the rate: this can be done in several ways. First, count the number of P (atrial rate) and then R (ventricular rate) waves in a 6-second strip and multiply by ten. Another option is to count the number of small boxes/squares between consecutive P waves and R waves and divide by 1500 (the number of small squares in one minute). The sequence method estimates HR based on the thicker black lines outlining the big squares/boxes. Locate a P wave that lands on a thicker black line. Locate the next P wave and determine how many big boxes are between them: - One big box: HR of 300 - Two big boxes: HR of 150 - Three big boxes: HR of 100 - Four big boxes: HR of 75 - Five big boxes: HR of 60 - Six big boxes: HR of 50 - Repeat this process with the R waves to determine the ventricular rate. - Evaluate the P waves: are they present with every QRS complex? Are they similar in size and shape? Are they of standard configuration? - Measure the PR interval duration by counting the number of small squares and multiplying by 0.04s. Measure from the beginning of the P wave to the beginning of the QRS complex. It should be consistent, measuring 0.12-0.2s and about twice the QRS complex. - Measure the QRS complex duration from the beginning of the Q wave to the end of the S wave by counting the number of small squares and multiplying by 0.04s. The expected duration is 0.06-0.1s, or about half the PR interval. Do they occur with every P wave? Are they consistent in size and shape? - Evaluate the T waves to see if they are present with every QRS complex and deflect in the same direction on each lead? The deflection should be upright in leads I, II, and V3-6, negative in lead aVR, and variable in leads III, aVF, aVL, and V1-2. Are they regular and consistent in amplitude and shape? - Measure the QT interval from the beginning of the Q wave to the end of the T wave by counting the number of small squares and multiplying by 0.04s. An average QT interval will be 0.36-0.44s or less than half the time between consecutive R waves. - Evaluate for any other characteristics, such as ectopic (extra) beats, ST segment abnormalities (elevation or depression), or the presence of U waves. Normal Sinus and Not-So-Normal Sinus Rhythms Normal sinus rhythm (see Figure 11) has a consistent regular rhythm, a rate of 60-100 bpm, a P wave preceding every QRS complex (1:1 ratio), typical PR and QT intervals, and standard/upright T waves. In addition, all P waves and QRS complexes are similar in size and shape (Jackson Health System, 2018; Shank Coviello, 2020). Sinus arrhythmia is a normal variant (see Figure 12). The HR is still 60-100, but the rhythm is irregular and cyclic, varying with the patient’s respirations. This variation is related to the reduction in vagal tone and an increase in HR during inspiration and an increase in vagal tone and decrease in HR during exhalation. Sinus arrhythmia should not be present when the patient is holding their breath. Although sinus arrhythmia is a normal variation, especially in younger people, it can be mistaken for other arrhythmias. Sinus arrhythmia has been associated with obesity, DM, and HTN. Patients with sinus arrhythmia should be asymptomatic. The difference between P-P and R-R intervals is usually > 0.12s, a P wave precedes every QRS complex, there are typical PR and QT intervals, and standard/upright T waves. In addition, all P waves and QRS complexes are similar in size and shape (Jackson Health System, 2018; Prutkin, 2021; Shank Coviello, 2020). Sinus bradycardia (see Figure 13) has a regular rhythm but a rate that is less than 60 bpm. The QT interval may also be slightly prolonged, but a P wave precedes every QRS complex, and there is a normal PR interval and standard/upright T waves. In addition, all P waves and QRS complexes are similar in size and shape. Sinus bradycardia occurs typically because of automaticity in the heart’s SA nodes decreasing due to excess vagal stimulation (such as Valsalva maneuver, carotid sinus massage, or vomiting) or decreased sympathetic stimulation (such as sleep or deep relaxation). It may also be caused by hyperkalemia, increased intracranial pressure, hypothyroidism, hypothermia, glaucoma, SA node disease, cardiomyopathy, myocarditis, or myocardial ischemia (especially after an inferior wall MI that involves the right coronary artery, which feeds the SA node). In addition, sinus bradycardia can be caused by certain medications (beta-blockers, opioids, sedatives, chemotherapeutic agents, digoxin (Lanoxin), calcium channel blockers, lithium (Lithobid), cimetidine (Tagamet), sotalol (Betapace), amiodarone (Cordarone), propafenone (Rhythmol), and quinidine (Quinora). No treatment is required for individuals with asymptomatic sinus bradycardia, such as elite athletes. Instead, carefully monitor the patient’s vital signs, airway, breathing, and HR. Sinus bradycardia may produce dizziness, hypotension, decreased level of consciousness (LOC), confusion, cool/clammy skin, blurred vision, chest pain, or bradycardia-induced syncope (Stokes-Adams attack). Sinus bradycardia in a child is an ominous sign and should be taken seriously and monitored very closely. An underlying cause should first be identified and corrected if treatment is required. In the interim, or if an underlying cause cannot be immediately identified, transcutaneous pacing can be used until a more definitive plan can be established. Alternatively, medications such as atropine (Atreza), epinephrine (Adrenalin), or dopamine (Intropin) can be given to increase the HR. A 3 to 10 mg bolus of IV glucagon should be given over 3 to 5 minutes if a beta-adrenergic or a calcium channel blocker overdose is suspected. This bolus may be repeated once if there is no response. If the glucagon bolus is effective, a continuous infusion should be started at 3 mg to 5mg per hour and titrated as needed (Homoud, 2022; Jackson Health System, 2018; Shank Coviello, 2020). Sinus tachycardia (see Figure 14) is a regular rhythm with a rate of 100-160 bpm. A P wave will precede every QRS complex, a normal PR interval, a shortened QT interval, and standard/upright T waves. In addition, all P waves and QRS complexes are similar in size and shape, but P waves may have a higher amplitude and become superimposed on the preceding T wave. Sinus tachycardia may be caused by exercise, increased stress, hypovolemia, pain, hemorrhage, CHF, cardiogenic shock, pericarditis, PE, sepsis, or hyperthyroidism. It can also be prompted by excessive alcohol, caffeine, cocaine, or nicotine intake. In addition, sinus tachycardia can be prompted by certain medications, including amphetamines, atropine (Atreza), dopamine (Intropin), dobutamine (Dobutrex), epinephrine (Adrenalin), isoproterenol (Isuprel), or aminophylline (Phyllocontin). Abrupt withdrawal of beta-blockers can also cause sinus tachycardia. Sudden onset of sinus tachycardia following an MI may indicate extension of the infarction. The increased HR causes increased myocardial demands and decreased cardiac output due to reduced ventricular filling time, leading to angina, palpitations, decreased peripheral perfusion, hypotension, syncope, nervousness/anxiety, and blurred vision. HCPs should carefully monitor the patient’s vital signs, airway, breathing, HR, and LOC. The environment should be kept as calm as possible. If untreated, tachycardia can lead to heart failure (as evidenced by respiratory crackles, S3 heart sounds, and jugular venous distention) or cardiogenic shock. An underlying cause should first be identified and corrected if treatment is required. In the interim, or if an underlying cause cannot be identified immediately, medications such as beta-blockers (metoprolol [Lopressor] or atenolol [Tenormin]) and/or calcium channel blockers (verapamil [Calan]) may be given to reduce the heart rate. If beta-blockers are used, monitor for first-degree heart block development or reports of digestive symptoms, trouble sleeping, or erectile dysfunction. If calcium channel blockers are selected, monitor for signs of AFib, lower extremity swelling, hypotension, and reports of digestive symptoms (Homoud, 2021; Jackson Health System, 2018; Shank Coviello, 2020). Sinus arrest (see Figure 15) or atrial standstill is secondary to a lack of electrical activity from the SA node in the atrium. The patient will have a previously regular rhythm, followed by one or more missing beats. If just one or two beats are missed, it is considered a sinus pause, while three or more skipped beats are regarded as a sinus arrest. The length of the pause is not a multiple of the previous R-R intervals and commonly ends with a junctional escape beat. When present, there will be a P wave preceding every QRS complex, typical PR and QT intervals, and standard/upright T waves. In addition, all existing P waves and QRS complexes are similar in size and shape. It closely resembles third-degree SA block and can be caused by SA node disease (fibrosis or idiopathic degeneration), increased vagal tone (such as Valsalva maneuver, carotid sinus massage, or vomiting), acute inferior wall MI, acute infection, chronic coronary artery disease (CAD), acute myocarditis, cardiomyopathy, hypertensive heart disease or sick sinus syndrome (SSS). In addition, various medications can cause sinus arrest, including digoxin (Lanoxin), quinidine (Quinora), procainamide (Procan), salicylates, or excessive dosages of beta-blockers such as metoprolol (Lopressor) or propranolol (Inderal). If asymptomatic, no treatment may be necessary. Normal adults may have 2-3 second pauses during sleep, increased vagal tone, or hypersensitive carotid sinus disease. However, a prolonged pause or arrest (usually 7 seconds or more) can cause syncope, leading to falls, injuries, car accidents, or other secondary injuries. Any pause over 2-3 seconds should be noted and considered significant. The patient may also have hypotension, altered mental status (AMS), dizziness, blurred vision, and cool/clammy skin. HCPs should carefully monitor the patient’s vital signs, airway, breathing, and HR. An underlying cause should first be identified and corrected if treatment is required. In the interim, or if an underlying cause cannot be identified immediately, a transcutaneous pacemaker may be used as well as medications such as atropine (Atreza) or epinephrine (Adrenalin) to prevent circulatory collapse until more definitive and long-term treatment can be established (Jackson Health System, 2018; Shank Coviello, 2020). Sick Sinus Syndrome Sick sinus syndrome (SSS) refers to various SA node abnormalities (see Figure 16). The syndrome can refer to both disturbances in the way impulses are generated or conducted. SSS is most common over the age of 60. The rhythm is irregular, and the rate may vary. P waves usually precede each QRS. The QRS complex, T wave, PR interval, and QT interval are generally normal but vary with rhythm changes. SSS typically presents with an insidious and progressive onset of bradycardia and episodes of sinus arrest or SA block with varying periods of rapid AFib. There can also be periods of atrial tachyarrhythmias such as atrial flutter or atrial tachycardia dispersed amongst periods of bradycardia, a condition known as bradycardia-tachycardia (or brady-tachy) syndrome. SSS may also present as an inappropriate response by the SA node to increase the HR during exercise. In addition, SSS can be caused by fibrosis of the SA node (advanced age, atherosclerotic heart disease, HTN, or cardiomyopathy), trauma to the SA node due to surgery, pericarditis, rheumatic heart disease, or autonomic disturbances. Various medications can also provoke SSS, including digoxin (Lanoxin), beta-blockers, or calcium channel blockers. It can develop after an inferior wall MI that involves the right coronary artery, which feeds the SA node. Patients may experience symptoms of cardiomyopathy (such as crackles, an s3 sound, and a dilated/displaced left ventricular apical pulse), AMS, hypotension, blurry vision, or syncope. If asymptomatic, no treatment may be necessary. If symptomatic, carefully monitor the patient’s mental status, vital signs, airway, breathing, and HR. An underlying cause should first be identified and corrected if treatment is required. In the interim, or if an underlying cause cannot be identified immediately, atropine (Atreza) or epinephrine (Adrenalin) may be administered for symptomatic bradycardia, or metoprolol (Lopressor) or digoxin (Lanoxin) may be given for tachyarrhythmias (although these may worsen underlying SA node disease. If digoxin (Lanoxin) is given, look for reports of nausea and the development of other arrhythmias such as heart block. Transcutaneous pacing may also be recommended as a short-term solution. Anticoagulants should be given to reduce the risk of blood clots and stroke if the patient develops AFib. If anticoagulants are prescribed, the HCP should educate the patient and family regarding the risks of bleeding and monitor for signs/symptoms of this in the future (Jackson Health System, 2018; Shank Coviello, 2020). Atrial or supraventricular arrhythmias affect the atrium. The AHA lists common symptoms of atrial arrhythmias as angina, shortness of breath, dizziness, lightheadedness, syncope, or heart palpitations. Premature atrial contractions (PACs) are ectopic or premature beats that do not originate in the SA node but instead from an irritable or ectopic spot elsewhere in the atria (see Figure 17). Unfortunately, this erroneous signal may be conducted through the AV node and the rest of the heart just as any other impulse. They can be caused by excessive alcohol or nicotine use, anxiety, extreme fear or fatigue, infection, coronary or valvular heart disease, acute renal failure, hypoxia, pulmonary disease, digoxin (Lanoxin) toxicity, or electrolyte imbalances. They are rarely dangerous in patients without heart disease and are typically asymptomatic. The ECG strip will show a premature P wave with an abnormal configuration compared to the others and an irregular rate. If conducted, a standard QRS complex will follow. If not conducted, no QRS complex will appear. The SA node is typically able to reset itself, causing a normal sinus beat to follow but appearing slightly earlier. If PACs occur every other beat, this is called bigeminy, and every third beat trigeminy. Two PACs in a row are called a couplet. If symptomatic, the patient may report palpitations, fluttering, or the feeling of skipped beats. If symptomatic, HCPs should monitor the patient’s vital signs and pulse. An underlying cause should first be identified and corrected (AHA, 2016c; Jackson Health System, 2018; Shank Coviello, 2020). Atrial tachycardia is also known as supraventricular tachycardia or SVT (see Figure 18). The AHA reports it is more common in children and more often found in women than men. This rhythm has an atrial rate of 150-250 bpm. This rapid rate may lead to shorter diastole, reduced atrial kick and cardiac output, decreased coronary perfusion, and myocardial ischemia. If untreated, this can eventually lead to angina, heart failure, or an MI. Atrial tachycardia can be due to a block (not every impulse is conducted through the AV node, so atrial and ventricular rates will differ), multifocal (multiple atrial foci firing impulses leads to various P wave configurations and an irregular rhythm), or paroxysmal (starts and stops suddenly, with a regular rhythm). In addition, atrial tachycardia can be caused by excessive caffeine or other stimulants, marijuana use, electrolyte imbalances, hypoxia, stress, MI, cardiomyopathy, congenital anomalies, WPW, valvular heart disease, SSS, cor pulmonale, hyperthyroidism, HTN, or digoxin (Lanoxin) toxicity (AHA, 2016c; Jackson Health System, 2018; Shank Coviello, 2020). In atrial tachycardias, the QRS complex and QT interval are usually normal but may be shorter with a faster rate. ST-segment changes and T wave inversion may occur if ischemia develops. Symptoms may include palpitations, blurry vision, syncope, and hypotension. If symptomatic, carefully monitor the patient’s mental status, airway/breathing, vital signs, and HR. An underlying cause should first be identified and corrected if treatment is required. Carotid sinus massage may be a treatment option for younger patients but should be avoided in elderly patients. Potential risks include hypotension, bradycardia, vasodilation, ventricular arrhythmias, stroke, and cardiac standstill. Valsalva maneuver is another option for vagal stimulation but carries many of the same risks. Medications such as digoxin (Lanoxin), beta-blockers, adenosine (Adenocard), or calcium channel blockers may be used to reduce HR. If adenosine (Adenocard) is used, watch for the development of chest pain, SOB, and flushing. If medications are unsuccessful or inappropriate, synchronized cardioversion or atrial overdrive pacing may also be used. Synchronized cardioversion works by precisely timing (at the peak of the R wave) a low-energy shock to the heart to restore a normal rhythm. The patient will be premedicated with a sedative, anxiolytic, and/or analgesic medication intravenously. Caution should be advised, as cardioversion can lead to new or worsening arrhythmias or a clot embolism (AHA, 2016c; Jackson Health System, 2018; Shank Coviello, 2020). Atrial flutter is an SVT with an atrial rate of 250-350 bpm (see Figure 19). The impulse is generated from a single atrial focus and results from circus reentry and increased automaticity. The ECG for this rhythm has a classic and unmistakable sawtooth pattern as the P waves lose their distinction and the waves blend. T waves are not usually discernible, and the QRS complexes may be widened if P waves are superimposed. Atrial flutter is often associated with a second-degree block, which does not allow all impulses through the AV node, resulting in a slower ventricular rate. If the ventricular rate is below 40 or above 150, cardiac output may be compromised, leading to reduced ventricular filling and coronary perfusion, angina, heart failure, pulmonary edema, hypotension, or syncope. Atrial flutter is often caused by conditions that raise the atrial pressure or cause atrial hypertrophy, such as severe mitral valve disease, hyperthyroidism, pericardial disease, or primary myocardial disease. It can also be seen in patients with recent cardiac surgery, acute MI, COPD, and systemic arterial hypoxia. If symptomatic, HCPs should carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. An underlying cause should first be identified and corrected if treatment is required. Then, treatment will focus on rate control and converting the arrhythmia to a normal rhythm. In a stable patient, direct current cardioversion (DCCV) may be considered if the atrial flutter has been present for less than 48 hours. If present for more than 48 hours, the risk of thromboembolism is greater, and cardioversion should not be considered unless the patient is adequately anticoagulated, or a transesophageal echocardiogram (TEE) has been completed to rule out the presence of a clot. If the patient is unstable, synchronized cardioversion should be considered. If pharmacologic treatment is necessary, ibutilide (Covert) is the preferred drug, reverting atrial flutter to a sinus rhythm in approximately 60% of patients. Other pharmacologic options that can be used include procainamide (Procan), sotalol (Betapace), or amiodarone (Cordarone; Jackson Health System, 2018; Phang & Prutkin, 2021; Shank Coviello, 2020). Atrial fibrillation, commonly referred to as AFib, is the most common arrhythmia (about 2 million people are affected in the US). It is defined as chaotic, random electrical activity in atrial tissue with an impulse rate of 400-600 per minute, resulting in atrial kick loss and quivering instead of coordinated contractions in the atria. The ECG baseline will contain erratic (fibrillatory) waves that may be coarse or fine and no discernible P waves (see Figure 20). AFib may be preceded by or caused by PACs. An irregularly irregular transfer of impulses through the AV node to the ventricles leads to an irregular ventricular rate and corresponding irregular pulse. AFib can be caused by cardiac surgery, hypotension, PE, COPD, electrolyte imbalances, mitral insufficiency or stenosis, hyperthyroidism, infection, coronary artery disease (CAD), acute MI, pericarditis, hypoxia, or atrial septal defects. It can also be triggered by excessive caffeine, alcohol, or nicotine combined with fatigue and stress. Medications such as aminophylline (Phyllocontin) or digoxin (Lanoxin) may also trigger AFib (AHA, 2016c; Jackson Health System, 2018; Shank Coviello, 2020). If left untreated, AFib can cause heart failure, angina, syncope, cardiovascular collapse, thrombus, and/or embolism formation. If the ventricular rate is greater than 100, it is termed uncontrolled AFib. The peripheral pulses may differ from the apical rate on the physical exam due to weaker contractions that do not produce a palpable pulse. Acute symptoms may include lightheadedness or hypotension due to reduced cardiac output. HCPs should assess for embolism symptoms such as PE or stroke in patients with chronic AFib. If symptomatic, HCPs should carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. Any underlying cause should first be identified and corrected if possible. Like atrial flutter, treatment is targeted at controlling the rate (below 100) with medications if needed and cardioverting to a normal rhythm if possible. Just as above, synchronized cardioversion is most successful if done within the first 48 hours of AFib development. Patients should be adequately anticoagulated. Vagal stimulation with carotid sinus massage or Valsalva maneuvers may temporarily slow the ventricular rate. Medications such as digoxin (Lanoxin), propranolol (Inderal), quinidine (Quinora), amiodarone (Cordarone), diltiazem (Cardizem), and verapamil (Calan) are often given to maintain normal sinus rhythm and control the ventricular rate after cardioversion. Radiofrequency ablation is sometimes required in patients with refractory ectopic sites in the atria. Ablation procedures use either a radiofrequency (high-energy) catheter, cryoablation (cold), or a laser to create a focal area of scar tissue to prevent the extraneous electrical activity from a specific ectopic site. The patient is premedicated with an anxiolytic, and the procedure carries many of the same risks as a cardiac catheterization (AHA, 2016c; Jackson Health System, 2018; Shank Coviello, 2020). A wandering pacemaker is an irregular rhythm when the heart's pacemaker changes from the SA node to another area above the ventricles or the AV junction. It is commonly transient and rarely serious. A wandering pacemaker is a rhythm with a standard rate of 60-100 bpm but varying P waves and PR intervals (see Figure 21). The QRS complex, T wave, and QT interval are usually standard, but the ventricular rate may be slightly irregular. It may be caused by increased vagal tone, digoxin (Lanoxin) toxicity, or heart disease such as rheumatic carditis. A wandering pacemaker is often, but not always, asymptomatic and requires no treatment other than monitoring. The nurse should carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse if symptomatic. If treatment is required, an underlying cause should first be identified and corrected if possible (Jackson Health System, 2018; Shank Coviello, 2020). Five junctional arrhythmias originate in the AV junction: the area around the AV node and the bundle of His. They occur when the SA fails as the heart’s pacemaker or the impulse fails to conduct properly. This abnormal signal from the AV junction may cause upward or retrograde depolarization of the atria, which appears as inverted P waves in leads II, III, and aVF, where they are generally upright. Another key to spotting a junctional arrhythmia is the PR interval. If a rhythm has an inverted P wave but a normal PR interval (0.12-0.2s), it is likely an atrial arrhythmia. If a rhythm has an inverted P wave and a shortened PR interval (<0.12s), it likely originated in the AV junction (Jackson Health System, 2018; Shank Coviello, 2020). Wolff-Parkinson-White (WPW) syndrome is typically a congenital rhythm disorder seen in young children and young adults aged 20-35. In this condition, impulses bypass the AV node through the bundle of Kent, traveling directly from the atria to the ventricles quickly. WPW can lead to circus reentry, retrograde conduction, or reentrant tachycardia. On ECG, this appears as a shortened PR interval (<0.1s), and a widened QRS complex (>0.1s) with a slurred beginning called a delta wave due to abnormal ventricular depolarization (see Figure 22). In asymptomatic patients, monitoring may be the only treatment necessary. WPW can also lead to tachyarrhythmias such as AFib or atrial flutter, thus necessitating additional treatment such as radiofrequency ablation and/or rate-controlling medications like amiodarone (Cordarone; Jackson Health System, 2018; Shank Coviello, 2020). Premature Junctional Contraction A premature junctional contraction (PJC) is an irregular ectopic beat originating from the AV junction. A PJC appears on ECG with an inverted P wave due to the retrograde depolarization of the atria that may fall before, during, or after the QRS complex. The PR interval is shortened (<0.12s) if the P wave occurs before the QRS complex. The QRS complex, T wave, and QT interval are usually normal (see Figure 23). A PJC can be caused by digoxin (Lanoxin) toxicity, excessive caffeine intake, inferior wall MI, rheumatic heart disease, valvular disease, hypoxia, heart failure, or swelling of the AV junction following surgery. Patients experiencing PJCs are often asymptomatic and do not require treatment other than monitoring, or the patient may report palpitations or a quickening in their chest and an irregular pulse. If cardiac output is compromised, they may be hypotensive or report dizziness, lightheadedness, blurry vision, or syncope. If symptomatic, carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. If treatment is required, an underlying cause should first be identified and corrected if possible (Jackson Health System, 2018; Shank Coviello, 2020). Junctional Escape Rhythm Junctional escape rhythm is a rapid succession of beats after a conduction delay from the atria. It is a compensatory mechanism to prevent ventricular standstill. Junctional Escape Rhythm appears on ECG with a rate of 40-60 bpm and an inverted P wave due to the retrograde depolarization of the atria that may fall before, during, or after the QRS complex. The PR interval is shortened (<0.12s) if the P wave occurs before the QRS complex. The QRS complex, T wave, and QT interval are usually normal (see Figure 24). It can be caused by SSS, vagal stimulation, digoxin (Lanoxin) toxicity, inferior wall MI, or rheumatic heart disease. Junctional escape rhythm may be asymptomatic depending on age and cardiovascular fitness level, or individuals may show signs of reduced cardiac output such as hypotension, decreased urine output, or syncope. If symptomatic, carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. An underlying cause should first be identified and corrected if treatment is required. Atropine (Atreza) may be indicated to speed up the patient’s HR. A transcutaneous temporary pacemaker may be used until a permanent one can be implanted (Jackson Health System, 2018; Shank Coviello, 2020). Accelerated Junctional Rhythm An accelerated junctional rhythm is like the junctional escape rhythm above but faster. An accelerated junction rhythm is especially problematic if the atria depolarize after the ventricles (the P wave follows the QRS complex), preventing the atrial kick, which is blood being pumped into the ventricles from the atria. It appears on ECG with a rate of 60-100 bpm (or > 80 bpm in toddlers up to age 3) and an inverted P wave due to the retrograde depolarization of the atria that may fall before, during, or after the QRS complex. The PR interval is shortened (<0.12s) if the P wave occurs before the QRS complex. The QRS complex, T wave, and QT interval are usually normal (see Figure 25). An accelerated junctional rhythm can lead to decreased cardiac output with symptoms of hypotension, dizziness, confusion, syncope, reduced urine output, and weak peripheral pulses. If symptomatic, carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. An underlying cause should first be identified and corrected if treatment is required. Pacing may be indicated with a transcutaneous temporary pacemaker until a permanent one can be implanted (Jackson Health System, 2018; Shank Coviello, 2020). Junctional tachycardia occurs when there are three or more consecutive PJCs. It originates from an irritable focus in the AV junction with increased automaticity, overriding the SA node. The rate on ECG is 100-200 bpm. The rhythm strip indicates an inverted P wave due to the retrograde depolarization of the atria that may fall before, during, or after the QRS. The PR interval is shortened (<0.12s) if the P wave occurs before the QRS complex. The QRS complex, T wave, and QT interval are usually normal, but may not be visible if the rate increases (see Figure 26). The significance of this rhythm depends on the rate, the underlying cause, and any accompanying or preexisting heart disease. Junctional tachycardia is most often caused by digoxin (Lanoxin) toxicity. However, it can also be related to hypokalemia, inferior or posterior myocardial ischemia or MI, congenital heart disease, or swelling of the AV junction after surgery. A lack of atrial kick and a high ventricular rate can quickly combine forces to reduce cardiac output. Assess for symptoms of hypotension, dizziness, confusion, syncope, decreased urine output, and weak peripheral pulses. If symptomatic, carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse. An underlying cause should first be identified and corrected if treatment is required. Vagal maneuvers may temporarily reduce the HR, and medications such as verapamil (Calan) may be indicated to slow the HR down further. Pacemakers, either temporary or permanent or ablation therapy, are sometimes indicated in resistant or recurrent junctional tachycardia (Jackson Health System, 2018; Shank Coviello, 2020). Ventricular arrhythmias start in the ventricle below the bundle of His and can be very dangerous, requiring immediate medical attention. For that reason, they should be studied, understood, and quickly recognized. The AHA lists common signs and symptoms of ventricular arrhythmias as dizziness, palpitations, shortness of breath, nausea, altered LOC, or cardiac arrest. Ventricular arrhythmias are often caused by reduced coronary blood flow, cardiomyopathy, inflammation related to sarcoidosis, sepsis, problems with the aorta, or certain medications. The myocardium is depolarized along a different pathway, causing widened QRS complexes on ECG. In addition, characteristic signs of ventricular arrhythmias include an absent P wave due to lack of atrial depolarization and a QRS complex and T wave that deflect in opposite directions due to the difference in the action potential during ventricular depolarization and repolarization. Without the atrial contraction or kick, cardiac output can decrease by 30%, causing hypotension, angina, syncope, and/or respiratory distress (AHA, 2016a; Jackson Health System, 2018; Shank Coviello, 2020). Premature Ventricular Contraction A premature ventricular complex or contraction (PVC) is an ectopic beat caused by electrical irritability in the ventricular conduction system or muscle tissue. PVCs may be caused by electrolyte imbalances, metabolic acidosis, hypoxia, myocardial ischemia or infarction, illicit drugs (cocaine, amphetamines), tricyclic antidepressants, ventricular hypertrophy, increased sympathetic stimulation, myocarditis, excessive alcohol, caffeine intake, antiarrhythmics medication, or tobacco use. If frequent or sustained, PVCs can reduce cardiac output. They can also be a precursor to more severe arrhythmias. PVCs can occur in couplets, bigeminy, or trigeminy. On ECG, PVCs appear wide and abnormally shaped at irregular intervals. There is no P wave, or a retrograde P wave may interfere with the ST segment. The QRS complex is > 0.12s with a deflection opposite the T wave (see Figure 27). There may or may not be a compensatory pause following the PVC, which should equal two regular P-P intervals. Patients with PVCs that are couplets, bigeminy, or variable in shape may indicate a more serious condition and require immediate medical evaluation. Patients with frequent PVCs may describe palpitations or experience hypotension or syncope. If asymptomatic, treatment beyond monitoring may not be necessary. If symptomatic, the HCP should carefully monitor the patient’s mental status, airway/breathing, vital signs, and pulse and place them on continuous ECG monitoring. An underlying cause should first be identified and corrected if treatment is required. Antiarrhythmic medications such as procainamide (Procan), amiodarone (Cordarone), or IV lidocaine (xylocaine) may be indicated (Jackson Health System, 2018; Shank Coviello, 2020). Idioventricular rhythms are the heart's safety mechanism to avoid ventricular standstill if no impulse is received by the SA node or the AV node above the bundle of His. The cells of the His-Purkinje system will generate electrical impulses at 20-40 bpm. As a result, cardiac output is markedly reduced with a slow rate and no atrial kick. If just a single beat is seen, it is called a ventricular escape beat. If multiple beats occur consecutively at a regular 20-40 bpm rate, it is called idioventricular rhythm (see Figure 28). If the rate is above 40, it is called accelerated idioventricular rhythm. On ECG, the P wave will be absent, the QRS complex will widen (>0.12s), the QT interval will be prolonged, and the T wave will deflect opposite the QRS complex. Signs and symptoms include palpitations, dizziness, lightheadedness, hypotension, weak peripheral pulses, syncope, decreased urine output, or confusion. Idioventricular rhythms may be caused by myocardial ischemia or infarction, digoxin (Lanoxin) toxicity, beta-blockers, pacemaker failure, or metabolic imbalances. It may accompany a third-degree heart block. The underlying cause should be identified and corrected in symptomatic patients if possible. Monitor the patient’s mental status, airway/breathing, vital signs, and pulse carefully and place on continuous ECG monitoring. Treatment should focus on increasing cardiac output by increasing the HR and establishing a normal rhythm. Atropine may be given, or a transcutaneous pacemaker may need to be used until a permanent treatment plan can be established. Never give lidocaine or other antiarrhythmic medications to someone with idioventricular rhythm, as this may suppress their last safety mechanism (Jackson Health System, 2018; Shank Coviello, 2020). Ventricular tachycardia, or V-tach, is a consecutive series of three or more PVCs. The ventricular rate exceeds 100 bpm. It may be a precursor to ventricular fibrillation and sudden cardiac death and should be identified and treated without delay. Ventricular tachycardia may occur in short bursts without symptoms or be sustained. On ECG, the atrial rate cannot be determined, and the ventricular rhythm is usually regular with a rate of 100-250 bpm. The QRS complex is widened (>0.12s) and typically with an increased amplitude. The QRS complex may be uniform (monomorphic) or variable (polymorphic). If the T wave is present, it deflects opposite the QRS complex, and the QT interval is not measurable (see Figure 29). Ventricular tachycardia may result from myocardial ischemia or infarction, CAD, valvular heart disease, heart failure, cardiomyopathy, electrolyte imbalances, digoxin (Lanoxin) toxicity, procainamide (Procan) toxicity, quinidine (Quinora) toxicity, cocaine use, or the proarrhythmic effects of some antiarrhythmic medications. If patients are stable, they still need quick and close attention as they can decompensate quickly. Poor cardiac output may lead to decreased LOC, dizziness, lightheadedness, hypotension, weak peripheral pulses, syncope, reduced urine output, or confusion. Assess the patient’s vital signs, level of consciousness, mental status, airway/breathing, and pulse carefully and place on continuous ECG monitoring. If no pulse is present, defibrillation and CPR should be started immediately. Synchronized cardioversion may be appropriate if a pulse is present, but the patient is hemodynamically unstable. If hemodynamically stable and the rhythm is monomorphic, amiodarone (Cordarone) could be administered first. If medication is ineffective, then cardioversion is recommended. In a stable patient with polymorphic ventricular tachycardia, treatments with beta-blockers, lidocaine (Xylocaine), amiodarone (Cordarone), or procainamide (Procan) could be attempted before cardioversion therapy. An implantable cardioverter-defibrillator may be recommended for those with chronic ventricular tachycardia (Jackson Health System, 2018; Shank Coviello, 2020). Torsades de Pointes Torsades de pointes (TdP), which means twisting around the points, is a polymorphic ventricular tachycardia. It shares many of the same characteristics of ventricular tachycardia above, plus a rotation of the QRS complexes deflecting above and below the baseline. The rate is 150-250 bpm, the rhythm is irregular, and the QRS complexes are wide with varying amplitude (see Figure 30). TdP may be paroxysmal and appear or cease suddenly. In addition, TdP may deteriorate into ventricular fibrillation. It is often caused by something reversible, such as medications (see the list of medications that prolong the QT interval located above; Jackson Health System, 2018; Shank Coviello, 2020). The AHA also lists the following risk factors for QT interval prolongation and potential TdP: - older age - female sex - heart disease (e.g., left ventricular hypertrophy, low left ventricular ejection fraction, myocardial ischemia) - bradyarrhythmia (e.g., sinus pauses, compensatory pauses after PVCs, pauses after conversion from AFib or flutter to sinus rhythm, complete heart block, or Mobitz II) - electrolyte abnormalities (e.g., hypomagnesemia, hypokalemia, malnutritional electrolyte disorders) - genetic or acquired metabolic impairment (e.g., renal failure, hepatic failure) - genetic predisposition to QT interval prolongation (e.g., family history of syncope, sudden death; Sandau et al., 2017) Treatment for TdP includes correcting the underlying cause. In addition, mechanical overdrive pacing may be utilized to override the ventricular rate. Magnesium sulfate or electrical cardioversion are also options that should be considered if other treatment options do not work (Jackson Health System, 2018; Shank Coviello, 2020). Ventricular fibrillation, or V-fib, is a chaotic rhythm of electrical activity. The impulses arise from multiple different foci in the ventricles. Ventricular fibrillation produces significant contraction and no cardiac output. If untreated, sudden cardiac death will occur. Ventricular fibrillation may be caused by myocardial ischemia or infarction, untreated ventricular tachycardia, underlying heart disease, acid-base imbalance, electric shock, severe hypothermia, electrolyte imbalances, drug toxicity (including digoxin [Lanoxin]), or severe hypoxia. On ECG, V-fib appears as fibrillatory waves with no discernible P waves, QRS complexes, or T waves. The larger the waves, the higher the electrical activity in the heart, and the easier it is to convert it back to a usable and acceptable rhythm (see Figure 31). Patients are in full cardiac arrest, with no detectable pulse or blood pressure, and are unresponsive. Shivering or electric razors can interfere with ECG monitors and even mimic ventricular fibrillation, so assess the patient first. Treatment for ventricular fibrillation is defibrillation, but CPR can help improve survival until the defibrillator arrives and is set up. Defibrillation causes the myocardium to depolarize and allows the SA node to resume pacemaker duties. Intubation to facilitate gas exchange and medications such as epinephrine (Adrenalin) or vasopressin (Pitressin) are vital components to help the heart respond to defibrillation and recover afterward. Other medications that may be given during resuscitation include amiodarone (Cordarone), lidocaine (Xylocaine), procainamide (Procan), or magnesium sulfate (Jackson Health System, 2018; Shank Coviello, 2020). Asystole or ventricular standstill corresponds with no electrical activity or cardiac output; it should be confirmed in multiple ECG leads. Asystole is predominantly related to inadequate blood flow to the heart, such as MI, severe electrolyte disturbances, massive PE, prolonged hypoxemia, severe acid-base disturbances, electric shock, drug overdose, cardiac tamponade, or hypothermia. On ECG, asystole looks like an essentially flat line except for fluctuations from chest compressions during CPR (see Figure 32). In a patient with an implanted pacemaker, pacer spikes may still be present. Assess the patient rapidly yet carefully- they will be unresponsive and have no pulse or blood pressure if truly in asystole. Treatment includes CPR initially with repeated doses of epinephrine (Adrenalin). Then, transcutaneous pacing should be initiated, and steps should be taken to correct the underlying cause (Jackson Health System, 2018; Shank Coviello, 2020). Pulseless Electrical Activity Pulseless electrical activity (PEA) is like asystole in that the heart is not contracting, and there is no cardiac output. The primary difference is that the heart’s electrical impulses are preserved in PEA. Therefore, the ECG will show organized electrical activity in PEA, yet the patient will be unresponsive without a pulse or measurable blood pressure. PEA can be caused by hypovolemia, hypoxia, acidosis, tension pneumothorax, cardiac tamponade, massive PE, hypothermia, electrolyte imbalances, massive acute MI, or drug overdose. CPR in conjunction with epinephrine is the recommended treatment of choice initially, followed by correcting the underlying cause. PEA can lead to asystole if left untreated (Jackson Health System, 2018; Shank Coviello, 2020). Sinoatrial blocks, or SA blocks, occur when the SA node discharges regularly, but some or all those discharges are delayed or blocked in-transit through the atria. There are three degrees of SA block (Jackson Health System, 2018; Shank Coviello, 2020): - A first-degree SA block is a delay between the SA node firing and depolarization of the atria. This type of SA block cannot be seen on the ECG as there is no specific indicator for SA node activity within the electrocardiograph. Typically, first-degree SA block is asymptomatic, with only monitoring required. Underlying causes should be treated if possible. - Second-degree SA block is further broken down into types I and II: - Type I (see Figure 33) is characterized by a progressively longer conduction time with each beat (causing an irregular rhythm and a progressively longer P-P interval) until an entire PQRST cycle is missed or dropped. The resulting pause is less than 2x the shortest P-P interval. Type II (see Figure 34) is characterized by a standard conduction time and a regular rhythm until a single impulse is blocked. The resulting pause is a multiple of the previous P-P interval. - Some impulses from the SA node are entirely blocked in third-degree SA block, causing sinus pauses. The pause is not a multiple of the previous R-R intervals. Third-degree SA block appears similar on ECG to sinus arrest but is due to lack of impulse conduction, not lack of impulse formation (see Figure 13). The pause can be indefinite and typically ends with a sinus beat (in contrast to sinus arrest, which typically ends with a junctional escape beat; Jackson Health System, 2018; Shank Coviello, 2020). Atrioventricular block (AV block) involves blocking the electrical impulses from the SA node at the AV node. In AV blocks, atrial rates are regular, with a rate of 60-100. However, ventricular rates vary depending on the type and severity of the block. If slowed significantly, the reduced ventricular rate can reduce cardiac output and produce signs and symptoms of lightheadedness, hypotension, and confusion. AV blocks can be caused by an inferior wall or anteroseptal MI, digoxin (Lanoxin) toxicity, acute myocarditis, calcium channel blockers, beta-blockers, cardiac surgery or radiofrequency ablation procedure, congenital abnormalities, or cardiomyopathy. There are three degrees of AV block (Jackson Health System, 2018; Shank Coviello, 2020): - First-degree AV block is simply a consistent delay of the impulses passing through the AV node, but every impulse eventually passes through. It may be temporary or permanent. On ECG, first-degree AV block will present with a longer than average PR interval (>0.2s) and a regular rate (see Figure 35, first strip). Typically, it is asymptomatic, with only monitoring required. If possible, underlying causes should be treated. - Second-degree AV block is further broken down into type I and type II. - Type I or Wenckebach/Mobitz type I block is characterized by progressively longer delay with each heartbeat until a beat is finally missed, and the cycle then restarts. It may be caused by an MI, CAD, rheumatic fever, or certain medications. In addition, type I AV block can also be caused by increased vagal stimulation. Treatment focuses on resolving the underlying condition and thus the consequent block. It may progress to a more severe form if it occurs during an MI. The atrial rhythm is regular (P-P interval) on ECG, yet the ventricular rhythm is regularly irregular (R-R interval). The PR interval progressively lengthens on each beat until a P wave fails to conduct and a QRS complex is missing (see Figure 35, second strip). Patients are usually asymptomatic but may present with signs of reduced cardiac output such as hypotension and dizziness/lightheadedness if the ventricular rate is slow. In symptomatic patients, atropine (Atreza) and/or a temporary pacemaker are indicated to help AV conduction. Any underlying cause should be assessed and corrected. - Type II or Mobitz type II block is less common yet more dangerous than type I. In type II AV block, impulses pass through the AV node regularly until a single beat fails to conduct. It can be caused by an anterior wall MI, severe CAD, or degenerative changes. The ventricular rate tends to be slower, thus decreasing cardiac output and causing symptoms such as hypotension and dizziness. The ratio of conducted to dropped beats can be as low as 2:1 (see Figure 35, fourth strip). On ECG, the atrial rate will be regular, but the ventricular rate may not be. There will simply be missing QRS complexes. The QRS complexes may be wide, and the PR interval may be prolonged but consistent (see Figure 35, third strip). As dropped beats increase, patients may report fatigue, dyspnea, chest pain, or lightheadedness. HR may be slow and blood pressure low. If symptomatic, atropine (Atreza), dopamine (Intropin), or epinephrine (Adrenalin) may be given to increase HR and improve cardiac output and oxygen to treat hypoxia. Transcutaneous pacing may also be indicated until a permanent pacemaker can be safely placed. Third-degree AV block, or complete heart block, occurs when all impulses are permanently or temporarily blocked at the AV node. The atrial rate will continue at 60-100 bpm, with regular P waves on ECG. However, the ventricular rate will be 40-60 bpm (if originating from the AV node) or 20-40 bpm (if arising from the Purkinje system) with no connection or coordination between the two (see Figure 35, fifth strip). It is most commonly congenital but may be caused by CAD, an anterior or inferior wall MI, degenerative changes, digoxin (Lanoxin) toxicity, calcium channel blockers, beta-blockers, or a surgical injury. With a decreased ventricular rate and no atrial kick, cardiac output can drop dangerously, causing severe fatigue, dyspnea, chest pain, lightheadedness, altered mental status, loss of consciousness, hypotension, pallor, diaphoresis, bradycardia, and variable pulses. Atropine (Atreza), dopamine (Intropin), epinephrine (Adrenalin), and/or a temporary pacemaker may be utilized to increase the ventricular rate and improve cardiac output. The underlying cause may be resolved or a permanent pacemaker inserted. Bundle Branch Block Bundle branch block (BBB) is a potential complication of an MI. Either the left or the right bundle branch does not conduct impulses. If the block happens further down the left bundle branch, it is called a hemiblock. If the block occurs as the HR increases, it is called rate-related. Ventricular depolarization is prolonged because the signal travels down one bundle branch and then across to the opposite ventricle via myocardial cell to cell conduction. This leads to a widened QRS complex (>0.12s) on the strip. Leads V1 and V6 help determine which side of the heart is affected. A right BBB (see Figure 36) can be caused by an anterior wall MI, CAD, cardiomyopathy, cor pulmonale, or PE. The QRS complex may be double notched in lead V1, which is an rSR (small R-wave, followed by an S-wave, followed by a large R-wave) due to late right ventricular depolarization and a negative T wave. In lead V6, a widened S wave may be seen. A left BBB (see Figure 36) may be caused by hypertension, aortic stenosis, degenerative changes in the conduction system, or CAD. If left BBB occurs with an anterior wall MI, it may indicate complete heart block and require pacemaker placement. A wide S wave will be seen after the widened QRS complex in lead V1 with a positive T wave. In lead V6, a tall, notched R wave with an inverted T wave will be seen. Most patients are asymptomatic, although BBBs may cause syncope if symptomatic and require pacing or cardiac resynchronization therapy (CRT) to help the ventricles get back in sync (Jackson Health System, 2018; Shank Coviello, 2020). A pacemaker is a device that generates electrical stimuli to the heart muscle when a patient has a slower-than-normal impulse formation or a symptomatic AV or ventricular conduction disturbance. Common indications for pacemaker placement include sinus node dysfunction, acquired AV block, and post-myocardial infarction. Other less common indications may include long QT syndrome, congenital complete heart block, and hypertrophic cardiomyopathy. Biventricular (i.e., both ventricles) pacing may be used to treat advanced heart failure. Most pacemakers are permanent; however, temporary pacemakers can be used in the hospital setting after an acute MI or open-heart surgery. The temporary pacemaker can be removed when the patient’s condition improves, or a permanent pacemaker is implanted (Hinkle & Cheever, 2018; Lak & Goyal, 2021). Pacemakers have two components: an electrical pulse generator and the pacemaker electrodes (see Figure 37). The electrical pulse generator determines the rate (in beats per minute) and strength (in milliamperes [mA]) of the stimulus delivered to the heart. The generator can detect intracardiac electrical activity to respond appropriately (e.g., sensitivity measured in millivolts [mV]). The sensitivity is set, and the intracardiac electrical activity must exceed this threshold to be sensed by the device. The pulse generator is most commonly placed in the infraclavicular region of the anterior chest wall. The pacemaker's leads carry the electrical impulse created by the generator to the heart. Most pacing systems use transvenous electrodes that transmit impulses from the generator to the heart musculature. These endocardial leads are threaded through a central vein into the heart (right atrium and ventricle). Less common systems work by direct stimulation: the generator attaches directly to the surface of the heart. These are epicardial wires and are always temporary (Hinkle & Cheever, 2018; Lak & Goyal, 2021). A pacemaker can pace the ventricle (see Figure 38), atrium (see Figure 39), or both. A pacemaker spike (i.e., a vertical line) is seen on an ECG when pacing is initiated. The appropriate ECG complex (termed capture) should immediately follow the pacemaker spike (i.e., a P wave when atrial pacing or a QRS complex when ventricular pacing). When pacing occurs, the impulse starts differently from the patient’s normal rhythm; therefore, the P wave and QRS complex look different from the typical ECG complex. There are three basic types of pacemakers: single chamber (i.e., one lead attaches to the upper or lower heart chamber), dual-chamber (i.e., one lead in the upper and one in the lower chamber), and biventricular (i.e., used for cardiac resynchronization therapy). The type of pacemaker and the settings selected are dependent on the patient’s dysrhythmia, cardiac function, and age. Generally, pacemakers are set to sense and respond to intrinsic activity (i.e., on-demand pacing). A fixed or asynchronous pacemaker is set to pace but not sense (i.e., paces at a constant rate regardless of intrinsic activity; Hinkle & Cheever, 2018; Lak & Goyal, 2021). Since pacemakers are widely used, universal codes are used to communicate the modes of each pacemaker. The coding system is known as NASPE-BPEG because it is sanctioned by the North American Society of Pacing and Electrophysiology and the British Pacing and Electrophysiology Group. The codes consist of five letters, with the fourth and fifth letters only used for permanent pacemakers. Most commonly, only the first three letters are used in the code (Hinkle & Cheever, 2018; Lak & Goyal, 2021). The letters refer to: - letter 1 refers to the area being paced (e.g., A designates the atria, V designates the ventricle, D designates dual, and O designates none) - letter 2 refers to the area which is sensed (e.g., A designates the atria, V designates the ventricle, D designates dual, and O designates none) - letter 3 refers to the response of the pacemaker to sensing (e.g., O designates non, I designates inhibiting, T designates triggering, and D designates dual) - letter 4 refers to rate adaptiveness (e.g., O designates none and R designates rate adaptiveness) - letter 5 indicates one of two things: - the permanent generator has multi-site pacing capability (A designates the atria, V designates the ventricle, D designates dual, and O designates none) - that the pacemaker has an anti-tachycardia function (Hinkle & Cheever, 2018; Lak & Goyal, 2021) The modes of a pacemaker can easily be identified by dividing them into single and dual chamber categories. Single-chamber modes include (Hinkle & Cheever, 2018; Lak & Goyal, 2021): - VOO (V designates pacing in the ventricle, O designates sensing is OFF, O means that the response to sensing is OFF) mode paces at a programmed rate regardless of intrinsic activity. - VVI (V designates pacing in the ventricle, V designates sensing in the ventricle, I designates inhibit) mode indicates that the pacemaker can sense electrical activity and withhold when not needed. - AOO (A designates pacing in the atrium, O designates sensing is OFF, O means that the response to sensing is OFF) mode paces at a programmed rate regardless of intrinsic activity. - AAI (A designates pacing in the atrium, A designates sensing in the atrium, I designates inhibit) mode indicates that the pacemaker can adapt to the intrinsic atrial rate and pace or inhibit if needed. Dual-chamber modes can be divided into tracking and non-tracking and include (Hinkle & Cheever, 2018; Lak & Goyal, 2021): - Tracking Modes - DDD (the first D designates pacing in the atrium and ventricle, the second designates sensing in the atrium and ventricle, and the third designates inhibit and/or trigger) mode can adapt to the intrinsic heart rhythm and mimic normal conduction. DDD has four distinct pacing patterns. - The AsVs (atrial sensed ventricular sensed) pattern is used when the sinus and AV node functions are good. - The AsVp (atrial sensed ventricular paced) pattern is used when sinus node function is good, but AV node function is poor. - The ApVs (atrial paced ventricular sensed) pattern is used when AV node function is good, but sinus node function is poor. - ApVp (atrial paced ventricular paced) pattern is used when sinus and AV node functions are poor. - VDD (V designates pacing in the ventricle, the first D designates sensing in the atrium and ventricle, and the second D designates inhibit and/or trigger) mode cannot pace the atrium. However, intrinsic atrial activity can trigger an AV delay, leading to P wave tracking and AV synchrony. VDD should only be used for patients with good SA node function because AV dissociation can occur if the sinus node function drops below the pacemaker rate. - Non-Tracking Modes - DDI (the first D designates pacing in the atrium and ventricle, the second designates sensing in the atrium and ventricle, and the I designates response to sensing will be either pace or inhibit) is primarily used in patients with atrial tachyarrhythmias. - DOO (D designates pacing in the atrium and ventricle, the first O designates sensing is OFF, and the second O establishes that the response to sensing is OFF) is an asynchronous pacing mode with AV sequential pacing at a lower rate regardless of intrinsic activity. - R (rate response or rate-adaptive pacing) is used for patients with chronotropic incompetence (i.e., the inability of the heart to increase the rate with increased activity). American Heart Association. (2016c). 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Sick sinus syndrome [Image]. https://commons.wikimedia.org/wiki/File:E265_(CardioNetworks_ECGpedia).jpg Sakr, M. (2013). Idioventricular rhythm [Image]. https://www.wikidoc.org/index.php/Idioventricular_rhythm Sandau, K. E., Funk, M., Auerbach, A., Blum, K., Cvach, M., Lampert, R., May, J. L., McDaniel, G. M., Perez, M. V., Sendelbach, S., Sommargren, C. E., & Wang, P. J. (2017). Update to practice standards for electrocardiographic monitoring in hospital settings: A scientific statement from the American Heart Association. Circulation, 136, e273-e344. https://doi.org/10.1161/CIR.0000000000000527 Shank Coviello, J. (Ed.). (2020). ECG interpretation made incredibly easy (7th ed.). Lippincott Williams & Wilkins Zuchinali, P., Souza, G. C., Pimentel, M., Chemello, D., Zimerman, A., Giaretta, V., Salamoni, J., Fracasso, B., Zimerman, L. I., & Rohde, L. E. (2016). Short-term effects of high-dose caffeine on cardiac arrhythmias in patients with heart failure: A randomized clinical trial. JAMA Internal Medicine, 176(12), 1752-1759. https://doi.org/10.1001/jamainternmed.2016.6374	2	32	1	0	0	0	0.972224	1	26,612
What is the ICD-10 classification system? International Classification of Diseases,Tenth Revision (ICD-10) The International Classification of Diseases (ICD) is designed to promote international comparability in the collection, processing, classification, and presentation of mortality statistics. Is ICD-10 a medical classification list? ICD-10 is the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD), a medical classification list by the World Health Organization (WHO). Who created the ICD-10 classification? ICD-10 codes were developed by the World Health Organization (WHO) . ICD-10-CM codes were developed and are maintained by CDC’s National Center for Health Statistics under authorization by the WHO. What is the latest ICD classification? ICD-11 was adopted at the World Health Assembly in May 2019 and Member States committed to start using it for mortality and morbidity reporting in 2022. What are the 3 classifications of disease? The most widely used classifications of disease are (1) topographic, by bodily region or system, (2) anatomic, by organ or tissue, (3) physiological, by function or effect, (4) pathological, by the nature of the disease process, (5) etiologic (causal), (6) juristic, by speed of advent of death, (7) epidemiological, and … What are the 3 volumes of ICD-10? The ICD-10 consists of three volumes: Volume 1 – Tabular list. Volume 2 – Instructions and guidelines manual. Volume 3 – Alphabetical index. How many ICD-10 codes are there? Key differences between ICD-9 and ICD-10 For example, ICD-10-CM has 68,000 codes, compared with 13,000 in ICD-9-CM, according to the Centers for Medicare & Medicaid Services (CMS). ICD-10 codes also have alphanumeric categories, while ICD-9 has numeric categories. What is the DSM ICD classification system? International Classification of Diseases (ICD) and Diagnostic and Statistical Manual of Mental Disorders DSM are systems that identify and classify diseases once the diagnosis is established. ICD and DSM are identical in some ways and differ in others. Are ICD-10 codes universal? Below you will find a basic explanation of ICD-10 codes, what they are and why they are used. Medical insurance companies have a billing system that consists of codes. These codes are universal among private health insurance companies, Medicaid and Medicare. What are ICD-10 codes examples? ICD-10-CM Code Examples - I25.110, Arteriosclerotic heart disease of native coronary artery with unstable angina pectoris. - K50.013, Crohn’s disease of small intestine with fistula. - K71.51, Toxic liver disease with chronic active hepatitis with ascites. What are ICD-10 diagnostic codes? ICD-9/ICD-10 are acronyms used in the medical field that stand for International Classification of Diseases, ninth/tenth revision. ICD diagnosis codes submitted by RREs on Section 111 Claim Input Files are used by Medicare claims paying offices to help process Medicare claims. What do ICD-10 codes mean? The ICD-10-CM (International Classification of Diseases, Tenth Revision, Clinical Modification) is a system used by physicians and other healthcare providers to classify and code all diagnoses, symptoms and procedures recorded in conjunction with hospital care in the United States. When will ICD-11 be implemented? 1 January 2022 When will ICD-11 come into effect? The new Revision of ICD was endorsed by the World Health Assembly at the 72nd meeting in 2019, and came into effect globally on 1 January 2022. From that date, health statistics will be reported in ICD-11. What is the difference between ICD-10 and 11? A central difference between ICD-11 and ICD-10 regarding chapter structure is the omission of a separate disorder grouping for mental and behavioral disorders with onset during childhood and adolescence. Can ICD-9 codes still be used? Currently, the U.S. is the only industrialized nation still utilizing ICD-9-CM codes for morbidity data, though we have already transitioned to ICD-10 for mortality. When was ICD-9 discontinued? ICD-9-CM is the official system of assigning codes to diagnoses and procedures associated with hospital utilization in the United States. The ICD-9 was used to code and classify mortality data from death certificates until 1999, when use of ICD-10 for mortality coding started. What is ICD-11 used for? ICD-11 is linked to the WHO non-proprietary names of pharmaceutical products, and it can be used for causes of death, primary care, cancer registration, patient safety, dermatology, pain documentation, allergology, reimbursement, clinical documentation, data dictionaries for WHO guidelines, digital documentation of … What does ICD-11 stand for? The International Classification of Diseases (ICD-11) is the 11th edition of a global categorization system for physical and mental illnesses published by the World Health Organization (WHO). Which ICD is currently used? The latest version of the ICD, ICD-11, was adopted by the 72nd World Health Assembly in 2019 and came into effect on 1st January 2022. … What are the 4 types of diseases with examples? There are four main types of disease: infectious diseases, deficiency diseases, hereditary diseases (including both genetic diseases and non-genetic hereditary diseases), and physiological diseases. How many versions of ICD are there? The ICD is revised periodically and is currently in its 11th revision. The ICD-11, as it is therefore known, was accepted by WHO’s World Health Assembly (WHA) on 25 May 2019 and officially came into effect on 1 January 2022.	4	7	0	0	0	6	0.590866	6	1,274
Type 2 Diabetes Type 2 diabetes is a chronic disease in which your body is unable to maintain a normal blood sugar (glucose) level. - Birth Control Facts about birth control to help decide which type is right for you. - Type 2 Diabetes Basic facts about type 2 diabetes & risk factors to be aware of. - COPD Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Flu Facts about influenza (flu), including symptoms and vaccines. - GERD Get the facts about gastroesophageal reflux disease (GERD). - Lupus Get the facts about lupus, including symptoms, risk factors, and the different types. - Menopause Facts about menopause, including the stages, symptoms, and types. - Ulcerative Colitis Facts about ulcerative colitis, including causes, signs and symptoms. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides About Dr. Mohammad S Ismail Dr. Mohammad S Ismail, MD is a Doctor primarily located in Paramount, CA. He has 37 years of experience. His specialties include Nephrology and Internal Medicine. Dr. Ismail is affiliated with California Hospital Medical Center, Lakewood Regional Medical Center and Promise Hospital of East Los Angeles, LP. He speaks English. Dr. Mohammad S Ismail has the following 2 specialties A nephrologist is a physician who is specially trained to diagnose and treat conditions affecting the kidneys or renal system. A nephrologist will determine through urine analysis, blood test, X-ray, sonogram, or kidney biopsy how well the kidneys are functioning and will then prescribe a special diet and exercise program, medication or dialysis - a process by which a machine filters the blood when the kidney is no longer capable of doing so. - Internal Medicine An internist is a physician who focuses on the diagnosis and treatment of conditions that affect the adult population—both acute and chronic. These doctors are often who adults see as their primary physicians because they treat a broad range of illnesses that do not require surgical or specialist interventions. They also work to help a patient maintain optimal health in order to prevent the onset of disease. In addition to treating the common cold and flu, internists also treat chronic diseases like diabetes and heart disease. - End Stage Renal Disease (ESRD) - Kidney Biopsy - Chronic Kidney Disease (CKD) - Chronic Renal Disease - Peritoneal Dialysis Showing 5 of 8 Arrived 15 minutes prior to the appointment given. The receptionists uses "mijo" or "Mija" for everyone whom checks in. Very unprofessional. However this specialist was assigned to my father due to an adonema found in his kidney, clearly stated on documents. She then returned asking why my father needed to be there? A little rude and horrible presentation on work ethic. After reviewing notes, it was clearly on the document and she then apologized and thanked us. Wow! I don't recommend it, it feels dirty. 12 patients waited, 8 seats, and doctor was a no show! Claiming he had to cover hospital?why schedule appointments then!? Very disappointing! Do not come! Dr. Ismail is affiliated (can practice and admit patients) with the following hospital(s). 37 Years Experience University Of Arizona College Of Medicine Graduated in 1981 U S Department Of Veterans Affairs Dr. Mohammad S Ismail accepts the following insurance providers. - Aetna Basic HMO - Aetna Choice POS II - Aetna HMO - Aetna HMO Deductible Plan CA only - Aetna Managed Choice POS Open Access - Aetna Savings Plus of CA - Aetna Signature Administrators PPO - Aetna Vitalidad Plus CA con Aetna BCBS Blue Card - BCBS Blue Card PPO Blue Cross California - BC CA California Care Small Group HMO - Blue Cross CA PPO Prudent Buyer Small Group - Blue Cross CA Advantage PPO Preferred DirectAccess Plus - Blue Cross CA California Care Large Group HMO - Blue Cross CA PPO Prudent Buyer Individual - Blue Cross CA PPO Prudent Buyer Large Group - Blue Cross CA Pathway X HMO - Blue Cross CA Priority Select HMO - Blue Cross CA Select HMO - Blue Cross CA Select Plus HMO Blue Shield California - Blue Shield CA Access Plus HMO - Blue Shield CA Access Plus Savenet - Blue Shield CA Bronze Full PPO 4500 - Blue Shield CA Local Access Plus HMO - Blue Shield CA PPO - Blue Shield CA Platinum Access+ HMO 25 - Blue Shield CA Platinum Local Access + HMO 25 - Blue Shield CA Trio ACO HMO - CIGNA HMO - CIGNA LocalPlus - CIGNA Open Access Plus - CIGNA Southern CA Value - First Health PPO - Health Net CA CommunityCare Network HMO - Health Net CA HMO Employer Group - Health Net CA HMO SmartCare - Health Net CA HMO Whole Care Network - Health Net CA Individual and Family PPO - Health Net CA PPO - Health Net EPO - PureCare One Small Business - Health Net PureCare HSP Individual and Family Plans - Health Net Salud EPO - Health Net SmartCare Large Group - Health Net SmartCare Small Group - Humana Choice POS LA Care Health - Multiplan PPO - PHCS PPO - PHCS PPO Kaiser - UHC SignatureValue Flex1 - UHC SignatureValue Flex2 - UHC SignatureValue Flex3 - UHC SignatureValue Focus - UHC West SignatureValue Alliance - UHC West SignatureValue HMO - UHC Choice Plus POS - UHC Options PPO - View by Location Locations & DirectionsMohammad Ismail Md, 16415 Colorado Ave Ste 207, Paramount, CA Dr. Mohammad S Ismail is similar to the following 3 Doctors near Paramount, CA.	2	8	1	0	0	0	0.936925	1	1,327
Tissue perfusion can be compromised in a number of disease conditions, including peripheral vascular disease (PVD), sepsis and septic shock, diabetic ulcers, pressure ulcers, and cancer, among others.1–4 Being able to measure tissue perfusion rates could prove highly impactful to clinical practice. However, few methods exist for doing so. One method for rough assessment of whole limb perfusion is assessment of cutaneous capillary refill time (CRT), which is done by pressing on a nail bed or a finger for 5 s and measuring the time for the pink color to return. However, CRT is devoid of metrics for diagnostic comparisons and is highly variable depending on the patient’s size, weight, tissue composition, and much more.5–8 Therefore, it is used to roughly gauge perfusion abnormality due to hypothermia, PVD, dehydration, and shock. It is not an imaging technique and provides no spatially resolved information about tissue perfusion. Laser Doppler speckle imaging (e.g., PeriMed), along with other speckle-based imaging such as laser speckle contrast imaging are examples of commercial perfusion-rate tracking system on a peripheral vascular system.9 However, these systems only measure very superficial blood-flow changes, are not fully quantitative, and provide no depth resolution.10,11 Depth-resolved perfusion imaging methods in peripheral vascular system used for preclinical purposes typically require contrast agents and thus limit applicability to screening tasks.12 Ultrasound imaging is able to measure the rate of perfusion by injecting microbubble contrast agents and destroying them at the imaging site. The reperfusion of these contrast agents is observed and signals are fit to models to estimate spatially resolved reperfusion times.13–15 While the destruction–reperfusion ultrasound imaging technique is a powerful means of imaging tissue perfusion, some work has shown that the destruction of microbubbles may damage microvascular walls.16,17 Other preclinical methods of measuring perfusion rates such as dynamic contrast-enhanced MRI also typically require contrast agents and are not sufficiently low cost or high throughput for widespread screening tasks. These applications have been applied to cerebral perfusion, myocardial perfusion, and cancer imaging, among others.18,19 Tissue perfusion has previously been defined as the volume-flow rate of blood exchange per given mass of tissue. In this study, we use a technique similar to ultrasound destruction–reperfusion imaging to estimate the so-called refill rate-associated tissue reperfusion. In the contrast-enhanced ultrasound approach, microbubble contrast agents are injected intravenously and those passing through the imaging plane are subsequently destroyed using an intense ultrasound pulse.13 The circulating microbubbles will re-establish flow through the imaging area and the signal recovery from the contrast agents is tracked using a recovering exponential model.13 The time-constant associated with this recovering exponential is the refill rate, and correlates with the tissue perfusion rate. In the present paper, instead of using microbubble contrast destruction–reperfusion methods, we instead use tissue compression and monitor reperfusion of blood using photoacoustic imaging. Similar to the destruction–reperfusion approach, we fit the replenishing blood flow signal to a recovering exponential model to estimate the refill rate. It should be understood that the volume of tissue which is monitored, such as the destruction–reperfusion technique, may not be well defined. However, relative reperfusion maps are still possible and may provide valuable data, which correlate with tissue perfusion. Photoacoustic imaging is a preclinical technology that enables a label-free method of visualizing various chromophores in the body, which includes but not limited to, oxy- and deoxy-hemoglobin, amino acids, and melanin. Being able to locate, image and differentiate oxy- and deoxy-hemoglobin allow the imaging technique to visualize vascular networks and quantify oxygen saturation within vessels.20–22 Furthermore, Doppler shift can occur for acoustic signals generated by moving chromophores, which can be used to determine the rate of blood flow.23–25 Ultrasound Doppler methods have likewise long been used to estimate blood flow velocities in vessels. However, to date no label-free ultrasound or photoacoustic technique has demonstrated the ability to estimate tissue perfusion rates in bulk tissue volumes involving many subresolvable or undetectable vessels. Our label-free method uses an ultrasound-photoacoustic (US-PA) dual imaging system to measure the perfusion rates in peripheral regions by pressing on the skin surface directly using the imaging transducer head. Due to the high resolution of PA imaging, we are able to detect net signals from tissue microvasculature, even if the vessel networks are not resolvable, allowing us to quantify changes in PA signals during compression and release. We fit the recovering mean photoacoustic signals to a first-order negative exponential model to create spatially resolved images which quantify perfusion rates. Perfusion-rate differences are seen in various cold- and hot-water-bath exposure conditions. Changes in capillary beds induced by compressions are imaged using the VEVO LAZR platform (Fujifilm, Visualsonics Inc.), a real-time US-PA dual imaging system. For this experiment, we used a LZ-550 transducer, which has a receive bandwidth of 32 to 55 MHz. Photoacoustic imaging is performed with 805-nm light delivered through a fiber bundle from the VEVO LAZR Optical Parametric Oscillator at the frame rate of 5 Hz. The laser power is , which is much lower than the ANSI safety limit of on the human skin and the frame rate is fast enough to detect PA signal changes during reperfusion as CRT in a healthy finger is known to be slightly under 2 s. This wavelength is selected because it is an isosbestic point where the molar extinction coefficient of oxy- and deoxy-hemoglobin is the same. At isosbestic point, the absorption coefficient for oxy- and deoxy-hemoglobin is equal, allowing for unbiased signatures from blood vessels with variable blood oxygenation. As shown in Fig. 1 below, when a soft tissue layer is compressed, blood is excluded from the compression volume, reducing the PA signal in the compressed area. The magnitude of loading rate is not measured nor it is constant. However, the load is measured by a force sensor (iLoad mini™, LoadStar Inc.) to maintain a maximum compressive pressure of 50 kPa for full ejection. The transducer is fixed to a transducer holder mounted on a stage, as shown in Fig. 2(b) for vertical compression. An example of sensing compression force synchronous with tracking the PA signal is shown in Fig. 2(c). Once the transducer is placed on the hand, where the area () is marked as shown in Fig. 2(a), it will not change its location for the duration of the experiment. In addition, a custom-made standoff pad () cut from a large ultrasound gel pad (Aquaflex®, Parker Laboratories Inc.) is placed within the transducer head casing to allow uniform pressure distribution along the area of compression. Analogous to US perfusion imaging with contrast agents, the recovering PA signals after compression is released will be fit to a negative exponential model, allowing us to quantify the perfusion rates. Human Subject Imaging Study To assess the change in perfusion rate, cold- and hot-water immersion tests are performed where the hand of a human subject is submerged in 4°C and 45°C water baths for various durations and the transducer head is used to directly compress on the hand to capture refill of blood vessels. The length of each compression-release (C-R) cycle is up to 10 s long with an interval between each cycle. The length of the interval time is set so that the durations of the C-R cycle and the interval time sum up to 30 s. Up to three cycles are performed. Human subject experiments were conducted in accordance with ethical protocols approved by the University of Alberta Health Research Ethics Board (Pro00007759). To demonstrate differences in refill rates of tissues subjected to temperature variations, we imaged a human palm in water immersion baths with different exposure times and temperatures as noted above. Perfusion-rate images were formed using the ultrasound and photoacoustic imaging data using the fitting methods described above. As shown in Fig. 3(a), on an initial image, a region of interest (ROI), marked by a yellow line, is chosen to define the boundary where the perfusion refill rate estimation will be performed. Within the chosen ROI boundary, a smaller ROI, which is denoted as a “sliding window,” shown as a green rectangle in the same figure, is defined. Mean PA signals inside the sliding window at each frame will be directly tracked. Note that the region of tissue associated with a sliding window may change in effective area as the tissue is compressed. We ideally want this sliding window to sample the same tissue volume during each stage of compression. Otherwise, vessels may move in or out of the window during compression, creating unwanted variation in the mean photoacoustic signal. For this reason, we use ultrasound data to track tissue motion to adaptively change the sliding window size to ensure that the same volume of tissue is being measured at each stage of compression. For this reason, we first separate each image into its respective US and PA components and use ultrasound data to track tissue motion to adaptively change the sliding window size to ensure that the same volume of tissue is being measured at each stage of compression, as shown in Fig. 3(b). Using a robust US strain estimation algorithm AM2D by Rivaz et al.,26 the RF signal of the B-scan at specific frame and the frame immediately after the chosen frame is processed to estimate both the axial and lateral displacements of the tissue, creating an ultrasound deformation map. The map is applied on the sliding window to create warping. After PA tracking in a sliding window, started in a specific location is completed, the tracking analysis repeats from the first frame with the sliding window starting from a location bounded by ROI, denoted by a blue arrow in Fig. 3(a). Also, AM2D only needs to run once as deformations are estimated on the entire image area. This process is repeated until the starting location of the sliding window covers the main ROI, the yellow boundary. During the tracking using MATLAB, (as measured with a 40-dB threshold) were detected and excluded from further PA analysis. This is to prevent flow rates from large vessels skewing the tissue perfusion rates as large vessels have much faster flow rate than arterioles and capillaries and they may be less affected by external temperature changes, which hinder accurate analysis of the perfusion rates of smaller vessel networks. To estimate the perfusion rates, a negative exponential equation, shown in Eq. (1), is used to model the PA signal recovery after the tissue is released from external loadingFig. 4. To demonstrate differences in reperfusion rates of tissues subjected to temperature variations, we imaged a human palm in water immersion baths with different exposure times and temperatures as noted above. Perfusion-rate images were formed using the ultrasound and photoacoustic imaging data using the fitting methods described above. The distribution of perfusion rates within the main ROI when the hand is submerged in the cold and hot water baths is shown in Fig. 5. Clear differences in the perfusion-rates are seen between differing temperature exposure conditions as visualized by the red-to-blue colormaps with red and blue colors representing fast and slow refill rates, respectively. Here the size of the main ROI is , and images in Fig. 5 had a sliding window size of to detect changes in PA signal. To explore how window sizes affect perfusion-rate estimates as larger window for averaging tends to reduce effect of noise, additional window sizes of , , and , respectively, are used and compared in Fig. 6. For 30, 60, and 90 s that the hand was submerged in the 4°C water bath, the mean refill rate constant ranges from 0.28 to , 0.29 to , and 0.14 to , respectively. In contrast, when the hand is submerged in a 45°C water bath for 0, 30, and 60 s, the mean refill rate constant ranges from 0.53 to , 0.84 to , and 1.15 to , respectively, depending on window sizes. The mean refill rates are listed in Table 1. The exposure time shown in Fig. 6 denotes the start time for each C-R cycle. The standard deviation of the perfusion rates varies with window size and is smallest when the sliding window is . The estimated mean refill rates. The submersion time is the duration the hand was submerged in a water bath before the start of the compression. \|Window size depth×length (mm×mm)\|\|Mean refill rate (s−1)\| \|Submersion time (s)\|\|30\|\|60\|\|90\|\|0\|\|30\|\|60\| We have demonstrated a method to image the perfusion rates in superficial tissues using a noninvasive, label-free, and US-PA imaging system. Statistically significant differences using paired -tests were observable () in mean perfusion-rates in multiple cold and hot temperature-exposure conditions. In addition, the distribution of perfusion rates in superficial tissue up to several mm depths can be estimated. Lower frequency probes and higher power light delivery could enable depths of multiple cms in future work. The ultrasound-photoacoustic system is capable of additional imaging modes, such as color Doppler and power Doppler. In principle, power Doppler could potentially be used to estimate the changes in flow associated with refill after release of compression. However, when tissue is compressed or released, the flow signal from vessels is overwhelmed with the Doppler signal from tissue motion. This takes several seconds to recover, which is too long considering that the capillary refill occurs within a couple of seconds (Fig. 6). The variance of the perfusion-rate estimates increases with increasing body temperature. This may be due to the fact that fewer data points exist on the recovering exponential fit when the reperfusion rate is high. Errors in displacement estimation impact how a given region of tissue deforms and thus impacts the associated window deformation for analysis. Future work should investigate the impact of errors in this changing window size. Using an imaging system with higher frame rate will improve the temporal resolution of the perfusion rates and will permit faster C-R cycles. Future work should investigate optimal sliding window sizes, optimize for large-vessel rejection, and push limits of depth and frame rate. Furthermore, more careful analysis from larger sets of data should be performed. Reperfusion rate measurements using ultrasound perfusion phantoms and in vivo studies involving multiple human subjects must be performed, which may improve data sensitivity and yield more accurate results. Perfusion-rate estimation method used in this paper deals with photoacoustic signal that are detectable but are spatially unresolvable. The large standard deviation may be due to the low SNR of unresolvable signals. In addition, the hand was not restrained during the measurement and the human subject had to intentionally focus on keeping the hand still. For future works, a laser system with higher power delivery and higher frequency transducer will allow improved SNR for small vessels, allowing more accurate analysis of perfusion-rate estimation. As tissue is compressed, both acoustic and optical properties of tissues may be impacted.27 Applied pressure may also exclude blood, lowering the net absorption, while effective distance to vessels will decrease. Thus compression may alter effective fluence at vessel locations. Currently, the present analysis ignores such effects but future work should aim to better incorporate these effects for more quantitative perfusion-rate estimation. The negative exponential model for calculating the perfusion rate, , is analogous to the wash-in rate (WIR) of contrast agents used in various contrast-enhanced perfusion imaging techniques. Future work could compare our label-free compression-reperfusion method with contrast-based perfusion imaging techniques. However, it is anticipated that quantitative perfusion rates from our approach may not necessarily match reperfusion rates from contrast-based approaches. For example, the WIR of destruction–reperfusion contrast ultrasound may depend on the thickness of the elevational beam waist, among other considerations. The WIR of our compression-reperfusion PA imaging will largely depend on the size of the compression area and internal pressures may be depth dependent. Future work should investigate these issues and correlate our label-free approach with contrast-enhanced methods. Label-free perfusion imaging could have a significant role in assessing tissue health in a number of diseases, including diabetes, cancer, etc.3,17,18 Dynamic contrast-enhanced MRI has show significant potential for discriminating malignant from healthy tissues in a range of cancers including breast cancer and prostate cancer. However, it is expense and the necessity of contrast injections precludes it from widespread screening applications. Our approach is not only label free but potentially high throughput and relatively low cost. Future work should investigate deeper tissues and various cancers and disease models. Viscoelasticity of the soft tissue may introduce tissue relaxation that can reopen vessels during the compression phase. However, the impact of these effects should be minimal as compression and relaxation are performed on timescales faster than tissue viscoelastic relaxation rates, typically several seconds.28–30 Future work should investigate the impact of tissue viscoelastic properties on perfusion-rate estimation and account for heterogeneity in tissue mechanical properties. We have demonstrated a label-free imaging method for mapping perfusion rates of tissues using C-R photoacoustic and ultrasound microimaging. Given it is real-time and label-free nature, the approach could have significant value in clinical settings where high-throughput screening is desirable. The authors have no relevant financial interests in this article and no potential conflicts of interest to disclose. R. Zemp is chief scientific officer and cofounder of illumiSonics Inc., which, however, did not support this work. We gratefully acknowledge funding from NSERC (355544-2008, 375340-2009, STPGP 396444), Terry-Fox Foundation and the Canadian Cancer Society (TFF 019237, TFF 019240, CCS 2011-700718), Alberta Innovates Health Solutions AIHS CRIO Team Award No. 201201154, the Alberta Cancer Research Institute (ACB 23728), the Canada Foundation for Innovation, Leaders Opportunity Fund (18472), Alberta Advanced Education and Technology, Small Equipment Grants Program (URSI09007SEG), and Alberta Ingenuity/Alberta Innovates scholarships for graduate and undergraduate students. Min Choi is pursuing an MSc degree at the University of Alberta, with a focus on using photoacoustic and ultrasound imaging to study the vascular system. A. M. James Shapiro is a clinician-scientist and professor of surgery at the University of Alberta. He led a clinical team to pioneer the “Edmonton Protocol” islet transplant program which has become internationally known. He has been the recipient of multiple awards, including the Hunterian Medal from the Royal College of Surgeons of England, the Gold Medal in Surgery from the Governor General of Canada, Physician of the Century, and was recently named one of Nature Biotechnology’s most remarkable and influential personalities. He was elected fellow of the Royal Society of Canada in 2012. He is a clinical hepatobiliary and pancreatic oncology and transplant surgeon, and also maintains an active immunology/transplant research laboratory. His group is actively researching personalized medicine approaches to pancreatic and other hepatobiliary cancers, with generation of human tumor transplantation in immunodeficient mouse models, using a novel prevascularized subcutaneous implantation site model. Roger Zemp is a professor of electrical and computer engineering and biomedical engineering at the University of Alberta. He earned his PhD from the University of California, Davis in 2004. He was a postdoctoral fellow in the Optical Imaging Laboratory of Lihong Wang at Texas A&M University and Washington University in St. Louis from 2004 to 2007. He joined the faculty at the University of Alberta in 2007. He has pioneered the development of novel photoacoustic imaging technologies including Photoacoustic Remote Sensing, and developed strategies for photoacoustic molecular imaging. He also has pioneered novel methods of ultrasonic three-dimensional and ultrafast imaging and leads a multidisciplinary team of engineers, biologists, chemists, and clinicians to develop new biomedical imaging strategies for clinical and biological applications.	2	13	1	0	0	0	0.795514	1	4,322
What are the ICD 10 Codes for Type 2 Diabetes? ICD-10-CM codes are a system used by doctors and healthcare professionals to code and classify symptoms, diagnoses, and procedures. The codes provide a level of detail that is essential for accuracy and specificity. Prior to the implementation of ICD-10-CM codes, the ICD-9-CM system was used. Both systems use unique alphanumeric codes to identify diseases and other health problems, as well as to help compile mortality and morbidity statistics. The system is published by the World Health Organization. ICD-10-CM codes are important as all Health Insurance Portability and Accountability Act-covered organizations must adhere to them. They also provide useful information about the severity of someone’s condition. Type 2 Diabetes ICD-10 A recent study found that ICD-10-CM coding changes that have recently been made for Type 2 diabetes have significantly improved coding for the condition, with the majority of codes sufficiently able to describe different diagnoses. The codes also represent an improvement over the ICD-9 system as rather than simply ‘controlled’ or ‘uncontrolled’ classifications, the system now has ‘inadequately controlled’, ‘out of control’ and ‘poorly controlled’ options. Categorizing diabetes in ICD-10-CM Diabetes is classified as an ‘endocrine, nutritional or metabolic disease’. It’s different forms are listed under these primary categories: - E08: Diabetes mellitus due to underlying condition - E09: Drug or chemical induced diabetes mellitus - E10: Type 1 diabetes mellitus - E11: Type 2 diabetes mellitus - E13: Other specified diabetes mellitus These categories are then divided into sub-categories, with codes used to specify characteristics of individual patient cases. Generally, diabetes ICD-10-CM codes are combinations of codes that identify the type of diabetes a patient has, any bodily systems that are affected, and any complications that have arisen. Let’s take an example. If a diabetic patient has chronic diabetes-related kidney disease, at stage 3, takes insulin daily and has a diagnosis of Type 2 diabetes, their codes would look like this: - E11.22: Type 2 diabetes mellitus with diabetic chronic kidney disease - N18.3: Chronic kidney disease, stage 3 (moderate) - Z79.4: Long term (current) use of insulin These codes help to specify the exact nature of the patient’s condition. Some types of diabetes have combination codes that cover all specifics of that type, and in these cases only one code is necessary. Coding in combination with other conditions To be truly accurate when coding ICD-10-CM for diabetes, you must note any underlying conditions that have caused or are impacting the diabetes. Category E08 defines ‘diabetes due to underlying condition’ and so these conditions must be noted first. The next category, E09, defines diabetes induced by drugs or chemicals – in these cases, the drug or chemical that has been determined as the cause should be noted first. In cases of gestational diabetes (diabetes that manifests in pregnancy but goes into remission after birth), the code should include a final character that indicates the trimester of the pregnancy. This is important as poorly controlled Type 2 diabetes in the second and third trimesters of pregnancy can cause babies to be born at above-average birth weight, which is a risk to both mother and child.	4	4	0	0	0	6	0.956943	6	744
I. Causes of Heart Failure: What every physician needs to know. What is heart failure? Heart failure (HF) is a clinical syndrome in which the heart is unable to provide sufficient blood flow to meet the metabolic needs of the body, or is only able to do so with significantly increased intracardiac filling pressure. However, this is a somewhat older definition, and many people now define heart failure as a clinical syndrome where there is structural heart disease and impaired function. Heart failure is often referred to as congestive heart failure (CHF). Many experts believe that the term “congestion” does not uniformly apply to all patients, and the syndrome is often referred to as “heart failure” in more contemporary times. Heart failure is most often characterized as systolic, if ejection fraction (EF) is decreased, and diastolic, if ejection fraction is preserved. “Diastolic” heart failure is also often called heart failure with preserved ejection fraction (HFPEF) or heart failure with preserved systolic function (HFPSF). Systolic heart failure is often due to damage to the heart, such as ischemia or alcohol exposure. Diastolic heart failure is often due to longstanding hypertension or intracardiac infiltration such as with amyloid protein, which “stiffens” both ventricles. It is not usually possible to distinguish systolic from diastolic heart failure based on signs and symptoms alone, as both share substantial clinical features. II. Diagnostic Confirmation: Are you sure your patient has Heart Failure? Heart failure is diagnosed at the bedside using history and physical findings. There is no absolute laboratory test for the diagnosis of heart failure. A characteristic history includes exertional dyspnea, fatigue, and edema. Physical exam would be notable for volume overload (jugular venous distention, dependent edema), S3 gallop, and often pulmonary rales. However, it is important to note that the absence of rales does not exclude heart failure, as the lymphatic system can provide substantial compensation, particularly in patients with chronic heart failure. Pulmonary congestion is typically evident on chest radiography. Plasma brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP) levels are typically elevated, although renal failure and obesity are commonly recognized confounders. The hallmark laboratory exam of heart failure is an echocardiogram. The presence of a decreased EF is sufficient to give a diagnosis of systolic heart failure; other modalities such as gated nuclear myocardial perfusion imaging, magnetic resonance imaging (MRI), or gated computed tomography (CT) are also able to determine EF. Diastolic heart failure is diagnosed in the presence of the clinical syndrome of heart failure with preserved EF. Efforts should be made to determine the etiology of heart failure. A. History Part I: Pattern Recognition Exertional dyspnea and fatigue are virtually universal. Volume overload is usually found, but in the modern era of powerful loop diuretics, some patients do not manifest fluid overload. Nocturnal symptoms such as orthopnea and/or paroxysmal nocturnal dyspnea (PND) are also highly suggestive of heart failure. Abdominal discomfort or fullness is also a frequently reported symptom. Patients with well-compensated heart failure may have minimal symptoms and no evidence of volume overload. Characteristics of end-stage heart failure may include cachexia and Cheyne-Stokes respirations. B. History Part 2: Prevalence Approximately half of patients with heart failure have systolic failure and the other half have diastolic heart failure. Some have features of both types. Heart failure is perhaps more common in men and in those who identify as black racially. Risk increases with age. Regardless, the most important risk factors for developing heart failure on a population basis are hypertension, myocardial infarction, and diabetes. The majority of diastolic heart failure is a result of longstanding hypertension, and this phenotype of heart failure is more common in women. Infiltrative diseases, such as amyloidosis and hemochromatosis, also typically lead to diastolic heart failure, often with restrictive physiology. Prior chest radiation is another potential cause of diastolic heart failure, and the incidence may increase as prolonged cancer survivorship increases. Once patients are hospitalized with diastolic heart failure, the prognosis is approximately the same as systolic heart failure. Hypertension and diabetes also increase the risk of systolic heart failure through the increased risk of coronary artery disease and myocardial infarctions. Thus efforts to minimize coronary artery disease would be anticipated to have substantial impact on risk of developing heart failure. The causes of systolic heart failure are myriad, including coronary artery disease, genetic or familial, viral (including HIV), idiopathic, valvular, inflammatory (myocarditis, sarcoidosis), thyroid disease, tachycardia-mediated (often from previously unrecognized atrial fibrillation), stress-induced (also known as Takotsubo cardiomyopathy), nutritional deficiency (such as thiamine or selenium), systemic myopathies (such as muscular dystrophies), and peripartum cardiomyopathy. Alcohol, cocaine, methamphetamines, anthracyclines, some antirheumatic medications (such as hydroxychloroquine) and tyrosine kinase inhibitors are common toxic causes of heart failure, which are typically identified through patient history. C. History Part 3: Competing diagnoses that can mimic Heart Failure Common alternative causes of volume overload include renal failure, liver failure, and hypoalbuminemia. Common alternative causes of exertional breathlessness are chronic obstructive pulmonary disease (COPD), pulmonary embolism (PE), and pneumonia. Coronary artery disease also may present with a dyspnea-predominant phenotype, rather than chest pain, as is classically expected. D. Physical Examination Findings Evaluation of the central venous pressure is of the utmost importance. Signs of volume overload (jugular venous distention, hepatomegaly, dependent edema, pulmonary rales) Extra heart sounds (S3 and/or S4) Severe hypertension suggests diastolic heart failure Profoundly low cardiac output states may be characterized by hypotension and/or narrow pulse pressure along with cool or mottled skin. Sinus tachycardia is common but may be compensatory. E. What diagnostic tests should be performed? Chest x-ray (CXR) and EKG are essential in the evaluation of a patient with suspected heart failure. Beyond providing information supporting the diagnosis of heart failure, these diagnostic tests often provide an alternative diagnosis (atrial fibrillation, pneumonia, lung mass, etc.) that implies heart failure may not be the key diagnosis. Echocardiogram is also essential and will provide substantial information about the size, structure, and function of the heart. MRI, which provides detailed information on the myocardium, should be considered. MRI can provide strong, noninvasive evidence of etiology in many cases, such as prior infarction, myocarditis, hypertrophic cardiomyopathy, left ventricular noncompaction, stress or “Takotsubo” cardiomyopathy, amyloidosis, sarcoidosis, or hemochromatosis, for example. Positron emission tomography (PET) can also be used to diagnose cardiac sarcoidosis. Endomyocardial biopsy should be considered in highly select circumstances, such as suspicion of myocarditis. Biopsy also has a role in defining the subtype of amyloid cardiomyopathy (e.g., transthyretin versus amyloid light-chain (AL)). Patients with a family history of cardiomyopathy should be offered the option of referral for genetic counseling and testing. 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? BNP (or NT-proBNP) should be considered, particularly if other causes of dyspnea such as COPD are being considered if there is doubt about the diagnosis. Very low levels virtually exclude heart failure, but can be confounded by obesity. Elevated levels can be seen in renal failure without substantial heart failure, but at least suggest volume overload. Elevated BNP in the absence of renal failure is strongly suggestive of heart failure, but BNP levels are increased with age. Basic metabolic parameters including renal and hepatic function should be measured. Renal dysfunction may be a seen as a result of low cardiac output or elevated venous filling pressure. Hepatic parameters (ALT, AST, total bilirubin) and international normalized ratio (INR) may be elevated as a consequence of hepatic congestion. Serum albumin is helpful in excluding malnutrition as an alternative explanation for peripheral edema. Minor elevations in troponin are characteristic of heart failure exacerbation, but typically do not rise and fall as in acute coronary syndrome; myocarditis should be considered if troponin is substantially elevated. Inflammatory markers such as C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR) would also be typically elevated in myocarditis, but are not diagnostic if elevated. Anemia (hemoglobin or hematocrit) and gout (uric acid) and are also frequent comorbidities to consider. Additional testing to establish the etiology of heart failure should be considered and may include: HIV, ferritin (hemochromatosis), thyroid-stimulating hormone, and serum free light chains (amyloid), for example. Testing for specific viruses other than HIV is not routinely performed, but Coxsackie, Epstein-Barr, and varicella zoster could be considered. Exclusion of connective tissue disease with antinuclear antibodies (ANA) is also often performed. 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? CXR film (Figure 1) may show cardiomegaly, but the absence does not exclude heart failure. Pulmonary edema is an important finding, but can be confused with pneumonitis or other causes of diffuse infiltrates. Accompanying findings such as pleural effusions may be helpful in interpretation. An echocardiogram will provide size; structural detail, including evaluation of valves; and myocardial function, including EF and presence of absence of regional wall motion abnormalities. This is the most important test when considering heart failure as a diagnosis. Abdominal ultrasound may demonstrate hepatomegaly and/or ascites, although these findings are often evident on exam and thus are not routinely indicated. The hallmarks of treatment of diastolic heart failure are control of congestion, heart rate in the presence of atrial fibrillation to increase ventricular filling time, and control of systolic blood pressure. The majority of evidence on the treatment of heart failure has been for systolic heart failure, and is thus the focus of the remainder of this section. There are three “to do’s” in the management of heart failure: (1) control congestion, (2) provide neurohormonal antagonists, and (3) consider sudden cardiac death prevention and cardiac resynchronization therapy (CRT). Since congestion causes most symptoms, this should be prioritized. Congestion control is achieved with sodium and sometimes (if there is hyponatremia) moderate fluid restriction; most patients need loop diuretics. Congestion is managed the same for systolic and diastolic heart failure. Second, neurohormonal antagonists (angiotensin-converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB), beta-blockers, aldosterone antagonists, and vasodilators in select populations) are critical for the treatment of systolic heart failure and should be initiated. Although it is critical to use the drugs and doses that were found to be effective in landmark clinical trials, very few patients will tolerate initiation of full doses of these medications, and a period of uptitration over several weeks to months should be anticipated. There is no consensus regarding whether beta-blockers or ACEI/ARB should be initiated first, and beta-blockers should be avoided in the setting of cardiogenic shock, severe bradycardia, complete heart block, or bronchospasm. Studies of neurohormonal antagonists for diastolic heart failure have been disappointing, but neurohormonal antagonists are still often used for their antihypertensive effect, and their safety in this population is well-established. Although there is no mortality benefit, digoxin has been noted to improve symptoms and reduce the risk of hospitalization. Finally, consideration should be given to “electrical therapy,” which consists of CRT (if QRS >130 ms) and sudden cardiac death prevention with an implantable cardioverter-defibrillator (ICD). The risk of sudden cardiac death is much higher with an EF <35%, and in the absence of contraindications such as life expectancy less than 1 year, electrical therapy should be considered. Patients should be receiving optimal medical therapy for at least 3 months before implanting an ICD. Wearable external defibrillators are sometimes used during the period of medical uptitration to permit reevaluation of EF; a sizable fraction of patients will improve on medical therapy to the point that an implantable ICD is not indicated. Patients who are unable to tolerate medical therapy or experience progression despite these therapies suggest a very poor prognosis and may be considered for advanced options, such as mechanical circulatory support (MCS, also known as ventricular assist device or VAD) or heart transplant. Author’s Preferred Method of Medication Uptitration ACEI/ARB and beta-blockers are both initiated at minimal dose, but uptitration of ACEI/ARB is typically prioritized, as full dose can typically be achieved more quickly. Beta-blocker dose is then increased every 1 to 2 weeks to the maximally tolerated dose. Timing of the introduction of aldosterone antagonist is individualized based on need for diuretics and potassium supplementation, and often deferred to prioritize ACEI/ARB and beta-blockers. A. Immediate management. Administration of diuretics is the hallmark of emergency treatment of heart failure and most patients respond promptly. Inadequate response should precipitate rapid escalation of a diuretic dose. A common rule of thumb is to double an ineffective dose. Consideration should be given to vasodilators if systemic blood pressure is adequate (e.g., mean arterial pressure greater than 70 to 75 mm Hg). In the event of hypotension or cardiogenic shock, initiation of inotropes, such as dobutamine and/or dopamine, may be necessary. Noninvasive positive pressure ventilation is an option and mechanical support such as an intraaortic balloon pump may also be indicated. B. Physical Examination Tips to Guide Management. In addition to evaluation of symptomatic response, physical exam findings that should be monitored while providing treatment for heart failure include frequent evaluation of jugular venous pressure and vital signs, with particular attention to blood pressure and heart rate. Sinus tachycardia is often a compensatory response and improvement is thought to represent a favorable response to treatment. Serial examinations should also focus attention on the presence of absence of S3/S4, liver span, presence or absence of ascites, and degree and distribution of edema. Urine output should also be monitored, as this provides the most direct measure of pace of decongestion and also indicates adequate (or not) renal perfusion. C. Laboratory Tests to Monitor Response To, and Adjustments in, Management. Basic metabolic panels are typically performed shortly after initiation of therapy (at least daily while hospitalized), to monitor renal function and ensure that diuretics are not causing dangerous perturbations in serum potassium levels. Following initiation of aldosterone antagonists, more frequent monitoring of potassium is indicated. A commonly used strategy is 1 week after initiation or dose increase, and then again at 1 month. Once a stable medication regimen has been established, routine intermittent testing in heart failure patients is not typically performed. There is some data to suggest that BNP levels may be used to guide therapy. D. Long-term management. Maintaining patients with heart failure on maximally tolerated doses of neurohormonal antagonists (ACEI/ARB, beta-blockers, aldosterone antagonists) are critical to minimizing risk of disease progression, symptomatic decompensation, hospitalization, and mortality. Application of CRT when indicated has also been shown to improve outcomes, and should be periodically reconsidered, as patient status or criteria for implant may change over time. Education on salt avoidance, the role of fluid restriction, and self-care including daily weight monitoring are all important to reduce burden of disease and avoid hospitalization. Over time, it is not uncommon for patients with heart failure to become intolerant to maximal doses of medical therapy. The need to decrease the doses of medications is a predictor of adverse outcome, and may be an appropriate time to consider evaluation for advanced options. For patients with end-stage disease, palliative care and/or hospice may be appropriate, and deactivation of ICD may be considered. E. Common Pitfalls and Side-Effects of Management Wrong medication (using atenolol, in lieu of carvedilol, metoprolol, or bisoprolol, for example) Inadequate dose (lisinopril 2.5 mg is insufficient) ACEI/ARB generally increase creatinine about 20% or less and this modest change should not precipitate cessation ICD too soon (placement immediately after diagnosis and before attempts to improve EF with medical therapy may result in placement of a device that is not ultimately indicated) Stopping neurohormonal therapy before considering decrement in diuretic dose Decreasing evidence-based neurohormonal therapy based on concern for hypotension with systolic blood pressure (SBP) above the goal range of 80 to 95 mm Hg (or ^mean arterial pressure MAP of 70 mm Hg) Use of non-steroidal anti-inflammatory drugs (NSAIDs), which can precipitate decompensation Not uptitrating beta blocker due to history of reactive airway disease or asymptomatic, mild bradycardia Overly aggressive fluid restriction in the absence of hyponatremia Captopril 50 mg t.i.d. Enalapril 10-20 mg b.i.d. Fosinopril 20-40 mg daily Lisinopril 20-40 mg daily Quinapril 20-40 mg daily Ramipril 10 mg daily Trandolapril 4 mg daily Candesartan 32 mg daily Losartan 150 mg daily Valsartan 160 mg b.i.d. Bisoprolol 2.5-20 mg/day (more popular in Europe) Carvedilol 25 mg b.i.d. or 80 mg daily of controlled release (CR) Metoprolol succinate 200 mg daily Spironolactone 25 mg daily Eplerenone 25-50 mg daily Digoxin is sometimes used in patients with atrial fibrillation and heart failure with insufficient rate control. Typical dose is 62.5 to 250 mcg daily. IV. Management with Co-Morbidities Concurrent anemia: Treatment may improve heart failure symptoms. Concurrent arrhythmias: Consider the role that uncontrolled arrhythmias may have on heart failure exacerbation. Improved control of atrial fibrillation or premature ventricular contractions (PVCs) may improve symptoms. Many antiarrhythmics are contraindicated in severe heart failure. Concurrent cancer: Consider whether antineoplastics may be contributing to heart failure (e.g., tyrosine kinase inhibitors) Concurrent chronic kidney disease: Continue ACEI/ARB if possible, but if necessary to discontinue, replace with isosorbide/hydralazine combination. May contribute to anemia. Aldosterone antagonists have a substantially increased risk of hyperkalemia. Concurrent diabetes: Avoid thiazolidinediones (TZDs) in all heart failure patients and metformin if there is significant renal dysfunction. Concurrent gout: It is likely that diuretic dosing will need to be increased if a gout flareup is treated with steroids. Concurrent liver disease: Liver congestion is common and may result in the need to alter dose of medications metabolized by the liver. Concurrent osteoarthritis: Avoid nonsteroidal antiinflammatory drugs (NSAIDs) Concurrent pulmonary disease: Does not typically contraindicate beta-blocker usage Concurrent prostatic hypertrophy: Alpha blockers should be avoided, as they have been shown to increase mortality in heart failure patients. Concurrent sleep apnea: Should be treated aggressively to minimize neurohormonal activation. Aldosterone antagonists should not be used in patients with underlying chronic kidney disease. Consider use of the Seattle Heart Failure Model or the Heart Failure Survival Score for estimating a patient’s prognosis. Consider referral to dietitian to augment teaching on importance of low-salt diet. V. Patient Safety and Quality Measures A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. Weigh yourself at the same time every day and record the result. Report increases of more than 3 lb in a day or 5 lb in a week to your doctor; however, not all decompensations are preceded by antecedent weight gain. Avoid sodium intake and strive to keep less than 2,000 mg daily. Avoid excess fluid intake. Take your medications as prescribed every day. Minimize alcohol intake. Report changes in symptoms promptly. B. What's the Evidence for specific management and treatment recommendations? “2009 Focused update incorporated into the ACC/AHA 2005 guidelines for the diagnosis and management of heart failure in adults: A report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines. Developed in collaboration with the International Society for Heart and Lung Transplantation”. C. DRG Codes and Expected Length of Stay. MS-DRG 291 (heart failure and shock with major comorbid conditions); mean length of stay 6 to 7 days MS-DRG 292 (heart failure and shock with comorbid conditions); mean length of stay 4 to 5 days MS-DRG 293 (heart failure and shock without comorbid conditions); mean length of stay 3 to 4 days (Data from UHC and Premier databases for 2010) Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM. - I. Causes of Heart Failure: What every physician needs to know. - II. Diagnostic Confirmation: Are you sure your patient has Heart Failure? - A. History Part I: Pattern Recognition - B. History Part 2: Prevalence - C. History Part 3: Competing diagnoses that can mimic Heart Failure - D. Physical Examination Findings - E. What diagnostic tests should be performed? - 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? - 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? - III. Management. - A. Immediate management. - B. Physical Examination Tips to Guide Management. - C. Laboratory Tests to Monitor Response To, and Adjustments in, Management. - D. Long-term management. - E. Common Pitfalls and Side-Effects of Management - IV. Management with Co-Morbidities - V. Patient Safety and Quality Measures - A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. - B. What's the Evidence for specific management and treatment recommendations? - C. DRG Codes and Expected Length of Stay.	2	23	0	2	0	0	0.985219	2	5,214
Identification and symptoms are necessary for classification through ovarian cancer ICD 10. Let’s know about symptoms, risk factors, and treatment. Ovarian cancer is among the fifth leading causes of cancer-related deaths of females in the United States. With that being said, the research done by the American Cancer Society suggests that the ovarian cancer survival rate has been higher in the United States over the past two decades due to more info and knowledge about the subject. In 2019, the ACS found that about 22,520 people will get an ovarian cancer diagnosis. About 13,980 people died due to this illness. Let’s get into detail about the ovarian cancer symptoms, how to know them, and what steps to follow when they’re there. What Is Ovarian Cancer? Cancer forms when cancer cells in the body start growing out of control. Cells in any body part may turn cancerous and may spread. Earlier ovarian cancers were thought to start in ovaries, but according to recent findings, many ovarian cancers begin in cells far from the end of the fallopian tube. Types Of Ovarian Cancers The ovaries consist of 3 main cell types. Each cell may turn into another tumor type: - Germ cell tumors begin from cells producing the eggs (ova). - Stromal tumors begin from the structure of tissue cells holding the ovary and producing female hormones progesterone and estrogen. - Epithelial tumors begin from cells covering the ovary’s outer surface. Most ovarian tumors are classified as epithelial cell tumors. Many such tumors are (non-cancerous) benign and don’t move beyond ovaries. Rather malignant (cancerous) and borderline (low malignant potential) ovarian tumors spread (metastasize) to other body parts and can turn fatal. How To Diagnose Ovarian Cancer? It is simple to treat ovarian cancer when the doctor finds it in its early stages. However, it is not so easy to find out in the early stages. The ovaries are deep inside the abdominal cavity, so you’re not likely to feel any tumor, unlike other types of cancers. It is important to report any constant or new symptoms to the doctor. When the doctor is concerned about ovarian cancer, they’ll most likely suggest a pelvic examination. Performing a pelvic exam helps the doctor find any irregularities, but it’s not easy to feel little ovarian tumors. As the tumor grows, it presses against the rectum and the bladder. The doctor would or would not be able to find any irregularities in a rectovaginal pelvic examination. The doctor might also suggest a few tests like: Transvaginal Ultrasound (TUVS) The imaging test TUVS uses sound waves for tumor findings in reproductive organs like the ovaries. However, TVUS can’t help the doctor know whether these are cancerous tumors. Pelvic CT & Abdominal Scan People who are allergic to the dye can order pelvic MRI scans. Blood tests to find cancer antigen 125 (CA-125) levels. The CA-125 test is needed to find treatment responses for ovarian cancer with other reproductive organ cancers. However, uterine fibroids, menstruation, and uterine cancer also affect the CA 125 blood levels. Chest CT Scan This is done to test lung metastasis. A small ovary tissue sample is collected and studied under a microscope in a biopsy. Positron Emission Tomography (PET) scan The test has a special dye with radioactive tracers injected or swallowed. Then tissues and organs absorb the tracer, allowing a doctor to note the working of those organs. While with the help of such tests, a doctor can move towards a diagnosis, the biopsy is the gold standard to confirm the presence of ovarian cancer. ICD 10 For Ovarian Cancer The ovarian cancer ICD 10 code is C 56.9, a billable code used to indicate a diagnosis for reimbursement. The C56.9 2022 ICD-10-CM came to effect on 1st Oct 2021. The ICD-10-CM American version and other international ICD-10 versions can be different. The codes mentioned below above the C56.9 have annotation back-references that apply to C56.9: - C00 – D49 for Neoplasms - C51 – C58 Malignant neoplasms of the female genital organs - C56 – Malignant ovarian neoplasm While bilateral ovarian cancer is prevalent, ICD 10 terminology was previously not there in the bilateral ovarian cancer code. The SGO Coding and Reimbursement Subcommittee appealed it. Annually, the Centers for Medicaid Services (CMS) takes papers that outline newly revised and deleted ICD 10 codes to be effective as the new fiscal year begins in October. The FY22 updates are effective from Oct. 1, 2021, including 159 more, 25 less, 27 revisions, and various updates for guideline coding. Two new diagnosis codes are there for the bilateral ovaries for primary and secondary malignancy. The coding committee in SGO requested such additions in 2019! - C56 ovary malignant neoplasm - C56.2 left ovary malignant neoplasm - C56.3 bilateral ovaries malignant neoplasm – New Code - C56.9 malignant neoplasm of any unspecified ovary These codes are for assigning the primary location, not the metastatic illness site. How To Treat Ovarian Cancer? Ovarian cancer treatment is based on the spread of the cancer cells in the body. Doctors determine treatment plans for the situation. It most likely includes two or more of the following: - Hormone therapy - Targeted therapy - Surgery to stage cancer and also remove the tumor It is among the main treatment choices for ovarian cancers. The surgery aims at taking out the tumor, but uterus removal can often be needed. Doctors can also suggest removing fallopian tubes and ovaries, nearby lymph nodes, and other pelvic tissues. It is difficult to find out all tumor locations. Researchers investigated ways to enhance surgical processes, making the process of removing the cancerous tissue simple. Targeted therapies like chemotherapy attack cancer cells without doing too much damage to the body’s normal cells. Other targeted therapies for treating advanced epithelial ovarian cancer cells include PARP inhibitors. Such drugs block an enzyme by cells to repair DNA damage. Cancer treatments like radiation, chemotherapy, and surgery can result in reproductive organ damage, making pregnancy difficult. When you want to become pregnant, talk to the doctor before starting treatment. They also discuss options to preserve fertility possibly. The options for preserving the fertility possibility are: Freezing embryo: It involves fertilized egg freezing Oocyte freezing: The process involves freezing an unfertilized egg Surgery for preserving fertility: Surgery can only remove a single ovary in some cases. It is mostly possible in the early stages of ovarian cancer. Suppression of ovaries: This means taking hormones to suppress ovarian function temporarily. Preservation of the ovarian tissue means taking out and freezing the ovarian tissue for future use. Preventing Ovarian Cancer Reducing the risk factor can be done in many ways: - Avoid any risk factors. - Manage a healthy weight and lifestyle - Avoid hormone replacement therapy post-menopause. - Gynecologic surgery: tubal ligation and hysterectomy minimize the chances of getting certain ovarian cancer types. However, these surgeries should be reserved for valid medical reasons rather than their ovarian cancer risk influence. - Prevention for women with ovarian cancer family history or BRCA mutation - Use of oral contraceptives - Genetic testing - Removing both fallopian tubes and ovaries: generally not only for ovarian cancer prevention. Most ovarian cancers begin in the outer lining or the epithelium of the ovary. You can have a few or no ovarian cancer symptoms in the early stages. When symptoms are there, they resemble other conditions, like premenstrual syndrome, irritable bowel syndrome, or a temporary bladder issue. However, the signs of ovarian cancer persist and become worse. Related Topic:- OUTLOOK ON RECTAL CANCER TREATMENT WITHOUT SURGERY Early Symptoms Can Include Early symptoms of ovarian cancer may include: - Pressure or pain in the pelvis - Pain in the abdomen, back - Unexpected vaginal bleeding - Urination pattern changes, like frequent urination. - Bowel habit changes, like constipation When any of such symptoms are there for two weeks, the person should consult a doctor. Additionally, there can be symptoms like: - Indigestion and nausea - Weight loss - Appetite loss The symptoms change when cancer spreads to other body areas. Risk Factors And Root Causes Ovarian cancer can develop when cells in this body area multiply and divide uncontrollably. However, why ovarian cancer occurs is not clear, but experts have identified many risk factors. These are: Having a close relative with a breast or ovarian cancer history increases someone’s chance of having ovarian cancer. Reproductive history of one or more full-term pregnancies is linked with lower ovarian cancer risk. With more pregnancies, the risk becomes lower. Breastfeeding can also minimize the risk factor. However, later having children or never having kids can increase the risk. People having some fertility treatment types have more chances of getting borderline cells, but many studies haven’t confirmed it. Women who use birth control pills or injectable contraceptive hormones are also at lower risk. Women with breast cancer history may have more chances of having ovarian cancer. It is because of changes in the BRCA gene. Due to this reason, some people with breast cancer test positive for such gene mutation and might go for surgery or oophorectomy to remove ovaries as a preventive therapy. If you’re having signs of ovarian cancer and suspect it in the first place, it’s best to consult your doctor about the same. However, the best advice would be to consult cancer specialists at University Cancer Centers, who aim to utilize the best technologies to offer intensive treatment and care to cancer patients. Our world-class infrastructure and the most modern cancer research technologies are patient-centric and have positive outputs	4	6	0	0	0	5	0.497014	5	2,165
Current Procedural Terminology (CPT) Current Procedural Terminology (CPT) is a standardized medical code set used to report medical procedures and services for billing and reimbursement purposes. What is Current Procedural Terminology (CPT)? Current Procedural Terminology (CPT) is a standardized medical coding system used to describe and report medical, surgical, and diagnostic services provided by healthcare professionals. It is developed and maintained by the American Medical Association (AMA) and is widely used in the United States healthcare system. CPT codes are alphanumeric and provide a uniform language that allows healthcare providers, insurance companies, and other stakeholders to communicate and understand the services rendered during patient encounters. CPT codes are used for billing and reimbursement purposes, as well as for tracking healthcare utilization, conducting research, and analyzing healthcare trends. They provide a detailed description of medical procedures, services, and tests, allowing for accurate documentation and appropriate reimbursement. CPT codes are regularly updated to reflect advancements in medical technology and changes in healthcare practices. Difference between CPT, ICD, and HCPCS While CPT is a coding system used to describe medical procedures and services, there are other coding systems that serve different purposes within the healthcare revenue cycle management (RCM) process. It is important to understand the differences between CPT, International Classification of Diseases (ICD), and Healthcare Common Procedure Coding System (HCPCS) codes. ICD codes, specifically ICD-10-CM (Clinical Modification), are used to classify and code diagnoses, symptoms, and medical conditions. They provide a standardized way to document and track patient conditions, which is essential for medical billing, research, and public health monitoring. ICD codes are used in conjunction with CPT codes to provide a comprehensive picture of the patient encounter.HCPCS codes, on the other hand, are used to identify and report healthcare services and supplies that are not included in the CPT coding system. HCPCS codes are divided into two levels: Level I codes, which are identical to CPT codes and used for physician services, and Level II codes, which are used for durable medical equipment, prosthetics, orthotics, and other supplies. HCPCS codes are essential for Medicare and Medicaid billing and reimbursement. In summary, CPT codes describe medical procedures and services, ICD codes classify diagnoses and conditions, and HCPCS codes identify additional healthcare services and supplies. Examples of CPT Codes CPT codes cover a wide range of medical procedures, services, and tests. Here are a few examples of commonly used CPT codes: 1. 99213 - Office or other outpatient visit for the evaluation and management of an established patient, which typically includes a detailed history, examination, and medical decision-making. 2. 36415 - Collection of venous blood by venipuncture for laboratory testing. 3. 29881 - Arthroscopy, knee, surgical; with meniscectomy (medial or lateral, including any meniscal shaving) including debridement/shaving of articular cartilage (chondroplasty), same or separate compartment(s), when performed. 4. 45378 - Colonoscopy, flexible, proximal to splenic flexure; diagnostic, with or without collection of specimen(s) by brushing or washing, with or without colon decompression (separate procedure). 5. 81002 - Urinalysis, by dipstick or tablet reagent for bilirubin, glucose, hemoglobin, ketones, leukocytes, nitrite, pH, protein, specific gravity, urobilinogen, any number of these constituents; non-automated, without microscopy. These examples illustrate the diversity of services covered by CPT codes, ranging from routine office visits to complex surgical procedures. Each code represents a specific service or procedure and is associated with a corresponding reimbursement value. Importance of CPT Codes in Healthcare Revenue Cycle Management CPT codes play a crucial role in healthcare revenue cycle management. They are used throughout the billing and reimbursement process to ensure accurate and timely payment for healthcare services. Here are some key reasons why CPT codes are important: 1. Accurate Billing: CPT codes provide a standardized way to document and bill for medical procedures and services. By using specific codes, healthcare providers can accurately communicate the services rendered to insurance companies, ensuring appropriate reimbursement. 2. Reimbursement: Insurance companies use CPT codes to determine the amount of reimbursement for healthcare services. Each code is associated with a relative value unit (RVU), which represents the complexity and resource utilization of the service. RVUs are then multiplied by a conversion factor to calculate the reimbursement amount. 3. Compliance: CPT codes help ensure compliance with coding and billing regulations. By using the appropriate codes and accurately documenting the services provided, healthcare providers can avoid potential audits, penalties, and legal issues. 4. Data Analysis: CPT codes allow for the collection and analysis of healthcare utilization data. By tracking the frequency and types of procedures performed, researchers and policymakers can identify trends, assess healthcare needs, and make informed decisions. 5. Cost Estimation: CPT codes facilitate cost estimation for healthcare services. Insurance companies and patients can use these codes to estimate the cost of specific procedures, helping them make informed decisions about healthcare utilization and financial planning. In conclusion, CPT codes are a vital component of healthcare revenue cycle management. They provide a standardized language for describing and reporting medical procedures and services, ensuring accurate billing, reimbursement, and data analysis. Understanding CPT codes is essential for healthcare professionals, insurance companies, and other stakeholders involved in the revenue cycle management process.	4	5	7	0	0	0	0.561455	7	1,176
Skip to content Broadly speaking, injury is physical harm or damage to the body. It may be intentional or unintentional. If intentional, it may be self-inflicted (for example, suicide) or inflicted by another (for example, assault and homicide). The harm can be caused by any of the following things: • an external force (such as a collision with an object) • energy (heat, electricity, etc.) • external or internal contact with a harmful substance (poisoning, etc.) • the absence of an essential element (such as oxygen or heat) . Normally, only harmful effects occurring over a short time are considered injury. (The term ‘injury' is used generally in reference to those conditions classified within the ICD group ‘external causes of morbidity and mortality' - V01 to Y98). Assessing the total impact of injury is difficult. The vast majority of injuries do not result in hospitalisation or death and there are little systematic data on them other than those collected as part of large-scale population health surveys (such as the periodic National Health Surveys conducted by the Australian Bureau of Statistics). Thus, the vast majority of injuries are not recorded in routine data collections, and may not be brought to the attention of health policy-makers and program managers. For injuries that are serious enough to be recorded in the routine data collections or are identified by specific studies, there are some issues with their classification. The classification of injury has generally followed the World Health Organization's International Classification of Diseases (ICD), which includes particular attention to the external cause and intention of the injury. The 10th revision of the World Health Organization's International Classification of Diseases (ICD) is now applied in Australia to deaths and (in the somewhat more extensive ‘Australian Modification') to hospitalisation . The ICD-10 classification codes injuries in terms of their nature (for example, fracture of the vault of the skull) and the external cause of the injury (for example, assault by blunt instrument) . Because it is more useful for preventive purposes, most reporting of injury is in terms of external causes, the broad categories of which are as follows: • accidents - transport accidents (including motor-vehicle accidents) and other external causes of accidental injury (falls, burns, accidental poisoning, etc.) • intentional self-harm (including suicide) • assault (including homicide) • events of undetermined intent • legal interventions and operations of war • complications of medical and surgical care • sequelae of external causes of morbidity and mortality • supplementary factors related to causes of morbidity and mortality classified elsewhere.(this coding provides for factors like alcohol involvement, including blood alcohol levels if known). The ICD categories are useful for broad epidemiological studies, but have serious limitations for detailed investigations for injury prevention. Many Indigenous injuries fall into categories in which there is little detail (for example, falls). In addition, it is apparent that culture affects the way in which information about an injury-causing event is described to investigators and clinicians, and the way this is interpreted through coding . An example is the uncertainty over how traditional Indigenous punishment practices should be coded - depending upon the perspective taken, they could be recorded as an accident, a legal intervention, or violence. The magnitude of a problem can be assessed, but a detailed understanding of the causes cannot be obtained using the ICD system . Attempting to address these issues, Weeramanthri and Plumber proposed an alternative system to the ICD for the classification of cause of death. Their system emphasises the underlying rather than the direct cause of death, and the ICD classifications were replaced with the following categories: Land (diseases of the physical environment), Body (so-called ‘lifestyle diseases'), Spirit (diseases of poverty and cultural dislocation, including injury deaths), and Smoking-related. The authors calculated proportional mortality ratios and presented the results of a mortality analysis based on these ratios at feedback sessions and a workshop. No formal evaluation of this process was conducted, but informal feedback suggested that health information presented in this way was relevant and useful to the participating communities, and resonated more with the participants' world view. The development of injury-prevention projects and programs depends on a solid understanding of the various factors contributing to specific injuries. Reflecting the great diversity of injuries - and the diversity of disciplines and backgrounds among involved in injury prevention - approaches investigating these factors range from the traditional epidemiological single-risk-factor approach to broad sociological methods. The ecological model proposed in the World Report on Violence and Health provides one way of conceptualising the types of factors that need to be considered in the development of injury-prevention strategies . With a particular focus on violence, this model involves four levels: There are, of course, other ways of conceptualising the factors contributing to injury. Regardless of which classification scheme is used, however, it is important that the scope is wide enough to ensure that the analysis of ‘causes' will reveal most of the factors that need to be taken into account in the development of preventive strategies. This broad ecological approach to seeking causal factors is well established in the field of justice, being a feature of the work of the Royal Commission into Aboriginal Deaths and Custody , and also in the area of self-inflicted injury. It is a feature also of an analysis of road injuries in South Australia , but does not appear to be as widely used in the more traditional injury-prevention literature. Overall, however, the understanding of contributory factors is weak for most areas of injury, including injury among Indigenous people . Information facilitating the analysis of risk factors and mechanisms of injury is relatively scarce and varies across the specific topic areas considered. Most studies that have sought to identify risk factors have failed to explore the interplay of risk factors (for example, young males' alcohol consumption, risk-taking, and exposure to hazardous environments) . If we are to understand how these factors influence each other, more longitudinal, in-depth research is required. Such research, with greater collaboration between fields of study, should also help us identify the point in the chain of events that can offer the greatest opportunity for intervention. © Australian Indigenous HealthInfoNet 2013 This product, excluding the Australian Indigenous HealthInfoNet logo, artwork, and any material owned by a third party or protected by a trademark, has been released under a Creative Commons BY-NC-ND 3.0 (CC BY-NC-ND 3.0) licence. Excluded material owned by third parties may include, for example, design and layout, images obtained under licence from third parties and signatures.	2	2	0	0	0	2	0.69668	2	1,374
Urinary tract infections (UTI) are a serious health problem affecting millions of people each year. Infections of the urinary tract are the second most common type of infection in the body. Only upper respiratory tract infections (colds and flu) happen more often. According to the Centers for Medicare and Medicaid Services (CMS), catheter-associated UTIs are the most common hospital-acquired infection, accounting for more than 1 million cases in hospitals and nursing homes nationwide. With the implementation of MS-DRG, the importance of coding MCCs and CCs, and the inclusion of catheter-associated UTIs as an initial preventable hospital acquired condition, correct coding and assignment of UTIs is more important than ever. A UTI is a bacterial infection of any part of the urinary tract. When bacteria get into the urinary tract and multiply in the urine, they cause a UTI. Predisposing factors for urinary tract infection include calculi or other urinary tract obstruction, foreign bodies such as stents or catheters, congenital urinary anomalies, pregnancy, diabetes mellitus, and neurogenic bladder. Women are approximately 10 times more likely to develop a UTI than men. Physicians often use the term UTI when referring to cystitis, urethritis and pyelonephritis. Lower UTIs include urethritis and cystitis, while pyelonephritis is an upper UTI. Not all patients have symptoms, but most do have some. Symptoms include dysuria (difficult or painful urination), increased urinary frequency, urgency and often hematuria. Other symptoms include chills, fever, nausea and vomiting, pain and a general feeling of malaise. Each type of UTI may result in more specific signs and symptoms, depending on which part of the urinary tract is infected. Cystitis is an infection or inflammation of the urinary bladder. Cystitis occurs when the lower urinary tract is infected by bacteria and becomes irritated and inflamed. Symptoms include pelvic pressure, lower abdomen discomfort, frequent, painful urination and cloudy, strong-smelling urine. Pyelonephritis or pyelitis is a urinary tract infection that has spread from the bladder to the kidneys. Kidney infection can cause upper back and flank pain, headache, high fever, shaking chills, and nausea or vomiting. In severe cases, delirium may be present. Urethritis is an infection of the urethra. Symptoms include urethral discharge, dysuria, urgency and increased frequency. Urinalysis may show significant bacteriuria, often accompanied by proteinuria, hematuria and pyuria. Urine or catheter culture reveals growth of >100,000 colonies of a single organism. Blood work may reveal neutrophilic leukocytosis and positive blood culture, especially if the infection involves the upper urinary tract. White blood cell shows count of >10,000. Coding Acute UTIs One of the most important aspects of coding an acute UTI is documentation of site specificity in the medical record. Acute pyelonephritis: 590.1X, Acute pyelonephritis Acute cystitis: 595.0, Acute cystitis Urethritis: 597.80, Urethritis, unspecified Code 599.0, Urinary tract infection, site not specified, should be assigned only if the physician has not identified or is unable to identify the site of the UTI. Code 599.0 should not be used in combination with codes that specifically identify the site of the UTI. For example, if the terms acute cystitis and UTI are both documented separately on the final diagnosis sheet, only the code for the acute cystitis, 595.0, should be assigned. However, if two specific sites are documented then both should be assigned. Coding guidelines require the assignment of an additional code if the causative organism is identified. The UTI code is sequenced first, followed by the organism. E. Coli is a common organism causing UTIs and is reported with code 041.4. Codes from category 599 should not be reported for UTIs in newborns. Code 771.82, Urinary tract infection of newborn, should be assigned instead. UTI due to Candidal yeast requires one code, 112.2, Candidiasis of other urogenital sites. However, UTI due to noncandidal yeast requires two codes. Code first the appropriate UTI code, followed by 117.9, Other and unspecified mycoses. UTI and Sepsis If the documentation indicates that the UTI has progressed to sepsis, code 038.X should be assigned first, then code 995.91, Sepsis, followed by the appropriate UTI code. In this instance sepsis indicates that the UTI has entered the bloodstream and becomes a generalized sepsis. The systemic infection, sepsis, should be sequenced before the localized infection, UTI. There has been considerable debate in the coding community regarding urosepsis. The term urosepsis is a nonspecific term. The ICD-9-CM Official Guidelines for Coding and Reporting states that if urosepsis is the only term documented, then only code 599.0 should be assigned based on the default for the term in the ICD-9-CM index. However, often the term urosepsis is used by physicians when in fact the infection has spread to the bloodstream and has progressed to sepsis. The physician should be queried to clarify whether the urosepsis is intended to mean sepsis or UTI. If the physician indicates that the UTI has developed into sepsis, assign the appropriate sepsis codes and then the appropriate UTI code as indicated above. If urosepsis is meant to mean UTI, then code 599.0 should be assigned along with the causal organism. If a UTI is diagnosed after a procedure is performed, the coder should not assume that the UTI is a complication of surgery. The physician should be queried. If the postoperative UTI is linked to the procedure, two codes are required; 997.5, Complications affecting specified body systems, urinary complications, and the appropriate UTI code. If the causative organism is identified it is also coded. If the physician states that the UTI is not linked to the procedure or is not result of the procedure, then only the appropriate UTI code is assigned. Foley Catheter Induced UTI A common source of UTIs is catheters placed in the urethra and bladder. Often urinary catheters are inserted during surgery. A perso who cannot void or who is unconscious or critically ill often needs a catheter that stays in place for a longer period of time. Bacteria on the catheter can infect the bladder, so hospital staff must take special care to keep the catheter clean and remove it as soon as possible. As indicated earlier, catheter-associated UTIs are the most common hospital-acquired infection, accounting for more than 1 million cases in hospitals and nursing homes nationwide. According to CMS, it is estimated that hospital-acquired UTIs require one extra hospital day per patient, at an estimated annual cost of $424 to $451 million. These conditions are reported with code 996.64, Infection and inflammatory reaction due to indwelling urinary catheter, and the appropriate UTI code. Recurrent and Chronic Recurrent or chronic UTI without current symptoms should not be coded to the acute infection. Code V13.02, Personal history of urinary (tract) infection, may be assigned to report this. Code V58.62, Long-term (current) use of antibiotics, may also be assigned if the patient is receiving prophylactic antibiotic therapy. UTIs should not be coded based upon lab results alone. Urine specimens are subject to contamination during collection from microorganisms or by improper handling and storage. Always verify the diagnosis with the physician. UTI is a commonly missed CC, which may affect MS-DRG assignment. If the medical record contains documentation suggestive of this condition, query the attending physician if it should be added as a secondary diagnosis. Check yourself on proper coding for UTIs with the quiz below: 1. A patient presents to the ED with a UTI that is due to an indwelling urinary catheter. Which of the following would be the appropriate diagnosis code(s) selection? a. 996.64, 599.0, E879.6 b. 599.0, 996.64, E879.6 c. 996.64, 599.0 d. 996.64, E879.6 2. Patient with chronic cholecystitis is admitted for an elective cholecystectomy. After admission it was noted by the physician that the patient had developed a UTI based on laboratory tests that were positive for E.Coli. The patient was started on antibiotics, the surgery was cancelled and the patient was discharged. Discharge orders indicate that the surgery was rescheduled in 3 weeks. Which of the following would be the appropriate diagnosis code(s) selection? a. 599.0, 041.4, 575.11, V64.1 b. 575.11, 599.0, 041.4, V64.1 c. 575.11, 599.0, V64.1 d. 599.0, 575.11, V64.1 3. A patient was discharged following prostate surgery with an indwelling catheter in place. He was readmitted with urinary sepsis due to staphylococcus aureus due to the catheter. The catheter was removed and the patient was started on antibiotic therapy. The patient improved over several days and he was discharged without the indwelling catheter. Which of the following would be the appropriate diagnosis code(s) selection? a. 996.64, 038.11, 995.91, 599.0, E879.6 b. 038.11, 995.91, 599.0, 996.64, E879.6 c. 996.64, 038.11, 995.91, E879.6 d. 038.11, 995.91, 966.64, E879.6 4. A preterm infant (2,450 grams at birth) was transferred to the current hospital for investigative studies after he was noted to have an elevated temperature and WBCs. A UTI was confirmed with a urine culture that was positive for E. coli. The infection was treated with intravenous antibiotics. Suspected septicemia was ruled out when all blood cultures were negative prior to institution of antibiotic therapy. Which of the following would be the appropriate diagnosis code(s) selection? a. 599.0, 041.4, 771.82, 765.18, 765.20 b. 771.82, 599.0, 041.4, 765.18, 765.20 c. 771.82, 041.4, 765.18, 765.20 d. 765.18, 771.82, 041.4, 765.20 This month's column has been prepared by Cheryl D'Amato, RHIT, CCS, director of HIM, facility solutions, Ingenix, and Melinda Stegman, MBA, CCS, clinical technical editor, Ingenix (www.ingenix.com). Ingenix develops software and e-commerce solutions for managing coding, reimbursement, compliance and denial management in the health care marketplace. Coding Clinic is published quarterly by the AHA. CPT is a registered trademark of the AMA. 1. a: Three codes are reported for this case. Code 996.64 is used to indicate that the infection is due to an indwelling urinary catheter. Code 599.0 is reported to identify the type of infection, UTI, site not specified. Code E879.6 may be reported to indicate that the urinary catheter is the cause of abnormal reaction or later complication. 2. b: The principal diagnosis does not change because the planned procedure was not carried out. Code 575.11, Chronic cholecystitis, is assigned as the principal diagnosis. Codes 599.0 and 041.4 are both assigned to report the UTI due to E.Coli. Code V64.1, Surgical or other procedure not carried out because of contraindications, is assigned to indicate that the procedure was cancelled due to the UTI. 3. a: Code 996.64 is reported as the principal diagnosis to indicate that the sepsis is due to an indwelling urinary catheter. Codes 038.11 and 995.91 are assigned to report the systemic infection, staphylococcus aureus sepsis. Code 599.0 is reported to identify UTI as the localized infection. Code E879.6 may be reported to indicate that the urinary catheter is the cause of abnormal reaction or later complication. 4. c: Code 771.82 is assigned as the principal diagnosis to report UTI of newborn. Code 041.4 is also assigned to report E. coli as the causative organism. UTI codes from category 599 are excluded for newborns and are not assigned. Code 765.18 is assigned to report the prematurity weighing 2,450 grams at birth. Code 765.20 is required to identify the number of weeks of gestation. In this case, it was unspecified.	4	4	0	0	0	4	0.725901	4	2,920
Alternative names for chronic fatigue syndrome Chronic fatigue syndrome (CFS) is the name currently used by the majority of the medical and scientific community to describe a condition or set of conditions characterized by fatigue and other symptoms. The term is contested, mostly by patients and patient advocacy groups, but also by some doctors. Several of the more common alternative names used to describe what most believe to be the same condition or subtypes include myalgic encephalomyelitis (ME), chronic fatigue immune dysfunction syndrome (CFIDS), and post-viral fatigue syndrome (PVFS). In 1959, E.D. Acheson an early investigator of benign myalgic encephalomyelitis, wrote: "The wisdom of naming a disorder, the nature of which cannot at present be proved, and which may be due to more than one agent, is debatable." The name used to label the condition (or set of conditions) is one of several controversies related to CFS. - 1 Current nomenclatures - 2 Historical nomenclatures - 3 References - 4 External links Chronic fatigue syndrome The name chronic fatigue syndrome (CFS) was used in the medical literature in 1987 to describe a condition resembling "chronic active Epstein-Barr virus (EBV) infection" but which presented no evidence of EBV as its cause. The initial case definition of CFS was proposed in 1988. These researchers had previously published their investigations of an apparent 1984 outbreak of mononucleosis-like symptoms in Lake Tahoe, Nevada, in the United States, where they found no strong evidence of a connection between Epstein-Barr virus and the symptoms. The name was specifically chosen to represent the major symptom of the condition and to avoid making a judgment about the cause, which was unknown. Today, the term CFS is accepted by scientists and most clinicians. Many patients, patient groups, and some clinicians perceive the term as trivializing, and as the 1994 Fukuda definition itself cedes, stigmatizing, which led to a movement in the United States to change the name and definition. The CFS Coordinating Committee of the United States Department of Health and Human Services formed a name change workgroup in 2000, recommending CFS be changed to "neuroendocrineimmune dysfunction syndrome" to imply specific underlying etiologies or pathologic processes. However, in 2003 implementation was halted when it was decided that a name change would be too disruptive at that time. Myalgic encephalomyelitis (ME) means "inflammation of the brain and spinal cord with muscle pain." ME is used in the current ICD10 classification system of the World Health Organization as a subheading under Brain Disorders - post-viral fatigue syndrome. A review from 2001 noted a report that indicates ME is a distinct condition from CFS, but CFS and ME are usually used as synonyms. The term "myalgic encephalomyelitis" is controversial as CFS has not been associated with any pathology of the muscles or central nervous system. For this reason, in 1996 the Royal Colleges of Physicians, Psychiatrists, and General Practitioners in the United Kingdom recommended use of the name chronic fatigue syndrome instead of myalgic encephalomyelitis. A letter to the editor in The Lancet explained the 1996 report received some acceptance, but also harsh criticism from patients and physicians who said their views had been excluded. Accordingly, the Working Party on CFS/ME that reported recommendations for the medical condition to the CMO in 2002, used both names in an attempt to reach out to patient groups and acknowledge the lack of consensus among professionals and patients on the name. The report declined to recommend one term over the other and preferred the compromise term ‘CFS/ME’. Many patients, and some doctors, especially in the United Kingdom, prefer to use ME on its own or in conjunction with CFS (ME/CFS or CFS/ME) because they believe it implies a more serious illness than the term chronic fatigue syndrome which focuses simply on fatigue. Margaret Williams, writing for the British campaign group MEActionUK, stated that the term "ME/CFS" was preferred because it more directly addressed a specific medical condition, while CFS/ME implied and placed emphasis on a condition of general fatigue. Some patient groups prefer the name myalgic encephalopathy as a synonym as this does not imply inflammation but simply an illness of the nervous system. The name myalgic encephalopathy has also been evaluated as being perceived as a more serious sounding illness than chronic fatigue syndrome by research outside of the UK. The first appearance of the term ME was in a 1956 Lancet editorial by Donald Acheson. Three years later, Acheson referred to several older reports and many different names that appeared to describe a similar syndrome, noting that the inflammation is presumed. Acheson also cites criticisms of the correctness of the name used in 1959, "benign myalgic encephalomyelitis", as the disease was not always benign, does not always cause muscle pain, and may not cause inflammation of the central nervous system. In 1962 Russell Brain included ME in his textbook of neurology. In 1978, the British Medical Journal reported that researchers at a Royal Society of Medicine conference to discuss the illness during that year clearly agreed that the symptoms described as myalgic encephalomyelitis made up a distinct nosological entity. The article also stated that, "From the patient's point of view the designation benign is also misleading, since the illness may be devastating." In 2011, an "international consensus criteria" for myalgic encephalomyelitis was published by a group of 26 individuals from 13 countries and consisting of clinicians, researchers, teaching faculty and an independent patient advocate. The authors rejected the name "chronic fatigue syndrome", re-asserted "myalgic encephalomyelitis" as the appropriate name and WHO classification based on "research and clinical experience that strongly point to widespread inflammation and multisystemic neuropathology", and criticized the primary emphasis on "fatigue". On the issue of ME vs CFS, the authors state that patients meeting this new criteria "should be removed from" the Reeves et al. CDC 2005 criteria for CFS and the 2007 NICE criteria for CFS. Chronic fatigue immune dysfunction syndrome Chronic fatigue immune dysfunction syndrome (CFIDS) is used by patients and some doctors in the USA, and by the Chronic Fatigue Immune Dysfunction Syndrome Association of America because of what they believe is the inaccuracy and the negative stigma attached to "chronic fatigue syndrome". The term calls attention to the immune dysfunction in patients, which extensive research suggests, is an integral part of the illness. The World Health Organization's ICD-10 classification system refers to CFS as post-viral fatigue syndrome (PVFS), based on the hypothesis that viruses can trigger chronic fatigue illnesses. After considerable research in this area, certain viruses have been implicated "as one of many possible precipitating and eventually perpetuating factors." Studies of chronic fatigue syndrome have reported increased rates of infection in subsets of patients with, among others, HHV-6, HHV-7, CMV, Epstein Barr, enteroviruses, and two retroviruses: an unnamed HTLV-II-like retrovirus, and XMRV. The "Oxford 1991" diagnostic criteria for CFS refer to a subtype of CFS called post-infectious fatigue syndrome (PIFS), which is broader than PVFS since it can include any infection, not just viral infections. Chronic Epstein-Barr virus Also known as Chronic Mononucleosis, the term CEBV was introduced in 1985 in the United States. The Epstein-Barr virus, a neurotropic virus that more commonly causes infectious mononucleosis, was thought to be the cause of CFS. Subsequent discovery of the closely related human herpesvirus 6 shifted the direction of biomedical studies, although a vastly expanded and substantial body of published research continues to show active viral infection or reinfection of CFS patients by these two viruses. These viruses are also found in healthy controls, lying dormant. This term was popularized in a November 1990 Newsweek cover story and was never official medical terminology. It reflects a stereotype that CFS mainly affects yuppies, and implies that it is a form of burnout. The phrase is considered offensive by patients and clinicians. CFS affects minorities at similar rates to Caucasians. Low natural killer syndrome This term, used mainly in Japan, reflected research showing diminished in-vitro activity of natural killer cells (NKs) isolated from patients and was used in a paper in 1987. Smaller proportions of NK cells and lower NK activity were also observed by some of the researchers who first used the term CFS. LNKS could be a condition separate from CFS. Other historical terms This is the term commonly used in New Zealand. It derives from the name of a town where numerous people suffered from the syndrome. Royal Free disease - Sharpe, Michael; Frankie Campling (2008). Chronic fatigue syndrome (CFS/ME). Oxford [Oxfordshire]: Oxford University Press. pp. 7–8. ISBN 0-19-923316-0. Retrieved 2009-10-23. - Acheson E (1959). "The clinical syndrome variously called benign myalgic encephalomyelitis, Iceland disease and epidemic neuromyasthenia." (PDF). Am J Med 26 (4): 569–95. doi:10.1016/0002-9343(59)90280-3. PMID 13637100. - Buchwald D, Sullivan JL, Komaroff AL (May 1987). "Frequency of 'chronic active Epstein-Barr virus infection' in a general medical practice". JAMA 257 (17): 2303–7. doi:10.1001/jama.257.17.2303. PMID 3033338. - Holmes G, Kaplan J, Gantz N, Komaroff A, Schonberger L, Straus S, Jones J, Dubois R, Cunningham-Rundles C, Pahwa S (1988). "Chronic fatigue syndrome: a working case definition.". Ann Intern Med 108 (3): 387–9. doi:10.7326/0003-4819-108-3-387. PMID 2829679. - Holmes GP, Kaplan JE, Stewart JA, Hunt B, Pinsky PF, Schonberger LB (May 1987). "A cluster of patients with a chronic mononucleosis-like syndrome. Is Epstein-Barr virus the cause?". JAMA 257 (17): 2297–302. doi:10.1001/jama.257.17.2297. PMID 3033337. - Prins JB, van der Meer JW, Bleijenberg G (January 2006). "Chronic fatigue syndrome". Lancet 367 (9507): 346–55. doi:10.1016/S0140-6736(06)68073-2. PMID 16443043. - Jason LA, Taylor RR. (2001). "Measuring Attributions About Chronic Fatigue Syndrome". J Chronic Fatigue Syndr 8 (3/4): 31–40. doi:10.1300/J092v08n03_04. TXT formal - Tuller, D (2007-07-17). "Chronic Fatigue Syndrome No Longer Seen as ‘Yuppie Flu’". The New York Times. Retrieved 2008-11-30. - "Advocacy Archives: Name Change". The CFIDS Association of America. Archived from the original on 8 January 2008. Retrieved 2008-01-16. - Lavrich, Carol (2003-09-29). Name Change Workgroup, CFSCC. Bethesda, Maryland: US Department of Health and Human Services, Chronic Fatigue Syndrome Advisory Committee. Archived from the original on 10 January 2008. Retrieved 2007-12-29. - Bell DS et al. (2003-12-03). Name Change. Washington, D.C.: US Department of Health and Human Services Chronic Fatigue Syndrome Advisory Committee (CFSAC) Second Meeting. Archived from the original on 27 February 2008. Retrieved 2008-01-16. - Whiting P, Bagnall AM, Sowden AJ, Cornell JE, Mulrow CD, Ramírez G (September 2001). "Interventions for the treatment and management of chronic fatigue syndrome: a systematic review". JAMA 286 (11): 1360–8. doi:10.1001/jama.286.11.1360. PMID 11560542. - Evangard B, Schacterie R.S., Komaroff A. L. (Nov 1999). "Chronic fatigue syndrome: new insights and old ignorance". Journal of Internal Medicine 246 (5): 455–469. doi:10.1046/j.1365-2796.1999.00513.x. PMID 10583715. Retrieved 2010-06-25. - "The Psychiatry Research Trust - Chronic Fatigue Syndrome". Archived from the original on 6 December 2008. Retrieved 2008-11-30. - Royal Colleges of Physicians, Psychiatrists and General Practitioners (1996). Chronic fatigue syndrome; Report of a joint working group of the Royal Colleges of Physicians, Psychiatrists and General Practitioners. London, UK: Royal College of Physicians of London. ISBN 1-86016-046-8. - Clark C, Buchwald D, MacIntyre A, Sharpe M, Wessely S (January 2002). "Chronic fatigue syndrome: a step towards agreement". Lancet 359 (9301): 97–8. doi:10.1016/S0140-6736(02)07336-1. PMID 11809249. - Working Party on CFS/ME (January 2002). "Report of the Working Party on CFS/ME to the Chief Medical Officer for England and Wales" (pdf). Department of Health. Archived from the original on 2003-03-22. Retrieved 2009-01-25. - Sharpe M (2002). "The report of the Chief Medical Officer's CFS/ME working group: what does it say and will it help?". Clin Med 2 (5): 427–9. doi:10.7861/clinmedicine.2-5-427. PMID 12448589. - Williams, M (2009-04-27). "ME/CFS Terminology". ME Action UK. Retrieved 2010-06-25. - Jason LA, Taylor RR, Plioplys S, Stepanek Z, Shlaes J (February 2002). 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I. Atrial Tachycardia: What every physician needs to know. An atrial tachycardia is a fast abnormal heart rhythm in which the electrical impulse originates in atrial tissue different than the sinoatrial node. Atrial electrical activation during atrial tachycardias is mostly regular and by definition at a rate faster than 100 bpm, although occasionally the rate may oscillate and be slower. Atrial tachycardias are the least frequent form of supraventricular tachycardias in the general population. The mechanisms of the arrhythmia could be abnormal automaticity, triggered activity, or reentry that is limited to atrial tissue. Atrial tachycardias are a form of supraventricular tachycardia and can be classified as focal or macro-reentrant, depending on their origin and propagation of the electrical impulse. They can also be classified by the mechanism of the arrhythmia. Focal atrial tachycardias are frequently assumed to be due to automaticity although micro-reentry and triggered activity are possible and difficult to demonstrate in practice. Macro-reentrant atrial tachycardias involve the participation of a reentry circuit within the atria. Neither the compact atrioventricular node (AV) node nor the accessory pathways participate in the mechanism of this arrhythmia. Atrial tachycardias can also be classified by the anatomic atrial structure where they originate from or that is involved in the reentry circuit (i.e., crista terminalis, tricuspid annulus, atrial appendages, mitral annulus-aorta continuity, and scar-related or mitral annulus reentry). The clinical presentation of this arrhythmia varies significantly from short, rather sporadic, paroxysmal runs to incessant tachycardia. Atrial tachycardias are often misdiagnosed as panic attacks and anxiety like any supraventricular tachycardia. The arrhythmia is commonly documented in the surface electrocardiogram as a narrow complex tachycardia and generically diagnosed as a supraventricular tachycardia. A definitive diagnosis of an atrial tachycardia may be only possible through specific clinical or electrophysiologic features of the arrhythmia requiring electrocardiography and sometimes even intracardiac recordings. Occasionally incessant atrial tachycardias can present as congestive heart failure secondary to dilated cardiomyopathy. The diagnosis of tachycardia-induced cardiomyopathy and adequate treatment of the atrial tachycardia could be life saving and result in complete resolution of the cardiomyopathy. Hemodynamic instability is possible but rather rare during atrial tachycardias. II. Diagnostic Confirmation: Are you sure your patient has an Atrial Tachycardia? The diagnostic confirmation of an atrial tachycardia requires detailed analysis of electrocardiographic or intracardiac electrogram recordings. Definitive diagnosis can be suspected but not confirmed in clinical grounds only. The clinical suspicion of an atrial tachycardia is usually brought by symptoms compatible with a supraventricular tachycardia. Sudden onset and offset of a regular narrow complex (QRS <120 ms) tachycardia coincidental with the presenting symptoms is very suggestive of a supraventricular tachycardia. Short bursts of tachycardia in between sustained episodes may suggest the diagnosis of atrial tachycardia but can be seen with any other form of supraventricular tachycardia. During atrial tachycardias, the P–R interval is usually normal, although in the presence of AV nodal disease the P–R interval could be prolonged. A rare but potentially fatal presentation is tachycardia induced cardiomyopathy with the constellation of symptoms caused by progressive systolic heart failure including edema, dyspnea on exertion, orthopnea and paroxysmal nocturnal dyspnea with documented depressed left ventricular systolic function in the setting of persistent tachycardia. A. History Part I: Pattern Recognition: Key symptoms: palpitations, chest discomfort, dyspnea at rest or exertion, dizziness, fatigue, near syncope, rarely syncope or could be asymptomatic. Key physical signs: regular tachycardia, unusual blood pressure (either higher or lower than baseline), pallor or flushing, rapid vascular peripheral pulsation waves, heart failure signs can be present when the tachycardia is associated with heart failure. Focal and reentrant atrial tachycardias have overlapping characteristics but may present with different clinical patterns and prevalence among different patient populations. Both can lead to tachycardia-induced cardiomyopathy when incessant over periods of several weeks or months. Focal atrial tachycardias Focal atrial tachycardias are rather infrequent and most commonly seen in normal hearts and younger patients, although they could develop at any age. They can present as an incessant tachycardia, which makes the diagnosis easier or as intermittent episodes of palpitations, as any other paroxysmal supraventricular tachycardia of sudden onset and offset. Focal atrial tachycardias can show acceleration and deceleration in response to changes in the autonomic tone with slight rate variations. Their origin has been described from multiple atrial and vascular structures within the atria, including crista terminalis, along the tricuspid and mitral annulus, pulmonary veins, fossa ovalis, coronary sinus, mitral annulus-aorta junction, vein of Marshall and atrial appendages (Figure 1, Macroreentrant atrial tachycardias Macro-reentrant atrial tachycardias are more frequent among patients with a history of structural heart disease, prior cardiac surgery, chronic obstructive pulmonary disease, pulmonary hypertension, prior ablation procedures involving the atria, or any other clinical condition leading to atrial dilatation or atrial scaring. Macro-reentrant atrial tachycardias could resemble typical cavo-tricuspid isthmus dependent atrial flutter (using a puristic definition atrial flutter itself could be considered a form of macroreentrant atrial tachycardia) and are frequently persistent although they could be paroxysmal as well (Figure 3, Figure 4). B. History Part 2: Prevalence: Atrial tachycardias, excluding atrial flutter, are thought to represent about 5% to 15% of sustained supraventricular tachycardias in the adult, and are more frequent in the pediatric population. Short, self-terminated runs of atrial tachycardia are rather common in ambulatory heart rhythm monitor recordings; but when asymptomatic and sporadic, they are frequently dismissed as incidental findings and not considered a clinical entity. Atrial tachycardias become more prevalent with age at the expense of a higher representation of macro-reentrant atrial tachycardias in the older population, while focal atrial tachycardias become rather rare. Focal atrial tachycardias are more common among younger patients with normal hearts. A gender difference in the prevalence of these arrhythmias is not clear, although higher prevalence of focal automatic atrial tachycardias in women has been reported. C. History Part 3: Competing diagnoses that can mimic an Atrial Tachycardia The other forms of supraventricular tachycardia are very difficult to differentiate from atrial tachycardias from the clinical standpoint: Typical AV nodal reentrant tachycardia (slow-fast AVNRT): cannon A waves appreciated at physical exam during tachycardia are suggestive of typical AVNRT. Undistinguishable P waves (simultaneous with the QRS), an RSr’ pattern during tachycardia in lead V1 that is absent during normal sinus rhythm, or P waves with a superior axis right after the QRS in the surface electrocardiogram are suggestive of typical AVNRT. Atypical AV nodal reentrant tachycardia (fast-slow or slow-slow AVNRT): electrocardiographically very difficult to differentiate from an atrial tachycardia. An electrophysiology study may be necessary to make a definitive diagnosis. Atrioventricular reentrant tachycardia (AVRT): during tachycardia, the P waves are usually close to the preceding QRS in AVRT resulting in a rather long P–R interval, which suggests a diagnosis different than atrial tachycardia but still possible in atrial tachycardias with prolonged P–R. An electrophysiology study may be necessary to make a definitive diagnosis. Permanent junctional reciprocating tachycardia (a unique form of AV reentrant tachycardia using a slow conducting retrograde accessory pathway): because of the participation of a slowly conducting accessory pathway as the retrograde limb of the tachycardia, this arrhythmia has retrograde P waves with a superior axis close to the following QRS resulting in a relatively normal P–R interval and very difficult to differentiate from an atrial tachycardia. The incessant nature of this arrhythmia, younger age of presentation, and frequent presentation as tachycardia-induced cardiomyopathy help lead the clinical differential diagnosis towards this entity. Junctional ectopic tachycardia: rather rare and more common in children. As a focal tachycardia originating in AV nodal tissue, it clinically presents similar to focal atrial tachycardias, but the electrocardiographic recordings are similar to those of typical AV nodal reentrant tachycardia usually showing absence of P waves that are simultaneous and obscured by the QRS. An electrophysiology study with pacing maneuvers is necessary to make a definitive diagnosis. Ventricular tachycardia: any supraventricular tachycardia that conducts to the ventricle with aberrancy resulting in a wide complex tachycardia, either due to bundle branch block or intraventricular conduction delay (underlying at baseline or developed during tachycardia), could resemble ventricular tachycardia. The clinical setting, characteristics of the arrhythmia, and AV relationship are useful in establishing the diagnosis, although not infrequently an electrophysiology study with intracardiac recordings is needed. In the differential diagnosis of supraventricular tachycardias, the administration of an intravenous bolus of adenosine (6, 12, or 18 mg administered rapidly and followed by a large bolus of saline flush) may be helpful. If intravenous adenosine does not terminate the tachycardia and results in AV block with continuation of the tachycardia in the atrium, the diagnosis of atrial tachycardia is confirmed. If the tachycardia terminates with intravenous adenosine, the mechanism of the tachycardia may be suspected as reentrant and possibly with the participation of the AV node but no definitive conclusion can be drawn since some focal atrial tachycardias, as well as macroreentrant atrial tachycardias, could terminate with intravenous adenosine. Atrial flutter: the characteristic electrocardiographic pattern and atrial rate around 300 bpm establishes the differential diagnosis. Nevertheless, in practice, there is an overlap between macroreentrant atrial tachycardias and atrial flutter. By mechanism, typical atrial flutter (a circuit of reentry in the right atrium using the cavo-tricuspid isthmus as the critical isthmus) is a form of macro-reentrant atrial tachycardia and many macro-reentrant atrial tachycardias could be considered forms of atypical atrial flutter, and manifest clinically and in the electrocardiogram as atrial flutter. A definitive diagnosis can only be made in the electrophysiology laboratory with the characterization of the tachycardia circuit. Atrial fibrillation: the characteristic electrocardiographic pattern of fibrillatory waves in the electrocardiogram and irregularity of the arrhythmia are diagnostic of atrial fibrillation. Inappropriate sinus tachycardia: often difficult to differentiate from an atrial tachycardia. Symptoms, clinical presentation, and patient population are very similar. The electrocardiogram during tachycardia shows P waves identical to the P waves during slower rates. Overlap of the P wave with the preceding T wave may make the comparison of the P waves difficult. An ambulatory heart rhythm monitor to document the progressive acceleration and deceleration of inappropriate sinus tachycardia without a sudden onset or change in P wave morphology is often required. Postural orthostatic tachycardia syndrome (POTS): P waves are identical to the P waves in sinus rhythm. The characteristic development of tachycardia and symptoms with upright posture and resolution of the symptoms help differentiate this syndrome from the other forms of supraventricular tachycardias. Physiologic sinus tachycardia: the P waves during tachycardia are identical to the P waves at slower rates. An underlying cause for the tachycardia eventually becomes apparent including anemia, volume depletion, hypoxia, pain, anxiety, or a drug effect (i.e., dopamine, albuterol). Frequent ectopy: a common reason for consultation; frequent atrial and/or ventricular ectopy could resemble the symptoms of atrial tachycardia and ambulatory rhythm monitoring devices can establish the diagnosis. D. Physical Examination Findings. The landmark physical finding in atrial tachycardias is the tachycardia itself, with a heart rate above 100 bpm and rarely dropping below but faster than the underlying sinus rhythm. Frequently, patients have no other physical findings related to the arrhythmia and while not in tachycardia their physical exam may be completely normal. Depending on the physiologic effects and rate of the tachycardia, like with any other supraventricular tachycardia, the patient may present the following symptoms during the arrhythmia: Change in blood pressure Underlying conditions that could predispose the development of atrial tachycardias may have more specific physical findings. Heart murmur in the case of valvular heart disease, hypertrophic cardiomyopathy, and congenital heart disease; sustained apical impulse in the case of left ventricular hypertrophy, barrel chest, tachypnea, or adventitious lung sounds in patients with underlying pulmonary disease. E. What diagnostic tests should be performed? The physical examination does not provide specific findings confirmatory of the diagnosis of atrial tachycardia. A suspicion is risen by the finding of tachycardia (heart rate >100 bpm) with no plausible physiologic explanation. Vagal maneuvers like Valsalva, carotid sinus massage (limited to patients without suspected carotid occlusive disease), and cold drinks, among others, may terminate the tachycardia and suggest the diagnosis of a supraventricular tachycardia but have no value on the specific diagnosis of an atrial tachycardia. Sinus tachycardia slows with vagal maneuvers with progressive deceleration/acceleration of the heart rate. Other findings in the physical exam could explain physiologic sinus tachycardia, including hypotension, hypoxia, signs of inflammation/infection, pallor, abdominal distention, or tenderness, etc. 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? Laboratory studies may be helpful in the diagnosis of underlying or associated conditions as well as in the differential diagnosis of sinus tachycardia but no laboratory test is useful in the specific diagnosis of an atrial tachycardia. Laboratory blood tests should be based on the physician’s clinical judgment after evaluation of the patient’s comorbidities, complaints, and physical findings. As an example, a complete blood count aids in the diagnosis of anemia or potential underlying infections; thyroid function tests help to address the clinical suspicion of thyroid disease; a blood gas analysis determines the presence and severity of hypoxia in predisposing pulmonary conditions; serum level of brain natriuretic peptide complements the evaluation of associated heart failure; and renal function tests and creatinine clearance have implications on the choice of antiarrhythmic drug. The diagnosis of supraventricular tachycardias and specifically atrial tachycardias is established with the documentation and characterization of the heart rhythm as discussed in section II E 2 below. 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? Documentation of the tachycardia and evaluation of the underlying cardiac condition are fundamental in the diagnosis and management of this arrhythmia: ECG: a 12-lead electrocardiogram recorded in sinus rhythm and during tachycardia. P waves during tachycardia exhibiting an axis different than expected for sinus tachycardia and a normal or short P–R interval is compatible with the diagnosis of atrial tachycardia. A similar electrocardiogram can be seen with the so-called atypical (fast-slow or slow-slow) variants of AV nodal reentrant tachycardia and in persistent junctional reciprocating tachycardia (atrioventricular reentrant tachycardia using a slow conducting retrograde accessory pathway). A long P–R interval during tachycardia does not exclude an atrial tachycardia since atrial tachycardias conducted either over the slow pathway of the AV node, a diseased AV node, or an AV node slowed by drugs or increased vagal tone could result in a prolonged P–R interval during the arrhythmia. Paroxysmal atrial tachycardias are frequently elusive and difficult to document in a 12-lead electrocardiogram (Figure 1, Figure 3). Heart rhythm monitors: when a 12-lead electrocardiogram has not been recorded during tachycardia, the correlation of the patient’s symptoms with an arrhythmia must be made either with an in-hospital or ambulatory heart rhythm monitor. Ambulatory heart rhythm monitors include the traditional Holter monitor (usually limited to 24 to 48 hours of heart rhythm recording), portable continuous tele-monitors with ongoing heart rhythm transmission and recording, snapshot event recorders and event loop recorders, which could be portable or implantable. Frequency of symptoms, availability and patient/physician preference determines the choice of monitoring modality. These monitors could be single or multichannel and the recording of a sudden onset and offset of tachycardia with P wave morphology different than the one in sinus rhythm is suggestive of the diagnosis of supraventricular tachycardia. Electrophysiology study: the definitive diagnosis confirmation of an atrial tachycardia is best done in the electrophysiology laboratory. Intracardiac recordings and response to pacing maneuvers during tachycardia, as well as characteristics of tachycardia induction and activation maps, provide a definitive diagnosis of the mechanism of the tachycardia as well as an opportunity for definitive treatment through ablation. Advanced nonfluoroscopic three-dimensional mapping systems with computerized representation of the cardiac chambers where activation and signal characteristics can be displayed are available today to facilitate the diagnosis and therapy in the electrophysiology laboratory (Figure 2, Figure 4). Echocardiogram: indicated for the evaluation of potential or known underlying structural heart disease. The treatment of atrial tachycardias is focused on rhythm control and prevention of arrhythmia recurrence. Persistent tachycardias with hemodynamic compromise must be terminated emergently with electrical cardioversion. In the case of persistent focal atrial tachycardias and frequently in the management of macro-reentrant atrial tachycardias that resemble atrial flutter, the initial treatment may be aimed at rate control but more definitive treatment and resolution of the clinical symptoms usually requires restoration of normal sinus rhythm. The treatment could be pharmacologic or catheter based ablation. The patient should participate in the decision of which strategy to use as long as he or she can be a candidate for both. Patients with multiple comorbidities and contraindications for antiarrhythmic drugs should be treated with an invasive strategy, and patients with vascular access problems, contraindication to sedatives, and inability to safely withstand an ablation procedure should be treated with a conservative approach until they can be candidates for an invasive procedure. Pharmacologic therapy includes beta-blockers, calcium channel blockers, and antiarrhythmic drugs. Among antiarrhythmic drugs, class IC seem to be particularly effective with focal atrial tachycardias, but its use is limited to patients without myocardial scar and by extension, patients without coronary artery disease or significant structural heart disease. Sotalol, amiodarone, and dofetilide can be used as well. Dofetilide have been reported to be more effective in the management of atrial flutter and hence suspected to be more effective for macro-reentrant atrial tachycardias but limited by the QTc and creatinine clearance. Treatment through catheter based ablation is the preferred approach given its high success rate and potential freedom of arrhythmias without the need for continued drug therapy. The catheter ablation procedure is a percutaneous invasive intervention that requires vascular access through the femoral and sometimes subclavian veins. Catheters with electrodes are advanced into the heart under fluoroscopy and used for recording intracardiac signals and stimulating the heart. With the use of pacing maneuvers and pharmacologic interventions, the tachycardia can be induced (if not present at the time of the procedure) and characterized establishing a definitive diagnosis of atrial tachycardia and differentiating between focal and macro-reentrant. Radiofrequency energy or cryoablation are delivered through a catheter tip to eliminate the tissue where a focal atrial tachycardia originates or tissue that is critical for the reentry circuit (Figure 4, Figure 5). The procedure may require access to the left atrium performing a transseptal puncture or retrograde through the aorta. The contemporary approach to catheter-based ablation using nonfluoroscopic mapping systems, intracardiac echocardiography, and irrigated tip catheters allows for improved safety during the procedure. Success rate is close to 90% and recurrence rates are low. Macro-reentrant atrial tachycardias associated to extensive atrial fibrosis in patients with valvular heart disease, multiple extensive atrial ablations, or surgeries may be particularly difficult to control both with drugs or ablation and eventually may require an aggressive rate-control strategy with AV junction ablation and the implantation of a permanent pacemaker. A. Immediate management. During tachycardia in the acute setting hemodynamic support and restoration of adequate perfusion should remain the priority as mandated by the ACLS protocols. When there is evidence of hemodynamic compromise restoration of normal sinus rhythm and perfusion should be achieved immediately through CPR, electrical cardioversion, and vasoactive drugs. Intravenous hydration is recommended during tachycardia in the absence of signs and symptoms of congestive heart failure. If the blood pressure is not critically compromised, a bolus of intravenous adenosine can help in establishing the diagnosis or terminating the tachycardia. Vagal maneuvers including carotid sinus massage are also helpful either in terminating a supraventricular tachycardia or causing transient AV nodal block to allow the diagnosis of an atrial tachycardia when the atrial tachycardia continues despite conduction block to the ventricle. Antiarrhythmic drugs in the acute setting can also be used for pharmacologic conversion of an incessant tachycardia or to achieve rate control and symptomatic relief. Beta-blockers are the first-line of therapy, particularly if there blood pressure is stable. Other antiarrhythmic drugs include class Ic antiarrhythmics given orally (flecainide 200 to 300 mg, propafenone 600 mg), intravenous amiodarone and ibutilide. Intravenous calcium channel blockers or digoxin can be attempted for rate control, especially in faster tachycardias (macro-reentrant atrial tachycardias that resemble atrial flutter) in which AV nodal blocking agents could be expected to slow ventricular response. Procainamide, disopyramide, and quinidine are rarely used. Class Ic antiarrhythmic drugs (flecainide, propafenone, procainamide, and disopyramide) should be only given to patients with macro-reentrant atrial tachycardias and atrial flutter when administered together with AV nodal blocking agents to prevent slowing of the tachycardia cycle length and 1:1 AV conduction that could lead to hemodynamic instability. B. Physical Examination Tips to Guide Management. Blood pressure and pulse rate should be monitored during the treatment of the arrhythmia in the acute setting, although a heart rhythm monitor or electrocardiogram is of most value. When the tachycardia is accompanied by signs of heart failure, dizziness, or syncope, monitoring respiratory rate, pulse oximetry, body weight, lung sounds, jugular venous distention, peripheral edema, urinary output, and neurologic and mental status is warranted to prevent worsening of those conditions that may be a consequence of coexisting with the tachycardia. C. Laboratory Tests to Monitor Response To, and Adjustments in, Management Continuous heart rhythm monitoring and 12-lead electrocardiograms to document and analyze rhythm changes is of most value to monitor the response to therapy. Laboratory tests are usually not needed unless used to guide response to concomitant therapy for other associated conditions or complications of the arrhythmia: Chest radiograph, BNP, electrolytes, and liver function for heart failure. Liver function and renal function in case of hypotension. Thyroid function in the setting of thyroid disease. Renal function for adjustment or dosing of drugs, such as sotalol and dofetilide. D. Long-term Management. Catheter ablation is the therapeutic strategy most likely to provide long-term elimination of the arrhythmia. Antiarrhythmic drugs can be used for maintenance of sinus rhythm. Patients may respond to beta-blockers and calcium channel blockers but commonly require the addition of a more potent antiarrhythmic drug, such as sodium (flecainide, propafenone) or potassium (sotalol, amiodarone, dronedarone, dofetilide) channel blockers. Recurrences are possible with both modalities of treatment. E. Common Pitfalls and Side-Effects of Management Lack of recognition of the arrhythmia. Incessant atrial tachycardias may be misinterpreted as sinus tachycardia. When incessant atrial tachycardias are not recognized could progress to the development of tachycardia-induced cardiomyopathy and heart failure. Paroxysmal atrial tachycardias may require the use of prolonged ambulatory heart rhythm monitors since recurrence may be sporadic and missed in 24 to 48 hours of ambulatory recordings. Prevention of recurrence. After termination of the tachycardia in the acute setting, the patient must be educated regarding the recurrent nature of the arrhythmia, and therapy to prevent recurrence should be considered. Patients discharged without a specific therapy or plan for management of recurrences are likely to be readmitted or present to the emergency department or outpatient clinic with recurrence of tachycardia and its symptoms. Associated conditions. The evaluation and management of atrial tachycardias should include the prompt diagnosis and treatment of associated conditions that could predispose, aggravate, or coexist with the tachycardia. Screening for thyroid, pulmonary, and ischemic heart disease should be considered. Substance abuse, prescription, and over-the-counter drugs (stimulants and decongestants) could be predisposing or aggravating factors. Avoidance of identified triggers if present, such as caffeinated drinks or exercise, should be recommended until an effective therapy is instituted. Side effects of drug therapy. The side effects are characteristic of each group or agent. The most common side effects include: Beta-blockers: bradycardia, fatigue, erectile dysfunction, depression, hypotension, and bronchospasm Calcium channel blockers: lower extremity edema, hypotension, and constipation Flecainide: nightmares, headaches, paresthesia, and proarrhythmia Propafenone: bradycardia, and proarrhythmia. Sotalol: bradycardia, Q–T prolongation, and proarrhythmia Dofetilide: Q–T prolongation and proarrhythmia Amiodarone: bradycardia and nausea, short and long term there is risk of neuropathy, thyroid, lung, and liver toxicity Dronedarone: bradycardia, nausea, and diarrhea Risks of catheter based ablation. The electrophysiology study and catheter-based ablation procedure is an invasive percutaneous procedure that involves vascular access and frequently access to the left atrium. In order to make the diagnosis and deliver the treatment, the tachycardia needs to be present or be inducible. If the arrhythmia cannot be induced during the procedure, neither the diagnostic confirmation nor an ablation can be performed. Ablation may be required close to vital structures such as coronary arteries, atrioventricular node, sinus node, phrenic nerve, etc. Complications of the procedure include radiation exposure, bleeding, vascular damage, hematoma, arteriovenous fistula, venous thrombosis, pneumothorax, stroke, heart perforation, cardiac tamponade, atrioventricular nodal block, phrenic nerve paralysis, myocardial infarction, and death. Acutely, a rapid bolus of IV adenosine (6, 12, or 18 mg) can be used for both the differential diagnosis or eventually termination of atrial tachycardias. Only a subset of atrial tachycardias are adenosine sensitive. Intravenous boluses or continuous infusions of beta-blockers, calcium channel blockers, procainamide or amiodarone may help terminate and/or prevent recurrence of the tachycardia. One milligram of IV Ibutilide administered over 10 minutes in a monitor setting with capability of adult cardiac life support may also be used for the acute termination and suppression of the arrhythmia. The drugs used in the long-term management of atrial tachycardias include beta-blockers, calcium channel blockers, sodium (class IC), and potassium (class III) channel blockers. Most commonly used oral beta-blockers: Atenolol 12.5-200 mg daily Metoprolol 12.5-100 mg b.i.d. Metoprolol extended release 12.5-200 mg daily Bisoprolol 5-20 mg daily Carvedilol 3.125-25 mg b.i.d Carvedilol controlled release 10-80 mg daily Calcium channel blockers Most commonly used oral calcium channel blockers: Diltiazem 30-120 mg q.i.d. Diltiazem sustained/extended release 120-420 mg daily Verapamil 40-120 mg t.i.d. Verapamil sustained/extended release 100-360 mg daily Class IC antiarrhythmic drugs (sodium channel blockers) Class IC antiarrhythmic drugs are contraindicated in patients with myocardial scar, left ventricular hypertrophy, structural heart disease ,and coronary artery disease. Flecainide 50-150 mg b.i.d. Propafenone 150-300 mg t.i.d. Propafenone sustained release 225-425 mg b.i.d. Class III antiarrhythmic drugs (potassium channel blockers) Sotalol 40-160 mg b.i.d.—monitoring of the QTc interval during loading for five half-lives (3 days) is recommended Dofetilide 125-500 mcg b.i.d.—restricted to certified prescribers, requires renal dose adjustment and monitoring of the QTc interval during loading for five half-lives (3 days) Amiodarone 200-800 mg daily—not recommended for long-term therapy due to potential side effects Dronedarone 400 mg b.i.d.—may be used off label, but no published literature is available regarding the use of dronedarone in the treatment of supraventricular tachycardias IV. Management with Co-Morbidities Thyroid disease. Patients with known thyroid disease on supplementation therapy or uncontrolled hyperthyroidisms may have a higher incidence of atrial tachycardias. Control of hyperthyroidism, including surgical removal or radioactive ablation of the thyroid gland may be necessary. In patients on supplementation therapy, occasionally a reduction on the supplementation dose could help present recurrences. Catheter ablation, if not contraindicated, may be an alternative to allow less aggressive treatment for the thyroid disease. Coronary artery disease. Patients with coronary artery disease require aggressive treatment of supraventricular tachycardias to prevent angina, heart failure, or myocardial infarction from demand ischemia during tachycardia. Beta-blockers are indicated for coronary artery disease, especially after myocardial infarction. Class IC antiarrhythmic drugs should be avoided in coronary artery disease and are contraindicated in patients with prior to myocardial infarction. Heart failure. Both systolic and diastolic heart failure could become decompensated during tachycardia. Beta-blockers are indicated in the treatment of heart failure. As previously discussed ,systolic heart failure could result from incessant atrial tachycardias. In the case of tachycardia-induced cardiomyopathy, control of the tachycardia frequently results in normalization of left ventricular function. Class IC antiarrhythmic drugs and dronedarone are in general contraindicated in patients with heart failure. Congenital heart disease. Atrial tachycardias are rather frequent among patients with history of congenital heart disease. Usually, tachycardia is not well tolerated due to the development of heart failure symptoms or hemodynamic compromise. Atrial tachycardias are commonly macro-reentrant involving scars from previous corrective surgery. Both antiarrhythmic drugs and catheter ablation may be effective in suppressing the arrhythmia. Multiple atrial tachycardias are common in this patient population. Hypertrophic cardiomyopathy. Atrial tachycardias are poorly tolerated in patients with hypertrophic cardiomyopathy due to the severe degree of diastolic heart failure. Tachycardia may result in heart failure decompensation with pulmonary edema. Multiple atrial tachycardias are also frequent due to severe atrial dilatation, which is frequent in these patients. Class IC antiarrhythmic drugs and sotalol are contraindicated. Dronedarone should be avoided since it is contraindicated in patients with symptomatic heart failure and that is how these patients usually present with atrial tachycardias. Chronic obstructive pulmonary disease. Atrial tachycardias are common and frequently poorly tolerated in patients with chronic lung disease. Beta-blockers may worsen a reactive airway disease component when present. Amiodarone should be avoided since pulmonary toxicity may be fatal in patients with baseline poor lung function. Renal insufficiency. Sotalol and dofetilide should be avoided in renal insufficiency. If no safe alternative is available or possible, the patient needs to be carefully monitored and the dose adjusted according to the creatinine clearance. Dofetilide is contraindicated in patients with creatinine clearance <20 ml/min. Deep venous thrombosis. Thrombosis of both femoral veins or the inferior vena cava prevents vascular access for an electrophysiology study and catheter ablation. Nevertheless, occlusion of the inferior vena cava or bilateral femoral veins is extremely rare. The presence of an inferior vena cava (IVC) filter does not prevent the advance of catheters, which should be avoided only after a recent IVC filter implant to prevent its dislodgement. Pharmacologic suppression should be the treatment of choice for patients with recently implanted IVC filters or acute/subacute femoral vein thrombosis. V. Patient Safety and Quality Measures A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. The best measure to prevent hospital readmissions and recurrence of atrial tachycardias is an effective treatment. The highest probability of achieving elimination atrial tachycardias is with catheter-based ablation. Class IC and III antiarrhythmic drugs are usually more effective than beta-blockers and calcium channel blockers. Nevertheless, recurrences are possible either after ablation or on pharmacologic therapy. Patient education in regards to symptoms recognition and self-determination of heart rate is of great value. A plan for management of recurrences should be in place including alternative dosing of ongoing medication or an additional prescription for rate control agents that could be used as needed in case of recurrence. The patient should be able to identify alarm signs and symptoms that require emergency attention, such as syncope, near syncope, decreased urinary output, severe dyspnea, or chest pain. Understanding that recurrences usually can be managed in the outpatient setting by adjusting or changing the medical regimen or scheduling an elective ablation procedure may help in lowering the patient’s anxiety and the likelihood of seeking unnecessary emergency care. It is crucial as well to provide adequate access and timely response by a medical provider in capacity to help manage persistent or frequently recurrent tachycardias in the outpatient setting. B. What's the Evidence for specific management and treatment recommendations? Blomstrðm-Lundqvist, C. “ACC/AHA/ESC guidelines for the management of patients with supraventricular arrhythmias–executive summary. a report of the American College of Cardiology/American Heart Association task force on practice guidelines and the European Society of Cardiology committee for practice guidelines (writing committee to develop guidelines for the management of patients with supraventricular arrhythmias) developed in collaboration with NASPE-Heart Rhythm Society”. J Am Coll Cardio. vol. 42. 2003. pp. 1493-1531. (Evidence-based guidelines for the management of patients with supraventricular arrhythmias, including specific recommendations and support references for the management of atrial tachycardias in the acute setting, prevention of recurrence, and long-term management. Includes tables with levels of evidence and references to support the recommendations when available.) Kumar, UN, Rao, RK, Scheinman, MM. “The 12-lead electrocardiogram in supraventricular tachycardia”. Cardiol Clin. vol. 24. 2006. pp. 427-37. (The authors review the characteristic electrocardiographic findings of the supraventricular tachycardias included in the differential diagnosis of atrial tachycardias.) Kistler, PM, Roberts-Thomson, KC, Haqqani, HM. “P-wave morphology in focal atrial tachycardia: development of an algorithm to predict the anatomic site of origin”. J Am Coll Cardiol. vol. 48. 2006. pp. 1010-7. (A comprehensive approach for the electrocardiographic diagnosis and classification of focal atrial tachycardias is presented. Clues on the origin of the atrial tachycardia can be found in the surface electrocardiogram.) Roberts-Thomson, KC, Kistler, PM, Kalman, JM. “Focal atrial tachycardia I: clinical features, diagnosis, mechanisms, and anatomic location”. Pacing Clin Electrophysiol. vol. 29. 2006. pp. 643-52. (Excellent, very complete review to expand on focal atrial tachycardias. Good source for references.) Roberts-Thomson, KC, Kistler, PM, Kalman, JM. “Focal atrial tachycardia II: management”. Pacing Clin Electrophysiol.. vol. 29. 2006. pp. 769-78. (A well written review on the management of focal atrial tachycardias. Excellent source of references.) Medi, C, Kalman, J, Haqqani, H. “Tachycardia-mediated cardiomyopathy secondary to focal atrial tachycardia”. J Am Coll Cardiol. vol. 53. 2009. pp. 1791-7. (The authors identified a 10% incidence of tachycardia-induced cardiomyopathy among 345 patients with focal atrial tachycardias. The article describes the associated clinical risk factors and recovery of cardiac function in 97% of patients three months after successful catheter ablation. This manuscript helps understand the risk and management of tachycardia-induced cardiomyopathy, which also applies to macro-reentrant atrial tachycardias and other clinical tachycardias.) Spector, P, Reynolds, MR, Calkins, H. “Meta-analysis of ablation of atrial flutter and supraventricular tachycardia”. Am J Cardiol. vol. 104. 2009. pp. 671-7. (In this well conducted meta-analysis 18 studies on ablation of atrial flutter (macro-reentrant atrial tachycardia) were evaluated. A high success rate (91.7%), and low risk of mortality (0.6%) and adverse events (0.5%) were identified.) Feld, GK, Chen, PS, Nicod, P. “Possible atrial proarrhythmic affects of class IC antiarrhythmic drugs”. Am J Cardiol. vol. 66. 1990. pp. 378-83. (The potential risk of facilitating 1:1 AV nodal conduction and the risk of proarrhythmia with the use of class IC antiarrhythmic drugs is expanded in this article.) Morady, F, Oral, H, Chugh, A. “Diagnosis and ablation of atypical atrial tachycardia and flutter complicating atrial fibrillation ablation”. Heart Rhythm. vol. 6. 2009. pp. S29-32. (A review that explores the mechanism, diagnosis, and management of a macro-reentrant atrial tachycardia of increasing frequency. With the improved life expectancy of patients with valvular and coronary disease, catheter ablation of atrial fibrillation and surgical Maze procedures have become more frequent, leading to higher incidence of postprocedure macro-reentrant atrial tachycardias, which are usually incessant.) C. DRG Codes and Expected Length of Stay. The in-hospital treatment of atrial tachycardias are classified into a diagnosis-related group (DRG) depending on the clinical presentation and admission diagnosis/purpose. Length of stay varies depending on the clinical presentation and comorbidities, as well as reason for admission. Different between an elective admission for a procedure versus new presentation, with ongoing need for management and diagnostic testing. Length of stay could be expected to be between 23 hours to 4 days . According to the 2011 inpatient prospective payment system final rule, the DRG codes that correspond to an admission for atrial tachycardia are: 308 Cardiac Arrhythmia and Conduction Disorder with Major Complications/Comorbidities 309 Cardiac Arrhythmia and Conduction Disorder with Complications/Comorbidities 310 Cardiac Arrhythmia and Conduction Disorder without Major Complications/Comorbidities When the patient is admitted for catheter-based ablation, the following DRG codes apply: 250 Percutaneous Cardiovascular Procedures without Coronary Artery Stent or Acute Myocardial Infarction with Major Complications/Comorbidities 251 Percutaneous Cardiovascular Procedures without Coronary Artery Stent or Acute Myocardial Infarction without Major Complications/Comorbidities If the admission diagnosis is syncope, chest pain, or heart failure, the corresponding codes are: 291 Heart Failure and Shock with Major Complications/Comorbidities 292 Heart Failure and Shock with Complications/Comorbidities 293 Heart Failure and Shock without Major Complications/Comorbidities 311 Angina Pectoris 312 Syncope and Collapse 313 Chest Pain Atrial tachycardia may be present as a comorbidity rather than the principal diagnosis constituting a complication/comorbidity. The corresponding complication/comorbidity (CC) code could be: 427.0 Paroxysmal Supraventricular Tachycardia 427.32 Atrial Flutter Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM. - I. Atrial Tachycardia: What every physician needs to know. - II. Diagnostic Confirmation: Are you sure your patient has an Atrial Tachycardia? - A. History Part I: Pattern Recognition: - B. History Part 2: Prevalence: - C. History Part 3: Competing diagnoses that can mimic an Atrial Tachycardia - D. Physical Examination Findings. - E. What diagnostic tests should be performed? - 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? - 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? - III. Management. - A. Immediate management. - B. Physical Examination Tips to Guide Management. - C. Laboratory Tests to Monitor Response To, and Adjustments in, Management - D. Long-term Management. - E. Common Pitfalls and Side-Effects of Management - IV. Management with Co-Morbidities - V. Patient Safety and Quality Measures - A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. - B. What's the Evidence for specific management and treatment recommendations? - C. DRG Codes and Expected Length of Stay.	2	26	0	0	0	1	0.790127	1	10,153
- Disseminated intravascular coagulation Disseminated intravascular coagulation or Disseminated intravascular coagulopathy Classification and external resources Micrograph showing a thrombotic microangiopathy, as may be seen in DIC. Kidney biopsy. PAS stain. ICD-10 D65 ICD-9 286.6 DiseasesDB 3765 eMedicine med/577 emerg/150 MeSH D004211 Disseminated intravascular coagulation (DIC), also known as disseminated intravascular coagulopathy or consumptive coagulopathy, is a pathological activation of coagulation (blood clotting) mechanisms that happens in response to a variety of diseases. DIC leads to the formation of small blood clots inside the blood vessels throughout the body. As the small clots consume coagulation proteins and platelets, normal coagulation is disrupted and abnormal bleeding occurs from the skin (e.g. from sites where blood samples were taken), the gastrointestinal tract, the respiratory tract and surgical wounds. The small clots also disrupt normal blood flow to organs (such as the kidneys), which may malfunction as a result. DIC can occur acutely but also on a slower, chronic basis, depending on the underlying problem. It is common in the critically ill, and may participate in the development of multiple organ failure, which may lead to death. Under homeostatic conditions, the body is maintained in a finely tuned balance of coagulation and fibrinolysis. The activation of the coagulation cascade yields thrombin that converts fibrinogen to fibrin; the stable fibrin clot being the final product of hemostasis. The fibrinolytic system then functions to break down fibrinogen and fibrin. Activation of the fibrinolytic system generates plasmin (in the presence of thrombin), which is responsible for the lysis of fibrin clots. The breakdown of fibrinogen and fibrin results in polypeptides called fibrin degradation products (FDPs) or fibrin split products (FSPs). In a state of homeostasis, the presence of plasmin is critical, as it is the central proteolytic enzyme of coagulation and is also necessary for the breakdown of clots, or fibrinolysis. In DIC, the processes of coagulation and fibrinolysis are dysregulated, and the result is widespread clotting with resultant bleeding. Regardless of the triggering event of DIC, once initiated, the pathophysiology of DIC is similar in all conditions. One critical mediator of DIC is the release of a transmembrane glycoprotein called tissue factor (TF). TF is present on the surface of many cell types (including endothelial cells, macrophages, and monocytes) and is not normally in contact with the general circulation, but is exposed to the circulation after vascular damage. For example, TF is released in response to exposure to cytokines (particularly interleukin 1), tumor necrosis factor, and endotoxin. This plays a major role in the development of DIC in septic conditions. TF is also abundant in tissues of the lungs, brain, and placenta. This helps to explain why DIC readily develops in patients with extensive trauma. Upon activation, TF binds with coagulation factors that then triggers the extrinsic pathway (via Factor VII) which subsequently triggers the intrinsic pathway (XII to XI to IX) of coagulation. The release of endotoxin is the mechanism by which Gram-negative sepsis provokes DIC. In acute promyelocytic leukemia, treatment causes the destruction of leukemic granulocyte precursors, resulting in the release of large amounts of proteolytic enzymes from their storage granules, causing microvascular damage. Other malignancies may enhance the expression of various oncogenes that result in the release of TF and plasminogen activator inhibitor-1 (PAI-1), which prevents fibrinolysis. Excess circulating thrombin results from the excess activation of the coagulation cascade. The excess thrombin cleaves fibrinogen, which ultimately leaves behind multiple fibrin clots in the circulation. These excess clots trap platelets to become larger clots, which leads to microvascular and macrovascular thrombosis. This lodging of clots in the microcirculation, in the large vessels, and in the organs is what leads to the ischemia, impaired organ perfusion, and end-organ damage that occurs with DIC. Coagulation inhibitors are also consumed in this process. Decreased inhibitor levels will permit more clotting so that a feedback system develops in which increased clotting leads to more clotting. At the same time, thrombocytopenia occurs and this has been attributed to the entrapment and consumption of platelets. Clotting factors are consumed in the development of multiple clots, which contributes to the bleeding seen with DIC. Simultaneously, excess circulating thrombin assists in the conversion of plasminogen to plasmin, resulting in fibrinolysis. The breakdown of clots results in excess amounts of FDPs, which have powerful anticoagulant properties, contributing to hemorrhage. The excess plasmin also activates the complement and kinin systems. Activation of these systems leads to many of the clinical symptoms that patients experiencing DIC exhibit, such as shock, hypotension, and increased vascular permeability. The acute form of DIC is considered an extreme expression of the intravascular coagulation process with a complete breakdown of the normal homeostatic boundaries. DIC is associated with a poor prognosis and a high mortality rate. There has been a recent challenge however to the basic assumptions and interpretations of the pathophysiology of DIC. A study of sepsis and DIC in animal models has shown that a highly-expressed receptor on the surface of hepatocytes, termed the Ashwell-Morell receptor, is responsible for thrombocytopenia in bacteremia and sepsis due to streptococcal pneumoniae (SPN) and possibly other pathogens. The thrombocytopenia observed in SPN sepsis was not due to increased consumption of coagulation factors such as platelets, but instead was the result of this receptor's activity enabling hepatocytes to ingest and rapidly clear platelets from circulation. By removing pro-thrombotic components before they participate in the coagulopathy of DIC, the Ashwell-Morell receptor lessens the severity of DIC, reducing thrombosis and tissue necrosis, and promoting survival. The hemorrhage observed in DIC and among some tissues lacking this receptor may thereby be secondary to increased thrombosis with loss of the mechanical vascular barrier. This discovery has possible significant clinical implications in devising new approaches to reducing the pathophysiology of DIC. - Cancers of lung, pancreas, prostate and stomach, as well as acute myeloid leukemia (particularly APML) - Obstetric: abruptio placentae, pre-eclampsia, amniotic fluid embolism - Massive tissue injury: Trauma, burns, extensive surgery - Infections: Gram-negative sepsis, Neisseria meningitidis, Streptococcus pneumoniae, malaria, histoplasmosis, aspergillosis, Rocky mountain spotted fever - Miscellaneous: Liver disease, snake bite, giant hemangioma, shock, heat stroke, vasculitis, aortic aneurysm, Serotonin syndrome - Viral: Arenaviruses causing Argentine hemorrhagic fever or Bolivian Hemorrhagic Fever Signs and symptoms The affected person is often acutely ill and shocked with widespread haemorrhage (common bleeding sites are mouth, nose and venepuncture sites), extensive bruising, renal failure and gangrene. The onset of DIC can be fulminant, as in endotoxic shock or amnioitic fluid embolism, or it may be insidious and chronic, as in cases of carcinomatosis. Diagnosis is usually suggested by following conditions: - Severe cases with hemorrhage: The PT and APTT are usually very prolonged and the fibrinogen level markedly reduced. High levels of fibrin degradation products, including D-dimer, are found owing to the intense fibrinolytic activity stimulated by the presence of fibrin in the circulation. There is severe thrombocytopenia. The blood film may show fragmented red blood cells (schistocytes). - Mild cases without bleeding: There is increased synthesis of coagulation factors and platelets. PT, APTT, and platelet counts are normal. fibrin degradation products are raised. Definitive diagnosis depends on the result of: - Prolongation of prothrombin time and activated partial thromboplastin time - A low fibrinogen concentration - Increased levels of fibrin degradation products Condition Prothrombin time Partial thromboplastin time Bleeding time Platelet count Vitamin K deficiency or warfarin prolonged prolonged unaffected unaffected Disseminated intravascular coagulation prolonged prolonged prolonged decreased von Willebrand disease unaffected prolonged prolonged unaffected Haemophilia unaffected prolonged unaffected unaffected Aspirin unaffected unaffected prolonged unaffected Thrombocytopenia unaffected unaffected prolonged decreased Early Liver failure prolonged unaffected unaffected unaffected End-stage Liver failure prolonged prolonged prolonged decreased Uremia unaffected unaffected prolonged unaffected Congenital afibrinogenemia prolonged prolonged prolonged unaffected Factor V deficiency prolonged prolonged unaffected unaffected Factor X deficiency as seen in amyloid purpura prolonged prolonged unaffected unaffected Glanzmann's thrombasthenia unaffected unaffected prolonged unaffected Bernard-Soulier syndrome unaffected unaffected prolonged unaffected The only effective treatment is the reversal of the underlying cause. Anticoagulants are given exceedingly rarely when thrombus formation is likely to lead to imminent death (such as in coronary artery thrombosis or cerebrovascular thrombosis). Platelets may be transfused if counts are less than 5,000-10,000/mm3 and massive hemorrhage is occurring, and fresh frozen plasma may be administered in an attempt to replenish coagulation factors and anti-thrombotic factors, although these are only temporizing measures and may result in the increased development of thrombosis. DIC results in lower fibrinogen levels (as it has all been converted to fibrin), and this can be tested for in the hospital lab. A more specific test is for "fibrin split products" (FSPs) or "fibrin degradation products" (FDPs) which are produced when fibrin undergoes degradation when blood clots are dissolved by fibrinolysis. In some situations, infusion with antithrombin may be necessary. Prognosis varies depending on the underlying disorder, and the extent of the intravascular thrombosis (clotting). The prognosis for those with DIC, regardless of cause, is often grim: Between 10% and 50% of patients will die. DIC with sepsis (infection) has a significantly higher rate of death than DIC associated with trauma. - ^ Churchill Livingstone Pocket Medical Dictionary 14th Edition. - ^ ISBN 0-443-07036-9 Davidson's Principles and Practice of Medicine 19th Edition. Churchill Livingstone. Page 200 - ^ a b c Robbins, Stanley L.; Cotran, Ramzi S.; Kumar, Vinay; Collins, Tucker (1999). Robbins' Pathologic Basis of Disease (6 ed.). Philadelphia: Saunders. ISBN 0-7216-7335-X. - ^ a b Davidson, Stanley; Haslett, C. (2002). Davidson's Principles and Practice of Medicine (19 ed.). Edinburgh: Churchill Livingstone. ISBN 0-443-07036-9. - ^ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; & Mitchell, Richard N. (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 469-471 ISBN 978-1-4160-2973-1 - ^ Rak J, Yu JL, Luyendyk J, Mackman N (2006). "Oncogenes, trousseau syndrome, and cancer-related changes in the coagulome of mice and humans". Cancer Res. 66 (22): 10643–6. doi:10.1158/0008-5472.CAN-06-2350. PMID 17108099. http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=17108099. - ^ Grewal, P.K., Uchiyama, S., Ditto, D., Varki,N., Le, D.T., Nizet, V., and Marth, J.D. (2008). The Ashwell receptor mitigates the lethal coagulopathy of sepsis. Nat. Med., 14, 648-655. - ^ a b c Clark, Michael; Kumar, Parveen J. (1998). Clinical Medicine: A Textbook for Medical Students and Doctors (4 ed.). Philadelphia: W.B. Saunders. ISBN 0-7020-2458-9. - ^ Boyer EW, Shannon M (2005). "The serotonin syndrome". N. Engl. J. Med. 352 (11): 1112–20. doi:10.1056/NEJMra041867. PMID 15784664. - ^ Oxford Handbook of Clinical Medicine 6th Edition. Page 650 - ^ ISBN 0-443-07036-9 Davidson's Principles and Practice of Medicine 19th Edition. Churchill Livingstone. Page 953. - ^ Kaplan QBook - USMLE Step 1 - 5th edition - page 254 - ^ a b Becker, Joseph U and Charles R Wira. Disseminated intravascular coagulation at eMedicine, 10 September 2009 - ^ "Medical slang". RadsWiki. 25 July 2008. http://www.radswiki.net/main/index.php?title=Medical_slang. Retrieved 4 January 2010. - ^ Norman K (2004). "Alternative treatments for disseminated intravascular coagulation.". Drug News Perspect 17 (4): 243–50. doi:10.1358/dnp.2004.17.4.829051. PMID 15334173. Diseases of the skin and appendages by morphology GrowthsPigmentedDermal and PurpuraMacularthrombocytopenic purpura · actinic purpuraPapulardisseminated intravascular coagulation · vasculitisIndurated Pathology: hematology · hematologic diseases of RBCs and megakaryocytes / MEP (D50-69,74, 280-287) Red Wikimedia Foundation. 2010.	2	10	4	0	0	3	0.823639	7	3,232
A frequently asked question on internet websites is: “What can I do?! Co-worker + BO at work! Help!” (just to quote one common phrase). The usual answers to this would be “Buy him some soap!” or “Ask him if he has ever heard of washing!” photo above \| “Dude, take a shower! Ever heard of deodorants?!” What most people do not know: In many cases the ‘air polluters’ are innocent. They are exactly free of blame in the matter because their body odour has nothing to do with personal hygiene, changing clothes or deodorants. The causes of the malodor are neither controllable nor avoidable. The unknown illness: permanent body odour Bromhidrosis (greek: bromos_hidros; ∼ the buck’s stench_sweat), also known as consecutive body odour (b.o.), is a common phenomenon in postpubertal individuals. In many cases, bromhidrosis may become pathologic if it is particularly overpowering or if the Bromhidrosis significantly interferes with the lives of the affected individuals. Bromhidrosis is a chronic condition in which excessive odour, usually an unpleasant one, emanates from the skin. Bromhidrosis, determined either by apocrine gland or by eccrine gland secretion, can substantially impair a person’s quality of life. The WHO’s official ICD-10 classification code for Bromhidrosis is: - L75.0 → Bromhidrosis Body odour, med. Bromhidrosis: severe and permanent body odour; diagnosable pathological condition; classified and registered by the WHO Symptoms: abnormal apocrine or eccrine odour, emanated from the skin, mostly from armpits, genitals, inner tights and scalp Side- and after-effects: psychological distress; social isolation; contaminated clothes ➊ special disinfectant soaps and washing foams special diet; abstinence (alcohol, nicotine, caffeine, drugs); medications Competent medical experts: dermatologist; internist; psychotherapist Online support and help: Excessive secretion from the sweat glands that becomes malodorous on bacterial breakdown is the predominant cause of Bromhidrosis. There are two types of human sweat glands: - Eccrine glands are distributed all over the skin. They are responsible for bodily thermoregulation by means of sweat production. They secrete large amounts of salty fluid, which is odourless upon reaching the skin surface. - Apocrine glands have a limited distribution involving the axilla, genital skin, scalp and breasts. Apocrine glands have no thermoregulatory role but are responsible for characteristic pheromonal odours. They secrete a small amount of oily fluid, which is rather odourless upon reaching the skin surface (most pheromones are not noticeable for human noses). fig. above \| The apocrine sweat gland is attached to the hair follicle. Its straight duct releases the apocrine secretion directly into the channel of the hair shaft. In contrast to this, a eccrine sweat gland secrets its salty fluid directly onto the skin’s surface. Apocrine body odour: Apocrine bromhidrosis is the most prevalent form of bromhidrosis. Bacterial decomposition of apocrine secretion yields ammonia and short-chained acids (mostly butyric and formic acids) with their characteristic strong odours. The individual secretion of pheromones is important, too. Pheromones are the main cause of subliminal odours which other people will notice unknowingly, although they are a contributing factor to one’s subjective decision to find an individual person attractive or not. Eccrine body odour: In certain circumstances, eccrine secretion, which is typically odourless, assumes an offensive aroma and causes eccrine bromhidrosis. When eccrine sweat softens keratin, bacterial degradation of the keratin yields a foul smell. Ingestion of some foods, including garlic, onion, curry, alcohol, nicotine, caffeine, certain drugs (for example penicillin) and toxins may cause eccrine odours. Lastly, eccrine bromhidrosis may result from underlying problems: - metabolic dysfunction - renal dysfunction - liver failure - other endogenous dysfunctions Excessive sweating, known as Hyperhidrosis, may promote the spread of apocrine sweat and contribute further to bromhidrosis by creating a moist environment, one ripe for bacterial overgrowth. Researchs suggested that Bromhidrosis is also caused by a genetic trait. Most patients can be genetically predisposed to body odour, especially when there is a known family history of Hyperhidrosis and family members had a certain bacterial skin flora. Other causes may include the following: - Diabetes mellitus - Intertrigo (rash) - Trichomycosis axillaris (underarm infection of the hair shafts) - Erythrasma (superficial skin disease) - wrong clothing (non-breathable fabrics) Medical experts frequently recommend the following measures: - appropriate clothing: breathable fabrics of natural origin (cotton, leather etc.) - disciplined washing routine (see below) - daily usage of special washing foams (e.g. BromEX foamer) or soaps - daily usage of prescription strength antiperspirants if Hyperhidrosis (excessive sweating) is the main cause (e.g. AHC20 sensitive) - regular shaving or trimming of bodily hair (e.g. armpits) - abstinence (no garlic and hot spices, no alcohol, caffeine, nicotine, drugs) - diet (natural and healthy food without artificial colourants, glutamates etc.) - general health check-up (in order to exclude hidden diseases as a cause) Interesting body odour facts: There is a significally difference between female and male odour. Males will develop much more odour, which has a masculine, strong characteristic because of the dominating testosterone hormone. Women emanate a more sweeter scent which is mostly affected by feminine estrogens. A Swiss study done by researchers in Geneva revealed that women’s body odour contained high levels of sulphur, which together with dermal bacteria produces the chemical compound thiol that has a smell of garlic or onion. According to the study, men’s sweat was found to contain more fatty acids, which when mixed with bacteria from the skin, produces a certain brute smell, resembling cheese. Excessive sweating is a more common problem for Caucasians and Africans, who tend to have more hair follicles to which the apocrine glands are attached. East Asian people appear to have less and smaller apocrine glands, which explains why they might not need to use deodorants as often as populations of Africa and Europe (see paragraph below). As a matter of fact the deodorant/antiperspirant market in Asia is much smaller than in the western world. Surely, though, there may be many exceptions since body odour is obviously influenced by many factors, for example by one’s personal diet (spicy asiatic food etc.). Earwax – the ethnic key to BO: Earwax comes in two types, wet and dry. The wet form predominates in Africa and Europe, where 97 percent or more of the people have it, and the dry form among East Asians, while populations of Southern and Central Asia are roughly half and half. By comparing the DNA of Japanese with each type, scientist Koh-Ichiro Yoshiura and his team from Nagasaki University were able to identify the gene that controls which type of earwax a person has. The Japanese team says that the earwax-affecting gene, known to geneticists as the ATP-binding cassette C11 gene, lies with three other genes in a long stretch of DNA that has very little variation from one person to another. This is why the Nagasaki scientists linked the earwax characteristics with sweating and individual body odour. They suggest that earwax type and armpit odour are correlated, since populations with dry earwax, such as those of East Asia, tend to sweat less and have little or no body odor, whereas the wet earwax populations of Africa and Europe sweat more and so may have greater body odor. Several Asian features, such as small nostrils and the fold of fat above the eyelid, are conjectured to be adaptations to the cold. Less sweating, the Japanese authors suggest, may be another adaptation to the cold climate in which the ancestors of East Asian peoples are thought to have lived. 1 Indicator for health problems: Some types of smell may reveal hidden health problems. It is known that if your sweat smells like peroxide (bleach), it may indicate a kidney or liver disease, while fruity body odour often points to diabetes. Also a rare genetic disorder, called TMAU-Syndrome (trimethylaminuria), makes a person produce constant fishy body odor. Unique as a fingerprint: Your body produces one of its kind odour – subtly, not noticeable for human noses – irrespective of outer influences. Individual odourtypes are genetically determined, they contain volatile organic compounds (VOCs) and pheromones that provide a certain smell, absolutely different from others, just like fingerprints or DNA samples. Scientists are working at creating special devices to identify individual odourtypes to find criminals, terrorists or missing children. photo above \| His mother may develop body odour because of her underarm sweating. His own infant apocrine sweat glands will not start to work before adolescence (see paragraph below). Childrens and BO: Smelly feet or armpits are usually not a problem for kids. Healthy children will not develop any unpleasant sweat odour until they enter adolescence between the ages 8 to 14 years. During puberty, the androgen hormones trigger increased sweating and thereby development of body odour. The Olf – the scale of smell: The Olf (latin olfactus; ∼ sense of smell) is used to measure the strength of a pollution source that can be inhaled and smelt by human nose. It was introduced by Danish professor P. Ole Fanger in 1988. One Olf is the sensory pollution strength from a standard person defined as “an average adult working in an office or similar non–industrial workplace, sedentary and in thermal comfort, with a hygienic standard equivalent of 0.7 bath per day and whose skin has a total area of 1.8 square metres“. It was defined to quantify the strength of pollution sources which can be perceived by humans. Nowadays, the Olf scale is used to rate smellable emissions from materials, for example at car design centers, where fabrics with a pleasant scent are designed (e.g. dashboard or seat coatings). To this day, there are no technical devices for the Olf measurement. Qualified testers still use their sensitive noses. Body odours of all kinds could also be measured in Olf. It is foreseeable that the WHO will use the Olf scale for classification and diagnosis of Bromhidrosis in near future. photo above \| Professor Povle Ole Fanger (* 16. Juli 1934; † 20. September 2006) at Syracuse University, NY. In 1988 he introduced the Olf scale for quantification of smellable pollution. Twin + twin = malodour² In a Swiss study (Kuhn F. et al. 2005), the aim was to use a group of qualified human ‘sniffers’ to match axillary odour samples from monozygotic (identical) twins who did not co-habit (so as to avoid cohabiting factors being relevant). The research showed that identical twins odour samples were matched by the human sniffers more often than chance would dictate. Dizygotic (non-identical) twins were also more often matched, but not as often as identical twins. Scientist regard this as a proof that body odour is sort of “blood-borne” and hereditary. Certain genes may play a significant role as a congenital disposition to individual malodour. Body odour gossip Library bans: No books for stinkers! At the Library of San Luis Obispo (California, USA) the staff has the right to ban people with unpleasant body odor from their library rooms. This right has been part of the official library rule since 1994. In 2005, though, the San Luis Obispo County council enacted an even stricter Library Rules of Conduct and Exclusion Process. By this, police officers also got the legitimation to ban stinkers from a total of 14 county libraries (plus one book bus). In 1989 Mr. Richard Kreimer from Morristown, New Jersey (USA) won a relevant case at the Federal Court. He had conducted this lawsuit after he was banned from an public library because of his body odour. The court mostly followed his argument, that in terms of human rights and honour it is highly discriminating to ban someone from a public place for such questionable reasons. However, the court of appeal refused to countersign this. In 1992 it overturned the 3 year old judicial decision. In the opinion of the appellate court, visitors and employees do not have to endure severe body odour emitted from a singular person. The library staff has officially the right to to banish such persons from their rooms. In Wigston, Leicestershire (UK), 27 year old worker Stuart Penman was banned from the public Wigston Library for 6 month. Members of the Leicestershire County Council tried to avoid this ban previously. They interwiewed Penman privately but he refused to cooperate. After many complaints from other visitors, Margaret Bellamy, head of library services for the council finally decided to enforce the ban. “We’ve had people leaving and saying it’s because of the smell. I feel very sorry about the whole situation and it’s not a decision we took lightly but we’ve done all we can to help and it’s still not getting any better. [...] When people were refusing to come into the library we felt we had no other option.” she said. Penman, who uses the library daily for internet surfing, said he was a victim of circumstances. “Some of the women that work there just tell me to get out because I smell, in front of everyone. It’s very embarrassing when they do that. [...] I have a bath every day but I realise my feet do smell. I live with my mum, who smokes, so I smell like cigarettes.” That could be the reason for his odour, he said in an interwiew with This is Leicestershire. In Honolulu, Hawaii (USA), bus riders with pungent body odour could have faced $ 500 in fines. Honolulu City Council members had considered a proposal to ban riders from buses if their body odor is too stinky. In 2009, the council introduced the bill to regulate a number of dangerous or annoying behaviors on city buses including excessive B.O. Under the bill, police could have cited riders if their body odour annoyed others and a convicted offender could have faced up to $ 500 in fines or six months in prison. However, many critics called the The bus behaviour bill discriminating and heavy handed. Bus drivers and regular passengers, though, said it was a positive sign that bad B.O. got banned along with other offences, such as spitting on buses, being drunk on a bus or urinating when you are on a bus or at a bus stop. After several public hearings and ongoing protests, the council eventually decided to drop the body odour ban from the bill. 2 Hands down, this must be a joke! Rollercoaster fans at Thorpe Park in in Chertsey, Surrey (UK) may wonder about strange signs (as shown above) which tell visitors to keep their arms down while riding and “Say NO to BO“. In summer, when temperatures reach 84 F (29 °C), park managers expect an excess of perspiration as visitors queue to go on rides. Wardens will also remind people to consider their fellow passengers and anyone ignoring the warnings will be escorted off. Those who continue to do so will be asked to leave the park. Mike Vallis, a director at Thorpe Park, said that the level of unpleasant smells can become unacceptable and that there were many complaints. “Our rides are really scary and people tend to sweat more than normal due to the fear and anticipation they experience while queueing up so it can get really pongy. Therefore, we felt a ban in temperatures of 25 °C plus would be the best way to ensure our guests have the most enjoyable experience and aren’t exposed to any unsavoury armpits.“ 3 Game over: Body odour now banned from the WSOP Tablemates with a severe stench now have a bad hand at poker tournaments. According to WSOP (World Series Of Poker) officials, players with bad body odour will be banned from the table. The 2010 Rule 37 B states: “All participants are entitled to expect civility and courtesy from one another at every Tournament table and throughout the Tournament area. Any individual who encounters behavior that is not civil or courteous — or is abusive in any way — is encouraged to immediately contact a Tournament official. This shall include, but is not limited to, any player whose personal hygiene has become disruptive to the other players seated at their table. The determination as to whether an individual’s personal hygiene is disruptive to other players shall be determined by the Tournament Staff which may, in its discretion, implement sanctions upon any such player who refuses to remedy the situation in a manner satisfactory.” 4 Facebook’s “clean air” group Brett Chandler from Manchester (UK) started a rather funny group at facebook.com: “Campaign To Ban Body Odour From Public Places“. In his profile Chandler descripes his idea in detail. “I’ve started this group to begin a major campaign which I will forward to the government if I get enough members. I want to give the rights to anybody to walk up to any Mr/Mrs Stinky and inform them that they smell awful. Also, I want to give bosses the authority to dismiss smelly employees. I believe that body odour in public places should be a punishable criminal offence.” By now, this group has over 240 members. Space hygiene – made in China It seems like only the ace of human race will go to space. According to China’s list of 100 taikonaut rules, aspiring space travellers need to have a “pleasant and adaptable disposition”. The guidelines make clear that only “super human beings” will go to space. In addition to all the rigorous physical requirements and demands placed on those entering China’s space program, those austronaut wannabes with bad breath, body odour or a family history of serious disease within the past three generations will be banned from space training. Shi Bing Bing, a doctor at the 454th Air Force Hospital in Nanjing, eastern China, said the new rules will help China send the best of the best into space. “Bad body odour will affect fellow colleagues in the narrow confines of a space shuttle,” he said. 5 Draconia Helvetia – Fresh air in Swiss city halls The Swiss city of Zurich has banned municipal workers from having overbearing body odour. The ban is part of a strict list of rules on behaviour and dress code issued by city officials, which also includes bans on strong perfume and plunging necklines. Workers have been provided with a four-page document with dos and don’ts for employees’ “professional presentation“. Sartorial restrictions are included as well. Too much bare back or stomach on display, mediocre shirts with patterns, trainers, messy beards and short trousers are also forbidden.6 However, city officials will not send dress code inspectors and municipal workers are still allowed to eat Swiss cheese at work. Perfidious body odour: 3-year prison sentence for Ghanaian rapist The body odour of a 20-year-old farmer, Raymond Ayeh Ensior gave him away when he attempted to rape Madam Vicentia Kwao in her sleep at Tsome-Afedo, Ghana. In the evening of January 26, 2004 Ensior sneaked into Madam Kwao’s bedroom while fellow tenants including her husband, a Teacher, were watching a football match on television on the compound. He entered the bedroom where Madam Kwao was fast asleep, removed her cover cloth and panties and attempted to rape her. Blessedly, Ensior’s approach was unusual to Madam Kwao, who also noticed that the body odour of Ensior was not that of her husband and, therefore, raised then alarm. The farmer, who pleaded guilty to indecent assault, unlawful entry and causing damage, was sentenced to three years’ imprisonment by the Ho Circuit Court presided over by Mr. Nicholas Abodakpi. 7	2	20	0	0	0	3	0.521102	3	4,451
Differences between ICD-9-CM and ICD-10-CMThe basic differences between ICD-9-CM and ICD-10-CM may be tabulated as below: \|14000 plus codes only\|\|More than 69000 codes\| \|Codes consists 3 – 5 characters\|\|Codes consists 3 – 7 characters\| \|First digit is numeric or alphanumeric\|\|First digit of every code is alpha\| \|Second, third, fourth and fifth digit always numeric\|\|Second and third digits are numeric, but fourth, fifth, sixth and seventh digit may be alpha or numeric\| \|No 7th character extension\|\|7th character extension in codes to identify episodes of care\| \|No concept of place holder\|\|Place holder "x" or dummy "x" concept, present\| \|Single excludes\|\|Excludes 1 and Excludes 2 present\| \|V and E codes as supplementary classification\|\|No supplementary classification\| \|Code titles may have incomplete code description\|\|Code titles may have incomplete code description Code titles have complete code description in all codes\| \|Code definitions lacks laterality\|\|Laterality is added to code descriptions\| Improvements In ICD-10-CM Over ICD-9-CM - ICD-10-CM has the same hierarchical structure as ICD-9-CM, but the codes are all alphanumeric and all letters except U are used. - New diseases and conditions that are not identified with unique codes in ICD-9-CM are identified with unique codes in ICD-10-CM (ex. Codes for blood type and alcohol level). In addition new diseases, etiology or treatment protocols have been reclassified more appropriately in ICD-10-CM. - ICD-10-CM provides additional information related to ambulatory & managed care encounters. - Injury codes are more expanded in ICD-10-CM than ICD-9-CM and are grouped by body parts (arm, leg, ankle and so on) instead by categories of injury or type (sprain, fracture, dislocation and so on) in ICD-9_CM. - More number of combination codes to reduce the number of codes required for fully describing a condition. Eg. Atherosclerotic heart disease with angina was coded with two codes in ICD-9-CM can be coded with a single code I25.119. - Incorporation of common fourth & fifth digit subclassifications. - Addition of sixth & seventh character subclassifications. - Introduction of laterality (right-left) to fully describe injury codes. - Greater specificity in code assignments. - No supplemental classifications (ex. V codes & E codes of ICD-9-CM), which are incorporated in the tabular list as individual chapters. - Provision of place holder dummy "x" at 5th or 6th character place for future expansion of the codes without disturbing the character structure that follows dummy "x". The new structure of code allows easy expansion in codes than was possible with ICD-9-CM. - Exclude notes are expanded to provide guidance on the hierarchy of chapters and to clarify priority in code assignments. - Creation of excludes1 & excludes2 in the conventions to segregate two codes incompatible on the same medical record by using exclude1 and to provide additional information to use additional code to fully describe a condition by using exclude2. - Postoperative complications codes have been expanded and a clear distinction has been made between intraoperative complications and postprocedureal disorders. - Obstetric codes in ICD-10-CM indicate which trimester the patient is in and no longer identify whether the patient has delivered or not, which is a constant source of confusion and error prone for coders in ICD-9-CM. - Many of the category code/subcategory code titles have been changed to reflect new technology and more recent medical terminology. - Classification refinement for increased data granularity. In general, the classification allows greater specificity in code assignments. Differences between ICD-9-CM Volume 3 and ICD-10-PCSThe basic differences between ICD-9-CM Volume 3 and ICD-10-PCS may be tabulated as follows: \|ICD-9-CM Volume 3\|\|ICD-10-PCS\| \|Approximately 3800 plus codes\|\|More than 71000 codes\| \|3 – 4 digits in codes with decimal point after first two digits\|\|All codes with 7 characters without decimal point\| \|All codes numeric\|\|Alphanumeric\| \|Less specific in description and explanation\|\|More specific\| \|More NOS and NEC codes\|\|Few NOS or NEC codes\| \|Diagnostic, clinical or pathologic terminology present in code description\|\|No diagnostic, clinical or pathological terms used in code description\| \|Laterally not attended\|\|Laterality described in code descriptions\| \|Less granular\|\|More granularity\| Apart from the above basic differences between ICD-10-CM and ICD-10-PCS, ICD-10-PCS has the following important characteristics that make it to be more acceptable in present day healthcare reimbursement system. - Completeness—there is a unique code for all substantially different procedures. - Expandability—as new procedures are developed, the structure of ICD-10-PCS allows them to be easily incorporated as unique codes. - Multi-axial—ICD-10-PCS consists of independent characters, with each individual component retaining its meaning across broad ranges of codes to the extent possible. - Standardized terminology—ICD-10-PCS includes definitions of the terminology used. Each term is assigned a specific meaning in the system. ICD-10-PCS will not affect physician practices, outpatient facilities and hospital outpatient departments usage of Current Procedural Terminology (CPT) codes on Medicare claims. CPT will still be in use. However, physicians should be aware that documentation requirements under ICD-CM-PCS are quite different, so their inpatient medical record documentation will be affected by this change.	4	8	4	0	0	2	0.367803	6	1,276
Get Coronavirus Disease 2019 essential facts below. View Videos or join the Coronavirus Disease 2019 discussion. Add Coronavirus Disease 2019 to your PopFlock.com topic list for future reference or share this resource on social media. Coronavirus Disease 2019 Infectious respiratory disease caused by severe acute respiratory syndrome coronavirus 2 The disease spreads between people most often when they are physically close.[a] It spreads very easily and sustainably through the air, primarily via small droplets or particles such as aerosols, produced after an infected person breathes, coughs, sneezes, talks or sings. It may also be transmitted via contaminated surfaces, although this has not been conclusively demonstrated. It can spread for up to two days prior to symptom onset, and from people who are asymptomatic. People remain infectious for 7-12 days in moderate cases, and up to two weeks in severe cases. The standard method of diagnosis is by real-time reverse transcription polymerase chain reaction (rRT-PCR) from a nasopharyngeal swab. Chest CT imaging may also be helpful for diagnosis in individuals where there is a high suspicion of infection based on symptoms and risk factors, however guidelines do not recommend using it for routine screening. Recommended measures to prevent infection include frequent hand washing, social distancing, quarantine, covering coughs, and keeping unwashed hands away from the face. The use of cloth face coverings such as a scarf or a bandana has been recommended by health officials in public settings to minimise the risk of transmissions, with some authorities requiring their use. Health officials also stated that medical-grade face masks, such as N95 masks, should be used only by healthcare workers, first responders, and those who directly care for infected individuals. Longer-term damage to organs (in particular lungs and heart) has been observed, and there is concern about a significant number of patients who have recovered from the acute phase of the disease but continue to experience a range of effects including severe fatigue, memory loss and other cognitive issues, low grade fever, muscle weakness, breathlessness and other symptoms for months afterwards. Symptoms of COVID-19 are variable, but usually include fever and a cough. People with the same infection may have different symptoms, and their symptoms may change over time. For example, one person may have a high fever, a cough, and fatigue, and another person may have a low fever at the start of the disease and develop difficulty breathing a week later. All of the symptoms of COVID-19 are non-specific, which means that they are also seen in some other diseases. Fever is the most common symptom of COVID-19. The fever may be high or low. Most people with COVID-19 develop a fever at some point. Most people with COVID-19 also have a cough, which could be either dry or a productive cough. Other typical symptoms include fatigue, shortness of breath and muscle and joint pains. Some symptoms, such as difficulty breathing, are more common in patients who need hospital care. Shortness of breath tends to develop later in the illness. About 40% of people temporarily lose their sense of smell (called anosmia), experience changes in how food tastes (dysgeusia), or have other disturbances to their normal abilities to smell or taste. This symptom, if it is present at all, often appears early in the illness. A disturbance in smell or taste is more commonly found in younger people, and perhaps because of this, it is associated with a lower risk of medical complications. Although most people with COVID-19 do not experience these symptoms, it is an unusual symptom for other respiratory diseases, so it is used for symptom-based screening. As is common with infections, there is a delay, known as the incubation period, between the moment a person first becomes infected and the appearance of the first symptoms. The median incubation period for COVID-19 is four to five days. Most symptomatic people experience symptoms within two to seven days after exposure, and almost all symptomatic people will experience one or more symptoms before day twelve. Some symptoms usually appear sooner than others. In August 2020, scientists at the University of Southern California reported the "likely" order of initial symptoms of the COVID-19 disease as a fever followed by a cough and muscle pain, and that nausea and vomiting usually appear before diarrhoea. This contrasts with the most common path for influenza where it is common to develop a cough first and fever later. Some people are infected with the virus but do not develop noticeable symptoms at any point in time. These asymptomatic carriers tend not to get tested, and they can spread the disease. Other infected people will develop symptoms later (called pre-symptomatic) or have very mild symptoms (called paucisymptomatic), and can also spread the virus. COVID-19 is a new disease, and how it spreads remains under investigation. It spreads from person to person, most often when they are physically close,[b] but also over longer distances, especially indoors. When an infected person breathes, coughs, sneezes, talks or sings, it produces contaminated droplets and particles such as aerosols. These travel through the air, over short and longer distances, infecting other people who inhale them. It transmits very easily and sustainably, with one infected person generally infecting between 2 and 3 others. This is more infectious than influenza, but less so than measles. It can transmit when people are symptomatic, also for up to two days prior to developing symptoms, and even if a person never shows symptoms (asymptomatic transmission), but it is unclear how often this happens. A July 2020 systematic review found that the proportion of asymptomatic cases ranges from 6% to 41%. People remain infectious in moderate cases for 7-12 days, and up to two weeks in severe cases. Airborne transmission occurs particularly in crowded and less ventilated indoor spaces, which are particularly effective for transmitting the virus, such as restaurants, nightclubs, public transport and gatherings such as funerals. It may be possible that people may be infected if they touch contaminated surfaces and then their eyes, nose or mouth with unwashed hands (fomite transmission), but this has not been conclusively demonstrated. Surfaces are easily decontaminated with household disinfectants which destroy the virus outside the human body or on the hands. Disinfectants or bleach are not a medical treatment for COVID-19, and cause health problems when not used properly, such as when used inside the human body. Estimates of the number of people infected by one person with COVID-19, the R0, have varied. The WHO's initial estimates of R0 were 1.4-2.5 (average 1.95); however, a review in early April 2020 found the basic R0 (without control measures) to be higher at 3.28 and the median R0 to be 2.79. However, a CDC study from the same month found that the median R0 of the virus was 5.7, with a basic R0 potentially as high as 8.9 without control measures, with a 95% confidence interval. Severe acute respiratory syndrome coronavirus2 (SARS-CoV-2) is a novel severe acute respiratory syndrome coronavirus. It was first isolated from three people with pneumonia connected to the cluster of acute respiratory illness cases in Wuhan. All features of the novel SARS-CoV-2 virus occur in related coronaviruses in nature. Outside the human body, the virus is destroyed by household soap, which bursts its protective bubble. SARS-CoV-2 is closely related to the original SARS-CoV. It is thought to have an animal (zoonotic) origin. Genetic analysis has revealed that the coronavirus genetically clusters with the genus Betacoronavirus, in subgenus Sarbecovirus (lineage B) together with two bat-derived strains. It is 96% identical at the whole genome level to other bat coronavirus samples (BatCov RaTG13). COVID-19 can affect the upper respiratory tract (sinuses, nose, and throat) and the lower respiratory tract (windpipe and lungs). The lungs are the organs most affected by COVID-19 because the virus accesses host cells via the enzyme angiotensin-converting enzyme 2 (ACE2), which is most abundant in type II alveolar cells of the lungs. The virus uses a special surface glycoprotein called a "spike" (peplomer) to connect to ACE2 and enter the host cell. The density of ACE2 in each tissue correlates with the severity of the disease in that tissue and some have suggested decreasing ACE2 activity might be protective,[unreliable medical source?] though another view is that increasing ACE2 using angiotensin II receptor blocker medications could be protective. As the alveolar disease progresses, respiratory failure might develop and death may follow.[unreliable medical source?] SARS-CoV-2 may also cause respiratory failure through affecting the brainstem as other coronaviruses have been found to invade the central nervous system (CNS). While virus has been detected in cerebrospinal fluid of autopsies, the exact mechanism by which it invades the CNS remains unclear and may first involve invasion of peripheral nerves given the low levels of ACE2 in the brain.[unreliable medical source?] The virus can cause acute myocardial injury and chronic damage to the cardiovascular system. An acute cardiac injury was found in 12% of infected people admitted to the hospital in Wuhan, China, and is more frequent in severe disease.[unreliable medical source?] Rates of cardiovascular symptoms are high, owing to the systemic inflammatory response and immune system disorders during disease progression, but acute myocardial injuries may also be related to ACE2 receptors in the heart. ACE2 receptors are highly expressed in the heart and are involved in heart function. A high incidence of thrombosis (31%) and venous thromboembolism (25%) have been found in ICU patients with COVID-19 infections, and may be related to poor prognosis.[unreliable medical source?][unreliable medical source?] Blood vessel dysfunction and clot formation (as suggested by high D-dimer levels) are thought to play a significant role in mortality, incidences of clots leading to pulmonary embolisms, and ischaemic events within the brain have been noted as complications leading to death in patients infected with SARS-CoV-2. Infection appears to set off a chain of vasoconstrictive responses within the body, constriction of blood vessels within the pulmonary circulation has also been posited as a mechanism in which oxygenation decreases alongside the presentation of viral pneumonia. Another common cause of death is complications related to the kidneys. Early reports show that up to 30% of hospitalized patients both in China and in New York have experienced some injury to their kidneys, including some persons with no previous kidney problems. Autopsies of people who died of COVID-19 have found diffuse alveolar damage (DAD), and lymphocyte-containing inflammatory infiltrates within the lung.[unreliable medical source?] Systemic inflammation results in vasodilation, allowing inflammatory lymphocytic and monocytic infiltration of the lung and the heart. In particular, pathogenic GM-CSF-secreting T-cells were shown to correlate with the recruitment of inflammatory IL-6-secreting monocytes and severe lung pathology in COVID-19 patients. Lymphocytic infiltrates have also been reported at autopsy.[unreliable medical source?] The WHO has published several testing protocols for the disease. The standard method of testing is real-time reverse transcription polymerase chain reaction (rRT-PCR). The test is typically done on respiratory samples obtained by a nasopharyngeal swab; however, a nasal swab or sputum sample may also be used. Results are generally available within a few hours to two days. Blood tests can be used, but these require two blood samples taken two weeks apart, and the results have little immediate value. Chinese scientists were able to isolate a strain of the coronavirus and publish the genetic sequence so laboratories across the world could independently develop polymerase chain reaction (PCR) tests to detect infection by the virus. As of 4 April 2020[update], antibody tests (which may detect active infections and whether a person had been infected in the past) were in development, but not yet widely used. Antibody tests may be most accurate 2-3 weeks after a person's symptoms start. The Chinese experience with testing has shown the accuracy is only 60 to 70%. The US Food and Drug Administration (FDA) approved the first point-of-care test on 21 March 2020 for use at the end of that month. The absence or presence of COVID-19 signs and symptoms alone is not reliable enough for an accurate diagnosis. Different clinical scores were created based on symptoms, laboratory parameters and imaging to determine patients with probable SARS-CoV-2 infection or more severe stages of COVID-19. Diagnostic guidelines released by Zhongnan Hospital of Wuhan University suggested methods for detecting infections based upon clinical features and epidemiological risk. These involved identifying people who had at least two of the following symptoms in addition to a history of travel to Wuhan or contact with other infected people: fever, imaging features of pneumonia, normal or reduced white blood cell count, or reduced lymphocyte count. A study asked hospitalised COVID-19 patients to cough into a sterile container, thus producing a saliva sample, and detected the virus in eleven of twelve patients using RT-PCR. This technique has the potential of being quicker than a swab and involving less risk to health care workers (collection at home or in the car). Along with laboratory testing, chest CT scans may be helpful to diagnose COVID-19 in individuals with a high clinical suspicion of infection but are not recommended for routine screening. Bilateral multilobar ground-glass opacities with a peripheral, asymmetric, and posterior distribution are common in early infection. Subpleural dominance, crazy paving (lobular septal thickening with variable alveolar filling), and consolidation may appear as the disease progresses. In late 2019, the WHO assigned emergency ICD-10 disease codes U07.1 for deaths from lab-confirmed SARS-CoV-2 infection and U07.2 for deaths from clinically or epidemiologically diagnosed COVID-19 without lab-confirmed SARS-CoV-2 infection. CT scan of rapid progression stage of COVID-19. Chest X-ray showing COVID-19 pneumonia. Few pieces of data were available in spring 2020 about microscopic lesions and the pathophysiology of COVID-19. The main pathological findings at autopsy are: Without pandemic containment measures--such as social distancing, vaccination, and use of face masks--pathogens can spread exponentially. This graphic shows how early adoption of containment measures tends to protect wider swaths of the population. Progressively stronger mitigation efforts to reduce the number of active cases at any given time--"flattening the curve"--allows healthcare services to better manage the same volume of patients. Likewise, progressively greater increases in healthcare capacity--called raising the line--such as by increasing bed count, personnel, and equipment, helps to meet increased demand. Mitigation attempts that are inadequate in strictness or duration--such as premature relaxation of distancing rules or stay-at-home orders--can allow a resurgence after the initial surge and mitigation. A COVID-19 vaccine is not expected until 2021 at the earliest. The US National Institutes of Health guidelines do not recommend any medication for prevention of COVID-19, before or after exposure to the SARS-CoV-2 virus, outside the setting of a clinical trial. Without a vaccine, other prophylactic measures, or effective treatments, a key part of managing COVID-19 is trying to decrease and delay the epidemic peak, known as "flattening the curve". This is done by slowing the infection rate to decrease the risk of health services being overwhelmed, allowing for better treatment of current cases, and delaying additional cases until effective treatments or a vaccine become available. Preventive measures to reduce the chances of infection include staying at home, wearing a mask in public, avoiding crowded places, keeping distance from others, washing hands with soap and water often and for at least 20 seconds, practising good respiratory hygiene, and avoiding touching the eyes, nose, or mouth with unwashed hands. Those diagnosed with COVID-19 or who believe they may be infected are advised by the CDC to stay home except to get medical care, call ahead before visiting a healthcare provider, wear a face mask before entering the healthcare provider's office and when in any room or vehicle with another person, cover coughs and sneezes with a tissue, regularly wash hands with soap and water and avoid sharing personal household items. Personal protective equipment For health care professionals who may come into contact with COVID-19 positive bodily fluids, using personal protective coverings on exposed body parts improves protection from the virus. Breathable personal protective equipment improves user-satisfaction and may offer a similar level of protection from the virus. In addition, adding tabs and other modifications to the protective equipment may reduce the risk of contamination during donning and doffing (putting on and taking off the equipment). Implementing an evidence-based donning and doffing protocol such as a one-step glove and gown removal technique, giving oral instructions while donning and doffing, double gloving, and the use of glove disinfection may also improve protection for health care professionals. Masks are also strongly recommended for those who may have been infected and those taking care of someone who may have the disease. Social distancing strategies aim to reduce contact of infected persons with large groups by closing schools and workplaces, restricting travel, and cancelling large public gatherings. Distancing guidelines also include that people stay at least 2 metres (6.6 ft) apart. After the implementation of social distancing and stay-at-home orders, many regions have been able to sustain an effective transmission rate ("Rt") of less than one, meaning the disease is in remission in those areas. Hand-washing and hygiene When not wearing a mask, the CDC, WHO, and NHS recommends covering the mouth and nose with a tissue when coughing or sneezing and recommends using the inside of the elbow if no tissue is available. Proper hand hygiene after any cough or sneeze is encouraged. The WHO also recommends that individuals wash hands often with soap and water for at least 20 seconds, especially after going to the toilet or when hands are visibly dirty, before eating and after blowing one's nose. The CDC recommends using an alcohol-based hand sanitiser with at least 60% alcohol, but only when soap and water are not readily available. For areas where commercial hand sanitisers are not readily available, the WHO provides two formulations for local production. In these formulations, the antimicrobial activity arises from ethanol or isopropanol. Hydrogen peroxide is used to help eliminate bacterial spores in the alcohol; it is "not an active substance for hand antisepsis". Glycerol is added as a humectant. The number of deaths vs total cases by country and approximate case fatality rate The severity of COVID-19 varies. The disease may take a mild course with few or no symptoms, resembling other common upper respiratory diseases such as the common cold. Mild cases typically recover within two weeks, while those with severe or critical diseases may take three to six weeks to recover. Among those who have died, the time from symptom onset to death has ranged from two to eight weeks. A European multinational study of hospitalized children published in The Lancet on 25 June 2020 found that about 8% of children admitted to a hospital needed intensive care. Four of those 582 children (0.7%) died, but the actual mortality rate could be "substantially lower" since milder cases that did not seek medical help were not included in the study. Most of those who die of COVID-19 have pre-existing (underlying) conditions, including hypertension, diabetes mellitus, and cardiovascular disease. The Istituto Superiore di Sanità reported that out of 8.8% of deaths where medical charts were available, 97% of people had at least one comorbidity with the average person having 2.7 diseases. According to the same report, the median time between the onset of symptoms and death was ten days, with five being spent hospitalised. However, people transferred to an ICU had a median time of seven days between hospitalisation and death. In a study of early cases, the median time from exhibiting initial symptoms to death was 14 days, with a full range of six to 41 days. In a study by the National Health Commission (NHC) of China, men had a death rate of 2.8% while women had a death rate of 1.7%. In 11.8% of the deaths reported by the National Health Commission of China, heart damage was noted by elevated levels of troponin or cardiac arrest. According to March data from the United States, 89% of those hospitalised had preexisting conditions. The University of Leicester and the University Hospitals of Leicester NHS Trust are undertaking a major study into the long-term health effects of COVID-19 as of August 2020[update]. The immune response by humans to CoV-2 virus occurs as a combination of the cell-mediated immunity and antibody production, just as with most other infections. However, it remains unknown if the immunity is long-lasting in people who recover from the disease.[needs update] Cases in which recovery from COVID-19 was followed by positive tests for coronavirus at a later date have been reported. However, these cases are believed to be lingering infection rather than reinfection, or false positives due to remaining RNA fragments.[medical ] Some other coronaviruses circulating in people are capable of reinfection after roughly a year. The virus is thought to be natural and has an animal origin, through spillover infection. The first known human infections were in China. A study of the first 41 cases of confirmed COVID-19, published in January 2020 in The Lancet, reported the earliest date of onset of symptoms as 1December 2019. Official publications from the WHO reported the earliest onset of symptoms as 8December 2019. Human-to-human transmission was confirmed by the WHO and Chinese authorities by 20 January 2020. According to official Chinese sources, these were mostly linked to the Huanan Seafood Wholesale Market, which also sold live animals. In May 2020, George Gao, the director of the Chinese Center for Disease Control and Prevention, said animal samples collected from the seafood market had tested negative for the virus, indicating that the market was the site of an early superspreading event, but it was not the site of the initial outbreak. Traces of the virus have been found in wastewater that was collected from Milan and Turin, Italy, on 18 December 2019. There are several theories about where the very first case (the so-called patient zero) originated. According to an unpublicised report from the Chinese government, the first case can be traced back to 17 November 2019; the person was a 55-year-old citizen in the Hubei province. There were four men and five women reported to be infected in November, but none of them were "patient zero". By December 2019, the spread of infection was almost entirely driven by human-to-human transmission. The number of coronavirus cases in Hubei gradually increased, reaching 60 by 20 December and at least 266 by 31 December. On 24 December, Wuhan Central Hospital sent a bronchoalveolar lavage fluid (BAL) sample from an unresolved clinical case to sequencing company Vision Medicals. On 27 and 28 December, Vision Medicals informed the Wuhan Central Hospital and the Chinese CDC of the results of the test, showing a new coronavirus. A pneumonia cluster of unknown cause was observed on 26 December and treated by the doctor Zhang Jixian in Hubei Provincial Hospital, who informed the Wuhan Jianghan CDC on 27 December. On 30 December, a test report addressed to Wuhan Central Hospital, from company CapitalBio Medlab, stated an erroneous positive result for SARS, causing a group of doctors at Wuhan Central Hospital to alert their colleagues and relevant hospital authorities of the result. That evening, the Wuhan Municipal Health Commission issued a notice to various medical institutions on "the treatment of pneumonia of unknown cause". Eight of these doctors, including Li Wenliang (punished on 3January), were later admonished by the police for spreading false rumours, and another, Ai Fen, was reprimanded by her superiors for raising the alarm. The Wuhan Municipal Health Commission made the first public announcement of a pneumonia outbreak of unknown cause on 31 December, confirming 27 cases--enough to trigger an investigation. During the early stages of the outbreak, the number of cases doubled approximately every seven and a half days. In early and mid-January 2020, the virus spread to other Chinese provinces, helped by the Chinese New Year migration and Wuhan being a transport hub and major rail interchange. On 20 January, China reported nearly 140 new cases in one day, including two people in Beijing and one in Shenzhen. Later official data shows 6,174 people had already developed symptoms by then, and more may have been infected. A report in The Lancet on 24 January indicated human transmission, strongly recommended personal protective equipment for health workers, and said testing for the virus was essential due to its "pandemic potential". On 30 January, the WHO declared the coronavirus a public health emergency of international concern. By this time, the outbreak spread by a factor of 100 to 200 times. On 31 January 2020, Italy had its first confirmed cases, two tourists from China. As of 13 March 2020, the World Health Organization (WHO) considered Europe the active centre of the pandemic. On 19 March 2020, Italy overtook China as the country with the most deaths. By 26 March, the United States had overtaken China and Italy with the highest number of confirmed cases in the world. Research on coronavirus genomes indicates the majority of COVID-19 cases in New York came from European travellers, rather than directly from China or any other Asian country. Retesting of prior samples found a person in France who had the virus on 27 December 2019 and a person in the United States who died from the disease on 6February 2020. Several measures are commonly used to quantify mortality. These numbers vary by region and over time and are influenced by the volume of testing, healthcare system quality, treatment options, time since the initial outbreak, and population characteristics such as age, sex, and overall health. The death-to-case ratio reflects the number of deaths divided by the number of diagnosed cases within a given time interval. Based on Johns Hopkins University statistics, the global death-to-case ratio is 3.1% (978,059/31,969,940) as of 24 September 2020. The number varies by region. Other measures include the case fatality rate (CFR), which reflects the percentage of diagnosed individuals who die from a disease, and the infection fatality rate (IFR), which reflects the percentage of infected individuals (diagnosed and undiagnosed) who die from a disease. These statistics are not time-bound and follow a specific population from infection through case resolution. Many academics have attempted to calculate these numbers for specific populations. Outbreaks have occurred in prisons due to crowding and an inability to enforce adequate social distancing. In the United States, the prisoner population is aging and many of them are at high risk for poor outcomes from COVID-19 due to high rates of coexisting heart and lung disease, and poor access to high-quality healthcare. Total confirmed cases over time Total deaths over time Total confirmed cases of COVID-19 per million people Total confirmed deaths due to COVID-19 per million people (spring 2020) Infection fatality rate Infection fatality rate or infection fatality ratio (IFR) is distinguished from case fatality rate (CFR). The CFR for a disease is the proportion of deaths from the disease compared to the total number of people diagnosed with the disease (within a certain period of time). The IFR, in contrast, is the proportion of deaths among all the infected individuals. IFR, unlike CFR, attempts to account for all asymptomatic and undiagnosed infections. Our World in Data states that, as of 25 March 2020, the IFR for coronavirus cannot be accurately calculated. In February, the World Health Organization reported estimates of IFR between 0.3% and 1%. On 2July, The WHO's Chief Scientist reported that the average IFR estimate presented at a two-day WHO expert forum was about 0.6%. The CDC estimated for planning purposes that the IFR was 0.65% and that 40% of infected individuals are asymptomatic, suggesting a fatality rate among those who are symptomatic of 1.1% (.65/60) (as of 10 July). Studies incorporating data from broad serology testing in Europe show IFR estimates converging at approximately 0.5-1%. According to the University of OxfordCentre for Evidence-Based Medicine (CEBM), random antibody testing in Germany suggested a national IFR of 0.4% (0.1% to 0.9%). Firm lower limits of IFRs have been established in a number of locations such as New York City and Bergamo in Italy since the IFR cannot be less than the population fatality rate. As of 10July, in New York City, with a population of 8.4million, 23,377 individuals (18,758 confirmed and 4,619 probable) have died with COVID-19 (0.3% of the population). May antibody testing in New York City suggested an IFR of 0.9%. In Bergamo province, 0.6% of the population has died. Early reviews of epidemiologic data showed greater impact of the pandemic and a higher mortality rate in men in China and Italy. The Chinese Center for Disease Control and Prevention reported the death rate was 2.8% for men and 1.7% for women. Later reviews in June 2020 indicated that there is no significant difference in susceptibility or in CFR between genders. One review acknowledges the different mortality rates in Chinese men, suggesting that it may be attributable to lifestyle choices such as smoking and drinking alcohol rather than genetic factors. Sex-based immunological differences, lesser prevalence of smoking in women and men developing co-morbid conditions such as hypertension at a younger age than women could have contributed to the higher mortality in men. In Europe, 57% of the infected people were men and 72% of those died with COVID-19 were men. As of April 2020, the US government is not tracking sex-related data of COVID-19 infections. Research has shown that viral illnesses like Ebola, HIV, influenza and SARS affect men and women differently. Percentage of infected people who are hospitalized Percentage of hospitalized people who go to Intensive Care Unit Percent of hospitalized people who die Percent of infected people who die – infection fatality rate (IFR) In the US, a greater proportion of deaths due to COVID-19 have occurred among African Americans. Structural factors that prevent African Americans from practicing social distancing include their concentration in crowded substandard housing and in "essential" occupations such as public transit and health care. Greater prevalence of lacking health insurance and care and of underlying conditions such as diabetes, hypertension and heart disease also increase their risk of death. Similar issues affect Native American and Latino communities. According to a US health policy non-profit, 34% of American Indian and Alaska Native People (AIAN) non-elderly adults are at risk of serious illness compared to 21% of white non-elderly adults. The source attributes it to disproportionately high rates of many health conditions that may put them at higher risk as well as living conditions like lack of access to clean water. Leaders have called for efforts to research and address the disparities. In the U.K., a greater proportion of deaths due to COVID-19 have occurred in those of a Black, Asian, and other ethnic minority background. Several factors such as poverty, poor nutrition and living in overcrowded properties, may have caused this. In January 2020, the World Health Organization recommended 2019-nCov and 2019-nCoV acute respiratory disease as interim names for the virus and disease per 2015 guidance and international guidelines against using geographical locations (e.g. Wuhan, China), animal species, or groups of people in disease and virus names in part to prevent social stigma. The official names COVID-19 and SARS-CoV-2 were issued by the WHO on 11 February 2020. WHO chief Tedros Adhanom Ghebreyesus explained: CO for corona, VI for virus, Dfor disease and 19 for when the outbreak was first identified (31 December 2019). The WHO additionally uses "the COVID-19 virus" and "the virus responsible for COVID-19" in public communications. The pandemic has had many impacts on global health beyond those caused by the COVID-19 disease itself. It has led to a reduction in hospital visits for other reasons. There have been 38% fewer hospital visits for heart attack symptoms in the United States and 40% fewer in Spain. The head of cardiology at the University of Arizona said, "My worry is some of these people are dying at home because they're too scared to go to the hospital." There is also concern that people with strokes and appendicitis are not seeking timely treatment.Shortages of medical supplies have impacted people with various conditions. In several countries there has been a marked reduction of spread of sexually transmitted infections, including HIV, attributable to COVID-19 quarantines and social distancing measures. Similarly, in some places, rates of transmission of influenza and other respiratory viruses significantly decreased during the pandemic. The pandemic has also negatively impacted mental health globally, including increased loneliness resulting from social distancing. Humans appear to be capable of spreading the virus to some other animals. A domestic cat in Liège, Belgium, tested positive after it started showing symptoms (diarrhoea, vomiting, shortness of breath) a week later than its owner, who was also positive. Tigers and lions at the Bronx Zoo in New York, United States, tested positive for the virus and showed symptoms of COVID-19, including a dry cough and loss of appetite.Minks at two farms in the Netherlands also tested positive for COVID-19. A study on domesticated animals inoculated with the virus found that cats and ferrets appear to be "highly susceptible" to the disease, while dogs appear to be less susceptible, with lower levels of viral replication. The study failed to find evidence of viral replication in pigs, ducks, and chickens. As of August 2020, dozens of domestic cats and dogs had tested positive, though according to the U.S. CDC, there was no evidence they transmitted the virus to humans. CDC guidance recommends potentially infected people avoid close contact with pets. No medication or vaccine other than remdesivir is approved with the specific indication to treat the disease. In Australia and the European Union, remdesivir (Veklury) is indicated for the treatment of COVID-19 in adults and adolescents (aged 12 years and older with body weight at least 40 kg) with pneumonia requiring supplemental oxygen. International research on vaccines and medicines in COVID-19 is underway by government organisations, academic groups, and industry researchers. In March, the World Health Organization initiated the "Solidarity Trial" to assess the treatment effects of four existing antiviral compounds with the most promise of efficacy. The World Health Organization suspended hydroxychloroquine from its global drug trials for COVID-19 treatments on 26 May 2020 due to safety concerns. It had previously enrolled 3,500 patients from 17 countries in the Solidarity Trial. France, Italy and Belgium also banned the use of hydroxychloroquine as a COVID-19 treatment. Modelling research has been conducted with several objectives, including predictions of the dynamics of transmission, diagnosis and prognosis of infection, estimation of the impact of interventions, or allocation of resources. Modelling studies are mostly based on epidemiological models, estimating the number of infected people over time under given conditions. Several other types of models have been developed and used during the COVID-19 including computational fluid dynamics models to study the flow physics of COVID-19, retrofits of crowd movement models to study occupant exposure, mobility-data based models to investigate transmission, or the use of macroeconomic models to assess the economic impact of the pandemic. There has been a great deal of COVID-19 research, involving accelerated research processes and publishing shortcuts to meet the global demand. To minimise the harm from misinformation, medical professionals and the public are advised to expect rapid changes to available information, and to be attentive to retractions and other updates. There is no available vaccine, but various agencies are actively developing vaccine candidates. Previous work on SARS-CoV is being used because both SARS-CoV and SARS-CoV-2 use the ACE2 receptor to enter human cells. Nine vaccine platforms are being investigated (as of August 2020), with 24 candidate vaccines being tested in clinical trials. First, researchers aim to build a whole virus vaccine. The use of such inactive virus aims to elicit a prompt immune response of the human body to a new infection with COVID-19. A second strategy, subunit vaccines, aims to create a vaccine that sensitises the immune system to certain subunits of the virus. In the case of SARS-CoV-2, such research focuses on the S-spike protein that helps the virus intrude the ACE2 enzyme receptor. A third strategy is that of the nucleic acid vaccines (DNA or RNA vaccines, a novel technique for creating a vaccination). Fourthly, scientists are attempting to use viral vectors to deliver the SARS-CoV-2 antigen gene into the cell. These can be replicating or non-replicating. Experimental vaccines from any of these strategies would have to be tested for safety and efficacy. The COVID-19 Clinical Research Coalition has goals to 1) facilitate rapid reviews of clinical trial proposals by ethics committees and national regulatory agencies, 2) fast-track approvals for the candidate therapeutic compounds, 3) ensure standardised and rapid analysis of emerging efficacy and safety data and 4) facilitate sharing of clinical trial outcomes before publication. There are mixed results as of 3 April 2020, as to the effectiveness of hydroxychloroquine as a treatment for COVID-19, with some studies showing little or no improvement. One study has shown an association between hydroxychloroquine or chloroquine use with higher death rates along with other side effects. A retraction of this study by its authors was published by The Lancet on 4June 2020. The studies of chloroquine and hydroxychloroquine with or without azithromycin have major limitations that have prevented the medical community from embracing these therapies without further study. On 15 June 2020, the FDA updated the fact sheets for the emergency use authorization of remdesivir to warn that using chloroquine or hydroxychloroquine with remdesivir may reduce the antiviral activity of remdesivir. In June, initial results from a randomised trial in the United Kingdom showed that dexamethasone reduced mortality by one third for patients who are critically ill on ventilators and one fifth for those receiving supplemental oxygen. Because this is a well tested and widely available treatment this was welcomed by the WHO that is in the process of updating treatment guidelines to include dexamethasone or other steroids. Based on those preliminary results, dexamethasone treatment has been recommended by the National Institutes of Health for patients with COVID-19 who are mechanically ventilated or who require supplemental oxygen but not in patients with COVID-19 who do not require supplemental oxygen. In September 2020, the WHO released updated guidance on using corticosteroids for COVID-19. The WHO recommends systemic corticosteroids rather than no systemic corticosteroids for the treatment of people with severe and critical COVID-19 (strong recommendation, based on moderate certainty evidence). The WHO suggests not to use corticosteroids in the treatment of people with non-severe COVID-19 (conditional recommendation, based on low certainty evidence). The updated guidance was based on a meta-analysis of clinical trials of critically ill COVID-19 patients. In September 2020, the European Medicines Agency (EMA) endorsed the use of dexamethasone in adults and adolescents (from twelve years of age and weighing at least 40 kg) who require supplemental oxygen therapy. Dexamethasone can be taken by mouth or given as an injection or infusion (drip) into a vein. A cytokine storm can be a complication in the later stages of severe COVID-19. There is preliminary evidence that hydroxychloroquine may be useful in controlling cytokine storms in late-phase severe forms of the disease. Lenzilumab, an anti-GM-CSF monoclonal antibody, is protective in murine models for CAR T cell-induced CRS and neurotoxicity and is a viable therapeutic option due to the observed increase of pathogenic GM-CSF secreting T-cells in hospitalised patients with COVID-19. Transferring purified and concentrated antibodies produced by the immune systems of those who have recovered from COVID-19 to people who need them is being investigated as a non-vaccine method of passive immunisation. The safety and effectiveness of convalescent plasma as a treatment option requires further research. This strategy was tried for SARS with inconclusive results.Viral neutralization is the anticipated mechanism of action by which passive antibody therapy can mediate defence against SARS-CoV-2. The spike protein of SARS-CoV-2 is the primary target for neutralizing antibodies. As of 8 August 2020, eight neutralizing antibodies targeting the spike protein of SARS-CoV-2 have entered clinical studies. It has been proposed that selection of broad-neutralizing antibodies against SARS-CoV-2 and SARS-CoV might be useful for treating not only COVID-19 but also future SARS-related CoV infections. Other mechanisms, however, such as antibody-dependent cellular cytotoxicity and/or phagocytosis, may be possible. Other forms of passive antibody therapy, for example, using manufactured monoclonal antibodies, are in development. Production of convalescent serum, which consists of the liquid portion of the blood from recovered patients and contains antibodies specific to this virus, could be increased for quicker deployment. Peru announced in April 2020 that it would begin working towards creating a vaccine, with the pharmaceutical company Farvet and Universidad Peruana Cayetano Heredia (UPCH) announcing plans to jointly develop a vaccine in Chincha. Peru's Experimental Station for Scientific Research and Genetic Improvement of Alpacas belonging to the Inca Group, selected on 5 June 2020 four alpacas for the development of a new vaccine that it had been developing in conjunction with Farvet and UPCH. They also indicated that alpacas have the ability to generate some types of antibodies known as "nanobodies", which are very small and have a greater potential to treat pathogens. According to Andina, research from the United States, Belgium, and Chile showed that antibodies from laminoid animals could possibly be formulated into inhaler or injection treatments for those infected with coronaviruses, with Teodosio Huanca of Peru's National Institute of Agricultural Innovation (INIA) National Camelid Program stating that Peruvian camelidae share the same genetic roots and antibodies. On 7 August, the Peruvian National Institute of Health (INS) announced that it would begin the development of a possible treatment for COVID-19 utilizing "recombinant nanoantibodies" from a llama named "Tito". According to the INIA, Peru holds "the only germplasm bank of South American camelids in the world, with 1,700 samples of alpacas and 1,200 of llamas". ^"ICD-10 Version:2019". World Health Organization. 2019. Archived from the original on 31 March 2020. Retrieved 2020. U07.2 - COVID-19, virus not identified - COVID-19 NOS - Use this code when COVID-19 is diagnosed clinically or epidemiologically but laboratory testing is inconclusive or not available. Use additional code, if desired, to identify pneumonia or other manifestations ^"For different groups of people: how to choose masks". NHC.gov.cn. National Health Commission of the People's Republic of China. 7 February 2020. Retrieved 2020. Disposable medical masks: Recommended for: · People in crowded places · Indoor working environment with a relatively dense population · People going to medical institutions · Children in kindergarten and students at school gathering to study and do other activities ^"WHO-recommended handrub formulations". WHO Guidelines on Hand Hygiene in Health Care: First Global Patient Safety Challenge Clean Care Is Safer Care. World Health Organization. 19 March 2009. Retrieved 2020. ^" :". Xinhua Net . 31 December 2019. Retrieved 2020. ^"Archived copy" . WJW.Wuhan.gov.cn (in Chinese). Wuhan Municipal Health Commission. 31 December 2019. Archived from the original on 9 January 2020. Retrieved 2020.CS1 maint: archived copy as title (link)	2	35	0	0	0	1	0.512372	1	9,612
Medical conditions are classified under either Diagnostic and Statistical Manual of Mental Disorders, currently in its fifth version (DSM-5) or International Classification of Diseases, tenth revision (ICD-10). They are the two international systems for classifying the illness in the US and World Health Organization's (WHO) member nations. DSM-5 contains diagnostic criteria reserved for mental disorders such as schizophrenia, depression, eating disorders, substance-related disorders etc. On the other hand, ICD-10-CM is a HIPAA-approved diagnostic code set reserved for classifying other illness associated with gastrointestinal, genitourinary, musculoskeletal, gastrointestinal, respiratory, etc. ICD-10 system shares some codes with DSM-5 although they are strictly unrelated. Read ahead to learn the major differences between ICD and DSM systems. One look at the ICD-10-CM coding system gives an impression that DSM-5 is almost an unneeded classification. However, that isn't the case. The DSM-5 gives clinicians with criteria and definitions to classify diseases symptomatically through a common language. Not only does it help in quick classification and diagnosis, but also streamlines patient care. After successful diagnosis, ICD-10-CM assigns a code number that is useful during the reimbursement process, statistical compilation, and for further documentation and reporting purposes. Not all diagnosis classified under DSM-5 is covered by ICD-10-CM. For instance, Under DSM-5 classification, dementia is considered as a major neurocognitive disorder or a minor one. However, it is unlisted in the ICD-10 code classification because it does not cover mental disorders in depth. It is one of the top differences between ICD-10 and DSM-5 systems. In such cases, it is essential to have an internal coding system that would allow specific diagnosis of neurocognitive disorders to assign the code. In psychology and psychiatry, there are fewer crosswalks. Perhaps because coding isn't straightforward and it is impossible to have a one-stop solution coding guidance. But then how to have a distinction between diagnoses? The DSM-5! It helps in providing usable definitions because creating crosswalks on commonly used codes can be tedious, but DSM-5 offers a crosswalk between ICD-9 and ICD-10. This is because it represents code sets for several key diagnoses. In a head-on comparison between ICD vs DSM, International Classification of Diseases (ICD) is a well-accepted global coding system used by clinicians for categorizing all health conditions because it is a code bible for all diseases, symptoms, abnormal findings, and reporting. But DSM is a coding system for diagnosis of mental disorder. Every “psychiatric disorder” is classified under “mental and behavioral disorders.” This classification is useful for diagnosing those under psychiatric care. The latter is more accurate because of its immense contribution to research and common language. American Psychiatric Association (APA) is collaborating with ICD-10-CM contributors for including the latest codes from the DSM-5 system. They were keen to classify social pragmatic communication disorder as code F80.82 under the ICD-10-CD. No doubt that there will be instances ahead where more DSM-5 codes will align with ICD-10. But that is not to say they will mirror diagnosis information in every manner. In the real world, the ideal way to deal with this scenario is to have an internal coding policy that will allow clinicians to include descriptive information of the diagnosis rather than plain codes. Similarities and differences between ICD-10 and DSM-5 are many. It provides a wealth of information to clinicians for classifying diagnosis and aiding. Both ICD-10 and DSM-5 provide ways to classify diseases ICD and DSM criteria are not based on empirical evidence but are consensus formulations approved by medical experts for research purpose ICD and DSM manuals are similar for several diagnoses. For example, ICD-10 and DSM-5 criteria coincide in the severe case of Alcohol Use Disorder (AUD) diagnosis, social pragmatic communication disorder, and its treatment IDC and DSM coding systems allow crosswalking between older and newer versions It is essential for clinicians to classify neurocognitive disorders with the right set of names and descriptions rather than simple codes. The specificity of coding details from diagnosis documented in ICD-10-CM had enhanced significantly after its implementation in 2015. Currently, the development is headed towards the 11th revision (ICD-11) which will be endorsed in 2018. ICD-10 system of code was frozen with strict rules preventing future modification. However, it soon became clear that the system needed expansion for covering more codes in depth. For example, obsessive-compulsive which was included under ICD-10-CM as F42 was expanded to cover the following specifics - Outsource2india is an ISO-certified organization which carries 21 years of experience in medical billing and coding services. During this time we have acquired innovative technologies, updated implementation methods, and tackled challenges as they come. We take pride that our solutions have resulted in several success stories. Our cost-effective medical coding services can help you minimize operational expenses by a huge margin. Whether it is following up on pending medical claims, tracking receivable balances, or initiating collections we have got it all covered through our top-quality healthcare BPO services and 24/7 support. So, you will no longer face worries about training new employees, no staff turnover, low accuracy, and much more. 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What is the ICD-10 code for right pseudoaneurysm? I72. 4 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2022 edition of ICD-10-CM I72. 4 became effective on October 1, 2021. What is the diagnosis code for aneurysm? I67. 1 – Cerebral aneurysm, nonruptured. ICD-10-CM. What is the ICD-10 code for abdominal aortic aneurysm? ICD-10 code I71. 4 for Abdominal aortic aneurysm, without rupture is a medical classification as listed by WHO under the range – Diseases of the circulatory system . What is a pseudoaneurysm? A pseudoaneurysm, or pseudoaneurysm of the vessels, occurs when a blood vessel wall is injured and the leaking blood collects in the surrounding tissue. It is sometimes called a false aneurysm. In a true aneurysm, the artery or vessel weakens and bulges, sometimes forming a blood-filled sac. What does aneurysm of unspecified site mean? Pathological outpouching or sac-like dilatation in the wall of any blood vessel (arteries or veins) or the heart (heart aneurysm). It indicates a thin and weakened area in the wall which may later rupture. What is a femoral artery pseudoaneurysm? Femoral artery pseudoaneurysm is the most common complication of femoral access following diagnostic or therapeutic cardiac and peripheral angiographic procedures. 1. A pseudoaneurysm or false aneurysm is a contained rupture in which blood leaks from an artery into the surrounding tissue (Figure 1). What is infrarenal Abdominal aortic aneurysm? The most common location of arterial aneurysm formation is the abdominal aorta, specifically, the segment of the abdominal aorta below the kidneys. An abdominal aneurysm located below the kidneys is called an infrarenal aneurysm. An aneurysm can be characterized by its location, shape, and cause. What is the difference between a pseudoaneurysm and an aneurysm? A pseudoaneurysm happens as a result of injury to a blood vessel. The artery leaks blood, which then pools near the damaged spot. It’s different from a true aneurysm, which happens when the wall of a blood vessel stretches and forms a bulge. Most pseudoaneurysms are complications from medical procedures. How is pseudoaneurysm different from aneurysm? A saccular-shaped aneurysm bulges or balloons out only on one side. A pseudoaneurysm, or false aneurysm, is not an enlargement of any of the layers of the blood vessel wall. A false aneurysm may be the result of a prior surgery or trauma. Where is an infrarenal aneurysm? An abdominal aneurysm located below the kidneys is called an infrarenal aneurysm. An aneurysm can be characterized by its location, shape, and cause. The shape of an aneurysm is described as being fusiform or saccular which helps to identify a true aneurysm. What part of the aorta is the infrarenal? The abdominal aorta is clinically divided into 2 segments: The suprarenal abdominal or paravisceral segment, inferior to the diaphragm but superior to the renal arteries. The Infrarenal segment, inferior to the renal arteries and superior to the iliac bifurcation. What is an aortic aneurysm? An aortic aneurysm is a balloon-like bulge in the aorta, the large artery that carries blood from the heart through the chest and torso. Aortic aneurysms can dissect or rupture: The force of blood pumping can split the layers of the artery wall, allowing blood to leak in between them. What exactly is a pseudoaneurysm? What is femoral pseudoaneurysm? INTRODUCTION. The femoral artery pseudoaneurysm (FAP) is a troublesome groin complication related to the femoral arterial access site used for invasive cardiovascular procedures. 1. FAP occur in 0.1% to 0.2% of diagnostic angiograms and 0.8% to 2.2% following interventional procedures. What is a small pseudoaneurysm? What are the new ICD 10 codes? The new codes are for describing the infusion of tixagevimab and cilgavimab monoclonal antibody (code XW023X7), and the infusion of other new technology monoclonal antibody (code XW023Y7). What ICD 10 cm code(s) are reported? What is the correct ICD-10-CM code to report the External Cause? Your Answer: V80.010S The External cause code is used for each encounter for which the injury or condition is being treated. What is the ICD 10 diagnosis code for? The ICD-10-CM is a catalog of diagnosis codes used by medical professionals for medical coding and reporting in health care settings. The Centers for Medicare and Medicaid Services (CMS) maintain the catalog in the U.S. releasing yearly updates. What does ICD 10 mean? ICD-10 is the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD), a medical classification list by the World Health Organization (WHO). It contains codes for diseases, signs and symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or diseases.	4	5	0	0	0	9	0.89653	9	1,262
A benign tumor is a mass of cells (tumor) that lacks the ability to invade neighboring tissue or metastasize. These characteristics are required for a tumor to be defined as cancerous and therefore benign tumors are non-cancerous. Also, benign tumors generally have a slower growth rate than malignant tumors and the tumor cells are usually more differentiated (cells have normal features). Benign tumors are typically surrounded by an outer surface (fibrous sheath of connective tissue) or remain with the epithelium. Common examples of benign tumors include moles (nevi) and uterine fibroids (leiomyomas). Although benign tumors will not metastasize or locally invade tissues, some types may still produce negative health effects. The growth of benign tumors produce a "mass effect" that can compress tissues and may cause nerve damage, reduction of blood to an area of the body (ischaemia), tissue death (necrosis) and organ damage. The mass effect of tumors are more prominent if the tumor is within an enclosed space such as the cranium, respiratory tract, sinus or inside bones. Tumors of endocrine tissues may overproduce certain hormones, especially when the cells are well differentiated. Examples include thyroid adenomas and adrenocortical adenomas. Although most benign tumors are not life-threatening, many types of benign tumors have the potential to become cancerous (malignant) through a process known as tumour progression. For this reason and other possible negative health effects, some benign tumors are removed by surgery. \|Cell Origin\|\|Cell type\|\|Tumor\| \|Liver\|\|Liver cell adenoma\| \|Renal\|\|Renal tubular adenoma\| \|Squamous\|\|Squamous cell papilloma\| Benign neoplasms are typically but not always composed of cells which bear a strong resemblance to a normal cell type in their organ of origin. These tumors are named for the cell or tissue type from which they originate, followed by the suffix "-oma" (but not -carcinoma, -sarcoma, or -blastoma, which are generally cancers). For example, a lipoma is a common benign tumor of fat cells (lipocytes), and a chondroma is a benign tumor of cartilage-forming cells (chondrocytes). Adenomas are benign tumors of gland-forming cells, and are usually specified further by their cell or organ of origin, as in hepatic adenoma (a benign tumor of hepatocytes, or liver cells). Teratomas contain many cell types such as skin, nerve, brain and thyroid, among others, because they are derived from germ cells. Hamartomas are a group of benign tumors that have relatively normal cellular differentiation but the architecture of the tissue is disorganised. There are a few cancers with 'benign-sounding' names which have been retained for historical reasons, including melanoma (a cancer of pigmented skin cells, or melanocytes) and seminoma (a cancer of male reproductive cells). Skin tags, vocal chord polyps and hyperplastic polyps of the colon are often referred to as benign but they are actually overgrowths of normal tissue rather than neoplasms. Benign tumors are very diverse, and may be asymptomatic or may cause specific symptoms depending on their anatomic location and tissue type. They grow outwards, producing large rounded masses, which can cause what is known as a "mass effect". This growth can cause compression of local tissues or organs, which can cause many effects such as blockage of ducts, reduced blood flow (ischaemia), tissue death (necrosis) and nerve pain or damage. Some tumors also produce hormones that can lead to life-threatening situations. Insulinomas can produce large amounts of insulin leading to hypoglycemia. Pituitary adenomas can cause elevated levels of hormones such as growth hormone and insulin-like growth factor-1, which cause acromegaly; prolactin; ACTH and cortisol, which cause Cushings disease; TSH, which causes hyperthyroidism; and FSH and LH. Bowel intussusception can occur with various benign colonic tumors. Cosmetic effects can be caused by tumors, especially those of the skin, possibly causing psychological effects on the person with the tumor. Vascular tumors can bleed, which in some cases can be substantial, leading to anemia. Some benign tumors need no treatment; others may be removed if they cause problems such as seizures, discomfort or cosmetic concerns. Surgery is usually the most effective approach and is used to treat most benign tumors. In some case other treatments may be of use. Adenomas of the rectum may be treated with sclerotherapy, a treatment in which chemicals are used to shrink blood vessels in order to cut off the blood supply. Most benign tumors do not respond to chemotherapy or radiation therapy, although there are exceptions; benign intercranial tumors are sometimes treated with radiation therapy and chemotherapy under certain circumstances. Radiation can also be used to treat hemangiomas in the rectum. Benign skin tumors are usually surgically ressected but other treatments such as cryotherapy, curettage, electrodesiccation, laser therapy, dermabrasion, chemical peels and topical medication are used. Benign vs malignant One of the most important factors in classifying a tumor as benign or malignant is its invasive potential. If a tumor lacks the ability to invade adjacent tissues or spread to distant sites by metastasizing then it is benign, whereas invasive or metastatic tumours are malignant. For this reason, benign tumours are not classed as cancer. Benign tumours will grow in a contained area usually encapsulated in a fibrous connective tissue capsule. The growth rates of benign and malignant tumors also differ; benign tumors generally grow more slowly than malignant tumors. Although benign tumors pose a lower health risk than malignant tumors, they can both be life-threatening in certain situations. There are many general characteristics which apply to either benign or malignant tumors, but sometimes one type may show characteristics of the other. For example, benign tumors are mostly well differentiated and malignant tumors are often undifferentiated. However, undifferentiated benign tumors and differentiated malignant tumors can occur. Although benign tumors generally grow slowly, cases of fast growing benign tumors have also been documented. Some malignant tumors are mostly non-metastatic such as in the case of basal cell carcinoma. Tumours are formed by carcinogenesis, a process in which cellular alterations lead to the formation of cancer. Multistage carcinogenesis involves the sequential genetic or epigenetic changes to a cell's DNA, where each step produces a more advanced tumour. It is often broken down into three stages; initiation, promotion and progression, and several mutations may occur at each stage. Initiation is where the first genetic mutation occurs in a cell. Promotion is the clonal expansion (repeated division) of this transformed cell into a visible tumour that is usually benign. Following promotion, progression may take place where more genetic mutations are acquired in a sub-population of tumor cells. Progression changes the benign tumor into a malignant tumor. A prominent and well studied example of this phenomenon is the tubular adenoma, a common type of colon polyp which is an important precursor to colon cancer. The cells in tubular adenomas, like most tumors which frequently progress to cancer, show certain abnormalities of cell maturation and appearance collectively known as dysplasia. These cellular abnormalities are not seen in benign tumors that rarely or never turn cancerous, but are seen in other pre-cancerous tissue abnormalities which do not form discrete masses, such as pre-cancerous lesions of the uterine cervix. Some authorities[who?] prefer to refer to dysplastic tumors as "pre-malignant", and reserve the term "benign" for tumors which rarely or never give rise to cancer. PTEN hamartoma syndrome PTEN hamartoma syndrome comprises four distinct hamartomatous disorders characterised by genetic mutations in the PTEN gene; Cowden syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome and Proteus-like syndrome. Although they all have distinct clinical features, the formation of hamartomas is present in all four syndromes. PTEN is a tumor suppressor gene that is involved in cellular signalling. Absent or dysfunctional PTEN protein allows cells to over-proliferate, causing hamartomas. Cowden syndrome is an autosomal dominant genetic disorder characterised by multiple benign hamartomas (trichilemmomas and mucocutaneous papillomatous papules) as well as a predisposition for cancers of multiple organs including the breast and thyroid. Bannayan-Riley-Ruvalcaba syndrome is a congenital disorder characterised by hamartomatous intestinal polyposis, macrocephaly, lipomatosis, hemangiomatosis and glans penis macules. Proteus syndrome is characterised by nevi, asymmetric overgrowth of various body parts, adipose tissue dysregulation, cystadenomas, adenomas, vascular malformation. Familial adenomatous polyposis Familial adenomatous polyposis (FAP) is a familial cancer syndrome caused by mutations in the APC gene. In this disorder adenomatous polyps are present in the colon that invariably progress into colon cancer. The APC gene is a tumor suppressor and its product is involved in many cellular processes. Inactivation of the APC gene leads to a buildup of a protein called β-catenin, which activates two transcription factors; T-cell factor (TCF) and lymphoid enhancer factor (LEF). These cause the upregulation of many genes involved in cell proliferation, differentiation, migration and apoptosis (programmed cell death), causing the growth of benign tumors. Tuberous sclerosis complex Tuberous sclerosis complex (TSC) is an autosomal dominant genetic disorder caused by mutations in the genesTSC1 and TSC2, which produce the proteins hamartin and tuberin, respectively. This disorder presents with many benign hamartomatous tumors including angiofibromas, renal angiomyolipomas, pulmonary lymphangiomyomatosis. Tuberin and hamartin inhibit the mTOR protein in normal cellular physiology and the inactivation of the TSC tumour suppressors causes an increase in mTOR activity. This leads to the activation of genes and the production of proteins that increase cell growth. Von Hippel-Lindau disease Von Hippel-Lindau disease is a dominantly inherited cancer syndrome that massively increases the risk of various tumors including benign hemangioblastomas and malignant pheochromocytomas, renal cell carcinomas, pancreatic endocrine tumors and endolymphatic sac tumors. It is caused by genetic mutations in the Von Hippel–Lindau tumor suppressor gene. The VHL protein (pVHL) is involved in cellular signalling in oxygen starved (hypoxic) cells. One role of pVHL is to cause the cellular degradation of another protein, HIF1α. Dysfunctional pVHL leads to accumulation of HIF1α, which in turn activates the production of several genes involved in cell growth and blood vessel production (VEGF, PDGFβ, TGFα and erythropoietin). - Wilson, Kathleen Atkins; Waugh, Anne; Chambers, Graeme; Grant, Allison; Ross, Janet (2006). Ross and Wilson anatomy and physiology in health and illness. Edinburgh: Churchill Livingstone. pp. 53–54. 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ISBN 0-7637-6357-8. - Inoki K, Corradetti MN, Guan KL (January 2005). "Dysregulation of the TSC-mTOR pathway in human disease". Nat. Genet. 37 (1): 19–24. doi:10.1038/ng1494. PMID 15624019. - Ramzi Cotran, Vinay Kumar, Tucker Collins (1999). Robbins Pathologic Basis of Disease, 6th Edition. W.B. Saunders. ISBN 0-7216-7335-X. - Marks V, Teale JD (June 1991). "Tumours producing hypoglycaemia". Diabetes Metab Rev 7 (2): 79–91. doi:10.1002/dmr.5610070202. PMID 1665409. - Grant CS (October 2005). "Insulinoma". Best Pract Res Clin Gastroenterol 19 (5): 783–98. doi:10.1016/j.bpg.2005.05.008. PMID 16253900. - Charis Eng; DeLellis, Ronald A.; Lloyd, Ricardo V.; Phillipp U. Heitz (2004). Pathology and genetics of tumours of endocrine organs. Lyon: IARC Press. ISBN 92-832-2416-7. - Gill SS, Heuman DM, Mihas AA (October 2001). "Small intestinal neoplasms". J. Clin. Gastroenterol. 33 (4): 267–82. doi:10.1097/00004836-200110000-00004. 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"Congenital melanocytic nevi: treatment modalities and management options". Semin Cutan Med Surg 26 (4): 231–40. doi:10.1016/j.sder.2008.03.007. PMID 18395671. - Skorić T, Korsić M, Zarković K, et al. (June 1999). "Clinical and morphological features of undifferentiated monomorphous GH/TSH-secreting pituitary adenoma". Eur. J. Endocrinol. 140 (6): 528–37. doi:10.1530/eje.0.1400528. PMID 10366409. - Song HJ, Xue YL, Qiu ZL, Luo QY (2012). "Uncommon metastases from differentiated thyroid carcinoma". Hell J Nucl Med 15 (3): 233–40. doi:10.1967/s002449910059. PMID 23106056. - Sagel SS, Ablow RC (November 1968). "Hamartoma: on occasion a rapidly growing tumor of the lung". Radiology 91 (5): 971–2. doi:10.1148/91.5.971. PMID 5681331. - Barrett JC (April 1993). "Mechanisms of multistep carcinogenesis and carcinogen risk assessment". Environ. Health Perspect. 100: 9–20. doi:10.1289/ehp.931009. PMC 1519586. PMID 8354184. - Hobert JA, Eng C (October 2009). "PTEN hamartoma tumor syndrome: an overview". Genet. Med. 11 (10): 687–94. doi:10.1097/GIM.0b013e3181ac9aea. PMID 19668082. - Pilarski R, Eng C (May 2004). "Will the real Cowden syndrome please stand up (again)? Expanding mutational and clinical spectra of the PTEN hamartoma tumour syndrome". J. Med. Genet. 41 (5): 323–6. doi:10.1136/jmg.2004.018036. PMC 1735782. PMID 15121767. - Eng C (November 2000). "Will the real Cowden syndrome please stand up: revised diagnostic criteria". J. Med. Genet. 37 (11): 828–30. doi:10.1136/jmg.37.11.828. PMC 1734465. PMID 11073535. - Eng C (September 2003). "PTEN: one gene, many syndromes". Hum. Mutat. 22 (3): 183–98. doi:10.1002/humu.10257. PMID 12938083. - Blumenthal GM, Dennis PA (November 2008). "PTEN hamartoma tumor syndromes". Eur. J. Hum. Genet. 16 (11): 1289–300. doi:10.1038/ejhg.2008.162. PMID 18781191. - Cohen MM (August 2005). "Proteus syndrome: an update". Am J Med Genet C Semin Med Genet 137C (1): 38–52. doi:10.1002/ajmg.c.30063. 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\|Classification and external resources\| CPR being administered during a simulation of cardiac arrest. Cardiac arrest, also known as cardiopulmonary arrest or circulatory arrest, is a sudden stop in effective blood circulation due to failure of the heart to contract effectively or at all. Medical personnel may refer to an unexpected cardiac arrest as a sudden cardiac arrest (SCA). A cardiac arrest is different from (but may be caused by) a heart attack, where blood flow to the muscle of the heart is impaired. It is different from congestive heart failure, where circulation is substandard, but the heart is still pumping sufficient blood to sustain life. Arrested blood circulation prevents delivery of oxygen and glucose to the body. Lack of oxygen and glucose to the brain causes loss of consciousness, which then results in abnormal or absent breathing. Brain injury is likely to happen if cardiac arrest goes untreated for more than five minutes. For the best chance of survival and neurological recovery, immediate and decisive treatment is imperative. Cardiac arrest is a medical emergency that, in certain situations, is potentially reversible if treated early. Unexpected cardiac arrest can lead to death within minutes: this is called sudden cardiac death (SCD). The treatment for cardiac arrest is immediate defibrillation if a "shockable" rhythm is present, while cardiopulmonary resuscitation (CPR) is used to provide circulatory support and/or to induce a "shockable" rhythm. - 1 Classification - 2 Signs and symptoms - 3 Causes - 4 Diagnosis - 5 Prevention - 6 Management - 7 Prognosis - 8 Epidemiology - 9 References - 10 External links Clinicians classify cardiac arrest into "shockable" versus "non–shockable", as determined by the ECG rhythm. This refers to whether a particular class of cardiac dysrhythmia is treatable using defibrillation. The two "shockable" rhythms are ventricular fibrillation and pulseless ventricular tachycardia while the two "non–shockable" rhythms are asystole and pulseless electrical activity. Signs and symptoms Cardiac arrest is an abrupt cessation of pump function in the heart (as evidenced by the absence of a palpable pulse). Prompt intervention can usually reverse a cardiac arrest, but without such intervention it will almost always lead to death. In certain cases, it is an expected outcome of a serious illness were death is expected. However, due to inadequate cerebral perfusion, the patient will be unconscious and will have stopped breathing. The main diagnostic criterion to diagnose a cardiac arrest (as opposed to respiratory arrest which shares many of the same features) is lack of circulation; however, there are a number of ways of determining this. Near death experiences are reported by 10-20% of people who survived cardiac arrest. Coronary heart disease is the leading cause of sudden cardiac arrest. Many other cardiac and non-cardiac conditions also increase one's risk. Coronary heart disease Approximately 60–70% of SCD is related to coronary heart disease. Among adults, ischemic heart disease is the predominant cause of arrest with 30% of people at autopsy showing signs of recent myocardial infarction. Non-ischemic heart disease In a group of military recruits aged 18–35, cardiac anomalies accounted for 51% of cases of SCD, while in 35% of cases the cause remained unknown. Underlying pathology included: coronary artery abnormalities (61%), myocarditis (20%), and hypertrophic cardiomyopathy (13%). Congestive heart failure increases the risk of SCD by 5 fold. Many additional conduction abnormalities exist that place one at higher risk for cardiac arrest. For instance, long QT syndrome, a condition often mentioned in young people's deaths, occurs in 1/5000-1/7000 newborns and is estimated to be responsible 3000 deaths each year compared to the approximately 300000 cardiac arrests seen by emergency services. These conditions are a fraction of the overall deaths related to cardiac arrest, but represent conditions which may be detected prior to arrest, which may be treatable. About 35% of SCDs are not caused by a heart condition. The most common non-cardiac causes are trauma, bleeding (such as gastrointestinal bleeding, aortic rupture, or intracranial hemorrhage), overdose, drowning and pulmonary embolism. Cardiac arrest can also be caused by poisoning (for example, by the stings of certain jellyfish). Hs and Ts - Hypovolemia - A lack of blood volume - Hypoxia - A lack of oxygen - Hydrogen ions (Acidosis) - An abnormal pH in the body - Hyperkalemia or Hypokalemia - Both excess and inadequate potassium can be life-threatening. - Hypothermia - A low core body temperature - Hypoglycemia or Hyperglycemia - Low or high blood glucose - Tablets or Toxins - Cardiac Tamponade - Fluid building around the heart - Tension pneumothorax - A collapsed lung - Thrombosis (Myocardial infarction) - Heart attack - Thromboembolism (Pulmonary embolism) - A blood clot in the lung - Traumatic cardiac arrest Cardiac arrest is synonymous with clinical death. A cardiac arrest is usually diagnosed clinically by the absence of a pulse. In many cases lack of carotid pulse is the gold standard for diagnosing cardiac arrest, but lack of a pulse (particularly in the peripheral pulses) may result from other conditions (e.g. shock), or simply an error on the part of the rescuer. Studies have shown that rescuers often make a mistake when checking the carotid pulse in an emergency, whether they are healthcare professionals or lay persons. Owing to the inaccuracy in this method of diagnosis, some bodies such as the European Resuscitation Council (ERC) have de-emphasised its importance. The Resuscitation Council (UK), in line with the ERC's recommendations and those of the American Heart Association, have suggested that the technique should be used only by healthcare professionals with specific training and expertise, and even then that it should be viewed in conjunction with other indicators such as agonal respiration. Various other methods for detecting circulation have been proposed. Guidelines following the 2000 International Liaison Committee on Resuscitation (ILCOR) recommendations were for rescuers to look for "signs of circulation", but not specifically the pulse. These signs included coughing, gasping, colour, twitching and movement. However, in face of evidence that these guidelines were ineffective, the current recommendation of ILCOR is that cardiac arrest should be diagnosed in all casualties who are unconscious and not breathing normally. With positive outcomes following cardiac arrest unlikely, an effort has been spent in finding effective strategies to prevent cardiac arrest. With the prime causes of cardiac arrest being ischemic heart disease, efforts to promote a healthy diet, exercise, and smoking cessation are important. For people at risk of heart disease, measures such as blood pressure control, cholesterol lowering, and other medico-therapeutic interventions are used. In medical parlance, cardiac arrest is referred to as a "code" or a "crash". This typically refers to "code blue" on the hospital emergency codes. A dramatic drop in vital sign measurements is referred to as "coding" or "crashing", though coding is usually used when it results in cardiac arrest, while crashing might not. Treatment for cardiac arrest is sometimes referred to as "calling a code". Extensive research has shown that patients in general wards often deteriorate for several hours or even days before a cardiac arrest occurs. This has been attributed to a lack of knowledge and skill amongst ward based staff, in particular a failure to carry out measurement of the respiratory rate, which is often the major predictor of a deterioration and can often change up to 48 hours prior to a cardiac arrest. In response to this, many hospitals now have increased training for ward based staff. A number of "early warning" systems also exist which aim to quantify the risk which patients are at of deterioration based on their vital signs and thus provide a guide to staff. In addition, specialist staff are being utilised more effectively in order to augment the work already being done at ward level. These include: - Crash teams (or code teams) - These are designated staff members who have particular expertise in resuscitation, who are called to the scene of all arrests within the hospital. This usually involves a specialized cart of equipment (including defibrillator) and drugs called a "crash cart" or "crash trolley". - Medical emergency teams - These teams respond to all emergencies, with the aim of treating the patient in the acute phase of their illness in order to prevent a cardiac arrest. - Critical care outreach - As well as providing the services of the other two types of team, these teams are also responsible for educating non-specialist staff. In addition, they help to facilitate transfers between intensive care/high dependency units and the general hospital wards. This is particularly important, as many studies have shown that a significant percentage of patients discharged from critical care environments quickly deteriorate and are re-admitted - the outreach team offers support to ward staff to prevent this from happening. In some medical facilities, the resuscitation team may purposely respond slowly to a patient in cardiac arrest, a practice known as slow code, or may fake the response altogether for the sake of the patient's family, a practice known as show code. This is generally done for patients for whom performing CPR will have no medical benefit. Such practices are ethically controversial, and are banned in some jurisdictions. Implantable cardioverter defibrillators A technologically based intervention to prevent further cardiac arrest episodes is the use of an implantable cardioverter-defibrillator (ICD). This device is implanted in the patient and acts as an instant defibrillator in the event of arrhythmia. Note that standalone ICDs do not have any pacemaker functions, but they can be combined with a pacemaker, and modern versions also have advanced features such as anti-tachycardic pacing as well as synchronized cardioversion. A recent study by Birnie et al. at the University of Ottawa Heart Institute has demonstrated that ICDs are underused in both the United States and Canada. An accompanying editorial by Simpson explores some of the economic, geographic, social and political reasons for this. Patients who are most likely to benefit from the placement of an ICD are those with severe ischemic cardiomyopathy (with systolic ejection fractions less than 30%) as demonstrated by the MADIT-II trial. Sudden cardiac arrest may be treated via attempts at resuscitation. This is usually carried out based upon basic life support (BLS) / advanced cardiac life support (ACLS), pediatric advanced life support (PALS) or neonatal resuscitation program (NRP) guidelines. Cardiopulmonary resuscitation (CPR) is a important part of the management of cardiac arrest. It is recommended that it be started as soon as possible and interrupted as little as possible. The component of CPR which seems to make the greatest difference in most cases is the chest compressions. Correctly performed bystander CPR has been shown to increase survival; however, it is performed in less than 30% of out of hospital arrests as of 2007. If high quality CPR has not resulted in return of spontaneous circulation and the person's heart rhythm is in asystole discontinuing CPR and pronouncing the person death is reasonable after 20 minutes. Exceptions to this include those with hypothermia or who have drowned. Longer durations of CPR may be reasonable in those who have cardiac arrest while in hospital. Tracheal intubation has not been found to improve survival rates in cardiac arrest and in the prehospital environment may worsen it. A 2009 study found that assisted ventilation may worsen outcomes over placement of an oral airway with passive oxygen delivery. CPR which involves only chest compressions results in the same outcomes as standard CPR for those who have gone into cardiac arrest due to heart issues. A 2013 review found some evidence that mechanical chest compressions (as performed by a machine) are better than manual chest compressions while a 2011 and 2012 review considered the evidence insufficient. It is unclear if a few minutes of CPR before defibrillation results in different outcomes than immediate defibrillation. Shockable and non–shockable causes of cardiac arrest is based on the presence or absence of ventricular fibrillation or pulseless ventricular tachycardia. The shockable rhythms are treated with CPR and defibrillation. In addition, there is increasing use of public access defibrillation. This involves placing automated external defibrillators in public places, and training staff in these areas how to use them. This allows defibrillation to take place prior to the arrival of emergency services, and has been shown to lead to increased chances of survival. Some defibrillators even provide feedback on the quality of CPR compressions, encouraging the lay rescuer to press the patient's chest hard enough to circulate blood. In addition, it has been shown that those who have arrests in remote locations have worse outcomes following cardiac arrest. Medications, while included in guidelines, have been shown not to improve survival to hospital discharge post out of hospital cardiac arrest. This includes the use of epinephrine, atropine, and amiodarone. Vasopressin overall does not improve or worsen outcomes but may be of benefit in those with asystole especially if used early. The 2010 guidelines, from the American Heart Association has removed its recommendation for using atropine in pulseless electrical activity and asystole due to the lack of evidence for its use. Evidence is insufficient for lidocaine and amiodarone may be considered in those who continue in ventricular tachycardia or ventricular fibrillation despite defibrillation. Thrombolytics when used generally may cause harm but may be of benefit in those with a pulmonary embolism as the cause of arrest. Cooling a person after cardiac arrest with return of spontaneous circulation (ROSC) but without return of consciousness may or may not improve outcomes. This procedure is called therapeutic hypothermia. People are cooled over a 24 hour period, with a target temperature of 32–34 °C (90–93 °F). Death rates in the hypothermia group were initially believed to be 35% lower with generally mild complications. A November 2013 trial, however, called this idea into question with findings that a temperature of 36 °C (97 °F) results in the same outcomes as 33 °C (91 °F). And a second trial looking at earlier versus later cooling found no difference. Do not resuscitate Some people choose to avoid aggressive measures at the end of life. A do not resuscitate order (DNR) in the form of an advance health care directive makes it clear that in the event of cardiac arrest, the person does not wish to receive cardiopulmonary resuscitation. Other directives may be made to stipulate the desire for intubation in the event of respiratory failure or, if comfort measures are all that are desired, by stipulating that healthcare providers should "allow natural death". Chain of survival Several organisations promote the idea of a chain of survival. The chain consists of the following "links": - Early recognition - If possible, recognition of illness before the patient develops a cardiac arrest will allow the rescuer to prevent its occurrence. Early recognition that a cardiac arrest has occurred is key to survival - for every minute a patient stays in cardiac arrest, their chances of survival drop by roughly 10%. - Early CPR - improves the flow of blood and of oxygen to vital organs - an essential component of treating a cardiac arrest. In particular, by keeping the brain supplied with oxygenated blood, chances of neurological damage are decreased. - Early defibrillation - is effective for the management of ventricular fibrillation and pulseless ventricular tachycardia - Early advanced care - Early post resuscitation care If one or more links in the chain are missing or delayed, then the chances of survival drop significantly. These protocols are often initiated by a code blue, which usually denotes impending or acute onset of cardiac arrest or respiratory failure, although in practice, code blue is often called in less life-threatening situations that require immediate attention from a physician. Resuscitation with extracorporeal membrane oxygenation devices has been attempted with better results for in-hospital cardiac arrest (29% survival) than out of hospital cardiac arrest (4% survival) in populations selected to benefit most. Cardiac catheterization in those who have survived an out of hospital cardiac arrest appears to improve outcomes. The precordial thump may be considered in those with witnessed, monitored, unstable ventricular tachycardia (including pulseless VT) if a defibrillator is not immediately ready for use, but it should not delay CPR and shock delivery or be used in those with unwitnessed out of hospital arrest. The survival rate to hospital discharge of people who receive initial emergency care by ambulance is 2%, with 15% experiencing return of spontaneous circulation. However, with defibrillation within 3–5 minutes, the survival rate increases to 30%. Since mortality in case of out-of-hospital cardiac arrest is high, programs were developed to improve survival rate. Although mortality in case of ventricular fibrillation is high, rapid intervention with a defibrillator increases survival rate. A 1997 review into outcomes following in-hospital cardiac arrest found a survival to discharge of 14% although the range between different studies was 0-28%. In those over the age of 70 who have a cardiac arrest while in hospital, survival to hospital discharge is less than 20%. How well these individuals are able to manage after leaving hospital is not clear. Survival is mostly related to the cause of the arrest (see above). In particular, people who have suffered hypothermia have an increased survival rate, possibly because the cold protects the vital organs from the effects of tissue hypoxia. Survival rates following an arrest induced by toxins is very much dependent on identifying the toxin and administering an appropriate antidote. A patient who has suffered a myocardial infarction due to a blood clot in the left coronary artery has a lower chance of survival. A study of survival rates from out of hospital cardiac arrest found that 14.6% of those who had received resuscitation by ambulance staff survived as far as admission to hospital. Of these, 59% died during admission, half of these within the first 24 hours, while 46% survived until discharge from hospital. This gives us an overall survival following cardiac arrest of 6.8%. Of these 89% had normal brain function or mild neurological disability, 8.5% had moderate impairment, and 2% suffered major neurological disability. 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PMID 8664715. - "Abstract 969: Lifetime Risk for Sudden Cardiac Death at Selected Index Ages and by Risk Factor Strata and Race: Cardiovascular Lifetime Risk Pooling Project -- Lloyd-Jones et al. 120 (10018): S416 -- Circulation". \|Find more about Cardiac arrest at Wikipedia's sister projects\| \|Definitions from Wiktionary\| \|Media from Commons\| \|Quotations from Wikiquote\| \|Source texts from Wikisource\| \|Textbooks from Wikibooks\| \|Learning resources from Wikiversity\|	2	58	9	0	2	0	0.907156	11	9,089
In healthcare, diagnosis codes are used as a tool to group and identify diseases, disorders, symptoms, poisonings, adverse effects of drugs & chemicals, injuries and other reasons for patient encounters. Diagnostic coding is the translation of written descriptions of diseases, illnesses and injuries into codes from a particular classification. In medical classification, diagnosis codes are used as part of the clinical coding process alongside intervention codes. Both diagnosis and intervention codes are assigned by a health professional trained in medical classification such as a clinical coder or Health Information Manager. Several diagnosis classification systems have been implemented to various degrees of success across the world. The various classifications have a focus towards a particular patient encounter type such as emergency, inpatient, outpatient, mental health as well as surgical care. The International Statistical Classification of Diseases and Related Health Problems (ICD) is one of the most widely used classification systems for diagnosis coding as it allows comparability and use of mortality and morbidity data. As the knowledge of health and medical advances arise, the diagnostic codes are generally revised and updated to match the most up to date current body of knowledge in the field of health. The codes may be quite frequently revised as new knowledge is attained. DSM (see below) changes some of its coding to correspond to the codes in ICD. In 2005, for example, DSM changed the diagnostic codes for circadian rhythm sleep disorders from the 307-group to the 327-group; the new codes reflect the moving of these disorders from the Mental Disorders section to the Neurological section in the ICD Diagnostic Coding Systems A number of diagnostic coding systems are currently implemented across the world to code the stay of patients within a typical health setting such as a hospital. The following table provides a basic list of the currently used coding systems: \|ICD-9-CM\|\|Volumes 1 and 2 only. Volume 3 contains Procedure codes\| \|ICD-10\|\|The Current International Standard\| \|ICPC-2\|\|Also includes reasons for encounter (RFE), procedure codes and process of care\| \|International Classification of Sleep Disorders\| \|Diagnostic and Statistical Manual of Mental Disorders\|\|Primarily psychiatric disorders\| \|Mendelian Inheritance in Man\|\|Genetic diseases\| \|Read code\|\|Used throughout United Kingdom General Practice computerised records\| Financial aspects of Diagnostic Coding Diagnosis codes are generally used as a representation of admitted episodes in health care settings. The principal diagnosis, additional diagnoses alongside intervention codes essentially depict a patient's admission to a hospital. Diagnoses codes are subjected to ethical considerations as they contribute to the total coded medical record in health services areas such as a hospital. Hospitals that are based on Activity Based Funding and Diagnoses Related Group Classification systems are often subjected to high end decision making that could affect the outcome of funding. It’s important to look at the scope of diagnoses codes in terms of their application in finance. The diagnoses codes in particular the Principal Diagnoses and Additional Diagnoses can significantly affect the total funding that a hospital may receive for any patient admitted. Ethically this highlights the fact that the assignment of the diagnoses code can be influenced by a decision to maximize reimbursement of funding. For example when looking at the activity based funding model used in the public hospital system in Victoria the total coded medical record is responsible for its reflected funding. These decisions also affect clinical documentation by physicians as recommendations from a Health Information Service can directly affect how a clinician may document a condition that a patient may have. The difference between the codes assigned for confusion and delirium can alter a hospitals DRG assignment as delirium is considered a higher level code than confusion within the ICD-10 coding hierarchy in terms of severity. A clinical coder or Health Information Manager may feel obliged to maximize funding above the ethical requirement to be honest within their diagnostic coding; this highlights the ethical standpoint of diagnoses codes as they should be reflective of a patient’s admission. Factors affecting accuracy in Diagnostic Coding Accuracy is a major component in diagnoses codes. The accurate assignment of diagnoses codes in clinical coding is essential in order to effectively depict a patients stay within a typical health service area. A number of factors can contribute to the overall accuracy coding which includes medical record legibility, physician documentation, clinical coder experience, financial decision making, miscoding as well as classification system limitations. Medical Record Legibility The legibility of a medical record is a contributing factor in the accuracy of diagnostic coding. The assigned proxy that is extracting information from the medical record is dependent on the quality of the medical record. Factors that contribute to a medical records quality are physician documentation, handwriting legibility, compilation of forms, duplication and inaccurate patient data. For example if a clinical coder or Health Information Manager was extracting data from a medical record in which the principal diagnoses was unclear due to illegible handwriting, the health professional would have to contact the physician responsible for documenting the diagnoses in order to correctly assign the code. In Australia, the legibility of records has been sufficiently maintained due to the implementation of highly detailed standards and guidelines which aim to improve the legibility of medical records. In particular the paper medical record standard 'AS 2828' created by Standards Australia focuses on a few key areas which are critical to maintaining a legible paper medical record. The following criteria should be used as a guideline when creating a medical record specific to the aid of providing clear documentation for diagnostic coding. In particular the legibility of a medical record is dependent on; - Durability: If a medical record wasn't durable, overtime if a coder was to revisit the record and it wasn't legible it wouldn't be feasible to code from that record. - Ready Identification: A coder must be able to identify the exact record being coded in order to effectively extract diagnoses codes. - Reproducible: A coder would need to make sure that the record is reproducible in that copies can be made to aid in effective coding. Clinical Coder Experience The experience of the health professional coding a medical record is an essential variable that must be accounted for when analysing the accuracy of coding. Generally a coder with years of experience is able to extract all the relevant information from a medical record whether it is paper, scanned or semi-electronic. The diagnoses codes selected from the extraction are generally compiled and sequenced in order to represent the admission. An experienced coder may incorrectly assign codes due a lack of application of a classification systems relevant standards. An example to highlight clinical coding experience would be the standard within the Australian Coding Standards 0010 General Abstraction Guidelines. These guidelines indicate that a coder must seek further detail within a record in order to correctly assign the correct diagnoses code. An inexperienced coder may simply just use the description from the discharge summary such as Infarction and may not use the correct detail which could be further found within the details of the medical record. This directly relates to the accuracy of diagnoses codes as the experience of the health professional coder is significant in its accuracy and contribution to finance. Weaknesses in Diagnostic Coding Generally coding is a concept of modeling reality with reduced effort but with physical copying. - Hence the result of coding is a reduction to the scope of representation as far as possible to be depicted with the chosen modeling technology. There will be never an escape, but choosing more than one model to serve more than one purpose. That led to various code derivatives, all of them using one basic reference code for ordering as e.g. with ICD-10 coding. However, concurrent depiction of several models in one image remains principally impossible. - Focusing a code on one purpose lets other purposes unsatisfied. This has to be taken into account when advertising for any coding concept. The operability of coding is generally bound to purpose. Inter-referring must be subject of evolutionary development, as code structures are subject of frequent change. - Unambiguous coding requires strict restriction to hierarchical tree structures possibly enhanced with multiple links, but no parallel branching for contemporary coding whilst maintaining bijectivity. - Spatial depictions of n-dimensional code spaces as coding scheme trees on flat screens may enhance imagination, but still leave the dimensionality of image limited to intelligibility of sketching, mostly as a 3D object on a 2D screen. Pivoting such image does not solve the intelligibility problem. - Projections of code spaces as flattened graphs may ease the depiction of a code, but generally reduce the contained information with the flattening. There is no explanation given with many of the codes for transforming from one code system to another. That leads to specialized usage and to limitations in communication between codes. The escape is with code reference structures (as e.g. not existing with SNOMED3). - Hierarchical ordering of more than one code system may be seen as appropriate, as the human body is principally invariant to coding. But the dependency implied with such hierarchies decrease the cross referencing between the code levels down to unintelligibility. The escape is with hyper maps that exceed planar views (as e.g. with SNOMED3) and their referring to other codes (as e.g. yet not existing with SNOMED3). - Purpose of documenting will be seen as essential just for the validation of a code system in aspects of correctness. However this purpose is timely subordinate to the generating of the respective information. Hence some code system shall support the process of medical diagnosis and of medical treatment of any kind. Escape is with a specialised coding for the processes of working on diagnosis as on working with treatment (as e.g. not intended with SNOMED3). - Intelligibility of results of coding is achieved by semantic design principles and with ontologies to support navigating in the codes. One major aspect despite the fuzziness of language is the bijectivity of coding. Escape is with explaining the code structure to avoid misinterpreting and various codes for the very same condition (as e.g. yet not served at all with SNOMED3). - Systematized Nomenclature of Medicine - Diagnosis-related group - Medical classification - Major Diagnostic Category - Clinical Audit - American Health Information Management Association - Hazelwood, A (2005). ICD-9-CM Diagnostic Coding and Reimbursement for Physician Services 2006 Edition. United States of America: American Health Information Management Association. p. 2. - Steindel, S (20 May 2010). "International classification of diseases, 10th edition, clinical modification and procedure coding system: descriptive overview of the next generation HIPAA code sets". Journal of American Medical Informatics Association 17 (3): 274–282. PMC 2995704. Retrieved 26 May 2013. - First, M (2005). "New Diagnostic Codes for Sleep Disorders". American Psychiatric Association. Retrieved 2008-08-08. - "Victorian Hospital Admission Policy". Department of Health. Retrieved 25 May 2013. - Uzkuraitis, C; Hastings, K. Torney,B. (2010). "Casemix funding optimisation: working together to make the most of every episode". Health Information Management Journal 39 (3): 47–49. - Lowe, A (2201). "Casemix accounting systems and medical coding Organisational actors balanced on ``leaky black boxes". Journal of Organizational Change Managemen 14 (1): 79–100. Retrieved 25 May 2613. Check date values in: - Cheng, P; Gilchrist, A. Robinson, K. Paul, L. (26 May 2013). "The risk and consequences of clinical miscoding due to inadequate medical documentation: a case study of the impact on health services funding". HEALTH INFORMATION MANAGEMENT JOURNAL 38 (1): 35–46. PMID 19293434. - Standards, Australia. "Paper-based Health Record". Standards Australia. Retrieved 30 May 2013. - "OVERVIEW OF ICD-10-AM/ACHI/ACS". University of Wollongong. Retrieved 29 May 2013. - O'Malley, K; Cook, K. Price, M. Wildes, K. Hurdle, J. Ashton, C. (2005). "Measuring Diagnoses: ICD Code Accuracy". Health Services Research 40 (5): 1620–1639. doi:10.1111/j.1475-6773.2005.00444.x. Retrieved 25 May 2013. - Towards Semantic Interoperability in Healthcare	3	13	1	0	0	0	0.715382	1	2,590
In developed countries, the number of maternal deaths associated with heart disease, including myocardial disorders, has been increasing during the past two decades.1,2 However, myocardial disorders represent a set of underrecognized, underreported, and poorly understood obstetric problems.3 Peripartum cardiomyopathy, a distinct clinical entity, has received the most attention in the obstetric literature because of its unknown etiology, unpredictable course, and frequently poor outcomes.4,5 The four diagnostic criteria for peripartum cardiomyopathy were adopted at a workshop on this disorder organized by the National Heart, Lung, and Blood Institute and the Office of Rare Diseases of the National Institutes of Health in April 1997.6 For a definitive diagnosis of peripartum cardiomyopathy, all four criteria must be present, and they include 1) the development of congestive heart failure in the past month of pregnancy or within 5 months after delivery, 2) the absence of preexisting cardiac dysfunction, 3) the absence of a determinable cause of cardiomyopathy, and 4) documented left ventricular systolic dysfunction, such as depressed shortening fraction or ejection fraction.6 The estimated prevalence of peripartum cardiomyopathy varies widely depending on the population, with the reported prevalence of this condition in the United States ranging from 1 per 1,300 live births in Little Rock, Arkansas, in 1963 to 1 per 15,000 live births in Dallas, Texas, in 1986.7 To improve the ascertainment and understanding of morbidity associated with peripartum cardiomyopathy among pregnant women, in October 2003 a specific International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM) code for peripartum cardiomyopathy (674.5) was separated from the nonspecific code 678.4, “Other complications of the puerperium, not elsewhere classified.”8 Peripartum cardiomyopathy is not the only myocardial disorder that complicates pregnancy. Because an increase in the prevalence of preexisting cardiac and noncardiac disease that can present as myocardial disorders has been observed in the obstetric population during the past 10 years,9,10 we hypothesized that many hospitalizations likely involved myocardial disorders other than peripartum cardiomyopathy. In addition, with the notable improvements in the treatment of congenital heart disease over the past few decades, congenital heart disease has become an important cause of heart disease (including myocardial disorders) in pregnancy in the developed world.11,12 However, population-level data on the prevalence of myocardial disorders in obstetric population are limited. Moreover, the risks of severe obstetric complications among women with myocardial disorders are not well understood. The objectives of the present report were to 1) evaluate the feasibility of using the ICD-9-CM code for peripartum cardiomyopathy for surveillance purposes, 2) estimate the rate of pregnancy hospitalizations with myocardial disorders, and 3) report the rate of severe obstetric complications by myocardial disorders status among delivery and postpartum hospitalizations using data from the 2004–2006 Nationwide Inpatient Sample of the Healthcare Cost and Utilization Project. MATERIALS AND METHODS The Healthcare Cost and Utilization Project is a federal–state–industry partnership sponsored by the Agency for Healthcare Research and Quality.13 The Nationwide Inpatient Sample is the largest all-payer inpatient care database in the United States. All of the nonfederal community hospitals from the states participating in the annual Healthcare Cost and Utilization Project data collection are stratified by rural/urban location, number of beds, region, teaching status, and ownership. Within each stratum, a systematic random 20% sample of hospitals is drawn. The sample size of this large cross-sectional study was predetermined by sampling methodology that allows using discharges from the sampled hospitals to produce nationwide estimates. A full description of the Nationwide Inpatient Sample methodology can be found on the Healthcare Cost and Utilization Project Web site.13 Because the Nationwide Inpatient Sample excludes data elements that could directly or indirectly identify individuals, this research was considered exempt from review by the institutional review board of the Centers for Disease Control and Prevention.14 For the myocardial disorders prevalence analysis, we included all 2004–2006 pregnancy-related hospitalizations, which were identified by ICD-9-CM diagnostic codes 630–677, V22, V23, V24, V28, and 792.3; ICD-9-CM obstetric procedure codes 72.xx, 73.xx, 74.xx, and 75.xx; and diagnosis related group (DRG) codes 370–384. The delivery, postpartum, and antenatal hospitalizations were identified hierarchically. The specific details about ICD-9-CM and DRG coding to identify delivery hospitalizations are described in detail elsewhere.15 Postpartum hospitalizations were identified by a fifth digit of 4 in ICD-9-CM codes for primary or secondary diagnosis; ICD-9-CM code V24 for any listed diagnosis; or postpartum DRG codes 376–377. Antenatal hospitalizations were identified by a fifth digit of 3 in ICD-9-CM codes for primary or secondary diagnosis; ICD-9-CM code V22, V23, V28, or 792.3 for any listed diagnosis; or antenatal DRG codes 378–384. We confined the analysis of severe obstetric complications associated with myocardial disorders to delivery hospitalizations and postpartum hospitalizations. Severe obstetric complications were identified by using condition-specific ICD-9-CM codes and additional information obtained from hospital discharge records, such as mortality, transfer from or to another health care facility, and duration of stay. The specific ICD-9-CM codes for severe complications and details of the method to identify delivery hospitalizations with these conditions can be found elsewhere.9,10 In addition to severe complications previously published, this study also included acute myocardial infarction (ICD-9-CM code 410.x), disorders of fluid, electrolyte, and acid–base balance (ICD-9-CM code 276.x), and cardiac arrest/ventricular fibrillation (ICD-9-CM codes 427.5, 427.41, and 427.42). The hospitalizations with myocardial disorders were identified by ICD-9-CM codes 674.5x (peripartum cardiomyopathy) or 425.x (other cardiomyopathies). In the classification of cardiomyopathy proposed by the American Heart Association in 2006, myocardial disorders associated with coronary artery disease, hypertension, valvular disease, and congenital heart disease were excluded.16 However, in our 2004–2006 sample these hospitalizations would still have ICD-9-CM codes for cardiomyopathy. Thus, in this analysis we classified hospital records that simultaneously had ICD-9-CM codes for cardiomyopathy and heart disease, such as coronary artery disease, hypertension, valvular disease, and congenital heart disease, as records with other myocardial disorders. The remaining hospitalizations with ICD-9-CM code 425.x or 674.5x were classified as hospitalizations with cardiomyopathy. As a next step of our analysis, we evaluated the feasibility of using the specific ICD-9-CM code 674.5x for surveillance of peripartum cardiomyopathy. Some hospital records with codes for peripartum cardiomyopathy also had ICD-9-CM codes for preexisting cardiac or cardiomyopathy-associated noncardiac disease (eg, diabetes mellitus) at the same time. We considered these records to be misclassified as peripartum cardiomyopathy because there was evidence of specific etiologies for cardiomyopathy. To describe a distribution of misclassified and correctly classified records with ICD-9-CM code 674.5x, we hierarchically categorized all these records into three mutually exclusive groups: 1) records with ICD-9-CM codes for heart disease (Table 1): valvular disease, congenital heart disease, coronary artery disease, hypertensive heart disease, conduction disorders and cardiac dysrhythmias, and pulmonary circulation disease; 2) records with ICD-9-CM codes for potential underlying conditions for cardiomyopathy (Table 2): myocarditis, diabetes (excluding gestational), thyroid disease, chronic hypertension (excluding hypertensive heart disease), chronic renal failure, rheumatoid arthritis/collagen vascular disease, rheumatic heart failure, severe anemia, human immunodeficiency virus, drug abuse, alcohol abuse, and nutrient deficiencies; and 3) records with ICD-9-CM codes for peripartum cardiomyopathy only. The unit of analysis was a hospitalization, not an individual; these data did not allow us to account for multiple pregnancy hospitalizations of the same women during the study period. However, because a woman can only deliver once, the analysis of delivery hospitalizations can be considered an analysis of individuals. We reported overall rates of pregnancy hospitalizations with cardiomyopathy and other myocardial disorders and rates by age, payer, and hospital region per 1,000 deliveries. Rates of severe complications per 1,000 deliveries or 1,000 postpartum hospitalizations were also calculated. We compared these rates using χ2 tests with adjustment of P values for multiple comparisons using the Bonferroni method. Because many hospitals do not collect or report race/ethnicity, we did not include this variable in the analysis.13 We used SAS 9.1 (SAS Institute Inc., Cary, NC) to manage data and SAS-callable SUDAAN 9.0 (RTI International, Research Triangle, NC) to account for the multistage probability sampling design. Thus, all results are based on the weighted estimates of pregnancy hospitalizations in the United States during the study period. During the 3-year study period, there were an estimated 14,323,731 pregnancy hospitalizations in the United States. Among the 16,824 records with myocardial disorders, 11,134 had codes for peripartum cardiomyopathy. Among 11,134 records with a code for peripartum cardiomyopathy, 6,097 (54.8%) also had ICD-9-CM codes for heart disease, and 2,704 (24.3%) also had codes for cardiomyopathy-associated noncardiac conditions (total 79.1%). Thus, only 2,332 records (20.9%) with the specific peripartum cardiomyopathy code had no codes for cardiac or noncardiac conditions. The overall rate of hospitalizations with any use of the ICD-9-CM code for peripartum cardiomyopathy was 0.88 per 1,000 deliveries (1 case per 1,136 deliveries), and the rate of hospitalizations with the ICD-9-CM code for peripartum cardiomyopathy without any code for cardiac and noncardiac conditions was 0.18 per 1,000 deliveries (1 case per 5,556 deliveries). For this analysis all hospitalizations with the ICD-9-CM code for peripartum cardiomyopathy were reclassified into two groups: cardiomyopathy (n=5,037) and other myocardial disorders (n=6,097). Among all pregnancy hospitalizations (Table 3), the overall prevalence of hospitalizations with myocardial disorders was 1.33 per 1,000 deliveries. The rate of pregnancy hospitalizations with cardiomyopathy was 0.46 per 1,000 deliveries (0.18 for apparent peripartum cardiomyopathy and 0.28 for other cardiomyopathies). The rate of pregnancy hospitalizations with other myocardial disorders was 0.87 per 1,000 deliveries. Myocardial disorders were rare during delivery hospitalizations (0.01%) but not uncommon among postpartum hospitalizations (4.2%). The rates of myocardial disorders per 1,000 deliveries by age groups, payer status, and region were also calculated (Table 4). The significantly (P<.01) higher rates of myocardial disorders were observed for women aged 35 years or older compared with women aged 15–24 years, hospitalizations covered by public payer compared with hospitalizations covered by private insurance, and in the South compared with other regions. The same pattern was observed when rates of cardiomyopathy and other myocardial disorders were estimated separately. The rate (per 1,000 deliveries) of severe complications among delivery hospitalizations without myocardial disorders ranged from 0.07 (95% confidence interval [CI] 0.06–0.08) for cardiac arrest to 1.88 (95% CI 951.77–1.99) for fluid and electrolyte disorders (Table 5). In contrast, among hospitalizations with myocardial disorders this rate ranged from 13.2 (95% CI 6.3–20.1) for acute myocardial infarction to 128.6 (95% CI 107.2–150.0) for adult respiratory distress syndrome. The rate (per 1,000 hospitalizations) of severe complications among postpartum hospitalizations without myocardial disorders ranged from 0.4 (95% CI 0.2–0.6) for cardiac arrest to 65.5 (95% CI 64.4–68.7) for fluid and electrolyte disorders (Table 6). The rate of severe complications among hospitalizations with myocardial disorders was significantly higher than among hospitalizations without myocardial disorders (P<.05) and ranged from 10.7 (95% CI 5.7–15.7) for pulmonary edema to 193.0 (95% CI 173.7–212.4) for fluid and electrolyte disorders. Our study reports the prevalence of cardiomyopathy and other myocardial disorders during pregnancy hospitalizations in the United States after the implementation of the specific ICD-9-CM code for peripartum cardiomyopathy at the end of 2003. The recently implemented ICD-9-CM code for peripartum cardiomyopathy was frequently used simultaneously with codes for other heart disease and comorbidities, and hence it is unlikely to be specific enough to track peripartum cardiomyopathy as a distinct entity in the United States. Thus, our results underlined the importance of being more precise about language and diagnostic criteria when documenting hospital course among pregnant women. In addition, our results support the continuation of efforts to modify ICD-9-CM codes to include more granularity about conditions that occur during pregnancy. The exact prevalence of cardiomyopathy during pregnancy hospitalization in the United States has not been known. Consistent with the existing obstetric literature focusing mostly on peripartum cardiomyopathy, recent reports have attempted to estimate the prevalence of this entity. A study attempting to estimate the national prevalence of peripartum cardiomyopathy in the United States from the 1990–2002 National Hospital Discharge Survey identified one hospitalization per 3,189 live births.17 Similarly, in a 1996–2005 study of 241,497 deliveries within the Southern California Kaiser health care system, the prevalence of peripartum cardiomyopathy based on the chart review of delivery hospitalizations with an ICD-9-CM code for heart failure was 1 in 4,025 deliveries.18 The results from these studies are difficult to compare, however, because of the difference in methodology applied to identify the hospitalizations with peripartum cardiomyopathy. In our study, approximately 1 in 1,136 delivery hospitalizations had the recently implemented ICD-9-CM code for peripartum cardiomyopathy. However, approximately 80% of these hospitalizations also had an ICD-9-CM code for heart disease or chronic conditions, potential underlying causes for dilated cardiomyopathy, and thus they could not fulfill the diagnostic criteria for peripartum cardiomyopathy. Moreover, a low sensitivity of ICD-9-CM codes for chronic conditions, resulting in their underreporting in obstetric hospital records, has been documented before.19 Accordingly, although we have reasonable confidence in using our data to estimate the prevalence of cardiomyopathy in a generic sense, our results highlight the limitations of using hospital discharge data to obtain reliable estimates for the prevalence of true peripartum cardiomyopathy. The clinical presentation of peripartum or dilated cardiomyopathy is similar to that for systolic congestive heart failure. However, dilated cardiomyopathy may be difficult to diagnose because of the tendency to mimic the physiologic alterations that take place during pregnancy, especially when early signs of congestive heart failure are subtle.6 Hence, a high index of suspicion is warranted. Unfortunately, no specific diagnostic test for peripartum cardiomyopathy is available currently. Regardless, peripartum cardiomyopathy should be diagnosed after ruling out cardiac and noncardiac causes for dilated cardiomyopathy.6 Because dilatation of the left ventricle can be a normal echocardiographic finding in pregnancy, a documentation of decreased left ventricular systolic function is necessary for the definitive diagnosis of dilated cardiomyopathy.20 Myocardial disorders are conditions that often result in cardiovascular and other major system complications.16,21 Among hospitalizations with myocardial disorders, the rate of severe complications ranged from 13.2 for acute myocardial infarction to 128.6 for adult respiratory distress syndrome and from 10.7 for pulmonary edema to 193.0 for fluid and electrolyte disorders per 1,000 delivery and postpartum hospitalizations, respectively. Among hospitalizations without myocardial disorders, the rate of severe complications ranged from 0.07 to 1.9 and from 0.4 to 65.5 for cardiac arrest and for fluid and electrolyte disorders per 1,000 hospitalizations in delivery and postpartum periods, respectively. Unfortunately, the cross-sectional study design and a lack of clinical details in hospital discharge data did not allow us to determine whether severe obstetric morbidity developed as a complication of myocardial disorders after admission or was a comorbid condition that existed before admission. Nevertheless, our surveillance data indicated that delivery and postpartum hospitalizations with myocardial disorders have extremely high rates of severe obstetric complications. Our study has several limitations that should be considered when interpreting the results. Our identification of severe complications is based solely on ICD-9-CM codes and data-driven criteria, such as mortality, transfer from or to other health care facilities, and duration of stay. There are potential risk factors for cardiomyopathy and other forms of myocardial disorders, such as race, body mass index, and use of tocolytic agents,21 for which we had no information. In addition, ICD-9-CM codes for myocardial disorders among pregnancy hospitalizations were not validated, and it is not known how practicing physicians used diagnostic criteria to diagnose cardiomyopathy. Thus, misclassification of exposure, outcomes, or both is possible in our study. The estimates for myocardial disorders reported in this study may be affected by several sources of error. Because the unit of our analysis was hospitalization and we could not identify the repeat hospitalizations for same patient, our rates may be overestimated. However, underestimation is also possible. Some cases of myocardial disorders with the clinical presentation ranging from mild heart failure to sudden cardiac death may be seen only in emergency departments20 and hence are not associated with an hospital admission. Finally, hospitalizations for peripartum cardiomyopathy that occurred after the 6-week time frame that is defined as the postpartum period and hence were not coded as postpartum hospitalizations may be not captured in our data set.22 In conclusion, the results of this nationwide study have shown that approximately 0.04% of delivery hospitalizations and approximately 4% of postpartum hospitalizations had ICD-9-CM codes for cardiomyopathy and other myocardial disorders. Although only a minority of hospitalizations for cardiomyopathy are consistent with peripartum cardiomyopathy, cardiomyopathy and other myocardial disorders are important contributors to severe obstetric complications. Because recent reports have indicated alarming increases in both maternal mortality from heart disease and the prevalence of risk factors for heart disease among women of reproductive age during the past decade, trends in hospitalizations with myocardial disorders need to be monitored. However, because of the inherent limitations of hospital discharge data, if we are to offer better diagnosis and treatment of this uncommon but potentially devastating condition, the collection of clinically detailed data are essential for making progress in understanding the differences and similarities between the entities currently designated as peripartum cardiomyopathy and other forms of myocardial disorders. 1.Barker D, Lewis N, Mason G, Tan L-B. Maternal cardiovascular medicine: towards better care for pregnant women with heart disease. Br J Cardiol 2006;13:399–404. 2.Whitehead SJ, Berg CJ, Chang J. Pregnancy-related mortality due to cardiomyopathy: United States, 1991–1997. Obstet Gynecol 2003;102:1326–31. 3.Gissler M, Deneux-Tharaux C, Alexander S, Berg CJ, Bouvier-Colle MH, Harper M, et al. Pregnancy-related deaths in four regions of Europe and the United States in 1999–2000: characterisation of unreported deaths. Eur J Obstet Gynecol Reprod Biol 2007;133:179–85. 4.Fett JD. Understanding peripartum cardiomyopathy, 2008. Int J Cardiol 2008;130:1–2. 5.Satpathy HK, Frey D, Satpathy R, Satpathy C, Fleming A, Mohiuddin SM, et al. Peripartum cardiomyopathy. Postgrad Med 2008;120:28–32. 6.Pearson GD, Veille JC, Rahimtoola S, Hsia J, Oakley CM, Hosenpud JD, et al. Peripartum cardiomyopathy: National Heart, Lung, and Blood Institute and Office of Rare Diseases (National Institutes of Health) workshop recommendations and review. JAMA 2000;283:1183–8. 7.Lampert MB, Lang RM. Peripartum cardiomyopathy. Am Heart J 1995;130:860–70. 9.Kuklina EV, Ayala C, Callaghan WM. Hypertensive disorders and severe obstetric morbidity in the United States. Obstet Gynecol 2009;113:1299–306. 10.Kuklina EV, Meikle SF, Jamieson DJ, Whiteman MK, Barfield WD, Hillis SD, et al. Severe obstetric morbidity in the United States: 1998–2005. Obstet Gynecol 2009;113(pt 1):293–9. 11.Gei AF, Hankins GD. Cardiac disease and pregnancy. Obstet Gynecol Clin North Am 2001;28:465–512. 12.Swan L, Lupton M, Anthony J, Yentis SM, Steer PJ, Gatzoulis MA. Controversies in pregnancy and congenital heart disease. Congenit Heart Dis 2006;1:27–34. 13.Healthcare Cost and Utilization Project. Overview of the Nationwide Inpatient Sample (NIS). Available at: www.hcup-us.ahrq.gov/databases.jsp . Retrieved December 23, 2008. 15.Kuklina EV, Whiteman MK, Hillis SD, Jamieson DJ, Meikle SF, Posner SF, et al. An enhanced method for identifying obstetric deliveries: implications for estimating maternal morbidity. Matern Child Health J 2008;12:469–77. 16.Maron BJ, Towbin JA, Thiene G, Antzelevitch C, Corrado D, Arnett D, et al. Contemporary definitions and classification of the cardiomyopathies: an American Heart Association Scientific Statement from the Council on Clinical Cardiology, Heart Failure and Transplantation Committee; Quality of Care and Outcomes Research and Functional Genomics and Translational Biology Interdisciplinary Working Groups; and Council on Epidemiology and Prevention. Circulation 2006;113:1807–16. 17.Mielniczuk LM, Williams K, Davis DR, Tang AS, Lemery R, Green MS, et al. Frequency of peripartum cardiomyopathy. Am J Cardiol 2006;97:1765–8. 18.Brar SS, Khan SS, Sandhu GK, Jorgensen MB, Parikh N, Hsu JW, et al. Incidence, mortality, and racial differences in peripartum cardiomyopathy. Am J Cardiol 2007;100:302–4. 19.Yasmeen S, Romano PS, Schembri ME, Keyzer JM, Gilbert WM. Accuracy of obstetric diagnoses and procedures in hospital discharge data. Am J Obstet Gynecol 2006;194:992–1001. 20.Egan DJ, Bisanzo MC, Hutson HR. Emergency department evaluation and management of peripartum cardiomyopathy. J Emerg Med 2009;36:141–7. 21.Elkayam U, Akhter MW, Singh H, Khan S, Bitar F, Hameed A, et al. Pregnancy-associated cardiomyopathy: clinical characteristics and a comparison between early and late presentation. Circulation 2005;111:2050–5. © 2010 by The American College of Obstetricians and Gynecologists. 22.Howard A. Review of pregnancy coding guidelines. For the Record 2009;21:31.	4	10	0	0	0	6	0.90487	6	5,496
Get the facts about bipolar disorder, including the different types and symptoms of each. - Bipolar Disorder Facts about bipolar disorder, including different types and symptoms. - ADHD Attention Deficit Hyperactivity Disorder (ADHD), including the different types and who gets them. - Adult ADHD Facts about attention deficit hyperactivity disorder (ADHD) in adults. - Alzheimer's Disease Facts about Alzheimer’s Disease, including the symptoms and stages. - Autism Spectrum Disorder Get the facts about Autism Spectrum Disorder (ASD). - Chronic Pain Facts about chronic pain, including body parts most commonly affected. - Clinical Depression Clinical depression facts; symptoms & other depressive disorders. - Depression Facts about depression, including the symptoms of the condition. - Diabetic Neuropathy Facts about diabetic neuropathy, including the symptoms and doctors. - Epilepsy Facts about epilepsy, including different types, symptoms and causes. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides About Dr. Benjamin L Walter Dr. Benjamin L Walter, MD is a Doctor primarily located in Cleveland, OH. He has 19 years of experience. His specialties include Neurology and Psychiatry. Dr. Walter is affiliated with University Of Cincinnati Medical Center. Dr. Walter has received 5 awards. He speaks English. Dr. Benjamin L Walter has the following 2 specialties A neurologist is a physician who diagnoses and treats disorders of the nervous system which is comprised of the brain, spinal cord and nerves. These doctors do not perform surgery, but refer patients to neurological surgeons when they determine that surgical intervention is necessary. Some of the conditions that neurologists diagnose and treat are epilepsy, aneurysms, hydrocephalus, Parkinson's disease, multiple sclerosis, stroke, spinal disc herniation, and spinal disease. In addition to using diagnostic tests like MRI, CT scans, EEG and EMG, neurologists also employ neurological testing to gauge muscle strength and movement, balance, reflexes, sensation, memory, speech, and other cognitive abilities. A psychiatrist is a doctor with specific training in the diagnosis and treatment of mental illness. He or she can not only provide the counseling necessary to both diagnose and treat a patient, but can also prescribe medication when needed. In some cases, a psychiatrist will only provide the medication and the counseling will be provided by another healthcare specialist, like a certified counselor or psychologist. Like other doctors, psychiatrists employ diagnostic tools like CT scans and MRI in order to observe the structure and function of a patient's brain. Once a diagnosis is made, these specialists may use behavior or cognitive therapy in order to address the patient's condition, or a multitude of other types of therapy, in conjunction with or in place of medication. - Dystonic Disorders - Multiple Sclerosis (MS) - Migraine Disorder - Nerve Conduction Studies - Movement Disorders - Endoscopic Deep Brain Simulation (DBS) - Restless Legs Syndrome - Alzheimer's Disease - Parkinson Disease See the board certifications this doctor has received. Board certifications provide confidence that this doctor meets the nationally recognized standards for education, knowledge and experience. Showing 5 of 20 I would never recommend this doctor to anyone - he screwed up my medications (I have Parkinson's) to the point where I am having panic attacks daily. It took 3 days of phone calls to finally get to speak with him and he was very confused as to who I was, when he saw me last and what he had prescribed. HORRIBLE EXPERIENCE! Dr. Walter was fantastic! Brought my husband in to be evaluated for Parkinson's. Dr. Walter answered all of our questions. He kept probing to get full answers to come to his diagnosis. Wonderful doctor - would recommend him to anyone. Compassionate Doctor Recognition (2015) Compassionate Doctor certification is granted to physicians who treat their patients with the utmost kindness. The honor is granted based on a physician's overall and bedside manner scores. On-Time Doctor Award (2015) Vitals On-Time + Promptness Award recognizes doctors with consistent high ratings for timeliness of appointments. The honor is granted based on a physician's overall and promptness scores. Top 10 Doctor - Metro Area (2014) Top 10 Doctors are chosen by the millions of patients who visit Vitals each year to find a new doctor and share their experiences by providing ratings and reviews. In order to differentiate highly-regarded doctors from the rest for patients in search of quality care, Vitals awards Top 10 Doctor honors to those physicians within a certain specialty and geographic area who are consistently given top ratings by their patients. Patients' Choice Award (2015) Patients' Choice recognition reflects the difference a particular physician has made in the lives of his/her patients. The honor is bestowed to physicians who have received near perfect scores, as voted by patients. Regional Top Doctors Castle Connolly is America's trusted source for the identification of Top Doctors. Their physician-led research team reviews and screens the credentials of tens of thousands of physicians who are nominated by their peers annually, via a nationwide online process, before selecting those physicians who are regionally or nationally among the very best in their medical specialties. Castle Connolly believes strongly that Top Doctors Make a Difference™. Dr. Walter is affiliated (can practice and admit patients) with the following hospital(s). 19 Years Experience Drexel University College Of Medicine Graduated in 1999 Dr. Benjamin L Walter accepts the following insurance providers. - Aetna Choice POS II - Aetna HMO - Aetna Health Network Option Open Access - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO - Anthem Blue Access PPO - Anthem Blue Preferred HMO BCBS Blue Card - BCBS Blue Card PPO - BCBS IL PPO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - First Health PPO - HealthSpan Access PPO - Humana Choice POS - Humana ChoiceCare Network PPO Medical Mutual of Ohio - MMOH SuperMed POS Select - MMOH SuperMed PPO Plus - Multiplan PPO - PHCS PPO - SummaCare Select Fully Insured - View by Location Locations & DirectionsUh Cleveland Medical Center, 11100 Euclid Ave, Cleveland, OH Take a minute to learn about Dr. Benjamin L Walter in this video. Dr. Benjamin L Walter is similar to the following 3 Doctors near Cleveland, OH.	2	8	1	0	0	0	0.905885	1	1,380
Patient professional reference Erythema nodosum is presumed to be a hypersensitivity reaction. It is often a dermatological manifestation of infectious, or other, disease. The eruptive phase begins with fever, aching and arthralgia whilst a painful rash usually appears within a couple of days. Lesions begin as red, tender nodules. The borders are poorly defined and they are 2-6 cms in diameter. In the first week the lesions become tense, hard and painful. In the second week, they may become fluctuant, rather like an abscess but they do not suppurate or ulcerate. Individual lesions last around two weeks but occasionally, new lesions continue to appear for three to six weeks. Aching legs and swollen ankles may persist for many weeks. In the first week they are bright red but in the second week there is a blue or purple hue, even turning yellow like a resolving bruise before disappearing in a couple of weeks. They can occur anywhere but are usually on the anterior aspect of the lower leg. When the aetiology is an infection the lesions usually heal in six to eight weeks but 30% of idiopathic cases last six months. Arthralgia occurs in more than half of patients and begins either during the eruptive phase or two to four weeks before. Joints are red, swollen and tender, sometimes with effusions. Morning stiffness may occur. The ankles, knees and wrists are most often involved. Synovitis resolves in a few weeks but joint pain and stiffness may last up to six months. There are no destructive changes in the joint and synovial fluid is acellular and the rheumatoid factor is negative. - Erythema nodosum is often indicative of an underlying infectious disease but a cause is not always found. Some underlying causes are not infectious. - Streptococcal infection is the most common underlying cause and so it may be a feature of other diseases, including scarlet fever and rheumatic fever, although in the UK nowadays the former is uncommon and the latter rare. - Sarcoidosis is also commonly involved in adults, although it is not infectious in origin. - Tuberculosis must be considered. - Leprosy can produce a clinical picture of erythema nodosum, although the histological picture of the lesions is different. - Various forms of gastroenteritis - especially Yersinia enterocolitica, Salmonella spp. and Campylobacter spp. - can be associated. - Lymphogranuloma venereum may be a cause. - Mycoplasma pneumonia can be associated. - Fungal infections are less common in the UK but coccidioidomycosis is important in Southwest USA. It may occur in histoplasmosis and blastoplasmosis. - Sulfonamides are used less often nowadays but other drugs to be implicated include sulfonylureas, gold and oral contraceptives. - It may correlate with flare-up of Crohn's disease or ulcerative colitis. - It can precede the diagnosis of Hodgkin's lymphoma and non-Hodgkin's lymphoma by months and it can accompany Behçet's syndrome. - It may occur in pregnancy when it is usually in the second trimester. It is likely to recur in future pregnancies and may occur with oral contraceptives. - There are rare cases (<1 in 100) associated with Epstein-Barr virus, hepatitis B and hepatitis C and HIV. - In many cases no cause is found. Although in many cases it is idiopathic, it is important to exclude serious underlying disease: - A throat swab for streptococcus is the first test, although it may well be negative, even with streptococcal disease. - Anti-streptococcal O (ASO) titre may be more helpful, although a normal titre does not exclude infection. A rising titre may be more valuable. - Arrange an FBC and ESR. ESR is often very high regardless of the aetiology, and CRP may be more contributory. - Stool examination for Y. enterocolitica, Salmonella spp. and Campylobacter spp. may yield results, as may blood cultures - In sarcoidosis, calcium and angiotensin-converting enzyme (ACE) are often raised. - CXR may show bilateral hilar lymphadenopathy (BHL) in sarcoidosis, unilateral or asymmetrical adenopathy in malignancy, or evidence of pulmonary tuberculosis. - Intradermal skin tests may be required to exclude tuberculosis and coccidioidomycosis. - Excisional biopsy may be helpful where the diagnosis is in doubt. - Erythema induratum (modular vasculitis). - Insect bites. - Acute urticaria. - Familial Mediterranean fever. - Superficial thrombophlebitis (standard or superficial migratory thrombophlebitis). - Most cases are self-limiting and require only symptomatic relief. - If an infective aetiology has been discovered then appropriate therapy is in order but it should not be given blind. - A degree of relief can be obtained with cool compresses and bed rest with elevation of the foot of the bed. Bed rest has been advocated for many years and is anecdotally useful but the evidence base is lacking. - Non-steroidal anti-inflammatory drugs (NSAIDs) are useful and no other drugs are usually needed. Steroids are beneficial but should be used with caution and may be contra-indicated if infection has not been excluded. - In difficult cases, oral potassium iodide may be valuable, as may tetracycline and, in erythema nodosum of leprosy, thalidomide has seen a resurgence but further research is required[10, 11, 12]. The condition usually resolves within six weeks but it may be more protracted, especially if the underlying cause remains or when it is idiopathic. Serious complications are unusual unless part of the underlying disease. Chronic or recurrent disease is rare. Lesions heal without atrophy or scarring. Further reading and references Shimizu M, Hamaguchi Y, Matsushita T, et al; Sequentially appearing erythema nodosum, erythema multiforme and Henoch-Schonlein purpura in a patient with Mycoplasma pneumoniae infection: a case report. J Med Case Rep. 2012 Nov 236(1):398. doi: 10.1186/1752-1947-6-398. Erythema Nodosum; Primary Care Dermatology Society Whig J, Mahajan V, Kashyap A, et al; Erythema nodosum: Atypical presentation of common disease. Lung India. 2010 Jul27(3):181-2. doi: 10.4103/0970-2113.68319. Min MS, Fischer R, Fournier JB; Unilateral Erythema Nodosum following Norethindrone Acetate, Ethinyl Estradiol, and Ferrous Fumarate Combination Therapy. Case Rep Obstet Gynecol. 20162016:5726416. doi: 10.1155/2016/5726416. Epub 2016 Mar 27. Mantadakis E, Arvanitidou V, Tsalkidis A, et al; Erythema nodosum associated with Salmonella enteritidis. Hippokratia. 2010 Jan14(1):51-3. Babamahmoodi F, Babamahmoodi A, Barani H, et al; Simultaneous occurrence of erythema nodosum in monozygotic twin sisters. Case Rep Med. 20122012:109427. doi: 10.1155/2012/109427. Epub 2012 Jun 5. Schwartz RA, Nervi SJ; Erythema nodosum: a sign of systemic disease. Am Fam Physician. 2007 Mar 175(5):695-700. Fowler A, Dargan P, Jones A; Puzzling hypercalcaemia: sarcoidosis without lung involvement. J R Soc Med. 2005 Feb98(2):60-1. Yi SW, Kim EH, Kang HY, et al; Erythema nodosum: clinicopathologic correlations and their use in differential diagnosis. Yonsei Med J. 2007 Aug 3148(4):601-8. Tabak F, Murtezaoglu A, Tabak O, et al; Clinical features and etiology of adult patients with Fever and rash. Ann Dermatol. 2012 Nov24(4):420-5. doi: 10.5021/ad.2012.24.4.420. Epub 2012 Nov 8. Passarini B, Infusino SD; Erythema nodosum. G Ital Dermatol Venereol. 2013 Aug148(4):413-7. Van Veen NH, Lockwood DN, van Brakel WH, et al; Interventions for erythema nodosum leprosum. Cochrane Database Syst Rev. 2009 Jul 8(3):CD006949. doi: 10.1002/14651858.CD006949.pub2. Gilchrist H, Patterson JW; Erythema nodosum and erythema induratum (nodular vasculitis): diagnosis and management. Dermatol Ther. 2010 Jul-Aug23(4):320-7. Wan P, Zhao X, Hunasehally RY, et al; Propylthiouracil-induced ANCA-positive erythema nodosum treated with thalidomide. Int J Dermatol. 2012 Mar51(3):345-8. doi: 10.1111/j.1365-4632.2011.05135.x. I have had EN for 5.5 months and it has gotten worse. I haven’t had 1 day without nodules. What is the longest outbreak you have had?ak52 Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. Patient Platform Limited has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.	2	6	2	0	0	0	0.580622	2	2,271
New Cpt codes for Antegrade Pyelogram(Nephrostogram) basics What is Antegrade Pyelogram? Antegrade Nephrostogram is also called Antegrade Pyelogram. It is a study of x-rays to diagnose the obstruction of the urinary tract. In Antegrade Pyelogram, the contrast dye is injected in the ureter, which carries the urine from the kidneys to the bladder. The flow of contrast is then studied with the x-rays images once it starts flowing from the kidneys into the ureters and urinary bladder. Read also: New CPT code for AV fistula Access 36901 Need of Antegrade Pyelogram Antegrade Pyelogram is used as an alternative when we don’t get any specific information after doing retrograde pyelogram or intravenous pyelogram. The antegrade pyelogram helps in the detecting the obstruction in the urinary tract. The obstruction can be due to stricture or narrowing of tract, kidney stone, tumor etc. So, whenever there is an obstruction, the contrast dye injected will be having problem to flow from kidneys to urinary bladder, which is getting detected in the x-ray images. image courtesy: http://radiopaedia.org/ Antegrade pyelogram is also useful in evaluating kidneys and ureters before or after the surgery. If there is obstruction in urinary tract, a nephrostomy tube is place to divert the flow of kidney directly from the kidneys to the abdominal wall. Nephrostomy tubes are placed within the kidney to collect the urine directly from the kidney as it is generated. Antegrade Pyelogram procedure At first the patient will be injected with local anaesthetic agent. Now, with the help of an imaging guidance a needle will be inserted in the kidney, till it reaches to renal pelvis. After reaching renal pelvis, the contrast dye is injected. Now, a series of x-rays will be taken through the ureters for study of urinary tract. Now, a guidewire is inserted through the needle to allow the placement of nephrostomy tube or catheter. Once the guidewire is placed, the needle is removed and then the catheter is placed along the guidewire. Once the catheter is placed, guidewire is also removed. After the placement, a sterile dressing will be applied. New CPT Codes for Nephrostogram in 2016 50430 – Injection procedure for antegrade nephrostogram and/or ureterogram, complete diagnostic procedure including imaging guidance (eg, ultrasound and fluoroscopy) and all associated radiological supervision and interpretation; new access 50431 ; existing access 50684- Injection procedure for ureterography or ureteropyelography through ureterostomy or indwelling ureteral catheter 50690 – Injection procedure for visualization of ileal conduit and/or ureteropyelography, exclusive of radiologic service 50435- Change of nephrostomy or pyelostomy tube (RS&I is bundled) 50387- Removal and replacement of externally accessible nephroureteral catheter (eg, external/internal stent) requiring fluoroscopic guidance, including radiological supervision and interpretation 50688 – Change of ureterostomy tube or externally accessible ureteral stent via ileal conduit (use CPT code 75984 as S&I ) 50382- Removal (via snare/capture) and replacement of internally dwelling ureteral stent via percutaneous approach, including radiological supervision and interpretation 50385 -Removal (via snare/capture) and replacement of internally dwelling ureteral stent via transurethral approach, without use of cystoscopy, including radiological supervision and interpretation 50386 -Removal (via snare/capture) of internally dwelling ureteral stent via transurethral approach, without use of cystoscopy, including radiological supervision and interpretation 50389 -Removal of nephrostomy tube, requiring fluoroscopic guidance (eg, with concurrent indwelling ureteral stent) Old CPT codes used for Antegrade Pyelogram For diagnostic antegrade pyelogram and Nephrostomy tube placement following cpt codes are used. Some of the CPT codes have been deleted in 2016 and New CPT codes have replaced them. 50390- for diagnostic antegrade pyelogram 50392- for placement for nephrostomy tube for drainage and/or injection percutaneous. 50394- for diagnostic antegrade pyelogram through the already placed catheter. 50393– for the placement of ureter catheter or stent into ureter through renal pelvis for drainage and/or injection percutaneous. The supervision and interpretation (S&I) and imaging guidance codes used along with above cpt codes are 74425, 74470, 76942, 77002, 77012 or 77021. When the findings are given with procedure performed we have to code the S&I code. And when the guidance is used we have to code the guidance code. The guidance codes are included with the S&I code, hence when we are coding S&I code we are not supposed to code the guidance code with them. Hope, now you can answer any question regarding pyelogram, if you like the article do share it.	4	11	11	0	0	0	0.608456	11	1,155
Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Birth Control Facts about birth control to help decide which type is right for you. - Type 2 Diabetes Basic facts about type 2 diabetes & risk factors to be aware of. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Flu Facts about influenza (flu), including symptoms and vaccines. - GERD Get the facts about gastroesophageal reflux disease (GERD). - Lupus Get the facts about lupus, including symptoms, risk factors, and the different types. - Menopause Facts about menopause, including the stages, symptoms, and types. - Ulcerative Colitis Facts about ulcerative colitis, including causes, signs and symptoms. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides Dr. Qiong Wu has the following 2 specialties - Internal Medicine An internist is a physician who focuses on the diagnosis and treatment of conditions that affect the adult population—both acute and chronic. These doctors are often who adults see as their primary physicians because they treat a broad range of illnesses that do not require surgical or specialist interventions. They also work to help a patient maintain optimal health in order to prevent the onset of disease. In addition to treating the common cold and flu, internists also treat chronic diseases like diabetes and heart disease. - Legal Medicine Legal medicine specialists advise on a variety of laws and regulations regarding health care and public health. This include disability claims, hospital law, privacy laws and physicians’ obligations and liabilities. They typically have a medical degree and a law degree so they're able to address specific legal issues that apply to medical professionals, hospitals and clinics. Compared to forensic medicine, which deals with determining cause of death in criminal investigations, they deal with issues that impact patient care and they’re also typically involved in assessing illegal substance use in athletes. See the board certifications this doctor has received. Board certifications provide confidence that this doctor meets the nationally recognized standards for education, knowledge and experience. Showing 1 of 1 Bridges to Excellence: Physician Office Systems Recognition Program This program is designed to recognize practices that use information systems to enhance the quality of patient care. To obtain Recognition, practices must demonstrate that they have implemented systematic office NCQA Patient-Centered Medical Home The patient-centered medical home is a way of organizing primary care that emphasizes care coordination and communication to transform primary care into what patients want it to be. Medical homes can lead to higher quality and lower costs, and can improve patients' and providers' experience of care. Dr. Qiong Wu accepts the following insurance providers. - Aetna Choice POS II - Aetna Elect Choice EPO - Aetna HMO - Aetna Managed Choice POS Open Access - Aetna NYC Community Plan - Aetna Signature Administrators PPO BCBS Blue Card - BCBS Blue Card PPO - BCBS MA Blue Care Elect PPO - BCBS MA Preferred Blue PPO Capital District Physicians Health Plan - CDPHP New York State of Health - Small Business - CDPHP PPO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - Connecticare Flex Connecticut - Empire Blue Priority EPO - Empire HMO - Empire PPO - Empire Prism EPO Blue Priority - First Health PPO - Healthfirst NY HMO ABCD - Humana ChoiceCare Network PPO MVP Health Plan - MVP Preferred PPO - Multiplan PPO - Oxford Freedom - Oxford Liberty - Oxford Metro - View by Location Locations & DirectionsIvy Medical Services Pc, 13527 38th Ave Ste 338, Flushing, NY Dr. Qiong Wu is similar to the following 3 Doctors near Flushing, NY.	2	4	1	0	0	0	0.816935	1	849
- Atypical moles; moles whose appearance is different from that of common moles. Dysplastic nevi are generally larger than ordinary moles and have irregular and indistinct borders. Their color frequently is not uniform and ranges from pink to dark brown; they usually are flat, but parts may be raised above the skin surface. English dictionary of cancer terms . 2015. Look at other dictionaries: Dysplastic nevi — Atypical moles; moles whose appearance is different from that of common moles. Dysplastic nevi are generally larger than ordinary moles and have irregular borders. Their color often is not uniform; they usually are flat, but parts may be raised… … Medical dictionary familial dysplastic nevi — A condition that runs in certain families in which at least two members have dysplastic nevi (atypical moles) and have a tendency to develop melanoma … English dictionary of cancer terms Dysplastic nevus — Classification and external resources ICD 10 D48.5 (ILDS D48.540) ICD 9 238.2 … Wikipedia dysplastic nevus syndrome — the occurrence of dysplastic nevi in persons having or at risk for having familial malignant melanoma. Called also atypical mole s., atypical nevus s., familial atypical moleâ€“malignant melanoma s., and FAMMM s … Medical dictionary Nevi and melanomas — Classification and external resources MeSH D018326 Nevi and melanomas are a group of neoplasia. Although a nevus and a melanoma are often treated as independent entities, there is evidence that a nevus can be a precursor for a melanoma … Wikipedia Melanocytic nevus — Classification and external resources Mole, more specifically an intradermal nevus ICD 10 D22 … Wikipedia Melanoma — Classification and external resources A melanoma ICD 10 C43 … Wikipedia Nevus — A pigmented spot on the skin, such as a mole. The plural of nevus is nevi. * * * 1. A circumscribed malformation of the skin, especially if colored by hyperpigmentation or increased vascularity; a n. may be predominantly epidermal, adnexal,… … Medical dictionary List of oncology-related terms — This is a list of terms related to oncology. The original source for this list was the U.S. National Cancer Institute s public domain Dictionary of Cancer Terms . NOTOC 1 * 10 propargyl 10 deazaaminopterin * 12 O tetradecanoylphorbol 13 acetate * … Wikipedia Nevus — Not to be confused with Naevius, Nevis, or Nevius. Naevus and Nevi redirect here. For the British experimental rock group, see Naevus (band). For the Eritrean footballer, see Nevi Ghebremeskez. For the Norwegian bank, see Nevi (company). Nevus… … Wikipedia	3	6	0	0	0	4	0.581263	4	645
As October 1st, 2015 approaches, we as healthcare providers are all preparing for the new ICD-10 codes and some of us, including myself have been using them for months. As a reference, ICD stands for International Classification of Disease. In 1990 the WHO (Would Health Organization) approved the 10th revision of the ICD – known as ICD-10. The purpose was to create a modified version in order to more accurately analyze health information due to a multitude of changes in disease states over the past several years. Here are some interesting facts: • Greater than 100 countries use ICD codes to help determine death and disease rates • Approximately 70% of the world’s health expenditures are allocated by using ICD for reimbursement • ICD-11 revision is scheduled to be released in 2018 • The first ICD edition (at which time was referred to as the International List of Causes of Death) was implemented in 1893 There are currently 13,000 codes with ICD-9, this will increase to 68,000 codes with ICD-10 version. The reason for significant more codes is to better specify the disease state for each individual patient. ICD-10 is required for everyone covered by HIPPA (Heath Insurance Portability Accountability Act). ICD-9 codes have been used for decades and with significant changes in medicine and diagnoses it was necessary for new codes to be implemented. The ICD-10 codes are much more specific – often times grouped as a “family of codes.” For example: A patient presents with Asthma. With the ICD 9 codes for asthma there were 14 options to specify asthma. However with the new ICD-10 codes, in order to correctly describe asthma, providers must document the following: • Cause of Asthma o Exercise induced o Related to smoking o Cough variant • Severity of Asthma o Mild intermittent o Mild persistent o Moderate persistent o Severe persistent • Temporal Factors o Status asthmaticus o Acute exacerbation J45.30 = mild persistent asthma, uncomplicated J45.991 = cough variant asthma	4	4	0	0	0	2	0.902467	2	451
What is an HCC? An HCC is a Hierarchical Condition Category which is used to calculate risk scores that may predict the future cost of healthcare for enrollees. HCCs were implemented by the Centers for Medicare and Medicaid (CMS) and have been adopted by Medicare Advantage Plans. Medicare Advantage Plans engage in prospective reviews to ensure accurate coding and payments. HCCs are used to ensure payers are properly compensated for the overall cost of care for each member. Accurate HCCs involve using the CMS-HCC model to determine the appropriate diagnosis codes. It is important for plans to review and make sure the appropriate HCC category is supported in the records and accurately reported to CMS. The Office of the Inspector General (OIG) found that 12 health conditions drove billions in risk-adjusted payments for 20 Medicare Advantage companies. One of those health conditions included Diabetes with Chronic Complications which is represented by HCC 018. Examples of ICD-10 codes included in HCC 018 are E08.21 (Diabetes mellitus due to underlying condition with diabetic nephropathy) and E13.65 (Other specified diabetes mellitus with hyperglycemia). HCC 019 (Diabetes without Complication) examples include ICD-10 codes such as E08.9 (Diabetes mellitus due to underlying condition without complications) and E10.9 (Type 1 diabetes mellitus without complications). What to look for? According to AHIMA, the top 10 Medicare Risk Adjustment Errors are : Plans can monitor specific HCCs of concern such as Diabetes by looking for - Providers billing for more patients in HCC 018 vs. HCC 019 - Reviewing data submitted to CMS and ensuring it’s accurate AI and Alerts Healthcare Fraud Shield monitors several HCCs that can be identified using both Artificial Intelligence models in AIShield and alerts in PostShield. One example of a PostShield alert is: [3066-20] – UPCODING, HCC 018, DIABETES WITH CHRONIC COMPLICATIONS If you have questions or comments you may email us at [email protected].	4	6	0	0	0	4	0.858282	4	458
Let’s look at the medical term “Arthrocentesis”. By breaking up the term into two sections, we find that “arthros” stands for “joint” and “kentesis” means “puncture”. So what is Arthrocentesis? Medical Coding Arthrocentesis When a healthcare provider surgically punctures a joint with a needle and withdraws (aspirates) synovial fluid (a gelatinous fluid found in the cavities of synovial joints which reduces friction between the articular cartilages and synovial joints during movement), or injects a synthetically produced anti-inflammatory such as cortisone to relieve arthritic pain, this is referred to as Arthrocentesis. There are three (3) CPT codes you can choose for Arthrocentesis: 20600: Arthrocentesis, aspiration and/or injection of a small joint or bursa. A bursa is a small fluid filled sac lined by synovial membrane that provides a cushion between bones and tendons and/or muscles around a joint. Infection or irritation of a bursa leads to bursitis (inflammation of a bursa). Fingers and toes are considered small joints. 20605: Arthrocentesis of the intermediate joint or bursa. Examples of intermediate joints are: the temporomandibular joint which is the joint of the jaw, often referred to as TMJ, acromioclavicular joint which is also referred to as the AC joint at the top of the shoulder between the acromion (part of the scapula) and the clavicle, wrist, elbow, and olecranon bursa, also known as elbow bump, water on the elbow or baker’s elbow. 20610: Arthrocentesis of a major joint such as a shoulder, hip, knee joint or subacromial bursa (the synovial membrane located just below the acromion). Typically, when coding for bilateral Arthrocentesis, you would append modifier 50 to one Arthrocentesis procedure code. For example, bilateral knees would be coded as 20610-50. For multiple joint procedures, you would add modifier 59 to each additional Arthrocentesis procedure site (excluding bilateral locations). If more than one (1) injection is entered into the same site, only bill the CPT code as one (1) unit. CPT codes 20600, 20605, and 20610 do not have a global period. If a patient sees the provider on day one (1) and he decides to perform Arthrocentesis on the same day, he would add modifier 25 to the E/M visit (separate identifiable service) and use the appropriate injection/aspiration code. If the patient returns on day two (2) for another injection to the same location, the coder should not bill another E/M visit but he CAN bill for another Arthrocentesis procedure. Only bill for the surgical injection/aspiration. If the provider knows the patient will have to return for multiple injections over a period of time, then modifier 58, staged or planned procedure, will indicate to the payer that the procedure is indicative for this particular case. Remember that coding rules change on a regular basis and you want to be sure your claim tells a story. LCDs and NCDs can affect your reimbursement if over utilized, so be clear at all times. Procedures performed on fingers and toes must include the location modifiers. For example, modifiers TA through T9 refer to each digit on the foot. In addition to the Arthrocentesis code, coders should always include the appropriate HCPCS “J” code to get reimbursed for the cost of the drug or biological used in the Arthrocentesis procedure if performed in a non-facility setting. The “J” code is identified by the generic name – not the brand name, but the descriptions are cross-referenced in the HCPCS code book. Be aware that payers’ rules may vary on bilateral coding. Maintain a list of who wants modifier 50 or LT, RT. Always sequence your modifiers by using your pricing modifier first, such as modifier 50, payment modifier second, such as 58, 59 or 51, and location modifiers last, such as FA, F1-F9, TA, T1-T9. Again, pay attention to how payers handle your claims and make note. More Medical Coding Arthrocentesis Related Posts: Submit your review	4	4	7	0	0	0	0.252891	7	972
When it comes to healthcare solutions, several services are offered by the health industry. Also, several terms are associated with it and have unique characteristics. For instance, ICD-9-CM is the International Classification of Diseases, ninth revision, and clinical modification is based on the World Health Organization’s Ninth Revision, International Classification of Diseases (ICD-9). It assigns codes to diagnoses and procedures related to hospital utilization in the United States. ICD-9 consists of a list containing a numerical list of the disease code numbers in a tabular form. Furthermore, it consists of a classification system for diagnostic, surgical, and therapeutic procedures. It includes an alphabetical index to the disease entries. It provides a format for reporting causes of death on the death certificate. The conditions are then converted into medical codes through the classification and structure modification rules that are embedded in the ICD, published by WHO. Similarly, ICD-10 is the 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD). It is a medical classification list by the WHO. It includes codes, abnormal findings, social circumstances, and other injuries and diseases. It was in 1983 when the work on ICD-10 began and then endorsed by the Forty-Third World Health Assembly in 1990. It was first used by members in 1994. It will be replaced by ICD-11 in 2021. As of 2020, there have been ten revisions of ICD. ICD-1 covered 1900 to 1909, ICD-2 covered 1910 to 1920, and so on. ICD-10 is from 1999 to the present. ICD periodically incorporates changes in the medical field. The WHO manages and publishes the base version of ICD. The members modified it to suit their needs. ICD-9-CM and ICD-10-CM Medical Diagnosis Codes The International Statistical Classification of Diseases and Related Health Problems provides alpha-numeric codes to classify several diseases and a variety of complaints, social circumstances, and external causes of disease. Every health condition is assigned to a different category and is given a code that is six characters long. The ICD-9-CM system is used in different avenues of healthcare to report diagnosis. The current ICD-9-CM have over 13000 codes. And the ICD-10-CM is expanding to over 68000 codes and can be further expanded. The ICD-9-CM has up to three to five characters. The ICD-10-CM has alphanumeric three to seven characters. How ICD-9 and ICD-10 Differ? The ICD-10 sets have changes in the structure that makes it different from ICD-9. Issues that occurred in ICD-9 are addressed in ICD-10. ICD-19 has a lack of specificity of the information in the codes. For instance, if a person has visited for the treatment of a burn on one arm (left), then this code couldn’t distinguish that burn is on which arm (left arm). If the same patient comes for another burn on the right arm, the same code would be displayed. Whereas, in the ICD-10, the code can identify where the burn is and other clinical information. Another problem with ICD-9 is that some chapters allow you to add new codes. New codes make it difficult to identify and recognize all available codes. On the other hand, the character length of ICD-10 is increased and has different codes for future use. Diagnosis Code Sets of ICD-9 and ICD-10 1. The codes in ICD-9 has three to five characters in length. The codes in ICD-10 has three-seven characters in length. 2. The first digit in ICD-9 can be alpha (E or V) or numeric (2-5). The first digit in ICD-10 is alpha, digit two, and three are numeric. Digits 4-7 are alpha or numeric. 3. ICD-9 has limited spaces to add new codes whereas, ICD-10 has flexible space for new codes. 4. In ICD-9, some codes require 4th and 5th digits. In ICD-10, full code titles, no 4th and 5th digits. Addition of 6th character in some chapters. Addition of code extensions via 7th character for injuries and external causes of injury. 5. In ICD-9, Etiology/manifestations coded are separately written. In ICD-10, Etiology/manifestation codes are combined. 6. ICD-9 codes lack detail and laterality. And ICD-10 is extremely specific and has laterality. The alpha characters in the ICD-10 are not case sensitive. Here’s an example of ICD-9 and ICD-10 codes. 382.9 Acute otitis media 540.9 Acute appendicitis ICD-10 Diagnosis Code B01.2 Varicella pneumonia K21.0 Gastro-esophageal reflux disease with esophagitis O30.003 Twin pregnancy, unspecified, third trimester. Code Structure of ICD-9-CM It has a minimum of three digits, maximum of five digits; decimal after 1st three digits: For example: X X X . X X Code Structure of ICD-10-CM In ICD-10-CM, character X is used as a dummy placeholder character. It can be used as a 5th character for six characters codes or when a code has less than six characters. Example 1: T37.0x1A Poisoning by sulfonamides. Example 2: S01.02xA Laceration of the scalp with Foreign Body scalp, initial encounter. The guidelines for the use of the ICD-CM-10 have been approved by the parties for ICD-10-CM: the American Hospital Association, Centres for Medicare & Medicaid Services. A few common guidelines are: 1. ICD-10-CM diagnosis codes are adopted under HIPAA for all healthcare settings. 2. Using appropriate codes to know the symptoms, complaints, and reasons for the encounter visit. 3. Signs and symptoms associated with the disease should not be coded unless the classification instructs otherwise. There may be multiple codes to describe a single condition. In the combination codes, the single code is used to identify two diagnoses or a diagnosis with an associated complication. 27 Countries are Using ICD-10 and Resource Allocation in the Healthcare System It is estimated that 27 countries use ICD-10 and resource allocation in their health system. The unchanged international version of ICD-10 is used in over 117 countries. The national version may differ from the base classification in the level of detail or the addition of procedure codes. In Australia, it was introduced in 1998. The ICD-10 Australian Modification was developed by the National Centre for classification in Health at the University of Sydney. It is maintained by the Australian Consortium for Classification Development. In China, the ICD-10 was adopted in 2002. In Germany, ICD-10 German Modification is based on ICD-10 AM. The ICD-10 - TM (Thai Modification) is a Thai language version. It is based on the 2016 ICD-10. In the United Kingdom, the ICD-10 was first mandated for use in the UK in 1995. It was in 2010 when the UK Government made an update to the UK version of ICD-10 every three years. We, at Covetus, have a team of technology experts who have experiences in working on different healthcare standards like DICOM , and more. We have an engineering team that has hands-on experience of the latest technologies and knows how to help grow your business.	4	8	4	0	0	5	0.594214	9	1,645
- Periosteal reaction Name = PAGENAME DiseasesDB = 31520 A periosteal reaction is the formation of new bonein response to injuryor other stimuli of the periosteumsurrounding the bone. It is most often identified on X-rayfilms of the bones. A periosteal reaction can result from a large number of causes, including injury and chronic irritation due to a medical condition such as hypertrophic osteopathy, bone healingin response to fracture, chronic stress injuries, subperiosteal hematomas, osteomyelitis, and cancerof the bone. It may occur as part of thyroid acropachy, a severe sign of the autoimmune thyroid disorder Grave's disease. The morphological appearance can be helpful in determining the cause of a periosteal reaction (for example, if other features of periostitisare present), but is usually not enough to be definitive. Diagnosis can be helped by establishing if bone formation is localized to a specific point or generalized to a broad area. The appearance of the adjacent bone will give clues as to which of these is the most likely cause. Hypertrophic pulmonary osteoarthropathy Wikimedia Foundation. 2010. Look at other dictionaries: reaction — 1. The response of a muscle or other living tissue or organism to a stimulus. 2. The color change effected in litmus and certain other organic pigments by contact with substances such as acids or alkalies; also the property that such substances… … Medical dictionary Ewing's sarcoma — Infobox Disease Name = Ewing sarcoma Caption = X Ray of a child with Ewing sarcoma of the tibia DiseasesDB = 4604 ICD10 = ICD9 = ICD9\|170.9 ICDO = 9260/3 \| OMIM = 133450 MedlinePlus = 001302 eMedicineSubj = ped eMedicineTopic = 2589 MeshID =… … Wikipedia Nail clubbing — Clubbing Clubbing of the fingernail. The red line shows the outline of a clubbed nail. ICD 10 R68.3 ICD 9 … Wikipedia Triceratops — Nephrozoa Triceratops Temporal range: Late Cretaceous, 68–65 Ma … Wikipedia Splints — is an ailment of the horse or pony, characterized by a hard, bony swelling, usually on the inside of a front leg, lying between the splint and cannon bone or on the splint bone itself. It may be hot, meaning that it occurred recently and is still … Wikipedia Osteofibrous dysplasia — is a rare, benign non neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. In 1942, however, Jaffe and Lichtenstein… … Wikipedia Mastication — Chew and Chewing redirect here. For other uses, see Chew (disambiguation). A monkey using mastication to process tough plant matter Mastication or chewing is the process by which food is crushed and ground by teeth. It is the first step of… … Wikipedia Osseointegration — derives from the Greek osteon, bone, and the Latin integrare, to make whole. The term refers to the direct structural and functional connection between living bone and the surface of a load bearing artificial implant. Osseointegration has… … Wikipedia Ossification — Bone is broken down by osteoclasts, and rebuilt by osteoblasts, both of which communicate through cytokine (TGF β, IGF) signalling. Ossification (or osteogenesis) is the process of laying down new bone material by cells called osteoblasts. It is… … Wikipedia Chondrogenesis — is the process by which cartilage is developed. Contents 1 Cartilage in fetal development 2 Mineralization 3 Repair … Wikipedia	2	7	1	0	0	2	0.346726	3	845
Can Chronic Hepatitis C Cause Hepatic Coma Hepatitis C is a blood-borne virus that affects the liver. Following infection, a small number of people can spontaneously clear the virus. However, others develop chronic hepatitis C. Left untreated, the infection can lead to advanced liver disease and even liver coma. Many people with chronic hepatitis C do not know they have an infection until it is revealed by a routine blood test. It may cause no symptoms, and people can live with an infection for decades before being diagnosed. Although chronic hepatitis C can lead to serious liver complications, doctors can treat it successfully with antiviral drugs. This prevents further damage to the liver, cures the infection and prevents it from spreading. However, if people do not seek treatment, they can develop complications that lead to a condition called hepatic encephalopathy. They can even fall into a hepatic coma. Keep reading to learn more about chronic hepatitis C, including complications that can lead to hepatic coma and how doctors treat hepatitis C in the absence of hepatic coma. Hepatitis C is a virus that damages the liver. Doctors classify it as acute or chronic depending on how long it inflames and damages the liver. Acute hepatitis C lasts and goes away alone. About 1015% of people infected with HCV live with this type. People with hepatitis C may have no symptoms initially. However, they can develop chronic liver disease after years of infection. If symptoms appear, they may include Next Treatment Of Hepatic Failure Treatment depends on the stage of the disease. Your doctor may prescribe medications. If only part of your liver is damaged, surgery may be recommended to remove the damaged part. A doctor can also take imagingtests of your liver to look for damage. If a healthy liver is damaged, it can grow back. If the damage is too severe, which can sometimes be the case with fast-acting acute liver failure, a liver transplant may be necessary. You May Like: Hepatitis B How Do You Catch It Whats The Difference Between Acute And Chronic Hepatitis C Hepatitis C is a contagious disease caused by HCV, which is spread through contact with blood and bodily fluids that contain HCV. This disease damages your liver. There are two types of hepatitis C infection: acute and chronic. Acute hepatitis C is a short-term viral infection. People with acute hepatitis C carry the infection for a small window of time, often just several months . Most people with the acute form of hepatitis C will experience illness and mild symptoms such as fatigue and vomiting within the first six months after exposure. In many cases, the disease causes no symptoms at all. Acute hepatitis C may improve or resolve without treatment. It leads to chronic infection in 75 to 85 percent of cases. The chronic form may cause long-term problems in your liver, including liver damage and liver cancer. HCV is spread through direct contact with blood or certain bodily fluids that contain HCV. Its safe to engage in the following activities without worry of transmission: If your doctor suspects that you have hepatitis C, they will draw blood to check for HCV antibodies. Antibodies are substances your body produces when its fighting an infection. If you have them, your doctor may order a second test to confirm that the virus is still present. Don’t Miss: What Are Symptoms Of Hepatitis Recommended Testing For Diagnosing Acute Hcv Infection RECOMMENDED RATING HCV antibody and HCV RNA testing are recommended when acute HCV infection is suspected due to exposure, clinical presentation, or elevated aminotransferase levels . I, C Recommendations for HCV testing are also found in the Testing and Linkage to Care section. Diagnosis of acute HCV infection enables estimation of annual incidence rates and transmission patterns, thereby facilitating implementation and assessment of prevention programs. At the individual level, a diagnosis of acute infection expedites linkage to care, counseling regarding high-risk behavior, and timely interventions to reduce virus transmission and liver disease progression . Some persons involved in high-risk behaviors practice serosorting, defined as using HCV antibody serostatus to determine whether to engage in high-risk behaviors with certain individuals . Thus, undiagnosed acutely infected persons may be at greater risk of transmitting HCV to their presumably seronegative contacts than would be expected by chance. The best laboratory evidence to support a diagnosis of acute HCV infection is a positive HCV RNA test in the setting of a negative HCV antibody test , or a positive HCV antibody test after a prior negative HCV antibody test . There are rare instances in which these approaches may be misleading, such as in immunosuppressed individuals with impaired antibody production . No Discrete Exposure Questions For Your Doctor When you visit the doctor, you may want to ask questions to get the information you need to manage your hepatitis C. If you can, have a family member or friend take notes. You might ask: Read Also: How To Check For Hepatitis Retreatment Of Persons With Prior Peginterferon And Ribavirin Failure The latest version of the AASLD-IDSA HCV Guidance no longer provides specific recommendations for retreatment of persons with a history of peginterferon plus ribavirin therapy, with or without an earlier generation direct-acting antiviral agent . The AASLD-IDSA HCV Guidance notes that these individuals respond to retreatment similar to treatment-naÃ¯ve persons, thus implying the treatment approach should be the same as with treatment-naÃ¯ve individuals. Although the pool of persons with a history of failure with a peginterferon-based regimen who need retreatment is small and diminishing, there are some individuals with this treatment history who need retreatment and may require special consideration that differs from that of treatment-naÃ¯ve individuals. The following outlines a few of these key considerations based on available data and previous guidance that should be noted when retreating an individual with a history of prior treatment failure with peginterferon plus ribavirin, with or without an earlier generation DAA . Note that except for the 8-week option of glecaprevir-pibrentasvir , when retreating these individuals with first-line DAA combinations that have pangenotypic activity , the treatment will be the same as their treatment-naÃ¯ve counterparts. Hcv And Changes In Metabolic Pathways On proton magnetic resonance spectroscopy, metabolic abnormalities of choline/creatine ratio in basal ganglia and white matter have been detected in patients with histologically proven mild hepatitis C with respect to both healthy volunteers and chronic hepatitis B patients, suggesting a role for HCV itself in affecting cerebral functions. Moreover, significant correlations have been reported between cognitive dysfunction and HCV replication and between degree of impairment and the choline/creatine ratio in the basal ganglia and white matter in contrast to what is commonly observed in hepatic encephalopathy, a higher content in cerebral choline has been recorded in these patients. Although the exact significance of elevated choline in the white matter remains uncertain, it may be implicated in glial activation secondary to oxidative stress a similar mechanism has been suggested for chronic fatigue syndrome in HIV infection. Don’t Miss: How Can You Get Hepatitis C What Are The Treatments For Hepatitis C Treatment for hepatitis C is with antiviral medicines. They can cure the disease in most cases. If you have acute hepatitis C, your health care provider may wait to see if your infection becomes chronic before starting treatment. If your hepatitis C causes cirrhosis, you should see a doctor who specializes in liver diseases. Treatments for health problems related to cirrhosis include medicines, surgery, and other medical procedures. If your hepatitis C leads to liver failure or liver cancer, you may need a liver transplant. Unspecified Viral Hepatitis C Without Hepatic Coma - 2016201720182019202020212022Billable/Specific Code - B19.20 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. - The 2022 edition of ICD-10-CM B19.20 became effective on October 1, 2021. - This is the American ICD-10-CM version of B19.20 other international versions of ICD-10 B19.20 may differ. - Applicable To annotations, or Also Check: Hepatitis B Vaccine Cost Without Insurance Don’t Miss: What Is Hepatic Steatosis Of The Liver Medications Used To Treat Hepatitis C The HCV Medications section on this website provides detailed information for each of the Food and Drug Administration -approved medications listed in the treatment recommendations, including links to the full prescribing information and to patient assistance programs. The DAAs exert their action at specific steps in the HCV life cycle. There are three major classes of DAA medications: nonstructural proteins 3/4A protease inhibitors, NS5A inhibitors, and NS5B polymerase inhibitors the NS5B polymerase inhibitors include the nucleoside analogs and nonnucleoside analogs. Adherence with the treatment regimen is of paramount importance. Thus, individuals should receive detailed counseling regarding the importance of adherence prior to starting therapy, as well as intensive monitoring and follow-up during therapy. What Is The Difference Between Hepatitis A Hepatitis B And Hepatitis C Although they are all viruses that infect the liver, how you get them and how they can cause long-term health problems differ. Hepatitis A can be contracted through food or water that has been contaminated by fecal matter and raw shellfish. It does not lead to chronic disease and can be prevented through vaccinations. People generally recover within three to six weeks with no permanent liver damage. Hepatitis B is also less common in the U.S. affecting less than five percent of our population. It is spread through blood and body fluids, including saliva. There are also vaccines to prevent hepatitis B and newborns are vaccinated against this form of hepatitis before they even leave the hospital. Unfortunately, there are no preventative vaccinations for hepatitis C, but early detection and advances in treatment can cure many strains of the disease. Also Check: How Can A Person Get Hepatitis C Hepatitis C Information Center Hepatitis C is a disease caused by a virus that infects the liver. The virus, called the Hepatitis C virus or HCV for short, is just one of the hepatitis viruses. The other common hepatitis viruses are A and B, which differ somewhat from HCV in the way they are spread and treated. According to the Centers for Disease Control , an estimated 2.7 million people in the United States have chronic Hepatitis C infection. The CDC now recommends one-time hepatitis C testing of all adults and all pregnant women during every pregnancy. CDC continues to recommend people with risk factors, including people who inject drugs, be tested regularly. Explore this Hepatitis C Information Center by clicking through to the Diagnosis, Treatment and Support landing pages where youll find more information to help you manage Hepatitis C. Electronic Medical Record Screening Protocol Where possible, the EMR offers an essential component of successful HCV screening through a best practice alert that notifies clinicians and staff when a patient is eligible for screening . Ideally this alert links to a one-time HCV screening test for eligible patients with the appropriate diagnosis code . After the test is completed, the BPA should turn off but highlight a positive result. The most efficient test to order is an anti-HCV antibody that reflexes to a quantitative HCV RNA on the same blood sample to confirm chronic HCV. This is essential as 15-35% of anti-HCV antibody positive patients have cleared the infection. In summary:Eligible patients for HCV screening: - Birth year 1945-1965 - Prior record of HCV diagnosis based on ICD-9-CM or ICD-10 codes - Prior record of any HCV test based on an array of Current Procedural Terminology codes . - Z11.59 Encounter for screening for other viral diseases - B17.11 Acute hepatitis C with hepatic coma - B18.2 Chronic viral hepatitis C - B17.10 Acute hepatitis C without hepatic coma - B19.20 Unspecified viral hepatitis C without hepatic coma - B19.21 Unspecified viral hepatitis C with hepatic coma - Z22.52 Carrier of Hepatitis C For a more complete list, visit Support Path Recommended Reading: Diet For Dogs With Chronic Hepatitis Cms National Coverage Policy Social Security Act Standard References: - Title XVIII of the Social Security Act, Section 1833 states that no payment shall be made to any provider of services or other person under this part unless there has been furnished such information as may be necessary in order to determine the amounts due such provider or other person under this part for the period with respect to which the amounts are being paid or for any prior period. You May Like: Is There Now A Cure For Hepatitis C Blood And Vessel Problems People with hepatitis C often get a condition called cryoglobulinemia. This happens when certain proteins in your blood stick together in cold weather. They can build up in vessels and block blood flow, which causes swelling and damage. The condition can affect your skin, organs, nerves, and joints. Hepatitis C also can cause problems with blood itself. You may not make enough white blood cells, which fight infections, or platelets, which help your blood clot. The infection can also make you bruise easily or get red or purple spots under your skin. Those are signs of a bleeding disorder called immune thrombocytopenic purpura. Also Check: Can Chronic Hepatitis B Be Cured Also Check: How Can I Catch Hepatitis How Is Acute Hepatitis C Treated Acute hepatitis C is typically monitored and not treated. Treatment during the acute stage doesnt change the risk that the disease will progress to the chronic form. An acute infection may resolve on its own without treatment. The following treatment may be all thats necessary: - adequate fluids Some people may need treatment with prescription medication. Your doctor will be able to work with you about what treatment options may be best for you. Those most at risk for acute and chronic hepatitis C are people who use or share contaminated needles. Mothers can transmit HCV to their babies during childbirth, but not through breastfeeding. Other risk factors for transmission of HCV include: - healthcare work, especially work around needles - getting a tattoo or body piercing with unsterile equipment - undergoing hemodialysis - living in a household with someone with HCV - sharing personal hygiene products, such as razors or toothbrushes - engaging in sexual activity with multiple partners without condoms or dental dams - having a blood transfusion or organ transplant before July 1992 or receiving clotting factors before 1987 The most serious long-term risk of acute hepatitis C is developing chronic hepatitis C, which can lead to cirrhosis and liver cancer. In 75 to 85 percent of those with acute hepatitis C, the disease will progress to the more serious chronic hepatitis C. What Is Viral Hepatitis Hepatitis means inflammation of the liver. The liver is a vital organ that processes nutrients, filters the blood, and fights infections. When the liver is inflamed or damaged, its function can be affected. Heavy alcohol use, toxins, some medications, and certain medical conditions can cause hepatitis. However, hepatitis is often caused by a virus. In the United States, the most common types of viral hepatitis are hepatitis A, hepatitis B, and hepatitis C. Read Also: Is Hepatitis C Sexually Transmitted Who Is At Risk For Hepatitis C You are more likely to get hepatitis C if you: - Have injected drugs - Had a blood transfusion or organ transplant before July 1992 - Have hemophilia and received clotting factor before 1987 - Have been on kidney dialysis - Were born between 1945 and 1965 - Have abnormal liver tests or liver disease - Have been in contact with blood or infected needles at work - Have had tattoos or body piercings - Have worked or lived in a prison - Were born to a mother with hepatitis C - Have had more than one sex partner in the last 6 months - Have had a sexually transmitted disease - Are a man who has had sex with men If you are at high risk for hepatitis C, your health care provider will likely recommend that you get tested for it. Who Should Get Tested Since universal screening of blood and blood products did not occur until 1992, anyone who had a blood transfusion or organ transplant before that time should be tested. It is recommended that people be tested for hepatitis C if they were/are: - Born between 1945 and 1965 - Exposed to blood and body fluids - Sharing toothbrushes, razors, nail clippers or other personal items with an infected individual - Using and/or sharing needles to inject drugs - Receiving tattoos and body piercings with unsterile needles - Women thinking about becoming pregnant - Born to an infected mother - Receiving long-term hemodialysis - Having unprotected sex with multiple sex partners or have a history of sexually transmitted diseases Don’t Miss: Dna Test For Hepatitis B What Are The Chances Of Surviving Liver And Kidney Failure Prognosis for Anyone Living with HRS The prognosis for people with liver failure is much worse if they develop HRS. Most patients die within weeks of the onset of renal failure without therapy. In fact, 50% of people die within 2 weeks of diagnosis and 80% of people die within 3 months of diagnosis. Hcv Infection And Peripheral Neuropathy Disorders In contrast to the brain, there is currently no evidence for peripheral nerves as permissive sites for HCV replication however, a wide variety of motor, sensory or sensorimotor mono- or polyneuropathies has been described during chronic HCV infection. Most peripheral neuropathies have been reported in patients with HCV-related mixed cryoglobulinemia, with prevalence up to 86% of cases. In particular, a sensory motor peripheral neuropathy has been found in up to 30% of HCV-positive cryoglobulinemic patients. Such neuropathy is the consequence of ischemic nerve changes, secondary to small-vessel vasculitis or necrotizing arteritis of medium-sized vessels. Frequently, the clinical onset is sub-acute as a distal, symmetric, sensory or sensorimotor polyneuropathy, although asymmetrical sensory impairment has also been reported. Small fiber sensory polyneuropathy , a painful condition mainly characterized by burning feet and tingling, is the most frequent neuropathy observed in patients with mild cryoglobulinemia syndrome, whereas the so called large fiber sensory neuropathy has been described less frequently. HCV-associated restless legs syndrome has also been reported as expression of SFSN. SFSN may later evolve in LFSN. LFSN symptoms include sensory loss, paresthesias, numbness, and cramps. Recommended Reading: How Hepatitis C Virus Spread	2	8	0	0	0	8	0.914366	8	4,079
Review the AMA’s updated definition of what constitutes a unique test. Many coders are asking the question, “What is a unique test?” As always, we must first turn to the guidelines to see how “test” is defined. In the original published guidelines, the American Medical Association (AMA) stated: “Tests are imaging, laboratory, psychometric, or physiologic data. A clinical laboratory panel (eg basic metabolic panel ) is a single test. The differentiation between single or multiple unique tests is defined in accordance with the CPT code set.” In the technical corrections, the AMA modified the above definition of “test,” removing the word “unique” and adding the following sentence: “For the purposes of data reviewed and analyzed, pulse oximetry is not a test.” The technical corrections further clarified this point by adding “unique” to its list of definitions for the elements of medical decision making (MDM): “A unique test is defined by the CPT code set. When multiple results of the same unique test (eg, serial blood glucose values) are compared during an E/M service, count it as one unique test. Tests that have overlapping elements are not unique, even if they are identified with distinct CPT codes. For example, a CBC with differential would incorporate the set of hemoglobin, CBC without differential, and platelet count. A unique source is defined as a physician or qualified heath care professional in a distinct group or different specialty or subspecialty, or a unique entity. Review of all materials from any unique source counts as one element toward MDM.” Break Down the Guidelines A unique test is defined by CPT® code, not the number of tests performed. If the provider orders a panel test that contains tests with individual CPT® codes, all of those individual CPT® codes are bundled into the CPT® code for the panel test and counted as one unique test. For example: The provider orders 80051 Electrolyte panel. Although this panel includes four tests — 82374 Carbon dioxide (bicarbonate), 82435 Chloride, 84132 Potassium, and 84295 Sodium — this is considered and counted as one unique test, not four, and reported with 80051 only. The AMA also states that serial tests — multiple results of the same unique test — with comparison count as one unique test, even though multiple results are reviewed. For example: The provider orders serial glucose testing over several hours or days and compares the results during an E/M service. Although the same test is repeated multiple times, the technical corrections state that serial tests count as one unique test. Lastly, the AMA gives an example to explain how tests that have overlapping elements are not unique, even if they are identified with two distinct CPT® codes: A complete blood count (CBC) with differential and a CBC without differential would constitute one unique test. For example, CPT® code 85025 Blood count; complete (CBC), automated (Hgb, Hct, RBC, WBC and platelet count) and automated differential WBC count and CPT® code 85027 Blood count; complete (CBC), automated (Hgb, Hct, RBC, WBC and platelet count) are considered one unique test because they have overlapping components. During the creation process of the changes, the AMA says they were not trying to incentivize the provider, but rather give credit to the provider for the work performed during the encounter. If a provider is going to bill for the professional component of a test, credit cannot be given for the ordering or reviewing of the test because they are already being paid for the test. Providers will not get credit for generating business for themselves. During a webinar released by the AMA last year, it was stated that the review of a unique test is inherent in the ordering of the unique test. Therefore, if the provider orders a test, the provider will not also get credit for the review of the test, even if the review is performed at a subsequent encounter. Remember that the AMA makes technical corrections to CPT® throughout the year. These corrections are not printed in your CPT® code book. You will need to refer to the errata and technical correction to apply the guidelines appropriately and code correctly. You can find the technical corrections made to the E/M guidelines on AMA’s website. For More Information: https://www.aapc.com/blog/82044-2021-e-m-changes-are-you-coding-unique-tests-properly/	4	7	7	0	0	0	0.670576	7	969
Basic Nutritional Support Heart and Cholesterol Finally, a Healthy Energy Drink for you and your kids! Chronic Fatigue Syndrome By Ronald Steriti, ND, PhD Chronic Fatigue Syndrome (CFS) is defined as debilitating fatigue and associated symptoms last-ing at least 6 months. Even though the Centers for Disease Control (CDC) officially recognized chronic fatigue syndrome in 1988, it remains a controversial issue. Chronic Fatigue Syndrome is closely related to another chronic condition, fibromyalgia (FMS). Muscle pain is the prominent symptom of fibromyalgia. However, preliminary studies by the Centers for Disease Control reveal that, for those individuals whose chronic fatigue does not significantly improve after a 5-year duration, the most prominent symptom changes from fatigue to muscle pain The criteria for diagnosing chronic fatigue syndrome was officially defined by the Centers for Disease Control (CDC) in 1988. They have recently revised their definition. The Oxford criteria differs slightly. The British criteria insists upon the presence of mental fatigue, while the American criteria includes a requirement for several physical symptoms, reflecting the belief that CFS has an underlying immune or infectious pathology. [1, 2] The CDC Criteria defines Chronic Fatigue Syndrome as: clinically evaluated, unexplained, persistent or relapsing fatigue that is: of new or definite onset; not a result of ongoing exertion; not alleviated by rest; and results in a substantial reduction in previous levels of occupational, social, or personal activity. Four or more of the following symptoms that persist or recur during 6 or more consecutive months of illness and that do not predate the fatigue: · Self-reported impairment of short-term memory or concentration · Sore throat · Tender lymph nodes · Muscle pain · Multi-joint pain without swelling or redness · Headaches of a new type, pattern, or severity · Unrefreshing and/or interrupted sleep Exclusion criteria includes: · Active, unresolved, or suspected disease that is likely to cause fatigue · Psychotic, melancholic or bipolar depression (but not uncomplicated major depression) · Psychotic disorders, Dementia, Anorexia or bulemia nervosa · Alcohol or other substance misuse · Severe obesity The Oxford (British) Criteria defines Chronic Fatigue Syndrome as: Severe disabling fatigue of at least six months duration that: affects both physical and mental functioning; and was present for more than 50% of the time. Other symptoms, particularly myalgia and sleep and mood disturbances, may be present. Exclusion criteria includes: · Active, unresolved, or suspected disease that is likely to cause fatigue · Psychotic, melancholic or bipolar depression (but not uncomplicated major depression) · Psychotic disorders, Dementia, Anorexia or bulemia nervosa Although the symptoms listed above are the official diagnostic criteria, many patients with chronic fatigue syndrome present with a variety of other symptoms, including: · Pain is almost universal in chronic fatigue · Chemical sensitivities · Secondary infections, including Candida and viral infections · Cognitive impairment, including short-term memory loss, difficulty concentrating, word searching, and math problems · Digestive disturbances, such as chronic constipation or diarrhea · Night sweats or spontaneous daytime sweats, unaccompanied by fever · Headaches, migraines · Weakness (paresis), muscle fatigue and pain (fibromyalgia) · Premenstrual syndrome (PMS) · Sleep disorders, including excessive sleep (hypersomnia), light sleep or an inability to sleep for more than an hour (hyposomnia), disturbing nightmares · A period of 1-3 hours after awakening during which they are too exhausted to get out of bed (dysania) · Cystitis (inflammation of the urinary bladder), particularly interstitial cystitis in which urine cultures are negative · Vision and eye problems, including sensitivity to light (photophobia), dry eyes, tunnel vision, night blindness and difficulty focusing An initial office exam may also find the following signs: · Low blood pressure, particularly on standing (orthostatic hypotension) · Low oral temperatures (less than 97˚ F) · Slightly elevated oral temperatures (less than 100˚ F), which are part of persistent flu-like symptoms. · Increased heart rate (tachycardia) · A positive Romberg test (unsteadiness when standing with eyes closed) Conventional Lab Tests Doctors usually perform the following labs when attempting to diagnose a patient with CFS: · Complete blood count (CBC) with differential · Chemistry panel · Erythrocyte Sedimentation Rate (ESR), a marker of inflammation Optional tests include: · Anti-nuclear antibodies (ANA) and rheumatoid factor (RF). These are tests for rheumatoid arthritis and systemic lupus erythematous (SLE) · Thyroid tests (T3, T4, TSH) · Adrenal tests (AM and PM cortisol levels) · Lyme titers and HIV serology Specific tests that support (but do not necessarily confirm) a diagnosis of chronic fatigue include: · Tests for viral infections, such as cytomegalovirus, Epstein-Barr virus, Human herpes virus 6, and coxsackie virus · Immune system tests, including low natural killer (NK) cell counts, elevated interferon alpha, tumor necrosis alpha, interleukins 1 and 2, T cell activation, altered T4/T8 cell ratios, low T cell suppressor cell (T8) count, fluctuating B and T cell counts, antinuclear antibodies, immunoglobin deficiency, antithyroid antibodies · Exercise testing may show decreased cortisol levels after exercise, decreased cerebral blood flow after exercise, inefficient glucose utilization, and erratic breathing patterns Research into the cause(s) of chronic fatigue syndrome touches upon a vast array of systems and etiologies. Several lab tests, in addition to those mentioned above may be helpful in guiding appropriate treatment. These would include: · Functional assessments of the adrenal gland, including measurements of cortisol, DHEA, and DHEA-S · Assessments of oxidative stress · Homocysteine levels · C-reactive protein, a sensitive marker of inflammation · Toxin analysis, including heavy metals, pesticides, and organic chemicals Possible Causes of Chronic Fatigue There is a considerable amount of research into the cause of chronic fatigue syndrome. Many researchers propose that there may be several different mechanisms that underly CFS. The possible causes of CFS fall into a few broad categories: · Immune system activation, particularly by viruses · Oxidative stress, glutathione deficiency · Endocrine dysfunction, including adrenal fatigue, thyroid deficiency and hypothalamic-pituitary axis abnormalities · Neurotransmitter deficiencies · Drug-induced fatigue As you will see from the following discussion, many of these causes are inter-related. For instance, oxidative stress can cause immune dysfunction through the nitric oxide and peroxynitrate systems. The immune system is also greatly influenced by the endocrine system (and the hormones involved, including DHEA, melatonin). For many people (and physicians) chronic fatigue syndrome is very confusing. In this article we will present current research on each of the components followed by a section on natural therapies that have been shown to be effective. The Immune System and Chronic Fatigue Syndrome Viruses and CFS Symptoms of CFS resemble a post-viral state and, for this reason, chronic viral conditions have been thought to contribute to CFS in some patients. Several viruses have been associated with CFS, including: · Herpes virus, particularly human herpes virus 6 (HHV-6) · Epstein-Barr virus (a herpes virus which causes infectious mononucleosis) · Cytomegalovirus (a herpes virus) · Coxsackievirus B1 and B4 Chronic viral infections have a detrimental impact on the body through several mechanisms: · Chronic viral infections cause the immune system to be activated in an effort to fight the infection · Chronic infections are a cause of inflammation in the body A primary strategy for chronic fatigue syndrome is to support the immune system in fighting viral infections. The Immune System The immune system is a complex system of cells and chemical messengers that work together to keep the body clear of pathogenic infections. The components specifically involved in viral immunity include: · Antigens (viruses) attach to T-helper cells which secrete a variety of chemical messengers (including interferon and interleukin-2) that activate NK cells, macrophages, cytotoxic T cells, and memory B cells. · Interferon is a group of glycoproteins that activate macrophages to form Natural Killer (NK) cells · NK cells lyse (split apart) cells that contain viruses. · Interleukin-2 stimulates proliferation of B and T cytotoxic cells. · Cytotoxic T cells are formed to attack specific antigens (viruses). An article published in the journal of Clinical Infectious Disease measured natural killer cell activity in 50 healthy individuals and 20 patients with clinically defined chronic fatigue immune dysfunction syndrome (CFIDS). The patients were divided into three groups based on severity of the clinical status. NK cell activity decreased with the increasing severity of the clinical condition. Researchers have found that human herpes virus 6 targets and kills NK cells. Supplements that have been shown to increase NK cell activity include beta-carotene, vitamin E, zinc, and DHEA. The herbs echinacea and ginseng have been shown to increase NK cell activity in CFS patients (see the Natural Therapies section). Viruses that “Fool” the Immune System There are two different types of T-helper cells which defend against different organisms: · T-helper 1 (Th1) cells target intracellular pathogens (organisms that invade cells), such as viruses. Interleukin-12 (IL-12) stimulates Th1 activation. · T-helper 2 (Th2) cells target organisms that are found outside of cells. T-helper 2 cells are involved in humoral or antibody-mediated immunity and are triggered by interleukin-10 (IL-10) which is stimulated by bacteria, parasites, toxins, and allergens. Each of the T-helper cells are activated by different cytokines (see Table). In health, there is a balance between Th1 and Th2 activity. When presented with an acute infection, the Th1 system predominates (and Th2 is supressed). In chronic infections, the Th2 system predominates leading to antibody production. Table: Cytokine profiles and functions of T helper cells Viruses, especially herpes viruses (like Epstein-Barr virus, cytomegalovirus, and human herpes virus 6) make proteins that mimic IL-10 which activates the Th2 system. Unfortunately, Th2 activation suppresses T-helper 1 (Th1) activity, particularly cytotoxic T cells and natural killer (NK) cells which are the main defense against viruses. In this way the viruses are able to “fool” the immune system and remain untouched by the bodies natural defenses. Addressing the two different types of T-helper cells has been the focus of work be Paul Cheney, MD. His protocols are are designed to stimulate Th1 and inhibit Th2. Several nutritional supplements, including essential fatty acids, vitamin A, vitamin E, DHEA and melatonin, have been found to have beneficial effects of the Th1:Th2 ratio (see the Natural Therapies section below). Infection and Inflammation A new theory has been published by Dr. Martin L. Pall (Professor of Biochemistry and Basic Medical Sciences at Washington State University). The theory involves a chain of events: · Chronic infections that often precede CFS act to induce excessive production of inflammatory cytokines. · Inflammatory cytokines induce nitric oxide synthase (iNOS) which synthesizes excessive amounts of nitric oxide · Nitric oxide reacts with superoxide to produce the potent oxidant peroxynitrite (nitrogen dioxide). · Peroxynitrite acts to increase the levels of both nitric oxide and superoxide which react to produce more peroxynitrite In this way, once peroxynitrite levels are elevated, they may act to continue the elevation, thus producing a self-sustaining vicious cycle. It is this cycle, according to the theory, that maintains the chronic symptoms of CFS and it is this cycle, therefore, that must be interrupted to effectively treat this condition. Figure: Peroxynitrate metabolism Breaking the infection-inflammation cycle Breaking the chain of inflammation caused by chronic viral infections would require a three-part protocol: · First, the underlying viral infection should be addressed with antiviral supplements (such as ginseng, echinacea and lactoferrin) and those that shift the Th1:Th2 ratio (such as essential fatty acids and vitamin E). · Second, inflammation should be reduced with anti-inflammatory agents (such as essential fatty acids and curcumin). · Third, the nitric oxide system should be supported with supplements such as arginine, vitamin B2 (riboflavin), vitamin B3 (niacin), and folate. Supplements that Support the Immune System Ginseng and Echinacea Commission E, the group of scientists that advises the German government about herbs, endorses ginseng "as a tonic to combat feelings of lassitude and debility, lack of energy and ability to concentrate, and during convalescence." Ginseng is highly prized in China as an herb that increases energy. The higher grades are extremely expensive. Most of the studies on ginseng have focused on its use in enhancing sports performance. Echinacea has become very popular in the United States as “the herb” to take for colds and flus. Echinacea has strong antiviral properties and has been shown to increase NK cell production. [10, 11] An article published in the journal Immunopharmacology found that both echinacea and ginseng (at concentrations greater or equal to 0.1 or 10 mcg/kg, respectively) significantly enhanced NK-function in patients with chronic fatigue syndrome or the acquired immunodeficiency syndrome. Essential Fatty Acids The use of essential fatty acids in chronic fatigue syndrome is controversial due to the results of one negative study. It has been proposed that essential fatty acids play a role in chronic fatigue syndrome. One possible mechanism is that viruses, as part of their attack strategy, may reduce the ability of cells to make 6-desaturated essential fatty acids. [14, 15] The use of essential fatty acids for post-viral fatigue syndrome was examined in a double blind, placebo-controlled study of 63 adults. The patients had been ill for one to three years after an apparent viral infection, suffering from severe fatigue, myalgia and a variety of psychiatric symptoms. The patients received either placebo or a preparation containing linoleic, gamma-linolenic (GLA), eicosapentaenoic (EPA) and docosahexaenoic acids (DHA) over a 3-month period (eight 500-mg capsules per day). Participants were asked to assess their improvement at months 1 and 3. The treatment group showed continual improvement, whereas many in the placebo group reverted towards baseline. Table: Improvement with essential fatty acid treatment The essential fatty acid composition of their red cell membrane phospholipids was analyzed at the first and last visits. The essential fatty acid levels were abnormal at the baseline and corrected by active treatment. The authors concluded that essential fatty acids provide a rational, safe and effective treatment for patients with post-viral fatigue syndrome. A follow-up study of 50 patients diagnosed with chronic fatigue syndrome found no significant difference between the placebo group and those treated with Efamol marine (a combination of Evening Primrose Oil and Marine Fish Oil that contains linoleic acid, GLA, EPA and DHA) In addition, no difference was seen in red cell membrane lipids between the patients and control group. These results sharply contrasted the previous study by Behan et al. Essential fatty acids have been shown to have an effect on the ratio of T-helper 1 and 2 cells. High dietary intake of linoleic acid results in high tissue production of prostaglandin E2, which in turn causes inhibition of the proliferation and cytokine production of Th1 cells, mediators of cellular immunity. A study examined the effects on the immune system of either a low-fat diet or a high-fat diet containing coconut oil (rich in saturated fatty acids), safflower oil (rich in omega-6 EFAs), or fish oil (rich in omega-3 EFAs) as the main fat sources on mice. The ratio of production of Th1- to Th2-type cytokines was lower for lymphocytes from mice fed the safflower oil or fish oil diets. Although all fatty acids decreased IL-2 production in a concentration-dependent manner, saturated fatty acids were the least potent and omega-3 EFAs the most potent inhibitors, with omega-6 EFAs falling in between in terms of potency. The authors concluded that EFAs act to inhibit production of Th1-type cytokines with little effect on Th2-type cytokines with omega-3 EFAs being particularly potent. Essential fatty acids are named “essential” because they play a vital role in health. Essential fatty acids are found in healthy oils, such as fish, flax, borage, and perilla. Unfortunately fatty acids are damaged by heat and many people are deficient due to the high heats used to process packaged foods. Fatty acid metabolism requires several nutritional cofactors. These include L-carnitine (to move fats in an out of cells), vitamin E (which protects fats against oxidation), and NADH (which breaks fats down to form energy). Each of these nutrients have been studied for use in chronic fatigue syndrome. The amino acid L-carnitine is used in the body to transport fats across cell membranes. Carnitine is synthesized in the body from lysine (an essential amino acid that has antiviral properties) and methionine (an amino acid involved in homocysteine metabolism). L-carnitine is often included in weight-loss supplements to aid in moving fats. L-carnitine is also known to boost energy levels. [20, 21] Several studies have found deficiencies of carnitine in patients with CFS, while more recent studies have contradicted these findings: · A study of 35 CFS patients (27 females and 8 males) found significantly lower serum total carnitine, free carnitine and acylcarnitine levels, and that higher serum carnitine levels correlated with better functional capacity. · Another study found low levels of acylcarnitine in both Japanese and Swedish patients with chronic fatigue syndrome. · A recent study of 25 women with CFS and 25 healthy controls in The Netherlands, however, found no difference in carnitine levels. A clinical trial of carnitine for the treatment of CFS found clinical improvement in 12 of 18 patients. The greatest improvement occurred between weeks four and eight of treatment. One patient was unable to complete the trial due to the development of diarrhea. Vitamin E is a powerful antioxidant that is found in vegetable oils. Vitamin E works to protect the fat-soluble parts of the body, such as LDL cholesterol. Recent research in mice has found that vitamin E may enhance Th1 cytokines, possibly as a result of reduced prostaglandin E2 (PGE2, an inflammatory compound) production. NADH (reduced B-nicotanimide dinucleotide) is a coenzyme molecule formed from vitamin B3 (niacin). NADH donates its hydrogen in many reactions throughout the body. It is involved in oxidative phosphorylation (the production of ATP, the energy molecule of the body), fatty acid oxidation (the breakdown of fats to make energy), and in carbohydrate metabolism. A recent randomized, double-blind, placebo-controlled crossover study examined the use of NADH with chronic fatigue syndrome. Twenty-six eligible patients diagnosed with CFS received either 10 mg of NADH or placebo for a 4-week period. Eight of 26 (31%) responded favorably to NADH in contrast to 2 of 26 (8%) to placebo. Based upon these encouraging results the authors decided to conduct a larger study to establish its efficacy in CFS. NADH (5 to 10 mg per day) is most effective when taken in the morning 30 minutes before breakfast. Vitamin A plays a role in the development of T-helper and B cells. Vitamin A deficiency impairs innate immunity by diminishing the function of neutrophils, macrophages, and natural killer cells. Although vitamin A does play a role in balancing Th1 and Th2 function, it does so by down-regulating Th1 cell IFN-gamma secretion directly, decreasing activated antigen presenting cell (APC) function, and promoting Th2 cell growth and/or differentiation. Therefore, although vitamin A is an important nutrient for immune function, chronic fatigue syndrome patients should avoid excessively high doses. [29-33] Whey protein is perhaps the oldest and most well-known supplement used by athletes and body builders. In recent years, scientists have begun to investigate the health benefits of proteins to improve immunity and prevent diseases. Whey has recently been shown to have significant antiviral properties (with much of the research on it’s ability to increase glutathione levels and inhibit HIV). [34, 35] Whey protein is comprised of four major protein fractions and six minor protein fractions. The major protein fractions are beta-lactoglobulin, alpha-lactalbumin, bovine serum albumin, and immunoglobulins. Each of these components have important disease-fighting effects. Lactoferrin, in particular, has been shown to have significant antiviral activity. [36-38] DHEA and Melatonin DHEA (dehydroepiandrosterone) is the most prevalent hormone produced by the adrenal glands. It circulates in the bloodstream as DHEA sulfate (DHEAS) and is converted into other hormones, including estrogen and testosterone. Melatonin is a natural hormone that regulates the human biological clock. Levels of melatonin are lower during the day and higher at night. Melatonin is commonly used before bed to aid in sleep. An article published in the journal Immunology described a study of the immune effects of DHEA and melatonin in mice infected with a leukemia retrovirus that caused AIDS. Treatment with DHEA or melatonin alone, as well as together, prevented the reduction of B- and T-cell proliferation as well as of Th1 cytokine secretion caused by retrovirus infection. Supplementation also suppressed the elevated production of Th2 cytokines stimulated by retrovirus infection. Arginine is made in the body from glutamic acid, and is therefore considered semi-essential. Arginine stimulates the first step in the urea cycle, which rids the body of nitrogenous waste. Arginine is concentrated in muscles, where it is responsible for the high energy compounds guanidophosphate, phosphoarginine and creatine. An article published in the European Journal of Clinical Investigation described a study of the effects of L-arginine on NK cell function in 20 subjects with chronic fatigue syndrome and 21 healthy individuals. Arginine was found to increase NK activity in the healthy subjects but not those with CFS. Further investigation, however, found that the effect of arginine on NK cell activity was mediated by nitric oxide. That is, the increase in NK activity induced by arginine was blocked by the addition of an inhibitor of inducible nitric oxide synthase. NK activity was increased by incubation with a nitric oxide donor. The authors concluded that a dysfunction in the nitric oxide mediated NK cell activation may exist in CFS patients. Caution Arginine has been found to promote the growth of Herpes simplex, especially if lysine levels are low. Both lysine and arginine contribute to immunity and have antiviral properties. Proteins (meats, fish and cheese) usually contain slightly more lysine than arginine, with eggs containing equal amounts. Supplementation with equal amounts of lysine and arginine is recommended for those considering this therapy. One 500 mg capsule of each can be taken once or twice daily. Antioxidants and CFS Free radical damage (oxidative stress) is probably the most significant cause of biologic aging. Free radicals are unstable molecules that damage cells and are implicated in most diseases associated with aging. Antioxidants are the bodies natural defense against free radical-induced cell damage. Recent studies have shown that oxidative stress plays a role in the development of chronic fatigue syndrome. [41-43] Exercise has been shown to increase the production of oxidants. Fortunately, regular endurance exercise results in adaptations in the skeletal muscle antioxidant capacity, which protects myocytes (muscle cells) against the deleterious effects of oxidants and prevents extensive cellular damage. [44, 45] A study of the oxygen delivery to muscles in patients with CFS found that oxygen delivery and oxidative metabolism was significantly reduced in CFS patients after exercise, when compared with sedentary controls. The issue of exercise in chronic fatigue syndrome is a topic of debate. Many women with CFS were active athletically. There is some overlap between CFS symptoms and overtraining syndrome. Physical exercise is sometimes recommended for those with CFS. Unfortunately, for some people with CFS even minimal exercise can cause extreme fatigue. The antioxidant theory offers a novel explanation for this situation and provides several powerful therapies for those who enjoyed an active lifestyle before the chronic fatigue symptoms developed. Elevated Homocysteine Levels and CFS Homocysteine is a toxic intermediate molecule formed in the body during cellular damage. Homocysteine, although toxic itself, is normally metabolized into other nutrients that are beneficial to the body, including cysteine, taurine and glutathione. Homocysteine is so toxic to the body that many consider it to be much worse than cholesterol. A study of 12 women who fulfilled the criteria for both fibromyalgia and chronic fatigue syndrome found that, in all the patients, the homocysteine levels were increased in the cerebrospinal fluid (CSF). There was a significant positive correlation between CSF homocysteine and B12 levels and fatigue-ability, as rated on the Comprehensive Psychopathological Rating Scale. The authors concluded: “increased homocysteine levels in the central nervous system characterize patients fulfilling the criteria for both fibromyalgia and chronic fatigue syndrome”. Homocysteine and glutathione metabolism are related in biochemical pathways that involve cysteine, glutamine, glycine and GABA (see Figure). Figure: Glutathione metabolism Antioxidant Therapy for Chronic Fatigue Syndrome Glutathione and N-Acetyl Cysteine Glutathione is a peptide-like molecule naturally synthesized in the body from three amino acids: L-glutamic acid, L-cysteine, and glycine. Glutathione is one of the body’s most important and powerful antioxidants. Glutathione also attaches to toxic molecules, which are then eliminated from the body (detoxification). An article published in the journal Medical Hypothesis proposed that glutathione, an antioxidant essential for lymphocyte function, may be depleted in chronic fatigue syndrome patients. Glutathione is needed for both the immune system and for aerobic muscular contraction. The authors proposed that glutathione depletion by an activated immune system also causes the muscular fatigue and myalgia associated with chronic fatigue syndrome. Cysteine is a precursor to glutathione. It has been hypothesized that glutathione and cysteine metabolism may play a role in skeletal muscle wasting and muscle fatigue. The combination of abnormally low plasma cysteine and glutamine levels, low natural killer (NK) cell activity (with a resulting susceptibility to viral infection), skeletal muscle wasting or muscle fatigue, and increased rates of urea production defines a complex of abnormalities that is tentatively called "low CG syndrome." These symptoms are found in patients with HIV infection, cancer, major injuries, sepsis, Crohn's disease, ulcerative colitis, chronic fatigue syndrome, and to some extent in over-trained athletes. N-acetyl-cysteine is a precursor of glutathione that has been shown to be helpful against viruses (most of the research has been with HIV and Hepatitis infections). [51, 52] Coenzyme Q10 has long been prescribed for CFS patients. CoQ10 is a potent antioxidant that aids in metabolic reactions including the process of forming ATP, the molecule the body uses for energy. Virtually every cell in the body contains CoQ10. It is concentrated in the mitochondria, the area of the cells where energy is produced. Judy presented a study of 20 female patients with CFS that required bed rest following mild exercise and 20 healthy controls. Eighty percent of the CFS patients were found to be deficient in CoQ10 which further decreased following mild exercise or over the course of normal daytime activity. After three months of CoQ10 supplementation (100 mg per day) exercise tolerance (400 kg-meters of work) more than doubled. All patients had improved. Ninety percent had reduction and/or disappearance of clinical symptoms, and 85 percent had decreased post-exercise fatigue. [21, 53] Coenzyme Q10, 100 mg taken 3 times a day, often helps victims of severe chronic fatigue syndrome. Folate plays a role in many key biochemical reactions in the body. Folic acid is involved in homocysteine metabolism (with vitamins B6 and B12). It is needed to form glutamate (a precursor of glutathione), and is involved in DNA replication. Folate is also needed to make SAMe (S-adenosyl methionine), a natural supplement which affects (and may improve) mood. An article published in the journal Neurology described a study in which serum folate levels were measured in 60 patients with chronic fatigue syndrome. Researchers found that 50% had values below 3.0 micrograms/L (the normal values are 2-20). The authors concluded that some patients with CFS are deficient in folic acid. Most people do not consume the recommended amount of folic acid in their diet. Mild folic acid deficiencies are common in Western societies, and women taking birth control pills are at higher risk. It is recommended that women who are or could become pregnant should take 400-800 mcg per day to reduce the risk of birth defects. Most nutritionally-oriented doctors recommend that everyone take 400 mcg of folic acid per day. Glutamine is a conditionally essential amino acid that is needed during periods of excessive stress. Glutamine is the preferred energy for enterocytes, the cells lining the gastrointestinal tract. An article published in the British Journal of Sports Medicine described a study of athletes during an intense training period before the 1992 Olympics. The athletes were divided into three groups who differed in training fatigue and were considered separately. Group A (21 track and field athletes) had no lasting fatigue; group B (12 judo competitors) reported heavy fatigue at night but recovered overnight to continue training; group C (18 track and field athletes, one rower) had chronic fatigue and had been unable to train normally for at least several weeks. Plasma amino acid analysis showed that group A had a normal amino acid pattern, and both groups B and C had decreased plasma glutamine (average 33%) with, especially in group B, decreased histidine, glucogenic, ketogenic, and branched chain amino acids. Ten athletes in group C presented with infection. After three weeks of additional protein intake, virtually all the low glutamine levels increased to above 500 micromol/L. Total amino acids increased, and the amino acid pattern normalized. Six of the ten athletes on this protein intake returned to increased training within the three weeks. Glutamine is a non-essential amino acid that supplies energy to the brain. It has been found to be helpful in reducing fatigue, improving exercise endurance, alleviating hypoglycemia, and strengthening the immune system. One or two grams may be used as needed and is often recommended before exercise. Insomnia, however, may occur if glutamine is consumed too close to bedtime. CFS and The Endocrine System The Hypothalamus-Pituitary-Adrenal Axis The HPA axis refers to the hypothalamus, pituitary, and adrenal glands which are part of the endocrine system. The hypothalamus secretes several hormones that control the pituitary gland. The pituitary gland is considered the “master gland” of the endocrine system because it secretes hormones that control other glands (including the ovaries, testes, adrenals, and thyroid glands). A major role of the HPA axis is to restrain the immune system and prevent tissue damage. Reciprocal interactions between the HPA axis and immune system constitutes a new endocrine feedback loop that has given rise to the field of neuroendocrine immunology. "Many experts now think that chronic fatigue syndrome may be an example of the hypothalamus failing to properly regulate the brain’s influence on the immune system," says Jay Lombard, M.D., assistant clinical professor of neurology at Weill Medical College of Cornell University in New York City and co-author of The Brain Wellness Plan. It has been proposed that CFS is a mild form of Addison’s disease (which may be referred to as adrenal insufficiency, adrenal fatigue or hypoadrenalism). The following evidence is presented: [58-60] · Many of the symptoms of chronic fatigue syndrome overlap those of Addison’s disease (adrenal failure). · Improvement in CFS patients has occurred after supplementation with mineralocorticoids (fludrocortisone), low-dose hydrocortisone (cortisol), and licorice (an old herbal remedy for Addison’s disease). Cortisol is the main glucocorticoid secreted the adrenal glands. It has two main functions: · Cortisol increases blood glucose levels during periods of stress (the “fight or flight response”) by mobilizing carbohydrates, lipids and protein. It also stimulates the breakdown of fats to release energy. Cortisol inhibits the effects of insulin and decreases the rate of glucose use by cells. · Glucocorticoids, including cortisol, are anti-inflammatory. They inhibit histamine secretion, inhibit lymphocyte production, and stabilize macrophage lysosomes. Cortisol production by the adrenal glands follows a diurnal rhythm: They are elevated in the morning and lower in the evening (during sleep). People under stress often have no diurnal variation in their cortisol levels. An article published in the journal Neuropsychobiology described a study in which morning and evening serum cortisol and ACTH concentrations, and diurnal changes in hormone levels, were measured in 30 patients with chronic fatigue syndrome (CFS) but without concurrent depressive disorder and a control group of 15 matched healthy volunteers. The diurnal change in cortisol levels was significantly less in CFS than in controls. In CFS subjects, evening levels of cortisol correlated significantly with measures of general health and physical functioning, while diurnal change in cortisol was positively correlated with measures of functional improvement over the past year and current social functioning. An article published in the Journal Clinical Endocrinology Metabolism described a study of cortisol levels in thirty CFS patients and 72 normal volunteers. Compared to normal subjects, CFS patients demonstrated significantly reduced basal evening glucocorticoid levels and low 24-hour urinary free cortisol excretion, but elevated basal evening ACTH concentrations. There was increased sensitivity to ACTH, but a reduced maximal response. The authors concluded that primary adrenal insufficiency or a pituitary source is unlikely, and that the data was compatible with a mild central adrenal insufficiency secondary to either a deficiency of CRH (a secretion of the hypothalamus) or some other central stimulus to the pituitary-adrenal axis. One study measured the morning and evening salivary cortisol levels obtained on consecutive days in the first 3 days of the menstrual cycle in 14 patients with chronic fatigue syndrome, 26 community cases of ICD-10 current depressive episodes and 131 healthy community controls. The mean evening cortisol was significantly lower in the chronic fatigue syndrome patients compared to controls with depression and healthy controls. Chronic fatigue syndrome patients without psychiatric disorder had significantly lower morning salivary cortisol levels compared to controls. An article published in the Journal of Affective Disorders described a study in which cortisol levels were measured in 10 patients with CFS, 15 patients with major depression, and 25 healthy controls. Baseline circulating cortisol levels were highest in the depressed, lowest in the CFS, and intermediate between the two in the control group. Prolactin responses to the selective serotonin-releasing agent d-fenfluramine were lowest in the depressed, highest in the CFS, and intermediate between both in the healthy group. The authors concluded that depression is associated with hypercotisolemia and reduced central serotonin neurotransmission and suggest that CFS may be associated with hypocortisolemia and increased 5-HT function. One interesting study measured the size of the adrenal glands in eight CFS patients that had evidence of adrenal hypofunction (determined by a subnormal resonse to an ACTH stimulation test). The right and left adrenal gland bodies were reduced by over 50% in the CFS subjects indicative of significant adrenal atrophy in a group of CFS patients with abnormal endocrine parameters. Natural Supplements to Support Adrenal Function DHEA (dehydroepiandrosterone) is a hormone secreted from the adrenal glands. It is a precursor of the sex hormones (estrogen and testosterone). DHEA-S has recently been shown to have beneficial effects on memory, stress, anxiety, sleep and depression. Therefore, the deficiency of DHEA-S might be related to the symptoms in patients with CFS. DHEA has been reported to improve energy levels in chronic fatigue patients. One study showed the value of DHEA and vitamin C infusion treatment in the control of chronic fatigue syndrome. A study of 15 subjects with CFS, 15 subjects with major depression, and 11 healthy subjects found that DHEA and DHEA-S levels were significantly lower in the CFS compared to the healthy group. DHEA-S levels, but not DHEA, were lower in the depressives. The authors concluded that DHEA has a potential role both therapeutically and as a diagnostic tool, in CFS. Another study of DHEA levels in 22 CFS patients and 14 healthy controls found normal basal DHEA levels, but a blunted serum DHEA response curve to ACTH (adreno-corticotropic hormone) injection. ACTH normally stimulates the adrenal glands to secrete DHEA. The authors concluded that endocrine abnormalities play a role in CFS and that a relative glucocorticoid deficiency might contribute to the overall clinical picture in CFS. Licorice is highly valued as a medicinal herb by the Chinese and is an ingredient in almost all of the Chinese patent herbal formulas. Licorice has a sweet taste and helps combat fatigue. The active constituent in licorice, glycyrrhizin, stimulates the production of hormones, including cortisone, and stimulates the production of interferon, which boosts immunity. Licorice is an old herbal remedy that was used medically for Addison’s disease and adrenal insufficiency. [72, 73] Licorice should be used with care since it is well-known to increase blood pressure. Two to four 500 mg capsules can be taken twice a day. Licorice may also be consumed as a tea (one cup in the morning). Orthostatic hypotension is defined as an excessive fall in blood pressure on standing, usually greater than 20/10 mmHg. It is considered to be a manifestation of abnormal blood pressure regulation due to a variety of causes. Hypotension, particularly orthostatic hypotension, is a common symptom in chronic fatigue patients. Many people with CFS have chronic low blood pressure (the normal is 120/80 mmHg), which is made even worse on standing. This may be a particular problem in the morning, when standing can cause dizziness. Exercise or a heavy meal may exacerbate the symptoms. Syncope is a loss of consciousness and postural tone caused by diminished cerebral blood flow. Syncope often occurs during the morning shower, perhaps due to the vasodilating effect of hot water. There are several mechanisms that govern blood pressure. Upon standing, a large amount of blood pools in the veins of the legs and trunk. The transient decrease in venous return to the heart results in a low blood pressure. The body responds with a sympathetic-mediated release of catacholamines (norepinephrine and epinephrine) that increase heart rate contraction and vasoconstricts the arteries. With continued standing, antidiuretic hormone (ADH) is secreted which activates the renin-angiotensin-aldosterone system subsequently causing sodium and water retention and an expansion of the circulating blood volume. There are many causes of orthostatic hypotension, including: · Hypovolemia (low blood volume) induced by excessive use of diuretic agents (e.g., loop diuretics such as furosemide, bumetanide, and ethacrynic acid) and relative hypovolemia due to vasodilator therapy with nitrate preparations and calcium antagonists (verapamil, nifedipine, or diltiazem) or with angiotensin converting enzyme (ACE) inhibitors. · Histamine, a key player in allergic reactions, induces vasodilation and hypotension. · Potassium deficiency (hypokalemia) impairs the reactivity of vascular smooth muscle and may limit the increase in peripheral vascular resistance on standing. · The adrenocortical hypofunction of Addison’s disease may lead to orthostatic hypotension in the absence of adequate salt intake. · Several classes of drugs reversibly impair autonomic reflexes and reduce blood pressure on standing as an important adverse effect. These include many drugs used to treat psychiatric disorders such as the monoamine oxidase inhibitors (MAOIs) (isocarboxazid, phenelzine, and tranylcypromine) used to treat depression; the tricyclic antidepressants (nortriptyline, amitriptyline, desipramine, imipramine, and protriptyline) or tetracyclic antidepressants; and the phenothiazine antipsychotic drugs (chlorpromazine, promazine, and thioridazine). Other drugs that may produce orthostatic hypotension are quinidine, l-dopa, barbiturates, and alcohol. Vasopressin is a hormone secreted by the posterior pituitary gland that is also called antidiuretic hormone (ADH) because its principle effect is to cause retention of water by the kidneys. Vasopressin has several effects on the body in addition to the effect on water retention: · Vasopressin causes vasoconstriction of blood vessels which can increase blood pressure · Vasopressin induces secretion of ACTH in the anterior pituitary which stimulates cortisol production in the adrenal glands · Vasopressin also has a role in memory. Vasopressin is made from several amino acids, including cysteine, tyrosine, proline, glycine and arginine. Vaspressin secretion is affected by several stimuli, including: · Increased secretion of ADH is diagnosed as syndrome of inappropriate antidiuretic hormone (SIADH) and can be caused by: o Increased osmotic water pressure o Decreased extracellular fluid volume o Nicotine; Morphine, barbiturates, chlorpropramide, clofibrate, carbamazepine, angiotensin II o Pain, emotion, stress, exercise, standing o Nausea and vomiting · Decreased secretion of ADH can be caused by: o Decreased osmotic water pressure or Increased extracellular fluid volume o Butophanol, oxilorphan o Diabetes insipidus is decreased ADH or insensitivity to ADH which results in the passage of large amounts of dilute urine (polyuria) and thirst (polydipsia). Several studies have linked problems with vasopressin to chronic fatigue: [74, 75] · One study of 19 patients with chronic fatigue syndrome and 19 healthy controls found that patients with chronic fatigue syndrome had a reduced ACTH response to vasopressin infusion and a more rapid cortisol response to the infusion. · Another study of nine patients with postviral fatigue syndrome found low baseline levels and an erratic secretion of arginine vasopressin in the patients with postviral fatigue syndrome. · An article published in the journal Biological Psychiatry described a study of chronic fatigue syndrome patients which found that the combination of CRH and desmopressin (a synthetic analog of vasopressin) normalized the pituitary-adrenal response to CRH. The most common form of vasopressin available in the United States is lysine vasopressin made by Sandoz Pharmaceuticals in the form of a nasal spray called Diapid (Lypressin). Many people are experimenting with Diapid to help increase their memory. It may also be of use in chronic fatigue syndrome. Sodium is known to increase blood pressure and cardiac patients are usually placed on sodium-restricted diets. Chronic fatigue syndrome patients, however, often have hypotension. One study examined the use of sodium chloride (1200 mg) in a sustained-release formulation for 3 weeks in 22 patients with CFS and orthostatic hypotension. Of these 22 patients, 10 redeveloped orthostatic hypotension, while 11 did not show an abnormal response to the test and reported an improvement of CFS symptoms. However, those CFS patients who again developed an abnormal response to tilt-test had a significantly reduced plasma renin activity compared both with healthy controls and with those 11 chronic fatigue patients who improved after sodium chloride therapy. The testing of the renin levels of chronic fatigue syndrome patients that did not respond to sodium chloride is an important distinction in this study. As discussed earlier, orthostatic hypotension can be due to a several factors. The focus of this study was on adrenal hypofunction with inadequate salt intake. Those that did not respond to the salt intake had a reduction in renin, which increases blood pressure. Figure: The Renin-Angiotensin-Aldosterone System Sodium restriction has become a popular for those with high blood pressure (and associated increased cardiovascular risk factors). Chronic fatigue syndrome patients with low blood pressure and orthostatic hypotension do not need to restrict dietary sodium intake. Using sodium therapeutically should be done under the care of a well-trained and knowledgeable physician or cardiologist. Neurotransmitters and CFS Deficiencies in brain hormones and neu-rotransmitters are also known to cause low levels of energy. Catecholamines are neurotransmitters that are primarily secreted during times of stress. The principal catecholamines found in the body include norepinephrine, epinephrine, and dopamine. They are formed from the amino acid tyrosine. Figure: Tyrosine Metabolism Supplements That Support Neurotransmitter Function A study of the catecholamine production was performed on rats after swimming for a period of 8 hours. There was a decreased production of catecholamines after swimming. In the presence or L-tyrosine, there is at first an activation of noradrenaline synthesis, followed by a gradual normalization (on the 7th day) of adrenaline formation. One study examined the alterations in metabolism of catecholamines (adrenaline, noradrenaline, their precursors DOPA and dopamine, and their acid metabolites vanillyl mandelic acid) in sportsmen after development of acute fatigue as a result of the test physical loading. The authors found that excretion of catecholamines and their precursors was decreased for a long time after development of chronic fatigue in the resting state and the increase in excretion of the substances studied was not observed after physical loading. An article published in the journal Medical Science of Sports Exercise described a study of the effects of tyrosine on exercise tolerance and brain neurochemistry of mice. Tyrosine injections improved maze performance and prevented increase of levels of serotonin (5-HT) in the hypothalamus that follows exercise. Tyrosine administration significantly improved food consumption, cognitive behavior, and activity performance. The authors concluded that tyrosine may improve exercise tolerance and delay fatigue. An article published in the journal Brain Research Bulletin described a study of the effects of tyrosine on a group of 21 cadets during a demanding military combat training course. Ten subjects received five daily doses of a protein-rich drink containing 2 grams of tyrosine, and 11 subjects received a carbohydrate rich drink with the same amount of calories (255 kcal). The group supplied with the tyrosine-rich drink performed better on a memory and a tracking task than the group supplied with the carbohydrate-rich drink. In addition, the supplementation of tyrosine decreased systolic blood pressure. No effects on mood were found. The authors concluded that these findings suggest that supplementation with tyrosine may, under operational circumstances characterized by psychosocial and physical stress, reduce the effects of stress and fatigue on cognitive task performance. The amino acids phenylalanine or tyrosine, taken in daily doses of 1500 mg, can boost epinephrine and norepinephrine levels. Caution phyenylalanine and tyrosine should not be taken by those using MAO inhibitors and should be used with caution in those on thyroid medication (Synthroid). Magnesium is involved primarily with muscle relaxation. Acetylcholine is the neurotransmitter with calcium and magnesium regulating the amount of acetylcholine released. Calcium causes muscular contraction, while magnesium causes muscular relaxation. Magnesium deficiency causes neuromuscular irritability with muscle tightness and spasm and nerve conduction problems. It also affects the heart and cause hypertension or hypotension. Magnesium deficiency is a common cause of premenstrual cramping, and also causes fatigue. An article published in Lancet described a randomized, double-blind, placebo-controlled study of 20 patients with CFS. The CFS patients were found to have lower red cell magnesium concentrations. In a clinical trial, 32 CFS patients received either placebo or intramuscular magnesium sulfate every week for 6 weeks. Patients treated with magnesium claimed to have improved energy levels, better emotional state, and less pain, as judged by changes in the Nottingham health profile. Red cell magnesium returned to normal in all patients on supplemental magnesium but in only 1 patient on placebo. The authors concluded that these results show that magnesium may have a role in CFS. One study, however, found no difference in red blood cell magnesium concentrations in samples from 89 patients with CFS when compared to age and sex matched group selected from the normal population. A magnesium-loading test on six patients found no evidence of deficiency. A study of 97 chronic fatigue patients (chronic fatigue syndrome, fibromyalgia or/and spasmophilia) was conducted in Belgium. An IV loading test showed a magnesium deficit in 44 patients. After magnesium supplementation in 24 patients, the loading test showed a significant decrease in magnesium retention. Mean values of magnesium in the serum, red blood cell, and urine showed no significant difference between patients with or without magnesium deficiency. Serum magnesium levels were found to be significantly lower in the patients with spasmophilia (muscle cramps, twitching and spasms) than in the other patients. A study of 93 patients with unexplained chronic fatigue (54% with CFS) examined the relationship between magnesium deficiency and oxidative stress. Magnesium deficient patients (47%) had lower total antioxidant capacity in plasma, which was related to serum albumin. Magnesium deficient patients whose magnesium body stores did not improve after oral supplementation with magnesium (10 mg/kg/day) had persistently lower blood glutathione levels. The authors concluded that magnesium supplementation was followed by an improvement in magnesium body stores, in serum vitamin E, and its interrelated stage of lipid peroxidation. Magnesium plays a crucial role in metabolism. It is needed for activating B vitamins, relaxing muscles, and forming ATP, the energy molecule. Fatigue, muscle cramps and constipation are signs a magnesium deficiency. Normal concentrations of magnesium in blood do not rule out the diagnosis of the nervous form of primary chronic magnesium deficiency. The diagnosis of magnesium deficiency requires an oral magnesium load test. Most people do not consume enough magnesium in their diet. Magnesium is often paired with calcium as they work together and compete for absorption. Determining the proper ratio of calcium to magnesium is important, but can be determined readily. Taking too much magnesium often leads to diarrhea. An easy method would be to begin with one capsule of magnesium once or twice a day. The dose is increased until the stools become watery, then backed off to maintain a normal consistency of stools. Tryptophan and 5-HTP Tryptophan is the precursor for the neurotransmitter serotonin (5-hydroxytryptamine, 5-HT), which is involved in fatigue and sleep. It is present in bound and free form in the blood, where the concentration is controlled by albumin binding to tryptophan. Figure: Tryptophan Metabolism Several older studies found that tryptophan was helpful in chronic fatigue. [90, 91] A recent study found that plasma-free tryptophan was significantly decreased in CFS patients. Tryptophan was banned for use as a supplement by the FDA following several deaths due to contaminated batches. Many companies are now making 5-HTP as a substitute for tryptophan. It has become a popular supplement used for depression. Unfortunately, 5-HTP is not as safe as tryptophan, particularly because it bypasses the enzyme tryptophan hydroxylase. As such, it can be converted into serotonin in peripheral tissue instead of in the brain. For this reason Life Extension Foundation recommends against the use of 5-HTP. Life Extension also encourages the FDA to reconsider its ban on tryptophan. Of particular concern to those that are considering supplementing with 5-HTP is that abnormally high levels (which can be achieved by supplementation or by prolonged exercise) can cause central fatigue. [93, 94] The following drugs are associated with side effects of chronic fatigue syndrome. Table: Medications that may cause fatigue Source: Physicians Desk Reference. Compiled by www.HolisticOnline.com There are currently no prescription medications approved by the FDA for use in chronic fatigue syndrome. There are, however, quite a number of medications that are used to treat the various symptoms of Chronic Fatigue Syndrome. Many are recommended for effects that may be unrelated the their primary use. These may include anti-depressants, anti-fungals, anti-histamines, anti-virals, CNS depressants (or stimulants), immunoglobulins, cardiac medications, anti-inflammatories, anti-convulsants, corticoids, and expectorants. Ampligen is an experimental anti-viral medication currently in phase III testing for the treatment of CFS. It is considered a “second generation interferon.” In clinical trials conducted outside the US, over 50% of test subjects taking Ampligen showed both physical and mental improvement of symptoms. Unfortunately, most of the study groups were too small for the results to be published in the scientific literature. Hemisherx, the drug manufacturer is hoping the current research trials will provide enough hard data to meet the criteria of FDA approval. Hydrocortisone is an anti-inflammatory that can be taken orally or administered topically. An article published in the journal Lancet described a study of 218 patients with chronic fatigue syndrome that received hydrocortisone (5 or 10 mg daily) for one month and placebo for one month. Self-reported fatigue scores for patients on hydrocortisone fell by 7.2 points, compared with 3.3 points for those on placebo. One researcher, however, concluded that although hydrocortisone treatment was associated with some improvement in symptoms, the degree of adrenal suppression precludes its practical use for CFS. Deprenyl, a MAO inhibitor Deprenyl or Derprenil, is also known as Eldepryl (Selegiline). It is an MAO-B inhibitor that is commonly used in Parkinson’s disease, in combination with levodopa or levodopa and carbodopa. Deprenyl inhibits the breakdown of dopamine by monoamine oxidase B (MAO-B). An article published in the journal Neuropsychobiology described a 6-week clinical trial of selegiline in 25 patients with chronic fatigue syndrome (CFS). Participants received placebo for two weeks, then 5 mg selegiline per day for two weeks, followed by two 5 mg tablets for two weeks. A significant improvement in tension/anxiety, vigor and sexual relations was found as compared with placebo. The authors concluded that selegiline has a small but significant therapeutic effect in CFS. Chronic Fatigue Syndrome is debilitating fatigue and associated symptoms lasting at least 6 months. The cause of CFS is as yet undetermined, but it may be triggered by infectious agents (especially viruses), stress, vitamin deficiencies, immunologic dysfunction, neurotransmitter deficits, adrenal or thyroid deficiency. 1. Ginseng (500 mg twice a day) has been found to enhance NK function in CFS patients. Ginseng is commonly used to help increase energy levels. 2. Echincea (500 mg twice a day) supports the immune system and has been found to enhance NK function in CFS patients. 3. Essential fatty acids may be of benefit in chronic fatigue. 4. Acetyl-L-carnitine (1000 to 2000 mg a day), Vitamin E (400 IU a day), and NADH (5 mg 2 times a day) support fat metabolism and may increase energy. 5. Whey protein should be considered as a source of amino acids and to enhance immunity and boost glutathione levels. 6. Lactoferrin (300 mg three times daily) has been shown to have significant antiviral properties and may be useful in chronic fatigue syndrome. 7. Glutathione (500 mg per day), and its precursors Glutamine (one gram per day) and N-Acetyl Cysteine (500 mg per day) are important antioxidants. Glutamine should not be taken at night as it may cause insomnia. 8. Vitamin B6, B12, folic acid (800 mcg per day) and trimethylglycine should be considered if homocysteine levels are elevated. SAMe (200 to 800 mg a day), a methyl donor, may be beneficial for symptoms of depression. 9. Coenzyme Q10 (100 mg 3 times a day) may be helpful in CFS for increased energy. 10. DHEA and melatonin can be considered based on appropriate lab testing. 11. Licorice (250 mg three times a day) may help with fatigue, particularly when it’s related to adrenal insufficiency. Care should be taken as high doses of licorice may increase blood pressure. 12. The amino acids phenylalanine or tyrosine, taken in daily doses of 1500 mg, will help to boost levels of brain hormones and neurotransmitters. 13. Magnesium may be deficient in 80% of all Americans and may be of particular importance in chronic fatigue. Everyone should consider supplementing with 500 mg of magnesium daily. Up to 3 grams of magnesium may be taken. Doseage should be reduced if an unwanted laxative effect occurs. 14. Few, if any, supplements contain sodium due to its adverse effect on blood pressure. Substituting sea salt for sodium chloride (common table salt) may be beneficial for those not on a sodium-restricted diet. 1. Reid, S., et al., Chronic fatigue syndrome. Bmj, 2000. 320(7230): p. 292-6. 2. 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Castell, L.M., et al., The role of tryptophan in fatigue in different conditions of stress. Adv Exp Med Biol, 1999. 467: p. 697-704. 94. Blomstrand, E., et al., Effect of sustained exercise on plasma amino acid concentrations and on 5-hydroxytryptamine metabolism in six different brain regions in the rat. Acta Physiol Scand, 1989. 136(3): p. 473-81. 95. Cleare, A.J., et al., Low-dose hydrocortisone in chronic fatigue syndrome: a randomised crossover trial. Lancet, 1999. 353(9151): p. 455-8. 96. McKenzie, R., et al., Low-dose hydrocortisone for treatment of chronic fatigue syndrome: a randomized controlled trial. Jama, 1998. 280(12): p. 1061-6. 97. Natelson, B.H., et al., Single-blind, placebo phase-in trial of two escalating doses of selegiline in the chronic fatigue syndrome. Neuropsychobiology, 1998. 37(3): p. 150-4. The next step, after you have been diagnosed with a disease, is to find out what is the specific cause, and then choose supplements that address it. Phone consults are my specialty. Please call my at (239) 659-2684 to schedule a consultation. I recommend that you make an informed choice, and the goal of this web site is to provide you with the information to make a wise choice when it comes to your health and wellness. As you can see, I don't sell any vitamins on this web site. There are thousands of vitamin stores that will sell you whatever vitamin you want, and many of them offer discounts. If you are interested in some high-quality basic nutritional supplements, I recommend visiting the Store You will find my recommendations for a multiple, tasty chewable fiber wafers, high-potency probiotics, and fish oils. Good luck in your journey towards health and wellness!! Unfortunately, that would be a clear violation of FDA regulations. Also, one of the most important aspect of naturopathy is: "Treat the person, not the disease". This is a profound statement, and many people have spent thousands of dollars on vitamins listed in books or web sites for a specific disease. This is what I call "vending machine medicine". It rarely works! The solution is to find a naturopathic physician, such as myself, to assist you.	2	38	0	0	0	7	0.985201	7	18,161
\|Part of a series on\| Transsexualism is when an individual identifies with a gender inconsistent or not culturally associated with their assigned sex, i.e. in which a person's assigned sex at birth conflicts with their psychological gender. A medical diagnosis can be made if a person experiences discomfort as a result of a desire to be a member of the opposite gender, or if a person experiences impaired functioning or distress as a result of that gender identification. Transsexualism is stigmatized in many parts of the world, but it has become more widely known in Western culture since the mid to late 20th century, concurrently with the sexual revolution and the development of sex reassignment surgery (SRS). There are cultures that have no difficulty integrating people who change gender roles, often holding them with high regard. Some maintain that transsexuals uphold the traditional role of what were called "Two-Spirit" people found among certain Native American tribes. - 1 Diagnosis - 2 Relation to gender roles - 3 Origins - 4 Relation to transgenderism - 5 Terminology - 6 Prevalence - 7 Causes - 8 Sex reassignment therapy - 9 Legal and social aspects - 10 Coming out - 11 In the media - 12 Thailand - 13 History and other cultures - 14 Customs and traditions - 15 Organizations - 16 See also - 17 References - 18 Bibliography - 19 External links Transsexualism appears in the two major diagnostic manuals used by mental health professionals worldwide, the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders (DSM, currently in its fifth edition) and the International Statistical Classification of Diseases and Related Health Problems (ICD, currently in its tenth edition). The ICD-10 incorporates transsexualism, dual role transvestism, and gender identity disorder of childhood into its gender identity disorder category. It defines transsexualism as "[a] desire to live and be accepted as a member of the opposite sex, usually accompanied by a sense of discomfort with, or inappropriateness of, one's anatomic sex, and a wish to have surgery and hormonal treatment to make one's body as congruent as possible with one's preferred sex." The DSM does not distinguish between gender identity disorder and transsexualism, and defines transvestic fetishism as a separate phenomenon which may co-occur with transsexualism. The DSM diagnosis requires four components: - A desire or insistence that one is of the opposite biological sex (that is not due to a perceived advantage of being the other sex). - Evidence of persistent discomfort with, and perceived inappropriateness of the individual's biological sex. - The individual is not intersex (although a diagnosis of GID Not Otherwise Specified is available, which enables intersex people who reject their sex-assignment to access transsexual treatments). - Evidence of clinically significant distress or impairment in work or social life. \|This section needs additional citations for verification. (July 2009)\| The current diagnosis for transsexual people who present themselves for psychological treatment is "gender dysphoria" (leaving out those who have sexual identity disorders without gender concerns). The DSM changed its terminology in 1994. According to the Standards Of Care formulated by the World Professional Association for Transgender Health (WPATH), formerly the Harry Benjamin International Gender Dysphoria Association, this diagnostic label is often necessary to obtain sex reassignment therapy with health insurance coverage, and states that the designation of gender identity disorders as mental disorders is not a license for stigmatization, or for the deprivation of gender patients' civil rights. However, some people diagnosed with gender identity disorder have no desire for sex reassignment therapy, nor for genital reassignment surgery, and/or are not appropriate candidates for such treatment. While some feel that formal diagnosis helps to destigmatize transsexualism, others feel that it only adds stigma. They contend that such a diagnosis is equivalent to saying something is wrong with transsexual people. Some transsexual people consider the diagnosis to be a causal factor in instances of harm or death of transsexual people as the result of prejudice and discrimination when deprived of their civil rights. (Brown 105) Many transsexual people have asked the American Psychiatric Association to remove Gender Identity Disorder from the DSM, and the World Health Organization to remove it from ICD-10, where it has been listed for some time. Some believe that at least some mental health professionals are being insensitive by labeling transsexualism as "a disease", rather than as an inborn trait, which those who have it believe it to be. The Principles 18 of The Yogyakarta Principles, documents on international human rights law opposes such diagnosis as mental illness as medical abuse, as well as "Activist's Guide to the Yogyakarta Principles". In response to these concerns, in 2013 the American Psychiatric Association revised the DSM-4's criteria for diagnosing gender identity disorder, and retitled this condition to "gender dysphoria" in the DSM-5. Relation to gender roles \|This section does not cite any references or sources. (January 2011)\| Transsexual people may refer to themselves as trans men or trans women. Transsexual people often desire to establish a permanent gender role as a member of the gender with which they identify. Some transsexual people pursue medical interventions as part of the process of expressing their gender. These medically based, physical alterations are collectively referred to as sex reassignment therapy, and may include female-to-male or male-to-female hormone replacement therapy, or various surgeries. Surgeries may include genital surgery such as orchiectomy or sex reassignment surgery; chest surgery such as top surgery or breast augmentation; or, in the case of trans women, facial surgery such as trachea shave or facial feminization surgery. The entire process of switching from one physical sex and social gender presentation to another is often referred to as transition, and usually takes several years. Not all transsexual people undergo a physical transition. Some find reasons not to, for example, the expense of surgery, the risk of medical complications, medical conditions which make the use of hormones or surgery dangerous. Some may not identify strongly with another binary gender role. Others may find balance at a mid-point during the process, regardless of whether they are binary-identified. Many transsexual people, including binary-identified transsexual people, do not undergo genital surgery, because they are comfortable with their own genitals, or because they are concerned about nerve damage and the potential loss of sexual pleasure and orgasm. This is especially so in the case of trans men, many of whom are dissatisfied with the current state of phalloplasty, which is typically very expensive, not covered by health insurance, and which does not result in a fully erectile, sexually sensate penis. Some transsexual people live heterosexual lifestyles and gender roles, while some identify as gay, lesbian, or bisexual. Many trans people find that a shift occurs in their sexual orientation as they undergo transition. Many transsexual people choose the language of how they refer to their sexual orientation based on their gender identity, not their morphological sex, though some transsexual people still find identification with their community: many trans men, for instance, are involved with lesbian communities. Gender was originally a linguistic term. In many languages, words can be considered masculine, feminine, or neutral, completely independently from the attributes of the things to which the word applies. Different languages manifest gender in various ways, recognizing two genders (female, male), three genders (female, male, neuter), or in some cases none at all. In some (e.g. the Romance languages), variation by gender is indicated by relatively simple changes in nouns and adjectives, while others require more complex grammatical changes. In English, a transsexual person's first step in transition often includes the request to be referred to using pronouns for their target gender (she rather than he, her rather than him, and hers rather than his, or vice versa). Some English speakers[who?] who feel that they are best described as something in between or other than masculine or feminine prefer to use "they" and "them", as well as “ze” and “hir” (examples of gender-neutral pronouns in English) or other invented neutral pronouns. Norman Haire reported that in 1921, Dora-R of Germany under the care of Magnus Hirschfeld, began surgical transition from 1921, ending in 1930 with a successful genital reassignment surgery. In 1930, Hirschfeld supervised the second genital reassignment surgery to be reported in detail in a peer-reviewed journal on Lili Elbe of Denmark. The German term, “Transsexualismus,” was introduced by Hirschfeld in 1923. The neo-Latin term “psychopathia transexualis” and English “transexual” were introduced by D. O. Cauldwell in 1949, who subsequently also used the term “trans-sexual” in 1950. Cauldwell appears to be the first to use the term to refer to those who desired a change of physiological sex. (In 1969, Benjamin claimed to have been the first to use the term “transsexual” in a public lecture, which he gave in December 1953.) This term continues to be used by the public and medical profession alike. It was included for the first time in the DSM-III in 1980 and again in the DSM-III-R in 1987, where it was located under Disorders Usually First Evident in Infancy, Childhood or Adolescence. The word transsexual was used by Harry Benjamin in his seminal 1966 book, The Transsexual Phenomenon, to describe transsexual people on a scale (later called the "Benjamin scale") that recognizes three levels of intensity of transsexualism: "Transsexual (nonsurgical)", "Transsexual (moderate intensity)", and "Transsexual (high intensity)". In his book, Benjamin described "true" transsexualism as the following: "True transsexuals feel that they belong to the other sex, they want to be and function as members of the opposite sex, not only to appear as such. For them, their sex organs, the primary (testes) as well as the secondary (penis and others) are disgusting deformities that must be changed by the surgeon's knife." Benjamin suggested that moderate intensity male to female transsexual people may benefit from estrogen medication as a "substitute for or preliminary to operation." Some people have had SRS but do not meet the common definition of a transsexual (e.g., Gregory Hemingway). Other people do not desire SRS although they meet Dr. Benjamin's definition of a "true transsexual". Beyond Benjamin's work, which focused on male-to-female transsexual people, there are cases of the female to male transsexual, for whom genital surgery may not be practical. Benjamin gave certifying letters to his MTF transsexual patients that stated 'Their anatomical sex, that is to say, the body, is male. Their psychological sex, that is to say, the mind, is female'. Beyond 1967 Benjamin abandoned his early terminology and adopted that of "gender identity." Relation to transgenderism \|This section needs additional citations for verification. (August 2009)\| Transsexualism is often included within the broader category of transgenderism, which is generally used as an umbrella term for people who do not conform to typical accepted gender roles. It includes cross-dressers, transvestites, and people who identify as genderqueer. Transsexualism refers to a specific condition in the transgender realm. Although a crossdresser and transsexual are both classified as transgender people, their conditions differ radically. Though some people use transgenderism and transsexualism interchangeably, they are not synonymous terms. The term transsexual originated in the medical and psychological communities, and is an older term than transgender. Many prefer the term transgender and consider transsexual to be outdated and derogatory, due to its roots in describing the identity as a disease. Additionally, transsexual is considered a misnomer by some people because the underlying condition is related to gender identity and not sexuality. Others prefer to identify as transsexual, and object to being included in the transgender spectrum. Anthropologist David Valentine provides context for this objection in his 2007 book Transgender, an Ethnography of a Category. He writes that transgender is a term coined and used by activists to include many people who do not necessarily identify with the term. He observes that many current health clinics and services set up to serve gender-variant communities employ the term, but that most of the service-seekers do not identify with this term. The rejection of this political category, first coined by self-identified activist Leslie Feinberg, illustrates the difference between a self-identifier and categories imposed by observers to understand other people. However, these observations are contested by the Transgender Health Program at Fenway Health in Boston. They note that there are no universally accepted definitions, and terminology confusion is common because terms that were popular in at the turn of the 21st century may now be deemed offensive. They suggest that transgender is the more widely accepted term. They recommend that clinicians avoid the term transsexual unless they are sure that a client is comfortable with it. It is best to ask an individual which term, if any, is preferred. Historically the reason that transsexual people rejected associations with the transgender or broader LGBT community is largely that the medical community in the 1950s through the late 1980s encouraged this rejection of such a grouping in order to qualify as a 'true transsexual' who would thus be allowed to access medical and surgical care. The animosity that is present today is no longer fed by this same kind of pressure from the medical community. Though the beliefs of some modern-day transsexual people that they are not "transgender" reflect this historical division, other transsexual people state that those who do not seek SRS are very different from those who need to be of "the other sex", and that these groups have different issues and concerns and are not doing the same things. The latter view is rather contested, with opponents pointing out that merely having or not having some medical procedures hardly can have such far-reaching consequences as to put those who have them and those who have not into such distinctive categories. Notably Harry Benjamin's original definition of transsexualism does not require that they need to have had SRS. The word "transsexual" is most often used as an adjective rather than a noun – a "transsexual person" rather than simply "a transsexual". Transsexual people prefer to be referred to by the gender pronouns and terms associated with their target gender. For example, a transsexual man is a person who was assigned the female sex at birth on the basis of his genitals but, despite that assignment, identifies as a man and is transitioning or has transitioned to a male gender role. He has or will have a masculine body. Transsexual people are sometimes referred to with "assigned-to-target" sex terms such as "female-to-male" for a transsexual man or "male-to-female" for a transsexual woman. These terms may be abbreviated as "M2F", "F2M", "MTF", "FTM", "F to M", etc. Individuals who have undergone and completed sex reassignment surgery (SRS) are sometimes referred to as transsexed individuals; however, the term transsexed is not to be confused with the term transexual, which can also refer to individuals who have not yet undergone SRS, and whose anatomical sex (still) does not match their psychological sense of personal gender identity. The term "gender dysphoria" and "gender identity disorder" were not used until the 1970s, when Laub and Fisk published several works on transsexualism using these terms. "Transsexualism" was replaced in the DSM-IV by "gender identity disorder in adolescents and adults". Male-to-female transsexualism has sometimes been called "Harry Benjamin's Syndrome" after the endocrinologist who pioneered the study of dysphoria. As noted above, the present-day medical study of gender variance is much broader than Benjamin's early description. Use of this term has been criticized for delegitimizing gender-variant people with different experiences. The DSM-IV (1994) says that roughly 1 in 30,000 assigned males and 1 in 100,000 assigned females seek sex reassignment surgery in the US. The most frequently quoted estimate of prevalence is from the Amsterdam Gender Dysphoria Clinic The data, spanning more than four decades in which the clinic has treated roughly 95% of Dutch transsexual clients, gives figures of 1:10,000 among assigned males and 1:30,000 among assigned females. Though no direct studies on the prevalence of GID have been done, a variety of clinical papers published in the past 20 years provide estimates ranging from 1:7,400 to 1:42,000 in assigned males and 1:30,040 to 1:104,000 in assigned females. Olyslager and Conway presented a paper at the WPATH 20th International Symposium (2007) arguing that the data from their own and other studies actually imply much higher prevalence, with minimum lower bounds of 1:4,500 male-to-female transsexual people and 1:8,000 female-to-male transsexual people for a number of countries worldwide. They estimate the number of post-op women in the US to be 32,000 and obtain a figure of 1:2500 male-to-female transsexual people. They further compare the annual incidences of SRS and male birth in the U.S. to obtain a figure of 1:1000 MTF transsexual people and suggest a prevalence of 1:500 extrapolated from the rising rates of SRS in the U.S. and a "common sense" estimate of the number of undiagnosed transsexual people. Olyslager and Conway also argued that the U.S. population of assigned males having already undergone reassignment surgery by the top three U.S. SRS surgeons alone is enough to account for the entire transsexual population implied by the 1:10,000 prevalence number. This excludes all other U.S. SRS surgeons, surgeons in countries such as Thailand, Canada, and others, and the high proportion of transsexual people who have not yet sought treatment, suggesting that a prevalance of 1:10,000 is too low. A study in 2008 examined the number of New Zealand passport holders who changed the sex on their passport and estimated that 1:3,639 birth-assigned males and 1:22,714 birth-assigned females were transsexual. A presentation at the LGBT Health Summit in Bristol, UK, based upon figures from a number of reputable European and UK sources, shows that this population is increasing rapidly (14% per year) and that the mean age of transition is actually rising. Psychological and biological causes for transsexualism have been proposed, i.a. by professor Dick Swaab, with evidence leaning toward prenatal and genetic causes. One such proposed cause is related to the bed nucleus of a stria terminalis, or BSTc, a constituent of the basal ganglia of the brain which is affected by prenatal androgens. In one study, the BSTc of male-to-female transsexual women was similar to those of cisgender women whose psychological gender identity and assigned sex are the same. However, those of both heterosexual and homosexual men were similar to each other but different from those of women (both cis- and transsexual). Another study suggests that transsexuality may have a genetic component. There is considerable evidence that prenatal exposure to endocrine-disrupting anti-miscarriage drugs such as diethylstilbestrol (DES) may also be positively associated with transsexualism, though research in this area has yet to establish a firm causal link. Some people consider research into the "causes" of transsexualism to be based on the possibility that it is a pathology, a possibility that is rejected by many transsexual people. Others think of the condition as a form of intersexuality, and support research into possible causes, believing that it will verify the theory of a biological origin and thereby reduce social stigma by demonstrating that it is not a delusion, a political statement, or a paraphilia. Note that social stigma has a role to play in the development of and adherence to both viewpoints. See the transfeminism article's section on GID for further discussion. Harry Benjamin wrote, "Summarizing my impression, I would like to repeat here what I said in my first lecture on the subject more than 10 years ago: Our genetic and endocrine equipment constitutes either an unresponsive, sterile, or a more or less responsive, that is to say, fertile soil on which the wrong conditioning and a psychic trauma can grow and develop into such a basic conflict that subsequently a deviation like transsexualism can result." Sex reassignment therapy Sex reassignment therapy (SRT) is an umbrella term for all medical treatments related to sex reassignment of both transgender and intersexual people. Though SRT is sometimes called "gender reassignment", those who use the word "sex" to describe an individual's biology and "gender" to describe their personal identity and social role consider this usage to be misleading. The process of changing from one gender presentation to another is often called transition. Individuals make different choices regarding sex reassignment therapy, which can include hormone replacement therapy (HRT) to modify secondary sex characteristics, sex reassignment surgery to alter primary sex characteristics, facial feminization surgery and permanent hair removal for trans women. Transsexual people who transition usually change their social gender roles, legal names and legal sex designation. To obtain sex reassignment therapy, transsexual people are generally required to undergo a psychological evaluation and receive a diagnosis of gender identity disorder in accordance with the Standards of Care (SOC) as published by the World Professional Association for Transgender Health. This assessment is usually accompanied by counseling on issues of adjustment to the desired gender role, effects and risks of medical treatments, and sometimes also by psychological therapy. The SOC are intended as guidelines, not inflexible rules, and are intended to ensure that clients are properly informed and in sound psychological health, and to discourage people from transitioning based on unrealistic expectations. Psychological techniques that attempt to alter gender identity to one considered appropriate for the person's assigned sex are typically ineffective. The widely recognized Standards of Care note that sometimes the only reasonable and effective course of treatment for transsexual people is to go through sex reassignment therapy. The need for treatment is emphasized by the high rate of mental health problems, including depression, anxiety, and various addictions, as well as a higher suicide rate among untreated transsexual people than in the general population. These problems may be alleviated by a change of gender role and/or physical characteristics. Many transgender and transsexual activists, and many caregivers, note that these problems are not usually related to the gender identity issues themselves, but the social and cultural responses to gender-variant individuals. Some transsexual people reject the counseling that is recommended by the Standards of Care because they don’t consider their gender identity to be a psychological problem. Brown and Rounsley noted that "[s]ome transsexual people acquiesce to legal and medical expectations in order to gain rights granted through the medical/psychological hierarchy." Legal needs such as a change of sex on legal documents, and medical needs, such as sex reassignment surgery, are usually difficult to obtain without a doctor and/or therapist's approval. Because of this, some transsexual people feel coerced into affirming outdated concepts of gender to overcome simple legal and medical hurdles (Brown 107). After an initial psychological evaluation, men and women may begin medical treatment starting with hormone replacement therapy or hormone blockers. People who change sex are usually required to live as members of their target sex for at least one year prior to genital surgery, gaining real-life experience sometimes called the "real-life test" (RLT). Transsexual individuals may undergo some, all, or none of the medical procedures available, depending on personal feelings, health, income, and other considerations. Some people posit that transsexualism is a physical condition, not a psychological issue, and assert that sex reassignment therapy should be given on request. (Brown 103) Regrets and detransitions People who undergo sex reassignment surgery can develop regret for the procedure later in life, largely due to lack of support from family or peers, with data from the 1990s suggesting a rate of 3.8%. A review of Medline literature suggests the total rate of patients expressing feelings of doubt or regret is estimated to be as high as 8%. In a 2001 study of 232 MTF patients who underwent GRS with Dr. Toby Meltzer, none of the patients reported complete regret and only 6% reported partial or occasional regrets. An issue reported by some is the inability to find sexual partners. Laws regarding changes to the legal status of transsexual people are different from country to country. Some jurisdictions allow an individual to change their name, and sometimes, their legal gender, to reflect their gender identity. Within the US, some states allow amendments or complete replacement of the original birth certificates. Some states seal earlier records against all but court orders in order to protect the transsexual's privacy. In many places, it is not possible to change birth records or other legal designations of sex, although changes are occurring. Estelle Asmodelle’s book documented her struggle to change the Australian birth certificate and passport laws, although there are other individuals who have been instrumental in changing laws and thus attaining more acceptance for transsexual people in general. Medical treatment for transsexual and transgender people is available in most Western countries. However, transsexual and transgender people challenge the "normative" gender roles of many cultures and often face considerable hatred and prejudice. The film Boys Don't Cry chronicles the case of Brandon Teena, a transsexual man who was raped and murdered after his status was discovered. The project Remembering Our Dead, founded by Gwendolyn Ann Smith, archives numerous cases of transsexual and transgender people being murdered. In the United States, November 20 has been set aside as the "Day of Remembrance" for all murdered transgender people. Some people who have switched their gender role enter into traditional social institutions such as marriage and parenting. They sometimes adopt or provide foster care for children, as complete sex reassignment therapy inevitably results in infertility.Some transsexual people have children from before transition. Some of these children continue living with their transitioning/transitioned parent, or retain close contact with them. Health-practitioner manuals, professional journalistic style guides, and LGBT advocacy groups advise the adoption by others of the name and pronouns identified by the person in question, including present references to the transgender or transsexual person's past. Family members and friends who may be confused about pronoun usage or the definitions of sex are commonly instructed in proper pronoun usage, either by the transsexual person or by professionals or other persons familiar with pronoun usage as it relates to transsexual people. Sometimes transsexual people have to correct their friends and family members many times before they begin to use the transsexual person's desired pronouns consistently. Deliberate mis-gendering is perceived[by whom?] to be a form of transphobia. Both "transsexualism" and "gender identity disorders not resulting from physical impairments" are specifically excluded from coverage under the Americans with Disabilities Act Section 12211. Gender dysphoria is not excluded. Transsexual people can have difficulty maintaining employment. Most find it necessary to remain employed during transition in order to cover the costs of living and transition. However, employment discrimination against trans people is rampant and many of them are fired when they come out or are involuntarily outed at work. Transsexual people must decide whether to transition on-the-job, or to find a new job when they make their social transition. Other stresses that transsexual people face in the workplace are being fearful of coworkers negatively responding to their transition, and losing job experience under a previous name—even deciding which rest room to use can prove challenging. Finding employment can be especially challenging for those in mid-transition. Laws regarding name and gender changes in many countries make it difficult for transsexual people to conceal their trans status from their employers. Because the Harry Benjamin Standards of Care require a one-year RLE[clarification needed] prior to SRS, some feel this creates a Catch 22 situation which makes it difficult for trans people to remain employed or obtain SRS. In many countries, laws provide protection from workplace discrimination based on gender identity or gender expression, including masculine women and feminine men. An increasing number of companies are including "gender identity and expression" in their non-discrimination policies. Often these laws and policies do not cover all situations and are not strictly enforced. California's anti-discrimination laws protect transsexual persons in the workplace and specifically prohibit employers from terminating or refusing to hire a person based on their transsexuality. The European Union provides employment protection as part of gender discrimination protections following the European Court of Justice decisions in P v S and Cornwall County Council. In the National Transgender Transgender Discrimination Survey, 44% of respondents reported not getting a job they applied for because of being transgender. 36% of trans women reported losing a job due to discrimination compared to 19% of trans men. 54% of trans women and 50% of trans men report having been harassed in the workplace. Transgender people who have been fired due to bias are more than 34 times likely than members of the general population to attempt suicide. Some transsexual men and women choose to live completely as members of their gender without being public about their past. This approach is sometimes called stealth. Some people feel that they have an obligation to be open about their past in order to further the cause of civil rights for LGBT people. There are examples of people having been denied medical treatment upon discovery of their trans status, whether it was revealed by the patient or inadvertently discovered by the doctors. For example, Leslie Feinberg was once turned away from a hospital emergency room where he had sought treatment for endocarditis.[unreliable source?] Feinberg was presenting as a man but had female genital anatomy. He nearly died after being denied treatment. Feinberg's case demonstrates one of the many dangers of having one's trans status discovered. Tyra Hunter died after being denied care by paramedics and emergency room physicians after she was injured in an automobile accident. In the media Transsexualism was discussed in the mass media as long ago as the 1930s. The American magazine Time in 1936 devoted an article to what it called "hermaphrodites", treating the subject with sensitivity and not sensationalism. It described the call by Avery Brundage, who led the American team to the 1936 Summer Olympics in Berlin, that a system be established to examine female athletes for "sex ambiguities"; two athletes changed sex after the Games. Before transsexual people were depicted in popular movies and television shows, Aleshia Brevard — an actual transsexual whose surgery took place in 1962 — was actively working as an actress and model in Hollywood and New York throughout the 1960s and '70s. Aleshia never portrayed a transsexual person, though she appeared in eight Hollywood produced films, on most of the popular variety shows of the day including The Dean Martin Show, and was a regular on The Red Skelton Show and One Life to Live before returning to University to teach Drama and Acting. Thomas Harris's Silence of the Lambs included a serial killer who considered himself a transsexual. After being turned down for sex reassignment surgery due to not meeting necessary psychological evaluations, he then harvested female bodies to make a feminine suit. In the novel, it is noted that the character is not actually a transsexual; this distinction is made only briefly in the film. Films depicting transgender issues include: Come Back to the Five and Dime, Jimmy Dean, Jimmy Dean, The World According to Garp, The Adventures of Priscilla, Queen of the Desert, All About My Mother and The Crying Game. The film Different for Girls is notable for its depiction of a transsexual woman who meets up with, and forms a romantic relationship with, her former best friend from her all-male boarding school. Ma Vie en Rose portrays a six-year-old child who is gender variant. The film Wild Zero features Kwancharu Shitichai, a transsexual Thai actor. When the main character is conflicted about falling in love with a "woman who is also a man", Guitar Wolf tells him "Love knows no race, nationality or gender!" Although Better Than Chocolate is primarily about the romance of two lesbians, a subplot in the 1999 Canadian film has Judy (Peter Outerbridge), a trans woman with a crush on Frances (Ann-Marie MacDonald), the owner of a lesbian bookstore. Within the film has a few scenes showing how Judy loses her parents who are unable to accept her, and buy her off with a bye forever present in purchasing a home for her. Southern Comfort is a 2001 documentary by filmmaker Katie Davis, which follows the final months of the life of Robert Eads, a female-to-male transsexual living in Georgia. Eads was diagnosed with ovarian cancer and rejected for treatment by over two dozen doctors due to his transsexuality. The documentary follows Eads and several of his closest friends, a support group of transsexual southerners known as "Southern Comfort". The documentary won several awards, including the Grand Jury Prize at the Sundance Film Festival, First Prize at the Seattle International Film Festival, and the Special Audience Award at the Berlin Film Festival. Two notable films depict transphobic violence based on true events: Soldier's Girl (about the relationship between Barry Winchell and Calpernia Addams, and Winchell's subsequent murder) and Boys Don't Cry (about Brandon Teena's murder). Calpernia Addams has appeared in numerous movies and television shows, including the 2005 movie Transamerica, in which Felicity Huffman portrays a transsexual woman. In fall 2005, the Sundance Channel aired a documentary series known as TransGeneration. This series focused on four transsexual college students, including two trans women and two trans men, in various stages of transition. In February 2006, Logo aired Beautiful Daughters, a documentary film about the first all-trans cast of The Vagina Monologues, which included Addams, Lynn Conway, Andrea James, and Leslie Townsend. Also in 2006, Lifetime aired a movie biography on the murder of "Eddie"/"Gwen" Araujo called A Girl Like Me: The Gwen Araujo Story. Transsexual people have also been depicted in popular television shows. In part of the first season of the 1970s t.v. comedy series, Soap, Billy Crystal plays Jodie Dallas, a gay man who is about to undergo a sex change in order to legally marry his male lover, who breaks off the relationship just before the surgery. In Just Shoot Me!, David Spade's character meets up with his childhood male friend, who has transitioned to living as a woman. After initially being frightened, he eventually forms sexual attraction to his friend, but is scorned, as he is 'not her type'. In an episode of Becker Dr. Becker gets an out-of-town visit from an old friend who turns out to have undergone SRS, it plays out very similar to the situations in Just Shoot Me!. In a 1980s episode of The Love Boat, McKenzie Phillips portrays a trans woman who is eventually accepted as a friend by her old high school classmate, series regular Fred Grandy. In the 1970s on The Jeffersons, George's Navy buddy Eddie shows up as Edie and is eventually accepted by George. Dramas including Law & Order and Nip/Tuck have had episodes featuring transsexual characters and actresses. While in Nip/Tuck the role was played by a non-transsexual woman, in Law & Order some were played by professional cross-dressers. Without a Trace and CSI: Crime Scene Investigation have had episodes dealing with violence against transsexual characters. Many transsexual actresses and extras appeared on the CSI episode, "Ch-Ch-Changes," including Marci Bowers and Calpernia Addams. The trans woman victim, Wendy, was played by Sarah Buxton, a cisgender woman. Candis Cayne, a transsexual actress, appeared in CSI: NY as a transsexual character. From 2007 to 2008, she also portrayed a transsexual character (this time recurring) in the ABC series Dirty Sexy Money. "Coronation Street" once had a transsexual woman named Hayley, who was Harold in her childhood. She died on 20 January 2014. Since 2004, with the goal of crowning the top transsexual of the world, a beauty pageant by the name of The World's Most Beautiful Transsexual Contest was held in Las Vegas, Nevada. The pageant accepted pre-operation and post-operation trans women, but required proof of their gender at birth. The winner of the 2004 pageant was a woman named Mimi Marks. Jenna Talackova, the 23-year-old woman who forced Donald Trump and his Miss Universe Canada pageant to end its ban on transgender contestants, competed in the pageant on May 19, 2012 in Toronto. On Saturday, January 12, 2013, Kylan Arianna Wenzel was the first transgender woman allowed to compete in a Miss Universe Organization pageant since Donald Trump changed the rules to allow women like Wenzel to enter officially. Miss Wenzel was the first transgender woman to compete in a Miss Universe Organization pageant since officials disqualified 23-year-old Miss Canada Jenna Talackova last year after learning she was transgender. Transgender researcher and activist Prempreeda Pramoj Na Ayutthaya claims that there is notable discrimination against transsexual people in relation to education and job opportunities in Thailand. An article in Bangkok Post in 2013, claims that there is societal discrimination against transsexuals in Thailand. An editorial in Bangkok Post in 2013, said "Yet it is also true that we don't find transgenders as high-ranking officials, doctors, lawyers, scientists, or teachers in state-run schools and colleges. Nor as executives in the corporate world. In short, the doors of government agencies and large corporations are still closed to transgender women. It is why they must be self-employed or work as freelancers." Thai law does not give "post-operation" male-to-female transexual people—who are government employees—the right to wear female uniforms at work. In 2013 Bangkok Post said that Jetsada "Note" Taesombat, coordinator of the Thai Transgender Alliance, "believes it is crucial for transgender men and women to be legally recognised as part of society. At present, they are legally identified as their sex at birth. 'Transgender men and women also want the civil partnership law to pass, since gender recognition is the most important issue. To legalise same-sex civil partnerships would mean that we, as people with sexual diversity, can finally be recognised legally. The most important thing for me and for everyone is to be accepted as part of society,' Note says." A 2014 Bangkok Post article said that a Mayathom 1 [grade school] textbook had been criticized for discrimination and lack of gender sensitivity, because the textbook denoted transgender people as gender confusion—khon long pate, and illustrations in the textbook "feature performances by transgender dancers". The word long "has negative connotations. Transgender or kham pate is more suitable". It was reported that Officials at the Education Ministry would look into the matter. A 2014 Bangkok Post article said that LGBT "still face discrimination affecting their social rights and job opportunities", according to a report by US Agency for International Development—and the United States Development Programme". Furthermore LGBT "still face difficulty gaining acceptance for non-traditional sexuality, even though the tourism authority has been promoting Thailand as a gay-friendly country". Furthermore "transgendered people cannot change identity papers, and male-to-female transgender people still have to perform military service". Furthermore "examples of inequality among LGBT" include the case of "a hospital which refused to allow a transexual to stay in a woman's ward, even though she had undergone sexual reassignment surgery". (The report was "prepared and researched over two years", and it "is part of the project, 'Being LGBT in Asia'—a project also held "in Cambodia, China, Indonesia, Mongolia, Nepal, the Philippines, Thailand and Vietnam".) History and other cultures Customs and traditions Transgender Day of Remembrance The Transgender Day of Remembrance is held every year on November 20. This event is held in honor of Rita Hester (killed Nov. 28, 1998), a victim of an anti-transgender hate crime. TDOR serves a number of purposes: - memorializes all of those who have been victims of hate crimes and prejudice - raises awareness about hate crimes towards the transgender community - honor the lost ones and their relatives by expressing respect for each other The Trans March is one of three protests held in San Francisco, California during "Pride Weekend" during the last weekend of June. Every year people from the transexual community gather in San Francisco, CA to protest social justice and equality for them. In addition, through the march they strive to inspire everyone from the transexual community to come out to an environment where power is shared and where one can feel safe and cared for. The event also hosts comedians, music, and dancing at the park. After parties are often followed after the event. All About Trans All About Trans is an organization in the UK whose goal is to influence and improve media professionals' understanding and portrayal of transsexual people. They do this by connecting media outlets with members of the transsexual community throughout the UK in order to foster a greater sensitivity toward this group of people. Paris Lees works as a facilitator with this organization and was recognized on The Independent on Sunday's Pink List in 2013 for being the most influential figure in the LGBT community in the UK. Transgender At Work Transgender At Work (TAW) is an organization with a focus on addressing issues in the workplace for transsexual individuals. Its goal is to allow transsexual employees to work productively without feeling as if they must hide an essential part of themselves. This includes addressing such issues as transsexual individuals being excluded from employer health care on the basis of their transsexuality. National Transgender Advocacy Coalition The National Transgender Advocacy Coalition is a lobbying organization in the United States dedicated to preserving the civil rights of transsexual individuals. It began in Virginia in 1999 and held its first lobbying event in 2001. It has no paid employees, but consists of a board of experienced lobbyists and activists. Renaissance Education Association The Renaissance Education Association is a non-profit organization founded in Pennsylvania that is dedicated to providing education and social support regarding transgender issues. This includes providing educational programs, support groups, and resources to community care providers. It also strives to provide personal and educational resources for individuals struggling with issues related to transsexuality and those close to them. Survivor Project is a non-profit organization founded in 1997 that is devoted to assisting intersex and transsexual survivors of domestic and sexual violence. This is done through caring action and education. The Project provides presentations, workshops, and consultation materials to many communities and universities across the United States. It also works to find information regarding the specific issues faced by intersex and transsexual individuals who are victimized. Empowering survivors and allowing them to participate in anti-violence activism is one major philosophy of the organization. Transgender Law and Policy Institute The Transgender Law and Policy Institute (TLPI) was founded by Paisley Currah, Associate Professor of Political Science at Brooklyn College. It is dedicated to engaging in effective advocacy for transgender people in our society. The TLPI brings experts together to work on law and policy initiatives designed to advance transgender equality. Their website provides information and resources on legislation, case law, employer and college policies and other resources.They also work with the Gay & Lesbian Advocates & Defenders (GLAD) in New England. Trans*topia is section of Youth Resource (a project of Advocates for Youth, a nonprofit organization located in Washington D.C.) designed for the needs of transsexual youth. Their website includes articles about being young and transgender, both personal accounts and scientific articles that are intended to help transgender youth become more informed and comfortable with their sexuality and gender. - List of transgender-related topics - List of transgender-rights organizations - List of LGBT-related organizations - List of transgender people - "ICD-10". Retrieved 2008-09-28. - American Psychiatric Association (2000). Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (Text Revision). American Psychiatric Publishing. ISBN 0-89042-025-4. - Two-Spirit People: Native American Gender Identity, Sexuality, and Spirituality 1997 U. Illinois, 1997, p. 100, Sabine Lang et al. - "Gender dysphoria". 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"Notes on Gender Role Transition - Crossdressing vs Transsexualism". Retrieved 2011-12-24. - Sullivan, L. (1990). Information for the female to male cross dresser and transsexual. (3 ed.). Seattle, WA: Ingersoll Gender Center. - Iyall Smith, K. E., & Leavy, P. (2008). Hybrid identities: theoretical and empirical examinations, Leiden, The Netherlands: IDC Publishers - "GLAAD Media Reference Guide". Retrieved 2013-12-27. - "Fenway Health Glossary of Gender and Transgender Terms". January 2010. Retrieved 2013-12-27. - Valentine, David. Imagining Transgender: An Ethnography of a Category, Duke University, 2007 - Stryker, Susan. Introduction. In Stryker and S. Whittle (Eds.), The Transgender Studies Reader, New York: Routledge, 2006. 1–17 - Denny, Dallas (2006). "Chapter 9: Transgender Communities of the United States in the Late Twentieth Century". In Currah, Paisley. Transgender Rights. - Journal of International Women's Studies: ...Treatment of UK Transsexed Individuals... - Laub, D. R.; N. Fisk (April 1974). "A rehabilitation program for gender dysphoria syndrome by surgical sex change". Plast Reconstr Surg. 53 (4): 388–403. doi:10.1097/00006534-197404000-00003. PMID 4592953. - Fisk, N. (1974). Laub, D.; Gandy P., ed. "Gender Dysphoria Syndrome". Proceedings of the Second Interdisciplinary Symposium on Gender Dysphoria Syndrome: 7–14. - Aggrawal, Anil (2008). Forensic and Medico-legal Aspects of Sexual Crimes and Unusual Sexual Practices. - Labonte, Richard and Lawrence Schimel, eds. (2009) Second Person Queer - Bornstein, Kate (2010). Gender Outlaws: The Next Generation. - van Kesteren, Paul J. M; Henk Asscheman, Jos A. J Megens, Louis J. G Gooren (1997). "Mortality and morbidity in transsexual subjects treated with cross-sex hormones". J. Clin. Endocrinol. (Blackwell, Oxford, UK) 47 (3): 337–343. doi:10.1046/j.1365-2265.1997.2601068.x. PMID 9373456. - Transgender Mental Health, "The Prevalence of Transgenderism" http://tgmentalhealth.com/2010/03/31/the-prevalence-of-transgenderism/ - Olyslager, Femke; Lynn Conway (2007). "On the Calculation of the Prevalence of Transsexualism" (PDF). - Veale, Jaimie F. (October 2008). "Prevalence of transsexualism among New Zealand passport holders". Australian and New Zealand Journal of Psychiatry 42 (10): 887–889. doi:10.1080/00048670802345490. PMID 18777233. - Reed, Bernard; Stephenne Rhodes (2008). "Presentation on prevalence of transsexual people in the UK". - Swaab, D.F. (2004). "Sexual differentiation of the human brain: relevance for gender identity, transsexualism and sexual orientation". Gynecological Endocrinology 19 (6): 301–312. doi:10.1080/09513590400018231. PMID 15724806. - Kruijver 2000 - Garcia-Falgueras A, Swaab DF (December 2008). "A sex difference in the hypothalamic uncinate nucleus: relationship to gender identity". Brain 131 (Pt 12): 3132–46. doi:10.1093/brain/awn276. PMID 18980961. - Psychology The Science Of Behaviour, pg 418, Pearson Education, Neil R.Carlson - Male transsexual gene link found BBC News 26 October 2008 (accessed 26 October 2008) - Hood E (2005). "Are EDCs blurring issues of gender?". Environmental Health Perspectives 113 (10): A670–7. doi:10.1289/ehp.113-a670. PMC 1281309. PMID 16203228. - Blackless M, Besser M, Carr S, Cohen-Kettenis PT, Connolly P, De Sutter P, Diamond M, Di Ceglie D, Higashi Y, Jones L, Kruijver FPM, Martin J, Playdon Z-J, Ralph D, Reed T, Reid R, Reiner WG, Swaab D, Terry T, Wilson P, Wylie K (2006). "Atypical Gender Development – A Review". International Journal of Transgenderism 9 (1): 29–44. doi:10.1300/J485v09n01_04. - Michel A, Mormont C, Legros JJ (2001). "A psycho-endocrinological overview of transsexualism". Eur. J. Endocrinol. 145 (4): 365–76. doi:10.1530/eje.0.1450365. PMID 11580991. - Selvaggi G, Ceulemans P, De Cuypere G, VanLanduyt K, Blondeel P, Hamdi M, Bowman C, Monstrey S (2005). "Gender identity disorder: general overview and surgical treatment for vaginoplasty in male-to-female transsexuals". Plast. Reconstr. Surg. 116 (6): 135e–145e. doi:10.1097/01.prs.0000185999.71439.06. PMID 16267416. - Benjamin, H. (1966). The transsexual phenomenon. New York: Julian Press. p. 85. ISBN 0-446-82426-7. - Endocrine Treatment of Transsexual People: A Review of Treatment Regimens, Outcomes, and Adverse Effects, Eva Moore, Amy Wisniewski and Adrian Dobs - Seattle and King County Health – Transgender Health[dead link] - The International Transsexual Sisterhood – Study On Transsexuality[dead link] - Brown, Mildred L.; Chloe Ann Rounsley (1996). True Selves: Understanding Transsexualism – For Families, Friends, Co-workers, and Helping Professionals. Jossey-Bass. ISBN 978-0-7879-6702-4. - Bunck, Mathijs C., Diamant, Michaela, Cornér, Anja, Eliasson, Bjorn, Malloy, Jaret L., Shaginian, Rimma M., Deng, Wei, Kendall, David M., Taskinen, Marja-Riitta, Smith, Ulf, Yki-Järvinen, Hannele, Heine, Robert J. (2008). "Long-term treatment of transsexuals with cross-sex hormones: extensive personal experience". J Clin Endocrinol Metab 32 (5): 19–25. doi:10.2337/dc08-1797. PMC 2671094. PMID 19196887. - Landén, M; Wålinder, J, Hambert, G, Lundström, B. (April 1998). "Factors predictive of regret in sex reassignment". Acta Psychiatr Scand. 97 (4): 284–9. doi:10.1111/j.1600-0447.1998.tb10001.x. PMID 9570489. - For examples see Jill Stark, 'I will never be able to have sex again. Ever', The Age, May 31, 2009. - Baranyi, A; Piber, D, Rothenhäusler, HB. (2009). "Male-to-female transsexualism. Sex reassignment surgery from a biopsychosocial perspective". Wien Med Wochenschr. 159 (21–22): 548–57. doi:10.1007/s10354-009-0693-5. PMID 19997841. - Lawrence MD, A. A. (Aug 2003). "Factors associated with satisfaction or regret following male-to-female sex reassignment surgery". Archives of Sexual Behavior 32 (4): 299–315. doi:10.1023/A:1024086814364. PMID 12856892. - "Anna Grodzka". Sejm Rzeczypospolitej Polskiej. Retrieved December 2, 2011. - Świerzowski, Bogusław. "Wybory 2011: Andrzej Duda (PIS) zdeklasował konkurentów w Krakowie". Info Kraków 24. October 10, 2011. - "The Transgender Law and Policy Institute: Home Page". Transgenderlaw.org. Retrieved 2011-07-06. - Remembering Our Dead – a memorial to transgender people who have been murdered - Don't Forget Transgender Day of Rememberance (sic) by Jamie Tyroler, January 18, 2008, Kansas City Camp - Glicksman, Eve (April 2013). "Transgender terminology: It's complicated". Vol 44, No. 4: American Psychological Association. p. 39. Retrieved 2013-09-17. "Use whatever name and gender pronoun the person prefers" - Sponsored by the American Medical Association and The Fenway Health with unrestricted support from Fenway Health and Pfizer. "Meeting the Health Care Needs of Lesbian, Gay, Bisexual, and Transgender (LGBT) People: The End to LGBT Invisibility" (PowerPoint Presentation). The Fenway Institute. p. 24. Retrieved 2013-09-17. "Use the pronoun that matches the person’s gender identity" - "Glossary of Gender and Transgender Terms" (PDF). Preface: Fenway Health. January 2010. p. 2. Retrieved 2013-09-17. "listen to your clients – what terms do they use to describe themselves" - "Therapists with Lesbian, Gay, Bisexual, and Transgender Clients" (Word Document). Association for Behavioral and Cognitive Therapies. 2010. p. 2. Retrieved 2013-09-17. "transsexuals prefer to be referred to using the pronoun of identified gender, regardless of their level of transition" - Paul M. Elizondo III, D.O.; Willy Wilkinson, M.P.H., & Christopher Daley, M.D. (6 September 2012). "Working With Transgender Persons". Phychiatric Times. Retrieved 2013-09-17. "If you are not sure which pronoun to use, you can ask the patient" - "Competencies for Counseling with Transgender Clients" (PDF). Association for Lesbian, Gay, Bisexual, and Transgender Issues in Counseling. 18 September 2009. p. 3. "honor the set of pronouns that clients select and use them throughout the counseling process" - "AP editors’ note on Manning". The AP Blog. Associated Press. 22 August 2013. Retrieved 2013-09-17. "Use the pronoun preferred by the individuals who have acquired the physical characteristics of the opposite sex or present themselves in a way that does not correspond with their sex at birth." - Division of Public Affairs (September 2011). "Style Guide". Vanderbilt University. p. 34. Retrieved 2013-09-17. "Use the pronoun preferred by the individuals who have acquired the physical characteristics of the opposite sex or present themselves in a way that does not correspond with their sex at birth." - "Frequently Asked Questions on Trans Identity". Common Ground – Trans Etiquette. University of Richmond. Retrieved 2013-09-17. "be considerate of one’s gender identity by using the pronouns of the respective gender pronouns, or gender-‐neutral pronouns, they use" - "Journalists: Commit to Fair and Accurate Coverage of Transgender People, including Pvt. Chelsea Manning". Transgender Law Center. 2013. Retrieved 2013-09-17. "Avoid pronoun confusion when examining the stories and backgrounds of transgender people prior to their transition." - "NAMES, PRONOUN USAGE & DESCRIPTIONS" (PDF). GLAAD Media Reference Guide. GLAAD. May 2010. p. 11. Retrieved 2013-09-17. "It is usually best to report on transgender people's stories from the present day instead of narrating them from some point or multiple points in the past, thus avoiding confusion and potentially disrespectful use of incorrect pronouns." - "Transgender FAQ". Resources. Human Rights Campaign. Retrieved 2013-09-17. "should be identified with their preferred pronoun" - "Americans with Disabilities Act of 1990 - ADA - 42 U.S. Code Chapter 126". find US law. Retrieved 2011-07-06. - "Americans with Disabilities Act of 1990 §512. DEFINITIONS.". United States Access Board, a Federal Agency. 2009-01-01. Retrieved 2013-06-05. - Work transition for transsexual women – TS Road Map - Making a successful transition at work – helpful guide by Jessica McKinnon and sample transition-related documents - Pepper 2008 - Weiss, Jillian Todd (2001). "The Gender Caste System: Identity, Privacy and Heteronormativity" (PDF). Tulane Law School. Retrieved 2007-02-25. - Workplace Discrimination: Gender Identity or Expression – Human Rights Campaign Foundation - Judgment of the Court of 30 April 1996. – P v S and Cornwall County Council. – Reference for a preliminary ruling: Industrial Tribunal, Truro – United Kingdom. – Equal treatment for men and women – Dismissal of a transsexual. – Case C-13/94 – European Court reports 1996 Page I-02143 - http://endtransdiscrimination.org/PDFs/NTDS_Report.pdf. Missing or empty - Stryker, Susan; Whittle, Stephen (2006). The Transgender Studies Reader. CRC Press. ISBN 9780415947091. Retrieved 2009-11-24. - Leslie Feinberg (1998). Trans Liberation: Beyond Pink Or Blue. Beacon Press. pp. 2–4. ISBN 0-8070-7951-0. - "Medicine: Change of Sex". Time. 24 Aug 1936. Retrieved 23 December 2010. - Aleshia Brevard at the Internet Movie Database - Aleshia Brevard (2001). The Woman I Was Not Born to Be: A Transsexual Journey. Philadelphia: Temple University Press. ISBN 1-56639-840-1. - Silence of the Lambs at the Internet Movie Database - Transamerica at the Internet Movie Database - TransGeneration at the Internet Movie Database - Beautiful Daughters – LOGO (TV channel) Documentary - Calpernia Addams at the Internet Movie Database - http://www.cnn.com/2012/05/19/showbiz/canada-miss-universe-transgender. Missing or empty - Bennettsmith, Meredith (2013-01-11). "Transgender Miss California Contestant Set To Make History". Huffington Post. - Katoey face closed doors - Chaiyot Yongcharoenchai (2013-09-08). "The two faces of Thai tolerance". Bangkok Post. - "Gender labels upset Gene". Bangkok Post. 2014-09-12. p. 12. - Chananthorn Kamjan (2014-09-17). "Gays still face a battle, report says". p. 4. - Roman Emperors – DIR Elagabalus Meckler, M. L. (August 26, 1997): - "About TDOR at Transgender Day of Remembrance". Transgenderdor.org. 1998-11-28. Retrieved 2011-07-06. - "San Francisco Trans March \| SF's premiere transgender Pride event, Friday, June 22, 2012". Transmarch.org. Retrieved 2011-07-06. - "Paris Lees: From prison to transgender role model". BBC News. 2013-10-27. - Burns, Christine: Why Phrasebook Diversity is not Enough (Press for Change) - Brown, Mildred L.; Chloe Ann Rounsley (1996). True Selves: Understanding Transsexualism – For Families, Friends, Coworkers, and Helping Professionals. Jossey-Bass. ISBN 978-0-7879-6702-4. - Feinberg, Leslie (1999). Trans Liberation : Beyond Pink or Blue. Beacon Press. ISBN 978-0-8070-7951-5. - Standards of Care for Gender Identity Disorders, Sixth Version. – World Professional Association for Transgender Health (2001) - Kruijver, Frank P. M.; Jiang-Ning Zhou, Chris W. Pool, Michel A. Hofman, Louis J. G. Gooren and Dick F. Swaab (2000). "Male-to-Female Transsexuals Have Female Neuron Numbers in a Limbic Nucleus". Journal of Clinical Endocrinology & Metabolism 85 (5): 2034–41. doi:10.1210/jc.85.5.2034. PMID 10843193. - Schneider, Harald J.; Johanna Pickel, Günter K. Stalla (2006). "Typical female 2nd–4th finger length (2D:4D) ratios in male-to-female transsexuals-possible implications for prenatal androgen exposure". International Society of Psychoneuroendocrinology 31 (2): 265–9. doi:10.1016/j.psyneuen.2005.07.005. PMID 16140461. - Xavier, J., Simmons, R. (2000) – The Washington transgender needs assessment survey, Washington, DC: The Administration for HIV and AIDS of the District of Columbia Government - Rathus, Spencer A.; Jeffery S. Nevid, Lois Fichner-Rathus (2002). Human Sexuality in a World of Diversity. Allyn & Bacon. ISBN 978-0-205-40615-9. - Pepper, Shanti M.; Peggy Lorah (2008). "Career Issues and Workplace Considerations for the Transsexual Community: Bridging a Gap of Knowledge for Career Counselors and Mental Health Care Providers". The Career Development Quarterly 56 (4): 330–343. doi:10.1002/j.2161-0045.2008.tb00098.x. \|Look up transsexualism in Wiktionary, the free dictionary.\| - Full service information and interactive dialogue for transsexual people - Asociación Española de Transexuales. AET Transexualia - Basic TG/TS/IS Information – including Successful Transwomen and Successful Transmen - FTM International – Female To Male International: practical and medical information - FTM Australia – Comprehensive information for all men identified female at birth in Australia. - Hudson's FTM Resource Guide – Comprehensive information for female to male trans men and allies – US-based - Gender.org – The home of Gender Education & Advocacy, a nonprofit corporation using the web to provide education and advocacy for transsexual and transgender issues. - Definition and Synopsis of the Etiology of Adult Gender Identity Disorder and Transsexualism – prepared by 24 internationally recognized experts, published by the Gender Identity Research and Education Society (GIRES)(See: http://www.gires.org.uk) - The International Journal of Transgenderism – The Official Journal of the World Professional Association for Transgender Health (formerly HBIGDA). An archive of IJT Volumes I through V is available, as are several books on transsexualism, including Harry Benjamin's "The Transsexual Phenomenon" - TransParentcy – supports transgender parents and their advocates (lawyers, mental health professionals, friends, family) by providing information and resources. - Transsexual Road Map – consumer information and advice for transsexual women. - Lynn Conway's transsexual resources pages - "Dr. Anne Lawrence On Transsexualism and Sexuality" - detailed information on medical aspects of transition - The Gender Identity Research and Education Society	2	51	8	0	0	0	0.717785	8	14,388
Coding is an unavoidable component of patient encounters. Use of the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM)1 became a statutory requirement for Medicare reimbursement in 1989. The International Classification of Diseases, Tenth Revision (ICD-10)2 was published in the early 1990s by the World Health Organization (WHO), and ICD-10 coded data have been adopted by over 100 countries in some form. While ICD-10 is currently used for mortality reporting in the United States, by early 2016 it will also incorporate morbidity reporting. The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM)3 was expected to have been adopted by October 1, 2014, but the Protecting Access to Medicare Act of 2014 prohibited adoption of this US-specific version before October 1, 2015.4 This new classification scheme is beneficial in several ways. ICD-10-CM allows for more succinct coding of disease severity (eg, the ability to code for “medically intractable,” or “… with status epilepticus”), providing the type of severity adjustment data needed to justify more appropriate reimbursements for the work that neurologists perform. Modern advanced health care reimbursement systems use these diagnoses to set relative acuity factors, which are one basis for payments to hospitals and physicians. ICD-10-CM also provides benefits at the public health level: greater specificity in diagnosis, etiology, and severity of disease and disease outcome; comparison of US morbidity to international reports; and identification of fraud and abuse. With more precise epidemiologic data, policy makers will be better informed to allocate health care resources more effectively. On a more practical level, ICD-10-CM greatly expands the capacity of disease description with a fivefold increase in the number of diagnosis codes as the available codes in the International Classification of Diseases, Ninth Revision (ICD-9) were full and new disorders have been identified since ICD-9 was published in 1977 (eg, Dravet syndrome).5 Medical knowledge continues to evolve and expand at a rapid pace. ICD-10 is designed to be a robust framework to encompass the current complexity and yet still be able to incorporate currently unknown disorders that may be identified in the future. This article guides readers through the coding for epilepsy using ICD-10. The good news is that the taxonomy is not much different between the old and new systems.6 THE INTERNATIONAL CLASSIFICATION OF DISEASES, TENTH REVISION, CLINICAL MODIFICATION While ICD-9-CM codes consist of five placeholders (the first is alphanumeric and the rest are all numerals), ICD-10-CM codes have three to seven characters (the first is a letter, the second and third are numeric, and the rest are alphanumeric). The first three characters specify the category; the next three specify etiology, severity, laterality, or other clinical detail. For neurologists, the last character is likely to be used only in the context of injuries, poisonings, and other conditions with external causes and not epilepsy; therefore, it will not be discussed further in this article.7 With ICD-10-CM, the epilepsy code is based on etiology, pathophysiology, determination of intractability or not, and whether the patient is in status epilepticus or not. However, one of the challenges with the transition to ICD-10-CM is that the most recent definitions and classifications promulgated by the International League Against Epilepsy (ILAE) do not always easily harmonize with ICD-10-CM.8,9 Under ICD-9, a first-time unprovoked seizure would be classified as 780.39, Other convulsions. However, this same code could be used for seizures associated with an external cause (eg, hypocalcemia, posttraumatic brain injury) or even nonepileptic events. Under ICD-10-CM, a first-time unprovoked seizure is coded as R56.9, Unspecified convulsions (CodingTable 1). However, seizures due to external causes are coded as G40.5, Epileptic seizures related to external causes. A conversion disorder with seizurelike symptoms is now coded as F44.5, Conversion disorder with seizures or convulsions, separate from the conversion disorder code for a symptom such as astasia-abasia. These are good examples of the increased granularity of ICD-10-CM compared to ICD-9-CM. ICD-10-CM no longer combines etiologically different seizures, yet the underlying conceptualization has not changed. Epilepsy, defined by the ILAE as recurrent unprovoked seizures more than 24 hours apart or a first-time unprovoked seizure with a high likelihood of recurrence, is subdivided into several categories in ICD-10-CM (CodingTable 1). A tabular list of the codes can also be found at the Centers for Disease Control and Prevention website: www.cdc.gov/nchs/data/icd/icd10cm/2016/ICD10CM_FY2016_Full_PDF.ZIP. Again, the conceptualization remains essentially the same between ICD-9-CM and ICD-10-CM: partial onset versus generalized onset, simple versus complex, symptomatic versus idiopathic, intractable versus well controlled. ICD-10-CM now specifically incorporates whether the patient is in status epilepticus. With this information in hand, coding is straightforward. To establish a partial-onset epilepsy, focal onset of the seizure, lateralizing clinical features (eg, unilateral blinking) or focal EEG or neuroradiographic findings should be documented. Absent a history of focality and with the presence of a history consistent with generalized seizures (eg, absence, myoclonic, atonic, tonic, clonic, or tonic-clonic), the documentation would be consistent with a generalized epilepsy. To establish a complex versus simple partial classification, documentation of an alteration in awareness during the seizure should be explicit. If awareness is preserved during the seizure, then the epilepsy would be classified as a simple partial seizure. If an etiologic basis exists for the epilepsy (eg, stroke, dementia, chromosomal anomaly, central nervous system lymphoma, mesial temporal sclerosis) or the neurologic examination demonstrates focal findings, then the epilepsy would be described as “symptomatic.” Otherwise, under the ICD-10-CM framework it would be coded as “idiopathic.” Under certain classification schemes, idiopathic implies an underlying genetic epilepsy, but in the context of ICD-10-CM, “idiopathic” can be thought of as “not symptomatic.” One caveat to keep in mind is that with better understanding of the genetic basis of the epilepsy syndromes (eg, juvenile myoclonic epilepsy), gene mutations may be identified for these syndromes. These would not be classified under symptomatic epilepsies, but rather continue to be classified under the more appropriate epilepsy syndromes (eg, G40.B, Juvenile myoclonic epilepsy [impulsive petit mal]). (Bear in mind that ICD-10 was released in the 1990s and is not consistent with the most recent classification of epilepsy by the ILAE and that archaic terms are included in ICD-10.)3,5 For the age-dependent genetic epilepsies, such as benign neonatal convulsions, myoclonic epilepsy in infancy, myoclonic astatic epilepsy, and grand mal seizures upon awakening, code families G40.0-, Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset, or G40.4-, Other generalized epilepsy and epileptic syndromes, would be used, depending on whether they are focal or generalized in onset, respectively. It is important to recall that absence epilepsy and juvenile myoclonic epilepsy have their own unique codes. The code G40.8, Other epilepsy and recurrent seizures, includes “epilepsy and epileptic syndromes undetermined as to whether they are focal or generalized” in onset. Landau-Kleffner syndrome is specifically mentioned under this heading. Lennox-Gastaut syndrome and epileptic (infantile) spasms are also given specific subheadings in this category (G40.81- and G40.82-, respectively). The codes under G40.9, Epilepsy, unspecified, should only be used infrequently when sufficient information is lacking for more specific coding. The guiding principle in coding is that each encounter should be coded to the highest level of certainty. Other seizures that do not fulfill a diagnosis of epilepsy are commonly seen in general practice but retain the same classification, only the codes used are different. For example, in pediatrics, the commonly encountered febrile seizures retain the same classifications: R56.00, Simple febrile convulsions, and R56.01, Complex febrile convulsions. Neonatal convulsions maintain their unique code as well: P90, Convulsions of newborn. With the information gathered in the epilepsy encounter, coding is straightforward. The first four characters are the fundamental descriptors for epilepsy in ICD-10-CM. The fifth placeholder usually signifies intractability (ie, failure of two appropriate anticonvulsants at high doses) with either “0” (not intractable) or “1” (intractable). The sixth placeholder usually signifies either active status epilepticus with “9” or not being in status epilepticus with “1.” Consider stepwise coding for an epilepsy encounter for a patient with an epilepsy characterized by complex partial seizures and an MRI demonstrating mesial temporal sclerosis (G40.2-), who continues to seize despite adequate trials with oxcarbazepine and lamotrigine (“intractable”, G40.21-), and who presents in status epilepticus (G40.211). Similarly, a 16-year-old patient with recent onset of generalized tonic-clonic seizures provoked by flashing lights could fall under several headings (G40.3-, Generalized idiopathic epilepsy and epileptic syndromes; G40.A-, Absence epileptic syndrome; G40.B-, Juvenile myoclonic epilepsy [impulsive petit mal]; G40.4-, Other generalized epilepsy and epileptic syndromes; G40.9-, Epilepsy, unspecified); however, if further history elicits a history of myoclonic seizures but no absence seizures, then the appropriate diagnosis would be G40.B, Juvenile myoclonic epilepsy [impulsive petit mal]. If the seizures are well controlled with medications and the patient is not in status epilepticus, the final code would be G40.B09, Juvenile myoclonic epilepsy, not intractable, without status epilepticus. The world has moved on from ICD-9, and the United States is moving to catch up. The transition to ICD-10-CM is not without its challenges, but tools exist to assist with the transition. In the long term, ICD-10-CM will be of benefit from a public health perspective, but it will also allow for more accurate coding of disease and disease severity, which will demonstrate the type of effort put forth by neurologists. This type of information can be used to inform policy makers’ decision making regarding reimbursements for the work performed by neurologists. 1. Centers for Disease Control and Prevention. International classification of diseases, ninth revision, clinical modification (ICD-9-CM). http://www.cdc.gov/nchs/icd/icd9cm.htm . Updated June 18, 2013. Accessed December 22, 2015. 2. The ICD-10 classification of mental and behavioural disorders: clinical descriptions and diagnostic guidelines. Geneva, Switzerland: World Health Organization, 1992. 3. Centers for Disease Control and Prevention. International classification of diseases, tenth revision, clinical modification (ICD-10-CM). www.cdc.gov/nchs/icd/icd10cm.htm . Updated October 29, 2015. Accessed December 22, 2015. 4. Bergen DC, Beghi E, Medina MT. Revising the ICD-10 codes for epilepsy and seizures. Epilepsia 2012; 53(suppl 2): 3–5. doi:10.1111/j.1528-1167.2012.03550.x. 8. Fisher RS, Acevedo C, Arzimanoglou A, et al. ILAE official report: a practical clinical definition of epilepsy. Epilepsia 2014; 55(4): 475–482. doi:10.1111/epi.12550. 9. Jette N, Beghi E, Hesdorffer D, et al. ICD coding for epilepsy: past, present, and future–a report by the International League Against Epilepsy Task Force on ICD codes in epilepsy. Epilepsia 2015; 6(3): 348–355. doi:10.1111/epi.12895.	4	5	3	0	0	19	0.60627	22	2,822
- Classification of mental disorders History · Subfields Basic science Applied science Lists Portal The classification of mental disorders, also known as psychiatric nosology or taxonomy, is a key aspect of psychiatry and other mental health professions and an important issue for consumers and providers of mental health services. There are currently two widely established systems for classifying mental disorders—Chapter V of the International Classification of Diseases (ICD-10) produced by the World Health Organization (WHO) and the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) produced by the American Psychiatric Association (APA). Both list categories of disorders thought to be distinct types, and have deliberately converged their codes in recent revisions so that the manuals are often broadly comparable, although significant differences remain. Other classification schemes may be in use more locally, for example the Chinese Classification of Mental Disorders. Other manuals have some limited use by those of alternative theoretical persuasions, such as the Psychodynamic Diagnostic Manual. The widely used DSM and ICD classifications employ operational definitions. There is a significant scientific debate about the relative validity of a "categorical" versus a "dimensional" system of classification, as well as significant controversy about the role of science and values in classification schemes and the professional, legal and social uses to which they are put. - 1 Definitions - 2 ICD-10 - 3 DSM-IV - 4 Other schemes - 5 Childhood diagnosis - 6 Usage - 7 Types of classification schemes - 8 Cultural differences - 9 Historical development - 10 Criticism - 11 See also - 12 External links - 13 References In the scientific and academic literature on the definition or categorization of mental disorders, one extreme argues that it is entirely a matter of value judgements (including of what is normal) while another proposes that it is or could be entirely objective and scientific (including by reference to statistical norms); other views argue that the concept refers to a "fuzzy prototype" that can never be precisely defined, or that the definition will always involve a mixture of scientific facts (e.g. that a natural or evolved function isn't working properly) and value judgements (e.g. that it is harmful or undesired). Lay concepts of mental disorder vary considerably across different cultures and countries, and may refer to different sorts of individual and social problems. The WHO and national surveys report that there is no single consensus on the definition of mental disorder/illness, and that the phrasing used depends on the social, cultural, economic and legal context in different contexts and in different societies. The WHO reports that there is intense debate about which conditions should be included under the concept of mental disorder; a broad definition can cover mental illness, mental retardation, personality disorder and substance dependence, but inclusion varies by country and is reported to be a complex and debated issue. There may be a criterion that a condition should not be expected to occur as part of a person's usual culture or religion. However, despite the term "mental", there is not necessarily a clear distinction drawn between mental (dys)functioning and brain (dys)functioning, or indeed between the brain and the rest of the body. Most international clinical documents avoid the term "mental illness", preferring the term "mental disorder". However, some use "mental illness" as the main over-arching term to encompass mental disorders. Some consumer/survivor movement organizations oppose use of the term "mental illness" on the grounds that it supports the dominance of a medical model. The term "serious mental illness" (SMI) is sometimes used to refer to more severe and long-lasting disorders while "mental health problems" may be used as a broader term, or to refer only to milder or more transient issues. Confusion often surrounds the ways and contexts in which these terms are used. Mental disorders are generally classified separately to neurological disorders, learning disabilities or mental retardation. The International Classification of Diseases (ICD) is an international standard diagnostic classification for a wide variety of health conditions. Chapter V focuses on "mental and behavioural disorders" and consists of 10 main groups: - F0: Organic, including symptomatic, mental disorders - F1: Mental and behavioural disorders due to use of psychoactive substances - F2: Schizophrenia, schizotypal and delusional disorders - F3: Mood [affective] disorders - F4: Neurotic, stress-related and somatoform disorders - F5: Behavioural syndromes associated with physiological disturbances and physical factors - F6: Disorders of personality and behaviour in adult persons - F7: Mental retardation - F8: Disorders of psychological development - F9: Behavioural and emotional disorders with onset usually occurring in childhood and adolescence - In addition, a group of "unspecified mental disorders". Within each group there are more specific subcategories. The ICD includes personality disorders on the same domain as other mental disorders, unlike the DSM. The ICD-10 states that mental disorder is "not an exact term", although is generally used "...to imply the existence of a clinically recognisable set of symptoms or behaviours associated in most cases with distress and with interference with personal functions." (WHO, 1992). The WHO is revising their classifications in this section as part of the development of the ICD-11 (scheduled for 2014) and an "International Advisory Group" has been established to guide this. The DSM-IV, produced by the American Psychiatric Association, characterizes mental disorder as "a clinically significant behavioral or psychological syndrome or pattern that occurs in an individual,...is associated with present distress...or disability...or with a significant increased risk of suffering" but that "...no definition adequately specifies precise boundaries for the concept of 'mental disorder'...different situations call for different definitions" (APA, 1994 and 2000). The DSM also states that "there is no assumption that each category of mental disorder is a completely discrete entity with absolute boundaries dividing it from other mental disorders or from no mental disorder." The DSM-IV-TR (Text Revision, 2000) consists of five axes (domains) on which disorder can be assessed. The five axes are: - Axis I: Clinical Disorders (all mental disorders except Personality Disorders and Mental Retardation) - Axis II: Personality Disorders and Mental Retardation - Axis III: General Medical Conditions (must be connected to a Mental Disorder) - Axis IV: Psychosocial and Environmental Problems (for example limited social support network) - Axis V: Global Assessment of Functioning (Psychological, social and job-related functions are evaluated on a continuum between mental health and extreme mental disorder) The main categories of disorder in the DSM are: - The Chinese Society of Psychiatry's Chinese Classification of Mental Disorders (currently CCMD-3) - The Latin American Guide for Psychiatric Diagnosis (GLDP). Child and adolescent psychiatry sometimes uses specific manuals in addition to the DSM and ICD. The Diagnostic Classification of Mental Health and Developmental Disorders of Infancy and Early Childhood (DC:0-3) was first published in 1994 by Zero to Three to classify mental health and developmental disorders in the first four years of life. It has been published in 9 languages. The Research Diagnostic criteria-Preschool Age (RDC-PA) was developed between 2000 and 2002 by a task force of independent investigators with the goal of developing clearly specified diagnostic criteria to facilitate research on psychopathology in this age group. The French Classification of Child and Adolescent Mental Disorders (CFTMEA), operational since 1983, is the classification of reference for French child psychiatrists. The ICD and DSM classification schemes have achieved widespread acceptance in psychiatry. A survey of 205 psychiatrists, from 66 different countries across all continents, found that ICD-10 was more frequently used and more valued in clinical practice and training, while the DSM-IV was more valued for research, with accessibility to either being limited, and usage by other mental health professionals, policy makers, patients and families less clear. A primary care (e.g. general or family physician) version of the mental disorder section of ICD-10 has been developed (ICD-10-PHC) which has also been used quite extensively internationally. A survey of journal articles indexed in various biomedical databases between 1980 and 2005 indicated that 15,743 referred to the DSM and 3,106 to the ICD. In Japan, most university hospitals use either the ICD or DSM. ICD appears to be the somewhat more used for research or academic purposes, while both were used equally for clinical purposes. Other traditional psychiatric schemes may also be used. Types of classification schemes The classification schemes in common usage are based on separate (but may be overlapping) categories of disorder schemes sometimes termed "neo-Kraepelinian" (after the psychiatrist Kraepelin) which is intended to be atheoretical with regard to etiology (causation). These classification schemes have achieved some widespread acceptance in psychiatry and other fields, and have generally been found to have improved inter-rater reliability, although routine clinical usage is less clear. Questions of validity and utility have been raised, both scientifically and in terms of social, economic and political factors—notably over the inclusion of certain controversial categories, the influence of the pharmaceutical industry, or the stigmatizing effect of being categorized or labelled. Some approaches to classification do not use categories with single cut-offs separating the ill from the healthy or the abnormal from the normal (a practice sometimes termed "threshold psychiatry" or "dichotomous classification"). Some approaches go further and propose continuously-varying dimensions that are not grouped into spectra or categories; each individual simply has a profile of scores across different dimensions. DSM-5 planning committees are currently seeking to establish a research basis for a hybrid dimensional classification of personality disorders. However, the problem with entirely dimensional classifications is they are said to be of limited practical value in clinical practice where yes/no decisions often need to be made, for example whether a person requires treatment, and moreover the rest of medicine is firmly committed to categories, which are assumed to reflect discrete disease entities. While the Psychodynamic Diagnostic Manual has an emphasis on dimensionality and the context of mental problems, it has been structured largely as an adjunct to the categories of the DSM. Nevertheless, non-categorical clinical formulation approaches are commonly employed in clinical psychology and some areas of psychiatry, where there may be limited or no reference to diagnostic categories. One such approach advocates taking each specific complaint reported by an individual on its own merits, treated as a phenomenon with its own causes. Descriptive vs Somatic Descriptive classifications are based almost exclusively on either descriptions of behavior as reported by various observers, such as parents, teachers, and medical personnel; or symptoms as reported by individuals themselves. As such, they are quite subjective, not amenable to verification by third parties, and not readily transferable across chronologic and/or cultural barriers. Somatic nosology, on the other hand, is based almost exclusively on the objective histologic and chemical abnormalities which are characteristic of various diseases and can be identified by appropriately trained pathologists. While not all pathologists will agree in all cases, the degree of uniformity allowed is orders of magnitude greater than that enabled by the constantly changing classification embraced by the DSM system. Classification schemes may not apply to all cultures. The DSM is based on predominantly American research studies and has been said to have a decidedly American outlook, meaning that differing disorders or concepts of illness from other cultures (including personalistic rather than naturalistic explanations) may be neglected or misrepresented, while Western cultural phenomena may be taken as universal. Culture-bound syndromes are those hypothesized to be specific to certain cultures (typically taken to mean non-Western or non-mainstream cultures); while some are listed in an appendix of the DSM-IV they are not detailed and there remain open questions about the relationship between Western and non-Western diagnostic categories and sociocultural factors, which are addressed from different directions by, for example, cross-cultural psychiatry or anthropology. In Ancient Greece, Hippocrates and his followers are generally credited with the first classification system for mental illnesses, including mania, melancholia, paranoia, phobias and Scythian disease (transvestism). They held that they were due to different kinds of imbalance in four humors. Middle ages to Renaissance An elaborate classification of mental disorders was developed in the 10th century by Arabian psychologist Najab ud-din Unhammad. His nosology included nine major categories of mental disorders, with 30 different mental illnesses in total. Some of the categories he described resembled obsessive-compulsive disorders, delusional disorders, degenerative diseases, involutional melancholia, and states of abnormal excitement. Avicenna (980−1037 CE) in the Canon of Medicine listed a number of mental disorders, including "passive male homosexuality". Laws generally distinguished between "idiots" and "lunatics". Thomas Sydenham (1624–1689), the "English Hippocrates", emphasized careful clinical observation and diagnosis and developed the concept of a syndrome, a group of associated symptoms having a common course, which would later influence psychiatric classification. Evolution in the scientific concepts of psychopathology (literally referring to diseases of the mind) took hold in the late 18th and 19th centuries following the Renaissance and Enlightenment. Individual behaviors that had long been recognized came to be grouped into syndromes. Boissier de Sauvages developed an extremely extensive psychiatric classification in the mid-18th century, influenced by the medical nosology of Thomas Sydenham and the biological taxonomy of Carl Linnaeus. It was only part of his classification of 2400 medical diseases. These were divided in to 10 "classes", one of which comprised the bulk of the mental diseases, divided into four "orders" and 23 "genera". One genus, melancholia, was subdivided into 14 "species". William Cullen advanced an influential medical nosology which included four classes of neuroses: coma, adynamias, spasms, and vesanias. The vesanias included amentia, melancholia, mania, and oneirodynia. Towards the end of the 18th century Pinel, influenced by Cullen's scheme, developed his own, again employing the terminology of genera and species. His simplified revision of this reduced all mental illnesses to four basic types. He argued that mental disorders are not separate entities but stem from a single disease that he called "mental alienation". Attempts were made to merge the ancient concept of delirium with that of insanity, the latter sometimes described as delirium without fever. The concept of partial insanity developed, and attempts were made to distinguish it from total insanity by criteria such as intensity, content or generalization of delusions. His successor, Esquirol, extended Pinel's categories to five. Both made a clear distinction between insanity (including mania and dementia) as opposed to mental retardation (including idiocy and imbecility). Esquirol developed a concept of monomania—a periodic delusional fixation or undesirable disposition on one theme—that became a broad and common diagnosis and a part of popular culture for much of the 19th century. The botanical taxonomic approach was abandoned in the 19th century, in favor of an anatomical-clinical approach that became increasingly descriptive. There was a focus on identifying the particular psychological faculty involved in particular forms of insanity, although some argued for a more central "unitary" cause. French and German psychiatric nosology was in the ascendency. The term "psychiatry" ("Psychiatrie") was coined by German physician Johann Christian Reil in 1808, from the Greek "ψυχή" (psychē: "soul or mind") and "ιατρός" (iatros: "healer or doctor"). The term "alienation" took on a psychiatric meaning in France, later adopted in to medical English. The terms psychosis and neurosis came in to use, the former viewed psychologically and the latter neurologically. In the second half of the century, Karl Kahlbaum and Ewald Hecker developed a descriptive categorizion of syndromes, employing terms such as dysthymia, cyclothymia, catatonia, paranoia and hebephrenia. Wilhelm Griesinger (1817–1869) advanced a unitary scheme based on a concept of brain pathology. French psychiatrists Jules Baillarger described "folie à double forme" and Jean-Pierre Falret described "la folie circulaire"—alternating mania and depression. The concept of adolescent insanity or developmental insanity was advanced by Scottish psychiatrist Thomas Coulston in 1873, describing a psychotic condition which generally afflicted those aged 18–24 years, particularly males, and in 30% of cases proceeded to "a secondary dementia". The concept of hysteria (wandering womb) had long been used, perhaps since ancient Egyptian times, and was later adopted by Freud. Descriptions of a specific syndrome now known as somatization disorder were first developed by the French physician, Briquet in 1859. Early 19th century psychiatrists also began to categorize personality disorders. The diagnosis of "moral insanity" became popular; those with the condition did not seem psychotic but seemed to have no ability to comprehend moral principles. In the late 19th century, Koch referred to "psychopathic inferiority", and in the 20th century the disorder became known as "psychopathy" or "sociopathy". Related studies led to the DSM-III category of antisocial personality disorder. An American physician, Beard, described "neurasthenia" in 1869. German neurologist Westphal, coined the term "obsessional neurosis" now termed obsessive-compulsive disorder, and agoraphobia. Alienists created a whole new series of diagnoses that highlighted single, impulsive behavior, such as kleptomania, dipsomania, pyromania, and nymphomania. The diagnosis of drapetomania was also developed in the Southern United States to explain the perceived irrationality of black slaves trying to escape what was thought to be a suitable role. The scientific study of homosexuality began in the 19th century, informally viewed either as natural or as a disorder. Kraepelin included it as a disorder in his Compendium der Psychiatrie that he published in successive editions from 1883. Influenced by the approach of Kahlbaum and others, and developing his concepts in publications spanning the turn of the century, German psychiatrist Emil Kraepelin advanced a new system. He grouped together a number of existing diagnoses that appeared to all have a deteriorating course over time—such as catatonia, hebephrenia and dementia paranoides—under another existing term "dementia praecox" (meaning "early senility", later renamed schizophrenia). Another set of diagnoses that appeared to have a periodic course and better outcome were grouped together under the category of manic-depressive insanity (mood disorder). He also proposed a third category of psychosis, called paranoia, involving delusions but not the more general deficits and poor course attributed to dementia praecox. In all he proposed 15 categories, also including psychogenic neurosis, psychopathic personality, and syndromes of defective mental development (mental retardation). He eventually included homosexuality in the category of "mental conditions of constitutional origin". The neuroses were later split into anxiety disorders and other disorders. Freud wrote extensively on hysteria and also coined the term, "anxiety neurosis", which appeared in DSM-I and DSM-II. Checklist criteria for this led to studies that were to define panic disorder for DSM-III. Early 20th century schemes in Europe and the US reflected a brain disease model that had emerged during the 19th century, as well as some ideas from Darwin's theory of evolution and/or Freud's psychoanalytic theories. Psychoanalytic theory did not rest on classification of distinct disorders, but pursued analyses of unconscious conflicts and their manifestations within an individual's life. The concept of borderline personality disorder developed from psychoanalytic theories. The philosopher and psychiatrist Karl Jaspers made influential use of a "biographical method" and suggested ways to diagnose based on the form rather than content of beliefs or perceptions. In regard to classification in general he prophetically remarked that: "When we design a diagnostic schema, we can only do so if we forego something at the outset ... and in the face of facts we have to draw the line where none exists... A classification therefore has only provisional value. It is a fiction which will discharge its function if it proves to be the most apt for the time". Adolph Meyer advanced a mixed biosocial scheme that emphasized the reactions and adaptations of the whole organism to life experiences. In 1945, William C. Menninger advanced a classification scheme for the US army, called Medical 203, synthesizing ideas of the time into five major groups. This system was adopted by the Veterans Administration in the US and strongly influenced the DSM. The term stress, having emerged out of endocrinology work in the 1930s, was popularized with an increasingly broad biopsychosocial meaning, and was increasingly linked to mental disorders. The diagnosis of post-traumatic stress disorder was later created. The Feighner Criteria group described fourteen major psychiatric disorders for which careful research studies were available, including homosexuality. These developed as the Research Diagnostic Criteria, adopted and further developed by the DSM-III. The DSM and ICD developed, partly in sync, in the context of mainstream psychiatric research and theory. Debates continued and developed about the definition of mental illness, the medical model, categorical vs dimensional approaches, and whether and how to include suffering and impairment criteria. There is some attempt to construct novel schemes, for example from an attachment perspective where patterns of symptoms are construed as evidence of specific patterns of disrupted attachment, coupled with specific types of subsequent trauma. The ICD-11 and DSM-5 are being developed at the start of the 21st century. Any radical new developments in classification are said to be more likely to be introduced by the APA than by the WHO, mainly because the former only has to persuade its own board of trustees whereas the latter has to persuade the representatives of over 200 different countries at a formal revision conference. In addition, while the DSM is a bestselling publication that makes huge profits for APA, the WHO incurs major expense in determining international consensus for revisions to the ICD. Although there is an ongoing attempt to reduce trivial or accidental differences between the DSM and ICD, it is thought that the APA and the WHO are likely to continue to produce new versions of their manuals and, in some respects, to compete with one another. There is some ongoing scientific doubt concerning the construct validity and reliability of psychiatric diagnostic categories and criteria even though they have been increasingly standardized to improve inter-rater agreement in controlled research. In the United States, there have been calls and endorsements for a congressional hearing to explore the nature and extent of harm potentially caused by this "minimally investigated enterprise". Other specific criticisms of the current schemes include: attempts to demonstrate natural boundaries between related syndromes, or between a common syndrome and normality, have failed; the disorders of current classification are probably surface phenomena that can have many different interacting causes, yet "the mere fact that a diagnostic concept is listed in an official nomenclature and provided with a precise operational definition tends to encourage us to assume that it is a "quasi-disease entity" that can be invoked to explain the patient's symptoms"; and that the diagnostic manuals have led to an unintended decline in careful evaluation of each individual person's experiences and social context. Psychodynamic schemes give this latter phenomenological aspect more consideration, but in psychoanalytic terms that have been long criticized on numerous grounds. Reliance on operational definition demands that intuitive concepts, such as depression need to be operationally defined before they become amenable to scientific investigation. However, John Stuart Mill pointed out the dangers of believing that anything that could be given a name must refer to a thing and Stephen Jay Gould and others have criticized psychologists for doing just that. One critic states that "Instead of replacing 'metaphysical' terms such as 'desire' and 'purpose', they used it to legitimize them by giving them operational definitions. Thus in psychology, as in economics, the initial, quite radical operationalist ideas eventually came to serve as little more than a 'reassurance fetish' (Koch 1992, 275) for mainstream methodological practice." Psychiatrist Joel Paris argues that psychiatry is sometimes susceptible to diagnostic fads. Some have been based on theory (overdiagnosis of schizophrenia), some based on etiological (causation) concepts (overdiagnosis of post-traumatic stress disorder), and some based on the development of treatments. Paris points out that psychiatrists like to diagnose conditions they can treat, and gives examples of what he sees as prescribing patterns paralleling diagnostic trends, for example an increase in bipolar diagnosis once lithium came into use, and similar scenarios with the use of electroconvulsive therapy, neuroleptics, tricyclic antidepressants, and SSRIs. He notes that there was a time when every patient seemed to have "latent schizophrenia" and another time when everything in psychiatry seemed to be "masked depression", and he fears that the boundaries of the bipolar spectrum concept, including in application to children, are similarly expanding. - Abnormal psychology - Diagnostic classification and rating scales used in psychiatry - Medical classification - Relational disorder (proposed DSM-5 new diagnosis) - Dalal PK, Sivakumar T. (2009) Moving towards ICD-11 and DSM-V: Concept and evolution of psychiatric classification. Indian Journal of Psychiatry, Volume 51, Issue 4, Page 310-319. - King LJ (March 1999). "A brief history of psychiatry: millennia past and present". Ann Clin Psychiatry 11 (1): 3–12. doi:10.3109/10401239909147041. PMID 10383169. http://www.springerlink.com/content/gt136u21p2x4t627/. - ^ Robin Murray (Md, M Phil; Murray, Robin (1997-10-28). The essentials of postgraduate psychiatry. ISBN 9780521578011. http://books.google.com/books?id=GnN2nEmpyIMC&pg=PA56. - ^ Berrios G E (April 1999). "Classifications in psychiatry: a conceptual history". Aust N Z J Psychiatry 33 (2): 145–60. doi:10.1046/j.1440-1614.1999.00555.x. PMID 10336212. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0004-8674&date=1999&volume=33&issue=2&spage=145. - ^ Perring, C. (2005) Mental Illness Stanford Encyclopedia of Philosophy - ^ Giosan C, Glovsky V, Haslam N (2001). "The Lay Concept of 'Mental Disorder': A Cross-Cultural Study". Transcultural Psychiatry 38 (3): 317–32. doi:10.1177/136346150103800303. http://tps.sagepub.com/cgi/content/full/38/3/317. - ^ a b c d World Health Organization (2005) (PDF). WHO Resource Book on Mental Health: Human rights and legislation. ISBN 924156282. http://www.who.int/mental_health/policy/who_rb_mnh_hr_leg_FINAL_11_07_05.pdf. - ^ Peck MC, Scheffler RM (September 2002). "An analysis of the definitions of mental illness used in state parity laws". Psychiatr Serv 53 (9): 1089–95. doi:10.1176/appi.ps.53.9.1089. PMID 12221306. http://ps.psychiatryonline.org/cgi/pmidlookup?view=long&pmid=12221306. - ^ Widiger TA, Sankis LM (2000). "Adult psychopathology: issues and controversies". Annu Rev Psychol 51: 377–404. doi:10.1146/annurev.psych.51.1.377. PMID 10751976. http://arjournals.annualreviews.org/doi/abs/10.1146/annurev.psych.51.1.377?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%3dncbi.nlm.nih.gov. PDF - ^ Office of the Surgeon General and various United States Government agencies (1999) Mental Health: A report of the Surgeon General - ^ US Department of Health and Human Sciences (2007) Mental Health & Mental Disorders: Terminology - ^ Parabiaghi A, Bonetto C, Ruggeri M, Lasalvia A, Leese M (June 2006). "Severe and persistent mental illness: a useful definition for prioritizing community-based mental health service interventions". Soc Psychiatry Psychiatr Epidemiol 41 (6): 457–63. doi:10.1007/s00127-006-0048-0. PMID 16565917. - ^ Economic and Social Research Council Mental Health and Mental Illness in the UK - ^ http://www.who.int/mental_health/evidence/en/ - ^ Berganza CE, Mezzich JE, Jorge MR (2002). "Latin American Guide for Psychiatric Diagnosis (GLDP)". Psychopathology 35 (2–3): 185–90. doi:10.1159/000065143. PMID 12145508. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=psp35185. - ^ Zero to Three. (1994). Diagnostic classification: 0–3: Diagnostic classification of mental health and developmental disorders in infancy and early childhood. Washington, DC. - ^ Zero to Three overview of the DC:0-3 - ^ Task Force on Research Diagnostic Criteria: Infancy Preschool (December 2003). "Research diagnostic criteria for infants and preschool children: the process and empirical support". J Am Acad Child Adolesc Psychiatry 42 (12): 1504–12. doi:10.1097/01.chi.0000091504.46853.0a (inactive 2009-06-30). PMID 14627886. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0890-8567&volume=42&issue=12&spage=1504. - ^ RDC-PA Online (PDF) - ^ Mises R, Quemada N, Botbol M, et al. (2002). "French classification for child and adolescent mental disorders". Psychopathology 35 (2–3): 176–80. doi:10.1159/000065141. PMID 12145506. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=psp35176. - ^ Mezzich JE (2002). "International surveys on the use of ICD-10 and related diagnostic systems". Psychopathology 35 (2–3): 72–5. doi:10.1159/000065122. PMID 12145487. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=psp35072. - ^ Jenkins R, Goldberg D, Kiima D, et al. (2002). "Classification in primary care: experience with current diagnostic systems". Psychopathology 35 (2–3): 127–31. doi:10.1159/000065132. PMID 12145497. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=psp35127. - ^ López-Muñoz F, García-García P, Sáiz-Ruiz J, et al. (2008). "A bibliometric study of the use of the classification and diagnostic systems in psychiatry over the last 25 years". Psychopathology 41 (4): 214–25. doi:10.1159/000125555. PMID 18408417. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=000125555. - ^ Nakane Y, Nakane H (2002). "Classification systems for psychiatric diseases currently used in Japan". Psychopathology 35 (2–3): 191–4. doi:10.1159/000065144. PMID 12145509. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=psp35191. - ^ Rogler LH (March 1997). "Making sense of historical changes in the Diagnostic and statistical manual of mental disorders: five propositions". J Health Soc Behav 38 (1): 9–20. doi:10.2307/2955358. JSTOR 2955358. PMID 9097505. - ^ James J. Hudziak; Helzer, John E. (2002). Defining Psychopathology in the 21st Century: Dsm-V and Beyond. American Psychopathological Association Series (1st ed.). Washington, DC: American Psychiatric Association. ISBN 1-58562-063-7. http://www.appi.org/book.cfm?id=62063. - ^ Cosgrove L, Krimsky S, Vijayaraghavan M, Schneider L (2006). "Financial ties between DSM-IV panel members and the pharmaceutical industry". Psychother Psychosom 75 (3): 154–60. doi:10.1159/000091772. PMID 16636630. http://content.karger.com/produktedb/produkte.asp?typ=fulltext&file=PPS2006075003154. - ^ Craddock, N., Owen, M. (2007) Rethinking psychosis: the disadvantages of a dichotomous classification now outweigh the advantages World Psychiatry v.6(2); Jun - ^ Maser JD, Akiskal HS (December 2002). "Spectrum concepts in major mental disorders". Psychiatr. Clin. North Am. 25 (4): xi–xiii. doi:10.1016/S0193-953X(02)00034-5. PMID 12462854. http://psych.theclinics.com/issues/contents?volume=25&issue=4. - ^ Krueger RF, Watson D, Barlow DH, et al. (November 2005). "Introduction to the Special Section: Toward a Dimensionally Based Taxonomy of Psychopathology". Journal of Abnormal Psychology 114 (4): 491–3. doi:10.1037/0021-843X.114.4.491. PMC 2242426. PMID 16351372. http://content.apa.org/journals/abn/114/4. - ^ Widiger TA, Simonsen E, Krueger R, Livesley WJ, Verheul R (June 2005). "PERSONALITY DISORDER RESEARCH AGENDA FOR THE DSM–V". J. Pers. Disord. 19 (3): 315–38. doi:10.1521/pedi.2005.19.3.315. PMC 2242427. PMID 16175740. http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2242427. - ^ a b c d Dalal PK, Sivakumar T. (2009) Moving towards ICD-11 and DSM-V: Concept and evolution of psychiatric classification. Indian Journal of Psychiatry, Volume 51, Issue 4, Page 310-319. - ^ Bentall R (2006). "Madness explained: why we must reject the Kraepelinian paradigm and replace it with a 'complaint-orientated' approach to understanding mental illness". Med. Hypotheses 66 (2): 220–33. doi:10.1016/j.mehy.2005.09.026. PMID 16300903. http://linkinghub.elsevier.com/retrieve/pii/S0306-9877(05)00509-8. - ^ Munro, Alistair; Bhugra, Dinesh (1997). Troublesome disguises: underdiagnosed psychiatric syndromes. Oxford: Blackwell Science. ISBN 0-86542-674-0. - ^ Millon, Theodore (2004). Masters of the Mind: Exploring the Story of Mental Illness from Ancient Times to the New Millennium. John Wiley & Sons. p. 38. ISBN 9780471469858. - ^ a b c Berrios GE (July 1987). "Historical aspects of psychoses: 19th century issues". Br. Med. Bull. 43 (3): 484–98. PMID 3322481. http://bmb.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=3322481. - ^ Bolme, A (1991). "Portraying Monomaniacs to Service the Alienist's Monomania: Gericault and Georget". Oxford Art Journal 14 (1): 79–91. JSTOR 1360279. - ^ O'Connell P, Woodruff PW, Wright I, Jones P, Murray RM (February 1997). "Developmental insanity or dementia praecox: was the wrong concept adopted?". Schizophr. Res. 23 (2): 97–106. doi:10.1016/S0920-9964(96)00110-7. PMID 9061806. http://linkinghub.elsevier.com/retrieve/pii/S0920-9964(96)00110-7. - ^ Mendelson G (December 2003). "Homosexuality and psychiatric nosology". Aust N Z J Psychiatry 37 (6): 678–83. doi:10.1111/j.1440-1614.2003.01273.x. PMID 14636381. http://www3.interscience.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0004-8674&date=2003&volume=37&issue=6&spage=678. - ^ Viner R (June 1999). "Putting Stress in Life: Hans Selye and the Making of Stress Theory". Social Studies of Science 29 (3): 391–410. doi:10.1177/030631299029003003. JSTOR 285410. - ^ Masten AS, Curtis WJ (2000). "Integrating competence and psychopathology: pathways toward a comprehensive science of adaptation in development". Dev. Psychopathol. 12 (3): 529–50. doi:10.1017/S095457940000314X. PMID 11014751. - ^ Kendell R, Jablensky A (January 2003). "Distinguishing between the validity and utility of psychiatric diagnoses". Am J Psychiatry 160 (1): 4–12. doi:10.1176/appi.ajp.160.1.4. PMID 12505793. http://ajp.psychiatryonline.org/cgi/pmidlookup?view=long&pmid=12505793. - ^ Baca-Garcia E, Perez-Rodriguez MM, Basurte-Villamor I, et al. (March 2007). "Diagnostic stability of psychiatric disorders in clinical practice". Br J Psychiatry 190 (3): 210–6. doi:10.1192/bjp.bp.106.024026. PMID 17329740. http://bjp.rcpsych.org/cgi/pmidlookup?view=long&pmid=17329740. - ^ Pincus, et al.; Zarin, DA; First, M (1998). ""Clinical Significance" and DSM-IV". Arch Gen Psychiatry 55 (12): 1145; author reply 1147–8. doi:10.1001/archpsyc.55.12.1145. PMID 9862559. http://archpsyc.ama-assn.org/cgi/content/extract/55/12/1145. - ^ http://www.psychdiagnosis.net/letter.html - ^ http://www.psychdiagnosis.net/endorsers.html - ^ DW Hands (2004) On Operationalisms and Economics Journal of Economic Issues - ^ Joel Paris, MD (2004) Psychiatric Diagnosis and the Bipolar Spectrum CPA Bulletin; 36:3) Wikimedia Foundation. 2010. Look at other dictionaries: Chinese Classification of Mental Disorders — The Chinese Classification of Mental Disorders (CCMD) (中国精神疾病分类方案与诊断标准), published by the Chinese Society of Psychiatry (CSP), is a clinical guide used in China for the diagnosis of mental disorders. It is currently on a third version, the CCMD 3 … Wikipedia Mental disorders diagnosed in childhood — Classification and external resources ICD 10 F70 F98 ICD 9 … Wikipedia History of mental disorders — The history of mental disorder spans prehistoric times, ancient civilisations, the Middle Ages, the early modern period, the enlightenment and modern times.Prehistoric timesThere is limited evidence by which to judge the existence or nature of… … Wikipedia Causes of mental disorders — Main article: Mental disorder The causes of mental disorders are complex, and interact and vary according to the particular disorder and individual. Genetics, early development, drugs, a loss of a family member, disease or injury, neurocognitive… … Wikipedia List of mental disorders — This list is incomplete; you can help by expanding it. The following is a list of mental disorders as defined by the DSM and ICD. The Diagnostic and Statistical Manual of Mental Disorders (DSM) is the American Psychiatric Association s standard… … Wikipedia Diagnostic and Statistical Manual of Mental Disorders — The Diagnostic and Statistical Manual of Mental Disorders (DSM) is published by the American Psychiatric Association and provides a common language and standard criteria for the classification of mental disorders. It is used in the United States… … Wikipedia Diagnostic and Statistical Manual of Mental Disorders — A system of classification, published by the American Psychiatric Association, that divides recognized mental disorders into clearly defined categories based on sets of objective criteria. Representing a majority view (rather than a consensus) of … Medical dictionary Diagnostic and Statistical Manual of Mental Disorders — Das Diagnostic and Statistical Manual of Mental Disorders (Diagnostisches und Statistisches Handbuch Psychischer Störungen) ist ein Klassifikationssystem der American Psychiatric Association (Amerikanische Psychiatrische Vereinigung), die diese… … Deutsch Wikipedia Diagnostic and Statistical Manual of Mental Disorders — Manuel diagnostique et statistique des troubles mentaux Pour les articles homonymes, voir DSM. Le Manuel diagnostique et statistique des troubles mentaux (en anglais : Diagnostic and Statistical Manual of Mental Disorders ou DSM), quatrième… … Wikipédia en Français Mental disorder — Classification and external resources Eight women representing prominent mental diagnoses in the 19th century. (Armand Gautier) ICD 10 F … Wikipedia	2	37	10	0	4	3	0.731172	17	9,369
New office's address : 9 avenue Mac Mahon, 75017 Paris - Métro Etoile Axillary lymph node surgery The lymph of the breast is drained by the lymphatic channels to the nodes in the armpit of the same side. These nodes form what is called the axillary lymph node chain. To find out the potential spread of cancer for each breast, axillary lymph nodes are examined for cancer cells. The only way of analysing these nodes is to remove them. A simple palpation or x-ray is not sufficient to determine if the nodes have been reached by the cancer or not. The removed nodes are sent to the pathologist for analysis. The data from the analysis are available a few days after the operation. This examination is important for determining the choice of postoperative treatments (radiotherapy, Chemotherapy, etc.) The invasion of the axillary lymph nodes is a significant element in planning breast cancer treatment. The lymphatic channels drain the lymph fluid (and therefore the tumour cells that are potentially circulating in it) primarily to the first nodes of the chain, known as the sentinel nodes (there are usually 2 or 3). These nodes – also called level 1 nodes – are located along the axillary edge of the breast, where the mammary gland joins the armpit. Level 2 corresponds to the axillary lymph nodes higher up, underneath the pectoralis minor muscle. Level 3 are the nodes located at the top of the arm pit, above the pectoralis minor muscle and under the collarbone. The lymph nodes in the armpit form a 'chain': if the level 1 nodes are free of cancer, then the level 2 and 3 nodes will not be affected. Si les Ganglions sentinelles ne contiennent pas de cellules tumorales, les autres ganglions de l’aisselle sont à priori indemnes. The surgeon removes a sample of the sentinel nodes to analyse them: if no tumour cells are found, he does not touch the other nodes. This technique can significantly reduce the number of nodes removed compared with Axillary dissection and thus reduce the risk of postoperative sequelae. It requires experienced surgeons. Identification, biopsy and analysis of sentinel nodes To identify the sentinel nodes The product is a blue dye and/or isotopic marker, i.e. a product which emits rays that are invisible to the eye (equivalent to X-rays). In the case of the latter, the marker is usually injected the day before the procedure, and a scintigraphic examination (a sort of x-ray) is performed to locate the sentinel lymph nodes. The sentinel lymph nodes are removed and examined, most often in the operating room, by the pathologist looking for tumour cells: this is known as a ‘frozen section examination’ (which takes place during the surgical procedure). If the sentinel lymph nodes are healthy, then it is not necessary to collect more nodes. However, if one or several sentinel nodes are affected, it is essential to carry out an axillary dissection (other nodes in the armpit are removed). If the sentinel lymph nodes are affected, the other nodes in the armpit are invaded in 40% of cases, which will mean that different treatments are proposed. Unfortunately, the frozen section examination is not completely reliable and the definitive answer can only be given by the pathologist a few days later. If the frozen section examination is negative but the final answer is positive, then it is necessary to complete the dissection... Around 5% of patients who undergo the sentinel lymph node technique must be re-operated on a few days later, to complete the dissection. Frozen section examination of sentinel lymph nodes The sentinel lymph node technique cannot be offered to everyone This technique is not considered : - if it is suspected either by clinical examination or by puncture test that the lymph nodes are invaded ; - when Chemotherapy or irradiation was performed before surgery (no study has demonstrated for the moment that the sentinel lymph node biopsy is reliable in these conditions) ; - when the patient has already had a surgical biopsy of a tumour near the armpit. This biopsy would have created a cavity in the breast, which as a result would have cut off the lymphatic channels leading from the tumour to the lymph nodes. - in the case of a large tumour (more than 5 cm) or multifocal cancer in several quadrants of the breast (multicentric cancer): in these cases, it is better to perform an axillary dissection. Axillary lymph node dissection - A traditional axillary dissection removes only the level 1 nodes and lymph nodes from the lower part of level 2. Generally, around ten nodes are removed (compared with around 20 in the case of a full axillary dissection). - A full axillary dissection removes the lymph nodes on all levels of the axillary chain (1, 2 and 3). This type of dissection is now very rarely carried out. In the case of breast-Conserving treatment, the incision for the Axillary dissection measures between 5 and 7 cm and is located in the armpit. For mastectomies with axillary dissection, the surgeon accesses the armpit using the same incision as for the mastectomy Innovation: Identifying lymph node involvement before surgery It is possible to get an idea of a patient’s lymph node involvement before surgery. In a woman with possible lymph node involvement, an ultrasound of the armpit coupled with fine needle aspiration (ultrasound-guided puncture) can sometimes show a lymph node involvement. The surgeon will then proceed immediately to an axillary dissection and not to a biopsy of the sentinel lymph nodes. In the event of lymph node involvement, certain scanners focusing on the lymph nodes can distinguish a partial involvement of the armpit, enabling a partial dissection; whereas without this information a more extensive dissection would have been carried out (ref...). During an axillary dissection, using very high quality imaging, the surgeon can carry out less aggressive dissection actions, while preserving the nodes that drain the arm (ref).	2	7	1	0	0	0	0.36109	1	1,312
\|Home \| About \| Journals \| Submit \| Contact Us \| Français\| Successful treatment of cancer is highly dependent on the stage at which it is diagnosed. Early diagnosis, when the disease is still localized at its origin, results in very high cure rates—even for cancers that typically have poor prognosis. Biopsies are often used for diagnosis of disease. However, because biopsies are destructive, only a limited number can be taken. This leads to reduced sensitivity for detection due to sampling error. A real-time fluorescence confocal microlaparoscope has been developed that provides instant in vivo cellular images, comparable to those provided by histology, through a nondestructive procedure. The device includes an integrated contrast agent delivery mechanism and a computerized depth scan system. The instrument uses a fiber bundle to relay the image plane of a slit-scan confocal microlaparoscope into tissue. It has a 3-μm lateral resolution and a 25-μm axial resolution. Initial in vivo clinical testing using the device to image human ovaries has been done in 21 patients. Results indicate that the device can successfully image organs in vivo without complications. Results with excised tissue demonstrate that the instrument can resolve sufficient cellular detail to visualize the cellular changes associated with the onset of cancer. This paper describes the first clinical confocal microlaparoscope system. We describe the instrument and how it evolved during clinical testing. We present both in vivo and ex vivo results showing that the device has the ability to nondestructively resolve cellular detail and has the potential to diagnose cancer in situ. Cancer frequently goes undetected until it reaches a late, difficult-to-treat, stage. Cancers that develop in epithelial cells (carcinomas) are the most common type of malignancy, and they account for 90% of all human cancers.1 Development of imaging devices that can more thoroughly interrogate epithelial surfaces for abnormalities will enable earlier detection of cancer, resulting in significant improvement in patient survival. The most effective treatments in terms of patient outcomes, quality of life, and cost are those that target cancer during its early development, before it has metastasized. Early diagnosis, when the disease is still localized at its origin, results in very high cure rates—even for cancers that typically have poor prognosis. Early detection provides the physician with more treatment options and the ability to use less invasive methods. Unfortunately, many cancers are not found until later stages due to inadequate detection methods or low-sensitivity diagnostic techniques. Initial detection and diagnosis of cancer is frequently based on biopsies. Small samples of tissue are removed, and a pathologist makes a diagnosis using thin sections of stained and processed tissue. Since biopsies require removal of the tissue, only a limited number can be taken. Sampling error plays a significant role in achieving accurate early detection of cancer. Development of devices that can nondestructively interrogate epithelial surfaces for abnormalities would reduce sampling error. Many groups are investigating new technologies to nondestructively image tissue at high resolution for the early diagnosis of cancer. Some of the promising methods are confocal microscopy,2–10 two-photon microscopy,11,12 spectroscopy,13–18 and optical coherence tomography (OCT).19–25 Conventional bright-field microscopy plays a central role in the diagnosis of disease from carefully prepared biopsy slides. Bright-field images of bulk tissue appear blurry due to simultaneous collection of out-of-focus planes. Improved imaging of thick samples can be achieved with confocal microscopy. Confocal images of bulk tissue are sharp because light from out-of-focus planes is rejected via a confocal aperture. Since the confocal microscope alleviates the need for cutting tissue into thin sections, it has significant potential for imaging of tissue in situ. However, a standard confocal microscope is a large device that is not well suited for in vivo assessment of epithelial surfaces inside the human body. We have previously reported on technology developed to enable in vivo confocal imaging using a coherent fiber optic bundle.26–30 In this paper, we describe development of a confocal microlaparoscope. A confocal microlaparoscope offers the potential for cellular imaging of a wide range of laparoscopically accessible organs. Almost every organ in the human body can be accessed using a laparoscope via a small incision.31 Organs that can be accessed in this manner include the stomach, intestines, pancreas, liver, esophagus, spleen, kidneys, peritoneal wall, bladder, gall bladder, lymph nodes, reproductive organs in females, and prostate in men. Laparoscopic surgery has significant advantages over open surgery. It minimizes the overall trauma to the skin and muscles, patient recovery time is shorter, and the infection rate is significantly reduced. In the following sections, we discuss the four-year development of a mobile confocal microlaparoscope imaging system. To demonstrate the device’s potential, we present in vivo and ex vivo human imaging results. We have developed a complete clinical confocal microlaparoscope system that can be wheeled into an operating room and set up to image within a few minutes. The system consists of a confocal microlaparoscope (hereafter referred to as a microlaparoscope) connected to a mobile cart housing an optical scan unit (OSU) and control systems. Once the system is running, the surgeon can interrogate regions of interest with the microlaparoscope; view live cellular images on the screen; and selectively record videos, depth scans, multispectral images, and still frames. Figure 1 shows the instrument in use in the surgical suite. The mobile cart is on the right, and the surgeon using the microlaparoscope is in the middle looking toward the left, where the gross anatomy is visible on a standard wide-field laparoscope screen. Directly adjacent to this screen is another display showing the live cellular images from the microlaparoscope system. The microlaparoscope provides live images of excited fluorescence at 30 frames per second with an optical resolution of 3 μm laterally and 25 μm axially. The system frame rate is fundamentally limited by the acquisition rate of the CCD camera used for detection. Fluorescent contrast agents are delivered locally to the field of view using an integrated contrast agent delivery system. Using controls on the microlaparoscope, the surgeon can adjust focus from the nominal epithelial position down to an optical limit of 200 μm below the surface. Table 1 provides a detailed listing of the system’s specifications. The evolution from a laboratory system26,28–30 to a successful surgical system entailed significant translational research over a four-year period.32–35 The work can be broken into two major areas: (1) the development of a mobile cart with a miniaturized confocal scan assembly (the OSU) and surgical control system and (2) the development of the clinical microlaparoscope. Both areas presented major challenges. The mobile cart required a redesign of the OSU and a change of the laser excitation system from a water-cooled gas laser to a small air-cooled solid-state laser. Making the microlaparoscope easy to use entailed significant developments to tightly integrate the focus and dye delivery systems to ensure quick and reliable response. In addition, the device had to be sterilizable, fit inside standard surgical trocars, and be safe to use inside a patient. In the following sections, we describe the mobile cart, the associated systems that it houses, and the functions that it provides. Then we will discuss the evolution of the microlaparoscope, beginning with the early prototypes and ending with the state-of-the-art instrumentation currently being used in human clinical trials. Details concerning the integrated in-handle axial focus and contrast agent delivery systems are also presented. The mobile cart houses the OSU and the control systems. The instrumentation fits on a standard endoscopy cart that has a 61 cm by 41 cm footprint. Figure 2 depicts the components on the mobile cart. The top shelf houses the sterile microlaparoscope and the surgical supplies that are used during the imaging procedure. The microlaparoscope cable connects to the OSU located on the second shelf. The third and fourth shelves house the laser, function generator, and computer. The bottom shelf houses a medical-grade isolation transformer. The operator display and controls for collecting data during surgery are located on a platform at standing height. A second mobile display is placed next to the operative field for viewing nearby the surgeon. The system has been designed to streamline all operations during surgery. Once the system is plugged in and the safety interlocks engaged, the system starts, and all hardware is initialized. Hardware initialization includes the solid-state laser, camera, dye delivery system, focus system, and function generator for scan mirror control. After the automatic initialization, the operator is presented with the software control interface for live imaging. Miniaturization of the OSU required a redesign of the previous bench-top system. By simplifying the optical layout and integrating the multispectral imaging segment into a common collection path,36 we were able to fit the whole scan system into a 50 cm by 30.5 cm housing. The housing also integrates the scan mirror power supply and control electronics, which were external in the previous system. The components of the OSU are shown in Fig. 3. The top of the figure shows how the system operates in the standard grayscale imaging mode. In this mode, a 488-nm solid-state laser beam is expanded and anamorphically shaped into a line via a cylindrical lens. The laser light is then reflected into the image path by a dichroic filter, imaged to a line by a microscope objective, and scanned by the object scan mirror across the coherent fiber bundle face in the microlaparoscope’s proximal connector. Tissue fluorescence is collected back through the microlaparoscope and descanned by the object scan mirror. The dichroic filter passes the fluorescence signal, which is focused down onto a stationary confocal slit. The light exiting the slit is recollimated and rescanned using the image scan mirror. An emission filter removes residual excitation light. The beam is refocused back into a line that sweeps across the camera to build up a two-dimensional (2-D) image every thirtieth of a second. In addition to 2-D grayscale imaging, the system can also collect multispectral data.27,29,36 This multispectral mode is activated in a fraction of a second via a software actuation button that deflects the image scan mirror to its extreme position (shown in the bottom of Fig. 3). During spectral collection, the image scan mirror is held stationary. The light passes through a prism dispersing the signal across the CCD. The CCD camera records spectral information in the direction perpendicular to the slit and one dimension of spatial data in the dimension parallel to the slit. The second spatial dimension of the image is collected over time by recording multiple frames as the object scan mirror is slowly stepped. The complete spectral data collection procedure executes in a few seconds. Once spectral collection is complete, the system reverts back to real-time grayscale operating mode. A software package that interfaces with the data collection devices and the system controls was written using Objective C 2.0 and Cocoa with automatic garbage collection on Mac OS X. Figure 4 provides a general overview of the software and hardware interface. The software links the surgical controls on the microlaparoscope to the control systems that manage contrast agent delivery, focus, and data acquisition. Parallel processing allows the software to run smoothly while live images are acquired, processed, and encoded at 30 frames per second. A custom image processing memory pool class enables efficient resource management under automatic garbage collection. Figure 5 shows the software interface. It provides a simple interface for viewing and collecting live images during the surgical procedure. The software has controls to (1) start live acquisition, (2) save the current frame, (3) record video, (4) deliver dye, (5) load dye, and (6) adjust image display. In addition to the basic controls, the system also records procedure and patient information, which is archived with each image. System information, including image dynamic range and imaging depth, are also visible. During operation, the surgeon can easily see real-time imagery on the surgical display (shown in Fig. 1) along with the instrument’s current status. Contrast agent delivery, focus, and data acquisition can be initiated by the surgeon via integrated controls on the third-generation microlaparoscope handle, described in the next section. Use of the surgical microlaparoscope entails placing the distal end in contact with the tissue, locally delivering fluorescent contrast agents to the field of view, adjusting focus, and collecting the resultant fluorescent confocal image. To create the surgical microlaparoscope, we had to develop (1) a reliable focus mechanism, (2) a localized contrast agent delivery system, and (3) an ergonomic sterilizable housing with surgical controls compatible with a 5-mm-diam trocar instrument port. The development process encompassed three generations of instruments, with the final version meeting all of the requirements. Table 2 summarizes the key properties of the three designs. In all three designs, the microlaparoscope contains a 30,000-element fiber bundle connected via an subminiature version A (SMA) connector to the OSU. The OSU generates a line of laser illumination that sweeps across the proximal face of the fiber. The fiber bundle spatially relays the illumination pattern to the distal end of the fiber bundle, where a miniature 0.46 NA objective lens30 images the illumination line to the desired tissue depth. Image depth is controlled by adjusting the axial spacing between the fiber bundle and the miniature objective lens. Contrast agent is locally delivered to the tissue through the microlaparoscope. The excited fluorescent signal is collected by the miniature lens and relayed back through the fiber bundle into the OSU. The first-generation design—depicted in Fig. 6—utilized a sterilizable semirigid 5-mm-diam by 500-mm-long dual-lumen polycarbonate sleeve through which a nonsterile imaging catheter was inserted.30 This simple design allowed us to rapidly move to clinical testing by creating a sterile housing for our existing 3-mm flexible imaging catheter. The polycarbonate also integrated a small secondary lumen to deliver dye locally to the field of view. The front end of the polycarbonate sleeve was sealed with a 160-μm-thick glass window. A 500-μm-diam secondary lumen, used to deliver contrast agent, ran parallel to the primary lumen. A 200-μm-diam hole in the window was placed over the secondary lumen, allowing contrast agent delivery to the tissue. The rear end of the polycarbonate housing contained the couplings to mate the imaging catheter and the dye delivery line. In the operating room, the compression coupling of the sterilized polycarbonate housing was loosened, and the non-sterile imaging catheter was fed through the main 3.1-mm lumen until the miniature objective was in contact with the glass window. The fitting was tightened, and then a sterile plastic cover was slid back over the portion of the imaging catheter that was in the operative field. A Luer Lock fitting coupled the fluid lumen to a 0.58-mm medical-grade Teflon tubing that ran back to the mobile cart. The 3-mm flexible catheter consisted of a miniature objective lens connected to durable polyetheretherketone (PEEK) tubing. The 30,000-element fiber optic bundle ran through the lumen of the PEEK tubing. Originally, focus was accomplished by a manual micrometer at the proximal end that moved the fiber bundle relative to the PEEK housing. However, focus control was unreliable due to an inexact fit of the fiber bundle inside the PEEK tubing. This resulted in hysteresis in the proximal movement of the fiber bundle relative to the fixed lens. Various methods were studied to improve the focus system.29,30,34 Ultimately, the manual focus micrometer was replaced by a computerized stepper motor. A hysteresis correction algorithm was developed to position the distal fiber face with an accuracy better than the system’s axial resolution. Figure 7 illustrates the ability of the first-generation focus system to image the epithelial surface of tissue (mouse peritoneal wall) and then to controllably focus the underlying tissue layers in 25-μm increments. Dye delivery was accomplished by loading a syringe with fluorescent contrast agent. The syringe was then inserted into a syringe pump located on the mobile cart. The syringe was connected to the secondary lumen of the polycarbonate housing through 0.58-mm-diam medical-grade Teflon tubing. Software control of the syringe pump enabled delivery of 1-to 3-μL droplets of contrast agent onto the tissue surface near the field of view of the miniature objective. Figure 8 shows a photograph of the distal end of the microlaparoscope with a droplet of dye coming out through the hole in the glass window. The device worked well using the 1.2-m-long imaging catheter. However, it was determined that a longer length imaging catheter was needed in the operating room. With the longer length catheter, the focus motor and the syringe pump were located far from the distal microlaparoscope tip, and focus would drift during use. Delivery of small droplets of contrast agent was unreliable. The surgeon also noted that positioning the proximal tip accurately was difficult because the polycarbonate housing was not rigid enough. A second-generation device was developed to address the issues concerning device rigidity, focus drift, and reliability of contrast agent delivery across a 6-m-long connection between the microlaparoscope and the OSU. The second design abandoned the use of the existing flexible imaging catheter. To address the reliability issues of focus and dye delivery in a 6-m-long device, the controls for focus and dye delivery were located close to the distal tip. To address the rigidity issue, a rigid instrument was made using a stainless steel tube with an inner diameter matching the fiber bundle’s outer diameter. Figure 9 shows a diagram of the second-generation microlaparoscope. The miniature objective attached to the distal end of the 3-mm-diam rigid stainless steel tube. The fiber bundle ran through the 1 mm inside diameter of the rigid tube. To adjust focus, the fiber bundle moved axially relative to the fixed lens position. The tube and fiber bundle were held in place by a round handle containing a manual focus mechanism. Rotation of the handle knob resulted in precise movement of the proximal fiber face relative to the rigid tube. To deliver contrast agents, a 21-gauge hypodermic tube ran parallel to the 3-mm rigid tube. Both tubes were sealed inside medical-grade fluorinated ethylene-propylene (FEP) heat-shrink tubing, resulting in a final 5-mm outer diameter. At the distal tip, the hypodermic tube made a 90-deg bend, dispensing contrast agents at the edge of the imaging field. At the back end of the handle, the hypodermic tubing passed into a computer controlled piezo valve immediately downstream from a pressurized syringe. A 6-m-long flexible cable ran from the handle to the OSU. The cable contained the fiber bundle and piezo valve electrical wires. The proximal end of the protective housing broke out into optical and electrical connectors. A standard SMA connector coupled the fiber bundle to the OSU. Compared to the first-generation system, the second-generation system was much easier to use because the whole microlaparoscope could be sterilized and then quickly connected to the OSU in the operating room. In contrast, the first-generation system required careful placement of the sterilized polycarbonate housing over the nonsterile imaging catheter permanently connected to the OSU. The rigid stainless steel tubing solved the rigidity issues, enabling the surgeon to precisely position the device. A press of a button at the operator console enabled rapid delivery of a small droplet of contrast agent, as shown in Fig. 10. Focus was extremely reliable and never drifted. In fact, during typical use, the surgeon would set the focus at the epithelial surface and image multiple sites without needing to refocus. Although the second-generation device addressed the primary issues encountered with the first-generation instrument, there were additional improvements that were important to make a successful clinical tool. Even though the manual focus system was reliable, a computerized system was desired to enable depth scans. Second, to allow the device to be controlled more efficiently by the surgeon, the microlaparoscope needed to integrate basic controls for focus, dye delivery, and data recording into the handle. The dye delivery system also needed some refinement. In the second-generation device, to ensure repeatable delivery of small contrast agent droplets, the pressure in the syringe had to be well regulated at a relatively high 1.76 kg/cm2. This pressure would sometimes cause contrast agent to squirt out of the distal tip rather than pool up in a localized droplet. To implement these improvements, a third-generation device was developed. The new microlaparoscope, shown in Fig. 11, has an ergonomic handle, control buttons, a computerized focus system, and a refined contrast agent delivery system. Control of focus and dye delivery are consistent and reliable since the control systems were miniaturized and located in the handle only 35 cm from the distal end of the device. A non-pressurized dye delivery system removed the problem encountered with dye ejection. Mechanically, the third-generation microlaparoscope followed the same design principles as the second-generation device. Figure 12 illustrates the mechanical aspects of the device. The fiber bundle couples to the focus motor inside the handle. A 1-cm3 syringe with contrast agent is placed into a spring-loaded receiver at the front end of the handle. A second miniature motor in the handle acts as a plunger for the syringe. A spring forces the syringe to mate with a Luer Lock connection that guides the fluid down a 21-gauge hypodermic tube. A customized tip helps to channel the dye and minimize tissue abrasion. The new tip is a 20-mm PEEK housing mated to the FEP outer tubing. A tiny 150-μm lumen inside the PEEK couples the hypodermic fluid line routing the dye to an exit port at the edge of the system’s field of view. In the second-generation design, the hypodermic tubing was bent around the tip of the miniature objective and had the potential to abrade the tissue surface. The new smooth tip helps to protect the tissue surface and allows the fluid exit port to be placed closer to the edge of the imaging field of view. The handle contains four ergonomically positioned controls. Two upper thumb controls on the back allow the surgeon to adjust focus and acquire a depth scan. The trigger button saves still frames with a short press and acquires video with a long press. The side button delivers a predefined amount of contrast agent to the field of view. The electrical wires and the fiber bundle are routed through the handle into a flexible 6-m protective cable. The rear end of the cable breaks out into an electrical connector and an SMA connector. Both connections couple to receptacles on the OSU. The electrical connection routes through the electronics of the OSU and exits as a single USB cable connecting to the computer which link the buttons and motors to the control software. The SMA connector couples the fiber bundle to the OSU. The third-generation microlaparoscope represents a viable surgical tool. Its rigid probe enables precise positioning control. The in-handle computerized focus system enables axial focus and depth scans with accurate 1-μm positioning. The contrast agent system delivers volumes with precision down to 50 nL. Last, the whole device has an ergonomic design that is comfortable for the surgeon to use and provides controls for all the tasks that the surgeon needs to perform. As previously mentioned, laparoscopic techniques can be used to access most organs in the body. To demonstrate the clinical viability of the confocal microlaparoscope system, we present results from two organs imaged using the device. The first set of results are in vivo and ex vivo images of the epithelial surface of ovarian tissue. Since there is no reliable way to detect ovarian cancer in its early stages,37 the microlaparoscope could be used to detect ovarian cancer in high-risk women. The second set of results are ex vivo images of human esophagus. Although laparoscopic esophageal surgery is a recent innovation, it is now second only to biliary tract surgery in the frequency of minimally invasive procedures performed in everyday surgical practice.38 The microlaparoscope’s ability to visualize the cellular boundaries of tumors could improve the success rates in laparoscopic staging of carcinoma of the esophagus and laparoscopic esophogectomy. Currently, the microlaparoscope is being evaluated in a clinical trial to image the epithelial surface of the ovary at the University of Arizona’s Medical Center in Tucson. The device was granted “nonsignificant risk” status by the University of Arizona’s Institutional Review Board and has been approved for use in humans using a protocol that includes topical application of contrast agents.33 To date, 21 patients have been imaged in vivo. The current protocol entails imaging human ovaries in vivo before oophorectomy or hysterectomy. The imaging protocol begins with the surgeon locating the ovary and isolating it in an endobag with the ovary still connected to the blood supply (see Fig. 13). Once the ovary is isolated, the surgeon brings the tip of the microlaparoscope into contact with the epithelial surface. Then the surgeon delivers the contrast agent, and live imaging begins. The endobag protects the patient from inadvertent exposure to contrast agent. After the microscopic imaging is done, the surgical procedure is completed as normal, and the ovaries are removed. The removed ovary is typically imaged again ex vivo using the microlaparoscope with additional contrast agents. Biopsies are also taken for correlated pathology. For the initial in vivo clinical studies, fluorescein sodium was selected because of its existing track record of safe use in humans.39,40 (All patients participating in the clinical trials were consented and imaged in accordance with human subjects protocols approved by the Institutional Review Board of the University of Arizona.) Although fluorescein provides limited diagnostic contrast when applied to the surface of the ovary, it is a safe contrast agent and allowed us to test the safety and basic functionality of the microlaparoscope system in vivo. Figure 14 shows nine examples of in vivo images obtained with the microlaparoscope system. The images demonstrate that the device functions as designed. The microlaparoscope can deliver controlled volumes of dye to the image site and then display real-time cellular-level images to the surgeon. The focus mechanism works well. After an initial adjustment of the focus, the instrument can be moved to various sites on the ovary while still maintaining good focus on the epithelial surface. After in vivo imaging with fluorescein, the surgeon removes the ovaries. The ovaries are reimaged ex vivo using acridine orange (AO). Compared to fluorescein, AO provides superior diagnostic contrast. Example images with AO, shown in Fig. 15, demonstrate the excellent cellular-level contrast achievable with the instrument. The epithelial surface of a healthy ovary is characterized by a homogeneous distribution of bright nuclei, as seen in Fig. 15(a). The epithelial surface cells of the ovary are delicate, and partial denuding can occur, exposing the underlying stroma [Fig. 15(b)]. Below the epithelial surface, healthy stroma also exhibits a characteristically homogenous structure albeit with a different nuclear size distribution and shape [Fig. 15(c)]. In the case of ovarian cancer, the tissue structure is visibly different [Fig. 15(d)]. The epithelial surface is irregular, and the high degree of heterogeneity in the size and spatial distribution of nuclei is indicative of ovarian cancer. We have previously shown41 that the microlaparoscope system can easily differentiate between normal epithelium and ovarian cancer using automated algorithms. It also appears that the microlaparoscope system may be able to visualize cellular changes that happen prior to the onset of cancer. Less distinct tissue changes such as tissue sclerosis may also be detectable [Figs. 15(e) and 15(f)]. With the success of our initial testing with fluorescein, we have now begun imaging in vivo with AO. (Approval from the FDA to use AO in this context has been granted under IND 102603.) Figure 16 shows our preliminary results. These images depict the same homogeneous structure of nuclei as seen in Figs. 15(a) and 15(b). Denuding of the delicate epithelium is also visible in some of the images. By optimizing the concentration of AO and giving surgeons more time to practice using the device, we believe we will achieve results comparable to the images obtained ex vivo. We have conducted ex vivo imaging of esophagus tissue biopsies from more than 30 patients. Figure 17(a) shows an example of normal squamous epithelium. Figure 17(b) shows epithelial tissue that closely resembles the intestine, with columnar-appearing mucosa and intestinal metaplasia, a condition known as Barrett’s esophagus. Finally, Fig. 17(c) illustrates a tumor in the esophagus. These images illustrate the device’s ability to resolve the cellular details of tissue in the esophagus. Nuclear distributions are easily characterized, and morphological tissue changes can be readily discerned. In the case of laparoscopic esophogectomy, the microlaparoscope would allow the surgeon to optically biopsy suspect tissues to potentially find locations containing cancer. A mobile confocal microlaparoscope system capable of performing live optical biopsies in vivo has been developed. The system is currently being evaluated in clinical trials to assess its safety and efficacy for detecting ovarian cancer. Results show that the instrument is safe and can successfully image ovaries in vivo. The in vivo and ex vivo results indicate that the microlaparoscope can resolve sufficient cellular detail to detect the transformation of normal epithelium and visualize cellular changes that happen with the onset of cancer. As we continue testing, we hope to show that the device can obtain the same high-quality images in vivo as have been obtained ex vivo. Because the microlaparoscope can nondestructively provide real-time cellular images in vivo, it has the potential to significantly improve the rate of early cancer detection in laparoscopically accessible organs. Today, early detection of cancer is highly dependent on the selection of biopsy sites. Since biopsies are destructive, only a few are typically taken. If cancer is in its early stages, it will often be small, reducing the probability that it will be biopsied. Moreover, it takes time to get the biopsies processed and evaluated. However, because the microlaparoscope nondestructively acquires optical biopsies instantly, a much larger region of tissue can be interrogated at the cellular level. Additionally, if the surgeon finds a small cancer, the microlaparoscope could be used to locate the cancer boundaries allowing for immediate resection. Thus, the device might improve the probability of early cancer detection and help the surgeon fully resect the tumor during the same procedure. In the future, we will continue to investigate the wide range of potential applications for the confocal microlaparoscope system. Since the microlaparoscope is based on an inherently flexible fiber bundle, we plan on making a device with a flexible probe. We have previously shown a flexible imaging catheter and plan to apply the technology developed for the microlaparoscope to a new confocal microgastroscope for interrogating the gastrointestinal tract. The authors wish to acknowledge the contribution of Dr. Molly Brewer, Dr. Richard Sampliner, Kathy Schmidt, and the surgical staff at UMC. Dr. Brewer helped motivate the development of the instrument and gave guidance on how such an instrument would be used in a clinical setting. Dr. Sampliner provided human esophagus biopsies for the ex vivo imaging studies. Kathy Schmidt and the surgical staff provided valuable assistance in the clinical trials. Last, we recognize the National Institutes of Health and the Arizona Disease Control Research Commission for their funding support provided through the following grants: NIH CA 73095, NIH CA 115780, and ADCRC 9711. This paper is a revision of a paper presented at the SPIE conference Endoscopic Microscopy IV, January 2009, San Jose, California. The paper presented there appears (unrefereed) in SPIE Proceedings Vol. 7172. Anthony A. Tanbakuchi, University of Arizona, Department of Radiology, Radiology Research Laboratories, 1609 North Warren Avenue, Tucson, Arizona 85724 and University of Arizona, College of Optical Sciences, 1630 East University Boulevard, Tucson, Arizona 85724. Andrew R. Rouse, University of Arizona, Department of Radiology, Radiology Research Laboratories, 1609 North Warren Avenue, Tucson, Arizona 85724. Joshua A. Udovich, University of Arizona, Department of Radiology, Radiology Research Laboratories, 1609 North Warren Avenue, Tucson, Arizona 85724 and University of Arizona, College of Optical Sciences, 1630 East University Boulevard, Tucson, Arizona 85724. Kenneth D. Hatch, University of Arizona, Arizona Health Sciences Center, Department of Obstetrics and Gynecology, P.O. Box 245078, Tucson, Arizona 85724. Arthur F. Gmitro, University of Arizona, Department of Radiology, Radiology Research Laboratories, 1609 North Warren Avenue, Tucson, Arizona 85724 and University of Arizona, College of Optical Sciences, 1630 East University Boulevard, Tucson, Arizona 85724.	2	21	6	0	0	0	0.394772	6	7,201
Muscular Dystrophies and Other Muscle Diseases Skeletal muscle diseases, or myopathies, are disorders with structural changes or functional impairment of muscle. These conditions can be differentiated from other diseases of the motor unit (e.g., lower motor neuron or neuromuscular junction pathologies) by characteristic clinical and laboratory findings. Most myopathies present with proximal, symmetric limb weakness (arms or legs) with preserved reflexes and sensation. However, asymmetric and predominantly distal weakness can be seen in some myopathies. An associated sensory loss suggests injury to a peripheral nerve or the central nervous system (CNS) rather than myopathy. On occasion, disorders affecting the motor nerve cell bodies in the spinal cord (anterior horn cell disease), the neuromuscular junction, or peripheral nerves can mimic findings of myopathy. Muscle Weakness Symptoms of muscle weakness can be either intermittent or persistent. Disorders causing intermittent weakness (Fig. 462e-1) include myasthenia gravis, periodic paralyses (hypokalemic, hyperkalemic, and paramyotonia congenita), and metabolic energy deficiencies of glycolysis (especially myophosphorylase deficiency), fatty acid utilization (carnitine palmitoyltransferase deficiency), and some mitochondrial myopathies. The states of energy deficiency cause activity-related muscle breakdown accompanied by myoglobinuria, appearing as light-brown- to dark-brown-colored urine. FIGURE 462e-1 Diagnostic evaluation of intermittent weakness. AChR AB, acetylcholine receptor antibody; CPT, carnitine palmitoyltransferase; EOMs, extraocular muscles; MG, myasthenia gravis; PP, periodic paralysis. Most muscle disorders cause persistent weakness (Fig. 462e-2). In the majority of these, including most types of muscular dystrophy, polymyositis, and dermatomyositis, the proximal muscles are weaker than the distal and are symmetrically affected, and the facial muscles are spared, a pattern referred to as limb-girdle. The differential diagnosis is more restricted for other patterns of weakness. Facial weakness (difficulty with eye closure and impaired smile) and scapular winging (Fig. 462e-3) are characteristic of facioscapulohumeral dystrophy (FSHD). Facial and distal limb weakness associated with hand grip myotonia is virtually diagnostic of myotonic dystrophy type 1. When other cranial nerve muscles are weak, causing ptosis or extraocular muscle weakness, the most important disorders to consider include neuromuscular junction disorders, oculopharyngeal muscular dystrophy, mitochondrial myopathies, or some of the congenital myopathies (Table 462e-1). A pathognomonic pattern characteristic of inclusion body myositis is atrophy and weakness of the flexor forearm (e.g., wrist and finger flexors) and quadriceps muscles that is often asymmetric. Less frequently, but important diagnostically, is the presence of a dropped head syndrome indicative of selective neck extensor muscle weakness. The most important neuromuscular diseases associated with this pattern of weakness include myasthenia gravis, amyotrophic lateral sclerosis, late-onset nemaline myopathy, hyperparathyroidism, focal myositis, and some forms of inclusion body myopathy. A final pattern, recognized because of preferential distal extremity weakness, is typical of a unique category of muscular dystrophy, the distal myopathies. FIGURE 462e-2 Diagnostic evaluation of persistent weakness. Examination reveals one of seven patterns of weakness. The pattern of weakness in combination with the laboratory evaluation leads to a diagnosis. ALS, amyotrophic lateral sclerosis; CK, creatine kinase; DM, dermatomyositis; EMG, electromyography; EOMs, extraocular muscles; FSHD, facioscapulohumeral dystrophy; IBM, inclusion body myositis; MG, myasthenia gravis; OPMD, oculopharyngeal muscular dystrophy; PM, polymyositis. FIGURE 462e-3 Facioscapulohumeral dystrophy with prominent scapular winging. NEUROMUSCULAR CAUSES OF PTOSIS OR OPHTHALMOPLEGIA It is important to examine functional capabilities to help disclose certain patterns of weakness (Table 462e-2). The Gowers’ sign (Fig. 462e-4) is particularly useful. Observing the gait of an individual may disclose a lordotic posture caused by combined trunk and hip weakness, frequently exaggerated by toe walking (Fig. 462e-5). A waddling gait is caused by the inability of weak hip muscles to prevent hip drop or hip dip. Hyperextension of the knee (genu recurvatum or back-kneeing) is characteristic of quadriceps muscle weakness; and a steppage gait, due to footdrop, accompanies distal weakness. OBSERVATIONS ON EXAMINATION THAT DISCLOSE MUSCLE WEAKNESS FIGURE 462e-4 Gowers’ sign showing a patient using his arms to climb up the legs in attempting to get up from the floor. FIGURE 462e-5 Lordotic posture, exaggerated by standing on toes, associated with trunk and hip weakness. Any disorder causing muscle weakness may be accompanied by fatigue, referring to an inability to maintain or sustain a force (pathologic fatigability). This condition must be differentiated from asthenia, a type of fatigue caused by excess tiredness or lack of energy. Associated symptoms may help differentiate asthenia and pathologic fatigability. Asthenia is often accompanied by a tendency to avoid physical activities, complaints of daytime sleepiness, necessity for frequent naps, and difficulty concentrating on activities such as reading. There may be feelings of overwhelming stress and depression. Thus, asthenia is not a myopathy. In contrast, pathologic fatigability occurs in disorders of neuromuscular transmission and in disorders altering energy production, including defects in glycolysis, lipid metabolism, or mitochondrial energy production. Pathologic fatigability also occurs in chronic myopathies because of difficulty accomplishing a task with less muscle. Pathologic fatigability is accompanied by abnormal clinical or laboratory findings. Fatigue without those supportive features almost never indicates a primary muscle disease. Muscle Pain (Myalgias), Cramps, and Stiffness Muscle pain can be associated with cramps, spasms, contractures, and stiff or rigid muscles. In distinction, true myalgia (muscle aching), which can be localized or generalized, may be accompanied by weakness, tenderness to palpation, or swelling. Certain drugs cause true myalgia (Table 462e-3). DRUGS THAT CAUSE TRUE MYALGIA There are two painful muscle conditions of particular importance, neither of which is associated with muscle weakness. Fibromyalgia is a common, yet poorly understood, type of myofascial pain syndrome. Patients complain of severe muscle pain and tenderness and have specific painful trigger points, sleep disturbances, and easy fatigability. Serum creatine kinase (CK), erythrocyte sedimentation rate (ESR), electromyography (EMG), and muscle biopsy are normal (Chap. 396). Polymyalgia rheumatica occurs mainly in patients >50 years and is characterized by stiffness and pain in the shoulders, lower back, hips, and thighs (Chap. 385). The ESR is elevated, while serum CK, EMG, and muscle biopsy are normal. Temporal arteritis, an inflammatory disorder of medium- and large-sized arteries, usually involving one or more branches of the carotid artery, may accompany polymyalgia rheumatica. Vision is threatened by ischemic optic neuritis. Glucocorticoids can relieve the myalgias and protect against visual loss. Localized muscle pain is most often traumatic. A common cause of sudden abrupt-onset pain is a ruptured tendon, which leaves the muscle belly appearing rounded and shorter in appearance compared to the normal side. The biceps brachii and Achilles tendons are particularly vulnerable to rupture. Infection or neoplastic infiltration of the muscle is a rare cause of localized muscle pain. A muscle cramp or spasm is a painful, involuntary, localized, muscle contraction with a visible or palpable hardening of the muscle. Cramps are abrupt in onset, short in duration, and may cause abnormal posturing of the joint. The EMG shows firing of motor units, reflecting an origin from spontaneous neural discharge. Muscle cramps often occur in neurogenic disorders, especially motor neuron disease (Chap. 452), radiculopathies, and polyneuropathies (Chap. 459), but are not a feature of most primary muscle diseases. Duchenne muscular dystrophy is an exception because calf muscle complaints are a common complaint. Muscle cramps are also common during pregnancy. A muscle contracture is different from a muscle cramp. In both conditions, the muscle becomes hard, but a contracture is associated with energy failure in glycolytic disorders. The muscle is unable to relax after an active muscle contraction. The EMG shows electrical silence. Confusion is created because contracture also refers to a muscle that cannot be passively stretched to its proper length (fixed contracture) because of fibrosis. In some muscle disorders, especially in Emery-Dreifuss muscular dystrophy and Bethlem myopathy, fixed contractures occur early and represent distinctive features of the disease. Muscle stiffness can refer to different phenomena. Some patients with inflammation of joints and periarticular surfaces feel stiff. This condition is different from the disorders of hyperexcitable motor nerves causing stiff or rigid muscles. In stiff-person syndrome, spontaneous discharges of the motor neurons of the spinal cord cause involuntary muscle contractions mainly involving the axial (trunk) and proximal lower extremity muscles. The gait becomes stiff and labored, with hyperlordosis of the lumbar spine. Superimposed episodic muscle spasms are precipitated by sudden movements, unexpected noises, and emotional upset. The muscles relax during sleep. Serum antibodies against glutamic acid decarboxylase are present in approximately two-thirds of cases. In neuromyotonia (Isaacs’ syndrome), there is hyperexcitability of the peripheral nerves manifesting as continuous muscle fiber activity. Myokymia (groups of fasciculations associated with continuous undulations of muscle) and impaired muscle relaxation are the result. Muscles of the leg are stiff, and the constant contractions of the muscle cause increased sweating of the extremities. This peripheral nerve hyperexcitability is mediated by antibodies that target voltage-gated potassium channels. The site of origin of the spontaneous nerve discharges is principally in the distal portion of the motor nerves. Myotonia is a condition of prolonged muscle contraction followed by slow muscle relaxation. It always follows muscle activation (action myotonia), usually voluntary, but may be elicited by mechanical stimulation (percussion myotonia) of the muscle. Myotonia typically causes difficulty in releasing objects after a firm grasp. In myotonic muscular dystrophy type 1 (DM1), distal weakness usually accompanies myotonia, whereas in DM2, proximal muscles are more affected; thus the related term proximal myotonic myopathy (PROMM) is used to describe this condition. Myotonia also occurs with myotonia congenita (a chloride channel disorder), but in this condition muscle weakness is not prominent. Myotonia may also be seen in individuals with sodium channel mutations (hyperkalemic periodic paralysis or potassium-sensitive myotonia). Another sodium channelopathy, paramyotonia congenita, also is associated with muscle stiffness. In contrast to other disorders associated with myotonia in which the myotonia is eased by repetitive activity, paramyotonia congenita is named for a paradoxical phenomenon whereby the myotonia worsens with repetitive activity. Muscle Enlargement and Atrophy In most myopathies muscle tissue is replaced by fat and connective tissue, but the size of the muscle is usually not affected. However, in many limb-girdle muscular dystrophies (and particularly the dystrophinopathies), enlarged calf muscles are typical. The enlargement represents true muscle hypertrophy; thus the term pseudohypertrophy should be avoided when referring to these patients. The calf muscles remain very strong even late in the course of these disorders. Muscle enlargement can also result from infiltration by sarcoid granulomas, amyloid deposits, bacterial and parasitic infections, and focal myositis. In contrast, muscle atrophy is characteristic of other myopathies. In dysferlinopathies (LGMD2B) and anoctaminopathies (LGMD2L), there is a predilection for early atrophy of the gastrocnemius muscles, particularly the medial aspect. Atrophy of the humeral muscles is characteristic of FSHD. A limited battery of tests can be used to evaluate a suspected myopathy. Nearly all patients require serum enzyme level measurements and electrodiagnostic studies as screening tools to differentiate muscle disorders from other motor unit diseases. The other tests described—DNA studies, the forearm exercise test, and muscle biopsy—are used to diagnose specific types of myopathies. Serum Enzymes CK is the preferred muscle enzyme to measure in the evaluation of myopathies. Damage to muscle causes the CK to leak from the muscle fiber to the serum. The MM isoenzyme predominates in skeletal muscle, whereas creatine kinase-myocardial bound (CK-MB) is the marker for cardiac muscle. Serum CK can be elevated in normal individuals without provocation, presumably on a genetic basis or after strenuous activity, minor trauma (including the EMG needle), a prolonged muscle cramp, or a generalized seizure. Aspartate aminotransferase (AST), alanine aminotransferase (ALT), aldolase, and lactic dehydrogenase (LDH) are enzymes sharing an origin in both muscle and liver. Problems arise when the levels of these enzymes are found to be elevated in a routine screening battery, leading to the erroneous assumption that liver disease is present when in fact muscle could be the cause. An elevated γ-glutamyl transferase (GGT) helps to establish a liver origin because this enzyme is not found in muscle. Electrodiagnostic Studies EMG, repetitive nerve stimulation, and nerve conduction studies (Chap. 442e) are essential methods for evaluation of the patient with suspected muscle disease. In combination, they provide the information necessary to differentiate myopathies from neuropathies and neuromuscular junction diseases. Routine nerve conduction studies are typically normal in myopathies but reduced amplitudes of compound muscle action potentials may be seen in atrophied muscles. The needle EMG may reveal irritability on needle placement suggestive of a necrotizing myopathy (inflammatory myopathies, dystrophies, toxic myopathies, myotonic myopathies), whereas a lack of irritability is characteristic of long-standing myopathic disorders (muscular dystrophies, endocrine myopathies, disuse atrophy, and many of the metabolic myopathies). In addition, the EMG may demonstrate myotonic discharges that will narrow the differential diagnosis (Table 462e-4). Another important EMG finding is the presence of short-duration, small-amplitude, polyphasic motor unit action potentials (MUAPs). Such MUAPs can be seen in both myopathic and neuropathic disorders; however, the recruitment or firing pattern is different. In myopathies, the MUAPs fire early but at a normal rate to compensate for the loss of individual muscle fibers, whereas in neurogenic disorders the MUAPs fire faster. The EMG is usually normal in steroid or disuse myopathy, both of which are associated with type 2 fiber atrophy; this is because the EMG preferentially assesses the physiologic function of type 1 fibers. The EMG can also be invaluable in helping to choose an appropriately affected muscle to sample for biopsy. aAssociated with myotonic discharges on electromyography but no clinical myotonia. DNA Analysis This serves as an important tool for the definitive diagnosis of many muscle disorders. Nevertheless, there are a number of limitations in currently available molecular diagnostics. For example, in Duchenne and Becker dystrophies, two-thirds of patients have deletion or duplication mutations in the dystrophin gene that are easy to detect, while the remainder have point mutations that are much more difficult to find. For patients without identifiable gene defects, the muscle biopsy remains the main diagnostic tool. Forearm Exercise Test In myopathies with intermittent symptoms, and especially those associated with myoglobinuria, there may be a defect in glycolysis. Many variations of the forearm exercise test exist. For safety, the test should not be performed under ischemic conditions to avoid an unnecessary insult to the muscle, causing rhabdomyolysis. The test is performed by placing a small indwelling catheter into an antecubital vein. A baseline blood sample is obtained for lactic acid and ammonia. The forearm muscles are exercised by asking the patient to vigorously open and close the hand for 1 min. Blood is then obtained at intervals of 1, 2, 4, 6, and 10 min for comparison with the baseline sample. A three- to fourfold rise of lactic acid is typical. The simultaneous measurement of ammonia serves as a control, because it should also rise with exercise. In patients with myophosphorylase deficiency or other glycolytic defects, the lactic acid rise will be absent or below normal, while the rise in ammonia will reach control values. If there is lack of effort, neither lactic acid nor ammonia will rise. Patients with selective failure to increase ammonia may have myoadenylate deaminase deficiency. This condition has been reported to be a cause of myoglobinuria, but deficiency of this enzyme in asymptomatic individuals makes interpretation controversial. Muscle Biopsy Muscle biopsy is an important step in establishing the diagnosis of a suspected myopathy. The biopsy is usually obtained from a quadriceps or biceps brachii muscle, less commonly from a deltoid muscle. Evaluation includes a combination of techniques—light microscopy, histochemistry, immunocytochemistry with a battery of antibodies, and electron microscopy. Not all techniques are needed for every case. A specific diagnosis can be established in many disorders. Endomysial inflammatory cells surrounding and invading muscle fibers are seen in polymyositis; similar endomysial infiltrates associated with muscle fibers containing rimmed vacuoles and amyloid deposits consisting of SMI-31-, p62-, and TDP-43-positive inclusions within fibers are characteristic of inclusion body myositis; and perivascular, perimysial inflammation associated with perifascicular atrophy is a feature of dermatomyositis. In addition, the congenital myopathies have distinctive light and electron microscopy features essential for diagnosis. Mitochondrial and metabolic (e.g., glycogen and lipid storage diseases) myopathies also demonstrate distinctive histochemical and electron-microscopic profiles. Biopsied muscle tissue can be sent for metabolic enzyme or mitochondrial DNA analyses. A battery of antibodies is available for the identification of abnormal proteins to help diagnose specific types of muscular dystrophies. Western blot analysis on muscle specimens can be performed to determine whether specific muscle proteins are reduced in quantity or are of abnormal size. PROGRESSIVE MUSCULAR DYSTROPHIES AUTOSOMAL DOMINANT LIMB-GIRDLE MUSCULAR DYSTROPHIES (LGMDs) AUTOSOMAL RECESSIVE LIMB-GIRDLE MUSCULAR DYSTROPHIES (LGMDs) DUCHENNE MUSCULAR DYSTROPHY This X-linked recessive disorder, sometimes also called pseudohypertrophic muscular dystrophy, has an incidence of ~1 per 5200 live-born males. Clinical Features Duchenne dystrophy is present at birth, but the disorder usually becomes apparent between ages 3 and 5 years. The boys fall frequently and have difficulty keeping up with friends when playing. Running, jumping, and hopping are invariably abnormal. By age 5 years, muscle weakness is obvious by muscle testing. On getting up from the floor, the patient uses his hands to climb up himself (Gowers’ maneuver [Fig. 462e-4]). Contractures of the heel cords and iliotibial bands become apparent by age 6 years, when toe walking is associated with a lordotic posture. Loss of muscle strength is progressive, with predilection for proximal limb muscles and the neck flexors; leg involvement is more severe than arm involvement. Between ages 8 and 10 years, walking may require the use of braces; joint contractures and limitations of hip flexion, knee, elbow, and wrist extension are made worse by prolonged sitting. Prior to the use of glucocorticoids, most boys became wheelchair dependent by 12 years of age. Contractures become fixed, and a progressive scoliosis often develops that may be associated with pain. The chest deformity with scoliosis impairs pulmonary function, which is already diminished by muscle weakness. By age 16–18 years, patients are predisposed to serious, sometimes fatal pulmonary infections. Other causes of death include aspiration of food and acute gastric dilation. A cardiac cause of death is uncommon despite the presence of a cardiomyopathy in almost all patients. Congestive heart failure seldom occurs except with severe stress such as pneumonia. Cardiac arrhythmias are rare. The typical electrocardiogram (ECG) shows an increased net RS in lead V1; deep, narrow Q waves in the precordial leads; and tall right precordial R waves in V1. Intellectual impairment in Duchenne dystrophy is common; the average intelligence quotient (IQ) is ~1 standard deviation (SD) below the mean. Impairment of intellectual function appears to be nonprogressive and affects verbal ability more than performance. Laboratory Features Serum CK levels are invariably elevated to between 20 and 100 times normal. The levels are abnormal at birth but decline late in the disease because of inactivity and loss of muscle mass. EMG demonstrates features typical of myopathy. The muscle biopsy shows muscle fibers of varying size as well as small groups of necrotic and regenerating fibers. Connective tissue and fat replace lost muscle fibers. A definitive diagnosis of Duchenne dystrophy can be established on the basis of dystrophin deficiency in a biopsy of muscle tissue or mutation analysis on peripheral blood leukocytes, as discussed below. Duchenne dystrophy is caused by a mutation of the gene that encodes dystrophin, a 427-kDa protein localized to the inner surface of the sarcolemma of the muscle fiber. The dystrophin gene is >2000 kb in size and thus is one of the largest identified human genes. It is localized to the short arm of the × chromosome at Xp21. The most common gene mutation is a deletion. The size varies but does not correlate with disease severity. Deletions are not uniformly distributed over the gene but rather are most common near the beginning (5′ end) and middle of the gene. Less often, Duchenne dystrophy is caused by a gene duplication or point mutation. Identification of a specific mutation allows for an unequivocal diagnosis, makes possible accurate testing of potential carriers, and is useful for prenatal diagnosis. A diagnosis of Duchenne dystrophy can also be made by Western blot analysis of muscle biopsy specimens, revealing abnormalities on the quantity and molecular weight of dystrophin protein. In addition, immunocytochemical staining of muscle with dystrophin antibodies can be used to demonstrate absence or deficiency of dystrophin localizing to the sarcolemmal membrane. Carriers of the disease may demonstrate a mosaic pattern, but dystrophin analysis of muscle biopsy specimens for carrier detection is not reliable. Pathogenesis Dystrophin is part of a large complex of sarcolemmal proteins and glycoproteins (Fig. 462e-6). Dystrophin binds to F-actin at its amino terminus and to β-dystroglycan at the carboxyl terminus. β-Dystroglycan complexes to α-dystroglycan, which binds to laminin in the extracellular matrix (ECM). Laminin has a heterotrimeric molecular structure arranged in the shape of a cross with one heavy chain and two light chains, β1 and γ1. The laminin heavy chain of skeletal muscle is designated laminin α2. Collagen proteins IV and VI are also found in the ECM. Like β-dystroglycan, the transmembrane sarcoglycan proteins also bind to dystrophin; these five proteins (designated α- through ε-sarcoglycan) complex tightly with each other. More recently, other membrane proteins implicated in muscular dystrophy have been found to be loosely affiliated with constituents of the dystrophin complex. These include caveolin-3, α7 integrin, and collagen VI. FIGURE 462e-6 Selected muscular dystrophy–associated proteins in the cell membrane and Golgi complex. Dystrophin localizes to the cytoplasmic face of the muscle cell membrane. It complexes with two transmembrane protein complexes, the dystroglycans and the sarcoglycans. The dystroglycans bind to the extracellular matrix protein merosin, which is also complexed with β1 and α7 integrins (Tables 462e-5, 462e-6, and 462e-7). Dysferlin complexes with caveolin-3 (which binds to neuronal nitric oxide synthase, or nNOS) but not with the dystrophin-associated proteins or the integrins. In some of the congenital dystrophies and limb-girdle muscular dystrophies (LGMDs), there is loss of function of different enzymes that glycosylate α-dystroglycan, which thereby inhibits proper binding to merosin: POMT1, POMT2, POMGnT1, Fukutin, Fukutin-related protein, and LARGE. The dystrophin-glycoprotein complex appears to confer stability to the sarcolemma, although the function of each individual component of the complex is incompletely understood. Deficiency of one member of the complex may cause abnormalities in other components. For example, a primary deficiency of dystrophin (Duchenne dystrophy) may lead to secondary loss of the sarcoglycans and dystroglycan. The primary loss of a single sarcoglycan (see “Limb-Girdle Muscular Dystrophy,” below) results in a secondary loss of other sarcoglycans in the membrane without uniformly affecting dystrophin. In either instance, disruption of the dystrophin-glycoprotein complexes weakens the sarcolemma, causing membrane tears and a cascade of events leading to muscle fiber necrosis. This sequence of events occurs repeatedly during the life of a patient with muscular dystrophy. BECKER MUSCULAR DYSTROPHY This less severe form of X-linked recessive muscular dystrophy results from allelic defects of the same gene responsible for Duchenne dystrophy. Becker muscular dystrophy is ~10 times less frequent than Duchenne. Clinical Features The pattern of muscle wasting in Becker muscular dystrophy closely resembles that seen in Duchenne. Proximal muscles, especially of the lower extremities, are prominently involved. As the disease progresses, weakness becomes more generalized. Significant facial muscle weakness is not a feature. Hypertrophy of muscles, particularly in the calves, is an early and prominent finding. Most patients with Becker dystrophy first experience difficulties between ages 5 and 15 years, although onset in the third or fourth decade or even later can occur. By definition, patients with Becker dystrophy walk beyond age 15, whereas patients with Duchenne dystrophy are typically in a wheelchair by the age of 12. Patients with Becker dystrophy have a reduced life expectancy, but most survive into the fourth or fifth decade. Mental retardation may occur in Becker dystrophy, but it is not as common as in Duchenne. Cardiac involvement occurs in Becker dystrophy and may result in heart failure; some patients manifest with only heart failure. Other less common presentations are asymptomatic hyper-CK-emia, myalgias without weakness, and myoglobinuria. Laboratory Features Serum CK levels, results of EMG, and muscle biopsy findings closely resemble those in Duchenne dystrophy. The diagnosis of Becker muscular dystrophy requires Western blot analysis of muscle biopsy samples, demonstrating a reduced amount or abnormal size of dystrophin or mutation analysis of DNA from peripheral blood leukocytes. Genetic testing reveals deletions or duplications of the dystrophin gene in 65% of patients with Becker dystrophy, approximately the same percentage as in Duchenne dystrophy. In both Becker and Duchenne dystrophies, the size of the DNA deletion does not predict clinical severity; however, in ~95% of patients with Becker dystrophy, the DNA deletion does not alter the translational reading frame of messenger RNA. These “in-frame” mutations allow for production of some dystrophin, which accounts for the presence of altered rather than absent dystrophin on Western blot analysis. LIMB-GIRDLE MUSCULAR DYSTROPHY The syndrome of LGMD represents more than one disorder. Both males and females are affected, with onset ranging from late in the first decade to the fourth decade. The LGMDs typically manifest with progressive weakness of pelvic and shoulder girdle musculature. Respiratory insufficiency from weakness of the diaphragm may occur, as may cardiomyopathy. A systematic classification of LGMD is based on autosomal dominant (LGMD1) and autosomal recessive (LGMD2) inheritance. Superimposed on the backbone of LGMD1 and LGMD2, the classification uses a sequential alphabetical lettering system (LGMD1A, LGMD2A, etc.). Disorders receive letters in the order in which they are found to have chromosomal linkage. This results in an ever-expanding list of conditions summarized in Tables 462e-6 and 462e-7. None of the conditions is as common as the dystrophinopathies; however, prevalence data for the LGMDs have not been systematically gathered for any large heterogeneous population. In referral-based clinical populations, Fukutin-related protein (FKRP) deficiency (LGMD2I), calpainopathy (LGMD2A), anoctaminopathy (LGMD2L), and to a lesser extent dysferlinopathy (LGMD2B) have emerged as the most common disorders. There are at least five genetically distinct forms of Emery-Dreifuss muscular dystrophy (EDMD). Emerin mutations are the most common cause of X-linked EDMD, although mutations in FHL1 may also be associated with a similar phenotype, which is X-linked as well. Mutations involving the gene for lamin A/C are the most common cause of autosomal dominant EDMD (also known as LGMD1B) and are also a common cause of hereditary cardiomyopathy. Less commonly, autosomal dominant EDMD has been reported with mutations in nesprin-1, nesprin-2, and TMEM43. Clinical Features Prominent contractures can be recognized in early childhood and teenage years, often preceding muscle weakness. The contractures persist throughout the course of the disease and are present at the elbows, ankles, and neck. Muscle weakness affects humeral and peroneal muscles at first and later spreads to a limb-girdle distribution. The cardiomyopathy is potentially life threatening and may result in sudden death. A spectrum of atrial rhythm and conduction defects includes atrial fibrillation and paralysis and atrioventricular heart block. Some patients have a dilated cardiomyopathy. Female carriers of the X-linked variant may have cardiac manifestations that become clinically significant. Laboratory Features Serum CK may be elevated two- to tenfold. EMG is myopathic. Muscle biopsy usually shows nonspecific dystrophic features, although cases associated with FHL1 mutations have features of myofibrillar myopathy. Immunohistochemistry reveals absent emerin staining of myonuclei in X-linked EDMD due to emerin mutations. ECGs demonstrate atrial and atrioventricular rhythm disturbances. X-linked EDMD usually arises from defects in the emerin gene encoding a nuclear envelope protein. FHL1 mutations are also a cause of X-linked scapuloperoneal dystrophy, but can also present with an X-linked form of EDMD. The autosomal dominant disease can be caused by mutations in the LMNA gene encoding lamin A and C; in the synaptic nuclear envelope protein 1 (SYNE1) or 2 (SYNE2) encoding nesprin-1 and nesprin-2, respectively; and most recently in TMEM43 encoding LUMA. These proteins are essential components of the filamentous network underlying the inner nuclear membrane. Loss of structural integrity of the nuclear envelope from defects in emerin, lamin A/C, nesprin-1, nesprin-2, and LUMA accounts for overlapping phenotypes (Fig. 462e-7). FIGURE 462e-7 Selected muscular dystrophy–associated proteins in the nuclear membrane and sarcomere. As shown in the exploded view, emerin and lamin A/C are constituents of the inner nuclear membrane. Several dystrophy-associated proteins are represented in the sarcomere including titin, nebulin, calpain, telethonin, actinin, and myotilin. The position of the dystrophin-dystroglycan complex is also illustrated. CONGENITAL MUSCULAR DYSTROPHY (CMD) This is not one entity but rather a group of disorders with varying degrees of muscle weakness, CNS impairment, and eye abnormalities. Clinical Features As a group, CMDs present at birth or in the first few months of life with hypotonia and proximal or generalized muscle weakness. Calf muscle hypertrophy is seen in some patients. Facial muscles may be weak, but other cranial nerve–innervated muscles are spared (e.g., extraocular muscles are normal). Most patients have joint contractures of varying degrees at elbows, hips, knees, and ankles. Contractures present at birth are referred to as arthrogryposis. Respiratory failure may be seen in some cases. The CNS is affected in some forms of CMD. In merosin and FKRP deficiency, cerebral hypomyelination may be seen by magnetic resonance imaging (MRI), although only a small number of patients have mental retardation and seizures. Three forms of congenital muscular dystrophy have severe brain impairment. These include Fukuyama’s congenital muscular dystrophy (FCMD), muscle-eye-brain (MEB) disease, and Walker-Warburg syndrome (WWS). Patients are severely disabled in all three of these conditions. In MEB disease and WWS, but not in FCMD, ocular abnormalities impair vision. WWS is the most severe congenital muscular dystrophy, causing death by 1 year of age. Laboratory Features Serum CK is markedly elevated in all of these conditions. The EMG is myopathic and muscle biopsies show nonspecific dystrophic features. Merosin, or laminin α2 chain (a basal lamina protein), is deficient in surrounding muscle fibers in merosin deficiency. Skin biopsies can also demonstrate defects in laminin α2 chain. In the other disorders (FKRP deficiency, FCMD, MEB disease, WWS), there is abnormal α-dystroglycan staining in muscle. In merosin deficiency, cerebral hypomyelination is common, and a host of brain malformations are seen in FCMD, MEB disease, and WWS. All forms of CMD are inherited as autosomal recessive disorders. Chromosomal linkage and specific gene defects are presented in Table 462e-8. With the exception of merosin, the other gene defects affect posttranslational glycosylation of α-dystroglycan. This abnormality is thought to impair binding with merosin and leads to weakening of the dystrophin-glycoprotein complex, instability of the muscle membrane, and/or abnormalities in muscle contraction. CMDs with brain and eye phenotypes probably involve defective glycosylation of additional proteins, accounting for the more extensive phenotypes. CONGENITAL MUSCULAR DYSTROPHIESa Myotonic dystrophy is also known as dystrophia myotonica (DM). The condition is composed of at least two clinical disorders with overlapping phenotypes and distinct molecular genetic defects: myotonic dystrophy type 1 (DM1), the classic disease originally described by Steinert, and myotonic dystrophy type 2 (DM2), also called proximal myotonic myopathy (PROMM). Clinical Features The clinical expression of DM1 varies widely and involves many systems other than muscle. Affected patients have a typical “hatchet-faced” appearance due to temporalis, masseter, and facial muscle atrophy and weakness. Frontal baldness is also characteristic of the disease. Neck muscles, including flexors and sternocleidomastoids, and distal limb muscles are involved early. Weakness of wrist extensors, finger extensors, and intrinsic hand muscles impairs function. Ankle dorsiflexor weakness may cause footdrop. Proximal muscles remain stronger throughout the course, although preferential atrophy and weakness of quadriceps muscles occur in many patients. Palatal, pharyngeal, and tongue involvement produce a dysarthric speech, nasal voice, and swallowing problems. Some patients have diaphragm and intercostal muscle weakness, resulting in respiratory insufficiency. Myotonia, which usually appears by age 5 years, is demonstrable by percussion of the thenar eminence, the tongue, and wrist extensor muscles. Myotonia causes a slow relaxation of hand grip after a forced voluntary closure. Advanced muscle wasting makes myotonia more difficult to detect. Cardiac disturbances occur commonly in patients with DM1. ECG abnormalities include first-degree heart block and more extensive conduction system involvement. Complete heart block and sudden death can occur. Congestive heart failure occurs infrequently but may result from cor pulmonale secondary to respiratory failure. Mitral valve prolapse also occurs commonly. Other associated features include intellectual impairment, hypersomnia, posterior subcapsular cataracts, gonadal atrophy, insulin resistance, and decreased esophageal and colonic motility. Congenital myotonic dystrophy is a more severe form of DM1 and occurs in ~25% of infants of affected mothers. It is characterized by severe facial and bulbar weakness, transient neonatal respiratory insufficiency, and mental retardation. DM2, or PROMM, has a distinct pattern of muscle weakness affecting mainly proximal muscles. Other features of the disease overlap with DM1, including cataracts, testicular atrophy, insulin resistance, constipation, hypersomnia, and cognitive defects. Cardiac conduction defects occur but are less common, and the hatchet face and frontal baldness are less consistent features. A very striking difference is the failure to clearly identify a congenital form of DM2. Laboratory Features The diagnosis of myotonic dystrophy can usually be made on the basis of clinical findings. Serum CK levels may be normal or mildly elevated. EMG evidence of myotonia is present in most cases of DM1 but may be more patchy in DM2. Muscle biopsy shows muscle atrophy, which selectively involves type 1 fibers in 50% of cases, and ringed fibers in DM1 but not in DM2. Typically, numerous internalized nuclei can be seen in individual muscle fibers as well as atrophic fibers with pyknotic nuclear clumps in both DM1 and DM2. Necrosis of muscle fibers and increased connective tissue, common in other muscular dystrophies, are less apparent in myotonic dystrophy. DM1 and DM2 are both autosomal dominant disorders. New mutations do not appear to contribute to the pool of affected individuals. DM1 is transmitted by an intronic mutation consisting of an unstable expansion of a CTG trinucleotide repeat in a serine-threonine protein kinase gene (named DMPK) on chromosome 19q13.3. An increase in the severity of the disease phenotype in successive generations (genetic anticipation) is accompanied by an increase in the number of trinucleotide repeats. A similar type of mutation has been identified in fragile × syndrome (Chap. 451e). The unstable triplet repeat in myotonic dystrophy can be used for prenatal diagnosis. Congenital disease occurs almost exclusively in infants born to affected mothers; it is possible that sperm with greatly expanded triplet repeats do not function well. DM2 is caused by a DNA expansion mutation consisting of a CCTG repeat in intron 1 of the ZNF9 gene located at chromosome 3q13.3-q24. The gene is believed to encode an RNA-binding protein expressed in many different tissues, including skeletal and cardiac muscle. The DNA expansions in DM1 and DM2 almost certainly impair muscle function by a toxic gain of function of the mutant mRNA. In both DM1 and DM2, the mutant RNA appears to form intranuclear inclusions composed of aberrant RNA. These RNA inclusions sequester RNA-binding proteins essential for proper splicing of a variety of other mRNAs. This leads to abnormal transcription of multiple proteins in a variety of tissues/organ systems, in turn causing the systemic manifestations of DM1 and DM2. FACIOSCAPULOHUMERAL (FSH) MUSCULAR DYSTROPHY This form of muscular dystrophy has a prevalence of ~1 in 20,000. There are two forms of FSHD that have similar pathogenesis, as will be discussed. Most patients have FSHD type 1 (95%), whereas approximately 5% have FSHD2. FSHD1 and FSHD2 are clinically and histopathologically identical. FSHD is not to be confused with the genetically distinct scapuloperoneal dystrophies. Clinical Features The condition typically has an onset in childhood or young adulthood. In most cases, facial weakness is the initial manifestation, appearing as an inability to smile, whistle, or fully close the eyes. Weakness of the shoulder girdles, rather than the facial muscles, usually brings the patient to medical attention. Loss of scapular stabilizer muscles makes arm elevation difficult. Scapular winging (Fig. 462e-3) becomes apparent with attempts at abduction and forward movement of the arms. Biceps and triceps muscles may be severely affected, with relative sparing of the deltoid muscles. Weakness is invariably worse for wrist extension than for wrist flexion, and weakness of the anterior compartment muscles of the legs may lead to footdrop. In most patients, the weakness remains restricted to facial, upper extremity, and distal lower extremity muscles. In 20% of patients, weakness progresses to involve the pelvic girdle muscles, and severe functional impairment and possible wheelchair dependency result. Characteristically, patients with FSHD do not have involvement of other organ systems, although labile hypertension is common, and there is an increased incidence of nerve deafness. Coats’ disease, a disorder consisting of telangiectasia, exudation, and retinal detachment, also occurs. Laboratory Features The serum CK level may be normal or mildly elevated. EMG usually indicates a myopathic pattern. The muscle biopsy shows nonspecific features of a myopathy. A prominent inflammatory infiltrate, which is often multifocal in distribution, is present in some biopsy samples. The cause or significance of this finding is unknown. An autosomal dominant inheritance pattern with almost complete penetrance has been established, but each family member should be examined for the presence of the disease, since ~30% of those affected are unaware of involvement. FSHD1 is associated with deletions of tandem 3.3-kb repeats at 4q35. The deletion reduces the number of repeats to a fragment of <35 kb in most patients. Within these repeats lies the DUX4 gene, which usually is not expressed. In patients with FSHD1 these deletions in the setting of a specific polymorphism leads to hypomethylation of the region and toxic expression of the DUX4 gene. In patients with FSHD2, there is no deletion, but a mutation in SMCHD1. Interestingly, in the setting of the same polymorphism, there again is seen hypomethylation of the region and the permissive expression of the DUX4 gene. In both FSHD1 and FSHD2, there is overexpression of the DUX4 transcript. This form of muscular dystrophy represents one of several disorders characterized by progressive external ophthalmoplegia, which consists of slowly progressive ptosis and limitation of eye movements with sparing of pupillary reactions for light and accommodation. Patients usually do not complain of diplopia, in contrast to patients having conditions with a more acute onset of ocular muscle weakness (e.g., myasthenia gravis). Clinical Features Oculopharyngeal muscular dystrophy has a late onset; it usually presents in the fourth to sixth decade with ptosis and/or dysphagia. The extraocular muscle impairment is less prominent in the early phase but may be severe later. The swallowing problem may become debilitating and result in pooling of secretions and repeated episodes of aspiration. Mild weakness of the neck and extremities also occurs. Laboratory Features The serum CK level may be two to three times normal. Myopathic EMG findings are typical. On biopsy, muscle fibers are found to contain rimmed vacuoles, which by electron microscopy are shown to contain membranous whorls, accumulation of glycogen, and other nonspecific debris related to lysosomes. A distinct feature of oculopharyngeal dystrophy is the presence of tubular filaments, 8.5 nm in diameter, in muscle cell nuclei. Oculopharyngeal dystrophy has an autosomal dominant inheritance pattern with complete penetrance. The incidence is high in French-Canadians and in Spanish-American families of the southwestern United States. Large kindreds of Italian and of eastern European Jewish descent have been reported. The molecular defect in oculopharyngeal muscular dystrophy is a subtle expansion of a modest polyalanine repeat tract in a poly-RNA-binding protein (PABP2) in muscle. A group of muscle diseases, the distal myopathies, are notable for their preferential distal distribution of muscle weakness in contrast to most muscle conditions associated with proximal weakness. The major distal myopathies are summarized in Table 462e-9. Clinical Features Welander’s, Udd’s, and Markesbery-Griggs type distal myopathies are all late-onset, dominantly inherited disorders of distal limb muscles, usually beginning after age 40 years. Welander’s distal myopathy preferentially involves the wrist and finger extensors, whereas the others are associated with anterior tibial weakness leading to progressive footdrop. Laing’s distal myopathy is also a dominantly inherited disorder heralded by tibial weakness; however, it is distinguished by onset in childhood or early adult life. Nonaka’s distal myopathy and Miyoshi’s myopathy are distinguished by autosomal recessive inheritance and onset in the late teens or twenties. Nonaka’s and Williams’ myopathy entails anterior tibial weakness, whereas Miyoshi’s myopathy is unique in that gastrocnemius muscles are preferentially affected at onset. Finally, the myofibrillar myopathies (MFMs) are a clinically and genetically heterogeneous group of disorders that can be associated with prominent distal weakness; they can be inherited in an autosomal dominant or recessive pattern. Of note, Markesbery-Griggs myopathy (caused by mutations in ZASP) and LGMD1B (caused by mutations in myotilin) are in fact subtypes of myofibrillar myopathy. Laboratory Features Serum CK level is particularly helpful in diagnosing Miyoshi’s myopathy because it is very elevated. In the other conditions, serum CK is only slightly increased. EMGs are myopathic. In the MFMs, myotonic or pseudomyotonic discharges are common. Muscle biopsy shows nonspecific dystrophic features and, with the exception of Laing’s and Miyoshi’s myopathies, often shows rimmed vacuoles. MFM is associated with the accumulation of dense inclusions, as well as amorphous material best seen on Gomori trichrome and myofibrillar disruption on electron microscopy. Immune staining sometimes demonstrates accumulation of desmin and other proteins in MFM, large deposits of myosin heavy chain in the subsarcolemmal region of type 1 muscle fibers in Laing’s myopathy, and reduced or absent dysferlin in Miyoshi’s myopathy. The affected genes and their gene products are listed in Table 462e-9. These rare disorders are distinguished from muscular dystrophies by the presence of specific histochemical and structural abnormalities in muscle. Although primarily disorders of infancy or childhood, three forms that may present in adulthood are described here: central core disease, nemaline (rod) myopathy, and centronuclear (myotubular) myopathy. Sarcotubular myopathy is caused by mutations in TRIM-32 and is identical to LGMD2H. Other types, such as minicore myopathy (multi-minicore disease), fingerprint body myopathy, and cap myopathy, are not discussed. CENTRAL CORE DISEASE Patients with central core disease may have decreased fetal movements and breech presentation. Hypotonia and delay in motor milestones, particularly in walking, are common. Later in childhood, patients develop problems with stair climbing, running, and getting up from the floor. On examination, there is mild facial, neck-flexor, and proximal-extremity muscle weakness. Legs are more affected than arms. Skeletal abnormalities include congenital hip dislocation, scoliosis, and pes cavus; clubbed feet also occur. Most cases are nonprogressive, but exceptions are well documented. Susceptibility to malignant hyperthermia must be considered as a potential risk factor for patients with central core disease. Recent series have demonstrated that many cases of late-onset axial myopathy in which patients manifest with bent spine (camptocormia) or neck extensor weakness (neck extensor myopathy) are caused by mutations in the ryanodine receptor gene (RYR1). This illustrates the interesting spectrum of RYR1 mutations. The serum CK level is usually normal. Needle EMG demonstrates a myopathic pattern. Muscle biopsy shows fibers with single or multiple central or eccentric discrete zones (cores) devoid of oxidative enzymes. Cores occur preferentially in type 1 fibers and represent poorly aligned sarcomeres associated with Z disk streaming. Autosomal dominant inheritance is characteristic; sporadic cases also occur. As alluded above, this myopathy is caused by point mutations of RYR1, encoding the calcium-release channel of the sarcoplasmic reticulum of skeletal muscle; mutations of this gene also account for some cases of inherited malignant hyperthermia (Chap. 23). Malignant hyperthermia is an allelic condition; C-terminal mutations of the RYR1 gene predispose to this complication. Specific treatment is not required, but establishing a diagnosis of central core disease is extremely important because these patients have a known predisposition to malignant hyperthermia during anesthesia. The term nemaline refers to the distinctive presence in muscle fibers of rods or threadlike structures (Greek nema, “thread”). Nemaline myopathy is clinically heterogeneous. A severe neonatal form presents with hypotonia and feeding and respiratory difficulties, leading to early death. Nemaline myopathy usually presents in infancy or childhood with delayed motor milestones. The course is nonprogressive or slowly progressive. The physical appearance is striking because of the long, narrow facies, high-arched palate, and open-mouthed appearance due to a prognathous jaw. Other skeletal abnormalities include pectus excavatum, kyphoscoliosis, pes cavus, and clubfoot deformities. Facial and generalized muscle weakness, including respiratory muscle weakness, is common. An adult-onset disorder with progressive proximal or distal weakness may be seen. Myocardial involvement is occasionally present in both the childhood and adult-onset forms. The serum CK level is usually normal or slightly elevated. The EMG demonstrates a myopathic pattern. Muscle biopsy shows clusters of small rods (nemaline bodies), which occur preferentially, but not exclusively, in the sarcoplasm of type 1 muscle fibers. Occasionally, the rods are also apparent in myonuclei. The muscle often shows type 1 muscle fiber predominance. Rods originate from the Z disk material of the muscle fiber. Six genes have been associated with nemaline myopathy. Five of these code for thin filament–associated proteins, suggesting disturbed assembly or interplay of these structures as a pivotal mechanism. Mutations of the nebulin (NEB) gene account for most cases, including both severe neonatal and early childhood forms, inherited as autosomal recessive disorders. Neonatal and childhood cases, inherited as predominantly autosomal dominant disorders, are caused by mutations of the skeletal muscle a-actinin (ACTA1) gene. In milder forms of the disease with autosomal dominant inheritance, mutations have been identified in both the slow a-tropomyosin (TPM3) and β;-tropomyosin (TPM2) genes accounting for <3% of cases. Muscle troponin T (TNNT1) gene mutations appear to be limited to the Amish population in North America. Mutations may also be seen in NEM6 that encodes a putative BTB/Kelch protein. No specific treatment is available. CENTRONUCLEAR (MYOTUBULAR) MYOPATHY Three distinct variants of centronuclear myopathy occur. A neonatal form, also known as myotubular myopathy, presents with severe hypotonia and weakness at birth. The late infancy–early childhood form presents with delayed motor milestones. Later, difficulty with running and stair climbing becomes apparent. A marfanoid, slender body habitus, long narrow face, and high-arched palate are typical. Scoliosis and clubbed feet may be present. Most patients exhibit progressive weakness, some requiring wheelchairs. Progressive external ophthalmoplegia with ptosis and varying degrees of extraocular muscle impairment are characteristic of both the neonatal and the late-infantile forms. A third variant, the late childhood–adult form, has an onset in the second or third decade. Patients have full extraocular muscle movements and rarely exhibit ptosis. There is mild, slowly progressive limb weakness that may be distally predominant (some of these patients have been classified as having Charcot-Marie-Tooth disease type 2 [CMT2; Chap. 459]). Normal or slightly elevated CK levels occur in each of the forms. Nerve conduction studies may reveal reduced amplitudes of distal compound muscle action potentials, in particular in adult-onset cases that resemble CMT2. EMG studies often give distinctive results, showing positive sharp waves and fibrillation potentials, complex and repetitive discharges, and rarely myotonic discharges. Muscle biopsy specimens in longitudinal section demonstrate rows of central nuclei, often surrounded by a halo. In transverse sections, central nuclei are found in 25–80% of muscle fibers. A gene for the neonatal form of centronuclear myopathy has been localized to Xq28; this gene encodes myotubularin, a protein tyrosine phosphatase. Missense, frameshift, and splice-site mutations predict loss of myotubularin function in affected individuals. Carrier identification and prenatal diagnosis are possible. Autosomal recessive forms are caused by mutations in BIB1 that encodes for amphyphysin-2, whereas some autosomal dominant cases, which are allelic to a form of CMT2, are associated with mutations in the gene that encodes dynamin-2. No specific medical treatments are available at this time. DISORDERS OF MUSCLE ENERGY METABOLISM There are two principal sources of energy for skeletal muscle—fatty acids and glucose. Abnormalities in either glucose or lipid utilization can be associated with distinct clinical presentations that can range from an acute, painful syndrome with rhabdomyolysis and myoglobinuria to a chronic, progressive muscle weakness simulating muscular dystrophy. GLYCOGEN STORAGE AND GLYCOLYTIC DEFECTS Disorders of Glycogen Storage Causing Progressive Weakness • α-GLUCOSIDASE, OR ACID MALTASE, DEFICIENCY (POMPE’S DISEASE) Three clinical forms of α-glucosidase, or acid maltase, deficiency (type II glycogenosis) can be distinguished. The infantile form is the most common, with onset of symptoms in the first 3 months of life. Infants develop severe muscle weakness, cardiomegaly, hepatomegaly, and respiratory insufficiency. Glycogen accumulation in motor neurons of the spinal cord and brainstem contributes to muscle weakness. Death usually occurs by 1 year of age. In the childhood form, the picture resembles muscular dystrophy. Delayed motor milestones result from proximal limb muscle weakness and involvement of respiratory muscles. The heart may be involved, but the liver and brain are unaffected. The adult form usually begins in the third or fourth decade but can present as late as the seventh decade. Respiratory failure and diaphragmatic weakness are often initial manifestations, heralding progressive proximal muscle weakness. The heart and liver are not involved. The serum CK level is 2–10 times normal in infantile or childhood-onset Pompe’s disease but can be normal in adult-onset cases. EMG examination demonstrates a myopathic pattern, but other features are especially distinctive, including myotonic discharges, trains of fibrillation and positive waves, and complex repetitive discharges. EMG discharges are very prominent in the paraspinal muscles. The muscle biopsy in infants typically reveals vacuoles containing glycogen and the lysosomal enzyme acid phosphatase. Electron microscopy reveals membrane-bound and free tissue glycogen. However, muscle biopsies in late-onset Pompe’s disease may demonstrate only nonspecific abnormalities. Enzyme analysis of dried blood spots is a sensitive technique to screen for Pompe’s disease. A definitive diagnosis is established by enzyme assay in muscle or cultured fibroblasts or by genetic testing. Pompe’s disease is inherited as an autosomal recessive disorder caused by mutations of the α-glucosidase gene. Enzyme replacement therapy (ERT) with IV recombinant human α-glucosidase has been shown to be beneficial in infantile-onset Pompe’s disease. Clinical benefits in the infantile disease include reduced heart size, improved muscle function, reduced need for ventilatory support, and longer life. In late-onset cases, ERT has not been associated with the dramatic response that can be seen in classic infantile Pompe’s disease, yet it appears to stabilize the disease process. OTHER GLYCOGEN STORAGE DISEASES WITH PROGRESSIVE WEAKNESS In de-branching enzyme deficiency (type III glycogenosis), a slowly progressive form of muscle weakness can develop after puberty. Rarely, myoglobinuria may be seen. Patients are usually diagnosed in infancy, however, because of hypotonia and delayed motor milestones, hepatomegaly, growth retardation, and hypoglycemia. Branching enzyme deficiency (type IV glycogenosis) is a rare and fatal glycogen storage disease characterized by failure to thrive and hepatomegaly. Hypotonia and muscle wasting may be present, but the skeletal muscle manifestations are minor compared to liver failure. Disorders of Glycolysis Causing Exercise Intolerance Several glycolytic defects are associated with recurrent myoglobinuria: myophosphorylase deficiency (type V glycogenosis), phosphofructokinase deficiency (type VII glycogenosis), phosphoglycerate kinase deficiency, phosphorylase kinase deficiency (type IX glycogenosis), phosphoglycerate mutase deficiency (type × glycogenosis), lactate dehydrogenase deficiency (glycogenosis type XI), and β-enolase deficiency. Myophosphorylase deficiency, also known as McArdle’s disease, is by far the most common of the glycolytic defects associated with exercise intolerance. These glycolytic defects result in a common failure to support energy production at the initiation of exercise, although the exact site of energy failure remains controversial. Clinical muscle manifestations in these conditions usually begin in adolescence. Symptoms are precipitated by brief bursts of high-intensity exercise such as running or lifting heavy objects. A history of myalgia and muscle stiffness usually precedes the intensely painful muscle contractures, which may be followed by myoglobinuria. Acute renal failure accompanies significant pigmenturia. Certain features help distinguish some enzyme defects. In McArdle’s disease, exercise tolerance can be enhanced by a slow induction phase (warm-up) or brief periods of rest, allowing for the start of the “second-wind” phenomenon (switching to utilization of fatty acids). Varying degrees of hemolytic anemia accompany deficiencies of both phosphofructokinase (mild) and phosphoglycerate kinase (severe). In phosphoglycerate kinase deficiency, the usual clinical presentation is a seizure disorder associated with mental retardation; exercise intolerance is an infrequent manifestation. In all of these conditions, the serum CK levels fluctuate widely and may be elevated even during symptom-free periods. CK levels >100 times normal are expected, accompanying myoglobinuria. All patients with suspected glycolytic defects leading to exercise intolerance should undergo a forearm exercise test. An impaired rise in venous lactate is highly indicative of a glycolytic defect. In lactate dehydrogenase deficiency, venous levels of lactate do not increase, but pyruvate rises to normal. A definitive diagnosis of glycolytic disease is made by muscle biopsy and subsequent enzyme analysis or by genetic testing. Myophosphorylase deficiency, phosphofructokinase deficiency, and phosphoglycerate mutase deficiency are inherited as autosomal recessive disorders. Phosphoglycerate kinase deficiency is X-linked recessive. Mutations can be found in the respective genes encoding the abnormal proteins in each of these disorders. Training may enhance exercise tolerance, perhaps by increasing perfusion to muscle. Dietary intake of free glucose or fructose prior to activity may improve function but care must be taken to avoid obesity from ingesting too many calories. LIPID AS AN ENERGY SOURCE AND ASSOCIATED DEFECTS Lipid is an important muscle energy source during rest and during prolonged, submaximal exercise. Fatty acids are derived from circulating very-low-density lipoprotein (VLDL) in the blood or from triglycerides stored in muscle fibers. Oxidation of fatty acids occurs in the mitochondria. To enter the mitochondria, a fatty acid must first be converted to an “activated fatty acid,” acyl-CoA. The acyl-CoA must be linked with carnitine by the enzyme carnitine palmitoyltransferase (CPT) I for transport into the mitochondria. CPT I is present on the inner side of the outer mitochondrial membrane. Carnitine is removed by CPT II, an enzyme attached to the inside of the inner mitochondrial membrane, allowing transport of acyl-CoA into the mitochondrial matrix for β-oxidation. Carnitine Palmitoyltransferase Deficiency CPT II deficiency is the most common recognizable cause of recurrent myoglobinuria, more common than the glycolytic defects. Onset is usually in the teenage years or early twenties. Muscle pain and myoglobinuria typically occur after prolonged exercise but can also be precipitated by fasting or infections; up to 20% of patients do not exhibit myoglobinuria, however. Strength is normal between attacks. In contrast to disorders caused by defects in glycolysis, in which muscle cramps follow short, intense bursts of exercise, the muscle pain in CPT II deficiency does not occur until the limits of utilization have been exceeded and muscle breakdown has already begun. Episodes of rhabdomyolysis may produce severe weakness. In young children and newborns, CPT II deficiency can present with a very severe clinical picture including hypoketotic hypoglycemia, cardiomyopathy, liver failure, and sudden death. Serum CK levels and EMG findings are both usually normal between episodes. A normal rise of venous lactate during forearm exercise distinguishes this condition from glycolytic defects, especially myophosphorylase deficiency. Muscle biopsy does not show lipid accumulation and is usually normal between attacks. The diagnosis requires direct measurement of muscle CPT or genetic testing. CPT II deficiency is much more common in men than women (5:1); nevertheless, all evidence indicates autosomal recessive inheritance. A mutation in the gene for CPT II (chromosome 1p36) causes the disease in some individuals. Attempts to improve exercise tolerance with frequent meals and a low-fat, high-carbohydrate diet, or by substituting medium-chain triglycerides in the diet, have not proven to be beneficial. Myoadenylate Deaminase Deficiency The muscle enzyme myoadenylate deaminase converts adenosine-5′-monophosphate (5′-AMP) to inosine monophosphate (IMP) with liberation of ammonia. Myoadenylate deaminase may play a role in regulating adenosine triphosphate (ATP) levels in muscles. Most individuals with myoadenylate deaminase deficiency have no symptoms. There have been a few reports of patients with this disorder who have exercise-exacerbated myalgia and myoglobinuria. Many questions have been raised about the clinical effects of myoadenylate deaminase deficiency, and, specifically, its relationship to exertional myalgia and fatigability, but there is no consensus. In 1972, Olson and colleagues recognized that muscle fibers with significant numbers of abnormal mitochondria could be highlighted with the modified trichrome stain; the term ragged red fibers was coined. By electron microscopy, the mitochondria in ragged red fibers are enlarged and often bizarrely shaped and have crystalline inclusions. Since that seminal observation, the understanding of these disorders of muscle and other tissues has expanded (Chap. 82). Mitochondria play a key role in energy production. Oxidation of the major nutrients derived from carbohydrate, fat, and protein leads to the generation of reducing equivalents. The latter are transported through the respiratory chain in the process known as oxidative phosphorylation. The energy generated by the oxidation-reduction reactions of the respiratory chain is stored in an electrochemical gradient coupled to ATP synthesis. A novel feature of mitochondria is their genetic composition. Each mitochondrion possesses a DNA genome that is distinct from that of the nuclear DNA. Human mitochondrial DNA (mtDNA) consists of a double-strand, circular molecule comprising 16,569 base pairs. It codes for 22 transfer RNAs, 2 ribosomal RNAs, and 13 polypeptides of the respiratory chain enzymes. The genetics of mitochondrial diseases differ from the genetics of chromosomal disorders. The DNA of mitochondria is directly inherited from the cytoplasm of the gametes, mainly from the oocyte. The sperm contributes very little of its mitochondria to the offspring at the time of fertilization. Thus, mitochondrial genes are derived almost exclusively from the mother, accounting for maternal inheritance of some mitochondrial disorders. Patients with mitochondrial myopathies have clinical manifestations that usually fall into three groups: chronic progressive external ophthalmoplegia (CPEO), skeletal muscle–CNS syndromes, and pure myopathy simulating muscular dystrophy or metabolic myopathy. PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA SYNDROMES WITH RAGGED RED FIBERS The single most common sign of a mitochondrial myopathy is CPEO, occurring in >50% of all mitochondrial myopathies. Varying degrees of ptosis and weakness of extraocular muscles are seen, usually in the absence of diplopia, a point of distinction from disorders with fluctuating eye weakness (e.g., myasthenia gravis). KEARNS-SAYRE SYNDROME (KSS) KSS is a widespread multiorgan system disorder with a defined triad of clinical findings: onset before age 20, CPEO, and pigmentary retinopathy, plus one or more of the following features: complete heart block, cerebrospinal fluid (CSF) protein >1 g/L (100 mg/dL), or cerebellar ataxia. Some patients with CPEO and ragged red fibers may not fulfill all of the criteria for KSS. The cardiac disease includes syncopal attacks and cardiac arrest related to the abnormalities in the cardiac conduction system: prolonged intraventricular conduction time, bundle branch block, and complete atrioventricular block. Death attributed to heart block occurs in ~20% of the patients. Varying degrees of progressive limb muscle weakness and easy fatigability affect activities of daily living. Endocrine abnormalities are common, including gonadal dysfunction in both sexes with delayed puberty, short stature, and infertility. Diabetes mellitus is a cardinal sign of mitochondrial disorders and is estimated to occur in 13% of KSS patients. Other less common endocrine disorders include thyroid disease, hyperaldosteronism, Addison’s disease, and hypoparathyroidism. Both mental retardation and dementia are common accompaniments to this disorder. Serum CK levels are normal or slightly elevated. Serum lactate and pyruvate levels may be elevated. EMG is myopathic. Nerve conduction studies may be abnormal related to an associated neuropathy. Muscle biopsies reveal ragged red fibers, highlighted in oxidative enzyme stains, many showing defects in cytochrome oxidase. By electron microscopy, there are increased numbers of mitochondria that often appear enlarged and contain paracrystalline inclusions. KSS is a sporadic disorder. The disease is caused by single mtDNA deletions presumed to arise spontaneously in the ovum or zygote. The most common deletion, occurring in about one-third of patients, removes 4977 bp of contiguous mtDNA. Monitoring for cardiac conduction defects is critical. Prophylactic pacemaker implantation is indicated when ECGs demonstrate a bifascicular block. In KSS, no benefit has been shown for supplementary therapies, including multivitamins or coenzyme Q10. Of all the proposed options, exercise might be the most applicable but must be approached cautiously because of defects in the cardiac conduction system. PROGRESSIVE EXTERNAL OPHTHALMOPLEGIA (PEO) This condition is caused by nuclear DNA mutations affecting mtDNA copy number and integrity and is thus inherited in a Mendelian fashion. Onset is usually after puberty. Fatigue, exercise intolerance, and complaints of muscle weakness are typical. Some patients notice swallowing problems. The neurologic examination confirms the ptosis and ophthalmoplegia, usually asymmetric in distribution. A sensorineural hearing loss may be encountered. Mild facial, neck flexor, and proximal weakness are typical. Rarely, respiratory muscles may be progressively affected and may be the direct cause of death. Serum CK is normal or mildly elevated. The resting lactate level is normal or slightly elevated but may rise excessively after exercise. CSF protein is normal. The EMG is myopathic, and nerve conduction studies are usually normal. Ragged red fibers are prominently displayed in the muscle biopsy. Southern blots of muscle reveal a normal mtDNA band at 16.6 kb and several additional mtDNA deletion bands with genomes varying from 0.5 to 10 kb. This autosomal dominant form of CPEO has been linked to loci on three chromosomes: 4q35, 10q24, and 15q22–26. In the chromosome 4q-related form of disease, mutations of the gene encoding the heart and skeletal muscle–specific isoform of the adenine nucleotide translocator 1 (ANT1) gene are found. This highly abundant mitochondrial protein forms a homodimeric inner mitochondrial channel through which adenosine diphosphate (ADP) enters and ATP leaves the mitochondrial matrix. In the chromosome 10q–related disorder, mutations of the gene C10orf2 are found. Its gene product, twinkle, co-localizes with the mtDNA and is named for its punctate, starlike staining properties. The function of twinkle is presumed to be critical for lifetime maintenance of mitochondrial integrity. In the cases mapped to chromosome 15q, a mutation affects the gene encoding mtDNA polymerase (POLG), an enzyme important in mtDNA replication. Autosomal recessive PEO has also been described with mutations in the POLG gene. Point mutations have been identified within various mitochondrial tRNA (Leu, Ile, Asn, Trp) genes in families with maternal inheritance of PEO. Exercise may improve function but will depend on the patient’s ability to participate. MITOCHONDRIAL DNA SKELETAL MUSCLE–CENTRAL NERVOUS SYSTEM SYNDROMES Myoclonic Epilepsy with Ragged Red Fibers (MERRF) The onset of MERRF is variable, ranging from late childhood to middle adult life. Characteristic features include myoclonic epilepsy, cerebellar ataxia, and progressive muscle weakness. The seizure disorder is an integral part of the disease and may be the initial symptom. Cerebellar ataxia precedes or accompanies epilepsy. It is slowly progressive and generalized. The third major feature of the disease is muscle weakness in a limb-girdle distribution. Other more variable features include dementia, peripheral neuropathy, optic atrophy, hearing loss, and diabetes mellitus. Serum CK levels are normal or slightly increased. The serum lactate may be elevated. EMG is myopathic, and in some patients nerve conduction studies show a neuropathy. The electroencephalogram is abnormal, corroborating clinical findings of epilepsy. Typical ragged red fibers are seen on muscle biopsy. MERRF is caused by maternally inherited point mutations of mitochondrial tRNA genes. The most common mutation found in 80% of MERRF patients is an A to G substitution at nucleotide 8344 of tRNA lysine (A8344G tRNAlys). Other tRNAlys mutations include base-pair substitutions T8356C and G8363A. Only supportive treatment is possible, with special attention to epilepsy. Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Strokelike Episodes (MELAS) MELAS is the most common mitochondrial encephalomyopathy. The term strokelike is appropriate because the cerebral lesions do not conform to a strictly vascular distribution. The onset in the majority of patients is before age 20. Seizures, usually partial motor or generalized, are common and may represent the first clearly recognizable sign of disease. The cerebral insults that resemble strokes cause hemiparesis, hemianopia, and cortical blindness. A presumptive stroke occurring before age 40 should place this mitochondrial encephalomyopathy high in the differential diagnosis. Associated conditions include hearing loss, diabetes mellitus, hypothalamic pituitary dysfunction causing growth hormone deficiency, hypothyroidism, and absence of secondary sexual characteristics. In its full expression, MELAS leads to dementia, a bedridden state, and a fatal outcome. Serum lactic acid is typically elevated. The CSF protein is also increased but is usually ≤1 g/L (100 mg/dL). Muscle biopsies show ragged red fibers. Neuroimaging demonstrates basal ganglia calcification in a high percentage of cases. Focal lesions that mimic infarction are present predominantly in the occipital and parietal lobes. Strict vascular territories are not respected, and cerebral angiography fails to demonstrate lesions of the major cerebral blood vessels. MELAS is caused by maternally inherited point mutations of mitochondrial tRNA genes. Most of the tRNA mutations are lethal, accounting for the paucity of multigeneration families with this syndrome. The A3243G point mutation in tRNALeu(UUR) is the most common, occurring in ~80% of MELAS cases. About 10% of MELAS patients have other mutations of the tRNALeu(UUR) gene, including 3252G, 3256T, 3271C, and 3291C. Other tRNA gene mutations have also been reported in MELAS, including G583A tRNAPhe, G1642A tRNAVal, G4332A tRNAGlu, and T8316C tRNALys. Mutations have also been reported in mtDNA polypeptide-coding genes. Two mutations were found in the ND5 subunit of complex I of the respiratory chain. A missense mutation has been reported at mtDNA position 9957 in the gene for subunit III of cytochrome C oxidase. No specific treatment is available. Supportive treatment is essential for the strokelike episodes, seizures, and endocrinopathies. PURE MYOPATHY SYNDROMES Muscle weakness and fatigue can be the predominant manifestations of mtDNA mutations. When the condition affects exclusively muscle (pure myopathy), the disorder becomes difficult to recognize. Occasionally, mitochondrial myopathies can present with recurrent myoglobinuria without fixed weakness and thus resemble a glycogen storage disorder or CPT deficiency. Mitochondrial DNA Depletion Syndromes Mitochondrial DNA depletion syndrome (MDS) is a heterogeneous group of disorders that are inherited in an autosomal recessive fashion and can present in infancy or adults. MDS can be caused by mutations in genes (TK2, DGUOK, RRM2B, TYMP, SUCLA1, and SUCLA2) that lead to depletion of pools of mitochondrial deoxyribonucleotide (dNTP) pools necessary for mtDNA replication The other major cause of MDS is a set of mutations in genes essential for mtDNA replication (e.g., POLG1 and C10orf2). The clinical phenotypes associated with MDS vary. Patients may develop a severe encephalopathy (e.g., Leigh’s syndrome), PEO, an isolated myopathy, myo-neuro-gastrointestinal-encephalopathy (MNGIE), and a sensory neuropathy with ataxia. DISORDERS OF MUSCLE MEMBRANE EXCITABILITY Muscle membrane excitability is affected in a group of disorders referred to as channelopathies. The heart may also be involved, resulting in life-threatening complications (Table 462e-10). CLINICAL FEATURES OF PERIODIC PARALYSIS AND NONDYSTROPHIC MYOTONIAS CALCIUM CHANNEL DISORDERS OF MUSCLE Hypokalemic Periodic Paralysis (HypoKPP) Onset occurs at adolescence. Men are more often affected because of decreased penetrance in women. Episodic weakness with onset after age 25 is almost never due to periodic paralyses, with the exception of thyrotoxic periodic paralysis (see below). Attacks are often provoked by meals high in carbohydrates or sodium and may accompany rest following prolonged exercise. Weakness usually affects proximal limb muscles more than distal. Ocular and bulbar muscles are less likely to be affected. Respiratory muscles are usually spared, but when they are involved, the condition may prove fatal. Weakness may take as long as 24 h to resolve. Life-threatening cardiac arrhythmias related to hypokalemia may occur during attacks. As a late complication, patients commonly develop severe, disabling proximal lower extremity weakness. Attacks of thyrotoxic periodic paralysis resemble those of primary HypoKPP. Despite a higher incidence of thyrotoxicosis in women, men, particularly those of Asian descent, are more likely to manifest this complication. Attacks abate with treatment of the underlying thyroid condition. A low serum potassium level during an attack, excluding secondary causes, establishes the diagnosis. Interattack muscle biopsies show the presence of single or multiple centrally placed vacuoles or tubular aggregates. Provocative tests with glucose and insulin to establish a diagnosis are usually not necessary and are potentially hazardous. In the midst of an attack of weakness, motor conduction studies may demonstrate reduced amplitudes, whereas EMG may show electrical silence in severely weak muscles. In between attacks, the EMG and routine nerve conduction studies are normal. However, a long exercise test may demonstrate a decrementing amplitude, and myopathic MUAPs may be seen on EMG in patients with fixed weakness. HypoKPP is caused by mutations in either of two genes. HypoKPP type 1, the most common form, is inherited as an autosomal dominant disorder with incomplete penetrance. These patients have mutations in the voltage-sensitive, skeletal muscle calcium channel gene, CALCL1A3 (Fig. 462e-8). Approximately 10% of cases are HypoKPP type 2, arising from mutations in the voltage-sensitive sodium channel gene (SCN4A). In either instance, the mutations lead to an abnormal gating pore current that predisposes the muscle cell to depolarize when potassium levels are low. It is also now recognized that some cases of thyrotoxic HypoKPP are caused by genetic variants in a potassium channel (Kir 2.6), whose expression is regulated by thyroid hormone. FIGURE 462e-8 The sodium and calcium channels are depicted here as containing four homologous domains, each with six membrane-spanning segments. The fourth segment of each domain bears positive charges and acts as the “voltage sensor” for the channel. The association of the four domains is thought to form a pore through which ions pass. Sodium channel mutations are shown along with the phenotype that they confer. HyperKPP, hyperkalemic periodic paralysis; PC, paramyotonia congenita; PAM, potassium-aggravated myotonia. See text for details. The chloride channel is envisioned to have 10 membrane-spanning domains. The positions of mutations causing dominantly and recessively inherited myotonia congenita are indicated, along with mutations that cause this disease in mice and goats.	2	16	0	0	0	1	0.939334	1	17,920
The Patient with a Dianne Smallidge, RDH, MDH and Esther M. Wilkins, BS, RDH, DMD III. Disease Process CONGENITAL HEART DISEASES I. The Normal Healthy Heart IV. Types of Defects VI. Clinical Considerations RHEUMATIC HEART DISEASE I. Rheumatic Fever II. The Course of Rheumatic Heart Disease MITRAL VALVE PROLAPSE II. Blood Pressure Levels III. Clinical Symptoms of Hypertension V. Hypertension in Children ISCHEMIC HEART DISEASE II. Manifestations of Ischemic Heart Disease I. Precipitating Factors III. Procedure During an Angina Attack III. Management During an Attack IV. Treatment After Acute Symptoms II. Clinical Manifestations III. Treatment During Chronic Stages IV. Emergency Care for Heart Failure and Acute Pul- LIFESTYLE MANAGEMENT FOR THE PATIENT WITH CARDIOVASCULAR DISEASE I. Coronary Dilation II. Coronary Bypass III. Cardiac Pacemaker and Implantable Cardioverter I. Clinical Procedures II. Postprocedural Instructions FACTORS TO TEACH THE PATIENT After studying this chapter, the student will be able to: 1. Identify the cardiovascular conditions that may be encountered in patients seeking oral health care. 2. Discuss the etiology, symptoms and risk factors associated with cardiovascular conditions. 3. Discuss the impact of cardiovascular diseases on the oral cavity and their relationship to oral health. 4. Plan dental hygiene treatment modifications for the patient with cardiovascular disease. Wilkins9781451193114-ch067.indd 1129 07/10/15 11:39 AM 1130 SECTION IX \| Patients with Special Needs Cardiovascular includes diseases of the heart and blood ▶ Patients with cardiovascular conditions are encoun- tered frequently in a dental office or clinic and may be from any age group, although the highest incidence is among older people. ▶ Although a causal relationship between periodontal disease and coronary heart disease (CHD) has not been proven, current data suggest the presence of periodon- tal disease may be a marker for CHD risk.¹ ▶ Dental hygienists need to take responsibility to inform patients of the significant relationship between oral and systemic health and the related need for maintenance of healthy oral tissues and prevention of periodontal disease. ▶ The major cardiovascular diseases are included in this chapter with their principle symptoms and treatments as well as applications for dental hygiene care. ▶ Key words and terminology are defined in Box 67-1. Prefixes and suffixes to clarify the terminology are listed in Appendix VII. Aneurysm: sac formed by the localized dilatation of the wall of an artery, a vein, or the heart. Angina: a condition marked by spasmodic suffocative attacks. Angina pectoris: acute pain in the chest from decreased blood supply to the heart muscle. Anoxia: absence of oxygen in the tissues; may be accompa- nied by deep respirations, cyanosis, increased pulse rate, and impairment of coordination. Anticoagulant: a substance that suppresses, delays, or nulli- fies coagulation of the blood. Apnea: temporary cessation of breathing. Arrhythmia: variation from the normal rhythm, especially with reference to the heart. Arthralgia: joint pain. Arterial blood: oxygenated blood carried by an artery away from the heart to nourish the body tissues. Arteriosclerosis: group of diseases characterized by thicken- ing and loss of elasticity of the arterial wall. Asphyxia: a condition in which there is a deficiency of oxy- gen in the blood and an increase in carbon dioxide. Atheroma: lipid (cholesterol) deposit on the intima (lining) of an artery; also called atheromatous plaque. Atherosclerosis: disease process caused by the deposit of atheromas on the inner lining of arteries that results in the obstruction of blood flow. Bradycardia: slowness of heartbeat with slowing of pulse rate to less than 60 per minute. Coronary heart disease: narrowing of the arteries that sup- ply blood and oxygen to the heart caused by the buildup of plaque in the arteries. Cyanosis: bluish discoloration of the skin and mucous mem- branes caused by excess concentration of reduced hemo- globin in the blood. Diaphoresis: profuse perspiration. Dyspnea: labored or difficult breathing. Echocardiography: recording of the position and motion of the heart walls and internal structures of the heart and neighboring tissue by the echo obtained from beams of ultrasonic waves directed through the chest wall; used to show valvular and other structural deformities; the record produced is called an echocardiogram. Edema: abnormal accumulation of fluid in the intercellular spaces of the body. Electrocardiography: the graphic recording from the body surface of the potential of electric currents generated by the heart as a means of studying the ac- tion of the heart muscle; the record produced is called an Embolism: the sudden blocking of an artery by a clot of for- eign material, an embolus, that has been brought to its site of lodgment by the bloodstream; the embolus may be a blood clot (most frequently) or an air bubble, a clump of bacteria, or a fat globule. Epistaxis: bleeding from the nose. Heparin: anticoagulant; prevents platelet agglutination and Hypoxia: diminished availability of oxygen to blood tissues. Infarct: localized area of ischemic necrosis produced by oc- clusion of the arterial supply or venous drainage of the Ischemia: deficiency of blood to supply oxygen in part result- ing from functional constriction or actual obstruction of a Lumen: the cavity or channel within a tube or tubular organ, such as a blood vessel or the intestine. Murmur: irregularity of heartbeat caused by a turbulent flow of blood through a valve that has failed to close. Myocardium: the middle and thickest layer of the heart wall, composed of cardiac muscle. Occlusion: blockage; state of being closed. Prolapse: when an organ falls out of its normal position due to lack of support from ligaments and muscles. Restenosis: recurrent stenosis. Sclerosis: induration, hardening. Shunt: abnormal communication between chambers or blood vessels; verb, to bypass, divert. Stenosis: narrowing or contraction of a body passage or Tachycardia: abnormally rapid heart rate, usually taken to be over 100 beats per minute. Tetralogy: a group or series of four. Tetralogy of fallot: congenital, cyanotic malformation of the heart that includes pulmonary stenosis, ventricular septal defect, hypertrophy of the right ventricle, and dextroposi- tion of the aorta. Thrombus: blood clot attached to the intima of a blood ves- sel; may occlude the lumen; contrast with embolus, which is detached and carried by the bloodstream. Venous blood: nonoxygenated blood from the tissues; blood pumped from the heart to the lungs for oxygenation. BOX 67-1 KEY WORDS: Cardiovascular Diseases Wilkins9781451193114-ch067.indd 1130 07/10/15 11:39 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1131 ▶ Anatomic classification • Diseases of the heart: pericardium, myocardium, en- docardium, heart valves • Diseases of the blood vessels and peripheral circulation ▶ Etiologic classification • Congenital anomalies • Atherosclerosis, hypertension • Infectious agents, immunologic mechanisms INFECTIVE ENDOCARDITIS (IE) Infective Endocarditis (IE) is a microbial infection of the heart valves or endocardium with a high mortality rate. ▶ IE is a serious disease, the prognosis of which depends on the degree of cardiac damage, the valves involved, the duration of the infection, and the treatment. ▶ IE is characterized by the formation of bacterial vegeta- tions on the heart valves or surface of the heart lining ▶ When IE develops, it directly affects the function of the • Streptococci and staphylococci are responsible for IE in most cases, with alpha-hemolytic streptococci being the most prevalent. • As yeast, fungi, and viruses have been implicated, the choice of the name “infective” endocarditis is more inclusive than “bacterial” endocarditis. • Incidence related to dental procedures: The majority of IE cases related to oral microflora are random bacte- remias that are a result of routine daily activities. An exceedingly small number of cases are believed to result from dental procedures. ▶ Risk factors • Preexisting cardiac abnormalities: Bacteria lodge on the endocardial (valvular) surface during bacteremia. • Prosthetic (artificial) heart valves: There is an increased number of patients who have had valve replacement surgery who are susceptible. Patients who have had prosthetic valve replacements have a risk of devel- oping prosthetic valve endocarditis. • History of previous endocarditis. • Intravenous drug abuse. Infected material is injected by contaminated needles directly into the blood- stream. Intravenous drug abusers are at high risk for endocarditis, which can initiate on previously nor- ▶ Precipitating factors • Self-induced bacteremia: In the oral cavity, self-in- duced bacteremias may result from eating, bruxism, chewing gum, or any activity that can force bacteria through the wall of a diseased sulcus or pocket. In- terdental aids for oral hygiene can also cause self- • Infection at portals of entry: Infections at sites where microorganisms may enter the circulating blood provide a constant source of potential infectious mi- croorganisms. In the oral cavity, organisms enter the blood by way of periodontal and gingival pockets, where multitudes of many species of microorgan- isms are harbored. An open area of infection, such as an ulcer caused by an ill-fitting denture, may also provide a site of entry. Patients are exposed daily to • Trauma to tissues by instrumentation: Bacteremias are created during general or oral surgery, endodontic procedures, periodontal therapy, scaling, and any therapy that causes bleeding. III. Disease Process ▶ Transient bacteremia initiated • Trauma to a mucosal surface such as the gingival sul- cus during instrumentation releases bacteria into the • Ease of entry of organisms directly relates to the se- verity of tissue trauma, quantity of bacterial biofilm, and the severity of inflammation or infection such ▶ Bacterial adherence • Circulating microorganisms attach to a damaged heart valve, prosthetic valve, or other susceptible area on the endocardium. ▶ Proliferation of bacteria • Microorganisms proliferate to form vegetative le- sions containing masses of plasma cells, fibrin, and • Heart valve becomes inflamed; function is • Clumps of microorganisms (emboli) may break off and spread by way of the general circulation (embo- lism); complications result. ▶ Clinical course • A small number of patients are symptomatic within 2 days, but usually symptoms appear within 2 weeks. • Severe symptoms of fever, loss of appetite and weight loss, weakness, arthralgia, and heart murmurs require hospitalization. Diagnosis is based on symp- toms, echocardiography, blood cell count, and posi- tive blood cultures. • Complications lead to eventual susceptibility to re- infection with IE, congestive heart failure, and cere- Wilkins9781451193114-ch067.indd 1131 07/10/15 11:39 AM 1132 SECTION IX \| Patients with Special Needs The basic areas for attention in dental and dental hygiene care that contribute to the prevention of IE are shown in ▶ Patient history • Special content: Specific questions need to be directed to elicit any history of congenital heart defects, car- diac transplant, presence of prosthetic valves, ac- quired valvular defects, or previous episode of IE. • Consultation with patient’s physician: Consultation can be assumed necessary for all patients with a history of heart defects, and any other condition suggesting the need for prophylactic antibiotic premedication. • Withhold instrumentation: The use of a probe or ex- plorer during assessment of the patient is withheld until the medical status is cleared. ▶ Prophylactic antibiotic premedication • Recommended regimens: The current recommen- dations of the American Heart Association are • Boxes 10-2 and 10-3 and Table 10-4 include the spe- cific information in Chapter 10. • When premedication is indicated, question the pa- tient at the time of the appointment to verify the an- tibiotic was taken on schedule. In the patient record, document the name of the antibiotic, time, and dos- age taken by the patient. ▶ Dental hygiene care • Oral health: Maintenance of a high degree of oral health is necessary for each patient susceptible to IE. • Education: Instruction in oral self-care such as brush- ing and flossing at initial appointments can be pro- vided while the patient is under antibiotic coverage. • Sequence of treatment: Biofilm removal instruction pre- cedes instrumentation for scaling to bring the tissues to a healthy state. The more severe the gingival or periodontal inflammation, the higher the incidence of bacteremia during and following instrumentation. • Instrumentation: Reduce the microbial population about the teeth and on the oral mucosa prior to instrumentation by having the patient brush, floss, and rinse thoroughly with an antimicrobial mouth rinse such as 0.12% chlorhexidine. CONGENITAL HEART DISEASES I. The Normal Healthy Heart ▶ A diagram of the normal heart is shown in Figure 67-1 to provide a comparison with the anatomic changes that may appear in a defective heart. ▶ In the healthy heart, the blood flows in one direction as each chamber contracts, with the valves acting as trap doors that snap shut after each contraction to prevent backflow of blood. ▶ The right side of the heart contains deoxygenated blood from the body cells on its way to the lungs for reoxygenation. The left side of the heart contains oxy- genated blood from the lungs being pumped out to the aorta on its way to the cells of the body. The septal wall divides the left and right sides of the heart. ▶ Anomalies of the anatomic structure of the heart or major blood vessels result following irregularities of de- velopment during the first 9 weeks in utero. ▶ The fetal heart is completely developed by the 9th week. ▶ Early diagnosis is necessary, but not all defects require ▶ Treatment usually involves surgical correction. Causes may be genetic, environmental, or a combination of both. Many are unknown. ▷ Identification of risk patients ▷ Medical and personal history ▷ Consultation with physician ▷ Prophylactic antibiotic coverage during appointments ▷ Upgrading and maintenance of the patient’s oral health ▷ Personal: daily dental biofilm removal ▷ Professional: supervision, instruction, and motivation Prevention of Infective Endocarditis FIGURE 67-1 The Normal Heart. The major vessels and the location of the tricuspid, pulmonary, aortic, and mitral valves are shown. Wilkins9781451193114-ch067.indd 1132 07/10/15 11:39 AM Was this article helpful? The Patient with a Cardiovascular DiseaseDianne Smallidge, RDH, MDH and Esther M. Wilkins, BS, RDH, DMDCLASSIFICATIONINFECTIVE ENDOCARDITIS I. Description II. Etiology III. Disease Process IV. PreventionCONGENITAL HEART DISEASES I. The Normal Healthy Heart II. Anomalies III. Etiology IV. Types of Defects V. Prevention VI. Clinical ConsiderationsRHEUMATIC HEART DISEASE I. Rheumatic Fever II. The Course of Rheumatic Heart DiseaseMITRAL VALVE PROLAPSE I. Description II. SymptomsHYPERTENSION I. Etiology II. Blood Pressure Levels III. Clinical Symptoms of Hypertension IV. Treatment V. Hypertension in ChildrenISCHEMIC HEART DISEASE I. Etiology II. Manifestations of Ischemic Heart DiseaseANGINA PECTORIS I. Precipitating Factors II. Treatment III. Procedure During an Angina AttackMYOCARDIAL INFARCTION I. Etiology II. Symptoms III. Management During an Attack IV. Treatment After Acute SymptomsHEART FAILURE I. Etiology II. Clinical Manifestations III. Treatment During Chronic Stages IV. Emergency Care for Heart Failure and Acute Pul-monary EdemaLIFESTYLE MANAGEMENT FOR THE PATIENT WITH CARDIOVASCULAR DISEASESURGICAL TREATMENT I. Coronary Dilation II. Coronary Bypass III. Cardiac Pacemaker and Implantable Cardioverter DefibrillatorsANTICOAGULANT THERAPY I. Clinical Procedures II. Postprocedural InstructionsCARDIAC SURGERY I. Presurgical II. PostsurgicalDOCUMENTATIONEVERYDAY ETHICSFACTORS TO TEACH THE PATIENTREFERENCESCHAPTER OUTLINE67LEARNING OBJECTIVESAfter studying this chapter, the student will be able to:1. Identify the cardiovascular conditions that may be encountered in patients seeking oral health care.2. Discuss the etiology, symptoms and risk factors associated with cardiovascular conditions.3. Discuss the impact of cardiovascular diseases on the oral cavity and their relationship to oral health.4. Plan dental hygiene treatment modifications for the patient with cardiovascular disease.Wilkins9781451193114-ch067.indd 1129 07/10/15 11:39 AM 1130 SECTION IX \| Patients with Special NeedsCardiovascular includes diseases of the heart and blood vessels. ▶ Patients with cardiovascular conditions are encoun-tered frequently in a dental office or clinic and may be from any age group, although the highest incidence is among older people. ▶ Although a causal relationship between periodontal disease and coronary heart disease (CHD) has not been proven, current data suggest the presence of periodon-tal disease may be a marker for CHD risk.¹ ▶ Dental hygienists need to take responsibility to inform patients of the significant relationship between oral and systemic health and the related need for maintenance of healthy oral tissues and prevention of periodontal disease. ▶ The major cardiovascular diseases are included in this chapter with their principle symptoms and treatments as well as applications for dental hygiene care. ▶ Key words and terminology are defined in Box 67-1. Prefixes and suffixes to clarify the terminology are listed in Appendix VII.Aneurysm: sac formed by the localized dilatation of the wall of an artery, a vein, or the heart.Angina: a condition marked by spasmodic suffocative attacks.Angina pectoris: acute pain in the chest from decreased blood supply to the heart muscle.Anoxia: absence of oxygen in the tissues; may be accompa-nied by deep respirations, cyanosis, increased pulse rate, and impairment of coordination.Anticoagulant: a substance that suppresses, delays, or nulli-fies coagulation of the blood.Apnea: temporary cessation of breathing.Arrhythmia: variation from the normal rhythm, especially with reference to the heart.Arthralgia: joint pain.Arterial blood: oxygenated blood carried by an artery away from the heart to nourish the body tissues.Arteriosclerosis: group of diseases characterized by thicken-ing and loss of elasticity of the arterial wall.Asphyxia: a condition in which there is a deficiency of oxy-gen in the blood and an increase in carbon dioxide.Atheroma: lipid (cholesterol) deposit on the intima (lining) of an artery; also called atheromatous plaque.Atherosclerosis: disease process caused by the deposit of atheromas on the inner lining of arteries that results in the obstruction of blood flow.Bradycardia: slowness of heartbeat with slowing of pulse rate to less than 60 per minute.Coronary heart disease: narrowing of the arteries that sup-ply blood and oxygen to the heart caused by the buildup of plaque in the arteries.Cyanosis: bluish discoloration of the skin and mucous mem-branes caused by excess concentration of reduced hemo-globin in the blood.Diaphoresis: profuse perspiration.Dyspnea: labored or difficult breathing.Echocardiography: recording of the position and motion of the heart walls and internal structures of the heart and neighboring tissue by the echo obtained from beams of ultrasonic waves directed through the chest wall; used to show valvular and other structural deformities; the record produced is called an echocardiogram.Edema: abnormal accumulation of fluid in the intercellular spaces of the body.Electrocardiography: the graphic recording from the body surface of the potential of electric currents generated by the heart as a means of studying the ac-tion of the heart muscle; the record produced is called an electrocardiogram.Embolism: the sudden blocking of an artery by a clot of for-eign material, an embolus, that has been brought to its site of lodgment by the bloodstream; the embolus may be a blood clot (most frequently) or an air bubble, a clump of bacteria, or a fat globule.Epistaxis: bleeding from the nose.Heparin: anticoagulant; prevents platelet agglutination and thrombus formation.Hypoxia: diminished availability of oxygen to blood tissues.Infarct: localized area of ischemic necrosis produced by oc-clusion of the arterial supply or venous drainage of the part.Ischemia: deficiency of blood to supply oxygen in part result-ing from functional constriction or actual obstruction of a blood vessel.Lumen: the cavity or channel within a tube or tubular organ, such as a blood vessel or the intestine.Murmur: irregularity of heartbeat caused by a turbulent flow of blood through a valve that has failed to close.Myocardium: the middle and thickest layer of the heart wall, composed of cardiac muscle.Occlusion: blockage; state of being closed.Prolapse: when an organ falls out of its normal position due to lack of support from ligaments and muscles.Restenosis: recurrent stenosis.Sclerosis: induration, hardening.Shunt: abnormal communication between chambers or blood vessels; verb, to bypass, divert.Stenosis: narrowing or contraction of a body passage or opening.Tachycardia: abnormally rapid heart rate, usually taken to be over 100 beats per minute.Tetralogy: a group or series of four.Tetralogy of fallot: congenital, cyanotic malformation of the heart that includes pulmonary stenosis, ventricular septal defect, hypertrophy of the right ventricle, and dextroposi-tion of the aorta.Thrombus: blood clot attached to the intima of a blood ves-sel; may occlude the lumen; contrast with embolus, which is detached and carried by the bloodstream.Venous blood: nonoxygenated blood from the tissues; blood pumped from the heart to the lungs for oxygenation.BOX 67-1 KEY WORDS: Cardiovascular DiseasesWilkins9781451193114-ch067.indd 1130 07/10/15 11:39 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1131CLASSIFICATION ▶ Anatomic classification• Diseases of the heart: pericardium, myocardium, en-docardium, heart valves• Diseases of the blood vessels and peripheral circulation ▶ Etiologic classification• Congenital anomalies• Atherosclerosis, hypertension• Infectious agents, immunologic mechanismsINFECTIVE ENDOCARDITIS (IE)2,3,4Infective Endocarditis (IE) is a microbial infection of the heart valves or endocardium with a high mortality rate.I. Description ▶ IE is a serious disease, the prognosis of which depends on the degree of cardiac damage, the valves involved, the duration of the infection, and the treatment. ▶ IE is characterized by the formation of bacterial vegeta-tions on the heart valves or surface of the heart lining (endocardium). ▶ When IE develops, it directly affects the function of the heart.II. Etiology ▶ Microorganisms• Streptococci and staphylococci are responsible for IE in most cases, with alpha-hemolytic streptococci being the most prevalent.• As yeast, fungi, and viruses have been implicated, the choice of the name “infective” endocarditis is more inclusive than “bacterial” endocarditis.• Incidence related to dental procedures: The majority of IE cases related to oral microflora are random bacte-remias that are a result of routine daily activities. An exceedingly small number of cases are believed to result from dental procedures. ▶ Risk factors• Preexisting cardiac abnormalities: Bacteria lodge on the endocardial (valvular) surface during bacteremia.• Prosthetic (artificial) heart valves: There is an increased number of patients who have had valve replacement surgery who are susceptible. Patients who have had prosthetic valve replacements have a risk of devel-oping prosthetic valve endocarditis.• History of previous endocarditis.• Intravenous drug abuse. Infected material is injected by contaminated needles directly into the blood-stream. Intravenous drug abusers are at high risk for endocarditis, which can initiate on previously nor-mal valves. ▶ Precipitating factors• Self-induced bacteremia: In the oral cavity, self-in-duced bacteremias may result from eating, bruxism, chewing gum, or any activity that can force bacteria through the wall of a diseased sulcus or pocket. In-terdental aids for oral hygiene can also cause self-induced bacteremia.• Infection at portals of entry: Infections at sites where microorganisms may enter the circulating blood provide a constant source of potential infectious mi-croorganisms. In the oral cavity, organisms enter the blood by way of periodontal and gingival pockets, where multitudes of many species of microorgan-isms are harbored. An open area of infection, such as an ulcer caused by an ill-fitting denture, may also provide a site of entry. Patients are exposed daily to bacteremias.• Trauma to tissues by instrumentation: Bacteremias are created during general or oral surgery, endodontic procedures, periodontal therapy, scaling, and any therapy that causes bleeding.III. Disease Process2 ▶ Transient bacteremia initiated• Trauma to a mucosal surface such as the gingival sul-cus during instrumentation releases bacteria into the bloodstream.• Ease of entry of organisms directly relates to the se-verity of tissue trauma, quantity of bacterial biofilm, and the severity of inflammation or infection such as periodontitis. ▶ Bacterial adherence• Circulating microorganisms attach to a damaged heart valve, prosthetic valve, or other susceptible area on the endocardium. ▶ Proliferation of bacteria• Microorganisms proliferate to form vegetative le-sions containing masses of plasma cells, fibrin, and bacteria.• Heart valve becomes inflamed; function is diminished.• Clumps of microorganisms (emboli) may break off and spread by way of the general circulation (embo-lism); complications result. ▶ Clinical course• A small number of patients are symptomatic within 2 days, but usually symptoms appear within 2 weeks.• Severe symptoms of fever, loss of appetite and weight loss, weakness, arthralgia, and heart murmurs require hospitalization. Diagnosis is based on symp-toms, echocardiography, blood cell count, and posi-tive blood cultures.• Complications lead to eventual susceptibility to re-infection with IE, congestive heart failure, and cere-brovascular disease.Wilkins9781451193114-ch067.indd 1131 07/10/15 11:39 AM 1132 SECTION IX \| Patients with Special NeedsIV. PreventionThe basic areas for attention in dental and dental hygiene care that contribute to the prevention of IE are shown in Box 67-2. ▶ Patient history• Special content: Specific questions need to be directed to elicit any history of congenital heart defects, car-diac transplant, presence of prosthetic valves, ac-quired valvular defects, or previous episode of IE.• Consultation with patient’s physician: Consultation can be assumed necessary for all patients with a history of heart defects, and any other condition suggesting the need for prophylactic antibiotic premedication.• Withhold instrumentation: The use of a probe or ex-plorer during assessment of the patient is withheld until the medical status is cleared. ▶ Prophylactic antibiotic premedication• Recommended regimens: The current recommen-dations of the American Heart Association are followed.3,4• Boxes 10-2 and 10-3 and Table 10-4 include the spe-cific information in Chapter 10.• When premedication is indicated, question the pa-tient at the time of the appointment to verify the an-tibiotic was taken on schedule. In the patient record, document the name of the antibiotic, time, and dos-age taken by the patient. ▶ Dental hygiene care• Oral health: Maintenance of a high degree of oral health is necessary for each patient susceptible to IE.• Education: Instruction in oral self-care such as brush-ing and flossing at initial appointments can be pro-vided while the patient is under antibiotic coverage.• Sequence of treatment: Biofilm removal instruction pre-cedes instrumentation for scaling to bring the tissues to a healthy state. The more severe the gingival or periodontal inflammation, the higher the incidence of bacteremia during and following instrumentation.• Instrumentation: Reduce the microbial population about the teeth and on the oral mucosa prior to instrumentation by having the patient brush, floss, and rinse thoroughly with an antimicrobial mouth rinse such as 0.12% chlorhexidine.CONGENITAL HEART DISEASES5,6I. The Normal Healthy Heart ▶ A diagram of the normal heart is shown in Figure 67-1 to provide a comparison with the anatomic changes that may appear in a defective heart. ▶ In the healthy heart, the blood flows in one direction as each chamber contracts, with the valves acting as trap doors that snap shut after each contraction to prevent backflow of blood. ▶ The right side of the heart contains deoxygenated blood from the body cells on its way to the lungs for reoxygenation. The left side of the heart contains oxy-genated blood from the lungs being pumped out to the aorta on its way to the cells of the body. The septal wall divides the left and right sides of the heart.II. Anomalies ▶ Anomalies of the anatomic structure of the heart or major blood vessels result following irregularities of de-velopment during the first 9 weeks in utero. ▶ The fetal heart is completely developed by the 9th week. ▶ Early diagnosis is necessary, but not all defects require treatment. ▶ Treatment usually involves surgical correction.III. EtiologyCauses may be genetic, environmental, or a combination of both. Many are unknown. ▷ Identification of risk patients ▷ Medical and personal history ▷ Consultation with physician ▷ Prophylactic antibiotic coverage during appointments ▷ Upgrading and maintenance of the patient’s oral health ▷ Personal: daily dental biofilm removal ▷ Professional: supervision, instruction, and motivationPrevention of Infective EndocarditisBOX 67-2FIGURE 67-1 The Normal Heart. The major vessels and the location of the tricuspid, pulmonary, aortic, and mitral valves are shown.Wilkins9781451193114-ch067.indd 1132 07/10/15 11:39 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1133 ▶ Genetic• Heredity is apparent in some types of defects.• An example of a chromosomal defect is Down syn-drome, in which congenital heart anomalies occur frequently (described in Chapter 61). ▶ Environmental• Most congenital anomalies originate between the 5th and 8th weeks of fetal life, when the heart is developing.• Viral infections from the mother (rubella, cytomegalovirus).• Drugs (thalidomide; isotretinoin).• Drinking alcohol and use of cocaine.• Exposure to industrial chemical solvents.IV. Types of Defects4 ▶ The types of heart defects that occur most frequently are the ventricular septal defect, atrial septal defect, pulmonary stenosis, and patent ductus arteriosis. ▶ Defects (openings) in the septal wall cause a mixing of oxygenated and deoxygenated blood. ▶ Atrial and/or ventricular septal defects result in mixing of the blood from the left and right sides of the heart. ▶ Other defects include a passageway between the great arteries and veins, which also causes mixing of oxygen-ated and deoxygenated blood. Two of the more com-mon congenital heart defects are described here. ▶ Ventricular septal defect• In this type of defect, the left and right ventricles exchange blood through an opening in their divid-ing wall (septum).• The oxygenated blood from the lung, which is normally pumped by the left ventricle to the aorta and then to the entire body, can pass across to the right ventricle through the septal defect, as shown in Figure 67-2.• The severity of symptoms is directly related to the specific location and size of the defect. Small defects may close without surgical correction. ▶ Patent ductus arteriosus• A patent ductus arteriosus means the passageway (shunt) is open between the two great arteries that arise from the heart, namely, the aorta and the pul-monary artery.• Normally, the opening closes during the first few weeks after birth.• When the opening does not close, blood from the aorta can pass back to the lungs, as shown in Figure67-3.• The heart compensates in the attempt to provide the body with oxygenated blood and becomes overburdened.V. Prevention6 ▶ Vaccination with rubella for women of childbearing age is highly advised for those not vaccinated in childhood FIGURE 67-2 Ventricular Septal Defect. The right and left ventricles are connected by an opening that permits oxygenated blood from the left ventricle to shunt across to the right ventricle and then recirculate to the lungs. Compare with Figure 67-1, in which the septum separates the ventricles.FIGURE 67-3 Patent Ductus Arteriosus. An open passageway between the aorta and the pulmonary artery permits oxygenated blood from the aorta to pass back into the lungs. Arrows show directions of flow through the patent ductus. Compare with normal anatomy in Figure 67-1.or those without confirmation of immunity by a labora-tory test. ▶ No medications, including over the counter and herbal medications, are to be taken during pregnancy without prior consultation with the physician. ▶ Avoid tobacco use at least 1 month before pregnancy and throughout the pregnancy.Wilkins9781451193114-ch067.indd 1133 07/10/15 11:40 AM 1134 SECTION IX \| Patients with Special Needs ▶ Attain and maintain a healthy weight prior to pregnancy. ▶ Genetic counseling.VI. Clinical Considerations ▶ Signs and symptoms of congenital heart diseaseGeneral conditions that may influence patient manage-ment include:• Easy fatigue• Exertional dyspnea; fainting• Cyanosis of lips and nail beds• Poor growth and development• Heart murmurs• Congestive heart failure. ▶ Dental hygiene concerns• Prevention of IE: Certain defective heart valves are at risk for endocarditis from bacteremia produced during oral treatments. The American Heart As-sociation Recommendations for premedication are consulted for procedure with this group of patients.2• Elimination of oral disease: Maintenance of a high level of oral health.RHEUMATIC HEART DISEASE7Rheumatic heart disease is a complication following rheu-matic fever. A rather high percentage of patients with a his-tory of rheumatic fever have permanent heart valve damage.I. Rheumatic Fever ▶ Incidence• Frequency of this condition in developed countries has declined significantly in the last several decades and is not common in the United States.• Primarily effects children between ages of 5 and 15. ▶ Etiology• The onset of acute rheumatic fever usually appears 2–3 weeks after a beta-hemolytic group A strepto-coccal pharyngeal infection.• Rheumatic fever and rheumatic heart disease are believed to be immunologic disorders caused by sensitization to antigens of beta-hemolytic group A streptococci. ▶ Prevention• The persistence and severity of the pharyngeal infec-tion are significant factors in determining whether rheumatic fever follows.• Early diagnosis and treatment of streptococcal throat and pharyngeal infections are necessary. ▶ Symptoms of acute rheumatic fever• Low grade fever. • Abdominal pain.• Shortness of breath and chest pain related to cardiac issues.• Joint pain with arthritis present in ankles, knees, el-bows and wrists as well as joint swelling with redness and warmth.• Nosebleeds.• Skin rash on trunk and upper parts of the arms and legs or nodules on skin.• Emotional instability.• Muscle weakness with quick uncontrolled jerky movements affecting the face, feet, and hands.II. The Course of Rheumatic Heart DiseaseFollowing the acute stage of rheumatic fever, usually symptoms do not persist except the effects of the valvular deformity. ▶ Symptoms• Stenosis or incompetence of valves; most commonly, the aortic and mitral valves.• Heart murmur influenced by the amount of scarring of the valves and myocardium.• Cardiac arrhythmias.• Late symptoms include shortness of breath, mur-mur, angina pectoris, epistaxis, elevation of diastolic blood pressure, enlargement of the left ventricle, and increasing signs of congestive cardiac failure. ▶ Practice applications• The American Heart Association no longer recom-mends antibiotic prophylaxis prior to dental treat-ment for patients with this condition due to their minimal risk of developing IE.2MITRAL VALVE PROLAPSE8I. Description ▶ The mitral valve is between the left atrium and the left ventricle (Figure 67-1). ▶ Oxygenated blood from the lungs passes from the pul-monary vein into the left ventricle, where it is pumped through the aortic valve and into the aorta for distribu-tion to the body cells. ▶ When the mitral valve leaflets are damaged, the clo-sure is imperfect and oxygenated blood can backflow or regurgitate. ▶ Mitral valve prolapse is the most common disorder of the valve that causes regurgitation. ▶ The mitral valve is prolapsed (becomes misaligned) backwards into the atrium during systole.II. Symptoms ▶ Most patients with mitral valve prolapse are without symptoms. ▶ A small number of cases will have symptoms of palpi-tations, fatigue, atypical chest pain, and a late systolic murmur.Wilkins9781451193114-ch067.indd 1134 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1135 ▶ When there is more severe involvement, an increase in frequency of palpitations and progressive mitral regurgi-tation is apparent along with a systolic click and murmur. ▶ Initial suspicion for diagnosis of valvular heart disease is the recognition of a heart murmur. ▶ The American Heart Association no longer recom-mends antibiotic prophylaxis during dental treatment for patients with this condition.2HYPERTENSION9,10Hypertension means an abnormal elevation of arterial blood pressure. It has been called the “silent killer,” as one-third of people who have it do not have symptoms. It is a contributing risk factor in many vascular diseases, or it may be a result or an effect of underlying pathologic changes. ▶ Detection of blood pressure for dental and dental hy-giene patients has become an essential step in patient assessment prior to treatment. ▶ Early detection, with referral for additional diagnosis and treatment when indicated, can prove to be lifesav-ing for certain people. ▶ Knowledge of the health problems of patients is needed to ensure treatment is safe and risk of emergencies.I. EtiologyA. Primary or Essential Hypertension ▶ Incidence: Approximately 90% of all hypertension is primary or essential. ▶ Predisposing or risk factors: Combinations of the factors listed are more significant than any one alone. Risk fac-tors for atherosclerosis are interrelated. ▶ Tobacco use ▶ Heredity ▶ Overweight ▶ Race: The incidence is higher among African-Ameri-cans than among white Americans, the illness is more severe, and the mortality rate is higher at a younger age. ▶ Salt: Particularly in excess in the diet. ▶ Sex: Men are more affected before age 45 years; women slightly more than men in later years. ▶ Age: General increase from birth to age 20 years; levels off until 40 years of age; then a slow increase into older age. ▶ Environment: Environmental conditions that increase stress factors.B. Secondary HypertensionAbout 10% of all hypertension is secondary to other un-derlying medical conditions. In secondary hypertension, usually both systolic and diastolic blood pressures are el-evated. Examples of causes are: ▶ Oral contraceptives8• Oral contraceptives may elevate blood pressure and is more likely to occur in women who are over-weight, have a history of hypertension during preg-nancy, have a family history, or have mild kidney disease.• The combination of birth control pills and cigarette use may be especially dangerous for some women. ▶ Renal disease• Renal artery obstruction• Pyelonephritis• Renal failure ▶ Endocrine disorders• Hyperthyroidism• Diabetes• Cushing’s syndrome• Thyroid or parathyroid disease ▶ Medications• Decongestants• SteroidsII. Blood Pressure Levels ▶ Blood pressure is documented in the patient’s record as the systolic pressure reading over the diastolic pressure reading (systolic/diastolic). ▶ The systolic blood pressure is the pressure exerted against the arterial walls during the ventricular con-traction. It is altered by the cardiac output, resistance of the capillary bed, and volume and viscosity of the blood. ▶ The diastolic blood pressure is the pressure exerted by the blood within the arteries during the total resting resistance after the contraction of the left ventricle. ▶ Diseases can have an effect on any of these factors, al-tering the blood pressure. ▶ Blood pressure fluctuates, so the baseline blood pres-sure needs to be measured two or three times and the average reading entered in the patient’s record when hypertension is suspected.A. Normal and High Blood Pressure9Table 67-1 lists the normal readings for blood pressure and the stages of hypertension for adults 18 years and over.B. Low Blood Pressure ▶ Many healthy people have a normal systolic pressure under 90 mm Hg, which may be considered “low blood pressure.” ▶ Such a level is normal for that person, and no clinical problems are evident.Wilkins9781451193114-ch067.indd 1135 07/10/15 11:40 AM 1136 SECTION IX \| Patients with Special Needs• Any of the early symptoms of high blood pressure.• Blurring of vision; possible loss of sight.• Severe dyspnea.• Chest pains similar to angina pectoris.• Mental confusion leading to stupor, coma, convulsions.• Activate emergency procedures as the situation can be fatal if not treated immediately or may result in dam-age to multiple body systems.IV. Treatment9A. Goals ▶ Primary hypertension• Achieve and maintain diastolic pressure level below 80 mm Hg.• Lower the risk of serious complications and prema-ture death. ▶ Secondary hypertension• Medical treatment of underlying systemic disease is needed.B. Lifestyle Changes (Box 67-3) ▶ Weight and exercise: Control weight and exercise daily. ▶ Diet: Sodium restriction, in those who are salt sensitive, and modest weight loss of 5%–10% of body weight may be all that are needed for the control of mild elevations of blood pressure. ▶ Tobacco use: All forms of tobacco must be eliminated. ▶ Other risk factors: In addition to factors listed in Box 67-3, life activity contributions to stress and tension need to be minimized.V. Hypertension in Children12 ▶ Children 3 years of age and older need to have blood pressure determinations made at least annually. ▶ A marked sudden drop in blood pressure is usually as-sociated with an emergency, such as severe blood loss, shock, myocardial infarction, sepsis, or other medical problem. ▶ Procedures to following during specific medical emer-gencies can be found in Table8-4 in Chapter 8.III. Clinical Symptoms of HypertensionEssential hypertension is frequently recognized only by blood pressure readings. The condition may go unrecog-nized because of the lack of clinical symptoms. ▶ High blood pressureThose who have early symptoms may describe them as:• Occipital headaches• Dizziness • Visual disturbances• Weakness• Ringing in the ears• Tingling of the hands and feet ▶ Major sequela of long-standing elevation of blood pressure• The brain, eyes, heart, or kidney may undergo marked changes in function. • Hypertensive heart disease; enlarged heart with eventual cardiac failure.• Cerebral vascular accident (stroke, described in Chapter 59).• Hypertensive renal disease.• Ischemic heart disease. ▶ Malignant hypertension11• Malignant hypertension is a life threatening that comes on suddenly with a diastolic reading often above 130 mm/Hg.• This disorder affects about 1% of patients with hy-pertension and symptoms can include:TABLE 67-1 Classification of Blood Pressure for Adults Aged 18 Years or OlderBLOOD PRESSURE CATEGORYSYSTOLIC (mm Hg)DIASTOLIC (mm Hg)Normal ,120 ,80Prehypertension 120–139 80–89Stage 1 hypertension 140–159 90–99Stage 2 hypertension≥160 ≥100Data from National High Blood Pressure Education Program. The Seventh Report of the Joint National Committee on Prevention, Detection, Evaluation, and Treatment of High Blood Pressure. Bethesda, MD: U.S. National Heart, Lung, and Blood Institute; 2003. NIH Publication No. 03-5233. ▷ If overweight, lose weight 5%–10% of body weight. ▷ Limit alcohol intake to no more than 1 ounce of ethanol per day (24 ounces of beer, 8 ounces of wine, or 2 ounces of 100-proof whiskey). ▷ Exercise (aerobic) daily. ▷ Reduce sodium intake. ▷ Maintain adequate dietary potassium, calcium, and magnesium intake. ▷ Stop use of tobacco. ▷ Reduce dietary saturated fat and cholesterol intake for overall cardiovascular health. Reducing fat intake also helps to reduce caloric intake.Lifestyle Modifications for Hypertension Control and/or Overall Cardiovascular RiskBOX 67-3Wilkins9781451193114-ch067.indd 1136 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1137 ▶ If the BP of a child or adolescent is equal to or above the 90th percentile, the BP measurement is repeated during the visit to determine if the patient is hyper-tensive (see Chapter 11, Table 11-1 for blood pressure values for children and adolescents).ISCHEMIC HEART DISEASEIschemic heart disease is the cardiac disability, acute and chronic, that arises from reduction or arrest of blood sup-ply to the myocardium. ▶ The heart muscle (myocardium) is supplied through the coronary arteries, which are branches of the de-scending aorta. ▶ Because of the relationship to the coronary arteries, the dis-ease is often referred to as CHD or coronary artery disease. ▶ Ischemia means oxygen deprivation in a local area from a reduced passage of fluid into the area. ▶ Ischemic heart disease is the result of an imbalance of the oxygen supply and demand of the myocardium re-sulting from a narrowing or blocking of the lumen of the coronary arteries.I. EtiologyOther factors may be involved, but the principal cause of reduction of blood flow to the heart muscle is atheroscle-rosis of the vessel walls, which narrows the lumen, thus obstructing the flow of blood. ▶ Definition of atherosclerosis13• Atherosclerosis is an inflammatory disease of medium and large arteries in which atheromas deposit and thicken the intimal layer of the involved blood vessel.• An atheroma is a fibro-fatty deposit or plaque con-taining several lipids, especially cholesterol.• With time, the plaques continue to thicken and, eventually, close the vessel (Figure 67-4).• Some plaques calcify, whereas others may develop an overlying thrombus. ▶ Risk factors for atherosclerosis13• Inflammation plays a significant role in the forma-tion of atheromas. Low-grade chronic inflammation in other parts of the body, including chronic peri-odontitis, has been shown to have a relationship to adverse cardiovascular outcomes.• Pathogenic microorganisms from these inflamma-tory processes have been associated with atheroma formation in the blood and the subsequent progres-sion of atherosclerosis.• Many risk factors for periodontal disease are also risk factors for atherosclerosis.14• Each risk factor is significant alone. When these factors occur in combinations, the risk of atherosclerosis, and therefore that of ischemic heart disease, is increased.FIGURE 67-4 Atherosclerosis. An atheroma develops within the lining of the normal blood vessel. The atheroma is made of a fatty deposit containing cholesterol. At first, the atheroma is small and no symptoms are apparent, but eventually it enlarges and completely blocks the vessel, thus depriving the area served by the vessel of oxygen. (Source: National Institute of Health. Report of the Working Group on Arteriosclerosis of the National Heart, Lung, and Blood Institute, National Institutes of Health, United States Department of Health and Human Services. Bethesda, MD: National Institute of Health; 1981. NIH Publication No. 81-2034.)Wilkins9781451193114-ch067.indd 1137 07/10/15 11:40 AM 1138 SECTION IX \| Patients with Special Needs• Risk factors include: elevated levels of blood lipids, the result of an increased dietary intake of choles-terol, saturated fat, carbohydrate (especially su-crose), alcohol, and calories.• Tobacco use, diabetes, obesity, insufficient physical activity, increased tensions, emotional stress, and family history may all be significant.• Genetic inheritance can be one factor along with the perpetuation of familial lifestyle habits such as diet, tobacco habits, tensions, and tendencies to-ward lack of exercise.• Prevention depends on educational programs along with early identification of persons at risk.II. Manifestations of Ischemic Heart Disease ▶ Angina pectoris ▶ Myocardial infarction ▶ Congestive heart failureANGINA PECTORIS15 ▶ Angina pectoris is chest pain, the most common symp-tom of coronary atherosclerotic heart disease. ▶ The pain is described as a heavy, squeezing pressure or tightness in the mid-chest region. ▶ The pain may radiate to the left or right arm and neck or even the mandible. On rare occasion, the pain may be limited to one of these areas and not occur in the chest area at all. ▶ The patient may be pale and also experience faint-ness, sweating, difficulty in breathing, anxiety, or fear. The pain lasts 1–5 minutes if precipitating factors are eliminated.I. Precipitating Factors ▶ Stable angina may be precipitated by exertion or exer-cise, emotion, or a heavy meal. In the dental office or clinic, a preventive atmosphere of calmness and quiet can do much to alleviate stress. Stable angina is pre-dictable and consistent in frequency, intensity, and duration. ▶ Unstable angina occurs without exertion or other pre-cipitating factors. The pain may occur while the pa-tient is at rest, and it may vary in intensity at each attack.II. Treatment ▶ A vasodilator, usually nitroglycerin, is administered sublingually. ▶ Basic life support that includes supplemental oxygen is part of the treatment provided in a dental office or clinic.III. Procedure During an Angina Attack ▶ Terminate treatment• Stop the dental or dental hygiene procedure.• Call for assistance and the emergency kit or cart. ▶ Position patient• Return the patient chair to a comfortable position.• Reassure the patient. ▶ Administer vasodilator• Administer nitroglycerin sublingually.• Use of the patient’s own supply is preferable. Prior to starting the appointment procedures make sure the patient’s supply is placed within reach.• The patient can be asked when the nitroglycerin was purchased because the potency is lost after 6 months out of a sealed storage container.• Patient with xerostomia may not have sufficient sa-liva to moisten the nitroglycerin. A few drops of wa-ter from the unit syringe can be placed on the tablet under the tongue. ▶ Check patient response• Give additional vasodilator. Usually, the first tablet relieves the condition within minutes.• When it is suspected that the patient’s supply may not be fresh and the first tablet has been ineffective, use of a second tablet from the dental office emer-gency kit may be advisable. ▶ Call for medical assistance• When the patient does not respond to the second dose of vasodilator, assume the attack to be a myo-cardial infarction.• Call emergency medical service (EMS).• Administer oxygen. ▶ Record vital signs• Use the Medical Emergency Report, Figure 8-1, Chapter 8.• Measure blood pressure, check pulse rate, and count respirations, as described in Chapter 11. ▶ Observe recovery• For the patient who recovers without additional medical assistance, allow a rest period before dismissal.• Record vital signs again. ▶ DocumentationThoroughly document the events that occurred and assessment data collected in the patient’s chart for future reference.MYOCARDIAL INFARCTION16 ▶ Myocardial infarction is the most extreme manifesta-tion of ischemic heart disease. ▶ Other names: heart attack, coronary occlusion, or coro-nary thrombosis.Wilkins9781451193114-ch067.indd 1138 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1139 ▶ The infarction results from a sudden reduction or arrest of coronary blood flow. ▶ The most common artery associated with a myocardial infarction is the anterior descending branch of the left coronary artery. That is also the most common site of advanced atherosclerosis.I. Etiology ▶ Immediate cause: can be a thrombosis that blocks an artery already narrowed by atherosclerosis. ▶ The blockage creates an area of infarction, which leads to necrosis of the area. ▶ Necrosis of the area can occur within a few hours. ▶ A few patients die immediately or within a few hours. Sudden death may be caused by ventricular fibrillation.II. Symptoms ▶ Pain• Location: Pain symptoms may start under the ster-num, with feelings of indigestion, or in the middle to upper sternum. Pain may last for extended periods, even hours.• When the pain is severe, it gives a pressing or crush-ing heavy sensation and is not relieved by rest or nitroglycerin.• Onset: The pain may have a sudden onset, sometimes during sleep or following exercise. The pain may be radial, similar to angina pectoris, which extends to the left or right arm, neck, and mandible. ▶ Other symptoms• Cold sweat, weakness and faintness, shortness of breath, nausea, and vomiting may occur.• Blood pressure falls below baseline.• Women do not always present with symptoms simi-lar to men and may not experience chest pain; faint-ing, pain in the upper back and lower abdomen, and extreme fatigue are chief symptoms.17III. Management During an Attack18 ▶ Terminate treatment• Sit the patient up for comfortable breathing.• Give nitroglycerin, and reassure the patient. ▶ Summon medical assistance• When nitroglycerin does not reduce the anginalike pain within 3 minutes, prepare for basic life support (Table 8-3, Chapter 8).• Call EMT. Administer oxygen.• Use Medical Emergency Report, Figure 8-1, and re-cord vital signs.• Apply basic life support measures, if indicated, while waiting for medical assistance.• Transport to hospital.IV. Treatment After Acute Symptoms19 ▶ Medical supervision• Current medical care for heart attack calls for a short-ened rest period with increased activity, in keeping with the strength and progress of the patient.• Most patients experience extreme fatigue during their convalescence. ▶ Lifestyle changes• Dietary changes and elimination of all forms of to-bacco and stressful activities, as well as control of diseases that exacerbate ischemic heart disease, are essential.• Periodontal health has particular significance.• Many patients need considerable education, reassur-ance, and motivation. ▶ Subsequent appointments• Elective dental appointments are postponed until the patient’s physician has given consent.HEART FAILURE20 ▶ Heart failure, often referred to as Congestive Heart Failure (CHF), is a syndrome in which an abnormality of cardiac function is responsible for the inability or failure of the heart to pump blood at a rate necessary to meet the oxygen needs of the body tissues. ▶ Considered an end-stage heart condition, it re-sults from many forms of cardiovascular diseases and can be related to a number of other systemic conditions.I. EtiologyExamples of diseases that contribute to heart failure are: ▶ Coronary heart disease ▶ Hypertension ▶ Diabetes ▶ Arrhythmias ▶ Congenital heart disease ▶ Thyroid disorders ▶ Alcohol or illegal drug use such as cocaine ▶ HIV/AIDS.II. Clinical Manifestations21 ▶ The clinical manifestations coincide with the parts of the heart involved. ▶ Signs and symptoms are different, depending, in gen-eral, on whether the left or the right side of the heart or both are affected. The general effects are extreme weakness, fatigue, fear, and anxiety.Wilkins9781451193114-ch067.indd 1139 07/10/15 11:40 AM 1140 SECTION IX \| Patients with Special NeedsA. Left Heart Failure ▶ The left side of the heart receives oxygenated blood from the lungs and pumps the blood into the aorta to the rest of the body. ▶ A pathologic condition of the left ventricle or the mi-tral valve alters output, and causes respiratory difficulty because of the backup of serous fluid into the lungs. ▶ Most heart failure, and the conditions leading to it, are a result of left ventricular failure, which is followed by right ventricular failure. ▶ Clinical symptoms are more prominent at night. The patient rests better in a sitting or semisitting position with more than one pillow. ▶ Signs and symptoms of left heart failure include the following:• Weakness, fatigue• Dyspnea, particularly evident on exertion. Shortness of breath when lying supine, relieved when sittingup• Cough and expectoration• Nocturia• Pallor; sweating, cold skin• Diastolic blood pressure increased• Heart rate rapid• Anxiety, fear.B. Right Heart Failure ▶ The right heart receives the venous blood from the vena cava and pumps it to the lungs for oxygenation. ▶ Right heart failure shows evidence of systemic venous congestion with peripheral edema. ▶ When left heart failure precedes right heart failure, the heart is already congested. Resistance to receiving the venous blood is an additional factor. ▶ Signs and symptoms of right heart failure include the following:• Weakness, fatigue• Swelling of the feet and/or ankles. The edema pro-gresses to the thighs and abdomen (ascites) in ad-vanced stages of heart failure• Cold hands and feet• Clubbing of fingers• Cyanosis of mucous membranes and nail beds• Prominent jugular veins• Congestion with edema in various organs: enlarged spleen and liver; gastrointestinal distress with nausea and vomiting; central nervous system involvement with headache and irritability• Anxiety, fear.III. Treatment During Chronic Stages20A patient with an appointment in a dental office or clinic may be receiving a variety of medical treatments. These are revealed by questioning during preparation of histories. Nearly all patients with heart failure complications have the following in their medical treatment plan: ▶ Drug therapy• Physicians may prescribe many different medica-tions for patients with cardiovascular disease. ▶ Dietary control• Limited sodium intake to alleviate fluid retention• Limited fluid intake• Weight reduction. ▶ Limitation of activity• Activity is limited depending on the sever-ity of the health problem and the advice of the physician.IV. Emergency Care for Heart Failure and Acute Pulmonary EdemaA medical emergency that demands urgent attention may occur anywhere. The patient with heart failure or acute pulmonary edema is usually conscious. ▶ Position the patient upright for comfortable breathing (Table 8-3 in Chapter 8). ▶ Call EMS. Administer oxygen. ▶ Use the Medical Emergency Report, Figure 8-1, Chapter 8, and monitor vital signs (blood pressure, respiratory rate, and pulse). ▶ Reassure the patient.LIFESTYLE MANAGEMENT FOR THE PATIENT WITH CARDIOVASCULAR DISEASE22The recommendation for the following is appropriate for all patients with cardiovascular disease: ▶ Education: The patient is counseled to be reassured that lifestyle changes are necessary but a productive life can be led. ▶ Lifestyle changes (Box 67-3)• Reduction of blood pressure• Tobacco cessation• Reduction of low-density lipoprotein and total cholesterol• Increase physical activity• Eat a heart healthy diet, like the DASH (dietary approaches to stop hypertension), which is high in vegetables, fruits, whole grains, low-fat dairy, lean poultry and fish, legumes, and nuts with limited added sugar and red meats.23 ▶ Medications: A variety of medications may be required depending on individual needs.Wilkins9781451193114-ch067.indd 1140 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1141SURGICAL TREATMENT19I. Coronary Dilation ▶ Percutaneous transluminal coronary angioplasty• Widely used procedure to stretch the coronary blood vessel using fluoroscopic guidance allows various tools to be inserted.• An inflatable balloon widens the narrowed lumen.• An atherectomy may be used to remove atheroma-tous plaque from the vessel lining. ▶ Coronary stent• The stent is placed to maintain the open vessel lu-men. Stents are made of metal and become covered with endothelium.• The coronary stent provides a semirigid scaffolding within the lumen, which helps prevent restenosis or renarrowing of the lumen.II. Coronary Bypass ▶ Coronary artery bypass grafting (CABG)• Coronary bypass is primarily for patients with sig-nificant obstruction.• The purpose is to “jump-pass” over arteries that have been narrowed with atherosclerosis.• The beneficial effects are relief from anginal pain, less workload for the heart, and an increase of oxy-gen and blood supply to the myocardium.• Figure 67-5 shows the use of a saphenous vein graft and the internal mammary artery for bypasses.FIGURE 67-5 Coronary Bypass Surgery. A: Heart showing infarcted (shaded) areas created by coronary arteries narrowed by atherosclerosis. B: Vein graft from saphenous vein connected with aorta to bypass narrowed area of right coronary artery, and internal mammary artery used to bypass narrowed left anterior descending artery.III. Cardiac Pacemakers and Implantable Cardioverter Defibrillators (ICD)24,25A. Cardiac Pacemakers ▶ The natural pacemaker, or center where the normal heartbeat is initiated, is the sinoatrial (S-A) node lo-cated in the right atrium. ▶ From that node, impulses are sent along the muscle walls to stimulate and regulate the contractions of the ventricles, which pump the blood throughout the body. ▶ When the natural pacemaker cells are not able to main-tain a reliable rhythm, or when the impulses are inter-rupted because of heart block, cardiac arrest, various arrhythmias, or other disease conditions, treatment by a cardiologist may include the placement of an artificial pacemaker.1. Description• A cardiac pacemaker is an electronic stimulator used to send a specified electrical current to the myocardium to control or maintain a minimum heart rate.• It may be single-chambered (to ventricle or atrium) or dual-chambered to sense and pace both heart chambers.2. Parts and power• A permanently implanted pacemaker has elec-trodes inserted transvenously to the endocardium. Less commonly, the leads may go to the pericar-dium of the external heart wall.Wilkins9781451193114-ch067.indd 1141 07/10/15 11:40 AM 1142 SECTION IX \| Patients with Special Needs• The electrodes are connected to the power source, a plastic- or metal-encased, hermetically sealed pulse generator containing a lithium anode battery.• The pulse generator is implanted under the skin in the thorax or upper abdomen. The area selected depends on the individual condition as deter-mined by the cardiologist (Figure 67-6).B. Implantable Cardioverter Defibrillator1. Description• An ICD is a device that is surgically placed in the chest or abdomen to help treat cardiac arrhythmias.• Patients with cardiac arrhythmias can experience life-threatening events that can result in sudden car-diac arrest.2. Parts and power• An ICD has wires with electrodes on the ends that connect to the heart chambers.• ICDs can detect when an arrhythmia has stopped the heart from beating and can automatically deliver a shock to the heart that will return the heart to a normal rate and rhythm.• ICDs use low-energy electrical pulses to restore a normal rhythm• If low-energy pulses do not restart heart rhythm, a high-energy pulse is delivered, which can be painful for the patient.C. Interferences and Their EffectsUse of ultrasonic and piezo powerscalers for patients with pacemakers and ICDs has previously been discour-aged due to concerns regarding electromagnetic inter-ference (EMI) and its disruptive impact on pacemakers and ICDs. ▶ However, current evidence indicates that EMI pro-duced from these technologies is not sufficient to dis-rupt the function of pacemakers and ICDs.25–27D. Prophylactic Antibiotic Premedication3,4 ▶ IE has occurred in patients with pacemakers and ICDs, and an increase in the incidence of infection following placement of these devices has prompted increased use of pre- and postantibiotic prophylaxis before and after implantation. ▶ Although evidence suggests the patient with a pace-maker is at low risk for endocarditis, the cardiologist may choose to use antibiotics to cover dental and den-tal hygiene procedures. Consultation with the patient’s physician regarding the need for antibiotic prophylaxis is necessary prior to providing dental treatment for the patient with a pacemaker or ICD.ANTICOAGULANT THERAPY28 ▶ Anticoagulants are used in the treatment of many car-diovascular diseases to prevent embolus and thrombus formation. ▶ A prescribed drug may be continued indefinitely by the patient as a preventive measure. ▶ Drugs most commonly used to prevent or delay blood coagulation are heparin (hospital-administered intra-venous) and coumarin derivatives. ▶ Although precautions are needed to prevent hemor-rhage, discontinuing the drug may be more hazard-ous for the patient than performing dental and dental hygiene therapy with precautions. ▶ Consultation with the patient’s physician must oc-cur prior to proceeding with invasive oral surgical procedures.I. Clinical ProceduresA. Consultation ▶ Information about the patient’s INR (international normalized ratio) is obtained from the primary care provider during an initial consultation. ▶ The INR is a test of the coagulation phase of blood clotting used to monitor therapy with anticoagulants.B. Treatment Planning ▶ Pretest for INR• Determine the INR within 24 hours before an ap-pointment. The patient can have the test made on the day of a dental appointment by preplanning with the physician and the laboratory.• Most patients have a routine appointment for moni-toring of the blood, and dental appointment dates can be planned to coincide.• Safe level for dental and dental hygiene procedures is considered to be 2–3, provided precautions are taken during instrumentation and postoperative care.FIGURE 67-6 Cardiac Pacemaker. The pulse generator is implanted under the skin in the thorax or upper abdomen. The lead electrodes may go to the ventricle or to the atrium or both to provide the necessary stimulus for regulation of the heartbeat.Wilkins9781451193114-ch067.indd 1142 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 1143 ▶ Quadrant nonsurgical periodontal therapy• Teach and emphasize daily dental biofilm control procedures in a series of appointments to prepare the gingival tissue for instrumentation. Healthy, healed tissue does not bleed as readily or as profusely.• Complete treatment, including removal of all cal-culus and subgingival biofilm and other irritants, is necessary to contribute to the goal of healthy tissue that does not bleed.C. Local Hemostatic Measures ▶ Instrumentation can be performed for most patients without complication, provided precautions are taken to minimize tissue trauma and control bleeding and not to dismiss the patient until bleeding has stopped. ▶ Pressure: Pressure with sponges or cotton pellets packed interdentally can aid in control.II. Postprocedural Instructions ▶ The practice by oral surgeons of closely observing patients for 6–8 hours following a surgical procedure has application following selected dental procedures. At the least, a check that postcare instructions are being followed is advisable. ▶ The patient is advised to avoid vigorous toothbrushing and rinsing on a treated area for several hours or until the next day. ▶ The use of extraoral icepacks may be helpful. ▶ General postcare instructions on for the care of an area with a periodontal dressing can be found in Table 43-2 in Chapter 43. ▶ The use of a soft diet, cool rather than hot foods. ▶ Moderation of physical activity may be advisable. ▶ Long-term instruction must emphasize the maintenance of gingival health to prevent future bleeding problems.CARDIAC SURGERY ▶ Patients in dental offices and clinics who have had or will have cardiac surgery such as CABG and heart trans-plantation are identified and need special procedures.I. Presurgical ▶ Before elective cardiac surgery, the patient is brought to a state of optimum oral health, with all sources of infection removed. ▶ All restorations and other dental procedures are completed. ▶ Patients requiring cardiac surgery need information and motivation relative to the importance of oral health in eliminating a potential source of IE. ▶ Vigilance in a preventive program that includes biofilm control and self-applied fluorides is essential.II. PostsurgicalA. Continuing Care AppointmentsFrequent appointments are necessary for supervision and dental hygiene care.B. Prophylactic Antibiotic3,4 ▶ Antibiotic coverage for all dental and dental hygiene procedures for patients with prosthetic cardiac valves is essential. Because of the high susceptibility to infections, a special regimen for high-risk patients may be indicated. ▶ Patients with implanted vascular autographs generally do not need antibiotic premedication before dental and dental hygiene appointments. An example of an im-planted vascular autograph is the use of a patient’s own blood vessel to provide a coronary bypass (Figure 67-5). Leo is a 68-year-old, obese, black male with a history of hypertension and hypercholesterolemia. He reminds Ker-stin, the dental hygienist, that he has an extreme dislike of dental appointments as he grasps very tightly to the armrests of the dental chair.During the medical history review, Leo admitted that he usually remembers to take his blood pressure medica-tions but since he does not feel well after the cholesterol-lowering medication, he does not take it regularly. Kerstin takes his right arm blood pressure of 165/90 mm Hg. Leo then starts rubbing his left arm, and Kerstin asks him how he is feeling. Leo says he is having heartburn from a spicy dinner last night and his left arm is sore, probably from doing some yard work a couple of days ago.Questions for Consideration1. What medical, legal, and ethical questions come to mind to be taken into account when a dental hygienist has a patient like Leo with a complicated medical his-tory that involves medications, patient symptoms, and is a nervous patient about any dental appointments?2. Which of the dental hygiene core values has applica-tion in this scenario? How does each of the core values selected affect the appointment plan?3. Using the questions in Table V-1 in Section V Introduc-tion, prepare at least three possible procedure outlines that Kerstin could consider as she decides steps in Leo’s treatment that day.EVERYDAY ETHICSWilkins9781451193114-ch067.indd 1143 07/10/15 11:40 AM 1144 SECTION IX \| Patients with Special NeedsC. Immunosuppressive Therapy ▶ Principal drugs used for patients with transplants are cyclosporin, azathioprine, and prednisolone to prevent rejection of the transplant. ▶ Among the side effects, particularly of cyclosporin, is gingi-val enlargement.27 Many patients may receive medication with the nifedipine group, also effective in causing gingival enlargement. Special periodontal care will be needed.DOCUMENTATIONDocumentation for a routine dental hygiene continuing care or periodontal maintenance appointment for a pa-tient with a cardiovascular illness would need to include a minimum of the following items: ▶ Note and record the responses to health history review questions about visitations to the cardiologist, and in addition to the patient’s report on the state of health, answers from the MD concerning changes that could influence procedures. ▶ Note, record, and compare all findings with previous findings: blood pressure determination, findings in the extraoral and intraoral examination, and the gingival and periodontal clinical examination with complete probing. ▶ An example documentation note may be reviewed in Box 67-4.Example Documentation: Patient with Uncontrolled HypertensionS –Forty-six year old African-American patient arrives in the dental office for a 3-month periodontal continuing care appointment. He reports he has been diagnosed with high blood pressure and is taking Procardia.O – Vital signs: blood pressure: 180/100. Patient reports he cannot afford his medication so he takes it every other day. Contact his cardiologist and/or primary care pro-vider to ensure it is safe to proceed with treatment. The providers gives permission, but recommends no vaso-constrictor in the local anesthetic until the blood pres-sure is under control.A – A comprehensive periodontal and caries examination findings include localized moderate chronic periodonti-tis with bleeding on probing in molar areas along with recurrent caries MO-#14, MO-#15.P – Oral self-care is reviewed with a focus on use of an inter-dental brush in posterior interproximal areas. Periodon-tal maintenance is completed except for in the area of #14–15 where localized nonsurgical periodontal therapy (NSPT) with local anesthesia is recommended. Patient to follow-up with his primary care provider and return for localized NSPT once BP is controlled. Office will follow-up in 2 weeks.Signed: _____________________________________, RDHDate: _______________________________________BOX 67-4 ▷ Encourage patients who have been diagnosed as hypertensive to continue their prescribed therapy.Stress Reduction Procedures ▷ Select an appointment time that is optimum with re-spect to that time of the day when the patient is feeling best and may be less fatigued. Most anxious patients prefer a morning appointment. ▷ Get adequate sleep and rest, and engage in non-fatiguing activities during the 24 hours before the appointment. ▷ Use premedication as prescribed for sleeping the night before. A sedative may be prescribed to be taken 60 minutes before an appointment or at the dental office, if possible. When taken at home 1 hour before, it is not recommended for the patient to drive a car. ▷ Allow time to get to the dental office or clinic; bring own reading material, knitting or sewing, or other relax-ing activity in the event that waiting is unavoidable. ▷ Eat breakfast, lunch, or other usual between-meal food and take usual medications on schedule. ▷ When other family members, especially children, have dental or dental hygiene appointments, do not add to their stress by relaying personal negative feelings.Factors To Teach The PatientReferences1. Humphrey L, Fu R, Buckley D, et al. Periodontal Disease and coronary heart disease incidence: a systematic review and meta-analysis. J Gen Intern Med. 2008;23(12):2079–2086. 2. Glenny AM, Oliver R, Roberts GJ, et al. Antibiotics for the prophylaxis of bacterial endocarditis in dentistry. Cochrane Database Syst Rev. 2013;(10):CD003813.3. Wilson W, Taubert KA, Gewitz M, et al. Prevention of in-fective endocarditis: guidelines from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Qual-ity of Care and Outcomes Research Interdisciplinary Work-ing Group. Circulation. 2007. http://circ.ahajournals.org/ content/116/15/1736.full. Accessed August 2013.4. Nishimura RA, Otto CM, Bonow RO, et al. American Col-lege of Cardiology/American Heart Association Task Force on Practice Guidelines. 2014 AHA/ACC guideline for the management of patients with valvular heart disease: execu-tive summary: a report of the American College of Cardi-ology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2014;63(22):2438–2488.5. March of Dimes. Congenital heart defects. March of Dimes Foundation. 2008. http://www.marchofdimes.com/baby/congenital-heart-defects.aspx. Accessed August 2013.6. Centers for Disease Control and Prevention. Facts about congenital heart defects. http://www.cdc.gov/ncbddd/heart-defects/facts.html. Updated July 18, 2014. Accessed August 20, 2015.Wilkins9781451193114-ch067.indd 1144 07/10/15 11:40 AM CHAPTER 67 \| The Patient with a Cardiovascular Disease 11457. National Institutes of Health, National Library of Medicine, Medline Plus. Rheumatic fever. http://www.nlm.nih.gov/medlineplus/ency/article/003940.htm. Updated May 11, 2014. Accessed August 30, 2015.8. National Institutes of Health, National Heart, Blood, and Lung Institute. What is mitral valve prolapse? http://www.nhlbi .nih.gov/health/health-topics/topics/mvp/. Updated July 1, 2011. Accessed August 30, 2015.9. James PA, Oparil S, Carter BL, et al. 2014 evidence-based guideline for the management of high blood pres-sure in adults: report from the panel members appointed to the Eighth Joint National Committee (JNC 8). JAMA. 2014;311(5):507–520.10. American Heart Association. High blood pressure. http://www.heart.org/HEARTORG/Conditions/High-BloodPressure/High-Blood-Pressure-or-Hypertension_UCM_002020_SubHomePage.jsp. Accessed June 30, 2014.11. National Institutes of Health, National Library of Medicine, Medline Plus. Malignant hypertension. http://www.nlm.nih.gov/medlineplus/ency/article/000491.htm. Updated April 5, 2013. Accessed August 30, 2015.12. Flynn JT, Daniels SR, Hayman LL, et al. American Heart Association Atherosclerosis, Hypertension and Obesity in Youth Committee of the Council on Cardiovascular Disease in the Young. Update: ambulatory blood pressure monitor-ing in children and adolescents: a scientific statement from the American Heart Association. Hypertension. 2014;63(5): 1116–1135.13. National Institutes of Health, National Heart, Lung, and Blood Institute. Atherosclerosis. http://www.nhlbi.nih.gov/health/health-topics/topics/atherosclerosis/atrisk.html. Up-dated August 4, 2014. Accessed August 30, 2015.14. Lockhart PB, Bolger AF, Papapanou PN, et al. Periodon-tal disease and atherosclerotic vascular disease: does the evidence support an independent association?: a scientific statement from the American Heart Association. Circula-tion. 2012;125(20):2520–2544.15. American Heart Association. Angina pectoris. http://www.heart.org/HEARTORG/Conditions/HeartAttack/SymptomsDiagnosisofHeartAttack/Angina-Pectoris-Chest-Pain_UCM_437515_Article.jsp. Updated May 15, 2015. Accessed August 30, 2015.16. National Institutes of Health, National Library of Medi-cine, Medline Plus. Heart attack. https://www.nlm.nih.gov/medlineplus/ency/article/000195.htm. Updated August 12, 2014. Accessed August 30, 2015.17. American Heart Association. Heart attack symptoms in women. http://www.heart.org/HEARTORG/Conditions/HeartAttack/WarningSignsofaHeartAttack/Heart-Attack-Symptoms-in-Women_UCM_436448_Article.jsp. Updated July 2015. Accessed August 30, 2015.18. Reed KL. Basic management of medical emergencies: recog-nizing a patient’s distress. J Am Dent Assoc. 2010;141 (Suppl 1):20S–24S.19. American Heart Association. Prevention and treat-ment of heart attack. http://www.heart.org/HEARTORG/Conditions/HeartAttack/PreventionTreatmentof-HeartAttack/Prevention-and-Treatment-of-Heart-At-tack_UCM_002042_Article.jsp. Updated August 21, 2015. Accessed August 30, 2015.20. National Institutes of Health, National Heart, Lung, and Blood Institute. What is heart failure? http://www.nhlbi.nih.gov/health/health-topics/topics/hf/. Updated March 27, 2014. Accessed August 30, 2015.21. American Heart Association. Types of heart failure. http://www.heart.org/HEARTORG/Conditions/HeartFailure/AboutHeartFailure/Types-of-Heart-Failure_UCM_306323_Article.jsp. Updated June 1, 2015. Accessed August 30, 2015.22. Eckel RH, Jakicic JM, Ard JD, et al. 2013 AHA/ACC guide-line on lifestyle management to reduce cardiovascular risk: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. Cir-culation. 2014;129(25, Suppl 2):S76–S99.23. National Institutes of Health, National Heart, Lung, and Blood Institute. What is the DASH eating plan? http://www.nhlbi.nih.gov/health/health-topics/topics/dash/. Updated June 6, 2014. Accessed July 27, 2014.24. Wilson BL, Broberg C, Baumgartner JC, et al. Safety of electronic apex locators and pulp testers in patients with implanted cardiac pacemakers or cardioverter/defibrillators. J Endod. 2006;32(9):847–852.25. National Institutes of Health; National Heart, Lung and Blood Institute. What is a pacemaker? http://www.nhlbi.nih.gov/health/health-topics/topics/pace/. Updated February 28, 2012. Accessed August 20, 2015.26. Gomez G, Duran-Sindreu F, Roig M, et al. The effects of six electronic apex locators on pacemaker function: an in vitro study. Int Endod J. 2013;46(5):399–405.27. Uslan D, Gleva M, Poole J, et al. Cardiovascular implant-able electronic device replacement infections and pre-vention: results from the REPLACE Registry. Pacing Clin Electrophysiol. 2012;35(1):81–87.28. Douketis JD, Spyropoulos AC, Spencer FA, et al. Periopera-tive management of antithrombotic therapy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Prac-tice Guidelines. Chest. 2012;141(2, Suppl):e326S–e350S.29. Dongari-Bagtzoglou A; and Research, Science and Ther-apy Committee, American Academy of Periodontol-ogy. Drug-associated gingival enlargement. J Periodontol. 2004;75(10):1424–1431.ENHANCE YOUR UNDERSTANDINGDIGITAL CONNECTIONS(see the inside front cover for access information)• Audio glossary• Quiz bankSUPPORT FOR LEARNING(available separately; visit lww.com)• Active Learning Workbook for Clinical Practice of the Dental Hygienist, 12th EditionINDIVIDUALIZED REVIEW(available separately; visit lww.com)• Adaptive quizzing with prepU for Wilkins’ Clinical Practice of the Dental HygienistWilkins9781451193114-ch067.indd 1145 07/10/15 11:40 AM Wilkins9781451193114-ch067.indd 1146 07/10/15 11:40 AM	2	29	1	0	0	1	0.875313	2	19,457
- Sudden cardiac death Sudden cardiac death Classification and external resources ICD-10 I46.1 eMedicine article/151907 MeSH D016757 Sudden cardiac death is natural death from cardiac causes, heralded by abrupt loss of consciousness within one hour of the onset of acute symptoms. Other forms of sudden death may be noncardiac in origin. Examples of this include respiratory arrest (such as due to airway obstruction, which may be seen in cases of choking or asphyxiation), toxicity or poisoning, anaphylaxis, or trauma. It is important to make a distinction between this term and the related term cardiac arrest, which refers to cessation of cardiac pump function which may be reversible. The phrase sudden cardiac death is a public health concept incorporating the features of natural, rapid, and unexpected. It does not specifically refer to the mechanism or cause of death. The most common cause of sudden cardiac death in adults over the age of 30 is coronary artery atheroma. The most common finding at postmortem examination is chronic high-grade stenosis of at least one segment of a major coronary artery, the arteries which supply the heart muscle with its blood supply. A significant number of cases also have an identifiable thrombus (clot) in a major coronary artery which causes transmural occlusion of that vessel. Death in these cases is thought to result from a period of transient or prolonged ischaemia (lack of blood supply) in the myocardium (muscle of the heart wall) which induces an arrhythmia (a conduction disturbance), usually a ventricular arrhythmia, which terminates into ventricular fibrillation. As a consequence there may be no changes in the myocardium. The absence of the histological signs of acute necrosis and a healed infarct are a common finding. Chronic high-grade stenosis may have caused previous episodes of ischaemia and areas of focal fibrosis may be seen histologically in the myocardium. Ventricular arrhythmias may arise from a myocardium which has been previously scarred by episodes of ischaemia. Left ventricular hypertrophy is the second leading cause of sudden cardiac death in the adult population. This is most commonly the result of longstanding raised high blood pressure which has caused secondary damage to the wall of the main pumping chamber of the heart, the left ventricle. Once again, hypertrophy is associated with cardiac arrhythmias. The mechanism of death in a the majority of patients dying of sudden cardiac death is ventricular fibrillation and as a consequence there may be no prodromal symptoms associated with the death. These patients may be going about their daily business and suddenly collapse without the typical features of myocardial infarction such as chest pain and shortness of breath. There are a number of cases in which patients feel the effect of myocardial ischaemia. Myocardial ischaemia is associated with referred pain, classically to the front of the chest, the left arm and the jaw. Patients may feel generally unwell, with nausea, dizziness and vomiting. These symptoms may precede the death for any length of time between a few minutes and several hours. Cardiac rapid response units and the use of portable defibrillators have contributed to the survival of many people who have collapsed with ventricular fibrillation. However, in cases of sudden cardiac death, the heart appears to be resistant to attempts of resuscitation. Early defibrillation in these cases rarely results in survival. The mechanism underlying this resistance is currently being investigated. Much attention in the press has been focused on young athletes who die suddenly whilst training as a consequence of sudden cardiac death. Though these cases are rare compared to the hundreds who die each year of coronary artery atheroma and left ventricular hypertrophy, they attract much more attention due to the sensational nature of young, otherwise healthy persons suddenly and unexpectedly dying. A great deal of time and money is being spent on researching these cases and implementing schemes to screen young athletes to prevent their occurrence. Although the most frequent cause of sudden cardiac death is coronary artery disease, other causes include: - Non-atherosclerotic coronary artery abnormalities - Hypertrophy of ventricular myocardium - Myocardial diseases and heart failure, including - Inflammatory, infiltrative, neoplastic, and degenerative processes - Diseases of the cardiac valves - Congenital heart disease - Primary electrophysiological abnormalities, such as - Long QT syndrome, both congenital and acquired - Sick sinus syndrome - Brugada syndrome - Catecholaminergic polymorphic ventricular tachycardia - Magnesium deficiency - Rhythm instability related to neurohumoral and central nervous system influences - Cystic tumour of the atrioventricular nodal region - Commotio cordis - Mechanical interference with venous return - Aortic dissection - Toxic/metabolic disturbances - ^ Myerburg, Robert J. "Cardiac Arrest and Sudden Cardiac Death" in Heart Disease: A Textbook of Cardiovascular Medicine, 7th edition. Philadelphia: WB Saunders, 2005. - ^ http://poptop.hypermart.net/sudden.html - Heart Rhythm Society Sudden Cardiac Arrest Page - Sudden Cardiac Arrest Association - Sudden Cardiac Arrest Foundation - Arrhythmia Alliance Sudden Cardiac Arrest Page - Cardiac Risk in the Young - Test My Heart screening tour from Philips and CRY (UK) Cardiovascular disease: heart disease · Circulatory system pathology (I00–I52, 390–429) IschaemicActive ischemia LayersValves Conduction/ arrhythmiaPremature contractionWolff-Parkinson-White · Lown-Ganong-LevineFlutter/fibrillationPacemakerOther/ungrouped Wikimedia Foundation. 2010. Look at other dictionaries: sudden cardiac death — n death occurring within minutes or hours following onset of acute symptoms of cardiac arrest resulting esp. from an arrhythmia * * * unexpected natural death from cardiac causes, occurring rapidly after onset of acute symptoms in a patient with… … Medical dictionary Sudden Cardiac Death — Klassifikation nach ICD 10 I21 I22 Plötzlicher Tod bei Myokardinfarkt I44 I45 Plötzlicher Tod bei Erregungsleitungsst … Deutsch Wikipedia sudden cardiac death — noun death from cardiac malfunction, with abrupt loss of consciousness as a preliminary symptom. Abbrev.: SCD … Australian English dictionary Sudden adult death syndrome — can refer to: * sudden unexpected nocturnal death syndrome * sudden cardiac death … Wikipedia sudden adult death syndrome — sudden arrhythmia death syndrome, sudden arrhythmic death syndrome (SADS) any of various conditions in which sudden cardiac death results from an arrhythmia, usually tachycardia but occasionally severe bradycardia … Medical dictionary Sudden unexplained death syndrome — Sudden unexpected death syndrome (SUDS) or Sudden unexpected nocturnal death syndrome (SUNDS) is sudden unexpected death of adolescents and adults during sleep.Fact\|date=September 2008 It is rarely observed in the Western world, and appears to… … Wikipedia Cardiac Risk in the Young — (CRY) humanitarian charitable organization helping to raise awareness of cardiac risk, Sudden Cardiac Death (SCD), Sudden Death Syndrome (SDS, SADS). Established in 1995 by Alison Cox and based in the United Kingdom.The organization promotes… … Wikipedia Sudden Death — may refer to: Sudden cardiac death Sudden death (sport) Sudden Death may refer to: 1958 NFL Championship Game, nicknamed Sudden Death for its dramatic ending Sudden Death (1977 film), starring Robert Conrad Sudden Death (1985 film), starring… … Wikipedia sudden death — n unexpected death that is instantaneous or occurs within minutes or hours from any cause other than violence <sudden death following coronary occlusion> esp SUDDEN CARDIAC DEATH … Medical dictionary Cardiac asthma — Classification and external resources ICD 10 I50.1 ICD 9 428.1 … Wikipedia	4	5	0	0	0	7	0.81939	7	1,739
The diagnostic group as a whole is characterized by a combination of disorders of thinking, perception and emotional-volitional disorders that last at least a month, but a more accurate diagnosis can only be made for 6 months. observations. Typically, the first step is a diagnosis of acute transient psychotic disorder with symptoms of schizophrenia or schizophrenia-like disorder. Disease stages: initial, manifest, remission, repeated psychosis, deficiency. In 10% of cases, spontaneous exit and prolonged (up to 10 years remission) are possible. The reasons for the differences in the prognosis are predominantly endogenous. In particular, the prognosis is better for women, with a picnic physique, high intelligence, life in a complete family, as well as with a short (less than 1 month) initial period, a short manifest period (less than 2 weeks), no abnormal premorbid background, no dysplasias, low resistance to psychotropic drugs. According to E. Bleiler, axial disorders of schizophrenia include disorders of thinking (tearing, resonance, paralogy, autism, symbolic thinking, narrowing of concepts and mantism, perseveration and poverty of thoughts) and specific emotional-volitional disorders (dulling of affect, coldness, paratimia, hypertrophy of emotions, ambivalence and ambivalence, apathy and abulia). M. Bleiler believed that axial disturbances should be outlined by the presence of manifest manifestations, the absence of exogenous type of reaction syndromes (amentia, delirium, quantitative changes in consciousness, seizures, amnesia), the presence of torn thinking, splitting in the sphere of emotions, facial expressions, motor skills, depersonalization, mental automatisms, catatonia and hallucinations. V. Meyer-Gross attributed the primary symptoms to mental disorders, passivity with a sense of influence, primary delirium with ideas of attitude, emotional flattening, sounding of thoughts and catatonic behavior. Symptoms of the first rank according to K. Schneider found the greatest recognition in diagnosis, which include: the sound of one’s own thoughts, auditory contradictory and mutually exclusive hallucinations, auditory commentary hallucinations, somatic hallucinations, effects on thoughts, effects on feelings, effects on motives, effects on actions, a symptom of open thoughts, sperrung and delusional perception, close to acute sensory delirium. Symptoms of the second rank include catatonia, pathological expression in speech, emotions, and feelings. Most of these symptoms are also taken into account in the current classification thanks to the International Schizophrenia Study in 9 countries. According to ICD 10, at least one of the following symptoms should be noted: - “Echo of thoughts” (sounding of one’s own thoughts), insertion or withdrawal of thoughts, openness of thoughts. - Delusions of exposure, motor, sensory, ideator automatism, delusional perception. Such a combination in domestic psychiatry is referred to as Kandinsky-Clerambo syndrome. - Auditory commenting on true and pseudo-hallucinations and somatic hallucinations. - Delusions that are culturally inadequate, ridiculous and grandiose in content. Or at least two of the following symptoms: - Chronic (more than a month) hallucinations with delusions, but without pronounced affect. - Neologisms, sperprings, fragmentation of speech. - Catatonic behavior. - Negative symptoms, including apathy, abulia, impoverishment of speech, emotional inadequacy, including coldness. - Qualitative changes in behavior with loss of interest, lack of focus, autism. The course of schizophrenia can be established already in the period of the manifesto, but more precisely – after the third attack. With a tendency to remissions of good quality, seizures are usually polymorphic, include the affect of anxiety, fear. A continuous course is distinguished, which means the absence of remission for more than a year, episodic with an increasing defect, when negative symptoms progressively (continuously) increase between psychotic episodes, episodic with a stable defect, when persistent negative symptoms are noted between psychotic episodes. The episodic course corresponds to the symptoms of a paroxysmal course accepted in domestic psychiatry. Episodic remitting when complete remissions between episodes are noted. This variant of the course corresponds to the symptoms of a periodic course accepted in domestic psychiatry. After the attack, incomplete remission is also possible. Earlier in Russian psychiatry, this concept corresponded to remissions “B” and “C” according to M. Ya. Sereysky, in which behavioral disorders, impaired disorders, an encapsulated psychosis clinic, or neurotic symptoms are found in a remission clinic. Complete remission corresponds to remission “A” according to M.Ya. Sereysky. Persistent negative symptoms during remissions (defect) include in your clinic the erased symptoms of productive symptoms (encapsulation), behavior disorders, decreased mood against the background of apathic-abulic syndrome, loss of communication, decreased energy potential, autism and fencing, loss of understanding, instinctive regression. In childhood, this diagnosis can be made quite accurately only after 2 years, from 2 to 10 years, nuclear forms predominate, which manifest in a slightly different form. Paranoid forms are described from the age of 9 years. Characteristic symptoms of childhood schizophrenia are regression, in particular, regression of speech, behavior (symptom of manege, ballet walking, choice of non-game items, neophobia), emotional-volitional disorders and developmental delay. The equivalents of delirium are overvalued fears, delusional fantasies. The premorbid background is often without features. The initial period is short – from several days to several months. In the clinic of this period – symptoms of anxiety, confusion, individual hallucinatory inclusions (hail), impaired concentration. The onset can also be a type of reactive paranoid or acute sensory delirium, which is initially regarded as an acute transient psychotic disorder with schizophrenia symptoms or schizophrenia-like. The manifest period is from 16 to 45 years old. Options for paranoid schizophrenia are: paraphrenic with symptoms of predominantly systematic paraphrenia; hypochondria, in which the delirium of infection is clearly associated with the content of auditory, olfactory, somatic hallucinations; hallucinatory-paranoid variant, proceeding with Kandinsky-Clerambo syndrome. Particular variants of paranoid schizophrenia are affective-delusional variants characteristic of the remitting course. These include depressive-paranoid and expansive-paranoid variants. The depressive-paranoid variant usually begins as hypochondriacal delirium, which grows to a degree of enormity, depressive affect is secondary. The expansive-paranoid variant proceeds with the clinic of expansive paraphrenia, however, the expansion continues less than the ideas of greatness. Classical paranoid schizophrenia is accompanied by polythematic delirium, in which it is difficult to separate the ideas of persecution, attitudes, meanings. With paranoid schizophrenia, all course options are possible (continuous, episodic, and remitting), and negative disorders during the period of remission include sharpening of character traits, fixation of apathetic-abulic symptoms, “encapsulation”, in which certain symptoms of hallucinations and delusions are found in the remission clinic. In the manifest period and the further course of the disease are characteristic: - Delusions of persecution, attitudes, values, high descent, special purpose or ridiculous delusions of jealousy, delusions of influence. - Auditory true and pseudo-hallucinations of a commenting, contradictory, judgmental and imperative nature. - Olfactory, gustatory and somatic, including sexual, hallucinations. The classical logic of the development of delirium described by V. Magnan corresponds to the sequence: paranoid (monotemic delirium without hallucinations) – paranoid (polythematic delirium with the addition of auditory hallucinations) – paraphrenic. However, this logic is not always noted, the development of acute paraphrenia and the absence of a paranoid stage are possible. At the first stages, it is necessary to differentiate with acute transient psychotic disorders, and then with chronic delusional and schizoaffective disorders, as well as organic delusional disorders. Acute transient psychotic disorders can occur with productive and negative symptoms of schizophrenia, however, these conditions are short-term and limited for about two weeks with a high probability of spontaneous release and good sensitivity to antipsychotics. This section, meanwhile, can be considered as “cosmetic” at the stage of manifest psychosis in paranoid schizophrenia. Chronic delusional disorders include monothematic delusions, if auditory hallucinations occur, they are often true. This group includes those delusions that were commonly called paranoid (love delirium, delirium of reformism, invention, and persecution). In schizoaffective disorders, delusional disorders are secondary to affect, and affect (manic, expansive, depressive) lasts longer than delirium. With organic delusional disorders, exogenous symptoms are often present, and neurologically, neuropsychologically and using objective research methods, it is possible to identify the main organic brain disease. In addition, personality changes in such disorders have a specific organic color. Until now, it is believed that treatment of acute manifest psychosis in paranoid schizophrenia is best to start with detoxification therapy, as well as antipsychotics. The presence of depressive affect in the structure of psychosis forces the use of antidepressants, but expansive affect can be stopped not only with tisercin, but also with carbamazepine and beta-blockers (propranolol, inderal). The onset of paranoid schizophrenia in adolescence is usually accompanied by an unfavorable course, so the increase in negative disorders can be prevented by insulinocomatous therapy, small doses of rispolept (up to 2 mg) and other antipsychotic drugs. In acute psychosis, rispolept doses increase to 8 mg. Antipsychotics – prolongs are used as maintenance therapy, and if there is an affect in the structure of psychosis, lithium carbonate. Therapy is based either on the principle of influencing the leading syndrome, which is chosen as the “target” of therapy, or on the principle of a complex effect on the amount of symptoms. Initiation of therapy should be careful to avoid dyskinetic complications. With resistance to antipsychotic therapy, monolateral ECT is used, while the application of electrodes depends on the structure of the leading syndrome. Maintenance therapy is carried out depending on the characteristics of the seizure clinic with either antipsychotic prolongs (haloperidol-depot, lioradin-depot), or antipsychotics in combination with lithium carbonate. Behavioral disorders are common in premorbid: antidisciplinary, antisocial, and criminal behavior. Dissociative personality traits, early puberty and homosexual excesses are frequent. This is often perceived as a distortion of the puberty crisis. The onset most often covers the age of 14-18 years, although the manifestation of later hebephrenia is also possible. Further, in the manifest period, a triad is characteristic, which includes the phenomenon of inaction of thoughts, unproductive euphoria and grimacing, reminiscent of uncontrolled tics. The style of behavior is characterized by regression in speech (obscene speech), sexuality (random and abnormal sexual intercourse) and in other instinctive forms of behavior (eating inedible, aimless dromomania, sloppy). In the structure of hebefren syndrome, the following are detected: - Motor-volitional changes in the form of grimacing, foolishness, regression of instincts, unmotivated euphoria, aimlessness and lack of focus. - Emotional inadequacy. - Formal paralogical disorders of thinking – resonance and fragmentation. - Unfolded delirium and hallucinations that do not come to the fore and are of the nature of inclusions. The course is often continuous or episodic with an increasing defect. In the structure of the defect is the formation of disocial and schizoid personality traits. Hebephrenic schizophrenia should be differentiated with tumors of the frontal lobes and dementia in Peak and Huntington’s disease. With tumors, cerebral symptoms, changes in the fundus, EEG and CT can be detected. Peak’s disease is noted at a much later age, and with Huntington’s disease, hyperkinesis of thinking, facial expressions, gesture, and posture is specific. On CT, in patients with schizophrenia who have been taking antipsychotics for a long time, there may be changes similar to Huntington’s disease. Treatment includes the use of insulin therapy, hypervitamin therapy, tranquilizers and large antipsychotics (chlorpromazine, majeptil, trisedil, haloperidol, zeprex, rispolepta in doses of about 4 mg per day). Maintenance therapy is carried out by combinations of antipsychotic prolongs and lithium carbonate, which allow you to control impulses, in particular aggression. Premorbid background is characterized by schizoid personality disorder, although it is possible to develop against a premorbid unchanged background. In the initial period, depressive episodes, simplex syndrome with fencing, loss of initiative and interests. The manifestation is likely as an acute reactive stupor, after traumatic brain injury, flu, although more often psychosis develops for no apparent reason. Classical catatonic schizophrenia occurs in the form of lucid catatonia, catatonic-paranoid states and oneiric catatonia, as well as febrile catatonia. The motor component in catatonia is expressed in the form of stupor and arousal. At present, classical catatonia has been replaced by microcatatonic states. Catatonic stupor includes mutism, negativism, catalepsy, rigidity, solidification, automatic subordination. Usually, Pavlov’s symptom is noted in the stupor (the patient responds to whispering speech, but does not respond to normal speech), gear symptom (jerking resistance is observed when the arm is bent and extended), a symptom of an air cushion (the head remains raised after the pillow is removed), a symptom of a hood ( the patient seeks to hide with his head or covers his head with clothing). Catatonic arousal proceeds with the phenomena of randomness, unfocusedness, perseveration and fragmentation of thinking. The entire clinic can be expressed either in a change of excitement and stupor, or in the form of repeated stupors (excitations). With lucid catatonia, purely motor psychosis is noted, and behind the facade of motor disorders, no productive disturbances are noted. The catatonic-paranoid variant suggests that delirium lies behind the catatonia. Often, such productive irregularities can be indirectly detected as a result of observing the patient’s facial expressions: he looks, the facial expression changes, regardless of the context of the doctor’s questions. With oneiric catatonia, behind the catatonia facade, an influx of fantastic visual images of a cosmic, apocalyptic nature is noted. The patient visits other worlds, heaven and hell. Amnesia after exiting this condition is absent. Febrile catatonia as a variant of catatonic schizophrenia is recognized only by some psychiatrists, most believe that the adherence of temperature to the stupor is due to either additional somatic pathology, or unrecognized stem encephalitis, or malignant antipsychotic syndrome. In the clinic, there are discrepancies in the pulse rate and the temperature, a petechial rash appears on the lower extremities, a gray film appears on the mucous membrane of the lips, and muscle tone gradually increases. Signs of microcatatonia include increased tone of the muscles of the shoulder girdle, increased activity of the oral zone, stereotyping of facial expressions, postures, gestures, gait, speech stereotypes, mutism, stereotyped playing with fingers, hypokinesia of the posture, decreased mobility of the hands with increased activity of the fingers, and the absence of blinking. Sometimes a catatonic stupor appears only in the form of mutism. All flow options are possible. The defect is usually expressed in apathetic-abulic states. The diagnosis is based on the identification of: - chaotic, non-targeted excitation; - catalepsy and negativism; - subordination and stereotypy (perseveration). Catatonic schizophrenia should be distinguished from organic catatonic disorders resulting from epilepsy, systemic diseases, tumors, encephalitis, and from depressive stupor. With organic catatonia, atypicality of motor disorders is noticeable. For example, against the background of catalepsy – tremor of the fingers, choreoathetoid movements, the difference in the symptoms of rigidity and catalepsy on the upper and lower extremities, muscle hypotension. CT, EEG and neurological examination data help to clarify the diagnosis. Depressive stupor is accompanied by a characteristic facial expression of depression with a fold of Veragut. Depression is revealed in the anamnesis. Symptoms of microcatatonia resemble both signs of neuroleptic intoxication and behavioral signs of a defect in schizophrenia, such as apathetic-abulic. In the latter case, they speak of secondary catatonia. For a differential diagnosis, detoxification therapy, tremblex, parkopan, cyclodol, or akineton is useful. The use of this course usually reduces the signs of antipsychotic intoxication. Catatonic mutism should be distinguished from selective (selective) mutism in children and adults with schizoid personality disorders. Medium and large doses of antipsychotic drugs for catatonia can lead to the fixation of symptoms and their translation into a chronic course. Therefore, with stupor, therapy should be prescribed with the intravenous administration of tranquilizers in increasing doses, sodium oxybutyrate, droperidol, nootropics, with careful monitoring of the patient’s somatic condition. A good effect is given by 5-6 ECT sessions with bilateral application of electrodes. The occurrence of a febrile state in the absence of contraindications forces an ECT or transfer to the intensive care unit. Catatonic excitation is stopped by chlorpromazine, haloperidol, tisercin. The clinic includes signs of paranoid, catatonic and hebephrenic schizophrenia in a state of psychosis. Such a high polymorphism within the framework of one psychosis usually involves an episodic remitting course. However, with the development of symptoms from one typology to another in a sequential chain of psychoses, the course can be continuous, for example, when a transition from paranoid to nuclear syndromes is noted in dynamics. The lack of differentiation of symptoms is sometimes associated with the fact that the disease proceeds against the background of dependence on drugs or alcohol, against the background of the immediate and long-term consequences of a traumatic brain injury. The diagnosis is based on the identification of symptoms of paranoid, catatonic and hebephrenic schizophrenia. A high polymorphism of psychosis is also characteristic of schizoaffective disorders, but with them affective disorders last longer than those characteristic of schizophrenia. The complexity of therapy lies in the choice of a “target” of exposure and a complex of maintenance therapy. For this purpose, the selection of axial symptoms is important, which is almost always visible in the dynamics of the disease. Post schizophrenic depression (F20.4). After a typical episode previously experienced with productive and negative symptoms of schizophrenia, a protracted depressive episode develops, which can be considered as a consequence of schizophrenic psychosis. Usually this episode is atypical. That is, there is no typical diurnal dynamics of mood disorders, for example, mood worsens by the evening as asthenic depression. Complex senestopathies, apathy, reduction of energy potential, aggressiveness may be present. Some patients interpret their condition as the result of a psychosis. If the level of depression corresponds to a mild to moderate depressive episode, it can be considered as a special remission clinic, and with the prevalence of negative disorders, as the dynamics of the defect. The diagnosis is based on the identification of: - a history of schizophrenic psychosis; - depressive symptoms, combined with negative symptoms of schizophrenia. At the onset of the disease after 50 years, it is necessary to differentiate these disorders with the initial period of Alzheimer’s disease, more precisely, with its variant, the disease with Levy bodies. In this case, additional neuropsychological and neurophysiological studies are needed to differentiate. Treatment includes a combination of tricyclic antidepressants and antipsychotics. It is possible to apply braking with nitrous oxide, as well as ECT with application of electrodes to the non-dominant hemisphere. This diagnosis can be considered as a delayed (for more than a year after psychosis) diagnosis of a typical defect in the emotional-volitional sphere after a psychotic episode that meets the criteria for schizophrenia. Diagnostic criteria are as follows: - Negative symptoms of schizophrenia in the emotional-volitional sphere (decreased activity, emotional smoothness, passivity, poor speech and non-verbal communication, decreased self-care and social communication skills). - In the past, at least one psychotic episode corresponding to schizophrenia. - A year has passed, during which productive symptoms have been reduced. The need for a differential diagnosis arises when there is no objective information about the past psychosis or the patient hides his past. In this case, the specified disorder can be considered schizotypic. In therapy, small, stimulating doses of antipsychotics, fluoxetine, nootropics in combination with long-term group psychotherapy and rehabilitation are used. This type of schizophrenia is not included in the American classification, since it is difficult to differentiate from the dynamics of schizoid personality disorder. However, if the personality was relatively harmonious in premorbid, its transformation and the appearance of regression traits in combination with emotional-volitional disorders suggest the specified diagnosis. The onset of the disease is from 14 to 20 years. In the initial period – obsessive-phobic, neurotic or affective episodes. In the manifest period, formal disorders of thinking (autistic, symbolic, resonant, paralogical), dysmorphopsia and senestopathy can be noted. Negative symptoms of schizophrenia are detected in the emotional-volitional sphere, activity decreases, emotional coldness occurs. Goal setting is disturbed, as a result of ambivalence passivity arises. Poverty of thinking is accompanied by complaints of emptiness in the head, speech is poor. Hypomimia, sometimes paramimia. Lost previous acquaintances and friends. The range of interests that may become fanciful is narrowed or stereotyped. Autistic thinking can be actively manifested and presented to others (autism inside out), but more often it is hidden from others by external self-absorption, staying in a fantasy world that has no points of contact with the world. Relatives often consider the patient lazy, stupid.	2	11	0	0	0	1	0.798532	1	4,849
There are many products that can help people with disabilities enhance their quality of life. Hearing aids, wheelchairs, braille equipment, communication devices, software, urine collection systems, oxygen apparatus, and mobile hoists all help people with disability to function better in their daily life and to participate in the society. Subdivisions of assistive products can be found in several classifications and nomenclatures. Important ones at the international level are ISO 9999 (Assistive products for persons with disability – Classification and terminology), GMDN (Global Medical Device Nomenclature) and SNOMED CT (Systematized Nomenclature of Medicine – Clinical Terms). This article focuses on ISO 9999 'Assistive products for persons with disability – Classification and terminology'. ISO 9999 is an international classification of assistive products in which all products that can be used by persons with disabilities are included. The goal of ISO 9999 is to promote communication internationally about the use of assistive products by people with disability including elderly people. In this article, several aspects of ISO 9999, such as its history, scope and content, are described. To place ISO 9999 in the general context of the classification of assistive products, this article starts with a short description of the others systems mentioned above: the GMDN and SNOMED CT. The Global Medical Device Nomenclature (GMDN) is a comprehensive system of internationally recognized coded descriptors in the format of preferred terms with definitions used to generically identify and characterize types of medical devices and related health care products. GMDN includes products used in the diagnosis, prevention, monitoring, treatment or alleviation of disease or injury in humans. The concept of a 'medical device' is much broader than that of an 'assistive product', and consequently the scope of GMDN is much broader than the scope of ISO 9999. Many of the assistive products included in ISO 9999 may be recognized on the market as medical devices and handled as such in accordance with applicable legislation and regulations. Other assistive products (e.g., home and environmental adaptations, general communication devices and some products relating to mobility) are not directly connected to the body or only indirectly influence body function. A product is 'assistive' if it contributes to the functioning of a person with disability. It may also improve the health of the user, in which case it may be classified as a medical device as well as an assistive product. For medical devices, dedicated legislation, with which the product has to comply, exists at the international and national level. For assistive products, no specific legislation exists and the assistive products are freely available on the consumer market. In case a product is classified as both a medical device and an assistive product, the legislation regulating medical devices applies fully. The main purpose of the GMDN is to provide a single naming system for medical devices that will support patient safety for national and international regulatory authorities, health care providers, medical device manufacturers and suppliers, conformity assessment bodies and other affiliated parties. GMDN codes indicate the generic descriptor by which a medical device can be identified; referring to GMDN's globally accepted generic medical device nomenclature. This means other devices having substantially similar generic features but originating from another source can be identified. The system allows data exchange between regular authorities and others, exchange of post-market vigilance information and inventory purposes. The GMDN is managed by the GMDN Agency, a non-profit organization. For more information on the GMDN see the website: http://www.gmdnagency.org. SNOMED CT (Systematized Nomenclature of Medicine – Clinical Terms) aimed to be a worldwide comprehensive, multilingual clinical healthcare terminology. SNOMED CT was originally created by the College of American Pathologists by combining SNOMED RT (related terms) and the UK National Health Service's Clinical Terms Version 3 (formerly known as Read Codes), a computer-based nomenclature and classification. The International Health Terminology Standards Development Organization (IHTSDO) is the organization entrusted with SNOMED CT. IHTSDO is a non-profit association that seeks to improve the health of humankind by fostering the development and use of suitable standardized clinical terminologies, notably SNOMED CT, in order to support safe, accurate, and effective exchange of clinical and related health information. The focus is on enabling the implementation of semantically accurate health records that are interoperable. SNOMED CT provides the core general terminology for the electronic health record and contains more than 311,000 active concepts with unique meanings and formal logic-based definitions organized into hierarchies. When implemented in software applications, SNOMED CT can be used to represent clinically relevant information as an integral part of producing electronic health records. The hierarchies (like clinical finding / disorder, procedure / intervention, observable entity and social context) have multiple levels of granularity. Assistive products are part of the 'physical objects' hierarchy. The subdivision of assistive products in SNOMED CT is not identical to the one presented in ISO 9999. More information on SNOMED CT can be found on the website of IHTSDO: http://www.ihtsdo.org. ISO 9999 is a product of ISO, the International Organization for Standardization (http://www.iso.org). ISO consists of technical committees (TC) and subcommittees (SC). Each subcommittee can have different working groups (WG). ISO 9999 is placed under TC173 Assistive products for persons with disability, SC2 Classification and terminology, WG11 Classification and terminology for the revision of ISO 9999. The secretariat is hosted by NEN, the Netherlands Standardization Institute. WG11 is composed of experts from China, Japan, Republic of Korea, United States of America and several European countries (Finland, France, Germany, the Netherlands, Portugal and Sweden). Short history of ISO 9999 In 1983, ISO/TC173/SC2 convened a meeting to establish an international classification system for technical aids for people with disability. The Nordic classification system, which was had been adopted in some of the Scandinavian countries in 1981 as a common classification system on technical aids for handicapped people (de Kleijn-de Vrankrijker and Valk 2003), served as the starting point for the new international classification. ISO initiated this international effort because there was a need for uniform terminology in the area of assistive products. With the increasing volume of international trade in assistive products, a classification was necessary to facilitate location and selection of technical aids and to provide a consistent basis for product information, prescription guidelines, legal documents, information systems, catalogues, administration of stocks and for surveys and the production of statistics (de Kleijn-de Vrankrijker 2002). The target audiences for the classification included consumers, governmental authorities, professionals, prescribers, and social security funds. In 1992 ISO published the first edition of ISO 9999, 'Technical aids for disabled persons – Classification' (ISO 9999: 1992) with an integral approval by CEN (European Committee for Standardization) of the new international standard as EN 29999: 1994 (de Kleijn-de Vrankrijker and Valk 2003). To adapt to the continuous innovation and expansion of the assistive product market, the classification has been revised three times since its first edition. The most recent version is the fourth edition published in 2007, 'Assistive products for persons with disability – Classification and terminology'. The next version, the fifth edition (fourth revision), will be published in 2011. The DIS (Draft International Standard) version of the fifth edition was published in February 2010. In this article we will refer to the 2007 version of ISO 9999. Definition of assistive product in ISO 9999 According to the 2007 version of ISO 9999, "an assistive product is any product (including devices, equipment, instruments, technology and software) especially produced or generally available, for preventing, compensating, monitoring, relieving or neutralizing impairments, activity limitations and participation restrictions". This definition was revised in the DIS version of the fifth edition of ISO 9999: "Any product (including devices, equipment, instruments and software), especially produced or generally available, used by or for persons with disability - for participation; - to protect, support, train, measure or substitute for body functions / structures and activities; or - to prevent impairments, activity limitations or participation restrictions." Scope of ISO 9999 Assistive products used by a person with disability, but which require the assistance of another person for their operation, are included in the classification. ISO 9999 excludes several products and services from the definition of an assistive product. These include the following: - items used for the installation of assistive products; - solutions obtained by combinations of assistive products which are individually classified in the classification; - assistive products and instruments used exclusively by healthcare professionals; - non-technical solutions and services, such as personal assistance, guide dogs or lip-reading; - implanted devices; and - financial support Relation of ISO 9999 with the WHO Family of International Classifications In 2003, ISO 9999 was accepted as a related member of the WHO Family of International Classifications (WHO-FIC). WHO-FIC is comprised of high quality classifications for relevant sectors of the health system, such as the ICD-10 (International Statistical Classification of Diseases and related health problems) and the ICF (International Classification of Functioning, Disability and Health) (Madden et al., 2007). The association of ISO 9999 with WHO-FIC provides the classification with expanded credibility on the international stage. ISO 9999 makes use of the terminology of the ICF. The ICF is a classification of health and health-related components (WHO, 2001). These components are classified from body, individual and societal perspectives. On the body level there are two classifications: a classification of body functions and a classification of body structures. For activities and participation there is a common classification. Since an individual's functioning and disability occurs in context, ICF also includes a list of environmental factors to define that context. Although personal factors are also relevant for functioning, there is not yet an international consensus about the items to be included in a list of personal factors. In Figure 1 the ICF scheme is presented. Figure 1: ICF Scheme (WHO, 2001); both with the neutral as well as the 'negative' terms An explanation of figure 1 is available. Chapter 1 'Products and technology' of the list of environmental factors of the ICF contains assistive products. Although the definition of assistive products is derived from ISO 9999, the subdivision of chapter 1 is not identical to the first level classes of ISO 9999 (see Figure 2). A proposal by ISO/TC173/SC2/WG11 to harmonize both subdivisions is in development. Figure 2: Relation between the list of environmental factors of the ICF and 1st level classes of ISO 9999 An explanation of figure 2 is available. More information on the ICF can be found on the WHO website: http://www.who.int/classifications/icf/en (application, training material, ICF and ICF-CY on line (multiple languages)). A working document indicating the relation between ISO 9999 and ICF can be found on the website of the Dutch WHO Collaborating Centre for the Family of International Classifications: http://www.rivm.nl/who-fic/ISO-9999eng.htm. Relation of ISO 9999 with other standards Where relevant product standards exist, their terminology is used in ISO 9999. These standards are included in the classification's bibliography, and are listed in Annex 1. Structure of the ISO 9999 Assistive products (including software) are classified according to their main function, such as mobility or employment. The classification consists of three hierarchical levels and the codes each consist of three pairs of digits. The first pair of digits indicates a class, the second pair of digits a subclass and the third pair of digits a division. When a class is named individually, it is indicated by only the first pair of digits (e.g., Class 12 instead of Class 12 00 00) and subclasses are indicated by two pairs of digits (e.g., Subclass 12 03 instead of Subclass 12 03 00). The numeric codes determine the positions of each class, subclass, or division in the classification. See table 1 for an overview of the classes and some examples of subclasses and divisions. Table 1: The classes of ISO 9999 and some examples of subclasses and divisions Classes (one-level classification) of ISO 9999 \|04\|\|Assistive products for personal medical treatment\| \|05\|\|Assistive products for training in skills\| \|06\|\|Orthoses and prostheses\| \|09\|\|Assistive products for personal care and protection\| \|12\|\|Assistive products for personal mobility\| \|15\|\|Assistive products for housekeeping\| \|18\|\|Furnishings and adaptations to homes and other premises\| \|22\|\|Assistive products for communication and information\| \|24\|\|Assistive products for handling objects and devices\| \|27\|\|Assistive products for environmental improvement, tools and machines\| \|30\|\|Assistive products for recreation\| Subclasses (two level) of class 12 Assistive products for personal mobility \|12 03\|\|Assistive products for walking, manipulated by one arm\| \|12 06\|\|Assistive products for walking, manipulated by both arms\| \|12 07\|\|Accessories for assistive products for walking\| \|12 12\|\|Car adaptations\| \|12 16\|\|Mopeds and motorcycles\| \|12 22\|\|Human driven wheelchairs\| \|12 23\|\|Powered wheelchairs\| \|12 24\|\|Wheelchair accessories\| \|12 31\|\|Assistive products for transfer and turning\| \|12 36\|\|Assistive products for lifting\| \|12 39\|\|Assistive products for orientation\| Divisions (three level) of subclass 12 31 Assistive products for transfer and turning \|12 31 03\|\|Sliding boards and sliding mats and turning sheets\| \|12 31 06\|\|Turntables\| \|12 31 09\|\|Free-standing rails for self-lifting\| \|12 31 12\|\|Grip ladders\| \|12 31 15\|\|Lifting belts and harnesses\| \|12 31 18\|\|Carrying chairs, harnesses and baskets\| \|12 31 21\|\|Transfer platforms\| Like other classifications, ISO 9999 provides codes, titles, explanatory notes, inclusions, exclusions and cross-references for each level of the classification. An explanation of Figure 3 is available. Besides the explanatory text and the classification itself, a table of conversion between the previous (2002) edition and the 2007 edition and an alphabetical index are provided in order to facilitate the use of and to improve the accessibility of the classification. Use of the ISO 9999 The ISO 9999 is used in several national databases of assistive products, including like AbleData (USA), Rehadat (Germany), HANDY-WIJZER (the Netherlands) and Vilatech (the Netherlands), and in financial systems from (local) government a/or health insurance. There are some attempts to add additional codes to the ISO 9999 to make it possible to describe characteristics of the assistive products in more detail (such as Cliq). AbleData provides free objective information on assistive technology and rehabilitation equipment available from domestic and international sources to consumers, organizations, professionals, and caregivers within the United States (http://www.abledata.com). AbleData is sponsored by the National Institute on Disability and Rehabilitation Research (NIDRR), part of the Office of Special Education and Rehabilitative Services (OSERS) of the U.S. Department of Education. AbleData's most significant resource is the AbleData database of assistive technology, which contains objective information on more than 37,000 assistive products (over 23,000 of which are currently available). For each product, a detailed description of the product's functions and features, price information (when available), and contact information for the product's manufacturer and/or distributors are provided. AbleData's product information is updated daily, and its staff add over 1,200 new products each year. Listing products on AbleData is free for assistive technology companies. The AbleData website receives over 260,000 visitors and over 900,000 page views each month. AbleData has maintained an independent classification system for over 20 years; however, it recently began to link that system to ISO 9999 so that AbleData's users will be able to locate product information using ISO 9999. Rehadat 'Information system on vocational rehabilitation' is an information system supporting the vocational integration of disabled persons that has been commissioned by the German Federal Ministry of Labour and Social Affairs and was established by the Cologne Institute for Economic Research (http://www.rehadat.de/rehadat/eng/). The database structure is based on ISO 9999 and can be searched in German or English. The descriptions of the products are available in English (translated manually or by translation software). Documenting more than 20,000 products, the database contains most of the technical aids for disabled persons that are available in Germany. The range of technical aids includes aids for housekeeping, orthoses and prostheses, and adapted machines and tools. Rehadat includes entries detailing each product's manufacturer, distributor and price, and it provides technical descriptions and information on the reimbursement of costs by health insurance organisations. Contact details for manufacturers or distributors include the e-mail and Web addresses of the companies as links that can be accessed directly from the database. Graphics and pictures supplement many documents. HANDY-WIJZER is a freely accessible website on assistive products from the Dutch company HANDY-BRAINS. It uses the Dutch language and is visited by users of assistive products as well as professionals mainly in the Netherlands and Belgium. The system is supported by financial contributions from participating companies that put commercially oriented information on their products and services on the website. The product information includes functional and technical characteristics, dimensions, compliance with international standards, available options and product variants, and pictures. The products are classified by HANDY-BRAINS following ISO 9999 with national additions (see Cliq) in order to specify in depth the intended use of the assistive product. Additionally, HANDY-BRAINS adds - in general and on the product level - information on law and legislation, policy and future-oriented topics, and developments in the wide field of assistive products. HANDY-BRAINS also performs quality control and checks the internal consistency of product information originating from different sources. The information may be retrieved using ISO 9999 or through free text search on key terms that include the intended use and specific user of the assistive product. The site is updated every three months with new products and other information and has early 2010 about 40.000 new visitors per month which have a look at up to 200.000 pages. For more information, go to http://www.handy-wijzer.nl or http://www.handy-wijzer.be (only in Dutch). Vilatech 'hulpmiddelenwijzer' is a Dutch database with independent information on assistive products for people with disability or chronic disease and elderly people. In three steps, people get an overview of the assistive products that might be useful for them. After selecting one or more products, more detailed information can be found, including a description of the functionality of the product(s), technical details, reimbursement systems from (local) government and/or health insurance, manufacturers and price. People can search the database by defining 'activity', 'environment in which the product will be used' and 'solution' or by using free text words. The subdivision of products is based on ISO 9999. For more information, visit http://www.vilatechdatabank.nl or http://www.vindeenhulpmiddel.nl (only in Dutch). Cliq (Classification with IQ) is derived from ISO 9999, providing more detailed categories than the original classification. It was developed in the Netherlands. To the original six-digit codes (3 pairs of two digits) of ISO 9999, a maximum of six extra digits (three pairs of two digits) are added. With these additional digits, the 'product related intended use' of the products can be described. 'Product related intended use' is a legal term that indicates what the user can and may expect of the assistive product. It encompasses the following characteristics: - functionality: activities (indirectly participation) for which the assistive product can be used (such as standing and work) and functions and structures supported by the product (such as respiration and a joint's range of motion) - technical characteristics - user friendliness - external and cosmetic features (like color) - other characteristics It is important to match the wishes of the user with respect to the assistive product (human related intended use) (coded in ICF terms) to the characteristics of the products (product related intended use) (in Cliq codes). As indicated, the fifth version of ISO 9999 will be published in 2011. Plans for the sixth version (to be published in 2015) include harmonization of the classification with ICF and the addition of a fourth level. Proposals for changes or additions to the ISO 9999, to revise existing classes/subclasses/divisions or propose new ones, in accordance with the rules of the classification, may be submitted to a national member body of ISO with an accompanying explanation of the proposal. More information on ISO 9999 More information can be found on the ISO website: http://www.iso.org. In the online bookstore, the ISO 9999 can be ordered in English and French. For other languages, the national standardization organization of the respective country can be consulted. For example, the Dutch version is available at the Netherlands Standardization Institute (NEN) (http://www.nen.nl). Annex: Standards used in ISO 9999 - ISO 1087-1, Terminology work — Vocabulary — Part 1: Theory and application - ISO 1087-2, Terminology work — Vocabulary — Part 2: Computer applications - ISO 6440, Wheelchairs — Nomenclature, terms and definitions - ISO 8549-1, Prosthetics and orthotics — Vocabulary — Part 1: General terms for external limb prostheses and external orthoses - ISO 8549-2, Prosthetics and orthotics — Vocabulary — Part 2: Terms relating to external limb prostheses and wearers of these prostheses - ISO 8549-3, Prosthetics and orthotics — Vocabulary — Part 3: Terms relating to external orthoses - ISO 8669-1, Urine collection bags — Part 1: Vocabulary - ISO 8670-1, Ostomy collection bags — Part 1: Vocabulary - ISO 9949-2, Urine absorbing aids — Vocabulary — Part 2: Products - ISO 9949-3, Urine absorbing aids — Vocabulary — Part 3: Identification of product types - ISO 10535, Hoists for the transfer of disabled persons — Requirements and test methods - ISO 11199-1, Walking aids manipulated by both arms — Requirements and test methods — Part 1: Walking frames - ISO 11199-2, Walking aids manipulated by both arms — Requirements and test methods — Part 2: Rollators - ISO 11334-1, Walking aids manipulated by one arm — Requirements and test methods — Part 1: Elbow crutches - ISO 11334-4, Walking aids manipulated by one arm — Requirements and test methods — Part 4: Walking sticks with three or more legs - ICF, WHO, Geneva, 2001, International Classification of Functioning, Disability and Health - EN 12182:1999, Technical aids for disabled persons — General requirements and test methods	3	11	2	0	0	0	0.508961	2	5,060
Malformations of the middle ear are classified as minor and major malformations. Minor malformations appear with regular external auditory canal, tympanic membrane and aerated middle ear space. The conducting hearing loss is due to fixation or interruption of the ossicular chain. The treatment is surgical, following the rules of ossiculoplasty and stapes surgery. In major malformations (congenital aural atresia) there is no external auditory canal and a deformed or missing pinna. The mastoid and the middle ear space may be underdevelopped, the ossicular chain is dysplastic. Surgical therapy is possible in patients with good aeration of the temporal bone, existing windows, a near normal positioned facial nerve and a mobile ossicular chain. Plastic and reconstructive surgery of the pinna should proceed the reconstruction of the external auditory canal and middle ear. In cases of good prognosis unilateral aural atresia can be approached already in childhood. In patients with high risk of surgical failure, bone anchored hearing aids are the treatment of choice. Recent reports of implantable hearing devices may be discussed as an alternative treatment for selected patients. PMID:22073077 Objective. To explore the strange beliefs and misconceptions related to the ear and its malformations, and how these have changed from ancient times until today.Methods. Ancient documents, journal articles, and history books were studied to research ancient and current beliefs and misconceptions with regard to the ear and its malformations.Results. The ear has been the centre of various beliefs and Irene E Gamatsi; Thomas P Nikolopoulos; Dimitra E Lioumi Summary Malformations of bony inner ear are rare anomalies occurring in approximately 20% of patients with congenital sensorineural hearing loss. Conductive hearing loss is usually associated with abnormalities of the external and middle ear. Recent reports of patients with lateral semicircular canal malformations indicate inner ear malformations to be associated with sensorineural or conductive hearing loss. Differential diagnosis of conductive hearing loss should include otosclerosis, isolated ossicular deformities, inner ear anomalies or a combination of these. In this report, a case is described with right vestibule-lateral semicircular canal dysplasia presenting at our centre with bilateral otosclerosis. De Stefano, A; Dispenza, F; Aggarwal, N; Russo, A The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl. Hearing impairment was identified at the age of three. She was referred to our university hospital in April 2005. Pure-tone audiogram showed conductive hearing impairments. Computed tomography (CT) revealed the incudo-stapedial disconnections in both ears. The exploratory tympanotomies on the right and left ears were performed in May and July 2005, respectively. The surgical findings showed absence of the long process and presence of the lenticular process of the incus in both ears. After the reconstructions of ossicular chain, the hearing of both ears improved. Case 2 was an 11-year-old girl. The hearing impairment of the right ear was identified in May 2008. She was referred to our university hospital three months later. Pure-tone audiogram showed the conductive hearing impairment in the right ear. CT revealed the incudo-stapedial disconnection in the right ear. The surgery showed the same findings as those of case 1. Anomalies of both cases suggest that the lenticular process of the incus and the stapes originate from a common primordium. PMID:24355584 Kidowaki, Naoko; Kamitani, Toru; Nakamura, Takashi; Taki, Masakatsu; Sakaguchi, Hirofumi; Suzuki, Toshihiro; Hisa, Yasuo Four cases with hereditary combinations of branchial fistulas and middle ear malformations in three generations of one family are reported. We found preauricular fistulas and lateral cervical fistulas combined with different types of middle ear dysplasias. In one case a preauricular fistula running through the middle ear and ending in the dura could be exstirpated. Such rare varieties must be considered, when preauricular fistulas are found together with congenital middle ear deafness. PMID:763058 Pau, H W; Koch, U The objective of this study was to assess the auditory performance of the neural structures in response to controlled electrical stimulation period. A prospective cohort study focused on the intracochlear electrical stimulation parameters and hearing performance of patients suffering different cochlear malformations who were treated by cochlear implants constituted the study design. The study sample constituted 16 patients, suffering profound prelingual hearing impairment, diagnosed on the basis of radiological criteria as having an inner ear malformation, and who underwent cochlear implantation and were followed for 24 months. Patients with common cavities, characterized by fewer nerve structures involved, less epithelial penetration, and deficient cochlear tonotopy distribution showed have higher thresholds and electrical charges than patients with cochlear hypoplasia, who in turn have higher thresholds than patients with minor malformations (p < 0.05). Furthermore, word perception was severely compromised in patients with a common cavity malformation and was also poor in patients with cochlear hypoplasia, who were unable to discriminate more than 50% of the words and relied on visual cues as a necessary aid to communication. Better results were reached by minor malformed inner ears. To conclude, the number of nerve structures involved, epithelial penetration and deficient cochlear tonotopy are responsible of inner ear functionality. PMID:21990053 Sainz, Manuel; Garcia-Valdecasas, Juan; Fernandez, Elena; Pascual, Maria Teresa; Roda, Olga Using a combination of in vivo computerized tomography and histological staining, a middle ear anomaly in two wild-caught American bullfrogs (Rana catesbeiana) is characterized. In these animals, the tympanic membrane, extrastapes, and pars media (shaft) of the stapes are absent on one side of the head, with the other side exhibiting normal morphology. The pars interna (footplate) of the stapes and the operculum are present in their normal positions at the entrance of the otic capsule on both the affected and unaffected sides. The pattern of deformity suggests a partial failure of development of tympanic pathway tissues, but with a preservation of the opercularis pathway. While a definitive proximate cause of the condition could not be determined, the anomalies show similarities to developmental defects in mammalian middle ear formation. Horowitz, Seth S.; Simmons, Andrea Megela; Ketten, Darlene R. We present a 4-year-old girl with congenital profound sensorineural deafness associated with inner ear malformation (incomplete partition type II, enlarged vestibule, and enlarged vestibular aqueduct). The proposita also had pseudocleft lips, skin defects, auricle abnormalities, and unilateral multicystic dysplastic kidney, leading to the diagnosis of branchio-oculo-facial (BOF) syndrome. Mutation analysis of the TFAP2A gene showed a de novo deletion of 18 and insertion of 6 nucleotides, resulting in deletion of amino acids LPGARR and insertion of RI between amino acids 276 and 281. Altered amino acids are located within the basic DNA binding and dimerization domains of TFAP2A. Previously reported amino acid substitutions in TFAP2A involved only DNA binding domain in four patients with BOF syndrome who were not reported to have profound sensorineural deafness. Our report implies that the localization of mutations in TFAP2A might be responsible with the phenotypic findings in BOF syndrome. PMID:19206157 Tekin, Mustafa; Sirmaci, Asli; Yüksel-Konuk, Berrin; Fitoz, Suat; Sennaro?lu, Levent Frog malformations have been reported from 42 states. The broad geographic distribution of these malformations warrants national attention. Scientists at the USGS National Wildlife Health Center in Madison, Wisconsin are studying this problem in an effort to document its scope and to determine the causes of the observed malformations. National Wildlife Health Center In this article, the author describes an art project wherein students make fanciful connections between art and medicine. This project challenges students to interpret "ear idioms" (e.g. "blow it out your ear," "in one ear and out the other") by relying almost entirely on realistic ear drawings, the placement of them, marks, and values. In that… This activity (on page 2 of the PDF) is a full inquiry investigation into using indicator species to assess the health of an environment. Groups of learners will collect as many frogs as they can from a pond, looking for malformations, which will be documented and further sorted as either symmetrical or asymmetrical. This data will then be graphed to identify a prevalent trend and possible cause of malformations. Relates to linked video, DragonflyTV: Malformed Frogs. Twin Cities Public Television, Inc. Arteriovenous malformations are serious high-flow vascular malformations. Four progressive stages have been described: dormancy, expansion, destruction and heart failure. Progression from one stage to another is not systematic but depends on events - physiological or traumatic, sometimes iatrogenic. Pulsed Doppler imaging of venous waveforms and magnetic resonance imaging (MRI) are the most informative examinations for both diagnosis and follow-up of arteriovenous malformations.Arteriography and angio-MRI help guide treatment decisions. Treatment of the malformation must not be envisioned until it reaches a symptomatic stage. It most often combines an endovascular procedures and wide surgical excision. A syndromic form must be considered in cases of systemic angioma. PMID:20206460 Naouri, Michael; Lorette, Gérard; Barbier, Charlotte; Zakine, Gilbert; Herbreteau, Denis Presents an activity in which students design, construct, and test "super ears" to investigate sound and hearing. Students work in groups of three and explore how the outer ear funnels sound waves to the inner ear and how human hearing compares to that of other animals. (NB) A new class of biometrics based upon ear features is introduced for use in the development of passive identification systems.\\u000a The availability of the proposed biometric is shown both theoretically in terms of the uniqueness and measurability over time\\u000a of the ear, and in practice through the implementation of a computer vision based system. Each subject’s ear is modeled as Mark Burge; Wilhelm Burger Anorectal malformations (ARM) are the result of an abnormal development of the terminal part of the digestive tract interesting anus and/or rectum that occur early between the sixth and tenth week of embryonic development. They carry a malformation spectrum of severity depending on the level of disruption of the anorectal canal and of the associated caudal malformations (sacrum and spine). ARM are associated in over half the cases with other malformations that can be integrated in some cases in known syndromes. If surgical treatment to restore anatomy as normal as possible is indispensable, post-operative care is essential for these patients whose defecation mechanisms are altered, to reach if not continence, at least a socially acceptable cleanliness. PMID:23992833 Cretolle, C; Rousseau, V; Lottmann, H; Irtan, S; Lortat-Jacob, S; Alova, I; Michel, J L; Aigrain, Y; Podevin, G; Lehur, P A; Sarnacki, S Introduction Ear wax only becomes a problem if it causes a hearing impairment or other ear-related symptoms. Ear wax is more likely to accumulate and cause a hearing impairment when normal extrusion is prevented — for example, by the use of hearing aids, or by the use of cotton buds to clean the ears. Ear wax can visually obscure the ear drum, and may need to be removed for diagnostic purposes. Methods and outcomes We conducted a systematic review and aimed to answer the following clinical question: What are the effects of methods to remove ear wax? We searched: Medline, Embase, The Cochrane Library, and other important databases up to June 2007 (Clinical Evidence reviews are updated periodically; please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA). Results We found nine systematic reviews, RCTs, or observational studies that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions. Conclusions In this systematic review, we present information relating to the effectiveness and safety of the following interventions: ear syringing; manual removal (other than ear syringing); and wax softeners (alone or prior to syringing). This activity (page 2 of the PDF) is a full inquiry investigation into animal behavior and communication. Groups of learners will fashion a headband with fake ears, similar in shape to those of the animal they are going to observe. Then, they record observations of the animalâs reactions when a learner, wearing the ears in different positions, brings it a snack. Learners develop categories of behavior to organize and evaluate the results. Safety Note: an adult handler must be present if working with a horse or even a large dog. Relates to linked video, DragonflyTV: Horse Ears. Twin Cities Public Television, Inc. Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, Marc A Levitt; Alberto Peña Spindly cells in the inner ear, called "hair" cells, are critical for both hearing and balance. Now, in a boon for research, neuro-scientists Jeffrey Corwin and Zhenqing Hu at the University of Virginia School of Medicine have finally grown and multiplied these cells in the lab. Science Update (AAAS;) Using these websites, you can practice your ear training skills. There are links for intervals, chords, and short melodic segments. First, you should go to this website to practice intervals. Intervals Begin with the first option (simple intervals up). After mastering this, move to simple intervals down. As you become more advanced, continue down the line of interval options. They will become more difficult as you ... Superficial venous malformations are part of a larger group now called superficial vascular anomalies and previously known as angiomas. These include vascular tumors or infantile hemangioma, low-flow vascular malformations (capillary, lymphatic, and superficial venous malformations) and high-flow vascular malformations (arteriovenous malformations). Some of these lesions are complex. This classification facilitates their multidisciplinary therapeutic management. Embolization is an effective curative treatment for malformations of small and intermediate size. The objective for large or complex malformations is to restore anatomic function and improve cosmetic results. PMID:20304597 Barbier, Charlotte; Martin, Arnaud; Papagiannaki, Chrisanti; Cottier, Jean-Philippe; Lorette, Gérard; Herbreteau, Denis Anorectal malformations comprise a wide spectrum of diseases, which can affect boys and girls, and involve the distal anus and rectum as well as the urinary and genital tracts. They occur in approximately 1 in 5000 live births. Defects range from the very minor and easily treated with an excellent functional prognosis, to those that are complex, difficult to manage, are often associated with other anomalies, and have a poor functional prognosis. The surgical approach to repairing these defects changed dramatically in 1980 with the introduction of the posterior sagittal approach, which allowed surgeons to view the anatomy of these defects clearly, to repair them under direct vision, and to learn about the complex anatomic arrangement of the junction of rectum and genitourinary tract. Better imaging techniques, and a better knowledge of the anatomy and physiology of the pelvic structures at birth have refined diagnosis and initial management, and the analysis of large series of patients allows better prediction of associated anomalies and functional prognosis. The main concerns for the surgeon in correcting these anomalies are bowel control, urinary control, and sexual function. With early diagnosis, management of associated anomalies and efficient meticulous surgical repair, patients have the best chance for a good functional outcome. Fecal and urinary incontinence can occur even with an excellent anatomic repair, due mainly to associated problems such as a poorly developed sacrum, deficient nerve supply, and spinal cord anomalies. For these patients, an effective bowel management program, including enema and dietary restrictions has been devised to improve their quality of life. Levitt, Marc A; Pena, Alberto Objectives. To investigate the best time of examination and section chosen of routine prenatal ultrasound screening for external ear abnormalities and evaluate the feasibility of examining the fetal external ear with ultrasonography. Methods. From July 2010 until August 2011, 42118 pregnant women with single fetus during 16–40 weeks of pregnancy were enrolled in the study. Fetal auricles and external auditory canal in the second trimester of pregnancy were evaluated by routine color Doppler ultrasound screening and systematic screening. Ultrasound images of fetal external ears were obtained on transverse-incline view at cervical vertebra level and mandible level and on parasagittal view and coronal view at external ear level. Results. Five fetuses had anomalous ears including bilateral malformed auricles with malformed external auditory canal, unilateral deformed external ear, and unilateral microtia. The detection rate of both auricles was negatively correlated with gestational age. Of the 5843 fetuses undergoing a routine ultrasound screening, 5797 (99.21%) had bilateral auricles. Of the 4955 fetuses following systematic screening, all fetuses (100%) had bilateral auricles. The best time for fetal auricles observation with ultrasonography is 20–24 weeks of pregnancy. Conclusions. Detection of external ear abnormalities may assist in the diagnosis of chromosomal abnormalities. Wei, Jun; Ran, Suzhen; Yang, Zhengchun; Lin, Yun; Tang, Jing The rate of malformed children in Utah of 11.7 per 1,000 liver births, derived from 128,857 birth certificates, ws not high compared with other non-Utah studies. Rates of selected malformations also were not high. The rate of malformed children varied by county of residence. San Juan County reported the highest percentage of mothers receiving late or infrequent prenatal care, the lowest mean level of public education, and the highest rate of malformed children in the state. The rate was not significantly associated with the large population of Indians residing in that county since by controlling for residence, the variation by race was eliminated. The overall rate was positively associated with maternal age rimarily due to an increased frequency of Down's syndrome. The impact of the "maternal age effect" on the state malformation rate, however, was not large. By controlling for maternal age, the slight association between increased rate of malformed children and increasing birth order was eliminated. The rate of malformed children was higher for parents having a low level of education, infrequent prenatal care, or who were not married. There was also a strong negative association of birth weight with the rate of malformation. Analysis of rates of selected malformations suggested that the low birth weight was a sequela to intrauterine growth retardation caused by severe congenital malformation. The validity and etiologic implications of these results await further investigation. PMID:6449752 Seegmiller, R E; Hansen, W N Arnold-Chiari malformation is a congenital malformation of the skull near the foramen magnum in which the cerebellum and the medulla are caudally displaced. This herniation of the brainstem causes down-beat nystagmus and oscillopsia, the most commonly presenting sign and symptom, respectively. Differential diagnoses for the Arnold-Chiari malformation include, but are not limited to, demyelinating disease, tumor, and vascular disorders. Symptoms will generally worsen with time and may even be brought on during exercise or valsalva maneuvers. A correct diagnosis can lead to timely surgical intervention which can improve the quality of eye movements. Treatment generally involves the surgical decompression of the surrounding spinal tissue. PMID:1565422 Russell, G E; Wick, B; Tang, R A The external ear is exposed to weathering and trauma; it also has sparse vascularity, making it prone to infection and disease. The external location of the cutaneous ear makes it easily visible for diagnosis and accessible for treatment. In this article, the authors focus on diseases of the ear that are most commonly encountered and may be subject to surgical and medical evaluation and/or treatment. Epidemiology, pathogenesis, clinical course, and treatment for each disease entity are discussed. Orengo, Ida; Robbins, Kerri; Marsch, Amanda Development of the mammalian inner ear requires coordination of cell proliferation, cell fate determination and morphogenetic movements. While significant progress has been made in identifying developmental signals required for inner ear formation, less is known about how distinct signals are coordinated by their downstream mediators. Members of the Rac family of small GTPases are known regulators of cytoskeletal remodeling and numerous other cellular processes. However, the function of Rac GTPases in otic development is largely unexplored. Here, we show that Rac1 and Rac3 redundantly regulate many aspects of inner ear morphogenesis. While no morphological defects were observed in Rac3-/- mice, Rac1CKO; Rac3-/- double mutants displayed enhanced vestibular and cochlear malformations compared to Rac1CKO single mutants. Moreover, in Rac1CKO; Rac3-/- mutants, we observed compromised E-cadherin-mediated cell adhesion, reduced cell proliferation and increased cell death in the early developing otocyst, leading to a decreased size and malformation of the membranous labyrinth. Finally, cochlear extension was severely disrupted in Rac1CKO; Rac3-/-mutants, accompanied by a loss of epithelial cohesion and formation of ectopic sensory patches underneath the cochlear duct. The compartmentalized expression of otic patterning genes within the Rac1CKO; Rac3-/- mutant otocyst was largely normal, however, indicating that Rac proteins regulate inner ear morphogenesis without affecting cell fate specification. Taken together, our results reveal an essential role for Rac GTPases in coordinating cell adhesion, cell proliferation, cell death and cell movements during otic development. Grimsley-Myers, Cynthia M.; Sipe, Conor W.; Wu, Doris K.; Lu, Xiaowei Diffuse cerebral arteriovenous malformation (AVM) is a rare disorder of the brain and defined as diffuse infiltration of brain by complex vascular structures. It is usually associated with hereditary syndromes and presented with hemorrhage or seizure. We report a 20-year-old male patient who presented with drooping of the left eyelid. He had no skin lesion. The ophthalmological examination was within normal limits except periorbital bruit on oscultation. Radiological examination revealed a diffuse AVM comprising multiple arteriovenous shunts, draining bihemispherically through numerous dilated veins but without a typical arteriovenous malformation nidus. No hereditary disorder was detected. No treatment was performed and the patient is still under follow-up. PMID:20963707 Solmaz, Ilker; Secer, Halil Ibrahim; Tehl?, Ozkan; Tem?z, Caglar; Izc?, Yusuf; Gonul, Engin The malformations of the cerebral cortex represent a major cause of developmental disabilities, severe epilepsy and reproductive disadvantage. The advent of high-resolution MRI techniques has facilitated the in vivo identification of a large group of cortical malformation phenotypes. Several malformation syndromes caused by abnormal cortical development have been recognised and specific causative gene defects have been identified. Periventricular nodular heterotopia Renzo Guerrini; Carla Marini Background: We undertook this study to characterize those patients who required reoperations for Chiari malformation and to determine whether modifications in surgical technique at the initial procedure might have obviated the need for repeat surgery. Methods: We reviewed the hospital records, imaging studies, operative reports, and follow-up data of those patients who were undergoing a second operation as part of David Sacco; R. Michael Scott With the advent of newer imaging techniques, the radiologist is now able to make very precise and accurate diagnoses of congenital uterine malformations and their complications. Because these anomalies are associated with reproductive dysfunction, they are often discovered during an infertility evaluation. By imaging parallel to the long axis of the uterus, the external contour can be evaluated, obviating laparoscopy for differentiating septate from bicornuate uteri. Obstructed uterovaginal anomalies (e.g., hematometros, hematometrocolpos), an important complication of abnormal müllerian duct development, can occur at any time from the newborn period to adulthood. Determining the site of obstruction is imperative for planning the proper surgical approach. To understand these malformations better, we review the relevant embryology. The most widely accepted classification scheme is discussed in detail, with an emphasis on diagnosis, prognosis, and therapeutic options. PMID:8536487 Woodward, P J; Sohaey, R; Wagner, B J One hundred sixty-five middle ear procedures were performed with an endoscope, a camera, and a video monitor instead of the microscope. The endoscope offers the following advantages: 1) it visualizes the whole tympanic membrane and the ear canal without having to manipulate the patient's head or the microscope, 2) it extends the operative field in transcanal procedures into structures usually hidden from the microscope (anterior tympanic perforation, posterior retraction pocket, facial recess, and hypotympanum), and 3) it visualizes structures from multiple angles as opposed to the microscope's single axis along the ear canal. Disadvantages of the endoscope include the one-handed surgical technique, a loss of depth perception, limited magnification, and the need for training. The endoscope holds the greatest promise in tympanoplasty and cholesteatoma surgery and should increase the utilization of transcanal over postauricular procedures. PMID:9930539 The prevalence of müllerian malformations is 1 in 200, or 0.5%. A third of the anomalies are septate, a third bicornuate uteri, 10% arcuate uterus, 10% didelphis and unicornuate uterus, and < 5% uterine and vaginal aplasia. Correct diagnosis of the malformation is most important but often very difficult. Correct treatment can only be performed if the malformation is clear. Longitudinal vaginal septums have to be removed due to potential obstetric problems. Transverse vaginal septums can cause hematocolpos and pain and have to be incised crosswise and excised so as not to shorten the vagina at the same time. Congenital vaginal agenesis occurs in Mayer-Rokitansky-Kuster-Hauser syndrome patients and in androgen insensitivity syndrome. The first choice for surgical treatment should be the new laparoscopic-assisted creation of a neovagina. Septate uterus has to be distinguished from a bicornuate uterus. Even if it is not proven to be a cause for infertility, the chance of miscarriage can be diminished by performing hysteroscopic metroplasty. Repair of a uterine septum in infertility patients often improves pregnancy rates. In contrast, surgical repair of a bicornuate uterus requires an abdominal metroplasty. This should only be performed if the patient has recurrent fetal loss due to the uterine structural defect. In a unicornuate uterus it is most important to determine if there is a second uterine horn that can cause cyclic pain if it has functioning endometrium. The only surgical option in these cases is to remove the rudimentary uterus with endometrium and hematometra, respectively. PMID:21437824 Brucker, Sara Yvonne; Rall, Katharina; Campo, Rudi; Oppelt, Peter; Isaacson, Keith Cholesteatoma is a hazardous condition because of the erosion and tissue destruction, which result in deafness, and the complications which threaten life. Early diagnosis and treatment provide the best opportunity for eradication of the disease and preservation of hearing. The patient usually complains of intermittent or persistent ear drainage and of diminished hearing acuity. Close examination of the tympanic membrane reveals a perforation, which at times may be quite small, with epithelial extension into the middle ear space. In most instances surgical intervention is necessary for eradication of the disease. ImagesFigure 1. Thomas, Gary L. Summary 5,376 consecutive births were examined for the presence of congenital malformations, to find out its incidence and to determine\\u000a the role of environmental factors in the causation of these anomalies.\\u000a \\u000a The incidence was 13.76 per one thousand births. Males dominated among the malformed infants with an incidence of 67.56 per\\u000a cent. The maximum number of malformed children were born to K. K. Khanna; L. S. N. Prasad Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. PMID:24093332 Lambru, Giorgio; Miller, Sarah; Matharu, Manjit S In this paper Doreen Kimura gives a personal history of the "right-ear effect" in dichotic listening. The focus is on the early ground-breaking papers, describing how she did the first dichotic listening studies relating the effects to brain asymmetry. The paper also gives a description of the visual half-field technique for lateralized stimulus… Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. This paper proposes a robust ear identification system which is developed by fusing SIFT features of color segmented slice regions of an ear. It makes use of Gaussian mixture model (GMM) to build ear model with mixture of Gaussian using vector quantization algorithm and K-L divergence is applied to the GMM framework for recording the color similarity in the specified Dakshina Ranjan Kisku; S. Gupta; P. Gupta; J. K. Sing A class of biometrics based upon ear features is introduced for use in the development of passive identification systems. The viability of the proposed biometric is shown both theoretically in terms of the uniqueness and measurability over time of the ear, and in practice through the implementation of a computer vision based system. Each subject's ear is modeled as an Mark Burge; Wilhelm Burger Arteriovenous malformations (AVM) are rare vascular lesions that can present with a myriad of clinical presentations. In our institutions, initial workup consists of a clinical exam, color Doppler imaging, and magnetic resonance imaging. After the initial noninvasive workup, arteriography, at times closed system venography, and ethanol endovascular repair of the AVM is performed under general anesthesia. Depending on the size of the lesion, additional Swan-Ganz line and arterial line monitoring are performed. Patients are usually observed overnight and uneventfully discharged the following day if no complication occurs. Patients are followed at periodic intervals despite cure of their lesion. Long-term follow-up is essential in AVM management. Yakes, Wayne F. [Interventional Radiology and Interventional Neuroradiology, Radiology Imaging Associates, P.C., Colorado NeurologicalInstitute, Swedish Medical Center, 501 E. Hampden Avenue, Englewood, CO 80110 (United States); Rossi, Plinio [Department of Radiology, Universita Degli Studi Di Roma, 'La Sapienza', 00161 Rome (Italy); Odink, Henk [Department of Radiology, De Wever Hospital, Henri Dunanstrat 5, 6419 PC Heerlen (Netherlands) Drug delivery to the ear is used to treat conditions of the middle and inner ear such as acute and chronic otitis media, Ménière's disease, sensorineural hearing loss and tinnitus. Drugs used include antibiotics, antifungals, steroids, local anesthetics and neuroprotective agents. A literature review was conducted searching Medline (1966-2012), Embase (1988-2012), the Cochrane Library and Ovid (1966-2012), using search terms 'drug delivery', 'middle ear', 'inner ear' and 'transtympanic'. There are numerous methods of drug delivery to the middle ear, which can be categorized as topical, systemic (intravenous), transtympanic and via the Eustachian tube. Localized treatments to the ear have the advantages of targeted drug delivery allowing higher therapeutic doses and minimizing systemic side effects. The ideal scenario would be a carrier system that could cross the intact tympanic membrane loaded with drugs or biochemical agents for the treatment of middle and inner ear conditions. PMID:23323784 Hoskison, E; Daniel, M; Al-Zahid, S; Shakesheff, K M; Bayston, R; Birchall, J P Human ear is a new class of relatively stable biometrics that has drawn researchers' attention recently. In this paper, we propose a complete human recognition system using 3D ear biometrics. The system consists of 3D ear detection, 3D ear identification, and 3D ear verification. For ear detection, we propose a new approach which uses a single reference 3D ear shape Hui Chen; Bir Bhanu Uterine arteriovenous malformation (AVM) is a little known condition of which, to date, very few cases have been described. It has a very diverse symptomatology, even though in most cases, it is diagnosed during a severe and acute haemorrhagic event. Its treatment can vary from expectant management to hysterectomy; however, current evidence suggests that the embolisation of uterine arteries is the most effective approach, especially if fertility is to be preserved. We present a case report classified as AVM, with additional images that show the appearance of this pathology in a short span of time. This case has a number of peculiarities: unusual persistence of human chorionic gonadotropin hormone (?-HCG), asymptomatic patient, quick establishment of the lesion and its duration with unchanging characteristics and finally its spontaneous resolution without further consequences. This entity shows an aetiopathogenesis, that is, not well established or described. We discuss its physiopathology and aetiopathogenesis. PMID:23396842 Sellers, Francisco; Palacios-Marqués, Ana; Moliner, Belen; Bernabeu, Rafael Background: The diagnosis and management of Chiari I malformations (CMI) remains controversial, particularly since it is often an incidental finding on cervical MR scans performed for neck pain and/or headaches. Recently, some surgeons “over-operated” on asymptomatic patients with Chiari I malformations, or even on those without the requisite radiographic diagnostic features for Chiari I malformations: unfortunately, only a subset were admonished for indiscriminate surgery. Nevertheless, when this hindbrain malformation is truly symptomatic, contributing to impaired cerebrospinal fluid (CSF) circulation, various valid surgical management strategies may be adopted. Methods: This review focuses on the current literature regarding the clinical presentation, diagnosis, and surgical management of Chiari I malformation. Variations in the surgical technique are also presented and critiqued. Results: The recommended treatment for Chiari I malformations I consists of decompressive suboccipital craniectomy and duroplasty when abnormal cine-flow MRI is observed preoperatively and blockage of CSF flow persists intraoperatively despite bony decompression. Conclusions: Controversy continues regarding the optimal surgical technique to address Chiari malformations I. Proper diagnostic studies and patient selection are needed to optimize patient outcomes, while avoiding unnecessary surgical procedures. Today, the designation "capillary malformation" is widely used as a modern name for what was formerly called a nevus flammeus or port-wine stain. This new terminology, however, is inaccurate and ambiguous. There are at least nine different skin disorders fulfilling the criteria of a capillary malformation. Examples include nevus anemicus, cutis marmorata telangiectatica congenita, angiokeratoma circumscriptum, and several vascular lesions that, in the author's view, do not represent nevi, such as the nuchal or glabellar salmon patch and the cutaneous changes of Rendu-Osler disease. Hence, I propose that we should use "capillary malformation" as an umbrella term and not as a name for a specific cutaneous entity. PMID:19022106 The purpose of this article is to give an overview of the cerebral and spinal cord pediatric malformations; we particularly describe three of them: Vein of Galen arteriovenous Malformation, Pial Arteriovenous Malformation, and Dural Sinus Malformation. We report the experience of Bicêtre since 1981 to 2003, with 317 VGAM, 302 Pial AVM and 30 DSM. We describe natural history, clinical A. Ozanne; H. Alvarez; T. Krings; P. Lasjaunias This game depicts a woman going through her day, faced with various loud sounds. In each situation, she has the choice of how to deal with the loud sound: Ear Plugs, Turn It Down, Move Away, or Do Nothing. You only have 3 of each choice available, symbolized by cards on screen. Your goal is to reach the end of the game with undamaged hearing and one set of earplugs, so you can enjoy an awesome rock concert. After each decision, you find out whether your hearing was damaged and if you made the best choice. Background: Uterine arteriovenous malformation is a rare but potential life-threatening source of bleeding. A high index of suspicion and accurate diagnosis of the condition in a timely manor are essential because instrumentation that is often used for other sources of uterine bleeding can be lead to massive hemorrhage. Case: We describe here a case of uterine arteriovenous malformation. A 32-year-old woman presented abnormal vaginal bleeding following the induced abortion. A diagnosis of uterine arteriovenous malformation made on the basis of Doppler ultrasonraphy was confirmed through pelvic angiography. The embolization of bilateral uterine arteries was performed successfully. Conclusion: Uterine arteriovenous malformation should be suspected in patient with abnormal vaginal bleeding, especially who had the past medical history incluing cesarean section, induced abortion, or Dillation and Curethage and so on. Although angiography remains the gold standard, Doppler ultrasonography is also a good noninvasive technique. The transcatheter uterine artery embolization offers a safe and effective treatment Chen, Yan; Wang, Guoyun; Xie, Fubo; Wang, Bo; Tao, Guowei; Kong, Beihua \\u000a Abstract\\u000a Background Congenital pancreaticobiliary malformations are sometimes associated with acute or chronic pancreatitis and biliary carcinoma. Currently, MRCP is one of the first choices for investigating and diagnosing pancreaticobiliary diseases noninvasively. We compared the accuracy of conventional MRCP and ERCP in making the diagnosis of congenital pancreaticobiliary malformations.Methods In patients with pancreas divisum (n = 17), pancreaticobiliary maljunction (n = 12), choledochocele (n = 2) and annular pancreas T. Kamisawa; Y. Tu; N. Egawa; K. Tsuruta; A. Okamoto; N. Kamata A classification for congenital malformations of dermatoglyphs is presented, dividing them into ridge aplasia, ridge hypoplasia, ridge dissociation, ridges-off-the-end, and a combination of the last two. The medical and genetic significance of these are considered in the light both of previous published cases and of new material. Malformations of dermatoglyphs are important as physical signs in paediatric diagnosis. ImagesFIG. 1FIG. 2FIG. 3FIG. 4FIG. 5FIG. 6FIG. 7 David, T. J. ... How Loud is Too Loud? Ear Infections in Children Tinnitus Resources Statistics on Hearing, Balance, Ear Infections, and Deafness Free Publications Order Form Directory of Organizations NIDCD Glossary Student and Teacher Activities — Games, videos, and education materials Have a Question? You can ... Summary We retrospectively evaluated 53 consecutive patients with cervicofacial venous malformation who had sclerotherapy. This review included a demographic analysis, MRI reexamination and tabulation of interventional therapeutic strategies. All patients whose MRI studies were included in this review demonstrated characteristic findings: space occupying lesion with hyperintense T2 signal abnormality, patchy contrast enhancement, and no flow signal on the gradient echo images. We concluded that a complete MRI work-up of these patients requires post-contrast scanning and gradient-echo imaging in addition to the standard T1 and T2 weighted spin echo imaging. The majority of patients had sporadic (non-familial) venous anomalies. Sinus pericranii (SP) was identified in six patients (11%) and blue rubber bleb nevus syndrome (BRB-NS) was found in two patients (4%). MRI findings of sinus pericranii are discussed in detail. Although sodium tetradecyl and/or absolute ethanol are the most commonly used sclerosants, a wide variety of therapeutic strategies (depending on the nature of the abnormality) are also needed for these patients. Konez, O.; Burrows, P. E.; Mulliken, J.B. Pulmonary arteriovenous malformations (PAVMs) are abnormal vascular structures that most often connect a pulmonary artery to a pulmonary vein, bypassing the normal pulmonary capillary bed and resulting in an intrapulmonary right-to-left shunt. As a consequence, patients with PAVM can have hypoxemia and paradoxical embolization complications, including stroke and brain abscess. PAVMs may be single or multiple, unilateral or bilateral, and simple or complex. Most PAVMs are hereditary and occur in hereditary hemorrhagic telangiectasia, an autosomal dominant vascular disorder, and screening for PAVM is indicated in this subgroup. PAVMs may also be idiopathic, occur as a result of trauma and infection, or be secondary to hepatopulmonary syndrome and bidirectional cavopulmonary shunting. Diagnostic testing involves identifying an intrapulmonary shunt, with the most sensitive test being transthoracic contrast echocardiography. Chest CT scan is useful in characterizing PAVM in patients with positive intrapulmonary shunting. Transcatheter embolotherapy is the treatment of choice for PAVM. Lifelong follow-up is important because recanalization and collateralization may occur after embolization therapy. Surgical resection is rarely necessary and reserved for patients who are not candidates for embolization. Antibiotic prophylaxis for procedures with a risk of bacteremia (eg, dental procedures) is recommended in all patients with PAVM because of the risk of cerebral abscess. PMID:24008954 Cartin-Ceba, Rodrigo; Swanson, Karen L; Krowka, Michael J Background: Because extra-axial cavernous malformations (CMs) are rare, the common clinical course remains unclear. We report the case of a patient with progressive CM originating from the cerebellar tentorium. Case Description: A 64-year-old woman was admitted to our hospital with the complaint of diplopia. Magnetic resonance (MR) imaging revealed a lesion attached to the left cerebellar tentorium, close to the cerebral peduncle. This well-demarcated lesion rapidly enlarged for 3 months and eroded into the midbrain. Cerebral angiography showed a branch of the middle meningeal artery supplying the lesion and pooling of the contrast medium in the venous phase. A dark reddish and mulberry-like mass of the tentorium was observed intraoperatively, allowing the diagnosis of a tentorial CM. The feeding artery was identified in the tentorium and was coagulated. Postoperative MR imaging showed remarkable mass reduction and central necrosis of the lesion. However, the lesion recurred in 3 months; consequently, gamma knife radiosurgery was performed. After an additional 2 months, the lesion shrank in response to the radiosurgery. Conclusions: We report an extremely rare case of tentorial CM which showed rapid growth in a short period. Coagulation of the feeding artery was not sufficient to control the lesion. Gamma knife radiosurgery may prove highly effective for recurrent lesions. Furuta, Takuya; Nakada, Mitsutoshi; Watanabe, Takuya; Hayashi, Yutaka; Hamada, Jun-Ichiro Pediatric dural arteriovenous malformations (dAVMs) are rare lesions that have a high mortality rate and require complex management. The authors report 3 cases of pediatric dAVMs that presented with macrocrania and extracranial venous distension. Dural sinus thrombosis developed in 2 of the cases prior to any intervention, which is an unusual occurrence for this particular disease. All 3 cases were treated using staged endovascular embolization with a favorable outcome in 1 case and a poor outcome in the other 2 cases. Complications developed in all cases and included dural sinus thrombosis, parenchymal hemorrhage, intracranial venous hypertension, and seizures. The strategies and challenges used in managing these patients will be presented and discussed, along with a review of the literature. While outcomes remain poor, the authors conclude that prompt treatment with endovascular embolization provides the best results for children with these lesions. A well-established venous collateral circulation draining directly to the internal jugular veins may further improve the rate of favorable outcome after embolization. PMID:24867128 Appaduray, Shaun P; King, James A J; Wray, Alison; Lo, Patrick; Maixner, Wirginia The malformations of the cerebral cortex represent a major cause of developmental disabilities, severe epilepsy and reproductive disadvantage. The advent of high-resolution MRI techniques has facilitated the in vivo identification of a large group of cortical malformation phenotypes. Several malformation syndromes caused by abnormal cortical development have been recognised and specific causative gene defects have been identified. Periventricular nodular heterotopia (PNH) is a malformation of neuronal migration in which a subset of neurons fails to migrate into the developing cerebral cortex. X-linked PNH is mainly seen in females and is often associated with focal epilepsy. FLNA mutations have been reported in all familial cases and in about 25% of sporadic patients. A rare recessive form of PNH due ARGEF2 gene mutations has also been reported in children with microcephaly, severe delay and early seizures. Lissencephaly-pachygyria and subcortical band heterotopia (SBH) are disorders of neuronal migration and represent a malformative spectrum resulting from mutations of either LIS1 or DCX genes. LIS1 mutations cause a more severe malformation in the posterior brain regions. Most children have severe developmental delay and infantile spasms, but milder phenotypes are on record, including posterior SBH owing to mosaic mutations of LIS1. DCX mutations usually cause anteriorly predominant lissencephaly in males and SBH in female patients. Mutations of DCX have also been found in male patients with anterior SBH and in female relatives with normal brain magnetic resonance imaging. Autosomal recessive lissencephaly with cerebellar hypoplasia, accompanied by severe delay, hypotonia, and seizures, has been associated with mutations of the reelin (RELN) gene. X-linked lissencephaly with corpus callosum agenesis and ambiguous genitalia in genotypic males is associated with mutations of the ARX gene. Affected boys have severe delay and seizures with suppression-burst EEG. Early death is frequent. Carrier female patients can have isolated corpus callosum agenesis. Among several syndromes featuring polymicrogyria, bilateral perisylvian polymicrogyria shows genetic heterogeneity, including linkage to chromosome Xq28 in some pedigrees, autosomal dominant or recessive inheritance in others, and an association with chromosome 22q11.2 deletion in some patients. About 65% of patients have severe epilepsy. Recessive bilateral frontoparietal polymicrogyria has been associated with mutations of the GPR56 gene. Epilepsy is often present in patients with cortical malformations and tends to be severe, although its incidence and type vary in different malformations. It is estimated that up to 40% of children with drug-resistant epilepsy have a cortical malformation. However, the physiopathological mechanisms relating cortical malformations to epilepsy remain elusive. PMID:16724181 Guerrini, Renzo; Marini, Carla Congenital limb malformations occur in 1 in 500 to 1 in 1000 human live births and include both gross reduction defects and more subtle alterations in the number, length and anatomy of the digits. The major causes of limb malformations are abnormal genetic programming and intra-uterine disruption to development. The identification of causative gene mutations is important for genetic counselling and also provides insights into the mechanisms controlling limb development. This article illustrates some of the lessons learnt from the study of human limb malformation, organized into seven categories. These are: (1) identification of novel genes, (2) allelic mutation series, (3) pleiotropy, (4) qualitative or (5) quantitative differences between mouse and human development, (6) physical and teratogenic disruption, and (7) unusual biological phenomena. Wilkie, Andrew OM Some arteriovenous malformations fed by the striate arteries and lying within the claustrolenticular region can be excised by using microsurgical techniques. Three patients operated on with successful removal of the malformation are discussed here. PMID:7444736 Viale, G L; Turtas, S; Pau, A Background Congenital pancreaticobiliary malformations are sometimes associated with acute or chronic pancreatitis and biliary carcinoma.\\u000a Currently, magnetic resonance cholangiopancreatography (MRCP) is one of the first choices for investigating and diagnosing\\u000a pancreaticobiliary diseases noninvasively. We compared the accuracy of conventional MRCP and endoscopic retrograde cholangiopancreatography\\u000a (ERCP) in making the diagnosis of congenital pancreaticobiliary malformations.\\u000a \\u000a \\u000a \\u000a Methods In patients with pancreas divisum (n = 17), T. Kamisawa; Y. Tu; N. Egawa; K. Tsuruta; A. Okamoto; N. Kamata Capillary malformations (CM) are defects of the dermal capillary bed. These slow-flow malformations can affect any part of the body and are always lateralized, despite Unna's naevus. Present at birth, they grow proportionally with the child. In rare instance, they can be part of a more complex syndrome such as Sturge-Weber syndrome. Ectatic CMs of telangiectatic types can be cutaneous, isolated, multiples, diffuse or generalized. In rare instance, they can be associated with epidermal modifications. They can also be part of a syndrome such as Fabry disease, Osler-Weber-Rendu disorder or Cutis marmorata telangiectatica congenita (CMTC). This chapter details the various clinical aspects of CMs. PMID:17007980 Bataille, A-C; Boon, L-M Several factors have led to the current epidemic of surgery for glue ear in children, including the widespread introduction of audiometry; greater recognition of the presence of fluid in the middle ear by general practitioners; the availability of more otolaryngologists; and technical advances such as the availability of antibiotics to treat postoperative infections and of flanged tympanostomy tubes (grommets). The need of surgeons to fill the vacuum caused by the decline in the number of adenotonsillectomies, and the fact that a diagnosis of glue ear legitimises the continued use of these operations, may also have contributed to the increase. Finally, glue ear may provide parents with a medical explanation of their children's poor educational performance, as the term dyslexia did in the past. The high social and public costs of this operation demand a reappraisal of its increasing use. The necropsy reports of 294 cases of anencephaly and 50 cases of iniencephaly have been examined, and a tubulated list of associated malformations produced. Cases were divided by sex and the presence or absence of spina bifida. Forty-one per cent of the series had other malformations, and other malformations were more common in those cases with spina bifida than in T J David; A Nixon Background Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver. It is commonly associated with multiple congenital anomalies. We present a case of Abernethy malformation, without associated congenital anomalies from India. Case presentation A 5-year-old female child presented with short history of jaundice. A provisional diagnosis of acute viral hepatitis was made in view of clinical presentation and local endemicity of viral hepatitis A. Persistence of jaundice on follow up after 4?weeks led to detailed investigations. Ultrasound and doppler study of abdomen revealed drainage of portal vein into inferior vena cava. CT angiography was performed which confirmed the diagnosis of Type 1 b Abernethy malformation without associated major anomalies. We discuss the common clinical presentations, associated anomalies, diagnostic workup and treatment options of this disorder. Conclusion The treatment of the patients with congenital porto-systemic shunts depends on the site of the shunt, associated congenital anomalies and the extent of liver damage but the prognosis depends on the complications irrespective of anatomical type. However, the extent of associated abnormalities should not deter paediatricians to refer patients for treatment. Whenever possible closure of the shunt should be advised for cure or to prevent complications. Only symptomatic type I patients with absence of possibility to close the shunt may require liver transplant. Long-term follow-up is indicated for all patients. BACKGROUND In pregnant women with epilepsy the use of antiepileptic drugs may increase the risk of harming the foetus. For the treating neurologist it may be challenging to find a balance between optimal seizure control and the lowest possible drug dosage. The aim of this study was to assess the prevalence and type of congenital malformations in children exposed to antiepileptic drugs during pregnancy.MATERIAL AND METHOD In Norway we have prospectively followed 813 pregnancies in women with epilepsy as part of an international cohort study. The women had three check-ups during the pregnancy, and the children were followed up twice during their first year of life.RESULTS We found a total of 34 congenital malformations in the children, of which 12 were heart defects, yielding a malformation rate of 4.5 %. Six of the malformations (18 %) were detected prenatally, 20 (59 %) were reported immediately after birth, and eight (24 %) were discovered during the child's first year of life.INTERPRETATION Our study shows that 95.5 %.of the women included who used antiepileptic drugs during pregnancy gave birth to a healthy child. This Norwegian cohort is too small to evaluate the teratogenic risk associated with the individual drugs. PMID:24989202 Nakken, Karl O; Lillestølen, Kari Mette; Brodtkorb, Eylert; Svendsen, Torleiv; Haggag, Kathrine Johannessen; Taubøll, Erik Occult vascular malformations are frequently associated with intractable seizures and are a common cause of lesional epilepsy. MR imaging can identify and characterize these lesions with accuracy. The presurgical evaluation must be tailored to the individual patient's presentation and circumstances. Surgical results following lesionectomy plus corticectomy may be slightly superior to lesionectomy alone, but the overall prognosis is excellent. PMID:10419577 Cosgrove, G R ABSTRACT Purpose To review the association of autism spectrum disorder (ASD) in individuals manifesting thalidomide embryopathy and Möbius sequence and compare them with three new studies in which ASD was also associated with ocular and systemic malformations: (1) a Swedish study of individuals with CHARGE association (Coloboma, Heart, choanal Atresia, developmental or growth Retardation, Genital anomaly, and Ear involvement); (2) a Swedish study of Goldenhar syndrome; and (3) Brazilian Möbius syndrome (sequence) study. Methods In the Swedish CHARGE study, 31 patients met the inclusion criteria (3+ or 4 of the common characteristics of the CHARGE syndrome). The same team of investigators also evaluated 20 Swedish patients with Goldenhar syndrome. In the Brazilian Möbius study, 28 children with a diagnosis of Möbius sequence were studied; some children had a history of exposure during their mother’s pregnancy to the abortifacient drug misoprostol in an unsuccessful abortion attempt Results In the CHARGE study, five patients had the more severe autism disorder and five had autistic-like condition. In the Goldenhar study, two had autism disorder and one had autistic-like condition. In the Brazilian Möbius study, the systemic findings of the misoprostol-exposed and misoprostol-unexposed patients were almost undistinguishable, and ASD was present in both groups (autism disorder in five and autistic-like condition in three). Conclusion Autism spectrum disorder has been reported in two conditions with known early pregnancy exposure to the teratogenic agents thalidomide and misoprostol. In the Brazilian Möbius study, autism also occurred in both the misoprostol-exposed and misoprostol-unexposed groups. Autism also was present in patients with both CHARGE association and Goldenhar syndrome. Miller, Marilyn T; Stromland, Kerstin; Ventura, Liana; Johansson, Maria; Bandim, Jose M; Gillberg, Christopher Vascular anomalies are a heterogeneous group of congenital blood vessel disorders more typically referred to as birthmarks. Subcategorized into vascular tumors and malformations, each anomaly is characterized by specific morphology, pathophysiology, clinical behavior, and management approach. Hemangiomas are the most common vascular tumors. Lymphatic, capillary, venous, and arteriovenous malformations make up the majority of vascular malformations. Arteriovenous malformation of the head and neck is a rare vascular anomaly but when present is persistent and progressive in nature and can represent a lethal benign disease. Here we present a case report of a 25-year-old male patient with arteriovenous malformation involving the base of tongue. PMID:24660070 Manjunath, S M; Shetty, Sujan; Moon, Ninad J; Sharma, Bhushan; Metta, Kiran Kumar; Gupta, Nitin; Goyal, Sandeep; Singh, Simranjit Malformations are common causes of pediatric morbidity and mortality, and genetic factors are a significant component of their etiology. Autosomal deletions, in almost all cases, cause a nonspecific embryopathy that presents after birth as growth failure, mental retardation, and multiple malformations. We have constructed a chromosome map of autosomal deletions associated with 47 different congenital malformations, using detailed clinical and cytogenetic information on 1,753 patients with nonmosaic single contiguous autosomal deletions. The 1,753 deletions involved 258 (89%) of 289 possible autosomal bands (by the use of ISCN 400-band nomenclature), giving a total of 4,190 deleted autosomal bands for analysis. We compared the band distributions of deletions associated with common major malformations with the distribution of all 1,753 deletions. We noted 283 positive associations between deleted bands and specific malformations, of which 199 were significant (P<.05, P>.001) and 84 were highly significant (P<.001). These "malformation-associated bands" (MABs) were distributed among 137 malformation-associated chromosome regions (MACRs). An average of 6 MABs in 2.9 MACRs were detected per malformation studied; 18 (6%) of 283 MABs contain a locus known to be associated with the particular malformation. A further 18 (6%) of 283 are in seven recognized specific malformation-associated aneuploid regions. Therefore, 36 (26%) of 137 of the MACRs contain an MAB coinciding with a previously recognized locus or malformation-associated aneuploid region. This map should facilitate identification of genes important in human development. Brewer, C; Holloway, S; Zawalnyski, P; Schinzel, A; FitzPatrick, D History of underwater diving dates back to antiquity. Breath-hold technique in diving was known to the ancient nations. However, deep diving progressed only in the early decades of the 19th century as the result of advancements in efficient underwater technologies which subsequently led to invention of sophisticated sets of scuba diving in the 20th century. Currently, diving is performed for various purposes including commercial, recreational, military, underwater construction, oil industry, underwater archeology and scientific assessment of marine life. By increasing popularity of underwater diving, dive-related medical conditions gradually became more evident and created a new challenge for the health care professionals, so that eventually, a specialty the so-called "diving medicine" was established. Most of the diving-associated disorders appear in the head and neck. The most common of all occupational disorders associated with diving are otologic diseases. External otitis has been reported as the most common otolaryngologic problem in underwater divers. Exostosis of the external ear canal may be formed in divers as the result of prolonged diving in cold waters. Other disorders of the ear and paranasal sinuses in underwater divers are caused by barometric pressure change (i.e., barotraumas), and to a lesser extent by decompression sickness. Barotrauma of the middle ear is the most prevalent barotrauma in divers. The inner ear barotraumas, though important, is less common. The present paper is a brief overview of diving-related ear disorders particularly in scuba divers. PMID:23022815 Azizi, M H The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing. PMID:23635097 Mannoor, Manu S; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A; Soboyejo, Winston O; Verma, Naveen; Gracias, David H; McAlpine, Michael C Stress of some kind is encountered everyday and release of stress hormones is essential for adaptation to change. Stress can be physical (pain, noise exposure, etc.), psychological (apprehension to impending events, acoustic conditioning, etc.) or due to homeostatic disturbance (hunger, blood pressure, inner ear pressure, etc.). Persistent elevated levels of stress hormones can lead to disease states. The aim of the present review is to bring together data describing morphological or functional evidence for hormones of stress within the inner ear. The present review describes possible multiple interactions between the sympathetic and the complex feed-back neuroendocrine systems which interact with the immune system and so could contribute to various inner ear dysfunctions such as tinnitus, vertigo, hearing losses. Since there is a rapidly expanding list of genes specifically expressed within the inner ear this clearly allows for possible genomic and non-genomic local action of steroid hormones. Since stress can be encountered at any time throughout the life-time, the effects might be manifested starting from in-utero. These are avenues of research which remain relatively unexplored which merit further consideration. Progress in this domain could lead towards integration of stress concept into the overall clinical management of various inner ear pathologies. PMID:14505685 Horner, K C The ability to three-dimensionally interweave biological tissue with functional electronics could enable the creation of bionic organs possessing enhanced functionalities over their human counterparts. Conventional electronic devices are inherently two-dimensional, preventing seamless multidimensional integration with synthetic biology, as the processes and materials are very different. Here, we present a novel strategy for overcoming these difficulties via additive manufacturing of biological cells with structural and nanoparticle derived electronic elements. As a proof of concept, we generated a bionic ear via 3D printing of a cell-seeded hydrogel matrix in the precise anatomic geometry of a human ear, along with an intertwined conducting polymer consisting of infused silver nanoparticles. This allowed for in vitro culturing of cartilage tissue around an inductive coil antenna in the ear, which subsequently enables readout of inductively-coupled signals from cochlea-shaped electrodes. The printed ear exhibits enhanced auditory sensing for radio frequency reception, and complementary left and right ears can listen to stereo audio music. Overall, our approach suggests a means to intricately merge biologic and nanoelectronic functionalities via 3D printing. Mannoor, Manu S.; Jiang, Ziwen; James, Teena; Kong, Yong Lin; Malatesta, Karen A.; Soboyejo, Winston O.; Verma, Naveen; Gracias, David H.; McAlpine, Michael C. When electrical resonances were observed in acoustic sensory cells of lower vertebrates, the hearing research community was presented with the exciting possibility that tuning in the ears of those animals might be explained directly in terms of familiar molecular devices. It is reported here that in the frog sacculus, where electrical resonances have been observed in isolated hair cells, the effects of those resonances are completely obscured in the tuning properties of the sacculus in the intact ear. This observation has important implications not only for students of the ear, but for reductionist biologists in general. All of the dynamic properties of a system of connected, bidirectional processes are consequences of all of those processes at once; in such a system, the properties of an experimentally isolated subsystem may be totally obscured in the operation of the system as a whole. Lewis, E R Phocomelia (flipper-like limbs) has long been recognized as a rare malformation. Numerous cases of phocomelia and other congenital malformations have recently been reported in the United Kingdom, Australia, Europe and Canada in which the common factor appears to have been the administration of the hypnotic compound thalidomide during early pregnancy. Two additional cases of infants born with phocomelia, amelia and alimentary abnormalities are presented. In both of these cases the administration of thalidomide was initiated early during pregnancy (five to eight weeks after the last normal menstrual period) and maintained for several weeks. Thalidomide (alpha-phthalimido glutarimide) is related chemically to other glutarimides currently in clinical use. The possibility that these compounds and/or their metabolites may induce teratogenic effects warrants consideration. Emphasis is added to the view that caution should be exercised when prescribing new drugs. ImagesFig. 1Fig. 2 Ing, George M.; Olman, C. L.; Oyd, John R. Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the development of local methods of drug delivery to the inner ear. Intratympanic approaches, which deliver therapeutics to the middle ear, rely on permeation through tissue for access to the structures of the inner ear, whereas intracochlear methods are able to directly insert drugs into the inner ear. Innovative drug delivery systems to treat various inner ear ailments such as ototoxicity, sudden sensorineural hearing loss, autoimmune inner ear disease, and for preserving neurons and regenerating sensory cells are being explored. Swan, Erin E. Leary; Mescher, Mark J.; Sewell, William F.; Tao, Sarah L.; Borenstein, Jeffrey T. The physiological functions of the ear and the role masking plays in speech communication are examined. Topics under investigation include sound analysis of the ear, the aural reflex, and various types of noise masking. ... FOOD AND DRUG ADMINISTRATION, DEPARTMENT OF HEALTH AND HUMAN SERVICES (CONTINUED) MEDICAL DEVICES GENERAL AND PLASTIC SURGERY DEVICES Prosthetic Devices Â§ 878.3590 Ear prosthesis. (a) Identification. An ear prosthesis is a... Scalp-ear-nipple (SEN) syndrome is a rare, autosomal-dominant disorder characterized by cutis aplasia of the scalp; minor anomalies of the external ears, digits, and nails; and malformations of the breast. We used linkage analysis and exome sequencing of a multiplex family affected by SEN syndrome to identify potassium-channel tetramerization-domain-containing 1 (KCTD1) mutations that cause SEN syndrome. Evaluation of a total of ten families affected by SEN syndrome revealed KCTD1 missense mutations in each family tested. All of the mutations occurred in a KCTD1 region encoding a highly conserved bric-a-brac, tram track, and broad complex (BTB) domain that is required for transcriptional repressor activity. KCTD1 inhibits the transactivation of the transcription factor AP-2? (TFAP2A) via its BTB domain, and mutations in TFAP2A cause cutis aplasia in individuals with branchiooculofacial syndrome (BOFS), suggesting a potential overlap in the pathogenesis of SEN syndrome and BOFS. The identification of KCTD1 mutations in SEN syndrome reveals a role for this BTB-domain-containing transcriptional repressor during ectodermal development. Marneros, Alexander G.; Beck, Anita E.; Turner, Emily H.; McMillin, Margaret J.; Edwards, Matthew J.; Field, Michael; de Macena Sobreira, Nara Lygia; Perez, Ana Beatriz A.; Fortes, Jose A.R.; Lampe, Anne K.; Giovannucci Uzielli, Maria Luisa; Gordon, Christopher T.; Plessis, Ghislaine; Le Merrer, Martine; Amiel, Jeanne; Reichenberger, Ernst; Shively, Kathryn M.; Cerrato, Felecia; Labow, Brian I.; Tabor, Holly K.; Smith, Joshua D.; Shendure, Jay; Nickerson, Deborah A.; Bamshad, Michael J. The frog inner ear contains three regions that are sensitive to airborne sound and which are functionally distinct. (1) The responses of nerve fibres innervating the low-frequency, rostral part of the amphibian papilla (AP) are complex. Electrical tuning of hair cells presumably contributes to the frequency selectivity of these responses. (2) The caudal part of the AP covers the mid-frequency portion of the frog's auditory range. It shares the ability to generate both evoked and spontaneous otoacoustic emissions with the mammalian cochlea and other vertebrate ears. (3) The basilar papilla functions mainly as a single auditory filter. Its simple anatomy and function provide a model system for testing hypotheses concerning emission generation. Group delays of stimulus frequency otoacoustic emissions (SFOAEs) from the basilar papilla are accounted for by assuming that they result from forward and reverse transmission through the middle ear, a mechanical delay due to tectorial membrane filtering and a rapid forward and reverse propagation through the inner ear fluids, with negligible delay. van Dijk, Pim; Mason, Matthew J.; Schoffelen, Richard L. M.; Narins, Peter M.; Meenderink, Sebastiaan W. F. ... speak. Sound, which is made up of invisible waves of energy, causes these vibrations. Every time you hear a sound, the various structures of the ear have to work together to make sure ... Hearing begins when sound waves that travel through the air reach the outer ... Intrauterine growth retardation is caused by factors that prevent adequate fetal nourishment or by factors that intrinsically affect the fetus. Limited available space due to a congenitally malformed uterus may prevent normal intrauterine development. Two cases are presented here. A review of the available literature confirms the tendency of women with congenital uterine malformations to have smaller offspring. Intrauterine growth retardation should be added to the more commonly known complications associated with a congenitally malformed uterus. ImagesFigure 1Figure 2 Poma, Pedro A. . Using the Swedish Cardiology Registry and the Registry of Congenital Malformations, and after excluding infants with a known\\u000a chromosomal anomaly, data on 397 infants (15%) born during the period 1981–1990 with a major cardiac defect and at least one\\u000a noncardiac malformation were analyzed. No clear-cut association appeared between specific heart defects and major groups of\\u000a noncardiac malformations except for Myelomeningocele (MMC) is a malformation characterized by the failure of closure of the neural tube, usually (but not only)\\u000a at the lumbo-sacral level. Synonyms are spina bifida aperta, open spinal dysra — phism, and Chiari II malformation complex.\\u000a MMC is typically associated with a metamerically consistent paraplegia, a posterior fossa deformity known as the Chiari II\\u000a malformation, hydrocephalus, and a Charles Raybaud; Elka Miller Ear is a new class of relatively stable biometric that is invariant from childhood to early old age (8 to 70). It is not affected with facial expressions, cosmetics and eye glasses. In this paper, we introduce a two-step ICP (Iterative Closest Point) algorithm for matching 3D ears. In the first step, the helix of the ear in 3D images Hui Chen; Bir Bhanu Immune inner ear disease represents a series of immune system mediated problems that can present with hearing loss, dizziness, or both. The etiology, presentation, testing, and treatment of primary immune inner ear disease is discussed. A review of secondary immune inner ear disease is presented for comparison. (Contains references.) (Author/CR) Kinney, William C.; Hughes, Gordon B. Malformations of cortical development (MCD) represent a major cause of developmental disabilities and severe epilepsy. Advances in imaging and genetics have improved the diagnosis and classification of these conditions. Up to now, eight genes have been involved in different types of MCD. Lissencephaly-pachygyria and subcortical band heterotopia (SBH) represent a malformative spectrum resulting from mutations of either LIS1 or DCX genes. LIS1 mutations cause a more severe malformation in the posterior brain regions. DCX mutations usually cause anteriorly predominant lissencephaly in males and SBH in female patients. Additional forms are X-linked lissencephaly with corpus callosum agenesis and ambiguous genitalia associated with mutations of the ARX gene. Lissencephaly with cerebellar hypoplasia (LCH) encompass heterogeneous disorders named LCH types a to d. LCHa is related to mutation in LIS1 or DCX, LCHb with mutation of the RELN gene, and LCHd could be related to the TUBA1A gene. Polymicrogyria encompasses a wide range of clinical, etiological, and histological findings. Among several syndromes, recessive bilateral fronto-parietal polymicrogyria has been associated with mutations of the GPR56 gene. Bilateral perisylvian polymicrogyria has been associated with mutations in the SRPX2 gene in a few individuals and with linkage to chromosome Xq28 in a some other families. X-linked bilateral periventricular nodular heterotopia (PNH) consists of PNH with focal epilepsy in females and prenatal lethality in males. Filamin A (FLNA) mutations have been reported in some families and in sporadic patients. It is possible to infer the most likely causative gene by brain imaging studies and other clinical findings. PMID:23622213 Bahi-Buisson, Nadia; Guerrini, Renzo AIMS—Orbital arteriovenous malformations (OAVM) are rare, mostly described with high flow characteristics. Two cases are reported with an OAVM of distinct haemodynamic abnormality. The clinical, angiographic features, and the management considerations are discussed.?METHODS—Case review of two patients with dural AVM (DAVM) who presented to referral neuro-ophthalmology and endovascular services because of clinical symptoms and signs consistent with a cavernous sinus dural AVM.?RESULTS—In each patient, superselective angiography revealed a small slow flow intraorbital shunt supplied by the ophthalmic artery. The transarterial and transvenous endovascular approaches to treat the malformation were partially successful. Although, the abnormal flow was reduced, complete closure of the DAVM could not be accomplished without significant risk of iatrogenic injury. Neither patient's vision improved after intervention.?CONCLUSION—A DAVM in the orbit can cause similar clinical symptoms and signs to those associated with a cavernous sinus DAVM. Even with high resolution magnetic resonance imaging, only superselective angiography can identify this small intraorbital slow flow shunt. The location in the orbital apex and the small size precludes a surgical option for treatment. The transarterial and transvenous embolisation options are limited.?? Huna-Baron, R.; Setton, A.; Kupersmith, M.; Berenstein, A. Summary \\u000a ?Background. A rare case of a left temporal arteriovenous malformation (AVM) with bleeding 10 days after removal of another hemorrhagic\\u000a AVM in the frontal lobe is reported.\\u000a \\u000a \\u000a \\u000a ?Method. A 47-year-old man had an attack of headache and vomiting and was admitted to our hospital. On admission, a CT scan showed\\u000a subcortical hemorrhage in the left frontal lobe. Left carotid S. Utsuki; A. Kurata; Y. Miyasaka; M. Takano; H. Ootaka; K. Fujii We report here a case of a 44-year-old female with a pulmonary varix. The patient was asymptomatic; a routine chest X-ray incidentally revealed a serpiginous mass in the left hilar region. Computed tomography revealed a serpiginous, dilated vascular structure in the left upper lobe that continued into the lower lobe and was suggestive of an arteriovenous malformation. Pulmonary angiography revealed no arterial abnormality; however, the filling of an abnormally dilated pulmonary vein was observed. Angiography is considered to be the most useful method for the confirmatory diagnosis of pulmonary varices. PMID:18164399 Kumazoe, Hiroyuki; Komori, Masashi; Ochiai, Reiji; Egashira, Ryoko; Nakazono, Takahiko; Kudo, Sho Pulmonary arteriovenous malformations are rare lesions with significant clinical complications. These lesions are commonly seen in patients with hereditary hemorrhagic telangiectasia (formerly Osler-Weber-Rendu syndrome). Interventional radiologists are a key part of the treatment team in this complex disease, and a thorough understanding of the disease process is critical to providing good patient care. In this article, the authors review the disease course and its association with hereditary hemorrhagic telangiectasia, discusses the clinical evaluation and treatment of these complex patients, and outlines complications and follow-up. Meek, Mary E.; Meek, James C.; Beheshti, Michael V. Investigation of the causes of vestibular symptoms in patients with chronic otitis media (COM) faces frustration, mainly because the bithermal caloric test using tap water is generally contraindicated in perforated ears. This study utilized audiometry, ocular vestibular-evoked myogenic potential (oVEMP) test, and cervical VEMP (cVEMP) test to evaluate inner ear deficits after COM. A total of 85 COM patients (117 ears) underwent otoscopy, image study, audiometry, oVEMP test, and cVEMP test. Mean bone-conducted (BC) hearing threshold ?25 dB was observed in 74 ears, 26-40 dB in 30 ears, and >40 dB in 13 ears. Restated, abnormal BC hearing threshold was identified in 43 ears (37 %). Percentages of abnormal cVEMP test, oVEMP test, and BC hearing threshold in 117 COM ears were 65, 62, and 37 %, respectively, exhibiting a significantly declining sequence in inner ear function. Furthermore, cVEMP/oVEMP test results were significantly correlated with BC hearing threshold, whereas no correlation existed between the cVEMP and oVEMP test results. In conclusion, the sequence of inner ear deficits after COM runs from the saccule/utricle to the cochlea and semicircular canals. Restated, in addition to BC hearing test, the cVEMP/oVEMP test may serve as a supplementary tool for early detection of inner ear involvement in COM patients. PMID:24061573 Chang, Chun-Wei; Cheng, Po-Wen; Young, Yi-Ho Imaging of the temporal bone is under continous developement. In the recent decades the technical advances of magnetic resonance imaging and computed tomography have contributed to improved imaging quality in assessment of the temporal bone. Dedicated imaging protocols have been developed and are routinely employed in most institutions. However, imaging interpretation remains challenging, since the temporal bone is an anatomically highly complex region and most diseases of the inner ear occur with low incidence, so that even radiologists experienced in the field may be confronted with such entities for the first time. The current review gives an overview about symptoms and imaging appearance of malformations and acquired lesion of the inner ear. PMID:17926042 Krombach, Gabriele A; Honnef, Dagmar; Westhofen, Martin; Di Martino, Ercole; Günther, Rolf W This article addresses the scope, etiology, important associations and imaging features of congenital bronchopulmonary foregut malformations. Etiologic concepts, including airway obstruction and vascular anomalies, are highlighted. Technical imaging advances, especially CT and MR, have greatly enhanced our diagnostic abilities in evaluating these lesions; however, thorough and careful assessment of all aspects of the malformation is still necessary. Several specific lesions Malformed messages in different protocols pose a serious threat because they are used to remotely launch malicious activity. Furthermore, they are capable of crashing servers and end points, sometimes with a single message. Recently, it was shown that a malformed SMS can crash a mobile phone or gain unfettered access to it. In spite of this, little research has been M Zubair Rafique; Muhammad Khurram Khan; Khaled Alghathbar; Muddassar Farooq Renal tract malformations are congenital anomalies of the kidneys and\\/or lower urinary tract. One challenging feature of these conditions is that they can present not only prenatally but also in childhood or adulthood. The most severe types of malformations, such as bilateral renal agenesis or dysplasia, although rare, lead to renal failure. With advances in dialysis and transplantation for young Larissa Kerecuk; Michiel F Schreuder; Adrian S Woolf Chiari type II malformation is a congenital deformity of the hindbrain. Square wave jerks are horizontal involuntary saccades that interrupt fixation. Cerebellar disorders may be associated with frequent square wave jerks or saccadic oscillations such as ocular flutter. The effects of Chiari type II malformation on visual fixation are unknown. We recorded eye movements using an eye tracker in 21 participants with Chiari type II malformation, aged 8 to 19 years while they fixated a target for 1 minute. Thirty-eight age-matched healthy participants served as controls. Square wave jerks' parameters were similar in the 2 groups. Saccadic oscillations were not seen. Chiari type II malformation is not associated with pathological square wave jerks or abnormal saccadic oscillations. The congenital nature of this deformity may permit compensation that preserves stable visual fixation. Alternatively, the deformity of Chiari type II malformation may spare parts of the cerebellum that usually cause fixation instability when damaged. PMID:19182152 Salman, Michael S; Sharpe, James A; Lillakas, Linda; Dennis, Maureen; Steinbach, Martin J Created in June, 1997 and funded by the US Geological Survey and Environmental Protection Agency, this site is a response to the discovery of numerous deformed amphibians (mostly frogs) in areas as widespread as Minnesota, California, and Florida. Malformations include extra or missing limbs, missing eyes, and split limbs. The site is intended to serve as a central repository for data on the type and relative frequency of such malformations throughout the US. Researchers hope to use the site to help discover the cause(s) of these deformities. The site contains a map of where malformations have been reported, background on the occurrence and possible causes of defects, numerous images of malformed amphibians, a searchable bibliography, instructions on how to report the discovery of a malformed amphibian, and links to eight related web sites. NARCAM's site also contains a toll-free number for citizen reports. Center., Northern P. ...tonsil guillotine; tonsil screw; tonsil snare; tonsil suction tube; tonsil suturing...rongeur; nasal saw; nasal scissors; nasal snare; sinus irrigator; sinus trephine; ear...excavator; ear rasp; ear scissor, ear snare; ear spoon; ear suction tube;... ...tonsil guillotine; tonsil screw; tonsil snare; tonsil suction tube; tonsil suturing...rongeur; nasal saw; nasal scissors; nasal snare; sinus irrigator; sinus trephine; ear...excavator; ear rasp; ear scissor, ear snare; ear spoon; ear suction tube;... Based on the clinical CT of normal right ear, a 3-D ?nite element (FE) model of the human ear consisting of the external ear canal, middle ear(tympanic membrane, ossicular chain, ligaments, tendons), and inner ear (including semicircular canals, vestibular, spiral cochlear)was constructed in this paper. The complicated structures and inner boundary conditions of middle ear were described in this model. Model analysis and acoustic-structure-?uid coupled dynamic frequency response analysis were conducted on the model. The validity of this model was confirmed by comparing the results with published experimental data. The amplitudes and velocities of tympanic membrane and stapes footplate, sound pressure gain across the middle ear, and the cochlear input impedance were derived. Besides, it was concluded that the ear canal can amplify the sound signal in low frequencies.The modes of vibration of middle ear auditory ossicles, oval window and round window have been analysed. This model can well simulate the acoustic behavior with the interaction of external ear, middle ear and inner ear, which can supply more valuable theoretical support for development and improvement of hearing-aid and artificial inner ear. Yao, W.; Ma, J.; Huang, X. Congenital malformations frequently arise sporadically, making it difficult to determine whether or not they are genetic in aetiology, let alone which gene(s) may be involved. Nevertheless, rapid progress has been made over recent years in the localisation and identification of gene mutations in specific malformations. This review draws from Mendelian inheritance in man (Johns Hopkins University Press, 11th ed, 1994) and the online version (OMIM) to catalogue 139 loci (including 65 specifically identified genes) implicated in congenital malformations. Some of the most interesting recent developments are discussed. Wilkie, A O; Amberger, J S; McKusick, V A The inner ear is a structurally complex vertebrate organ built to encode sound, motion, and orientation in space. Given its complexity, it is not surprising that inner ear dysfunction is a relatively common consequence of human genetic mutation. Studies in model organisms suggest that many genes currently known to be associated with human hearing impairment are active during embryogenesis. Hence, the study of inner ear development provides a rich context for understanding the functions of genes implicated in hearing loss. This chapter focuses on molecular mechanisms of inner ear development derived from studies of model organisms. Wu, Doris K.; Kelley, Matthew W. Repeated trauma to the ear very often results in "cauliflower ear." Many methods have been suggested to prevent an injured ear from demonstrating a cauliflowerlike deformity. The principles of treatment are evacuation of the hematoma, control of the reaccumulation of fluid, and maintenance of the cartilage contour. The authors studied the effect of ionizing radiation on deformed rabbit ears induced by repeated trauma. Twenty ears (10 rabbits) were used in the experiment. The animals were divided into four groups (control, preradiation, low dose, and high dose). Hematoma was produced by pounding the lateral side of the auricle 10 times with a 50-g weight at a height of 15 cm. The thickness of the injured and uninjured sites was measured, and histological analysis was performed for each group. The thickness of the ears of the irradiated groups was significantly less than the control group. The authors think that radiation treatment of repeatedly injured ears could prevent ear deformity, and could possibly be an adjunctive form of management of cauliflower ear in addition to hematoma evacuation and compression therapy. PMID:11216608 Hwang, K; Kim, C W; Lee, S I; Park, I S; Kim, W C; Loh, J J The literature abounds with reports of malformation syndromes in which human external ears are variously described as dysplastic, abnormal, large/small, low set, typical, or in some way unusual. Rarely is the ear well illustrated or described in meaningful detail. With few exceptions, such as Down syndrome, there is no real understanding of the degree to which ear morphology is affected in a specific syndrome. This paper describes a retrospective attempt to apply the recently published Morphological Definitions of the ear to compare a control sample of convenience with a group of patients with Cornelia de Lange syndrome (CdLS) (all six papers in this issue are available online, open access at http://www3.interscience.wiley.com/journal/121641055/issue). Although this study has a number of limitations, it demonstrates that the method can be successfully applied and is capable of producing data that can be subjected to statistical analysis. The ears of the patients with CdLS were significantly different from the controls over a number of descriptors, the most significant of which included more frequent apparent posterior rotation, a shorter more serpiginous antihelical stem and sharper antihelical to inferior crus angle, a shorter crus helix, a more V-shaped incisura, and a smaller lobe. PMID:19764039 Hunter, Alasdair G W; Collins, Julianne S; Deardorff, Matthew A; Krantz, Ian D Capillary malformation-arteriovenous malformation syndrome is an autosomal dominant disorder caused by mutations in the RASA1 gene and characterized by multiple small, round to oval capillary malformations with or without arteriovenous malformations. Ateriovenous malformations occur in up to one-third of patients and may involve the brain and spine. Although making the diagnosis is straightforward in some patients, there are other patients for whom diagnostic criteria may be helpful in their evaluation. Here we review the literature regarding capillary malformation-arteriovenous malformation syndrome, propose diagnostic criteria, and discuss the care of patients with this condition. PMID:23662773 Orme, Charisse M; Boyden, Lynn M; Choate, Keith A; Antaya, Richard J; King, Brett A Full blown cases of cleidocranial dysplasia (CCD) have been reported earlier but a case with a rarity of 60 teeth associated with bony malformations, is seldom observed Because of the oral findings this condition has been diagnosed at an early age, thus helping to achieve a better oral harmony. This article reports an atypical case with 16 supernumerary teeth associated with bony malformations. PMID:22524086 Kamatham, R; Sharada, J; Mohapatra, A; Nuvvula, S We report a very rare case of a high flow arteriovenous malformation (AVM) of the upper limb that caused high output cardiac failure at birth. There was early transfer of the baby to the care of a multidisciplinary team. After radiological intervention, the arm distal to the malformation became ischaemic and an urgent amputation through the upper-humerus followed. Methods of treatment are discussed, together with a review of results in the literature. PMID:15544778 Webb, Jill B; O'Brien, Mary; John, Philip R; Nishikawa, Hiroshi A case of a Chiari malformation with an extraordinarily thick occipital bone is described. The thick occipital bone might make the posterior fossa narrow with consequent herniation of the cerebellar tonsils to the foramen magnum and formation of a syrinx. At dural plasty, well-developed marginal and occipital sinuses should be deliberately handled with the preservation of normal venous drainage. This case gives us the essence of the occurrence mechanisms of Chiari malformation and foramen magnum decompression. PMID:21339798 Yasuhara, Takao; Miyoshi, Yasuyuki; Date, Isao Risk factors were studied in 370 children with internal urinary system (IUS) anomalies, coming from 105,374 consecutive births of known outcome. The incidence of IUS malformations was 3.51 per 1,000 births. Diagnosis was performed prenatally in 54.4% of patients. Two hundred and fifty-two patients had isolated IUS anomalies; 118 (31.8%) of the children had at least one non-urinary malformation. Fifty-five C. Stoll; Y. Alembik; M. P. Roth; B. Dott; P. Sauvage We report on three children from two families with a new pattern recognition malformation syndrome consisting of severe congenital microcephaly (MIC), intractable epilepsy including infantile spasms, and generalized capillary malformations that was first reported recently in this journal [Carter et al. (2011); Am J Med Genet A 155: 301–306]. Two of our reported patients are an affected brother and sister, suggesting this is an autosomal recessive severe congenital MIC syndrome. Mirzaa, Ghayda M.; Paciorkowski, Alex R.; Smyser, Christopher D.; Willing, Marcia C.; Lind, Anne C.; Dobyns, William B. Congenital pseudoarthrosis is a pathologic entity that may be isolated, or may be associated with neurofibromatosis. We report the case of a 3-year-old female with congenital pseudoarthrosis involving the right tibia and fibula. Magnetic resonance imaging (MRI) and complementary magnetic resonance angiogram (MRA) revealed a lobulated mass with vivid enhancement, which led to the diagnosis of venous malformation. This is the first report of congenital pseudoarthrosis caused by the presence of a vascular malformation. PMID:16967289 Al-Hadidy, A; Haroun, A; Al-Ryalat, N; Hamamy, H; Al-Hadidi, S Tbx1 belongs to the family of T-box containing transcription factors. In humans, TBX1 is implicated in the etiology of the DiGeorge syndrome. Inactivation of the Tbx1 gene in mice produces a variety of malformations including abnormal branching of the heart outflow tract, deficiencies in the branchial arch derivatives, agenesis of pharyngeal glands and abnormal development of the auditory system. We analyze here the middle and inner ear phenotypes of the Tbx1 null mice. The middle ear is strongly affected. Its skeletal components are malformed to varying degrees, some being slightly hypoplastic and others completely absent. However, a seemingly normal-looking tympanic membrane can still be recognized. Middle ear anomalies are associated with other skeletal deficiencies in the branchial arch-derived skeleton. These phenotypes derive from a combination of the failure of the posterior branchial arches to develop and the misrouting of neural crest cells. The inner ears of Tbx1(-/-) animals are hypoplastic. No vestibular or cochlear structures are detectable, but the endolymphatic duct, the cochleovestibular ganglia and residual sensory patches are still identifiable. Molecular analyses revealed a seemingly normal spatial distribution of a variety of patterning markers in the otic vesicles of Tbx1 null mutants at E9.0. However, 1 day later, several of these markers presented altered domains of expression in the otocysts of these mutant embryos, suggesting that Tbx1 is not required for the establishment of spatial patterns in the otocyst, but rather for their maintenance. The inability of the Tbx1(-/-) embryos to keep properly segregated functional domains in the otocyst is likely the cause of the strong inner ear phenotypes observed in these mutants. PMID:15652707 Moraes, Filipa; Nóvoa, Ana; Jerome-Majewska, Loydie A; Papaioannou, Virginia E; Mallo, Moisés Pineal hemorrhage only occurs in rare cases, and this known to have several different causes such as germ cell tumors, pineal cysts and vascular malformations, including the cavernous malformations. Pineal cavernous malformations are extremely rare: to date only fifteen cases have been reported worldwide. Although the diagnosis of pineal cavernous malformation is not easy because of the extreme rareness of this condition, the presence of this lesion can be suspected based on its typical radiological findings. Case 1. A 42-year- old man presented with a limitation in his upward gazing. Radiologic examinations showed acute hemorrhage in the pineal region. He underwent ventriculo-peritoneal (VP) shunting but the patient's condition deteriorated after the shunting surgery. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. Case 2. A 37-year-old man presented with diplopia. Radiologic examinations showed acute hemorrhage in the third ventricle. He underwent VP shunting, and after this procedure the diplopia was aggravated. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. If there is no doubt about the pineal cavernous malformation on MR imaging, we strongly recommend early surgical intervention without performing a risky biopsy. In this study, we describe our experiences for the diagnosis of cavernous malformations in the pineal region with special emphasis on the radiological aspects and the clinical course of this disease. Kim, Dong-Seok; Shim, Kyu-Won; Kim, Tae-Gon; Chang, Jong-Hee; Park, Yong-Gou Pineal hemorrhage only occurs in rare cases, and this known to have several different causes such as germ cell tumors, pineal cysts and vascular malformations, including the cavernous malformations. Pineal cavernous malformations are extremely rare: to date only fifteen cases have been reported worldwide. Although the diagnosis of pineal cavernous malformation is not easy because of the extreme rareness of this condition, the presence of this lesion can be suspected based on its typical radiological findings. Case 1. A 42-year- old man presented with a limitation in his upward gazing. Radiologic examinations showed acute hemorrhage in the pineal region. He underwent ventriculo-peritoneal (VP) shunting but the patient's condition deteriorated after the shunting surgery. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. Case 2. A 37-year-old man presented with diplopia. Radiologic examinations showed acute hemorrhage in the third ventricle. He underwent VP shunting, and after this procedure the diplopia was aggravated. We operated and totally removed the tumor and the hemorrhages via an occipital-transtentorial approach. If there is no doubt about the pineal cavernous malformation on MR imaging, we strongly recommend early surgical intervention without performing a risky biopsy. In this study, we describe our experiences for the diagnosis of cavernous malformations in the pineal region with special emphasis on the radiological aspects and the clinical course of this disease. PMID:16385664 Kim, Dong-Seok; Shim, Kyu-Won; Kim, Tae-Gon; Chang, Jong-Hee; Park, Yong-Gou; Choi, Joong-Uhn The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area. Dompmartin, Anne; Vikkula, Miikka; Boon, Laurence M The anatomy and developmental molecular genetics of the inner ear from establishment of the otic placode to formation of the definitive cochlea and vestibular apparatus will be reviewed and the complex 3-D structural changes that shape the developing inner ear will be illustrated... Objective: To describe and evaluate an ear piercing and earring retention method for individuals with metal hypersensitivity. Setting: Private facial plastic surgery practice associated with a tertiary care medical center. Methods: Thirty-one patients with a history of hypersensitivity to metallic jewelry (62 ears) underwent earlobe piercing with an intravenous catheter. Results: None of the patients experienced an infection or hypersensitivity Anthony J. Cornetta; David Reiter Many inner ear disorders cannot be adequately treated by systemic drug delivery. A blood-cochlear barrier exists, similar physiologically to the blood-brain barrier, which limits the concentration and size of molecules able to leave the circulation and gain access to the cells of the inner ear. However, research in novel therapeutics and delivery systems has led to significant progress in the Erin E. Leary Swan; Mark J. Mescher; William F. Sewell; Sarah L. Tao; Jeffrey T. Borenstein Patient with a round and soft lesion completely covered with skin without modification in color fulfilling approximately 90% of external ear canal diameter. Patient’s complain was dullness sensations on affected ear. CT scan demonstrated lack of tympanic membrane commitment and pathological findings were compatible with hemangioma. Conclusion: It is important to define whether there is hemangioma's involvement of tympanic membrane, José Faibes Lubianca Neto; Mauricio Schreiner Miura; Catia Saleh; Marina de Andrade; Melina Assmann Animal studies on inner ear development, repair and regeneration provide understanding of molecular pathways that can be harnessed for treating inner ear disease. Use of transgenic mouse technology, in particular, has contributed knowledge of genes that regulate development of hair cells and innervation, and of molecular players that can induce regeneration, but this technology is not applicable for human treatment, for practical and ethical reasons. Therefore other means for influencing gene expression in the inner ear are needed. We describe several gene vectors useful for inner ear gene therapy and the practical aspects of introducing these vectors into the ear. We then review the progress toward using gene transfer for therapies in both auditory and balance systems, and discuss the technological milestones needed to advance to clinical application of these methods. Fukui, Hideto; Raphael, Yehoash Previous works have shown that the ear is a promising candidate for biometric identification. However, in prior work, the preprocessing of ear images has had manual steps and algorithms have not necessarily handled problems caused by hair and earrings. We present a complete system for ear biometrics, including automated segmentation of the ear in a profile view image and 3D shape matching for recognition. We evaluated this system with the largest experimental study to date in ear biometrics, achieving a rank-one recognition rate of 97.8 percent for an identification scenario and an equal error rate of 1.2 percent for a verification scenario on a database of 415 subjects and 1,386 total probes. PMID:17568136 Yan, Ping; Bowyer, Kevin W Geometrical methods of feature extraction from ear images in order to perform human identification are presented. Geometrical approach is motivated by the actual procedures used by police and forensic experts (so-called ear otoscopy). In their work, geometrical features of ears such as size, height, width, and shapes of earlobe are useful and valid proofs of identity. The contribution of the article is development of the new and original methods of geometrical feature extraction from 2D ear images. Four novel algorithms of ear feature extraction from contour images are described in detail. Moreover, identification results obtained for each of the methods, based on the distance of feature vectors in the feature space, are presented. Earth Sciences: Instrumentation and Facilities (EAR/IF) Program Solicitation NSF 04-507 Replaces ... of Earth Sciences (EAR) supports research and education focused on understanding Earth dynamics ... Lesion of ossicular chain is a common ear disease impairing the sense of hearing. A comprehensive numerical model of human ear can provide better understanding of sound transmission. In this study, we propose a three-dimensional finite element model of human ear that incorporates the canal, tympanic membrane, ossicular bones, middle ear suspensory ligaments/muscles, middle ear cavity and inner ear fluid. Numerical analysis is conducted and employed to predict the effects of middle ear cavity, malleus handle defect, hypoplasia of the long process of incus, and stapedial crus defect on sound transmission. The present finite element model is shown to be reasonable in predicting the ossicular mechanics of human ear. Liu, Yingxi; Li, Sheng; Sun, Xiuzhen History.—Primary and secondary types.—Incidence in (a) Phthisical patients, (b) Patients with suppurating ears, (c) Children.—Modes of infection: (a) by Eustachian tube, (b) by blood-stream.—Signs and symptoms: Painless onset; Early appearances of middle-ear; Infiltration of tympanic membrane; Multiple perforation; Granulation tissue; Type of discharge.—Examination of discharge and of granulation tissue.—Course of disease in middle-ear; Extensions; Complications.—Special types: Mastoiditis in children; Tumour formation.—Deafness.—Reports of twenty cases. Ormerod, F. C. Students learn about the anatomy of the ear and how the ears work as a sound sensor. Ear anatomy parts and structures are explained in detail, as well as how sound is transmitted mechanically and then electrically through them to the brain. Students use LEGOÂ® robots with sound sensors to measure sound intensities, learning how the NXT brick (computer) converts the intensity of sound measured by the sensor input into a number that transmits to a screen. They build on their experiences from the previous activities and establish a rich understanding of the sound sensor and its relationship to the TaskBot's computer. GK-12 Program, Computational Neurobiology Center, College of Engineering, During the mid-1960s, 22 977 pregnant women in Scotland and England were followed up prospectively for the incidence of malformations in their infants evident at birth or within six weeks. During the first 13 weeks of gestation 620 of these women had been prescribed Debendox (dicyclomine-doxylamine-pyridoxine) and 743 other women agents other than Debendox containing pyridoxine. Of the 620 women given Debendox, 589 (95%) had a normal outcome of pregnancy, 8 (13%) delivered a malformed infant, and 23 (3.7%) had other outcomes. Of the 22 357 women who were given Debendox, 445 (2.0%) produced infants with malformation; and the rates for all abnormal outcomes among women given Debendox and those not given the drug were 5.0% and 5.4% respectively. These results support the hypothesis that Debendox is not teratogenic. Fleming, D M; Knox, J D; Crombie, D L In spite of their congenital origin, only 18-20% of cerebral AVMs are diagnosed during infancy and childhood. Intracranial haemorrhage is the presenting clinical manifestation in 75-80% of paediatric patients and is associated with a high morbidity and mortality. The natural history of untreated cerebral AVMs in children is worse than in adults, in relation to a longer life expectation, a higher annual risk of AVM bleeding (3.2% vs. 2.2%) and a higher incidence of posterior fossa and basal ganglia AVMs, most of which present with massive haemorrhages. The surgical excision remains the treatment of choice for parenchymal AVMs in children; AVM complete removal is currently achieved in 70-90% of the patients. With the advent of new agents for endovascular management, preoperative AVM embolization has further improved surgical results. Stereotactic radiosurgery appears to be a successful treatment option in small or moderate sized AVMs. Recent studies have demonstrated low complication rates with this technique in paediatric patients. We reviewed our experience with 37 paediatric AVMs treated at the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1980 and 1997. Twenty-three patients underwent surgery as the only treatment modality; endovascular embolization was combined with the surgical treatment in a further four cases. Radiosurgery was utilized as the only treatment in three patients and in combination with other techniques in an other three children (with surgery in one case and with AVM embolization in the remaining two subjects). No treatment was carried out in three patients because of excessively critical condition on admission; endovascular embolization failed in a further patient because of the anatomical complexity of the malformation. Previous studies have demonstrated a quite strict correlation between AVM complexity based on Spetzler and Martin's grading system and patients outcome. A less direct relationship has been observed in the present study. In our experience the factors which were more closely predictive of patients' outcome were the occurrence of an AVM bleeding and the neurological status on admission. In spite of the low number of cases in the single subsets of patients this study seems to support the role of AVM embolization and radiosurgery as effective adjuvant techniques in the management of cerebral AVMs in children. PMID:10795888 Di Rocco, C; Tamburrini, G; Rollo, M Ear canal acoustics was examined using a one-dimensional lossy transmission line with a distributed load impedance to model the ear. The acoustic input impedance of the ear was derived from sound pressure measurements in the ear canal of healthy human ears. A nonlinear least squares fit of the model to data generated estimates for ear canal radius, ear canal length, and quantified the resistance that would produce transmission losses. Derivation of ear canal radius has application to quantifying the impedance mismatch at the eardrum between the ear canal and the middle ear. The length of the ear canal was found, in general, to be longer than the length derived from the one-quarter wavelength standing wave frequency, consistent with the middle ear being mass-controlled at the standing wave frequency. Viscothermal losses in the ear canal, in some cases, may exceed that attributable to a smooth rigid wall. Resistance in the middle ear was found to contribute significantly to the total resistance. In effect, this analysis "reverse engineers" physical parameters of the ear from sound pressure measurements in the ear canal. PMID:23917695 Withnell, Robert H; Gowdy, Lauren E Introduction. Despite modern radiological workup, surgeons can still be surprised by intraoperative findings or by the pathologist's report. Materials & Methods. We describe the case of a 52-year-old male who was referred to our clinic with a single sided conductive hearing loss. He ultimately underwent middle ear exploration and excision of a middle ear tumour followed by second look and ossiculoplasty a year later. Results. Though preoperative CT and MRI scanning were suggestive of a congenital cholesteatoma, the pathologist's report diagnosed a middle ear adenoma. Discussion. Middle ear glandular tumors are extremely rare and, despite numerous histological techniques, continue to defy satisfactory classification. Most surgeons advocate surgical excision though evidence of the tumour's natural course and risk of recurrence is lacking. Vrugt, B.; Huber, A. M. The present report describes a case of percutaneous cryotherapy in a 36-year-old woman with a large and painful pectoral venous malformation. Cryoablation was performed in a single session for this 9-cm mass with 24 h hospitalisation. At 2- and 6-month follow-up, the pain had completely disappeared, and magnetic resonance imaging demonstrated a significant decrease in size. Percutaneous cryoablation shows promise as a feasible and apparently safe method for local control in patients with symptomatic venous vascular malformations. Cornelis, F., E-mail: [email protected] [Institut Bergonie, Department of Radiology (France); Neuville, A. [Institut Bergonie, Department of Pathology (France); Labreze, C. [Pellegrin Hospital, Department of Pediatric Dermatology (France); Kind, M. [Institut Bergonie, Department of Radiology (France); Bui, B. [Institut Bergonie, Department of Oncology (France); Midy, D. [Pellegrin Hospital, Department of Vascular Surgery (France); Palussiere, J. [Institut Bergonie, Department of Radiology (France); Grenier, N. [Pellegrin Hospital, Department of Radiology (France) We describe the presentation and management of gastrointestinal perforation in four neonates with anorectal malformations. Two neonates with high malformation had pneumoperitoneum on X-ray; surgery revealed sigmoid perforation in one patient and transverse colon perforation in the other. Colostomy was done, followed by posterior sagittal anorectoplasty at four months; both recovered satisfactorily. The third neonate had no radiological feature of gut perforation but cecal perforation was found at surgery; the neonate recovered following right hemicolectomy with stoma followed by anorectoplasty at five months. The fourth neonate presented with clinical and radiological features of perforation and recovered satisfactorily after anoplasty and colostomy. PMID:15250569 Sharma, Shyam B; Gupta, Vipul; Sharma, Vinod ... help with understanding microcephaly-capillary malformation syndrome? angiogenesis ; apoptosis ; autophagy ; autosomal ; autosomal recessive ; capillaries ; cell ; degrade ; developmental delay ; ... Vascular tumors affect the head and neck commonly but arteriovenous malformations are rare. Vascular malformations are often present at birth and grow with the patient, usually only becoming significant later in childhood. Embolization has been the mainstay of treatment in massive and complex arteriovenous malformations. We present a case of massive extracranial arteriovenous malformation in a 7-year-old boy causing significant workload on right heart and respiratory distress. The management of angioembolization under general anaesthesia and anaesthetic concerns are presented. Shamim, Faisal; Ullah, Hameed; Rehman, Azhar ... help with understanding VMCM? angiogenesis ; autosomal ; autosomal dominant ; calcium ; cell ; cutaneous ; deficiency ; endothelial cells ; gene ; inflammation ; inherited ; kinase ; lesion ; malformation ; ... An essential component in the process of hearing is the transformation of sound from acoustic to mechanical vibration in the middle ear. In order to study this phenomenon, computational models have been introduced to model the behavior of the tympanic membrane and its interaction with the surrounding acoustical spaces. Using such a computational model, one gains an increased understanding of the relationship between its structure and performance, which may assist in preventive, diagnostic, and reconstructive medical applications. The goal of this present work is to expand the computational simulation capabilities of current eardrum models using modern finite element modeling techniques. A fully coupled structural acoustic model is presented using modern shell element technology. Anatomical geometries for the eardrum, acoustic spaces of the ear canal and middle ear cavity, and the ossicles are utilized via muCT imaging. A new computational algorithm is used to compute the frequency response of this model over a wide frequency range. This approach uses the matrix Pade-via-Lanczos algorithm to construct reduced-order models around chosen reference frequencies, which can be solved efficiently at many frequencies within a frequency window. An adaptive algorithm is introduced to span a given frequency range by introducing new reference frequencies as necessary. Results for the middle ear model, using this multifrequency algorithm, are presented for intact and modified middle ear anatomies. These modifications serve to demonstrate the utility of the computational approach in understanding the relationships between the morphological structure of the middle ear and its functionality. Tuck-Lee, James Peter In a large scale screen for genetic defects in zebrafish embryogenesis we identified mutations affecting several aspects of ear development, including: specification of the otic placode, growth of the otic vesicle (otocyst), otolith formation, morphogenesis of the semicircular canals and differentiation of the otic capsule. Here we report initial phenotypic and genetic characterization of 20 of these mutations defining 13 independent loci. Embryos mutant at the quadro locus display abnormal specification of the otic placode. As revealed by dlx-3 expression, the otic field in the mutant embryos is smaller or split into two fields. At later stages of development the ear of quadro mutants is frequently divided into two smaller, incomplete units. Four loci affect ear shape shortly after formation of the otic vesicle. All of them also display abnormal brain morphology. Mutations in five loci result in the absence of otolith formation; two of these also produce changes of ear morphology. Two loci, little richard and golas, affect morphology of the otic vesicle shortly before formation of the semicircular canals. In both cases the morphogenesis of the semicircular canals is disrupted. Finally, the antytalent locus is involved in late expansion of the ear structure. Analysis of mutations presented here will strengthen our understanding of vertebrate ear morphogenesis and provide novel entry points to its genetic analysis. PMID:9007247 Malicki, J; Schier, A F; Solnica-Krezel, L; Stemple, D L; Neuhauss, S C; Stainier, D Y; Abdelilah, S; Rangini, Z; Zwartkruis, F; Driever, W Infants with limb reduction deficiencies (LRD) often have other associated congenital malformations. The purpose of this investigation was to assess the prevalence and the types of associated malformations in a defined population. This study included special strengths: each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for malformations was continued until 1 year Claude Stoll; Yves Alembik; Beatrice Dott; Marie-Paule Roth The Finnish Register of Congenital Malformations, a case-referent register, was used to analyze the associations between the parental occupation and the children born with malformations. The women working in industrial and construction occupations had more children with central nervous system (CNS) and musculoskeletal malformations than the referent mothers. The women employed in transport and communication occupations had more children with K. Hemminki; P. Mutanen; K. Luoma; I. Saloniemi A series of 24 patients with Chiari malformation and hydromyelia, treated at the Prince of Wales Children's Hospital between 1975 and 1991, is reviewed. The age range of these patients was 3 to 19 years. Eleven had a Chiari 1 malformation and 13 had a Chiari 2 malformation. The follow-up period ranged from 6 months to 16 years with a R. F. C Jones; J. G. J Ayer; W. A Stening Nine angiomatous malformations situated on the outer surface of the dura and one situated in the pelvis, from which draining veins pierced the dura and joined the coronal venous plexus are described. The clinical manifestations and myelographic appearances are similar to those of the majority of intradural angiomatous malformations. This suggests that neurological deficiencies with both types of malformation are B. E. Kendall; V. Logue The head is anatomically the most sophisticated part of the body and its evolution was fundamental to the origin of vertebrates; understanding its development is a formidable problem in biology. A synthesis of embryology, evolution and mouse genetics is shaping our understanding of head development and in this review we discuss its application to studies of human craniofacial malformations. Many Andrew O. M. Wilkie; Gillian M. Morriss-Kay Only limited information is available concerning dural arteriovenous malformations (AVMs) found in association with cerebral aneurysms. The present report focuses on six such cases, concentrating attention on clinical characteristics and significance. Of a total of 46 dural AVMs encountered over a given period, six (13%) were linked with cerebral aneurysms. Particularly strong associations were noted for dural AVMs in the Sachio Suzuki; Ryusui Tanaka; Yoshio Miyasaka; Akira Kurata; Makoto Takano; Kiyotaka Fujii; Hiroshi Takagi Arteriovenous malformations of the brain can carry considerable morbidity and mortality in the setting of rupture. The complex angioarchitecture and hemodynamic alteration requires careful consideration in diagnostic and management approaches. In this review, the authors define the pathophysiology, outline diagnostic methods, and highlight current management approaches. PMID:24504610 Asif, Kaiz; Leschke, John; Lazzaro, Marc A We report the first familial cases with two different types of posterior fossa cystic malformation and a leukodystrophic-like aspect on cerebral magnetic resonance imaging (MRI). The girl and her brother had severe encephalopathy, marked hypotonia, absent deep tendon reflexes, macrocrania, gigantism, and dysmorphic face and extremities. The girl had generalized seizures. The boy had unilateral cataract and bilateral optic atrophy. Véronique T. Humbertclaude; Philippe A. Coubes; Nicolas Leboucq; Bernard B. Echenne Congenital Pulmonary Airway Malformation (CPAM) and lung sequestration are often discovered on routine prenatal sonography. While some lesions may be large and cause serious complications in the fetus or newborn, many will be asymptomatic at birth. Some authors advocate simple observation because of the lack of data on the incidence of long-term complications. However, there are very few described cases Jean-Martin Laberge; Ioana Bratu; Hélène Flageole Summary A rare case of dural sinus malformation with dural arteriovenous fistula in an infant is presented. Presenting symptom was progressive macrocrania without hydrocephalus. A high flow arteriovenous of the sigmoid sinus associated with jugular bulb diaphragm was demonstrated. Reflux in the intracranial sinus was present. The shunt was successfully occluded by transarterial embolization. The natural history and treatment strategy are discussed. Yau, C.Kwong; Alvarez, H.; Lasjaunias, P. Summary A rare case of dural sinus malformation with dural arteriovenous fistula in an infant is presented. Presenting symptom was progressive macrocrania without hydrocephalus. A high flow AVS of the sigmoid sinus associated with jugular bulb diaphragm was demonstrated. Reflux in the intracranial sinus was present. The shunt was successfully occluded by transarterial embolization. The natural history and treatment strategy are discussed. Yau, C.K.; Alvarez, H.; Lasjaunias, P. The craniometric linear dimensions of the posterior fossa have been relatively well studied, but angular craniometry has been poorly studied and may reveal differences in the several types of craniocervical junction malformation. The objectives of this study were to evaluate craniometric angles compared with normal subjects and elucidate the main angular differences among the types of craniocervical junction malformation and the correlation between craniocervical and cervical angles. Angular craniometries were studied using primary cranial angles (basal and Boogard's) and secondary craniocervical angles (clivus canal and cervical spine lordosis). Patients with basilar invagination had significantly wider basal angles, sharper clivus canal angles, larger Boogard's angles, and greater cervical lordosis than the Chiari malformation and control groups. The Chiari malformation group does not show significant differences when compared with normal controls. Platybasia occurred only in basilar invagination and is suggested to be more prevalent in type II than in type I. Platybasic patients have a more acute clivus canal angle and show greater cervical lordosis than non-platybasics. The Chiari group does not show significant differences when compared with the control, but the basilar invagination groups had craniometric variables significantly different from normal controls. Hyperlordosis observed in the basilar inavagination group was associated with craniocervical kyphosis conditioned by acute clivus canal angles. PMID:23640096 Botelho, Ricardo Vieira; Ferreira, Edson Dener Zandonadi Auricular reattachment and reconstruction following traumatic ear avulsion is a challenging surgical problem. Suggested reconstruction methods include direct reattachment, composite grafting, pocket methods, coverage with periauricular flaps, and microsurgical repair. A published alternative is reattachment and burial of the amputated part under a postauricular flap. If circumstances delay the surgical intervention, what is a safe window to still consider this form of treatment? In the current report, we present a case of a complete partial ear avulsion, which was reattached in the ER as a free graft. Two days later the cartilage was banked under a postauricular flap. A 22 year-old male had his ear was completely bitten off which was re-attached. Two days later the skin of the avulsed segment was dark and bloodless. The avulsed segment was removed from the ear. The overlying skin was dissected off of the attached ear leaving perichondrium on the medial aspect of the ear. The cartilage was then reattached. A postauricular pocket/flap was created and the ear tucked and secured to the postauricular fascia and skin closed overtop. Five weeks later, the patient had division and inset of the flap with a full thickness skin graft to the posterior aspect of the ear. Our results and experiences suggest that immediate reconstruction may not be crucial. PMID:24793138 Brockhoff, Hans C; Zide, Michael This article describes the effect of various pathologies on power reflectance (PR) and absorbance measured in human adults. The pathologies studied include those affecting the tympanic membrane, the middle-ear ossicles, the middle-ear cavity, the inner ear, and intracranial pressure. Interesting pathology-induced changes in PR that are statistically significant have been reported. Nevertheless, because measurements of PR obtained from normal-hearing subjects have large variations and some pathology-induced changes are small, it can be difficult to use PR alone for differential diagnosis. There are, however, common clinical situations without reliable diagnostic methods that can benefit from PR measurements. These conditions include ears with a normal-appearing tympanic membrane, aerated middle-ear cavity, and unknown etiology of conductive hearing loss. PR measurements in conjunction with audiometric measurements of air–bone gap have promise in differentiating among stapes fixation, ossicular discontinuity, and superior semicircular canal dehiscence. Another possible application is to monitor an individual for possible changes in intracranial pressure. Descriptions of mechanisms affecting PR change and utilization of PR measurements in clinical scenarios are presented. PMID:23900180 Nakajima, Hideko Heidi; Rosowski, John J; Shahnaz, Navid; Voss, Susan E Besides eradication of chronic middle ear disease, the reconstruction of the sound conduction apparatus is a major goal of modern ear microsurgery. The material of choice in cases of partial ossicular replacement prosthesis is the autogenous ossicle. In the event of more extensive destruction of the ossicular chain diverse alloplastic materials, e.g. metals, ceramics, plastics or composits are used for total reconstruction. Their specialised role in conducting sound energy within a half-open implant bed sets high demands on the biocompatibility as well as the acoustic-mechanic properties of the prosthesis. Recently, sophisticated titanium middle ear implants allowing individual adaptation to anatomical variations are widely used for this procedure. However, despite modern developments, hearing restoration with passive implants often faces its limitations due to tubal-middle-ear dysfunction. Here, implantable hearing aids, successfully used in cases of sensorineural hearing loss, offer a promising alternative. This article reviews the actual state of affairs of passive and active middle ear implants. Beutner, Dirk; Huttenbrink, Karl-Bernd The middle ear cavity is exposed and vulnerable to waterborne infection in patients with grommets, perforated tympanic membranes and after radical mastoidectomy. Patients suffering from chronic otitis externa and those receiving radiotherapy to the head and neck also have an increased susceptibility to such infections. Many advocate the use of waterproof ear protectors in such patients when swimming. The choice of a suitable ear protector is complicated as many are now available commercially. This study was therefore designed to evaluate the degree of protection afforded by seven different ear protectors in a group of six swimmers. A very sensitive, original method of water detection was devised incorporating a pH indicator strip. The results showed conclusively that cotton wool coated in paraffin jelly BPC was the most effective method of ear protection and was found to be comfortable and easy to use. Other methods, including custom-made silicone rubber plugs, were not adequate in sealing the external auditory canal and are considerably more expensive. PMID:2614235 Robinson, A C This paper describes the effect of various pathologies on power reflectance (PR) and absorbance measured in human adults. The pathologies studied include those affecting the tympanic membrane, the middle-ear ossicles, the middle-ear cavity, the inner ear, and intracranial pressure. Interesting pathology-induced changes in PR that are statistically significant have been reported. Nevertheless, because measurements of PR obtained from normal-hearing subjects have large variations and some pathology-induced changes are small, it can be difficult to use PR alone for differential diagnosis. There are, however, common clinical situations without reliable diagnostic methods that can benefit from PR measurements. These conditions include ears with a normal-appearing tympanic membrane, aerated middle-ear cavity and unknown etiology of conductive hearing loss. PR measurements in conjunction with audiometric measurements of air-bone gap have promise in differentiating among stapes fixation, ossicular discontinuity and superior semicircular canal dehiscence. Another possible application is to monitor an individual for possible changes in intracranial pressure. Descriptions of mechanisms affecting PR change and utilization of PR measurements in clinical scenarios are presented. Nakajima, Hideko Heidi; Rosowski, John J.; Shahnaz, Navid; Voss, Susan E. Mouse embryonic fibroblasts (MEFs) are commonly utilized as a primary cell culture model and have several advantages over other types of ex vivo-derived cells. However, the successful generation of MEFs is time consuming and requires a certain level of mouse expertise to successfully complete. Thus, primary ear-derived fibroblasts offer an acceptable alternative to MEFs. Fibroblasts derived from the pinna of adult mice are easily attainable with minimal skill, proliferate rapidly, and are easy to manipulate. Likewise, because they are derived from adult mice, other organs can be concurrently harvested for the isolation of additional types of primary cells. Similar to MEFs, ear fibroblasts are an excellent ex vivo model system to study mechanisms associated with virus infection and produce a diverse array of inflammatory mediators, such as cytokines and interferon. Here, we describe a highly versatile and simple method for the derivation, maintenance, and viral challenge of primary ear-derived fibroblasts from mice. PMID:23824888 Moore, Chris B; Allen, Irving C Surgical reconstruction of an earlobe requires adequate support without sacrificing the delicacy necessary for an attractive result. A two-stage ear lobule reconstruction using a mastoid skin pocket and cartilage from the nasal septum was performed in six patients. The earlobe aesthetics were acceptable and allowed ear piercing. There were no major complications, including no loss of flap, graft extrusion, septal perforation, or infection. Range of follow-up was 1 to 6 years, with an average of 3 years. No revisions have been performed. A two-stage technique for ear lobule reconstruction is described using septal cartilage to preserve shape and definition that has the additional advantage of minimal morbidity. PMID:23542248 Bastidas, Nicholas; Jacobs, Jordan M S; Thorne, Charles H The cosmetic result of an ear restored surgically or via prosthetics is dependent on the surgeon''s ability to carve a precise cartilage armature at the time of surgery or the prosthetist''s ability to sculpt in wax an exact duplicate of the patient''s " missing" ear. Introducing CAD/CAM technology into the process benefits the esthetic outcome of these procedures. By utilizing serial section information derived from CAT MRI or moulage techniques a mirrorimage of the patient''s " donor" ear is generated. The resulting earform data is then used for the design of a cartilage armature produced by multi-axis milling or to produce by stereolithography a model which serves as the basis for a prosthesis. Evenhouse, Raymond J.; Chen, Xiaoming Dermoid cysts of the head and neck are rare lesions comprised of epidermal and mesodermal elements. We report a dermoid cyst presenting in the middle ear of the youngest patient reported to date. Structures of endodermal descent were also identified, but, given that the entire middle ear mucosa is of endodermal origin, specific classification as a teratoma would be imprecise. This lesion is interesting in that it did not directly involve the mastoid. Possible embryologic sites of origin are discussed. ImagesFigure 1Figure 2Figure 3Figure 4Figure 5 Farris, Paul E.; Meyerhoff, William L.; Vuitch, Frank Mayer Rokitansky Kuster Houser syndrome (MRKH syndrome) is characterized by Mullerian duct structures agenesis, vaginal atresia\\u000a being the commonest variant. It can be associated with renal, skeletal, spine and other malformations. Patient with Mayer\\u000a Rokitansky syndrome has a varied presentation from newborn period to adolescence. Thorough investigations are required for\\u000a classification of the syndrome and diagnosis of associated anomalies. The Shreeprasad P. Patankar; Vijay Kalrao I; Shilpa S. Patankar Cutaneous cystic lymphatic malformations consist of dilatations of various sizes, developed from the lymphatic system. Various clinical presentations exist, which are more or less complex and severe. MRI is the best examination to determine the extent of deep lesions. Therapeutic decisions usually follow multidisciplinary consultations. In localized superficial forms, whether surgical excision or abstention is required. In macrocystic forms, sclerotherapy is the first-line treatment. PMID:21074349 Lorette, Gérard; Vaillant, Loïc; Wiersbicka, Ewa; Herbreteau, Denis The treatment of arteriovenous malformations (AVMs) requires a multidisciplinary management including microsurgery, endovascular embolization, and stereotactic radiosurgery (SRS). This article reviews the recent advancements in the multimodality treatment of patients with AVMs using endovascular neurosurgery and SRS. We describe the natural history of AVMs and the role of endovascular and radiosurgical treatment as well as their interplay in the management of these complex vascular lesions. Also, we present some representative cases treated at our institution. Plasencia, Andres R.; Santillan, Alejandro Malformations of cortical development (MCDs) are macroscopic or microscopic abnormalities of the cerebral cortex that arise as a consequence of an interruption to the normal steps of formation of the cortical plate. The human cortex develops its basic structure during the first two trimesters of pregnancy as a series of overlapping steps, beginning with proliferation and differentiation of neurons, which then migrate before finally organizing themselves in the developing cortex. Abnormalities at any of these stages, be they environmental or genetic in origin, may cause disruption of neuronal circuitry and predispose to a variety of clinical consequences, the most common of which is epileptic seizures, A large number of MCDs have now been described, each with characteristic pathological, clinical, and imaging features. The causes of many of these MCDs have been determined through the study of affected individuals, with many MCDs now established as being secondary to mutations in cortical development genes. This review will highlight the best-known of the human cortical malformations associated with epilepsy. The pathological, clinical, imaging, and etioiogic features of each MCD will be summarized, with representative magnetic resonance imaging (MRI) images shown for each MCD, The malformations tuberous sclerosis, focal cortical dysplasia, hemimegalencephaiy, classical iissencephaly, subcortical band heterotopia, periventricular nodular heterotopia, polymicrogyria, and schizencephaly will be presented. Leventer, Richard J.; Guerrini, Renzo; Dobyns, William B. Malformations of the inferior vena cava (IVC) are rare presentations, exceptional in children, and are usually asymptomatic.They are caused by disturbances in the embryological formation of the venous system or can develop as a result of perinatal venous thrombosis with secondary impairment of the venous development. We report the case of a 14 year old boy,admitted for pediatric evaluation before undergoing plastic surgery in order to remove superficial varicose veins of the lower abdomen. The patient presents with inequality in circumference and length of the legs. Laboratory investigations are normal and the abdominal ultrasound describes hypoplasia of the retrohepatic segment of the inferior vena cava. The diagnosis of complex malformation of the abdominal deep venous system (retrohepatic vena cava atresia, cavo-caval anastomosis through azygos veins, abnormal formation of the inferior vena cava with the absence of the left iliac vein) was established through a CT angiography. The presence of abdominal varicose dilations should indicate the necessity to closely look for malformations of the portal and/or caval venous systems. PMID:24742422 Lesanu, G; Balanescu, R; Pacurar, D; Iaru, O; Vlad, R M; Topor, L; Oraseanu, D Introduction Congenital lung malformations are rare lesions that are most commonly diagnosed antenatally. Management of such lesions, particularly those that are asymptomatic, remains controversial. We undertook a survey to ascertain current practice of surgeons in the UK and Ireland. Methods All consultant members of the British Association of Paediatric Surgeons were asked to complete a survey on congenital lung malformations with respect to antenatal management, symptomatic and asymptomatic lesions, and operative techniques. Results Responses were received from 20 paediatric surgical centres and highlighted the ongoing variability in management of such lesions, particularly those that are asymptomatic. Twenty per cent of surgeons never resect an asymptomatic lesion and twenty-four per cent always do. The remainder intervene selectively, with size being the most commonly stated indication. Most resections are undertaken via thoracotomy although 35% of surgeons use thoracoscopy for some procedures. Conclusions National data based on congenital anomaly registers are needed to determine the natural history of these malformations and to guide future management. Burge, DM; Marven, SS Modern neuroembryology integrates descriptive morphogenesis with more recent insight into molecular genetic programing and data enabled by cell-specific tissue markers that further define histogenesis. Maturation of individual neurons involves the development of energy pumps to maintain membrane excitability, ion channels, and membrane receptors. Most malformations of the nervous system are best understood in the context of aberrations of normal developmental processes that result in abnormal structure and function. Early malformations usually are disorders of genetic expression along gradients of the three axes of the neural tube, defective segmentation, or mixed lineages of individual cells. Later disorders mainly involve cellular migrations, axonal pathfinding, synaptogenesis, and myelination. Advances in neuroimaging now enable the diagnosis of many malformations in utero, at birth, or in early infancy in the living patient by abnormal macroscopic form of the brain. These images are complimented by modern neuropathological methods that disclose microscopic, immunocytochemical, and subcellular details beyond the resolution of MRI. Correlations may be made of both normal and abnormal ontogenesis with clinical neurological and EEG maturation in the preterm or term neonate for a better understanding of perinatal neurological disease. Precision in terminology is a key to scientific communication. PMID:23622157 Sarnat, Harvey B; Flores-Sarnat, Laura The impedance of the middle-ear air space was measured on three human cadaver ears with complete mastoid air-cell systems. Below 500 Hz, the impedance is approximately compliance-like, and at higher frequencies (500-6000 Hz) the impedance magnitude has several (five to nine) extrema. Mechanisms for these extrema are identified and described through circuit models of the middle-ear air space. The measurements demonstrate that the middle-ear air space impedance can affect the middle-ear impedance at the tympanic membrane by as much as 10 dB at frequencies greater than 1000 Hz. Thus, variations in the middle-ear air space impedance that result from variations in anatomy of the middle-ear air space can contribute to inter-ear variations in both impedance measurements and otoacoustic emissions, when measured at the tympanic membrane. Stepp, Cara E.; Voss, Susan E. According to the development of body temperature measurement mode, an ear thermometer with infrared thermopiles sensor is designed for body thermometry Compared with oral thermometer, the accuracy of ear thermometer is acceptable. PMID:24409789 Xie, Haiyuan; Qian, Mingli Complex combined vascular malformations affecting extremities are an interesting group of vascular malformations because, in addition to the vascular channel anomalies present, they can be associated with other tissue changes and sometimes altered limb growth. At present, magnetic resonance imaging is the gold standard imaging tool to evaluate such complex conditions in children because of its inherent tissue specificity and vascular capabilities that enables characterization of tissues and the vascular channel anomalies both for diagnosis and management of the patient. A brief review of some of these conditions is presented, including Klippel-Trénaunay syndrome, Parkes Weber syndrome, extensive diffuse low-flow venous malformations, Bannayan-Riley-Ruvalcaba syndrome, cutis marmorata telangiectatica congenita, Maffucci's syndrome, and Gorham-Stout disease. PMID:19724993 Lobo-Mueller, Edrise; Amaral, Joao G; Babyn, Paul S; Wang, Qiuyan; John, Philip Inner ear pathology was studied in adult rats with lipoid nephrosis induced by puromycin aminonucleoside. Although no abnormality was observed in auditory brain-stem responses, significant changes were noted in the stria vascularis. The most striking observation was that intermediate cells were markedly swelled, there-by pressing adjacent marginal cells. Severely affected marginal cells have vacuoles and increased lysosomes and protruded toward H. Yamane; Y. Nakai R Mike Mullane is a US astronaut who has flown into space three times on the Space Shuttle. He resigned from NASA in 1990 and has since pursued a career as a professional speaker and author. Do Your Ears Pop in Space? is his third book, and is based on the simple idea of writing down the 500 questions he This curriculum was designed to help teachers teach their fourth-grade students about hearing and the effects of loud noises on hearing. The program describes the human ear and how it works, explains the health effects of noise, and offers ways for students to protect their hearing from unsafe noise levels. Students are taught how hearing is… Olson, Dianne R. This article suggests that the development of listening skills should extend to the "soundscape" of nonspeech acoustical information. It presents a model for effective aural processing, identifies categories of information obtained from nonverbal sound, and explores "ear-tuning" or listening exercises that use sound to glean information about… The general status of a grant to investigate the origins and evolution of two hair cell types in the ears of a teleost fish, Astronotus ocellatus (the oscar), is presented. First, it was demonstrated that the cells in the rostral end of the saccule of the... A. N. P. I. Popper The overall objective in defining feature space is to reduce the dimensionality of the original pattern space, whilst maintaining discriminatory power for classification. To meet this objec- tive in the context of ear biometrics a new force field transformation treats the image as an array of mutually attracting particles that act as the source of a Gaussian force field. Under- David J. Hurley; Mark S. Nixon; John N. Carter Audiobooks have been around for years in various formats, like cassette tapes and CDs. This article describes a new type of audiobook on the market which is generating an interest in "reading." The device, called Playaway, is the size of a MP3 player and comes with a lanyard and ear buds. Buttons on the back of the player control the speed and… Fues, Marianne Cole Malformations of neocortical development are associated with cognitive dysfunction and increased susceptibility to epileptogenesis. Rodent models are widely used to study neocortical malformations and have revealed important genetic and environmental mechanisms that contribute to neocortical development. Interestingly, several inbred mice strains commonly used in behavioral, anatomical, and/or physiological studies display neocortical malformations. In the present report we examine the cytoarchitecture and myeloarchitecture of the neocortex of 11 inbred mouse strains and identified malformations of cortical development, including molecular layer heterotopia, in all but one strain. We used in silico methods to confirm our observations and determined the transcriptional profiles of cells found within heterotopia. These data indicate cellular and transcriptional diversity present in cells in malformations. Furthermore, the presence of dysplasia in nearly every inbred strain examined suggests that malformations of neocortical development are a common feature in the neocortex of inbred mice. Ramos, Raddy L.; Smith, Phoebe T.; DeCola, Christopher; Tam, Danny; Corzo, Oscar Malformations can occur in all living species, but there is little information about anomalies that occur in snakes and their frequency. This study assessed malformations in newborn South American pit vipers (Bothrops jararaca) and South American rattlesnakes (Crotalus durissus) from wild captured pregnant females (240 and 35 litters, respectively). Newborn snakes were measured, weighed, sexed and studied grossly and by radiography for the presence of malformations. Ninety-five malformed pit vipers were identified from 4,087 births (2.3%), while 36 malformed rattlesnakes were found from 324 births (11.1%). Spinal abnormalities were the most common in both species, followed by fusion of ventral scales. Pit vipers showed a greater range of malformations including schistosomia (22.1%), kinked tail (13.7%), bicephaly (3.1%) and hydrocephaly (2.1%). PMID:23885804 Sant'Anna, S S; Grego, K F; Lorigados, C A B; Fonseca-Pinto, A C B C; Fernandes, W; Sá-Rocha, L C; Catão-Dias, J L Ear recognition, as a biometric, has several advantages. In particular, ears can be measured remotely and are also relatively static in size and structure for each individual. Unfortunately, at present, good recognition rates require controlled conditions. For commercial use, these systems need to be much more robust. In particular, ears have to be recognized from different angles (poses), under different John D. Bustard; Mark S. Nixon ...part. Publicly available technology and software not subject to the EAR are described in...States, or release of technology or software subject to the EAR to a foreign national...encryption source code and object code software subject to the EAR. (2) Export... Ear decomposition of a graph has been extensively studied in relation to graph connectivity. In this paper, a connection of ear decomposition to graph embeddings is exhibited. It is shown that constructing a maximumpaired ear decomposition of a graph and constructing a maximum-genus embedding of the graph are O (e log n)-time equivalent. This gives a polynomial time algorithm for Jianer Chen; Saroja P. Kanchi Inspired by the micro-scale ears of the fly Ormia, which show remarkable sound localization ability, the overall objective of this project is to achieve a further understanding of the mechanism the fly ear and use this understanding to develop Fly-Ear Ins... Background.- Use of MRI makes the association Chiari malformation-cranio-facial synostosis more frequent than expected. The aim of this work was to ascertain the true incidence of Chiari malformation and to understand the reasons of the association between a bone pathology and a CNS malformation.Patients and methods.- The anatomy of the posterior cranial fossa in Crouzon syndrome was retrospectively studied on G Cinalli; D Renier; G Sebag; C Sainte-Rose; E Arnaud; A Pierre-Kahn Congenital cardiovascular malformations (CCVMs) of the left side of the heart show familial recurrence of various forms of\\u000a obstructive malformations, including hypoplastic left heart (HLH), interrupted aortic arch, coarctation of the aorta, and\\u000a aortic stenosis. In a previous population-based study in the Baltimore–Washington region, these malformations were associated\\u000a with parental reports of occupational or leisure solvent exposure, overt diabetes, and K. S. Kuehl; C. A. Loffredo The patient is a healthy 11-year-old girl with no history of trauma or hearing impairment. She developed pneumococcal meningitis three times, at ages 7, 10, and 11. Intracranial examination revealed, pore expansion and cerebrospinal fluid leakage in the right internal acoustic foramen, which were attributed to a bone malformation of the base of the skull. A procedure was performed to close the cerebrospinal fluid leakage; no relapse has been observed thus far. Previous case reports indicate that repetitive bacterial meningitis is often caused by internal ear malformation, trauma, tumors, or surgical operation. This case suggests the possibility that underlying disorders may not be apparent in cases of repetitive bacterial meningitis and, more proactive investigations are required to prevent further recurrence of meningitis. PMID:24084029 Kajimoto, Madoka; Hasegawa, Shunji; Nomura, Sadahiro; Inoue, Hirofumi; Matsushige, Takeshi; Ichiyama, Takashi Endovascular embolization is being increasingly used to treat intracranial arteriovenous malformations (AVMs). However, we experienced two patients with retained microcatheters after AVM embolization using Onyx. Lee, Jae Il; Ko, Jun Kyeung; Lee, Tae Hong Malformations are significant contributions to childhood mortality and disability. Their co-occurrence with intellectual disability may compound the health burden, requiring additional evaluation and management measures. Overall, malformations of greater or lesser severity occur in at least some cases of almost half of the 153 XLID syndromes. Genitourinary abnormalities are most common, but tend to contribute little or no health burden and occur in only a minority of cases of a given XLID syndrome. Some malformations (e.g., lissencephaly, hydranencephaly, long bone deficiency, renal agenesis/dysplasia) are not amenable to medical or surgical intervention; others (e.g., hydrocephaly, facial clefting, cardiac malformations, hypospadias) may be substantially corrected. PMID:24166814 Stevenson, Roger E; Schwartz, Charles E; Rogers, R Curtis Endovascular embolization is being increasingly used to treat intracranial arteriovenous malformations (AVMs). However, we experienced two patients with retained microcatheters after AVM embolization using Onyx. PMID:22949969 Lee, Jae Il; Choi, Chang Hwa; Ko, Jun Kyeung; Lee, Tae Hong Infants with neural tube defects (NTDs) often have associated congenital anomalies. The reported frequency and types of associated malformations vary between different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with NTDs in a geographically well-defined population from 1979 to 2008 of 402,532 consecutive births. Of the 441 infants with NTDs born during this period, 20.4% had associated malformations. Infants with associated malformations were divided into those with recognizable conditions [11 (2.5%) infants with chromosomal and 23 (5.2%) with non-chromosomal conditions], and those without recognizable conditions [56 (12.7%) infants with multiple malformations]. Associated malformations were more frequent among infants with encephalocele (36.8%) than those with anencephaly (11.5%) or spina bifida (23.8%). Oral clefts and malformations in the musculoskeletal, renal and cardiovascular systems were the most commonly observed associated anomalies. The frequency of associated malformations in infants with NTDs emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations, especially facial clefts and musculoskeletal, renal and cardiac anomalies, may need to be considered in infants with NTDs, and referral of these infants for genetics evaluation and counseling seems warranted. PMID:21337695 Stoll, Claude; Dott, Beatrice; Alembik, Yves; Roth, Marie-Paule Infants with radial ray deficiencies very often have other associated congenital anomalies. The reported frequency and types of associated malformations vary between different studies. The purpose of this investigation was to assess the frequency and types of associated malformations among infants with radial ray deficiencies in a geographically well-defined population from 1979 to 2004 of 346,831 consecutive births. Of the 73 infants with radial ray deficiencies born during this period (prevalence at birth of 2.1 per 10,000), 75% had associated malformations. Infants with associated malformation were divided into recognizable conditions (16 (22%) infants with chromosomal and 20 (27%) with non chromosomal conditions), and non recognizable conditions (19 (26%) infants with multiple malformations). Trisomies 18 and autosomal deletions were the most frequent chromosomal abnormalities. VACTERL association, thrombocytopenia absent radii syndrome, Fanconi anemia and Holt-Oram syndrome were most often present in recognizable non chromosomal conditions. Malformations in the musculoskeletal, cardiovascular and urogenital systems were the most common other anomalies in infants with multiple malformations and non recognizable conditions. The frequency of associated malformations in infants with radial ray deficiencies emphasizes the need for a thorough investigation of these infants. Routine screening for other malformations especially musculoskeletal, cardiac and urogenital systems anomalies may need to be considered in infants with radial ray deficiencies, and referral of these infants for genetic evaluation and counseling seems warranted. PMID:24032294 Stoll, C; Dott, B; Alembik, Y; Roth, M-P The ear canal sound pressure and the malleus umbo velocity with bone conduction (BC) stimulation were measured in nine ears from five cadaver heads in the frequency range 0.1 to 10 kHz. The measurements were conducted with both open and occluded ear canals, before and after resection of the lower jaw, in a canal with the cartilage and soft tissues removed, and with the tympanic membrane (TM) removed. The sound pressure was about 10 dB greater in an intact ear canal than when the cartilage part of the canal had been removed. The occlusion effect was close to 20 dB for the low frequencies in an intact ear canal; this effect diminished with sectioning of the canal. At higher frequencies, the resonance properties of the ear canal determined the effect of occluding the ear canal. Sectioning of the lower jaw did not significantly alter the sound pressure in the ear canal. The sound radiated from the TM into the ear canal was investigated in four temporal bone specimens; this sound is significantly lower than the sound pressure in an intact ear canal with BC stimulation. The malleus umbo velocity with air conduction stimulation was investigated in nine temporal bone specimens and compared with the umbo velocity obtained with BC stimulation in the cadaver heads. The results show that for a normal open ear canal, the sound pressure in the ear canal with BC stimulation is not significant for BC hearing. At threshold levels and for frequencies below 2 kHz, the sound in the ear canal caused by BC stimulation is about 10 dB lower than air conduction hearing thresholds; this difference increases at higher frequencies. However, with the ear canal occluded, BC hearing is dominated by the sound pressure in the outer ear canal for frequencies between 0.4 and 1.2 kHz. Stenfelt, Stefan; Wild, Timothy; Hato, Naohito; Goode, Richard L. Oral Vascular Malformations (OVM) are congenital anomalies characterized by morph-structural and/or functional changes of nature in severity and extension. OVM can affect any type of vessels arterial, venous or lymphatic and any capillary or anatomical. They are divided into two categories: low and high flow. In this study were treated 40 patients with OVM with a range size from 2 mm to 44 mm; they were subjected to clinical examination supported by Colour-Doppler Ultrasound instrumental examination and only for doubt cases the Magnetic Resonance Imaging (MRI) was prescribed. Only low flow venous and capillary malformations were treated by GaAlAs laser (Wiser®, Lambda, Brindole,Italy, 980nm) and KTP laser (SmartLite®, DEKA, Florence, Italy, 532nm) with two different techniques: the Transmucosal Thermophotocoagulation (TMT) and the Intralesional Photocoagulation (ILP). These techniques permitted a good control of haemostasis, avoiding bleeding both during surgery and in the postoperative. It is obtained an excellent and good healing respectively in 10% and 60% of cases, a moderate and poor resolution respectively in 22.5% and 7.5% of cases. A clear diagnosis allowed the management of Venous malformations (VM) by laser devices with wavelengths highly absorbed in haemoglobin in safety and efficacy and according to the principles of minimal invasive surgery. The aim of this study was to verify if the laser is effective in the treatment of OVM for the purpose of the clinical findings and the postoperative course. The Authors concluded that the laser can be considered the "gold standard" for treating OVM. Romeo, U.; Gaimari, G.; Mohsen, M.; Tenore, G.; Palaia, G. A 1-year-old castrated male Saanen goat was observed to have drooping and edema of the left ear consistent with published accounts of gotch ear in cattle associated with a tick bite. The goat's left ear was edematous from the tip of the pinna to the base of the ear. No signs of trauma or infectious processes were observed. Three engorged Gulf Coast ticks (Amblyomma maculatum) were observed attached inside the ear. Ticks were removed and the ear biopsied at tick attachment sites. The affected ear was treated topically with betadine after removal of the ticks. No other treatment was administered. The goat remained free of clinical signs and the edema of the ear resolved within 3 days after tick removal. No clinical adverse effects of the condition were evident. All three ticks were positive for spotted fever group rickettsia by polymerase chain reaction analysis and showed 100% similarity with the homologous sequence of Rickettsia parkeri. There was no immunohistochemical evidence of spotted fever group rickettsia in the ear samples, supporting the hypothesis that gotch ear is not due to rickettsial infection. This report represents the first apparent case of gotch ear in a goat. PMID:21395411 Edwards, Kristine T; Varela-Stokes, Andrea S; Paddock, Christopher D; Goddard, Jerome Spinal arteriovenous malformations (sAVMs) are rare vascular lesions whose natural history remains incompletely defined. Several classification schemes for sAVMs have evolved based on an improved understanding of the anatomic characteristics as well as pathophysiologic behavior of these arteriovenous shunts. Advances in endovascular technology have inspired the adoption of interventional techniques both as stand-alone treatment and as part of a multi-modality management paradigm for sAVMs. Further refinements in liquid embolic agents as well as improved microcatheter navigability will contribute to an ever-expanding role for endovascular intervention in the management of these lesions. PMID:22935350 Ducruet, Andrew F; Crowley, R Webster; McDougall, Cameron G; Albuquerque, Felipe C Zoster sine herpete (ZSH) is difficult to diagnosis during an acute period due to the absence of the characteristic zosteriform dermatomal rash; therefore, progression to postherpetic neuralgia is more common than typical zoster. In addition, misdiagnosis of other neuropathic pain as ZSH is common in clinical situations. Here, we report a case of spinal arteriovenous malformation that mimics ZSH. This is a rare condition; therefore, high clinical suspicion for a correct diagnosis and proper examination are not easy. However, early diagnosis and definitive treatment are essential to prevent neurologic deficit and mortality. Lee, Ji Young; Ok, Se Jin; Oh, Chang Keun; Park, Sun Kyung; Kim, Do Wan Pediatric aneurysms are different from adult aneurysms – they are more rare, are giant and in the posterior circulation more frequently than in adults and may be associated with congenital disorders. Infectious and traumatic aneursyms are also seen more frequently. Vein of Galen malformations are even rarer entities. They may be of choroidal or mural type. Based on the degree of AV shunting they may present with failure to thrive, with hydrocephalus or in severe cases with heart failure. The only possible treatment is by endovascular techniques – both transarterial and transvenous routes are employed. Rarely transtorcular approach is needed. These cases should be managed by an experienced neurointerventionist. Rao, V. R. K.; Mathuriya, S. N. We report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM. Jin, Sung-Chul; Ahn, Jae-Sung; Kwon, Do Hoon Background Both the diagnosis and treatment regimens for the Chiari I malformation (CIM) are varied and controversial. The present paper\\u000a analyzes the literature regarding this form of hindbrain herniation in regard to definition, anatomy, pathobiology, symptoms,\\u000a findings, treatment, and outcomes.\\u000a \\u000a \\u000a \\u000a Discussions Appropriate literature germane to the CIM is reviewed and discussed. There is variation in the reported anatomy, outcome,\\u000a and treatment for R. Shane Tubbs; Michael J. Lyerly; Marios Loukas; Mohammadali M. Shoja; W. Jerry Oakes Summary \\u000a ?Background. The present study was conducted to establish an animal model for the investigation of the pathophysiology and haemodynamics\\u000a of cerebral arteriovenous malformation (AVM) but also to assess therapeutic aspects.\\u000a \\u000a \\u000a \\u000a ?Method. For anatomic and haemodynamic reasons, dogs were chosen as the animal model. An arteriovenous fistula was created by interposing\\u000a a segment of the superficial temporal artery between one T. A. Pietilä; J. M. Zabramski; A. Thèllier-Janko; K. Duveneck; W. D. Bichard; M. Brock; R. F. Spetzler We describe measurements of middle-ear input admittance in chinchillas (Chinchilla lanigera) before and after various manipulations that define the contributions of different middle-ear components to function. The chinchilla’s middle-ear air spaces have a large effect on the low-frequency compliance of the middle ear, and removing the influences of these spaces reveals a highly admittant tympanic membrane and ossicular chain. Measurements of the admittance of the air spaces reveal that the high-degree of segmentation of these spaces has only a small effect on the admittance. Draining the cochlea further increases the middle-ear admittance at low frequencies and removes a low-frequency (less than 300 Hz) level dependence in the admittance. Spontaneous or sound-driven contractions of the middle-ear muscles in deeply anesthetized animals were associated with significant changes in middle-ear admittance. Rosowski, John J.; Ravicz, Michael E.; Songer, Jocelyn E. The anatomy of the middle ear has been studied in nine species of birds, with particular reference to the structure of the ossicle and its relationship to the tympanic membrane. The morphology of the avian middle ear has been compared to that of the reconstructed human middle ear. Drum to stapes foot plate assemblies created during ossiculoplasty operations differ from the pattern found in the avian middle ear in a number of important respects and this may help to explain why they are often unsuccessful. It is not technically feasible to reproduce the avian middle ear pattern exactly in the human middle ear and developments in reconstructive technique should therefore be directed towards reproducing the three ossicle pattern of the mammalian ear. Images Figure 1. Figure 2. The D0 endcap calorimeter outer hadronic (OH) modules play a major structural role in the calorimeter assembly. The disrete modules, once connected together, form a ring within which other massive calorimetry will reside. It has been proposed that the connection of the OH at the downstream end be accomplished by extending the downstream endplates in the radial direction to form 'ears', and then through-bolting between adjacent ears as shown in Fig. 1. A single 2 1/4 in. dia. bolt is used, and previous calculations have determined that the design load on this joint should be 130,000 lbs tension. The high load and serious consequences of failure make this a critical component in the calorimeter assembly. The purpose of this analysis is to investigate the stresses in the connection and other mechanical characteristics which determine joint performance. Wands, Bob; /Fermilab We identified nine individuals from three unrelated Turkish families with a unique autosomal recessive syndrome characterized by type I microtia, microdontia, and profound congenital deafness associated with a complete absence of inner ear structures (Michel aplasia). We later demonstrated three different homozygous mutations (p.S156P, p.R104X, and p.V206SfsX117) in the fibroblast growth factor 3 (FGF3) gene in affected members of these families, cosegregating with the autosomal recessive transmission as a completely penetrant phenotype. These findings demonstrate the involvement of FGF3 mutations in a human malformation syndrome for the first time and contribute to our understanding of the role this gene plays in embryonic development. Of particular interest is that the development of the inner ear is completely disturbed at a very early stage—or the otic vesicle is not induced at all—in all of the affected individuals who carried two mutant FGF3 alleles. Tekin, Mustafa; Hismi, Burcu Ozturk; Fitoz, Suat; Ozdag, Hilal; Cengiz, Filiz Basak; S?rmac?, Asl?; Aslan, Idil; Inceoglu, Bora; Yuksel-Konuk, E. Berrin; Y?lmaz, Seda Tas?r; Yasun, Oztan; Akar, Nejat It is estimated that a 4200 kg elephant generates as much as 5.12 kW of heat. How the elephant dissipates its metabolic heat and regulates its body temperature has been investigated during the past seven decades. Findings and conclusions differ sharply. The high rate of metabolic heat coupled with low surface area to volume ratio and the absence of sweat glands eliminate surface convection as the primary mechanism for heat removal. Noting that the elephant ears have high surface area to volume ratio and an extensive vascular network, ear flapping is thought to be the principal thermoregulatory mechanism. A computational and experimental program is carried out to examine flow and heat transfer characteristics. The ear is modeled as a uniformly heated oscillating rectangular plate. Our computational work involves a three-dimensional time dependent CFD code with heat transfer capabilities to obtain predictions of the flow field and surface temperature distributions. This information was used to design an experimental setup with a uniformly heated plate of size 0.2m x 0.3m oscillating at 1.6 cycles per second. Results show that surface temperature increases and reaches a steady periodic oscillation after a period of transient oscillation. The role of the vortices shed off the plate in heat transfer enhancement will be discussed. Koffi, Moise; Jiji, Latif; Andreopoulos, Yiannis Porcine ear necrosis was investigated in 23 conveniently chosen farms, consisting of 14 case farms and 9 control farms. Biopsies of lesions and oral swabs from pigs on 11 case farms were examined by histology and bacterial culture. All farms were visited for observations and a survey on management, housing, and the presence of other clinical signs or behavioral vices. Histological examination revealed that the lesions began on the surface and progressed to deeper layers, and that vascular damage did not appear to be the initiating cause. Spirochetes were only rarely observed in histological examination and were not cultured from biopsies and oral swabs. Staphylococcus aureus and Staphylococcus hyicus were cultured from 91% and 66% of samples, respectively. Ear biting and a humid environment were associated with ear necrosis. On some farms large numbers of pigs were affected and lesions were sometimes extensive. The condition appears to be an infectious disease beginning on the surface of the skin; contributing environmental and management factors are likely. Park, Jeonghwa; Friendship, Robert M.; Poljak, Zvonimir; DeLay, Josepha; Slavic, Durda; Dewey, Catherine E. Pursed lips breathing (PLB) training is often used in the management of patients with chronic obstructive lung disease (COLD). Previous clinical studies have demonstrated that PLB improves arterial oxygen saturation (SaO2) and CO2 removal as well as relieving dyspnea. Twelve hypoxemic subjects with stable COLD were randomly assigned to either the pursed lips (P) or control group consisting of general relaxation (R). The SaO2 was monitored via ear oximetry, and respiratory rate and tidal volume were monitored using a strain gage transducer and the minute volume was calculated. The PLB was taught by an experienced instructor using the ear oximeter as a monitoring display with a goal toward increasing SaO2. The subject was taught general relaxation (Rlx) with the aid of pleasant music. We compared PLB and Rlx treatments using an A-B-A crossover study design. In both groups, PLB significantly improved SaO2 over baseline (p less than 0.001) whereas Rlx did not. We conclude that patients can learn to increase their SaO2 by PLB using ear oximetry adjunctively. PMID:3731893 Tiep, B L; Burns, M; Kao, D; Madison, R; Herrera, J This paper explores the historical context of the dramatic rise in surgery for glue ear in the mid-20th century, and questions the published assertion that this represented a manufactured 'epidemic'. In examining historical sources, the reader's theoretical viewpoint greatly influences their conclusions: the sustained rise in treatment for glue ear may be seen as the advance of science in a golden age or the resistance of insular professionals to reason in the light of new scientific study methods. Current views on the practice of medicine, consumerism, science and standardisation, rationing and the nature of 'truth' all affect the way that we see this period. Technological advances clearly allowed better diagnosis and more effective treatment, but these did not appear to drive an 'epidemic', rather they were developed to meet the pre-existing challenges of otological practice. The proposition that an 'epidemic' was created does not appear to have any solid grounding. Society's perception of what constitutes disease and what needs treatment may have evolved, but the prevalence of other important diseases changed dramatically over this time period, and a real change in the epidemiology of glue ear cannot be dismissed. In defining the case for and against surgical treatment, a solely positivist, quantitative worldview cannot give us a complete picture of benefit and risk to individuals, families and society at large. PMID:21653931 Summary The purpose of this study is to determine the effectiveness of embolization of each type of vascular malformation. Thirty three patients with a diagnosis of vascular malformations in head and neck regions who were treated by embolization at Siriraj Hospital, Thailand, between 1997-2002 were reviewed retrospectively. There were 19 arteriovenous malformations (AVMs), two arteriolar-capillary types, ten venous malformations, one veno-lymphatic malformation and one mixed capillary and venous types. The goal of treatment is to restore function and to prevent bleeding complications with particularly good cosmetic results. The technique and therapeutic agents depended on the types and flow characteristics of each malformation. Transarterial embolization with N-butyl cyanoacrylate were used in AVMs, unless no arterial route approach, then direct puncture was tried. This was carried out in five patients with AVMs. Polyvinyl alcohol was used in two capillary lesions. All venous malformations were treated by absolute alcohol injection, percutaneously. All had good outcome without serious complications. Two patients had further plastic surgery, one was AVM of eyelid and the other was facial venolymphatic malformation. Embolization is considered to be the primary treatment by eradication of those abnormal vessels directly at the target, whereas delayed plastic surgery may be needed later. Churojana, A.; Chiewwit, P.; Chuangsuwanich, A.; Aojanepong, C.; Chawalaparit, O.; Suthipongchai, S. The advent of universal antenatal ultrasonography in many countries has revealed the full spectrum of congenital thoracic malformations (CTMs) and presented clinicians with a number of practical dilemmas to do with diagnosis and management. We present a review of the most common forms of CTMs, including congenital cystic adenomatoid malformation, bronchopulmonary sequestration, and lobar and segmental emphysema. PMID:22305631 Davenport, Mark; Eber, Ernst Background Congenital malformations remain a common cause of perinatal deaths and even though ultrasonogram can give fairly accurate diagnosis, perinatal autopsy is essential to confirm the diagnosis and look for associated malformations. Objectives To emphasize the importance of perinatal autopsy in diagnosing congenital malformations and to compare the same with the prenatal ultrasound findings. Methods The present study comprises 100 consecutive perinatal autopsies conducted after obtaining the approval from the Institutional Ethics Committee. In cases where prenatal ultrasound findings were available they were compared with the autopsy findings. Results Out of 100 perinatal autopsies, 44 cases were congenital anomalies with M:F = 1:1.5. Majority of the fetuses with congenital malformations (36.36%) were therapeutically terminated, Cental nervous system malformations being the commonest indication. The most common timing of therapeutic termination being 20 -24weeks. Congenital malformations were common between 35-39 weeks gestational age and birth weight range 350- 1000g. The malformations involving the central nervous system were commonest, seen in 15 cases (34.09%) followed by renal anomalies in 9 cases (20.45%) and multiple malformations in 7cases ( 15.91%). Autopsy confirmed the prenatal ultrasound findings in 50% of the cases, added to diagnosis in 29.54%, while it completely changed the primary diagnosis in 9.09% of the cases. Conclusion This study highlights the importance of perinatal autopsy in confirming the diagnosis of congenital anomalies by prenatal ultrasound findings. Andola, Uma S; AM, Anita; Ahuja, Mukta; Andola, Sainath K Background: Superficial venous malformations (SVM) are the most frequent vascular malformations. Outpatient percutaneous treatment with ethanol injection has rarely been described. Objective: To analyze the results from treating SVM patients with ethanol sclerotherapy. Methods: 81 patients were followed up prospectively over a median period of 18 months. 47 were female and 34 were male with a median age of 21 José Luiz Orlando; Jose Guilherme Mendes Pereira Caldas; Heloisa Galvão do Amaral Campos; Kenji Nishinari; Nelson Wolosker Reports of malformation induced by cadmium such as the appearance of vertebral anomalies in carp have been described by the present author. In this paper, the appearance of such malformed fish by exposure to cadmium was confirmed in a repeat experiment. Decalcification of the fish was studied from spinal x-ray photographs and the results of some elements analysis. IntroductionThe difficulty in prognosticating the clinical and intellectual outcome of fetuses presenting with a Dandy-Walker malformation (DWM) comes from the great variety of cystic, median, and retrocerebellar malformations that probably have nothing in common and the variability of the definitions given to these lesions. In addition, many of these lesions can mimic each other. A correct diagnosis cannot be made O. Klein; A. Pierre-Kahn; N. Boddaert; D. Parisot; F. Brunelle Background: Mayer-Rokitansky-Küster-Hauser syndrome is defined as Müllerian aplasia with vaginal agenesis and uterine remnants. It is commonly associated with renal and sometimes vertebral anomalies. MRKH syndrome or distal vaginal atresia is sometimes associated with anorectal malformations. The purpose of this study was to describe seven females with vaginal agenesis or distal vaginal atresia and an anorectal malformation and review the Tomas Wester; Juan A. Tovar; Risto J. Rintala Summary Carotid-vertebral anastomoses are commonly detected as incidental findings. But sometimes these channels are important for the clinical condition of the patient. Here a case of right thalamo-capsular arteriovenous malformation is described where a persistent primitive hypoglossal artery was the only route for embolization of the arteriovenous malformation. BACKGROUND: The percentage of children born after IVF will continue to increase due to demographic changes such as increasing maternal age and new developments in assisted reproduction techniques. IVF conceptions may carry an increased risk of congenital malformations. METHODS: We compared overall and specific congenital malformation rates calculated for IVF children (n 4224) and naturally conceived children (n 314 605), S. Anthony; S. E. Buitendijk; C. A. Dorrepaal; K. Lindner; D. D. M. Braat; A. L. den Ouden We report two patients aged 65 and 61 years, who presented a subacute dementia with normal CT scan without contrast injection. Angiography showed a dural arteriovenous malformation. The patients improved dramatically with treatment of the malformation. Dementia was characterised by frontal dysfunction, emotional disorders, mnesic and praxic impairment. This neuropsychological pattern suggests a profile of global dementia in the field Assia Serradj Jaillard; Blandine Peres; Marc Hommel Of 76 patients in the pediatric age group suffering from cerebrovascular diseases treated in the years 1970–1983, 26 patients (34%) did not harbor intracranial vascular malformations (aneurysms or arterovenous malformations). Two groups of patients were identified: (a) those suffering from a spontaneous intracranial hemorrhage (16 cases); (b) those suffering from an ischemic stroke (10 cases). Of those with spontaneous intracranial C. Mazza; A. Pasqualin; P. Cavazzani; B. Dalla Bernardina; R. Pian Benzimidazole compounds have teratogenic effects in domestic and experimental animals. In this study, 14 Manchega ewes were treated orally, under controlled conditions, with 20 mg netobimin (a prodrug of a benzimidazole compound) per\\/kg bodyweight on the 17th day of pregnancy. Congenital malformations and abortions affected 60 per cent of the lambs. The main malformations were skeletal and renal, but vascular M. Navarro; C. Cristofol; A. Carretero; M. Arboix; J. Ruberte The Klippel-Feil deformity is a complex of osseous and visceral anomalies, which include low hairline, platybasia, fused cervical vertebrae with a short neck, and deafness. Associated central nervous system abnormalities include occipital cephalocele, Chiari I malformation, syrinx, microcephaly, and hydrocephalus. Herein, we report a case with Klippel-Feil syndrome and Dandy-Walker malformation. PMID:22303802 Karaman, A; Kahveci, H Thirty patients with Chiari I malformation were examined by MRI over 2-year period. All patients underwent MRI scan before and after surgical decompression of the posterior fossa. Images of the craniocervical junction confirmed tonsillar herniation in all cases and allowed the definition of two anatomically distinct types of Chiari malformation. Twenty-one of the 30 patients (70%) had concomitant syringomyelia and Talal A. Amer; Omran M. El-Shmam Purpose Cortical malformations (CMs) are increasingly recognized as the epileptogenic substrate in patients with medically refractory neocortical epilepsy (NE). The aim of this study was to test the hypotheses that: 1. CMs are metabolically heterogeneous. 2. The structurally normal appearing perilesional zone is characterized by similar metabolic abnormalities as the CM. Methods Magnetic resonance spectroscopic imaging (MRSI) in combination with tissue segmentation was performed on eight patients with NE and CMs and 19 age-matched controls. In controls, NAA, Cr, Cho, NAA/Cr and NAA/Cho of all voxels of a given lobe were expressed as a function of white matter content and thresholds for pathological values determined by calculating the 95% prediction intervals. These thresholds were used to identify metabolically abnormal voxels within the CM and in the perilesional zone. Results 30% of all voxels in the CMs were abnormal, most frequently because of decreases of NAA or increases of Cho. Abnormal voxels tended to form metabolically heterogeneous clusters interspersed in metabolically normal regions. Furthermore, 15% of all voxels in the perilesional zone were abnormal, the most frequent being decreases of NAA and Cr. Conclusion In CMs metabolically normal regions are interspersed with metabolically heterogeneous abnormal regions. Metabolic abnormalities in the perilesional zone share several characteristics of CMs and might therefore represent areas with microscopic malformations and/or intrinsic epileptogenicity. Mueller, Susanne G.; Barakos, Jerome A.; Cashdollar, Nathan; Flenniken, Derek L.; Vermathen, Peter; Matson, Gerald B.; Weiner, Michael W. Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis. PMID:15081131 Rossi, Andrea; Biancheri, Roberta; Cama, Armando; Piatelli, Gianluca; Ravegnani, Marcello; Tortori-Donati, Paolo In the human, malformations of lymphatic vessels can be observed as lymphangiectasia, lymphangioma and lymphangiomatosis, with a prevalence of 1.2-2.8 per thousand. Their aetiology is unknown and a causal therapy does not exist. We investigated the origin of lymphatic endothelial cells (LECs) in avian and murine embryos, and compared the molecular profile of LECs from normal and malformed lymphatics of children. In avian embryos, Prox1+ lymphangioblasts are located in the confluence of the cranial and caudal cardinal veins, where the jugular lymph sac (JLS) forms. Cell lineage studies show that the JLS is of venous origin. In contrast, the lymphatics of the dermis are derived from mesenchymal lymphangioblasts located in the dermatomes, suggesting a dual origin of LECs in avian embryos. The same may hold true for murine embryos, where Lyve1+ LEC precursors are found in the cardinal veins, and in the mesenchyme. The mesenchymal cells express the pan-leukocyte marker CD45, indicating a cell type with lymphendothelial and leukocyte characteristics. In the human, such cells might give rise to Kaposi's sarcoma. Microarray analyses of LECs from lymphangiomas of children show a large number of regulated genes, such as VEGFR3. Our studies show that lymphvasculogenesis and lymphangiogenesis occur simultaneously in the embryo, and suggest a function for VEGFR3 in lymphangiomas. PMID:18300425 Wilting, Jörg; Buttler, Kerstin; Rössler, Jochen; Norgall, Susanne; Schweigerer, Lothar; Weich, Herbert A; Papoutsi, Maria We report on two families with co-occurrence of sirenomelia and caudal malformations. In the first family, the mother had undergone surgery for a short form of imperforate anus. Her first pregnancy was terminated because of bilateral renal agenesis with oligohydramnios. Her second pregnancy was interrupted because of sirenomelia. The second family was referred to us because of caudal malformation in their two children. The parents' spinal radiographs were normal. The first pregnancy resulted in a girl with imperforate anus, absence of S3-S5 and coccyx, abnormal pelvic floor, and an almost bifid anteriorly located bladder. The second pregnancy resulted in a baby girl with sirenomelia. No diabetes was present during the pregnancies in either of these two families. These families confirm the hypothesis that major genes are responsible for the embryogenesis of the caudal part of the embryo, with variable expression, as has been already described in sirenomelia mouse models (CYP26A1, BMP7/tsg). Molecular studies are underway in these families and in sporadic cases in our laboratory to explore the genetic basis of sirenomelia in humans. PMID:22522670 Gerard, Marion; Layet, Valérie; Costa, Teresa; Roumazeilles, Yves; Chenal, Pierre; Cailliez, Daniel; Gerard, Bénédicte A study was undertaken in Jawaharlal Institute of Postgraduate Medical Education and Research, Pondicherry, South India, to understand the relation between congenital developmental disorders and consanguinity and also reproductive wastage and consanguinity. Four hundred children with existing congenital developmental disorders were studied with regard to their consanguineous parentage and compared with 1,000 randomly selected patients attending the paediatric outpatient department. There was a significantly higher prevalence of consanguinity in the study group (p < 0.001) and greater frequency in rural areas. The common types of consanguineous marriages were between first cousins (50.6%) and uncle and niece (42.4%). Frequency of consanguinity was not significantly related to religion and caste. The mean coefficient of inbreeding was 0.056. Consanguinity had no significant effect on average pregnancy rate and reproductive wastage. The frequency of consanguinity was significantly higher especially with autosomal recessive disorders (p < 0.001), congenital heart diseases (p < 0.001), multiple malformations (p < 0.001), neurological malformations (p < 0.005), chromosomal disorders (p < 0.01), genitourinary disorders (p < 0.02) and mental retardation-developmental disorders (p < 0.02). These observations stress the need for communicating the deleterious effects of inbreeding to the public through regular health education. PMID:7684587 Jain, V K; Nalini, P; Chandra, R; Srinivasan, S Although genetic defects are the leading cause of central nervous system malformations including in the posterior fossa, specific malformative patterns should alert the clinician to consider rather a teratogenic etiology. We discuss the imaging features of 2 mid-hindbrain malformations consecutive to the intake of isotretinoin (Roaccuatane®; case 1) and misoprostol (Cytotec®; case 2) during pregnancy and review the pertinent literature. We correlate the morphological appearance of the mid-hindbrain malformation, as seen on high-resolution magnetic resonance imaging to possible drug-induced pathogenetical mechanisms. The recognition of characteristic imaging patterns enables diagnosis of and/or confirmation of suspected drug-induced hindbrain malformations. This has important medicolegal implications and also clinical significance to avoid unsuccessful and misleading genetic testing. PMID:23390117 Merlini, Laura; Fluss, Joël; Dhouib, Amira; Vargas, Maria I; Becker, Minerva Vascular malformations are congenital lesions that are present at birth and do not regress. However, they often present later in life. They are subdivided into two categories: (1) slow- or low-flow and (2) fast- or high-flow malformations. Low-flow malformations contain combinations of capillary, venous, and lymphatic components. Venous malformations can occur anywhere in the body, but are most frequently seen in the head and neck (40%). These lesions present in a variety of ways, from a vague blue patch to a soft blue mass, which may be single isolated or may occur in multiple areas. Treatment depends on the type of lesion, the location, degree of involvement, and the clinical symptoms. Here we are report the imaging and histopathologic findings in a patient with multiple venous malformations affecting the left side of the face and trunk. Chava, Venkateswara Rao; Shankar, Ashwini Naveen; Vemanna, Naveen Shankar; Cholleti, Sudheer Kumar Lymphatic malformations are uncommon, benign and congenital malformations of the lymphatic system exhibiting lack of development of communication between the lymphatic and venous circulation. We report the unusual case of rapidly expanding giant lymphatic malformation of the torso, bilateral axillae and left upper extremity of a newborn. As the first-line treatment, aspiration and sclerotherapy with bleomycin were performed. The sclerotherapy failed to cause regression of the mass, and rapid expansion of the malformation necessitated surgery. Partial resection of the mass was performed. Clinical symptoms of respiratory distress resolved in the early postoperative period, and the patient became hemodynamically stable. However, intrathoracic invasion of the mass developed, leading to restriction of thoracic expansion, ending in death. In conclusion, surgical treatment of giant lymphatic malformations remains challenging. PMID:24292043 Cal??, Mert; Kona?, Ersoy; Takc?, ?ahin; Yurdakök, Murat; Tunçbilek, Gökhan From a research sample of 138 corpses, divided into four subgroups of ambient storage temperature (0-5 degrees C, 6-10 degrees C, 11-15 degrees C and 16-23 degrees C) four linear regression formulae of actual versus estimated post-mortem interval were obtained ('interval' formulae) using a single outer ear temperature measurement on both sides. This method showed the best correlation coefficient among five other methods previously proposed for time of death determination (rectal temperature, vitreous K+, CSF K+, blood log NA+/K+ and log Cl-), however its results were less accurate than those obtained with a multivariate equation combining several of the above mentioned methods. Eventually an equation expressing time of death (TOD) as a function of outer ear temperature (OE T degrees) and ambient temperature was also established from the whole research sample ('global' formulae). On a different sample of 141 corpses the regression formulae ('interval' and 'global') for the outer ear temperature were compared to three methods based on a single rectal temperature measurement ('rule of thumb' 1 and 2, Henssge nomogram) and therefore useful at the scene; the results of all methods were compared within the four subgroups of ambient temperature as well as in three subgroups of different post-mortem interval lengths (< 7 h, < 10 h, < 15 h). In all cases the outer ear temperature formulae provided better results than the rectal temperature methods (especially Henssge nomogram and rule of thumb 1). Moreover they did not show any post-mortem plateau which was present in almost 30% of cases when rectal temperature was measured in corpses kept at ambient temperature above 15 degrees C. Our results show that outer ear temperature measurement is the method which provides the best simplicity/quality ratio and should therefore be proposed for use at the scene when conditions are similar to those of our experiment (within buildings). A software equipped thermometer is required in order to use in each case the appropriate formula and confidence interval. PMID:9022275 Baccino, E; De Saint Martin, L; Schuliar, Y; Guilloteau, P; Le Rhun, M; Morin, J F; Leglise, D; Amice, J To rehabilitate most cases of conductive hearing loss closure of ear drum perforations and rebuilding of the ossicular chain can be performed. Due to the great number of biocompatible bone substitute materials available it is occasionally difficult for the surgeon to choose the most favorable substitute. Autogenous structures (ossicles, cortical bone, cartilage) and allogenous tissues (ossicles, cortical bone, cartilage, dentin) are possible bone replacement materials. Xenogenic tissue is currently not used in middle ear surgery. Ionomer cement is a hybrid material for replacement of bone but does not fit direct classification of the various classes of alloplastic materials in current use: that is, metals (gold, steel wire, platinum, titanium), plastics (polyethylene, polytetrafluorethylene) and ceramics (ceramic oxide, carbon, calcium-phosphate ceramic, vitreous ceramic). For restoration of the sound conductive apparatus preference is given to autogenous ossicles because cortical bone is resorbed and cartilage weakens over time. Most surgeons do not use allogenous tissue, because of the possible transmission of such infectious disease as immunodeficiency syndrome or Creutzfeldt-Jakob disease. Only dentin deserves special attention as a possible bone substitute in the middle ear because its form can be preserved during sterilization. Based on the observations available to date, it becomes apparent that titanium implants hold greater promise than gold. Form-stable synthetic materials are not generally recommended due to foreign body reactions which have been confirmed by many investigators. Ceramic materials (e.g. ceramic oxide, carbon, calcium-phosphate ceramic, glass ceramic) are well tolerated in the middle ear and have also proved to be useful over time. Hybrid bone substitute ionomer cement is easily workable and well integrated, showing a good functional outcome. For many years good results in otosclerosis surgery have been achieved with a prosthesis made of platinum-wire and Teflon. Short-term follow-up periods hold great promise with pistons made of gold. Autogenous ossicles, ionomer cement and recently titanium protheses--as far as usable--are employed by the author for reconstructing the middle ear. For the time being platinum-Teflon prostheses and gold are used in otosclerosis surgery. PMID:10197273 Five kinds of ear mushrooms are commercially available in Taiwan, including black, red, jin, snow, and silver ears. Methanolic extracts were prepared from these ear mushrooms, and their antioxidant properties were studied. For all methanolic extracts from ear mushrooms, the antioxidant activities in the 1,3-diethyl-2-thiobarbituric acid method were moderate (38.6 approximately 74.6%) at 1.0-5.0 mg/mL. Methanolic extracts from red, jin, and snow ears showed excellent antioxidant activities in the conjugated diene method at 5.0 mg/mL. At 5.0 mg/mL, reducing powers of methanolic extracts were in the descending order of snow > black approximately red approximately jin > silver ears. The scavenging effect of methanolic extracts from ear mushrooms on 1,1-diphenyl-2-picrylhydrazyl radicals was excellent except for that from silver ears. Ear mushroom extracts were not good scavengers for hydroxyl free radicals but were good chelators for ferrous ions. Naturally occurring antioxidants, including ascorbic acid, tocopherols, and total phenols, were found in the methanolic extracts. However, beta-carotene was not detected. Total antioxidant components were 15.69, 30.09, 27.83, 49.17, and 31.70 mg/g for black, red, jin, snow, and silver ears, respectively. PMID:11714344 Mau, J L; Chao, G R; Wu, K T Infants with limb reduction deficiencies (LRD) often have other associated congenital malformations. The purpose of this investigation was to assess the prevalence and the types of associated malformations in a defined population. This study included special strengths: each affected child was examined by a geneticist, all elective terminations were ascertained, and the surveillance for malformations was continued until 1 year of age. The associated malformations in infants with LRD were collected in all livebirths, stillbirths and terminations of pregnancy during 25 years in 347,810 consecutive births in the area covered by our population based registry of congenital malformations. Of the 271 LRD infants born during this period, representing a prevalence of 7.8 per 10,000, 57.9% had associated malformations. There were 17(6.3%) patients with chromosomal abnormalities including 10 trisomies 18, and 62 (22.9%) nonchromosomal recognized dysmorphic conditions. There were no predominant recognized dysmorphic conditions, but VA(C)TER(L) association. However numerous recognized dysmorphic conditions were registered including Poland, ectrodactyly-ectodermal dysplasia-clefting, oral-facial-digital, Klippel-Trenaunay-Weber, oculo-auriculo-vertebral defect spectrum, CHARGE, Townes-Brocks, Moebius, Du Pan, Smith-Lemli-Opitz, hypoglossia-hypodactyly, amniotic band, De Lange, Rubinstein-Taybi, Fanconi, radius aplasia- thrombocytopenia, Roberts, Holt-Oram, and fetal diethylstilbestrol. Seventy eight (28.8%) of the patients were multiply, non-syndromic, non chromosomal malformed infants (MCA). Malformations in the cardiac system, in the genital system, and in the central nervous system were the most common other malformations, 11.4%, 9.4%, and 7.7% of the associated malformations, respectively, followed by malformations in the renal system (4.8%), and in the digestive system (4.6%). Prenatal diagnosis was performed in 48.4% of dysmorphic syndromes with LRD. The overall prevalence of associated malformations, which was more than one in two infants, emphasizes the need for a thorough investigation of infants with LRD.A routine screening for other malformations especially cardiovascular system, urogenital system, central nervous system, and digestive system may be considered in infants and in fetuses with LRD. PMID:20670696 Stoll, Claude; Alembik, Yves; Dott, Beatrice; Roth, Marie-Paule CHARGE syndrome is a rare human disorder caused by mutations in the gene encoding chromodomain helicase DNA binding protein 7 (CHD7). Characteristics of CHARGE are varied and include developmental ear and hearing anomalies. Here we report a novel mouse model of CHD7 dysfunction, termed Looper. The Looper strain harbours a nonsense mutation (c.5690C>A, p.S1897X) within the Chd7 gene. Looper mice exhibit many of the clinical features of the human syndrome, consistent with previously reported CHARGE models, including growth retardation, facial asymmetry, vestibular defects, eye anomalies, hyperactivity, ossicle malformation, hearing loss and vestibular dysfunction. Looper mice display an otosclerosis-like fusion of the stapes footplate to the cochlear oval window and blepharoconjunctivitis but not coloboma. Looper mice are hyperactive and have vestibular dysfunction but do not display motor impairment. Ogier, Jacqueline M.; Carpinelli, Marina R.; Arhatari, Benedicta D.; Symons, R. C. Andrew; Kile, Benjamin T.; Burt, Rachel A. Purpose: To report and discuss four cases of ear pain which were treated successfully with manual therapy. Methods: Report of four cases. Results: Four patients with ear pain were referred for chiropractic consult. They were all treated with a combination of manual therapy and exercise with resolution of their ear symptoms. Conclusions: The mechanism of idiopathic ear pain that may be amenable to manual therapy is not fully known. Further research is needed to investigate the etiology of this disorder and to determine whether manual therapy and exercise are viable options in some patients with idiopathic ear pain. In the meantime, it may be advantageous for otolaryngologists to seek input from physicians skilled in assessment and treatment of the musculoskeletal system in cases ear pain for which an otolarygologic etiology cannot be found. Murphy, Donald R.; Gay, Charles W. Upper limb bud appears in the cervical region of the embryo during the fifth week of development. It is made of epithelia and underlying mesenchyme. Diffusible growth factors, expressed by the apical ectodermal ridge, direct the proximal-distal growth. Other factors are expressed by zone of polarizing activity and ectoderm. They induce together anterior-posterior growth and dorsal-ventral polarity of the limb bud. The development of axial skeleton pattern is controlled by transcription factors from the HOX family, which are expressed in a stripe along the proximal and distal edges of the limb bud. Embryologic mechanisms of the main hand malformations are described, as well as their known genetic or mechanical aetiologies. PMID:18838286 Perrin, J; Geoffroy-Siraudin, C; Metzler-Guillemain, C Breeding experiments were conducted on cats with congenital taillessness, to test the dissemination pattern of taillessness in their offspring. Clinical evaluation, radiographic analysis of the vertebral column and histological studies of the digestive tract and central nervous tissue were conducted to determine the association of malformations of these systems in cats born with different degrees of taillessness noted in the rumpy and stumpy cats. The mode of transmission of the tailless (Manx) condition assumed to be through an autosomal dominant factor (M) was confirmed by this investigation. It is hypothesized that the problems associated with the tailless condition such as spina bifida, urinary and faecal incontinence and locomotor disturbances of the pelvic limbs may all be related to a disturbance affecting the development of the central nervous system in the early embryonic life. ImagesFigure 3.Figure 4.Figure 5.Figure 6.Figure 7.Figure 8.Figure 9.Figure 10. DeForest, M. E.; Basrur, P. K. Summary Embolization is recognized as an important adjunct in the treatment of cerebral arteriovenous malformations (AVMs). We reviewed our results of embolizations for AVMs and discussed procedure-related complications. Eleven complications were recorded in 68 consecutive patients (16%). Of these, four were technical problems including a glued catheter, inability to withdraw the catheter, vessel perforation by the microcatheter, and coil migration. Other complications included three cases of ischemic symptoms due to retrograde thrombosis, two cases of asymptomatic cerebral infarction, one case of asymptomatic small haemorrhage due to venous occlusion, and one case of post-embolization haemorrhage of unknown etiology. Our morbidity rate was 7%, mortality rate was 0%, and asymptomatic complication torospectively. Further improvements to endovascular techniques and devices are required. Sugiu, K.; Tokunaga, K.; Sasahara, W.; Watanabe, K.; Nishida, A.; Ono, S.; Nishio, S.; Date, I.; Rufenacht, D. A. We report the first familial cases with two different types of posterior fossa cystic malformation and a leukodystrophic-like aspect on cerebral magnetic resonance imaging (MRI). The girl and her brother had severe encephalopathy, marked hypotonia, absent deep tendon reflexes, macrocrania, gigantism, and dysmorphic face and extremities. The girl had generalized seizures. The boy had unilateral cataract and bilateral optic atrophy. The parents were first cousins, suggesting autosomal recessive transmission. MRI showed Dandy-Walker variant in the girl, with cerebellar vermis hypoplasia and expansion of the cisterna magna, which communicated with the fourth ventricle. Her brother had mega cisterna magna communicating with the fourth ventricle and a normal cerebellum. The 2 children had abnormally high signal in the supratentorial white matter. Visual and auditory evoked potentials revealed prolonged latencies. Motor and sensory conduction velocities were normal. Muscle and nerve biopsies were normal. Metabolic exploration demonstrated no abnormality. PMID:9258968 Humbertclaude, V T; Coubes, P A; Leboucq, N; Echenne, B B Pulmonary arteriovenous malformation is a rare anomaly that presents in several different ways. It can present as an isolated finding, or more often in the context of hereditary haemorrhagic telangiectasia. It can also complicate palliative surgery such as the Glenn operation for complex congenital heart disease with single ventricle physiology. Its management includes transcatheter embolization, which is the preferred mode of therapy, surgery (including resection of the affected lobe, segment, or the fistula itself), or rarely, medical therapy. Complications of the disease itself and of various modes of treatment are relatively common, and patients require close surveillance for possible recurrence, or development of new fistulas. In cases related to the Glenn operation, redirection of hepatic venous flow or heart transplantation may cure the problem. Papagiannis, J; Apostolopoulou, S; Sarris, GE; Rammos, S Although ears capable of detecting airborne sound have arisen repeatedly and independently in different species, most animals that are capable of hearing have a pair of ears. We review the advantages that arise from having two ears and discuss recent research on the similarities and differences in the binaural processing strategies adopted by birds and mammals. We also ask how these different adaptations for binaural and spatial hearing might inform and inspire the development of techniques for future auditory prosthetic devices. Schnupp, Jan W H; Carr, Catherine E In the past few years, the increasing accessibility of next-generation sequencing technology has translated to a number of significant advances in our understanding of brain malformations. Genes causing brain malformations, previously intractable due to their complex presentation, rarity, sporadic occurrence, or molecular mechanism, are being identified at an unprecedented rate and are revealing important insights into central nervous system development. Recent discoveries highlight new associations of biological processes with human disease including the PI3K-AKT-mTOR pathway in brain overgrowth syndromes, the trafficking of cellular proteins in microcephaly-capillary malformation syndrome, and the role of the exosome in the etiology of pontocerebellar hypoplasia. Several other gene discoveries expand our understanding of the role of mitosis in the primary microcephaly syndromes and post-translational modification of dystroglycan in lissencephaly. Insights into polymicrogyria and heterotopias show us that these 2 malformations are complex in their etiology, while recent work in holoprosencephaly and Dandy-Walker malformation suggest that, at least in some instances, the development of these malformations requires "multiple-hits" in the sonic hedgehog pathway. The discovery of additional genes for primary microcephaly, pontocerebellar hypoplasia, and spinocerebellar ataxia continue to impress upon us the significant degree of genetic heterogeneity associated with many brain malformations. It is becoming increasingly evident that next-generation sequencing is emerging as a tool to facilitate rapid and cost-effective molecular diagnoses that will be translated into routine clinical care for these rare conditions in the near future. PMID:23793931 Dyment, David A; Sawyer, Sarah L; Chardon, Jodi Warman; Boycott, Kym M Advances in neuroimaging, developmental biology and molecular genetics have increased the understanding of developmental disorders affecting the midbrain and hindbrain, both as isolated anomalies and as part of larger malformation syndromes. However, the understanding of these malformations and their relationships with other malformations, within the central nervous system and in the rest of the body, remains limited. A new classification system is proposed, based wherever possible, upon embryology and genetics. Proposed categories include: (i) malformations secondary to early anteroposterior and dorsoventral patterning defects, or to misspecification of mid-hindbrain germinal zones; (ii) malformations associated with later generalized developmental disorders that significantly affect the brainstem and cerebellum (and have a pathogenesis that is at least partly understood); (iii) localized brain malformations that significantly affect the brain stem and cerebellum (pathogenesis partly or largely understood, includes local proliferation, cell specification, migration and axonal guidance); and (iv) combined hypoplasia and atrophy of putative prenatal onset degenerative disorders. Pertinent embryology is discussed and the classification is justified. This classification will prove useful for both physicians who diagnose and treat patients with these disorders and for clinical scientists who wish to understand better the perturbations of developmental processes that produce them. Importantly, both the classification and its framework remain flexible enough to be easily modified when new embryologic processes are described or new malformations discovered. Millen, Kathleen J.; Dobyns, William B. The Chiari type 1 malformation is common. Unlike the Chiari type 2 and 3 malformations, it may remain latent for a long time, becoming symptomatic only in adulthood. The introduction of MRI has resulted in an increased number of diagnoses of this malformation in pediatric patients. It appears to be related to underdevelopment of the posterior cranial fossa. It must be differentiated from acquired tonsillar herniation, particularly when herniation results from intracranial hypotension; these cases are sometimes reported as acquired Chiari I malformation with spontaneous resolution. Tonsillar ectopia may cause symptoms by its direct effect on any or all of the medulla and the cerebellar and upper spinal cord. The most suggestive of the oculomotor disturbances is oscillopsia with downbeat nystagmus. Dysphonia and dysphagia are common. Potentially serious autonomic disturbances are also frequent: sleep apnea, respiratory failure, syncope and even sudden death. Another risk is syrinx formation, resulting from obstruction of CSF circulation in the cisterna magna. Syringomyelia is detected in 32 to 74% of patients with Chiari I malformation. Treatment is surgical. Posterior fossa decompression is achieved by suboccipital craniectomy combined with laminectomy of the upper cervical segments. Surgical intervention is indicated when the malformation is symptomatic and there is no doubt that it is the cause of the symptoms. When a Chiari I malformation is identified fortuitously on MRI, long-term monitoring is essential. The risk of developing symptoms increases over time. Patients should be advised not to participate in contact sports. PMID:16327707 Masson, C; Colombani, J-M We report a surgical case of tectal cavernous malformation presented by hydrocephalus. This 30-year-old man suffered from headache and nausea due to an obstructive hydrocephalus caused by a cavernous malformation in the tectum. Magnetic resonance (MR) images on admission showed a small lesion depicted as isointensity on the T1-weighted image, hyperintensity on the T2-weighted image, and accompanied with peripheral low-signal intensity rim, suggesting a tectal cavernous malformation. After admission, ventricular drainage was performed and the patient's symptoms improved immediately. He underwent a ventriculoperitoneal shunt ten days after the ventricular drainage. Postoperative CT scan showed an enlargement of the tectal mass with hemorrhagic change, but there was no deterioration in his neurological status. Cerebral angiography demonstrated no vascular stain or venous malformation. Microsurgical removal of the tectal mass was then performed via an occipital transtentorial approach. Histopathology proved a cavernous malformation and MR images at follow-up demonstrated total excision. Postoperatively, an upward gaze palsy appeared, but gradually improved within a month. Management strategy of brain stem cavernous malformation is controversial. Occasionally, brain stem hemorrhage may become critical. Therefore, we recommend aggressive surgical extirpation of symptomatic brain stem cavernous malformation, if it is accessible, if hemorrhage is present, and if the patient's condition permits it. PMID:11806112 Fujiwara, Satoshi; Ohta, Masahiro; Takeda, Tetsuji; Kohno, Kanehisa; Takechi, Akihiko; Kawada, Yasuchika; Shinohara, Naoki; Sasaki, Ushio Background. The question is debated on whether maternal hypothyroidism or use of thyroxin in early pregnancy affects the risk for infant congenital malformations. Objectives. To expand the previously published study on maternal thyroxin use in early pregnancy and the risk for congenital malformations. Methods. Data from the Swedish Medical Birth Register were used for the years 1996-2011 and infant malformations were identified from national health registers. Women with preexisting diabetes or reporting the use of thyreostatics, anticonvulsants, or antihypertensives were excluded from analysis. Risk estimates were made as odds ratios (ORs) or risk ratios (RRs) after adjustment for year of delivery, maternal age, parity, smoking, and body mass index. Results. Among 23?259 infants whose mothers in early pregnancy used thyroxin, 730 had a major malformation; among all 1?567?736 infants, 48012 had such malformations. The adjusted OR was 1.06 (95% CI 0.98-1.14). For anal atresia the RR was 1.85 (95% CI 1.00-1.85) and for choanal atresia 3.14 (95% CI 1.26-6.47). The risk of some other malformations was also increased but statistical significance was not reached. Conclusions. Treated maternal hypothyroidism may be a weak risk factor for infant congenital malformations but an association with a few rare conditions is possible. PMID:24744955 Källén, Bengt; Norstedt Wikner, Birgitta Background: Amplatzer vascular plugs (AVPs) are devices ideally suited to close medium-to-large vascular communications. There is limited published literature regarding the utility of AVPs in congenital cardiovascular malformations (CCVMs). Aims: To describe the use of AVPs in different CCVMs and to evaluate their safety and efficacy. Materials and Methods: All patients who required an AVP for the closure of CCVM were included in this retrospective review of our catheterization laboratory data. The efficacy and safety of AVPs are reported. Results: A total of 39 AVPs were implanted in 31 patients. Thirteen (33%) were AVP type I and 23 (59%) were AVP type II. AVP type III were implanted in two patients and type IV in one patient. The major indications for their use included closure of pulmonary arteriovenous malformation (AVM) (n = 7), aortopulmonary collaterals (n = 7), closure of a patent Blalock-Taussig shunt (n = 5), systemic AVM (n = 5), coronary AVM (n = 4), patent ductus arteriosus (PDA) (n = 3), pulmonary artery aneurysms (n = 3), and venovenous collaterals (n = 2). Deployment of the AVP was done predominantly via the 5 – 7F Judkin's right coronary guide catheter. Overall 92% of the AVPs could be successfully deployed and resulted in occlusion of the target vessel in all cases, within 10 minutes. No procedure-related or access site complication occurred. Conclusions: AVPs are versatile, easy to use, and effective devices to occlude the vascular communications in a variety of settings. AVP II is especially useful in the closure of tubular structures with a high flow. Barwad, Parag; Ramakrishnan, Sivasubramanian; Kothari, Shyam S; Saxena, Anita; Gupta, Saurabh K; Juneja, Rajnish; Gulati, Gurpreet Singh; Jagia, Priya; Sharma, Sanjiv Transumbilical surgical procedures have been reported to be a feasible, safe, and cosmetically excellent procedure for various pediatric surgical diseases. Umbilical loop colostomies have previously been created in patients with Hirschsprung's disease, but not in patients with anorectal malformations (ARMs). We assessed the feasibility and cosmetic results of temporal umbilical loop colostomy (TULC) in patients with ARMs. A circumferential skin incision was made at the base of the umbilical cord under general anesthesia. The skin, subcutaneous tissue, and fascia were cored out vertically, and the umbilical vessels and urachal remnant were individually ligated apart from the opening in the fascia. A loop colostomy was created in double-barreled fashion with a high chimney more than 2 cm above the level of the skin. The final size of the opening in the skin and fascia was modified according to the size of the bowel. The bowel wall was fixed separately to the peritoneum and fascia with interrupted 5-0 absorbable sutures. The bowel was opened longitudinally and everted without suturing to the skin. The loop was divided 7 days postoperatively, and diversion of the oral bowel was completed. The colostomy was closed 2-3 months after posterior saggital anorectoplasty through a peristomal skin incision followed by end-to-end anastomosis. Final wound closure was performed in a semi-opened fashion to create a deep umbilicus. TULCs were successfully created in seven infants with rectourethral bulbar fistula or rectovestibular fistula. Postoperative complications included mucosal prolapse in one case. No wound infection or spontaneous umbilical ring narrowing was observed. Skin problems were minimal, and stoma care could easily be performed by attaching stoma bag. Healing of umbilical wounds after TULC closure was excellent. The umbilicus may be an alternative stoma site for temporary loop colostomy in infants with intermediate-type anorectal malformations, who undergo radical anorectoplasty via a non-abdominal approach. PMID:23001137 Hamada, Yoshinori; Takada, Kohei; Nakamura, Yusuke; Sato, Masahito; Kwon, A-Hon Background Congenital malformations are present in approximately 2–3% of liveborn babies and 20% of stillborn fetuses. The mechanisms underlying the majority of sporadic and isolated congenital malformations are poorly understood, although it is hypothesized that the accumulation of rare genetic, genomic and epigenetic variants converge to deregulate developmental networks. Methodology/Principal Findings We selected samples from 95 fetuses with congenital malformations not ascribed to a specific syndrome (68 with isolated malformations, 27 with multiple malformations). Karyotyping and Multiplex Ligation-dependent Probe Amplification (MLPA) discarded recurrent genomic and cytogenetic rearrangements. DNA extracted from the affected tissue (46%) or from lung or liver (54%) was analyzed by molecular karyotyping. Validations and inheritance were obtained by MLPA. We identified 22 rare copy number variants (CNV) [>100 kb, either absent (n?=?7) or very uncommon (n?=?15, <1/2,000) in the control population] in 20/95 fetuses with congenital malformations (21%), including 11 deletions and 11 duplications. One of the 9 tested rearrangements was de novo while the remaining were inherited from a healthy parent. The highest frequency was observed in fetuses with heart hypoplasia (8/17, 62.5%), with two events previously related with the phenotype. Double events hitting candidate genes were detected in two samples with brain malformations. Globally, the burden of deletions was significantly higher in fetuses with malformations compared to controls. Conclusions/Significance Our data reveal a significant contribution of rare deletion-type CNV, mostly inherited but also de novo, to human congenital malformations, especially heart hypoplasia, and reinforce the hypothesis of a multifactorial etiology in most cases. Serra-Juhe, Clara; Rodriguez-Santiago, Benjamin; Cusco, Ivon; Vendrell, Teresa; Camats, Nuria; Toran, Nuria; Perez-Jurado, Luis A. Cholesterol granuloma (CG) is a histologic description of foreign body giant cell formation toward cholesterol crystals. The majority of temporal bone CG is unilateral and most common in the petrous apex. Middle ear CG is usually the result of underlying ear diseases. Primary middle ear CG is very rare. Most reported CG has not been associated with familial hypercholesterolemia (FH). FH, an autosomal dominant disorder, manifests as high levels of serum cholesterol and low density lipoprotein (LDL) cholesterol. We report a rare case of FH and bilateral aggressive primary middle ear CG. This publication has been approved by the IRB, Hospital Alor Setar. PMID:18503863 Masaany, Mansor; Siti, Hashim Sabzah; Nurliza, Idris; Mazita, Ami To achieve mass customization and collaborative product design, human factors and ergonomics should play a key development role. The purpose of this study was to provide product designers with the anthropometic dimensions of outer ears for different demographic data, including gender and age. The second purpose was to compare the dimensions of various ear-related products (i.e., earphone, bluetooth earphone and ear-cup earphone) with the anthropometic database and recommend appropriate solutions for design. Two hundred subjects aged 20-59 was selected for this study and divided into four age stratifications. Further, three different dimensions of the outer ear (i.e., the earhole length, the ear connection length and the length of the pinna) were measured by superimposed grid photographic technique. The analysis of variance (ANOVA) was used to investigate the effects of gender, and age on ear dimensions. The results showed that all ear dimensions had significant gender effects. A comparison between the anthropometric dimensions and those of current products revealed that most current ear-related products need to be redesigned using anthropometric data. The shapes of earhole and pinna are not circular. Consequently, ear products need to be elongated so that users may feel more comfortably and not have the product slip off easily. PMID:17374520 Extracranial meningioma with extension into a middle ear is very uncommon. A 74-year-old female was admitted to our hospital with right ear bleeding when removing earwax. In this case, magnetic resonance imaging, computed tomography, her past history and operative findings would consider as infiltrative growth from the right sphenoid ridge meningioma to the right middle ear via the right petrous pyramid and bilateral optic nerve. She underwent only partial extirpation with decompression for optic nerve, rather than total extirpation including middle ear and temporal bone, due to wide invasion of the middle cranial fossa and caversinus sinus. Kusunoki, Takeshi; Ikeda, Katsuhisa; Miyashita, Mie Extracranial meningioma with extension into a middle ear is very uncommon. A 74-year-old female was admitted to our hospital with right ear bleeding when removing earwax. In this case, magnetic resonance imaging, computed tomography, her past history and operative findings would consider as infiltrative growth from the right sphenoid ridge meningioma to the right middle ear via the right petrous pyramid and bilateral optic nerve. She underwent only partial extirpation with decompression for optic nerve, rather than total extirpation including middle ear and temporal bone, due to wide invasion of the middle cranial fossa and caversinus sinus. PMID:24765466 Kusunoki, Takeshi; Ikeda, Katsuhisa; Miyashita, Mie Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, exposing the ossicles requires the removal of the eardrum, with the result that the ossicles can no longer be stimulated acoustically. To overcome this we devised a new set up in which the ossicles can be driven magnetically. After measuring the response of the eardrum to an acoustic signal, we then remove the eardrum and attach a small magnet to the exposed manubrium (the part of the first auditory ossicle, the malleus, which is normally attached to the eardrum). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a set-up has uses in research on middle ear mechanics, such as the measurement of non-linearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis, or in chronic otitis media. We describe our set up in which the vibrometer unit is attached to a surgical microscope, offering accurate positioning of the laser beam. We discuss the viability of our method and its future potential by presenting some measurements on artificially fixated ears. Peacock, John; Unge, Magnus Von; Dirckx, Joris Twenty-five out of 205 (i.e. 12%) babies born to diabetic mothers in the Birmingham Maternity Hospital in the period 1969-1974 were malformed as against 6% in a control group. The incidence was highest in the group where mothers were on insulin at the time of conception (17 out of 117, i.e. 15%). No correlation was observed between major malformation in this group and age of onset or duration of the diabetes, progressive vascular complications, maternal age, or parity. Cardiovascular malformations were over-represented. Day, R E; Insley, J Cavernous hemangiomas are rare congenital venous malformations having propensity for the head and neck. These venous malformations, especially the large and intraoral ones, may cause severe symptoms requiring aggressive treatment. Today, the main treatment is based on sclerotherapy, associated or no with surgery. We report on a patient presenting an enormous venous malformation of the face and tongue, which was treated successfully in our department by embolization, sclerotherapy and maxillo-facial surgery, with a special focus on Surgiflo in our technique. PMID:20399053 Slaba, S; Braidy, C; Sader, R B; Hokayem, N; Nassar, J The diagonal ear-lobe crease, detectable especially after the age of 40, is still accepted as a sign of coronary heart disease risk. In the literature some authors report an association between anxiety and coronary heart disease. In our work a group of 143 patients with ear-lobe crease showed - in both sexes and in all examined decades (5th, 6th, 7th) - higher levels of anxiety than in the control group. The possible significance of the crease has been considered on the grounds of present knowledge of ear-acupuncture and the somatotopic mapping of CNS on the ear-lobe. PMID:2575346 Romoli, M; Tordini, G; Giommi, A Summary Background Middle ear surgery techniques can improve hearing destroyed by disease, but results of treatment are difficult to predict. Therefore, researchers use a Laser Doppler Vibrometer to measure vibrations of human middle ear ossicles. Material/Methods Measurements of ossicular chain vibrations are performed on fresh human temporal bone specimens using Laser Doppler Vibrometer. Vibrations of stapes are recorded in 3 cases: 1) for intact ossicular chain, 2) when incus long process is removed, and 3) after long process reconstruction with bone cement. A typical analysis of transfer function is completed by other methods applied in dynamics. Results Measurements and analysis of stapes vibrations in case of intact and damaged ossicular chain show regular and irregular behavior which can be recognize with the help of phase portraits, recurrence plots, correlation dimension, and Hurst and Lyapunov exponents. The long process reconstruction with bone cement gives good results in improving hearing. Conclusions Recurrence plots, and Lyapunov and Hurst exponents used in the study complete information obtained from transfer function and can be employed to enrich the classical approach to ossicular chain vibrations. Rusinek, Rafal; Szymanski, Marcin; Warminski, Jerzy; Zadrozniak, Marek; Morshed, Kamal A forward genetic screen of N-ethyl-N-nitrosourea mutagenized Xenopus tropicalis has identified an inner ear mutant named eclipse (ecl). Mutants developed enlarged otic vesicles and various defects of otoconia development; they also showed abnormal circular and inverted swimming patterns. Positional cloning identified specificity protein 8 (sp8), which was previously found to regulate limb and brain development. Two different loss-of-function approaches using transcription activator-like effector nucleases and morpholino oligonucleotides confirmed that the ecl mutant phenotype is caused by down-regulation of sp8. Depletion of sp8 resulted in otic dysmorphogenesis, such as uncompartmentalized and enlarged otic vesicles, epithelial dilation with abnormal sensory end organs. When overexpressed, sp8 was sufficient to induce ectopic otic vesicles possessing sensory hair cells, neurofilament innervation in a thickened sensory epithelium, and otoconia, all of which are found in the endogenous otic vesicle. We propose that sp8 is an important factor for initiation and elaboration of inner ear development. PMID:24722637 Chung, Hyeyoung A; Medina-Ruiz, Sofia; Harland, Richard M The purpose of the study was to describe the magnitude of the selected sports medicine problems (i.e. cauliflower ear and skin infections) among wrestlers in Tehran. A number of 411 wrestlers were randomly selected from wrestling clubs in Tehran employing cluster sample setting method. The participants were interviewed using a specially designed and validated questionnaire. Nearly half of the participants (44%) had "cauliflower ears". Only 23% of these participants had received any kind of treatment for their acute ear haematomas that are known to result in "cauliflower ears". The prevalence of reported hearing loss among participants with cauliflower ears (11.5%, 95%CI: 6.9 to 16.2) was significantly more than this prevalence among those participants without cauliflower ears (1.8%, 95%CI: 0.1 to 3.5) (p < 0.05). More than half of the participants (52%) had skin infection diagnosed by a physician during the previous year. This study has identified evidence of an increase in hearing loss as a possible side effect of either cauliflower ear or ear injury in wrestling in Iran. There has been an outbreak of ringworm and there is a significant potential for an outbreak of impetigo among wrestlers in Tehran. Key pointsSkin infections are prevalent among wrestlers in Tehran.Commonly wrestlers in Tehran continue to carry out wrestling training while affected by skin infections.Cauliflower ear "is common among wrestlers in Tehran.More research is needed to investigate hearing loss as a possible side effect of either cauliflower ear or ear injury in wrestling in Iran. PMID:24198702 Kordi, Ramin; Mansournai, Mohammad Ali; Nourian, Roh Allah; Wallace, W Angus Background The senses of hearing and balance depend upon mechanoreception, a process that originates in the inner ear and shares features across species. Amphibians have been widely used for physiological studies of mechanotransduction by sensory hair cells. In contrast, much less is known of the genetic basis of auditory and vestibular function in this class of animals. Among amphibians, the genus Xenopus is a well-characterized genetic and developmental model that offers unique opportunities for inner ear research because of the amphibian capacity for tissue and organ regeneration. For these reasons, we implemented a functional genomics approach as a means to undertake a large-scale analysis of the Xenopus laevis inner ear transcriptome through microarray analysis. Results Microarray analysis uncovered genes within the X. laevis inner ear transcriptome associated with inner ear function and impairment in other organisms, thereby supporting the inclusion of Xenopus in cross-species genetic studies of the inner ear. The use of gene categories (inner ear tissue; deafness; ion channels; ion transporters; transcription factors) facilitated the assignment of functional significance to probe set identifiers. We enhanced the biological relevance of our microarray data by using a variety of curation approaches to increase the annotation of the Affymetrix GeneChip® Xenopus laevis Genome array. In addition, annotation analysis revealed the prevalence of inner ear transcripts represented by probe set identifiers that lack functional characterization. Conclusions We identified an abundance of targets for genetic analysis of auditory and vestibular function. The orthologues to human genes with known inner ear function and the highly expressed transcripts that lack annotation are particularly interesting candidates for future analyses. We used informatics approaches to impart biologically relevant information to the Xenopus inner ear transcriptome, thereby addressing the impediment imposed by insufficient gene annotation. These findings heighten the relevance of Xenopus as a model organism for genetic investigations of inner ear organogenesis, morphogenesis, and regeneration. In the vertebrate inner ear, the ability to detect angular head movements lies in the three semicircular canals and their sensory tissues, the cristae. The molecular mechanisms underlying the formation of the three canals are largely unknown. Malformations of this vestibular apparatus found in zebrafish and mice usually involve both canals and cristae. Although there are examples of mutants with only defective canals, few mutants have normal canals without some prior sensory tissue specification, suggesting that the sensory tissues, cristae, might induce the formation of their non-sensory components, the semicircular canals. We fate-mapped the vertical canal pouch in chicken that gives rise to the anterior and posterior canals, using a fluorescent, lipophilic dye (DiI), and identified a canal genesis zone adjacent to each prospective crista that corresponds to the Bone morphogenetic protein 2 (Bmp2)-positive domain in the canal pouch. Using retroviruses or beads to increase Fibroblast Growth Factors (FGFs) for gain-of-function and beads soaked with the FGF inhibitor SU5402 for loss-of-function experiments, we show that FGFs in the crista promote canal development by upregulating Bmp2. We postulate that FGFs in the cristae induce a canal genesis zone by inducing/upregulating Bmp2 expression. Ectopic FGF treatments convert some of the cells in the canal pouch from the prospective common crus to a canal-like fate. Thus, we provide the first molecular evidence whereby sensory organs direct the development of the associated non-sensory components, the semicircular canals, in vertebrate inner ears. PMID:15280215 Chang, Weise; Brigande, John V; Fekete, Donna M; Wu, Doris K Introduction Mesenteric lymphatic malformations are rare, benign tumors that are most commonly found in children. The presentation of these tumors is variable and may either be innocuous or life threatening. It has been suggested that mesenteric lymphatic malformations are congenital; however, there is evidence that their growth may be stimulated by local trauma. Case presentation We describe the first case of a mesenteric lymphatic malformation associated with acute appendicitis in a 13-year-old Caucasian boy. The patient is well six months after surgical excision of the tumor. Conclusion The reader should be aware that growth and/or development of mesenteric lymphatic malformations may be associated with trauma and other pro-inflammatory processes. When embryological development of the internal and/or external genitalia is disrupted, the patient presents with a disorder of sex development (DSD) in the neonatal period or sometime later in life. Some of these patients have other, nongenital malformations, which makes their overall management more complex than if they just had a DSD. This Review summarises these malformation syndromes and discusses the recent research into their aetiology. The genetic causes of these malformation syndromes, when they are known, will also be described. Many specific genetic mutations are now known in malformation syndromes with a defect in hormonal function. By contrast, the genetic causes remain unknown in many nonhormonal morphological anomalies that affect the genitalia. PMID:24913517 Hutson, John M; Grover, Sonia R; O'Connell, Michele; Pennell, Samuel D Macrocephaly-capillary malformation (OMIM 602501) is a rare overgrowth and asymmetry syndrome. Cardiac arrhythmias were reported to occur in few patients. We present a case in which fetal arrhythmia was the presenting symptom of the syndrome. PMID:22329570 Kuint, Jacob; Globus, Omer; Ben Simon, Guy J; Greenberger, Shoshana Mice used to model induction of cleft palate and kidney malformations in offspring following maternal treatment with TCDD, were dosed on gestation day with hexachlorobiphenyl (HCB) and/or with tetrachlorodibenzo-p-dioxin (TCDD) to investigate the potentia... R. E. Morrissey M. W. Harris J. J. Diliberto L. S. Birnbaum Summary Cerebral arteriovenous malformations (C-AVMs) are rarely diagnosed in utero. Most prenatal imaging of intracranial vascular malformations relates to Vein of Galen aneurysmal malformations (VGAMs) or Dural Arteriovenous Malformations (D-AVMs). We report a case of a fetal pial AVF with multiple fistulae and venous pouches, which appeared as an anechoic lesion on the prenatal ultrasound scan. The patient was asymptomatic with normal postnatal growth. No haemodynmaic disturbance was evident. Postnatal Computed tomography (CT), Magnetic Resonance Imaging (MRI) and catheter Digital Subtraction Angiography (DSA) confirmed the presence of a pial AVF. The angiographic findings and family history of nose bleeds suggests the diagnosis of Hereditary Hemorrhagic Telangiectasia. The largest AVF was embolized with tissue adhesive; the residual AVF subsequently removed by surgical excision. Auyeung, K.M.; Laughlin, S.; TerBrugge, K.G. Background\\/purposeMany reports have addressed the feasibility and safety of using robotic surgery in children. To our knowledge, no published report has described the use of a surgical robot in the repair of anorectal malformations (ARMs). Abdulrahman AlBassam; Abdulmonem Gado; Mohammed Saquib Mallick; Mohammed AlNaami; Weaam Al-shenawy Vascular malformations and tumors comprise a broad spectrum of lesions that can cause significant morbidity and even mortality\\u000a in children and adults. Classification of vascular malformations into high flow and low flow has significant impact on management\\u000a since the main treatment of the former is transarterial embolization and the later percutaneous sclerotherapy. Magnetic resonance\\u000a imaging (MRI) is a noninvasive effective Hicham Moukaddam; Jeffrey Pollak; Andrew H. Haims Two patients (11 and 23 months old, respectively) had successful transcatheter occlusion of congenital arterio-venous or veno-venous malformations using detachable silicone balloons. Patient 1 with a cervicofacial hemangioma had selective embolization of the feeding artery with two balloons. Patient 2, with a veno-venous malformation between a systemic vein and pulmonary vein also had selective embolization of the lesion with two balloons. Follow-up examinations confirm a successful outcome in both cases. PMID:8894781 Sreeram, N; Miller, P; John, P A 31-year-old woman was referred to our hospital because of gross hematuria. Radiologic examinations with computed tomography and magnetic resonance imaging revealed renal arteriovenous malformation in the right kidney and an entrapped retroaortic left renal vein. Because the patient was free of hematuria, she refused additional treatment for economic reasons. Renal arteriovenous malformation associated with the posterior nutcracker phenomenon is a very rare condition. PMID:23031411 Qin, Jie; Zheng, Xiang-Yi; Jiang, Hai A close relation between different forms of dysganglionosis such as intestinal neuronal dysplasia (IND) type B and aganglionosis\\u000a has been established. No systematic analysis of other malformations and diseases accompanying IND has been made as yet. Congenital\\u000a malformations and perinatal morbidity were analyzed in 109 patients with IND seen at the Department of Pediatric Surgery in\\u000a Mainz from 1977 to S. Berger; P. Ziebell; M. Kessler; S. Hofmann-von Kap-herr Lymphatic malformations of the head and neck, also known as lymphangiomas or cystic hygromas, are a diverse group of lesions. Lymphangiomas represent benign hamartomatous tumors of lymphatic vessels with a marked predilection for the head, neck and oral cavity. These lesions, like most of the swellings occurring in the neck, frequently pose a dilemma in diagnosis and treatment. This is a case report of a lymphatic malformation which presented as a painless mass in the lower border of mandible. Ramashankar; Prabhakar, Chandan; Shah, Nishit Kumar; Giraddi, Girish The possibility of preventing or reducing the prevalence of human congenital malformations is discussed in the light of current knowledge about their aetiology. The original data presented are derived from an epidemiological study of all the infants (92,982) born in the three years 1964-6 to women resident in a defined area of South Wales and all the congenital malformations (3,242) discovered in those infants by the second anniversary of their birth. Lowe, C. R. PURPOSE: To illustrate that orbital venous-lymphatic malformations (lymphangiomas) may rarely simulate cavernous hemangiomas.METHODS: Retrospective case review.RESULTS: Five patients were identified from a series of 85 patients with venous-lymphatic malformations. The age range was 21 to 69 years, and all cases presented with a history of slowly progressive or long-standing proptosis. Computerized tomography revealed relatively homogeneous intraconal masses that were well Dinesh Selva; Diego Strianese; Giulio Bonavolonta; Jack Rootman This recently recognised entity (OMIM # 602501) (OMIM 2006) is characterised by the association of macrocephaly (megalencephaly),\\u000a capillary malformation of the cutis marmorata telangectatica congenita type, cavernous haemangioma, asymmetric growth pattern,\\u000a central nervous system malformations, and neurological abnormalities (Clayton-Smith et al. 1997, Gerritsen et al. 2000, Moore et al. 1997, Lapunzina et al. 2004). Despite extensive investigation of many of Pablo Lapunzina; Jill Clayton-Smith ...Ear, nose, and throat synthetic polymer material. 874.3620 Section...Ear, nose, and throat synthetic polymer material. (a) Identification. Ear, nose, and throat synthetic polymer material is a device... ...Ear, nose, and throat synthetic polymer material. 874.3620 Section...Ear, nose, and throat synthetic polymer material. (a) Identification. Ear, nose, and throat synthetic polymer material is a device... IN the previous issue, the first of two articles on feline ear, nose and throat disease concentrated on diseases of the nose (In Practice, April 1995, pp 154-161). Here, in the second article, diseases of the throat and ear are covered. Kim Willoughby; Alison Coutts Although acoustics of the external ear has been studied extensively for auralization and hearing aids, the acoustic behavior with insert headphones is not as well known. Our research focused on the eects of outer ear physical dimensions, particularly to sound pressure at the eardrum. The main parameter was the length of the canal, but eardrum's damping of resonances was also Marko Hiipakka; Miikka Tikander; Matti Karjalainen This paper presents a description and comparison of the Nazlet Khater 2 (NK 2) inner ear structures. This specimen is the only complete modern human skeleton from the earliest Late Stone Age in Africa. The interest in the inner ear structures lies with their strong genetic component. The morphology and biometrical characteristics of the NK 2 bony labyrinth are described L. Bouchneb; I. Crevecoeur The efficient and systematic development of a middle ear prosthesis necessitates the use of computer models for the prosthesis itself and the reconstructed middle ear. The structure and parameters of the computer model have to be verified by specific measurements of the implant and the reconstructed ear. To obtain a realistic model of a reconstructed ear, three steps of modeling and measurements have been carried out. To get a first approach of the coupling elements a mechanical test rig representing a simplified reconstructed middle ear was built. The velocity of the stapedial footplate was measured with a laser Doppler vibrometer. The corresponding computer model was formulated, and the respective parameters were determined using the measured dynamical transfer functions. In the second step, a prosthesis was implanted into a human temporal bone without inner ear. Exciting this system with noise, the velocity of the stapes footplate was measured with the laser Doppler vibrometer. Based on the multibody system approach, a mechanical computer model was generated to describe the spatial motions of the reconstructed ossicular chain. Varying some significant parameters, simulations have been carried out. To describe the dynamical behavior of the system consisting of middle and inner ear, the computer model used in the second step has been enlarged by adding a simplified structure of the inner ear. The results were compared with in situ measurements taken from living humans. PMID:10187927 Eiber, A; Freitag, H G; Burkhardt, C; Hemmert, W; Maassen, M; Rodriguez Jorge, J; Zenner, H P ...4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal shank. The... ...4140 Ear, nose, and throat bur. (a) Identification. An ear, nose, and throat bur is a device consisting of...nose, or throat area. The bur consists of a carbide cutting tip on a metal shank or a coating of diamond on a metal shank. The... The effects of direct variation in static middle ear pressure were studied in the acute guinea pig. Pressure differences of from - 500 to + 500 mm of H2O were examined. Pure tones were delivered to the test ear in a closed system and the sound pressure le... D. L. McPherson J. M. Miller A. Axelsson Researchers may place a catheter in the ear vessel of a rabbit for a short period of time in order to collect repeated blood samples without extensive restraint of the animal. Maintaining such a catheter in a healthy rabbit can be challenging, as the animal may scratch at the ear, removing the catheter or forming a large hematoma that might Rekha N. Orchard; Aphroditi J. Antonopoulos; Donald L. Hamilton; Francesca Sampieri The rabbit ear microcirculation was analyzed in a chronic unanesthetized model to evaluate alpha adrenergic microvascular control in a thermoregulatory end organ. This model allowed direct measurement of microcirculatory responses without the effects of anesthetics or inflammatory responses induced by acute surgical intervention. The ipsilateral facial artery was catheterized for drug injections into the experimental ear. Microvascular diameter changes following Zhongyu Li; L. Andrew Koman; Beth P. Smith; E. Stanley Gordon; Thomas L. Smith The middle ear is a composite organ formed from all three germ layers and the neural crest. It provides the link between the outside world and the inner ear, where sound is transduced and routed to the brain for processing. Extensive classical and modern studies have described the complex morphology and origin of the middle ear. Non-mammalian vertebrates have a single ossicle, the columella. Mammals have three functionally equivalent ossicles, designated the malleus, incus and stapes. In this review, I focus on the role of genes known to function in the middle ear. Genetic studies are beginning to unravel the induction and patterning of the multiple middle ear elements including the tympanum, skeletal elements, the air-filled cavity, and the insertion point into the inner ear oval window. Future studies that elucidate the integrated spatio-temporal signaling mechanisms required to pattern the middle ear organ system are needed. The longer-term translational benefits of understanding normal and abnormal ear development will have a direct impact on human health outcomes. Chapman, Susan Caroline Patch testing of 40 patients with chronic inflammatory ear disease demonstrated medicament allergic contact dermatitis in 35%. The most frequent sensitizers were neomycin, framycetin, clioquinol and gentamicin. Although allergic contact dermatitis to dewaxing ear drops was unusual, irritant reactions were common. Holmes, R C; Johns, A N; Wilkinson, J D; Black, M M; Rycroft, R J This study established a normal middle ear resonance estimated from sweep frequency tympanometry, established normal equivalent ear canal volume, static acoustic admittance, and tympanometric peak pressure at 226 hertz in 90 children with normal hearing and 68 children with deafness, ages 6-15. No significant intergroup or age differences were… Hanks, Wendy D.; Rose, Katie J. Pressure damage to the middle ear in 74 cases of pressure trauma was studied. It was found that pressure trauma can be most serious with respect to reducing work capability of personnel in flight, and that pressure trauma of the middle ear developed with acute rhinitis. Medvezhova, R. A. Declines in amphibian populations, and amphibians with gross malformations, have prompted concern regarding the biological status of many anuran species. A survey of bullfrogs, Rana catesbeiana, and green frogs, Rana clamitans, conducted in central and southern New Hampshire showed malformed frogs at 81% of the sites sampled (13 of 16 sites). Brain gonadotropin-releasing hormone (GnRH) and the synthesis of androgens and estradiol, hormones essential to reproductive processes, were measured from limb-malformed and normal (no limb malformation) frogs. Normal frogs had significantly higher concentrations (nearly 3-fold) of in vitro produced androgens and of brain GnRH than malformed frogs. Because most malformations are thought to occur during development, we propose that environmental factors or endocrine-disrupting chemicals that may cause developmental abnormalities also act during early development to ultimately cause abnormally reduced GnRH and androgen production in adult frogs. The consequences of reduced GnRH and androgens on anuran reproductive behavior and population dynamics are unknown but certainly may be profound and warrant further research. Sower, S A; Reed, K L; Babbitt, K J Malformations of cortical development are disorders of altered brain anatomy and architecture that arise from abnormalities in the usual processes of cerebral cortical development. Although they often lead to epilepsy, cognitive delay, and motor impairment, little is known about their effect on sleep. Since malformations may anatomically or functionally disrupt the cerebral circuits that mediate sleep spindles, we hypothesized that these disorders would be associated with abnormal spindle characteristics. We analyzed the density, maximum frequency, laterality and distribution of sleep spindles seen in routine and long-term electroencephalographic recordings performed in ten brain malformation subjects and ten matched controls. There were no significant differences in spindle density or maximum frequency between the two groups, but malformation subjects had a significantly lower proportion of bilateral spindles and a significantly higher proportion of anterior and diffuse spindles compared to controls. In addition, unilateral malformations appeared to be associated with a skewing of unilateral spindles toward the contralateral side. Our findings suggest that brain malformations disrupt the thalamocortical circuits responsible for sleep spindle generation, and support the need for further studies on the relationships between cortical maldevelopment and sleep. Selvitelli, Megan F.; Krishnamurthy, Kaarkuzhali B.; Herzog, Andrew G.; Schomer, Donald L.; Chang, Bernard S. In recent years, the segmentation, i.e. the identification, of ear structures in video-otoscopy, computerised tomography (CT) and magnetic resonance (MR) image data, has gained significant importance in the medical imaging area, particularly those in CT and MR imaging. Segmentation is the fundamental step of any automated technique for supporting the medical diagnosis and, in particular, in biomechanics studies, for building realistic geometric models of ear structures. In this paper, a review of the algorithms used in ear segmentation is presented. The review includes an introduction to the usually biomechanical modelling approaches and also to the common imaging modalities. Afterwards, several segmentation algorithms for ear image data are described, and their specificities and difficulties as well as their advantages and disadvantages are identified and analysed using experimental examples. Finally, the conclusions are presented as well as a discussion about possible trends for future research concerning the ear segmentation. PMID:22994296 Ferreira, Ana; Gentil, Fernanda; Tavares, João Manuel R S As a new biometrics authentication technology, ear recognition remains many unresolved problems, one of them is the occlusion problem. This paper deals with ear recognition with partially occluded ear images. Firstly, the whole 2D image is separated to sub-windows. Then, Neighborhood Preserving Embedding is used for feature extraction on each subwindow, and we select the most discriminative sub-windows according to the recognition rate. Thirdly, a multi-matcher fusion approach is used for recognition with partially occluded images. Experiments on the USTB ear image database have illustrated that using only few sub-window can represent the most meaningful region of the ear, and the multimatcher model gets higher recognition rate than using the whole image for recognition. Yuan, Li; Wang, Zhen-Hua; Mu, Zhi-Chun The effects of early right ear deafness on lateralisation of auditory language functions are not fully known. A 36 year old right handed man, with a history of perinatal right ear deafness and undergoing evaluation for surgical treatment of seizures that began at age 10 years was studied. Language lateralisation testing by intracarotid sodium amobarbital injection showed receptive and expressive language functions to be strongly lateralised to the left hemisphere. Results with intracarotid sodium amobarbital injection further suggested that transmission of auditory input to the patient's left hemisphere was partially dependent on ipsilateral left ear pathways. Cortical language mapping through implanted subdural electrodes localised auditory language functions to traditional left posterior perisylvian language areas. These results suggest that early right ear deafness does not impede left hemisphere lateralisation and localisation of auditory language functions. Moreover, transmission of auditory information to the patient's left hemisphere seems to be accomplished, in part, by recruitment of ipsilateral left ear pathways.?? Boatman, D.; Krauss, G. Context: Self-ear-cleaning has been reported to be common from several hospital-based studies and it has been associated with some diseases of the ear. Aims: To determine community-based prevalence of self-ear-cleaning and its sociodemographic correlates among educated young adults in Nigeria. Settings and Design: A cross-sectional survey conducted in a National Youth Service Corps camp in Nigeria. Subjects and Methods: Semistructured questionnaires were administered on a randomly selected sample of 1280 respondents. The outcome variable was self-ear-cleaning. Independent variables were sociodemographic variables, materials used and ear-cleaning habits. Statistical Analysis Used: Statistical Package for the Social Sciences (SPSS) version 15 was utilized for univariate, bivariate, and multiple logistic regression analysis. Results: There were 1012 respondents (M: F = 1.05:1). Mean age was 25.3 (standard deviation, 2.34). Prevalence of self-ear-cleaning was 93.4%. Mean age at first cleaning was 7.6 years. Cotton buds were the most frequently used objects (in 85.1%). Prevalence was high irrespective of sociodemographic class, significantly higher among females (?2 = 4.549, P = 0.033), those who believed the habit was beneficial (?2 = 114.185, P < 0.001) and those whose parents and siblings practiced the habit. Significant predictive factors were self-ear-cleaning in respondent's father [odds ratio (OR) P = 0.011) and owning cotton buds (OR = 0.192, P = 0.007). Conclusions: Self-ear-cleaning is almost universal. Most of the population is, therefore, at risk of possible harmful effects. Also, medical advice against self-ear-cleaning is not widely known. Rather, the erroneous perception that self-ear-cleaning is beneficial is common. Collaborative health education efforts targeted at families and schools and campaigns and advocacy for legislation regulating the sale of cotton buds are recommended. Olaosun, Adedayo Olugbenga We describe the complications of endovascular embolization for cerebral arteriovenous malformations (AVMs). 164 consecutive patients with AVMs treated by endovascular embolization between 1998 and 2003 were retrospectively reviewed. Over a five-year period, 292 embolization procedures were performed in 164 patients with embolization as the primary treatment modality (n=126) or as an adjunct to surgery (n =14) or radiosurgery (n=24). There were a total of 15 (9.1% per patient, 5.1% per procedure) complications in this series, of which 14 were clinically significant (8.5% of patients, 4.8% per procedure), and one was a technical complication (0.6% of patients, 0.3% per procedure). Excellent or good outcomes (Glasgow Outcome Scale ? 4) were observed in 136 (82.9%) patients at discharge. Neurological deficits (Glasgow Outcome Scale 1-4) as a direct result of embolization were 7.3% at discharge. In a single-center, retrospective, nonrandomized study, 82.9% of patients had excellent or good outcomes at discharge after AVM embolization, with a complication rate of 5.1%. PMID:24029089 Sun, Y; Lv, X; Li, Y; Li, A Stereotactic linear accelerator (linac) radiosurgery has been in operation in the West Midlands since 1987, the first of its kind in the United Kingdom. Forty two patients with high-flow cerebral arteriovenous malformations have been treated, 26 of whom have been followed up. Angiography one year after treatment showed that five lesions were obliterated, 11 were reduced in size and/or flow rate and 10 were unchanged. Overall results show that nine out of 10 patients reviewed at 24 months had total obliteration. Three patients had complications; one has fully recovered, one died of an unrelated cause at 36 months and the other died from recurrent haemorrhage at nine months. Two patients had recurrent non-fatal haemorrhage within 24 months of treatment; both recovered without further deficit. All patients are fit to work but eight are unemployed. Although the follow up period is short, the early results indicate a success rate similar to those published by others using linac radiosurgery. Images Kenny, B G; Hitchcock, E R; Kitchen, G; Dalton, A E; Yates, D A; Chavda, S V The synthesis of tooth development biology with human studies focusing on inherited conditions that specifically interfere with tooth development is improving our understanding of normal and pathological tooth formation. The type of inherited dental malformations observed in a given kindred relate to when, during odontogenesis, the defective gene is critically expressed. Information about the protein encoded by the defective gene and the resulting dental phenotype helps us understand the major processes underway at different stages during tooth development. Genes affecting early tooth development (PAX9, MSX1, and AXIN2) are associated with familial tooth agenesis or oligodontia. Genes expressed by odontoblasts (COL1A1, COL1A2, and DSPP), and ameloblasts (AMELX, ENAM, MMP20, and KLK4) during the crown formation stage, are associated with dentinogenesis imperfecta, dentin dysplasia, and amelogenesis imperfecta. Late genes expressed during root formation (ALPL and DLX3) are associated with cementum agenesis (hypophosphatasia) and taurodontism. Understanding the relationships between normal tooth development and the dental pathologies associated with inherited diseases improves our ability to diagnose and treat patients suffering the manifestations of inherited dental disorders. PMID:17552940 Hu, Jan C-C; Simmer, James P Congenital pulmonary airway malformation, or congenital cystic adenomatoid malformation, is postulated to be a disorder of pulmonary airway morphogenesis and encompasses 5 different types with distinct levels or stages of tracheobronchial development. We present a unique case of type 2 congenital pulmonary airway malformation with a previously undocumented combination of multiple extrapulmonary anomalies, featuring ipsilateral multicystic renal dysgenesis, contralateral renal Truc T. Pham; Kurt Benirschke; Eliezer Masliah; J. Thomas Stocker; Eunhee S. Yi Culturing middle ear fluid samples from children with chronic otitis media with effusion (OME) using standard techniques results in the isolation of bacterial species in approximately 30-50% of the cases. Haemophilus influenzae, Streptococcus pneumoniae and Moraxella catarrhalis, the classic middle ear pathogens of acute otitis media, are involved but, recently, several studies suggested Alloiococcus otitidis as an additional pathogen. In the present study, we used species-specific PCRs to establish the prevalence, in both the nasopharyngeal cavity and the outer ear, of H. influenzae, M. catarrhalis, S. pneumoniae and A. otitidis. The study group consisted of 70 healthy volunteers (aged 19-22 years). The results indicate a high prevalence (>80%) of A. otitidis in the outer ear in contrast to its absence in the nasopharynx. H. influenzae was found in both the outer ear and the nasopharynx (6% and 14%, respectively), whereas S. pneumoniae and M. catarrhalis were found only in the nasopharynx (9% and 34%, respectively).A. otitidis, described as a fastidious organism, were able to be cultured using an optimized culture protocol, with prolonged incubation, which allowed the isolation of A. otitidis in five of the nine PCR-positive samples out of the total of ten samples tested. Given the absence of the outer ear inhabitant A. otitidis from the nasopharynx, its role in the aetiology of OME remains ambiguous because middle ear infecting organisms are considered to invade the middle ear from the nasopharynx through the Eustachian tube. PMID:19895585 De Baere, T; Vaneechoutte, M; Deschaght, P; Huyghe, J; Dhooge, I This study was undertaken to develop a functional model of otitis media with effusion (OME) in the pig (Sus scrofa), with the purpose of investigating the origin of lymphocytes populating the middle ear during the course of an inflammatory process. The relevance of the model to the human condition of OME is to a large extent dependent on the anatomical and physiological similarities between the middle ear cavity and the pharyngeal lymphoid tissue of the pig and man. Anatomical specimens were collected from 7 young Large White pigs to determine the gross anatomy of the middle ear cavity and the histological characteristics of the middle ear mucosa. It was found that the anatomy of the 3 parts of the middle ear cavity in man and in the pig is broadly similar, although some minor differences were observed. The porcine eustachian tube was seen to be cartilaginous throughout its length in contrast to the part osseous, part cartilaginous structure found in man; the porcine ossicles were slightly different in shape to those of man and the air cell system was situated inferior to the tympanic cavity in the pig as opposed to posteriorly in man. This paper describes the structure and morphology of the pig middle ear cavity and compares and contrasts it with that of man. The minor differences observed are of anatomical importance but do not diminish the usefulness of the pig middle ear cleft as a potential model for human middle ear disorders. PRACY, J. P.; WHITE, A.; MUSTAFA, Y.; SMITH, D.; PERRY, M. E. The achievement of volume geometry data from middle ear structures and surrounding components performs a necessary supposition for the finite element simulation of the vibrational and transfer characteristics of the ossicular chain. So far those models base on generalized figures and size data from anatomy textbooks or particular manual and one- or two-dimensional distance measurements of single ossicles, mostly obtained by light microscopy, respectively. Therefore the goal of this study is to create a procedure for complete three-dimensional imaging of real middle ear structures (tympanic membrane, ossicles, ligaments) in vitro or even in vivo. The main problems are their microscopic size with relevant structures from 10 micrometer to 5 mm, representing various tissue properties (bone, soft tissue). Additionally, these structures are surrounded by the temporal bone, the most solid bone of the human body. Generally there exist several established diagnostic tools for medical imaging that could be used for geometry data acquisition, e.g., X-ray computed tomography and magnetic resonance imaging. Basically they image different tissue parameters, either bony structures (ossicles), or soft tissue (tympanic membrane, ligaments). But considering this application those standard techniques allow low spatial resolution only, usually in the 0.5 - 1mm range, at least in one spatial direction. Thus particular structures of the middle ear region could even be missed completely because of their spatial location. In vitro there is a way out by collecting three complete data sets, each distinguished by 90 degree rotation of a cube-shaped temporal bone specimen. That allows high-resolution imaging in three orthogonal planes, which essentially supports the three-dimensional interpolation of the unknown elements, starting from the regularly set elements of the cubic grid with an edge extension given by the original two-dimensional matrix. A different approach represents the application of a micro- tomographic imaging device. Therefore an X-ray beam focused down to few microns passes the object in a tomographic arrangement. Subsequently the slices become reconstructed. Generally spatial resolution down to 10 micrometer may be obtained by using this procedure. But there exist few devices only, it is not available as standard equipment. The best results concerning spatial resolution should be achieved by applying conventional histologic sectioning techniques. Of course the target will become destroyed during the procedure. It is cut into sections (e.g., 10 micrometer thick), every layer is stained, and the image acquired and stored by a digital still-camera with appropriate resolution (e.g., 2024 X 3036). Three-dimensional reconstruction is done with the computer. The staining allows visual selection of bones and soft tissues, resolutions down to 10 micrometer are possible without target segmentation. But there arise some practical problems. Mainly the geometric context of the layers is affected by the cutting procedure, especially if cutting bone. Another problem performs the adjustment of the -- possibly distorted -- slices to each other. Artificial markers are necessary, which could allow automatic adjustment too. But the introduction and imaging of the markers is difficult inside the temporal bone specimen, that is interspersed by several cavities. Of course the internal target structures must not be destroyed by the marker introduction. Furthermore the embedding compound could disturb the image acquisition, e.g., by optical scattering of paraffin. A related alternative is given by layered ablation/grinding and imaging of the top layer. This saves the geometric consistency, but requires very tricky and time-consuming embedding procedures. Both approaches require considerable expenditures. The possible approaches are evaluated in detail and first results are compared. So far none of the above-mentioned procedures has been established as a standard tool for three-dimensional geometry data acquisition of the middle ear. Otherwise the establi Vogel, Uwe; Schmitt, Thomas The cup-drawing of a strongly anisotropic sheet metal is simulated using a commercial finite element software along with a user material subroutine. In order to accurately describe the plastic anisotropy of the material the well-known recent yield function 'Yld2004-18p' is extended. Regarding the experimental characterization of the considered material the occurrence of dynamic strain aging lead to an oscillating signal of the width change of the tensile samples, which prevented a reliable determination of plastic strain ratios (r-values). Thus, an improved measurement concept was developed that leads to a very robust and reproducible determination of r-values. Furthermore, a novel plane-strain tensile test sample is presented which is used for the characterization of the plastic anisotropy in biaxial loading states. A quantitative comparison with measured earing profiles of deep drawn cups illustrates the predictive capabilities of the numerical simulation. Aretz, Holger; Aegerter, Johannes; Engler, Olaf [Hydro Aluminium Deutschland GmbH, Research and Development, Georg-von-Boeselager-Str. 21, D-53117 Bonn (Germany) A musical canon consists of two melodic lines with the second part copying the first exactly after some time delay. Right-handed\\u000a adults listened to canons presented dichotically at time delays between the ears of 2, 4, and 8 sec. Presentation rate varied\\u000a from 1.0 to 4.4 notes\\/sec in one part. Different groups of subjects heard the canons with the left W. J. Dowling Capillary malformation-arteriovenous malformation (CM-AVM) is an autosomal-dominant disorder, caused by heterozygous RASA1 mutations, and manifesting multifocal CMs and high risk for fast-flow lesions. A limited number of patients have been reported, raising the question of the phenotypic borders. We identified new patients with a clinical diagnosis of CM-AVM, and patients with overlapping phenotypes. RASA1 was screened in 261 index patients with: CM-AVM (n = 100), common CM(s) (port-wine stain; n = 100), Sturge-Weber syndrome (n = 37), or isolated AVM(s) (n = 24). Fifty-eight distinct RASA1 mutations (43 novel) were identified in 68 index patients with CM-AVM and none in patients with other phenotypes. A novel clinical feature was identified: cutaneous zones of numerous small white pale halos with a central red spot. An additional question addressed in this study was the "second-hit" hypothesis as a pathophysiological mechanism for CM-AVM. One tissue from a patient with a germline RASA1 mutation was available. The analysis of the tissue showed loss of the wild-type RASA1 allele. In conclusion, mutations in RASA1 underscore the specific CM-AVM phenotype and the clinical diagnosis is based on identifying the characteristic CMs. The high incidence of fast-flow lesions warrants careful clinical and radiologic examination, and regular follow-up. PMID:24038909 Revencu, Nicole; Boon, Laurence M; Mendola, Antonella; Cordisco, Maria Rosa; Dubois, Josée; Clapuyt, Philippe; Hammer, Frank; Amor, David J; Irvine, Alan D; Baselga, Eulalia; Dompmartin, Anne; Syed, Samira; Martin-Santiago, Ana; Ades, Lesley; Collins, Felicity; Smith, Janine; Sandaradura, Sarah; Barrio, Victoria R; Burrows, Patricia E; Blei, Francine; Cozzolino, Mariarosaria; Brunetti-Pierri, Nicola; Vicente, Asuncion; Abramowicz, Marc; Désir, Julie; Vilain, Catheline; Chung, Wendy K; Wilson, Ashley; Gardiner, Carol A; Dwight, Yim; Lord, David J E; Fishman, Leona; Cytrynbaum, Cheryl; Chamlin, Sarah; Ghali, Fred; Gilaberte, Yolanda; Joss, Shelagh; Boente, Maria Del C; Léauté-Labrèze, Christine; Delrue, Marie-Ange; Bayliss, Susan; Martorell, Loreto; González-Enseñat, Maria-Antonia; Mazereeuw-Hautier, Juliette; O'Donnell, Brid; Bessis, Didier; Pyeritz, Reed E; Salhi, Aicha; Tan, Oon T; Wargon, Orli; Mulliken, John B; Vikkula, Miikka A pedigree of branchio-oto-renal dysplasia (the BOR syndrome) is reported, including the documentation by serial audiometric studies of the onset and rapid progression of hearing loss in the twin sister of an affected child. The literature on this syndrome is analyzed to derive some figures for use in genetic counseling of such families. Branchio-oto-renal dysplasia is an autosomal dominant disorder in which affected individuals may have preauricular pits, lachrymal duct stenosis, hearing loss, branchial fistulas or cysts, structural defects of the outer, middle, and inner ear, and renal anomalies, which may range from mild hypoplasia to complete absence. Not all features of the syndrome are expressed in all carriers of the gene, but few carriers lack all the features, and the pits, branchial clefts, and hearing loss, are frequently expressed. Those offspring of affected persons who have pits or fistulas are likely (about 80%) to have hearing loss of varying degrees of severity. A minority of heterozygotes (about 7%) may have hearing loss without pits or fistulas. The risk of severe renal malformation is probably fairly low. Whether families that show dominant inheritance of pits, clefts, and deafness without renal anomalies represent variants of the BOR syndrome or a separate entity (the BO syndrome), is still not clear. At present, any individual with preauricular pits and branchial clefts deserves both otologic and renal investigation. PMID:263442 Fraser, F C; Ling, D; Clogg, D; Nogrady, B The present study summarizes the experimental findings obtained on the pressure in the inner ear fluids and on the effects of pressure changes on cochlear function in the guinea pig. Two types of pressures have to be distinguished in the inner ear fluid compartments: (i) hydrostatic fluid pressure and (ii) superimposed hydrodynamic high frequency (> 100 Hz) sound pressure oscillations. Hydrostatic pressure in the inner ear fluids in guinea pigs is in the order of 200 Pa (2 cm H2O) and shows slow (< 5 Hz) respiratory and pulsatory oscillations as well as considerable physiological variations in the range of -100 to +700 Pa. In normal ears, hydrostatic pressure in the perilymph equals pressure in the endolymph, and pressure changes applied to one compartment are immediately transmitted to the other one. A high compliance of Reissner's membrane seems to be the cause of this endolymphatic-perilymphatic pressure equalization. In experimental endolymphatic hydrops, a unique animal model for Meniere's disease, endolymphatic pressure is higher (100 Pa and above) than perilymphatic pressure. These pressure gradients occur only in late stages of hydrops, probably when Reissner's membrane has lost its high compliance after long standing distension. Positive endolymphatic-perilymphatic pressure gradients are secondary to and not the primary cause of hydrops formation. Changes of hydrostatic pressure do not affect auditory function as long as they stay in the physiological range. This includes the sudden loss of positive inner ear pressure that occurs in perilymph fistulas. The rationale for surgical repair of perilymph fistulas in patients in order to restore the hearing function thus becomes questionable. Other aspects of surgical repair, however, as e.g. prevention of labyrinthitis due to permanently open fistula, could not be investigated in this model, because in guinea pigs even large fistulas heal spontaneously within a few days. In experimental endolymphatic hydrops, deterioration of auditory thresholds was partially correlated to the presence of positive endolymphatic-perilymphatic pressure gradients. A change in pressure, however, occurred later than the first deterioration in auditory function. Therefore positive endo-perilymphatic pressure gradients may contribute to, but are not the only cause of hearing impairment. PMID:8273452 A number of acoustical applications require the transformation of acoustical quantities, such as impedance and pressure that are measured at the entrance of the ear canal, to quantities at the eardrum. This transformation often requires knowledge of the shape of the ear canal. Previous attempts to measure ear-canal area functions were either invasive, non-reproducible, or could only measure the area function up to a point mid-way along the canal. A method to determine the area function of the ear canal from measurements of acoustic impedance at the entrance of the ear canal is described. The method is based on a solution to the inverse problem in which measurements of impedance are used to calculate reflectance, which is then used to determine the area function of the canal. The mean ear-canal area function determined using this method is similar to mean ear-canal area functions measured by other researchers using different techniques. The advantage of the proposed method over previous methods is that it is non- invasive, fast, and reproducible. Rasetshwane, Daniel M.; Neely, Stephen T. CHARGE syndrome is a multiple congenital anomaly disorder that leads to life-threatening birth defects, such as choanal atresia and cardiac malformations as well as multiple sensory impairments, that affect hearing, vision, olfaction and balance. CHARGE is caused by heterozygous mutations in CHD7, which encodes an ATP-dependent chromatin remodeling enzyme. Identification of the mechanisms underlying neurological and sensory defects in CHARGE is a first step toward developing treatments for CHARGE individuals. Here, we used mouse models of Chd7 deficiency to explore the function of CHD7 in the development of the subventricular zone (SVZ) neural stem cell niche and inner ear, structures that are important for olfactory bulb neurogenesis and hearing and balance, respectively. We found that loss of Chd7 results in cell-autonomous proliferative, neurogenic and self-renewal defects in the perinatal and mature mouse SVZ stem cell niche. Modulation of retinoic acid (RA) signaling prevented in vivo inner ear and in vitro neural stem cell defects caused by Chd7 deficiency. Our findings demonstrate critical, cooperative roles for RA and CHD7 in SVZ neural stem cell function and inner ear development, suggesting that altered RA signaling may be an effective method for treating Chd7 deficiency. PMID:24026680 Micucci, Joseph A; Layman, Wanda S; Hurd, Elizabeth A; Sperry, Ethan D; Frank, Sophia F; Durham, Mark A; Swiderski, Donald L; Skidmore, Jennifer M; Scacheri, Peter C; Raphael, Yehoash; Martin, Donna M The epidemiology and natural history of cerebral arteriovenous malformations (AVMs) remains incompletely elucidated. Several factors are responsible. With regard to the incidence and prevalence of AVMs, the results of prior studies have suffered because of the retrospective design, the use of nonspecific ICD-9 codes, and a focus on small genetically isolated populations. Recent data from the New York Islands AVM Hemorrhage Study, an ongoing, prospective, population-based survey determining the incidence of AVM-related hemorrhage and the associated rates of morbidity and mortality in a zip code-defined population of 10 million people, suggests that the AVM detection rate is 1.21/100,000 person-years (95% confidence interval [CI] 1.02-1.42) and the incidence of AVM-hemorrhage is 0.42/100,000 person-years (95% CI 0.32-0.55). Contemporaneous data from the Northern Manhattan Stroke Study, a prospective, longitudinal population-based study of nearly 150,000 patients in which the focus is to define the incidence of stroke, suggest the crude incidence for first-ever AVM-related hemorrhage to be 0.55/100,000 person-years (95% CI 0.11-1.61). Efforts are ongoing to study the natural history of both ruptured and unruptured AVMs in these datasets to examine the relevance of prior studies of patients selected for conservative follow up in Finland. In addition, data are being gathered to determine whether risk factors for future hemorrhage, which have previously been established in small case series, are valid when applied to whole populations. Together, these data should help inform therapeutic decisionmaking. PMID:16466233 Stapf, C; Mohr, J P; Pile-Spellman, J; Solomon, R A; Sacco, R L; Connolly, E S Objective To estimate the frequency, mechanisms and predictive factors of sleep apnoea syndrome (SAS) in a large group of children and adults with type I (CMI) and II (CMII) Chiari malformation (CM). Background The anatomical and functional integrity of both respiratory circuits and lower cranial nerves controlling the upper airway is necessary for breathing control during sleep. These latter structures may be altered in CM, and a few investigations have reported CM related sleep disordered breathing. Methods Forty?six consecutive unrelated patients with CM (40 CMI, six CMII), of which 20 were children (eight males) and 26 were adults (12 males), underwent physical, neurological and oto?rhino?laryngoscopic examination, MRI and polysomnography. Results SAS was present in 31 (67.4%) of the patients with CM (70% of CMI, 50% of CMII, including mainly children). Sixty per cent of children with CM exhibited SAS, including 35% with obstructive (OSAS) and 25% with central (CSAS) sleep apnoea syndrome. SAS was observed in 73% of CM adults (57.7% OSAS, 15.4% CSAS). Severe SAS was found in 23% of CM adults. Multiple regression analysis revealed that age, type II Chiari and vocal cord paralysis predicted the central apnoea index. Conclusion SAS is highly prevalent in all age groups of patients suffering from CM. CSAS, a rare condition in the general population, was common among the patients with CM in our study. Sleep disordered breathing associated with CM may explain the high frequency of respiratory failures observed during curative surgery of CM. Our results suggest that SAS should be systematically screened for in patients with CM, especially before surgery. Dauvilliers, Y; Stal, V; Abril, B; Coubes, P; Bobin, S; Touchon, J; Escourrou, P; Parker, F; Bourgin, P The development and evolution of mechanosensory cells and the vertebrate ear is reviewed with an emphasis on delineating the cellular, molecular and developmental basis of these changes. Outgroup comparisons suggests that mechanosensory cells are ancient features of multicellular organisms. Molecular evidence suggests that key genes involved in mechanosensory cell function and development are also conserved among metazoans. The divergent morphology of mechanosensory cells across phyla is interpreted here as ‘deep molecular homology’ that was in parallel shaped into different forms in each lineage. The vertebrate mechanosensory hair cell and its associated neuron are interpreted as uniquely derived features of vertebrates. It is proposed that the vertebrate otic placode presents a unique embryonic adaptation in which the diffusely distributed ancestral mechanosensory cells became concentrated to generate a large neurosensory precursor population. Morphogenesis of the inner ear is reviewed and shown to depend on genes expressed in and around the hindbrain that interact with the otic placode to define boundaries and polarities. These patterning genes affect downstream genes needed to maintain proliferation and to execute ear morphogenesis. We propose that fibroblast growth factors (FGFs) and their receptors (FGFRs) are a crucial central node to translate patterning into the complex morphology of the vertebrate ear. Unfortunately, the FGF and FGFR genes have not been fully analyzed in the many mutants with morphogenetic ear defects described thus far. Likewise, little information exists on the ear histogenesis and neurogenesis in many mutants. Nevertheless, a molecular mechanism is now emerging for the formation of the horizontal canal, an evolutionary novelty of the gnathostome ear. The existing general module mediating vertical canal growth and morphogenesis was modified by two sets of new genes: one set responsible for horizontal canal morphogenesis and another set for neurosensory formation of the horizontal crista and associated sensory neurons. The dramatic progress in deciphering the molecular basis of ear morphogenesis offers grounds for optimism for translational research toward intervention in human morphogenetic defects of the ear. Fritzsch, Bernd; Pauley, Sarah; Beisel, Kirk W. Biometrics based personal authentication is an effective way for automatically recognizing, with a high confidence, a person’s identity. Recently, 3D ear shape has attracted tremendous interests in research field due to its richness of feature and ease of acquisition. However, the existing ICP (Iterative Closet Point)-based 3D ear matching methods prevalent in the literature are not quite efficient to cope with the one-to-many identification case. In this paper, we aim to fill this gap by proposing a novel effective fully automatic 3D ear identification system. We at first propose an accurate and efficient template-based ear detection method. By utilizing such a method, the extracted ear regions are represented in a common canonical coordinate system determined by the ear contour template, which facilitates much the following stages of feature extraction and classification. For each extracted 3D ear, a feature vector is generated as its representation by making use of a PCA-based local feature descriptor. At the stage of classification, we resort to the sparse representation based classification approach, which actually solves an l1-minimization problem. To the best of our knowledge, this is the first work introducing the sparse representation framework into the field of 3D ear identification. Extensive experiments conducted on a benchmark dataset corroborate the effectiveness and efficiency of the proposed approach. The associated Matlab source code and the evaluation results have been made publicly online available at http://sse.tongji.edu.cn/linzhang/ear/srcear/srcear.htm. Zhang, Lin; Ding, Zhixuan; Li, Hongyu; Shen, Ying Biometrics based personal authentication is an effective way for automatically recognizing, with a high confidence, a person's identity. Recently, 3D ear shape has attracted tremendous interests in research field due to its richness of feature and ease of acquisition. However, the existing ICP (Iterative Closet Point)-based 3D ear matching methods prevalent in the literature are not quite efficient to cope with the one-to-many identification case. In this paper, we aim to fill this gap by proposing a novel effective fully automatic 3D ear identification system. We at first propose an accurate and efficient template-based ear detection method. By utilizing such a method, the extracted ear regions are represented in a common canonical coordinate system determined by the ear contour template, which facilitates much the following stages of feature extraction and classification. For each extracted 3D ear, a feature vector is generated as its representation by making use of a PCA-based local feature descriptor. At the stage of classification, we resort to the sparse representation based classification approach, which actually solves an l1-minimization problem. To the best of our knowledge, this is the first work introducing the sparse representation framework into the field of 3D ear identification. Extensive experiments conducted on a benchmark dataset corroborate the effectiveness and efficiency of the proposed approach. The associated Matlab source code and the evaluation results have been made publicly online available at http://sse.tongji.edu.cn/linzhang/ear/srcear/srcear.htm. PMID:24740247 Zhang, Lin; Ding, Zhixuan; Li, Hongyu; Shen, Ying We report a rare case of granuloma annulare (GA), affecting both ear antihelixes, in a 28-year old male patient that presented with a 1-year history of non-tender, firm, skin-colored, 1~5 mm papules on both ear antihelixes. There was no history of trauma. An excisional biopsy specimen taken from one of the lesions of the right ear revealed infiltration of histiocytes and lymphocytes around a zone of collagen alteration in the dermis. Based on the clinical and pathological findings, the patient was diagnosed with a rare case of bilateral GA of both antihelixes; this is the first report in the Korean dermatology literature. Kim, Jin Gu; Lee, Seung Hun Single field, fixed irradiation of bilateral tympanic cavities using 200-kV x-rays was administered to five guinea pigs. The irradiation dose was 30 Gy. They were killed immediately after irradiation, and bilateral middle ear mucosa was examined for ciliary activity and epithelial structure. Significant deterioration of the ciliary activity in the middle ear mucosa was observed, proximal as well as distal to the eustachian tube. Electron microscopy showed various changes in the irradiated middle ear mucosa. The most conspicuous findings were hyperreactivity in secretion, vacuolation of ciliated cells, and stomal edema. Ohashi, Y.; Nakai, Y.; Esaki, Y.; Ikeoka, H.; Koshimo, H.; Onoyama, Y. This paper summarizes terminology and general concepts involved in animal development for the purpose of providing background for the study and understanding of frog malformations. The results of our radiographic investigation of rear limb malformations in Rana pipiens provide evidence that frog malformations are the product of early developmental errors. Although bacteria, parasites and viruses were identified in these metamorphosed frogs, the relevant window to look for the teratogenic effect of these agents is in the early tadpole stage during limb development. As a result, our microbiological findings must be regarded as inconclusive relative to determining their contribution to malformations because we conducted our examinations on metamorphosed frogs not tadpoles. Future studies need to look at teratogenic agents (chemical, microbial, physical or mechanical) that are present in the embryo, tadpole, and their environments at the stages of development that are relevant for the malformation type. The impact of these teratogenic agents then needs to be assessed in appropriate animal models using studies that are designed to mimic field conditions. The results of these laboratory tests should then be analyzed in such a way that will allow comparison with the findings in the wild-caught tadpoles and frogs. Meteyer, C. U.; Cole, R. A.; Converse, K. A.; Docherty, D. E.; Wolcott, M.; Helgen, J. C.; Levey, R.; Eaton-Poole, L.; Burkhart, J. G. Vascular diseases of the human brain are one of the reasons of deaths and people's incapacitation not only in Russia, but also in the world. The danger of an arteriovenous malformation (AVM) is in premature rupture of pathological vessels of an AVM which may cause haemorrhage. Long-term prognosis without surgical treatment is unfavorable. The reduced impact method of AVM treatment is embolization of a malformation which often results in complete obliteration of an AVM. Pre-surgical mathematical modeling of an arteriovenous malformation can help surgeons with an optimal sequence of the operation. During investigations, the simple mathematical model of arteriovenous malformation is developed and calculated, and stationary and non-stationary processes of its embolization are considered. Various sequences of embolization of a malformation are also considered. Calculations were done with approximate steady flow on the basis of balanced equations derived from conservation laws. Depending on pressure difference, a fistula-type AVM should be embolized at first, and then small racemose AVMs are embolized. Obtained results are in good correspondence with neurosurgical AVM practice. Nadezhda Telegina, Ms; Aleksandr Chupakhin, Mr; Aleksandr Cherevko, Mr The general status of a grant to investigate the origins and evolution of two hair cell types in the ears of a teleost fish, Astronotus ocellatus (the oscar), is presented. First, it was demonstrated that the cells in the rostral end of the saccule of the , Carassius auratus, are type 1-like, while those at the caudal end are type 2 cells. It was demonstrated that the dichotomy of hair cell types found in the utricle of the oscar is also found in the goldfish. Second, the lateral line system of the oscar was examined using gentamicin sulphate, an ototocix drug that destroys type 1- like hair cells but does not appear to damage type 2 hair cells. It was demonstrated that the hair cells found in neuromasts of lateral line canal organs were totally destroyed within 1 day of treatment, while the hair cells in free neuromasts were undamaged after 12 days of treatment. Third, it was demonstrated that the calyx, the specialized nerve ending, is not unique to amniotes and that it is present at least in the cristae of semicirular canals in goldfish. These results have demonstrated that: (1) there are multiple hair cell types in the vestibular endorgans of the ear of fishes, (2) these hair cell types are very similar to those found in the mammalian vestibular endorgans, (3) the nerve calyx is also present in fishes, and (4) multiple hair cell types and the calyx have evolved far earlier in the course of vertebrate evolution than heretofore thought. Understanding the structure of the vestibular endorgans has important implications for being able to understand how these organs respond to gravistatic, acceleration and acoustic input. The vestibular endorgans of fishes may provide an ideal system in which to analyze functional differences in hair cells. Not only are the two hair cell types similar to those found in mammals, they are located in very discrete regions in each endorgan. Thus, it is relatively easy to gain access to cells of one or the other type. The presence of two cell types in the lateral line have equally significant implications for studies of the vestibular system. Popper, Arthur N. (Principal Investigator) Multiple venous malformations (VMs) pose some of the most difficult challenges in the practice of medicine today. Clinical manifestations of these lesions are extremely protean. Because of the rarity of these lesions, experience in their diagnosis and management by most clinicians is limited. This augments the enormity of the problem and can lead to misdiagnoses, inadequate treatment, high complication rates and poor patient outcomes. Because these lesions can recur, removal of the nidus is the main priority. Vascular malformations are best treated in medical centers where patients with these maladies are seen regularly and the team approach is utilized. The presence of intralesional nerve in arteriovenous malformation (AVM) and sometimes in VMs, as reported in this study, provides an additional diagnostic criterion that is simple and reliable and can be readily used to differentiate VMs from hemangiomas. Lakkasetty, Yogesh T; Malik, Sangeeta; Shetty, Akshay; Nakhaei, Kourosh Purpose. To evaluate the usefulness of a mixture of absolute ethanol and lipiodol in the management of venous malformations. Methods. Percutaneous sclerotherapy was performed with a mixture of absolute ethanol and lipiodol (9:1) in 17 patients with venous malformations, once in 12 patients, twice in 5. The therapeutic efficacy was evaluated by pain reduction. Conventional radiographs (n=15) and posttreatment magnetic resonance imaging (n=5) were obtained for the follow-up evaluation. Results. Sclerotherapy was successful in all but two patients. The therapeutic effect was excellent in two patients, good in seven, fair in five, and poor in one. Radiopacity of lipiodol was beneficial for monitoring the procedure rather than for follow-up evaluations. Areas with low signal-intensity strands were increased on T2-weighted images obtained after the sclerotherapy. Conclusion. Sclerotherapy with a mixture of ethanol and lipiodol is effective in treating venous malformations. Suh, Jin-Suck [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Shin, Kyoo-Ho [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of); Na, Jae-Bum [Kyungsang University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Won, Jong-Yun [Yonsei University, College of Medicine, Department of Diagnostic Radiology (Korea, Republic of); Hahn, Soo-Bong [Yonsei University, College of Medicine, Department of Orthopedic Surgery (Korea, Republic of) White lions (Panthera leo krugeri) have never been common in the wild, and at present, the greatest population is kept in zoos where they are bred for biological and biodiversity conservation. During the years 2003 to 2008 in a zoological garden in northern Italy, 19 white lions were born to the same parents, who were in turn paternally consanguineous. Out of the 19 lions, 4 (21%) were stillborn, 13 (69%) died within 1 month, and 1 (5%) was euthanatized after 6 months because of difficulty with prehension of food. Six lions (32%) showed malformations involving the head (jaw, tongue, throat, teeth, and cranial bones). One lion (5%) still alive at 30 months revealed an Arnold-Chiari malformation upon submission for neurological evaluation of postural and gait abnormalities. Paternal consanguinity of the parents, along with inbreeding among white lions in general, could account for the high incidence of congenital malformations of the head in this pride of white lions. PMID:20826844 Scaglione, F E; Schröder, C; Degiorgi, G; Zeira, O; Bollo, E Brain vascular malformations are recognized as having potential to produce hemorrhage, but leading to sudden death in children is uncommon. Arteriovenous malformations may be situated in any region of the brain, but very rarely, they can be restricted to the choroid plexus. We report here a rare case of sudden death in a child, caused by a ruptured vascular malformation with an unusual location, which was not identified grossly but only on histological examination. The size and the location of the lesion, as well as the age of our patient, were contributing factors of the massive bleeding. Autopsy remains an important tool because it provides valuable information about the etiology of such bleedings, improves knowledge about these lesions, and enhances epidemiologic data. PMID:24781402 Cioca, Andreea; Gheban, Dan; Perju-Dumbrava, Dan; Chiroban, Ovidiu; Mera, Mihaela Human ear has been long proved to be a promising candidate for personal identification. Human ear, with its 3D structure stability and detail expressive, has drawn researchers' attention recently. The posture of ear always changes to the motion of head, it is necessary to normalize an ear to a standard posture to ensure the accuracy of feature extraction. This paper Chao Huang; Guangming Lu; Yahui Liu Researchers have suggested that the ear may have advantages over the face for biometric recognition. Our previous experiments with ear and face recognition, using the standard principal component analysis approach, showed lower recognition performance using ear images. We report results of similar experiments on larger data sets that are more rigorously controlled for relative quality of face and ear images. Kyong I. Chang; Kevin W. Bowyer; Sudeep Sarkar; Barnabas Victor The use of spontaneous and evoked otacoustic emissions is now a standard clinical tool for diagnosis of the function of the inner ear. However, it is not possible to extract this information over the entire, functionally relevant frequency range because of imperfect coupling of: (1) stapedial to ear-drum vibrations through the ossicular chain of the middle ear and (2) ear-drum J. Rodriguez Jorge; Werner Hemmert; C. Burkhardt; Hans-Peter Zenner; Anthony W. Gummer ... Vaccines Hearing Loss and Ear Infection Find an ENT Last Name ZIP Code More Options About Otolaryngology ... United States. Otolaryngologists are commonly referred to as ENT physicians. Learn More Free Download AAO-HNS MarketPlace ... The pathophysiology, differential diagnosis, and currently available management of barotrauma affecting the ears and sinuses after scuba diving are reviewed, along with medical standards for resuming scuba diving after barotrauma has resolved. PMID:11407445 Becker, G D; Parell, G J Size, proportions, level, inclination and shape of the ears were assessed by anthrompometric methods in eight patients with Treacher Collins' syndrome, eleven with Apert's, and 25 with Crouzon's syndrome. L. G. Farkas; Walter Zingg The use of tympanostomy tubes to treat middle ear disease including otitis media is discussed with sections on the eustachian tube; acute otitis media; persistent effusion; changes in the tympanic membrane; special populations; and complications. (DB) Roland, Peter S.; Brown, Orval ... has and if antibiotics would help. Acute otitis media The type of ear infection that is usually ... for AOM, but are not always necessary. Otitis media with effusion Otitis media with effusion (uh-FEW- ... Acoustic communication is widespread in animals. According to the sensory drive hypothesis [Endler JA (1993) Philos Trans R Soc Lond B Biol Sci 340(1292):215-225], communication signals and perceptual systems have coevolved. A clear illustration of this is the evolution of the tetrapod middle ear, adapted to life on land. Here we report the discovery of a bone conduction-mediated stimulation of the ear by wave propagation in Sechellophryne gardineri, one of the world's smallest terrestrial tetrapods, which lacks a middle ear yet produces acoustic signals. Based on X-ray synchrotron holotomography, we measured the biomechanical properties of the otic tissues and modeled the acoustic propagation. Our models show how bone conduction enhanced by the resonating role of the mouth allows these seemingly deaf frogs to communicate effectively without a middle ear. PMID:24003145 Boistel, Renaud; Aubin, Thierry; Cloetens, Peter; Peyrin, Françoise; Scotti, Thierry; Herzog, Philippe; Gerlach, Justin; Pollet, Nicolas; Aubry, Jean-François This study aimed to determine the mean values of the different morphometric measurements from right and left ears. These measurements\\u000a were taken from 341 healthy young adults (150 women and 191 men) ages 18 to 25 years using an electronic digital caliper.\\u000a The results showed the mean values for total ear height, lobular height and width, distances from tragus to M. Gülhal Bozk?r; P?nar Karaka?; Metin Yavuz; Fahri Dere We present a new technique called disjoint decreasing ear paths, which is based on a graph's open ear decomposition. We apply this technique in CRCW PRAM parallel algorithms for the two vertex disjoint s — t paths problem and the maximal path problem in planar graphs. These run in O(log n) time with n + m processors and O(log2\\u000an) Louis Ibarra; Dana S. Richards In order to investigate the sound transmission in the middle ear in detail and the changes brought about by operations, an\\u000a appropriate transducer was developed. Modern techniques in operative treatment of otosclerosis change the acoustic parameters\\u000a of the ear; one of the operations is to penetrate the fixed stapedial footplate with a wire aftached to the incus to transmit\\u000a sound H. Fischler; E. H. Frei; M. Rubinsteins; D. Spira A number of species of anuran amphibians from different regions across North America have recently exhibited an increased occurrence of, predominantly, hind limb malformations. Research concerning factors potentially responsible for these malformations has focused extensively on ... There is significant confusion in the literature when describing vascular anomalies, and vascular malformations are often misnamed or incorrectly classified. Part I of this two-part series on the diagnosis and management of extensive vascular malformations of the lower limbs will discuss the dermatologist's role in the diagnosis of these lesions. At least nine types of vascular malformations with specific clinical and radiologic characteristics must be distinguished in the lower limbs: Klippel-Trénaunay syndrome, port-wine stain with or without hypertrophy, cutis marmorata telangiectatica congenita, macrocephaly-capillary malformation, Parkes Weber syndrome, Stewart-Bluefarb syndrome, venous malformation, glomuvenous malformation, and lymphatic malformation. This article highlights the differences in clinical appearance and discusses the differential diagnosis of extensive vascular malformations in an attempt to ensure earlier diagnosis and better outcomes for these patients. PMID:22000870 Redondo, Pedro; Aguado, Leyre; Martínez-Cuesta, Antonio Congenital abnormalities of the kidneys and the urinary tract are the most common sonographically identified malformations in the prenatal period. Obstructive uropathies account for the majority of cases. The aim of prenatal diagnosis and management is to detect those anomalies having impact on the prognosis of the affected child and requiring early postnatal evaluation or treatment to minimize adverse outcomes. In this paper, we summarize the embryology of kidneys and urinary tract, the normal sonographic appearance throughout pregnancy and the prenatal diagnosis of their congenital malformations. Hindryckx, A.; De Catte, L. Dandy Walker malformation (DWM) is a rare congenital brain anomaly characterized by cystic dilation of the fourth ventricle and hypoplasia of the cerebellar vermis. Other extracranial anomalies can be associated, including cardiac defects. We report a rare patient with DWM associated with progressive heart failure secondary to hypertrophic cardiomyopathy. He was diagnosed at 2 months of age and died 5 months later. We conclude that hypertrophic cardiomyopathy can be associated with DWM with poor prognosis. A careful cardiac evaluation is needed in all infants with DWM for early recognition of such potentially serious associated cardiac malformations. PMID:21048653 Kurdi, Maher E; Chamsi-Pasha, Mohammed A; Baeesa, Saleh S; Jan, Mohammed M Since the first discovery of malformed frogs by an adventurous middle school teacher and her after-school science club in 1995, outreach programs like A Thousand Friends of Frogs (see Resources) have been created to connect students and teachers with scientists so that they can better understand frogs and their habitats. Many of the amphibian-malformation activities published in education-practitioner journals approach this subject through internet investigations (Webster 2002), hypothetical case studies (Murphy and Fortner 2001), or reading with discussion (Davidson, Matthews, and Patrick 2001). These activities can be supplemented with inquiry-based labs designed to instruct and engage students about conservation biology, as described in this article. Talley, Brooke L. Cavernous malformation (CM) is a vascular malformation disorder characterized by a berry-like mass of expanded blood vessels. CM, originating from the optic chiasm. usually leads to chiasma syndrome presenting with bitemporal hemianopsia. We report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans. We investigated the contribution of the middle ear to the physiological response to bone conduction stimuli in chinchilla. We measured intracochlear sound pressure in response to air conduction (AC) and bone conduction (BC) stimuli before and after interruption of the ossicular chain at the incudo-stapedial joint. Interruption of the chain effectively decouples the external and middle ear from the inner ear and significantly reduces the contributions of the outer ear and middle ear to the bone conduction response. With AC stimulation, both the scala vestibuli Psv and scala tympani Pst sound pressures drop by 30 to 40 dB after the interruption. In BC stimulation, Psv decreases after interruption by about 10 to 20 dB, but Pst is little affected. This difference in the sensitivity of the BC induced Psv and Pst to ossicular interruption is not consistent with a BC response to ossicular motion, but instead suggests a significant contribution of an inner-ear drive (e.g. cochlear fluid inertia or compressibility) to the BC response. Chhan, David; Roosli, Christof; McKinnon, Melissa L.; Rosowski, John J. Retinoic acid is a biologically active derivative of vitamin A that is indispensable for inner ear development. The normal function of retinoic acid is achieved only at optimal homeostatic concentrations, with an excess or deficiency in retinoic acid leading to inner ear dysmorphogenesis. We present an overview of the role of retinoic acid in the developing mammalian inner ear, discussing both how and when retinoic acid may act to critically control a program of inner ear development. Molecular mechanisms of otic teratogenicity involving two members of the fibroblast growth factor family, FGF3 and FGF10, and their downstream targets, Dlx5 and Dlx6, are examined under conditions of both retinoic acid excess and deficiency. We term the effect of too little or too much retinoic acid on FGF/Dlx signaling a Goldilocks phenomenon. We demonstrate that in each case (retinoic acid excess, retinoic acid deficiency), retinoic acid can directly affect FGF3/FGF10 signaling within the otic epithelium, leading to downregulated expression of these essential signaling molecules, which in turn, leads to diminution in Dlx5/Dlx6 expression. Non-cell autonomous affects of the otic epithelium subsequently occur, altering transforming growth factor beta (TGF?) expression in the neighboring periotic mesenchyme and serving as a putative explanation for retinoic acid-mediated otic capsule defects. We conclude that retinoic acid coordinates inner ear morphogenesis by controlling an FGF/Dlx signaling cascade, whose perturbation by deviations in local retinoid concentrations can lead to inner ear dysmorphogenesis. Frenz, Dorothy A.; Liu, Wei; Cvekl, Ales; Xie, Qing; Wassef, Lesley; Quadro, Loredana; Niederreither, Karen; Maconochie, Mark; Shanske, Alan Background: Ear cartilage piercing is increasingly popular and has a significant complication rate. Contrary to popular belief, there are no minimum qualifications required to practice ear piercing. This study evaluated ear cartilage piercing practices in London, UK. Method: Practitioners at 25 piercing parlours completed a telephone questionnaire assessing piercing practice. Results: Ninety-six per cent of practitioners were aware of the risk of infection post-piercing. Four per cent, 12 per cent and 0 per cent of practitioners were aware of keloid scarring, hypertrophic scarring and cauliflower ear respectively. No practitioners were aware of any other complications. Their consent forms did not document any ear cartilage complications. Twenty-eight per cent of participants advised clients to seek medical attention following a complication. Forty per cent did not provide written post-piercing guidance. Conclusion: Piercing practitioners were insufficiently aware of ear cartilage piercing complications. It is unlikely that informed consent was obtained prior to piercing. The post-piercing practice of the majority of parlours did not follow published national guidance. PMID:24909453 Mandavia, R; Kapoor, K; Ouyang, J; Osmani, H Background When presented with different sounds in each ear (dichotic listening), healthy subjects typically show a preference for stimuli heard in the right ear, an effect termed “right ear advantage”. Previous studies examining right ear advantage in schizophrenia have been inconsistent, showing either decreased or increased advantage relative to comparison subjects. Given evidence for enhanced semantic processing in schizophrenia, some of this inconsistency may be due to the type of stimuli presented (words or syllables). The present study examined right ear advantage in patients and controls using both words and syllables as stimuli. Methods Right ear advantage was compared between 20 patients with schizophrenia and 17 healthy controls. Two versions of the task were used, ie, a consonant-vowel pairing task and a fused rhymed words task. Results A significant group × task interaction was observed. Relative to healthy controls, patients showed a greater difference on the syllable-based task compared with the word-based task. The number of distractors marked during the syllable-based task was inversely correlated with score on the Global Assessment of Function Scale. Conclusion The findings are consistent with a left hemisphere dysfunction in schizophrenia, but also suggest that differences may be stimulus-specific, with a relative sparing of the deficit in the context of word stimuli. Performance may be related to measures of social, occupational, and psychological function. Smucny, Jason; Wylie, Korey; Tregellas, Jason The middle ear muscle (MEM) reflex is one of two major descending systems to the auditory periphery. There are two middle ear muscles (MEMs): the stapedius and the tensor tympani. In man, the stapedius contracts in response to intense low frequency acoustic stimuli, exerting forces perpendicular to the stapes superstructure, increasing middle ear impedance and attenuating the intensity of sound energy reaching the inner ear (cochlea). The tensor tympani is believed to contract in response to self-generated noise (chewing, swallowing) and nonauditory stimuli. The MEM reflex pathways begin with sound presented to the ear. Transduction of sound occurs in the cochlea, resulting in an action potential that is transmitted along the auditory nerve to the cochlear nucleus in the brainstem (the first relay station for all ascending sound information originating in the ear). Unknown interneurons in the ventral cochlear nucleus project either directly or indirectly to MEM motoneurons located elsewhere in the brainstem. Motoneurons provide efferent innervation to the MEMs. Although the ascending and descending limbs of these reflex pathways have been well characterized, the identity of the reflex interneurons is not known, as are the source of modulatory inputs to these pathways. The aim of this article is to (a) provide an overview of MEM reflex anatomy and physiology, (b) present new data on MEM reflex anatomy and physiology from our laboratory and others, and (c) describe the clinical implications of our research. Mukerji, Sudeep; Windsor, Alanna Marie; Lee, Daniel J. We report a case of malignant melanoma of Eustachian tube with extension to the middle ear cavity and nasopharynx in a 51-year-old woman who presented with right ear fullness. Computed tomography showed a soft tissue mass in the middle ear cavity and causedthe widening and eroding of the bony eustachian tube. Magnetic resonance imaging showed well enhancing mass in eustachian tube extending nasopharynx to middle ear cavity. A biopsy of the middle ear cavity mass revealed a malignant amelanotic melanoma. Kim, Hong Chul; Jang, Han Won The infant mortality from cardiovascular malformations in a region with a population of 2 million inhabitants during a 10-year period has been studied. The study involved validation of the diagnoses and judgement whether the cardiovascular malformation was the dominating or a contributing cause of death. It is shown that the incidence of fatal cardiovascular malformations is probably overestimated in the E Esscher; M Michaëlsson; B Smedby Arteriovenous malformations of the lower urinary tract are uncommon lesions, usually presenting as scrotal masses. A case of recurrent acute scrotal pain mimicking testicular torsion that was attributed to the presence of an arteriovenous malformation of the spermatic cord is described. To our knowledge this is the first reported case of an arteriovenous malformation of the spermatic cord presenting with Petros Sountoulides; Athanasios Bantis; Irene Asouhidou; Hellen Aggelonidou A role for the Notch signalling pathway in the formation of arteriovenous malformations during development has been suggested. However, whether Notch signalling is involved in brain arteriovenous malformations in humans remains unclear. Here, we performed immunohistochemistry on surgically resected brain arteriovenous malformations and found that,… ZhuGe, Qichuan; Zhong, Ming; Zheng, WeiMing; Yang, Guo-Yuan; Mao, XiaoOu; Xie, Lin; Chen, Gourong; Chen, Yongmei; Lawton, Michael T.; Young, William L.; Greenberg, David A.; Jin, Kunlin Chiari malformation type 1 (CM1) is a developmental abnormality of the cerebellar tonsils. Patients with CM1 commonly present with headache. Papilloedema is rarely seen in CM1. However, a 52-year-old woman presented to the hospital with a headache and her ophthalmological examination revealed bilateral papilloedema. Her cranial MRI was compatible with borderline CM1. Bilateral papilloedema and headache suggested idiopathic intracranial hypertension (IIH) as the preliminary diagnosis. IIH is a rare case in CM1. This article argues about this association and discusses as to whether it is an acquired or congenital Chiari malformation. PMID:24898995 It has been reported that the physiological motion of the stapes in human and several animals in response to acoustic stimulation is mainly piston-like at low frequencies. At higher frequencies, the pattern includes rocking motions around the long and short axes of the footplate in human and animal ears. Measurements of such extended stapes motions are highly sensitive to the exact angulation of the stapes in relation to the measurement devices and to measurement errors. In this study, velocity in a specific direction was measured at multiple points on the footplates of human temporal bones using a Scanning Laser Doppler Vibrometer (SLDV) system, and the elementary components of the stapes motions, which were the piston-like motion and the rocking motions about the short and long axes of the footplate, were calculated from the measurements. The angular position of a laser beam with respect to the stapes and coordinates of the measurement points on the footplate plane were calculated by correlation between the SLDV measurement frame and the footplate-fixed frame, which was obtained from micro-CT images. The ratios of the rocking motions relative to the piston-like motion increased with frequency and reached a maximum around 7 kHz.A novel method for quantitatively assessing measurements of complex stapes motions and error boundaries of the motion components is presented. In the frequency range of 0.5 to 8 kHz, the magnitudes of the piston-like and two rocking motions were larger than estimated values of the corresponding upper error bounds. PMID:20165895 Sim, Jae Hoon; Chatzimichalis, Michail; Lauxmann, Michael; Röösli, Christof; Eiber, Albrecht; Huber, Alexander M Polymicrogyria (PMG) is one of the most common malformations of cortical development. It is characterized by overfolding of the cerebral cortex and abnormal cortical layering. It is a highly heterogeneous malformation with variable clinical and imaging features, pathological findings, and etiologies. It may occur as an isolated cortical malformation, or in association with other malformations within the brain or body as part of a multiple congenital anomaly syndrome. Polymicrogyria shows variable topographic patterns with the bilateral perisylvian pattern being most common. Schizencephaly is a subtype of PMG in which the overfolded cortex lines full-thickness clefts connecting the subarachnoid space with the cerebral ventricles. Both genetic and non-genetic causes of PMG have been identified. Non-genetic causes include congenital cytomegalovirus infection and in utero ischemia. Genetic causes include metabolic conditions such as peroxisomal disorders and the 22q11.2 and 1p36 continguous gene deletion syndromes. Mutations in over 30 genes have been found in association with PMG, especially mutations in the tubulin family of genes. Mutations in the (PI3K)-AKT pathway have been found in association PMG and megalencephaly. Despite recent genetic advances, the mechanisms by which polymicrogyric cortex forms and causes of the majority of cases remain unknown, making diagnostic and prenatal testing and genetic counseling challenging. This review summarizes the clinical, imaging, pathologic, and etiologic features of PMG, highlighting recent genetic advances. © 2014 Wiley Periodicals, Inc. PMID:24888723 Stutterd, Chloe A; Leventer, Richard J When the size of an arteriovenous malformation (AVM) in any one direction is more than 4 cm, an attempt is made to treat it in two stages, separated by 6 months. The first stage treats one-half of the AVM and the remainder is treated in the second stage, thus giving rise to a higher prescription dose for each stage. Therefore, Hideo D. Kubo; Richard B. Wilder; Conrad T. Pappas; John D. Earle; Robin L. Stern C57BL/6N mice, used to model induction of cleft palate and kidney malformations in offspring following maternal treatment with TCDD, were dosed on gestation day (gd) 9 with ,2',4,4',5,5'-hexachloro-biphenyl (HCB) (62.5, 125, 250, 500. 1000 mg/kg) and/or gd 10 with 2,3,7,8-tetrach... In order to evaluate the teratogenic potential of acetazolamide in rabbits, three groups of 18 artificially inseminated females were treated orally with 50, 100, or 150 mg/kg/day of acetazolamide on days 6-18 of gestation. These doses induced maternal acidosis and electrolyte changes, consistent with those reported in rats and considered to be a result of carbonic anhydrase inhibition, as well as reductions in maternal body weight gain. At cesarean sections, average fetal body weights in the acetazolamide groups were dose-dependently decreased compared with controls. There were no effects of acetazolamide on embryonic survival or external morphology of live fetuses. In the fetal skeletal examination, thoracic and lumbar vertebral malformations occurred in 0.7%, 3.9%, and 6.1% of fetuses in the 50, 100, and 150 mg/kg/day groups, respectively, compared with none in the control group. In addition, missing vertebra was seen in a small number of fetuses in the 100 and 150 mg/kg/day groups. These axial skeletal malformations were, in some cases, associated with costal malformations. These results indicate that acetazolamide at maternotoxic doses can produce axial skeletal malformations in the rabbit. PMID:1412055 Nakatsuka, T; Komatsu, T; Fujii, T The vertebrate limb is a powerful model system for studying the cellular and molecular interactions that determine morphological pattern during embryonic development. Recent advances in our understanding of these interactions have shed new light on the molecular mechanisms of vertebrate limb development, evolution and congenital malformations. The transfer of information has, until recently, been largely one way, with developmental studies Martin J. Cohn; Philippa E. Bright Sonography is the method of choice for prenatal malformation screening but it does not always provide sufficient information for correct diagnosis or adequate abnormality evaluation. Fetal magnetic resonance imaging (MRI) is considered as a valuable second line imaging tool for confirmation, completion and correction of sonographic findings. Fetal MRI has proven its value in the evaluation of central nervous system Izabela Herman-Sucharska; Monika Bekiesi?ska-Figatowska; Andrzej Urbanik The cause of limb deformities in wild amphibian populations remains unclear, even though the apparent increase in prevalence of this condition may have implications for human health. Few studies have simultaneously assessed the effect of multiple exposures on the risk of limb deformities. In a cross-sectional survey of 5,264 hylid and ranid metamorphs in 42 Vermont wetlands, we assessed independent risk factors for nontraumatic limb malformation. The rate of nontraumatic limb malformation varied by location from 0 to 10.2%. Analysis of a subsample did not demonstrate any evidence of infection with the parasite Ribeiroia. We used geographic information system (GIS) land-use/land-cover data to validate field observations of land use in the proximity of study wetlands. In a multiple logistic regression model that included land use as well as developmental stage, genus, and water-quality measures, proximity to agricultural land use was associated with an increased risk of limb malformation (odds ratio = 2.26; 95% confidence interval, 1.42–3.58; p < 0.001). The overall discriminant power of the statistical model was high (C = 0.79). These findings from one of the largest systematic surveys to date provide support for the role of chemical toxicants in the development of amphibian limb malformation and demonstrate the value of an epidemiologic approach to this problem. Taylor, Brynn; Skelly, David; Demarchis, Livia K.; Slade, Martin D.; Galusha, Deron; Rabinowitz, Peter M. Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM. PMID:23524491 Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F Public concern exists about the potential for reproductive damage that may result from exposures to environmental contaminants. Therefore, the authors sought to determine if there was an association between a child's congenital malformation or a child's lowered weight at birth and his or her mother's residence in a census tract where a site of environmental contamination had been documented. Exposure G. M. Shaw; J. Schulman; J. D. Frisch; J. A. Harris; S. K. Cummins Public concern exists about the potential for reproductive damage that may result from exposures to environmental contaminants. Therefore, the authors sought to determine if there was an association between a child's congenital malformation or a child's lowered weight at birth and his or her mother's residence in a census tract where a site of environmental contamination had been documented. Exposure Gary M. Shaw; Jane Schulman; Jonathan D. Frisch; Susan K. Cummins; John A. Harris A study of 2620 pregnancies ending in spontaneous abortion revealed a CNS defect in 3.6% of embryos and fetuses, and 3% of all complete conceptuses. The type of malformation observed varied with the gestational age at expulsion, encephaloceles being predominant in earlier specimens, while more typical anencephalus and spina bifida were more common among later abortions. Chromosome abnormalities were found M R Creasy; E D Alberman Pathological manifestations in fishes from the polluted environment reflect the deleterious effects of environmental damage to higher animals in the food chain including man. Industrial and mining wastes containing cadmium ions induced several abnormalities and metabolic disorders in aquatic animals. Though cadmium is reported to be toxic to all tissues of animals, cadmium-induced malformations in eyes of fish have not V. Pragatheeswaran; B. Loganathan; R. Natarajan; V. K. Venugopalan We describe six cases from three unrelated consanguineous Egyptian families with a novel characteristic brain malformation at the level of the diencephalic-mesencephalic junction. Brain magnetic resonance imaging demonstrated a dysplasia of the diencephalic-mesencephalic junction with a characteristic "butterfly"-like contour of the midbrain on… Zaki, Maha S.; Saleem, Sahar N.; Dobyns, William B.; Barkovich, A. James; Bartsch, Hauke; Dale, Anders M.; Ashtari, Manzar; Akizu, Naiara; Gleeson, Joseph G.; Grijalvo-Perez, Ana Maria Summary An 11-year-old girl was operated on for a frontal arteriovenous malformation in 1978; a postoperative carotid angiogram showed no filling of the AVM. The patient still had epilepsy. In 1981 a carotid angiogram showed no AVM. Some months later a new carotid angiography was done. In the first lateral series no AVM was seen but on the second injection M. Porras; A. Servo; J. Jääskinen Uterine arteriovenous malformation (AVM) is an uncommon problem and traditional treatment by hysterectomy excludes the possibility of future pregnancy. Developments in interventional techniques make transcatheter embolization of the feeding vessel(s) a therapeutic alternative, potentially preserving the patient's fertility. We present a case of successful endovascular treatment of uterine AVM. Rangarajan, R. D.; Moloney, J. C.; Anderson, H. J. [Eastbourne District General Hospital, Department of Radiology (United Kingdom)], E-mail: [email protected] Anorectal malformation (ARM) can be divided in high, intermediate, and low forms according to the level of termination of the rectum in relation to the pubococcygeal and ischiatic lines. Patients with Down's syndrome have a high incidence of gastrointestinal anomalies, such as tracheoesophageal fistula, duodenal obstruction, annular pancreas, Hirschsprung's disease, and ARM. In these children, ARM is generally low with Anthony S. de Buys Roessingh; Claudia Mueller; Chad Wiesenauer; Arié L. Bensoussan; Mona Beaunoyer An adult male of Pachycheles serratus with a malformation on the right cheliped was found during a collection of anomuran crabs in coastal waters of the peninsula de Macanao, Margarita island, Venezuela. The specimen was found at La Carmela beach (11 degrees 04'N-64 degrees 20'W), and featured a bifurcated fixed finger on the right cheliped. PMID:15264565 Lira, C; Hernández, G; Bolaños, J A A midline exploratory laparotomy on a 1-year-old, neutered male, West Highland terrier with a history of lethargy, anorexia, and intermittent vomiting revealed a cranial abdominal cyst. The lining of the excised cyst was histologically identical with that of the small intestine and may have represented an uncommon intestinal malformation. Cooper, Johanna C. Background: Congenital cystic adenomatoid malformation of the lung (CCAM) is an embryonic developmental anomaly of an unknown etiology usually diagnosed antenatally by imaging techniques. A minority of cases may not be identified by prenatal imaging techniques and may go unnoticed for the first 6 months of their extrauterine life. Due to its rarity, physicians are unlikely to suspect the condition. Manel Luján; Montserrat Bosque; Rosa M. Mirapeix; M. Teresa Marco; Oscar Asensio; Christian Domingo The Northern Prairie Wildlife Research Center (NPWRC) has posted several more resources online. This article introduces readers to The North American Reporting Center for Amphibian Malformations and discusses availability and uses of the Center's databases and resources. The resource may be browsed online or downloaded as a .zip file. Fowle, Suzanne C.; Johnson, Douglas H.; Jundt, Jeffrey A. While congenital malformations of the genital tract are not common, the sequelae of their presence can be serious. The practising gynaecologist must be aware of the range of congenital abnormalities that may occur and the symptoms that may result from them. Failure to manage these patients correctly may have long-term sequelae for their psychological, sexual and reproductive health. The involvement D. Keith Edmonds Seven subjects with Chiari I malformations and seizures (four males, three females; age range 11 years, 7 months to 36 years; mean, 22.28 ± 7.58 years; median, 21) were identified in four different centers from among a group of 10 patients. Our aim was to analyze clinical and electroencephalographic characteristics of seizures in this etiologically homogeneous group of patients. Most Maurizio Elia; Roberto Biondi; Vito Sofia; Sebastiano A. Musumeci; Raffaele Ferri; Giuseppe Capovilla; Paolo Curatolo Purely extradural lumbar spinal arteriovenous malformations (AVMs) are rare lesions that have diverse presentations and imaging features. The treatment of a symptomatic high flow paraspinal AVM with multiple feeders remains a challenge. We report the first use of an Ascent balloon (dual lumen balloon catheter) to deliver Onyx with excellent penetration to a paraspinal AVM. PMID:23543731 Martínez-Galdámez, Mario; Rodriguez-Arias, Carlos A; Utiel, Elena; Arreba, Emilio; Gonzalo, Miguel; Arenillas, Juan F From an observation of in utero suspected myelomeningocele, the authors underline interest of pattern of cholinesterases using acrylamide gel electrophoresis. The AChE isoenzyme band appears in some fetal malformations, particularly NTD. This biochemical test is considered as complementary of the AF alpha-fetoprotein assay. PMID:7276919 Guibaud, S; Simplot, A; Bonnet, M; Fara, J F; Thoulon, J M; Dumont, M; Guibaud, P; Robert, J M Colobomatous cyst of the orbit is a rare congenital cystic malformation associated with ocular maldevelopment. Usually, the cyst is associated with a microphthalmic globe. We present a rare case of a unilateral large colobomatous cyst associated with a normal-sized globe, giving the appearance of a double globe on imaging. Kim, Usha R; Arora, Vipul; Shah, Akash D; Srinivasan, KG Uterine arteriovenous malformation (AVM) is rare but potentially life-threatening from excessive vaginal bleeding. All uterine AVMs reported to date have been found in the endometrial or myometrial layers. Here we present a patient with a subserosal type AVM on the fundus of uterus, which spontaneously ruptured. Seo, Kyung Jin; Kim, Jin; Sohn, In Sook; Kwon, Han Sung; Park, Sang Woo Abstract ,Genital malformations constitute the most common,birth defects encountered in man,and domes- tic animals. They occur more ,frequently in genetic males since the participation of many genes is required for sex differentiation to proceed in the male direction. The emerging insight, through the identification of genes involved in the sex differentiation cascade, is that over 85 percent of sex anomalies P. K. Basrur; V. R. Basrur Background: We present our experience over a 10-year period of staged radiosurgery for large arteriovenous malformations (AVMs) including patient outcomes and methods. Methods: From July 2000 to December 2010, 80 patients with AVMs were treated with gamma knife radiosurgery (GKS) at our institution; of these patients, 5 were treated for large AVMs with staged GKS (volumes >20 cm3). The mean Kwame Amponsah; Thomas L. Ellis; Michael D. Chan; J. Daniel Bourland; Stephen S. Glazier; Kevin P. McMullen; Edward G. Shaw; Stephen B. Tatter BACKGROUND AND PURPOSE: Conventional catheter angiography (CCA) is the current reference standard for the diagnosis, assessment, and management of pial brain arteriovenous malformations (AVMs). The purpose of this study was to develop an MR angiographic tech- nique that produces dynamic images comparable to those provided by CCA and to apply the technique to the investigation of pial brain AVMs. METHODS: Paul D. Griffiths; Nigel Hoggard; Daniel J. Warren; Iain D. Wilkinson; Bob Anderson; Charles A. Romanowski Acquired cerebellar lesions in adults and children can lead to the development of a complex behavioural pattern termed 'Cerebellar Cognitive Affective Syndrome' (Schmahmann and Sherman, Brain, 1998; 121: 561-79), which is characterized by reduced cognitive efficiency associated with specific neuropsychological deficits (executive and visuospatial disorders), expressive language disorders (mild agrammatism and anomia) and affective disorders with blunting of affect. It is not known whether a symptomatological picture such as this can also be found in congenital cerebellar malformations. We studied the behavioural developmental profile of 27 patients including children and adults with congenital malformations confined to the cerebellum, the largest studied sample to date. Extensive clinical and neuropsychological investigations highlight the presence of a wide range of disorders supporting the important role played by the cerebellum in the acquisition of higher-order cognitive and affective skills. The type and extent of cerebral reorganization processes in the presence of malformative lesions are difficult to predict and may possibly account for the variability of clinical phenotypes. It is, therefore, more difficult to identify a syndromic picture defined as exactly as is the case with acquired lesions. However, the pattern of deficits that we document is in remarkable agreement with the general profile of the Cerebellar Cognitive Affective Syndrome. Malformations affecting the cerebellar vermis induce affective and social disorders and evolve towards more unfavourable pictures often associated with an autistic symptomatology. Malformations of cerebellar hemispheres are more frequently associated with selective neuropsychological deficits involving mainly executive functions and visuospatial and linguistic abilities. Motor deficits are generally less severe, and tend to improve slowly and progressively, in some cases reaching almost complete functionality. Finally, the overall favourable evolution with an onset of skills in advanced age in a consistent subset of subjects suggests that individual follow-ups should be performed in order to monitor the quality and stability of impairments and acquired abilities over time. PMID:17872929 Tavano, Alessandro; Grasso, Rita; Gagliardi, Chiara; Triulzi, Fabio; Bresolin, Nereo; Fabbro, Franco; Borgatti, Renato The underlying mutations that cause human limb malformations are often difficult to determine, particularly for limb malformations that occur as isolated traits. Evidence from a variety of studies shows that cis-regulatory mutations, specifically in enhancers, can lead to some of these isolated limb malformations. Here, we provide a review of human limb malformations that have been shown to be caused by enhancer mutations and propose that cis-regulatory mutations will continue to be identified as the cause of additional human malformations as our understanding of regulatory sequences improves. VanderMeer, Julia E.; Ahituv, Nadav Congenital pulmonary malformations represent a heterogeneous group of developmental disorders affecting the lung parenchyma, the arterial supply to the lung, and the lung's venous drainage. In both asymptomatic and symptomatic pediatric patients with congenital pulmonary malformations, the diagnosis of such malformations usually requires imaging evaluation, particularly in cases of surgical lesions for preoperative assessment. The goal of this article is to review the current imaging techniques for evaluating congenital pulmonary malformations and their characteristic imaging findings, which can allow differentiation among various congenital pulmonary malformations in pediatric patients. PMID:21889015 Lee, Edward Y; Dorkin, Henry; Vargas, Sara O The pathogenesis of Chiari malformation Type I (CM-I) and associated syringomyelia is incompletely understood. Patients often present in middle age with incidental or minimally symptomatic CM-I, whose management is controversial. One option is clinical and radiographic observation of asymptomatic and minimally symptomatic patients. The authors here present the case of a 36-year-old woman who had been monitored for 6 years for a minimally symptomatic CM-I and cervicothoracic syrinx. After 5 years of follow-up, she suffered spontaneous rupture of a cerebral cavernous malformation when she was 27 weeks pregnant. The ruptured cavernous malformation and hematoma were operatively managed via a right frontal craniotomy. Ten months after the craniotomy for resection of the ruptured cavernous malformation, follow-up MRI demonstrated resolution of the CM-I and syrinx. Few similar cases have been reported in adults. This case argues for the presence of dynamic factors in the development and maintenance of CM-I and supports the nonoperative treatment of asymptomatic and minimally symptomatic patients. PMID:22324421 Miele, William R; Schirmer, Clemens M; Yao, Kevin C; Heilman, Carl B In this paper the problem of human ear detection in the thermal infrared (IR) spectrum is studied in order to illustrate the advantages and limitations of the most important steps of ear-based biometrics that can operate in day and night time environments. The main contributions of this work are two-fold: First, a dual-band database is assembled that consists of visible and thermal profile face images. The thermal data was collected using a high definition middle-wave infrared (3-5 microns) camera that is capable of acquiring thermal imprints of human skin. Second, a fully automated, thermal imaging based ear detection method is developed for real-time segmentation of human ears in either day or night time environments. The proposed method is based on Haar features forming a cascaded AdaBoost classifier (our modified version of the original Viola-Jones approach1 that was designed to be applied mainly in visible band images). The main advantage of the proposed method, applied on our profile face image data set collected in the thermal-band, is that it is designed to reduce the learning time required by the original Viola-Jones method from several weeks to several hours. Unlike other approaches reported in the literature, which have been tested but not designed to operate in the thermal band, our method yields a high detection accuracy that reaches ~ 91.5%. Further analysis on our data set yielded that: (a) photometric normalization techniques do not directly improve ear detection performance. However, when using a certain photometric normalization technique (CLAHE) on falsely detected images, the detection rate improved by ~ 4%; (b) the high detection accuracy of our method did not degrade when we lowered down the original spatial resolution of thermal ear images. For example, even after using one third of the original spatial resolution (i.e. ~ 20% of the original computational time) of the thermal profile face images, the high ear detection accuracy of our method remained unaffected. This resulted also in speeding up the detection time of an ear image from 265 to 17 milliseconds per image. To the best of our knowledge this is the first time that the problem of human ear detection in the thermal band is being investigated in the open literature. Abaza, Ayman; Bourlai, Thirimachos The finite element (FE) model of the human ear has been developed to analyze the middle ear and cochlea function in relation to the ear structures. However, the energy absorbance or energy reflectance used in the research and clinical audiology test has not been reported in the FE model. The relationship between the middle ear structure and the energy absorbance (EA) needs to be identified using the FE model. In this study, a FE model of the human ear, including the ear canal, the middle ear and the spiral cochlea constructed from the histological sections of a human temporal bone, was used to calculate EA. The viscoelastic material properties were applied to the middle ear soft tissues. Three middle ear disorders were simulated in the FE model: otitis media, otosclerosis, and ossicular chain disarticulation. Multi-physics (acoustic, structure, and fluid) coupled analysis was conducted in the model. The FE model was first validated with the published experimental data on the middle ear input impedance and EA of the normal ear. The EA in three disordered ears was obtained from the model and compared with the published results measured in the clinics and the temporal bone experiments. The consistence of the model-derived EA with the published data demonstrates that the FE model is feasible to analyze EA. The effects of middle ear pressure, middle ear effusion, and mechanical properties of soft tissues on EA were estimated and discussed. This article is part of a special issue entitled "MEMRO 2012". PMID:23274858 Zhang, Xiangming; Gan, Rong Z Objective: A chronic arteriovenous malformation (AVM) model using the swine retia mirabilia (RMB) was developed and compared with the human extracranial AVM (EAVM) both in hemodynamics and pathology, to see if this brain AVM model can be used as an EAVM model. Methods: We created an arteriovenous fistula between the common carotid artery and the external jugular vein in eight animals by using end-to-end anastomosis. All animals were sacrificed 1 month after surgery, and the bilateral retia were obtained at autopsy and performed hematoxylin and eosin staining and immunohistochemistry. Pre- and postsurgical hemodynamic evaluations also were conducted. Then, the blood flow and histological changes of the animal model were compared with human EAVM. Results: The angiography after operation showed that the blood flow, like human EAVM, flowed from the feeding artery, via the nidus, drained to the draining vein. Microscopic examination showed dilated lumina and disrupted internal elastic lamina in both RMB of model and nidus of human EAVM, but the thickness of vessel wall had significant difference. Immunohistochemical reactivity for smooth muscle actin, angiopoietin 1, and angiopoietin 2 were similar in chronic model nidus microvessels and human EAVM, whereas vascular endothelial growth factor was significant difference between human EAVM and RMB of model. Conclusions: The AVM model described here is similar to human EAVM in hemodynamics and immunohistochemical features, but there are still some differences in anatomy and pathogenetic mechanism. Further study is needed to evaluate the applicability and efficacy of this model. Lv, Ming-ming, E-mail: [email protected] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China); Fan, Xin-dong, E-mail: [email protected] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Radiology (China); Su, Li-xin, E-mail: [email protected] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China)] [Ninth People's Hospital, Shanghai Jiao Tong University School of Medicine, Department of Oral and Maxillofacial Surgery, Shanghai Key Laboratory of Stomatology (China) Deep drawing of a cylindrical cup from a rolled sheet is one of the typical forming operations where the effect of this anisotropy is most evident. Indeed, it is well documented in the literature that the number of ears and the shape of the earing pattern correlate with the r-values profile. For the strongly textured aluminum alloy AA 5042 (Numisheet Benchmark 2011), the experimental r-value distribution has two minima between the rolling and transverse direction data provided for this show that the r-value along the transverse direction (TD) is five times larger than the value corresponding to the rolling direction. Therefore, it is expected that there are more that the earing profile has more than four ears. The main objective of this paper is to assess whether a new form of CPB06ex2 yield function (Plunkett et al. (2008)) tailored for metals with no tension-compression asymmetry is capable of predicting more than four ears for this material. Yoon, J. H.; Cazacu, O. The aim of our investigations was to characterize fungal colonization of the ear in immunocompetent patients. From 1993 to 2000, 128 patients supposed to suffer from otomycosis were included. Mycological examination conducted by direct microscopy and fungal cultures was performed on 139 specimens. Among these, 115 patients suffered from chronic otitis media with persisting tympanum perforation and otorrhea. A further 13 patients had clinical signs of an otitis externa only. Out of 139 samples, fungi were identified in the auditory canal (n = 54), on the tympanic membrane (n = 5), and in the middle ear (n = 5). Two-thirds were as moulds and one-third yeasts. The dominating species were Aspergillus niger and Candida parapsilosis. Samples from 15 patients supposed to have mastoiditis or cholesteatoma were examined histologically. Fungal hyphae were observed in the middle ear cavity and/or between horny lamellae of cholesteatoma in four patients. In the middle ear of immunocompetent patients chronic-hyperplastic (polypoid) inflammation was detected with increased production of mucus, which probably promotes colonization by pathogenic fungi in the middle ear as well as in the auditory canal. Invasive fungal growth into the subepithelial connective tissue was not observed. PMID:12588477 Vennewald, I; Schönlebe, J; Klemm, E The external ear is a location with high risk of keloid scar formation. Its incidence is growing since general use of piercings and performance of plastic surgery of the external ear. The external ear keloid can be a devasting process for adolescent population which is worried about their appearance. Our aim is to attract attention about the risk of keloid scars of the external ear, reviewing our experience. After dismissing radiotherapy, corticoid infiltration and surgical removal are the most used options, with a high recurrence risk. We have reviewed traumatic, surgical and piercing wounds of the external ear, with a subsequent keloid formation treated in our outpatient clinic, collecting data about wound etiology, treatment and results. During the last 10 years we have found 11 keloid scars, 2 of them improved with topical corticosteroid. Treatment has been surgical in 9 cases, 4 of them with skin graft: 5 recovered and 4 recurred; 2 of them were reoperated. 2 of them were treated with intralesional corticosteroid solely, one recovered and the other one had improved. Treatment management of keloid scars is complex and there isn't a procedure with superior results than the others. Risk of complication must be explained within adolescent population. PMID:24783642 Bejarano Serrano, M; Parri Ferrandis, F J; García Smith, N I; Martínez-Herrada, S; Manzanares Quintela, A; Albert Cazalla, A Investigations for co-occurring malformations can serve as a first step to discover new risk factors and provide insights into malformation etiologies. Our objective was to explore the risks of occurrence of structural congenital malformation phenotypes in 5,481 infants with hypospadias, using data from a population-based active surveillance system. Ascertainment was performed among 1,816,258 liveborn and 12,203 stillborn male offspring of women who were residents of registry counties during 1983-1997. Malformations other than hypospadias were grouped according to the 3- and 4-digit BPA malformation codes. Prevalences of each malformation grouping were calculated among all births with hypospadias and their three subgroups and all births without hypospadias but with another structural malformation. Relative risks were estimated based on the ratio of two prevalences, and the corresponding 95% confidence intervals were computed using Poisson regression models. Observed relative risks indicated that all malformation groupings were less likely to co-occur with hypospadias and their three subgroups than with other types of malformations, with relative risks ranging from 0.04 to 0.93. For most malformations, risks were relatively similar among all births with hypospadias and the three subgroups of hypospadias. The observed relative risks were not substantially changed in analyses that adjusted for maternal age, race/ethnicity, and parity. Computations were extended to 4-digit level BPA codes. Almost all observed relative risks for malformations co-occurring with hypospadias overall and three subgroups were less than 1. PMID:17937439 Yang, Wei; Carmichael, Suzan L; Shaw, Gary M This report describes relationships between middle-ear measurements of acoustic admittance and energy reflectance (YR) and measurements of hearing status using visual reinforcement audiometry in a neonatal hearing-screening population. Analyses were performed on 2638 ears in which combined measurements were obtained [Norton et al., Ear Hear. 21, 348-356 (2000)]. The measurements included distortion-product otoacoustic emissions (DPOAE), transient evoked otoacoustic emissions (TEOAE), and auditory brainstem responses (ABR). Models to predict hearing status using DPOAEs, TEOAEs, or ABRs were each improved by the addition of the YR factors as interactions, in which factors were calculated using factor loadings from Keefe et al. [J. Acoust. Soc. Am. 113, 389-406 (2003)]. This result suggests that information on middle-ear status improves the ability to predict hearing status. The YR factors were used to construct a middle-ear dysfunction test on 1027 normal-hearing ears in which DPOAE and TEOAE responses were either both present or both absent, the latter condition being viewed as indicative of middle-ear dysfunction. The middle-ear dysfunction test classified these ears with a nonparametric area (A) under the relative operating characteristic curve of A=0.86, and classified normal-hearing ears that failed two-stage hearing-screening tests with areas A=0.84 for DPOAE/ABR, and A=0.81 for TEOAE/ABR tests. The middle-ear dysfunction test adequately generalized to a new sample population (A=0.82). Keefe, Douglas H.; Gorga, Michael P.; Neely, Stephen T.; Zhao, Fei; Vohr, Betty R. Vertebrate hearing and balance are based in complex asymmetries of inner ear structure. Here, we identify retinoic acid (RA) as an extrinsic signal that acts directly on the ear rudiment to affect its compartmentalization along the anterior-posterior axis. A rostrocaudal wave of RA activity, generated by tissues surrounding the nascent ear, induces distinct responses from anterior and posterior halves of the inner ear rudiment. Prolonged response to RA by posterior otic tissue correlates with Tbx1 transcription and formation of mostly nonsensory inner ear structures. By contrast, anterior otic tissue displays only a brief response to RA and forms neuronal elements and most sensory structures of the inner ear. PMID:21173260 Bok, Jinwoong; Raft, Steven; Kong, Kyoung-Ah; Koo, Soo Kyung; Dräger, Ursula C; Wu, Doris K The pertinent clinical literature concerning the research project is reviewed. The middle ear of guinea pigs was first damaged on one side (inflicting a subtotal perforation of the tympanic membrane, dislocation of the incudo-stapedial joint followed by otitis media) and the animals were then exposed to a specified narrow-band noise. The induced inner ear changes were quantified by means of cochleographic studies using the surface specimen technique. In spite of the inner ear changes due to middle ear manipulation prior to the noise exposure, the cochleographic studies revealed an obvious protective effect of the induced middle ear changes upon the inner ear during noise exposure. PMID:138031 A case study of clinical practice in children with glue ear is presented. The case is part of a larger project, funded by the North Thames Research and Development Programme, that sought to explore the part played by clinicians in the implementation of research and development into practice in two areas: adult asthma and glue ear in children. What is striking about this case is the differences found in every area of the analysis. That is, diversity was found in views about diagnosis and treatment of glue ear; the organisation of related services; and in the reported practice of our interviewees, both between particular groupings of clinical staff and within these groupings. The challenge inherent in the case is to go beyond describing the complexity and differences that were found, and look for patterns in the accounts of practice and tease out why such patterns may occur. PMID:10557674 Dopson, S; Miller, R; Dawson, S; Sutherland, K Blood pressure (BP) measurement and BP control are important for the prevention of lifestyle diseases, especially hypertension, which can lead to more serious conditions, such as cardiac infarction and cerebral apoplexy. The purpose of our study is to develop a ubiquitous blood pressure sensor that is more comfortable and less disruptive of users’ daily activities than conventional blood pressure sensors. Our developed sensor is worn at an ear orifice and measures blood pressure at the tragus. This paper describes the concept, configuration, and the optical and electronic details of the developed ear-worn blood pressure sensor and presents preliminary evaluation results. The developed sensor causes almost no discomfort and produces signals whose quality is high enough for detecting BP at an ear, making it suitable for ubiquitous usage. Koizumi, Hiroshi; Shimada, Junichi; Uenishi, Yuji; Tochikubo, Osamu To realize the ubiquitous eating habits monitoring, we proposed the use of sounds sensed by an in-ear placed wireless wearable microphone. A prototype of wireless wearable in-ear microphone was developed by utilizing a common Bluetooth headset. We proposed a robust chewing action recognition algorithm which consists of two recognition stages: “chew-like” signal detection and chewing sound verification stages. We also provide empirical results on other action recognition using in-ear sound including swallowing, cough, belch, and etc. The average chewing number counting error rate of 1.93% is achieved. Lastly, chewing sound mapping is proposed as a new prototypical approach to provide an additional intuitive feedback on food groups to be able to infer the eating habits in their daily life context. Nishimura, Jun; Kuroda, Tadahiro In this article, a beamformer is proposed as a functional model for the spatial and temporal filtering characteristics of the external ear. The output of a beamformer is a weighted combination of the data received at an array of spatially distributed sensors. The beamformer weights and array geometry determine its spatial and temporal filtering characteristics. A procedure is described for choosing the weights to minimize the mean-squared error between the beamformer response and the measured response of the external ear. The effectiveness of the model is demonstrated by designing a beamformer of several hundred weights that duplicates and interpolates the measured external ear response of a cat over broad ranges of frequency and direction. A limited investigation of modeling performance as a function of array geometry is reported. PMID:1401538 Chen, J; Van Veen, B D; Hecox, K E Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, laser vibrometry requires free visual access to the object under investigation, and acquiring free visual access to the ossicles through the ear canal requires the removal of the tympanic membrane (TM), with the result that the ossicles can no longer be stimulated acoustically. To overcome this, we devised a new setup in which the ossicles can be driven magnetically. After measuring the response of the TM to an acoustic signal, we then remove it and attach a small magnet to the exposed manubrium (a part of the most lateral auditory ossicle, the malleus, which is normally attached to the TM). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a setup may have uses in research on middle ear mechanics, such as the measurement of nonlinearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis or in chronic otitis media. We describe our setup and discuss the viability of our method and its future clinical potential by presenting some measurements on an artificially fixated ear. Peacock, J.; von Unge, M.; Dirckx, J. Vibrations of the middle ear ossicles are easily measured by means of laser vibrometry. However, laser vibrometry requires free visual access to the object under investigation, and acquiring free visual access to the ossicles through the ear canal requires the removal of the tympanic membrane (TM), with the result that the ossicles can no longer be stimulated acoustically. To overcome this, we devised a new setup in which the ossicles can be driven magnetically. After measuring the response of the TM to an acoustic signal, we then remove it and attach a small magnet to the exposed manubrium (a part of the most lateral auditory ossicle, the malleus, which is normally attached to the TM). An electromagnetic excitation coil is then used to drive the magnet, and the output to the coil adjusted until the vibration of the manubrium, as measured by the vibrometer, matches that measured in response to the acoustic signal. Such a setup may have uses in research on middle ear mechanics, such as the measurement of nonlinearities in their response, as well as applications in the diagnosis of middle ear conditions such as the fixation of the ossicles by otosclerosis or in chronic otitis media. We describe our setup and discuss the viability of our method and its future clinical potential by presenting some measurements on an artificially fixated ear. PMID:24387412 Peacock, J; von Unge, M; Dirckx, J Epizootic outbreaks of congenital malformations in sheep are rare and have, to the best of our knowledge, never been reported before in Europe. This paper describes relevant preliminary findings from the first epizootic outbreak of ovine congenital malformations in the Netherlands. Between 25 November and 20 December 2011, congenital malformations in newborn lambs on sheep farms throughout the country were reported to the Animal Health Service in Deventer. Subsequently, small ruminant veterinary specialists visited these farms and collected relevant information from farmers by means of questionnaires. The deformities varied from mild to severe, and ewes were reported to have given birth to both normal and deformed lambs; both male and female lambs were affected. Most of the affected lambs were delivered at term. Besides malformed and normal lambs, dummy lambs, unable to suckle, were born also on these farms. None of the ewes had shown clinical signs during gestation or at parturition. Dystocia was common, because of the lambs' deformities. Lambs were submitted for post-mortem examination, and samples of brain tissue were collected for virus detection. The main macroscopic findings included arthrogryposis, torticollis, scoliosis and kyphosis, brachygnathia inferior, and mild-to-marked hypoplasia of the cerebrum, cerebellum and spinal cord. Preliminary data from the first ten affected farms suggest that nutritional deficiencies, intoxication, and genetic factors are not likely to have caused the malformations. Preliminary diagnostic analyses of precolostral serum samples excluded border disease virus, bovine viral diarrhoea virus, and bluetongue virus. In December 2011, samples of brain tissue from 54 lambs were sent to the Central Veterinary Institute of Wageningen University Research, Lelystad. Real-time PCR detected the presence of a virus, provisionally named the Schmallenberg virus, in brain tissue from 22 of the 54 lambs, which originated from seven of eight farms that had submitted lambs for post-mortem examination. This Schmallenberg virus was first reported in Germany and seems to be related to the Shamonda, Aino, and Akabane viruses, all of which belong to the Simbu serogroup of the genus Orthobunyavirus of the family Bunyaviridae. These preliminary findings suggest that the Schmallenberg virus is the most likely cause of this epizootic of ovine congenital malformations, which is the first such outbreak reported in Europe. PMID:22393844 van den Brom, R; Luttikholt, S J M; Lievaart-Peterson, K; Peperkamp, N H M T; Mars, M H; van der Poel, W H M; Vellema, P The influence of Eustachian tube (ET) dysfunction on the inner ear fluid pressure and thus on the inner ear function in Meniere's disease has been discussed controversially. So far, most of the studies examining ET function in inner ear disorders indirectly analyzed ET function by tympanometric methods. The present study directly studied ET function in inner ear disorders by sonotubometry. Healthy subjects and patients with Meniere's disease, sudden sensorineural hearing loss, cholesteatoma and chronic suppurative otitis media were examined by sonotubometry. Mean increase of sound pressure intensity (dB) and mean duration of sound pressure increase (s) were analyzed. Highest mean increase of sound pressure intensity was seen in healthy subjects when using >5 dB peaks (11.6 ± 0.7 dB) and >0 dB peaks (9.6 ± 0.6 dB). Comparative analysis including bilateral ears showed decreased ET function in patients with cholesteatoma (p = 0.002) and in patients with Meniere's disease (p = 0.003) when using >0 dB peaks. Examination of each specific ET opening maneuver showed impaired ET function in pathological ears of patients with cholesteatoma and with Meniere's disease, during yawning (p = 0.001; p < 0.001), dry swallowing (p = 0.010; p = 0.049), Toynbee maneuver (p = 0.033; p = 0.032) and drinking (p = 0.044; p = 0.027). Mild ET dysfunction is detected in patients with Meniere's disease by direct sonotubometric assessment of ET function. PMID:22941437 Park, Jonas J-H; Luedeke, Inger; Luecke, Kerstin; Emmerling, Oliver; Westhofen, Martin The ion channel genome is still being defined despite numerous publications on the subject. The ion channel transcriptome is even more difficult to assess. Using high-throughput computational tools, we surveyed all available inner ear cDNA libraries to identify genes coding for ion channels. We mapped over 100,000 expressed sequence tags (ESTs) derived from human cochlea, mouse organ of Corti, mouse and zebrafish inner ear, and rat vestibular end organs to Homo sapiens, Mus musculus, Danio rerio, and Rattus norvegicus genomes. A survey of EST data alone reveals that at least a third of the ion channel genome is expressed in the inner ear, with highest expression occurring in hair cell-enriched mouse organ of Corti and rat vestibule. Our data and comparisons with other experimental techniques that measure gene expression show that every method has its limitations and does not per se provide a complete coverage of the inner ear ion channelome. In addition, the data show that most genes produce alternative transcripts with the same spectrum across multiple organisms, no ion channel gene variants are unique to the inner ear, and many splice variants have yet to be annotated. Our high-throughput approach offers a qualitative computational and experimental analysis of ion channel genes in inner ear cDNA collections. A lack of data and incomplete gene annotations prevent both rigorous statistical analyses and comparisons of entire ion channelomes derived from different tissues and organisms. Electronic supplementary material The online version of this article (doi:10.1007/s10162-007-0082-y) contains supplementary material, which is available to authorized users. Sokolowski, Bernd H.A.; Morton, Cynthia C.; Giersch, Anne B.S. Sensory epithelia of the inner ear contain two major cell types: hair cells and supporting cells. It has been clear for a long time that hair cells play critical roles in mechanoreception and synaptic transmission. In contrast, until recently the more abundant supporting cells were viewed primarily as serving primarily structural and homeostatic functions. In this review we discuss the growing information about the roles that supporting cells play in the development, function and maintenance of the inner ear, their activities in pathological states, their potential for hair cell regeneration, and the mechanisms underlying these processes. Wan, Guoqiang; Corfas, Gabriel; Stone, Jennifer S Lupus vulgaris is the most common morphological variant of cutaneous tuberculosis. Classical lupus lesions are often seen in the head and neck region. Turkey ear is a clinically descriptive term, previously being used for the earlobe with reddish indurated plaque lesions, which recently can be a sign for lupus vulgaris. A 65-year-old man presented with lupus vulgaris of the earlobe. The diagnosis was confirmed by conventional laboratory investigations and the patient showed well response to antituberculous therapy. This is the second reported case of “turkey ear” as a manifestation of cutaneous tuberculosis. Kucukunal, Asl?; Ekmekci, Tugba R; Sak?z, Damlanur Lupus vulgaris is the most common morphological variant of cutaneous tuberculosis. Classical lupus lesions are often seen in the head and neck region. Turkey ear is a clinically descriptive term, previously being used for the earlobe with reddish indurated plaque lesions, which recently can be a sign for lupus vulgaris. A 65-year-old man presented with lupus vulgaris of the earlobe. The diagnosis was confirmed by conventional laboratory investigations and the patient showed well response to antituberculous therapy. This is the second reported case of "turkey ear" as a manifestation of cutaneous tuberculosis. PMID:23248385 Küçükünal, Asl?; Ekmekçi, Tu?ba R; Sak?z, Damlanur Multiple congenital malformations in a young girl with bilateral conductive hearing loss are described. Facial dysmorphic features include prominent supraorbital ridges, facial hypoplasia, facial asymmetry, downward-slanting palpebral fissures, high prominent nasal bridge with bifid nasal tip and a small lower jaw, and hypoplastic ear lobules with bilaterally narrow and oblique external auditory canals. Recognisable skeletal abnormalities include hypoplastic facial bones, hypoplastic clavicles, narrow and anteriorly sloping shoulders, bowing of both forearm bones, brachydactyly due to short metacarpals and hypoplastic terminal phalanges, thoracolumbar kyphoscoliosis, narrow transverse measurements of most vertebrae with prominent coccyx, spinal canal narrowing, hypoplasia of lower ilia, medially bowed femora, tibiae and fibulae and brachysyndactyly of the second, third and fourth toes bilaterally. Gynaecological evaluation revealed abnormalities of the Mullerian duct structures: urogenital sinus, a vestigial uterus, a posteriorly placed small but patent vagina and a septum at the vaginal introitus. The pattern of MCA probably refers to a new syndrome within the "community of syndromes" involving anomalies of the Mullerian duct structures, limbs, spine and external ears. PMID:9272710 Kumar, D; Masel, J P Abernethy malformation is a rare anomaly of the splanchnic venous system. We report a case of an unusual portosystemic shunt via a dilated inferior mesenteric vein. A 20-year-old woman was referred to our hospital with complains of nonspecific abdominal pain for almost 3 years and hematochezia since 15 months old. Computed tomography and further transhepatic splenoportography revealed a hypoplastic portal vein and a giant inferior mesenteric vein, via which part of the portal venous blood drained into the inferior vena cava. The patient underwent a surgical ligation of the portocaval shunt and recovered well. We believe that this is the first case of type II Abernethy malformation presenting as a portosystemic shunt via the giant inferior mesenteric vein. PMID:24530574 Lu, Jingbo; Lin, Zhiqi; Liu, Hao; Liu, Zhengjun Arteriovenous malformations (AVMs) of the jaws are relatively rare, with fewer than 200 cases reported in the literature. Their real importance lies in their potential to result in exsanguination, which usually follows an unrelated treatment, such as tooth extraction, surgical intervention, puncture wound or blunt injury in involved areas, with the dentist unaware of the existence of the AVM. The present case illustrates an AVM in an 18-year-old female with swelling on the right side of the face. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigation diagnosed it as high-flow vascular malformation. We ascertain the importance of dentists' awareness of the fatal outcome of these lesions and emphasize that, prior to performing any procedure, necessary investigations should always be done. PMID:22282511 Shailaja, S R; Manika; Manjula, M; Kumar, L V Arteriovenous malformations (AVMs) of the jaws are relatively rare, with fewer than 200 cases reported in the literature. Their real importance lies in their potential to result in exsanguination, which usually follows an unrelated treatment, such as tooth extraction, surgical intervention, puncture wound or blunt injury in involved areas, with the dentist unaware of the existence of the AVM. The present case illustrates an AVM in an 18-year-old female with swelling on the right side of the face. This case report is unique because although there was no history of bleeding episodes, thorough examination and investigation diagnosed it as high-flow vascular malformation. We ascertain the importance of dentists' awareness of the fatal outcome of these lesions and emphasize that, prior to performing any procedure, necessary investigations should always be done. Shailaja, S R; Manika; Manjula, M; Kumar, L V In this video, we illustrate a right far-lateral craniotomy for resection of a 13-mm cavernous malformation of the pons in a healthy 53-year-old female patient presenting with diplopia and right 6th nerve palsy. The cavernous malformation was surrounded by normal pons, but was within 1 mm of the pontomedullary sulcus. The lesion was exposed from below through a far lateral craniotomy and accessed through the vasoaccessory triangle, superior to olivary nucleus and 12th cranial nerve. The alternative retrosigmoid craniotomy would have involved significant transgression of the middle cerebellar peduncle. The patient had gross-total resection and some temporary increase in her abducens nerve palsy without any complication. The video can be found here: http://youtu.be/8nOnrnTk3Tg . PMID:24380516 Abla, Adib Adnan; Clark, Aaron J; Lawton, Michael L Objective\\u000a In patients with non-traumatic subarachnoid hemorrhage (SAH) and no evidence for a cerebral aneurysm on angiography, a frequent\\u000a cause of the hemorrhage is perimesencephalic hemorrhage or other cerebral vascular pathology. In some patients no cause is\\u000a found. The exact incidence of a spinal vascular malformation (SVM) as the origin for the SAH is not known. We assessed the\\u000a occurrence M. R. Germans; F. A. Pennings; M. E. S. Sprengers; W. P. Vandertop The investigators present a novel image-guided embolization, not previously described, of a uterine arteriovenous malformation (AVM) resistant to endovascular management. The uterus was exposed surgically, and Histoacryl (Braun, Fulda, Germany) was injected directly into the nidus using ultrasound guidance and fluoroscopy. The patient had a successful full-term pregnancy after this procedure. This technique may be a useful alternative management strategy in patients with uterine AVM who fail traditional endovascular embolization and who still desire fertility. Przybojewski, Stefan J., E-mail: [email protected]; Sadler, David J. [University of Calgary, Diagnostic Imaging Department, Foothills Hospital (Canada) During an 8-year period, facial defects were observed in 146 (7%) of the 2,086 fetuses that underwent karyotyping in our unit because of fetal malformations and\\/or growth retardation. Chromosomal abnormalities were detected in 37 of 56 (66%) fetuses with micrognathia, in 10 of 13 (77%) with macroglossia, in 31 of 64 (48 %) with cleft lip and palate, in 5 K. H. Nicolaides; D. R. Salvesen; R. J. M. Snijders; C. M. Gosden The authors report their experience on one patient with hypersexuality from resection of left occipital arteriovenous malformation.\\u000a To the best of our knowledge, this is the first case reported in the literature. A 35-year-old right-handed female farmer\\u000a suffered a sudden left occipital hemorrhage with subarachnoid and subdural hemorrhages of the left hemisphere. Transient left\\u000a uncal herniation occurred at the onset Yong Cao; Zhaohui Zhu; Rong Wang; Shuo Wang; Jizong Zhao We present a polysomnogram-documented report of central sleep apnea (427 events) and moderately severe decreases in arterial oxygen saturation (to 81%) associated with the Arnold-Chiari malformation (ACM). Daytime hypersomnolence and other symptoms had significantly impaired our patient's work performance. After surgical correction of the ACM, there was marked improvement in symptomatology. A post-surgery polysomnogram revealed marked improvement in the number of central apneas (74 events) and only mild decreases in oxygen saturation (to 94%). PMID:4051328 Balk, R A; Hiller, F C; Lucas, E A; Scrima, L; Wilson, F J; Wooten, V Background\\/Purpose: Patients with esophageal atresia (EA) often have skeletal malformations. The purpose of this study is to examine if similar defects occur in rat fetuses prenatally exposed to Adriamycin, a chemical capable of causing EA in these animals.Methods: The charts of 443 babies with EA were reviewed to assess the incidence and nature of these defects in them. Time-mated female Huimin Xia; Lucia Migliazza; Sandra Montedonico; Jose I. Rodriguez; Juan A. Diez-Pardo; Juan A. Tovar A 75-year-old woman presented with acute onset dyspnoea, and was found to have signs of pulmonary congestion on clinical examination. Imaging revealed cardiomegaly and coincident congestive hepatopathy, secondary to a left renal arteriovenous malformation. The presence of a high flow vascular shunt in the left kidney was possibly the causative factor behind both the high-output cardiac failure and congestive hepatopathy. PMID:23349173 Khalife, Mohammad; Faraj, Walid; Salah, Fatima; Haydar, Ali A Anomalies involving the pulmonary vein are so rare that most of them have been reported as a case or a series of several cases. Some of them often simulate pulmonary arteriovenous malformation (AVM), and patients undergo pulmonary angiography for embolization. Herein we report a case of anomalous unilateral single pulmonary vein that was confirmed on pulmonary angiography after initial diagnosis of pulmonary AVM on contrast medium-enhanced chest computed tomography. PMID:23989502 Hyun, Dongho; Do, Young Soo; Lim, Seong Joo; Park, Hong Suk; Park, Kwang Bo Despite almost a century of efforts in the treatment of brain arteriovenous malformations (BAVMs) no clinical trial has yet\\u000a been performed to demonstrate the benefits of intervention versus conservative (medical) management for those not yet bled.\\u000a Only insufficient information on the natural history of unbled BAVMs exists to certify that intervention is needed and that\\u000a such intervention produces a better J. P. Mohr Malformation disease of Mango (Mangifera indica L.) caused by Fusarium moniliforme var. subglutinans is one of the most destructive diseases, which is a major production constraint in the mango-growing regions of India. In this study, The bioagents Trichoderma viride (Tr1), Trichoderma virens (Tr2) and Trichoderma harzianum (Tr3) were evaluated in culture with the pathogens to monitor the antagonistic effect and Pradeep Kumar; Ashok Kumar Misra; Dinesh Raj Modi; Vijai Kumar Gupta ...CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Therapeutic Devices Â§ 874.5300 Ear, nose, and throat examination...blowing apparatus, and receptacles for connection of specialized lights and examining instruments. (b) Classification.... ...CONTINUED) MEDICAL DEVICES EAR, NOSE, AND THROAT DEVICES Therapeutic Devices Â§ 874.5300 Ear, nose, and throat examination...blowing apparatus, and receptacles for connection of specialized lights and examining instruments. (b) Classification.... ...120524116-2116-01] RIN 0694-AF70 Revisions to the Export Administration Regulations (EAR): Unverified...Security (BIS) proposes to amend the Export Administration Regulations (EAR) by: requiring exporters to file an Automated Export System (AES) record for all... ...110310188-2058-03] RIN 0694-AF17 Revisions to the Export Administration Regulations (EAR): Export Control Classification Number 0Y521 Series...publishes this final rule, which amends the Export Administration Regulations (EAR) by... Fetal malformations are very frequent in industrialized countries. Although advanced maternal age may affect pregnancy outcome adversely, 80%-90% of fetal malformations occur in the absence of a specific risk factor for parents. The only effective approach for prenatal screening is currently represented by an ultrasound scan. However, ultrasound methods present two important limitations: the substantial absence of quantitative parameters and the dependence on the sonographer experience. In recent years, together with the improvement in transducer technology, quantitative and objective sonographic markers highly predictive of fetal malformations have been developed. These markers can be detected at early gestation (11-14 wk) and generally are not pathological in themselves but have an increased incidence in abnormal fetuses. Thus, prenatal ultrasonography during the second trimester of gestation provides a “genetic sonogram”, including, for instance, nuchal translucency, short humeral length, echogenic bowel, echogenic intracardiac focus and choroid plexus cyst, that is used to identify morphological features of fetal Down’s syndrome with a potential sensitivity of more than 90%. Other specific and sensitive markers can be seen in the case of cardiac defects and skeletal anomalies. In the future, sonographic markers could limit even more the use of invasive and dangerous techniques of prenatal diagnosis (amniocentesis, etc.). Renna, Maria Daniela; Pisani, Paola; Conversano, Francesco; Perrone, Emanuele; Casciaro, Ernesto; Renzo, Gian Carlo Di; Paola, Marco Di; Perrone, Antonio; Casciaro, Sergio The incidence of obesity in pregnancy has increased over the past two decades, with nearly 50% of U.S. women aged 15–49 are classified as overweight or obese. Obesity (independent of diabetes) among gravidae poses unique risks which extend towards the fetus, with several large population-based analyses demonstrating independent increased risks for fetal malformations including neural tube defects, cardiac anomalies, and orofacial clefts as well as stillbirth and macrosomia. Unfortunately, several lines of evidence also suggest that the quality of the prenatal fetal anatomic survey and certain aspects of prenatal diagnostic screening programs are significantly limited. The net effect is that among obese gravidae, the increased risk of fetal anomalies is further offset by a concomitant diminished ability to sonographically detect such malformation in the prenatal interval. The purpose of this summary review is to systematically examine the evidence suggesting an increased risk of fetal malformations in obese gravidae, the contributing role of diabetes, and the limitations of prenatal diagnostic and sonographic screening among this at-risk population. RACUSIN, Diana; STEVENS, Blair; CAMPBELL, Genevieve; AAGAARD-TILLERY, Kjersti Peripheral vascular malformations are now described according to some accepted guidelines, and the principle of proper treatment (nidus ablation) is becoming clear. An appropriate classification scheme for vascular anomalies and definite indications for treatment are important to successful treatment overall. The findings from noninvasive imaging (ie, Doppler ultrasonography, computed tomography, or magnetic resonance imaging) in association with clinical findings are critical in establishing the diagnosis, evaluating the extent of the malformation, and planning appropriate treatment. Direct opacification of the nidus is useful, not only in making a correct diagnosis, but also in treating the lesion with sclerotherapy. In most cases, conservative treatment is recommended, but when a patient suffers clinical complications (eg, ulceration, pain, hemorrhage, cardiac failure, or unacceptable cosmetic consequences), the nidus sclerotherapy becomes mandatory. If the vascular malformation has blood outflow to a drainage vein during nidus opacification, flow control (with balloon occlusion, tourniquet, or embolization) is necessary to achieve sclerosant stasis within the nidus. Embolotherapy (with a coil, n-butyl cyanoacrylate, or small particles) should be used for subsequent multifaceted palliative therapy. A multi-disciplinary approach is needed in the treatment of a high-flow lesion, and a dedicated team approach is necessary for appropriate management in most cases. PMID:16227489 Hyodoh, Hideki; Hori, Masakazu; Akiba, Hidenari; Tamakawa, Mitsuharu; Hyodoh, Kazusa; Hareyama, Masato Malformations of cortical development are increasingly recognized as important causes of epilepsy, developmental delay and other neurological disorders. Our purpose is to present the relevance of the MRI in these pathologies with the clinical, genetic and therapeutic aspects. This classification is based on the three fundamental events of cortical formation: proliferation of neurons and glie in the periventricular zone, migration of postmitotic neurons to the periphery, subsequent cortical organization. MR analysis evaluates particularly the cortical thickness, sulcal and cortical morphology, gray-white matter junction, and looks for gray matter in abnormal location. These data coupled with the familial history, the seizure characteristics and genetic findings should allow an appropriate classification of the lesions. MR imaging allows the detection and classification of cortical malformations. MR imaging findings are primordial to consider surgery when the epilepsy becomes refractory to the anti-epileptic drugs. An adequate classification of these malformations should help to provide to the family an appropriate counseling both in terms of genetics and outcome. PMID:17095957 Cottier, J P; Toutain, A; Hommet, C; Sembely, C; Bosq, M; Texier, N; Herbreteau, D; Sirinelli, D Over the past ten years, polyethylene tube, stainless steel wire and absorbable gelatin sponge have come to be used in middle ear surgery. A modified Williams' steel chamber with a transparent central area was embedded in a rabbit ear lobe surgically, and cross-sectioned polyethylene tube, stainless steel wire or absorbable gelatin sponge were inserted respectively. The in vivo tissue reaction to each of these installed materials was observed daily microscopically. Finally, in the case of both the cross-sectioned polyethylene tube and the stainless steel wire, the chambers were fixed with 10% formalin solution and the contents stained for histological study. The results were as follows: Absorbable gelatin sponge was absorbed completely by phagocytosis in 62 days. Polyethylene tube was encapsulated by a layer of fibroblasts by day 110 in one case and by day 140 in another case; these findings were confirmed histologically on day 110 and 281. Almost all the surface of the stainless steel wire was encapsulated by a layer consisting of giant cells, on which a dense fibrous layer was superimposed by day 82. It is concluded that polyethylene tube, stainless steel wire, and absorbable gelatin sponge should be well tolerated in middle ear surgery. PMID:137659 Ohsaki, K; Saito, R Middle and inner ears from human cadaver temporal bones were stimulated in the forward direction by an ear-canal sound source, and in the reverse direction by an inner-ear sound source. For each stimulus type, three variables were measured: (a) Pec-ear-canal pressure with a probe-tube microphone within 3 mm of the eardrum, (b) Vst-stapes velocity with a laser interferometer, and (c) This paper investigates an ear worn sensor for the development of a gait analysis framework. Instead of explicitly defining gait features that indicate injury or impairment, an automatic method of feature extraction and selection is proposed. The proposed framework uses multi-resolution wavelet analysis and margin based feature selection. It was validated on three datasets; the first simulating a leg injury, Louis Atallah; Omer Aziz; Benny P. L. Lo; Guang-zhong Yang We are interested in stable gene network activities operating sequentially during inner ear specification. The implementation of this patterning process is a key event in the generation of functional subdivisions of the otic vesicle during early embryonic development. The vertebrate inner ear is a complex sensory structure that is a good model system for characterization of developmental mechanisms controlling patterning and specification. Meis genes, belonging to the TALE family, encode homodomain-containing transcription factors remarkably conserved during evolution, which play a role in normal and neoplastic development. To gain understanding of the possible role of homeobox Meis genes in the developing chick inner ear, we comprehensively analyzed their spatiotemporal expression patterns from early otic specification stages onwards. In the invaginating otic placode, Meis1/2 transcripts were observed in the borders of the otic cup, being absent in the portion of otic epithelium closest to the hindbrain. As development proceeds, Meis1 and Meis2 expressions became restricted to the dorsomedial otic epithelium. Both genes were strongly expressed in the entire presumptive domain of the semicircular canals, and more weakly in all associated cristae. The endolymphatic apparatus was labeled in part by Meis1/2. Meis1 was also expressed in the lateral wall of the growing cochlear duct, while Meis2 expression was detected in a few cells of the developing acoustic-vestibular ganglion. Our results suggest a possible role of Meis assigning regional identity in the morphogenesis, patterning, and specification of the developing inner ear. PMID:21120931 Sánchez-Guardado, Luis Óscar; Ferran, José Luis; Rodríguez-Gallardo, Lucía; Puelles, Luis; Hidalgo-Sánchez, Matías The effectiveness of ten different ear-protectors (6 types of earmuffs and 4 types of earplugs) has been tested under laboratory conditions and in the real occupational environment. Three methods were used: (1) physical, utilizing a dummy head; (2) subjective, real-ear, executed on trained human subjects; (3) subjective, measuring TTS2 resulting from occupational, one-workday exposure. It could be shown that the ear protection efficiency ascertained on the basis of TTS2 measurements on workers exposed to noise in their occupational environment is, in nearly all cases, smaller than the efficiency expected, taking into account the sound damping of the same protectors, tested under laboratory conditions, using the physical or real-ear method. Measurements of TTS2 were found to give the best data needed to define the protectors' efficiency, since they include, simultaneously, the impact of various environmental factors, the subjective reactiveness, the nature of the professional task and the acoustical features of the protector used. Therefore this method enables the estimation of the real protection given to workers with a risk of noise-induced hearing loss. PMID:2379964 Pawlas, K; Grzesik, J Most maize cultivars have been developed for grain production. Because superior cultivars may differ in their exploiting purposes, interest has been demonstrated for the evaluation of corn cultivars with regard to their baby corn, green ear, and grain yields production ability. In the present work ten corn cultivars (AG 405, AG 1051, AG 2060, AG 6690, AG 7575, AG 8080, Itala Paula de C. Almeida; Paulo Sérgio L. e Silva; Maria Z. de Negreiros; Zenaide Barbosa An outbreak of erysipelas killed an estimated 5,000 aquatic birds on Great Salt Lake (Utah) in late November, 1975. Although several thousand ducks and gulls were using the lake, at least 99 percent of the victims were eared grebes. A hypothetical explanation for the selective mortality is offered. Jensen, W. I.; Cotter, S. E.	2	131	4	0	0	0	0.397529	4	89,185
Chronic, recurrent skin disorder with reddish, slightly elevated patches or bumps covered with silvery-white scales. Spots may coalesce into large patches around a normal area. If the nails are involved, they may become pitted, thick, and separated from the nail bed. Skin injury, infection, stress, and certain drugs may trigger psoriasis. Skin cells move at an accelerated rate from the dermis into the epidermis, where they slough off, causing inflammation. In some cases, patients also have arthritis. Psoriasis often becomes less severe in the summer and during pregnancy. There is no cure, but treatment with drugs and ultraviolet light may help. Learn more about psoriasis with a free trial on Britannica.com. The disorder is a chronic recurring condition which varies in severity from minor localised patches to complete body coverage. Fingernails and toenails are frequently affected (psoriatic nail dystrophy) - and can be seen as an isolated finding. Psoriasis can also cause inflammation of the joints, which is known as psoriatic arthritis. Ten to fifteen percent of people with psoriasis have psoriatic arthritis. The cause of psoriasis is not known, but it is believed to have a genetic component. Several factors are thought to aggravate psoriasis. These include stress, excessive alcohol consumption, and smoking. Individuals with psoriasis may suffer thereby from depression and loss of self-esteem. As such, quality of life is an important factor in evaluating the severity of the disease. There are many treatments available but because of its chronic recurrent nature psoriasis is a challenge to treat. While it may have been visually, and later semantically, confused with leprosy it was not until 1841 that the condition was finally given the name psoriasis by the Viennese dermatologist Ferdinand von Hebra. The name is derived from the Greek word psora which means to itch. It was during the 20th century that psoriasis was further differentiated into specific types. The symptoms of psoriasis can manifest in a variety of forms. Variants include plaque, pustular, guttate and flexural psoriasis. This section describes each type (with ICD-10 code ). Plaque psoriasis (psoriasis vulgaris) (L40.0) is the most common form of psoriasis. It affects 80 to 90% of people with psoriasis. Plaque psoriasis typically appears as raised areas of inflamed skin covered with silvery white scaly skin. These areas are called plaques. Flexural psoriasis (inverse psoriasis) (L40.83-4) appears as smooth inflamed patches of skin. It occurs in skin folds, particularly around the genitals (between the thigh and groin), the armpits, under an overweight stomach (pannus), and under the breasts (inframammary fold). It is aggravated by friction and sweat, and is vulnerable to fungal infections. Guttate psoriasis (L40.4) is characterized by numerous small round spots (differential diagnosis - pityriasis rosea - oval shape lesion). These numerous spots of psoriasis appear over large areas of the body, such as the trunk, limbs, and scalp. Guttate psoriasis is associated with streptococcal throat infection. Pustular psoriasis (L40.1-3, L40.82) appears as raised bumps that are filled with non-infectious pus (pustules). The skin under and surrounding pustules is red and tender. Pustular psoriasis can be localised, commonly to the hands and feet (palmoplantar pustulosis), or generalised with widespread patches occurring randomly on any part of the body. Nail psoriasis (L40.86) produces a variety of changes in the appearance of finger and toe nails. These changes include discolouring under the nail plate, pitting of the nails, lines going across the nails, thickening of the skin under the nail, and the loosening (onycholysis) and crumbling of the nail. Psoriatic arthritis (L40.5) involves joint and connective tissue inflammation. Psoriatic arthritis can affect any joint but is most common in the joints of the fingers and toes. This can result in a sausage-shaped swelling of the fingers and toes known as dactylitis. Psoriatic arthritis can also affect the hips, knees and spine (spondylitis). About 10-15% of people who have psoriasis also have psoriatic arthritis. Erythrodermic psoriasis (L40.85) involves the widespread inflammation and exfoliation of the skin over most of the body surface. It may be accompanied by severe itching, swelling and pain. It is often the result of an exacerbation of unstable plaque psoriasis, particularly following the abrupt withdrawal of systemic treatment. This form of psoriasis can be fatal, as the extreme inflammation and exfoliation disrupt the body's ability to regulate temperature and for the skin to perform barrier functions. The Psoriasis Area Severity Index (PASI) is the most widely used measurement tool for psoriasis. PASI combines the assessment of the severity of lesions and the area affected into a single score in the range 0 (no disease) to 72 (maximal disease). Nevertheless, the PASI can be too unwieldy to use outside of trials, which has led to attempts to simplify the index for clinical use. Individuals with psoriasis may also feel self-conscious about their appearance and have a poor self-image that stems from fear of public rejection and psychosexual concerns. Psychological distress can lead to significant depression and social isolation. The prevalence of psoriasis in Western populations is estimated to be around 2-3%. The prevalence of psoriasis among 7.5 million patients who were registered with a general practitioner in the United Kingdom was 1.5%. A survey conducted by the National Psoriasis Foundation (a US based psoriasis education and advocacy group) found a prevalence of 2.1% among adult Americans. The study found that 35% of people with psoriasis could be classified as having moderate to severe psoriasis. Around one-third of people with psoriasis report a family history of the disease, and researchers have identified genetic loci associated with the condition. Studies of monozygotic twins suggest a 70% chance of a twin developing psoriasis if the other twin has psoriasis. The concordance is around 20% for dizygotic twins. These findings suggest both a genetic predisposition and an environmental response in developing psoriasis. Onset before age 40 usually indicates a greater genetic susceptibility and a more severe or recurrent course of psoriasis. The immune-mediated model of psoriasis has been supported by the observation that immunosuppressant medications can clear psoriasis plaques. However, the role of the immune system is not fully understood, and it has recently been reported that an animal model of psoriasis can be triggered in mice lacking T cells. Animal models, however, reveal only a few aspects resembling human psoriasis. Psoriasis is a fairly idiosyncratic disease. The majority of people's experience of psoriasis is one in which it may worsen or improve for no apparent reason. Studies of the factors associated with psoriasis tend to be based on small (usually hospital based) samples of individuals. These studies tend to suffer from representative issues, and an inability to tease out causal associations in the face of other (possibly unknown) intervening factors. Conflicting findings are often reported. Nevertheless, the first outbreak is sometimes reported following stress (physical and mental), skin injury, and streptococcal infection. Conditions that have been reported as accompanying a worsening of the disease include infections, stress, and changes in season and climate. Certain medicines, including lithium salt and beta blockers, have been reported to trigger or aggravate the disease. Excessive alcohol consumption, smoking and obesity may exacerbate psoriasis or make the management of the condition difficult. Individuals suffering from the advanced effects of the Human immunodeficiency virus, or HIV, often exhibit psoriasis. This presents a paradox to researchers as traditional therapies that reduce T-cell counts generally cause psoriasis to improve. Yet, as CD4-T-cell counts decrease with the progression of HIV, psoriasis worsens. In addition, HIV is typically characterized by a strong Th2 cytokine profile, whereas psoriasis vulgaris is characterized by a strong Th1 secretion pattern. It's hypothesized that the diminished CD4-T-Cell presence causes an over-activation of CD8-T-Cells, which are responsible for the exacerbation of psoriasis in HIV positive patients. It is important to remember that most individuals with psoriasis are otherwise healthy and the presence of HIV accounts for less than 1% of cases. The prevalence of psoriasis in the HIV positive population ranges from 1 to 6 percent, which is about 3 times higher than the normal population. Medications with the least potential for adverse reactions are preferentially employed. If the treatment goal is not achieved then therapies with greater potential toxicity may be used. Medications with significant toxicity are reserved for severe unresponsive psoriasis. This is called the psoriasis treatment ladder. As a first step, medicated ointments or creams, called topical treatments, are applied to the skin. If topical treatment fails to achieve the desired goal then the next step would be to expose the skin to ultraviolet (UV) radiation. This type of treatment is called phototherapy. The third step involves the use of medications which are taken internally by pill or injection. This approach is called systemic treatment. Over time, psoriasis can become resistant to a specific therapy. Treatments may be periodically changed to prevent resistance developing (tachyphylaxis) and to reduce the chance of adverse reactions occurring. This is called treatment rotation. The disadvantages of topical agents are variably that they can often irritate normal skin, can be time consuming and awkward to apply, cannot be used for long periods, can stain clothing or have a strong odour. As a result, it is sometimes difficult for people to maintain the regular application of these medications. Abrupt withdrawal of some topical agents, particularly corticosteroids, can cause an aggressive recurrence of the condition. This is known as a rebound of the condition. Some topical agents are used in conjunction with other therapies, especially phototherapy. Sunlight contains many different wavelengths of light. It was during the early part of the 20th century that it was recognised that for psoriasis the therapeutic property of sunlight was due to the wavelengths classified as ultraviolet (UV) light. Ultraviolet wavelengths are subdivided into UVA (380–315 nm) UVB (315–280 nm), and UVC (< 280 nm). Ultraviolet B (UVB) (315–280 nm) is absorbed by the epidermis and has a beneficial effect on psoriasis. Narrowband UVB (311 to 312 nm), is that part of the UVB spectrum that is most helpful for psoriasis. Exposure to UVB several times per week, over several weeks can help people attain a remission from psoriasis. Ultraviolet light treatment is frequently combined with topical (coal tar, calcipotriol) or systemic treatment (retinoids) as there is a synergy in their combination. The Ingram regime, involves UVB and the application of anthralin paste. The Goeckerman regime combines coal tar ointment with UVB. The three main traditional systemic treatments are methotrexate, cyclosporine and retinoids. Methotrexate and cyclosporine are immunosupressant drugs; retinoids are synthetic forms of vitamin A. Other additional drugs, not specifically licensed for psoriasis, have been found to be effective. These include the antimetabolite tioguanine, the cytotoxic agent hydroxyurea, sulfasalazine, the immunosupressants mycophenolate mofetil, azathioprine and oral tacrolimus. These have all been used effectively to treat psoriasis when other treatments have failed. Although not licensed in many other countries fumaric acid esters have also been used to treat severe psoriasis in Germany for over 20 years. Biologics are manufactured proteins that interrupt the immune process involved in psoriasis. Unlike generalised immunosuppressant therapies such as methotrexate, biologics focus on specific aspects of the immune function leading to psoriasis. These drugs (interleukin antagonists) are relatively new, and their long-term impact on immune function is unknown. They are very expensive and only suitable for very few patients with psoriasis. Ustekinumab (IL-12 and IL-23 blocker) shows hopeful results for psoriasis therapy. Climatotherapy involves the notion that some diseases can be successfully treated by living in a particular climate. Several psoriasis clinics are located throughout the world based on this idea. The Dead Sea is one of the most popular locations for this type of treatment. In Turkey & in Croatia (Altermedica), doctor fish which live in the outdoor pools of spas, are encouraged to feed on the psoriatic skin of people with psoriasis. The fish only consume the affected areas of the skin. The outdoor location of the spa may also have a beneficial effect. This treatment can provide temporary relief of symptoms. A revisit to the spas every few months is often required. Treatment in this hot spring has been examined until now in two small clinical trials, with positive results. A number of patients have reported significant improvements from sun and sea water: unfortunately, salt alone does not have any effect. Sea water contains so many minerals and different life forms (thousands of species of bacteria alone) that it will be hard to determine which of these is causing the observed effects. Interestingly, people in the tropics differentiate between "live" and "dead" sea water: "live" sea water is water that has never been covered. A psychological symptom management programme has been reported as being a helpful adjunct to traditional therapies in the management of psoriasis. It has been suggested that Milk thistle may prevent psoriasis outbreaks by encouraging proper liver function; the claimed mechanism is neutralization of "certain toxins associated with psoriasis attacks" but neither the identity of these toxins, the nature of the association nor the mechanism of neutralization is cited. In the more recent past Fowler's solution, which contains a poisonous and carcinogenic arsenic compound, was used by dermatologists as a treatment for psoriasis during the 18th and 19th centuries. Grenz rays (also called ultrasoft X-rays or Bucky rays) was a popular treatment of psoriasis during the middle of the 20th century. This type of therapy was superseded by ultraviolet therapy. Undecylenic acid was investigated and used for psoriasis some 40 years ago(cir. 1950~). All these treatments have fallen out of favour. Sulphur was fashionable as a treatment for psoriasis in the Victorian and Edwardian eras. It has recently re-gained some credibility as a safe alternative to steroids and coal tar. It has been suggested that cannabis might treat psoriasis, due to the anti-inflammatory properties of its cannabinoids, and the regulatory effects of THC on the immune system. The adverse effects of cannabis might be overcome by use of more specific cannabinoid receptor medications, to inhibit keratinocyte proliferation. Future innovation should see the creation of additional drugs that refine the targeting of immune-mediators further. Research into antisense oligonucleotides carries the potential to provide novel therapeutic strategies for treating psoriasis. ABT-874 is a human anti-IL-12 monoclonal antibody being developed by Abbott Laboratories in conjunction with Cambridge Antibody Technology for the treatment of multiple autoimmune diseases including psoriasis. Phase II trials have been completed and showed promising results. Abbott was planning to initiate Phase III trials in 2007. In 2004, Tas and Avci demonstrated cyclopamine’s clinical potential for the treatment of psoriasis and basal cell carcinoma in two preliminary proof of concept studies. By treating 31 psoriatic lesions in 7 patients, these authors asserted that topical cyclopamine was more effective in the clinical and histological clearance of guttate and plaque psoriasis than the topical steroid clobetasol-17 propionate.Furthermore, they demonstrated that concurrent application of cylopamine and clobetasol-17 propionate accelerated regression and clearance of selected lesions greater than cyclopamine alone with clearance times as early as 48 hours.They assert that cyclopamine inhibits the abnormal proliferation of epithelial cells, induces terminal differentiation, and is associated with the decreased presence of inflammatory cells, including CD41 lymphocytes. On August 27, 2006, scientists led by Jeung-Hoon Lee created in the laboratory synthetic lipids called pseudoceramides which are involved in skin cell growth and could be used in treating skin diseases such as atopic dermatitis, a form of eczema characterized by red, flaky and very itchy skin; psoriasis, and glucocorticoid-induced epidermal atrophy, in which the skin shrinks due to skin cell loss. According to one study, psoriasis is linked to 2.5-fold increased risk for non melanoma skin cancer in men and women, with no preponderance of any specific histologic subtype of cancer. This, however could be linked to antipsoriatic treatment.	2	12	0	0	0	8	0.732922	8	3,722
Explore health content from A to Z. I need information about... Premature ventricular contractions (PVCs) are a type of arrhythmia (irregular heartbeat). They are common and can affect people of all ages. PVCs are almost never dangerous. But if other heart problems are present, PVCs can cause serious health issues. This sheet tells you more about PVCs and how they are treated. To understand how PVCs occur, it helps to first understand how your heart works. Your heart is a muscle that pumps blood throughout the body. It is made up of 4 chambers: 2 atria and 2 ventricles. Electrical signals are sent to these chambers, making them contract (squeeze) in a certain order. This rhythm, which pumps blood through and out of your heart, is your heartbeat. The process begins in the sinoatrial (SA) or sinus node. This is the heart's natural pacemaker: The SA node. This group of cells in the right atrium sends a signal to both atria, telling them to contract. When the atria contract, blood is pumped into the ventricles. The AV node. This is another group of cells in the right atrium. It receives the signal from the SA node after it passes through the atria. The AV node transmits the electrical signal from the atria to the ventricles. During a PVC, an abnormal signal disrupts the normal heartbeat. This signal comes from the ventricle instead of the SA node. The signal causes the ventricles to contract too soon, and the heart skips the next normal beat. This results in an irregular heartbeat. Sometimes PVCs cause no symptoms at all. Other times, a patient may feel palpitations (irregular heartbeats). These can feel like “skipped” beats, or “flopping” in the chest. If PVCs are frequent, other symptoms can occur. These include tiredness, feeling faint, or shortness of breath. They also include fullness or pressure in the neck, and chest pain. These symptoms occur because less oxygen is delivered to the body. This is because PVCs make the heart pump blood less effectively. In some cases, no cause of PVCs is found. When a cause is found, it is either chemical or structural: Chemical. Changes in the body’s chemistry can prompt PVCs. For instance, raised levels of certain hormones, such as adrenaline or thyroid, can cause PVCs. Consuming substances such as alcohol and caffeine can also cause them. Structural. This involves existing problems in the heart and/or cardiovascular system. Coronary artery disease (CAD) is 1 type of problem that can be related to PVCs. Others are heart failure and heart valve problems. The doctor will take your medical history and ask you to describe your symptoms. You’ll also have a physical exam. And certain tests may be done. These include: Electrocardiogram (ECG or EKG). This test records the electrical activity of your heart. During an ECG, small pads (electrodes) are placed on your chest, arms, and legs. Wires connect the pads to a machine, which records your heart’s electrical signals. Heart monitor. There are 2 types of external heart monitors: Holter monitor. This monitor can be worn for 1 to 7 days. It provides a constant recording of heart activity. After the test is done, your health care provider analyzes the recording. Event monitors. These monitors can be used for 3 to 4 weeks. One kind is a memory loop recorder. This monitor records constantly, but stores the recording only when you press a button. The other kind is a credit card-sized recorder. This monitor is turned on only during an episode. With both types, you send the recordings of symptoms to your health care provider over the phone. Treatment depends on whether structural heart problems are present. It is also determined by the severity of symptoms: If you have no other heart problems and your symptoms are not bothersome, treatment may not be needed. If it is needed, treatment can involve: Lifestyle changes. Your doctor may suggest that you exercise and limit caffeine and alcohol. If you smoke, you’ll be advised to quit. Medications. Two types of medication can help with PVCs. Beta-blockers and calcium channel blockers both can lower the heart rate and reduce blood pressure. If you have structural heart problems, you’ll likely be referred to a doctor who specializes in heart rhythm problems. This may be a cardiologist or an electrophysiologist. An electrophysiologist is a cardiologist who specializes in the electrical system of the heart. You will need a referral because for you, PVCs can be a bigger threat to your health. Depending on the nature of your heart disease, you may need treatment for an underlying heart problem. In severe cases, an ICD (implantable cardioverter defibrillator) may be implanted. This is done to treat the underlying heart disease. It can help normalize the heart rhythm. Copyright © 2014 Baylor Health Care System All Rights Reserved. \| 3500 Gaston Avenue, Dallas, TX 75246-2017 \| 1.800.4BAYLOR	2	6	1	0	0	0	0.958414	1	1,112
Type 2 Diabetes Type 2 diabetes is a chronic disease in which your body is unable to maintain a normal blood sugar (glucose) level. - Birth Control Facts about birth control to help decide which type is right for you. - Type 2 Diabetes Basic facts about type 2 diabetes & risk factors to be aware of. - COPD Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Flu Facts about influenza (flu), including symptoms and vaccines. - GERD Get the facts about gastroesophageal reflux disease (GERD). - HIV/AIDS The differences between HIV & AIDS; signs, symptoms & complications. - Lupus Get the facts about lupus, including symptoms, risk factors, and the different types. - Menopause Facts about menopause, including the stages, symptoms, and types. - Shingles Facts about shingles, including symptoms & possible long-term effects. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides Dr. Johnny A Liquete has the following 2 specialties - Infectious Disease An infectious disease specialist has specialized training in the diagnosis and treatment of contagious diseases. Infectious diseases, also known as contagious or transmissible diseases, are those that stem from pathogen from a host organism. These infections may spread to other carriers through physical touch, airborne inhalation, bodily fluids or contaminated foods. Infectious disease specialists identify whether the disease is caused by bacteria, a virus, a fungus or a parasite often through blood tests and then determine what course of treatment, if any, is necessary. - Internal Medicine An internist is a physician who focuses on the diagnosis and treatment of conditions that affect the adult population—both acute and chronic. These doctors are often who adults see as their primary physicians because they treat a broad range of illnesses that do not require surgical or specialist interventions. They also work to help a patient maintain optimal health in order to prevent the onset of disease. In addition to treating the common cold and flu, internists also treat chronic diseases like diabetes and heart disease. - AIDS/HIV (Acquired Immunodeficiency Syndrome) - Weight Loss - Hepatitis C - HIV Infections - Weight Loss (non-surgical) See the board certifications this doctor has received. Board certifications provide confidence that this doctor meets the nationally recognized standards for education, knowledge and experience. Showing 5 of 13 He is by far the rudest doctor I have ever encountered. He was assigned to me after my c section developed into a septic infection. He would regularly be rude, yell, and call me crazy and stupid. He even called another doctor of mine to tell him I was crazy, loudy, where all of the staff and other patients could hear. His bheavier was so awfuk, his Nurse Practitioner kept apologizing for him when he was out of the room. This was 10 years ago. I nearly died with all the medical papers to prove it, yet he was convinced I was faking it or crazy. Ihaving staph, an infected c section, infected blood, and a blood clot with high fevers and weight loss is not fake. I will never forget him as the worst doctor I have ever come across. Linda, Dr Liquete's front office assistant who also does BPs and vitals, is one of the most rude and uncaring PERSONS (not medical professionals only) I've ever encountered. She makes getting an appointment nearly impossible and will never work with you to get you in around the indigenous persons that populate the office. Maybe if I spoke Spanish it would be better as she clearly prefers her fellow Hispanic customers. She has no "shame" about saying "no" and simply seems to enjoy her perceived position of power. Once you get past her, the hour long wait between the waiting room and waiting on the doctor in the room, Dr Liquete is a competent doctor whose only weakness might be his discernment in hiring his assistant. No matter how good he is, Linda makes it not worth taking your patronage here. Dr Liquete - fire Linda and let her take her controlling, overbearing personality to the unemployment line where she might learn some good life lessons. i developed a bone infection requiring several months of IV antibiotics. Fortunately these could be administered at home through the use of a portable pump. I was started on two medications, but within a few days i started developing a reaction to one of them, which began escalating with each use. Finally, on a day before a 3-day holiday i was at physical therapy when i began to develop what was now a pretty severe reaction. My friend had to drive me home, and by the time i got there i had a fever of 104 degrees. fortunately my friend was a nurse and quickly packed me in ice packs, gave aspirin, etc. (i chose not to go to the ER because i knew they would basically do the same thing.)i called Liquete's office, but it was closed for the holiday. On the following Monday i again called the office and attempted to explain what had happened. the girl who answered the phone kept telling me to go to the ER. i repeated that the symptoms had occurred 3 days ago, i had stopped taking that particular med, and i was just calling to inform the doctor of what happened and find out if he wanted to substitute another medication. I kept being told to the ER. I asked if Liquete was going to meet me there? no. then why were they insisting i go when the symptoms had occurred three days ago? I just kept getting the same answer, as if i were talking to a robot. I said i wasn't going to go and ended the call. Imagine my surprise when two days later i received a letter from Liquete saying that he was no longer going to treat me as i was 'non-compliant.'! He referred me to no other doctor, and in fact there were no other infectious disease specialists in my area. I ended up going to UCLA, 50 miles away just to have someone oversee my treatment.As a postscript: i was recently in the hospital and dr. liquete was assigned to be my infectious disease specialist. After i told him about our previous interaction i told him he was fired and to get out of my room. He mumbled something and stomped out. I have to say i enjoyed the experience immensely. 36 Years Experience University Of Santo Tomas Graduated in 1982 Lac Usc Medical Center Dr. Johnny A Liquete accepts the following insurance providers. - Aetna Basic HMO - Aetna Choice POS II - Aetna HMO - Aetna HMO Deductible Plan CA only - Aetna Managed Choice POS Open Access - Aetna Savings Plus of CA - Aetna Signature Administrators PPO - Aetna Vitalidad Plus CA con Aetna - Aetna Whole Health PrimeCare HMO CA - Aetna Whole Health PrimeCare OAMC CA BCBS Blue Card - BCBS Blue Card PPO Blue Cross California - BC CA California Care Small Group HMO - Blue Cross CA California Care Large Group HMO - Blue Cross CA Pathway X HMO - Blue Cross CA Priority Select HMO - Blue Cross CA Select HMO - Blue Cross CA Select Plus HMO Blue Shield California - BS CA Platinum 90 PPO - Blue Shield CA Access Plus HMO - Blue Shield CA Access Plus Savenet - Blue Shield CA Bronze 60 PPO SHOP - Blue Shield CA Bronze Full PPO 4500 - Blue Shield CA Local Access Plus HMO - Blue Shield CA PPO - Blue Shield CA Platinum Access+ HMO 25 - Blue Shield CA Platinum Local Access + HMO 25 - Blue Shield CA Silver 70 PPO - Blue Shield CA Trio ACO HMO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - Health Net CA CommunityCare Network HMO - Health Net CA HMO Employer Group - Health Net CA HMO SmartCare - Health Net CA HMO Whole Care Network - Health Net CA Individual and Family PPO - Health Net CA PPO - Health Net EPO - PureCare One Small Business - Health Net PureCare HSP Individual and Family Plans - Health Net SmartCare Large Group - Health Net SmartCare Small Group - Humana Choice POS - Molina Mrktplace CA - Multiplan PPO - PHCS PPO - PHCS PPO Kaiser - UHC SignatureValue Flex1 - UHC SignatureValue Flex2 - UHC SignatureValue Flex3 - UHC SignatureValue Focus - UHC West SignatureValue Alliance - UHC West SignatureValue HMO - UHC Choice Plus POS - UHC Options PPO - View by Location Locations & DirectionsJohnny Ang Liquete Md, 14114 Business Center Dr Ste D, Moreno Valley, CA Dr. Johnny A Liquete is similar to the following 3 Doctors near Moreno Valley, CA.	2	13	1	0	0	0	0.935083	1	1,952
29 Aug Using Modifiers effectively in Family Practice and Surgery The National Correct Coding Initiative (NCCI) was developed by the Centers for Medicare & Medicaid Services (CMS) in 2010. This was formulated to promote national coding methodologies and to control improper coding. The purpose of NCCI edits is to stop improper payments when incorrect code combinations are used. Modifiers are simple two-character designators that alert billing professionals and claims handlers of a change in how the code for a service, treatment, or procedure should be applied on the claim. Using modifiers correctly will add accuracy to your billing practices. In addition, modifiers detail the record of the encounter. Four New Modifiers to Replace Modifier 59 Modifier 59 is used to define as a “Distinct Procedure Service.” These are services and procedures performed by a physician that are not usually reported together, but are separately billable under certain circumstances. Modifier 59 is added to a CPT code to indicate that the service is distinct and separate from another service or procedure. This modifier is the most used modifier in the billing world, and it is often the most abused. CMS has identified a need to make this modifier more specific, and with ICD-10, changes have occurred to reduce overpayment errors. Modifier 59 is not going away completely, but new options are available. Before using 59, the biller should see if it can be replaced with XE, XS, XP, and XU. With modifier XE, this means a separate encounter and service that is distinct because it occurred during a different encounter. Modifier XP refers to a separate practitioner. It alerts claims handlers that the service is distinct because it was performed by a different provider. When a separate structure is involved, you must use modifier XS. This indicates that the service or procedure involves a separate structure or organ. Finally, with XU, the unusual non-overlapping services is considered. This modifier denotes the use of a service that does not overlap usual components of the main service. Use of Other Modifiers - Modifier 22 – This denotes increased procedural services, and it may be used with computed tomography (CT) codes when additional slices are required. - Modifier 24 – Specifies unrelated E&M service by the same physician during a postoperative time period. - Modifier 25 – Corresponds to a significant and separately identified E&M service by the same doctor on the same day as the other service or procedure. - Modifier 27 – Specifies increased procedural services. - Modifier 33 – Used for preventive services, and claims billed with this are not subject to specific ICD-10 inclusion and/or exclusion criteria. - Modifier 47 – Shows that anesthesia was given by the surgeon, and is not to be used with anesthesia codes. - Modifier 50 – Denotes a bilateral procedure. - Modifier 51 – Shows the claims handler that multiple procedures were performed. - Modifier 52 – Indicates reduced services, and it requires “by report” documentation for certain surgery codes. - Modifier 53 – Specifies a discontinued procedure, and it requires “by report” documentation. - Modifier 54 – This is a “surgical care only” code. - Modifier 55 – Used for postoperative management only. - Modifier 58 – Indicates a staged or related procedure or service by the same provider during the postoperative time period. It can be used with codes 15002 thru 15429 to address subsequent parts of the staged procedure. - Modifier 62 – Specifies that two surgeons were involved in the procedure. - Modifier 73 – Used by a hospital outpatient department or surgical center only, and it requires “by report” documentation. - Modifier 76 – Used when a repeat service or procedure is performed by the same physician. - Modifier 78 – Used when there is an unplanned return to the procedure room or surgical center by the same physician following the initial surgery/procedure.	4	20	1	0	0	0	0.43428	1	835
Methods \| Statistics \| Clinical \| Educational \| Industrial \| Professional items \| World psychology \| Biological: Behavioural genetics · Evolutionary psychology · Neuroanatomy · Neurochemistry · Neuroendocrinology · Neuroscience · Psychoneuroimmunology · Physiological Psychology · Psychopharmacology (Index, Outline) \|Classification and external resources\| Chest spiral CT scan with radiocontrast agent showing multiple filling defects of principal branches of the pulmonary arteries, due to acute and chronic pulmonary embolism. \|eMedicine\|\|med/1958 emerg/490 radio/582\| Pulmonary embolism (PE) is a blockage of the main artery of the lung or one of its branches by a substance that has travelled from elsewhere in the body through the bloodstream (embolism). Usually this is due to embolism of a thrombus (blood clot) from the deep veins in the legs, a process termed venous thromboembolism. A small proportion is due to the embolization of air, fat or amniotic fluid. The obstruction of the blood flow through the lungs and the resultant pressure on the right ventricle of the heart leads to the symptoms and signs of PE. The risk of PE is increased in various situations, such as cancer and prolonged bed rest. Symptoms of pulmonary embolism include difficulty breathing, chest pain on inspiration, and palpitations. Clinical signs include low blood oxygen saturation and cyanosis, rapid breathing, and a rapid heart rate. Severe cases of PE can lead to collapse, abnormally low blood pressure, and sudden death. Diagnosis is based on these clinical findings in combination with laboratory tests (such as the D-dimer test) and imaging studies, usually CT pulmonary angiography. Treatment is typically with anticoagulant medication, including heparin and warfarin. Severe cases may require thrombolysis with drugs such as tissue plasminogen activator (tPA) or may require surgical intervention via pulmonary thrombectomy. Signs and symptoms Symptoms of PE are sudden-onset dyspnea (shortness of breath), tachypnea (rapid breathing), chest pain of a "pleuritic" nature (worsened by breathing), cough and hemoptysis (coughing up blood). More severe cases can include signs such as cyanosis (blue discoloration, usually of the lips and fingers), collapse, and circulatory instability. About 15% of all cases of sudden death are attributable to PE. On physical examination, a pleural rub may be audible by stethoscope over affected areas of the lung. Strain on the right ventricle may be detected as a left parasternal heave, a loud pulmonary component of the second heart sound, raised jugular venous pressure, and more rarely leg swelling. The diagnosis of PE is based primarily on validated clinical criteria combined with selective testing because the typical clinical presentation (shortness of breath, chest pain) cannot be definitively differentiated from other causes of chest pain and shortness of breath. The decision to do medical imaging is usually based on clinical grounds, i.e. the medical history, symptoms and findings on physical examination, followed by an assessment of clinical probability. The most commonly used method to predict clinical probability, the Wells score, is a clinical prediction rule, whose use is complicated by multiple versions being available. In 1995, Wells et al. initially developed a prediction rule (based on a literature search) to predict the likelihood of PE, based on clinical criteria. The prediction rule was revised in 1998 This prediction rule was further revised when simplified during a validation by Wells et al. in 2000. In the 2000 publication, Wells proposed two different scoring systems using cutoffs of 2 or 4 with the same prediction rule. In 2001, Wells published results using the more conservative cutoff of 2 to create three categories. An additional version, the "modified extended version", using the more recent cutoff of 2 but including findings from Wells's initial studies were proposed. Most recently, a further study reverted to Wells's earlier use of a cutoff of 4 points to create only two categories. The Wells score: - clinically suspected DVT - 3.0 points - alternative diagnosis is less likely than PE - 3.0 points - tachycardia - 1.5 points - immobilization/surgery in previous four weeks - 1.5 points - history of DVT or PE - 1.5 points - hemoptysis - 1.0 points - malignancy (treatment for within 6 months, palliative) - 1.0 points - Score >6.0 - High (probability 59% based on pooled data) - Score 2.0 to 6.0 - Moderate (probability 29% based on pooled data) - Score <2.0 - Low (probability 15% based on pooled data) - Score > 4 - PE likely. Consider diagnostic imaging. - Score 4 or less - PE unlikely. Consider D-dimer to rule out PE. Early primary research has shown that in low/moderate suspicion of PE, a normal D-dimer level (shown in a blood test) is enough to exclude the possibility of thrombotic PE. This has been corroborated by a recent systematic review of studies of patients with low pre-test probability (PTP) of PE and negative D-dimer results that found the three month risk of thromboembolic events in patients excluded in this manner was 0.14%, with 95% confidence intervals from 0.05 to 0.41%, though this review was limited by its use of only one randomized-controlled clinical trial, the remainder of studies being prospective cohorts. When a PE is being suspected, a number of blood tests are done, in order to exclude important secondary causes of PE. This includes a full blood count, clotting status (PT, APTT, TT), and some screening tests (erythrocyte sedimentation rate, renal function, liver enzymes, electrolytes). If one of these is abnormal, further investigations might be warranted. Template:FixBunching The gold standard for diagnosing pulmonary embolism (PE) is pulmonary angiography. Pulmonary angiography is used less often due to wider acceptance of CT scans, which are non-invasive. - Non-invasive imaging CT pulmonary angiography (CTPA) is a pulmonary angiogram obtained using computed tomography (CT) with radiocontrast rather than right heart catheterization. Its advantages are clinical equivalence, its non-invasive nature, its greater availability to patients, and the possibility of identifying other lung disorders from the differential diagnosis in case there is no pulmonary embolism. Assessing the accuracy of CT pulmonary angiography is hindered by the rapid changes in the number of rows of detectors available in multidetector CT (MDCT) machines.. According to a cohort study, single-slice spiral CT may help diagnose detection among patients with suspected pulmonary embolism . In this study, the sensitivity was 69% and specificity was 84%. In this study which had a prevalence of detection was 32%, the positive predictive value of 67.0% and negative predictive value of 85.2% (click here to adjust these results for patients at higher or lower risk of detection). However, this study's results may be biased due to possible incorporation bias, since the CT scan was the final diagnostic tool in patients with pulmonary embolism. The authors noted that a negative single slice CT scan is insufficient to rule out pulmonary embolism on its own. A separate study with a mixture of 4 slice and 16 slice scanners reported a sensitivity of 83% and a specificity of 96%. This study noted that additional testing is necessary when the clinical probability is inconsistent with the imaging results. CTPA is non-inferior to VQ scanning, and identifies more emboli (without necessarily improving the outcome) compared to VQ scanning. Ventilation/perfusion scan (or V/Q scan or lung scintigraphy), which shows that some areas of the lung are being ventilated but not perfused with blood (due to obstruction by a clot). This type of examination is used less often because of the more widespread availability of CT technology, however, it may be useful in patients who have an allergy to iodinated contrast or in pregnancy due to lower radiation exposure than CT. - Low probability diagnostic tests/non-diagnostic tests Tests that are frequently done that are not sensitive for PE, but can be diagnostic. - Chest X-rays are often done on patients with shortness of breath to help rule-out other causes, such as congestive heart failure and rib fracture. Chest X-rays in PE are rarely normal, but usually lack signs that suggest the diagnosis of PE (e.g. Westermark sign, Hampton's hump). - Ultrasonography of the legs, also known as leg doppler, in search of deep venous thrombosis (DVT). The presence of DVT, as shown on ultrasonography of the legs, is in itself enough to warrant anticoagulation, without requiring the V/Q or spiral CT scans (because of the strong association between DVT and PE). This may be valid approach in pregnancy, in which the other modalities would increase the risk of birth defects in the unborn child. However, a negative scan does not rule out PE, and low-radiation dose scanning may be required if the mother is deemed at high risk of having pulmonary embolism. An electrocardiogram (ECG) is routinely done on patients with chest pain to quickly diagnose myocardial infarctions (heart attacks). An ECG may show signs of right heart strain or acute cor pulmonale in cases of large PEs - the classic signs are a large S wave in lead I, a large Q wave in lead III and an inverted T wave in lead III ("S1Q3T3"). This is occasionally (up to 20%) present, but may also occur in other acute lung conditions and has therefore limited diagnostic value. The most commonly seen signs in the ECG is sinus tachycardia, right axis deviation and right bundle branch block. Sinus tachycardia was however still only found in 8 - 69% of people with PE. In massive and submassive PE, dysfunction of the right side of the heart can be seen on echocardiography, an indication that the pulmonary artery is severely obstructed and the heart is unable to match the pressure. Some studies (see below) suggest that this finding may be an indication for thrombolysis. Not every patient with a (suspected) pulmonary embolism requires an echocardiogram, but elevations in cardiac troponins or brain natriuretic peptide may indicate heart strain and warrant an echocardiogram. The specific appearance of the right ventricle on echocardiography is referred to as the McConnell sign. This is the finding of akinesia of the mid-free wall but normal motion of the apex. This phenomenon has a 77% sensitivity and a 94% specificity for the diagnosis of acute pulmonary embolism. Combining tests into algorithms Recent recommendations for a diagnostic algorithm have been published by the PIOPED investigators; however, these recommendations do not reflect research using 64 slice MDCT. These investigators recommended: - Low clinical probability. If negative D-dimer, PE is excluded. If positive D-dimer, obtain MDCT and based treatment on results. - Moderate clinical probability. If negative D-dimer, PE is excluded. However, the authors were not concerned that a negative MDCT with negative D-dimer in this setting has an 5% probability of being false. Presumably, the 5% error rate will fall as 64 slice MDCT is more commonly used. If positive D-dimer, obtain MDCT and based treatment on results. - High clinical probability. Proceed to MDCT. If positive, treat, if negative, additional tests are needed to exclude PE. Pulmonary Embolism Rule-out Criteria The Pulmonary Embolism Rule-out Criteria, or PERC rule, helps assess patients in whom pulmonary embolism is suspected, but unlikely. Unlike the Wells Score and Geneva score, which are clinical prediction rules intended to risk stratify patients with suspected PE, the PERC rule is designed to rule-out risk of PE in patients when the physician has already stratified them into a low-risk category. Patients in this low risk category without any of these criteria may undergo no further diagnostic testing for PE: Hypoxia - Sa02 <95%, unilateral leg swelling, hemoptysis, prior DVT or PE, recent surgery or trauma, age >50, hormone use, tachycardia. The rationale behind this decision is that further testing (specifically CT angiogram of the chest) may cause more harm (from radiation exposure and contrast dye) than the risk of PE. The PERC rule has a sensitivity of 97.4% and specificity of 21.9% with a false negative rate of 1.0% (16/1666). - Main article: anticoagulant In most cases, anticoagulant therapy is the mainstay of treatment. Heparin, low molecular weight heparins (such as enoxaparin and dalteparin), or fondaparinux is administered initially, while warfarin, acenocoumarol, or phenprocoumon therapy is commenced (this may take several days, usually while the patient is in hospital). Low molecular weight heparin may reduce bleeding among patients with pulmonary embolism as compared to heparin according to a systematic review of randomized controlled trials by the Cochrane Collaboration. The relative risk reduction was 40.0%. For patients at similar risk to those in this study (2.0% had bleeding when not treated with low molecular weight heparin), this leads to an absolute risk reduction of 0.8%. 125.0 patients must be treated for one to benefit (number needed to treat = 125.0. Click here to adjust these results for patients at higher or lower risk of bleeding). It may however, be possible to treat low risk patients as outpatients. An ongoing clinical trial is looking into the safety of this practice on the basis of evidence summarized by a recent systematic review of observational studies. This systematic review examined outpatient treatment of symptomatic pulmonary embolism and found that death from all causes ranged from 5 to 44%, and incidence of other complications such as recurrent thromboembolism ranged from 1 to 9%, and major bleeding from 0 to 4%. Warfarin therapy often requires frequent dose adjustment and monitoring of the INR. In PE, INRs between 2.0 and 3.0 are generally considered ideal. If another episode of PE occurs under warfarin treatment, the INR window may be increased to e.g. 2.5-3.5 (unless there are contraindications) or anticoagulation may be changed to a different anticoagulant e.g. low molecular weight heparin. In patients with an underlying malignancy, therapy with a course of low molecular weight heparin may be favored over warfarin based on the results of the CLOT trial. Similarly, pregnant women are often maintained on low molecular weight heparin to avoid the known teratogenic effects of warfarin, especially in the early stages of pregnancy. People are usually admitted to hospital in the early stages of treatment, and tend to remain under inpatient care until INR has reached therapeutic levels. Increasingly, low-risk cases are managed on an outpatient basis in a fashion already common in the treatment of DVT. Warfarin therapy is usually continued for 3–6 months, or "lifelong" if there have been previous DVTs or PEs, or none of the usual risk factors is present. An abnormal D-dimer level at the end of treatment might signal the need for continued treatment among patients with a first unprovoked pulmonary embolus. - Main article: Thrombolysis Massive PE causing hemodynamic instability (shock and/or hypotension, defined as a systolic blood pressure <90 mmHg or a pressure drop of 40 mmHg for>15 min if not caused by new-onset arrhythmia, hypovolemia or sepsis) is an indication for thrombolysis, the enzymatic destruction of the clot with medication. It is the best available medical treatment in this situation and is supported by clinical guidelines. The use of thrombolysis in non-massive PEs is still debated. The aim of the therapy is to dissolve the clot, but there is an attendant risk of bleeding or stroke. The main indication for thrombolysis is in submassive PE where right ventricular dysfunction can be demonstrated on echocardiography, and the presence of visible thrombus in the atrium. Surgical management of acute pulmonary embolism (pulmonary thrombectomy) is uncommon and has largely been abandoned because of poor long-term outcomes. However, recently, it has gone through a resurgence with the revision of the surgical technique and is thought to benefit selected patients. Inferior vena cava filter - Main article: inferior vena cava filter If anticoagulant therapy is contraindicated and/or ineffective, or to prevent new emboli from entering the pulmonary artery and combining with an existing blockage, an inferior vena cava filter may be implanted. Mortality from untreated PE is said to be 26%. This figure comes from a trial published in 1960 by Barrit and Jordan, which compared anticoagulation against placebo for the management of PE. Barritt and Jordan performed their study in the Bristol Royal Infirmary in 1957. This study is the only placebo controlled trial ever to examine the place of anticoagulants in the treatment of PE, the results of which were so convincing that the trial has never been repeated as to do so would be considered unethical. That said, the reported mortality rate of 26% in the placebo group is probably an overstatement, given that the technology of the day may have detected only severe PEs. Prognosis depends on the amount of lung that is affected and on the co-existence of other medical conditions; chronic embolisation to the lung can lead to pulmonary hypertension. After a massive PE, the embolus must be resolved somehow if the patient is to survive. In thrombotic PE, the blood clot may be broken down by fibrinolysis, or it may be organized and recanalized so that a new channel forms through the clot. Blood flow is restored most rapidly in the first day or two after a PE. Improvement slows thereafter, and some defects may remain permanently. There is controversy over whether or not small subsegmental PEs need to be treated at all and some evidence exists that patients with subsegmental PEs may do well without treatment. The PESI and Geneva prediction rules can estimate mortality and so may guide selection of patients who can be considered for outpatient therapy. After a first PE, the search for secondary causes is usually brief. Only when a second PE occurs, and especially when this happens while still under anticoagulant therapy, a further search for underlying conditions is undertaken. This will include testing ("thrombophilia screen") for Factor V Leiden mutation, antiphospholipid antibodies, protein C and S and antithrombin levels, and later prothrombin mutation, MTHFR mutation, Factor VIII concentration and rarer inherited coagulation abnormalities. The most common sources of embolism are proximal leg deep venous thrombosis (DVTs) or pelvic vein thromboses. Any risk factor for DVT also increases the risk that the venous clot will dislodge and migrate to the lung circulation, which happens in up to 15% of all DVTs. The conditions are generally regarded as a continuum termed venous thromboembolism (VTE). The development of thrombosis is classically due to a group of causes named Virchow's triad (alterations in blood flow, factors in the vessel wall and factors affecting the properties of the blood). Often, more than one risk factor is present. - Alterations in blood flow: immobilization (after surgery, injury or long-distance air travel), pregnancy (also procoagulant), obesity (also procoagulant) - Factors in the vessel wall: of limited direct relevance in VTE - Factors affecting the properties of the blood (procoagulant state): - Oestrogen-containing hormonal contraception - Genetic thrombophilia (factor V Leiden, prothrombin mutation G20210A, protein C deficiency, protein S deficiency, antithrombin deficiency, hyperhomocysteinemia and plasminogen/fibrinolysis disorders). - Acquired thrombophilia (antiphospholipid syndrome, nephrotic syndrome, paroxysmal nocturnal hemoglobinuria) Once anticoagulation is stopped, the risk of a fatal pulmonary embolism is 0.5% per year. - ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Goldhaber SZ (2005). "Pulmonary thromboembolism" Kasper DL, Braunwald E, Fauci AS, et al. Harrison's Principles of Internal Medicine, 16th, 1561–65, New York, NY: McGraw-Hill. - ↑ Stein PD, Afzal A, Henry JW, Villareal CG (January 2000). Fever in acute pulmonary embolism. Chest 117 (1): 39–42. - ↑ 3.0 3.1 Wells PS, Hirsh J, Anderson DR, Lensing AW, Foster G, Kearon C, Weitz J, D'Ovidio R, Cogo A, Prandoni P (1995). Accuracy of clinical assessment of deep-vein thrombosis. Lancet 345 (8961): 1326–30. - ↑ 4.0 4.1 Wells PS, Ginsberg JS, Anderson DR, Kearon C, Gent M, Turpie AG, Bormanis J, Weitz J, Chamberlain M, Bowie D, Barnes D, Hirsh J (1998). Use of a clinical model for safe management of patients with suspected pulmonary embolism. Ann Intern Med 129 (12): 997–1005. - ↑ 5.0 5.1 5.2 5.3 5.4 Wells P, Anderson D, Rodger M, Ginsberg J, Kearon C, Gent M, Turpie A, Bormanis J, Weitz J, Chamberlain M, Bowie D, Barnes D, Hirsh J (2000). Derivation of a simple clinical model to categorize patients probability of pulmonary embolism: increasing the models utility with the SimpliRED D-dimer.. Thromb Haemost 83 (3): 416–20. - ↑ 6.0 6.1 Wells PS, Anderson DR, Rodger M, Stiell I, Dreyer JF, Barnes D, Forgie M, Kovacs G, Ward J, Kovacs MJ (2001). Excluding pulmonary embolism at the bedside without diagnostic imaging: management of patients with suspected pulmonary embolism presenting to the emergency department by using a simple clinical model and d-dimer. Ann Intern Med 135 (2): 98–107. - ↑ Sanson BJ, Lijmer JG, Mac Gillavry MR, Turkstra F, Prins MH, Büller HR (2000). Comparison of a clinical probability estimate and two clinical models in patients with suspected pulmonary embolism. ANTELOPE-Study Group. Thromb. Haemost. 83 (2): 199–203. - ↑ 8.0 8.1 van Belle A, Büller H, Huisman M, Huisman P, Kaasjager K, Kamphuisen P, Kramer M, Kruip M, Kwakkel-van Erp J, Leebeek F, Nijkeuter M, Prins M, Sohne M, Tick L (2006). Effectiveness of managing suspected pulmonary embolism using an algorithm combining clinical probability, D-dimer testing, and computed tomography. JAMA 295 (2): 172–9. - ↑ Roy PM, Meyer G, Vielle B, Le Gall C, Verschuren F, Carpentier F, Leveau P, Furber A (2006). Appropriateness of diagnostic management and outcomes of suspected pulmonary embolism. Ann. Intern. Med. 144 (3): 157–64. - ↑ Neff MJ (2003). ACEP releases clinical policy on evaluation and management of pulmonary embolism. American family physician 68 (4): 759–60. - ↑ Yap KS, Kalff V, Turlakow A, Kelly MJ (2007). A prospective reassessment of the utility of the Wells score in identifying pulmonary embolism. Med. J. Aust. 187 (6): 333–6. - ↑ 12.0 12.1 12.2 12.3 Stein PD, Woodard PK, Weg JG, Wakefield TW, Tapson VF, Sostman HD, Sos TA, Quinn DA, Leeper KV, Hull RD, Hales CA, Gottschalk A, Goodman LR, Fowler SE, Buckley JD (2007). Diagnostic pathways in acute pulmonary embolism: recommendations of the PIOPED II Investigators. Radiology 242 (1): 15–21. - ↑ Bounameaux H, de Moerloose P, Perrier A, Reber G (1994). Plasma measurement of D-dimer as diagnostic aid in suspected venous thromboembolism: an overview. Thromb. Haemost. 71 (1): 1–6. - ↑ Carrier, M.; Righini, M.; Djurabi, R.K.; Huisman, M.V.; Perrier, A.; Wells, P.S.; Rodger, M.; Wuillemin, W.A.; et al. (2009), "VIDAS D-dimer in combination with clinical pre-test probability to rule out pulmonary embolism. A systematic review of management outcome studies", Thromb Haemost 101 (5): 886–92, PMID 19404542 - ↑ Schaefer-Prokop C, Prokop M (2005). MDCT for the diagnosis of acute pulmonary embolism. European radiology 15 Suppl 4: D37–41. - ↑ (2005)Accuracy of single-detector spiral CT in the diagnosis of pulmonary embolism: a prospective multicenter cohort study of consecutive patients with abnormal perfusion scintigraphy.. Journal of thrombosis and haemostasis : JTH. - ↑ 17.0 17.1 Stein PD, Fowler SE, Goodman LR, et al. (2006). Multidetector computed tomography for acute pulmonary embolism. N. Engl. J. Med. 354 (22): 2317–27. - ↑ Anderson DR, Kahn SR, Rodger MA et al. (2007). Computed tomographic pulmonary angiography vs ventilation-perfusion lung scanning in patients with suspected pulmonary embolism. JAMA 298 (23): 2743–53. - ↑ Scarsbrook AF, Gleeson FV (2007). Investigating suspected pulmonary embolism in pregnancy. BMJ 334 (7590): 418–9. - ↑ Worsley D, Alavi A, Aronchick J, Chen J, Greenspan R, Ravin C (1993). Chest radiographic findings in patients with acute pulmonary embolism: observations from the PIOPED Study.. Radiology 189 (1): 133–6. - ↑ McGinn S, White PD (1935). Acute cor pulmonale resulting from pulmonary embolism. J Am Med Assoc 104: 1473–80. - ↑ Rodger M, Makropoulos D, Turek M, et al. (October 2000). Diagnostic value of the electrocardiogram in suspected pulmonary embolism. Am. J. Cardiol. 86 (7): 807–9, A10. - ↑ Amal Mattu; Deepi Goyal; Barrett, Jeffrey W.; Joshua Broder; DeAngelis, Michael; Peter Deblieux; Gus M. Garmel; Richard Harrigan; David Karras; Anita L'Italien; David Manthey (2007). Emergency medicine: avoiding the pitfalls and improving the outcomes, 10, Malden, Mass: Blackwell Pub./BMJ Books. - ↑ Kucher N, Goldhaber SZ (2003). Cardiac biomarkers for risk stratification of patients with acute pulmonary embolism. Circulation 108 (18): 2191–4. - ↑ McConnell MV, Solomon SD, Rayan ME, Come PC, Goldhaber SZ, Lee RT (1996). Regional right ventricular dysfunction detected by echocardiography in acute pulmonary embolism. Am. J. Cardiol. 78 (4): 469–73. - ↑ Kline, Jeffrey A (2004). Clinical criteria to prevent unnecessary diagnostic testing in emergency department patients with suspected pulmonary embolism. Journal of Thrombosis and Haemostasis 2 (8): 1247–1255. - ↑ Kline, Jeffrey A (2008). Prospective multicenter evaluation of the pulmonary embolism rule-out criteria. Journal of Thrombosis and Haemostasis 6 (5): 772–780. - ↑ (2004)Fixed dose subcutaneous low molecular weight heparins versus adjusted dose unfractionated heparin for venous thromboembolism.. Cochrane database of systematic reviews (Online). ACPJC Review - ↑ BestBets: Outpatient treatment of pulmonary embolism. URL accessed on December 6, 2008. - ↑ Safety Study of Outpatient Treatment for Pulmonary Embolism - Full Text View - ClinicalTrials.gov. URL accessed on December 6, 2008. - ↑ Squizzato, A.; Galli, M.; Dentali, F.; Ageno, W. (2009), "Outpatient treatment and early discharge of symptomatic pulmonary embolism: a systematic review", Eur Respir J 33 (5): 1148–55, PMID 19407049 - ↑ Lee AY, Levine MN, Baker RI, Bowden C, Kakkar AK, Prins M, Rickles FR, Julian JA, Haley S, Kovacs MJ, Gent M (2003). Low-molecular-weight heparin versus a coumarin for the prevention of recurrent venous thromboembolism in patients with cancer.. N Engl J Med 349 (2): 146–53. - ↑ Davies CW, Wimperis J, Green ES, et al. (October 2007). Early discharge of patients with pulmonary embolism: a two-phase observational study. Eur. Respir. J. 30 (4): 708–14. - ↑ Palareti G, Cosmi B, Legnani C, et al. (2006). D-dimer testing to determine the duration of anticoagulation therapy. N. Engl. J. Med. 355 (17): 1780–9. - ↑ (June 2003)British Thoracic Society guidelines for the management of suspected acute pulmonary embolism. Thorax 58 (6): 470–83. - ↑ Torbicki A, Perrier A, Konstantinides S, et al. (September 2008). Guidelines on the diagnosis and management of acute pulmonary embolism: the Task Force for the Diagnosis and Management of Acute Pulmonary Embolism of the European Society of Cardiology (ESC). Eur. Heart J. 29 (18): 2276–315. - ↑ Hirsh J, Guyatt G, Albers GW, Harrington R, Schünemann HJ (June 2008). Executive summary: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th Edition). Chest 133 (6 Suppl): 71S–109S. - ↑ Dong B, Jirong Y, Liu G, Wang Q, Wu T (2006). Thrombolytic therapy for pulmonary embolism. Cochrane Database Syst Rev (2): CD004437. - ↑ Goldhaber SZ (2004). Pulmonary embolism. Lancet 363 (9417): 1295–305. - ↑ Augustinos P, Ouriel K (2004). Invasive approaches to treatment of venous thromboembolism. Circulation 110 (9 Suppl 1): I27–34. - ↑ Decousus H, Leizorovicz A, Parent F, Page Y, Tardy B, Girard P, Laporte S, Faivre R, Charbonnier B, Barral F, Huet Y, Simonneau G (1998). A clinical trial of vena caval filters in the prevention of pulmonary embolism in patients with proximal deep-vein thrombosis. Prévention du Risque d'Embolie Pulmonaire par Interruption Cave Study Group. N Engl J Med 338 (7): 409–15. - ↑ Barritt DW, Jordan SC (1960). Anticoagulant drugs in the treatment of pulmonary embolism: a controlled trial.. Lancet 1 (7138): 1309–12. - ↑ (17 October 1970)Resolution of Pulmonary Embolism. British Medical Journal 4 (5728): 135–139. - ↑ Le Gal G, Righini M, Parent F, van Strijen M, Couturaud F (2006). Diagnosis and management of subsegmental pulmonary embolism. J Thromb Haemost 4 (4): 724–31. - ↑ Perrier A, Bounameaux H (2006). Accuracy or outcome in suspected pulmonary embolism. N Engl J Med 354 (22): 2383–5. - ↑ Jiménez D, Yusen RD, Otero R, et al. (2007). Prognostic models for selecting patients with acute pulmonary embolism for initial outpatient therapy. Chest 132 (1): 24–30. - ↑ White RH (October 2008). Risk of fatal pulmonary embolism was 0.49 per 100 person-years after discontinuing anticoagulant therapy for venous thromboembolism. Evid Based Med 13 (5): 154. Pathology of respiratory system (J, 460-519) \|Acute upper respiratory infections\| \|Influenza and Pneumonia\| \|Other acute lower respiratory infections\| \|Other diseases of upper respiratory tract\| \|Chronic lower respiratory diseases\| \|Lung diseases due to external agents\| \| Other, principally affecting\| \| Suppurative and necrotic conditions\| of lower respiratory tract \|This page uses Creative Commons Licensed content from Wikipedia (view authors).\|	2	43	0	0	0	3	0.95553	3	7,481
Chronic fatigue syndrome Chronic Fatigue Syndrome Chronic fatigue syndrome (CFS) is a complicated and poorly understood disease that causes persistent and profound fatigue. CFS is also known as systemic exertional intolerance disease (SEID) or myalgic encephalomyelitis (ME/CFS). The causes of CFS are unknown. Symptoms of CFS can come and go in cycles. People may feel better for a while, and then have a relapse. The main symptoms of CFS are: People with CFS may also experience many other symptoms, including problems with thinking and memory, worsening of symptoms when standing or sitting up, pain, and sensitivities to external stimuli. Treatment for CMS focuses on symptom relief. Cognitive behavioral therapy (CBT) and graded exercise therapy (GET) are two approaches that have been advocated for people with CFS and are supported by a few clinical studies. While these may work well for some patients, their appropriateness as a general indication in CFS has recently been under scrutiny. In 2017, the CDC decided to remove CBT and GET from the list of recommended therapeutic approaches for people with CFS/ME. New Name and Diagnostic Criteria In 2015, the Institute of Medicine (IOM) recommended systemic exertion intolerance disease (SEID) as the new name for CFS. The IOM's goal was to have the name reflect some of the disease's core symptoms, and to hopefully remove some of the stigma that surrounds this challenging condition. Chronic fatigue syndrome (CFS) is a serious and complex medical condition characterized by a cluster of systemic symptoms that affect physical and cognitive functioning. The hallmark symptom is persistent and profound fatigue, which often worsens after physical or mental exertion. This fatigue lasts for more than 6 months, impairs normal activities, and has no identifiable medical or psychological factors to account for it. Fatigue is just one symptom of the condition. In addition to fatigue, people usually experience other problems such as unrefreshing sleep, difficulty with memory or concentration ("brain fog"), muscle and joint pain, and worsening of symptoms when in an upright position. CFS is a serious condition that has a profound impact on the people who have it. Many people with CFS are forced to curtail or find new coping strategies to handle normal daily activities. About 1 in 4 people with CFS are so severely disabled that they cannot get out of bed or leave their home. Symptoms can come and go in cycles, and even when people feel better, they may experience a relapse triggered by exertion or an unknown cause. There are a number of challenges, confusions, and controversies surrounding CFS, including defining its possible causes, exact symptoms, diagnosis, and treatment. There is even disagreement about what to call the condition. The term "myalgic encephalomyelitis" or "ME" is sometimes added to CFS, with the disease being abbreviated as ME/CFS or CFS/ME. However, some researchers believe that ME is not appropriate because it implies brain inflammation (encephalomyelitis) and muscle pain (myalgia), which are not its main symptoms. Many people feel that the term CFS is not accurate either, because it contributes to belittling misunderstandings that undercut the serious nature of this condition. People with CFS are not lazy, neurotic, unmotivated, or suffering from the "yuppie flu." They struggle to live with a bewildering illness that is not completely understood by medical practitioners, much less the general public. In 2015, the Institute of Medicine (IOM) proposed replacing ME/CFS with a new name: systemic exertion intolerance disease (SEID). The IOM's rationale for this name change is that SEID focuses on a core symptom of post-exertional malaise, which involves the entire body. The name also emphasizes that this condition is a "real" disease, not a psychological disorder. However, the term SEID has not been universally embraced. Some people feel that it is still open to distortion and misrepresentation. Until experts agree upon the terminology, CFS may remain more commonly recognized as the name for this condition, and will be the term used in this report. The exact causes of CFS are not known. Researchers think that infection, immune system problems, genetics, and the effects of stress on hormone production may play roles in different people. There may also be a genetic component. It is likely that CFS is due to a combination of factors rather than one single cause. There is also no standard way that CFS develops. Some people have an abrupt onset of symptoms after an infectious disease or psychiatric problem. Other people experience a slowly progressive emergence of symptoms, and have no history of mental health issues. It is not clear what sequence of events actually leads to the fatigue and other symptoms of this disorder. No primary cause has been found that explains all cases of CFS, and no blood tests or brain scans can definitively diagnose the condition. Researchers have focused on the possibility that a virus or other type of infection may trigger CFS. Many people report having a flu-like illness or bacterial infection prior to developing CFS. Possible viral causes include the Epstein-Barr virus (EBV) and herpesvirus type 6 (HHV-6). EBV is related to mononucleosis, which can cause severe fatigue. However, scientists have been unable to establish a causal link between EBV and CFS. Researchers are also investigating whether CFS may be associated with the reactivation of a latent infection. Again, there is no conclusive evidence to support this theory. Immune System Dysfunction A number of studies have suggested that there may be problems of regulation of the immune system in people with CFS. Researchers are investigating whether over- or under-activation of T cells, B cells, and natural killer (NK) cells, which help regulate the immune system, may play a role in CFS. It is not yet clear whether people with CFS have the autoantibodies (antibodies that attack the body's own tissues) found in people with autoimmune disorders. There is no indication at the current time to use immune system modulating therapies to treat CFS. Many people with CFS have allergies or sensitivities to foods, pollen, molds, metals (such as nickel or mercury), or other substances. One theory is that allergens, like viral infections, may trigger a cascade of immune abnormalities that lead to CFS. However, most people with allergies do not have CFS. Central Nervous System Abnormalities Abnormal levels of certain chemicals in the brain system known as the hypothalamus-pituitary-adrenal (HPA) axis have been proposed as a cause of CFS. This system controls important functions, including sleep, the stress response, and depression. Some people with CFS appear to have imbalances in hormones controlled by the HPA axis, such as cortisol. Autonomic Nervous System Abnormalities Researchers are investigating links between autonomic system dysfunction and CFS. The autonomic nervous system controls involuntary actions, including regulating blood pressure. Some people who have CFS also have symptoms of a condition known as neurally mediated hypotension (NMH). NMH causes a dramatic drop in blood pressure when a person stands up, for even as few as 10 minutes. Its immediate effects can be light-headedness, nausea, and fainting. A related condition experienced by some people with CFS is called postural orthostatic tachycardia syndrome (POTS). POTS causes a rapid increase in blood pressure when changing from a lying down to a standing up position. The Institute of Medicine estimates that up to 2.5 million Americans have CFS, but millions more may have the disease and not yet been diagnosed. CFS usually affects adults in their 30s, 40s, or 50s, but it can develop in people of all ages from children and adolescents to the elderly. CFS is more common in women than men. CFS sometimes occurs among members of the same family, which implies there may be a genetic link. CFS is not contagious. There is some evidence that stress, or difficulty managing stress, may trigger CFS in people who are at risk for the disease because of genetic and other underlying factors. Stress may trigger the condition through its effects on the central nervous system, immune system, and neuroendocrine system (which is related to both nerves and hormones.) Studies have not found any consistent association between psychological, emotional, or personality factors and CFS. A number of conditions overlap or coexist with chronic fatigue syndrome and have similar symptoms. They include fibromyalgia, irritable bowel syndrome, and sleep disorders. People with CFS often report extreme sensitivities and physical reactions to environmental chemicals, such as those found in perfumes, cigarette smoke, or paint fumes. There is controversy and disagreement surrounding "multiple chemical sensitivities" and whether these symptoms constitute a real disorder. The main symptoms of CFS are: Other symptoms may include: Additional symptoms that have been associated with CFS include abdominal bloating, urinary problems, alcohol intolerance, and sensitivity to light and loud noise. It is very difficult to diagnose chronic fatigue syndrome. Diagnosis focuses on symptom criteria, especially unexplained and profound fatigue that has lasted at least 6 months. The symptoms of CFS tend to come on suddenly in most individuals affected by the condition. Personal and Medical History The health care provider will take a careful personal and family medical history, and perform a thorough physical examination and psychological assessment. The provider will ask questions such as: The provider may also ask about any changes in weight or recent illnesses. You should tell your provider about any drugs you are taking, including over-the-counter medications, and vitamin, herbal, or dietary supplements. You may be asked to keep a diary for several weeks to record your activities and symptoms. No specific laboratory test can diagnose CFS. The following tests may be used to rule out other conditions that can cause persistent fatigue: If any test is abnormal, it is not useful for diagnosing CFS specifically, and the provider should look for other possible causes suggested by the positive test (such as decreased thyroid function.) Other tests for CFS may include: Ruling Out Other Conditions Other conditions can mimic symptoms of CFS. They include: CFS remains poorly understood and many people with the disease face challenges finding good care. It is important to recognize that there is no cure for CFS. As scientific understanding of the disease evolves, researchers hope to discover new treatments that address the disease systemically. Current treatment focuses on relieving symptoms and helping people self-manage the condition. You should work with your provider to develop a treatment plan that addresses your specific symptoms and concerns. It is best to begin treatment by focusing on the symptoms that cause you the most problems. Treatment approaches by symptom may include. Fatigue and postexertional malaise: Emotional and Mental Health Problems: People with CFS often experience post-exertional malaise after physical activity, which can last days to weeks. Creating an individualized plan balancing activity with appropriate periods of rest can help improve quality of life. Activity Pacing (Adaptive Pacing Therapy) Try to plan a daily schedule that allows you to spread out and accomplish a certain number of activities but also allows time for adequate rest. People with CFS often find that doing too much at once can worsen symptoms and exhaustion. A strategy that allows you to plan daily rest breaks, and find a rhythm that works for your energy level, will help you feel more in control. When you begin activity pacing (also called adaptive pacing therapy), you may find it helpful to keep a diary that tracks your activities, rest, sleep, and symptoms so that you can identify patterns and set goals. Activity pacing should involve: The goals of CBT include improving a person's ability to deal with stressful situations, and better manage their illness, as well as manage sleep problems and regulate activity levels. CBT is and has been extensively used to manage CFS, however questions have been raised regarding its effectiveness. As a consequence, in 2017 the CDC has removed CBT from its list of recommended therapeutic approaches for CFS/ME. Graded Exercise Therapy Graded exercise therapy (GET) involves slowly increasing the duration and intensity of exercise over time. In general, people start with about 3 to 5 minutes of exercise, and then gradually increase activity over the course of several weeks. GET does not work for all people with CFS and may worsen the condition in some. Some people experience profound fatigue after even mild or moderate exercise. Other people cannot exercise at all. Due to concerns over its effectiveness, in 2017, the CDC has removed GET from its list of recommended treatment approaches for CFS/ME. Many specialists advise against pursuing aerobic exercise or any exercise that overly increases heart rate. Your provider may suggest that you wear a heart rate monitor while exercising. Recommended types of exercise include yoga, tai chi, and resistance and weight training. People with CFS appear to have problems with aerobic metabolism, and must be careful not to exercise beyond their threshold. Over-exercise can worsen symptoms. For those who follow a GET approach, it is important to gradually build up to a level that is appropriate for you. Sleep hygiene involves simple self-help measures and behavioral changes that can help improve sleep. These include: Relaxation and stress-reduction techniques can be helpful. They include: There is no evidence that specific foods or restrictive diets influence CFS. It is best to take a commonsense approach to a healthy diet that includes: Discuss with your provider whether you should take a multivitamin supplement. Your provider may recommend a specific vitamin supplement if you have deficiencies in vitamin D or vitamin B12. Only use the recommended dose for vitamins: megadoses can be dangerous. Herbs and Dietary Supplements Many people with CFS turn to herbal and so-called "natural" remedies but there is no scientific evidence that they are effective. Be wary of supplements advertised as "immune system boosters" because they may contain unlisted steroids or pharmaceutical products. If you choose herbal remedies, stick to safe and simple ones such as peppermint or ginger tea for stomach problems and relaxation. Be aware that the FDA does not regulate herbal remedies or dietary supplements. The amounts of the active ingredients in these remedies may not always match what is claimed on the label. Be sure to let your provider know of any herbs or supplements you are taking. Some of these products may interact with medications. Strong, supportive relationships with family and friends are important. Attending support groups in which you share experiences with others who have CFS can be very helpful for sharing your concerns and strategies, and improving your coping abilities. No medications are specifically approved to treat CFS. However, some medications may be useful for pain, sleep, or other symptoms. People with CFS are often very sensitive to drugs, so try to limit medication use as much as possible and use the lowest effective dose. Nonsteroidal anti-inflammatory drugs (NSAIDs) NSAIDs are common non-prescription pain relievers. They include aspirin, ibuprofen (Motrin, Advil), and naproxen (Aleve), and COX-2 inhibitors. Daily use of NSAIDs can increase the risk for stomach bleeding and ulcers. Talk to your provider before taking these medicines if you have kidney disease or heart disease. Your provider should be aware if you take these medicines on most days. Nonprescription medications for sleep usually include the antihistamine diphenhydramine (Benadryl). Antihistamines can be effective when occasionally used, but in the long-term can cause problems with rebound insomnia. Non-Benodiazepine Sedative Hypnotics Are the preferred prescription medications for treating insomnia. They include zolpidem (Ambien), zaleplon (Sonata), and eszopiclone (Lunesta). Newer types of sedative hypnotics include ramelteon (Rozerem) and suvorexant (Belsomra). It is important that your provider explain the risks of these drugs and the precautions you need to take. These drugs are usually prescribed for short-term use and should be taken at the lowest possible dose because they can affect next-day mental alertness and physical coordination. You should never use alcohol before taking these drugs. Long-term use of these drugs may cause problems with short-term memory. A supplement that is a synthetic version of a hormone found in the body that is associated with regulating the circadian rhythms associated with sleep. Melatonin is a dietary supplement; therefore, it does not require FDA approval. There are no consistent standards on melatonin doses. General recommendations are to take 0.3 mg to 1 mg about 90 minutes before going to sleep. Taking higher doses may disrupt sleep and may cause daytime sleepiness, headaches, dizziness, nausea, and stomach cramps. Other types of medications, such as tricyclic antidepressants or anticonvulsants, may be used to treat sleep problems, as well as pain. Antidepressants known as tricyclics affect brain chemicals that are involved in managing pain. The tricyclic antidepressant amitriptyline (Elavil) is sometimes prescribed to treat pain and sleep problems associated with CFS. Other tricyclics include doxepin (Sinequan, generic), desipramine (Norpramin), nortriptyline (Pamelor), clomipramine (Anafranil), and imipramine (Tofranil). Tricyclics for CFS pain and sleep management are given at lower doses than when used to treat depression. Selective Serotonin-Reuptake Inhibitors (SSRIs), Selective Serotonin and Norepinephrine Reuptake Inhibitors (SNRI), and Seratonin Antagonist and Reuptake Inhibitors (SARI) SSRIs may be helpful for people with CFS who experience significant depression. These drugs include fluoxetine (Prozac), sertraline (Zoloft), paroxetine (Paxil, Pexeva), citalopram (Celexa) and escitalopram (Lexapro). Duloxetine (Cymbalta) and venlafaxine (Effexor) are antidepressants classified as SNRIs because they affect both neurotransmitters. SSRIs and SNRIs should not be taken with tricyclics, because the combination may cause dangerous side effects. A tetracyclic antidepressant that works as a SARI. Trazodone is often prescribed for sleep problems. Other Drugs Being Investigated for CFS Rituximab (Rituxan) is a biologic drug used to treat the autoimmune disease rheumatoid arthritis and certain cancers. The drug targets and blocks the activation of certain type of B cells. A small early-stage trial investigated rituximab's effectiveness in treating CFS. Results suggest that the drug may improve fatigue and other symptoms in some people with the disease. The results are preliminary and further study is needed before it can be determined if rituximab is likely to be helpful in treating CFS. In 2016, Rintatolimod (Ampligen) an antiviral and immunomodulatory agent, was approved for the treatment of chronic fatigue syndrome in Argentina. It is also available now in Canada and Europe, but is still not approved for use in the United States. It is the only drug to receive an approval for the treatment of chronic fatigue syndrome anywhere in the world. Brigden A, Loades M, Abbott A, Bond-Kendall J, Crawley E. Practical management of chronic fatigue syndrome or myalgic encephalomyelitis in childhood. Arch Dis Child. 2017;102(10):981-986. PMID: 28659269 www.ncbi.nlm.nih.gov/pubmed/28659269. Castro-Marrero J, Sáez-Francàs N, Santillo D, Alegre J. Treatment and management of chronic fatigue syndrome/myalgic encephalomyelitis: all roads lead to Rome. Br J Pharmacol. 2017;174(5):345-369. PMID: 28052319 www.ncbi.nlm.nih.gov/pubmed/28052319. Clauw DJ. Fibromyalgia, chronic fatigue syndrome, and myofascial pain. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 258. Collatz A, Johnston SC, Staines DR, Marshall-Gradisnik SM. A Systematic systematic review of drug therapies for chronic fatigue syndrome/myalgic encephalomyelitis. Clin Ther. 2016;38(6):1263.e9-1271.e9. PMID: 27229907 www.ncbi.nlm.nih.gov/pubmed/27229907. Committee on the Diagnostic Criteria for Myalgic Encephalomyelitis/Chronic Fatigue Syndrome; Board on the Health of Select Populations; Institute of Medicine. Beyond myalgic encephalomyelitis/chronic fatigue syndrome: redefining an illness. National Academies Press, Washington, DC; 2015. PMID: 25695122 www.ncbi.nlm.nih.gov/pubmed/25695122. Crawley E. Pediatric chronic fatigue syndrome: current perspectives. Pediatric Health Med Ther. 2018;9:27-33. PMID: 29722371 www.ncbi.nlm.nih.gov/pubmed/29722371. Dreher M, Murray MT. Chronic fatigue syndrome. In: Pizzorno JE, Murray MT, eds. Textbook of Natural Medicine. 4th ed. St Louis, MO: Elsevier Churchill Livingstone; 2013:chap 157. Engleberg NC. Chronic fatigue syndrome (systemic exertion intolerance disease). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 130. International Association for Chronic Fatigue Syndrome/Myalgic Encephalomyelitis website. Chronic fatigue syndrome/Myalgic encephalomyelitis: A primer for clinical practitioners. 2014 Edition. www.iacfsme.org/assets/docs/PrimerFinal.pdf. Accessed March 10, 2020. Jason LA, Brown A, Evans M, Sunnquist M, Newton JL. Contrasting chronic fatigue syndrome versus myalgic encephalomyelitis/chronic fatigue syndrome. Fatigue. 2013;1(3):168-186. PMID: 23914329 www.ncbi.nlm.nih.gov/pubmed/23914329. Larun L, Brurberg KG, Odgaard-Jensen J, Price JR. Exercise therapy for chronic fatigue syndrome. Cochrane Database Syst Rev. 2019;10:CD003200. PMID: 31577366 www.ncbi.nlm.nih.gov/pubmed/31577366. Marshall GS, Carter BD. Chronic fatigue syndrome. In: Long SS, Prober CG, Fischer M, eds. Principles and Practice of Pediatric Infectious Diseases. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 200. Santhouse A, Hotopf M, David AS. Chronic fatigue syndrome. BMJ. 2010;340:c738. PMID: 20150199 www.ncbi.nlm.nih.gov/pubmed/20150199. Smith MEB, Nelson HD, Haney E, et al. Diagnosis and treatment of myalgic encephalomyelitis/Chronic fatigue syndrome. Evidence Report/Technology Assessment No. 219. July 2016 Addendum. AHRQ Publication No. 15-E001-EF. Rockville, MD: Agency for Healthcare Research and Quality; 2014. www.ncbi.nlm.nih.gov/books/NBK379582. White PD, Goldsmith KA, Johnson AL, et al. Comparison of adaptive pacing therapy, cognitive behavior therapy, graded exercise therapy, and specialist medical care for chronic fatigue syndrome (PACE): a randomised trial. Lancet. 2011;377(9768):823-836. PMID: 21334061 www.ncbi.nlm.nih.gov/pubmed/21334061. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.	2	9	0	0	0	1	0.985804	1	5,323
- Can V codes be used as primary diagnosis? - What are V codes in the DSM 5? - Is burnout in the DSM 5? - What is a DSM category? - What are the 6 types of anxiety disorders? - What is the ICD 10 code for major depression? - What is the ICD 10 for anxiety? - What is anxiety f41 9? - What is the difference between DSM 4 and 5? - What is the code for anxiety? - Are DSM 5 and ICD 10 codes the same? - What does DSM 5 include? - What is the CPT code for major depression? - What is the code for depression? - What is the ICD 10 code for alcohol abuse? - What is the DSM V code for depression? - What is the DSM 5 code for adjustment disorder? - What are the 5 DSM categories? - What is the DSM 5 criteria for anxiety? - What is the purpose of Z codes? Can V codes be used as primary diagnosis? ANSWER: Per Coding Clinic, you can report V codes, including code V66. Report V codes either as primary diagnosis codes in an outpatient setting or as secondary codes in an inpatient setting, depending on the circumstances of the encounter.. What are V codes in the DSM 5? V codes, described in the ICD-9-CM chapter “Supplementary Classification of Factors Influencing Health Status and Contact with Health Services,” are designed for occasions when circumstances other than a disease or injury result in an encounter or are recorded by providers as problems or factors that influence care. Is burnout in the DSM 5? Classification. Burnout is not recognized as a distinct disorder in the current revision (dating from 2013) of the Diagnostic and Statistical Manual of Mental Disorders (DSM-5). Its definitions for Adjustment Disorders, and Unspecified Trauma- and Stressor-Related Disorder in some cases reflect the condition. What is a DSM category? The Diagnostic and Statistical Manual of Mental Disorders is the handbook widely used by clinicians and psychiatrists in the United States to diagnose psychiatric illnesses. Published by the American Psychiatric Association (APA), the DSM covers all categories of mental health disorders for both adults and children. What are the 6 types of anxiety disorders? 6 Major Types of Anxiety DisordersSeparation Anxiety Disorder.Specific Phobia.Social Anxiety Disorder (Social Phobia)Panic Disorder.Agoraphobia.Generalized Anxiety Disorder. What is the ICD 10 code for major depression? F33.1Code F33. 1 is the diagnosis code used for Major Depressive Disorder (MDD), Recurrent, Moderate. What is the ICD 10 for anxiety? 9 – Anxiety disorder, unspecified is a sample topic from the ICD-10-CM. To view other topics, please sign in or purchase a subscription. ICD-10-CM 2020 Coding Guide™ from Unbound Medicine. What is anxiety f41 9? Code F41. 9 is the diagnosis code used for Anxiety Disorder, Unspecified. It is a category of psychiatric disorders which are characterized by anxious feelings or fear often accompanied by physical symptoms associated with anxiety. What is the difference between DSM 4 and 5? In the DSM-IV, patients only needed one symptom present to be diagnosed with substance abuse, while the DSM-5 requires two or more symptoms in order to be diagnosed with substance use disorder. The DSM-5 eliminated the physiological subtype and the diagnosis of polysubstance dependence. What is the code for anxiety? Code F41. 1 is the diagnosis code used for Generalized Anxiety Disorder. It is an anxiety disorder characterized by excessive, uncontrollable and often irrational worry, that is, apprehensive expectation about events or activities. Are DSM 5 and ICD 10 codes the same? DSM-5 and ICD-9 and ICD-10 are related, but not the same: the DSM provides diagnostic criteria, to which the ICD billing codes are then applied. The DSM-5 manual contains descriptions of diagnoses of various behavioral health conditions. … Therefore, the codes you are using will probably not change. What does DSM 5 include? DSM–5 is a manual for assessment and diagnosis of mental disorders and does not include information or guidelines for treatment of any disorder. That said, determining an accurate diagnosis is the first step toward being able to appropriately treat any medical condition, and mental disorders are no exception. What is the CPT code for major depression? Code F33. 0 is the diagnosis code used for Major depressive disorder, recurrent, mild. This falls under the category of mood [affective] disorders. What is the code for depression? Coding the diagnosis Code 300.4, “Neurotic depression,” also referred to as “reactional depression,” can be used to code depression brought on by personal change or unexpected circumstances. What is the ICD 10 code for alcohol abuse? Chronic Causes100% AttributableCauseICD-9ICD-10Alcoholic psychosis291F10.3-F10.9Alcohol abuse305.0, 303.0F10.0, F10.1Alcohol dependence syndrome303.9F10.29 more rows What is the DSM V code for depression? 4-F32. 5 (major depressive disorder, in remission) and codes ICD9 296.30-296.36 / ICD10 F33. 0-F33. 9 (major depressive disorder, recurrent episode) are included in the code set. What is the DSM 5 code for adjustment disorder? Adjustment Disorder: Causes, Symptoms, Treatment DSM-5 309.9 (F43. What are the 5 DSM categories? Contents1.2.1 Neurodevelopmental disorders.1.2.2 Schizophrenia spectrum and other psychotic disorders.1.2.3 Bipolar and related disorders.1.2.4 Depressive disorders.1.2.5 Anxiety disorders.1.2.6 Obsessive-compulsive and related disorders.1.2.7 Trauma- and stressor-related disorders.1.2.8 Dissociative disorders.More items… What is the DSM 5 criteria for anxiety? Excessive anxiety and worry (apprehensive expectation), occurring more days than not for at least 6 months, about a number of events or activities (such as work or school performance). B. The person finds it difficult to control the worry. What is the purpose of Z codes? Z codes are designated as the principal/first listed diagnosis in specific situations such as: ➢ To indicate that a person with a resolving disease, injury or chronic condition is being seen for specific aftercare, such as the removal of internal fixation devices such as orthopedic pins.	4	8	0	0	0	16	0.845775	16	1,443
Icd 10 Ear Pain ICD-10 code H92.09 for Otalgia, unspecified ear is a medical classification medical classification A medical classification is used to transform descriptions of medical diagnoses or procedures into standardized statistical code in a process known as clinical coding. https://en.wikipedia.org › wiki › Medical_classification Medical classification – Wikipedia as listed by WHO under the range – Diseases of the ear and mastoid process mastoid process The mastoid part of the temporal bone is the posterior (back) part of the temporal bone, one of the bones of the skull. Its rough surface gives attachment to various muscles (via tendons) and it has openings for blood vessels. Mastoid part of the temporal bone – Wikipedia ICD-9 code 388.70 for Otalgia unspecified is a medical classification as listed by WHO under the range -DISEASES OF THE EAR AND MASTOID PROCESS (380-389). What is the ICD-9 code for right ear infection? Ear Infections – \|Unspecified otitis media\|\|Otitis media, unspecified, unspecified ear\| Ear infections are a common cause of ED visits, particularly in children. On its surface, the ICD-9 code (382.9) converts pretty smoothly to the equivalent ICD-10 code (H66.90). But when you look closely, a straight conversion doubles the number of “unspecified” elements. - That’s a lot of “unspecifieds” to present to a payer. - Under ICD-9, the code description for 382.9 includes the terms “acute” and “NOS” in its notes, so ED coding/billing entities have been able to code “acute otitis media” as long as the provider uses those terms on the chart. - While more specific ICD-9 codes are available, this code has generally been sufficient for billing and reimbursement purposes in the emergency department.) However, in ICD-10, the description “acute otitis media” leads to “double unspecified” coding unless further documentation is provided. As with ICD-9, more specific codes, (e.g., H66.009, acute suppurative otitis media with spontaneous rupture of eardrum) can be used if more specific terminology is documented. For physicians who have relied on “acute otitis media,” we strongly recommend: Use of descriptors “serous” and “suppurative” Note “with” or “without spontaneous rupture of eardrum” To eliminate the second “unspecified,” the provider must also document whether the infection is on the “right,” “left” or “bilateral.” In fact, ICD-10 requires documentation of laterality for all injuries and for illnesses involving bilateral organs (like ears). When HAP reviewed thousand of pediatric ED charts (where otitis is rampant), we found that documentation of laterality is often lacking in the diagnosis or impression. At times, the location can be inferred from other parts of the chart. But many coders, particularly those who are hospital-based, are taught that the diagnosis, if one is documented, overrides symptoms in the HPI or ROS. For this reason, we strongly recommend that ED providers get in the habit of identifying otitis as “right,” “left” or “bilateral.” We understand the position of many providers that treatment is usually the same regardless of laterality. But documentation of the specific location allows the coder to eliminate at least one “unspecified” element, which is likely to impact reimbursement. Often fever is noted with otitis. The good news is that ICD-9 code 780.60 (fever, unspecified) converts seamlessly to ICD-10 code R50.9 (fever, unspecified). Fever is typically well documented on ED charts, so most physicians can continue using their current notation system. (We are surprised that ICD-10 does not get more granular stratifying fever codes by actual temperature. Maybe in the next edition!) A final caveat: we at HAP understand that certain symptoms or conditions go with certain diagnoses (including otitis) and should be easily inferred by a capable coder. But remember, for compliance purposes, coders are trained to never infer. What is the ICD-9 code for inner ear? 20.99 Mid -inner ear ops NEC – ICD-9-CM Vol.3 Procedure Codes. What is the ICD-10 code for right ear symptoms? Unspecified disorder of right ear – 2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code - H93.91 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. - The 2023 edition of ICD-10-CM H93.91 became effective on October 1, 2022. - This is the American ICD-10-CM version of H93.91 – other international versions of ICD-10 H93.91 may differ. The following code(s) above H93.91 contain annotation back-references Annotation Back-References In this context, annotation back-references refer to codes that contain: - Applicable To annotations, or - Code Also annotations, or - Code First annotations, or - Excludes1 annotations, or - Excludes2 annotations, or - Includes annotations, or - Note annotations, or - Use Additional annotations that may be applicable to H93.91 : - H60-H95 2023 ICD-10-CM Range H60-H95	4	3	0	0	0	16	0.477458	16	1,227
Cardiopulmonary resuscitation has become the default treatment for all patients who suffer cardiac arrest. The history of how this came to be suggests the clinical and ethical importance of establishing more humane and appropriate indications for extracorporeal membrane oxygenation and other aggressive therapies for patients at the end of life. Development of new medical technologies has potential to greatly improve patients’ lives but also raises questions about how to establish standards of care for applying them. Increasing application of extracorporeal membrane oxygenation (ECMO)—especially as an adjunct to cardiopulmonary resuscitation (CPR), termed ECPR—presents us with an opportunity to establish sensitive and humane use standards. Such standards would pose a stark contrast to using CPR and other “life-saving” procedures to treat cardiac arrest in all critically ill and dying patients without a do-not-resuscitate (DNR) order. CPR became the default treatment for cardiac arrest in hospitals in the early 1970s and established a precedent for the current standard of “doing everything”—that is, applying all indicated procedures, regardless of whether they are expected to help a specific patient. It was only later that DNR orders were established, placing the onus on patients or surrogates to opt out. The do-everything precedent, however, suggests that as ECMO becomes increasingly accessible, it, too, will likely be added to the list of what is included in everything physicians do by default and that patients come to expect, perhaps prompting the need for do-not-ECMO orders. This article examines parallels between the early history of CPR and ECMO. We argue that similar forces that led to CPR becoming indicated in all cases of cardiac arrest are currently driving the expansion of indications for ECMO. Understanding these forces is essential to establishing more humane and appropriate indications for these aggressive therapies and may prevent them from becoming “default” treatments for all dying patients. A Brief History of ECMO ECMO is a life-supporting treatment that supplants the function of the lungs, the heart, or both, typically applied when patients’ illnesses are refractory to other standard procedures. ECMO’s earliest incarnation was referred to as the “mechanical heart and lung.”1 First successfully applied in the 1950s to bypass a patient’s heart, thereby revolutionizing cardiac surgery, ECMO was initially confined to operating rooms.1 After development of a compact portable battery-operated “roller pump” in the 1960s, ECMO could be used outside operating rooms, and its indications expanded to include acute respiratory distress syndrome, bridge to transplant, sepsis, and resuscitation.2 The first successes of ECMO as an adjunct to CPR in select cases of cardiac arrest were reported in 1976.3 Although use of ECPR has increased in the last decade, 2 recent meta-analyses of the procedure suggest that ECMO should be used as an adjunct to CPR or as an alternative to resuscitation alone in patients with reversible etiologies for their arrest (ie, not at the terminus of diseases expected to end in death).4,5 This limited indication for ECPR appears to be fairly well established and was echoed in an editorial commenting on 12 years of data from the Extracorporeal Life Support Organization,6 which defined ECPR as “implantation of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) in a patient who experienced a sudden and unexpected pulseless condition attributable to cessation of cardiac mechanical activity.”7 Innovating CPR Protocol? Although current standards for ECPR stipulate limited indications and specific locations, experts cannot help but wonder about where and for whom the procedure should be used. This concern was articulated in an editorial titled “ECPR: Are We Ready for Primetime?,”8 which accompanied a previously mentioned meta-analysis. In addition to implying that the indications for and application of ECPR had expanded, the title of the editorial raises an unspoken question: Should ECPR become part of the CPR protocol? Although the meta-analysis reported overall greater survival to hospital discharge for ECPR compared to conventional CPR,4 a reasonable answer to this question, for the time being, is “not yet.” The authors of the editorial suggested that the meta-analysis included too few publications, was “incomplete,” and that “ECPR should only be performed in selected circumstances and in those institutions that have 24-hours-a-day extracorporeal support systems in place.”8 The authors also stipulate, as do many articles on ECPR, that “Ethical considerations as to who should receive ECPR, and who should not get it, need to be properly addressed.”8 What should be the role of ethics in establishing standards for ECPR? Responses to this question have included calls for more evidence about benefits and risks, for evaluating potential patients’ preferences, and for consideration of economics.9 Establishing default CPR for all cardiac arrest meant that many patients, the vast majority of whom died in hospitals, underwent the procedure despite its not offering hope of meaningful life extension. The parallel early histories of CPR and ECMO suggest that the forces that contributed to CPR’s expanding indications and that ultimately led to its default application are also driving ECMO’s use trajectory. History of Default Care for Cardiac Arrest Treatment of cardiac arrest was mostly confined to the operating room until the 1950s when a few bold surgeons encouraged applying the technique, which involved open cardiac massage, in other areas of the hospital and beyond. This development meant that, until 1950, cardiac arrest was for the most part only diagnosed in the operating room because it was there that the procedure to treat the condition could be applied. The definition of cardiac arrest and, with it, the indications for resuscitation, greatly expanded when the much-less-invasive closed method of resuscitation was developed in the late 1950s. When the originators of CPR, which combined the closed method with mouth-to-mouth ventilation, convened for a roundtable discussion at the Chest meetings in 1962, they began by defining indications for the new therapy.10 Peter Safar, a developer of and advocate for mouth-to-mouth ventilation, opened the roundtable by posing a definition of cardiac arrest as an indication for the new technique: “I would like to define cardiac arrest as the clinical picture of cessation of circulation in a patient who was not expected to die at the time.”10 He then asked James Jude if he agreed with his definition. Jude, one of the developers of external cardiac massage at the Johns Hopkins University School of Medicine and the sole physician on the first article on the technique, published in 1960,11 replied, “It’s a very good one.”10 The markedly diminished burdens of CPR compared to the more invasive open technique that it replaced led to an expansion of CPR’s indications throughout the 1960s, but the limits of the procedure were still appreciated by many and guided practice in those hospitals accordingly.12 Evidence that CPR had not yet become the default treatment for cardiac arrest during the 1960s comes from several sources. Jude, along with James Elam, another developer of CPR also at the 1962 Chest conference roundtable, published the first CPR manual in 1965, The Fundamentals of Cardiopulmonary Resuscitation. The manual begins with a description of patients for whom CPR is indicated: “The patient must be salvable … resuscitative measures on terminal patients will, at best, return them to the dying state.”13 Three years later, in an article about uses of life-saving treatments such as cardiac resuscitation, the attorney John Fletcher states: The moral of our circular journey is that doctors are in a position to fashion their own law to deal with cases of prolongation of life. By establishing customary standards, they may determine the expectations of their patients and thus regulate the understanding and the relationship between doctor and patient. And by regulating that relationship, they may control their legal obligations to render aid to doomed patients.14 Fletcher’s call for developing “customary standards” for cardiac resuscitation speaks to the lack of an established standard at the time. It was only after CPR was established as the default treatment for cardiac arrest that DNRorders became necessary. The first mention of the DNR order does not appear in the medical literature until 1972,15 and it was formally codified by the American Heart Association in 1974.16 The standard of default CPR thus was not established before 1970 (see Table). \|1965\|\|Jude and Elam publish The Fundamentals of Cardiopulmonary Resuscitation, which began, “The patient must be salvable … resuscitative measures on terminal patients will, at best, return them to the dying state.”a\| \|1968\|\|Fletcher argues for the development of sensitive and humane standards for the care of patients who are clearly dying, implying a standard is not yet in place.b\| \|1972\|\|First mention of “Do Not Resuscitate” orders appears in the medical literature in a perspectives piece on patient death.c\| \|1974\|\|The American Heart Association officially codifies the “Order not to Resuscitate.”d\| \|a Quoted from Jude and Elam.13 b From Fletcher.14 c From Janes.15 d From “Standards for Cardiopulmonary Resuscitation (CPR) and Emergency Cardiac Care (ECC).”16 Proponents of ECPR are to be congratulated for their prudence to date in limiting its indications, but the parallels between ECPR and CPR more than half a century earlier are startling: ECPR appears poised to follow in CPR’s footsteps by becoming the default treatment for cardiac arrest—an example of the technological imperative. The Technological Imperative and Coding CPR as a Billable Procedure The technological imperative—the overapplication of technological solutions to an increasing range of problems—has been appreciated in medicine since at least the 1980s, when Howard Spiro discussed it at an eponymous conference. Spiro, then chief of general internal medicine at Yale University, identified the force driving the ever-increasing application of technological procedures when he remarked: We are all encouraged to do more in the way of technological activities today than 10 or 30 years ago simply because the third-party payers pay for technology and not for thinking. When you talk with the officials, they point out that it is easy to assess the costs of the procedures but difficult to assess the cost of a thought.17 The truth of Spiro’s remarks is manifest in the temporal relationship between CPR becoming the default option for all patients who died in hospitals in the early 1970s and its listing as a billable procedure in the second edition of the Current Procedural Terminology (CPT) manual, published in 1970 by the American Medical Association (AMA).18 In 1966 the AMA published the first CPT manual, which was much smaller and narrower in scope than the second edition, so that physicians and administrators could begin billing Medicare for procedures.19 The new 1970 CPT code read “CPR for Cardiac Arrest … 96000” and, very soon, cardiac arrest came to mean all cases of cessation of circulation, regardless of the context of the patient. The default application of CPR thus became the standard of care. A brief overview of the CPT codes for ECMO highlights an important difference between ECMO and CPR codes. The first instance of CPT coding for ECMO occurs in the 4th edition, published in 1977: “33960 … Prolonged extracorporeal circulation for cardiopulmonary insufficiency.”20 After several revisions, the current CPT codes for ECMO were established in 2015,21 with 16 different codes referring to specific aspects of the procedure: placement, repositioning, and removal of peripheral or central cannulas, for example, are further subdivided into open or percutaneous approaches. Of note, these various CPT codes are not linked to specific indications, as were CPR codes for cardiac arrest. This history suggests that one way to avoid ECPR becoming the default treatment for all cardiac arrest would be not to create a CPT code that links it to cardiac arrest. The gauntlet has already been thrown down for hospitals to provide around-the-clock ECMO teams before they will be sanctioned to provide ECPR, paralleling the creation of code teams for CPR in the 1960s.22 Although American Heart Association guidelines state that “[t]here is insufficient evidence to recommend the routine use of ECPR for patients with cardiac arrest,”23 it is likely only a matter of time before data will be collected that incontrovertibly show ECPR to be superior to CCPR, but only in select patients. One lesson from the history of CPR is this: cardiac arrest that includes all cases of cessation of blood flow should never be a blanket indication for ECPR. Another is that patients should not be forced to become do-not-ECMO to avoid the harms of ECPR. Instead, physicians need to make judgments about who would likely benefit from the procedure and decide with patients and families if this is something they would want. The history of CPR suggests the importance of defining the limits of any kind of resuscitation. By raising the stakes of resuscitation, ECPR also pushes us to better define these limits. By illuminating the temporal link between CPT codes and standards of care for CPR, this history also raises the question of CPT codes’ influence on other procedures and their standards. - Gibbon JH Jr. Application of a mechanical heart and lung apparatus to cardiac surgery. Minn Med. 1954;37(3):171-185. - Makdisi G, Wang IW. Extra corporeal membrane oxygenation (ECMO): review of a lifesaving technology. J Thorac Dis. 2015;7(7):E166-E176. - Mattox KL, Beall AC Jr. Resuscitation of the moribund patient using portable cardiopulmonary bypass. Ann Thorac Surg. 1976;22(5):436-442. Kim SJ, Kim HJ, Lee HY, Ahn HS, Lee SW. Comparing extracorporeal cardiopulmonary resuscitation with conventional cardiopulmonary resuscitation: a meta-analysis. Resuscitation. 2016;103:106-116. Ahn C, Kim W, Cho Y, Choi KS, Jang BH, Lim TH. Efficacy of extracorporeal cardiopulmonary resuscitation compared to conventional cardiopulmonary resuscitation for adult cardiac arrest patients: a systematic review and meta-analysis. Sci Rep. 2016;6:34208. Richardson AS, Schmidt M, Bailey M, Pellegrino VA, Rycus PT, Pilcher DV. ECMO cardio-pulmonary resuscitation (ECPR), trends in survival from an international multicentre cohort study over 12-years. Resuscitation. 2017;112:34-40. Pappalardo F, Montisci A. What is extracorporeal cardiopulmonary resuscitation? J Thorac Dis. 2017;9(6):1415-1419. Ocegueda-Pacheco C, Varon J. ECPR: are we ready for primetime? Resuscitation. 2016;103:A6-A7. Riggs KR, Becker LB, Sugarman J. Ethics in the use of extracorporeal cardiopulmonary resuscitation in adults. Resuscitation. 2015;91:73-75. Safar P, Elam JO, Jude JR, Wilder RJ, Zoll PM. Resuscitative principles for sudden cardiopulmonary collapse. Dis Chest. 1963;43:34-49. - Kouwenhoven WB, Jude JR, Knickerbocker GG. Closed-chest cardiac massage. JAMA. 1960;173(10):1064-1067. Jude JR, Kouwenhoven WB, Knickerbocker GG. External cardiac resuscitation. Monogr Surg Sci. 1964;1:59-117. Jude JR, Elam JO. Fundamentals of Cardiopulmonary Resuscitation. Philadelphia, PA: FA Davis; 1965. - Fletcher GP. Legal aspects of the decision not to prolong life. JAMA. 1968;203(1):65-68. - Janes RG. A clinical perspective on dying. Can Med Assoc J. 107(5):425-427. Standards for cardiopulmonary resuscitation (CPR) and emergency cardiac care (ECC). V. Medicolegal considerations and ecommendations. JAMA. 1974;227(7)(suppl):864-868. Wolf S, Berle BB, eds. The Technological Imperative in Medicine. New York, NY: Plenum Press; 1981. Gordon BL, ed. Physicians’ Current Procedural Terminology. 2nd ed. Chicago, IL: American Medical Association; 1970. Gordon BL, ed. Current Procedural Terminology. Chicago, IL: American Medical Association; 1966. American Medical Association. Physicians’ Current Procedural Terminology. 4th ed. Chicago, IL: American Medical Association; 1977. American Medical Association. CPT 2015 Professional Edition. Chicago, IL: American Medical Association; 2015. - Jung MA, Selby A, Johnson JR, Beanlands DS, Lenkei SC. Value of a cardiac arrest team in a university hospital: results in a series of 100 patients. Can Med Assoc J. 1968;98(2):74-78. Link MS, Berkow LC, Kudenchuk PJ, et al. Part 7: Adult advanced cardiovascular life support: 2015 American Heart Association guidelines update for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2015;132(18)(suppl 2):S444-S464.	2	8	5	0	0	0	0.771303	5	3,764
How do you code cardiac arrest? Code 427.5, Cardiac arrest, may be used as a secondary code in the following instances: The patient arrives in the hospital’s emergency service unit in a state of cardiac arrest and is resuscitated (and admitted) with the condition prompting the cardiac arrest known, such as ventricular tachycardia or trauma. What are the 3 conditions of cardiac arrest? Signs of sudden cardiac arrest are immediate and drastic and include: Sudden collapse. No pulse. No breathing. Is cardiac arrest the same as cardiogenic shock? Causes of Death in Cardiogenic Shock and Cardiac Arrest The causes and predictors of death differ between CS and CA: ABI is the primary cause of death in patients with CA, whereas CS patients typically die via refractory shock, organ failure, and arrhythmias. Is cardiac arrest an MCC? If the patient dies during the admission, the cardiac arrest will not serve as a major complication or comorbidity (MCC). If the patient dies in-house from the cardiac arrest without attempt at resuscitation, such that the cardiac arrest is their terminal event, you do not code the arrest. What is the ICD-10 code for cardiogenic shock? ICD-10 \| Cardiogenic shock (R57. 0) What is the difference between myocardial infarction and cardiac arrest? “A heart attack, technically called a myocardial infarction or MI, happens when there is a blockage that prevents the oxygen-rich blood from getting to the heart,” explains William Harris III, M.D., a cardiologist with Riverside Cardiology Specialists. “Cardiac arrest is when the heart suddenly stops functioning. What is sudden cardiac arrest? Cardiac arrest, also known as sudden cardiac arrest, is when the heart stops beating suddenly. The lack of blood flow to the brain and other organs can cause a person to lose consciousness, become disabled or die if not treated immediately. If a loved one experiences symptoms of cardiac arrest, call 911 immediately. What is cardiogenic arrest? Cardiogenic shock is a life-threatening condition in which your heart suddenly can’t pump enough blood to meet your body’s needs. The condition is most often caused by a severe heart attack, but not everyone who has a heart attack has cardiogenic shock. Cardiogenic shock is rare. What is the ICD 10 code for cardiogenic shock? Do we code CPR? Answer: Current Procedural Terminology (CPT) code 92950 is intended to reimburse for CPR performed to restore and maintain the patient’s respiration and circulation after cessation of heartbeat and breathing. CPR is a separately billable procedure. What to do after cardiac arrest? – Brentford boss Thomas Frank reveals how he managed to sign Christian Eriksen – The 29-year-old has agreed to join the Bees until the end of the current season – The Danish midfielder last played on June, 12 when he suffered a cardiac arrest When to code cardiac arrest? No efforts were made to resuscitate a 55-year-old resident, nor were calls made to 911, after the individual was discovered in cardiac arrest and not breathing on While classified as a “full code,” with the family requesting aggressive life-saving What are the warning signs of cardiac arrest? Chest pain prior to 46% of cardiac arrests. What are the reasons for cardiac arrest? Abstract. There is limited information on pulseless electrical activity (PEA)/asystolic cardiac arrest (CA) in the infant population.	4	5	2	0	0	2	0.857771	4	765
A 6 year old is prepared to go under anaesthesia Anesthesia, or anaesthesia (from Greek ἀν-, an-, "without"; and αἴσθησις, aisthēsis, "sensation", see spelling differences) is a temporary state consisting of unconsciousness, loss of memory, lack of pain, and muscle relaxation. Anesthesia is a unique medical intervention which does not itself offer any particular medical benefit and instead enables the performance of other medical interventions. The best anesthetic is therefore one with the lowest risk to the patient that still achieves the end points required to complete the other intervention. There are many different needs and goals of anesthesia. The goals (end points) are traditionally described as unconsciousness and amnesia, analgesia, and muscle relaxation. To reach multiple end points one or more drugs are commonly used (such as general anesthetics, hypnotics, sedatives, paralytics, narcotics, and analgesics), each of which serves a specific purpose in creating a safe anesthetic. The types of anesthesia are broadly classified into general anesthesia, sedation and regional anesthesia. General anesthesia refers to the suppression of activity in the central nervous system, resulting in unconsciousness and total lack of sensation. Sedation (or dissociative anesthesia) uses agents that inhibit transmission of nerve impulses between higher and lower centers of the brain inhibiting anxiety and the creation of long-term memories. Regional anesthesia renders a larger area of the body insensate by blocking transmission of nerve impulses between a part of the body and the spinal cord. It is divided into peripheral and central blockades. Peripheral blockade inhibits sensory perception within a specific location on the body, such as when a tooth is "numbed" or when a nerve block is given to stop sensation from an entire limb. Central blockades place the local anesthetic around the spinal cord (such as with spinal and epidural anesthesia) removing sensation to any area below the level of the block. There are both major and minor risks of anesthesia. Examples of major risks include death, heart attack and pulmonary embolism whereas minor risks can include postoperative nausea and vomiting and readmission to hospital. The likelihood of a complication occurring is proportional to the relative risk of a variety of factors related to the patient's health, the complexity of the surgery being performed and the type of anesthetic. Of these factors, the person's health prior to surgery (stratified by the ASA physical status classification system) has the greatest bearing on the probability of a complication occurring. Patients typically wake within minutes of an anesthetic being terminated and regain their senses within hours. One exception is a condition called long-term post-operative cognitive dysfunction, characterized by persistent confusion lasting weeks or months, which is more common in those undergoing cardiac surgery and in the elderly. The first documented general anesthetic was performed by Crawford W. Long in 1842. Unfortunately for Long, he did not publish his successes with ether for general anesthesia until 1849. The first public demonstration of general anesthesia was in 1846 by a Boston dentist named William T.G. Morton at the Massachusetts General Hospital. Dr. Morton gave an ether anesthetic for the removal of a neck tumor by surgeon John Collins Warren (the first editor of the New England Journal of Medicine and dean of Harvard Medical School). About a decade later, cocaine was introduced as the first viable local anesthetic. It wasn't until the 1930s that Dr. Harvey Cushing tied the stress response to higher mortality rates and began using local anesthetic for hernia repairs in addition to general anesthesia. - 1 Medical uses - 2 Techniques - 3 Risks and complications - 4 Recovery - 5 History - 6 Society and culture - 7 Special populations - 8 References - 9 External links The purpose of anesthesia can be distilled down to three basic goals or end points::236 - hypnosis (a temporary loss of consciousness and with it a loss of memory) - analgesia (lack of sensation which also blunts autonomic reflexes) - muscle relaxation Different types of anesthesia (which are discussed in the following sections) affect the endpoints in different ways. Regional anesthesia, for instance affects analgesia, benzodiazepine type sedatives (used in twilight sleep) favor amnesia and general anesthetics can affect all of the endpoints. The goal of anesthesia is to achieve the necessary endpoints with the least amount of risk possible to the patient. To achieve the goals of anesthesia, drugs act on different but interconnected parts of the nervous system. Hypnosis, for instance, is generating through actions on the nuclei in the brain and is similar to the activation of sleep. The effect is to make people less aware and less reactive to non-noxious stimuli.:245 Loss of memory (amnesia) is created by action of drugs on multiple (but specific) regions of the brain. Memories are created as either declarative or non-declarative memories in several stages (short-term, long-term, long-lasting) the strength of which is determined by the strength of connections between neurons termed synaptic plasticity.:246 Each anesthetic produces amnesia through unique effects on memory formation at variable doses. Inhalational anesthetics will reliably produce amnesia through general suppression of the nuclei at doses below those required for loss of consciousness. Drugs like midazolam produce amnesia through different pathways by blocking the formation of long-term memories.:249 Tied closely to the concepts of amnesia and hypnosis is the concept of consciousness. Consciousness is the higher order process that synthesizes information. For instance, the “sun” conjures up feelings, memories and a sensation of warmth rather than a description of a round, orange warm ball seen in the sky for part of a 24 hour cycle. Likewise, a person can have dreams (a state of subjective consciousness) during anesthetic or have consciousness of the procedure despite having no indication of it under anesthetic. It is estimated that 22% of people dream during general anesthesia and 1 or 2 cases per 1000 have some consciousness termed “awareness during general anesthesia”.:253 Anesthesia is unique, in that it does not offer any particular benefit, rather it allows others to do things that might be beneficial. The best anesthetic, therefore is the one with the lowest risk to the patient that still achieves the endpoints required to complete the procedure. The first stage of an anesthetic is the pre-operative risk assessment made up of the medical history, physical examination and lab tests. Diagnosing a person's pre-operative physical status allows the clinician to minimize anesthetic risks. A well completed medical history will arrive at the correct diagnosis 56% of the time which increases to 73% with a physical examination. Lab tests help in diagnosis but only in 3% of cases, underscoring the need for a full history and physical examination prior to anesthetics. Incorrect pre-operative assessments or preparations are the root cause of 11% of all adverse anesthetic events.:1003 \|ASA class\|\|Physical status\| \|ASA 1\|\|Healthy person\| \|ASA 2\|\|Mild systemic disease\| \|ASA 3\|\|Severe systemic disease\| \|ASA 4\|\|Severe systemic disease that is a constant threat to life\| \|ASA 5\|\|A moribund person who is not expected to survive without the operation\| \|ASA 6\|\|A declared brain-dead person whose organs are being removed for donor purposes\| \|E\|\|Suffix added for patients undergoing emergency procedure\| One part of the risk assessment is based on the patients' health. The American Society of Anesthesiologists have developed a six-tier scale which stratifies the pre-operative physical state of the patient called the ASA physical status. The scale assesses a high-order of risk as the patient's general health relates to an anesthetic. The more detailed pre-operative medical history aims to discover genetic disorders (such as malignant hyperthermia or pseudocholinesterase deficiency), habits (tobacco, drug and alcohol use), physical attributes (such as obesity or a difficult airway) and any coexisting diseases (especially cardiac and respiratory diseases) that might impact the anesthetic. The physical examination helps quantify the impact of anything found in the medical history in addition to lab tests.:1003–1009 Aside from the generalities of the patients health assessment, an evaluation of the specific factors as they relate to the surgery also need to be considered for anesthesia. For instance, anesthesia during childbirth must consider not only the mother but the baby. Cancers and tumors that occupy the lungs or throat create special challenges to general anesthesia. After determining the health of the person undergoing anesthetic and the endpoints that are required to complete the procedure, the type of anesthetic can be selected. Choice of surgical method and anaesthetic technique aims to reduce risk of complications, shorten time needed for recovery and minimise the surgical stress response. Anesthesia is the combination of the endpoints (discussed above) which are reached by drugs acting on different but overlapping sites in the central nervous system. General anesthesia (as opposed to sedation or regional anesthesia) has three main goals: lack of movement (paralysis), unconsciousness, and blunting of the stress response. In the early days of anesthesia, anesthetics could reliably achieve the first two, allowing surgeons to perform necessary procedures, but many patients died because the extremes of blood pressure and pulse caused by the surgical insult were ultimately harmful. Eventually, the need for blunting of the surgical stress response was identified by Harvey Cushing, who injected local anesthetic prior to hernia repairs.:30 This led to the development of other drugs that could blunt the response leading to lower surgical mortality rates. The most common approach to reach the endpoints of general anesthesia is through the use of inhaled general anesthetics. Each has its own potency which is correlated to its solubility in oil. This relationship exists because the drugs bind directly to cavities in proteins of the central nervous system, although several theories of general anaesthetic action have been described. Inhalational anesthetics are thought to exact their effects on different parts of the central nervous system. For instance, the immobilizing effect of inhaled anesthetics results from an effect on the spinal cord whereas sedation, hypnosis and amnesia involve sites in the brain.:515 The potency of an inhalational anesthetic is quantified by its minimum alveolar concentration or MAC. The MAC is the percentage dose of anaesthetic that will prevent a response to painful stimulus in 50% of subjects. The higher the MAC, generally, the less potent the anesthetic. The ideal anesthetic drug would provide hypnosis, amnesia, analgesia, and muscle relaxation without undesirable changes in blood pressure, pulse or breathing. In the 1930s, physicians started to augment inhaled general anesthetics with intravenous general anesthetics. The drugs used in combination offered a better risk profile to the person under anesthetic and a quicker recovery. A combination of drugs was later shown to result in lower odds of dying in the first 7 days after anesthetic. For instance, propofol (injection) might be used to start the anesthetic, fentanyl (injection) used to blunt the stress response, midazolam (injection) given to ensure amnesia and sevoflurane (inhaled) during the procedure to maintain the effects. More recently, several intravenous drugs have been developed which, if desired, allow inhaled general anesthetics to be avoided completely.:720 The core instrument in an inhalational anesthetic delivery system is an anesthetic machine. It has vaporizers, ventilators, an anesthetic breathing circuit, waste gas scavenging system and pressure gauges. The purpose of the anesthetic machine is to provide anesthetic gas at a constant pressure, oxygen for breathing and to remove carbon dioxide or other waste anesthetic gases. Since inhalational aenesthetics are inflammable, various checklists have been developed to confirm that the machine is ready for use, that the safety features are active and the electrical hazards are removed. Intravenous anesthetic is delivered either by bolus doses or an infusion pump. There are also many smaller instruments used in airway management and monitoring the patient. The common thread to modern machinery in this field is the use of fail-safe systems that decrease the odds of catastrophic misuse of the machine. Patients under general anesthesia must undergo continuous physiological monitoring to ensure safety. In the US, the American Society of Anesthesiologists (ASA) have established minimum monitoring guidelines for patients receiving general anesthesia, regional anesthesia, or sedation. This includes electrocardiography (ECG), heart rate, blood pressure, inspired and expired gases, oxygen saturation of the blood (pulse oximetry), and temperature. In the UK the Association of Anaesthetists (AAGBI) have set minimum monitoring guidelines for general and regional anesthesia. For minor surgery, this generally includes monitoring of heart rate, oxygen saturation, blood pressure, and inspired and expired concentrations for oxygen, carbon dioxide, and inhalational anesthetic agents. For more invasive surgery, monitoring may also include temperature, urine output, blood pressure, central venous pressure, pulmonary artery pressure and pulmonary artery occlusion pressure, cardiac output, cerebral activity, and neuromuscular function. In addition, the operating room environment must be monitored for ambient temperature and humidity, as well as for accumulation of exhaled inhalational anesthetic agents, which might be deleterious to the health of operating room personnel. Sedation (also referred to as dissociative anesthesia or twilight anesthesia) creates hypnotic, sedative, anxiolytic, amnesic, anticonvulsant, and centrally produced muscle-relaxing properties. From the perspective of the person giving the sedation, the patient will appear sleepy, relaxed and forgetful, allowing unpleasant procedures to be more easily completed. Sedatives such as benzodiazepines are usually given with pain relievers (such as narcotics, or local anesthetics or both) because they don't, by themselves, provide significant pain relief. From the perspective of the person receiving sedative, the effect is a feeling of general relaxation, forgetfulness and time passing quickly. Many drugs can produce a sedative effect including benzodiazepines, propofol, thiopental, ketamine and inhaled general anesthetics. The advantage of sedation over a general anesethetic is that it generally doesn't require support of the airway or breathing (no tracheal intubation or mechanical ventilation) and can have less of an effect on the cardiovascular system which may add to a greater margin of safety in some patients.:736 When pain is blocked from a part of the body using local anesthetics, it is generally referred to as regional anesthesia. There are many types of regional anesthesia either by injecting into the tissue itself, a vein that feeds the area or around a nerve trunk that supplies sensation to the area. The later are called nerve blocks and are divided into peripheral or central nerve blocks. The following are the types of regional anesthesia::926–931 - Infiltrative anesthesia: a small amount of local anesthetic is injected in a small area to stop any sensation (such as during the closure of a laceration, as a continuous infusion or "freezing" a tooth). The effect is almost immediate. - Peripheral nerve block: local anesthetic is injected near a nerve that provides sensation to particular portion of the body. There is significant variation in the speed of onset and duration of anesthesia depending on the potency of the drug (e.g. Mandibular block). - Intravenous regional anesthesia (also called a Bier block): dilute local anesthetic is infused to a limb through a vein with a tourniquet placed to prevent the drug from diffusing out of the limb. - Central nerve blockade: Local anesthetic is injected or infused in or around a portion of the central nervous system (discussed in more detail below in Spinal, epidural and caudal anesthesia). - Topical anesthesia: local anesthetics that are specially formulated to diffuse through the mucous membranes or skin to give a thin layer of analgesia to an area (e.g. EMLA patches). - Tumescent anesthesia: a large amount of very dilute local anesthetics are injected into the subcutaneous tissues during liposuction. - Systemic local anesthetics: local anesthetics are given systemically (orally or intravenous) to relieve neuropathic pain When local anesthetic is injected around a larger diameter nerve that transmits sensation from an entire region it is referred to as a nerve block. Nerve blocks are commonly used in dentistry, when the mandibular nerve is blocked for procedures on the lower teeth. With larger diameter nerves (such as the interscalene block for upper limbs or psoas compartment block for lower limbs) the nerve and position of the needle is localized with ultrasound or electrical stimulation. The use of ultrasound may reduce complication rates and improve quality, performance time, and time to onset of blocks. Because of the large amount of local anesthetic required to affect the nerve, the maximum dose of local anesethetic has to be considered. Nerve blocks are also used as a continuous infusion, following major surgery such as knee, hip and shoulder replacement surgery, and may be associated with lower complications. Nerve blocks are also associated with a lower risk of neurologic complications when compared to neuraxial blocks.:1639–1641 Spinal, epidural and caudal anesthesia Central neuraxial anesthesia is the injection of local anesthetic around the spinal cord to provide analgesia in the abdomen, pelvis or lower extremities. It is divided into either spinal (injection into the subarachnoid space), epidural (injection outside of the subarachnoid space into the epidural space) and caudal (injection into the cauda equina or tail end of the spinal cord). Spinal and epidural are the most commonly used forms of central neuraxial blockade. Spinal anesthesia is a "one-shot" injection that provides rapid onset and profound sensory anesthesia with lower doses of anesethetic, and is usually associated with neuromuscular blockade (loss of muscle control). Epidural anesthesia uses larger doses of anesthetic infused through an indwelling catheter which allows the anesthetic to be augmented should the effects begin to dissipate. Epidural anesethesia does not typically affect muscle control. Because central neuraxial blockade causes arterial and vasodilation, a drop in blood pressure is common. This drop is largely dictated by the venous side of the circulatory system which holds 75% of the circulating blood volume. The physiologic effects are much greater when the block is placed above the 5th thoracic vertebra. An ineffective block is most often due to inadequate anxiolysis or sedation rather than a failure of the block itself.:1611 Acute pain management Pain that is well managed during and immediately after surgery improves the health of patients (by decreasing physiologic stress) and the potential for chronic pain. Nociception (pain sensation) is not hard-wired into the body. Instead, it is a dynamic process wherein persistent painful stimuli can sensitize the system and either make pain management difficult or promote the development of chronic pain. For this reason, preemptive acute pain management may reduce both acute and chronic pain and is tailored to the surgery, the environment in which it is given (in-patient/out-patient) and the individual patient.:2757 Pain management is classified into either pre-emptive or on-demand. On-demand pain medications typically include either opioid or non-steroidal anti-inflammatory drugs but can also make use of novel approaches such as inhaled nitrous oxide or ketamine. On demand drugs can be administered by a clinician ("as needed drug orders") or by the patient using patient-controlled analgesia (PCA). PCA has been shown to provide slightly better pain control and increased patient satisfaction when compared with conventional methods. Common preemptive approaches include epidural neuraxial blockade or nerve blocks. One review which looked at pain control after abdominal aortic surgery found that epidural blockade provides better pain relief (especially during movement) in the period up to three postoperative days. It reduces the duration of postoperative tracheal intubation by roughly half. The occurrence of prolonged postoperative mechanical ventilation and myocardial infarction is also reduced by epidural analgesia. Risks and complications Risks and complications as they relate to anesthesia are classified as either morbidity (a disease or disorder that results from anesthesia) or mortality (death that results from anesthesia). Attempting to quantify how anesthesia contributes to morbidity and mortality can be difficult because a person's health prior to surgery and the complexity of the surgical procedure can also contribute to the risks. Prior to anesthetic in the early 19th century, the physiologic stress from surgery caused significant complications and many deaths from shock. The faster the surgery was, the lower the rate of complications (leading to reports of very quick amputations). The advent of anesthesia allowed more complicated and life-saving surgery to be completed, decreased the physiologic stress of the surgery, but added an element of risk. It was two years after the introduction of ether anesthetics that the first death directly related to anesthetic was reported. Morbidity can be major (myocardial infarction, pneumonia, pulmonary embolism, renal failure/insufficiency, postoperative cognitive dysfunction and allergy) or minor (minor nausea, vomiting, readmission). There is usually overlap in the contributing factors that lead to morbidity and mortality between the health of the patient, the surgery being performed and the anesthetic. To understand the relative risk of each contributing factor, consider that the rate of deaths totally attributed to the patient's health is 1:870. Compare that to the rate of deaths totally attributed to surgical factors (1:2860) or anesthesia alone (1:185,056) illustrating that the single greatest factor in anesthetic mortality is the health of the patient. These statistics can also be compared to the first such study on mortality in anesthesia from 1954, which reported a rate of death from all causes at 1:75 and a rate attributed to anesthesia alone at 1:2680.:993 Direct comparisons between mortality statistics cannot reliably be made over time and across countries because of differences in the stratification of risk factors, however, there is evidence that anesthetics have made a significant improvement in safety but to what degree is uncertain. Rather than stating a flat rate of morbidity or mortality, many factors are reported as contributing to the relative risk of the procedure and anesthetic combined. For instance, an operation on a person who is between the ages of 60–79 years old places the patient at 2.32 times greater risk than someone less than 60 years old. Having an ASA score of 3, 4 or 5 places the person at 10.65 times greater risk than someone with an ASA score of 1 or 2. Other variables include age greater than 80 (3.29 times risk compared to those under 60), gender (females have a lower risk of 0.77), urgency of the procedure (emergencies have a 4.44 times greater risk), experience of the person completing the procedure (less than 8 years experience and/or less than 600 cases have a 1.06 times greater risk) and the type of anesthetic (regional anesthetics are lower risk than general anesthetics).:984 Obstetrical, the very young and the very old are all at greater risk of complication so extra precautions may need to be taken.:969–986 The immediate time after anesthesia is called emergence. Emergence from general anesthesia or sedation requires careful monitoring because there is still a risk of complication. Nausea and vomiting are reported at 9.8% but will vary with the type of anesthetic and procedure. There is a need for airway support in 6.8%, there can be urinary retention (more common in those over 50 years of age) and hypotension in 2.7%. Hypothermia, shivering and confusion are also common in the immediate post-operative period because of the lack of muscle movement (and subsequent lack of heat production) during the procedure.:2707 Postoperative cognitive dysfunction (also known as POCD and post-anesthetic confusion) is a disturbance in cognition after surgery. It may also be variably used to describe emergence delirium (immediate post-operative confusion) and early cognitive dysfunction (diminished cognitive function in the first post-operative week). Although the three entities (delirium, early POCD and long-term POCD) are separate, the presence of delirium post-operatively predicts the presence of early POCD. There does not appear to be an association between delirium or early POCD and long-term POCD. According to a recent study conducted at the David Geffen School of Medicine at UCLA, the brain navigates its way through a series of activity clusters, or "hubs" on its way back to consciousness. Dr. Andrew Hudson, an assistant professor in anesthesiology states, "Recovery from anesthesia is not simply the result of the anesthetic 'wearing off,' but also of the brain finding its way back through a maze of possible activity states to those that allow conscious experience. Put simply, the brain reboots itself." Long-term postoperative cognitive dysfunction is a subtle deterioration in cognitive function, that can last for weeks, months, or longer. Most commonly, relatives of the person report a lack of attention, memory and loss of interest in activities previously dear to the person (such as crosswords). In a similar way, people in the workforce may report an inability to complete tasks at the same speed they could previously. There is good evidence that POCD occurs after cardiac surgery and the major reason for its occurrence is the formation of microemboli. POCD also appears to occur in non-cardiac surgery. Its causes in non-cardiac surgery are less clear but older age is a risk factor for its occurrence.:2805–2816 Throughout Europe, Asia, and the Americas a variety of Solanum species containing potent tropane alkaloids were used for anesthesia. In 13th century Italy, Theodoric Borgognoni used similar mixtures along with opiates to induce unconsciousness, and treatment with the combined alkaloids proved a mainstay of anesthesia until the nineteenth century. Local anesthetics were used in Inca civilization where shamans chewed coca leaves and performed operations on the skull while spitting into the wounds they had inflicted to anesthetize. Cocaine was later isolated and became the first effective local anesthetic. It was first used in 1859 by Karl Koller, at the suggestion of Sigmund Freud, in eye surgery in 1884. German surgeon August Bier (1861–1949) was the first to use cocaine for intrathecal anesthesia in 1898. Romanian surgeon Nicolae Racoviceanu-Piteşti (1860–1942) was the first to use opioids for intrathecal analgesia; he presented his experience in Paris in 1901. Early Arab writings mention anesthesia by inhalation. This idea was the basis of the "soporific sponge" ("sleep sponge"), introduced by the Salerno school of medicine in the late twelfth century and by Ugo Borgognoni (1180–1258) in the thirteenth century. The sponge was promoted and described by Ugo's son and fellow surgeon, Theodoric Borgognoni (1205–1298). In this anesthetic method, a sponge was soaked in a dissolved solution of opium, mandragora, hemlock juice, and other substances. The sponge was then dried and stored; just before surgery the sponge was moistened and then held under the patient's nose. When all went well, the fumes rendered the patient unconscious. The most famous anesthetic, ether, may have been synthesized as early as the 8th century, but it took many centuries for its anesthetic importance to be appreciated, even though the 16th century physician and polymath Paracelsus noted that chickens made to breathe it not only fell asleep but also felt no pain. By the early 19th century, ether was being used by humans, but only as a recreational drug. Meanwhile, in 1772, English scientist Joseph Priestley discovered the gas nitrous oxide. Initially, people thought this gas to be lethal, even in small doses, like some other nitrogen oxides. However, in 1799, British chemist and inventor Humphry Davy decided to find out by experimenting on himself. To his astonishment he found that nitrous oxide made him laugh, so he nicknamed it laughing gas. Davy wrote about the potential anesthetic properties of nitrous oxide, but nobody at that time pursued the matter any further. American physician Crawford W. Long noticed that his friends felt no pain when they injured themselves while staggering around under the influence of ether. He immediately thought of its potential in surgery. Conveniently, a participant in one of those “ether frolics", a student named James Venable, had two small tumors he wanted excised. But fearing the pain of surgery, Venable kept putting the operation off. Hence, Long suggested that he have his operation while under the influence of ether. Venable agreed, and on 30 March 1842 he underwent a painless operation. However, Long did not announce his discovery until 1849. In 1846, Boston dentist William Thomas Green Morton conducted the first public demonstration of the inhalational anesthetic. Morton, who was unaware of Long's previous work, was invited to the Massachusetts General Hospital to demonstrate his new technique for painless surgery. After Morton had induced anesthesia, surgeon John Collins Warren removed a tumor from the neck of Edward Gilbert Abbott. This occurred in the surgical amphitheater now called the Ether Dome. The previously skeptical Warren was impressed and stated, "Gentlemen, this is no humbug." In a letter to Morton shortly thereafter, physician and writer Oliver Wendell Holmes, Sr. proposed naming the state produced "anesthesia", and the procedure an "anesthetic". Morton at first attempted to hide the actual nature of his anesthetic substance, referring to it as Letheon. He received a US patent for his substance, but news of the successful anesthetic spread quickly by late 1846. Respected surgeons in Europe including Liston, Dieffenbach, Pirogov, and Syme quickly undertook numerous operations with ether. An American-born physician, Boott, encouraged London dentist James Robinson to perform a dental procedure on a Miss Lonsdale. This was the first case of an operator-anesthetist. On the same day, 19 December 1846, in Dumfries Royal Infirmary, Scotland, a Dr. Scott used ether for a surgical procedure. The first use of anesthesia in the Southern Hemisphere took place in Launceston, Tasmania, that same year. Drawbacks with ether such as excessive vomiting and its flammability led to its replacement in England with chloroform. Discovered in 1831 by an American physician Samuel Guthrie (1782–1848), and independently a few months later by Frenchman Eugène Soubeiran (1797-1859) and Justus von Liebig (1803–73) in Germany, chloroform was named and chemically characterised in 1834 by Jean-Baptiste Dumas (1800–84). Its anaesthetic properties were noted early in 1847 by Marie-Jean-Pierre Flourens (1794–1867). The use of chloroform in anesthesia is linked to James Young Simpson, who, in a wide-ranging study of organic compounds, found chloroform's efficacy on 4 November 1847. Its use spread quickly and gained royal approval in 1853 when John Snow gave it to Queen Victoria during the birth of Prince Leopold. Unfortunately, though free of ether's flammability and consequent explosion hazard, chloroform is not as safe pharmacologically, especially when administered by an untrained practitioner (medical students, nurses, and occasionally members of the public were often pressed into giving anesthetics at this time). This led to many deaths from the use of chloroform that (with hindsight) might have been preventable. The first fatality directly attributed to chloroform anesthesia was recorded on 28 January 1848 after the death of Hannah Greener. John Snow of London published articles from May 1848 onwards "On Narcotism by the Inhalation of Vapours" in the London Medical Gazette. Snow also involved himself in the production of equipment needed for the administration of inhalational anesthetics, the forerunner of today's anesthesia machines. Of these first famous anesthetics, only nitrous oxide is still widely used today, with chloroform and ether having been replaced by safer but sometimes more expensive general anesthetics, and cocaine by more effective local anesthetics with less abuse potential. Society and culture Almost all healthcare providers use anesthesia to some degree, however most health professions have their own field of specialists in the field including medicine, nursing and dentistry. Doctors specializing in perioperative care, development of an anesthetic plan, and the administration of anesthetics are known in the US as anesthesiologists and in the UK, Canada, Australia, and NZ as anaesthetists or anaesthesiologists. All anesthetics in the UK, Australia, New Zealand, Hong Kong and Japan are administered by doctors. Nurse anesthetists also administer anesthesia in 109 nations. In the US, 35% of anesthetics are provided by physicians in solo practice, about 55% are provided by anesthesia care teams (ACTs) with anesthesiologists medically directing anesthesiologist assistants or certified registered nurse anesthetists (CRNAs), and about 10% are provided by CRNAs in solo practice. There can also be anesthesiologist assistants (US) or physician assistant (anaesthesia) (UK) who assistant with anesthesia There are many circumstances when anesthesia needs to be altered for special circumstances due to the procedure (such as in cardiac surgery, cardiothoracic anesthesiology or neurosurgery), the patient (such as in pediatric anesthesia, geriatric, bariatric or obstetrical anesthesia) or special circumstances (such as in trauma, prehospital care, robotic surgery or extreme environments). - Anesthesia. Merriam-Webster. Retrieved 2012-06-13. - Miller, RD (2010). Miller's Anesthesia 7th edition. Elsevier Health Sciences. ISBN 9780443069598. - Fitz-Henry, J (Apr 2011). "The ASA classification and peri-operative risk". Ann R Coll Surg Engl 93 (3): 185–187. doi:10.1308/147870811X565070. PMC 3348554. PMID 21477427. - Goneppanavar, U; Prabhu, M (Sep–Oct 2013). "Anaesthesia Machine: Checklist, Hazards, Scavenging". Indian J Anaesth 57 (5): 533–540. doi:10.4103/0019-5049.120151. PMC 3821271. PMID 24249887. - Subrahmanyam M, Mohan S. (Sep 2013). "Safety Features in Anaesthesia Machine.". Indian J Anaesth. 57 (5): 472–480. doi:10.4103/0019-5049.120143. PMC 3821264. PMID 24249880. - . Committee of Origin: Standards and Practice Parameters (Approved by the ASA House of Delegates on 21 October 1986, amended 20 Oct 2010 with an effective date of 1 July 2011) - Birks RJS, ed. (March 2007). "RECOMMENDATIONS FOR STANDARDS OF MONITORING DURING ANAESTHESIA AND RECOVERY 4th Edition". Association of Anaesthetists of Great Britain and Ireland. Retrieved 21 February 2014. - Reddy S, Patt RB. (Nov 1994). "The benzodiazepines as adjuvant analgesics.". J Pain Symptom Manage. 9 (8): 510–4. doi:10.1016/0885-3924(94)90112-0. - Walker KJ et al (Oct 2009). "Ultrasound guidance for peripheral nerve blockade.". Cochrane Database Syst Rev. 7 (4): CD006459. doi:10.1002/14651858.CD006459.pub2. - Ullah H et al (April 2014). "Continuous interscalene brachial plexus block versus parenteral analgesia for postoperative pain relief after major shoulder surgery.". Cochrane Database Syst Rev. 2 (CD007080). - Andreae, MH Andreae DA (Oct 2012). "Local anaesthetics and regional anaesthesia for preventing chronic pain after surgery". Cochrane Database Syst Rev 17. doi:10.1002/14651858.CD007105.pub2. - Klomp T et al (Sep 2012). "Inhaled analgesia for pain management in labour.". Cochrane Database Syst Rev. 12 (9): CD009351. doi:10.1002/14651858.CD009351.pub2. - Bell, Rae F; Dahl, Jørgen B; Moore, R Andrew; Kalso, Eija A (Jan 2006). "Perioperative ketamine for acute postoperative pain". Cochrane Database Syst Rev. 25 (1): CD004603. doi:10.1002/14651858.CD004603.pub2. PMID 16437490. - Hudcova J, et al (Oct 2006). "Patient controlled opioid analgesia versus conventional opioid analgesia for controlling postoperative pain". Cochrane Database of Systematic Reviews 18 (4): CD003348. doi:10.1002/14651858.CD003348.pub2. - Jones L et al (Mar 2012). "Pain management for women in labour: an overview of systematic reviews". Cochrane Database of Systematic Reviews 14 (3): CD009234. doi:10.1002/14651858.CD009234.pub2. PMID 22419342. - Novikova N et al (Sep 2012). "Local anaesthetic nerve block for pain management in labour". Cochrane Database of Systematic Reviews 12 (9): CD009351. doi:10.1002/14651858.CD009351.pub2. - Gilpin GL (Oct 2006). "Patient controlled opioid analgesia versus conventional opioid analgesia for postoperative pain.". Cochrane Database Syst Rev. 2006 Oct 18;(4):CD003348. 18 (4): CD003348. doi:10.1002/14651858.CD005059.pub3. - Lagasse, RS (Dec 2002). "Anesthesia Safety:Model or Myth?". Anesthesiology 97 (6): 1609–17. doi:10.1097/00000542-200212000-00038. PMID 12459692. - Chaloner EJ, Ham RJ (Aug 2001). "Amputations at the London Hospital 1852-1857". J R Soc Med 94 (8): 409–412. PMC 1281639. PMID 11461989. - Braz LG (Oct 2009). "Mortality in Anesthesia: A Systematic Review". Clinics (Sao Paulo) 64 (10): 999–1006. doi:10.1590/S1807-59322009001000011. PMC 2763076. PMID 19841708. - Whitaker DK, Booth H (Mar 2013). "Immediate post-anaesthesia recovery 2013: Association of Anaesthetists of Great Britain and Ireland.". Anaesthesia. 68 (3): 288–97. doi:10.1111/anae.12146. - Rudolph JL et al (Sep 2008). "Delirium is associated with early postoperative cognitive dysfunction". Anaesthesia 63 (9): 941–947. doi:10.1111/j.1365-2044.2008.05523.x. PMC 2562627. PMID 18547292. - Deiner S, Silverstein JH (2009). "Postoperative delirium and cognitive dysfunction". Br J Anaesth 103 (Suppl 1): i41–i46. doi:10.1093/bja/aep291. - Ruetsch, YA; Böni, T; Borgeat, A (2001). "From Cocaine to Ropivacaine: The History of Local Anesthetic Drugs". Current Topics in Medicinal Chemistry 1 (3): 175–82. doi:10.2174/1568026013395335. PMID 11895133. - Koller, K (1884). "Über die verwendung des kokains zur anästhesierung am auge" [On the use of cocaine for anesthesia on the eye]. Wiener Medizinische Wochenschrift (in German) 34: 1276–1309. - Bier, A (1899). "Versuche über cocainisirung des rückenmarkes" [Experiments on the cocainization of the spinal cord]. Deutsche Zeitschrift für Chirurgie (in German) 51 (3–4): 361–9. doi:10.1007/BF02792160. - Brill, S; Gurman, GM; Fisher, A (2003). "A history of neuraxial administration of local analgesics and opioids". European Journal of Anaesthesiology 20 (9): 682–9. doi:10.1017/S026502150300111X. ISSN 0265-0215. PMID 12974588. - Toski, Judith A; Bacon, Douglas R; Calverley, Rod K (2001). The history of Anesthesiology. In: Barash, Paul G; Cullen, Bruce F; Stoelting, Robert K. Clinical Anesthesia (4 ed.) (Lippincott Williams & Wilkins). p. 3. ISBN 978-0-7817-2268-1. - Hademenos, George J.; Murphree, Shaun; Zahler, Kathy; Warner, Jennifer M. (2008-11-12). McGraw-Hill's PCAT. McGraw-Hill. p. 39. ISBN 978-0-07-160045-3. Retrieved 2011-05-25. - Fenster, JM (2001). "Power Struggle". Ether Day: The Strange Tale of America's Greatest Medical Discovery and the Haunted Men Who Made It. New York: HarperCollins. pp. 106–16. ISBN 978-0-06-019523-6. - Long, CW (1849). "An account of the first use of Sulphuric Ether by Inhalation as an Anesthetic in Surgical Operations". Southern Medical and Surgical Journal 5: 705–713. - Morkel, H (16 Oct 2013). "The painful story behind modern anesthesia". - BAILLIE T.W. (1965). "The first European trial of anaesthetic ether. The Dumfries claim". British Journal of Anaesthesia 37: 952. doi:10.1093/bja/37.12.952. - McAuliffe, MS; Henry, B (2010). "Nurse anesthesia worldwide: practice, education and regulation". Downloads. Silver Spring, Maryland: International Federation of Nurse Anesthetists. Retrieved 2012-06-13. - Abenstein, JP; Long, KH; McGlinch, BP; Dietz, NM (2007). "Is Physician Anesthesia Cost-Effective?". Anesthesia & Analgesia 98 (3): 750–7. doi:10.1213/01.ANE.0000100945.56081.AC. PMID 14980932. - Rosenbach, ML; Cromwell, J (2007). "When do anesthesiologists delegate?". Med Care 27 (5): 453–65. doi:10.1097/00005650-198905000-00002. PMID 2725080. - "Five facts about AAs". American Academy of Anesthesiologist Assistants. Archived from the original on 2006-09-26. Retrieved 2010-11-25. - NICE Guidelines on pre-operative tests - ASA Physical Status Classification - DMOZ link to anesthesia society sites	2	45	6	0	1	0	0.761287	7	9,435
Type 2 Diabetes Type 2 diabetes is a chronic disease in which your body is unable to maintain a normal blood sugar (glucose) level. - Birth Control Facts about birth control to help decide which type is right for you. - Type 2 Diabetes Basic facts about type 2 diabetes & risk factors to be aware of. - COPD Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Flu Facts about influenza (flu), including symptoms and vaccines. - GERD Get the facts about gastroesophageal reflux disease (GERD). - HIV/AIDS The differences between HIV & AIDS; signs, symptoms & complications. - Lupus Get the facts about lupus, including symptoms, risk factors, and the different types. - Menopause Facts about menopause, including the stages, symptoms, and types. - Shingles Facts about shingles, including symptoms & possible long-term effects. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides About Dr. Sharafali Y Diwan Dr. Sharafali Y Diwan, MD is a Doctor primarily located in Houston, TX. He has 29 years of experience. His specialties include Infectious Disease and Internal Medicine. Dr. Diwan is affiliated with Houston Northwest Medical Center, Tomball Regional Medical Center and Houston Methodist Willowbrook Hospital. He speaks English. Dr. Sharafali Y Diwan has the following 2 specialties - Infectious Disease An infectious disease specialist has specialized training in the diagnosis and treatment of contagious diseases. Infectious diseases, also known as contagious or transmissible diseases, are those that stem from pathogen from a host organism. These infections may spread to other carriers through physical touch, airborne inhalation, bodily fluids or contaminated foods. Infectious disease specialists identify whether the disease is caused by bacteria, a virus, a fungus or a parasite often through blood tests and then determine what course of treatment, if any, is necessary. - Internal Medicine An internist is a physician who focuses on the diagnosis and treatment of conditions that affect the adult population—both acute and chronic. These doctors are often who adults see as their primary physicians because they treat a broad range of illnesses that do not require surgical or specialist interventions. They also work to help a patient maintain optimal health in order to prevent the onset of disease. In addition to treating the common cold and flu, internists also treat chronic diseases like diabetes and heart disease. - Bone marrow inflammation (Osteomyelitis) - AIDS/HIV (Acquired Immunodeficiency Syndrome) - Hepatitis C - STD (Sexually Transmitted Diseases) - Human Immunodeficiency Virus (HIV/AIDS) - Sinus Infection (Sinusitis) - Lyme Disease - Tropical Diseases Showing 5 of 8 Dr. Diwan treated me in 2005 while I was as in patient at HNW Medical Center he saved my life, if it had not been for his course of treatment I would not be here today. I have continued on as a patient of his since then and would not see ANYONE else. Excellent bedside manor, he remembers you and is a caring professional. His office staff is second to none. I was critically ill when Dr Diwan treated me, when my regular doctor had given up Dr. Diwan who was also treating me stepped up and changed the course of treatment changing antibiotics and just treating me instead of deciding to take me out of ICU because it was a waste of money to continue to care for me and also that there were other patients who needed the ICU unit and the ventilator more than I(my regular Dr expressed this to my family) I have seen Dr. Diwan since 2005 and her is the best in everything he does. Dr. Diwan is affiliated (can practice and admit patients) with the following hospital(s). 29 Years Experience Dow Medical College Graduated in 1989 Dr. Sharafali Y Diwan accepts the following insurance providers. - Aetna Choice POS II - Aetna HMO - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO BCBS Blue Card - BCBS Blue Card PPO - BCBS TX BlueChoice - BCBS TX HMO Blue Texas - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - Multiplan PPO - UHC Choice Plus POS - UHC Navigate HMO - UHC Navigate POS - UHC Options PPO - View by Location Locations & DirectionsSharafali Y Diwan Md, 18220 State Highway 249 Ste 230, Houston, TX Dr. Sharafali Y Diwan is similar to the following 3 Doctors near Houston, TX.	2	6	1	0	0	0	0.877374	1	1,053
Describe the capsular structure of a lymph node; what are the functions of the LN? Capsule of lymph node is made up of type III collagen (made by reticulin fibers!), extension of this collagen extends into the splenic tissue as trabeculae; nonspecific filtration of lymph by Macrophages, Antibody production (via activation), storage and activation of B and T cells via bringing antigens from lymph by APCs or alone T/F a lymph node is a primary lymphoid organ. Describe the interstitial tissue of a spleen including the sinuses. What type of cells are found in the four structures (cortex, paracortex, medulla and sinuses)? The interstitial tissue of a lymph node is structured into the cortex on the outside which is densely cellular which transitions into the paracortex which is less dense and then the medulla which is least dense. The cells that inhabit these tissues are B cells (in both inactive and active follicles), T cells, and plasma cells respectively. The cells came from the lymph and vasculature. Afferent lymphatics pierce the capsular structure and empty into the subscapular sinuses which are continuous with the cortical sinuses which are then continuous with the paracortical sinuses and then the medullary sinuses. These sinuses are closely adjunct to the cords of the corresponding tissue and allow for interaction between lymph and the lymphocytes in the ln tissue and are lined with macrophages that engulf antigens and present them to the lymphocytes in the adjunct cords. The sinuses empty in the efferent lymphatics. T/F the secondary follicles found in the cortex of a lymph node are densely colored. False, secondary follicles have pale central germinal centers; primary follicles are dense What are HEV? Where are they found? where does the vasculature of the lymph node travel to? High endothelial venules are post capillary cuboidal endothelial cells that contain specific adhesion molecules for lymphocytes with specific binders that allows for lymphocytes to drain out of the vasculature into the lymph node structure (this is how these lymphocytes get there in the first place!); They are found in the paracortical section of the LN; the vasculature travels through the entire ln, once lymph dont find an antigen to attack they empty back into veins or into efferent lymphatics What happens in a secondary follicle? isotype switching (activated by Th2 cell signals and cytokines), somatic hypermutation What happens when a T helper cell in the paracortical section encounters an antigen? a cytotoxic t cell? a B cell in the cortical section? An antigen encounter (presented by APC for Th2) with Th2 will result in it to go to the cortical section and help with B cell activation; Cytotoxic T cell will enter vasculature/efferent lymph to go kill; B cell will wait for Th2 for activation and then follicular dendritic cells will select for plasma cell which will go to medulla and secrete Ab What part of the lymph node specifically expands during a cellular immune response? when would this occur? Paracortex; viral infection what can cause a lymph node enlargement? local infection/inflammation; infection of the ln itself; metastasis What bugs can actually infect the lymph node itself? What portion of the lymph node is not well developed in DiGeorge Syndrome? what lymph node drains the upper limb? what lymph node drains the breast? what lymph node drains the stomach? what lymph node drains the duodenum, jejunum? what lymph node drains the sigmoid colon? what lymph node drains the rectum (above the pectinate line)? what lymph node drains the anal canal (below the pectinate line)? what lymph node drains the scrotum? what lymph node drains the testes? what lymph node drains the thigh? what lymph node drains the lateral side of the dorsum of the foot? what are the sinusoids of the spleen? what is the difference between a spleen and a lymph node? the red pulp of the spleen its where the vasculature channels go through and interact with the parenchyma of the spleen (has macrophages) which empty in the sinuses; they are both encapsulated (with trabeculae) secondary lymphoid organs that trap antigens from local tissue but the spleen also does the same for the blood! what type of fenestrations are found in the red pulp of the spleen? barrel hoop basement membrane fenestrations what is the white pulp of the spleen? surrounds the red pulp and begins with PALS and then turns into follicles with B cells/follicles what is the marginal zone of the spleen? what happens there? found in the red pulp; contains the cords of billroth or the splenic parenchyma that have APCs/Macrophages that closely interact with the basement membrane of the vasculature and where RBCs squeeze through into the parenchyma and interact with Macrophages and those not eaten empty back into venous sinuses what are the PALS? periarteriolar lymphatic sheath in the white pulp that surround that dead end of the capillaries in the red pulp, contain T cells what is the two fates of the RBCs that go through the spleen? what happens eventually to all of them> closed circuit where go directly to veins and open where squeeze out of capillary sinusoids and interact with either PALS or with macrophages in the cords of Billroth (for antigen immune function or for blood cleaning or for both) and squeeze back into venous sinuses; all end up going through open circuit other than eat and bite RBCs what else do Macrophages of spleen do> what bacteria are a splenectomy patient most susceptible to? why? N. meningitidis, H. influenzae, S. pneumonia, Salmonella d/t lack of C3b opsonization for MAC d/t lack of complement activation d/t lack of IgM; MAC is needed by encapsulated avoid by humoral and cell mediated through their capsule what are howell jolly bodies? nuclear remnants that are usually taken out of RBC before complete maturation by Macrophages what are target cells? cells that stil have weird parts of their membrane that macrophages usually bite off what is the thymus ? where is it located? is it encapsulated? how many lobes does it have? the place of T cell diff and maturation; ant mediastinum above heart; yes; 2 T/F the thymus and its comp derived from 3rd branchial pouch false- the lymphocytes are mesenchymal origin how is the thymus organized? what happens in each section? Into cortex and medulla; cortex is where immature T lymphocytes enter, at the corticomedullar junction is where positive and negative selection occur; and at the medulla is where the mature T lymphs, reticulin cells and hassalls corpuscles are (dead reticular cells) in thymic development, what is the positive selection? negative selections? when you select for which MHC it will have; take out the lymphs that self react are the MALT/GALT encapsulated? which type of selection of thymic development provides central tolerance? what links the adaptive and innate immunity? complement activation (active in both) what kinds of receptors activate innate immunity? TLR ad nuclear receptors T/F RBCs have blood group antigens. T/F RBCs have MHC class glycoproteins. What are MHC Class molecules (ie what macromolecule are they made out of)? what gene are responsible for MHC? Which HLA's are included in MHC I? MHC II? A, B, C; all the D's What are MHC's necessary for? By themselves? T cell activation; no with CD 4 or CD 8 What is MHC I made out of? where is it found? What does it bind to? What type of antigens does it present? an alpha chain and a B2 microglobulin; on all nucleated cells; TCR and CD8; intracellular antigens Which TCR MHC system is important for viral immunity? neoplastic? donor graft cells? All MHC 1/CD8 What is MHC II made out of? where is it found? what does it bind to? What type of antigens does it present? an alpha and beta chain; only on APCs; TCR and CD4; extracellular (phagocytosed by APCs) how is the antigen loaded onto a MHC II? an acidified endosome with the antigen fuses with the MHC which causes the release of the invariant chain which is sitting in spot of antigen and stabilizing the MHC II which MHC presents intracellular peptides? how so? MHC I; from RER with help of the B2 microglobulin which disease is associated with HLA A3? which disease is associated with B27? (PAIR) Psoriasis, Ankylosis Spondylitis, IBD, Reiters; all seronegative spondylopathies Which disease is associated with B8? which diseases are associated with DR2? MS, hay fever, SLE, goodpastures which disease is associated with DR3? Diabetes mellitus (DM) type I Which diseases are associated with DR4? DM type I and rheumatoid arthritis (RA) Which diseases are associated with DR5? Pernicious Anemia and Hashimotos which disease is associated with DR7? Steroid responsive nephrotic syndrome What are the three types of lymphocytes? B, T, and NK cells T/F all the lymphocytes are apart of the innate system. False! only NK cells What type of cells do NK cells attack? with what tools? by necrosis or apoptosis? tumor cells and virally infected cells; perforin pore to secrete granzymes through it; apoptosis which interleukin receptor is required for NK development? activation? IL 12, interferon Beta and interferon alpha what are the two signals to kill for NK cells? lack of MHC class I constant region which it binds or a nonspecific activation signal (TLR) what prevents NK cells from killing normal cells if their default is to kill? MHC class molecules bind to KIRS or CD94 to prevent killing Which T helper cell activated Macrophages? by secreting what? what else does Th1 secrete? for what? Th1; interferon gamma; IL 2 for cytotoxic T cell activation How is Th1 helper cell inhibited? cytokine IL 10 secreted by Th2 what do macrophages secrete that activate Th1 cells to secrete interferon gamma? TNF alpha and IL1 T/F the Th2 regulate the cell mediated response. Which cytokines do Th2 release and for what? IL 4,5,6 for B cell class switching, IL 10 to inhibit TH1 what secretes IL 4? basophils! THey want IG E class switch! which cytokine inhibits TH2 cells? secreted by who? Interferon gamma; Th1 what does granzyme do? who secretes it? its a serine protease that activates apoptosis; NK and CD8 what does granulysin do? antimicrobial also secreted by CD8 and NK cells that induces apoptsosis what is the general structure of an Ab? 2 heavy chains and two light chains T/F the two constant parts of the light chains of an antibody contribute to the Fc region FALSE. only the heavy chains contribute to the Fc region T/F the two heavy chains of an antibody contribute to the Fab portion T/F the Fab (antigen binding) determines the isotype false! idiotype; the Fc portion determines the isotype To what portion of the Antibody do the complements bind? T/F the Fc region is found on the N terminal False; found on the carboxy terminal what type of side chains are found on Fc region of an antibody? So antibodies are the effectors for the humoral response. List some of their functions. cross link antigens which increases their phagocytability; activate complement for MAC attack; opsonization; neutralization Name 5 ways Antibody diversity is generated? 1) VJ light chain random creation 2) VDJ (heavy chain) random creation 3) random combination of light with heavy 4) somatic hypermutation (high frequency mutating that occurs on activation) 5) terminal deoxynucleotidyl transferase (TDT) random addition of nucleotides at the end of the chains where do somatic hypermutation and class switching occur? in the germinal center of secondary follicles (In the paler center) what do multimeric antibodies require for assembly? A j chain Which antibodies can be multimeric? IgM and IgA which antibodies can bind complement? IgM and IgG which antibodies prevent antigens from binding mucosal surfaces? which antibody activate mast cells, basophils, and eosinophils? what happens in order for class switching to occur (after being activated by IL and cd40 L)? alternative splicing of mRNA which is the main antibody in the delayed or secondary response to an antigen? which is the most abundant antibody in blood? can igG cross the placenta? which is the main antibody that provides passive immunity to infants? T/F IgG opsonizes. T/F IgG can not neutralize. T/F IgA fixes complement. T/F IgA is a dimer in circulation. False! Monomer in circulation, dimer when secreted How does igA cross the epithelium? what is colostrum? the igA found in breast milk What does IgA pick up from epithelial cells before being secreted? which antibody is involved in the primary response or immediate response to an antigen? T/F IgM can both fix complement and cross the placenta false! can fix complement but cant cross placenta T/F IgM is a monomer False! IgM can exist as a pentamer also What does it mean if there are igM in the serum at birth? since cant cross placenta most likely means that child was forced early to produce due to an infection (most likely CMV) what do mature naive B lymphocytes express? IgM and IgD T/F IgE has the lowest concentration in the serum What does IgE do on the surface of the mast cell to induce inflammatory mediator release? Which antibody mediates immunity to worms? how? IgE; by activating eosinophils If an antigen lacks a peptide component how does the adaptive immunity attack it? what type of response is this called. give an example of bugs that do this. what implications does this have on splenectomy? This means that MHC can not recognize it and thus will not be phagocytosed. The humoral response rescues- IgM is the primary response attack. Since there is no class switching (without MHC activation no CD40 L and interleukins to activate!) then there is memory and igE attack is all we get; this is called Thymus independent antigens; Bugs that do this are bugs with lipopolysaccharide from envelope of gram negative or polysaccharide capsular antigen. Since spleen is where all igM are housed and produced, then there will be increased risk for infection by these types of organisms that lack a peptide component. how do we use thymus dependent antigens to prevent infection from organisms that lack a peptide component? give a vaccine with a peptide comp from it that the cell mediated immunity otherwise cant get to! like h.influenzae vaccine. then class switching and memory response can occur what ensure that a memory response is generated? if there is class switching and plasma cell production (that is when memory cells are produced) what are complements in the complement system? what activates them? there seems to be different ones, what are these pathways called? adaptive uses classic pathway to kill; innate uses alternative pathway to kill What is the end result of complement activation? what bugs are this important for? through what pathway and why? MAC (membrane attack complex) which is activated by C5b-C9 and results in lysis and cytoxicity by creating pores in the target membrane; encapsulated organisms (S. pnuemonia, H.influenzae, B perfussis for example) and other organisms with non peptide antigens because IgM coats these and through the classic pathway these can then be killed what part of the complement system also acts as an opsonin? what is opsonization? can you name two other opsonins? C3b; coating of a pathogen with molecules that promotes phagocytosis; IgG and CRP T/F complements are acute phase reactants. where are complements produced? liver! (they are proteins circulating in the blood) How does the alternative pathway lead to MAC activation? soluble C3 spontaneously hydrolyzes spontaneously to C3b and opsonizes microbial and host cell surfaces and accumulates on surfaces; C3b activates C5 convertase which leads to MAC activation T/F the alternative pathway is the only constutively active complement pathway If the alternative pathway is constitively active, how come normal cells don't get attacked with MAC? DAF and C1 esterase inhibitor on cells prevent complement activation on their surfaces (microbial surfaces lack this) after C3 spontaneously hydrolyzes to C3b and C3a, what happens to C3a? its involved in inflammation and results in anaphylaxis and neutrophil chemotaxis. other than C3a, what other complement acts as an anaphyloxin? Describe the Mannose Lectin pathway some microbial surfaces (only) have mannan binding lectin which activates a protease that cleaves C2 and C4 which combine to make C3. the rest follows the alternative pathway describe the classic complement pathway. IgG and IgM bind to pathogens activating C1 convertase and leads to C2 and C4 and then C3 convertase activated what are C1, C2, C3, C4 important for? viral neutralization of igM and IgG! what happens in a deficiency of C1 esterase inhibitor? DAF? hereditary angioedema; PNH What happens in a deficiency of C3? severe recurrent pyogenic sinus and resp tract AND increased susceptibility to type III hypersensitivity like SLE what happens in a deficiency of C5-C8? why dont you get recurrent pyogenic infections like in C3 def? septicemia with neisseria; because at least you have C3 to act like a neutrophilic chemotactic and an acute phase reactant what is hereditary angioedema? What are the C3 levels? Aut. Dominant; C1 esterase inhibitor deficiency resulting in continued C1 which results in increased C2 and C4 cleavage products which have anaphyltoxic activity and result in swelling of face and oropharynx In order to produce Antibodies, does the antigen have to be phagocytosed? give an example with a bug and an autoimmune (type II hypersensitivity for example). no! That is why they are different from T cells- they can recognize antigens in free solution; if a bug has a peptide fragment that a mature naive B cell recognizes it will bind to it and cause activation, it then gets activated into a plasma cell! same for type II or III hypersensitivity except the antigen is usually harmless (like an RBC cell slight altered with penicillin on it). explain the process from beginning (ie phagocytosis of the peptide) to end of how Abs are formed in Goodpasteurs. for some reason a mature naive B lymphocyte got away from tolerance and the result was a production an Ab on its surface with an Fab region that recognized the alpha 3 collagen subunit on the BM as non self. It then gets activated by a Th2 cell (entire immune system recognizes it as non self) is IgM an opsonizer? Name the three opsonins CRP, C3b, IgM name two endogenous pyrogens IL 1 and IL 6 Name three things that IL 1 does as a cytokine. other than the liver, who secretes IL 1 induces fever, chemokine secretion to recruit, activates adhesion molecules; macrophages Other than stimulating fever, what else does IL 6 do? stimulate the liver to release acute phase reactants what is the main function of IL 8? a chemotactic factor for neutrophils from where do cytokines come from? what is the main function of IL 12? other than macrophages who else can release IL 12? induce differentiation of T cells into Th1 helper cells and activates NK cells also; B cells what is the main function of TNF alpha? how does it do this? mediates septic shock; activates the endothelium for adhesion and dilation and leukocyte recruitment results in vascular leak other than mediating shock, what else does TNF alpha do? who releases it mainly? activates Th1 helper cells; Macrophages What cytokines do macrophages release? who else can secrete IL 6? IL 12? IL 1, IL 6, IL 12, TNF alpha; IL 6- Th2 cells; IL 12- B cells what is the main cytokine released by T cells? what does it do IL 3; supports growth and differentiation of bone marrow stem cells What cytokines are released by Th1 cells? Interferon gamma and IL 2 what does IL 2 do? activates cytotoxic CD 8 T cells as second signal What does interferon gamma do? what two type of cells does it attack mostly? activates Macrophages and Th1 cells; suppresses Th2 cells; antiviral and antitumor What cytokines to Th2 secrete? IL 4, 5, 10, 6 What does IL 4 do? acts as second signal on B cells to induce class switching to IgE and IgG what does IL 5 do? acts as second signal on B cells to induce class switching to IgA; also stimulates eosinophils what is the main cytokine that activates eosinophils? what cytokine does basophils secrete? what does IL 10 do? who is secreted by? suppresses immune response (also specifically Th1 cells) and activates Th2; regulatory T cells Which are the only two antiinflammatory cytokines? TGF beta and IL 10 what is the main function of interferons? antiviral for uninfected cells (for future protection) and kills virally infected cells (NK activation) what does Interferon alpha and beta do? how? antiviral by inducing ribonuclease which degrades only viral mRNA (prevents protein synthesis) and activates NK cells to kill virus infected cells what does interferon gamma do to be antiviral? increases expression of MHC I and MHC II and also activates NK cells what else does interferon gamma do other than inducing ribonuclease production, activating NK cells, inducing increased MHC expression? what are the main Cell surface proteins on T cells? what are the main cell surface proteins on B cells? Ig, CD19, CD20, CD21, CD40, MHC II, B7 what is the receptor for EBV? on what cells is that located? CD21 on B cells (although there is T cell lymphocytosis in EBV) What are the cell surface proteins for Macrophages? which two are for opsonins? MHC II, B7, CD40 and CD14; CD40 and D14 for FcG and C3b resp what cell surface proteins are on all APCs? MHC II, B7 what are the cell surface proteins on NK cells? MHC I, CD16, CD56 what does CD16 on NK cells do? bind FcG for antibody dependent cellular cytotoxicity what cell surface marker is used for NK cells as it is unique to them? what will NK cells do to cells covered in IgG Ab? why? kill them because they have CD16 on them that recognize the FcG portion what is anergy? why does this occur? lack of response from lymphocytes when activated; because they are self reactive, this is a form of tolerance which cells have more complete tolerance- B or T cells? what are superantigens? give two examples. CROSS LINK Beta region on TCR of CD4 cells to the MHC class II on APCs this results in uncoordinated release of Interferon gamma from CD4 Th1 cells and subsequent release of IL1, IL6 and TNF alpha from Macrophages --> toxic shock syndrome; s. pyogene and s. aureus both have this toxin how do endotoxin/LPS of gram negative bacteria stimulate the immune system if they do not have a peptide fragment? they directly stimulate Macrophages by binding CD14; also the alternative complement pathway binds to these host surfaces and induces MAC complex; also TLRs exist for endotoxins; also IgM though not an opsonin can act as a pentamer and trap the antigen which can activate classical pathway complement activation are Th cells involved in trapping of antigens of endotoxin/LPS? no because no peptide fragment! Give three examples of bacteria that use antigenic variation and how. Salmonella- 2 flagellar variants; Borrelia (relapsing fever); and Neisseria gonorrhoaea- pilus protein give an example of a virus that uses antigenic variation. what does a major variation result in? minor? influenza; antigenic shift; antigenic drift what is an example of a parasite showing antigenic variation? trypanosomes with programmed rearrangement (results in recurring fever-- sleeping sickness!) give an example of how influenza does a major antigenic shift. RNA segment reassortment which type of immunity is slow but long lasting? as opposed to... active; passive- fast but short half life (3 weeks!) what is the most common example of passive immunity? IgAs in mothers breast milk! For which toxins are preformed antibodies (passive) given? Tetanus, Botulinum, HBV, Rabies what is passive immunity? receiving preformed Antibodies what is a type I hypersensitivity reaction? what is atopic? an anaphylactic immediate reaction; atopy refers to the inherited propensity to develop asthmatic or allergic reactions what is the pathogenesis of a hypersensitivity reaction? first a B cell gets sensitized to an allergen, after sensitization, it gets induce by Th2 secreting IL4 to class switch from IgM to IgE, next time blood stream is exposed to allergen these antigens cross like IgEs on mast cells and result in chemical mediator release what amine is the main chemical mediator released by mast cells? where does it act what does it result in? histamine; post capillary venules- vasodilation what is the late phase reaction of anaphylaxis allergy? what mediates it? hours later (instead of minutes); instead of release of preformed mediators, mast cells synthesize PGs and LTs that mediate the late phase reaction (edema, inflammation, decreased airflow) what are the mediators that mast cells release? histamine (causes vasodilation), leukotrienes (causes bronchoconstriction), chemotaxins for eos, and heparin (increased blood flow needs to be anticoagulated!) what two ways do you test for a type 1 hypersensitivity reaction? what will you see? scratch test - histamine mediated wheal; radioimmunosorbent assay (detects specific IgEs in serum) what mediates the type II hypersensitivity? what are the two different methods? Antibody mediated cytotoxicity; either complement dependent or complement independent Describe complement dependent Type II hypersensitivity. Give an example. IgM or IgG antibodies coat the antigen and result in activation of MAC complex via the classical pathway OR fixed macrophages in the spleen phagoctyose the opsonized (C3b or igG) antigens- ex penicillin reaction; IgM AIHA, anti B IgMs in a group A blood donor to a group B recipient Describe the complement independent Type II hypersenstivity reaction. Give an example. The antibodies (igM or IgG) either neutralize the target directly (cytoxic!) or recruit leukocytes (Neutrophils and Macrophages!) to incite tissue damage what are the three types of Type III hypersensitivity ? what is the common mechanism between them? Immune complex, Arthus reaction and Serum sickness; they are all a result of antibody-antigen complex deposition resulting in complement activation what is immune complex disease? give an example. type III hypersensitivity where an internal antigen antibody complexes activate classical complement pathway resulting in C3a neutrophilic chemotaxis and neutrophilic lysosomal death. SLE what is serum sickness? give an example. type III hypersensitivity where an exogenous antigen results in a systemic antigen antibody complex disease (takes around 5 days after exposure for the Abs to develop); immune complexes then fix in tissue and activate complement resulting in tissue damage; horse antithymocyte globulin Ab what is the arthus reaction? what is the difference between arthus and serum sickness? give an example. how do you test for it? it is a localized type III hypersensitivity reaction to an external antigen; localized instead of systemic; ?; intradermal injection of the antigen results in antibody production and antigen antibody complexes deposit in the skin what characterizes an arthus reaction? edema and necrosis in that region how do you test for type III hypersensitivity? immunoflourescent staining of tissue biopsies which of the hypersensitivity reactions is not Ab mediated? T/F type Iv hypersensitivity is an immediate reaction T/F type IV hypersensitivity is induceable by a serum transfer false! not Ab mediated describe the pathogenesis of delayed type IV hypersensitivity T cells react to the angtigen and activate leukocyted (macrophage acitivation) T/F all transplant rejections are mediated by Type IV hypersensitivity false- all except hyperacute T/F DTH (delayed type hypersensitivity) is the pathogenesis of a PPD reaction what is the pathogenesis of a candida skin test? delayed type hypersensitivity T/F the pathogenesis of contact dermatitis is type III hypersensitivity false! type four what is the pathogenesis of IgG AIHA ABO hemolytic disease of a newborn? describe what happens. Type II hypersensitivity, complement dependent resulting in phagocytosis of RBCs coated with C3b by fixed macrophages in the spleen; Group O mother has anti A,B, IgG Abs that cross placenta and attach to fetal blood group A or B RBCs what is the difference of IgE AIHA and IgG AIHA if they are both complement dependent type II hypersensitivities? in IgE AIHA- it results in MAC attack (remember IgE is not an opsonin!), in IgG AIHA C3b (and IgG) opsonization results in phagocytosis by fixed macrophages in the spleen what results in symptoms of shock in an acute hemolytic transfusion reaction? since it is a comp dependent type II hypersensitivity reaction; C3a brings anaphylotoxins that bring in mediators of shock what is the autoantibody for SLE that is nonspecific? Specific? Anti nuclear Antibodies (ANA); Anti dsDNA, anti Smith what are the autoantibodies for drug induced lupus? To what disease do the autoantibodies to IgG (rheumatoid factor)? what are the autoantibodies for scleroderma (CREST)? scleroderma diffuse? anticentromere; anti Scl-70 (anti DNA topoisomerase I) what are the autoantibodies for primary biliary cirrhosis? what are the autoantibodies for Celiac disease? Antigliadin, antiendomysial (both are IgAs- anti tissue transgluataminase igA) what are the autoantibodies for goodpastures syndrome? Anti alpha subunit 3 of collagen on type IV bm what are the autoantibodies for pemphigus bulgaris? what are the autoantibodies for hashimotos? antimicrosomal and antithyroglobulin what are the autoantibodies for polymyositis and dermatomyositis? what are the autoantibodies for sjorgens syndrome? Anti SS-A (anti RO) and Anti SS-B what are the autoantibodies for Mixed connective tissue disease? Anti U1 RNP (ribonucleoprotein) what are the autoantibodies for autoimmune hepatitis? anti smooth muscle what are the autoantibodies for type I diabetes mellitus? anti glutamate carboxylase and anti insulin what are the autoantibodies for wegeners granulomatosis? c-ANCA (antiproteinase); in neutrophil; c= cytoplasmic what are the autoantibodies for other vasculitides? p-ANCA (antimyeloperoxidase); in neutrophil; p= perinuclear what are the autoantibodies for myasthenia gravis? anti Ach receptor what are the autoantibodies for graves? anti TSh receptor what are the autoantibodies for pernicious anemia? what are the autoantibodies for systemic sclerosis? what are the three immune privileged sites? why are they called that? what happens after infection in these areas? brain, eyes, placenta/fetus, testicles; because they can have an antigen in there and not get attacked by immune system because dont have contact with immune system via blood and lymph; if an infection occurs such that trauma results in exposure to immune response- takes a long time time- prolonges uveitis and orchitis what is the defect in Brutons agammaglobulinemia? what is its effect on B cells? what is its inheritance pattern? defect in BTK a tyrosine kinase gene; failed maturation of B cells; X linked recessive with failed maturation of B cells in Brutons agammaglobulinemia, what is its effect on immune pathways and why? its main effect is a defect in Ab opsonization for killing what is the presentation of Brutons agammaglobulinemia? recurrent sinopulmonary bacterial infections after 6 months (d/t mothers igGs transplacental) due to opsonization defect what are some sinopulmonary infections? sinusitis, otitis media, pneumonia what are the labs in brutons agammaglobulinemia? normal pro B (CD 19 +), no mature B cells results in decreased total B cell level, and decreased amount of immunoglobulins in each class what is the defect in hyper IgM syndrome? what are the lab results? defect in CD40L results in inability to class switch; a lot of IgM and no IgG, A, E what is the presentation of hyperIgM syndrome? severe pyogenic infections early in life What is the most common selective Ig deficiency? what is the presentation? IgA; sinus and lung infections from lack of secretory defense, milk allergies and diarrhea from giardiasis; Anaphylasix on exposure to blood products with igA what is the common variable immunodeficiency ? how is it different from Brutons? defect in B cell maturation; idiopathic, presents at older age and normal number of B cells and hyperplastic germinal centers because B cells can be activated but can not produce Abs what is the presentation of common variable immunodef? and what are the labs? can be acquired in 20s-30s; increased risk of autoimmune disease, lymphoma, Sp infections; normal number of B cells and no plasma cells and immunoglobulin what are the main symptoms of B cell immunodeficiencies? what are the main symptoms of T cell immunodeficiencies? intracellular pathogens (acute and chronic viruses and virally induced cancers) what is the cause of thymic aplasia? what is its presentation? what are the labs? Digeorge syndrome- 22q11 deletion resulting in failure to develop 3rd and 4th pharyngeal pouches; cardiac and great vessel congenital defects, tetany from hypocalcemia, recurrent viral/fungal infections from T cell deficiency; hypoPTH, hypoCa, and absent thymic shadow on CXR what is the result of an IL 12 deficiency? what is the presentation? what are the labs? what is the defect in Leukocyte adhesion defect? what is the presentation? what are the labs? defect in LFA 1 integrin (CD 18) protein on phagocytes (neutrophils!); recurrent bacterial infections, severe gingivitis, poor wound healing, absent pus formation, and delayed separation of the umbilicus; neutrophilia T/F leukocyte adhesion defect presents with neutropenia which immunodeficiency presents with delayed separation of the umbilicus? ataxia? telangiectasia?albinism? anaphylaxis on exposure to blood products with IgA? tetany?retained primary teeth? peripheral neuropathy? what is a factor that is a predictor for a bad transplantation? patient has had previous exposure to blood products which result in presence of preformed anti HLA cytotoxic Abs what is an autograft? syngeneic graft? allograft? xenograft? what is an ex of an allograft? xenograft? from self; from identical twin or clone; from nonidentical individual of same species; from different species; fetus; pig valve which of the transplant rejections is antibody mediated? why does it occur? hyperacute; presence of preformed antibodies in the recipient ( to blood groups or HLA ) give an example of someone who could get hyperacute transplant rejection. previous transfusion; pregnant woman whose fetus had paternal antigens how fast does it occur? the patient could become cyanotic in the OR! what is the pathology in hyperacute transplant rejection? ischemia and necrosis of graft due to occlusion of vessels and fibrinoid necrosis and thrombosis within the vessels what is the pathogenesis of acute transplant rejection? when does it occur? cell mediated due to T cell cytotoxicity reacting to foreign MHCs; few weeks after what is the pathology of acute transplant rejection? is it reversible? vasculitis of graft vessels with dense interstitial lymphocytic infiltrate; yes! with immunosuppressants what is the treatment of acute transplant rejection? what is the pathogenesis of chronic transplant rejection? when does it occur? is it reversible? T cell and antibody mediated vascular damage due to MHC non self being recognized as self by self lymphocytes and resulting in attack of the foreign antigens it presents (all of them); months to years after; no :( what is the pathology seen in chronic transplant rejection? fibrosis of blood vessels and graft tissue from ischemia; obliterative vascular fibrosis with intimal thickening when can graft versus host disease? what is the result? in any situation where immunologically competent cells are transplanted into immunologically crippled recipient; graft rejects all the cells due to foreign proteins resulting in severe organ dysfunction what is the symptoms involved in graft versus host disease? it what transplant cases does it usually occur? give an example maculopapular rash (palm, soles, back, neck), jaundice with bile duct necrosis, hepatosplenomegaly, diarrhea; bone marrow and liver transplants (rich with lymphocytes); SCID patient receiving whole blood transfusion	2	10	0	0	0	3	0.365816	3	9,016
Coding Screening for Colorectal Polyp. Colorectal cancer typically develops from a polyp. Polyps are abnormal growths of tissue (neoplasms) that can be found in any organ that has blood vessels — and the colorectal area has a rich supply of blood vessels. Most polyps are benign, but they can become cancerous. Colon polyps found in the early stages usually can be removed safely and completely. Symptoms may include bleeding from the anus, constipation or diarrhea that lasts more than a week, and hematochezia (blood in the stool) that can make stool look black or show up as red streaks in the stool. Most colon polyps are asymptomatic and people are unaware of them until one or more is found during a screening or other testing. When selecting an ICD-10 diagnosis code for colon polyps, the precise location of the polyp(s) is key. The type of polyp (e.g., benign, inflammatory, etc.), as confirmed by biopsy, is also required. Codes for benign adenomatous polyp of the colon are found in the neoplasm chapter of ICD-10-CM, by location: - 0Benign neoplasm of cecum - 1Benign neoplasm of appendix - 2Benign neoplasm of ascending colon - 3Benign neoplasm of transverse colon - 4Benign neoplasm of descending colon - 5Benign neoplasm of sigmoid colon - 6Benign neoplasm of colon, unspecified [Note: This code includes adenomatous polyp of colon and polyposis of colon] - 7Benign neoplasm of rectosigmoid junction - 8Benign neoplasm of the rectum Codes for inflammatory colon polyps, found in category K51 Ulcerative colitis, also are considered possible complications: - 40Inflammatory polyps of colon without complications - 411Inflammatory polyps of colon with rectal bleeding - 412Inflammatory polyps of colon with intestinal obstruction - 413Inflammatory polyps of colon with fistula - 414Inflammatory polyps of colon with abscess - 418Inflammatory polyps of colon with other complication ICD-10 instructs to use an additional code with category K51 to identify manifestations (e.g., pyoderma gangrenosum). Polyps of the colon not documented as adenomatous, benign, or inflammatory are coded to K63.5 Polyp of colon. If a colon polyp is specified as hyperplastic, assign K63.5, even if greater specificity is provided regarding the location, according to the American Hospital Association’s (AHA) Coding Clinic for ICD-10-CM and ICD-10-PCS (Second Quarter 2015, pages 14-15). Latest changes in Otorhinolaryngology. Latest changes in Otorhinolaryngology: CPT® 2017 brings several code changes for Otorhinolaryngology, a specialty that has seen few, if any, code changes in the past several years. The changes are primarily new codes, with some code revisions, to keep the codes up to date with contemporary clinical practice. The laryngoscopy codes revised for 2017, and what’s different from 2016, are shown in Table A. These procedures involve the use of a flexible laryngoscope and are typically performed in the office setting, but may occur in the operating room. New guidelines state not to report 31575 with 31231 Nasal endoscopy, diagnostic, unilateral or bilateral (separate procedure), unless both are performed for separate conditions using separate endoscopes. Reporting both codes is not common, as both procedures are performed transnasal. The clinical rationale to support both codes should be documented clearly if both are reported. Both procedures should be separately documented, as well. New flexible laryngoscopy codes allow us to report procedures more commonly performed by otolaryngologists in the past several years. These procedures are also typically performed in the office setting, using a flexible laryngoscope, but may occur in the operating room. Here are the 2017 codes, with examples for practical application: - 31572Laryngoscopy, flexible; with ablation or destruction of lesion(s) with laser, unilateral. Code 31572 includes the removal of any number of lesions using the laser. - 31573Laryngoscopy, flexible; with therapeutic injection(s) (eg, chemodenervation agent or corticosteroid, injected percutaneous, transoral, or via endoscope channel), unilateral. Code 31573 includes one or more injections at the same session. - 31574Laryngoscopy, flexible; with injection(s) for augmentation (eg, percutaneous, transoral), unilateral. Code 31574 includes one or more injections at the same session. The procedure is commonly known as a percutaneous injection laryngoplasty. All of these laryngoscopy codes are unilateral, which means modifier 50 Bilateral procedure may be appended if performed on both paired structures of the larynx/pharynx. CPT® guidelines state paired structures include true vocal cords, arytenoids, false vocal cords, ventricles, pyriform sinuses, and aryepiglottic folds. There are six new, three revised, and two deleted codes in the Larynx Repair section of CPT® 2017. Direct laryngoscopy and bronchoscopy are included in all the open larynx repair codes for airway evaluation prior to the definitive procedure at the same operative session. The following four codes reflect procedures for resection of laryngeal stenosis. These codes are differentiated by the age of the patient, and whether an indwelling stent was placed. CPT® codes for patients younger than 12 years of age: - 31551Laryngoplasty; for laryngeal stenosis, with graft, without indwelling stent placement, younger than 12 years of age - 31553 for laryngeal stenosis, with graft, with indwelling stent placement, younger than 12 years of age CPT® code for patients aged 12 years or older: - 31552Laryngoplasty; for laryngeal stenosis, with graft, without indwelling stent placement, age 12 years or older - 31554 for laryngeal stenosis, with graft, with indwelling stent placement, age 12 years or older The graft harvest and placement is included in the above codes and should not be separately reported, even when performed through a separate incision. The remaining two new airway CPT® codes are for specific procedures: - 31591Laryngoplasty, medialization, unilateral. Code 31591 is typically performed for vocal fold paralysis. - 31592Cricotracheal resection. Code 31592 is usually performed for subglottic stenosis. There are three revised airway procedure codes in 2017. These are listed in Table B, with an explanation of the difference in code description from 2016. Two airway procedure codes, 31582 and 31588, were removed to correspond with the new and revised codes, noted above. Deletion of the “moderate sedation included” symbol () affected many otolaryngology endoscopy codes, such as 31615 Tracheobronchoscopy through established tracheostomy incision, the bronchoscopy codes (Eg: 31622 Bronchoscopy, rigid or flexible, including fluoroscopic guidance, when performed; diagnostic, with cell washing, when performed (separate procedure)), and many esophagoscopy codes (Eg: 43200 Esophagoscopy, flexible, transoral; diagnostic, including collection of specimen(s) by brushing or washing, when performed (separate procedure)). Reporting Mental Health in Drug Screening. Major changes have occurred in mental health coding and drug screen services.The evolving public awareness of mental health, parity laws, revisions to CDC guidelines, and HCPCS Level II dual coding for drug screen services prompted more changes in 2017. In 2013, CPT® created separate reporting guidelines for physicians and other mental health clinicians. CPT® instructed physicians to report medication management through evaluation and management (E/M) codes (99201-99499), with psychotherapy reported as an add-on code, because physicians focus on the medication management of the patient, with referrals to therapists and psychologists. For clinicians practicing only psychotherapy, CPT® created new psychotherapy codes. This prompted the creation of new guidelines, new definitions, and clarification on time reporting. Time-based psychotherapy codes began to adopt the Time Rule, which can be found in the introduction section of the CPT® codebook. In 2015, CPT® changed drug screening services to define them as either presumptive or definitive. The Centers for Medicare & Medicaid Services (CMS) still required providers to use an appropriate HCPCS Level II code, which CMS subsequently updated in 2016. HCPCS Level II codes adopted by CMS in 2016 for presumptive drug screen services became popular among coders and payers, forcing CPT® to adopt these code definitions in 2017. With the adoption of the new CPT® codes, CMS deleted the dual coding methodology for presumptive drug screen services in the HCPCS Level II codebook, creating a uniformed coding system for presumptive drug screen services. The new CPT® codes are: - 80305Drug test(s), presumptive, any number of drug classes, any number of devices or procedures (eg, immunoassay); capable of being read by direct optical observation only (eg, dipsticks, cups, cards, cartridges) includes sample validation when performed, per date of service [The old code was G0477.] - 80306 read by instrument assisted direct optical observation only (eg, dipsticks, cups, cards, cartridges) includes sample validation when performed, per date of service [The old code was G0478.] - 80307Drug test(s), presumptive, any number of drug classes, any number of devices or procedures, by instrument chemistry analyzers (eg, utilizing immunoassay [eg, EIA, ELISA, EMIT, FPIA, IA, KIMS, RIA]), chromatography (eg, GC, HPLC), and mass spectrometry either with or without chromatography, (eg, DART, DESI, GC-MS, GC-MS/MS, LC-MS, LC-MS/MS, LDTD, MALDI, TOF) includes sample validation when performed per date of service [The old code was G0479.] Presumptive drug screening services may be performed prior to definitive drug screen testing when a provider wants to: - Rule out illicit drug uses; - Confirm the presence of a particular drug class without identifying individual drugs; or - Distinguish between structural isomers. For example, a patient using prescription opioids for pain management may receive a randomized drug screen service to test for the presence of opioids and illicit drugs, or other prescription drugs that may cause risk to the patient when on opioids. One such risky combination, per a CDC warning, is the use of benzodiazepines with opioids. According to CPT® guidelines, sample validation in each of the codes “may include, but are not limited to, pH, specific gravity, and nitrite.” These sample validation tests are included in the drug screen services, so urinalysis, immunoassay tests, and other lab tests bundle into these codes, by definition. It is not appropriate to use modifiers 59 Distinct procedural service, XE Separate encounter, XP Separate practitioner, XS Separate structure, or XU Unusual non-overlapping service to override the bundling combination. Chapter 10 of the National Correct Coding Initiative (NCCI) Policy Manual confirms providers should not separately report the validity testing (p. X-7). CPT® also limits each of these codes to one unit per billing. Because CPT® is adopting the CMS model, CMS advises providers to report these codes for presumptive drug screening services, and is including these codes in the Clinical Laboratory Fee Schedule. Because substance abuse requires mental health intervention, it’s important to address mental health changes, as well. When the original psychotherapy codes were created in 2015, the term “with patient and/or family” was included in psychotherapy codes 90832-90838. This caused confusion, and may have caused coders and providers to use these codes erroneously for family psychotherapy (which should be reported with 90846-90847). To correct this, CPT® has removed this terminology from the codes and clarified the guidelines. The guidelines now state: Codes 90832, 90833, 90834, 90836, 90837, 90838 describe psychotherapy for the individual patient, although times are for face-to-face services with patient and may include informant(s). See codes 90846, 90847 when utilizing family psychotherapy techniques, such as focusing on family dynamics. This clarification instructs us not to use the individual psychotherapy codes for family psychotherapy, and directs us to the correct code. Doing so ensures 90832-90838 remain as individual psychotherapy codes that may involve an informant(s) to help the clinician better treat the individual patient and their individual needs. In making these changes and to provide consistency, CPT® made psychotherapy codes 90846 and 90847 time-based, 50-minute codes. Coders are further advised not to report family psychotherapy services less than 26 minutes in length. Auditors and payers will also be looking at coding based on time: - Did the documentation support the time spent performing the services? - Did the physician use time for both the E/M and the psychotherapy for the same encounter, against coding guidelines? - Did the clinician actually perform psychotherapy, or was only medication management performed? - Did the service meet the time criteria for the psychotherapy services? Regarding drug screen services: Auditors and investigators look for orders, lab results, medical necessity, frequency, and custom panels. All lab studies require orders, results, and medical necessity. Frequency is important, particularly with drug screen services for an individual who is compliant with medication regimen and no risk of illicit drug use, for example. Custom panels do not provide convincing evidence of medical necessity because the ordering provider determines what tests are required. The lab should not bill for services not ordered and not deemed medically necessary. The correct way of using Modifier L1. Modifier L1 Separately payable lab test was implemented in 2014 by the Centers for Medicare & Medicaid Services (CMS) as part of the July Outpatient Prospective Payment System (OPPS) package updates (CMS 2014). Under the 2014 OPPS packaging edits, if a laboratory service with status indicator A Services furnished to a hospital outpatient that are paid under a fee schedule or payment system other than OPPS is on the same claim as a service with status indicator Q1, Q2, or Q3, the laboratory service is denied and bundled into the payment for the other service. Modifier L1 bypasses this bundling edit, indicating the laboratory service is unrelated to the other ancillary services provided on the same day. “Unrelated” is defined as: - Ordered by a different practitioner than the practitioner who ordered the other hospital outpatient services; and - For a different diagnosis than the other hospital outpatient services (CMS Pub. 100-04). - Implementation of status indicator Q4Conditionally packaged laboratory tests has revised the instances where modifier L1 may be used. The functions of the Q1-Q4 edit set are based on CPT®/HCPCS Level II status indicators. Most laboratory services are assigned status indicator A. The edit looks to see if there are any other ancillary services on the same date of service with a status indicator Q1, Q2, or Q3. If one of these services is included on the claim with the same date of service, the laboratory service is flipped to status N Items and services packaged into APC rates and bundled into the line item with the status indicator Q Packaged services subject to separate payment under OPPS payment criteria. Status indicator Q1 codes are STVX-packaged codes. These codes are packaged when billed on the same date of service with any other code with a status indicator of S, T, V, or X: - Indicator S codes are significant procedures, not subject to the multi-procedure discount. - Indicator T codes are significant procedures subject to the multiple procedure discount. - Indicator V codes include clinic or emergency department visits. - Indicator X codes are ancillary services. When not packaged, these codes are paid separately under an Ambulatory Payment Classification (APC). APCs are a method of paying facilities for outpatient services provided to Medicare patients. Each APC is composed of services that are similar in clinical intensity, resource utilization, and cost. Codes with a status indicator of Q2 T-packaged codes are packaged only if they are billed on the same date of service with any other status indicator T codes. Otherwise, they are separately payable under a separate APC. If you report more than one STVX or T-packaged code without a separately payable service into which it would otherwise be packaged, CMS makes separate payment only for the highest paying service, and packages all others into that code. Status indicator Q3 May be paid through a composite APC-based on composite-specific criteria or separately through single code APCs when the criteria are not met codes are subject to payment as part of a composite rate when all criteria for that composite are met. Otherwise, Q3 services become separately payable if assigned to a separate APC, or packaged into other services. For 2016, CMS implemented status indicator Q4. This status indicator designates packaged APC payment for codes billed on the same claim as a HCPCS Level II code assigned status indicator J1, J2, S, T, V, Q1, Q2, or Q3. Status indicator Q4 identifies outpatient bills (TOB 13x) where there are only laboratory HCPCS Level II codes on the Clinical Laboratory Fee Schedule (CLFS), automatically changes their status indicator to A, and pays them separately at the CLFS payment rates. When status indicator Q4 applies, modifier L1 may only be used to identify laboratory tests ordered for a different diagnosis and by a different provider unrelated to the other OPPS services on the claim. Ways to minimize NCCI denial risk. NCCI denial risk: Becoming knowledgeable about National Correct Coding Initiative (NCCI) policies and edits may be the difference between having a profitable revenue cycle or placing your facility at risk for denials. In recent years, NCCI policies and edits have become key factors in outpatient facility and professional claims denials. You must have a strong understanding of these guidelines to ensure coding compliance and to mitigate risk. Follow NCCI Policy Manual Annual Updates: The NCCI Policy Manual is updated annually. The new guidance becomes effective Jan. 1 of each year. Special attention should be paid to claims denied during the first quarter of the year to ensure you are consistent with the most recent published guidelines. NCCI Edits Are Updated Quarterly: NCCI edits are updated quarterly and are effective Jan. 1, April 1, July 1, and Oct. 1 each year. Because the NCCI Policy Manual is updated only annually, the quarterly updates may not correlate with the information published in the NCCI Policy Manual. The July 2016 NCCI updates eliminated the procedure-to-procedure code edits precluding the assignment of code 29823 Arthroscopy, shoulder, surgical; debridement, extensive with several other ipsilateral shoulder surgeries; however, the 2016 NCCI Policy Manual maintained the following language, “With the exception of the knee joint, arthroscopic debridement should not be reported separately with a surgical arthroscopy procedure when performed on the same joint at the same patient encounter.” In this case, the encoder would no longer flag the debridement as not separately reportable by the procedure-to-procedure edits, but the language in the NCCI Policy Manual would still preclude the assignment of a debridement code with another ipsilateral arthroscopic shoulder surgery. The quarterly updates must be reviewed when they are published and compare them to the guidance published in the NCCI Policy Manual. Not Every NCCI Policy Guideline Has an Edit You may think that, if there isn’t an edit to preclude a particular code assignment, you can combine codes as you please. One should remember that not every NCCI policy has an associated NCCI edit. Per the NCCI Policy Manual, “Providers are obligated to code correctly even if edits do not exist to prevent the use of an inappropriate code combination.” Become familiar with guidelines published in the NCCI Policy Manual. Do not rely on your encoder, alone, to flag NCCI edit violations for code pairs. CPT® Assistant Versus NCCI Policy Guidance One may encounter cases where the guidance published in the NCCI Policy Manual differs from that published in CPT® Assistant. When this occurs, establish which set of guidelines has precedence. For Medicare claims, the NCCI policies prevail. According to page I-28 of the NCCI Policy Manual: The American Medical Association publishes CPT® Assistant which contains coding guidelines. CMS does not review nor approve the information in this publication. In the development of NCCI PTP edits, CMS occasionally disagrees with the information in this publication. If a physician utilizes information from CPT® Assistant to report services rendered to Medicare patients, it is possible that Medicare Carriers (A/B MACS processing practitioner service claims) and Fiscal Intermediaries may utilize different criteria to process claims. For commercial claims, know whether the payer follows NCCI edits prior to code assignment to ensure compliance. Apply Policies to Your Healthcare Organization These are just a few of the potential risks your practice or facility may prevent by having a thorough understanding of the NCCI policies. Coding managers should review the NCCI Policy Manual, and ensure their coding staff receives training on the sections applicable to their place of service. The National Correct Coding Initiative (NCCI) was developed by the Centers for Medicare & Medicaid Services (CMS) to promote national correct coding methodologies and to control improper coding that leads to inappropriate payment of Medicare Part B claims. Although the NCCI policies were initially established for the Medicare program, several commercial insurers have adopted it. Easy ways to correct surgical coding. Surgical coding: Many coders struggle with coding operative reports because there are so many guidelines and policies that affect code selection. The process is easier when you break it into seven steps: - Review the header of the report. - Review the CPT®codebook (start in the Index). - Review the report/documentation. - Make a preliminary code selection. - Review the guidelines (for the preliminary codes). - Review policies and eliminate the extras. - Add any needed modifiers. These seven steps will ensure all the factors that may affect code selection are accounted. Step 1 Review the Header of the Report PREOPERATIVE DIAGNOSIS: Left medial compartment osteoarthritis of the knee. POSTOPERATIVE DIAGNOSIS: Left medial compartment osteoarthritis of the knee. PROCEDURE PERFORMED: Left unicompartmental knee replacement. Based on the documentation above, a unicompartmental knee replacement on the left knee was performed. A unicompartmental knee replacement indicates only one of the three compartments of the knee (medial, lateral, or patellofemoral) was altered during the procedure. The postoperative diagnosis field indicates the altered compartment was the medial compartment of the left knee. Verification of the statement will take place as part of Step 3. Step 2 Review the CPT® Codebook (Start in the Index) Based on the header information from the report, review the CPT® codebook to identify the code options. Also identify the differences between the codes and the documentation required to support one service over another. Continuing with the example given, the Index is reviewed first to identify all possible code options for knee replacement procedures. - Intraoperative Use, Kinetic Balance - Sensor … +0396T - Partial … 27446 - Total … 27447 Three options are given: - +0396TIntra-operative use of kinetic balance sensor for implant stability during knee replacement arthroplasty (List separately in addition to code for primary procedure), - 27446Arthroplasty, knee, condyle and plateau; medial OR lateral compartment, and - 27447 Arthroplasty, knee, condyle and plateau; medial AND lateral compartments with or without patella resurfacing (total knee arthroplasty). Upon reviewing the three options, 0396T is found to be an add-on code, so it may not be reported alone, nor may it be the first-listed CPT® code reported. But it does indicate a specific technology was used during the procedure. If that technology is included in the full report (which will be reviewed in Step 3), +0396T will be included in Step 4 (Preliminary Code Selection). CPT® codes 27446 and 27447 differ based on one key word: “OR” vs. “AND.” Code 27446 indicates a partial knee replacement (including either the medial OR the lateral compartment) was performed; whereas, 27447 indicates a total knee replacement (including both the medial AND lateral compartments) was performed. When the documentation is reviewed fully (Step 3), the primary focus will be to determine which compartments were altered during the procedure. Step 3 Review the Report/Documentation Details Based on the documentation, a unicompartmental knee replacement using a Biomet, Inc., prosthesis was performed. The components were cemented into the tibia and distal femur after the necessary cuts and trial fit/placement were performed. According to the Cleveland Clinic, “Medial knee joint degeneration is the most common deformity of arthritis.” Step 4 Make a Preliminary Code Selection The emphasis here is to make a preliminary code selection based on the documentation. It’s preliminary because reviewing the guidelines, policies, etc., may lead to eliminating certain codes, or the need for additional codes and/or modifiers. Based on the documentation above, a unicompartmental knee replacement is supported. A unicompartmental knee replacement is also referred to as a “partial” knee replacement, so based on the code options, CPT® code 27446 is supported, preliminarily. Guidelines, policies, and the like still need to be reviewed (Steps 5-7). Note: If coding for a facility (as this procedure is fairly common in the outpatient facility or ambulatory surgery center setting), the implant also needs to be reported. Step 5 Review the Guidelines Review all relevant CPT® guidelines, including parenthetical references, to ensure all rules are followed, additional, supported services are captured, etc. Steps 5, 6, and 7 are all related, and are frequently performed concurrently. The “Femur (Thigh Region) and Knee Joint/ Repair, Revision, or Reconstruction” CPT® codes do not include specific subsection guidelines. But there are two parenthetical references below code 27447 to review, and the general surgery guidelines (at the beginning of the Surgery section of CPT®) still apply. The parenthetical references under 27447 read: - (For revision of total knee arthroplasty, use 27487) - (For removal of total knee prosthesis, use 27488) Both parenthetical references are specific to total knee arthroplasties — particularly revision or removal of previously placed prosthesis — and are not relevant. Based on the documentation for this scenario, a partial knee arthroplasty was performed in a knee without a previous prosthesis or implant. No additional CPT® guidelines appear to be relevant for this scenario. Step 6 Review Policies and Eliminate the Extras Because there is only one service supported based on the documentation and steps above, it does not appear there are any extras. Review all of the relevant edits and policies (National Correct Coding Initiative (NCCI) edits, local and national coverage determinations (LCDs, NCDs), payer contracts, medical policies, etc.) to ensure bundled services are appropriately eliminated (and tracked internally, if applicable). This step is essential in scenarios where more than one service is performed and more than one code may be warranted. Reviewing the NCCI edits and payer policies will help you identify bundled services, instances where modifiers may be needed, or situations where a contract limitation restricts reporting a service that would otherwise be reportable (e.g., a colonoscopy in which multiple procedures were performed such as snare polypectomy, biopsy polypectomy, and submucosal injection). Step 7 Add Necessary Modifiers Based on the description for 27446, there is no indication as to which knee was repaired. The story is incomplete. A modifier is needed to indicate which knee was affected. Modifier options are: - LTeft side - RTight side - 50ilateral procedure Based on the documentation, the left knee was replaced because the patient had a right-side replacement previously. For this scenario, modifier LT is added to the procedure to indicate the procedure was performed on the left knee. Without this modifier, a denial or request for additional information may be received from the payer because the patient had the previous knee replacement. Final code selection for this scenario is: 27446-LT. Although the scenario used in this example is fairly straight-forward, these seven steps will work for any level of sophistication in an operative report. Use them for consistent review of all relevant factors, and correct coding is certain. Final updates to Prescription Drug Program. Prescription Drug Program: The Center Medicare & Medicaid Services (CMS) released final updates to the Medicare Advantage and Part D Prescription Drug Program for 2018, as an ongoing effort to strengthen the Medicare Advantage and the Prescription Drug Program by supporting flexibility, efficiency, and innovation. The final policies are similar to the proposal released in February’s 2018 Advance Notice and Draft Call Letter. There are several changes, however, in response to feedback received during the public comment period. Some of the policies in the rate announcement will according to CMS’ April 3 press release will provide: - Flexibility and incentives to encourage organizations to develop new plan offerings - Incentives for plans to submit complete encounter data - Modify the phase-in of the use of encounter data - Use encounter data for 15 percent of the risk adjustment payment to Medicare Advantage plans - Help combat overuse of opioids by encouraging safeguards before an opioid prescription is dispensed at the pharmacy - Employ best practices and the most appropriate care management interventions for enrollees using high dosage opioids The “Year-to-Year Percentage Change in Payment” is: \|Effective Growth Rate\|\|2.8%\|\|2.7%\| \|Change in Star Ratings\|\|-0.4%\|\|-0.4%\| \|MA coding intensity adjustment\|\|-0.25%\|\|-0.25%\| \|Expected Average Change in Revenue\|\|0.25%\|\|0.45%\| \|Expected Average Change in Revenue with Coding trend Proper Reporting of Modifier 99. Modifier 99 Multiple modifiers do not get a lot of attention as it is rarely needed , but knowing when to apply it can make the difference in getting a claim paid. Appendix A — Modifiers tells us: Under certain circumstances, 2 or more modifiers may be necessary to completely delineate a service. In such situations, modifier 99 should be added to the basic procedure, and other applicable modifiers may be listed as part of the description of the service. In practice, call on modifier 99 only if a single line item requires five or more modifiers. The reason is the standard 1500 Health Insurance Claim Form (or electronic equivalent) field 24D accommodates the entry of up to four modifiers: If a single line item requires more than four modifiers, enter modifier 99 (and only modifier 99) in the first space available for modifiers in field 24D. All other applicable modifiers should be entered in field 19 “Additional Claim Information,” or the equivalent electronic data field. You may use modifier 99, when applicable, with any CPT® code. Medicare Carriers Manual Part 4 – Professional Relations, Transmittal 25, Change Request (CR) 1910 (Nov. 1, 2001) further specifies, “If modifier -99 is entered on multiple line items of a single claim form, all applicable modifiers for each line item containing a -99 modifier should be listed as follows: 1=(mod), where the number 1 represents the line item and ‘mod’ represents all modifiers applicable to the referenced line item.” When listing multiple modifiers for the same line item, take care to sequence the modifiers affecting payment first. Level I payment modifiers include: - 26Professional component - 50Bilateral procedure - 53Discontinued procedure - 54Surgical care only - 55Postoperative management only - 56Preoperative management only - 62Two surgeons66 Surgical team - 78Unplanned return to operating/procedure room by the same physician or other qualified healthcare professional following an initial procedure for a related procedure during postoperative period - 80Assistant surgeon - 81Minimum assistant surgeon - 82Assistant surgeon (when qualified resident surgeon not available) - 91Repeat clinical diagnostic laboratory test Informational or statistical modifiers (any modifier not classified as a payment modifier) are sequenced after payment modifiers. If multiple informational/statistical modifiers apply, you may sequence them in any order (as long as they are sequenced after any payment modifiers). Effective Date of Home Health Agency CoPs delayed. The Centers for Medicare & Medicaid Services (CMS) issued on March 31 a proposed rule (CMS-3819-P2) to delay the effective date for the Medicare and Medicaid Programs: Conditions of Participation for Home Health Agencies (HHA CoPs) final rule. The current effective date for the final rule is July 13. The proposed rule would delay the effective date an additional six months, until Jan. 13, 2018. CMS also proposes to revise a phase-in date for the requirements at §484.65(d) — “Standard: Performance improvement projects” from Jan. 13, 2018, to July 13, 2018, and to extend the July 13, 2017, effective date at §484.115(a)(1) and (2) to Jan. 13, 2018. Following publication of the January 2017 HHA CoPs final rule, CMS says it received inquiries that represented a large number of HHAs requesting that the agency delay the effective date for the new HHA CoPs. The inquiries asserted that HHAs were not able to effectively implement the new CoPs until CMS issued its revised Interpretive Guidelines (State Operations Manual, CMS Pub. 100-07, Appendix B). In addition, one of the inquiries stated that HHAs were unable to effectively implement the new CoPs until CMS issued further sub-regulatory guidance related to converting subunits to branches or independent HHAs, which would affect 216 HHAs nationwide. One of the inquiries cited the estimated $300 million cost to implement the new requirements as a reason for delaying the effective date. The January 2017 HHA CoPs final rule revised the conditions of participation home health agencies must meet o participate in the Medicare and Medicaid programs. “The requirements focus on the care delivered to patients by HHAs, reflect an interdisciplinary view of patient care, allow HHAs greater flexibility in meeting quality care standards, and eliminate unnecessary procedural requirements,” CMS states in the proposed rule. Coding Carpal Tunnel Syndrome. Carpal Tunnel Syndrome. Carpal tunnel syndrome is diagnosed when there is pressure on the median nerve in the wrist. Some symptoms include pain, numbness, tingling, and weakness in the hand. The physician may order physical therapy, or prescribe a wrist brace or nonsteroidal anti-inflammatory drugs (NSAID). To achieve coverage and payment, the provider should document the conservative treatment provided, along with the patient’s response to these methods. Following more conservative treatments, an injection performed in the wrist with corticosteroids and/or anesthetics can provide temporary relief of the symptoms. The injection is reported with 20526 Injection, therapeutic (Eg: Local anesthetic corticosteroid), carpal tunnel. If one performs this service in an office setting and purchase the medication, the corticosteroid using the appropriate HCPCS Level II code (In a hospital or outpatient setting, the facility codes for the drug) should be coded. A more productive intervention is for the physician to perform a release of the ligament, through either an endoscopic or open approach. The endoscope is placed into the wrist through a small incision in the wrist joint. The scope is used to identify the carpal ligament, which is divided to relieve pressure on the median nerve and tendons. An endoscopic carpal tunnel release is reported with CPT® code 29848 Endoscopy, wrist, surgical, with release of transverse carpal ligament. The National Correct Coding Initiative Policy Manual for Medicare Services, updated Jan. 1, states: CPT code 29848 describes endoscopic release of the transverse carpal ligament of the wrist. CPT code 64721 describes a neuroplasty and/or transposition of the median nerve at the carpal tunnel and includes open release of the transverse carpal ligament. The procedure coded as CPT code 64721 includes the procedure coded as CPT code 29848 when performed on the same wrist at the same patient encounter. If an endoscopic procedure is converted to an open procedure, only the open procedure may be reported. In an open approach, an incision is made over the carpal tunnel. The ligament is divided to release pressure on the median nerve, or the nerve may be relocated to relieve the pressure. An endoscope is not used in this procedure. The CPT® code to report this procedure is 64721 Neuroplasty and/or transposition; median nerve at carpal tunnel. Both endoscopic and open carpal tunnel release surgeries are unilateral codes. To report bilateral injections, either append modifier 50 to the single code or bill the code on two lines and append modifiers RT and LT, depending on the insurance carrier’s preference. Whether the patient is having an injection or surgery, consent is needed. Consent may be verbal for an injection given in a physician office, but must be in writing for surgery in a hospital or other outpatient setting. Most hospitals require consent forms to be filled out by the provider and signed by both the provider and the patient. For verbal consent, the provider must document that consent was obtained by the patient or the patient’s guardian. Additionally, the physician should document the prep, the location, the needle, aspiration (if performed), drug, dosage, and how the patient tolerated the procedure. ICD-10-CM breaks down carpal tunnel syndrome based on laterality. The codes are in Chapter 6, Diseases of the Nervous System: - 00Carpal tunnel syndrome, unspecified upper limb - 01Carpal tunnel syndrome, right upper limb - 02Carpal tunnel syndrome, left upper limb - 03Carpal tunnel syndrome, bilateral upper limb Carpal tunnel surgery has a 90-day global period. Global periods can be found in the Physician Fee Schedule. Any evaluation and management (E/M) visits to the surgeon or the surgeon’s associates related to the carpal tunnel surgery are inclusive in the reimbursement for the surgery and cannot be separately reported. If the physician performs internal neurolysis (such as a pain block) using an operating microscope during a carpal tunnel release using an open approach, report add-on code 64727 Internal neurolysis, requiring use of operating microscope (List separately in addition to for neuroplasty) (Neuroplasty includes external neurolysis) in addition to 64721. A parenthetical note states, “Do not report code 69990 in addition to code 64727.” It is better to check with the insurance if they require modifier 51 Multiple procedures when billing bilateral procedures on two lines using the anatomic modifiers RT/LT It should be noted that Novitas Solutions has a local coverage policy for CPT® 20526. Under the utilization guidelines it states, “More than 3 injections per anatomic site in a six month period will be denied. More than two anatomic sites injected at any one session will be denied.” It’s important to check these guidelines with the different payers.	4	46	57	0	5	6	0.45581	68	8,768
Coma ICD-10 R40.2 ICD-9 780.01 In medicine, a coma (from the Greek κῶμα koma, meaning deep sleep) is a state of unconsciousness, lasting more than 6 hours in which a person cannot be awakened, fails to respond normally to painful stimuli, light or sound, lacks a normal sleep-wake cycle and does not initiate voluntary actions. A person in a state of coma is described as comatose. According to the Glasgow Coma Scale though, a person with confusion is considered to be in the mildest coma. Coma may result from a variety of conditions, including intoxication (such as illicit drug abuse, overdose or misuse of over the counter medications, prescribed medication, or controlled substances), metabolic abnormalities, central nervous system diseases, acute neurologic injuries such as strokes or herniations, hypoxia, hypothermia, hypoglycemia or traumatic injuries such as head trauma caused by falls or vehicle collisions. It may also be deliberately induced by pharmaceutical agents in order to preserve higher brain functions following brain trauma, or to save the patient from extreme pain during healing of injuries or diseases. In order for a patient to maintain consciousness, two important neurological components must function impeccably. The first is the cerebral cortex which is the gray matter covering the outer layer of the brain, and the other is a structure located in the brainstem, called reticular activating system (RAS or ARAS). Injury to either or both of these components is sufficient to cause a patient to experience a coma. The human cortex is a group of tight, dense, "gray matter" composed of the nucleus of the neurons whose axons then form the "white matter", and is responsible for the perception of the universe, relay of the sensory input (sensation) via the thalamic pathway, and most importantly directly or indirectly in charge of all the neurological functions, from simple reflexes to complex thinking. Reticular activating system (RAS) on the other hand is a more primitive structure in the brainstem that is tightly in connection with reticular formation (RF), a critical anatomical structure needed for maintenance of arousal and sleep-wake transitions. Reticular activating system (RAS) takes its name from the effect it has on the reticular formation, which is via its stimulation. It is therefore necessary to investigate in a comatose patient, the integrity of the bilateral cerebral cortices, as well as that of the reticular activating system (RAS). - 1 Signs and symptoms - 2 Diagnosis and findings - 3 Prognosis - 4 Treatment and recovery - 5 Society and culture - 6 See also - 7 References Signs and symptoms Generally, a patient who is unable to voluntarily open the eyes, does not have a sleep-wake cycle, is unresponsive in spite of strong tactile (painful), or verbal stimuli and who generally scores between 3 to 8 on the Glasgow Coma Scale is considered to be in coma. Coma may have developed in humans as a response to injury to allow the body to pause bodily actions and heal the most immediate injuries before - if at all - waking. It therefore could be a compensatory state in which the body's expenditure of energy is not superfluous. The severity and mode of onset of coma depends on the underlying cause. For instance, severe hypoglycemia (low blood sugar) or hypercapnia (increased carbon dioxide levels in the blood) initially cause mild agitation and confusion, but progress to obtundation, stupor and finally complete unconsciousness. In contrast, coma resulting from a severe traumatic brain injury or subarachnoid hemorrhage can be instantaneous. The mode of onset may therefore be indicative of the underlying cause. A state of unconsciousness lasting less than 6 hours is not by definition a coma, but a concussion, therefore a patient who loses consciousness for less than this time period should not be immediately labeled as having experienced a coma. Diagnosis and findings Diagnosis of coma is simple; however, diagnosing the cause of the underlying disease process often proves to be challenging. The first priority in treatment of a comatose patient is stabilization following the basic ABCs (standing for airway, breathing, and circulation). Once a person in a coma is stable, investigations are performed to assess the underlying cause. Investigative methods are divided into physical examination findings and imaging (such as CAT scan, MRI, etc.) and special studies (EEG, etc.) Initial assessment and evaluation In the initial assessment of coma, it is common to gauge the level of consciousness by spontaneously exhibited actions, response to vocal stimuli ("Can you hear me?"), and painful stimuli; this is known as the AVPU (alert, vocal stimuli, painful stimuli, unresponsive) scale. More elaborate scales, such as the Glasgow Coma Scale, quantify an individual's reactions such as eye opening, movement and verbal response on a scale; Glasgow Coma Scale (GCS) is an indication of the extent of brain injury varying from 3 (indicating severe brain injury and death) to a maximum of 15 (indicating mild or no brain injury). In those with deep unconsciousness, there is a risk of asphyxiation as the control over the muscles in the face and throat is diminished. As a result, those presenting to a hospital with coma are typically assessed for this risk ("airway management"). If the risk of asphyxiation is deemed to be high, doctors may use various devices (such as an oropharyngeal airway, nasopharyngeal airway or endotracheal tube) to safeguard the airway. Physical examination findings Physical examination is critical after stabilization. It should include vital signs, a general portion dedicated to making observations about the patient's respiration (breathing pattern), body movements (if any), and of the patient's body habitus (physique); it should also include assessment of the brainstem and cortical function through special reflex tests such as the oculocephalic reflex test (doll's eyes test), oculovestibular reflex test (cold caloric test), nasal tickle, corneal reflex, and the gag reflex. Vital signs in medicine are temperature (rectal is most accurate), blood pressure, heart rate (pulse), respiratory rate, and oxygen saturation. It should be easy to evaluate these vitals quickly in order to gain insight into a patient's metabolism, fluid status, heart function, vascular integrity, and tissue oxygenation. Respiratory pattern (breathing rhythm) is significant and should be noted in a comatose patient. Certain stereotypical patterns of breathing have been identified including Cheyne-Stokes a form of breathing in which the patient's breathing pattern is described as alternating episodes of hyperventilation and apnea. This is a dangerous pattern and is often seen in pending herniations, extensive cortical lesions, or brainstem damage. Another pattern of breathing is apneustic breathing which is characterized by sudden pauses of inspiration and is due to a lesion of the pons. Ataxic breathing is irregular and is due to a lesion (damage) of the medulla. Assessment of posture and body habitus is the next step. It involves general observation about the patient's positioning. There are often two stereotypical postures seen in comatose patients. Decorticate posturing is a stereotypical posturing in which the patient has arms flexed at the elbow, and arms adducted toward the body, with both legs extended. Decerebrate posturing is a stereotypical posturing in which the legs are similarly extended (stretched), but the arms are also stretched (extended at the elbow). The posturing is critical since it indicates where the damage is in the central nervous system. A decorticate posturing indicates a lesion (a point of damage) at or above the red nucleus, whereas a decerebrate posturing indicates a lesion at or below the red nucleus. In other words, a decorticate lesion is closer to the cortex, as opposed to a decerebrate cortex that is closer to the brainstem. Oculocephalic reflex also known as the doll's eye is performed to assess the integrity of the brainstem. Patient's eye lids are gently elevated and the cornea is visualized. The patient's head is then moved to the patient's left, to observe if the eyes stay or deviate toward the patient's right; same maneuver is attempted on the opposite side. If the patient's eyes move in a direction opposite to the direction of the rotation of the head, then the patient is said to have an intact brainstem. However, failure of both eyes to move to one side, can indicate damage or destruction of the affected side. In special cases, where only one eye deviates and the other does not, this often indicates a lesion (or damage) of the medial longitudinal fasciculus (MLF) which is a brainstem nerve tract. Caloric reflex test also evaluates both cortical and brainstem function; cold water is injected into one ear and the patient is observed for eye movement; if the patient's eyes slowly deviate toward the ear where the water was injected, then the brainstem is intact, however failure to deviate toward the injected ear indicates damage of the brainstem on that side. Cortex is responsible for a rapid nystagmus away from this deviated position and is often seen in patients who are conscious or merely lethargic. An important part of the physical exam is also assessment of the cranial nerves. Due to the unconscious status of the patient, only a limited number of the nerves can be assessed. These include the cranial nerves number 2 (CN II), number 3 (CN III), number 5 (CN V), number 7 (CN VII), and cranial nerves 9 and 10 (CN IX, CN X). Gag reflex helps assess cranial nerves 9 and 10. Pupil reaction to light is important because it shows an intact retina, and cranial nerve number 2 (CN II); if pupils are reactive to light, then that also indicates that the cranial nerve number 3 (CN III) (or at least its parasympathetic fibers) are intact. Corneal reflex assess the integrity of cranial nerve number 7 (CN VII), and cranial nerve number 5 (CN V). Cranial nerve number 5 (CN V), and its ophthalmic branch (V1) are responsible for the afferent arm of the reflex, and the cranial nerve number 7 (CN VII) also known a facial nerve, is responsible for the efferent arm, causing contraction of the muscle orbicularis oculi resulting in closing of the eyes. Pupil assessment is often a critical portion of a comatose examination, as it can give information as to the cause of the coma; the following table is a technical, medical guideline for common pupil findings and their possible interpretations: Pupil sizes (Left eye vs. Right eye) Possible interpretation •ʖ• Normal eye with two pupils equal in size and reactive to light. This means that the patient is probably not in a coma and is probably lethargic, under influence of a drug, or sleeping. •ʖ• "Pinpoint" pupils indicate heroin or opiate overdose, and can be responsible for a patient's coma. The pinpoint pupils are still reactive to light, bilaterally (in both eyes, not just one). Another possibility is the damage of the pons. •ʖ• One pupil is dilated and unreactive, while the other is normal (in this case the L eye is dilated but the R eye is normal in size). This could mean a damage to the oculomotor nerve (cranial nerve number 3, CN III) on the right side, or possibility of vascular involvement. •ʖ• Both pupils are dilated and unreactive to light. This could be due to overdose of certain medications, hypothermia or severe anoxia (lack of oxygen). Imaging and special tests findings Imaging basically encompasses computed tomography (CAT or CT) scan of the brain, or MRI for example, and is performed to identify specific causes of the coma, such as hemorrhage in the brain or herniation of the brain structures. Special tests such as an EEG can also show a lot about the activity level of the cortex such as semantic processing, presence of seizures, and are important available tools not only for the assessment of the cortical activity but also for predicting the likelihood of the patient's awakening. The autonomous responses such as the Skin Conductance Response may also provide further insight on the patient's emotional processing. When diagnosing any neurological condition, history and examination are fundamental. History is obtained by family, friends or EMS. The Glasgow Coma Scale is a helpful system used to examine and determine the depth of coma, track patients progress and predict outcome as best as possible. In general a correct diagnosis can be achieved by combining findings from physical exam, imaging, and history components and will direct the appropriate therapy. Severity and classification Plum and Posner classify coma as either (1) supratentoral (above Tentorium cerebelli), (2) infratentoral (below Tentorium cerebelli), or (3) metabolic or (4) diffuse. This classification is merely dependent on the position of the original damage that caused the coma, and does not correlate with severity or the prognosis. The severity of coma impairment however is categorized into several levels. Patients may or may not progress through these levels. In the first level, the brain responsiveness lessens, normal reflexes are lost, the patient no longer responds to pain and cannot hear. The Rancho Los Amigos Scale is a complex scale that has eight separate levels, and is often used in the first few weeks or months of coma while the patient is under closer observation, and when shifts between levels are more frequent. Outcomes range from recovery to death. Comas can last from several days to several weeks. In more severe cases a coma may last for over 5 weeks, while some have lasted as long as several years. After this time, some patients gradually come out of the coma, some progress to a vegetative state, and others die. Some patients who have entered a vegetative state go on to regain a degree of awareness. Others remain in a vegetative state for years or even decades (the longest recorded period being 37 years). The outcome for coma and vegetative state depends on the cause, location, severity and extent of neurological damage. A deeper coma alone does not necessarily mean a slimmer chance of recovery, because some people in deep coma recover well while others in a so-called milder coma sometimes fail to improve. People may emerge from a coma with a combination of physical, intellectual and psychological difficulties that need special attention. Recovery usually occurs gradually—patients acquire more and more ability to respond. Some patients never progress beyond very basic responses, but many recover full awareness. Regaining consciousness is not instant: in the first days, patients are only awake for a few minutes, and duration of time awake gradually increases. This is unlike the situation in many movies where people who awake from comas are instantly able to continue their normal lives. In reality, the coma patient awakes sometimes in a profound state of confusion, not knowing how they got there and sometimes suffering from dysarthria, the inability to articulate any speech, and with many other disabilities. Predicted chances of recovery are variable owing to different techniques used to measure the extent of neurological damage. All the predictions are based on statistical rates with some level of chance for recovery present: a person with a low chance of recovery may still awaken. Time is the best general predictor of a chance of recovery: after 4 months of coma caused by brain damage, the chance of partial recovery is less than 15%, and the chance of full recovery is very low. Occasionally people come out of coma after long periods of time. After 19 years in a minimally conscious state, Terry Wallis spontaneously began speaking and regained awareness of his surroundings. Similarly, Polish railroad worker Jan Grzebski woke up from a 19-year coma in 2007. A brain-damaged man, trapped in a coma-like state for six years, was brought back to consciousness in 2003 by doctors who planted electrodes deep inside his brain. The method, called deep brain stimulation (DBS) successfully roused communication, complex movement and eating ability in the 38-year-old American man who suffered a traumatic brain injury. His injuries left him in a minimally conscious state (MCS), a condition akin to a coma but characterized by occasional, but brief, evidence of environmental and self-awareness that coma patients lack. Coma lasting seconds to minutes results in post-traumatic amnesia (PTA) that lasts hours to days; recovery plateau occurs over days to weeks. Coma that lasts hours to days results in PTA lasting days to weeks; recovery plateau occurs over months. Coma lasting weeks results in PTA that lasts months; recovery plateau occurs over months to years. Treatment and recovery Coma is a medical emergency, and attention must first be directed to maintaining the patient's respiration and circulation, using intubation and ventilation, administration of intravenous fluids or blood and other supportive care as needed. Once a patient is stable and no longer in immediate danger, the medical staff may concentrate on maintaining the health of patient’s physical state. The concentration will be directed on preventing infections such as pneumonias, bedsores (decubitus ulcers) and providing a balanced nutrition. These infections may appear from the patient not being able to move around, and being confined to the bed. The nursing staff will move the patient every 2–3 hours from side to side and depending on the state of consciousness sometimes to a chair. The goal is to move the patient as much as possible to try to avoid bedsores, atelectasis and pneumonia. Pneumonia can occur from the person’s inability to swallow leading to aspiration, lack of gag reflex or from feeding tube, (aspiration pneumonia). Physical therapy may also be used to prevent contractures and orthopedic deformities that would limit recovery for those patients who emerge from coma. A person in a coma may become restless, or seize and need special care to prevent them from hurting themselves. Medicine may be given to calm such individuals. Patients who are restless may also try to pull on tubes or dressings so soft cloth wrist restraints may be put on. Side rails on the bed should be kept up to prevent patient from falling. Coma has a wide variety of emotional reactions from the family members of the affected patients, as well as the primary care givers taking care of the patients. Common reactions, such as desperation, anger, frustration, and denial are possible. The focus of the patient care should be on creating an amicable relationship with the family members or dependents of a comatose patient as well as creating rapport with the medical staff. Society and culture Research by Dr. Eelco Wijdicks on the depiction of comas in movies was published in Neurology in May 2006. Dr. Wijdicks studied 30 films (made between 1970 and 2004) that portrayed actors in prolonged comas, and he concluded that only two films accurately depicted the state of a coma victim and the agony of waiting for a patient to awaken: Reversal of Fortune (1990) and The Dreamlife of Angels (1998). The remaining 28 were criticized for portraying miraculous awakenings with no lasting side effects, unrealistic depictions of treatments and equipment required, and comatose patients remaining muscular and tanned. - Brain death Lack of activity in both cortex, and lack of brainstem function. - Coma scale, a system to assess the severity of coma - Locked-in syndrome Paralysis of most muscles, except ocular muscles of the eyes, while patient is conscious. - Persistent vegetative state (vegetative coma), deep coma without detectable awareness. Damage to the cortex, with an intact brainstem. - Process Oriented Coma Work, for an approach to working with residual consciousness in comatose patients. - ^ a b c d e Weyhenmyeye, James A.; Eve A. Gallman (2007). Rapid Review Neuroscience 1st Ed. Mosby Elsevier. pp. 177–9. ISBN 0-323-02261-8. - ^ a b c d e f Hannaman, Robert A. (2005). MedStudy Internal Medicine Review Core Curriculum: Neurology 11th Ed. MedStudy. pp. (11–1)-(11–2). ISBN 1-932703-01-2. - ^ "Video of Aaron Cohen at beginning of documented 3 month coma.". http://www.youtube.com/watch?v=IW8rBQgiU_c. - ^ "Video of Aaron Cohen still nonresponsive to stimuli while in coma.". http://www.youtube.com/watch?v=I6t6dDEDVXg. - ^ "Video of Aaron Cohen nearly out of 3 month coma.". http://www.youtube.com/watch?v=FlwRC7E4TQI. - ^ "Video of Aaron Cohen walking 2 weeks after 3 month coma.". http://www.youtube.com/watch?v=WcIbULrMBsQ. - ^ Russ Rowlett. "Glasgow Coma Scale". University of North Carolina at Chapel Hill. http://www.unc.edu/~rowlett/units/scales/glasgow.htm. - ^ Daltrozzo, J., Wioland, N., Mutschler, V., Lutun, P., Jaeger, A., Calon, B., Meyer, A., Pottecher, T., Lang, S., Kotchoubey, B. (2009c). Cortical Information Processing in Coma, Cognitive & Behavioral Neurology, 22(1), 53-62. - ^ Daltrozzo, J., Wioland, N., Mutschler, V., Kotchoubey, B. (2007). Predicting Coma and other Low Responsive Patients Outcome using Event-Related Brain Potentials: A Meta-analysis. Clinical Neurophysiology, 118, 606-614. - ^ Daltrozzo, J., Wioland, N., Mutschler, V., Lutun, P., Calon, B., Meyer, A., Jaeger, A., Pottecher, T., Kotchoubey, B. (2010a). Electrodermal Response in Coma and Other Low Responsive Patients. Neuroscience Letters, 475(1), 44-47. - ^ According to the Guinness Book of Records, the longest period spent in coma was by Elaine Esposito. She did not wake up after being anaesthetized for an appendectomy on August 6, 1941, at age 6. She died on November 25, 1978 at age 43 years 357 days, having been in a coma for 37 years 111 days. - ^ NINDS (October 29, 2010). "Coma Information Page: National Institute of Neurological Disorders and Stroke (NINDS)". http://www.ninds.nih.gov/disorders/coma/coma.htm#What_is_the_prognosis. Retrieved 2010-12-08. - ^ Formisano R, Carlesimo GA, Sabbadini M, et al. (May 2004). "Clinical predictors and neuropleropsychological outcome in severe traumatic brain injury patients". Acta Neurochir (Wien) 146 (5): 457–62. doi:10.1007/s00701-004-0225-4. PMID 15118882. - ^ brain injury .com \| Coma traumatic brain injury - Brain Injury Coma - ^ "Mother stunned by coma victim's unexpected words". The Sydney Morning Herald. 2003-07-12. http://www.smh.com.au/articles/2003/07/11/1057783356390.html. - ^ "Electrodes stir man from six-year coma-like state". Cosmos Magazine. 2 August 2007. http://www.cosmosmagazine.com/node/1513. - ^ a b "Coma". http://medicalcenter.osu.edu/PatientEd/Materials/PDFDocs/dis-cond/general/coma.pdf. Retrieved 2010-12-08. - ^ Coma Care (2010-03-30). "Caring for Care Giver and Family". http://www.comacare.com/cgi-bin/giga.cgi?. Retrieved 2010-12-08. - ^ Eelco F.M. Wijdicks, MD and Coen A. Wijdicks, BS (2006). "The portrayal of coma in contemporary motion pictures". Neurology 66 (9): 1300–1303. doi:10.1212/01.wnl.0000210497.62202.e9. PMID 16682658. http://www.neurology.org/cgi/content/abstract/66/9/1300. Retrieved 2009-11-25. General Related Symptoms and signs: cognition, perception, emotional state and behaviour (R40–R46, 780.0–780.5, 781.1) CognitionFainting/SyncopeOther Emotional state Behavior Perception/ Wikimedia Foundation. 2010. Look at other dictionaries: coma — coma … Dictionnaire des rimes COMA — Le coma est un état caractérisé par la perte de la conscience et par la perte plus ou moins complète des fonctions de relation: sensibilité et motricité. Cependant, certaines fonctions végétatives (qui entretiennent la nutrition) sont… … Encyclopédie Universelle Coma — puede tener los siguientes significados: en medicina, coma (del griego κῶμα o κωμα sopor) es un estado severo de pérdida de consciencia. la coma [,] (del griego κόμμα corte) es un signo de puntuación. en matemáticas, al separador decimal entre… … Wikipedia Español coma — m. neurol. Estado de sopor profundo debido a una alteración del sistema reticular activador ascendente. El paciente ha perdido la sensibilidad, la movilidad y se muestra inconsciente pero conserva las funciones vegetativas (respiración,… … Diccionario médico coma — \|ô\| s. f. 1. Cabeleira. 2. Cabelo comprido. 3. Crina do cavalo. 4. Juba do leão. 5. Penas compridas que adornam a cabeça de certas aves. = PENACHO 6. Plumas do capacete. = PENACHO 7. Copa (do arvoredo). 8. [Astronomia] Cabeleira de cometa. 9. … … Dicionário da Língua Portuguesa COMA — olim libertatis insigne, apud Gallos praesertim: unde Regalis capillitii ius priscis Francis, adeo celebre, apud Hottomannum, Francogalliae, c. 9. Aimoinum, de Gestis Francorum, l. 3. c. 61. etc. Certe omnes Galliae Reges usque ad Pippinum Caroli … Hofmann J. Lexicon universale Coma I — es un grupo de galaxias disperso que recibe éste nombre por estar muchos de sus miembros en la constelación de Coma Berenices. Se halla a apenas 5 millones de parsecs de la galaxia elíptica M87, el miembro central del Cúmulo de Virgo, y parece… … Wikipedia Español Coma — Пётр Рогуцкий лидер группы COMA во время концерта в Мегаклу … Википедия coma — CÓMA, come, s.f. (fiz.) 1. Aberaţie a sistemelor optice care constă în apariţia unei imagini în formă de cometă. 2. Aberaţie a lentilelor electronice, caracterizată prin apariţia unei estompări spre periferia imaginii. – Din fr. coma. Trimis de… … Dicționar Român coma — sustantivo masculino 1. (no contable) Pérdida de conciencia prolongada en que permanecen algunos enfermos graves: El accidentado estuvo un mes en estado de coma. sustantivo femenino 1. Signo ortográfico de puntuación que indica una separación o… … Diccionario Salamanca de la Lengua Española Coma — Verbrauchermärkte GmbH Co KG Rechtsform GmbH Co KG Gründung 1977 Sitz Meppen Mitarbeiter ca. 1.400 Website … Deutsch Wikipedia	2	20	2	0	0	4	0.907887	6	6,342
What is E29 testicular hypofunction? ICD-10 code E29. 1 for Testicular hypofunction is a medical classification as listed by WHO under the range – Endocrine, nutritional and metabolic diseases . What is icd10 testicular hypofunction? E29. 1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. What is the ICD 9 code for hypogonadism? ICD-9 code 257.2 for Other testicular hypofunction is a medical classification as listed by WHO under the range -DISEASES OF OTHER ENDOCRINE GLANDS (249-259). What causes testicular hypofunction? Male hypogonadism is a condition in which the body doesn’t produce enough of the hormone that plays a key role in masculine growth and development during puberty (testosterone) or enough sperm or both. You can be born with male hypogonadism, or it can develop later in life, often from injury or infection. What happens if testes do not produce testosterone? If it occurs after puberty, there may be infertility and sexual dysfunction. In adult men, symptoms begin within a few weeks of the onset of testosterone deficiency. Hypogonadism may increase the risk for cardiovascular disease, type 2 diabetes, metabolic syndrome, premature death in older men, and Alzheimer’s disease. What are the symptoms of low testosterone in a male? Symptoms of low testosterone depend on the age of person, and include the following: - Low sex drive. - Erectile dysfunction. - Decreased sense of well-being. - Depressed mood. - Difficulties with concentration and memory. - Moodiness and irritability. - Loss of muscular strength. What are the signs of low testosterone in a man? What happens if a man’s testosterone is low? If a male has low testosterone, symptoms can include erectile dysfunction, and males and females may have reduced bone mass and sex drive. The hormone has many important functions, including: the development of the bones and muscles. the deepening of the voice, hair growth, and other factors related to appearance.	4	4	0	0	0	6	0.966631	6	476
From Wikipedia, the free encyclopedia - View original article Diagram showing normal anatomy of anal canal and rectum. Diagram showing normal anatomy of anal canal and rectum. Fecal incontinence (FI), also called faecal incontinence, bowel incontinence, anal incontinence, or accidental bowel leakage, is a lack of control over defecation, leading to involuntary loss of bowel contents—including flatus (gas), liquid stool elements and mucus, or solid feces. FI is a sign or a symptom, not a diagnosis. Incontinence can result from different causes and might occur with either constipation or diarrhea. Continence is maintained by several inter-related factors, and usually there is more than one deficiency of these mechanisms for incontinence to develop. The most common causes are thought to be immediate or delayed damage from childbirth, complications from prior anorectal surgery (especially involving the anal sphincters or hemorrhoidal vascular cushions) and altered bowel habits (e.g. caused by irritable bowel syndrome, Crohn's disease, ulcerative colitis, food intolerance, or constipation with overflow incontinence). An estimated 2.2% of community dwelling adults are affected. Fecal incontinence has three main consequences: local reactions of the perianal skin and urinary tract, including maceration (softening and whitening of skin due to continuous moisture), urinary tract infections, or decubitus ulcers (pressure sores); a financial expense for individuals (due to cost of medication and incontinence products, and loss of productivity), employers (days off), and medical insurers and society generally (health care costs, unemployment); and an associated decrease in quality of life. There is often reduced self-esteem, shame, humiliation, depression, a need to organize life around easy access to bathroom and avoidance of enjoyable activities. FI is an example of a stigmatized medical condition, which creates barriers to successful management. People may be too embarrassed to seek medical help, and attempt to self-manage the symptom in secrecy from others. FI is one of the most psychologically and socially debilitating conditions in an otherwise healthy individual, but it is generally treatable. Management may be achieved through an individualized mix of dietary, pharmacologic and surgical measures. Health care professionals are often poorly informed about treatment options, and may fail to recognize the impact of FI. There is no globally accepted definition, but fecal incontinence is generally defined as the recurrent inability to voluntarily control the passage of bowel contents through the anal canal and expel it at a socially acceptable location and time, occurring in individuals over the age of four. "Social continence" has been given various precise definitions for the purposes of research, however generally it refers to symptoms being controlled to an extent that is acceptable to the individual in question, with no significant impact on their life. There is no consensus about the best way to classify FI, and several methods are used. Symptoms can be directly or indirectly related to the loss of bowel control. The direct (primary) symptom is a lack of control over bowel contents which tends to worsen without treatment. Indirect (secondary) symptoms, which are the result of leakage, include pruritus ani (an intense itching sensation from the anus), perianal dermatitis (irritation and inflammation of the skin around the anus), and urinary tract infections. Due to embarrassment, people may only mention secondary symptoms rather than acknowledge incontinence. Any major underlying cause will produce additional signs and symptoms, such as protrusion of mucosa in external rectal prolapse. Symptoms of fecal leakage (FL) are similar, and may occur after defecation. There may be loss of small amounts of brown fluid and staining of the underwear. FI can be divided into those people who experience a defecation urge before leakage (urge incontinence), and those who experience no sensation before leakage (passive incontinence or soiling). Urge incontinence is characterized by a sudden need to defecate, with little time to reach a toilet. Urge and passive FI may be associated with weakness of the external anal sphincter (EAS) and internal anal sphincter (IAS) respectively. Urgency may also be associated with reduced rectal volume, reduced ability of the rectal walls to distend and accommodate stool, and increased rectal sensitivity. There is a continuous spectrum of different clinical presentations from incontinence of flatus (gas), through incontinence of mucus or liquid stool, to solids. The term anal incontinence often is used to describe flatus incontinence, however it is also used as a synonym for FI generally. It may occur together with incontinence of liquids or solids, or it may present in isolation. Flatus incontinence may be the first sign of FI. Once continence to flatus is lost, it is rarely restored. Anal incontinence may be equally disabling as the other types. Fecal leakage, fecal soiling and fecal seepage are minor degrees of FI, and describe incontinence of liquid stool, mucus, or very small amounts of solid stool. They cover a spectrum of increasing symptom severity (staining, soilage, seepage and accidents). Rarely, minor FI in adults may be described as encopresis. Fecal leakage is a related topic to rectal discharge, but this term does not necessarily imply any degree of incontinence. Discharge generally refers to conditions where there is pus or increased mucus production, or anatomical lesions that prevent the anal canal from closing fully, whereas fecal leakage generally concerns disorders of IAS function and functional evacuation disorders which cause a solid fecal mass to be retained in the rectum. Solid stool incontinence may be called complete (or major) incontinence, and anything less as partial (or minor) incontinence (i.e. incontinence of flatus (gas), liquid stool and/or mucus). In children over the age of four who have been toilet trained, a similar condition is generally termed encopresis (or soiling), which refers to the voluntary or involuntary loss of (usually soft or semi-liquid) stool. The term pseudoincontinence is used when there is FI in children who have anatomical defects (e.g. enlarged sigmoid colon or anal stenosis). Encopresis is a term that is usually applied when there are no such anatomical defects present. The ICD-10 classifies nonorganic encopresis under "behavioural and emotional disorders with onset usually occurring in childhood and adolescence" and organic causes of encopresis along with FI. FI can also be classified according to gender, since the cause in females may be different from males, for example it may develop following radical prostatectomy in males, whereas females may develop FI as an immediate or delayed consequence of damage whilst giving birth. Pelvic anatomy is also different according to gender, with a wider pelvic outlet in females. Several severity scales exist. The Cleveland Clinic (Wexner) fecal incontinence score takes into account five parameters that are scored on a scale from zero (absent) to four (daily) frequency of incontinence to gas, liquid, solid, of need to wear pad, and of lifestyle changes. The Park's incontinence score uses four categories: The fecal incontinence severity index is based on four types of leakage (gas, mucus, liquid stool, solid stool) and five frequencies (once to three times per month, once per week, twice per week, once per day, twice or more per day). Other severity scales include: AMS, Pescatori, Williams score, Kirwan, Miller score, Saint Mark's score and the Vaizey scale. FI may present with signs similar to rectal discharge (e.g. fistulae, proctitis or rectal prolapse), pseudoincontinence, encopresis (with no organic cause) and irritable bowel syndrome. The mechanisms and factors contributing to normal continence are multiple and inter-related. The puborectalis sling, forming the anorectal angle (see diagram), is responsible for gross continence of solid stool. The IAS is an involuntary muscle, contributing about 55% of the resting anal pressure. Together with the hemorrhoidal vascular cushions, the IAS maintains continence of flatus and liquid during rest. The EAS is a voluntary muscle, doubling the pressure in the anal canal during contraction, which is possible for a short time. The rectoanal inhibitory reflex (RAIR) is an involuntary IAS relaxation in response to rectal distension, allowing some rectal contents to descend into the anal canal where it is brought into contact with specialized sensory mucosa to detect consistency. The rectoanal excitatory reflex (RAER) is an initial, semi-voluntary contraction of the EAS and puborectalis which returns prevents incontinence following the RAIR. Other factors include the specialized anti-peristaltic function of the last part of the sigmoid colon, which keeps the rectum empty most of the time, sensation in the lining of the rectum and the anal canal to detect when there is stool present, its consistency and quantity, and the presence of normal rectoanal reflexes and defecation cycle which completely evacuates stool from the rectum and anal canal. Problems affecting any of these mechanisms and factors may be involved in the cause. FI is a sign or a symptom, not a diagnosis, and represents an extensive list of causes. Usually, it is the result of a complex interplay of several coexisting factors, many of which may be simple to correct. Up to 80% of people may have more than one abnormality that is contributing. Deficits of individual functional components of the continence mechanism can be partially compensated for a certain period of time, until the compensating components themselves fail. For example, obstetric injury may precede onset by decades, but postmenopausal changes in the tissue strength reduce in turn the competence of the compensatory mechanisms. The most common factors in the development are thought to be obstetric injury and after effects of anorectal surgery, especially those involving the anal sphincters and hemorrhoidal vascular cushions. The majority of incontinent persons over the age of 18 fall into one of several groups. These are: those with structural anorectal abnormalities (sphincter trauma, sphincter degeneration, perianal fistula, rectal prolapse), neurological disorders (multiple sclerosis, spinal cord injury, spina bifida, stroke, etc.), constipation/fecal loading (presence of a large amount of feces in the rectum with stool of any consistency), cognitive and/or behavioral dysfunction (dementia, learning disabilies), diarrhea, inflammatory bowel diseases (e.g. ulcerative colitis, Crohn's disease), irritable bowel syndrome, disability related (people who are frail, acutely unwell, or have chronic/acute disabilities), and those cases which are idiopathic (of unknown cause). Diabetes mellitus is also known to be a cause, but the mechanism of this relationship is not well understood. Anorectal anaomalies and spinal cord defects may be a cause in children. These are usually picked up and operated upon during early life, but continence is often imperfect thereafter. The functioning of the anal canal can be damaged, traumatically or atraumatically. The resting tone of the anal canal is not the only factor which is important, both the length of the high pressure zone and its radial translation of force are required for continence. This means that even with normal anal canal pressure, focal defects such as the keyhole deformity can be the cause of substantial symptoms. EAS dysfunction is associated with impaired voluntary control, whereas IAS dysfunction is associated with impaired fine tuning of fecal control. Lesions which mechanically interfere with, or prevent the complete closure of the anal canal can cause a liquid stool or mucous rectal discharge. Such lesions include piles (inflamed hemorrhoids), anal fissures, anal cancer or fistulae. Obstetric injury may tear the anal sphincters, and some of these injuries may be occult (undetected). The risk of injury is greatest when labor has been especially difficult or prolonged, when forceps are used, with higher birth weights or when an episiotomy is performed. Only when there is post operative investigation of FI such as endoanal ultrasound is the injury discovered. FI is a much under-reported complication of surgery. The IAS is easily damaged with an anal retractor (especially the Park's anal retractor), leading to reduced resting pressure postoperatively. Since the hemorrhoidal vascular cushions contribute 15% of the resting anal tone, surgeries involving these structures may affect continence status. Partial internal sphincterotomy, fistulotomy, anal stretch (Lord's operation), hemorrhoidectomy or transanal advancement flaps may all lead to FI post operatively, with soiling being far more common than solid FI. The "keyhole deformity" refers to scarring within the anal canal and is another cause of mucus leakage and minor incontinence. This defect is also described as a groove in the anal canal wall, and may occur after posterior midline fissurectomy or fistulotomy, or with lateral IAS defects. Rare causes of traumatic injury to the anal sphincters include military or traffic accidents complicated by pelvic fractures, spine injuries or perineal lacerations, insertion of foreign bodies in the rectum, and sexual abuse. Nontraumatic conditions causing anal sphincter weakness include scleroderma, damage to the pudendal nerves and IAS degeneration of unknown etiology. Radiation induced FI may involve the anal canal as well as the rectum, when proctitis, anal fistula formation and diminished function of internal and external sphincter occur. Irradiation may occur during radiotherapy, e.g. for prostate cancer. Many people with FI have a generalized weakness of the pelvic floor, especially puborectalis. A weakened puborectalis leads to widening of the anorectal angle, and impaired barrier to stool in the rectum entering the anal canal, and this is associated with incontinence to solids. Abnormal descent of the pelvic floor can also be a sign of pelvic floor weakness. Abnormal descent manifests as descending perineum syndrome (>4 cm perineal descent). This syndrome initially gives constipation, and later FI. The pelvic floor is innervated by the pudendal nerve and the S3 and S4 branches of the pelvic plexus. With recurrent straining, e.g. during difficult labour or long term constipation, then stretch injury can damage the nerves supplying levator ani. The pudendal nerve is especially vulnerable to irreversible damage, (stretch induced pudendal neuropathy) which can occur with a 12% stretch. If the pelvic floor muscles lose their innervation, they cease to contract and their muscle fibres are in time replaced by fibrous tissue, which is associated with pelvic floor weakness and incontinence. Increased pudendal nerve terminal motor latency may indicate pelvic floor weakness. The various types of pelvic organ prolapse (e.g. external rectal prolapse, mucosal prolapse and internal rectal intussusception & solitary rectal ulcer syndrome) may also cause coexisting obstructed defecation. The rectum needs to be of a sufficient volume to store stool until defecation. The rectal walls need to be "compliant" i.e. able to distend to an extent to accommodate stool. Rectal sensation is required to detect the presence, nature and amount of rectal contents. The rectum must also be able to evacuate its contents fully. There must also be efficient co-ordination of rectal sensation and relaxation of the anal canal. If the sensory nerves are damaged, detection of stool in the rectum is dulled or absent, and the person will not feel the need to defecate until too late. Rectal hyposensitivity may manifest as constipation, FI, or both. Rectal hyposensitivty was reported to be present in 10% of people with FI. Pudendal neuropathy is one cause of rectal hyposensitivity, and may lead to fecal loading/impaction, megarectum and overflow FI. Normal evacuation of rectal contents is 90-100%. If there is incomplete evacuation during defecation, residual stool will be left in the rectum and threaten continence once defecation is finished. This is a feature of people with soiling secondary to obstructed defecation. Obstructed defecation is often due to anismus (paradoxical contraction or relaxation failure of the puborectalis).:38 Whilst anismus is largely a functional disorder, organic pathologic lesions may mechanically interfere with rectal evacuation. Other causes of incomplete evacuation include non-emptying defects like a rectocele. Straining to defecate pushes stool into the rectocele, which acts like a diverticulum and causes stool sequestration. Once the voluntary attempt to defecate, albeit dysfunctional, is finished, the voluntary muscles relax, and residual rectal contents are then able to descend into the anal canal and cause leaking.:37 \|Drug/mechanism of action\|\|Common examples\| \|Drugs altering sphincter tone\| \|Broad spectrum antibiotics\| \|Topical drugs applied to anus (reducing pressure)\| \|Drugs causing profuse diarrhea\| \|Tranquilisers/hypnotics (reducing alertness)\| Continence requires conscious and subconscious networking of information from and to the anorectum. Defects/brain damage may affect the central nervous system focally (e.g. stroke, tumor e.g. spinal cord lesions, trauma, multiple sclerosis) or diffusely (e.g. dementia, multiple sclerosis, infection, Parkinson's disease or drug-induced). FI (and urinary incontinence) may also occur during epileptic seizures. Dural ectasia is an example of a spinal cord lesion that may affect continence. Liquid stool is more difficult to control than formed, solid stool. Hence, FI can be exacerbated by diarrhea. Some consider diarrhea to be the most common aggravating factor. Orlistat is an anti-obesity (weight loss) drug that blocks the absorption of fats. This may give side effects of FI, diarrhea and steatorrhea. This may occur when there is a large mass of feces in the rectum (fecal loading), which may become hardened (fecal impaction). Liquid stool elements are able to pass around the obstruction, leading to incontinence. Megarectum (enlarged rectal volume) and rectal hyposensitivity are associated with overflow incontinence. Hospitalized patients and care home residents may develop FI via this mechanism, possibly a result of lack of mobility, reduced alertness, constipating effect of medication and/or dehydration. Identification of the exact causes usually begins with a thorough medical history, including detailed questioning about symptoms, bowel habits, diet, medication and other medical problems. Digital rectal examination is performed to assesses resting pressure and voluntary contraction (maximum squeeze) of the sphincter complex and puborectalis. Anal sphincter defects, rectal prolapse, and abnormal perineal descent may be detected. Anorectal physiology tests assess the functioning of the anorectal anatomy. Anorectal manometry records the pressure exerted by the anal sphincters and puborectalis during rest and during contraction. The procedure is also able to assess sensitivity of the anal canal and rectum. Anal electromyography tests for nerve damage, which is often associated with obstetric injury. Pudendal nerve terminal motor latency tests for damage to the pudendal motor nerves. Proctography, also known as defecography, shows how much stool the rectum can hold, how well the rectum holds it, and how well the rectum can evacuate the stool. It will also highlight defects in the structure of the rectum such as internal rectal intussusception. Dynamic pelvic MRI, also called MRI defecography is an alternative which is better for some problems but not as good for other problems. Proctosigmoidoscopy involves the insertion of an endoscope (a long, thin, flexible tube with a camera) into the anal canal, rectum and sigmoid colon. The procedure allows for visualization of the interior of the gut, and may detect signs of disease or other problems that could be a cause, such as inflammation, tumors, or scar tissue. Endoanal ultrasound, which some consider to be the gold standard for detection of anal canal lesions, evaluates the structure of the anal sphincters, and may detect occult sphincter tears that otherwise would go unseen. Functional FI is common. The Rome process published diagnostic criteria for functional FI, which they defined as "recurrent uncontrolled passage of fecal material in an individual with a developmental age of at least 4 years". The diagnostic criteria are, one or more of the following factors present for the last 3 months: abnormal functioning of normally innervated and structurally intact muscles, minor abnormalities of sphincter structure/innervation (nerve supply), normal or disordered bowel habits, (i.e., fecal retention or diarrhea), and psychological causes. Furthermore, exclusion criteria are given. These are factors which all must be excluded for a diagnosis of functional FI, and are abnormal innervation caused by lesion(s) within the brain (e.g., dementia), spinal cord (at or below T12), or sacral nerve roots, or mixed lesions (e.g., multiple sclerosis), or as part of a generalized peripheral or autonomic neuropathy (e.g., due to diabetes), anal sphincter abnormalities associated with a multisystem disease (e.g., scleroderma), and structural or neurogenic abnormalities that are the major cause. \|Stool consistency\|\|Cause\|\|First line\|\|Second line\| \|Diarrhea\|\|Inflammatory\|\|Anti-inflammatory drugs\|\|Constipating drugs\| \|Solid\|\|Pelvic floor\|\|Biofeedback\|\|Sacral nerve stimulation\| \|Sphincter intact\|\|Sacral nerve stimulation\|\|Lavage\| \|Sphincter rupture\|\|Anal repair\|\|Sacral nerve stimulation/Neosphincter\| \|Rectal prolapse\|\|Rectopexy\|\|Perineal resection\| \|Soiling\|\|Keyhole defect\|\|Lavage\|\|PTQ implant\| FI has an impact on virtually all aspects of peoples' lives, greatly diminishing physical and mental health, and affect personal, social and professional life. Emotional effects may include stress, tearfulness, anxiety, exhaustion, fear of public humiliation, feeling dirty, poor body-image, reduced desire for sex, anger, humiliation, depression, isolation, secrecy, frustration and embarrassment. Some people may need to be in control of life outside of FI as means of compensation. The physical symptoms such as skin soreness, pain and odor may also impact quality of life. Physical activity such as shopping or exercise is often affected. Travel may be affected, requiring careful planning. Working is also affected for most. Relationships, social activities and self-image likewise often suffer. Symptoms may worsen over time, but FI is generally treatable with conservative management, surgery or both. The success of treatment depends upon the exact causes and how easily these are corrected. Treatment choice depends on the cause and severity of disease, and the motivation and general health of the person effected. Commonly, conservative measures are used together, and if appropriate surgery carried out. Treatments may be attempted until symptoms are satisfactorily controlled. A treatment algorithm based upon the cause has been proposed, including conservative, non-operative and surgical measures (neosphincter refers to either dynamic graciloplasty or artificial bowel sphincter, lavage refers to retrograde rectal irrigation). Conservative measures include dietary modification, drug treatment, retrograde anal irrigation, biofeedback retraining anal sphincter exercises. Incontinence products refer to devices such as anal plugs and perineal pads and garments such as diapers/nappies. Perineal pads are efficient and acceptable for only minor incontinence. Dietary modification may be important for successful management. Both diarrhea and constipation can contribute to different cases, so dietary advice must be tailored to address the underlying cause or it may be ineffective or counter productive. In persons with disease aggravated by diarrhea or those with rectal loading by soft stools, the following suggestions may be beneficial: increase dietary fiber; reduce wholegrain cereals/bread; reduce fruit and vegetables which contain natural laxative compounds (rhubarb, figs, prunes/plums); limit beans, pulses, cabbage and sprouts; reduce spices (especially chilli); reduce artificial sweeteners (e.g. sugar free chewing gum); reduce alcohol (especially stout, beer and ale); reduce lactose if there is some degree of lactase deficiency; and reduce caffeine. Caffeine lowers the resting tone of the anal canal and also causes diarrhea. Excessive doses of vitamin C, magnesium, phosphorus and/or calcium supplements may increase FI. Reducing olestra fat substitute, which can cause diarrhea, may also help. Pharmacological management may include anti-diarrheal/constipating agents and laxatives/stool bulking agents Stopping or substituting any previous medication that causes diarrhea may be helpful in some (see table). There may a benefit from constipating agents in those with coexisting diarrhea. There is little evidence regarding the relative efficacy of medications to other treatments or the use of medications in those with normal stool consitency. Anti-diarrheal/ constipating agents such as loperamide, codeine phosphate or diphenoxylate with atropine (co-phenotrope) may be used. Loperamide reduces stool weight, decreases bowel motility, rectoanal inhibitory reflex sensitivity and may slightly alter resting anal tone. Codeine phosphate and co-phenotrope are less commonly used because greater side effects. In people who have undergone gallbladder removal, the bile acid sequestrant colestyramine may help minor degrees of FI. Laxatives may be used in elderly people where difficulties are secondary to constipation or fecal impaction (paradoxical diarrhea/ overflow incontinence). A common example is lactulose. Regular use of this laxative is intended to prevent recurrence of impaction. Stool bulking agents may reduce symptoms of obstructed defecation. Bulking agents also absorb water, so may be helpful those with diarrhea. A common side effect is bloating and flatulence. Topical agents to treat and prevent dermatitis may also be used, such as topical antifungals when there is evidence of perianal candidiasis or occasionally mild topical anti-inflammatory medication. Prevention of secondary lesions is carried out by perineal cleansing, moisturization, and use of a skin protectant. Evacuation aids (suppositories or enemas) e.g. glycerine or bisacodyl suppositories may be prescribed. People may have poor resting tone of the anal canal, and consequently may not be able to retain an enema, in which case transanal irrigation (retrograde anal irrigation) may be a better option, as this equipment utilizes an inflatable catheter to prevent loss of the irrigation tip and to provide a water tight seal during irrigation. A volume of luke warm water is gently pumped into the colon via the anus. People can be taught how to perform this treatment in their own homes, but it does require special equipment. If the irrigation is efficient, stool will not reach the rectum again for up to 48 hours. By regularly emptying the bowel using transanal irrigation, controlled bowel function is often re-established to a high degree in patients with bowel incontinence and/or constipation. This enables control over the time and place of evacuation and development of a consistent bowel routine. However, persistent leaking of residual irrigation fluid during the day may occur and make this option unhelpful, particularly in persons with obstructed defecation syndrome who may have incomplete evacuation of any rectal contents. Consequently, the best time to carry out the irrigation is typically in the evening, allowing any residual liquid to be passed the next morning before leaving the home. Complications such as electrolyte imbalance and perforation are rare. The impact of transanal irrigation varies considerably. Some individuals experience complete control of incontinence, and other report little or no benefit. It has been suggested that if appropriate, people be offered home retrograde anal irrigation. Biofeedback (the use equipment to record or amplify and then feed back activities of the body) is a commonly used and researched treatment, but the benefits are uncertain. Biofeedback therapy varies in the way it is delivered, but it is unknown if one type has benefits over another. The role of pelvic floor exercises and anal sphincter exercises in FI is poorly determined. While there may be some benefit they appear less useful than implanted sacral nerve stimulators. These exercises aim to increase the strength of the pelvic floor muscles (mainly levator ani). The anal sphincters are not technically part of the pelvic floor muscle group, but the EAS is a voluntary, striated muscle which therefore can be strengthened in a similar manner. It has not been established whether pelvic floor exercises can be distinguished from anal sphincter exercises in practice by the people doing them. This kind of exercise is more commonly used to treat urinary incontinence, for which there is a sound evidence base for effectiveness. More rarely are they used in FI. The effect of anal sphincter exercises are variously stated as an increase in the strength, speed or endurance of voluntary contraction (EAS). Electrical stimulation can also be applied to the anal sphincters and pelvic floor muscles, inducing muscle contraction without traditional exercises (similar to transcutaneous electrical nerve stimulation, TENS). The evidence supporting its use is limited, and any benefit is tentative. In light of the above, intra-anal electrical stimulation (using an anal probe as electrode) appears to be more efficacious than intra-vaginal (using a vaginal probe as electrode). Rarely, skin reactions may occur where the electrodes are placed, but these issues typically resolve when the stimulation is stopped. Surgically implanted sacral nerve stimulation may be more effective than exercises, and electrical stimulation and biofeedback may be more effective than exercises or electrical stimulation by themselves. TENS is also sometimes used to treat FI by transcutaneous tibial nerve stimulation. In a minority of people, anal plugs may be useful for either standalone therapy or in concert with other treatments. Anal plugs (sometimes termed tampons) aim to block involuntary loss of fecal material, and they vary in design and composition. Polyurethane plugs were reported to perform better than those made of polyvinyl-alcohol. Plugs are less likely to help those with frequent bowel movements, and many find them difficult to tolerate. Surgery may be carried out if conservative measures alone are not sufficient to control incontinence. There are many surgical options, and their relative effectiveness is debated due to a lack of good quality evidence. The optimal treatment regime may be a complex combination of various surgical and non-surgical therapies. The surgical options can be considered in four categories: restoration and improvement of residual sphincter function (sphincteroplasty, sacral nerve stimulation, tibial nerve stimulation, correction of anorectal deformity), replacement / imitation of the sphincter or its function (anal encirclement, SECCA procedure, non-dynamic graciloplasty, perianal injectable bulking agents), dynamic sphincter replacement (artificial bowel sphincter, dynamic graciloplasty), antegrade continence enema (Malone procedure), and finally fecal diversion (e.g. colostomy). A surgical treatment algorithm has been proposed. Isolated sphincter defects (IAS/EAS) may be initially treated with sphincteroplasty and if this fails, the person can be assessed for sacral nerve stimulation. Functional deficits of the EAS and/or IAS (i.e. where there is no structural defect, or only limited EAS structural defect, or with neurogenic incontinence) may be assessed for sacral nerve stimulation. If this fails, neosphincter with either dynamic graciloplasty or artificial anal sphincter may be indicated. Substantial muscular and/or neural defects may be treated with neosphincter initially. FI is thought to be very common, but much under-reported due to embarrassment. One study reported a prevalence of 2.2% in the general population. It affects people of all ages, but is more common in older adults (but it should not be considered a normal part of aging). Females are more likely to develop it than males (63% of those with FI over 30 may be female).In 2014, the National Center for Health Statistics reported that one out of every six seniors in the U.S. who lived in their own home or apartment had FI. Men and women were equally affected. 45–50% of people with FI have severe physical and/or mental disabilities. Risk factors include age, female gender, urinary incontinence, history of vaginal delivery (non-Caesarean section childbirth), obesity, prior anorectal surgery, poor general health and physical limitations. Combined urinary and fecal incontinence is sometimes termed double incontinence, and it is more likely to be present in those with urinary incontinence. Traditionally, FI was thought to be an insignificant complication of surgery, but it is now known that a variety of different procedures are associated with this possible complication, and sometimes at high levels. Examples are midline internal sphincterotomy (8% risk), lateral internal sphincterotomy, fistulectomy, fistulotomy (18-52%), hemorrhoidectomy (33%), ileo-anal reservoir reconstruction, Lower anterior resection, total abdominal colectomy, ureterosigmoidostomy, and anal dilation (Lord's procedure, 0-50%). Some authors consider obstetric trauma to be the most common cause. While the first mention of urinary incontinence occurs in 1500 BC in the Ebers Papyrus, the first mention of FI in a medical context is unknown. For many centuries, colonic irrigation was the only treatment available. Stoma creation was described in 1776, FI associated with rectal prolapse in 1873 and anterior sphincter repair in 1875. During the mid 20th Century, several operations were developed for instances where the sphincters were intact but weakened. Muscle transpositions using the gluteus maximus or the gracilis were devised, but did not become used widely until later. End-to-end sphincteroplasty is shown to have a high failure rate in 1940. In 1971 Parks and McPartlin first describe an overlapping sphincteroplasty procedure. Biofeedback is first introduced in 1974. In 1975, Parks describes post anal repair, a technique to reinforce the pelvic floor and EAS to treat idiopathic cases. Endoanal ultrasound is invented in 1991, which starts to demonstrate the high number of occult sphincter tears following vaginal deliveries. In 1994, the use of an endoanal coil during pelvic MRI shows greater detail of the anal canal than previously. During the last 20 years, dynamic graciliplasty, sacral nerve stimulation, injectable perianal bulking agents and radiofrequency ablation have been devised, mainly due to the relatively poor success rates and high morbidity associated with the earlier procedures. Persons with this symptom are frequently ridiculed and ostracized in public. It has been described as one of the most psychologically and socially debilitating conditions in an otherwise healthy individual. In older people, it is one of the most common reasons for admission into a care home. Persons who develop FI earlier in life are less likely to marry and obtain employment. Often, people will go to great lengths to keep their condition secret. It has been termed "the silent affliction" since many do not discuss the problem with their close family, employers or clinicians. They may be subject to gossip, hostility, and other forms of social exclusion. The economic cost has not received much attention. In the Netherlands, outpatients were reported to have total costs of €2169 annually, and over half of this was productivity loss in work. In the USA, the average lifetime cost (treatment and follow-up) was $17,166 per person in 1996. The average hospital charges for sphincteroplasty was $8555 per procedure. Overall, in the USA, the total charges associated with surgery increased from $34 million in 1998 to $57.5 million in 2003. Sacral nerve stimulation, dynamic graciloplasty and colostomy were all shown to be cost effective. Doses of 30% - 40% phenylephrine gel may increase anal canal resting pressure to the levels comparable with the normal range. Engineered anal sphincters grown from stem cells have been successfully implanted in mice. New blood vessels developed and the tissue displayed normal contraction and relaxation. In the future, these methods may become part of the management of FI, replacing the need for high morbidity implanted devices such as the artificial bowel sphincter.	2	7	0	0	0	1	0.941511	1	7,928
Coding for Low Back Pain Low Back Pain is one of the most common Chief Complaints and problems by patients presenting to physicians in general and to Orthopedic Surgeons in the practice of general orthopedics, right up there with knee pain and shoulder pain. For practical purposes, at some point in their life, most everybody suffers an episode of lower back pain. Much of the introduction to this Blog is spent discussing the anatomy of the lower back, and its complexities. This discussion may seem excessive to Coders, but it also helps explain why the exact diagnosis for the cause of the low back pain is so difficult to zero in on. When I refer to the topic of Low Back Pain, I am including the Thoracolumbar Spine (functionally from T11-L2), the Lumbar Spine (L1-L5), the Lumbosacral Spine/junction (L5-S1), the Sacrum, and the Sacroiliac Joints. All of these areas can be the source of low back pain. Although technically the Thoracolumbar Spine includes T12 and L1, from a functional standpoint I consider the thoracolumbar spine/junction/region to include T11, T12, L1, and L2. The ribs of the T11 and T12 vertebrae do not have a direct anatomical connection of the rest of the rib cage (T1-T10). The rib cage contributes to the stability of the Thoracic Spine, but since the lower 2 ribs are not connected to the rib cage, they do not provide the same additional support and stability to T11 and T12. In that respect, the T11 and T12 vertebrae function more like Lumbar Spine vertebrae. This is a segment of the spine that transitions from the more stable, less mobile Thoracic Spine (T1-T10) to the more mobile and flexible Lumbar Spine. Also, this is the transition zone from the dorsal kyphotic curve of the Thoracic Spine to the lordotic curve of the Lumbar Spine and Sacrum. Because of these transitions, this segment of the spine can be exposed to greater stresses and strains. In the Lumbar Spine, normally there are 5 lumbar vertebrae. However, during the development of the lower lumbar spine and its junction with the sacrum (which is several fused vertebral segments), variations in the anatomy do occur. Occasionally the 5th Lumbar vertebra can be partially or completely fused to the Sacrum in such a way that it functions more as a Sacral vertebra than a Lumbar vertebra. This is considered incomplete segmentation of the spine. In the situation sited, this is referred to as “Sacralization of L5.” At the other end of the spectrum is another developmental segmentation problem in which the 1st Sacral vertebrae is not completely fused or incorporated into the sacrum, and from a functional standpoint functions more like a 6th Lumbar vertebra. This variation is referred to as “Lumbarization of S1.” There are also some developmental abnormalities in the completion of the development and closure of the bony neural arch such that it does not completely close, which is Spinal Bifida. Most commonly this is very limited to about the S1 level and is called Spina Bifida Occulta. This is often seen on x-rays of the lumbar and sacral spine, but in this very limited form is not a cause of low back pain. The more extreme version of Spina Bifida can cover several segments of the lumbar and sacral spine with associated meningomyelocele, and which can cause devastating neurologic and orthopedic problems. These appear at birth and require immediate surgical attention. Another significant aspect of the anatomy of the spine, both from the neurologic and the skeletal aspect, is that the spinal cord does not go the entire length of the spine. It generally terminates at about the level of L1 and beyond that point is converted into the nerve roots of the Lumbar and Sacral spine, called the Cauda Equina. This means that from about T12-L1 on down through the remaining Lumbar Spine, disorders of the Lumbar Spine affect only the nerve roots, not the spinal cord itself. Therefore, Myelopathy which is neurologic damage to the spinal cord can only occur in the cervical and thoracic regions down to the terminal spinal cord at L1. Myelopathy cannot occur where there is no spinal cord. However, Radiculopathy, which is damage to the spinal nerve roots (radicals) can occur at any level of the spine. However, since there is no spinal cord in the Lumbar Spinal Canal, disorders of the lumbar spine can only result in radicular problems such as radiculitis or radiculopathy. As stated, although this may seem to be an excessive amount of “education” regarding anatomy, it helps explain the difficulty in accurately identifying the cause of a patient’s low back pain, i.e. with specificity. From the standpoint of the incidence of Low Back Pain, Idiopathic Low Back Pain, which essentially means back pain for which we cannot identify a specific cause, is the most common. This would be coded as a Regional Pain. Often however there is associated muscular involvement resulting in muscular pain (myalgia, myofascial), with or without associated muscle spasm. But, muscular pain and/or spasm can be the “primary” abnormality/element of the back pain (i.e. the cause), or the muscular pain and spasm can be “secondary,” i.e. the result of some other spinal or associated soft tissue disorder or disease. That disorder causes the pain, but the muscles are recruited into the process as they are activated and work to help support and protect the injured bone or soft tissue. It is sometimes impossible to determine whether the muscular involvement is primary or secondary. This makes “Low Back Pain with Muscular Pain and/or Spasm” a common diagnosis. The other frequent elements or causes of Low Back Pain are Intervertebral Disc Disorders, with or without nerve root or radicular involvement. This would include such things as disc degeneration and disc herniations, ruptures, protrusions, extrusions (which are included in the term “displacement“ in ICD-10). "Bulging disc” is a mild form of early disc degeneration, but according to most authorities is not considered a symptom causing lesion. In other words, if a patient suffers from low back pain, it is not the result of a “bulging disc.” With time and as a result of the aging process degenerative processes occur through the lower spine leading to Arthritis/Spondylosis of the spine. Spondylosis/Spondylopathy is the term used in ICD-10. The wear and tear and degenerative processes in the spine can affect all the anatomic elements including the discs, the vertebral bodies (spurs and spur formation), facet joints, and other soft tissues and ligaments that can then cause pain in and of themselves, and stenosis of the spinal canal and/or the neural foramina which can affect the nerves as they pass down the spinal canal or as they exit through the foramina to go out to the rest of the body, i..e. radicular involvement. In the process of coding for Low Back Pain, which is also/sometimes called Lumbago, the most generic code for this Regional, Site of Pain, comes from Dorsalgia (Other Dorsopathies) and comes from the M54 Code Set. The Code for Low Back Pain is M54.5 (Lumbago NOS), which Excludes strain, disc disorders, and Lumbago with Sciatica (M54.4). This is the code that would apply to Idiopathic Low Back Pain, the Regional Pain in the lower back, which may be an isolated phenomenon, without any other identifiable spinal or soft tissue disorder causing or contributing to the back pain. There are other codes in the Dorsalgia Code Set that add to the confusion of coding for Low Back Pain. There is code M54.3: Sciatica/Sciatic Neuropathy, but this Excludes Disc Disorders as a cause. Isolated Sciatica can occur, but rarely as an isolated phenomenon not caused by some other disorder. Then there is M54.4: Lumbago with sciatica, but this also Excludes Intervertebral Disc Disorders. This code/diagnosis is too vague and nonspecific to be very useful when compared to the more specific codes from the M51 Code Set: Thoracic, Thoracolumbar, Lumbar, and Lumbosacral Intervertebral Disc Disorders with myelopathy (M51.0 ), with radiculitis or radiculopathy (M51.1 ), Disc “Displacement" (M51.2 ) which includes disc herniation, rupture, protrusion, and extrusion, and Disc Degeneration (M51.3 ). Of course, there is always “Other" (M51.4 ) which literally means something other than the above. One of the codes in the Dorsalgia Code Set is M54.0: “Panniculitis,” which literally means “inflammation of the subcutaneous fat characterized by the development of single or multiple subcutaneous nodules.” Many patients have tender subcutaneous “nodules" in the lumbosacral region. I do not know whether these are fatty or fibrous nodules. In 40 years of clinical practice, I have never used the term Panniculitis as an explanation of the patient's low back pain. As it refers to the Dorsalgia Code Set, the 5th Characters are 5 for the Thoracolumbar Spine, 6 for the Lumbar Spine, 7 for the Lumbosacral Spine/junction, and 8 for the Sacral Spine. Although I will discuss muscular and soft tissue aspects of low back pain later in this Blog, it is important to note that more often than not there is not a single diagnosis or code that can be used for coding Low Back Pain. Often it will require a list of codes starting with the generic code for Low Back Pain and supplemented by other codes that may either indicate associated findings such as muscular pain or spasm, or other diagnoses that could be possible or contributing causes for the back pain. In other words the coding may result in something like "Low Back Pain with or associated with…” It may be impossible with a list of several possible/probable contributing problems to prioritize them accurately. The next major category of Spinal Disorders is the Deforming Dorsopathies (M40-M43) which Includes Kyphosis and Lordosis (M40), but which Excludes Congenital (Q76) and Post-procedural Deformities (M96); Scoliosis (M41) (which Excludes Congenital forms (Q codes) and Post-procedural Deformities (M96), but Includes Infantile (M41.0), Juvenile and Adolescent (M41.1), Other Idiopathic (M41.2), Thoracogenic (M41.35 for the TL spine), Neuromuscular (M41.4), “Other” Secondary (M41.5), and finally “Other" Forms (M41.8) of Scoliosis. During the ages of growth and development, these deformities are not necessarily a cause of back pain, but as time and aging occur, the spine starts to degenerate as result from these deformities, and then these can evolve into secondary spondylopathies/arthritis of the spine in adulthood that can be a source of back pain. Next, comes Spinal Osteochondroses (Osteochondritis) (M42). The only one of any relevance is Scheuermann's disease (Juvenile Osteochondrosis of the Spine) seen during the teenage years and more frequently in boys than girls, and most commonly occurs in the mid and lower Thoracic Spine, but can go into the upper Lumbar Spine. This can be a source of back pain. It causes a Kyphotic “hump" of the back best seen when the patient bends forward, and particularly when viewed from the side. The "Inflammation” (Chondritis) involves the vertebral body at the epiphyseal rings resulting in abnormal growth of the vertebral body resulting in wedging deformities of the vertebrae. This causes the kyphotic “hump." In the M43 Code Set for Other Deforming Dorsopathies, are the codes for Spondylolysis and Spondylolisthesis. The code for Spondylolysis is M43.0, which Excludes Congenital Spondylolysis (Q76.2) and Spondylolisthesis (M43.1). Spondylolysis is a defect in the bony ring (neural arch) surrounding the spinal canal that can be a cause of pain, and can result in spinal instability Spondylolisthesis (M43.1), which is a displacement (slippage) of the vertebrae above with the defect in relation to the vertebrae below without this bony defect. There are different gradations (I-IV) which designate the progression from mild to severe, depending on the amount of displacement. This can be painful, but surprisingly, not always. The most common levels involved are L5-S1, followed by L4-L5, and far less frequently above this level. There is a type of Acquired Spondylolysis, but rarely with any listhesis (displacement) which has been identified in young adult athletes, particularly gymnasts and college level football players. This has been categorized as a "Stress Fracture” of the neural arch, i.e. Spondylolysis. This is most common in the low lumbar spine, and would be coded from the M48.4 Code Set (M48.46X for the Lumbar Spine above L5, and M48.47X for the Lumbosacral Spine, which would involve the L5 vertebrae). This particular Code Set requires 7 characters to complete the code, with options of A, D, G, or S. Another code from the M43 Code Set is M43.24: “Spontaneous" Spinal Fusion, which can occur in the lumbar spine, but is very rare, and more often than not, is the result of advanced and aggressive arthritic processes such as Hypertrophic Spondylopathy (M48.1). This “Spontaneous Fusion” results from bony spurs from the vertebral body margin that bridge over the intervening disc space and unite with the spurs from the adjacent vertebra. The next group would be the Spondylopathies (M45-M49), Arthritis of the spine. M45.0 is for Ankylosing Spondylitis, which is isolated Rheumatoid Arthritis of the Spine. It is a variation of Rheumatoid Arthritis, but isolated to the spine. Rheumatoid Arthritis is a generalized disorder of the body, but can affect the spine, most significantly and severely in the cervical region. As for Ankylosing Spondylitis, it generally starts in the sacroiliac regions then progresses upward through the entire spine all the way to the skull when the process is complete. It can result in significant pain and deformity of the spine. The M46 Code Set applies to "Other" Inflammatory or Infectious Spondylopathies. These are usually a manifestation of a systemic inflammatory or infectious disease which has some spinal involvement. However, occasionally some of these disorders such as Osteomyelitis and Pyogenic and/or Non-pyogenic Discitis can occur as isolated spinal disorders causing pain. M46.2 is for Vertebral Osteomyelitis. M46.3 is for Pyogenic Discitis, i.e. result of a bacterial infection of the disc. M46.4 is for Non-pyogenic Discitis, which is a Discitis for which an infectious agent cannot be confirmed. M46.5 is for Other Infectious Spondylopathies, and M46.8 is for Other Specified Inflammatory Spondylopathies. These again would tend to be spinal manifestations of a systemic disease either inflammatory or infectious. If it as an infection, the infectious disease and/or infecting agent would also have to be coded. A Code Set M47 is for Spondylosis, which is Degenerative Arthritis of the Spine, which is very common, and when compared to the disorders discussed above for M45 and M46, is the most common degenerative disorder of the spine. The most common is M47.8: Other Spondylosis, without myelopathy or radiculopathy, i.e. without neurologic involvement. As it regards Spondylosis with Myelopathy (spinal cord compression) (M47.1), this can only occur at the T11-T12-L1 segments, as that is the only level of the lower spine for this discussion which contains the terminal end of the spinal cord. Therefore, this would be limited to M47.15 for the T11 or T12 level, and M47.16 for the L1 level. Spondylosis with Radiculopathy (nerve damage) is M47.2 , with a 5th Character of 5, 6, or 7 as previously defined. This causes neuritic pain originating in the lower back and extending into the lower extremity (Sciatica or Femoral Neuropathy). As an isolated disorder, Spondylosis rarely does this on its own, but as a part of other aspects of spinal degeneration that all add up and combined to result in nerve compression. These include the "Other" Spondylopathies of the M48 Code Set, and in particular Spinal Stenosis (M48.0 ), possibly Ankylosing Hyperostosis (M48.1 ), and Traumatic Spondylopathy (M48.3 ). I would be careful using M48.3 unless the previous “trauma" is vague and ill-defined. There may be a history of “trauma” which resulted in the Traumatic Spondylopathy which if identifiable should also be coded. Also, Disc Disorders in the Other Dorsopathies (M51 Code Set) can go along with these Spondylosis disorders. This would include Disc Disorder with Myelopathy (M51.0 ) (T11-T12, T12-L1 only), with Radiculopathy (M51.1 ), and Disc "Displacement" (M51.2 (which includes disc rupture, herniation, extrusion, and protrusion), and Degenerative Disc Disorder (M51.3 ). It may take multiple codes from these code sets to adequately cover all the possible/probable causes of the Low Back Pain, with or without neural involvement, as more than one may be at play. Fatigue/Stress Fractures of the vertebrae (Code Set M48.4) are very rare in normal, healthy bone such that these would be rare in the lower back region. Furthermore, this Code Set Excludes all forms of pathologic fractures. The only one that I think would fit into this category is the one described previously in the paragraph regarding Spondylolysis. As stated there, in young adult athletes with healthy bone an Acquired Spondylolysis has been identified. This is considered by most of the people that have studied it to be a Stress Fracture of the bony vertebral arch. From what I know about this, it occurs most commonly at the L5 level, which is part of the lumbosacral segment of the spine, and would be coded as M408.47X . If it were to occur at the L4 level or above, it would be M48.46X . As also stated before, these require a 7th Character of A, D, G, or S. As for the Code Set M48.5: Collapse/Wedge Compression Vertebral Fracture, this is not a particularly useful Code Set in the lower back either because this code set confines itself to this occurring in basically normal healthy bone. This Code Set Excludes current injury or trauma (S codes), all forms of pathologic fractures, and Stress/Fatigue Fractures (M48.4). These codes imply that "normal, healthy” vertebral bone fractured “spontaneously” during normal daily levels of activity. For practical purposes, this does not really happen in the Thoacolumbar, Lumbar, or Lumbosacral Spine. If a vertebral fracture of this type is identified, then you would have to look for some other underlying disease process, i.e. a pathologic process/fracture, or some traumatic insult of sufficient magnitude to cause normal bone to collapse (S Code). When it comes to Soft Tissue Causes (M60-M79), the Code Set of M60: Myositis, which is "inflammation" of the muscle, has some application to lower back pain. Myositis can be the result of an infectious process (M60.0), non-infectious or sterile myositis (M60.1), or secondary to an intramuscular embedded foreign body (M60.2) such as a residual foreign body from a gunshot wound, etc. These could cause low back pain. The most useful of this Code Set would be in M62.8: Other Specified Disorders of Muscles, and in particular M62.830: Muscle Spasm or Cramp of the back. As for Synovial and/or Tendinous problems (M65-M67), there is really nothing that I can identify that is really relevant/useful to coding Low Back Pain. In Other Soft Tissue Disorders (M70-M79), there is the M70 Codes Set which includes Occupational Disorders for Pain secondary to use, overuse, and pressure, i.e. Occupational Low Back Pain for which there is no other clearly identifiable cause of the low back pain other than the stresses of work activities by the patient, particularly repetitive spinal motions. The code for this would be M70.88, with the 5th Character 8 standing for “other site”, i.e. the low back. This code also requires an Activity Code from the Y93 Code Set. Then there is good old plain “Muscular Pain” from M79.1, which includes Myalgia and Myofascial Pain (Syndrome), but Excludes Fibromyalgia (M79.7) and Myositis (M60.). Myalgia as such is not particularly specific so I would not use it as an isolated or primary code for low back pain. Myalgia has to be differentiated from Fibromyalgia (M79.7) (which is also called fibromyositis, myofibrositis, or fibrositis). Fibromyalgia is a more generalized pain disorder involving multiple areas of the back including the neck and shoulders, upper back, and can include the low back and hip regions. Confusion arises from the mixture of terms that are used. Many physicians will use the term Myofascial Pain (without necessarily including Syndrome) as a diagnosis, even though there is no identifiable pathology of the muscle or fascia, and the term just means there is localized muscle and/or fascial pain and tenderness. Others may use such terms as Localized Fasciitis or Fibrositis for the same thing. The M79.1 code is for localized muscle fascia pain and tenderness, whereas M79.7 is for the more generalized disorder of which localized findings in the lower back and hips may be just a part of the whole picture. In the category of Osteopathies (M80-M85), there are a large group of disorders that can cause Low Back Pain, in particular the Pathologic Fractures that occur from Osteoporosis (M80.0) for Age-related Osteoporosis including Involutional, Postmenopausal, and Senile Osteoporosis; M80.84 Other Osteoporosis (i.e. Secondary Osteoporosis), and M84.4, 5, and/or 6 for various Other Types of Pathologic Fractures. For these codes, the 6th Character is 8 for vertebral fractures. The M80 and M84 codes do require 7 characters to complete the code, with options of A, D, G, K, P, and S. The M81 Code Set is for Osteoporosis without current Pathologic Fracture and is the basic or primary code for Osteoporosis, which in and of itself does not cause pain. If there is low back pain in the presence of generalized osteoporosis and without a current pathologic fracture, then another cause for the pain needs to be identified and coded. Most often this would be associated arthritis of the spine. M83: Adult Osteomalacia (which literally means "softening of the bone”) would not in and of itself be a cause of Low Back Pain, and again some other cause for the pain would need to be identified. However, the softened bone might result in a pathologic fracture. As for Other Osteopathies (M86-M90) these do not really contain any useful codes for common causes for Low Back Pain except for Paget’s Disease of Bone (Osteitis Deformans), M88.1 for vertebral involvement, but not as a manifestation of a neoplastic disorder. If there is a neoplastic disease, Paget's Disease can be a manifestation, and the most correct code would be M90.68, but the neoplastic disorder also needs to be coded. There is also the possibility of Low Back Pain resulting from the Code Set M96: Intra-operative and Post-procedural Complications and Disorders of the Musculoskeletal System. This has many possible diagnoses that could result in chronic low back pain as the lumbar spine is a common site of surgical intervention, particularly if that surgery involves the use of spinal implants and devices for stabilization. The code for a Failed Spinal Fusion/Arthrodesis which would be a Pseudoarthrosis is M96.0. Post-Laminectomy Syndrome (M96.3) would be for ongoing low back pain following a lumbar spine surgical procedure/laminectomy, i.e. a failed procedure. M96.69: Bone Fracture following insertion of Orthopedic Implant or Device could certainly occur in lumbar spine surgery resulting in persistent postoperative low back pain. M96.8 includes Intra-operative and Postoperative Complications and Disorders of the Musculoskeletal System, which includes Intra-operative bleeding disorders such as Hemorrhage or Hematoma from a spinal procedure. The code for this would be M96.810. There also could be Accidental Puncture or Laceration of a Musculoskeletal Structure during a Musculoskeletal Procedure, which would be code M96.820. For Post-procedural bleeding disorder (hemorrhage or hematoma) following a Musculoskeletal procedure the code would be M96.830. As of the 2017 coding updates and changes, Intra-operative and Post-procedural hemorrhage and hematoma have not been separated into separate codes for hemorrhage and for hematoma as has been done for these complications for Skin and Subcutaneous Procedures and for Neurosurgical procedures. (See the Coding Blog for 2017 Coding Changes and Updates) The last of the Musculoskeletal categories is M99: Biomechanical lesions, not elsewhere classified (5th Character 2 for the TL spine, 3 for the L-spine, and 4 for the Sacral region). Most of these do apply to the spine (M99.1, 2, 3, 4, 5, 6, and 7) and could be causes for low back pain. However, these codes should not be used if the condition or cause of the pain can be identified more specifically elsewhere. This is a last resort code. There are Congenital Malformations, Deformations (Q00-Q07) that can result in low back pain problems in older patients such as Q05: Spina Bifida with or without hydrocephalus; Q67: Congenital Musculoskeletal Deformities of the Spine (Q67.5: Congenital Scoliosis and Postural Scoliosis); Q76: Congenital Malformations of the Spine, including Spina Bifida Occulta (Q76.0) which as stated earlier is not a cause for low back pain; Q76.2: Congenital Spondylolysis and Spondylolisthesis; Q76.3: Congenital Scoliosis; and Q76.4: Other Congenital Malformations of the Spine not associated with scoliosis. As has been previously stated, these are not necessarily painful disorders during the growth and development of the patient, but when older and in adulthood, they could cause pain, particularly with degenerative processes occurring in the spine with aging. Of the R Codes: General Symptoms and Signs (R50-R69) there is R52: Unspecified pain, which Includes acute pain and generalized pain, but Excludes Acute and Chronic Pain (G89) and Localized pain such as low back pain, with the coding directive to Code to Pain by Site. For practical purposes, this has no use for coding of low back pain problems. For the G Codes: Diseases of the Nervous System (G00-G99), there are the Code Sets for Nerve, Nerve Root, and Plexus Disorders (G50-G59). These have little use in the coding of Low Back Pain with neurologic involvement (radiculopathy, radiculitis, etc.) since all of these codes Exclude identifiable spinal and/or disc disorders resulting in nerve and nerve root compression. The code(s) for the Spinal Disorder causing the neurologic dysfunction would have to be used. This goes back to the M Codes previously discussed. This leaves the code set of G89 for coding Pain (NEC). The useful codes from this set are not "stand alone” codes, but can be used as supplemental codes to the underlying disease code and Site of Pain Codes such as M54 for Low Back Pain. These would be “second listed” to the primary diagnosis, “first listed" code to help provide additional emphasis to the nature and severity of the patient’s pain. The exception to this is in the field of Pain Management where the purpose of the evaluation and treatment is for the treatment of the pain, not the underlying disorder causing the pain. In this case the G89 code would be "first listed," and supported or supplemented by the underlying diagnosis/disease code (“second listed”). The codes from this set that would be useful in coding for Low Back Pain are G89.1: Acute pain (NEC) including G89.11: Acute pain due to trauma (however, the injury to the lower back could not have an identifiable trauma or S code to explain the pain), and G89.18: Other Acute post-procedural or postoperative pain, i.e. more severe pain and would be expected from the procedure, and not explained by an identifiable surgical complication (T84 code). G89.2 codes are for Chronic Pain (NEC) with G89.21: Chronic pain due to trauma (with the same restrictions as for G89.11), and G89.28: Other chronic post-procedural and postoperative pain (again not related to an identifiable surgical complication (T84 code)). G89.3 is for for Neoplasm related pain, acute or chronic. This could be used to supplement back pain secondary to primary or metastatic neoplastic pain in the lower spine. This is a long and extensive discourse on the coding of Low Back Pain, trying to cover as many relevant areas as possible out of the ICD-10 Codes. As stated at the beginning of this Blog, this is a very complicated region of the body with multiple bones, joints, muscles, ligaments, and tendons from which pain can arise. It has to be quite lengthy in order to provide sufficient education in detail for anyone who is involved with this area of coding. It is impossible to make it simple and straightforward. Posted in Coding Blogs on Sep 19, 2016	4	10	0	0	0	75	0.519331	75	7,032
What is a Ventral Hernia? When a separation or hole is present in the abdominal wall, it can allow abdominal tissue, such as part of an intestine, to push out through this hole forming a hernia. If the hernia is positioned in the vertical center of the abdominal wall it is classified as a ventral hernia. Ventral hernias are broken down into three categories which include: Epigastric Hernia – Found in both adults and children, epigastric hernias are usually located in the area between the sternum and the belly button and makeup about 3% of all abdominal hernias. Umbilical Hernia – These hernias form around the belly button and are common in infants but can also occur in adults. Incisional Hernia – Appears around the scar or incision site of a former abdominal surgical procedure. Incisional hernias can form months or even years after surgery and makeup about 20% of abdominal hernias. What causes a Ventral Hernia? Incision site weakness (possibly caused by infection or failed mesh surgical repair); At birth, there was a weakness in an area of the abdominal wall; Conditions that cause exertion or straining of the abdominal wall can create weaknesses and hernias. Straining can be caused by conditions such as: - Bowel injuries. - Chronic coughing. - Excessive or acute vomiting. - Pushing or lifting heavy objects. - Weakness of abdominal wall due to aging. - Constipation causes straining to have a bowel movement. - The prostate enlargement causes men to strain to urinate. - Lung diseases cause individuals to struggle to breathe and put a strain on the abdominal wall. What are the Symptoms of a Ventral Hernia? A ventral hernia may cause no symptoms at all or may create a bulge in the abdominal wall. This bulge may expand when coughing or exerting pressure such as while lifting a heavy object. The bulge may not create any discomfort or can be tender and painful. Symptoms that are commonly associated with a ventral hernia include: - A painful lump in the abdomen. - The skin over the abdominal bulge is red. - A lump or area that bulges on the abdomen but is not painful. - A lump in the abdomen that can be temporarily reduced by pushing with gentle pressure. - An abdominal bulge that gets bigger when pressure is increased such as while coughing or lifting a heavy object. Uncommon, but serious ventral hernia symptoms include: In rare instances, ventral hernia symptoms can be life-threatening and require immediate medical attention. Some of the symptoms to watch for include: - Chronic nausea or vomiting. - Sharp or severe pain in the abdomen. - Tight skin that is red and stretched over a swelling bulging area. - Difficulty having bowel movements or passing gas. - Fever of over 101 degrees. - Low output or no output of urine (infant who is not producing wet diapers with normal frequency). What are the Symptoms of a Ventral Hernia? Adults or children who have a weakness in their abdominal wall causing internal organs or tissue to protrude outside of the abdominal cavity may be candidates for ventral hernia repair. Hernia repair may be performed if: What Treatments are available? As a rule, ventral hernias do not go away on their own and eventually require surgical intervention to mitigate the risks of intestinal strangulation. A ventral hernia repair with mesh is the most commonly used treatment. The exception to this rule is when a ventral hernia is present in an infant (umbilical hernia) they normally go away by the time the child reaches the age of 4 or 5. If the hernia is still present after that time, then surgery may be necessary. Ventral Hernia Surgery Procedures Ventral hernia repairs can be performed through one of three techniques. Open ventral hernia repair, laparoscopic hernia repair, or robotic-assisted laparoscopic hernia repair. 1. Laparoscopic Hernia Repair Laparoscopic ventral hernia repair is considered a minimally invasive procedure and is normally done while the patient is asleep under general anesthesia. The ventral hernia repair is performed through about 3 to 5 small incisions made in the abdomen. A hollow tube-shaped surgical tool with an attached camera called a laparoscope guides the surgeon during the procedure. The surgeon will push the bulging tissue back into place and close and repair the weakness in the abdominal wall using a special surgical mesh. This material will help support and strengthen the abdominal weakness in this area and help prevent future occurrences. Some benefits of laparoscopic ventral hernia repair include: - Fewer complications. - Shorter recovery times. - Reduces the risks of post-operative infection. 2. Robotic-Assisted Laparoscopic Hernia Repair During a robotic ventral hernia repair, the same procedure steps and tools are used as during laparoscopic ventral hernia repair. The only difference is that instead of the surgeon manually holding the surgical tools, robotic arms are holding the tools while the surgeon controls them using a computer console with a viewing screen. 3. Open Hernia Repair Open ventral hernia repair can be done using general anesthesia or using local anesthesia and is often a good choice for patients who are intolerant to general anesthesia. During open ventral hernia repair, the surgeon creates an incision in the abdomen targeting the location of the hernia. The protruding abdominal or intestinal tissue is pushed back through the opening and normally a mesh material is used to repair and reinforce the area to avoid a future recurrence. The incision is normally closed with dissolvable sutures and surgical glue. How long is Recovery Time from Ventral Hernia Surgery? The recovery time from ventral hernia repair will vary from patient to patient and be dependent on the size, location, and extent of the repair and the technique performed. Many ventral hernias can be repaired in an outpatient setting while some large ventral hernias or more complicated hernias may require a hospital stay. The surgeon will provide aftercare instructions to each patient specifically tailored for them which may include instructions about when they may return to work, shower, drive, resume normal activities, etc. Some patients may be required to wear a special compression garment for 4 to 6 weeks that will provide stability and help prevent excessive swelling while the hernia repair site heals. The typical recovery from laparoscopic ventral hernia surgery is between two to six weeks. Deciphering information about your Hernia Repair from your Medical Records What does it mean when you see laparoscopic ventral hernia repair CPT 49652 in your medical records? With all the HIPPA laws and advancements in technology, more and more of our medical records are being loaded into an online program for us to access. After we attend an appointment, have lab tests, or undergo a procedure, we receive notification that the results have been posted to “our chart”. In the results, you will probably find numbers called CPT codes, or “current procedural terminology” codes. These codes are maintained by the American Medical Association and are universally recognized for the condition, illness, or treatment they are describing in the chart. So if you see CPT 49652 in your medical chart, this is the universally recognized procedural code for a laparoscopic ventral hernia repair. Where can I get a Laparoscopic Ventral Hernia Repair in Atlanta? If you are concerned about a hernia that has formed in your abdomen, contact IBI Healthcare Institute and set up a free consultation. Dr. Chris Ibikunle, MD, FACS, and the professionals at IBI Healthcare Institute perform laparoscopic ventral hernia repairs in Atlanta at their state-of-the-art surgery center serving Buckhead, Loganville, Duluth, and the surrounding areas. How much does Hernia Repair cost? The cost of Ventral Hernia Repair surgery will vary by patient taking into consideration different factors such as the size of the hernia, the type of hernia repair performed, the type of mesh used if any, and other patient-specific factors. The average cost of ventral hernia repair in the United States ranges between $4,000 and $11,000. Ventral hernia surgery is usually covered by health insurance as it is not considered cosmetic. The financial responsibility of the patient may be offset through the use of insurance, FSA, or HSA programs. Schedule a consultation or contact us to inquire most about Laparoscopic or Open Ventral Hernia Repair procedure costs and flexible financing options.	2	6	4	0	0	0	0.716152	4	1,860
NR 511 Completed Midterm study guide \| NR511 Midterm Study Guide Worksheet(2020) Complete A+ Guide;Chamberlain College of Nursing. Disease Risk Subjective Finding Objective Findings Diagnostics Treat... ment Education GI DISORDERS Appendicitis Celiac disease (autoimmune disorder caused by an immunologic response to gluten) Cholelithiasis Crohn’s Diverticulitis GERD Giardia H. Pylori Infection Irritable bowel syndrome Peptic ulcer disease (includes gastric ulcers and duodenal ulcers) Pancreatitis ACUTE\ CHRONIC Salmonella Shigella Ulcerative Colitis Viral gastroenteritis Bacterial conjunctivitis (viral is most contagious) Corneal abrasion Epiglotittis Eustachian tube disorder Hyphema (a layer of RBCs - hemorrhage) Meniere’s disease Mononucleosis: primarily caused by EBV Angie Nasal polyps Otitis Externa (AKA swimmer’s ear) Otitis Media Presbycusis (sensorineural hearing loss; not reversible) Rhinosinusitis - viral Rhinosinusitis - bacterial Streptococcus Tinnitus Viral conjunctivitis (highly contagious; usually caused by adenovirus which are associated with URIs or the common cold. Other viruses include HXV, HZV (zoster), and Molluscum contagiosum. 2 types of herpes viruses. HSV-1 typically occurs above the waist, and HSV-2 typically occurs below the waist. HSV conjunctivitis spread by contact with persons who have visible, infected lesions and with persons symptomatically shedding the virus. Meaning, the patient may be experiencing a prodrome of ill-related symptoms such as malaise, low grade fever, pain or tingling near site of the lesions (but the lesions are not yet visible). Allergic conjunctivitis is usually caused by an environmental allergen such as pollen, grass, trees, and so on. Occurrence can be seasonal and can be isolated to the eyes or include upper respiratory allergy symptoms such as rhinitis. Acanthosis nigricans: Benign dermatosis characterized by velvety, hyperpigmented, hyperkeratotic plaques Acne Actinic keratosis: Premalignant lesion that can progress to SCC. Alopecia areata, temporary loss of hair (nonscarring alopecia) Atopic dermatitis (eczema) is not considered a distinct entity but is a descriptive term for a group of skin disorders characterized by pruritus and inflammation whose distinct cause is unknown. Eczema is a more general term that is often used collectively to describe skin of an erythematous and inflamed appearance Basal Cell carcinoma (most common”): malignant tumor of the skin that originates in the basal cells of the epidermis. It is a slow-growing and locally invasive tumor that rarely metastasizes. Contact dermatitis: A common condition categorized as either irritant dermatitis or allergic dermatitis. Allergic dermatitis is from immunologically mediated response and irritant dermatitis is the result of repeated insults to atopic skin by caustic or irritant substances. Stages of contact dermatitis are: Acute: Erythema and edema Clear, fluid filled vesicles or bullae Exudate, clear fluid Distinct margins Subacute: Lessening edema Formation of papules Less distinct margins Chronic: Minimal edema Scaling skin Lichenification Minimal erythema Eczema Atopic dermatitis(eczema) is not considered a distinct entity but is a descriptive term for a group of skin disorders characterized by pruritus and inflammation whose distinct cause is unknown. Eczema is a more general term that is often used collectively to describe skin of an erythematous and inflamed appearance (SEE ATOPIC DERMATITS) Erythema infectiosum (AKA 5th disease or EI); primarily caused by human parvovirus B19 Erythema migrans (bull’s eye rash) associated with Lyme Disease Folliculitis: a superficial to deep skin infection of the hair follicles. Mainly caused by gram-positive bacteria, occasional by fungus or by gram-negative bacilli. Bacteria infect the hair follicle at a superficial level which leads to the clinical presentation of little pustules or erythema surrounding the base of the hair follicle. Pseudomonas folliculitis presents as follicular erythematous papules, pustules, or vesicles over the back, buttocks, and upper arms. Associated features include pruritus, malaise, low-grade fever, sore throat and eyes, and axillary lymphadenopathy. This type of folliculitis usually resolves spontaneously within 10 days. Shaving folliculitis is the result of sebaceous follicles which are colonized by gram negative bacteria become infected due to trauma from shaving. “Hot tub” folliculitis is a form of folliculitis that is caused by pseudomonas aeruginosa, which can withstand temperatures of up to 107 degrees F and chlorine levels up to 3mg/L. Hand Foot and Mouth Disease (HFMD) a contagious virus mostly occurring in young children which is caused by the coxsackievirus A16 and enterovirus 71. Keratosis pilaris – benign skin disorder resulting in hyperkeratinization of the hair follicles; Lichen Planus Jessica Sparks Melanoma Melasma Butterfly face Pregnancy mask Molluscum Contagiosum (viral infection that causes a mild skin rash, wart-like bump); caused by the Poxviridae virus. This virus is encased in a protective sac that prevents the immune system from being triggered. Pediculosis (head lice infestation) Pityriasis rosea Psoriasis Rosacea Roseola Infantum (AKA 6th disease) – caused by the human herpes virus types 6 and 7. Virus is mild and common in children under age 2; spread via saliva. Short lives 3-5 days Scabies Squamous Cell carcinoma: a malignant tumor originating from keratinocytes, can invade the dermis and occasionally metastasize to distant sites. Telogen Effluvium – can be a cause of non scarring alopecia. Is excessive shedding of scalp hair that results from an increases number of hair follicles entering the resting stage (telogen). Tinea versicolor (aka pityriasis versicolor) - Jenna Trichotillomania (hair pulling) - jenna Varicella Verruca vulgaris (Common warts): p.203 Small, hardened growths of keratinized tissue. Warts usually grow around nails, on fingers, and the backs of hands, but can appear anywhere on the body. Common warts (verruca vulgaris) are primarily caused by HPV serotypes 1 to 5, 7, 27, or 29, whereas HPV serotypes 3, 10, 28, and 29 cause flat warts. HPV serotypes 1 to 4, 27, 29, and 57 typically causes plantar warts, whereas HPV serotypes 6 and 11 cause anogenital warts. Vitiligo BUSINESS ESSENTIALS (jenna) Medical coding Medical billing CPT codes ICD10 codes Medicare Part A Medicare Part B Medicare Part D Health maintenance organization (HMO) (couldn’t find in text book) Preferred provider organization (PPO) (couldn’t find in text book) Indemnity insurer (couldn’t find in text book) Features Hepatitis A Virus (HAV) Hepatitis B Virus (HBV) Hepatitis C Virus (HCV) Hepatitis D Virus (HDV) Hepatitis E Virus (HEV) Transmission Incubation period (days) Laboratory tests Immunity/immunization Prevalence Course/mortality TABLE 15.2 Herpes Simplex Infections Infection Location Commonly Affected Age Group Oral—labial herpes simplex Herpetic keratoconjunctivitis Herpetic tracheobronchitis Herpes simplex encephalitis Herpes gladiatorum Herpetic whitlow Lumbosacral herpes Herpes simplex of the buttocks Genital herpes Eczema herpeticum Erythema multiforme Question 1. Treatment for achalasia may include: Balloon dilation of the lower esophageal sphincter. Question 2. Which oral medication might be used to treat a client with chronic cholelithiasis who is a poor candidate for surgery? Question 3. All of the following medications are used for the control of nausea and vomiting. Which medication works by affecting the chemoreceptor trigger zone, thereby stimulating upper gastrointestinal motility and increasing lower esophageal sphincter pressure? Question 4. Which is the most common presenting symptom of gastric cancer? Question 5. Margie, age 52, has an extremely stressful job and was just given a diagnosis of gastric ulcer. She tells you she is sure it is going to be malignant. How do you respond? Question 6. Marcie just returned from Central America with traveler’s diarrhea. Which is the best treatment? Question 7. Rose has gastroesophageal reflux disease (GERD). You know she misunderstands your teaching when she tells you she will: Have a snack before retiring so that the esophagus and stomach are not empty at bedtime. Question 8. Marty, age 52, notices a bulge in his midline every time he rises from bed in the morning. You tell him that it is a ventral hernia, also known as an: Question 9. You are trying to differentiate between functional (acquired) constipation and Hirschsprung disease in a neonate. Distinguishing features of Hirschsprung disease include which of the following? Question 10. You are doing routine teaching with a patient who has a family history of colorectal cancer. You know she misunderstands the teaching when she tells you she will: Question 11. Bobby, age 6, has constant periumbilical pain shifting to the right lower quadrant, vomiting, a small volume of diarrhea, absence of headache, a mild elevation of the white blood cell count with an early left shift, and white blood cells in the urine. You suspect: Question 12. Melva, age 63, presents with an acute exacerbation of pancreatitis, and you are going to admit her to the hospital. Which is the most important factor in determining a negative long-term outcome for her? Question 13. A mother brings in her 4-year-old child, who she states has acute abdominal pain and a rash. Which of the following do you initially rule out? Question 14. Icterus due to hyperbilirubinemia is seen when the serum level of bilirubin is greater than? 2.5 mg/dL. Question 15. A 45-year-old homeless man presents to your urgent care clinic for evaluation. His chief complaint is diarrhea. The patient states he started to have diarrhea 2-3 days ago, and it is getting progressively worse. He also notes nausea without vomiting, dry mouth, and double vision. On exam you notice his pupillary reflex is absent. The patient states he lives on the street and eats mostly canned goods that he scavenges from a grocery store dumpster. What is the likely cause of the patient’s symptoms? Question 16. Hepatitis D is an RNA virus that requires a coinfection with which of the following strains of hepatitis in Question 17. Which of the following is not true regarding hepatitis C? If hepatitis is asymptomatic it doesn’t cause cirrhosis or liver cancer. Question 18. A 54-year-old female presents to your primary care office for routine reevaluation for gastroesophageal reflux disease (GERD). She has been treated with diet modifications and 6 weeks of omeprazole without improvement of her symptoms. What is the next step in management of this patient’s GERD? Question 19. A 75-year-old male presents for a routine physical. He is obese and has no abdominal pain or recent injuries or problems. He has no complaints. He lies supine for his abdominal exam. He is nontender to palpation and has a normal exam. When he sits up you see a large mass protrude from his abdomen. It is central to his abdomen and inferior to his rib cage. It disappears when he is sitting up fully. What is the patient’s diagnosis? Question 20. Which gastrointestinal disease below could theoretically be completely eradicated with a total colectomy? Question 1. You auscultate Julie’s abdomen and hear a peritoneal friction rub. Which condition do you rule out? Question 2. Susan, age 59, has no specific complaints when she comes in for her annual examination. She does, however, have type 2 diabetes mellitus (DM), slight hypertension, dyslipidemia, and central obesity. How would you diagnose her? Question 3. Timothy, age 68, complains of an abrupt change in his defecation pattern. You evaluate him for: Question 4. Ruby has a colostomy and complains that her stools are too loose. What food(s) do you suggest to help thicken the stools? Cheese Question 5. Martina, age 34, has AIDS and currently suffers from diarrhea. You suspect she has which protozoal infection of the bowel? Question 6. When Sammy asks you what he can do to help his wife, who has dumping syndrome, what do you suggest he encourage her to do? Question 7. Lucy, age 49, has pain in both the left and right lower quadrants. What might you suspect? Question 8. You suspect appendicitis in Andrew, who is 18. With his right hip and knee flexed, you slowly rotate his right leg internally to stretch a muscle. He states that it is painful over his right lower quadrant. Which sign did you elicit? Rationales Option 1: Rovsing sign is pain elicited with deep palpation over the left lower quadrant (LLQ), with sudden resultant pain in the right lower quadrant (RLQ). Option 2: Psoas sign is pain elicited when the patient is instructed to try to lift the right leg against gentle pressure applied by the examiner or when the patient is placed in the left lateral decubitus position, extending the right leg at the hip. An increase in pain is considered positive and is an indication of the inflamed appendix irritating the psoas muscle. Option 3: Obturator sign is elicited when, with the patient’s right hip and knee flexed, the examiner slowly rotates the right leg internally, which stretches the obturator muscle. Pain over the right lower quadrant (RLQ) is considered a positive sign. Option 4: McBurney sign is pain elicited when pressure is applied to McBurney point, which is located halfway between the umbilicus and the anterior spine of the ilium. Question 9. You elicit costovertebral angle tenderness in Gordon, age 29. Which condition do you suspect? Question 10. Marvin, a known alcoholic with cirrhosis, is frequently admitted for coagulopathies and occasionally receives blood transfusions. His wife asks you why he has bleeding problems. How do you respond? Question 11. Olive has an acute exacerbation of Crohn’s disease. Which laboratory test value(s) would you expect to be decreased? Question 12. You suspect that Harry has a peptic ulcer and tell him that it has been found to be strongly associated with: Question 14. Dottie brings in her infant, who has gastroesophageal reflux. What do you tell her about positioning her infant? Question 15. To differentiate among the different diagnoses of inflammatory bowel diseases, you look at the client’s histological, culture, and radiological features. Mary has transmural inflammation, granulomas, focal involvement of the colon with some skipped areas, and sparing of the rectal mucosa. What do you suspect? Question 18. What is the best diagnostic test to confirm the diagnosis of celiac disease? Question 19. A 50-year-old female presents to the urgent care clinic complaining of left lower quadrant pain. She has associated nausea and vomiting, and her vital signs are as follows: temperature 102.5°F, pulse 110, blood pressure 150/90, pulse oximetry 99% on room air. What is the best test to evaluate this patient? Question 20. The screening guidelines for colon cancer recommend which of the following for the general population? Question 2. Zena just had a hemorrhoidectomy. You know she has not understood your teaching when she tells you she will: . Question 3. Simon, age 72, states that he is worried because he has a bowel movement only every third day. You respond: Question 4. Matt, age 26, recently returned from a camping trip and has gastroenteritis. He says that he has been eating only canned food. Which of the following pathogens do you suspect? Question 7. Anson tells you he thinks his antacids are causing his diarrhea. You respond: Question 9. Sandy, age 52, presents with jaundice, dark urine, and light-colored stools, stating that she is slightly improved over last week’s symptoms. Which stage of viral hepatitis do you suspect? Question 10. Ellie, age 42, has a seizure disorder and has been taking phenytoin (Dilantin) for years. Which supplement should she also be taking if no other problems exist? Question 13. Harvey just came back from Mexico. Which pathogen do you suspect is responsible for his diarrhea? Question 14. Which of the following medications/drugs are not known to cause heartburn or dyspepsia? Question 17. A 25-year-old male presents complaining of hematochezia. The patient states he has noticed this for 2 days. He states there is a streak of bright blood along his stool every time he has a bowel movement. The patient has no pain with his bowel movements. He admits to eating a poor diet. The patient has no abdominal pain, nausea, or vomiting. On physical exam the patient has a positive fecal occult blood test but has no noticeable rectal bleeding or lesions. What is the likely diagnosis? Question 20. Which of the following is the most common cause of acute pancreatitis? Question 1. The metabolism of which drug is not affected in Marsha, age 74? Question 2. Nausea is difficult to discern in a young child. What question might you ask to determine if a child has nausea? Question 3. Sally, age 21, is to undergo a tonsillectomy. She has heard about permanent taste changes after a tonsillectomy. What do you tell her? Question 7. Cydney has been given a diagnosis of ascariasis. Which symptoms would you expect to see? Question 8. Marian, age 52, is obese. She complains of a rapid onset of severe right upper quadrant abdominal cramping pain, nausea, and vomiting. Your differential diagnosis might be: Question 9. A 7-year-old male presents with his mother to the urgent care clinic complaining of abdominal pain. He started to complain of pain prior to going to bed; however, it has gotten progressively worse and is now preventing him from sleeping. He is nauseous but hasn’t vomited and didn’t eat dinner due to the pain. The patient appears pale and is complaining of right-sided abdominal pain. His vitals are as follows: blood pressure 130/85, pulse 120, temperature 100.5°F, pulse oximetry 98% on room air. On physical exam he is tender in the right lower quadrant. His complete blood count (CBC) shows a white blood cell count (WBC) of 17.0. What is the patient’s likely diagnosis? Question 10. What would you expect to see on an abdominal series that would lead toward a diagnosis of small-bowel obstruction? Question 2. What is the most common bacterial pathogen associated with acute otitis media? Question 4. When the Weber test is performed with a tuning fork to assess hearing and there is no lateralization, the nurse practitioner should document this finding as: Question 6. You are assessing a first grader and find that the tonsils are touching the uvula. How would you grade this finding? Question 1. Which of the following is not a cause of conductive hearing loss? Question 2. Jill, a 34-year-old bank teller, presents with symptoms of hay fever. She complains of nasal congestion, runny nose with clear mucus, and itchy nose and eyes. On physical assessment, you observe that she has pale nasal turbinates. What is your diagnosis? 1. Question 3. Mrs. Johnson, a 54-year-old accountant, presents to the office with a painful red eye without discharge. You should suspect: Question 4. April, age 50, presents with soft, raised, yellow plaques on her eyelids at the inner canthi. She is concerned that they may be cancerous skin lesions. You tell her that they are probably: Question 5. A 62-year-old woman presents to your clinic with a sudden right-sided headache that is worse in her right eye. She states that her vision seems blurred, and her right pupil is dilated and slow to react. The right conjunctiva is markedly injected, and the eyeball is firm. You screen her vision and find that she is 20/30 OS and 20/30 OD. She most likely has: 2. Rationales Option 1: With open-angle glaucoma, the onset is more insidious. Option 2: In angle-closure glaucoma, the patient presents with a sudden onset of symptoms as described in this case. This client has a visual deficit and pain as well as fullness of the affected eye. This is a medical emergency, and she should be referred immediately because, without intervention, blindness can occur within days. Option 3: Herpetic conjunctivitis is generally associated with a herpetic rash, and the pain is dull in character. Option 4: Diabetic retinopathy is a complication of diabetes that affects both eyes. It is caused by damage to the blood vessels of the light-sensitive tissue at the back of the eye (ie, the retina). Question 1. A 25-year-old male presents with “bleeding in my eye” for 1 day. He awoke this morning with a dark area of redness in his eye. He has no visual loss or changes. He denies constitutional symptoms, pruritus, drainage, or recent trauma. The redness presents on physical exam as a dark red area in the patient’s sclera of the right eye only and takes up less than 50% of the eye. The patient’s remaining sclera is clear and white. He also notes he was drinking alcohol last night and vomited afterward. What is the best treatment? Rationales Option 1: This would be the treatment for an infectious process; however, topical steroids need to be used in the eye with care. Option 2: This would be the treatment for a globe rupture or acute angle-closure glaucoma. Option 3: This is the classic presentation of a subconjunctival hemorrhage. It will resolve without treatment in 2 to 4 weeks. Vomiting probably caused his hemorrhage. Option 4: This is the treatment for viral conjunctivitis. Question 2. You prescribe Levaquin (levofloxacin) for a severe sinus infection. What is not a possible adverse reaction to this medication? Question 3. A 20-year-old male presents to your primary care clinic. This patient is a college student. He complains of fatigue, sore throat, and low-grade fever for 3 days. On physical exam, he has a temperature of 100.7°F. His ear exam is normal. His nose and throat exam shows mild erythema of the nasal mucosa and edematous, enlarged tonsils bilaterally, with erythema of the pharyngeal wall and tonsillar exudates. He has inflamed posterior cervical lymph nodes. He has a mild nonproductive cough and clear lung exam. What is his most likely diagnosis? Rationales Option 1: This presentation could be a viral pharyngitis; however, with posterior cervical lymphadenitis, you would suspect mononucleosis. Option 2: This presentation could be a viral pharyngitis; however, with posterior cervical lymphadenitis, you would suspect mononucleosis. Option 3: You would expect fever and tonsillar exudates without posterior cervical lymphadenitis. Option 4: Upper respiratory infections usually present with cough that may be productive and milder ear, nose, and throat symptoms. Question 4. When you are assessing the internal structure of the eye of your 59-year-old patient, the absence of a red reflex may indicate: Rationales Option 1: When assessing the internal structure of the eye, absence of a red reflex may indicate the total opacity of the pupil because of a cataract or hemorrhage into the vitreous humor. It may also be a result of improper positioning of the ophthalmoscope. Option 2: Acute iritis is noted by constriction of the pupil accompanied by pain and circumcorneal redness (ciliary flush). Option 3: The absence of a red reflex is not a normal finding. It may be caused by a cataract or a hemorrhage into the vitreous humor. Option 4: If areas of hemorrhage, exudate, and white patches are present when the internal structure of the eye is assessed, they are usually a result of diabetes or long-standing hypertension. Question 5. What significant finding(s) in a 3-year-old child with otitis media with effusion would prompt more There is a change in the child’s hearing threshold to greater than 25 dB. Rationales Option 1: If a child with otitis media with effusion has a change in the hearing threshold greater than 25 dB and has notable speech and language delays, more aggressive treatment is indicated. When the child’s hearing examination reveals a change in the hearing threshold, it is extremely important that the provider evaluate the child’s achievement of developmental milestones in speech and language. Any abnormal findings warrant referral. Option 2: A child with otitis media with effusion might become irritable during meals. Option 3: A slight delay in language and speech does not warrant aggressive treatment or referral. Option 4: Persistent rhinitis does not warrant aggressive treatment or referral. Question 6. Max, age 35, states that he thinks he has an ear infection because he just flew back from a business trip and feels unusual pressure in his ear. You diagnose barotrauma. What is your next action? Prescribe nasal steroids and oral decongestants. Question 7. Susan is a 19-year-old college student and avid swimmer. She frequently gets swimmer’s ear and asks if there is anything she can do to help prevent it other than wearing earplugs, which do not really work for her. What do you suggest? Start using a cotton-tipped applicator to dry the ears after swimming. Use ear drops made of a solution of equal parts alcohol and vinegar in each ear after swimming. Rationales Option 1: The adage “You shouldn’t put anything smaller than your elbow in your ear” holds true today. Option 2: Using ear drops made of a solution of equal parts alcohol and vinegar in each ear after swimming is effective in drying the ear canal and maintaining an acidic environment, therefore preventing a favorable medium for the growth of bacteria, the cause of swimmer’s ear. Option 3: A hair dryer operated on the lowest setting several inches from the ear may be used to dry the canal. Option 4: There is no reason for the client to stop swimming. Question 8. Mary, age 82, presents with several eye problems. She states that her eyes are always dry and look “sunken in.” What do you suspect? Normal age-related changes. Rationales Option 1: With hyperthyroidism, the eyes appear to bulge out (exophthalmos), but in hypothyroidism, the eyes do not appear any different. Option 2: Dryness of the eyes and the appearance of “sunken” eyes are normal age-related changes. Option 3: A moon face is apparent with Cushing syndrome, and this might make the eyes appear to be sunken in, although on close examination, they are not. Option 4: With a detached retina, the outward appearance is normal, but the client complains of seeing floaters or spots in the visual field and describes the sensation as like a curtain being drawn across the vision. Question 9. Nystatin (Mycostatin) is ordered for Michael, a 56-year-old banker who has an oral fungal infection. What instructions for taking the medication do you give Michael? “Swish and swallow the medication.” Rationales Option 1: The oral medication should not be diluted, as that may compromise the absorption. Option 2: Taking the medication with meals may compromise the absorption. Option 3: When ordering nystatin (Mycostatin) for an oral fungal infection, tell the client to swish the medication in the mouth to coat all the lesions and then to swallow it. Option 4: It is almost impossible to apply this liquid medication to only the lesions; swishing it in the mouth coats all the lesions more effectively. Question 10. The most common cause of a white pupil (leukokoria or leukocoria) in a newborn is: A congenital cataract. Rationales Option 1: The most common cause of a white pupil (leukokoria or leukocoria) in a newborn is a congenital cataract. The incidence may be as high as 1 in every 500 to 1000 live births, and there is usually a family history. Some infants require no treatment; however, in other cases surgery may be performed during the first few weeks of life. Option 2: Retinoblastoma, a common intraocular malignancy, is detected within the first few weeks of life and is the second most common cause of a white pupil. Option 3: Persistent hyperplastic primary vitreous is the third most common cause of a white pupil and is a congenital developmental abnormality. Option 4: Retinal detachment may occur as a result of trauma or disease and only rarely occurs in infancy. Question 11. You diagnose acute epiglottitis in Sally, age 5, and immediately send her to the local emergency room. Which of the following symptoms would indicate that an airway obstruction is imminent? Question 12. A 27-year-old female comes in to your primary care office complaining of a perioral rash. The patient noticed burning around her lips a couple days ago that quickly went away. She awoke from sleep yesterday and noticed a group of vesicles with erythematous bases where the burning had been before. There is no burning today. She is afebrile and has no difficulty eating or swallowing. What test would confirm her diagnosis? Question 13. Which condition is not included in the atopic triad? Question 14. The ABCDEs of melanoma identification include which of the following? Question 15. Your 24-year-old client whose varicella rash just erupted yesterday asks you when she can go back to work. What do you tell her? Question 16. Tom, age 50, is complaining of an itchy rash that occurred about a half hour after putting on his leather jacket. He recalls having a slightly similar rash last year when he wore his jacket. The annular lesions are on his neck and both arms. They are erythematous, sharply circumscribed, and both flat and elevated. His voice seems a little raspy, although he states that his breathing is normal. What is your first action? Question 17. You suspect a platelet abnormality in a 40-year-old woman who presents to your clinic with: Question 18. Justin, an obese 42-year-old, cut his right leg 3 days ago while climbing a ladder. Today his right lower leg is warm, reddened, and painful, without a sharply demarcated border. What do you suspect? Question 19. During a camping trip, Jim, age 35, abruptly developed fever, headache, and joint pain. A few days after the onset of the fever, a blanchable macular rash began on his wrists and ankles and quickly spread to the palms and soles before becoming generalized. The rash is now petechial. You suspect Jim has: Question 20. You’re teaching Mitch, age 18, about his tinea pedis. You know he doesn’t understand your directions when he tells you which of the following? Question 1. A 75-year-old female presents to your office complaining of dizziness and hearing loss. The patient states she awoke yesterday with dizziness, which she described as feeling the room spinning. She also notes intermittent ringing in her ears. On physical exam, the patient has lateralization of her hearing loss to the unaffected ear. Rinne test shows air conduction lasts longer than bone conduction. What is the next step in helping this patient’s symptoms? Question 3. Which of the following is not recommended for hoarseness? Question 4. Kevin, a 56-year-old lawyer, has throbbing pain in the left eye, an irregular pupil shape, marked photophobia, and redness around the iris. What is your initial diagnosis? Question 5. You are the nurse practitioner caring for Martha, a 47-year-old accountant. You have made a diagnosis of acute sinusitis based on Martha’s history and the fact that she complains of pain behind her eye. Which sinuses are affected? 2. Question 6. Marlene, a 57-year-old cashier, comes to the clinic because she is unable to differentiate between sharp and dull stimulation on both sides of her face. You suspect: Question 7. While doing a face, head, and neck examination on a 16-year-old patient, you note that the palpebral fissures are abnormally narrow. What are you examining? Question 8. A 44-year-old banker comes to your office for evaluation of a pulsating headache over the left temporal region, and he rates the pain as an 8 on a scale of 1 to 10. The pain has been constant for the past several hours and is accompanied by nausea and sensitivity to light. He has had frequent, though less severe, headaches for many years, and they are usually relieved by over-the-counter medicines. He is unclear as to a precipitating event but notes that he has had visual disturbances before each headache and he has been under a lot of stress in his job. Based on this description, what is the most likely diagnosis of this type of headache? Question 9. Your client, a 72-year-old smoker of 50 years, is at the office today for a routine physical. During your inspection of the oral mucosa, you discover a white lesion on the lateral surface of the tongue that you suspect to be cancerous. You document your finding as: Question 10. Which of the following conditions produces sharp, piercing facial pain that lasts for seconds to minutes? Question 11. Mary, age 82, presents with several eye problems. She states that her eyes are always dry and look “sunken in.” What do you suspect? Question 12. A 64-year-old obese woman comes in complaining of difficulty swallowing for the past 3 weeks. She states that “some foods get stuck” and she has been having “heartburn” at night when she lies down, especially if she has had a heavy meal. Occasionally, she awakes at night coughing. She denies weight gain and/or weight loss, vomiting, or change in bowel movements or color of stools. She denies alcohol and tobacco use. There is no pertinent family history or findings on review of systems (ROS). Physical examination is normal, with no abdominal tenderness, and the stool is occult blood (OB) negative. What is the most likely diagnosis? Question 13. Mr. Johnson, age 69, has had Meniere disease for several years. He has some hearing loss but now has persistent vertigo. What treatment might be instituted to relieve the vertigo? Question 14. Marjorie, age 37, has asthma and has been told she has nasal polyps. What do you tell her about them? 2. Cataracts are a common occurrence in patients over 60 years of age. You counsel your patient that the best cure for cataracts is: Corrective lens surgery. In a young child, unilateral purulent rhinitis is most often caused by: Kathleen, age 54, has persistent pruritus of the external auditory canal. External otitis and dermatological conditions, such as seborrheic dermatitis and psoriasis, have been ruled out. What can you advise her to do? Question 19. At the clinic, you are assessing Kyle, a 4-month-old baby, for the first time and notice that both eyes are turning inward. What is this called? Question 20. Kevin, age 26, has AIDS and presents to the clinic with complaints of a painful tongue covered with what look like creamy white, curdlike patches overlying erythematous mucosa. You are able to scrape off these “curds” with a tongue depressor, which assists you in making which of the following diagnoses? Which of the following patients would not be at risk of Candida infection? 1. Which of the following has/have not been linked to the use of isotretinoin? Sophie brings in her husband, Nathan, age 72, who is in a wheelchair. On his sacral area, he has a deep crater with full-thickness skin loss. Subcutaneous tissue is visible but muscle and bone are not. Which pressure ulcer stage is this? A Gram stain of a lesion reveals large, square-ended, gram-positive rods that grow easily on blood agar. Which diagnosis does this finding confirm? Question 6. Roy, age 13, was recently diagnosed with epilepsy and prescribed carbamazepine for control of his seizures. He has developed erythematous papules, dusky appearing vesicles, purpura, and target lesions that have erupted rapidly and are more centrally distributed on the face. He has hemorrhagic crusts on his lips. He tells you his skin feels tender and burns. Additionally, he has developed exudative conjunctivitis. These findings are indicative of: Question 8 Which disease usually starts on the cheeks and spreads to the arms and trunk? Erythema infectiosum (fifth disease). Jennifer, age 32, is pregnant and has genital warts (condylomata) and would like to have them treated. What should you order? Question 9 Louis, age 52, presents with pruritus with no rash present. He has hypertension, diabetes, and end-stage renal disease (ESRD). Which of the following would be included in the differential diagnosis? Question 10 Susan states that her fiancé has been frostbitten on the nose while skiing and is fearful that it will happen again. What do you tell her? Question 11 What is the most important thing a person can do to maintain healthy skin and hopefully reduce wrinkles? Question 12 Which of the following statements about malignant melanomas is true? Lee brings her 13-year-old son to your clinic. He has been complaining of a rash on the buttocks, anterior thighs, and posterolateral aspects of his upper arms. He tells you it is mildly pruritic and looks like “gooseflesh.” On examination, the rash appears as small, pinpoint, follicular papules on a mildly erythematous base. You explain to Lee that the benign condition is likely to resolve by the time her son reaches adulthood, and it is known as: Question 14. Jim, age 59, presents with recurrent, sharply circumscribed red papules and plaques with powdery white scale on the extensor aspects of his elbows and knees. What do you suspect? Question 15. Mildred, age 72, presents to the clinic with a blistering rash that is generalized but located mostly in skin folds and on flexural areas. She describes the course of the rash as beginning with pruritic urticarial papules that coalesced into plaques that turned dark red in about 2 weeks, followed by the development of vesicles and bullae. She tells you that the lesions are moderate to severely pruritic. During your exam, you determine the bullae are very tense and do not rupture when pressure is applied. Her daily medications include an angiotensin-converting enzyme (ACE) inhibitor, a loop diuretic, and a nonsteroidal anti-inflammatory drug (NSAID). What is your diagnosis? Question 16. Sandra, age 69, is complaining of dry skin. What do you advise her to do? Question 17. Caroline has a 13-year-old daughter who has had 2 recent infestations of lice. She asks you what she can do to prevent this. You respond: Question 18. Candidiasis may occur in many parts of the body. James, age 29, has it in the glans of his penis. What is your diagnosis? Question 19. Mr. Swanson, age 67, presents to the clinic for his annual health exam. He asks you if there is anything he can do to prevent the painful, blistering sores that develop on his lip in the summertime when he plays golf. You explain to Mr. Swanson that the way to prevent the development of these lesions is to: Question 20. A biopsy of a small, yellow-orange papulonodule on the eyelid will probably show: Question 1. A 75-year-old African American male presents to your family practice office complaining of visual impairment. He has worn corrective lenses for many years but has noticed that his vision has gotten progressively worse the past 6 months. He denies pain. He states his vision is worse in both eyes in the peripheral aspects of his visual field. He also notes trouble driving at night and halos around street lights at night. You test his intraocular pressure, and it is 23 mm Hg. What is his most likely diagnosis? Question 2. Which of the following is not a complication of untreated group A streptococcal pharyngitis? Question 3. The antibiotic of choice for recurrent acute otitis media (AOM) and/or treatment failure in children is: Question 4. Mattie, age 64, presents with blurred vision in 1 eye and states that it felt like “a curtain came down over my eye.” She doesn’t have any pain or redness. What do you suspect? Question 5. While doing a face, head, and neck examination on a 16-year-old patient, you note that the palpebral fissures are abnormally narrow. What are you examining? Question 6. Mia, a 27-year-old school teacher, has a 2-day history of severe left ear pain that began after 1 week of upper respiratory infection (URI) symptoms. On physical examination, you find that she has acute otitis media (AOM). She has a severe allergy to penicillin. The most appropriate antimicrobial option for this patient is: Question 7. Mario, a 17-year-old high school student, came to the office for evaluation. He is complaining of persistent sore throat, fever, and malaise not relieved by the penicillin therapy prescribed recently at the urgent care center. As the nurse practitioner, what would you order next? Question 8. Cynthia, a 31-year-old woman with a history of depression, is seen in the office today for complaints of headaches. She was recently promoted at her job, and this has caused increased stress. She describes the headache as a tightening (viselike) feeling in the temporal and nuchal areas. The pain is bilateral and tends to wax and wane. It started approximately 2 days ago and is still present. What kind of headache is she describing? Question 9. Ellen, a 56-year-old social worker, is seen by the nurse practitioner for complaints of fever; left-sided facial pain; moderate amounts of purulent, malodorous nasal discharge; and pain and headache when bending forward. The symptoms have been occurring for approximately 6 days. On physical assessment, there is marked redness and swelling of the nasal passages and tenderness/pain on palpation over the cheekbones. The nurse practitioner should suspect: Question 10. Sally, age 19, presents with pain and pressure over her cheeks and discolored nasal discharge. You cannot transilluminate the sinuses. You suspect which sinus to be affected? Question 11. A 3-year-old patient presents to your pediatric office with her mother. She has recently been started in day care. Her mother noted slight perioral erythema on the right side of the patient’s mouth prior to bed last night. The patient awoke today with 3 small, superficial, honey-colored vesicles where the erythema was last night. The patient has no surrounding erythema. She had no difficulty eating this morning and is active and energetic and doesn’t appear lethargic or fatigued. She is also afebrile. How would you treat this child? Question 12. Sandra, age 32, comes in to the clinic. She has painful joints and a distinctive rash in a butterfly distribution on her face. The rash has red papules and plaques with a fine scale. What do you suspect? Question 14. The nurse practitioner (NP) tells Samantha, age 52, that she has an acrochordon on her neck. What is the NP referring to? Question 17. Thomas, age 35, uses a high-potency corticosteroid cream for a dermatosis. He also currently has tinea corporis. You tell him the following regarding the cream: Question 19. A mother complains that her newborn infant, while lying on his side, appears red on the dependent side of the body and pale on the upper side. When she picks up the baby, this coloring disappears. You explain to her about which of the following? Question 20. Helen, age 39, comes to your clinic for generalized joint pain, especially in the knees and hands. She tells you that the pain is moderate, spreading, and symmetrical. She reports having had a sore throat and low-grade fever for a few days and then developing a rash, described as warm erythema of the cheeks, which lasted about 4 days before disappearing. You ask her if the rash on the cheeks included the nasolabial folds or circumoral skin, and she tells you that it did not cover these areas. Two days later, she developed a nonspecific macular eruption that preceded the joint pain. Her rash faded within 2 weeks, but she tells you that it comes and goes if she bathes in hot water or spends time in the sunlight. Your patient’s symptoms are most consistent with a diagnosis of: Question 1. Which of the following statements about macular degeneration is not true? Question 2. A 6-year-old female presents to your pediatric office with her mother complaining of right ear pain for 3 days. This pain resolved with Tylenol. The patient has also had noted fevers of 101.3°F over the last 2 nights. The patient had a nonproductive cough for 7 days prior to the ear pain. On physical exam, the patient has tenderness with tugging on the auricle of the ear. The tympanic membrane is not mobile with pneumatic otoscopy and is erythematous and full. The patient has no drainage from the ear and no mastoid tenderness. What is the next step? Question 3. What is the most common cause of epistaxis? Question 4. Mallory brings her 4-week-old infant to the office because she noticed small, yellow-white, glistening bumps on her infant’s gums. She says they look like teeth, but she is worried that they may be cancer. You diagnose these bumps as: Question 5. Jonathan, age 19, has just been given a diagnosis of mononucleosis. Which of the following statements is true? Question 6. Martin, age 24, presents to the office with an erythematous ear canal and pain on manipulation of the auricle. He is on vacation and has been swimming daily at the resort. What is your diagnosis? Question 7. Samantha, age 12, presents with ear pain. When you begin to assess her ear, you tug on her normal-appearing auricle, eliciting severe pain. This leads you to suspect: Question 8. Which manifestation is noted with carbon monoxide poisoning? Question 9. A 22-year-old African American female presents to your family practice office complaining of progressive skin discoloration. She is adopted and has no known family history of skin problems. The patient notes nonpalpable patches of skin loss and blanching of her forehead and both hands and feet. It has developed over a period of 6 months and appears to have stopped. It is not pruritic, and there is no erythema or sign of infectious etiology. What is the most likely diagnosis? Question 11. A 16-year-old male presents to your office. He was sent by an orthopedist. He has recently had surgical fixation of a humerus fracture. The patient has been going to physical therapy and has been developing a rash on his arm after therapy that disappears shortly after returning home. He does not have the rash prior to therapy. The patient denies fevers and chills, and his incision is well healed, with no signs of infection. Of note, the patient has been experiencing more hand edema than the average patient and has had edema wraps used at the end of therapy to help with his swelling. The wraps are made of a synthetic plastic material. The rash the patient gets is erythematous and blotchy, not raised; it is on the operative upper extremity. What is the most likely diagnosis? Question 13. Maryann, age 28, presents to the clinic because of a rapid onset of patchy hair loss. The skin within these oval patches of hair loss is very smooth. Tapered hairs that resemble exclamation points are seen at the margin of a patch of hair loss. Based on these findings, you suspect Maryann has: Question 14. Which treatment is considered the gold standard in tissue-conserving skin cancer removal? Question 15. You are examining Barbara, age 27, who presents with multiple dry, dusky red, well-localized plaques with a “stuck-on” appearance. They are 5 to 20 mm in diameter and located on her face, scalp, and external ears. You note there is atrophy, telangiectasia, depigmentation, and follicular plugging present. On examination of the scalp, there are areas of total hair loss. There is depigmented scarring of the concha of the ear. Your most likely diagnosis is: Question 16. Michael, a 25-year-old military reservist, presents to your clinic for a rash that began on his chest and has since developed into smaller lesions that are more concentrated on the lower abdomen and pubic area. In obtaining a history of the present illness, he reports that he had an upper respiratory infection 1 month before the rash developed. He tells you it started with 1 large oval-shaped lesion on his left chest, and 1 to 2 weeks later he developed numerous smaller lesions on the lower abdomen and groin. It has been 2 weeks since the smaller lesions developed, and he tells you he is concerned that the rash isn’t improving. As you examine the patient, you note that the lesions are salmon-colored and have a thin collarette of scale within them. The original lesion is still present. You suspect Michael has: Question 17. Shelby, age 14, has a blister on her arm that is filled with clear fluid. It is the result of contact with a hot iron. How do you document this? Question 18. Deanna, age 6, was bitten by a friend’s dog. Her mother asks you if the child needs antirabies treatment. You tell her: Question 19. Buddy, age 13, presents with annular lesions with scaly borders and central clearing on his trunk. What do you suspect? Question 20. Sandy asks what she can do for Dolores, her 90-year-old mother, who takes a bath every day and who has extremely dry skin. You respond: Question 6. Erica, age 39, has a sudden onset of shivering, sweating, headache, aching in the orbits, and general malaise and misery. Her temperature is 102°F. The nurse practitioner diagnosed her with influenza (flu). What is your next course of action? Question 7. You diagnose 46-year-old Mabel with viral conjunctivitis. Your treatment should include: Question 8. Which presentation is most concerning for skin cancer? Question 9. A 4-year-old male presents to your pediatric clinic with his mother complaining of an itchy rash, mostly between his fingers. This has been going on for multiple days and has been getting worse. The patient recently started at a new day care. On physical exam, the patient is afebrile and has multiple small (1-2 mm) red papules in sets of 3 located in the web spaces between his fingers. He also has signs of excoriation. What is the treatment for this problem? Question 10. Which of the following statements about psoriasis is not true? Question 11. Eric, age 52, has gout. What do you suggest? Question 12. Marge, age 36, is planning to go skiing with her fiancé. He has warned her about frostbite, and she is wondering what to do if frostbite should occur. You know she’s misunderstood the directions when she tells you which of the following? Question 13. Which disease usually starts on the cheeks and spreads to the arms and trunk? Question 15. A dark-field microscopic examination is used to diagnose: Question 16. Which of the following should be used with all acne medications? Question 18. In a burn trauma, which blood measurement rises as a secondary result of hemoconcentration when fluid shifts from the intravascular compartment? Question 2. Alexandra, age 34, was treated with oral antibiotics 2 weeks ago for a urinary tract infection. She is seen in the office today for a follow-up visit. On physical examination, the nurse practitioner notices that she has some painless, white, slightly raised patches in her mouth. This is probably caused by: Question 4. Aaron, age 4, is brought in to the clinic by his father. His tympanic membrane is perforated from otitis media. His father asks about repair of the eardrum. How do you respond? Question 5. Sharon, a 47-year-old bank teller, is seen by the nurse practitioner in the office for a red eye. You are trying to decide between a diagnosis of conjunctivitis and iritis. One distinguishing characteristic between the two is: Question 7. A 25-year-old client who plays in a band complains that he finds it difficult to understand his fellow musicians at the end of a night of performing, a problem that is compounded by the noisy environment of the club. These symptoms are most characteristic of which of the following? Question 8. Marcia, age 4, is brought in to the office by her mother. She has a sore throat, difficulty swallowing, copious oral secretions, respiratory difficulty, stridor, and a temperature of 102°F but no pharyngeal erythema or cough. What do you suspect? Question 9. An 80-year-old woman comes in to the office with complaints of a rash on the left side of her face that is blistered and painful and accompanied by left-sided eye pain. The rash broke out 2 days ago, and she remembers being very tired and feeling feverish for a week before the rash appeared. On examination, the rash follows the trigeminal nerve on the left, and she has some scleral injection and tearing. You suspect herpes zoster ophthalmicus. Based on what you know to be complications of this disease, you explain to her that she needs: Question 10. Regular ocular pressure testing is indicated for older adults taking: Question 14. Client teaching is an integral part of successfully treating pediculosis. Which of the following statements would you incorporate into your teaching plan? Question 15. A 70-year-old client with herpes zoster has a vesicle on the tip of the nose. This may indicate: Question 16. Which skin cancer that arises from skin cells, characteristically occurs on body areas exposed to the sun, most commonly presents as a pearly nodule with fine telangiectasias over the surface and a border that appears rolled, and is the most common skin cancer? Question 17. Ashley, age 6 months, has a Candida infection in the diaper area. What do you suggest to the parent? “Keep the area as dry as possible. Question 18. Danny, age 18, presents with a pruritic rash on his upper trunk and shoulders. You observe flat to slightly elevated brown papules and plaques that scale when they are rubbed. You also note areas of hypopigmentation. What is your initial diagnosis? Question 19. Elizabeth, age 83, presents with a 2-day history of pain and burning in the left forehead. This morning she noticed a rash with erythematous papules at that site. What do you suspect? Question 1. A 10-year-old male in 5th grade presents to the pediatric office with his mother complaining of itchy and red eyes for 1 day. The patient complains of watery drainage in both eyes, associated with repetitive itching. On physical exam, he has no fever or constitutional symptoms. His vision is normal, with no decrease in extraocular movements. The patient has a sibling that just started day care recently. He also has bilateral preauricular lymph nodes that are inflamed. What is the patient’s diagnosis? Question 2. A 65-year-old man presents complaining of a left-sided, deep, throbbing headache and mild fatigue. On examination, the client has a tender, tortuous temporal artery. You suspect giant cell arteritis (GCA), or temporal arteritis. What is the least invasive procedure to help with diagnosis? Question 4. Mandy, 44, was given a diagnosis of flu 1 day ago and wants to start on the “new flu medicine” right away. What do you tell her? Question 5. Mr. Clark, age 78, is being treated with timolol maleate (Timoptic) drops for his chronic open-angle glaucoma. While performing a new client history and physical, you note that he is taking other medications. Which medication would you be most concerned about? Question 7. How would you describe the cervical lymphadenopathy associated with asymptomatic human immunodeficiency virus (HIV) infection? Question 8. A 22-year-old college student presents to your urgent care clinic complaining of a rash. She was recently on spring break and spent every night in the hot tub at her hotel. On physical exam, she has multiple small areas of 1- to 2-mm erythematous pustules that are present mostly where her bathing suit covered her buttocks. What is the most likely pathogen causing these lesions? Question 9. Which human papillomavirus serotypes most commonly cause cancer? Question 10. An eczematous skin reaction may result from: Question 11. When palpating the skin over the clavicle of Norman, age 84, you notice tenting, which is: Question 15. You are teaching Harvey, age 55, about the warts on his hands. What is included in your teaching? Question 16. Jill, age 29, has numerous transient lesions that come and go, and she is diagnosed with urticaria. What do you order? Question 19. Dan, age 57, has just been given a diagnosis of herpes zoster. He asks you about exposure to others. You tell him: Question 20. Martin, age 13, just started taking amoxicillin for otitis media. His mother said that he woke up this morning with a rash on his trunk. What is your first action? . Question 1. Which of the following is not a modifiable risk factor? Question 2. Which of the following is not a subjective finding? Question 3. What intervention would not be included in the definition of secondary health promotion? Question 4. Which of the following refers to an aspect of a patient’s health that can be changed or affected by a health intervention? Question 5. Which of the following refers to an aspect of a patient’s health that cannot be changed or affected by a health intervention? Question 6. Which of the following patients is health literate? Question 7. All of the following statements about the US Preventive Services Task Force (USPSTF) are true except? Question 8. Between ages 7 and 18, both boys and girls are immunized against the following diseases: Question 9. Telehealth has shown a drastic increase in utilization by patients in which of the following fields of medicine? Question 10. Eileen, a 42-year-old woman, comes to your office with a chief complaint of fatigue, weight loss, and blurred vision. Eileen has a past medical history that is negative for any chronic medical problems. You obtain a fasting chemistry panel, lipid profile, complete blood count (CBC), and hemoglobin A1c (HbA1c). The results of the blood work show Eileen’s blood sugar elevated at 356 mg/dL, total cholesterol elevated at 255, high-density lipoprotein (HDL) cholesterol low at 28, low-density lipoprotein (LDL) cholesterol elevated at 167, triglycerides 333, and HbA1c 12. On questioning Eileen further, you discover that both her grandmothers had adult-onset diabetes mellitus. You diagnose type 2 diabetes mellitus. Your treatment plan should include a cholesterol-lowering agent, an agent that lowers blood sugar, and which other class of medication? Question 11. A lab value that is commonly decreased in older adults is: Question 12. Dennis, age 62, has benign prostatic hyperplasia (BPH). He tells you that he voids at least 4 times per night and that he has read about a preventive drug called terazosin hydrochloride (Hytrin) that might help him. What do you tell him? Question 13. You are sharing with your client the idea that he needs to get some counseling to deal with his severe stress because it is affecting his physiological condition. Which of the following hormonal changes occurs during severe stress? . Question 14. Margaret, age 29, is of medium build and 5 ft 4 in tall. You estimate that she should weigh about: Question 15. Martha, age 82, has an asymptomatic carotid bruit on the left side. What do you recommend? Question 16. When performing a sports physical exam on Kevin, a healthy 16-year-old boy, which question in the history is important to ask Kevin or his guardian? Question 17. If a screening test used on 100 individuals known to be free of breast cancer identified 80 individuals who did not have breast cancer while missing 20 of the individuals, the specificity would be: Question 18. Harvey, age 55, comes to the office with a blood pressure (BP) of 144/96 mm Hg. He states that he did not know if it was ever elevated before. When you retake his blood pressure at the end of the examination, it remains 144/96. What should your next action be? Question 19. Mimi, age 52, asks why she should perform a monthly breast self-examination (BSE) when she has her mammograms on schedule. You respond: Question 20. Which of the following individuals should get the shingles (herpes zoster) vaccine? Question 3. As a primary care provider, which of the following topics is not typically important for adults aged 20 to 40? Question 4. As a nurse practitioner, which of the following would not be an example of primary health promotion? Question 5. Which of the following initiatives does not fall under the National Prevention Strategy? Question 7. The OLD CARTS (onset, location, duration, character, aggravating/alleviating factors, radiation, timing, severity) mnemonic is best used in which part of your chart note? Question 8. A nurse practitioner is practicing based on the rule-based actions he was taught in school and doesn’t understand when/where those rules may be inappropriate. Which following statement best describes this skill level? Question 9. Mildred, an independent 92-year-old woman, is moving into her daughter’s home. Her daughter comes to see you seeking information to help keep her mother from falling. Which of the following interventions would you suggest she do to help prevent Mildred from falling? Question 10. Joseph, a 55-year-old man with diabetes, is at your office for his diabetes follow-up. On examining his feet with monofilament, you discover that he has developed decreased sensation in both feet. There are no open areas or signs of infection on his feet. What health teaching should Joseph receive today regarding the care of his feet? Question 11. Sandra, a 27-year-old nurse, states that she does not want to get the hepatitis B virus vaccine because of its adverse effects. You tell her that the most common adverse effect is: Question 12. Harry is taking his entire family to Central America and is wondering about protection against bites from malaria-causing mosquitoes. What advice do you give him? Question 14. Susie, age 5, comes to the clinic for a well-child visit. She has not been in since she was 2. Her immunizations are up to date. What immunizations would you give her today? Question 15. Mary, a 70-year-old woman with diabetes, is at your office for her 3-month diabetic checkup. Mary’s list of medications includes metformin (Glucophage XR) 1000 mg daily, an angiotensin-converting enzyme (ACE) inhibitor daily, and 1 baby aspirin (ASA) daily. Mary’s blood work showed a fasting blood sugar (FBS) of 112 and glycosylated hemoglobin (HbA1c) of 6.5. You tell Mary that her blood work shows: Question 16. Sandy, a 68-year-old woman, presents to your office for screening for osteoporosis. She states that her grandmother and mother both lost inches in their old age. She has been postmenopausal for the past 15 years and never took any hormone replacement medications. She is Caucasian, weighs 108 lb, and is 5 ft 1 in tall on today’s measurement. When do women lose the greatest amount of bone density? Question 18. Julia, age 18, asks you how many calories of fat she is eating when 1 serving has 3 g of fat. You tell her: Question 19. Marian’s husband, Stu, age 72, has temporal arteritis. She tells you that his physician wants to perform a biopsy of the temporal artery. She asks if there is a less invasive diagnostic test. What test do you tell her is less invasive? Question 20. For which patient would you administer the human papillomavirus (HPV) vaccination? Question 1. Which of the following demonstrates a subjective finding? Question 3. Which of the following statements about health promotion is false? Question 4. Which of the following statements defines health literacy? Question 5. The study of the way diseases are spread through groups and what causes and helps spread these diseases is called? Question 6. Which of the following statements does not belong in the past medical history portion of your chart note? Question 7. An 81-year-old patient presents for a physical. She recently had a fall and now has problems walking up her stairs. The only restroom in the house is on the second floor. She also has a flight of stairs outside her house she has to navigate in order to reach street level, and this is difficult for her. Where does this information belong in your chart note? Question 10. Mark, a 56-year-old man, comes to your practice seeking help quitting smoking. You prescribe varenicline (Chantix), a prescription medication, to aid with his attempt. What instructions do you give Mark regarding how to stop smoking with Chantix? Question 11. How do you respond when Jill, age 42, asks you how long she should work out each week? Question 12. Marvin is a gay man who is ready to “come out.” What is the last step in the process of coming out? Question 16. Jan’s mother has Alzheimer disease (AD). Jan tells you that her mother’s recent memory is poor and that she is easily disoriented, incorrectly identifies people, and is lethargic. Jan asks you, “Is this as bad as it gets?” You tell her that her mother is in which stage of the disease? Question 17. Herbert, a 69-year-old man, comes to your office complaining of nocturia. On questioning Herbert, you find that for the past 3 months he has been getting up at least 5 times a night to void. He came in to seek help today because of his wife’s insistence that he be checked out. When you perform the digital rectal exam, you find that his prostate protrudes 3 to 4 cm into the rectum. What grade would you assign to Herbert’s prostate enlargement? [Show More] Last updated: 1 year ago Preview 1 out of 110 pages NURSING> STUDY GUIDE > NR341 Exam 1 Study Guide-comprehensive (All) Chapter 1: Overview of Critical Care Nursing ▪ Describe the importance of professional organizations & characteristics of the AACN & SCCM o American Association of Critical-Care Nursing (AACN) ▪ Mi... By Studyrepository , Uploaded: Apr 27, 2022 NURSING> STUDY GUIDE > NR 507 week7 quiz study guide (All) NR 507 week7 quiz study guide By ExpertAnswer , Uploaded: Apr 20, 2021 NURSING> STUDY GUIDE > NURS-611 Patho Exam 3 Review Study Guide ( Maryville University) (All) NURS-611 Patho Exam 3 Review Study Guide ( Maryville University) By Study Hacks , Uploaded: Jun 06, 2021 NURSING> STUDY GUIDE > NR 228-Nutrition Final exam -study guide (All) CHAPTER 1 (2 left to answer) How many calories are provided by protein, carbohydrates, fat and alcohol? Protein/Carbs: 4 kcal/gram Fat: 9 kcal/gram Alcohol: 7 kcal/gram What does “nutrient densit... By Studyrepository , Uploaded: Mar 22, 2022 NURSING> STUDY GUIDE > NR 512 Week 1 Pre-TANIC TIGER Assessment plus Discussion Topic-Chamberlain College Of Nursing:GRADED A (All) NR 512 Week 1 Pre-TANIC TIGER Assessment plus Discussion Topic-Chamberlain College Of Nursing:GRADED A By Succeed , Uploaded: May 17, 2020 NURSING> STUDY GUIDE > Math Misc: Dosage Calculations for Nursing Students B Wojcik (All) Math Misc: Dosage Calculations for Nursing Students B Wojcik By quizprof , Uploaded: Jun 12, 2021 NURSING> STUDY GUIDE > NASM CPT 4 COMPLETE Study Guide GREAT PIECE FOR EXCELLENT STUDENT! (All) NASM CPT 4 COMPLETE Study Guide GREAT PIECE FOR EXCELLENT STUDENT! NASM CPT 4 COMPLETE Study Guide GREAT PIECE FOR EXCELLENT STUDENT! NASM CPT 4 COMPLETE Study Guide GREAT PIECE FOR EXCELLENT STUDEN... By GradesProff , Uploaded: Jun 25, 2023 NURSING> STUDY GUIDE > ATI Leadership Proctored Remediation (New, 2020)\|100% Correct, SATISFACTION GUARANTEED (All) ATI Leadership Proctored Remediation (New, 2020)\|100% Correct, SATISFACTION GUARANTEED By Succeed , Uploaded: Jul 05, 2020 NURSING> STUDY GUIDE > NR 533 Financial Management In Healthcare Organizations Week 4 Staffing Budgets FTEs Variance Analysis Assignment Guidelines with Scoring Rubric 3 Graded A (All) NR 533 Financial Management In Healthcare Organizations Week 4 Staffing Budgets FTEs Variance Analysis Assignment Guidelines with Scoring Rubric 3 Graded A By TopScholar , Uploaded: Mar 15, 2021 NURSING> STUDY GUIDE > Study Guide for Health Assessment Exam 1 (All) Study Guide for Health Assessment Exam 1 Ch. 1-7 Concepts to know CH. 1 Evidence-Based Practice Types of Data • Objective – visibly able to measure on a patient o Vital signs, (BP, Pulse) Re... 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Prostate cancer occurs when a group is found abnormal cells in the prostate. First, the cells will be limited to the gland, but may eventually spread or metastasize to other areas. There is a better prognosis if the cancer is detected early while still confined to the prostate. Prostate cancer is coded according to the International Classification of Disease (ICD). The current version is ICD-9. ICD-10 comes into effect in the USA October 2013, whilst ICD-11 is in progress and will be completed in 2015. ICD was first adopted in France back in 1893 and the ICD-9 was adopted in 1979 by the World Health Association. The codes are used by medical coders to classify diseases into categories. However, physicians will use alternative databases such as SNOMED CT (Systematized Nomenclature of Medicine Clinical Terms) developed by the IHTSDO, which includes 18 countries. Prostate Cancer ICD-9 Code (and ICD-10) For some aspects of prostate disease there is a direct match between ICD-9 and ICD-10. For instance, the code for primary prostate cancer in ICD-9 is 185, while the ICD-10 code is C61. Prostate intraepithelial neoplasia (PIN) is a pre-cancerous stage also included in the ICD classification. Stages 1 and 2 are classed as ICD-9 code 602.3, while stage 3 is ICD-9 code 233.4 for carcinoma in situ (early, local cancer) of the prostate. Treatments are also recorded in the ICD classification. For instance, prostatectomy surgery is 60.21 to 60.69, chemotherapy is 99.25, external beam radiotherapy is 92.29, hormonal therapy is 99.24 and brachytherapy is 92.27. ICD-9 codes for malignant prostate cancer outside the prostate include bony metastases (198.5), bladder or urethral metastases (198.1), pelvic lymph nodes (196.6) and seminal vesicles (198.82). Incoming search terms: - icd 9 code for prostate cancer - icd 9 code prostate cancer - diagnosis code for prostate cancer - icd code for prostate cancer - icd 9 prostate cancer - ICD9 PROSTATE CANCER - prostate cancer icd code - Diagnosis Code Prostate Cancer - icd9 code for prostate cancer	4	5	0	0	0	2	0.394405	2	526
I'm a provider You will be redirected to myBlue. Would you like to continue? Please wait while you are redirected. DESCRIPTIONImmune globulin is an antibody-containing solution obtained from the pooled plasma of healthy blood donors. There are several different commercial preparations available. Intravenous immune globulin (IVIg) therapy is used to provide antibodies to patients who are susceptible to disease for which there is no immunization. Prior authorization is required. Primary immune deficiency syndromes, including combined immunodeficiencies. Acute Humoral Rejection Autoimmune Mucocutaneous Blistering Diseases, in patients with severe, progressive disease despite treatment with conventional agents (corticosteroids, azathioprine, cyclophosphamide, etc.) Autoimmune and inflammatory disorders * FDA-labeled indications IVIg is considered not medically necessary as a treatment of relapsing/remitting multiple sclerosis. Intravenous immune globulin therapy is considered investigational, including, but not limited to, the following conditions: Subcutaneous Immune Globulin (SCIg) Therapy SCIg may be considered medically necessary for the treatment of primary immunodeficiencies (FDA-labeled indications) including congenital agammaglobulinemia, hypogammaglobulinemia, common variable immunodeficiency (CVID), severe combined immunodeficiency, Wiskott-Aldrich syndrome, and X-linked agammaglobulinemia (XLA). POLICY GUIDELINESPrimary Immunodeficiency Syndromes. The diagnosis of immunodeficiency and post immunization titers must be taken in context with the clinical presentation of the patient, and may vary dependent on the type of vaccine given and the prior immunization history of the patient. The following parameters are examples of criteria for diagnosis of the primary immunodeficiency syndromes. According to a 2010 national guideline from Canada on immune globulin for primary immune deficiency, although higher trough levels of IVIg may be associated with clinical response; the goal of IVIg dose increases should be to improve clinical effectiveness and not merely to increase trough levels. Acute, severe ITP may be defined by the following parameters: Patients with chronic inflammatory demyelinating neuropathy (CIDP) should meet the diagnostic criteria established by the American Academy of Neurology, particularly if the patient also is diagnosed with chronic fatigue syndrome. (See Appendix A for the diagnostic criteria.) In addition, by intravenous immunoglobulin infusion (IVIg), treatment should be limited to CIDP patients who do not respond to initial therapy with prednisone and are experiencing serious clinical worsening. In patients treated for chronic diseases, such as CIDP, multifocal motor neuropathy, and dermatomyositis, the effect of IVIg is transitory and therefore periodic infusions of IVIg are needed to maintain treatment effect. The frequency of transfusions is titrated to the treatment response; typically, biweekly or monthly infusions are needed. Investigative service is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized by certifying boards and/or approving or licensing agencies or published peer review criteria as standard, effective medical practice for the treatment of the condition being treated and as such therefore is not considered medically necessary. The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language. POLICY HISTORY8/1997: Approved by Medical Policy Advisory Committee (MPAC) 8/1999: Revisions approved by MPAC 11/1999: Revisions approved by Pharmacy & Therapeutics (P & T) Committee 5/2001: Reviewed by MPAC; IgIV considered medically necessary for CIDP 1/30/2002: Venoglobulin I® deleted from ITP; Venoglobulin S®, Gammagond SD, Polygam SD added to Kawasaki syndrome 2/15/2002: Investigational definition added 5/1/2002: Type of Service and Place of Service deleted 5/29/2002: Code Reference section updated 11/6/2002: Prior authorization required 3/25/2003: Policy section revised, "CLL with frequent infections" deleted, CPT code range 90281-90399 deleted 7/2003: Reviewed by MPAC, Policy section aligned with BCBSA, treatment dosage information deleted, ICD-9 diagnosis code ranges 356.0-356.9, 493.00-493.91, 203.00-203.81, 714.0-714.9 listed separately, ICD-9 diagnosis code 695.4 deleted covered codes, ICD-9 diagnosis code 203.00-203.81, 288.0, 493.00-493.91 moved to non-covered codes, ICD-9 diagnosis code 493.92 added non-covered codes 10/13/2004: Code Reference section updated, CPT code 90780, 90781 deleted covered codes, ICD-9 diagnosis code 279.00, 279.04, 279.05, 279.12, 279.2, 357.82, 358.01, 776.1, V42.0, V42.1, V42.6, V42.7, V42.83 added covered codes, ICD-9 diagnosis code 357.0, 446.1 description revised, ICD-9 diagnosis 358.0 5th digit added, HCPCS J0850 added covered codes, HCPCS J1561 effective deletion date added, HCPCS J1562 deleted, non-covered ICD-9 diagnosis code 203.00-203.81, 288.0, 493.00-493.91, 493.92, 710.0, 714.0-714.9 deleted 11/11/05: Policy description revised: Intravenous immune globulin (IgIV) changed to (IVIg). Policy section language revised: Preferred provider changed to Accredo; telephone # changed from 1-866-591-9075 to 1-866-240-3373; fax # changed from 1-866-591-9094 to 1-800-711-3526. Policy section description revised for intravenous immune globulin therapy diagnoses related to medical necessity. Added paragraph for intravenous immune globulin therapy. Deleted under intravenous immune gloublin therapy: agammaglobulinemia - primary humoral, hypogammaglobulinemia - primary humoral immundeficiency, bacterial infections associated with B-Cell chronic lymphocytic leukemia (CLL), HIV-ITP with severe bleeding using pooled nonspecific IVIG preparations, prevention of infection in HIV-infected children(Gamimune N® only), solid organ transplant recipients at risk for cytomegalovirus infections and pneumonia (Cytogam® only). Vital capacity less than 1L, dysphagia associated with aspiration and inability to ambulate 100 feet without assistance deleted from definition of myasthenia gravis. Deleted hereditary and idiopathic peripheral neuropathy, bone marrow transplant patients; and relapsing/remitting multiple sclerosis. Policy section description revised for polymyositis under the intravenous immune globulin therapy section related to investigational diagnoses; added chronic progressive multiple sclerosis. Code Reference section updated: 5th digit added to ICD9 diagnosis code 287.31, description revised; HCPCS codes J1564, Q9941-Q9944 added, J1561 deleted. 11/2005: Approved by Pharmacy & Therapeutics (P & T) Committee 3/14/2006: Coding updated. HCPCS 2006 revisions added to policy 3/20/2006: Policy reviewed, no changes 7/27/2006: Policy revised. Revisions approved by Medical Policy Advisory Committee (MPAC) 6/14/2007: Code Reference section updated per quarterly HCPCS and Category III revisions 12/19/2007: Coding updated per 2008 CPT/HCPCS revisions 1/18/2008: Policy revised to add "Policy" section on SCIg and maternal-fetal tolerance 1/21/2008: Code reference section revised; CPT 90284 and HCPCS J1562 added to covered codes. Added non-covered codes table; CPT 86021, 86355, 86357, 86360, 86361, and 86849 added to non-covered codes for techniques to investigate immunologic abnormalities affecting maternal-fetal tolerance. Policy named changed from "Intravenous Immune Globulin (IVIg)" to "Immune Globulin Replacement Therapy". 9/22/2008: Annual ICD-9 updates effective 10-1-2008 applied 1/1/2009:Accredo preferred provider information removed. BCBSMS information added. 1/6/2009: Policy reviewed, the following specifically listed under investigational: 4/23/2009: ICD-9 code 340 deleted from covered table 10/11/2010: Updated the Code Reference section to remove 86360 and 86361 from the non-covered codes table. 02/28/1011: Added new HCPCS codes J1559 and J1599 to the Code Reference section. 01/17/2012: Policy statement reformatted for clarity purposes. Add the following as medically necessary indications: Acute Humoral Rejection, Autoimmune Mucocutaneous Blistering Diseases, warm antibody autoimmune hemolytic anemia, refractory to corticosteroids and immunosuppressive agents, anti-phospholipid syndrome, and toxic shock syndrome. Added the following indications to the investigational policy statement: complex regional pain syndrome, Alzheimer’s disease, IGG sub-class deficiency, and sepsis. Deleted the statement regarding laboratory tests to investigate immunologic abnormalities affecting maternal-fetal tolerance. Policy guidelines updated regarding laboratory testing. Deleted the Non-Covered codes table. Deleted outdated references from the Sources section. 04/09/2013: Policy reviewed. Added neonatal sepsis and Crohn's disease as investigational indications. Removed deleted codes J1567 and Q4087 - Q4092 from the Code Reference section. 11/15/2013: Policy statement updated to add severe anemia due to parvovirus B19 as medically necessary. Added opsoclonus-myoclonus, birdshot retinopathy, epidermolysis bullosa acquisita, necrotizing fasciitis, and polyradiculoneuropathy (other than CIDP) as investigational indications. SOURCE(S)Blue Cross Blue Shield Association Policy # 8.01.05 CODE REFERENCEThis may not be a comprehensive list of procedure codes applicable to this policy. The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.	4	17	8	0	9	3	0.923848	20	2,353
Every woman is at risk for developing breast cancer, and the risk increases with age. According to the Centers for Disease Control and Prevention (CDC), approximately 94 percent of breast cancers are diagnosed in women over 40. Mammography is the best available way to detect breast cancer early, when it is most curable. A mammography is a low-dose X-ray of the breast that can find lumps too small to be felt during a breast examination. The breast is compressed firmly between two planes and pictures are taken. A screening mammogram is used to detect breast changes in women who have no signs of breast cancer. When the patient has signs or symptoms of a suspected disease, a diagnostic mammography is performed instead. It is essential coders understand the ICD-9-CM diagnosis, CPT and HCPCS coding guidelines for accurate code assignment for mammography. The diagnoses codes used to report encounters for a screening mammography are: • V76.11, Special screening for malignant neoplasm, screening mammogram for high-risk patients • V76.12, Special screening for malignant neoplasm, other screening mammogram The following codes may be assigned along with code V76.11 to identify why the patient is considered to be at high-risk. • V10.3, Personal history of malignant neoplasm, breast • V16.3, Family history of malignant neoplasm, breast • V15.89, Other specified personal history presenting hazards to health, other For Medicare patients, diagnosis codes V76.11 and V76.12 must be reported as the first listed diagnosis when a claim contains only screening mammography services. When billing a screening mammogram with other services billed on the same claim, codes V76.11 or V76.12 may be listed as a secondary diagnosis. If a condition is found during the screening, the code for the condition may be used as an additional diagnosis. The rationale for this is even though a condition is found during the mammography, the visit is still considered a screening. A screening mammogram is inherently bilateral and is reported with the following procedure codes: • 77057, Screening mammography, bilateral (two view film study of each breast) • G0202, Screening mammography, producing direct digital image, bilateral, all views Code 77052, Computer aided detection, screening mammography, may be assigned when it is performed in addition to the primary procedure. Computer aided detection (CAD) indicates a laser beam was used to scan the mammography film and the image was then converted to digital data for computer analysis. As mentioned earlier, if a condition is discovered during the screening, the code for the condition may be used as an additional diagnosis. In this instance, if the radiologist orders additional films based on the condition discovered during the screening mammogram, both procedures may be coded. When this occurs, append modifier GG, Performance and payment of a screening mammography and diagnostic mammography on same patient, same day to the diagnostic mammography code. Modifier GG indicates the test changed from a screening test to a diagnostic test. If not performed on both breasts, it is also important to append the appropriate anatomic modifier, RT or LT, to indicate on which side the diagnostic mammogram was performed. A diagnostic mammography is performed when the patient has a complaint, such as a lump, or to investigate an abnormality found during a screening mammogram. For diagnostic mammograms, report the confirmed or definitive diagnosis that is documented in the medical record. It is not necessary to code related signs or symptoms in addition to the confirmed diagnosis. If the final interpretation is not available when the case is coded, or if there is no definitive diagnosis, report the signs or symptoms that prompted the mammography. Diagnoses typically assigned with a diagnostic mammography include but are not limited to: • Lump or mass in breast (611.72) • Pain in breast (611.71) • Nipple discharge (611.79) • Specified anomalies of breast (757.6) • Neoplasms of breast, both male and female (174.0-175.9, 198.81, 217, 233.0, 238.3, 239.3) The assignment of procedure codes is dependent on the services provided and the technologies used to perform the mammography. The following codes are used to report a diagnostic mammography: • 77055, Mammography; unilateral • 77056, Mammography; bilateral • G0204, Diagnostic mammography, producing direct digital image, bilateral, all views • G0206, Diagnostic mammography, producing direct digital image, unilateral, all views Code assignment will be determined by whether or not direct digital images were taken and if the procedure was bilateral or unilateral. A diagnostic mammography without a direct digital image is coded to either 77055 or 77056, depending on the laterality of the procedure. A diagnostic mammography generating a direct digital image is coded to either G0204 or G0206, depending on the laterality of the procedure. Add-on code 77051, Computer-aided detection, with further physician review for interpretation, with or without digitization of film images; diagnostic mammography, may be assigned in additional to codes 77055, 77056, G0204, or G0206 when performed in addition to the primary procedure. CAD uses computer software to review the mammography after the radiologist has interpreted it. If the software identifies any abnormalities, it marks them for further radiologist review. Test your knowledge with the following quiz: 1. An asymptomatic 65-year-old woman had a screening mammogram, which revealed a breast mass. The physician scheduled the patient for follow-up biopsy of the breast mass at a later date. How should the diagnoses and procedures for this case be coded? a. V76.12, 611.72, 77057 b. 611.72, 77057 c. V76.12, 77057 d. V76.11 611.72, 77056 2. An asymptomatic 70-year-old woman with a family history of breast cancer has a screening mammogram that reveals a right breast mass. As a result of the positive finding, a diagnostic mammogram of the right breast with direct digital image is then performed. How should the diagnoses and procedures for this case be coded? a. V76.11, 611.72, G0206GGRT b. 611.72, V76.11, V16.3, 77057, G0206GGRT c. 611.72, V16.3, 77057, 77055GGRT, G0206 d. V7611, 611.72, V16.3, 77057, G0206GGRT This month's column has been prepared by Cheryl D'Amato, RHIT, CCS, director of HIM, facility solutions, Ingenix, and Melinda Stegman, MBA, CCS, clinical technical editor, Ingenix (www.ingenix.com). CPT is a registered trademark of the American Medical Association. 1. a. Assign diagnosis code V76.12 to indicate the screening for malignant neoplasm of breast and code 611.72, Lump or mass in breast, as an additional diagnosis to identify the breast mass. Procedure code 77057 is assigned to for the screening mammography. 2. d. Assign diagnosis code V76.11 to indicate that the breast cancer screening is being performed on a high risk patient. Secondary diagnosis codes V16.3, family history of breast cancer and 611.72, lump or mass in breast are also assigned. Procedure code 77057 is reported for the screening mammogram. Procedure code G0206 is assigned for the unilateral diagnostic mammogram producing direct digital image. Modifiers GG and RT are appended to code G0206 indicating that the screening mammogram was converted to a diagnostic mammogram of the right breast.	4	6	9	0	8	10	0.719388	27	1,690
Home > Preview The flashcards below were created by user on FreezingBlue Flashcards. What is the pathogenesis of squamous cell carcinoma of the skin? - 1. UVB causes mutation in basal cells. - 2. Proliferation of abnormal basal cells causes actinic keratosis. - 3. Further mutations occur and lead to full thickness dysplasia – see in situ. - 4. SCC in situ develops the ability to invade the dermis due to an accumulation of further mutations. - 5. In immune suppressed people HPV plays a role; and there are some inherited diseases with an increase in SCC (albinism, xeroderma). What is the pathogenesis of SCC of the lung? - 1. Inhalation of smoke causes squamous metaplasia. - 2. Exposure to carcinogens in the smoke causes squamous metaplasia to become dysplastic. This is through accumulation of mutations. - 3. The dysplasia progresses to squamous cell carcinoma in situ as the dysplastic cells fill up the entire thickness of the epithelium. - 4. By now the genomics is unstable and more mutations mean the cells acquire the ability to invade through the basement membrane. - 5. The squamous cell carcinoma expands and invades the bronchus, lung parenchyma, and blood vessels. Metastasis occurs. What is the pathogenesis sporadic of colonic carcinoma? - 1. There is an initiating mutation of the APC gene. - 2. A tubular adenoma forms. More mutations occur, particularly in KRAS. - 3. The tubular adenoma enlarges and becomes more dysplastic. More mutations occur. - 4. A key mutation in P53 means that the tumour progresses to high-grade dysplasia/carcinoma in situ. - 5. Accumulation of more mutations results and the ability to invade through the basement membrane and become invasive adenocarcinoma. What is the pathogenesis of colonic carcinoma in Lynch syndrome? - 1. The patient inherits an abnormality of one of the genes used for DNA mismatch repair. - 2. The matching gene on the opposite chromosome is lost due to a sporadic mutation. (Two hit hypothesis) - - autosomal dominant - 3. DNA repair abnormalities mean that mutations accumulate. A mutation occurs in the APC gene. - 4. The APC mutation means that tubulous adenoma’s form. These progress through increasing levels of dysplasia as more mutations occur. - 5. High-grade dysplasia develops, which then acquires the ability, through further mutations, to invade through the basement membrane and into the bowel wall. What is the pathogenesis of urothelial carcinoma of the urinary bladder? - 1. Carcinogens such as those found in cigarette smoke or aromatic amines cause mutations in the urothelial cells. This causes them to become dysplastic. - 2. The dysplastic cells proliferate and gain more mutations. - 3. The proliferation of dysplastic cells can result in either flat atypia or by forming papillary proliferations. - 4. When the papillary proliferations grow they can accumulate more mutations and become more dysplastic. This forms urothelial papillary carcinoma, this is an insitu proliferation. - 5. With more mutations the cells gain the ability to invade through the basement membrane and an invasive urothelial carcinoma. What will determine the outcome of a patient with bladder cancer? - Pass must mention stage and grade - Whether the tumour is invasive or in situ (1 mark) - • Depth of invasion - • Extension outside the bladder - • Vascular invasion - • lymph node invasion extension (1 mark for 2 of these things) - High grade has a worse prognosis What are the risk factors of bladder cancer? - The most important risk factor is cigarette smoking. - Industrial carcinogens, such as naphthylamine and benzidine, play a role among the workers in dye, rubber, plastic, and insulation industries. - Schistosoma haematobium infection in Egypt is associated with an increased incidence of urinary bladder cancer. What is the pathogenesis of Pagets disease of the bone? - 1. The exact initial cause of Paget’s disease is unknown, some people think it might be by rest and others think it might be due to a mutation in the genes such as RANK. - 2. The initial activity is increased osteoclastic activity. There is much bone resorption increases in the rate of bone resorption. - 3. There is a next phase of osteoclastic, osteoblastic activity. The osteoblasts compensate by an accelerated deposition of lamellar bone. This produces a characteristic mosaic pattern. - 4. Osteoblastic activity. The osteoblastic activity predominates. The marrow spaces are filled with blood vessels causing the bone to become hypervascular. The bone become sclerotic. - 5. Burned out. The osteoblastic activity eventually decreases leaving dense bone which is very thick for example in the skull Describe the pathogenesis of the aortic aneurysm. (6 marks) What would have caused the femoral artery occlusion. (4 marks) - MUST say: The aneurysm is caused by atherosclerosis weakening the wall. The hydrostatic pressure then causes the aorta to dilate. ( 2 marks) - Atherosclerosis causes weakening oft he wall by the following mechanism; ½ mark for each point. - - Endothelium is injured - - Dysfunctional endothelial cells are more permeable to lipids. - - Oxidation of lipids and an invasion of the vessel wall by macrophages. - - Accumulation and attachment of platelets to the vessel wall; platelets degranulate, releasing growth factors and permeability factors, and also help recruit macrophages into the site of vascular injury. - Oxidation of lipids deposited in the stroma; macrophages releasing reactive free oxygen radicals injure the muscular wall - Macrophages die and release cell debris, enzymes, and lipids that form the semiliquid core of atheromas. - The underlying media is injured by the inflammation and becomes weak and thin. - The aorta dilated because of luminal pressure. - ---PART 2--- - Ulceration and thrombosis of the atheroscleotic plaque ( in the Aorta). - The aortic plaque ulcerates exposing the thrombogenic lipid core. (1 mark) - A thrombus forms. (1 mark) - Embolism of the thrombus (1 mark) - Thrombus occludes the femoral artery (1 mark) - An arterial dissection arises when blood enters the arterial wall itself, as a hematoma dissecting between its layers. - Dissections are often but not always aneurysmal (see also below). - - In contrast, a false aneurysm (also called pseudo-aneurysm) is a defect in the vascular wall leading to an extravascular hematoma that freely communicates with the intravascular space (“pulsating hematoma”). - Both true and false aneurysms as well as dissections can rupture, often with catastrophic consequences. - More common (and dangerous) proximal lesions (called type A dissections), involving either both the ascending and descending aorta or just the ascending aorta (types I and II of the DeBakey classification) - Hypertension is the major risk factor in aortic dissection. - - suggesting that pressure-related mechanical injury is contributory. - A considerably smaller number of dissections are related to inherited or acquired connective tissue disorders causing abnormal vascular ECM (e.g., Marfan syndrome, Ehlers-Danlos syndrome, vitamin C deficiency, copper metabolic defects). - Regardless of the underlying etiology causing medial weakness, the trigger for the intimal tear and initial intramural aortic hemorrhage is not known in most cases. Nevertheless, once the tear has occurred, blood flow under systemic pressure dissects through the media, fostering progression of the medial hematoma. - Accordingly, aggressive pressure-reducing therapy may be effective in limiting an evolving dissection. In some cases disruption of penetrating vessels of the vasa vasorum can give rise to an intramural hematoma without an intimal tear. - In lobar pneumonia, four stages of the inflammatory response have classically been described; congestion, red hepatization, gray hepatization, and resolution. - In the first stage of congestion the lung is heavy, boggy, and red. It is characterized by vascular engorgement, intra-alveolar fluid with few neutrophils, and often the presence of numerous bacteria. - The stage of red hepatization that follows is characterized by massive confluent exudation with neutrophils, red cells, and fibrin filling the alveolar spaces. On gross examination, the lobe now appears distinctly red, firm, and airless, with a liver-like consistency, hence the term hepatization. - The stage of gray hepatization follows with progressive disintegration of red cells and the persistence of a fibrinosuppurative exudate, giving the gross appearance of a grayish brown, dry surface. - In the final stage of resolution the consolidated exudate within the alveolar spaces undergoes progressive enzymatic digestion to produce granular, semifluid debris that is resorbed, ingested by macrophages, expectorated, or organized by fibroblasts growing into it. Pleural fibrinous reaction to the underlying inflammation, often present in the early stages if the consolidation extends to the surface (pleuritis), may similarly resolve. More often it undergoes organization, leaving fibrous thickening or permanent adhesions. Asbestosis and sequalae - Asbestosis is marked by diffuse pulmonary interstitial fibrosis, which is indistinguishable from diffuse interstitial fibrosis resulting from other causes, except for the presence of multiple asbestos bodies. - They arise when macrophages attempt to phagocytose asbestos fibers; the iron is presumably derived from phagocyte ferritin. - Asbestosis begins as fibrosis around respiratory bronchioles and alveolar ducts and extends to involve adjacent alveolar sacs and alveoli; eventually the affected regions become honeycombed. - The scarring may trap and narrow pulmonary arteries and arterioles, causing pulmonary hypertension and cor pulmonale. - Both lung carcinomas and mesotheliomas (pleural and peritoneal) develop in workers exposed to asbestos. The risk of lung carcinoma is increased about fivefold for asbestos workers; the relative risk of mesotheliomas, normally a rare tumor (2 to 17 cases per 1 million persons), is more than 1000-fold greater. - Concomitant cigarette smoking greatly increases the risk of lung carcinoma but not that of mesothelioma. - The alveolar capillary membrane is formed by two separate barriers; the microvascular endothelium and the alveolar epithelium. In ARDS the integrity of this barrier is compromised by either endothelial or epithelial injury or, more commonly, both. - Markers of endothelial injury and activation such as endothelin and von Willebrand factor can be detected at high levels in the serum of patients with ARDS. - The acute consequences of damage to the alveolar capillary membrane include increased vascular permeability and alveolar flooding, loss of diffusion capacity, and widespread surfactant abnormalities caused by damage to type II pneumocytes. - Endothelial injury also triggers the formation of microthrombi that add the insult of ischemic injury. Hyaline membranes so characteristic of ALI/ARDS result from inspissation of protein rich edema fluid that entraps debris of dead alveolar epithelial cells. Diffuse alveolar damage: what is it and describe the pathological features (a) immediately and (b) at 6 months - Diffuse alveolar damage is a histological pattern in lung disease. It is seen in acute respiratory distress syndrome (ARDS), transfusion related acute lung injury (TRALI) and acute interstitial pneumonia (AIP). - Macroscopic findings: The lungs are heavy, filled with fluid, firm, red, and boggy. - Microscopic findings in acute stage: There is congestion, interstitial and intraalveolar edema, inflammation, and fibrin deposition along the inside of alveoli in form of hyaline membranes - ---6 months--- - Must mention fibrosis (1 mark) 1/2 mark for any of the ones below. - • Marked thickening of the alveolar septa. - • Lungs are stiff. - • Alveoli are smaller and can resemble a honeycomb What is the pathogenesis of coronary artery atheroma? - 1. There is endothelial cell injury by a variety of mechanisms including hypertension and turbulent blood flow. Chemicals in the blood such as homocystine and high blood glucose and free radicals can also damage it. - 2. The damaged endothelial cells are leaky and admit lipids. The lipids sit under the endothelium forming a fatty streak. - 3. Macrophages and other white blood cells are attracted by the damaged endothelium. They migrate into the fatty streak and cause release of cytokines which means that there are more inflammatory cells attracted. They ingest lipid. - 4. The cytokines also cause smooth-muscle migration and proliferation. The end result is an atheroma with endothelium overlying a fibrous and smooth-muscle cap, with a lipid core. And associated chronic inflammation. The outside smooth-muscle wall can become thin. - 5. If the atheroma is unstable because they are fibrous cap is thin than it can rupture and thrombus can form on it. What is the pathogenesis of MI? - 1. An unstable atheroma forms in a coronary artery. - 2. The thin cap overlying the lipid core ruptures exposing the thromobogenic.lipid. - 3. Platelets adhere and the clotting cascade is initiated. - 4. The coronary artery is occluded by thrombus . This results in ischaemia to the myocardium supplied by the coronary artery. - 5. The myocardium undergoes coagulative necrosis. What is the pathogenesis of heart valve damage in chronic rheumatic fever? - 1. A streptococcus infection in the throat generates antibodies. - 2. These cross-react with proteins in the heart valve. Inflammation occurs by activation of the complement cascade, recruitment of inflammatory cells. The mitral valve is particularly affected. - 3. Cytokines produced by the inflammatory cells cause fibrosis by activation of fibroblasts. - 4. Repeat infections mean that this cycle is repeated resulting in marked fibrosis of the valve with shortening of the cordi tendonae. - 5. The repeated cycles of fibrosis result in a markedly distorted valve with a gaping fishlike mouth. The distorted valve is incompetent and regurgitatent. What is the pathogenesis of Coal miners pneumoconiosis? - 1. The coalminer breathes in coal dust and silica. - 2. This is not able to be cleared by the lung cilia, or by the type 2 pnuemocytes. It accumulates in the interstitium of the lungs. - 3. The silica and carbon cause activation of fibroblasts and chronic inflammation resulting in fibrosis and expansion of the alveolar walls. - 4. The fibroblasts produce collagen which then organises and contracts. - 5. As the as spaces contracts the become smaller with very wide alveolar walls. This is honeycomb lung. Gas exchange is unable to occur over the thick walls. The patient becomes very short of breath. What is the pathogenesis of cervical cancer? - 1. Human papilloma virus of a high risk type such as 16 or 18 infects squamous cells. This particularly happens at the transformation zone. - 2. Initially the infection is episomal, and produces a low grade squamous intraepithelial lesion. Eventually, HPV DNA integrates into the nuclear DNA of the squamous cell. - 3. The HPV oncogenes E6 and E7 are transcribed and block the retinoblastoma and P 53 pathways. This allows proliferation of atypical cells. - 4. The atypical cells accumulate to fill up the entire thickness of the epithelium. This is high-grade squamous intraepithelial lesion. HS I L. - 5. More mutations accumulate in the cell and the cell has the ability to invade through the basement membrane forming a large tumour mass of squamous cell carcinoma. Cytoplasmic changes in CIN - Atypical cells with: - - High N/C ratio - - Increased mitotic activity - - Nuclear atypia - - Koilocytic change (structural changes in a cell as a result of HPV infection) What is the pathogenesis of endometrioid carcinoma of the ovary? - Endometrioid tumors are distinguished from serous and mucinous tumors by the presence of tubular glands bearing a close resemblance to benign or malignant endometrium - 1. Endometriosis occurs and endometrial glands and stroma are deposited in the ovary. - 2. The ectopic endometrium is exposed to unopposed oestrogen, such as from oestrogen made in fat, or iaterogenic sources - 3. There is hyperplasia of the endometrium, mutations occur in some cells and this becomes atypical hypoplasia. - 4. Further mutations occur and small foci of endometrioid adenocarcinoma form in the ovary. - 5. This grows and becomes a large tumour which can metastasise by invasion of blood vessels and transit coelomic spread. What is the pathogenesis of hepatocellular carcinoma caused by hepatitis B? - 1. Hepatocellular carcinoma is caused by hepatitis B in two ways. The hepatitis B has a direct carcinogenic effect by integrating its DNA and a into the nucleus of the cell. The cirrhosis caused by chronic hepatitis B infection also helps cause hepatocellular carcinoma. - 2. Hepatitis B integration into the nucleus of a cell causes of activation of oncogenes. This causes the cell to proliferate abnormally and the DNA to become unstable so that more mutations occur. - 3. The chronic inflammation from cirrhosis causes increased cell turnover. This means that any cells that have abnormal mutations are given the opportunity to proliferate more - 4. Eventually the atypical cells which are caused by this process form a nodule. Initially this may be an adenoma. As the mutations accumulate, this progress is to carcinoma. - 5. The carcinoma cells grow uncontrollably and form a large tumour mass. This can invade blood vessels and metastasise. What is the pathogenesis of alcoholic cirrhosis? - 1. Alcohol causes injury to cells resulting in cell death and fatty change within the remainder of cells. - 2. The cell death and fatty change attract inflammatory cells. This forms steatohepatitis. - 3. The inflammatory cells release cytokines which causes fibroblast proliferation. Preparative processes mean that the liver regenerate and forms small nodules. - 4. Continued cell death, reparative growth and fibrosis result in bands appearing between the portal tracts and the central veins. This is called bridging fibrosis. - 5. These fibrous band expand to surround nodules of regenerating liver cells are form the characteristic appearance of cirrhosis. What is the pathogenesis of gallstone induced pancreatitis? - 1. Gallstone travels down the common bile duct and blocks the pancreatic duct. - 2. There is some reflux of bile into the pancreatic duct. This causes activation of the pancreatic enzymes lipase amylase within the duct. - 3. The enzymes start to auto digests the adjacent pancreatic acini. This releases more enzymes and a positive cycle of enzyme release and inflammation occurs. - 4. If this settles down it will become chronic fibrotic pancreatitis. - 5. If the positive reinforcement cycle continues there will be continuing digestion and fat necrosis, protein necrosis in collagen from proteases, ascites, haemorrhage as blood vessels are digestive and eventually acute haemorrhagic pancreatitis and shock. A 69 year old man is involved a severe motorcycle crash. He is admitted to ICU with multiple fractures. On day 3 he has develops low blood pressure, a and tense abdomen. Ascitic fluid is drained and is cloudy…..his amylase is 8765. 1. Describe the sequence of events leading to his acute decline. (5 marks) 2. Describe the typical sequence for healing of his tibeal fracture (4 marks) - ---Part 1 (5 marks)--- - To pass MUST say (2 marks) - The patient has acute pancreatitis. - The inappropriate activation of enzymes inside the pancreas leading to autodigestion is thought to occur in the following sequence (1 mark for each of these) - ▪ Shock damages the pancreas through ischaemia. - ▪ Rupture of damaged acinar cells leading to a release of digestive enzymes into the interstitial spaces and peripancreatic tissue - ▪ Autodigestion of proteins, lipids, and carbohydrate in of cells forming the pancreas, peripancreatic fat tissue, and blood vessels - ---Part 2--- 1 mark for explaining each part of the healing sequence - Bone healing can be divided into four stages: - • inflammation; - • soft callus formation; - • hard callus formation; - • remodeling. What is granulation tissue and its constituents? What are the 4 stages of tissue repair from scarring - Granulation tissue: is new connective tissue and tiny blood vessels that form on the surfaces of a wound during the healing process - - contains new vessels, fibroblasts laying down collagen, and ECM - (1) Formation of new blood vessels ; angiogenesis - (2) Migration and proliferation of fibroblasts -> laying down collagen - (3) Deposition of ECM - (4) Maturation and organisation of fibrous scar Describe the two types of skin healing - Primary intention: edges are brought together by suturing; no apparent deficit of flesh - Secondary intention: close apposition of wound not possible - - large deficit 4 stages of bone healing - (1) Haematoma formation: the initial trauma will damage blood vessels, leading to clot formation around the site of injury. In this and other early phases of healing there will also be necrosis and phagocytosis of damaged tissues. - (2) Granulation tissue/soft callous: growth of new blood vessels/angiogenesis, influx of macrophages and fibroblasts. - - This occurs early in healing [2-5 days] and prepares the area for osteogenesis; fibrogenesis will only deposit a small amount of collagen before chondrocytes and osteoblasts become the main ECM secreting cells. - (3) Hard callous formation: where cartilage and bone is laid down - - Cartilage formation: laid down by chondrocytes/chondroblasts. - - Bone formation: progenitor cells migrate in from the periosteum and differentiate into osteoblasts → extracellular matrix proteins (including collagen) → mineralised to form bone. - (4) Remodelling: both osteoblasts and osteoclasts [which break down bone] will be active to convert woven/spongy bone [Weak] to cortical/compact bone. List 4 factors which may result in delayed or abnormal bone healing - Movement: too much will disrupt callous but too little will reduce the size and delay healing - Foreign body - Medication: e.g. corticosteroids - Diabetes and other medical conditions Types of bone healing failures (2); and subsequent treatment - Non-union: bone ends are too far apart - Fibrous union: - Surgery: to remove the excess fibrous tissue, correct alignment and possible add a bone graft - - reproducing haemotoma to start the healing process What is the pathogenesis of oesophageal adenocarcinoma? - 1. There is damage to the normal oesophageal squamous epithelium at the gastro-oesophageal junction by reflux. - 2. The squamous epithelium undergoes metaplasia to intestine or type. There is chronic inflammation. This is called Barrett’s oesophagus. - 3. There is an accumulation of mutations, some of which is due to the increased cell turnover caused by the inflammation, this causes dysplasia within the intestinal epithelium. - 4. More mutations accumulate and the epithelium undergoes severe dysplasia and carcinoma in situ - 5. Further accumulation of mutations results in cells being able to invade through the basement membranes and proliferate forming a large mass which is oesophageal adenocarcinoma. What is the pathogenesis of duodenal peptic ulcer? -> gastric cancer - 1. Helicobacter pylori causes 90% of duodenal ulcers. - 2. With Helicobacter inflammation of the stomach mucosa is more marked in the distal (antral) region, with relative sparing of body (acid-secreting) - 3. This pattern results in increased gastrin release, which makes the acid secretion in the stomach go up. - 4. High acid output from the stomach is dumped in the duodenum. There are other factors that can increase rapid emptying. - 5. The high acid erodes the relatively unprotected duodenal mucosa and an ulcer forms. - Helicobacter causes antral gastritis by - - Releasing urease and breaking down the protective mucus layer - - Causing an antigenic reaction – T cells which cause epithelial damage - - Causing inflammation which kills the epithelial cells - - Causes gastric atrophy. - Must say the ulcer is malignant, that there is intestinal metaplasia and dysplasia leading to carcinoma. (3 marks) - + Gastric atrophy and inflammation lead to intestinal metaplasia - Further exposure to inflammation and carcinogens lead to dysplasia - An accumulation of mutations lead to high grade dysplasia and carcinoma What is the pathogenesis of a haemorrhagic stroke? - 1. An embolus from a more proximal artery lodges in a cerebral vessel. - 2.The vessel occludes and the area of the brain it supplies becomes ischaemic. The vessels in the iscaemic area are damaged too with the iscaemia. - 3.The embolus dissolves and blood flows back into the vessels - 4.The damaged vessels rupture and blood flows into the brain - 5. With no blood supply and the haemorrhage the infarct becomes established What is the pathogenesis of a subarachnoid haemorrhage? - 1. There is a congenital weakness at the branch point of a circle of Willis vessel – a defect in the media. - 2. The weakness bulges out and forms a small berry aneurism - 3. A combination of hypertension, which increases the pressure and la place’s law means the aneurism expands like a balloon - 4. The larger it gets the easier it is to expand and the wall becomes very thin - 5. the berry anuerism bursts and hoses blood into the subarachnoid space. What is the pathogenesis of acute death following an ischaemic stroke? - 1. The blood vessels around the stroke and damaged and become leaky - 2. The fluid leaking out of them causes the brain to swell. - 3. The cranium has a limited volume so the brain starts to push through the tentorium - 4. The herniation (uncal herniation, the uncus is part of the temporal lobe) compresses vital midbrain structures. - 5. With increasing pressure and progression of the hernia there will be distortion of the brainstem leading to Duret hemorrhages (tearing of small vessels in the parenchyma) in the pons., respiratory center depression and death. What is the pathogenesis of renal failure in diabetes? - 1. The basement membrane of glomeruli becomes thickened by complex glycosylation products - 2. There is increase in the mesangial matrix by increased matrix production or glycation of matrix proteins. If they are nodular they are called Kimmelsteil-Wilson nodules. - 3. Hyalinisation of arterioles narrows them and leads to glomerular ischaemia - 4. Narrowing of larger vessels leads to global ischaemia and loss of tubules with interstitial scarring. - 5. The glomeruli become sclerosed and there is extensive renal scarring What is the pathogenesis of lytic bone lesions in a patient with prostate cancer? - 1. The prostate cancer metastasises to bone - 2. The cancer cells release cytokines that cause osteoclast proliferation and activation - 3. The osteoclasts destroy the bony trabeculae - 4.The loss of calcified bone leads to radiolucency What is the pathogenesis of unstable metacarpophalangeal joints in rheumatoid arthritis? - 1. Complex autoimmune activation of B and T cells and cytokines results in joint inflammation - 2. Inflammatory cells infiltrate the synovial membrane and cause synovitis. - 3. Pannus forms and destroys bone, enzymes secreted by synoviocytes and chondrocytes degrade cartilage - 4. The inflammation also weakens the tendons and ligaments - 5. Combined they lead to malformation of the MCP joint. What is the pathogenesis of bullus formation in pemphigoid? - 1. Auto-antibodies form against the basement membrane ( hemidesmosomes) - 2. These bind to the skin basement membrane - 3. Complement is activated and the hemidesmosomes are destroyed - 4. The epidermis separates from the dermis - 5. Fluid accumulate in the gap and causes a bulla Invasive ductal carcinoma of no special type and high-grade ductal carcinoma in situ (DCIS). - The invasive ductal carcinoma will have developed from preceding high-grade ductal carcinoma in situ. - High-grade and extensive ductal carcinoma in situ has a relatively high risk of progression to invasive ductal carcinoma - Ductal carcinoma in situ arises as a result of clonal proliferations due to accumulated DNA alterations/abnormalities and epigenetic changes in epithelial cells - Further DNA alterations in abnormal intraductal epithelial cell clones, as well as changes in the adjacent stroma, lead to stromal invasion i.e the development of invasive ductal carcinoma. Describe pathological changes in osteolytic metastatic cancer - The osteolytic lesions are almost certainly due to metastatic breast cancer - Cytokines released by the metastatic cancer cells activate osteoclast activity in the site of metastases leading to osteolysis in these sites. - Typical pathology of these lesions would be of groups of malignant epithelial cells (metastatic carcinoma) in the marrow spaces. - Bony trabeculae are thin or absent - Osteoclast activity leading to decreased thickness of the adjacent bone trabeculae and no significant increased osteoblastic activity. - Bone metastases are typically multifocal and most commonly involve the axial skeleton. - Triple assessment: Clinical and radiological examination supplemented with tissue diagnosis is the standard of care for all. - Hereditary Breast and Ovarian Cancer Syndrome - 60-80% lifetime risk breast cancer - 20-40% lifetime risk ovarian cancer - 40-60% lifetime risk breast cancer (5-10% male) - 10-20% lifetime risk ovarian cancer - Pancreas and prostate cancer O and G: Abnormal Uterine Bleeding Diagnosis and investigations - AUB in women >40 yr requires an endometrial biopsy to rule out cancer even if known to have fibroids O and G: Abnormal Uterine Bleeding Management - Adenomyosis: surgery is the only definitive - - iron suppliments, analgesia and NSAIDs, Depo-Provera/Mirena or a progesterone type non-phasic contraceptive - Fibroid: Only needs treatment if symptomatic; e.g. rapidly enlarging - - treat anemia if present - - medical approach; antiprostaglandins (ibuprofen, other NSAIDs) and tranexamic acid - - Interventional radiology (embolise blood supply), or surgery O and G: Abnormal Uterine Bleeding Treatment - resuscitate patient if hemodynamically unstable - treat underlying disorders; if anatomic lesions and systemic disease have been ruled out, consider DUB - Mild DUB (dysfunctional uterine bleeding): not attributable to organic (anatomic/systemic) disease DUB is a diagnosis of exclusion. Anovulatory AUB often used synonymously with DUB - - NSAIDs - - combined OCP - - progestins (Provera®) on first 10-14 d of each month or every 3 mo if oligomenorrheic - - Mirena® IUD - - endometrial ablation; consider pretreatment with danazol or GnRH agonists - - if finished childbearing - - repeat procedure may be required if symptom reoccur especially if over 40 YOA - - hysterectomy: definitive treatment	2	12	0	0	0	1	0.617194	1	7,439
Danish guidelines for management of non-APC-associated hereditary polyposis syndromes Hereditary Cancer in Clinical Practice volume 19, Article number: 41 (2021) Hereditary Polyposis Syndromes are a group of rare, inherited syndromes characterized by the presence of histopathologically specific or numerous intestinal polyps and an increased risk of cancer. Some polyposis syndromes have been known for decades, but the development in genetic technologies has allowed the identification of new syndromes.. The diagnosis entails surveillance from an early age, but universal guideline on how to manage and surveille these new syndromes are lacking. This paper represents a condensed version of the recent guideline (2020) from a working group appointed by the Danish Society of Medical Genetics and the Danish Society of Surgery on recommendations for the surveillance of patients with hereditary polyposis syndromes, including rare polyposis syndromes. Hereditary Polyposis Syndromes (HPSs) are a group of rare, inherited syndromes characterized by the presence of histopathologically specific or numerous intestinal polyps and sometimes extra-intestinal manifestations. HPSs is associated with an increased risk of cancer in and outside the gastrointestinal (GI)- tract -tract and timely diagnosis is important in order to offer specific organ-targeted surveillance programs with the purpose of reducing morbidity and mortality. The classification of HPS has traditionally been based on the histopathology of the removed polyps as presented in Fig. 1. Some HPSs have been known for decades, but the possibility of sequencing many genes in a very short time (Next Generation Sequencing (NGS)), has revealed several genes now known to be associated with HPSs, and genetic testing is therefore a part of the diagnostic pipeline for patients with (or suspected of having) a HPS. Both autosomal dominant and autosomal recessive inheritance is seen. Genetics diagnostics and genetic counselling Genetic testing includes gene-panel screening using NGS with genes known to be related to polyposis syndromes. As of 2020, the panel should include the genes listed in Fig. 1. The finding of causative monoallelic (autosomal dominant) or biallelic (autosomal recessive) germline pathogenic variations (PVs) is crucial in order to make an accurate diagnosis which in turn is the prerequisite for tailoring the optimal surveillance program for each patient. Additionally, detecting a genetic cause also makes prenatal diagnosis, including preimplantation diagnosis (PGT), possible in some cases. Somatic mosaicism should be considered in patients with a clinically convincing HPS, where standard genetic analyses of blood does not identify the cause. How to manage HPS? There is a high demand for guidelines addressing questions like: how many and what types of polyps should cause concern? When should a patient be referred for genetic counselling? How should we manage patients and their families when detecting (or not detecting) a PV in an HPS gene? In order to address these questions, the Danish Society of Medical Genetics and the Danish Society of Surgery appointed a group of experts in the field in 2017. This paper is a summarized version of this work and guidelines, approved by the two societies in 2020. The guideline points out referral criteria for genetic work-up and counselling (Fig. 2) and suggests surveillance programs for HPS-patients with or without a known genetic etiology. APC-associated polyposis and PTEN-hamartoma-tumor syndrome are not included in the work. The working group agreed on general recommendations (Suppl. Table 1) and more specific surveillance for each HPS (Suppl. Table 2). General considerations of surveillance and prophylactic GI-operations Endoscopic investigations are the core of surveillance. There is no evidence for recommending prophylactic intestinal resections in any of the HPSs, which are described here, but some patients may have a massive polyp burden in part(s) of the GI-tract, making endoscopic surveillance challenging; gastrointestinal resection is indicated in some patients. In the case of colorectal cancer (CRC), a subtotal or total colectomy should be considered, but taking the polyp burden, age and co-morbidity into consideration. If (large) polyps causes complications such as invagination and/or bleeding, segmental resections with or without peroperative enteroscopy should be performed. After surgery surveillance must be resumed. HAMARTOMATOUS polyposis syndromes Peutz-Jeghers syndrome (PJS) is characterized by the presence of hamartomatous Peutz-Jeghers polyps in the GI-tract and mucocutaneous pigmentations (MPs) especially on the lips and buccal mucosa. MPs typically presents in childhood and tend to fade after puberty. The polyps are mainly found in the small intestines and 50–75% of patients experience GI symptoms before 20 years of age, with invagination as the most common complication . PJS is inherited in an autosomal dominant manner and STK11 is the only gene known to be associated with the condition . An age dependent increased risk of cancer in the GI-tract as well as various extra-intestinal cancers are well documented (Table 1) . Surveillance is comprehensive and should start in childhood (see Supp Table 2) . Management of patients with a solitary Peutz-Jeghers-polyp or isolated MPs Patients with a solitary Peutz-Jeghers polyp should be referred to a clinical genetics department for STK11 analysis. Endoscopy with gastroscopy, colonoscopy and video capsule enteroscopy could be performed in order to rule out PJS. If both genetic and endoscopic work-up is negative, PJS is unlikely and the patient (and family members) should not be subjected to further investigations or follow-up. Isolated MPs suggestive of PJS should be managed as described by Latchford et al. Juvenile polyposis syndrome Juvenile Polyposis Syndrome (JPS) is characterized by the presence of few to over a 100 hamartomatous juvenile polyps in the GI-tract, mostly in the large intestine and stomach. A subgroup of patients with JPS and a PV in SMAD4 may have symptoms of hereditary hemorrhagic telangiectasia (HHT) as well as an increased risk of aortic aneurisms . The phenotypic spectrum is broad and there is significant intra – and interfamilial variability in expressivity. JPS is inherited in an autosomal dominant manner. The distinction between patients – especially children – with solitary or few juvenile polyps from juvenile polyposis can be difficult, but for patients with only one juvenile polyp the risk for having JPS is low . The risk of CRC and gastric cancer is increased with the risk of gastric polyposis, gastric cancer being highest in SMAD4 carriers . The clinical approach may vary depending on the clinical picture. For SMAD4 carriers surveillance for HHT should start at 12 years, while the starting point for screening for aortopathy is less clear. Autosomal dominant adenomatous polyposis syndromes PVs in the exonuclease domains of POLE (exon 9–14) were described in adult patients with colonic polyps and/or early-onset CRC in 2013 [15, 16]. Since then, additional cases have been reported [17, 18]. Still, data regarding the phenotypic characteristics, penetrance and estimation of cancer risk are limited. Café-au-lait pigmentations may be part of the phenotypic spectrum and are important to discover, as this manifestation can be suggestive of a more aggressive phenotype. Several PVs have been reported with c.1270C > G, p.Leu424Val, (NM_006231.3) as the most frequent. Other pathogenic missense variants seem to be associated with a more severe phenotype with cancer including medulloblastoma) and CRC [19, 20]. There is an increased risk of CRC, and a high frequency of extraintestinal cancer has been reported. There is no evidence that truncating, loss-of-function variants cause POLE-associated polyposis. The genotype should guide surveillance strategies: p.Leu424Val results in a more Lynch-like phenotype with adult-onset cancer, while other pathogenic missense variants (often de novo) result in a more severe phenotype including childhood cancer and skin pigmentations (see Suppl. Table 2). For carriers of other pathogenic missense variants, surveillance programs should be tailored case by case, but in general beginning in childhood. PVs in the exonuclease domains of POLD1 (exon 6–12) were first described in patients with multiple adenomas and CRCs [21, 22]. Knowledge about phenotype, penetrance and risk of extracolonic cancers is limited, but polyposis and cancer develops in adulthood. As with POLE, PVs are missense variants and there is no evidence that truncating, loss-of-functions variants cause polyposis and/or cancer. AXIN2-associated polyposis (also referred to as oligodontia-colorectal cancer syndrome) is a rare autosomal dominant syndrome, characterized by adenomatous colonic polyps varying in number from 0 to > 100. In most families, affected patients also have oligodontia and/or other aspects of ectodermal dysplasia. There is an increased risk of CRC, which is most often diagnosed in adulthood. Other variants in the gene seem to cause oligodontia/ectodermal dysplasia only [21, 22]. Autosomal recessive adenomatous polyposis syndromes MUTYH- associated polyposis MUTYH-associated polyposis (MAP) is characterized by multiple colorectal adenomas. The phenotypic spectrum is wide, and the number of polyps varies from few to over a 100. Approximately 2% of patients with MAP develop CRC without polyps. The lifetime risk of CRC in patients with MAP is well-documented and high (43–100%). Duodenal adenomas and gastric polyps are also found in a significant part of patients. MAP is caused by biallelic PVs in MUTYH thus the inheritance pattern is autosomal recessive. The carrier frequency in the Northern European population is estimated to be 1–2%. Two-point mutations, p.Y179C and p.G396D (NM_001128425), account for approximately. 85% of all PVs in individuals with Northern European ancestry. A high carrier frequency is also found in the Northern African population as well as in the non-Ashkenazi Jewish population. Risk of cancer in heterozygotes with pathogenic MUTYH variants The risk for colorectal adenomas in monoallelic carriers of pathogenic MUTYH-variants has been investigated in a prospective study from 2019 which found no evidence of an adenomatous polyposis phenotype in monoallelic carriers. Carriers of a PV who have a first-degree relative with MAP have up to a 5-fold increased risk of CRC , while carriers in general have an over 3-fold increased risk. It is debated whether the finding of one PV in an individual should result in genetic testing of relatives (cascade-testing) as recommendations are contradictive [2, 25]. It is recommended that siblings of a patient with MAP are tested for the PV(s) in the family. Spouses of patients with MAP and spouses of patients, who are heterozygous carriers of a PV should be offered genetic screening of MUTYH. NTHL1-associated polyposis (or NTHL1-tumour syndrome) was described for the first time by Weren et al. in 2015 in patients with adenomatous polyposis in the lower GI-tract . As of January 2020, reports of 34 patients with NTHL1-associated polyposis have been published [6, 26,27,28,29,30,31,32]. Development of NTHL1-associated polyposis is caused by biallelic PVs in NTHL1 and the inheritance pattern is autosomal recessive. Most patients are homozygous for the recurrent PV, NTHL1, c.268C > T, p.Gln90* (NM_002528). There is a high frequency of CRC in the published cases, but also of breast- and duodenal cancer suggesting a broader cancer predisposition syndrome [32,33,34,35,36,37,38,39]. Thus, the phenotypic spectrum of this syndrome is still emerging. Constitutional mismatch repair deficiency syndrome Constitutional mismatch repair deficiency syndrome (CMMRD) is a distinct childhood cancer predisposition syndrome characterized by an increased risk of a broad spectrum of malignancies, and GI-polyposis in both the upper and lower GI-tract. Often café-au-lait spots and other findings that mimic neurofibromatosis type 1 are detected. The patients carry biallelic PVs in the MMR genes (MLH1, MSH2, MSH6 and PMS2) and thus the inheritance pattern is autosomal recessive. More than half of the patients known with CMMRD have bi-allelic PVs in PMS2 . Recommendations for GI-surveillance are listed in Suppl.Table 2. Suggested surveillance for other malignancies is described by the European Consortium of CMMRD . MSH3- and MLH3-associated polyposis MSH3: As of January 2020, a total of four individuals from two families have been reported with biallelic PVs in MSH3 . The inheritance pattern is autosomal recessive and the associated phenotype is characterized by the presence of colorectal adenomatous polyposis. Polyposis was accompanied by benign and malignant lesions in the GI- tract and extracolonic manifestations such as duodenal adenomas, thyroid adenomas, gastric cancer and astrocytoma. MLH3: Olkinuora et al. reported five patients from four families to be homozygous for PVs in MLH3,. The patients had 50–200 adenomatous polyps (age range 48–52 years). One of three female patients had breast cancer at age 52, and the male patient had CRC at age 48. Other polyposis syndromes GREM1-associated mixed polyposis GREM1-associated Mixed Polyposis (previously Hereditary Mixed Polyposis syndrome) is an extremely rare condition with an unknown incidence. The condition was first described by Whitelaw et al. in 1997 in an Ashkenazi Jewish family with mixed GI-polyposis and CRC. A genetic cause was reported in 2012 by Jaeger et al, who detected a 40 kb duplication upstream of GREM1 . Since then other duplications have been reported [21, 39, 40]. The mode of inheritance is autosomal dominant and the condition is caused by upstream GREM1 duplications . The histopathology of the polyps is variable and includes atypical juvenile polyps and/or hyperplastic polyps as well as adenomas and serrated adenomas, and there is a phenotypic overlap with other syndromes, although the phenotypic description is limited. CRC occurs with an increased frequency in adulthood . Serrated polyposis syndrome Serrated Polyposis Syndrome (SPS) (previously named Hyperplastic Polyposis Syndrome) Is a condition characterized by numerous serrated polyps in the colon. Although the prevalence is unknown, the syndrome is probably more common than anticipated. In fecal occult blood test-based screening cohorts 1:111–1:238 individuals were diagnosed [42, 43]. SPS is commonly grouped with the HPSs but does not appear to be inherited in a simple Mendelian fashion. Some studies link PVs in RNF43 to SPS; however, studies of larger cohorts suggest that RNF43 only explains a small proportion of cases [44, 45]. Individuals with SPS have an increased risk of CRC, and relatives have a recognized substantial risk of developing CRC, but the risk is not well defined . Polyposis with unknown etiology In some patients with a significant number of adenomas in the L-GI-tract, both with and without a family history of polyposis, the etiology is not identified by gene analysis. These patients/families may have the diagnosis of “polyposis of unknown etiology” although there is no clear definition of the term “polyposis”. The definition seen in Table 2 can be used as guidance. Few publications have focused on this group of patients, and these are likely influenced by selection bias. Cancer occurrence has been reported in relatives, both in the colon and in the U-GI tract, but the inclusion criteria in these studies differ from those listed in Table 2 [47,48,49]. The National Comprehensive Cancer Network (NCCN) suggests surveillance/management guided by the phenotype of the patient and by the family history . In recent years, HPSs have been identified due to the development in genetic technologies. Patients with these syndromes should be offered surveillance in order to reduce mortality and morbidity, and genetic analysis is crucial in the diagnostic pipeline. Long-term follow-up studies are needed in order to obtain evidence but are complicated by the small number of patients, lack of population-based data and risk of ascertainment bias. The guidelines presented will have to undergo revision as knowledge increases and new polyposis syndromes are identified. Availability of data and materials Congenital mismatch repair deficiency - FAP : Familial adenomatous polyposis Hereditary hemorrhagic telangiectasia Hereditary polyposis syndrome Juvenile polyposis syndrome Lower gastrointestinal tract - PJS : - PV : - U-GI : Upper gastrointestinal tract Serrated Polyposis Syndrome - VCE : Hinds R, Philp C, Hyer W, Fell JM. Complications of childhood Peutz-Jeghers syndrome: implications for pediatric screening. J Pediatr Gastroenterol Nutr. 2004;39(2):219–20. https://doi.org/10.1097/00005176-200408000-00027. Syngal S, Brand RE, Church JM, Giardiello FM, Hampel HL, Burt RW. 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New insights into POLE and POLD1 germline mutations in familial colorectal cancer and polyposis. Hum Mol Genet. 2014;23(13):3506–12. https://doi.org/10.1093/hmg/ddu058. Bellido F, Pineda M, Aiza G, Valdes-Mas R, Navarro M, Puente DA, et al. POLE and POLD1 mutations in 529 kindred with familial colorectal cancer and/or polyposis: review of reported cases and recommendations for genetic testing and surveillance. Genet Med. 2016;18(4):325–32. https://doi.org/10.1038/gim.2015.75. Rosner G, Gluck N, Carmi S, Bercovich D, Fliss-Issakov N, Ben-Yehoyada M, et al. POLD1 and POLE gene mutations in Jewish cohorts of early-onset colorectal Cancer and of multiple colorectal adenomas. Dis Colon Rectum. 2018;61(9):1073–9. https://doi.org/10.1097/DCR.0000000000001150. Lindsay H, Scollon S, Reuther J, Voicu H, Rednam SP, Lin FY, et al. Germline POLE mutation in a child with hypermutated medulloblastoma and features of constitutional mismatch repair deficiency. Cold Spring Harbor Mol Case Stud. 2019;5(5):a004499. https://doi.org/10.1101/mcs.a004499. Wimmer K, Beilken A, Nustede R, Ripperger T, Lamottke B, Ure B, et al. A novel germline POLE mutation causes an early onset cancer prone syndrome mimicking constitutional mismatch repair deficiency. Familial Cancer. 2017;16(1):67–71. https://doi.org/10.1007/s10689-016-9925-1. Rohlin A, Rambech E, Kvist A, Torngren T, Eiengard F, Lundstam U, et al. Expanding the genotype-phenotype spectrum in hereditary colorectal cancer by gene panel testing. Familial Cancer. 2017;16(2):195–203. https://doi.org/10.1007/s10689-016-9934-0. Rivera B, Perea J, Sanchez E, Villapun M, Sanchez-Tome E, Mercadillo F, et al. A novel AXIN2 germline variant associated with attenuated FAP without signs of oligondontia or ectodermal dysplasia. Eur J Hum Genet. 2014;22(3):423–6. https://doi.org/10.1038/ejhg.2013.146. El Hachem N, Abadie C, Longy M, Colas C, Fert-Ferrer S, Leroux D, et al. Endoscopic phenotype of Monoallelic carriers of MUTYH gene mutations in the family of polyposis patients: a prospective study. Dis Colon Rectum. 2019;62(4):470–5. https://doi.org/10.1097/DCR.0000000000001323. Win AK, Dowty JG, Cleary SP, Kim H, Buchanan DD, Young JP, et al. Risk of colorectal cancer for carriers of mutations in MUTYH, with and without a family history of cancer. Gastroenterology. 2014;146:1208–11 e1–5. van Leerdam ME, Roos VH, van Hooft JE, Dekker E, Jover R, Kaminski MF, et al. Endoscopic management of polyposis syndromes: European Society of Gastrointestinal Endoscopy (ESGE) guideline. Endoscopy. 2019;51(9):877–95. https://doi.org/10.1055/a-0965-0605. Weren RD, Ligtenberg MJ, Kets CM, de Voer RM, Verwiel ET, Spruijt L, et al. A germline homozygous mutation in the base-excision repair gene NTHL1 causes adenomatous polyposis and colorectal cancer. Nat Genet. 2015;47(6):668–71. https://doi.org/10.1038/ng.3287. Rivera B, Castellsague E, Bah I, van Kempen LC, Foulkes WD. Biallelic NTHL1 mutations in a woman with multiple primary tumors. N Engl J Med. 2015;373(20):1985–6. https://doi.org/10.1056/NEJMc1506878. Belhadj S, Quintana I, Mur P, Munoz-Torres PM, Alonso MH, Navarro M, et al. NTHL1 biallelic mutations seldom cause colorectal cancer, serrated polyposis or a multi-tumor phenotype, in absence of colorectal adenomas. Sci Rep. 2019;9(1):9020. https://doi.org/10.1038/s41598-019-45281-1. Fostira F, Kontopodis E, Apostolou P, Fragkaki M, Androulakis N, Yannoukakos D, et al. Extending the clinical phenotype associated with biallelic NTHL1 germline mutations. Clin Genet. 2018;94(6):588–9. https://doi.org/10.1111/cge.13444. Groves A, Gleeson M, Spigelman AD. NTHL1-associate polyposis: first Australian case report. Familial Cancer. 2019;18(2):179–82. https://doi.org/10.1007/s10689-018-0107-1. Broderick P, Dobbins SE, Chubb D, Kinnersley B, Dunlop MG, Tomlinson I, et al. Validation of recently proposed colorectal Cancer susceptibility gene variants in an analysis of families and patients-a systematic review. Gastroenterology. 2017;152(1):75–7 e4. https://doi.org/10.1053/j.gastro.2016.09.041. Altaraihi M, Gerdes AM, Wadt K. A new family with a homozygous nonsense variant in NTHL1 further delineated the clinical phenotype of NTHL1-associated polyposis. Hum Genome Variation. 2019;6(1):46. https://doi.org/10.1038/s41439-019-0077-3. Bakry D, Aronson M, Durno C, Rimawi H, Farah R, Alharbi QK, et al. Genetic and clinical determinants of constitutional mismatch repair deficiency syndrome: report from the constitutional mismatch repair deficiency consortium. Eur J Cancer. 2014;50:987–96. Vasen HF, Ghorbanoghli Z, Bourdeaut F, Cabaret O, Caron O, Duval A, et al. Guidelines for surveillance of individuals with constitutional mismatch repair-deficiency proposed by the European consortium "care for CMMR-D" (C4CMMR-D). J Med Genet. 2014;51(5):283–93. https://doi.org/10.1136/jmedgenet-2013-102238. Adam R, Spier I, Zhao B, Kloth M, Marquez J, Hinrichsen I, et al. Exome sequencing identifies Biallelic MSH3 germline mutations as a recessive subtype of colorectal adenomatous polyposis. Am J Hum Genet. 2016;99(2):337–51. https://doi.org/10.1016/j.ajhg.2016.06.015. Olkinuora A, Nieminen TT, Martensson E, Rohlin A, Ristimaki A, Koskenvuo L, et al. Biallelic germline nonsense variant of MLH3 underlies polyposis predisposition. Genet Med. 2019;21(8):1868–73. https://doi.org/10.1038/s41436-018-0405-x. Whitelaw SC, Murday VA, Tomlinson IP, Thomas HJ, Cottrell S, Ginsberg A, et al. Clinical and molecular features of the hereditary mixed polyposis syndrome. Gastroenterology. 1997;112(2):327–34. https://doi.org/10.1053/gast.1997.v112.pm9024286. Jaeger E, Leedham S, Lewis A, Segditsas S, Becker M, Cuadrado PR, et al. Hereditary mixed polyposis syndrome is caused by a 40-kb upstream duplication that leads to increased and ectopic expression of the BMP antagonist GREM1. Nat Genet. 2012;44(6):699–703. https://doi.org/10.1038/ng.2263. McKenna DB, Van Den Akker J, Zhou AY, Ryan L, Leon A, O'Connor R, et al. Identification of a novel GREM1 duplication in a patient with multiple colon polyps. Familial Cancer. 2019;18(1):63–6. https://doi.org/10.1007/s10689-018-0090-6. Lieberman S, Walsh T, Schechter M, Adar T, Goldin E, Beeri R, et al. Features of patients with hereditary mixed polyposis syndrome caused by duplication of GREM1 and implications for screening and surveillance. Gastroenterology. 2017;152(8):1876–80 e1. https://doi.org/10.1053/j.gastro.2017.02.014. Tomlinson I, Rahman N, Frayling I, Mangion J, Barfoot R, Hamoudi R, et al. Inherited susceptibility to colorectal adenomas and carcinomas: evidence for a new predisposition gene on 15q14-q22. Gastroenterology. 1999;116(4):789–95. https://doi.org/10.1016/S0016-5085(99)70061-2. Bleijenberg AG, Je IJ, van Herwaarden YJ, Carballal S, Pellise M, Jung G, et al. Personalised surveillance for serrated polyposis syndrome: results from a prospective 5-year international cohort study. Gut. 2020;69(1):112–21. https://doi.org/10.1136/gutjnl-2018-318134. Jeg IJ, Bevan R, Senore C, Kaminski MF, Kuipers EJ, Mroz A, et al. Detection rate of serrated polyps and serrated polyposis syndrome in colorectal cancer screening cohorts: a European overview. Gut. 2017;66(7):1225–32. https://doi.org/10.1136/gutjnl-2015-310784. Yan HHN, Lai JCW, Ho SL, Leung WK, Law WL, Lee JFY, et al. RNF43 germline and somatic mutation in serrated neoplasia pathway and its association with BRAF mutation. Gut. 2017;66(9):1645–56. https://doi.org/10.1136/gutjnl-2016-311849. Buchanan DD, Clendenning M, Zhuoer L, Stewart JR, Joseland S, Woodall S, et al. Lack of evidence for germline RNF43 mutations in patients with serrated polyposis syndrome from a large multinational study. Gut. 2017;66(6):1170–2. https://doi.org/10.1136/gutjnl-2016-312773. Win AK, Walters RJ, Buchanan DD, Jenkins MA, Sweet K, Frankel WL, et al. Cancer risks for relatives of patients with serrated polyposis. Am J Gastroenterol. 2012;107(5):770–8. https://doi.org/10.1038/ajg.2012.52. Tieu AH, Edelstein D, Axilbund J, Romans KE, Brosens LA, Wiley E, et al. Clinical characteristics of multiple colorectal adenoma patients without germline APC or MYH mutations. J Clin Gastroenterol. 2016;50(7):584–8. https://doi.org/10.1097/MCG.0000000000000416. Kallenberg FGJ, Latchford A, Lips NC, Aalfs CM, Bastiaansen BAJ, Clark SK, et al. Duodenal adenomas in patients with multiple colorectal adenomas without germline APC or MUTYH mutations. Dis Colon Rectum. 2018;61(1):58–66. https://doi.org/10.1097/DCR.0000000000000868. Giarola M, Stagi L, Presciuttini S, Mondini P, Radice MT, Sala P, et al. Screening for mutations of the APC gene in 66 Italian familial adenomatous polyposis patients: evidence for phenotypic differences in cases with and without identified mutation. Hum Mutat. 1999;13(2):116–23. https://doi.org/10.1002/(SICI)1098-1004(1999)13:2<116::AID-HUMU3>3.0.CO;2-2. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Ethics approval and consent to participate Consent for publication The authors declare that there is no conflict of interest. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. About this article Cite this article Jelsig, A.M., Karstensen, J.G., Jespersen, N. et al. Danish guidelines for management of non-APC-associated hereditary polyposis syndromes. Hered Cancer Clin Pract 19, 41 (2021). https://doi.org/10.1186/s13053-021-00197-8	2	18	6	0	1	0	0.504856	7	8,485
The implantable cardioverter-defibrillator (ICD) is a surgically implanted electronic device that directs an electric charge directly into the heart to treat life-threatening arrhythmias. The implantable cardioverter-defibrillator is used to detect and stop life-threatening arrhythmias and restore a productive heartbeat that is able to provide adequate cardiac output to sustain life. The exact indications for the implantation of the device are controversial, but patients suffering from ventricular fibrillation (unproductive heartbeat), ventricular tachycardia (abnormally fast heartbeat), long QT syndrome (an inherited heart disease), or others at risk for sudden cardiac death are potential candidates for this device. A study by the National Institute for Heart, Lung, and Blood of the National Institutes of Health showed a significant increase in survival for patients suffering from ventricular arrhythmias when ICD implant is compared to medication. Several follow-up studies indicate that this may be due to the marked increase in survival for the sickest patients, generally defined as those having a heart weakened to less than 50% of normal, as measured by the ability of the left side of the heart to pump blood. Overall, studies have documented a very low mortality rate of 1–2% annually ICD implant is limited to patients that face the risk of sudden cardiac death from sustained ventricular arrhythmia, including ventricular tachycardia and ventricular fibrillation. Less than 1% of the more than 100,000 device implants done in the United States are performed on pediatric patients. Reduction in the risk of sudden cardiac death improves to less than 2% for both populations. Patients experiencing syncope (fainting) will be monitored with a cardiac monitor for arrhythmias. Following unsuccessful medical treatment for sustained ventricular arrhythmias, ICD implant will be indicated. Similar in structure to a pacemaker, an ICD has three main components: a generator, leads, and an electrode. The generator is encased in a small rectangular container, usually about 2 in (5 cm) wide and around 3 oz (85 g) in weight. Even smaller generators have been developed, measuring 1 in (2.5 cm) in diameter and weighing about 0.5 oz (14 g). The generator is powered by lithium batteries and is responsible for generating the electric shock. The generator is controlled by a computer chip that can be programmed to follow specific steps according to the input gathered from the heart. The programming is initially set and can be changed using a wand programmer, a device that communicates by radio waves through the chest of the patient after implantation. One or two leads, or wires, are attached to the generator. These wires are generally made of platinum with an insulating coating of either silicone or polyurethane. The leads carry the electric shock from the generator. At the tip of each lead is a tiny device called an electrode that delivers the necessary electrical shock to the heart. Thus, the electric shock is created by the generator, carried by the leads, and delivered by the electrodes to the heart. The decision of where to put the leads depends on the needs of the patient, but they can be located in the left ventricle, the left atrium, or both. According to the American College of Cardiology, more than 100,000 persons worldwide currently have an ICD. The battery-powered device rescues the patient from a life-threatening arrhythmia by performing a number of functions in order to reestablish normal heart rhythm, which varies with the particular problem of the patient. Specifically, if encountered with ventricular tachycardia, many devices will begin treatment with a pacing regimen. If the tachycardia is not too fast, the ICD can deliver several pacing signals in a row. When those signals stop, the heart may go back to a normal rhythm. If the pacing treatment is not successful, many devices will move onto cardioversion . With cardioversion, a mild shock is sent to the heart to stop the fast heartbeat. If the problem detected is ventricular fibrillation, a stronger shock called a defibrillation is sent. This stronger shock can stop the fast rhythm and help the heartbeat return to normal. Finally, many ICDs can also detect heartbeats that are too slow; they can act like a pacemaker and bring the heart rate up to normal. ICDs that defibrillate both the ventricles and the atria have also been developed. Such devices not only provide dual-chamber pacing but also can distinguish ventricular from atrial fibrillation. Patients that experience both atrial and ventricle fibrillations, or atrial fibrillation alone, that would not be controlled with a single-chamber device are candidates for this kind of ICD. ICD insertion is considered minor surgery, and can be performed in either an operating room or an electrophysiology laboratory. The insertion site in the chest will be cleaned, shaved, and numbed with local anesthetic. Generally, left-handed persons have ICDs implanted on the right side, and visa versa, to speed return to normal activities. Two small cuts (incisions) are made, one in the chest wall and one in a vein just under the collarbone. The wires of the ICD are passed through the vein and attached to the inner surface of the heart. The other ends of the wires are connected to the main box of the ICD, which is inserted into the tissue under the collarbone and above the breast. Once the ICD is implanted, the physician will test it several times before the anesthesia wears off by causing the heart to fibrillate and making sure the ICD responds properly. The doctor then closes the incision with sutures (stitches), staples, or surgical glue. The entire procedure takes about an hour. Immediately following the procedure, a chest x ray will be taken to confirm the proper placement of the wires in the heart. The ICD's programming may be adjusted by passing the programming wand over the chest. After the initial operation, the physician may induce ventricular fibrillation or ventricular tachycardia one more time prior to the patient's discharge, although recent studies suggest that this final test is not generally necessary. A short stay in the hospital is usually required following ICD insertion, but this varies with the patient's age and condition. If there are no complications, complete recovery from the procedure will take about four weeks. During that time, the wires will firmly take hold where they were placed. In the meantime, the patient should avoid heavy lifting or vigorous movements of the arm on the side of the ICD, or else the wires may become dislodged. After implantation, the cardioverter-defibrillator is programmed to respond to rhythms above the patient's exercise heart rate. Once the device is in place, many tests will be conducted to ensure that the device is sensing and defibrillating properly. About 50% of patients with ICDs require a combination of drug therapy and the ICD. Morbidity and mortality rates Perioperative mortality demonstrates a 0.4–1.8% risk of death for primary non-thoracotomy implants. The ICD showed improved survival compared to medical therapy, improving by 38% at one year. There is a 96% survival rate at four years for those implanted with ICD. Less then 2% of patients require termination of the device, with a return to only medical therapy. Ventricular tachycardia can be successfully relieved by pacing in 96% of instances with the addition of defibrillation converting 98% of patients to a productive rhythm that is able to sustain cardiac output. Ventricular fibrillation is successfully converted in 98.6–98.8% of all cases. Atrial fibrillation and rapid ventricular response leads to erroneous fibrillation in as many as 11% of patients. Environmental conditions that can affect the functioning of the ICD after installation include: - strong electromagnetic fields such as those used in arcwelding - contact sports - shooting a rifle from the shoulder nearest the installation site - cell phones used on that side of the body - magnetic mattress pads such as those believed to treat arthritis - some medical tests such as magnetic resonance imaging (MRI) Environmental conditions often erroneously thought to affect ICDs include: - microwave ovens (the waves only affect old, unshielded pacemakers and do not affect ICDs) - airport security (although metal detector alarms could be set off, so patients should carry a card stating they have an ICD implanted) - anti-theft devices in stores (although patients should avoid standing near the devices for prolonged periods) Patients should also be instructed to memorize the manufacturer and make of their ICD. Although manufacturing defects and recalls are rare, they do occur and a patient should be prepared for that possibility. In general, if the condition of the patient's heart, drug intake, and metabolic condition remain the same, the ICD requires only periodic checking every two months or so for battery strength and function. This is done by placing a special device over the ICD that allows signals to be sent over the telephone to the doctor, a process called trans-telephonic monitoring. If changes in medications or physical condition occur, the doctor can adjust the ICD settings using a programmer, which involves placing the wand above the pacemaker and remotely changing the internal settings. One relatively common problem is the so-called "ICD storm," in which the machine inappropriately interprets an arrhythmia and gives a series of shocks. Reprogramming can sometimes help alleviate that problem. When the periodic testing indicates that the battery is getting low, an elective ICD replacement operation is scheduled. The entire signal generator is replaced because the batteries are sealed within the case. The leads can often be left in place and reattached to the new generator. Batteries usually last from four to eight years. Patients are treated with medical therapy to reduce the chance of arrhythmia. This alternative has been shown to have a higher rate of sudden death when compared to ICD over the initial three years of treatment, but has not been compared at five years. If the site of ventricular tachycardia generation can be mapped by electrophysiology studies, the aberrant cells can be removed or destroyed. Less then 5% of patients suffer peri-operative mortality with this cell removal. Gersh, Bernard J., ed. Mayo Clinic Heart Book. New York: William Morrow and Company, Inc., 2000. Gregoratos, Gabriel, et al. "ACC/AHA Guidelines for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices." Journal of the American College of Cardiologists 31, no. 5 (April 1998): 1175–1209. Moss, A. "Implantable Cardioverter-Defibrillator Therapy: The Sickest Patients Benefit Most." Circulation 101 (April 2000): 1638–1640. Sears, Samuel F. Jr., et al. "Fear of Exertion Following ICD Storm: Considering ICD Shock and Learning History." Journal of Cardiopulmonary Rehabilitation 21 (January/February 2001): 47. American Heart Association. National Center. 7272 Greenville Avenue, Dallas, TX, 75231-4596. (214) 373-6300. http://www.americanheart.org . North American Society of Pacing and Electrophysiology. 6 Strathmore Road, Natick, MA, 01760-2499. (508) 647-0100. http://www.naspe.org/index.html . "Implantable Cardioverter-Defibrillator." American Academy of Family Physicians. May 7, 2001. http://www.familydoctor.org/handouts/270.html . "Implantable Cardioverter-Defibrillators (ICDs)" North American Society of Pacing and Electrophysiology. 2000. http://www.naspe.org/your_heart/treatments/icds.html . Michelle L. Johnson, MS,JD Allison J. Spiwak, MSBME WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED? Electrophysiologists are specially trained cardiologists or thoracic surgeons who study and treat problems with the heart conduction system. In a hospital operating room, they often implant the ICD system and oversee the programming or reprogramming of the device. Electrophysiologists receive special continuing medical education to provide successful implantation. Implantation, follow-up, and replacement can be limited at any one institution, therefore an experienced well-trained electrophysiologist should perform these procedures. QUESTIONS TO ASK THE DOCTOR - How many of these procedures have been performed by the physician? - What type of longevity can be expected from the device? - What will happen during device activation? - What precautions should be taken in the weeks immediately following implant? - After implantation how long will it be before normal daily activities can be resumed such as driving, exercise, and work? - What indications of device malfunction will there be, and when should emergency treatment be sought? - What precautions should be taken by bystanders when the device activates? - How will device recalls be communicated? - Can psychological counseling benefit patient satisfaction and comfort?	3	8	2	0	0	0	0.717374	2	2,825
Every woman is at risk for developing breast cancer, and the risk increases with age. According to the Centers for Disease Control and Prevention (CDC), approximately 94 percent of breast cancers are diagnosed in women over 40. Mammography is the best available way to detect breast cancer early, when it is most curable. A mammography is a low-dose X-ray of the breast that can find lumps too small to be felt during a breast examination. The breast is compressed firmly between two planes and pictures are taken. A screening mammogram is used to detect breast changes in women who have no signs of breast cancer. When the patient has signs or symptoms of a suspected disease, a diagnostic mammography is performed instead. It is essential coders understand the ICD-9-CM diagnosis, CPT and HCPCS coding guidelines for accurate code assignment for mammography. The diagnoses codes used to report encounters for a screening mammography are: • V76.11, Special screening for malignant neoplasm, screening mammogram for high-risk patients • V76.12, Special screening for malignant neoplasm, other screening mammogram The following codes may be assigned along with code V76.11 to identify why the patient is considered to be at high-risk. • V10.3, Personal history of malignant neoplasm, breast • V16.3, Family history of malignant neoplasm, breast • V15.89, Other specified personal history presenting hazards to health, other For Medicare patients, diagnosis codes V76.11 and V76.12 must be reported as the first listed diagnosis when a claim contains only screening mammography services. When billing a screening mammogram with other services billed on the same claim, codes V76.11 or V76.12 may be listed as a secondary diagnosis. If a condition is found during the screening, the code for the condition may be used as an additional diagnosis. The rationale for this is even though a condition is found during the mammography, the visit is still considered a screening. A screening mammogram is inherently bilateral and is reported with the following procedure codes: • 77057, Screening mammography, bilateral (two view film study of each breast) • G0202, Screening mammography, producing direct digital image, bilateral, all views Code 77052, Computer aided detection, screening mammography, may be assigned when it is performed in addition to the primary procedure. Computer aided detection (CAD) indicates a laser beam was used to scan the mammography film and the image was then converted to digital data for computer analysis. As mentioned earlier, if a condition is discovered during the screening, the code for the condition may be used as an additional diagnosis. In this instance, if the radiologist orders additional films based on the condition discovered during the screening mammogram, both procedures may be coded. When this occurs, append modifier GG, Performance and payment of a screening mammography and diagnostic mammography on same patient, same day to the diagnostic mammography code. Modifier GG indicates the test changed from a screening test to a diagnostic test. If not performed on both breasts, it is also important to append the appropriate anatomic modifier, RT or LT, to indicate on which side the diagnostic mammogram was performed. A diagnostic mammography is performed when the patient has a complaint, such as a lump, or to investigate an abnormality found during a screening mammogram. For diagnostic mammograms, report the confirmed or definitive diagnosis that is documented in the medical record. It is not necessary to code related signs or symptoms in addition to the confirmed diagnosis. If the final interpretation is not available when the case is coded, or if there is no definitive diagnosis, report the signs or symptoms that prompted the mammography. Diagnoses typically assigned with a diagnostic mammography include but are not limited to: • Lump or mass in breast (611.72) • Pain in breast (611.71) • Nipple discharge (611.79) • Specified anomalies of breast (757.6) • Neoplasms of breast, both male and female (174.0-175.9, 198.81, 217, 233.0, 238.3, 239.3) The assignment of procedure codes is dependent on the services provided and the technologies used to perform the mammography. The following codes are used to report a diagnostic mammography: • 77055, Mammography; unilateral • 77056, Mammography; bilateral • G0204, Diagnostic mammography, producing direct digital image, bilateral, all views • G0206, Diagnostic mammography, producing direct digital image, unilateral, all views Code assignment will be determined by whether or not direct digital images were taken and if the procedure was bilateral or unilateral. A diagnostic mammography without a direct digital image is coded to either 77055 or 77056, depending on the laterality of the procedure. A diagnostic mammography generating a direct digital image is coded to either G0204 or G0206, depending on the laterality of the procedure. Add-on code 77051, Computer-aided detection, with further physician review for interpretation, with or without digitization of film images; diagnostic mammography, may be assigned in additional to codes 77055, 77056, G0204, or G0206 when performed in addition to the primary procedure. CAD uses computer software to review the mammography after the radiologist has interpreted it. If the software identifies any abnormalities, it marks them for further radiologist review. Test your knowledge with the following quiz: 1. An asymptomatic 65-year-old woman had a screening mammogram, which revealed a breast mass. The physician scheduled the patient for follow-up biopsy of the breast mass at a later date. How should the diagnoses and procedures for this case be coded? a. V76.12, 611.72, 77057 b. 611.72, 77057 c. V76.12, 77057 d. V76.11 611.72, 77056 2. An asymptomatic 70-year-old woman with a family history of breast cancer has a screening mammogram that reveals a right breast mass. As a result of the positive finding, a diagnostic mammogram of the right breast with direct digital image is then performed. How should the diagnoses and procedures for this case be coded? a. V76.11, 611.72, G0206GGRT b. 611.72, V76.11, V16.3, 77057, G0206GGRT c. 611.72, V16.3, 77057, 77055GGRT, G0206 d. V7611, 611.72, V16.3, 77057, G0206GGRT This month's column has been prepared by Cheryl D'Amato, RHIT, CCS, director of HIM, facility solutions, Ingenix, and Melinda Stegman, MBA, CCS, clinical technical editor, Ingenix (www.ingenix.com). CPT is a registered trademark of the American Medical Association. 1. a. Assign diagnosis code V76.12 to indicate the screening for malignant neoplasm of breast and code 611.72, Lump or mass in breast, as an additional diagnosis to identify the breast mass. Procedure code 77057 is assigned to for the screening mammography. 2. d. Assign diagnosis code V76.11 to indicate that the breast cancer screening is being performed on a high risk patient. Secondary diagnosis codes V16.3, family history of breast cancer and 611.72, lump or mass in breast are also assigned. Procedure code 77057 is reported for the screening mammogram. Procedure code G0206 is assigned for the unilateral diagnostic mammogram producing direct digital image. Modifiers GG and RT are appended to code G0206 indicating that the screening mammogram was converted to a diagnostic mammogram of the right breast.	4	6	9	0	8	10	0.719388	27	1,690
Type 2 Diabetes Type 2 diabetes is a chronic disease in which your body is unable to maintain a normal blood sugar (glucose) level. - Birth Control Facts about birth control to help decide which type is right for you. - Type 2 Diabetes Basic facts about type 2 diabetes & risk factors to be aware of. - Arrhythmia Facts about arrythmia, inclding the types, symptoms and causes. - Atrial Fibrillation Facts about atrial fibrillation, including symptoms and risk factors. - COPD Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Coronary Artery Angioplasty with Stent Coronary artery angioplasty with stent facts, including who needs it. - Coronary Heart Disease Get the facts about coronary heart disease. - Deep Vein Thrombosis Facts about deep vein thrombosis (DVT), including symptoms & causes. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Erectile Dysfunction Facts about erectile dysfunction (ED), including causes. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides About Dr. Melissa A Stevens Dr. Melissa A Stevens, MD is a Doctor primarily located in Farmington Hills, MI. She has 28 years of experience. Her specialties include Cardiovascular Disease and Internal Medicine. Dr. Stevens is affiliated with Beaumont Hospital - Troy, St Mary Mercy Hospital and Henry Ford Hospital. Dr. Stevens has received 2 awards. She speaks English. Dr. Melissa A Stevens has the following 2 specialties - Cardiovascular Disease - Internal Medicine An internist is a physician who focuses on the diagnosis and treatment of conditions that affect the adult population—both acute and chronic. These doctors are often who adults see as their primary physicians because they treat a broad range of illnesses that do not require surgical or specialist interventions. They also work to help a patient maintain optimal health in order to prevent the onset of disease. In addition to treating the common cold and flu, internists also treat chronic diseases like diabetes and heart disease. - Unstable Angina - Blood Clot - Heart Failure - Heart Diseases - High Blood Pressure (Hypertension) - Heart Block - Heart Attack - High Cholesterol - Acute Coronary Syndrome (ACS) Showing 4 of 4 Patients' Choice Award (2014) Patients' Choice recognition reflects the difference a particular physician has made in the lives of his/her patients. The honor is bestowed to physicians who have received near perfect scores, as voted by patients. On-Time Doctor Award (2014) Vitals On-Time + Promptness Award recognizes doctors with consistent high ratings for timeliness of appointments. The honor is granted based on a physician's overall and promptness scores. Dr. Stevens is affiliated (can practice and admit patients) with the following hospital(s). 28 Years Experience Wayne State University School Of Medicine Graduated in 1990 Wayne State University Dr. Melissa A Stevens accepts the following insurance providers. - Aetna Choice POS II - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - First Health PPO - HAP Preferred PPO - Humana Choice POS - Humana ChoiceCare Network PPO - View by Location Locations & DirectionsBeaumont Cardiovascular Specialists, 29645 W 14 Mile Rd Ste 200, Farmington Hills, MI Dr. Melissa A Stevens is similar to the following 3 Doctors near Farmington Hills, MI.	2	5	1	0	0	0	0.951056	1	804
Peripheral Neuropathy information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS). Neuropathy is when nerve damage interferes with the peripheral nervous system’s ability to function. Idiopathic neuropathy is when no cause is determined. Idiopathic sensory-motor polyneuropathy is an illness where sensory and motor nerves of the peripheral nervous system are affected and no obvious underlying etiology. Re idiopathic progressive polyneuropathy [ Follow Ups ] [ Post Followup ] [ The Neurology Forum ] [ FAQ ] Posted by CCF Neuro MD on August. Idiopathic means no known cause and polyneuropathy means many nerves. Idiopathic polyneuropathy is a length dependent degeneration of the peripheral nerves starting. Idiopathic Peripheral Neuropathy causes have no known origin. Discover the associated symptoms, signs, diagnoses, and treatment. Idiopathic progressive neuropathy. 2015 2016 Billable Code. Progressive neuropathy, idiopathic; ICD-10-CM G60.3 is grouped within Diagnostic Related Group(s). 1. J Neurol. 2004 Mar;251(3)269-78. Progressive idiopathic axonal neuropathy--a comparative clinical and histopathological study with vasculitic neuropathy. Progressive inflammatory neuropathy (PIN) is a disease that was identified in a report, released on January 31, 2008, by the Centers for Disease Control and. Sometimes peripheral neuropathy seems to happen for no particular reason. Doctors call this disorder "idopathic", which means "of unknown cause." Idiopathic progressive neuropathy; Polyneuropathy Progressive neuropathy, idiopathic; ICD-9-CM Volume 2 Index entries containing back-references to 356.4 Polyneuropathy, the most common form of a group of disorders known as peripheral neuropathy, is caused by damage to peripheral nerves (defined as all nerves beyond. Learn about Idiopathic Progressive Polyneuropathy from patients' first hand experiences and trusted online health resources, including common treatments and. Meaning of idiopathic polyneuropathy medical See also neuropathy. acute idiopathic polyneuropathy. idiopathic proctitis; Idiopathic Progressive Bilateral. Welcome to the Official Website of Stop Shootin Inc. IDIOPATHIC PROGRESSIVE POLYNEUROPATHY and 30 were atactica the are progressive disease. Clinical progressive a. Helpful, trusted answers from doctors Dr. Machanic on polyneuropathy idiopathic progressive Small fibre neuropathy could be immune origin like sensory cidp, more. Question - My doctor has diagnosed me with Idiopathic Progressive - AP. Find the answer to this and other Health questions on JustAnswer. Its symptoms are also similar to progressive inflammatory neuropathy. An asymmetrical variant of CIDP is known as Lewis Sumner-Syndrome. [2 Cause. Structure of a. Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disorder characterized by slowly progressive weakness and a loss of sensation in the legs. Can Immunoglobulins Intravenous be an effective treatment for Idiopathic Progressive Polyneuropathy? It is safe or dangerous to use Immunoglobulins Intravenous while. Polyneuropathy Information, Treatment and Therapy 'Idiopathic ,' meaning, 'of When more aggressive treatment is required for progressive types of neuropathy. Introduction idiopathic axonal neuropathy. In the last century, various names have been suggested for axonal polyneuropathy without a clear cause, such as. Idiopathic Progressive Polyneuropathy disease Malacards - Research Articles, Symptoms, Drugs, Genes, Clinical Trials What can help or stop the progression of peripheral neuropathy? to the neuropathy. I am experiencing progressive pain in idiopathic neuropathy you. Idiopathic polyneuropathy is a type of peripheral known disorder. Q What is small fiber sensory neuropathy and what causes it? Summary G60.3 Idiopathic progressive neuropathy - ICD-10-CM Diagnosis Codes - FindACode. Keywords G60.3 Idiopathic progressive neuropathy - ICD-10-CM Diagnosis. Define progressive neuropathy. progressive neuropathy synonyms, progressive neuropathy; Progressive non-fluent aphasia; Progressive nonfluent aphasia; Polyneuropathy information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis. For the past eight 1 2 months I have had peripheral sensory neuropathy. I have had numbness 24 hours a day in my legs and fingers. Within the past two months this has. Progressive neuropathy, idiopathic; ICD-9-CM Volume 2 Index entries containing back-references to 356.4 Neuropathy, neuropathic (see also Disorder, nerve) 355.9. See also neuropathy. acute idiopathic polyneuropathy. hypothyroid polyneuropathy. a progressive lower motor neuron disease associated with hypothyroidism. Abstract. Patients with a progressive disabling idiopathic axonal neuropathy could have a potentially treatable immune mediated neuropathy. Summary Polyneuropathy idiopathic progressive is reported only by a few people who take Synthroid. We study 89,107 people who have side effects while taking. WebMD's guide to the causes and types of peripheral neuropathy. Peripheral neuropathy — Comprehensive overview covers diagnosis, causes and treatment of this often painful disorder. Page 8- Idiopathic Peripheral Neuropathy Peripheral Neuropathy Welcome to the NeuroTalk Communities! You are currently viewing our boards as a guest. Chronic inflammatory polyneuropathy is one cause of damage to nerves outside the brain or spinal cord (peripheral neuropathy). Polyneuropathy means several. Slowly progressive ataxia and neuropathy, with no significant family history, leads one to consider autosomal recessive ataxias as potential causes. Enter the code or terms for which you want to search (like '823' or 'lymphoma') Search Chronic idiopathic axonal polyneuropathy is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability. I have had CFIDS for 28 years. The last several years I've had increasing neurological problems. I wonder if I could have progressive idiopathic peripheral neuropathy. Patients with a progressive disabling idiopathic axonal neuropathy could have a potentially treatable immune mediated neuropathy. To evaluate whether progressive. ICD-10 G60.3 is idiopathic progressive neuropathy (G603). This code is grouped under diagnosis codes for diseases of the nervous system. Question Hi. I'm Janice. I have peripheral neuropathy, but the doctor says it's idiopathic. What does that mean? Answer Janice, that is a great question. ICD 10 Clinical Modification Diagnosis Code G60.3 for Idiopathic progressive neuropathy Polyneuropathy idiopathic progressive, Ask a Doctor about Polyneuropathy What is Multifocal Motor Neuropathy? Multifocal motor neuropathy is a progressive muscle disorder characterized by muscle weakness in the hands, with. Peripheral neuropathy — Comprehensive overview covers diagnosis, causes and treatment of this often painful disorder. CIDP (or Chronic Inflammatory Demyelinating Polyneuropathy) is one of several inflammatory Peripheral Neuropathy causes. Learn about its associated signs and treatment. 270 hands,and axonal polyneuropathy on electrophysiolog-ical examination [22,27,40,44,60].Patients remain am-bulatory without severe disability or handicap [22, 27, - idiopathic progressive neuropathy icd 9 - signs and symptoms of polyneuropathy - what is polyneuropathy in diabetes - idiopathic sensory neuropathy - peripheral neuropathy in feet - stages of cidp - what is polyneuropathy disease	4	11	0	0	0	10	0.568115	10	1,721
General description of procedure, equipment, technique Ventricular assist devices (VAD) can be used to support the left ventricle (LVAD), right ventricle (RVAD), or both ventricles (BiVAD). VAD were developed to support patients with circulatory failure with failing organ systems and/ or near death to sustain life until cardiac transplantation became feasible. The refinement of the devices and the improvements in outcomes has led to the widespread use of LVAD to support patients with advanced heart failure indefinitely. A durable VAD is implanted by a cardiac surgeon via a sternotomy and has a percutaneous driveline or connection to provide power and control of the device. Current devices weigh 1 lb or less and can deliver up to 10 L of flow per minute. The HeartMate II (Thoratec Corp) is FDA approved for bridge to transplant and destination therapy. It is implanted by a sternotomy and is a continuous flow LVAD (Figure 1, Figure 2). VADs do not have sealed batteries contained internally, hence the need for the driveline to provide electrical power; the potential for infection remains a limitation of VAD. All VADs require anticoagulation and antiplatelet therapy; bleeding and stroke are complications. Indications and patient selection Left ventricular assist devices (LVAD) are approved for two indications: bridge to transplant (BTT) and destination therapy (DT). The Food and Drug Administration (FDA) approves devices based upon these two indications. Although not designated by Centers for Medicare and Medicaid Services (CMS), many patients fall into an undifferentiated category or “bridge to decision.” Essentially, a bridge to decision patient is quite ill with comorbidities that might preclude an immediate decision regarding transplant candidacy. With successful LVAD support, many patients improve and become eligible for cardiac transplantation consideration. VAD BTT are considered for patients listed for cardiac transplantation that require hemodynamic support. Generally patients considered for VAD BTT are inotrope dependent and/or have cardiorenal limitations (hypotension and renal insufficiency), negating the use of neurohormonal antagonists. Patients intolerant of ACE inhibitors and beta blockers are generally quite ill and referred for LVAD consideration. Patients with pulmonary hypertension may also be considered for an LVAD, which can fully unload the left ventricle and reduce the pulmonary artery pressure. Patients with advanced heart failure considered for LVAD as DT are generally ineligible for cardiac transplantation due to age and or comorbidities. LVAD is a potentially life-sustaining therapy that will enhance the quality of life in patients otherwise unlikely to survive more than 6 to 12 months. In clinical trials, about 75% of patients were inotrope dependent. In properly selected patients, LVAD will prolong life and improve quality of life dramatically compared to medical therapy. Patient selection is based upon standard criteria established by CMS and published guidelines. (http://www.ishlt.org/ContentDocuments/JHLT_Feb13_MCS_Guidelines.pdf) Approved for heart transplant by a local selection committee and active or planned UNOS (United Network of Organ Sharing) listing of patient. Suitability for cardiac transplantation is based upon functional limitation and is generally evaluated with a metabolic stress test and other variables to estimate risk. Brain natriuretic peptide level, frequent hospitalizations for heart failure, and Seattle Heart Failure Model score are commonly used to predict mortality and to guide suitability for listing for transplantation. Stable outpatients are infrequently advised to consider LVAD BTT. An LVAD is often needed to sustain the patient’s life and end organ function until a heart donor is available. Examples include: Failing end organs with renal and hepatic dysfunction Inability to tolerate BB or ACE Often inotropic dependent Poor functional capacity and quality of life Frequent hospitalization for heart failure Various risk scores have been developed to estimate the mortality rate before hospital discharge after LVAD implant. The HeartMate II risk score multivariate predictors of 90-day mortality include age, albumin, creatinine, international normalized ration (INR), and implanting center volume. Per CMS definitions, the patient should be ineligible for heart transplant: often due to chronologic age and comorbid conditions. Typically, approximately 75% of patients are on continuous inotropes. NYHA class IV on maximal medical therapy for >60 to 90 days. Ejection fraction <25% Most with peak oxygen consumption <12 ml/kg/min. The patient has the appropriate body size (≥1.5 m2) to support the LVAD implantation. Use of INTERMACS levels guides patient selection http://www.uab.edu/intermacs/ Patients are assigned an INTERMACS level, 1-7, with a modifier for uncontrolled ventricular arrhythmias. INTERMACS level 1 patients, which are the sickest (“crash and burn”), also have the highest 30-day mortality after LVAD. Most patients undergoing LVAD today are INTERMACS level 2 and 3, which indicates chronic inotropic support. Risk scores have been developed to examine, low/medium/high risk patients using common clinical variables that predict mortality in hospitals postoperation until discharge. In general avoid critically ill patients with frank or impending renal, hepatic, or pulmonary failure due to elevated mortality rates. Patients with multiorgan failure are very high risk. Patients with severe heart failure, on a ventilator, with impending renal failure with elevated MELD scores and right heart failure are complex to care for and have increased mortality. For patients in shock and or multiorgan failure, ECMO or a percutaneous VAD such as Tandem Heart or Impella may be used. A heart failure specialist will help determine whether the patient should go to the OR for heart transplant, versus LVAD, versus no surgical therapy due to risk. Contraindications to LVAD therapy are complex and require consultation with a heart failure cardiologist and an experienced LVAD surgeon. Inability to provide informed consent Inability to receive prolonged anticoagulation and antiplatelet therapy ESRD requiring renal replacement therapy Other irreversible life-limiting disease (e.g., malignancy, advanced emphysema, cirrhosis with elevated MELD score) Cerebrovascular disease and increased risk of stroke based upon neurologic consultation Inability or refusal to receive blood products Inability to be educated regarding VAD operation and or be compliant with a complex medical regimen Prohibitive and irreversible right heart failure Prohibitive and irreversible pulmonary hypertension and elevated pulmonary vascular resistance Inability to be placed on cardiopulmonary bypass due to aortic calcification and or additional anatomic limitations Lack of support from family and others to comply with a complex treatment regimen Lack of resources (e.g., insurance) to provide for ongoing medical care including dressing changes and follow-up care after VAD implant Details of how the procedure is performed VAD implantation is performed by a cardiac surgeon using standardized surgical techniques. Most often a full incision median sternotomy is required. The patient is placed on cardiopulmonary bypass and typically cannulated after the chest is opened. An apical core is removed from the left ventricle (LV) and the inflow cannula is inserted in the LV and sutured in place. The outflow graft is an end to side anastomosis to the descending aorta. The VAD is placed in the chest or in the abdominal cavity, depending on the size of the VAD used. A driveline is tunneled and exits via the skin in the right or left lower quadrant. The batteries and controller are external to the body. Interpretation of results Recent published clinical trials and postmarket approval studies have clearly demonstrated the outcomes in patients implanted with VAD. Additionally, the INTERMACS registry contains outcomes from over 145 participating sites and over 9,200 subjects enrolled. INTERMACS is a prospective registry that collects clinical data including follow-up early postoperation and every 6 months. Major outcomes, including death, explant, rehospitalization, and adverse events, are publicly reported at the website. Quality of life and level of function is also collected and reported. BTT clinical trials typically require 6 months of follow-up and assess mortality, survival to transplant, explant, device failure, and major adverse events, including stroke, device failure, infection, and bleeding. DT clinical trials typically require 24 months of follow-up and they assess mortality, explant, device failure, and major adverse events. A smaller percentage of patients in DT clinical trials receive a transplant, as inclusion criteria specifies that those enrolled are not eligible for heart transplantation. The FDA requires postmarket approval studies such that a cohort of patients implanted after approval are followed and outcomes are compared to a reference group of patients implanted with a previously approved VAD. Typically the “control” group is obtained from the INTERMACS registry and postapproval trials are nonrandomized. These postapproval trials ensure that outcomes are equal or exceed the outcomes observed when the VAD was initially implanted in the context of a clinical trial. Outcomes (applies only to therapeutic procedures) Survival with VAD will be reviewed for BTT, DT clinical trials, and the INTERMAC registry. Expected survival rate for patients with LVAD BTT at 6 months is approximately 90% and the 30-day survival rate is approximately 95%. Competing outcomes for patients implanted with the HeartMate II LVAD BTT in a postmarket approval study at 6 months were: 90% transplanted, recovery, or ongoing support Expected survival with HeartMate II continuous flow LVAD DT at 12 months and 24 months are 68% and 58%, respectively. Major adverse events include disabling stroke, and reoperation to repair or replace VAD. In the HeartMate II DT trial, the following adverse events were observed with the continuous flow LVAD: Pump replacement 9%; 0.06 events/patient-year Ischemic and hemorrhagic stroke 18%; 0.13 events/patient-year LVAD thrombosis 4%; 0.02 events/patient-year Current INTERMACS registry data for VAD continuous flow survival in a cohort of 5,436 patients: 80%: 12 months survival 70%: 24 months survival 59%: 36 months survival 47%: 48 months survival The INTERMACS registry demonstrates that the majority of LVAD recipients have improved ability to perform usual activities of daily living as shown in the Figure 4. The HeartMate II experience has shown an increase in the 6-minute walk test of +156 meters, which is remarkable. Patient self-reported measures including the Minnesota Living with Heart Failure and the Kansas City Cardiomyopathy Scores both favorably improved. Although survival, functional capacity, and quality of life improve challenges remain as many patients are readmitted to the hospital and suffer complications, including GI bleeding, infections, and heart failure (Figure 3, Figure 4). Alternative and/or additional procedures to consider No alternatives to VAD therapy except cardiac transplantation or palliative care. Patients considered for VAD in general have very poor quality of life and expected mortality in excess of 30% at 12 months or higher. The ACC/AHA heart failure guidelines suggest for stage D heart failure: cardiac transplantation, LVAD as BTT or DT, palliative inotropes, or hospice/palliative care. The projected mortality for heart failure patients on chronic inotropic therapy is approximately 50% at 6 months and over 90% at 1 year. In addition, only cardiac transplantation and VAD therapy can provide a remarkable improvement in functional capacity (improved 6-minute walk) and quality of life. Complications and their management Complications after VAD remain challenging. All patients require anticoagulation and antiplatelet agents. All devices have a percutaneous drive line to link the implanted pump with the power supply and the controller. Major complications include: Ischemic and hemorrhagic stroke Right heart failure The INTERMACS registry indicates the only 30% of patients are free of infection, bleeding, stroke, device malfunction, and death 12 months after device implant (Figure 5). Infection: driveline infection is well controlled with mobilization of the drive line at the time of implant, patient education regarding dressing changes, and being careful to maintain the integrity of the driveline interface with the skin by immobilizing the driveline. Experienced centers report driveline infection rates of less than 5% to 10%. A serious driveline infection can spread and involve the pump pocket, often leading to bacteremia and the need for chronic antibiotic suppression. Rarely a device change or urgent transplant is required for uncontrolled pump pocket infection and bacteremia. Incision and drainage is uncommon but can be effective for recurrent infections. GI Bleeding: 20% of continuous flow VAD patients experience bleeding that can be epistaxis, and upper or lower GI bleeding. There is a propensity for bleeding from AV malformations. This may be related to a relative lack of pulsatile flow. When transfusions are required, both aspirin and warfarin are stopped and an upper and lower endoscopy is performed for GI bleeding. A negative capsule endoscopy is sometimes required. Occasionally angiography and interventional radiology may be consulted to control bleeding. Patients with continuous flow LVAD may develop acquired reduction in Von Willebrand multimers and hence be at risk for bleeding. Right heart failure: It is important to carefully select patients for LVAD expected to have improvement in right ventricular function. If a patient requires inotropic therapy to support RV function >14 days after LVAD implant, increased mortality is expected. A variety of risk scores to predict RV failure after LVAD have been developed. In general a patient with severe RV dysfunction assessed by echo, CVP >20 cm H20, and severe tricuspid regurgitation is of concern for RV failure. Alternatives are to consider BIVAD support or a total artificial heart. Patients that go back to the operating room for an RVAD have increased mortality. A small percentage of patients that have done well for many months will return with volume overload and poor RV function. An echo and right heart catheter should be performed to assess the severity of the hemodynamic abnormality and to determine if the RV failure can be ameliorated by additional unloading of the left ventricle. In general, most patients respond to intravenous diuretics. A small percentage may require chronic inotropic therapy in addition to the LVAD. Device malfunction is rare. If alarms sound immediate, interrogation by a VAD coordinator is required. Very rarely a patient will require urgent surgery for pump replacement for a catastrophic device malfunction. Device thrombosis: This is a spectrum usually characterized by hemolysis, abnormal pump function, inadequate unloading of the left ventricle, and clinical heart failure. The published clinical trials indicate this occurs in <5% to 10% of cases treated with adequate anticoagulation. If a patient presents with heart failure, check for hemolysis and request a VAD interrogation by the VAD coordinator. If hemolysis and anemia are present, the VAD should be checked for alarms and the patient should be very carefully assessed for evidence of heart failure/inadequate unloading of the LV. Rarely device thrombosis will require urgent VAD replacement. Most cases are treated with heparin and intensified anticoagulation and improve. Stroke is a devastating complication and can be ischemic or hemorrhagic. In general adequate anticoagulation is mandated to reduce the risk of VAD thrombosis and stroke. Hemorrhagic stroke is often fatal. Any change in neurologic function is a medical emergency and requires immediate imaging of the brain and consultation with the stroke service. Anticoagulation is generally held until brain imaging is completed and evaluated. No specific VAD parameters need to be changed when stroke occurs. Patients with known extensive cerebrovascular disease and or prior SAH may be at increased risk and should be carefully assessed by a neurologist before considering VAD therapy. Emerging data suggests that meticulous blood pressure control is also important to reduce the risk of stroke and that the mean arterial pressure (MAP) should be <85 mm Hg. After prolonged VAD support (>18 months), the leaflets of the aortic valve may become fused and lead to the development of aortic insufficiency. Any VAD patient that has a change in their condition from a cardiac perspective should undergo a careful echo assessment. What’s the evidence? Miller, LW. “Use of a continuous-flow device in patients awaiting heart transplantation”. N Engl J Med,. vol. 357. 2007. pp. 885-96. (This report describes the pivotal clinical trial providing evidence for the U.S. FDA approval of the continuous flow LVAD as a bridge to transplant and demonstrates the advances compared to the pulsatile LVAD.) Slaughter, MS, Rogers, JG, Milano, CA. “HeartMate II Investigators. Advanced heart failure treated with continuous-flow left ventricular assist device”. N Engl J Med. vol. 361. 2009. pp. 2241-51. (This report describes the pivotal clinical trial providing evidence for the U.S. FDA approval of the continuous flow LVAD as chronic destination therapy and demonstrates the advances compared to the pulsatile LVAD.) Rogers, JG. “Continuous flow left ventricular assist device improves functional capacity and quality of life of advanced heart failure patients”. J Am Coll Cardiol. vol. 55. 2010. pp. 1826-34. (This review describes the impressive improvements in NYHA class, quality of life, and 6-minute walk distance with the use of a continuous flow LVAD.) Starling, RC. “Results of the post-U.S. Food and Drug Administration-approval study with a continuous flow left ventricular assist device as a bridge to heart transplantation: a prospective study using the INTERMACS (Interagency Registry for Mechanically Assisted Circulatory Support)”. J Am Coll Cardiol. vol. 57. 2011. pp. 1890-8. (This report demonstrates that results with the continuous flow LVAD post-BTT approval after wide use are comparable or superior to outcomes in the pivotal clinical trial.) Aaronson, KD. “Use of an intrapericardial, continuous-flow, centrifugal pump in patients awaiting heart transplantation”. Circulation,. vol. 125. 2012. pp. 3191-200. (This report reviews the pivotal clinical trial comparing the HVAD (centrifugal pump) with the axial flow LVAD in a bridge to transplant clinical trial.) Matthews, JC. “The right ventricular failure risk score a pre-operative tool for assessing the risk of right ventricular failure in left ventricular assist device candidates”. J Am Coll Cardiol. vol. 51. 2008. pp. 2163-72. (Predicting adequate RV function post-LVAD is challenging; this study provides a risk assessment tool to assess the severity of RV function and outcomes post-LVAD.) Kirklin, JK, Naftel, DC, Kormos, RL. “Fifth INTERMACS annual report: risk factor analysis from more than 6,000 mechanical circulatory support patients”. J Heart Lung Transplant. vol. 32. 2013. pp. 141-56. (A wealth of LVAD information is contained in this INTERMACS report of the U.S. registry with over 6,000 implants.) Cowger, J, Sundareswaran, K, Rogers, JG. “Predicting survival in patients receiving continuous flow left ventricular assist devices: the HeartMate II risk score”. J Am Coll Cardiol. vol. 61. 2013. pp. 313-21. (The derivation and validation of the HeartMate II Risk score is a valuable article to review to help in selection of patients for LVAD.) Park, SJ, Milano, CA, Tatooles, AJ. “Outcomes in advanced heart failure patients with left ventricular assist devices for destination therapy”. Circ Heart Fail. vol. 5. 2012. pp. 241-8. (This report demonstrates that results with the continuous flow LVAD post-DT approval after wide use are comparable or superior to outcomes in the pivotal clinical trial in destination therapy patients.) Russell, SD, Rogers, JG, Milano, CA. “Renal and hepatic function improve in advanced heart failure patients during continuous-flow support with the HeartMate II left ventricular assist device”. Circulation. vol. 120. 2009. pp. 2352-7. (This impressive article chronicle that end organ function will improve in critically ill patients successfully treated with continuous flow LVAD therapy.) Copyright © 2017, 2013 Decision Support in Medicine, LLC. All rights reserved. No sponsor or advertiser has participated in, approved or paid for the content provided by Decision Support in Medicine LLC. The Licensed Content is the property of and copyrighted by DSM. - General description of procedure, equipment, technique - Indications and patient selection - Details of how the procedure is performed - Interpretation of results - Outcomes (applies only to therapeutic procedures) - Alternative and/or additional procedures to consider - Complications and their management - What’s the evidence?	2	10	0	0	0	1	0.740556	1	4,586
Device Therapy for Patients with Heart Failure Device Therapy for Patients with Heart Failure Implantable cardiac defibrillator An implantable cardiac defibrillator (ICD) is an electronic device that constantly monitors the patient’s heart rhythm. When the device detects certain abnormal heart rhythms, it delivers a small shock to the heart muscle to restore a normal heart rhythm. The shock will be brief and may feel very uncomfortable. Studies show that an ICD can reduce the risk of sudden cardiac arrest (SCA). Heartmate II implanted Sudden cardiac arrest Sudden cardiac arrest (SCA, sometimes called sudden cardiac death [SCD]) is a sudden, unexpected death caused by a loss of heart function due to an abnormal heart rhythm (arrhythmia). The ventricles (lower chambers of the heart) flutter or quiver (ventricular fibrillation) or the heart beats dangerously fast (ventricular tachycardia), and blood is not delivered to the rest of the body. A person suffering SCA will first lose consciousness, and death will follow. SCA is responsible for 30 percent of deaths among patients with mild to moderate heart failure. Patients with heart failure are 6 to 9 times more likely than the general population to experience ventricular arrhythmias that can lead to SCA. Because you are at risk for SCA, talk to your family members so they understand your condition and the importance of seeking immediate care in the event of an emergency. Family members and friends of those at risk for SCA should know how to perform cardiopulmonary resuscitation (CPR). Although more than 90 percent of people who suffer SCA die, it can be treated and reversed if the person receives immediate emergency action. Survival is as high as 90 percent if treatment is initiated within the first minutes after arrhythmia begins, but the survival rate decreases by about 10 percent each minute the patient waits for treatment. Prevention of SCA and death includes placement of an implantable cardioverter defibrillator (ICD). Emergency treatment for SCA includes CPR and defibrillation. Cardiac resynchronization therapy Some patients with systolic heart failure can benefit from cardiac resynchronization therapy (CRT), also referred to as biventricular pacing, which helps the heart’s lower chambers (ventricles) contract normally. About 30% of patients with heart failure have electrical problems in one or more ventricles that cause delays in ventricular muscle contraction (pumping). When the ventricular muscles do not contract together, the amount of oxygen-rich blood the heart normally pumps to the body’s organs and muscles is decreased. Pumping delays make heart failure worse (increased fatigue, shortness of breath and other symptoms) and can increase the risk of sudden cardiac arrest (SCA) and death from heart failure. CRT involves the implantation of a pacemaker with 3 leads (wires) that keeps the left and right ventricles pumping together. The pacemaker sends small, painless electrical impulses to the heart muscle through the leads that cause the right and left ventricles to be stimulated at the same time. Some patients with heart failure may benefit from combination therapy with CRT and an ICD. The use of a combination device (CRT-D) keeps the left and right ventricles of the heart beating together and can shock the heart back into normal rhythm if ventricular tachycardia or ventricular fibrillation occurs. CRT, with or without an ICD, improves symptoms in about half of all patients with heart failure who still have symptoms after exhausting all medication options. CRT improves survival, quality of life, heart function, the ability to exercise, and helps decrease hospitalizations in some patients with systolic heart failure. Internal monitoring devices Many internal cardiac devices have monitoring features and software so your doctor or nurse can monitor not only your heart rhythm, but also your heart function and activity level. You may be asked to use a telephone to send information from your device to a computer server so your condition can be monitored. Or, your device may use a handheld personal digital assistant (PDA) to send data to your healthcare provider. Other types of internal monitoring devices are emerging. For example, one device that is the size of a small paper clip can be placed in a blood vessel near your heart to monitor pressure in the blood vessel and left ventricle. The information can be sent to a computer server so your doctor or nurse can remotely review your heart function and give you treatment advice. These and other devices may let your healthcare team watch trends in your heart function that could lead to changes in the medications you take and reinforce the importance of a healthy diet, exercise, medication and monitoring to stay healthy. © Copyright 1995-2011 The Cleveland Clinic Foundation. All rights reserved. Can't find the health information you’re looking for? This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 12/10/2010…#12906	2	6	1	0	0	0	0.903798	1	1,075

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