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Individual differences \| Methods \| Statistics \| Clinical \| Educational \| Industrial \| Professional items \| World psychology \| Idiopathic inflammatory demyelinating diseases (IIDDs), sometimes known as borderline forms of multiple sclerosis, is a collection of multiple sclerosis variants, sometimes considered different diseases, but considered by others to form a spectrum differing only in terms of chronicity, severity, and clinical course. Diseases included in this categoryEdit The list of these diseases depends of the author, but usually are included: - Standard multiple sclerosis, the most known and extended variant. - Optic-spinal MS, or Opticospinal, variant which often include visual symptoms and have a more severe course than typical MS. Though multiple scars (scleroses) are present in CNS, and sometimes Poser criteria classifies it as clinically definite multiple sclerosis, currently is considered outside the scope of Multiple Sclerosis and inside the scope of Devic's disease, though is not sure if this applies to all cases. - Devic's disease, currently considered a separate disease - Acute disseminated encephalomyelitis or ADEM, a closely related disorder in which a known virus or vaccine triggers autoimmunity against myelin. - Acute hemorrhagic leukoencephalitis, possibly a variant of Acute disseminated encephalomyelitis - Balo concentric sclerosis, an unusual presentation of plaques forming concentrenic circles, which can sometimes get better spontaneously. - Schilder disease or diffuse myelinoclastic sclerosis: is a rare disease that presents clinically as a pseudotumoural demyelinating lesion; and is more common in children. - Marburg multiple sclerosis, an aggressive form, also known as malignant, fulminant or acute MS. - Tumefactive Multiple sclerosis: lesions whose size is more than 2 cm, with mass effect, oedema and/or ring enhancement - Solitary sclerosis: This variant has been recently proposed (2012) by Mayo Clinic researches. It is defined as isolated demyelinating lesions which produce a progressive myelopathy similar to primary progressive MS. Some others sources include in the list also other conditions associated with the presence of the scleroses As MS is an active field for research, the list is not closed or definitive. For example, some diseases like Susac's syndrome (MS has an important vascular component), Myalgic encephalomyelitis (aka Chronic fatigue syndrome) or autoimmune variants of peripheral neuropathies like Guillain-Barré syndrome or Progressive inflammatory neuropathy could be included assuming the autoimmune model. Also Leukodystrophy (which see) and its sub-conditions: Adrenoleukodystrophy and Adrenomyeloneuropathy could be in the list. Venous induced demyelination has also been proposed as a hypothetical MS variant produced by CCSVI. Clinical courses inside standard MSEdit Also inside standard MS different clinical courses can be separated. Some authors also think that primary progressive multiple sclerosis should be considered a different entity from standard MS. Others maintain the opposite. In any case, the lesions in PPMS can be diffused instead of the normal focal ones. Finally, also a dual classification of these diseases has been proposed, according to the shape of edges of the scars, in MS-like and ADEM-like Subclinical, preclinical, CIS and CDMSEdit The first manifestation of MS is the so called Clinically isolated syndrome, or CIS, which is the first isolated attack. The current diagnosis criteria for MS does not allow doctors to give an MS diagnosis until a second attack takes place. Therefore the concept of "clinical MS", for a MS that can be diagnosed has been developed. Until MS diagnosis has been established, nobody can tell whether the disease dealing with is MS. Cases of MS before the CIS are sometimes found during other neurological inspections and are referred to as subclinical MS. Preclinical MS refers to cases after the CIS but before the confirming second attack. After the second confirming attack the situation is referred to as CDMS (clinically defined multiple sclerosis). Aggressive multiple sclerosisEdit Relapsing-Remitting MS is considered aggressive when the frequency of exacerbations is not less than 3 during 2 years. Special treatment is often considered for this subtype. Pediatric and pubertal MSEdit MS cases are rare before puberty, but they can happen. Whether they constitute a separate disease is still an open subject. Anyway, even this pubertal MS could be more than one disease, because early-onset and late-onset have different demyelination patterns Controversy for the definitionEdit Clinical vs. pathological definitionsEdit Probably the most implicitly used definition can be found into the McDonald criteria proposal, which is the currently considered as the gold standard for MS diagnosis. These authors state that "MS is a clinical entity and therefore should be diagnosized with clinical and paraclinical criteria" . Currently the McDonald criteria are considered a clinical definition of MS. Some other authors consider MS as a pathological entity instead, and propose a pathological definition. According to Hans Lassmann, the pathological definition should be preferred because clinical definitions have problems with differential diagnosis. Of course, using a pathological definition would not prevent performing clinical diagnosis, but would require to calibrate any diagnosis criteria against it. McDonald et al. do not agree with this, and they remark the clinical character of MS. They state that "Whereas it might be said that the only proved diagnosis of MS can be made upon autopsy, or occasionally upon biopsy, where lesions typical of MS can be directly detected through standard histopathological techniques, MS is essentially a clinical problem and can be diagnosed using clinical and paraclinical criteria"" At this moment, both definitions are currently used by each of their supporters and the relationship among them is not well documented. The list of diseases included in the MS-spectrum is not closed because no formal definition of MS is normally given. For example, the World Health Organization does not give any explicit definition with ICD-10 MS entry . In ICD-9 it used to say "chronic disease characterized by presence of numerous areas of demyelination in the central nervous system with symptoms such as weakness, incoordination, paresthesis, and speech disturbances". The Unified Medical Language System also gives very loose definitions of MS . The Medline medical dictionary defines it as "a demyelinating disease marked by patches of hardened tissue in the brain or the spinal cord and associated especially with partial or complete paralysis and jerking muscle tremor" . It uses the anatomical hallmark of the lesions, but also imposes the existence of clinical problems (paralysis and jerking muscle tremor) Assuming a definition as weak as the previous ones, several diseases could be included inside the MS-spectrum. Other authors use a definition for MS based in its clinical course . Clinical definitions refer to the lesions and their location, but not to the nature of the lesions and this kind of definitions are potentially heterogeneous. As with any definition, patients have to be considered non-affected until the definition conditions are satisfied. - ↑ Fontaine B (2001). [Borderline forms of multiple sclerosis]. Rev. Neurol. (Paris) 157 (8-9 Pt 2): 929–34. - ↑ Wingerchuk DM, Lucchinetti CF (2007). Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis. Curr. Opin. Neurol. 20 (3): 343–50. - ↑ Poser CM, Brinar VV (October 2007). Disseminated encephalomyelitis and multiple sclerosis: two different diseases - a critical review. Acta Neurol Scand. 116 (4): 201–6. - ↑ Weinshenker B, Miller D (1998). "Multiple sclerosis: one disease or many?" Thompson AB, Siva A, Kesselring J Frontiers in Multiple Sclerosis, 2nd, 37–46, London: Taylor & Francis Group. - ↑ Hartung HP, Grossman RI (May 2001). ADEM: distinct disease or part of the MS spectrum?. Neurology 56 (10): 1257–60. - ↑ Kalanie H, Kholghie Y, Shamsai GR, Ghorbani M (October 2008). Opticospinal multiple sclerosis in Iran. J Neurol Sci. 276 (1-2): 130–2. - ↑ Kira J (October 2008). Neuromyelitis optica and asian phenotype of multiple sclerosis. Ann NY Acad Sci. 1142 (1): 58–71. - ↑ (2009). Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese.. Multiple sclerosis (Houndmills, Basingstoke, England) 15 (7): 834–47. - ↑ Garrido C, Levy-Gomes A, Teixeira J, Temudo T (2004). [Schilder's disease: two new cases and a review of the literature]. Revista de neurologia 39 (8): 734–8. - ↑ Afifi AK, Bell WE, Menezes AH, Moore SA (1994). Myelinoclastic diffuse sclerosis (Schilder's disease): report of a case and review of the literature. J. Child Neurol. 9 (4): 398–403. - ↑ Lucchinetti CF, Gavrilova RH, Metz I, et al. (July 2008). Clinical and radiographic spectrum of pathologically confirmed tumefactive multiple sclerosis. Brain 131 (Pt 7): 1759–75. - ↑ Given CA, Stevens BS, Lee C (1 January 2004). The MRI appearance of tumefactive demyelinating lesions. AJR Am J Roentgenol 182 (1): 195–9. - ↑ Schmalstieg WF, Keegan BM, Weinshenker BG (Feb 2012). Solitary sclerosis: progressive myelopathy from solitary demyelinating lesion. Neurology 78 (8): 540–4. - ↑ Paul O'Connor, James Marriott, Differential Diagnosis and Diagnostic Criteria for Multiple Sclerosis: Application and Pitfalls - ↑ Minagar A, Jy W, Jimenez JJ, Alexander JS (2006). Multiple sclerosis as a vascular disease. Neurol. Res. 28 (3): 230–5. - ↑ http://health.nytimes.com/health/guides/disease/chronic-fatigue-syndrome/overview.html?WT.z_gsac=1 ; "The following test results... are seen consistently...: Brain MRI showing swelling in the brain or destruction of part of the nerve cells (demyelination) - ↑ Vukusic S, Confavreux C (2003). Primary and secondary progressive multiple sclerosis. J. Neurol. Sci. 206 (2): 153–5. - ↑ Dressel A, Kolb AK, Elitok E, Bitsch A, Bogumil T, Kitze B, Tumani H, Weber F (2006). Interferon-beta1b treatment modulates cytokines in patients with primary progressive multiple sclerosis. Acta Neurol. Scand. 114 (6): 368–73. - ↑ Ebers GC (2004). Natural history of primary progressive multiple sclerosis. Mult. Scler. Suppl 1 (3): S8–13; discussion S13–5. - ↑ Zwemmer JN, Bot JC, Jelles B, Barkhof F, Polman CH (April 2008). At the heart of primary progressive multiple sclerosis: three cases with diffuse MRI abnormalities only. Mult Scler. 14 (3): 428–30. - ↑ Poser CM, Brinar VV (2004). The nature of multiple sclerosis. Clinical neurology and neurosurgery 106 (3): 159–71. - ↑ Hakiki B, Goretti B, Portaccio E, Zipoli V, Amato MP. (2008). Subclinical MS: follow-up of four cases. European Journal of Neurology 15 (8): 858–61. - ↑ Lebrun C, Bensa C, Debouverie M, et al. (2008). Unexpected multiple sclerosis: follow-up of 30 patients with magnetic resonance imaging and clinical conversion profile. J Neurol Neurosurg Psychiatry 79 (2): 195–198. - ↑ Frisullo G, Nociti V, Iorio R, et al. (December 2008). The persistency of high levels of pSTAT3 expression in circulating CD4+ T cells from CIS patients favors the early conversion to clinically defined multiple sclerosis. J Neuroimmunol. 205 (1-2): 126–34. - ↑ (2009). Combined therapy of aggressive remitted multiple sclerosis with mitoxantrone in combination with copaxone. Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova / Ministerstvo zdravookhraneniia i meditsinskoi promyshlennosti Rossiiskoi Federatsii, Vserossiiskoe obshchestvo nevrologov [i] Vserossiiskoe obshchestvo psikhiatrov 109 (12): 76–9. - ↑ Chabas D, Castillo-Trivino T, Mowry EM, Strober JB, Glenn OA, Waubant E (September 2008). Vanishing MS T2-bright lesions before puberty: a distinct MRI phenotype?. Neurology 71 (14): 1090–3. - ↑ McDonald WI (2001). Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann. Neurol. 50 (1): 121–7. - ↑ H. Lassmann, Acute disseminated encephalomyelitis and multiple sclerosis, DOI:10.1093/brain/awp342 , - ↑ McDonald WI, Compston A, Edan G, et al. (2001). Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann. Neurol. 50 (1): 121–7. \|Signs and symptoms\| \|Diagnosis and evolution following\| \|This page uses Creative Commons Licensed content from Wikipedia (view authors).\|	2	26	0	0	0	1	0.864037	1	3,050
Genetic Testing for Hereditary Hearing Loss Genetic testing for hereditary hearing loss mutations (GJB2, GJB6, and other hereditary hearing loss-related mutations) in individuals with hearing loss to confirm the diagnosis of hereditary hearing loss (see Policy Guidelines) may be considered medically necessary. Preconception genetic testing (carrier testing) for hereditary hearing loss mutations (GJB2, GJB6, and other hereditary hearing loss-related mutations) in parents may be considered medically necessary when at least one of the following conditions has been met: Genetic testing for hereditary hearing loss mutations is considered investigational for all other situations, including, but not limited to, testing in patients without hearing loss (except as addressed in Related Policies, e.g., 12.04.305 Preimplantation Genetic Testing in Embryos) Hereditary hearing loss can be classified as syndromic or non-syndromic. The definition of non-syndromic hearing loss (NSHL) is hearing loss that is not associated with other physical signs and symptoms at the time of hearing loss presentation. It is differentiated from syndromic hearing loss, which is hearing loss associated with other signs and symptoms characteristic of a specific syndrome. Physical signs of a syndrome often include dysmorphic changes in the maxillofacial region and/or malformations of the external ears. Malfunction of internal organs may also be part of a syndrome. The physical signs can be subtle and easily missed on physical exam; therefore exclusion of syndromic findings is ideally done by an individual with expertise in identifying dysmorphic physical signs. The phenotypic presentation of NSHL varies, but generally involves the following features: This policy primarily focuses on the use of genetic testing to identify a cause of suspected hereditary hearing loss. The diagnosis of syndromic hearing loss may be able to be made on the basis of associated clinical findings. However, at the time of hearing loss presentation, associated clinical findings may not be apparent; furthermore, mutations in certain genetic loci may cause both syndromic and NSHL. Given this overlap, the policy focuses on genetic testing for hereditary hearing loss more generally. Genetic evaluation and counseling should be offered to all patients who are being considered for hereditary hearing loss genetic testing. Genetic evaluation and counseling can help define the familial patterns of inheritance, exclude the presence of syndromic hearing loss, and provide information to patients on the future risk of hereditary hearing loss in offspring. In addition to mutations in the GJB6 and GJB2 genes, there are many less common pathologic mutations found in other genes. Some of these are: ACTG1, CDH23, CLDN14, COCH, COL11A2, DFNA5, DFNB31, DFNB59, ESPN, EYA4, GJB2, GJB6, KCNQ4, LHFPL5, MT-TS1, MYO15A, MYO6, MYO7A, OTOF, PCDH15, POU3F4, SLC26A4, STRC, TECTA, TMC1, TMIE, TMPRSS3, TRIOBP, USH1C, and WFS1 genes. Testing for mutations associated with hereditary hearing loss should be confined to known pathologic mutations. While research studies using genome-wide associations have uncovered numerous single nucleotide polymorphisms and copy number variations associated with hereditary hearing loss, (1,2) the clinical significance of these findings is unclear. For carrier testing, outcomes are expected to be improved if parents alter their reproductive decision making as a result of genetic test results. This may occur through the use of preimplantation genetic testing in combination with in vitro fertilization. Other ways that prospective parents may alter their reproductive choices are to proceed with attempts at pregnancy, or to avoid attempts at pregnancy, based on carrier testing results. Evaluation of a patient with suspected hereditary hearing loss should involve a careful physical exam and family history to assess for associated clinical findings that may point to a specific syndrome or non-syndromic cause of hearing loss (e.g., infectious, toxic, autoimmune, other causes). Consideration should also be given to temporal bone computed tomography scanning in cases of progressive hearing loss and to testing for cytomegalovirus (CMV) in infants with sensorineural hearing loss. If there is not high suspicion for a specific hearing loss etiology, ideally the evaluation should occur in a step-wise fashion. About 50% of individuals with autosomal recessive hereditary hearing loss have mutations in GJB2 gene. In the remainder of patients with apparent autosomal recessive hereditary hearing loss, numerous other genes are implicated. In autosomal dominant hereditary hearing loss, there is not a single identifiable gene that is responsible for most cases. If there is suspicion for autosomal recessive congenital hearing loss, it would be reasonable to begin testing with testing of GJB2 and GJB6. If this is negative, screening for other genetic causes of hearing loss with a multigene panel would be reasonable. An alternative strategy for suspected autosomal recessive or autosomal dominant hearing loss would be to obtain a multigene panel that includes GJB2 and GJB6 as a first step. Given the extreme heterogeneity in genetic causes of hearing loss, either strategy may be considered reasonable.3 Genetic mutations in GJB2, GJB6, and numerous other genes are found in a substantial percent of patients with hereditary hearing loss. The analytic validity of genetic testing for hereditary hearing loss is high. Of all patients with suspected hereditary hearing loss after clinical examination, a substantial minority, in the range of 30% to 60% will be found to have a genetic mutation. False-positive results on mutation testing are expected to be very low. Congenital deafness and childhood-onset hearing loss is caused by genetic mutations in a large percentage of cases. Genetic testing for hearing loss is primarily intended either to determine whether hearing loss is hereditary, or to determine carrier status of parents in order to better define the likelihood of hearing loss in their offspring. Description of disease Hearing loss is a common birth defect. Approximately one of every 500 newborns in developed countries is affected by bilateral, permanent hearing loss of moderate or greater severity (≥40 db). (6) Syndromic hearing loss refers to hearing loss associated with other medical or physical findings, including visible abnormalities of the external ear. Because syndromic hearing loss occurs as part of a syndrome of multiple clinical manifestations, it is often recognized more readily as hereditary in nature. NSHL is defined as hearing loss that is not associated with other physical signs or symptoms. For NSHL, it is more difficult to determine whether the etiology is hereditary or acquired, because by definition, there are no other clinical manifestations at the time of the hearing loss presentation. NSHL accounts for 70% to 80% of genetically determined deafness. (7) Autosomal recessive patterns of inheritance predominate and account for 80% of congenital NSHL. A typical clinical presentation of autosomal recessive NSHL involves the following characteristics: Most of the remaining 20% of patients have an autosomal dominant inheritance pattern, with a small number having X-linked or mitochondrial inheritance. Patients with autosomal dominant inheritance typically show progressive NSHL, which begins in the second through fourth decades of life. (8) Diagnosis of nonsyndromic hearing loss requires an evaluation with appropriate core medical personnel with expertise in the genetics of hearing loss, dysmorphology, audiology, otolaryngology, genetic counseling, and communication with deaf patients. The evaluation should include a family history, as well as a physical examination consisting of otologic examination, airway examination, documentation of dysmorphisms, and neurologic evaluation. (4) However, the clinical diagnosis of nonsyndromic hearing loss is non-specific because there are a number of underlying etiologies, and often it cannot be determined with certainty whether a genetic cause for hearing loss exists. Treatment of congenital and early-onset hearing loss typically involves enrollment in an educational curriculum for hearing impaired persons and fitting with an appropriate hearing aid. In some patients with profound deafness, a cochlear implant can be performed. Early identification of infants with hearing impairment may be useful in facilitating early use of amplification by 6 months of age and early intervention to achieve age-appropriate communication, speech, and language development. (9) Delays in development of hearing treatment have been shown to delay development of communication. The primary method for identification of hearing impairment has been newborn screening with audiometry. Genetic testing has not been proposed as a primary screen for hearing loss. Genetic Mutations in Hereditary Hearing Loss Genes associated with hereditary hearing loss may be associated with an autosomal dominant, autosomal recessive, X-linked, or mitochondrial inheritance pattern. The genetic loci on which mutations associated with hereditary hearing loss are usually found are termed DFN, and hereditary hearing loss is sometimes called DFN-associated hearing loss. DFN loci are named based on their mode of inheritance: DFNA associated with autosomal dominant inheritance; DFNB with autosomal recessive inheritance; and DFNX with x-linked inheritance. Dozens of deafness-associated loci have been identified. (10) There are more than 300 individual mutations known to be associated with NSHL. (11) Two DFN loci commonly associated with hereditary hearing loss are DFNA3 and DFNB1, both of which map to chromosome 13q12. DFNA3-associated hereditary hearing loss is caused by autosomal dominant mutations present in the GJB2 or GJB6 genes. (12) DFNB1-associated hereditary hearing loss are autosomal recessive syndromes in which more than 99% of cases are caused by mutations to the GJB2 gene with less than 1% of remaining cases arising from mutations to GJB6. (13) A list of available tests for genetic mutations at the DFNA3 and DFNB1 loci is given in Table 1. Two of the most commonly mutated genes are GJB2 and GJB6. GJB2 is a small gene with a single coding exon. Mutations of this gene are most common in hereditary hearing loss, causing an estimated 50% of the cases of nonsyndromic hereditary hearing loss. (14) The carrier rate in the general population for a recessive deafness-causing GJB2 mutation is approximately 1 in 33. (6) Specific mutations have been observed to be more common in certain ethnic populations. (15, 16) Mutations in the GJB2 gene will impact expression of the Cx26 connexin protein and almost always cause prelingual, but not necessarily congenital, deafness. (11) Differing mutations to GJB2 can present high phenotypic variation, but it has been demonstrated that it is possible to correlate the type of associated hearing loss with findings on molecular analysis. A systematic review of publications reporting GJB2 mutation prevalence suggests that the overall prevalence of GJB2 mutations is similar around the world, although specific mutations differ. (17) Mutations in the GJB6 gene are the second most common genetic defect in hereditary hearing loss and lead to similar effects on abnormal expression of connexin protein Cx30. However, GJB6 mutations are much less common than mutations in GJB2. Of all the patients with hereditary hearing loss, approximately 3% are found to have a mutation in the GJB6 gene. Table 1. Clinical Characteristics and Testing Methods for GJB2 and GJB6 Mutations at the DFNA3 and DFNB1 Loci Mutation analysis for GJB6 and GJB2 mutations can be performed by Sanger sequencing analysis of individual genes. This method has a high degree of validity and reliability but is limited by the ability to sequence one gene at a time. With Sanger sequencing, the gene with the most common mutations is generally sequenced first, followed by sequencing of additional genes if a pathogenic mutation is not found. In addition to the most common mutations that are associated with hereditary hearing loss, GJB6 and GJB2, there are many less common pathologic mutations. Some of these are: ACTG1, CDH23, CLDN14, COCH, COL11A2, DFNA5, DFNB31, DFNB59, ESPN, EYA4, GJB2, GJB6, KCNQ4, LHFPL5, MT-TS1, MYO15A, MYO6, MYO7A, OTOF, PCDH15, POU3F4, SLC26A4, STRC, TECTA, TMC1, TMIE, TMPRSS3, TRIOBP, USH1C, and WFS1 genes. Novel genetic mutations continue to be identified in cases of hereditary. (18,19) Because of the large number of genes associated with hereditary hearing loss, there are a variety of genetic panels for hereditary deafness. Next generation genetic sequencing technology allows targeted sequencing of multiple genes simultaneously, expanding the ability to examine multiple genes. These panels are alternatives to sequencing of individual genes such as GJB6 and GJB2. Some examples of these panels are given in Table 3. These panels include the most common genes associated with NSHL. They may also include many of the less common genes associated with NSHL, as well as genes that are associated with syndromic hearing loss. In addition, whole exome sequencing and whole genome sequencing have been used to identify novel mutations in subjects with a history suggestive of genetic hereditary hearing loss. (20-22) Overlap Between NSHL and Recognized Syndromes There is overlap between hereditary NSHL and hearing loss associated with recognized syndromes. Some genetic mutations may be associated with clinical findings other than hearing loss, but they are not necessarily present at the time of presentation with hearing loss. For example, Jervell and Lange-Nielsen syndrome is associated with congenital deafness and prolonged QT interval, but it may present only with deafness without an apparent history to suggest cardiac dysfunction. Additionally, some of the genes associated with NSHL are also associated with recognized syndromes. A summary of some of the genetic syndromes and mutations that may have overlap with NSHL is shown in Table 2. Table 2. Genetic Mutations With Overlap Between Syndromic and Nonsyndromic Hearing Loss SIDS: sudden infant death syndrome. No U.S. Food and Drug Administration (FDA)‒cleared molecular diagnostic tests were found. Thus, molecular evaluation is offered as a laboratory-developed test. Clinical laboratories may develop and validate tests in-house (laboratory-developed tests, formerly “home-brew”) and market them as a laboratory service; such tests must meet the general regulatory standards of the Clinical Laboratory Improvement Act (CLIA). The laboratory offering the service must be licensed by CLIA for high-complexity testing. More than a dozen commercial laboratories currently offer a wide variety of diagnostic procedures for GJB2 and GJB6 genetic testing. Medical policies are systematically developed guidelines that serve as a resource for Company staff when determining coverage for specific medical procedures, drugs or devices. Coverage for medical services is subject to the limits and conditions of the member benefit plan. Members and their providers should consult the member benefit booklet or contact a customer service representative to determine whether there are any benefit limitations applicable to this service or supply. This medical policy does not apply to Medicare Advantage. Literature was sought on genetic testing for hereditary hearing loss in the following areas: analytic validity (ability to detect a mutation that is known to be present and the ability to rule out mutations when they are absent); clinical validity (ability to detect a mutation in a patient with hereditary hearing loss, or to exclude a mutation in a patient without hereditary hearing loss); and clinical utility (the impact of a mutation on the management of patients and on relevant health outcomes). The analytic validity of Sanger sequencing is known to be high. Although there is not a robust evidence base for Sanger sequencing specifically for genes involved in hereditary hearing loss, it is reasonable to assume that sequencing has an analytic sensitivity and specificity that approaches 100% under ideal testing conditions. The analytic validity of targeted panels, such as the available microchips for hereditary hearing loss mutations that have been described, is less certain. The studies identified for this review are summarized in Table 3. These data are only available for some of the panels that are commercially available. In all cases where data were presented, the analytic sensitivity was greater than 99%, and in most studies it was 100%. The analytic specificity was 100% when it was reported, usually in a small number of normal individuals. Table 4 summarizes some of the commercially available targeted panels for hereditary hearing loss. The largest of these studies was published by Abe et al. (23) This study included 338 patients from Japan with congenital or childhood onset hearing loss before age 10 years. The population included a broad range of patients with hereditary hearing loss, including those with inheritance patterns that were autosomal dominant, autosomal recessive, mitochondrial, or sporadic. A targeted microarray panel (Invader Assay) was used to detect genetic mutations, which included 41 mutations in 9 different genes, one of which was GJB2. A total of 13,858 assays were performed. The correct genotype was identified after a single Invader analysis in 13,748 cases (99.2%). A total of 110 assays incorrectly identified the genotype. When these samples were re-assayed with a larger amount of DNA, 108/110 were correctly genotyped. The remaining 2 assays were invalid because of insufficient DNA. Other studies used different patient populations and different panels of genes, but all included the GJB2 mutations as part of the panel. Despite the heterogeneity in populations and genes examined, the analytic specificity was 100% in these other studies. (24-26) There is limited evidence on the analytic validity of testing for hereditary hearing loss mutations. When performed by direct sequencing, the analytic validity approaches 100%. When performed as part of a next-generation testing panel, the error rate is expected to be higher than for direct sequencing. However, the available evidence reports high sensitivity and specificity for available next-generation genetic panels, and the difference in accuracy between direct sequencing and targeted panels is not well-defined in the literature. Table 3. Mutation Chips Including GJB2 and GJB6 Genes PCR: polymerase chain reaction. Table 4. Genomic Mutations Panels for Hereditary Hearing Loss (11) A number of publications have evaluated the clinical sensitivity and specificity of genetic testing for hereditary hearing loss in general and nonsyndromic hearing loss (NSHL) more specifically. The clinical sensitivity is reported as the percent of patients with hereditary hearing loss who have a pathologic genetic mutation, and the clinical specificity is reported as the percent of patients without hereditary hearing loss who do not have a pathologic genetic mutation. The clinical validity will vary as a function of the number of different genes examined, and also by whether the population includes patients with hearing loss that is not strictly hereditary hearing loss. A representative sample of articles on clinical validity is given in Table 5. Studies were selected that were published within the past 10 years, had populations of primarily NSHL. Mixed populations that included patients with syndromic hearing loss, or with non-hereditary hearing loss were not included. Table 5: Clinical Validity of Genetic Testing for Hereditary Hearing Loss Vona et al reported testing results for targeted next generation sequencing of 2 panels of deafness-associated genes, 1 with 80 genes and 1 with 129 genes, in the evaluation of NSHL for cases in which GJB2 testing was negative. (36) Testing with 1 of the 2 panels was performed on 30 patients from 23 families (23 index patients) with hearing loss and 9 normal-hearing controls. Pathogenic mutations in a gene associated with autosomal dominant hearing loss (ACTG1, CCDC50, EYA4, MYH14, M7O6, TCF21, MYO1A) or autosomal recessive hearing loss (MYO15A, MYO7A, GJB2, USH2A) were identified in 8/23 index patients and 5/23 index patients, respectively, for a success rate of 57%. Gu et al. reported results for targeted next generation sequencing of a panel of 131 genetic mutations related to hearing loss in 63 subjects with NSHL with negative testing for mutations in the GJB2, MT-RNR1, and SLC26A4 genes. (37) The mutation detection rate was 12.7%, with 10 of 14 mutations detected as novel compound heterozygotes. In general, these studies indicate that the clinical sensitivity is low to moderate and the clinical specificity is high. There is a high degree of variability among these studies in the type of sequencing used and the number of genes examined. For example, the study with the highest sensitivity (62%), Duman et al. (2011) tested for mutations on 38 different genes, which was the highest number examined in any of these studies. The other studies generally tested for one or several genes, and reported lower sensitivities in the range of 30% to 40%. There is some data on clinical validity, but it is incomplete. The available studies indicate that a substantial percent of patients with hereditary hearing loss will have a pathologic mutation (clinical sensitivity). This rate varies widely in available studies due to differences in specific genes tested, patient population used, and the type of genetic testing performed. As a result, the clinical sensitivity is not well-defined. There is limited information on the clinical specificity. Some studies with relatively small numbers of normal individuals have reported specificities approaching 100%. There are several potential ways in which genetic testing for hereditary hearing loss may have clinical utility. For this policy review, clinical utility will be considered in the following areas: Diagnostic test for etiology of Hereditary Hearing Loss Clinical Utility of Genetic testing for Diagnosis of Hereditary Hearing Loss Genetic testing in patients with suspected hereditary hearing loss can be performed to confirm the diagnosis of hereditary hearing loss, which is distinguished from acquired hearing loss. There is no direct evidence on the impact of genetic testing on outcomes when used as a diagnostic test in this manner. Therefore, an indirect chain of evidence is considered to determine the impact on health outcomes. The high analytic sensitivity indicates that if a genetic mutation is present and included within test repertoires, it is very likely to be detected by current testing methods. The high analytic specificity indicates that if a genetic mutation is absent, a false-positive result on genetic testing is very unlikely to occur. Therefore, a positive genetic test with a known pathologic mutation indicates that hereditary hearing loss is present with a high degree of certainty. In contrast, the low to moderate clinical sensitivity indicates that a negative test is not definitive for ruling out hereditary hearing loss. False negative results on genetic testing are not uncommon, therefore the utility of a negative test in discriminating between hereditary and acquired hearing loss is low. To have clinical utility, the confirmation of the diagnosis must be accompanied by changes in clinical management that improve outcomes. No published evidence was identified to evaluate whether management changes occur, and no clinical practice guidelines were identified that recommend these actions. However, the confirmation of a genetic basis for hereditary hearing loss may be useful in differentiating hereditary hearing loss from other causes of deafness, and thereby precluding other testing such as computed tomography or magnetic resonance imaging. Given that some cases of apparent NSHL may, in actuality, represent an initial presentation of a known syndrome that is associated with hearing loss, identification of specific mutations may prompt additional action. For example if a KNCQ1 mutation is found, additional cardiac workup may be warranted because mutations in this gene are also associated with cardiac rhythm abnormalities. In addition, genetic counseling can provide patients and families with further information and assistance on issues such as reproductive decision making. Genetic testing has also been proposed as a method to predict response to cochlear implantation. Expression of GJB2 and GJB6 is in the cochlea. In addition, patients with hereditary hearing loss mutations have been found to have intact spiral ganglion cells in the cochlea. Intact spiral ganglion cells have been associated with success following cochlear implantation. These factors lend credence to the theory that patients with GJB2 and GJB6 mutations may have a favorable prognosis following cochlear implantation and that patients with other mutations or without a documented mutation may have a less favorable prognosis. The evidence on this question consists of several small, retrospective, single center studies that compared outcomes of cochlear implantation in patients with and without genetic mutations. Two small series from Japan initially reported that hearing outcomes were superior in patients with genetic mutations. Fukushima et al compared 3 patients with genetic mutation with 4 patients without mutations. (38) Patients with GJB2 mutations had a larger vocabulary compared with patients without a mutation (1,243 words vs 195 words), and a higher mean developmental quotient. Matsushiro et al. evaluated 15 patients with hearing loss, 4 with genetic mutations and 11 without. (39) These authors reported that speech perception was higher among patients with mutations compared with those without. In 2014, in a retrospective cohort study, Popov et al evaluated the impact of GJB2 mutations on hearing outcomes after cochlear implantation for congenital nonsyndromic sensorineural hearing loss. (40) The study included 60 patients who had received a cochlear implant, 30 with GJB2 mutations and 30 without, who were a subset of 71 patients included in a larger registry of cochlear implant patients evaluated at a single institution from 2009 to 2013. At 36 months of follow-up, results on several hearing test metrics were significantly better for the patients with GJB2 mutations than for those without mutations, including the Listening Progress Profile test (p<0.05), the Monosyllabic-Trochee-Polysyllabic test with 3, 6, or 12 items (p=0.005, p=0.002, and p=0.001, respectively). Yan et al reported results from a series of 41 children who received cochlear implants for severe bilateral sensorineural hearing loss treated at a single center in China, 15 of whom had GJB2 mutations and 10 of whom had SLC26A4 mutations. (41) Compared with patients with no mutation, patients with GJB2 mutations but not those with SLC26A4 mutations, had improved outcomes on a number of hearing-related tests, including the Meaningful Auditory Integration Scale, categories of auditory performance, and speech intelligibility rating. At least 2 similar series have been published in the U.S. Sinnathuray et al. published 2 articles on overlapping series of patients who were treated with cochlear implants. (42, 43) In the larger series, 38 patients were included, 14 patients with genetic mutations and 24 without. A standardized measure of speech, the Speech Intelligibility Rating (SIR) score, was used as the primary outcome measure. At 1 year, the median SIR scores were higher in the patients with GJB2 mutations (median, 3; range, 2-4) compared with patients without mutations (median, 2; range, 1-4), and the difference between the 2 groups was statistically significant (p=0.007). The percent of patients achieving intelligible speech was 82% in the GJB2 group compared with 30% in patients without mutations (p=0.02). In a second U.S. study by Connell et al., (44) these findings were not completely replicated. This series included 31 patients with congenital hearing loss, 12 with genetic mutations and 19 without. The main outcome measure was speech perception category ranging from 1 to 6. The mean speech perception category was not different between patients with and without mutations (4.1 vs 4.9 respectively, p =NS). The percent of patients achieving speech perception category 6 was higher in the mutation group (75% vs 53%), but statistical testing for this difference was not performed. On multivariate analysis, the variability in speech perception was explained primarily by the length of time since cochlear implantation, and cause of hearing loss was not a significant predictor of outcomes. Clinical Utility of Genetic Panel Testing for Diagnosis of Hereditary Hearing Loss Given the large quantity of genes associated with hereditary hearing loss, multiple genetic panel tests are commercially available. The Medical Policy No. 12.04.92, General Approach to Evaluating the Utility of Genetic Panels (see Related Policies), outlines criteria that can be used to evaluate the clinical utility of panel testing for hereditary or genetic conditions. Panel testing for hereditary hearing loss generally falls into the category of panels containing mutations associated with a single condition such as hearing loss, for which the following criteria apply: For next generation sequencing panels for hereditary hearing loss, criteria 2 to 4 generally apply. Some, but not all, of the mutations evaluated in hereditary hearing loss genetic panels would be associated with the need for additional subspecialist referral or additional testing; based on an indirect chain of evidence, testing for these mutations would have demonstrated clinical utility. Testing with a panel that includes only genes that have an association with hereditary hearing loss would be associated with low potential for harm, as they would not be likely to lead to further investigations that are of unproven benefit. Hereditary hearing loss can be confirmed if genetic testing reveals a pathologic mutation known to be associated with hereditary hearing loss, but a negative genetic test does not rule out hereditary hearing loss. For the individual patient, there is no evidence from literature and no specialty society guidelines that recommend specific actions or changes in management as a result of a positive genetic test. However, the use of genetic testing can streamline the diagnostic workup, and knowledge of specific mutations may prompt further action such as referral to specialists. Also, genetic counseling can be provided and may impact future decisions by the patient in areas such as reproductive planning. It is possible that the presence of a genetic mutation, and/or the presence of a specific type of mutation, is associated with the degree of response to cochlear implantation. This evidence is from small case series and therefore is not definitive. In addition, there are not treatment guidelines that recommend genetic testing as part of the decision to perform a cochlear implant. Therefore it is not possible to conclude that genetic testing has clinical utility in predicting response to cochlear implantation. Clinical Utility of Genetic Testing for Carrier Testing for Hereditary Hearing Loss in High-Risk Individuals People who are contemplating having children may desire to know the probability of hereditary hearing loss. This is most relevant when parents have had a previous child with hearing loss, or when there is a strong family history of hereditary hearing loss. In this situation, testing of the index case for a genetic mutation can first be performed. If a pathologic mutation is found, then targeted testing for that specific mutation can be performed in the parents to confirm the presence of the carrier state, and to determine the risk of hereditary hearing loss in future offspring. The specific mutation identified will give substantial information on the usual inheritance patterns, and the probability of a future offspring being affected. Carrier testing can also be performed in people who do not have an offspring with hereditary hearing loss. If there is a strong family history of hearing loss, the likelihood of a genetic mutation is increased, but is still considerably less than for parents with a child who has hereditary hearing loss. For individuals with neither a family history of hearing loss nor an offspring with hearing loss, the probability of detecting a pathologic mutation is much lower. For individuals with a low pretest likelihood of being a carrier for a hereditary hearing loss mutation, the positive and negative predictive values of testing are not certain. Because the clinical specificity is not well established, it is not possible to determine the likelihood that a positive result represents a true positive versus a false positive. At prevalences that approach the population rate, it is possible that a substantial number of positive results are false positives, even in the presence of a low false-positive rate. Carrier testing has clinical utility if it aids in reproductive decision making. Parents may decide to change their plans for attempting pregnancy based on results of genetic testing. Carrier testing combined with preimplantation genetic testing and in vitro fertilization may be effective in reducing the number of infants born with hereditary hearing loss. While there is no direct evidence that carrier testing leads to a higher percentage of live births without hereditary hearing loss, there is evidence from other disorders, such as Tay-Sachs disease and cystic fibrosis, that carrier testing can result in a decrease in offspring with those disorders. Theoretically, a similar decrease should be expected with carrier testing for hereditary hearing loss. Carrier testing is most accurate when the mutation in the index case with hereditary hearing loss is known. In those cases, targeted mutation testing for a single mutation can be performed in lieu of comprehensive genetic testing for the full range of mutations associated with hereditary hearing loss. Targeted testing has a higher accuracy for confirming and excluding the presence of a pathologic mutation. It is particularly useful for excluding the presence of a mutation, because comprehensive testing has a suboptimal sensitivity and negative predictive value. Therefore, targeted testing can rule out a genetic mutation with certainty whereas comprehensive testing cannot. Clinical Utility of Genetic Panel Testing for Carrier Testing for Hereditary Hearing Loss in High-Risk Individuals The Medical Policy: General Approach to Evaluating the Utility of Genetic Panels (See Related Policies) outlines criteria that can be used to evaluate the clinical utility of reproductive panel testing for at-risk individuals. The following criteria apply for the use of panel testing for carrier testing in hereditary hearing loss: In line with the reasoning for the clinical utility of panel testing for diagnosis of hereditary hearing loss, panel testing for hearing loss for carrier testing can be considered to meet these criteria for individuals who will make reproductive decisions based on the test results. Carrier testing can be performed in parents who are planning offspring to determine their likelihood of a child with hereditary hearing loss. If there is a previous child with hereditary hearing loss, there is a high likelihood of subsequent offspring having hereditary hearing loss. In other situations, a family history of hereditary hearing loss is sufficient to conclude that the likelihood of an offspring with hereditary hearing loss is increased. Examples of these situations are when a first- or second-degree relative has hereditary hearing loss. Carrier testing has clinical utility in these high-risk situations when used as an aid in reproductive decision making. Carrier testing is most useful when the specific mutation causing hereditary hearing loss in the family is known, because targeted mutation testing is more accurate than comprehensive testing, and can confirm or exclude the presence of a mutation with higher certainty. Because of the low prevalence of mutations in unselected populations, the positive predictive value of finding a mutation is not known in unselected populations and the value of carrier testing is uncertain for these individuals. Genetic Testing to Screen for Hearing Loss Routine screening of newborns for congenital hearing loss via audiometric testing is standard of care and has been recognized to be associated with improved outcomes. However, audiometric testing does not identify all newborns with congenital hearing loss. As a result, genetic testing has been investigated as a way to identify early-onset hearing loss. Several studies have evaluated the use of genetic testing, either by itself or as a complement to audiometric screening, in the detection of congenital hearing loss. Lim et al reported results from genetic panel testing for 14 genetic mutations associated with hearing loss (SoundGene panel) of 3806 infants without major congenital malformations. (45) Thirty-five subjects (0.95%) had a positive panel test; of those, 3 patients (8.6%) had persistent hearing loss compared with 5 (0.21%) of 2398 subjects with no report of a mutation (p<0.01). Two of the 35 (6%) subjects with a positive genetic panel test panel had a positive newborn audiometric screen. Han et al demonstrated the feasibility of testing newborns for mutations related to hereditary hearing loss on a large scale using the types of filter paper blood samples that are used for routine newborn screening, using a PCR-based panel test designed to detect high-risk deafness-associated mutations, including GJB2 c.235delC, SLC26A4 c.919-2A>G, mtDNA 12S rRNA mt.1555A>G and mt.1494C>T.46 Among 1181 newborns tested, 29 had 1 or 2 mutant alleles, for a carrier rate of 2.46% (29/1181). Although a few studies have demonstrated the feasibility of genetic testing to screen for congenital hearing loss, the positive and negative predictive values of genetic testing for hereditary hearing loss in unselected populations is not well-defined. There are no studies that demonstrate that such testing is associated with incremental improvement in outcomes. Ongoing and Unpublished Clinical Trials An online search of ClinicalTrials.gov in July 2014 found several ongoing trials related to genetic testing for hereditary hearing loss: Clinical Input Received From Physician Specialty Societies and Academic Medical Centers While the various physician specialty societies and academic medical centers may collaborate with and make recommendations during this process through the provision of appropriate reviewers, input received does not represent an endorsement or position statement by the physician specialty societies or academic medical centers, unless otherwise noted. In response to requests, input was received from two physician specialty societies and 2 academic medical centers while this policy was under review in 2013. Reviewers agreed with the medically necessary indication for carrier testing, and with additional indications for carrier testing. There was support for testing the index case to confirm NSHL among a majority of reviewers. Reviewers in favor of genetic testing cited the ability to distinguish NSHL from other causes of hearing loss, to streamline the diagnostic workup and avoid further unnecessary testing and to provide referrals to specialists when specific types of mutations were identified that are associated with disorders in other organ systems. It was considered that two contextual factors were present: barriers to performing high-quality trials, and the potential to reduce harms by avoiding unnecessary testing. Summary of Evidence Genetic mutations in GJB2, GJB6, and numerous other genes are found in a substantial percent of patients with hereditary hearing loss. The analytic validity of genetic testing for hereditary hearing loss is high. Of all patients with suspected hereditary hearing loss after clinical examination, a substantial minority, in the range of 30% to 60% will be found to have a genetic mutation. False-positive results on mutation testing are expected to be very low. There are several situations for which there is potential clinical utility of testing for hereditary hearing loss mutations. For diagnosis alone, there is a lack of evidence from the literature or from clinical practice guidelines on specific management changes that result from genetic testing. Clinical input received from physician specialty societies and academic medical centers demonstrated support for genetic testing to differentiate NSHL from other causes of hearing loss and to improve the efficiency of the diagnostic workup by avoiding unnecessary testing. Clinical input also suggested that knowledge of specific mutations may lead to further management changes, such as referral to specialists. Therefore, genetic testing to confirm the diagnosis of hereditary hearing loss may be considered medically necessary. For parents at high risk of an offspring with hereditary hearing loss, genetic testing can be useful as an aid in reproductive decision making. Parents may alter their attempts at pregnancy following testing, or can increase the likelihood of a birth free of genetic mutations through preimplantation genetic testing followed by in vitro fertilization. Based on the available evidence and results of clinical vetting, genetic testing for hereditary hearing loss carrier status may be considered medically necessary when one of the following is present: (1) an offspring with hereditary hearing loss, (2) one or both parents with suspected hereditary hearing loss, (3) A first-degree relative with an offspring who has hereditary hearing loss, (4) a first- or second-degree relative with hereditary hearing loss, and the parents desire to have further offspring and wish to know the likelihood of another offspring with hereditary hearing loss. Although genetic testing for hereditary hearing loss has been investigated as an adjunct to audiologic testing for identification of congenital hearing loss, there are no studies that demonstrate that such testing is associated with incremental improvement in outcomes. Therefore, genetic testing for hereditary hearing loss in patients without identified hearing loss is considered investigational. Practice Guidelines and Position Statements American College of Medical Genetics and Genomics (ACMG) In 2014, the ACMG issued a practice guideline (47) for the clinical evaluation and etiologic diagnosis of hearing loss. The guideline recommends obtaining testing for acquired hearing loss if there is clinical suspicion, including testing for cytomegalovirus (CMV), imaging, or other testing based on the suspected etiology. For individuals lacking physical findings suggestive of a known syndrome and having medical and birth histories that do not suggest an environmental cause of hearing loss, the guidelines make the following recommendations for a tiered diagnostic approach: The Joint Committee on Infant Hearing (JCIH) The JCIH issued the following recommendations in 2007 (5): This information may influence parents' decision making regarding intervention options for their child. There is a 2013 supplement to the JCIH 2007 position statement on early intervention after confirmation that a child is deaf or hard of hearing. (48) Genetic testing was not addressed. U.S. Preventive Services Task Force Recommendations Genetic testing is not a preventive service under normal circumstances. Medicare National Coverage There is no national coverage determination (NCD). In the absence of an NCD, coverage decisions are left to the discretion of local Medicare carriers. GJB2 (gap junction protein, beta 2, 26kDa; connexin 26)(e.g., nonsyndromic hearing loss) gene analysis, full gene sequence known familial variants GJB6 (gap junction protein, beta 6, 30kDa, connexin 30)(e.g., nonsyndromic hearing loss) gene analysis, common variants (e.g., 309kb [del(GJB6-D13S1830)] and 232kb [del(GJB6-D13S1854)]) Hearing loss (e.g., nonsyndromic hearing loss, Usher syndrome, Pendred syndrome); genomic sequence analysis panel, must include sequencing of at least 60 genes, including CDH23, CLRN1, GJB2, GPR98, MTRNR1, MYO7A, MYO15A, PCDH15, OTOF, SLC26A4, TMC1, TMPRSS3, USH1C, USH1G, USH2A, and WFS1. (new code effective 01/1/15) Hearing loss (e.g., nonsyndromic hearing loss, Usher syndrome, Pendred syndrome); duplication/deletion analysis panel, must include copy number analyses for STRC and DFNB1 deletions in GJB2 and GJB6 genes (new code effective 01/1/15) New Policy. Policy created with literature search through June 30, 2013 and clinical input reviewed; may be considered medically necessary for confirmation of the diagnosis of hereditary nonsyndromic hearing loss, and for carrier testing in parents under certain conditions. Annual Review. Policy title and policy statements changed to refer to “hereditary hearing loss” (from “nonsyndromic hearing loss”) to reflect significant overlap between nonsyndromic and syndromic hearing loss. Added related policy 12.04.92 General Approach to Evaluating the Utility of Genetic Panels. Added Table 2 - a summary of some of the genetic syndromes and mutations that may have overlap with NSHL. Replaced the word “proband” with “index patient”. Policy updated with literature review through July 29, 2014. References 3, 10-11, 17-22, 36, 37, 40-41, 45-48 added. Policy statements changed as noted. New CPT code 81430 added effective 1/15. Coding update. New CPT code 81431, effective 1/1/15, added to policy. Update related policy title 7.01.105. Disclaimer: This medical policy is a guide in evaluating the medical necessity of a particular service or treatment. The Company adopts policies after careful review of published peer-reviewed scientific literature, national guidelines and local standards of practice. Since medical technology is constantly changing, the Company reserves the right to review and update policies as appropriate. Member contracts differ in their benefits. Always consult the member benefit booklet or contact a member service representative to determine coverage for a specific medical service or supply. CPT codes, descriptions and materials are copyrighted by the American Medical Association (AMA).	2	36	4	0	0	0	0.665101	4	9,357
Cleft Lip and Cleft Palate The concepts of cleft palate and cleft lip and palate will be covered in detail in the BGDB Face and Ear Development lecture/practical. Atresia and Stenosis The gastrointestinal tract can be considered as a simple tube or pipe, anything which blocks the tube (at different levels) can have different effects. Gastrointestinal tract duplication sites based upon 78 clinical studies. There are two types of abnormalities that impact upon the continuity of the gastrointestinal tract lumen. Atresia - interuption of the lumen (esophageal atresia, duodenal atresia, extrahepatic biliary atresia, anorectal atresia) Stenosis - narrowing of the lumen (duodenal stenosis, pyloric stenosis) Duplication - incomplete recanalization resulting in parallel lumens, this is really a specialized form of stenosis. \|\|Classification of Oesophageal Atresia - A Esophageal atresia (EA) without tracheoesophageal fistula (TEF) - B Proximal TEF with distal EA - C Distal TEF with proximal EA - D Proximal and distal TEF - E TEF without EA or “H”-type TEF fistula - abnormal connection between either hollow or tubular organs. Persistent Vitelline Duct - Links: Meckel's Diverticulum \| Meckel's Image 1 \| Meckel's Image 2 \| Meckel's Image 3 Abnormal Gut Rotation \|\|Presents clinically in symptomatic malrotation as: Neonates - bilious vomiting and bloody stools. Newborn - bilious vomiting and failure to thrive. Infants - recurrent abdominal pain, intestinal obstruction, malabsorption/diarrhea, peritonitis/septic shock, solid food intolerance, common bile duct obstruction, abdominal distention, and failure to thrive. Ladd's Bands - are a series of bands crossing the duodenum which can cause duodenal obstruction. - Links: Midgut Volvulus \| Situs Inversus \| Gastrointestinal Tract - Abnormalities Extrahepatic Biliary Atresia, Accessory Pancreatic Tissue, Anular Pancreas, Accessory Spleen Aganglionic colon (Hirschprung's disease) - abnormalities of neural crest migration. - Links: Aganglionic colon \| Enteric Nervous System \| Neural Crest Development - omphalocele - herniation of the bowel, liver and other organs into the intact umbilical cord, the tissues being covered by membranes unless the latter are ruptured - gastroschisis - intact umbilical cord and evisceration of the bowel through a defect in the abdominal wall, generally to the right of the cord, with no membrane covering Omphalocele ruptured during birth exposing liver and small intestine. Abdominal wall herniation abnormality occurs in both omphalocele and gastroschisis. Omphalocele involves "covered by membranes" and a lack of normal return of the bowel to the abdominal cavity and has a different position relative to the umbilical cord. The origin differs, as this is a failure of midgut loops to return to the body cavity after initial herniation into the umbilical cord during week 6 - 10. \| Gastroschisis is a developmental abnormality occurs due to an abdominal wall defect, that allows the evisceration of the intestine. Cleft lip and palate can affect postnatal nutrition, due to the inability of the infant to form a liquid seal on the breast during feeding. Note - this topic will be covered in detail in the BGDB Practical - Face and Ear Development practical. - Links: Palate Development \| Cleft Palate Foundation - feeding your baby An abnormality of face development leading to an opening in the upper lip. Due to failure during the embryonic period of maxillary process fusion with the frontonasal prominence. Clefting of the lip and or palate occurs with 300+ different abnormalities. Depending on many factors, this cleft may extend further into the oral cavity leading to a cleft palate. In most cases clefting of the lip and palate can be repaired by surgery. An abnormality of face development leading to an opening in the palate, the roof of the oral cavity between the mouth and the nose. If it occurs alone, due to failure during the early fetal period of palatal shelves. Clefting of the lip and or palate occurs with 300+ different abnormalities. In most cases clefting of the lip and palate can be repaired by surgery. \|\| Polyhydramnios (hydramnios, amniotic fluid disorder) refers to abnormally high amniotic fluid levels. \|Decreased Fluid Swallowing or Absorption \|\|Increased Fluid Production - Lung disorders - Multiple pregnancy - Hydrops fetalis \|Maternal Causes - Poorly controlled diabetes. A condition caused by intra-uterine intestinal perforation leading to a sterile inflammatory reaction of the peritoneum. Inborn Errors of Metabolism (IEMs) are caused by mutations in the specific genes that lead to impaired proteins or enzymes production and are usually detected by the neonatal Guthrie test. - phenylketonuria (PKU) - 1 in 10,000 live births (about 10 babies per year). PKU causes high blood levels of phenylalanine and severe intellectual disability. A diet low in phenylalanine, started in the first two to three weeks results in normal development. - galactosaemia - 1 in 40,000 births (about 1-3 cases per year), incidence rate is different for other groups. Babies cannot process galactose, a component of lactose, (enzyme galactose-1-phosphate uridyl transferase) metabolizes galactose in milk sugar. Life-threatening liver failure and infections can occur. A galactose-free diet instituted in the first week is life saving. - Rarer metabolic disorders - Some fatty acid, organic acid and other amino acid defects can now be detected using Tandem Mass Spectrometry. These much rarer metabolic disorders affect about 15 – 18 babies per year. Early detection is important as diet and medications can treat most of these disorders. Without appropriate management they can cause severe disability or death. - Links: Guthrie test \| neonatal diagnosis \| milk \| MedlinePlus - Galactosemia \| Gastrointestinal Tract Terms - allantois - An extraembryonic membrane, endoderm in origin extension from the early hindgut, then cloaca into the connecting stalk of placental animals, connected to the superior end of developing bladder. In reptiles and birds, acts as a reservoir for wastes and mediates gas exchange. In mammals is associated/incorporated with connecting stalk/placental cord fetal-maternal interface. - amnion - An extra-embryonic membrane, ectoderm and extraembryonic mesoderm in origin, also forms the innermost fetal membrane, that produces amniotic fluid. This fluid-filled sac initially lies above the trilaminar embryonic disc and with embryoic disc folding this sac is drawn ventrally to enclose (cover) the entire embryo, then fetus. The presence of this membane led to the description of reptiles, bird, and mammals as amniotes. - amniotic fluid - The fluid that fills amniotic cavity totally encloses and cushions the embryo. Amniotic fluid enters both the gastrointestinal and respiratory tract following rupture of the buccopharyngeal membrane. The late fetus swallows amniotic fluid. - buccal - (Latin, bucca = cheek) A term used to relate to the mouth (oral cavity). - bile salts - Liver synthesized compounds derived from cholesterol that function postnatally in the small intestine to solubilize and absorb lipids, vitamins, and proteins. These compounds act as water-soluble amphipathic detergents. liver - buccopharyngeal membrane - (oral membrane) (Latin, bucca = cheek) A membrane which forms the external upper membrane limit (cranial end) of the early gastrointestinal tract (GIT). This membrane develops during gastrulation by ectoderm and endoderm without a middle (intervening) layer of mesoderm. The membrane lies at the floor of the ventral depression (stomodeum) where the oral cavity will open and will breakdown to form the initial "oral opening" of the gastrointestinal tract. The equivilent membrane at the lower end of the gastrointestinal tract is the cloacal membrane. - cloacal membrane - Forms the external lower membrane limit (caudal end) of the early gastrointestinal tract (GIT). This membrane is formed during gastrulation by ectoderm and endoderm without a middle (intervening) layer of mesoderm. The membrane breaks down to form the initial "anal opening" of the gastrointestinal tract. - cholangiocytes - epithelial cells that line the intra- and extrahepatic ducts of the biliary tree. These cells modify the hepatocyte-derived bile, and are regulated by hormones, peptides, nucleotides, neurotransmitters, and other molecules. liver - coelom - Term used to describe a space. There are extraembryonic and intraembryonic coeloms that form during vertebrate development. The single intraembryonic coelom will form the 3 major body cavities: pleural, pericardial and peritoneal. - crypt of Lieberkühn - (intestinal gland, intestinal crypt) intestinal villi epithelia extend down into the lamina propria where they form crypts that are the source of epithelial stem cells and immune function. - foregut - The first of the three part/division (foregut - midgut - hindgut) of the early forming gastrointestinal tract. The foregut runs from the buccopharyngeal membrane to the midgut and forms all the tract (esophagus and stomach) from the oral cavity to beneath the stomach. In addition, a ventral bifurcation of the foregut will also form the respiratory tract epithelium. - galactosemia - Metabolic abnormality where the simple sugar galactose (half of lactose, the sugar in milk) cannot be metabolised. People with galactosemia cannot tolerate any form of milk (human or animal). Detected by the Guthrie test. - gastrula - (Greek, gastrula = little stomach) A stage of an animal embryo in which the three germ layers (Endoderm/ Mesoderm/Ectoderm) have just formed. - gastrulation - The process of differentiation forming a gastrula. Term means literally means "to form a gut" but is more in development, as this process converts the bilaminar embryo (epiblast/hypoblast) into the trilaminar embryo (Endoderm/ Mesoderm/Ectoderm) establishing the 3 germ layers that will form all the future tissues of the entire embryo. This process also establishes the the initial body axes. (More? Gastrulation) - Guthrie test - (heel prick) A neonatal blood screening test developed by Dr Robert Guthrie (1916-95) for determining a range of metabolic disorders and infections in the neonate. (More? Guthrie test) - hindgut - The last of the three part/division foregut - midgut - hindgut) of the early forming gastrointestinal tract. The hindgut forms all the tract from the distral transverse colon to the cloacal membrane and extends into the connecting stalk (placental cord) as the allantois. In addition, a ventral of the hindgut will also form the urinary tract (bladder, urethra) epithelium. - intraembryonic coelom - The "horseshoe-shaped" space (cavity) that forms initially in the third week of development in the lateral plate mesoderm that will eventually form the 3 main body cavities: pericardial, pleural, peritoneal. The intraembryonic coelom communicates transiently with the extraembryonic coelom. - mesothelium - The mesoderm derived epithelial covering of coelomic organs and also line their cavities. - neuralation - The general term used to describe the early formation of the nervous system. It is often used to describe the early events of differentiation of the central ectoderm region to form the neural plate, then neural groove, then neural tube. The nervous system includes the central nervous system (brain and spinal cord) from the neural tube and the peripheral nervous system (peripheral sensory and sympathetic ganglia) from neural crest. In humans, early neuralation begins in week 3 and continues through week 4. - neural crest - region of cells at the edge of the neural plate that migrates throughout the embryo and contributes to many different tissues. In the gastrointestinal tract it contributes mainly the enteric nervous system within the wall of the gut responsible for peristalsis and secretion. - pharynx - uppermost end of gastrointestinal and respiratory tract, in the embryo beginning at the buccopharyngeal membrane and forms a major arched cavity within the phrayngeal arches. - recanalization - describes the process of a hollow structure becoming solid, then becoming hollow again. For example, this process occurs during GIT, auditory and renal system development. - somitogenesis The process of segmentation of the paraxial mesoderm within the trilaminar embryo body to form pairs of somites, or balls of mesoderm. A somite is added either side of the notochord (axial mesoderm) to form a somite pair. The segmentation does not occur in the head region, and begins cranially (head end) and extends caudally (tailward) adding a somite pair at regular time intervals. The process is sequential and therefore used to stage the age of many different species embryos based upon the number visible somite pairs. In humans, the first somite pair appears at day 20 and adds caudally at 1 somite pair/4 hours (mouse 1 pair/90 min) until on average 44 pairs eventually form. - splanchnic mesoderm - Gastrointestinal tract (endoderm) associated mesoderm formed by the separation of the lateral plate mesoderm into two separate components by a cavity, the intraembryonic coelom. Splanchnic mesoderm is the embryonic origin of the gastrointestinal tract connective tissue, smooth muscle, blood vessels and contribute to organ development (pancreas, spleen, liver). The intraembryonic coelom will form the three major body cavities including the space surrounding the gut, the peritoneal cavity. The other half of the lateral plate mesoderm (somatic mesoderm) is associated with the ectoderm of the body wall. - stomodeum - (stomadeum, stomatodeum) A ventral surface depression on the early embryo head surrounding the buccopharyngeal membrane, which lies at the floor of this depression. This surface depression lies between the maxillary and mandibular components of the first pharyngeal arch. \| Additional Information - Content shown under this heading is not part of the material covered in this class. It is provided for those students who would like to know about some concepts or current research in topics related to the current class page. \| Abnormalities and Development - How these abnormalities may be generated in development. - When they first occur. - How can we detect these abnormalites. - Do the abnormalites have a direct or indirect effect on the GIT. - How serious to the embryo, fetus, newborn, child and adult are these conditions. - What therapeutics are available for these conditions. Ladd's band endoscopic view in adult. \| Australian GIT Abnormalities (2002-2003) \| Oesophageal atresia/stenosis - (2.0 per 10,000 births) ICD-10 Q39.0–Q39.3 - A congenital anomaly characterised by the absence of continuity or narrowing of the oesophagus, with or without tracheal fistula, including tracheoesophageal fistula with or without mention of atresia or stenosis of oesophagus. - More males (58.6%) than females were affected with this anomaly. - Women aged 40 years or older had the highest rate of affected pregnancies. \| Small intestinal atresia/stenosis - (2.4 per 10,000 births) ICD-10 Q41.0-Q41.2 - Complete or partial occlusion of the lumen of a segment of the small intestine. It can involve a single area or multiples areas of the duodenum, jejunum or ileum. - Half of the babies (49.2%) with small intestinal atresia or stenosis were born pre-term. \| Anorectal atresia/stenosis - ( 3.1 per 10,000 births) ICD-10 Q42.0–Q42.3 - A congenital anomaly characterised by absence of continuity of the anorectal canal or of communication between rectum and anus, or narrowing of anal canal, with or without fistula to neighbouring organs. It excludes mild stenosis which does not need correction, and ectopic anus. \| Hirschsprung’s disease - (1.3 per 10,000 births) ICD-10 Q43.1 - A condition characterised by partial or complete bowel obstruction resulting from absence of peristalsis in a segment of bowel due to an aganglionic section of the bowel. - More than two-thirds (66.7%) of the babies born with this anomaly were males. - Women aged 40 years or older had the highest rate of affected pregnancies. \| Exomphalos - (Omphalocele) (2.1 per 10,000 births) ICD-10 Q79.2 - A congenital anomaly characterised by herniation of abdominal contents through the umbilical insertion and covered by a membrane which may or may not be intact. The anomaly excludes gastroschisis, hypoplasia of abdominal muscles and skin covered umbilical hernia. - A significantly higher rate of births with exomphalos was seen in women who had multiple births. \| Gastroschisis - (2.6 per 10,000 births) ICD-10 Q79.3 - A congenital anomaly characterised by visceral herniation through a right side abdominal wall defect with an intact umbilical cord and not covered by a membrane. This anomaly excludes hypoplasia of abdominal muscles, skin covered umbilical hernia and exomphalos. - Similar proportions of males and females with this anomaly. - Half of the affected pregnant women delivered preterm. - Links: Gastrointestinal Tract - Abnormalities \| ICD-10 GIT \| Australian Statistics - Reference: Abeywardana S & Sullivan EA 2008. Congenital Anomalies in Australia 2002-2003. Birth anomalies series no. 3 Cat. no. PER 41. Sydney: AIHW National Perinatal Statistics Unit. \| Australian Palate Abnormalities (2002-2003) \| Cleft lip with or without cleft palate (9.2 per 10,000 births) ICD-10 Q36.0, Q36.1, Q36.9, Q37.0–Q37.5, Q37.8, Q37.9 \| A congenital anomaly characterised by a partial or complete clefting of the upper lip, with or without clefting of the alveolar ridge or the hard palate. Excludes a midline cleft of the upper or lower lip and an oblique facial fissure (going towards the eye). - 17% of the affected pregnancies were terminated in early pregnancy or resulted in fetal deaths. Most of the fetal deaths or terminations of pregnancy (95%) had multiple abnormalities. - more commonly seen in males than in females. - babies born before 25 weeks of gestation, 150 per 10,000 births had this anomaly. Most babies (80.0%) were born at term with a birthweight of 2,500 grams or more. - Maternal age group was not associated with the anomaly. - Rates significantly higher among Indigenous women than non Indigenous women. \| Cleft palate without cleft lip (8.1 per 10,000 births) ICD-10 Q35.0–Q35.9 \| A congenital anomaly characterised by a closure defect of the hard and/or soft palate behind the foramen incisivum without a cleft lip. This anomaly includes sub-mucous cleft palate, but excludes cleft palate with a cleft lip, a functional short palate and high narrow palate. - overall rate has increased to 9.1 when the rate was estimated using data from the four states that include TOP data. The reported number of fetal deaths or early terminations of pregnancy with this anomaly was small and these deaths or terminations could be due to other associated anomalies. - proportion of females with this anomaly was higher (56.9%) than males. - 52.7 per 10,000 babies born before 25 weeks of gestation. - 83.0% were born at term and most of the babies (82.7%) had a birthweight of 2,500 grams or more. - Women aged 40 years or older and women born in South Central America or the Caribbean region had the highest rates of affected births. - Multiple births had a significantly higher rate of affected babies than singleton births. - Rates did not differ significantly by Indigenous status or areas of residence. - Links: Palate Development \| Head Development \| Gastrointestinal Tract - Abnormalities \| ICD-10 GIT \| Australian Statistics - Reference: Abeywardana S & Sullivan EA 2008. Congenital Anomalies in Australia 2002-2003. Birth anomalies series no. 3 Cat. no. PER 41. Sydney: AIHW National Perinatal Statistics Unit. \| ICD10 Other congenital malformations of the digestive system (Q38-Q45) \| XVII Congenital Malformations - Other congenital malformations of the digestive system (Q38-Q45) \| Q38 Other congenital malformations of tongue, mouth and pharynx Excl.: macrostomia (Q18.4) microstomia (Q18.5) - Q38.0 Congenital malformations of lips, not elsewhere classified Congenital: fistula of lip malformation of lip NOS Van der Woude's syndrome Excl.: cleft lip (Q36.-) cleft lip with cleft palate (Q37.-) macrocheilia (Q18.6) microcheilia (Q18.7) - Q38.1 Ankyloglossia Tongue tie - Q38.2 Macroglossia - Q38.3 Other congenital malformations of tongue Aglossia Bifid tongue Congenital: adhesion fissure malformation NOS of tongue Hypoglossia Hypoplasia of tongue Microglossia - Q38.4 Congenital malformations of salivary glands and ducts Absence Accessory Atresia (of) salivary gland or duct Congenital fistula of salivary gland - Q38.5 Congenital malformations of palate, not elsewhere classified Absence of uvula Congenital malformation of palate NOS High arched palate Excl.: cleft palate (Q35.-) cleft palate with cleft lip (Q37.-) - Q38.6 Other congenital malformations of mouth Congenital malformation of mouth NOS - Q38.7 Pharyngeal pouch Diverticulum of pharynx Excl.: pharyngeal pouch syndrome (D82.1) - Q38.8 Other congenital malformations of pharynx Congenital malformation of pharynx NOS \| Q39 Congenital malformations of oesophagus - Q39.0 Atresia of oesophagus without fistula Atresia of oesophagus NOS - Q39.1 Atresia of oesophagus with tracheo-oesophageal fistula Atresia of oesophagus with broncho-oesophageal fistula - Q39.2 Congenital tracheo-oesophageal fistula without atresia Congenital tracheo-oesophageal fistula NOS - Q39.3 Congenital stenosis and stricture of oesophagus - Q39.4 Oesophageal web - Q39.5 Congenital dilatation of oesophagus - Q39.6 Diverticulum of oesophagus Oesophageal pouch - Q39.8 Other congenital malformations of oesophagus Absent Congenital displacement Duplication (of) oesophagus - Q39.9 Congenital malformation of oesophagus, unspecified \| Q40 Other congenital malformations of upper alimentary tract - Q40.0 Congenital hypertrophic pyloric stenosis Congenital or infantile: constriction hypertrophy spasm stenosis stricture of pylorus - Q40.1 Congenital hiatus hernia Displacement of cardia through oesophageal hiatus Excl.: congenital diaphragmatic hernia (Q79.0) - Q40.2 Other specified congenital malformations of stomach Congenital: displacement of stomach diverticulum of stomach hourglass stomach Duplication of stomach Megalogastria Microgastria - Q40.3 Congenital malformation of stomach, unspecified - Q40.8 Other specified congenital malformations of upper alimentary tract - Q40.9 Congenital malformation of upper alimentary tract, unspecified Congenital: anomaly deformity NOS of upper alimentary tract \| Q41 Congenital absence, atresia and stenosis of small intestine Incl.: congenital obstruction, occlusion and stricture of small intestine or intestine NOS Excl.: meconium ileus (E84.1) - Q41.0 Congenital absence, atresia and stenosis of duodenum - Q41.1 Congenital absence, atresia and stenosis of jejunum Apple peel syndrome Imperforate jejunum - Q41.2 Congenital absence, atresia and stenosis of ileum - Q41.8 Congenital absence, atresia and stenosis of other specified parts of small intestine - Q41.9 Congenital absence, atresia and stenosis of small intestine, part unspecified Congenital absence, atresia and stenosis of intestine NOS \| Q42 Congenital absence, atresia and stenosis of large intestine Incl.: congenital obstruction, occlusion and stricture of large intestine - Q42.0 Congenital absence, atresia and stenosis of rectum with fistula - Q42.1 Congenital absence, atresia and stenosis of rectum without fistula Imperforate rectum - Q42.2 Congenital absence, atresia and stenosis of anus with fistula - Q42.3 Congenital absence, atresia and stenosis of anus without fistula Imperforate anus - Q42.8 Congenital absence, atresia and stenosis of other parts of large intestine - Q42.9 Congenital absence, atresia and stenosis of large intestine, part unspecified \| Q43 Other congenital malformations of intestine - Q43.0 Meckel's diverticulum Persistent: omphalomesenteric duct vitelline duct - Q43.1 Hirschsprung's disease Aganglionosis Congenital (aganglionic) megacolon - Q43.2 Other congenital functional disorders of colon Congenital dilatation of colon - Q43.3 Congenital malformations of intestinal fixation Congenital adhesions [bands]: omental, anomalous peritoneal Jackson's membrane Malrotation of colon Rotation: failure of incomplete insufficient of caecum and colon Universal mesentery - Q43.4 Duplication of intestine - Q43.5 Ectopic anus - Q43.6 Congenital fistula of rectum and anus Excl.: congenital fistula: rectovaginal (Q52.2) urethrorectal (Q64.7) pilonidal fistula or sinus (L05.-) with absence, atresia and stenosis (Q42.0,Q42.2) - Q43.7 Persistent cloaca Cloaca NOS - Q43.8 Other specified congenital malformations of intestine Congenital: blind loop syndrome diverticulitis, colon diverticulum, intestine Dolichocolon Megaloappendix Megaloduodenum Microcolon Transposition of: appendix colon intestine - Q43.9 Congenital malformation of intestine, unspecified \| Q44 Congenital malformations of gallbladder, bile ducts and liver - Q44.0 Agenesis, aplasia and hypoplasia of gallbladder Congenital absence of gallbladder - Q44.1 Other congenital malformations of gallbladder Congenital malformation of gallbladder NOS Intrahepatic gallbladder - Q44.2 Atresia of bile ducts - Q44.3 Congenital stenosis and stricture of bile ducts - Q44.4 Choledochal cyst - Q44.5 Other congenital malformations of bile ducts Accessory hepatic duct Congenital malformation of bile duct NOS Duplication: biliary duct cystic duct - Q44.6 Cystic disease of liver Fibrocystic disease of liver - Q44.7 Other congenital malformations of liver Accessory liver Alagille's syndrome Congenital: absence of liver hepatomegaly malformation of liver NOS \| Q45 Other congenital malformations of digestive system Excl.: congenital: diaphragmatic hernia (Q79.0) hiatus hernia (Q40.1) - Q45.0 Agenesis, aplasia and hypoplasia of pancreas Congenital absence of pancreas - Q45.1 Annular pancreas - Q45.2 Congenital pancreatic cyst - Q45.3 Other congenital malformations of pancreas and pancreatic duct Accessory pancreas Congenital malformation of pancreas or pancreatic duct NOS Excl.: diabetes mellitus: congenital (E10.-) neonatal (P70.2) fibrocystic disease of pancreas (E84.-) - Q45.8 Other specified congenital malformations of digestive system Absence (complete)(partial) of alimentary tract NOS Duplication Malposition, congenital of digestive organs NOS - Q45.9 Congenital malformation of digestive system, unspecified Congenital: anomaly deformity NOS of digestive system World Health Organisation. International Statistical Classification of Diseases and Related Health Problems. (1992) 10th Revision (ICD-10). Geneva: WHO ICD-10 - 2016 Online (English) \| Links: Gastrointestinal Abnormalities \| ICD10 - Gastrointestinal \| Genital \| Renal \| Integumentary \| Australian Statistics - GIT (1981 - 1992). \|\| The pie diagram shows the relative contribution of major gastrointestinal tract abnormalities as a percentage of the total number of congenital abnormalities in Australia beween 1981 - 92. Note that the digestive system represents approximately 6% of all major congenital abnormalities. One of the most common abnormalities occurring in (2% - 3% population) is Meckel's Diverticulum. The mouth (cleft lip, cleft palate) is part of the digestive tract, but more accurately reflects an abnormality of face formation. \| Data shown as a percentage of all major abnormalities based upon published statistics using the same groupings as Congenital Malformations Australia 1981-1992 P. Lancaster and E. Pedisich ISSN 1321-8352. \| USA Statistics - CDC National estimates for selected GIT related major birth defects (2004–2006). \| Birth Defects \|\| Cases per Births (1 in ...) \|\| Estimated Annual Number of Cases \| Cleft palate without cleft lip \| Cleft lip with and without cleft palate \| Esophageal atresia/tracheoesophageal fistula \| Rectal and large intestinal atresia/stenosis \| Diaphragmatic hernia \| Trisomy 21 (Down syndrome) - ↑ Bower RJ, Sieber WK & Kiesewetter WB. (1978). Alimentary tract duplications in children. Ann. Surg. , 188, 669-74. PMID: 718292 - ↑ Akakpo-Numado GK, Gnassingbe K, Boume MA, Sakiye KA, Mihluedo-Agbolan K, Attipou K & Tekou H. (2012). Emergency treatment of a ruptured huge omphalocele by simple suture of its membrane. Ann Surg Innov Res , 6, 2. PMID: 22325297 DOI. - ↑ Feldkamp ML, Carey JC & Sadler TW. (2007). Development of gastroschisis: review of hypotheses, a novel hypothesis, and implications for research. Am. J. Med. Genet. A , 143A, 639-52. PMID: 17230493 DOI. - ↑ Panda N, Bansal NK, Narasimhan M & Ardhanari R. (2014). Laparoscopic correction of intestinal malrotation in adult. J Minim Access Surg , 10, 90-2. PMID: 24761085 DOI. : Lecture - Gastrointestinal System \| Practical - Gastrointestinal System \| Lecture - Face and Ear \| Practical - Face and Ear \| Lecture - Endocrine \| Lecture - Sexual Differentiation \| Practical - Sexual Differentiation - Glossary: A \| B \| C \| D \| E \| F \| G \| H \| I \| J \| K \| L \| M \| N \| O \| P \| Q \| R \| S \| T \| U \| V \| W \| X \| Y \| Z \| Numbers \| Symbols \| Term Link Cite this page: Hill, M.A. (2018, June 18) Embryology BGDB Gastrointestinal - Abnormalities. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/BGDB_Gastrointestinal_-_Abnormalities - What Links Here? - © Dr Mark Hill 2018, UNSW Embryology ISBN: 978 0 7334 2609 4 - UNSW CRICOS Provider Code No. 00098G	4	17	0	0	0	29	0.726711	29	7,713
International Statistical Classification of Diseases and Related Health Problems(Redirected from ICD-9) The International Classification of Diseases (ICD) is the international "standard diagnostic tool for epidemiology, health management and clinical purposes." Its full official name is International Statistical Classification of Diseases and Related Health Problems. The ICD is maintained by the World Health Organization (WHO), the directing and coordinating authority for health within the United Nations System. The ICD is originally designed as a health care classification system, providing a system of diagnostic codes for classifying diseases, including nuanced classifications of a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or disease. This system is designed to map health conditions to corresponding generic categories together with specific variations, assigning for these a designated code, up to six characters long. Thus, major categories are designed to include a set of similar diseases. ICD-11 is a major step forward, because it has the necessary terminological and ontological elements for seamless use in digital health. The ICD is published by the WHO and used worldwide for morbidity and mortality statistics, reimbursement systems, and automated decision support in health care. This system is designed to promote international comparability in the collection, processing, classification, and presentation of these statistics. Like the analogous Diagnostic and Statistical Manual of Mental Disorders (which is limited to psychiatric disorders and almost exclusive to the United States), the ICD is a major project to statistically classify all health disorders, and provide diagnostic assistance. The ICD is a core statistically based classificatory diagnostic system for health care related issues of the WHO Family of International Classifications (WHO-FIC). The ICD is revised periodically and is currently in its 10th revision. ICD-10, as it is therefore known, is from 1992 and the WHO publishes annual minor updates and triennial major updates. The final draft of the ICD-11 system is expected to be submitted to WHO's World Health Assembly (WHA) for official endorsement in 2019. The version for preparation of approval at the WHA is released on 18 June 2018. The ICD is part of a "family" of international classifications (WHOFIC) that complement each other, including also the International Classification of Functioning, Disability and Health (ICF) which focuses on the domains of functioning (disability) associated with health conditions, from both medical and social perspectives, and the International Classification of Health Interventions (ICHI) that classifies the whole range of medical, nursing, functioning and public health interventions. This section needs additional citations for verification. (July 2017) (Learn how and when to remove this template message) In 1860, during the international statistical congress held in London, Florence Nightingale made a proposal that was to result in the development of the first model of systemic collection of hospital data. In 1893, a French physician, Jacques Bertillon, introduced the Bertillon Classification of Causes of Death at a congress of the International Statistical Institute in Chicago. A number of countries adopted Bertillon's system, which was based on the principle of distinguishing between general diseases and those localized to a particular organ or anatomical site, as used by the City of Paris for classifying deaths. Subsequent revisions represented a synthesis of English, German, and Swiss classifications, expanding from the original 44 titles to 161 titles. In 1898, the American Public Health Association (APHA) recommended that the registrars of Canada, Mexico, and the United States also adopt it. The APHA also recommended revising the system every 10 years to ensure the system remained current with medical practice advances. As a result, the first international conference to revise the International Classification of Causes of Death took place in 1900, with revisions occurring every ten years thereafter. At that time, the classification system was contained in one book, which included an Alphabetic Index as well as a Tabular List. The book was small compared with current coding texts. The revisions that followed contained minor changes, until the sixth revision of the classification system. With the sixth revision, the classification system expanded to two volumes. The sixth revision included morbidity and mortality conditions, and its title was modified to reflect the changes: International Statistical Classification of Diseases, Injuries and Causes of Death (ICD). Prior to the sixth revision, responsibility for ICD revisions fell to the Mixed Commission, a group composed of representatives from the International Statistical Institute and the Health Organization of the League of Nations. In 1948, the WHO assumed responsibility for preparing and publishing the revisions to the ICD every ten years. WHO sponsored the seventh and eighth revisions in 1957 and 1968, respectively. It later became clear that the established ten year interval between revisions was too short. The ICD is currently the most widely used statistical classification system for diseases in the world. In addition, some countries—including Australia, Canada, and the United States—have developed their own adaptations of ICD, with more procedure codes for classification of operative or diagnostic procedures. Versions of ICDEdit The ICD-6, published in 1949, was the first to be shaped to become suitable for morbidity reporting. Accordingly, the name changed from International List of Causes of Death to International Statistical Classification of Diseases. The combined code section for injuries and their associated accidents was split into two, a chapter for injuries, and a chapter for their external causes. With use for morbidity there was a need for coding mental conditions, and for the first time a section on mental disorders was added . The international Conference for the Seventh Revision of the International Classification of Diseases was held in Paris under the auspices of WHO in February 1955. In accordance with a recommendation of the WHO Expert Committee on Health Statistics, this revision was limited to essential changes and amendments of errors and inconsistencies. The 8th Revision Conference convened by WHO met in Geneva, from 6 to 12 July 1965. This revision was more radical than the Seventh but left unchanged the basic structure of the Classification and the general philosophy of classifying diseases, whenever possible, according to their etiology rather than a particular manifestation. During the years that the Seventh and Eighth Revisions of the ICD were in force, the use of the ICD for indexing hospital medical records increased rapidly and some countries prepared national adaptations which provided the additional detail needed for this application of the ICD. In the USA, a group of consultants was asked to study the 8th revision of ICD (ICD-8a) for its applicability to various users in the United States. This group recommended that further detail be provided for coding hospital and morbidity data. The American Hospital Association's "Advisory Committee to the Central Office on ICDA" developed the needed adaptation proposals, resulting in the publication of the International Classification of Diseases, Adapted (ICDA). In 1968, the United States Public Health Service published the International Classification of Diseases, Adapted, 8th Revision for use in the United States (ICDA-8a). Beginning in 1968, ICDA-8a served as the basis for coding diagnostic data for both official morbidity [and mortality] statistics in the United States. The International Conference for the Ninth Revision of the International Statistical Classification of Diseases, Injuries, and Causes of Death, convened by WHO, met in Geneva from 30 September to 6 October 1975. In the discussions leading up to the conference, it had originally been intended that there should be little change other than updating of the classification. This was mainly because of the expense of adapting data processing systems each time the classification was revised. There had been an enormous growth of interest in the ICD and ways had to be found of responding to this, partly by modifying the classification itself and partly by introducing special coding provisions. A number of representations were made by specialist bodies which had become interested in using the ICD for their own statistics. Some subject areas in the classification were regarded as inappropriately arranged and there was considerable pressure for more detail and for adaptation of the classification to make it more relevant for the evaluation of medical care, by classifying conditions to the chapters concerned with the part of the body affected rather than to those dealing with the underlying generalized disease. At the other end of the scale, there were representations from countries and areas where a detailed and sophisticated classification was irrelevant, but which nevertheless needed a classification based on the ICD in order to assess their progress in health care and in the control of disease. A field test with a bi-axial classification approach—one axis (criterion) for anatomy, with another for etiology—showed the impracticability of such approach for routine use. The final proposals presented to and accepted by the Conference in 1978 retained the basic structure of the ICD, although with much additional detail at the level of the four digit subcategories, and some optional five digit subdivisions. For the benefit of users not requiring such detail, care was taken to ensure that the categories at the three digit level were appropriate. For the benefit of users wishing to produce statistics and indexes oriented towards medical care, the 9th Revision included an optional alternative method of classifying diagnostic statements, including information about both an underlying general disease and a manifestation in a particular organ or site. This system became known as the dagger and asterisk system and is retained in the Tenth Revision. A number of other technical innovations were included in the Ninth Revision, aimed at increasing its flexibility for use in a variety of situations. It was eventually replaced by ICD-10, the version currently in use by the WHO and most countries. Given the widespread expansion in the tenth revision, it is not possible to convert ICD-9 data sets directly into ICD-10 data sets, although some tools are available to help guide users. Publication of ICD-9 without IP restrictions in a world with evolving electronic data systems led to a range of products based on ICD-9, such as MeDRA or the Read directory. When ICD-9 was published by the World Health Organization (WHO), the International Classification of Procedures in Medicine (ICPM) was also developed (1975) and published (1978). The ICPM surgical procedures fascicle was originally created by the United States, based on its adaptations of ICD (called ICDA), which had contained a procedure classification since 1962. ICPM is published separately from the ICD disease classification as a series of supplementary documents called fascicles (bundles or groups of items). Each fascicle contains a classification of modes of laboratory, radiology, surgery, therapy, and other diagnostic procedures. Many countries have adapted and translated the ICPM in parts or as a whole and are using it with amendments since then. International Classification of Diseases, Clinical Modification (ICD-9-CM) is an adaption created by the U.S. National Center for Health Statistics (NCHS) and used in assigning diagnostic and procedure codes associated with inpatient, outpatient, and physician office utilization in the United States. The ICD-9-CM is based on the ICD-9 but provides for additional morbidity detail. It is updated annually on October 1. It consists of two or three volumes: - Volumes 1 and 2 contain diagnosis codes. (Volume 1 is a tabular listing, and volume 2 is an index.) Extended for ICD-9-CM - Volume 3 contains procedure codes for surgical, diagnostic, and therapeutic procedures. ICD-9-CM only The NCHS and the Centers for Medicare and Medicaid Services are the U.S. governmental agencies responsible for overseeing all changes and modifications to the ICD-9-CM. Work on ICD-10 began in 1983, and the new revision was endorsed by the Forty-third World Health Assembly in May 1990. The latest version came into use in WHO Member States starting in 1994. The classification system allows more than 155,000 different codes and permits tracking of many new diagnoses and procedures, a significant expansion on the 17,000 codes available in ICD-9. Adoption was relatively swift in most of the world. Several materials are made available online by WHO to facilitate its use, including a manual, training guidelines, a browser, and files for download. Some countries have adapted the international standard, such as the "ICD-10-AM" published in Australia in 1998 (also used in New Zealand), and the "ICD-10-CA" introduced in Canada in 2000. Adoption of ICD-10-CM was slow in the United States. Since 1979, the US had required ICD-9-CM codes for Medicare and Medicaid claims, and most of the rest of the American medical industry followed suit. On 1 January 1999 the ICD-10 (without clinical extensions) was adopted for reporting mortality, but ICD-9-CM was still used for morbidity. Meanwhile, NCHS received permission from the WHO to create a clinical modification of the ICD-10, and has production of all these systems: - ICD-10-CM, for diagnosis codes, replaces volumes 1 and 2. Annual updates are provided. - ICD-10-PCS, for procedure codes, replaces volume 3. Annual updates are provided. On 21 August 2008, the US Department of Health and Human Services (HHS) proposed new code sets to be used for reporting diagnoses and procedures on health care transactions. Under the proposal, the ICD-9-CM code sets would be replaced with the ICD-10-CM code sets, effective 1 October 2013. On 17 April 2012 the Department of Health and Human Services (HHS) published a proposed rule that would delay, from 1 October 2013 to 1 October 2014, the compliance date for the ICD-10-CM and PCS. Once again, Congress delayed implementation date to 1 October 2015, after it was inserted into "Doc Fix" Bill without debate over objections of many. Revisions to ICD-10-CM Include: - Relevant information for ambulatory and managed care encounter. - Expanded injury codes. - New combination codes for diagnosis/symptoms to reduce the number of codes needed to describe a problem fully. - Addition of sixth and seventh digit classification. - Classification specific to laterality. - Classification refinement for increased data granularity. ICD-10-CA is a clinical modification of ICD-10 developed by the Canadian Institute for Health Information for morbidity classification in Canada. ICD-10-CA applies beyond acute hospital care, and includes conditions and situations that are not diseases but represent risk factors to health, such as occupational and environmental factors, lifestyle and psycho-social circumstances. ICD-11 (stable version for implementation release June 2018)Edit The World Health Organization has revised the International Classification of Diseases (ICD) towards the ICD-11. Its development has taken place on an internet-based workspace that continues to be used as the maintenance platform for discussions, and proposals for updates of ICD. Anybody can submit an evidence based proposal. The proposals are processed in an open tranparent way with reviews for scientific evidence, and usability and utility in the various uses of ICD. It is envisaged, that there will be no need for national modifications of ICD-11, due to its richness and flexibility in the reportable detail The final draft of the ICD-11 system is expected to be submitted to WHO's World Health Assembly (WHA) for official endorsement in 2019. The version for implementation (preparation of approval at the WHA) is released on 18 June 2018. ICD-11 comes with an implementation package that includes transition tables from and to ICD-10, a translation tool, a coding tool, web-services, a manual, training material, and more. All tools are accessible after self-registration from the maintenance platform . The official release is accessed via icd.who.int - About 300 specialists of 55 countries, organized in 30 main working groups have provided their input to make ICD-11 scientifically up-to-date, and structural problems that became apparent using ICD-10 have been solved. - ICD-11 is much easier to use than ICD-10. Its ontological infrastructure allows for an improved user guidance compared to ICD-10. - The systematic reliance on the use of code combinations and extension codes makes ICD finally clinically relevant. With the combinations any condition can be coded to the desired level of detail. - Primary care, cancer coding, traditional medicine (currently module 1: ancient Chinese medicine - China, Korea and Japan), and a section for assessment of functioning are now included. - Specialty versions, as for mental health, primary care, or dermatology are produced from the common core, the foundation. - ICD-11 is truly multilingual. ICD-10 exists in 43 Languages in electronic versions and ICD-11 has already 15 translations in progress. The tooling produces all files and formats from the core translation tooling on the maintenance platform . For authorization of translations, requests have to be made to WHO. - ICD-11 is digital health (formerly e-health) ready due to the use of uniform resource identifiers and its ontological underpinning. The system allows connection of any software through a standard API. The same package is also prepared for use offline. - ICD-11 is based on the electronic foundation component that conains all content, structural information, references and discriptors in a machine readable format. The content is then rendered for machine or human use, electronically or in print. - In ICD-11, each disease entity has a description that give key descriptions and guidance on what the meaning of the entity/category is in human-readable terms, to guide users. This is an advancement over ICD-10, which had only title headings. The Definitions have a standard structure according to a template with standard definition templates and further features exemplified in a "Content Model". The Content Model is a structured framework that captures the knowledge that underpins the definition of an ICD entity. The Content Model therefore allows computerization (with links to ontologies and SNOMED CT). Each ICD entity can be seen from different dimensions or "parameters". For example, there are currently 13 defined main parameters in the Content Model (see below) to describe a category in ICD. An external review of the ICD-11 Revision has been completed. The report notes the progress in the ICD Revision, and makes clear recommendations about forward progress in the revision. - ICD Entity Title - Fully Specified Name - Classification Properties - disease, disorder, injury, etc. - Textual Definitions - short standard description - Terms - synonyms, other inclusion and exclusions - Body System/Structure Description - anatomy and physiology - Temporal Properties - acute, chronic or other - Severity of Subtypes Properties - mild, moderate, severe, or other scales - Manifestation Properties - signs, symptoms - Causal Properties - etiology: infectious, external cause, etc. - Functioning Properties - impact on daily life: activities and participation - Specific Condition Properties - relates to pregnancy etc. - Treatment Properties - specific treatment considerations: e.g. resistance - Diagnostic Criteria - operational definitions for assessment ICD-11 invokes a more sophisticated architecture than historical versions, consistent with its generation as a digital resource. The core content of the system, called the Foundation Component, is a semantic network of words and terms, where any given term can have more than one parent. To address the requirement that statistical classifications exhibit mutual exclusiveness (so events are not counted more than once) and exhaustiveness (so there is a place to tally all events), ICD11 supports the serialization of the Foundation Component into an arbitrary number of linearizations, optimized for use cases. The main linearization, presently called the Joint Linearization for Morbidity and Mortality Statistics, is the tabular format with which most traditional users will become familiar. However, other linearizations, for primary care, multiple sub-specialty derivatives, or applications such as clinical decision support are possible. Finally, preliminary work in partnership with the IHTSDO is underway to ensure that the ICD-11 Foundation Component is semantically coherent through development of the Common Ontology, a subset of SNOMED CT which will anchor the Foundation Component to terms defined through description logic. Usage in the United StatesEdit In the United States, the U.S. Public Health Service published The International Classification of Diseases, Adapted for Indexing of Hospital Records and Operation Classification (ICDA), completed in 1962 and expanding the ICD-7 in a number of areas to more completely meet the indexing needs of hospitals. The U.S. Public Health Service later published the Eighth Revision, International Classification of Diseases, Adapted for Use in the United States, commonly referred to as ICDA-8, for official national morbidity and mortality statistics. This was followed by the ICD, 9th Revision, Clinical Modification, known as ICD-9-CM, published by the U.S. Department of Health and Human Services and used by hospitals and other healthcare facilities to better describe the clinical picture of the patient. The diagnosis component of ICD-9-CM is completely consistent with ICD-9 codes, and remains the data standard for reporting morbidity. National adaptations of the ICD-10 progressed to incorporate both clinical code (ICD-10-CM) and procedure code (ICD-10-PCS) with the revisions completed in 2003. In 2009, the U.S. Centers for Medicare and Medicaid Services announced that it would begin using ICD-10 on April 1, 2010, with full compliance by all involved parties by 2013. The years for which causes of death in the United States have been classified by each revision as follows: Cause of death on United States death certificates, statistically compiled by the Centers for Disease Control and Prevention (CDC), are coded in the ICD, which does not include codes for human and system factors commonly called medical errors. Mental health issuesEdit The ICD includes a section classifying mental and behavioral disorders (Chapter V). This has developed alongside the Diagnostic and Statistical Manual of Mental Disorders (DSM) of the American Psychiatric Association and the two manuals seek to use the same codes. The WHO is revising their classifications in these sections as part the development of the ICD-11 (scheduled for 2018), and an "International Advisory Group" has been established to guide this. Section F66 of the ICD-10 deals with classifications of psychological and behavioural disorders that are associated with sexual development and orientation. It explicitly states that "sexual orientation by itself is not to be considered a disorder," in line with the DSM and other classifications that recognise homosexuality as a normal variation in human sexuality. The Working Group has reported that there is "no evidence that [these classifications] are clinically useful" and recommended that section F66 be deleted for the ICD-11. An international survey of psychiatrists in 66 countries comparing use of the ICD-10 and DSM-IV found that the former was more often used for clinical diagnosis while the latter was more valued for research. The ICD is actually the official system for the US, although many mental health professionals do not realize this due to the dominance of the DSM. A psychologist has stated: "Serious problems with the clinical utility of both the ICD and the DSM are widely acknowledged." - Clinical coder - Medical classifications - Medical diagnosis - Medical terminology - Current Procedural Terminology - MedDRA (Medical Dictionary for Regulatory Activities) - Systematized Nomenclature of Medicine Clinical Terms (SNOMED CT) - WHO Family of International Classifications - "International Classification of Diseases (ICD)". World Health Organization. Archived from the original on 12 February 2014. - "About WHO". World Health Organization. Archived from the original on 9 February 2014. Retrieved 14 March 2014. - "The WHO Family of International Classifications". World Health Organization. Archived from the original on 22 December 2013. Retrieved 14 March 2014. - Information from World Health Organization (WHO): List of Official ICD-10 Updates. For the ICD-11 revision: The ICD 11th Revision is due by 2017 (Archived, Feb. 2014); ICD Revision Timelines and ICD-11 Beta Draft (online beta-version of ICD-11). - "History of the development of the ICD" (PDF). Who.int. Retrieved 11 December 2017. - International health statistics using this system are available at the Global Health Observatory (GHO) and the WHO Statistical Information System (WHOSIS). - Katsching, Heinz (February 2010). "Are psychiatrists an endangered species? Observations on internal and external challenges to the profession". World Psychiatry. World Psychiatric Association. 9 (1): 21–28. doi:10.1002/j.2051-5545.2010.tb00257.x. PMC . PMID 20148149. - ICD-10 Volume 2, online at http:// www.who.int/classifications - "International Classification Of Diseases - 9 - CM, (1979)". Wonder.cdc.gov. Retrieved 11 December 2017. - O'Malley KJ, Cook KF, Price MD, Wildes KR, Hurdle JF, Ashton CM (2005). "Measuring diagnoses: ICD code accuracy". Health Serv Res. 40: 1620–39. doi:10.1111/j.1475-6773.2005.00444.x. PMC . PMID 16178999. - World Health Organization. FAQ on ICD. Accessed 12 July 2011. - National Center for Health Statistics, CDC. ICD-9-CM Guidelines, Conversion Table, and Addenda. Classification of Diseases, Functioning, and Disability. Retrieved 2010-01-24. - InstaCode Institute. ICD-10 Demystified. - "ICD-9-CM Diagnosis Codes - International Classisfication of Diseases - Medical Diagnosis Codes". www.findacode.com. Retrieved 2015-12-30. - WHO. International Classification of Diseases (ICD). - CMS Office of Public Affairs (February 11, 2008). "HHS Proposes Adoption of ICD-10 Code Sets and Updated Electronic Transaction Standards" (Press release). U.S. Department of Health & Human Services. Retrieved 2009-02-11. - New Zealand Health Information Service. ICD-10-AM. Accessed 12 July 2011. - Canadian Institute for Health Information. ICD-10-CA. Archived 2015-05-01 at the Wayback Machine. Accessed 12 July 2011. - International Classification Of Diseases - 9 - CM, (1979). Wonder.cdc.gov. Retrieved on 2014-06-20. - "Classification of Diseases, Functioning, and Disability". U.S. Centers for Disease Control. Retrieved 29 October 2010. - ICD-11 project plan WHO team for classifications, terminologies and standards/IER (12 May 2015) Retrieved on 2018-06-01. - "Revision of International Classification Diseases by 2018 - ABC Live". Abclive.in. Retrieved 11 December 2017. - Makary, MA; Daniel, M (3 May 2016). "Medical error—the third leading cause of death in the US". BMJ: i2139. doi:10.1136/bmj.i2139. - Moriyama, IM; Loy, RM; Robb-Smith, AHT (2011). Rosenberg, HM; Hoyert, DL, eds. History of the Statistical Classification of Diseases and Causes of Death (PDF). Hyattsville, MD: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention, National Center for Health Statistics. ISBN 978-0-8406-0644-0. - Who \| Mental Health Evidence And Research (Mer). Who.int. Retrieved on 2014-06-20. - Cochran, Susan D.; Drescher, Jack; Kismödi, Eszter; Giami, Alain; García-Moreno, Claudia; Atalla, Elham; Marais, Adele; Meloni Vieira, Elisabeth; Reed, Geoffrey M. (2014). "Proposed declassification of disease categories related to sexual orientation in the International Statistical Classification of Diseases and Related Health Problems (ICD-11)". Bulletin of the World Health Organization. 92 (9): 672–679. doi:10.2471/BLT.14.135541. PMC . - Juan E. Mezzich (2002). "International Surveys on the Use of ICD-10 and Related Diagnostic Systems" (guest editorial, abstract). Psychopathology. 35 (2–3): 72–75. doi:10.1159/000065122. PMID 12145487. - Geoffrey M. Reed (2010). "Toward ICD-11: Improving the clinical utility of WHO's International Classification of mental disorders" (PDF). Professional Psychology: Research and Practice. 41 (6): 457–464. doi:10.1037/a0021701. Note: Since adoption of ICD-10 CM in the USA, several online tools have been mushrooming. They all refer to that particular modification and thus are not linked here.	3	31	1	0	0	4	0.791881	5	6,135
As the name suggests, vascular malformations of the brain is an umbrella term for at least six conditions in which blood vessels of the brain are affected. Such malformations are classified into several types in which the symptoms, severity, and causes vary. These types of VMB are: (1) arteriovenous malformations (AVM), abnormal arteries and veins; (2) cavernous malformations (CM), enlarged blood-filled spaces; (3) venous angiomas (VA), abnormal veins; (4) telangiectasias (TA), enlarged capillary-sized vessels; (5) vein of Galen malformations (VGM); and (6) mixed malformations (MM). Vascular malformations of the brain may cause headaches, seizures, strokes, or bleeding in the brain (cerebral hemorrhage). Some researchers believe that the type of malformation determines the symptoms and progression of the disease. Other researchers believe that only the severity rather than the type of malformation is important. Arteriovenous malformations or AVMs affect arteries, veins, and middle- sized vessels but not capillaries. These blood vessels are enlarged, twisted, and tangled. Arteries and veins may be connected directly instead of being connected through fine capillaries for which reason they are often referred to as “shunt lesions” since the capillaries are by-passed. These abnormal “feeding” arteries progressively enlarge and as a result the “draining” veins dilate as well. The brain tissue between these vessels may be hardened or rigid (atrophied), full of a network of fine small fibers (fibrils) interspersed with flattened cells (gliotic), and sometimes may be calcified. Such malformations may, by drawing blood away from the brain, cause brain cell atrophy. Hemorrhages or seizures are commonly experienced with AVMs. (For more information on this disorder choose “Arteriovenous” for your search term in the Rare Disease Database.) Cavernous malformations, CMs (also called cavernous angiomas, or cavernous hemangiomas, or cavernomas) present as abnormally enlarged collections of blood-filled spaces. A cavernous hemangioma acts like a “blood sponge” soaking up blood that has found its way between capillaries, in the spaces between tissues (sinusoids) and “larger cavernous spaces.” These are “slow-flow lesions.” There is not usually any brain tissue in these spaces in contrast with symptoms of AVMs. Hemorrhages or seizures are also common with CMs. (For more information on this disorder choose “cavernous hemangioma” for your search term in the Rare Disease Database.) Venous angiomas (VAs) involve enlarged, tangled, and twisted veins that vary in size but do not involve the arteries. The site of these “growths” is most often just after the capillary stage of the vessel (post-capillary malformation). They may be isolated defects or associated with cavernous malformations. The defect shows itself as a “crown” of small veins (venules) that meet to form part of a larger vein (trunk). Telangiectasias are the malformations that arise as a result of the enlarging (dilation) of the tiny capillaries. These dilated capillaries make themselves known as small pink-red spots in various parts of the body such as the face, eyes, membranes that cover the brain (dura) and spinal cord (meninges), and mucous membranes (the thin moist layer lining the body’s internal surfaces). (For more information on a disorder involving telangiectasias choose “hemorrhagic telangiectasia, hereditary” for your search term in the Rare Disease Database.) Vein of Galen malformations (VGMs begin while the embryo is developing. The vein of Galen is located under the cerebral hemispheres and drains the forward (anterior) and central regions of the brain into the proper sinuses. The malformations occur when the vein of Galen is not supported within the head by surrounding tissue and lacks the normal fibrous wall. Thus, the vein of Galen appears free-floating within the fluids of the cerebral spaces (sinuses). Should the pressure increase within the vein of Galen, its shape changes from a cylinder to that of a sphere. Such changes are accompanied by abnormal fetal blood circulation. In extreme cases, there may be cardiac failure or swelling of the brain (hydrocephalus). Mixed malformation is a phrase used to include any of several multiple-mixed malformations. Frequently, these malformations appear to be mixes of arteriovenous malformations with telangiectasias. Three types or forms of VMB have a genetic component. The evidence for a genetic cause is strong in the case of cavernous hemangiomas and telangiectasias. The case is much weaker for arteriovenous malformation of the brain (AVM). In each of these cases, the condition is transmitted as an autosomal dominant trait. The malfunctioning gene in the case of cavernous malformations has been tracked to gene map locus 7q11.2-q21, and in the case of telangiectasia to gene map locus 9q34.1. Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Human body cells normally have 46 chromosomes. Pairs of human chromosomes are numbered from 1 through 22 and the sex chromosomes are designated X and Y. Males have one X and one Y chromosome and females have two X chromosomes. Each chromosome has a short arm designated “p” and a long arm designated “q”. Chromosomes are further sub-divided into many bands that are numbered. For example, “chromosome 9q34.1″ refers to band 34.1 on the long arm of chromosome 7. The numbered bands specify the location of the thousands of genes that are present on each chromosome. To say that the abnormal gene is located at 7q11.2-q21 means that the gene in question is located in a region on the long arm of chromosome 7 between bands 11.2 and 21. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child. Recessive genetic disorders occur when an individual inherits the same abnormal gene for the same trait from each parent. If an individual receives one normal gene and one gene for the disease, the person will be a carrier for the disease but usually will not show symptoms. The risk for two carrier parents to both pass the defective gene and, therefore, have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier like the parents is 50% with each pregnancy. The chance for a child to receive normal genes from both parents and be genetically normal for that particular trait is 25%. The risk is the same for males and females. All individuals carry a few abnormal genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder. X-linked recessive genetic disorders are conditions caused by an abnormal gene on the X chromosome. Females have two X chromosomes but one of the X chromosomes is “turned off” and all of the genes on that chromosome are inactivated. Females who have a disease gene present on one of their X chromosomes are carriers for that disorder. Carrier females usually do not display symptoms of the disorder because it is usually the X chromosome with the abnormal gene that is “turned off”. A male has one X chromosome and if he inherits an X chromosome that contains a disease gene, he will develop the disease. Males with X-linked disorders pass the disease gene to all of their daughters, who will be carriers. A male cannot pass an X-linked gene to his sons because males always pass their Y chromosome instead of their X chromosome to male offspring. Female carriers of an X-linked disorder have a 25% chance with each pregnancy to have a carrier daughter like themselves, a 25% chance to have a non-carrier daughter, a 25% chance to have a son affected with the disease, and a 25% chance to have an unaffected son. X-linked dominant disorders are also caused by an abnormal gene on the X chromosome, but in these rare conditions, females with an abnormal gene are affected with the disease. Males with an abnormal gene are more severely affected than females, and many of these males do not survive. Vascular malformations of the brain affect males and females in equal numbers. A hereditary form of cavernous malformations tends to occur more frequently in Mexican-Americans. Arteriovenous malformations occur more frequently in males. Imaging apparatus, such as magnetic resonance imaging (MRI), computed tomography (CT) scans, venograms and/or digital intravenous or common angiography can take pictures of the brain's blood vessels to see if vascular malformations are present. Current treatment options vary according to the severity and location of the malformation. Surgical removal (resection), multiple embolization (an operation in which pellets are put into the circulatory system in order to block blood flow to and/or from the abnormal blood vessels), and irradiation are the treatments currently in use. In some cases, treatment may not be necessary. Recently introduced techniques involve particle beam and stereotaxic radio-surgery. Genetic counseling may be of benefit for patients and their families if they have a hereditary form of this disorder. Other treatment is symptomatic and supportive. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website. For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office: Tollfree: (800) 411-1222 TTY: (866) 411-1010 For information about clinical trials sponsored by private sources, contact: Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:244-45; 1427; 1482. Smith WS, Johnston SC, Easton JD. Cerebrovascular Diseases. In: Kasper, DL, Fauci AS, Longo DL, et al. Eds. Harrison’s Principles of Internal Medicine. 16th ed. McGraw-Hill Companies. New York, NY; 2005:2392-93. Marchuk DA, Berg JN. ENG and ALK1 and Hereditary Hemorrhagic Telangiectasia (Osler-Weber-Rendu Syndrome) and Vascular Morphogenesis. In: Epstein CJ, Erickson RP, Wynshaw-Boris, eds. Inborn Errors of Development. 1st ed. Oxford University Press. New York, NY; 2004:319-28. Johnston MV. Acute Stroke Syndromes. In: Behrman RE, Kliegman RM, Jenson HB. Eds. Nelson Textbook of Pediatrics. 17th ed. Elsevier Saunders. Philadelphia, PA; 2005:2036-37. Solomon RA, Pile-Spellman J, Mohr JP. Vascular Tumors and Malformations. In: Rowland LP. Ed. Merritt’s Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:367-71. Guttmacher AE, McDonald JE. Hereditary Hemorrhagic Telangiectasia. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:418. Choi JH, Mohr JP. Brain arteriovenous malformations in adults. Lancet Neurol. 2005;4:299-308. Hussain MS, Qureshi AI, Kirmani JF, et al. Update on endovascular treatment of cerebrovascular diseases. J Endovasc Ther. 2004;11 Suppl 2:II32-42. Brown RD Jr, Flemming KD, Meyer FB, et al. Natural history, evaluation, and management of intracranial vascular malformations. Mayo Clin Proc. 2005;80:269-81. Schauble B, Cascino GD, Pollock BE, et al. Seizure outcomes after stereotactic radiosurgery for cerebral arteriovenous malformations. Yamada S, Brauer FS, Colohan AR, et al. Concept of arteriovenous malformation compartments and surgical management. Neurol Res. 2004;26:288-300. Raychaudhuri R, Batjer HH, Awad IA. Intracranial cavernous hemangioma: a practical review of clinical and biological aspects. Surg Neurol. 2005;63:319-28. Byrne JV. Cerebrovascular malformations. Eur Radiol. 2005;15:448-52. Yasui T, Komiyama M, Iwai Y, et al. A brainstem cavernoma demonstrating a dramatic, spontaneous decrease in size during follow-up: case report and review of the literature. Surg Neurol. 2005;63:170-73; discussion 173. Baumgartner JE, Ater JL, Ha CS, et al. Pathologically proven cavernousangiomas of the brain following radiation therapy for pediatric brain tumors. Pediatr Neurosurg. 2003;39:201-07. Zhou LF, Mao Y, Chen L. Diagnosis and surgical treatment of cavernous sinus hemangiomas: an experience of 20 cases. Surg Neurol. 2003;60:31-36; discussion 36-37. Bilaniuk LT. Vascular lesions of the orbit in children. Neuroimaging Clin N Am. 2005;15:107-20. Chiller KG, Frieden IJ, Arbiser JL. Molecular pathogenesis of vascular anomalies: classification into three categories based upon clinical and biochemical characteristics. Lymphat Res Biol. 2003;1:267-81. Metry DW. Potential complications of segmental hemangiomas of infancy. Semin Cutan Med Surg. 2004;23:107-15. Tille JC, Pepper MS. Hereditary vascular anomalies: new insights into their pathogenesis. Arterioscler Thromb Vasc Biol. 2004;24:1578-90. Vein of Galen malformations Kubi N, Levy BI. Understanding angiogenesis: a clue for understanding vascular malformations. J Neuroradiol. 2004;31:365-68. Greene AK, Burrows PE, Smith L, et al. Periorbital lymphatic malformation: clinical course and management in 42 patients. Plast Reconstr Surg. 2005;115:22-30. Gupta AK, Varma DR. Vein of Galen malformations: review. Neurol India. 2004;52:43-53. Punt J. Surgical management of paediatric stroke. Pediatr Radiol. 2004;34:16-23. Freedom RM, Yoo SJ, Perrin D. The biological “scrabble” of pulmonary arteriovenous malformations: considerations in the setting of cavopulmonary surgery. Cardiol Young. 2004;14:417-37. Woods CG. Human microcephaly. Curr Opin Neurobiol. 2004;14:112-17. Marchuk DA, Srinivasan S, Squire TL, et al. Vascular morphogenesis: tales of two syndromes. Hum Mol Genet. 2003; 12 Spec No 1:R97-112. Sabba C, Pasculli G, Cirulli A, et al. Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). Minerva Cardioangiol. 2002;50:221-38. Ataxia telangiectasia with vascular abnormalities in the brain parenchyma: report of an autopsy case and literature review. Pathol Int. 2001;51:271-76. FROM THE INTERNET McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Cerebral Cavernous Malformations; CCM. Entry Number; 116860: Last Edit Date; 11/2/2005. McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Telangiectasia, Hereditary Hemorrhagic, of Rendu, Osler, and Weber; HHT. Entry Number; 187300: Last Edit Date; 2/18/2005.. McKusick VA, ed. Online Mendelian Inheritance In Man (OMIM). The Johns Hopkins University. Arteriovenous malformations of the Brain. Entry Number; 108010: Last Edit Date; 2/7/2000. Wagner AL. Brain, Venous Vascular Malformations. emedicine. Last Updated: August 21, 2002. 8pp.	2	7	0	0	0	1	0.925237	1	3,849
The Merriam-Webster Dictionary defines hypersexual as "exhibiting unusual or excessive concern with or indulgence in sexual activity." Sexologists have been using the term hypersexuality since the late 1800s, when Krafft-Ebing described several cases of extreme sexual behaviours in his seminal 1886 book, Psychopathia Sexualis. The author used the term "hypersexuality" to describe conditions that would now be termed premature ejaculation. Hypersexuality may be a primary condition, or the symptom of another medical disease or condition, for example Klüver-Bucy syndrome or bipolar disorder. Hypersexuality may also present as a side effect of medication such as drugs used to treat Parkinson's disease. Clinicians have yet to reach a consensus over how best to describe hypersexuality as a primary condition, or to determine the appropriateness of describing such behaviors and impulses as a separate pathology. Some authors have questioned whether it makes sense to discuss hypersexuality at all, arguing that labeling sexual urges "extreme" merely stigmatizes people who do not conform to the norms of their culture or peer group. Hypersexual behaviours are viewed variously by clinicians and therapists as: an addiction; a type of obsessive-compulsive disorder (OCD) or “OCD-spectrum disorder”; or a disorder of impulsivity. A number of authors do not acknowledge such a pathology and instead assert that the condition merely reflects a cultural dislike of exceptional sexual behavior. Consistent with there not being any consensus over what causes hypersexuality, authors have used many different labels to refer to it, sometimes interchangeably, but often depending on which theory they favor or which specific behavior they were studying. Contemporary names include compulsive masturbation, compulsive sexual behavior, cybersex addiction, erotomania, “excessive sexual drive”, hyperphilia, hypersexuality, hypersexual disorder, problematic hypersexuality, sexual addiction, sexual compulsivity, sexual dependency, sexual impulsivity, “out of control sexual behavior”, and paraphilia-related disorder. There is little consensus among experts as to the causes of hypersexuality. Some research suggests that some cases can be linked to biochemical or physiological changes that accompany dementia. Psychological needs also complicate the biological explanation, which identifies the temporal/frontal lobe of the brain as the area for regulating libido. Persons suffering from injuries to this part of the brain are at increased risk for aggressive behavior and other behavioral problems including personality changes and socially inappropriate sexual behavior such as hypersexuality. The same symptom can occur after unilateral temporal lobotomy. There are other biological factors that are associated with hypersexuality such as premenstrual changes, and the exposure to virilising hormones in childhood or in utero. In research involving use of anti-androgens to reduce undesirable sexual behaviour such as hypersexuality, testosterone is deemed necessary, but not sufficient, for sexual drive. Other proposed factors include a lack of physical closeness, and forgetfulness of the recent past. Pathogenic overactivity of the dopaminergic mesolimbic pathway in the brain—forming either psychiatrically, during mania, or pharmacologically, as a side effect of dopamine agonists, specifically D3-preferring agonists—is associated with various addictions and has been shown to result among some in overindulgent, sometimes hypersexual, behavior. As a symptom Hypersexuality is known to present itself as a symptom in connection to a number of mental and neurological disorders. Some people with borderline personality disorder (sometimes referred to as BPD) can be markedly impulsive, seductive, and extremely sexual. Sexual promiscuity, sexual obsessions, and hypersexuality are very common symptoms for both men and women with BPD. On occasion for some there can be paraphilic drives such as voyeurism, necrophilia, sadomasochism, urolagnia, and other more extreme forms of paraphilic drives and desires. 'Borderline' patients, due in the opinion of some to the use of splitting, experience love and sexuality in perverse and violent qualities which they cannot integrate with the tender, intimate side of relationships. People who suffer from bipolar disorder may often display tremendous swings in sex drive depending on their mood. As defined in the DSM-IV-TR, hypersexuality can be a symptom of hypomania or mania in bipolar disorder or schizoaffective disorder. Pick’s disease causes damage to the temporal/frontal lobe of the brain; people suffering with Pick’s disease show a range of socially inappropriate behaviors. Several neurological conditions such as Alzheimer's disease, Attention deficit/hyperactivity disorder(ADHD), Autism, various types of brain injury, Klüver-Bucy syndrome, Kleine-Levin syndrome, and many more neurodegenerative diseases can cause hypersexual behavior. Sexually inappropriate behavior has been shown to occur in 7-8% of Alzheimer's patients living at home, at a care facility or in a hospital setting. Hypersexuality has also been reported to result as a side-effect of some medications used to treat Parkinson's disease. Some street drugs, such as methamphetamine, may also contribute to hypersexual behavior. A positive link between the severity of dementia and occurrence of inappropriate behavior has also been found. Hypersexuality can be caused by dementia in a number of ways, including disinhibition due to organic disease, misreading of social cues, understimulation, the persistence of learned sexual behaviour after other behaviours have been lost, and the side-effects of the drugs used to treat dementia. Other possible causes of dementia-related hypersexuality include an inappropriately expressed psychological need for intimacy and forgetfulness of the recent past. As this illness progresses, increasing hypersexuality has been theorized to sometimes compensate for declining self-esteem and cognitive function. As a disorder Hypersexuality may negatively impact an individual. The concept of hypersexuality as an addiction was started in the 1970s by former members of Alcoholics Anonymous who felt they experienced a similar lack of control and compulsivity with sexual behaviors as with alcohol. Multiple 12-step style self-help groups now exist for people who identify as sex addicts, including Sex Addicts Anonymous, Sexaholics Anonymous, Sex and Love Addicts Anonymous, and Sexual Compulsives Anonymous. As of 2010[update], a proposal to add Sexual Addiction to the Diagnostic and Statistical Manual of Mental Disorders (DSM) system has failed to get support of the American Psychiatric Association (APA). Some authors have questioned whether it makes sense to discuss hypersexuality at all, arguing that labeling sexual urges "extreme" merely stigmatizes people who do not conform to the norms of their culture or peer group. The DSM does include an entry called Sexual Disorder Not Otherwise Specified (Sexual Disorder NOS) to apply to, among other conditions, "distress about a pattern of repeated sexual relationships involving a succession of lovers who are experienced by the individual only as things to be used." The International Statistical Classification of Diseases and Related Health Problems (ICD-10) of the World Health Organization (WHO), includes two relevant entries. One is "Excessive Sexual Drive" (coded F52.7), which is divided into satyriasis for males and nymphomania for females. The other is "Excessive Masturbation" or "Onanism (excessive)" (coded F98.8). - Persistent genital arousal disorder - Pornography addiction - Sexual Compulsivity Scale - Sexual obsessions - Krafft-Ebing, R. von (1886/1965). Psychopathia sexualis: A medico–forensic study (H. E. Wedeck, Trans.). New York: Putnam. - Kafka, M. P. (2010). Hypersexual Disorder: A proposed diagnosis for DSM-V. Archives of Sexual Behavior, 39, 377–400. - Stein, D. J. (2008). Classifying hypersexual disorders: Compulsive, impulsive, and addictive models. Psychiatric Clinics of North America, 31, 587–592. - Bancroft, J., & Vukadinovic, Z. (2004). Sexual addiction, sexual compulsivity, sexual impulsivity or what? Toward a theoretical model. Journal of Sex Research, 41, 225–234. - Coleman, E. (July 1986). "Sexual Compulsion vs. Sexual Addiction: The Debate Continues" (PDF). SIECUS Report (ProQuest Academic Research Library) 14 (6): 7–11. Retrieved 2012-10-15. - Levine, M. P; Troiden, R. R. (1988). "The Myth of Sexual Compulsivity". Journal of Sex Research 25 (3): 347–363. doi:10.1080/00224498809551467. - Orford, J. (1985). Excessive appetites: A psychological view of the addictions. Chichester, England: John Wiley & Sons. - Carnes, P. (1983). Out of the shadows: Understanding sexual addiction. Minneapolis, MN: CompCare. - Levine, S. B. (2010). What is sexual addiction? Journal of Sex & Marital Therapy, 36, 261–275. - Rinehart, N. J., & McCabe, M. P. (1997). Hypersexuality: Psychopathology or normal variant of sexuality? Sexual and Marital Therapy, 12, 45–60. - Quadland, M. C. (1985). Compulsive sexual behavior: Definition of a problem and an approach to treatment. Journal of Sex and Marital Therapy, 11, 121–132. - Coleman, E. (1990). The obsessive–compulsive model for describing compulsive sexual behavior. American Journal of Preventive Psychiatry & Neurology, 2, 9–14. - "ICD-10 entry for "Excessive sexual drive."". Apps.who.int. Retrieved 2012-06-22. - Money, J. (1980). Love and love sickness. The science of sex, gender difference, and pair bonding. Baltimore, MD: Johns Hopkins University Press. - Miller, B. L., Cummings, J. L., & McIntyre, H. (1986). Hypersexuality and altered sexual preferences following brain injury. Journal of Neurology, Neurosurgery and Psychiatry, 49, 867–873. - Orford, J. (1978). Hypersexuality: Implications for a theory of dependence. British Journal of Addiction, 73, 299–310. - Krueger, R. B., & Kaplan, M. S. (2001). The paraphilic and hypersexual disorders. Journal of Psychiatric Practice, 7, 391–403. - Kingston, D. A., & Firestone, P. (2008). Problematic hypersexuality: Review of conceptualization and diagnosis. Sexual Addiction and Compulsivity, 15, 284–310. - Dodge, B., Reece, M., Cole, S. L., & Sandfort, T. G. M. (2004). Sexual compulsivity among heterosexual college students. Journal of Sex Research, 41, 343–350. - Kafka, M. P. (1995b). Sexual impulsivity. In E. Hollander & D. J. Stein (Eds.), Impulsivity and aggression (pp. 201–228). Chichester, England: John Wiley. - Bancroft, J. (2008). Sexual behavior that is "out of control": A theoretical conceptual approach. Psychiatric Clinics of North America, 31, 593-601. - Kafka, M. P. (1994). Paraphilia-related disorders: Common, neglected, and misunderstood. Harvard Review of Psychiatry, 2, 39–40. - Kafka, M. P. (2000). The paraphilia-related disorders: Nonparaphilic hypersexuality and sexual compulsivity/addiction. In S. R. Leiblum & R. C. Rosen (Eds.), Principles and practice of sex therapy (3rd ed., pp. 471–503). New York: Guilford Press. - Kafka, M. P. (2001). The paraphilia-related disorders: A proposal for a unified classification of nonparaphilic hypersexuality disorders. Sexual Addiction & Compulsivity, 8, 227–239. - Orford, J. (1978). Hypersexuality: Implications for a theory of dependence. British Journal of Addiction, 73, 299-320. - Ellis, A., & Sagarin, E. (1965). Nymphomania: A Study of oversexed women. London: Ortolan. - Robinson, Karen M. DNS, RN, CS, FAAN (January 2003). "Understanding Hypersexuality: A Behavioral Disorder of Dementia". Home Healthcare Nurse: the Journal for the Home Care and Hospice Professional 21: 43. doi:10.1097/00004045-200301000-00010. - Devinsky, Julie; Oliver Devinsk; Orrin Sacks (18 Nov 2009). "Neurocase: The Neural Basis of Cognition". Klüver–Bucy syndrome, hypersexuality, and the law 16 (2): 140–145. doi:10.1080/13554790903329182. - Catalan, (1995). Hypersexuality revisited, The Journal of Forensic Psychiatry, 6:2, 255–258. - Catalan, J. and Singh, A., "Hypersexuality Revisited", The Journal of Forensic Psychiatry Volume 6, Issue 2, 1995. - Robinson, K. M. (2003). Understanding Hypersexuality: A Behavioral Disorder of Dementia. Home Healthcare Nurse: The Journal for the Home Care and Hospice Professional, 43-47. - Silverstone T, T (1985). "Dopamine in manic depressive illness. A pharmacological synthesis". Journal of Affective Disorders 8 (3): 225–31. doi:10.1016/0165-0327(85)90020-5. PMID 2862169. - "MedlinePlus Drug Information: Pramipexole (Systemic)". United States National Library of Medicine. Archived from the original on 2006-09-26. Retrieved 2006-09-27. - Boyd, Alan (1995). "Bromocriptine and psychosis: A literature review". Psychiatric Quarterly 66 (1): 87–95. doi:10.1007/BF02238717. PMID 7701022. Retrieved 2008-09-06. - Arias-Carrión O, Pöppel E (2007). "Dopamine, learning and reward-seeking behavior". Act Neurobiol Exp 67 (4): 481–488. - Eric J. Nestler, Department of Psychiatry and Center for Basic Neuroscience, The University of Texas Southwestern Medical Center (2005). "Is There A Common Molecular Pathway For Addiction?" (PDF). Nature Neuroscience 8 (11): 1445–1449. doi:10.1038/nn1578. PMID 16251986. - Mitchell, Stephen (1995). Freud and Beyond: A History of Modern Psychoanalytic Thought. New York: Basic Books. ISBN 978-0-465-01405-7. - Cummings, J. L. Dementia: A clinical approach (2nd ed). Boston: Butterworth-Heinemann. - Jones, M. C.; Okere, K. (2008). "Treatment of Hypersexual Behavior with Oral Estrogen in an Autistic Male". Southern Medical Journal 101 (9): 959–960. doi:10.1097/SMJ.0b013e318180b3de. PMID 18708975. - Dhikav V, Anand K, Aggarwal N (April 2007). "Grossly disinhibited sexual behavior in dementia of Alzheimer's type". Arch Sex Behav 36 (2): 133–4. doi:10.1007/s10508-006-9144-1. PMID 17308974. - Miller BL, Cummings JL, McIntyre H, Ebers G, Grode M (August 1986). "Hypersexuality or altered sexual preference following brain injury". J. Neurol. Neurosurg. Psychiatr. 49 (8): 867–73. doi:10.1136/jnnp.49.8.867. PMC 1028946. PMID 3746322. - National Institute of Neurological Disorders and Stroke. "NINDS Klüver-Bucy Syndrome Information Page". Retrieved 2009-10-10. - Arnulf I, Zeitzer JM, File J, Farber N, Mignot E (December 2005). "Kleine-Levin syndrome: a systematic review of 186 cases in the literature". Brain 128 (Pt 12): 2763–76. doi:10.1093/brain/awh620. PMID 16230322. - Vogel, H. P., & Schiffter, R. (1983). Hypersexuality: A complication of dopaminergic therapy in Parkinson's disease. Pharmacopsychiatria, 16, 107-110. - Uitti, R. J., Tanner, C. M., & Rajput, A. H. (1989). Hypersexuality with antiparkinsonian therapy. Clinical Neuropharmacology, 12, 375-383. - Mansergh, G., Purcell, D. W., Stall, R., McFarlane, M., Semaan, S., Valentine, J., et al. (2006). CDC consultation on methamphetamine use and sexual risk behavior for HIV/STD infection: Summary and suggestions. Public Health Reports, 121, 127-132. - Burns, A.; Jacoby, R. & Levy, R. (1990). "Psychiatric phenomena in Alzheimer's disease. IV: Disorders of behavior.". British Journal of Psychiatry 157: 86–94. doi:10.1192/bjp.157.1.86. PMID 2397368. - Series, H.; Dégano, P. (2005). "Hypersexuality in dementia". Advances in Psychiatric Treatment 11 (6): 424. doi:10.1192/apt.11.6.424. - Karen M. Robinson, DNS, RN, DS, FAAN. (Jan 2003). Understanding Hyper sexuality, A Behavioral Disorder of Dementia. Home Healthcare Nurse. Vol.21, no.1. - Goleman, Daniel (October 16, 1984). "Some Sexual Behavior Viewed as an Addiction". New York Times: Cl, C9. Retrieved 2012-10-15. - Rubin, Rita (2010-02-09). "Psychiatry's bible: Autism, binge-eating updates proposed for 'DSM'". USA Today. - "Black Friday deals for Target, H&M, Forever21, Old Navy, Radio Shack and more". Daily News (New York). 2010-02-10.[dead link] - "New Diagnostic Guidelines for Mental Illnesses Proposed". Health.usnews.com. 2010-02-10. Retrieved 2012-06-22. - American Psychiatric Association. (2000). Diagnostic and statistical manual of mental disorders (4th ed., text revision). Washington, DC: Author. - "2012 ICD-10 Diagnosis Code F52.7 : Excessive sexual drive". Retrieved 2013-02-22. - "2012 ICD-10-CM Diagnosis Code F98.8 : Other specified behavioral and emotional disorders with onset usually occurring in childhood and adolescence". Icd10data.com. Retrieved 2012-06-22.	2	12	3	0	0	7	0.781837	10	4,153
Clinical and Health Affairs What You Can Do Now to Prepare for ICD-10 By Patrice Kuppe ■ The United States is moving toward adoption of the 10th version of the World Health Organization’s International Classification of Diseases (ICD) codes. Because the change will have a significant impact on electronic health record and billing systems, ICD-10 is being rolled out in phases over the next couple of years. Physicians will need to begin using the new diagnosis codes starting in October 2013. This article describes the differences between ICD-9 and ICD-10 and the steps physicians and clinics can take now to prepare for the implementation. Big changes are underway in the realm of medical coding. Thanks to a mandate under the Health Insurance Portability and Accountability Act, hospitals, clinics, and health plans throughout the country are in the process of preparing for the implementation of the new International Classification of Diseases (ICD) 10 diagnosis and procedural codes for medical documentation and billing. The United States is one of the last countries in the world to adopt the 10th version of the codes, which was endorsed by the World Health Organization in 1990. Because the change will have an enormous impact on electronic health record (EHR) and billing systems and the people who use them, it will take place in phases over the next several years. Billing and information technology personnel are already working to meet a January 1, 2012, deadline for upgrading systems to meet new standards for electronic claims and other revenue cycle transactions. But for most physicians, October 1, 2013, is the deadline to pay attention to, as that is when they will have to begin using ICD-10-CM, the new diagnosis codes. (Physician services will continue to be coded using Current Procedural Terminology [CPT] codes.) By this same date, in addition to the ICD-10-CM codes, hospitals must start using ICD-10-PCS codes to report the resources and procedures used in inpatient cases (Table 1). There will be no grace period for implementation. The ICD-10 code set replaces ICD-9, which was adopted by the World Health Organization in 1975, the year Microsoft was founded and eight years before the virus that causes AIDS was identified. Obviously, much has changed with regard to computing and medicine since then. Not only does ICD-9 no longer accurately describe the practice of medicine, it is inadequate for meeting the demands of medical record-keeping in the 21st century, which include tracking quality measures, monitoring potential public health risks, and submitting utilization data. In some cases, ICD-9 simply has no code for a condition. For example, it initially had no code for severe acute respiratory syndrome (SARS); a special update had to be made in 2003 to add codes for SARS. In other cases, terminology is outdated and inconsistent with current Questions to Ask Your EHR and Billing System Vendors - How will their application, business processes, or systems address your needs during implemention of ICD-10? Will they maintain tables for each code set? How long will ICD-9 be available for use? Some will propose an embedded or proprietary solution, while others will delegate the responsibility to the user organization. - What is the migration strategy for making the change to ICD-10? Will multiple upgrades be required? This may be a concern for organizations that are not using the latest release of a vendor-supported application. - Will they ensure you have the right tools in place to help you select the more specific diagnosis codes? Will they provide specific provider templates for each specialty? medical practice. Asthma is one example where ICD-10-CM is much more precise and accurate than ICD-9-CM. With ICD-10-CM, asthma is classified as mild intermittent, mild persistent, moderate, or severe. Current guidelines base diagnosis and treatment of asthma according to these categories. But ICD-9-CM classifies asthma as intrinsic and extrinsic, which is no longer relevant for treatment. Thus, using ICD-9-CM codes to analyze treatment outcomes, prevalence of asthma in their patient population, and occurrences of acute episodes of asthma would not yield good data. ICD-9 has simply outlasted its usefulness. ICD-10-CM versus ICD-9-CM Physicians need to understand that the ICD-10-CM system is significantly different than the ICD-9-CM system. First, the number of codes will increase dramatically—from about 13,000 to 68,000. Second, the codes themselves are very different. The ICD-9-CM codes are only three to five characters long. The ICD-10-CM codes will be three to seven characters. In ICD-10-CM, the first character is alpha; characters 2 and 3 are numeric; characters 4 through 7 are alpha or numeric (Table 2). The increased number of codes and the change in code length, combined with considerably more code granularity, allows for much greater specificity. For example, under ICD-9-CM, there is one code for a patient with a traumatic closed fracture of the shaft of the radius and ulna (813.23). Under ICD-10-CM, there are multiple possibilities, as the fourth character of the code will identify the type of fracture (eg, greenstick or transverse), the fifth and sixth characters the location and condition (right or left side and in some cases whether the fracture was considered displaced or nondisplaced), and the final character if the encounter was initial, subsequent, or sequela. The resulting code might look like this: A52.131A—indicating displaced fracture of neck of right radius, initial encounter for closed fracture. Implementing the Change Given these differences, all provider organizations and health plans will need to engage in significant planning to make EHR and billing system modifications or upgrades. They also will need to provide training and ongoing support to staff. The key for successful migration to ICD-10 is to establish an environment in which new and old technology, along with like and unlike data sets, can co-exist and where information exchange can occur while the re-engineering of existing workflow and software takes place. Each provider organization will need to review all of its processes, systems, and reports and document where ICD-9 codes are currently used. In addition, each organization should conduct a financial impact analysis to determine if the new levels of specificity will change the reimbursements they receive from the government or commercial health plans. To navigate the challenges, the Center for Medicare and Medicaid Services is developing general equivalence mapping (GEM) tools to convert data from ICD-9-CM to ICD-10-CM and vice versa.1 The GEMs will be like dictionaries that will enable users to translate from one code set to the other. The mapping tools can be used to help you calculate reimbursement, format new provider-specific prompts, and update reports or forms. But the GEMs should be used with care for a number of reasons: - There are new concepts in ICD- 10-CM that are not present in ICD-9-CM; - In a few cases, the GEMs may have no matching codes; - There may be multiple ICD-9-CM codes for a single ICD-10-CM code; and - There may be multiple ICD-10-CM codes for a single ICD-9-CM code. Although these tools will aid during the transition period, organizations will still need to work with their EHR and billing-system vendors to ensure that the transition goes smoothly (see “Questions to Ask Your EHR and Billing System Vendors”). They will have to decide how long they will keep ICD-9 codes since the codes are attached to the date of service and not to the date the record or claim was created. And those organizations that do not use EHRs will need to update their charge sheets and make sure their billing system is ready. Finally, no tool will be a substitute for learning the ICD-10 codes. Thus, all health care providers, coders, and support and billing staff will need to be trained. Start Preparing Now Clearly, organizations should be getting ready for this change. A number of steps should be taken well before October 1, 2013: - Create a project team. Assign an executive to spearhead the work and to create awareness of the coming changes among both clinical and financial staff. - Conduct an assessment. List the places where codes are used and stored. - Talk to your software vendors about what the change could mean in terms of your systems. Successful conversion to ICD-10 will depend heavily on when your vendor has the upgrades completed and when they can be installed in your system. - Identify the changes that you need to make in your practice to convert to the ICD-10 code set. For example, your diagnosis coding tools, “super bills,” and public health reporting tools will need to be updated, and you will need to make it clear which code list to use based on the date of service. - Identify staff training needs and complete the necessary training. - Conduct internal testing to make sure you can generate transactions with the ICD-10 codes. - Conduct external testing with your clearinghouses and payers to make sure you can send and receive transactions with the ICD-10 codes. - Conduct a financial assessment. The transition from ICD-9 to ICD-10 presents health care providers with a number of financial opportunities and risks, both during the transition period and over the long term. You should identify how the change could affect your organization in terms of financial performance, availability of working capital, and financial reporting. We’ve handled changes in coding before. After an initial outcry over the conversion to the “new” CPT E/M code system in the early 1990s, we all adapted. In the end, the transition went relatively smoothly for those who took the time to plan and prepare. The move to ICD-10-CM will also be smooth if we start preparing now. MM Patrice Kuppe is director of administrative simplification for Allina Health System. 1. Centers for Medicare and Medicaid Services. ICD-10 Provider Resources. Available at: https://www.cms.gov/ICD10/05a_ProviderResources.asp#TopOfPage. Accessed: March 9, 2011. ICD-10 Summary 15-Minute Webinars *discount for MMA Members!	4	6	0	0	0	2	0.397866	2	2,211
Coding Complications of Pregnancy: Hypertension, Pre-eclampsia, Eclampsia and ICD-10 Hypertension is dangerous during pregnancy because it may interfere with the placenta's ability to deliver oxygen and nutrition to the fetus and has also been noted to be a contributing factor in low-birthweight babies. Pregnant patients may have other health problems too, such as gestational diabetes, that can contribute to the complexity of the pregnancy. These pregnancy complication may necessitate a patient be induced for delivery prior to the “normal” timeframe of 38-40 weeks of gestation. If induced for delivery, the patient will be closely monitored for a vaginal, or if more complications arrise, be delivered via cesarean section. Women with hypertension in pregnancy have a higher risk of complications such as: · Abruptio placentae. (Placental abruption) · Cerebrovascular accident. (CVA) · Disseminated intravascular coagulation. (DIC) The fetus has an increased risk of: · Intrauterine growth restriction. (IUGR) · Intrauterine death. As you can see in the table below, ICD-10cm gives us these codes to be used when hypertension is a factor in pregnancy, childbirth and the puerperium. O10 Pre-existing hypertension complicating pregnancy, childbirth and the puerperium O11 Pre-existing hypertension with pre-eclampsia O12 Gestational [pregnancy-induced] edema and proteinuria without hypertension O13 Gestational [pregnancy-induced] hypertension without significant proteinuria O16 Unspecified maternal hypertension As we can see, not only do coders have to choose the correct code, the providers need to give good clear documentation for the coders to choose from. However, before we can correctly choose these codes, we need to have a good working knowledge of what the definitions are of the pregnancy hypertensive code-set. Unfortunately , the cause of pre-eclampsia is still unknown. Pre-existing hypertension is defined as: · a systolic blood pressure (BP) of 140 mm Hg or greater, · and/or a diastolic BP of 90 mm Hg or more, · either pre-pregnancy or before 20 weeks Gestational hypertension (aka pregnancy-induced hypertension) · Is the development of a new hypertension diagnosis in a pregnant woman after 20 weeks gestation without the presence of protein in the urine or other signs of preeclampsia. · Can be considered severe when systolic blood pressure is ≥160 mmHg and/or diastolic blood pressure is ≥110 mmHg on two consecutive blood pressure measurements at least four hours apart Preeclampsia is defined as: · A condition in pregnancy characterized by abrupt hypertension (a sharp rise in blood pressure), · Albuminuria (leakage of large amounts of the protein albumin into the urine) · Edema (swelling) of the hands, feet, and face · A headache that will not go away · Seeing spots or changes in eyesight · Pain in the upper abdomen or shoulder · Nausea and vomiting (in the second half of pregnancy) · Sudden weight gain · Difficulty breathing · Severe hypertension and signs/symptoms of end-organ injury are considered within the severe spectrum of the pre-eclampsia disease process. o Note: In 2013, the American College of Obstetricians and Gynecologists (ACOG) removed proteinuria as an essential criterion for diagnosis of preeclampsia with severe features. Eclampsia is defined as: · The development of grand mal seizures in a pregnant patient with diagnosed pre-eclampsia, (in the absence of other neurologic conditions that could account for the seizure activity) HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) is commonly defined as : · a severe form of pre-eclampsia, OR · HELLP syndrome can be considered an independent disorder from pre-eclampsia based upon the providers documentation) Preeclampsia affects 3% to 5% of all pregnancies and any pregnant woman can get preeclampsia, but studies have shown that a patient is at a higher risk of pre-eclampsia if the provider has noted any of these risk factors: · This is the first pregnancy · A family history where the patient’s mother or sister had preeclampsia or eclampsia during pregnancy · Patient is pregnant with a multiple gestation (eg: twins, triplets) · Patient is under age 20 or over age 40 at the time of pregnancy · The patient has a pre-existing diagnosis of high blood pressure, kidney disease, or diabetes · The patient has a pre-pregnancy body mass index (BMI) greater than 30 (potential obesity) · The patient was diagnosed with preeclampsia in a previous pregnancy What to look for clinically – to choose the correct codes in ICD-10 Now that we are fully entrenched in ICD-10 coders will need to look for the above and verify that the provider has clearly stated the diagnosis when coding for a pregnant patient with symptoms of hypertension and/or pre-eclampsia. If only the symptoms are noted, it is warranted to then query the physician and ask if the symptoms correlate to a specific diagnosis, or are simply “separately identifiable” signs and symptoms. Clinical Emergency Department Note: HPI: 41-year female, G2P0A1, at 36 and 3/7 weeks, presents to the Emergency room with sever headache and confusion. Husband and mother both report that the patient has had episodes of muddled thinking for last ten days or more. Pregnancy has been uneventful. While in the Emergency Department, the pt complains of bilateral pulsing headache with no visual disturbances. Headache is aggravated with any movement and has not responded to Tylenol. Pt has had nausea x 3 days, no vomiting, but has symptoms of oliguria. Pt states “Cannot remember when I last urinated”. ROS includes RUQ pain. Patient reports good fetal movement, denies contractions, vaginal bleeding, or pelvic cramping. Patient also denies dizziness, loss of coconsciousness, tremors, seizures, SOB, chest pain. Patient denies tobacco, alcohol, or drug use. Patient states she took Tylenol 2 hrs ago, but without relief of headache. Vital Signs: BP 142/94, T 98.9°F, P 94, R 22. Ht: 5’ 0” Wt: 151 lb. Well nourished, well-groomed, A&Ox3, mood distressed. HEENT, Respiratory and Cardiac exams all normal. Abdomen: Fundal height consistent with 36 weeks, single fetus, vertex and engaged; fetal weight ~ 2,200g, FHR 142 bpm. Fetus small for gestational age. Musculoskeletal: Adequate muscle tone + full AROM x4. Deep tendon reflexes were 4+/4+ with sustained knee and ankle clonus. Extremities: Generalized edema present, 3+ bilateral edema LE. No cyanosis. Vaginal exam: Cervix fingertip dilated and 5% effaced. The vertex was presenting at 0 station. Membranes intact. Laboratory: U/A 3+ proteinuria +2 glucose Assessment/Plan: Severe pre-eclampsia. Will obtain Fetal ultrasound with bio-physicial profile and fetal non-stress test to assess fetal status. Proceed with Direct admit to Labor and Delivery unit for induction of labor. Admission orders: called and faxed to L&D unit nurse. Will contact patient’s primary OB to meet the patient at L&D and assume care for induction of labor in a pre-eclamptic advanced maternal age patient. Indication: 41-year-old patient that has been admitted to Labor and Delivery unit for induction of labor due to Severe pre-eclampsia. Induction attempted with IV Pitocin, but patient failed to progress. Fetus is cephalic per bedside ultrasound, and we will proceed with low transverse c/s Patient was prepared and draped in the usual manner. Incision was made as noted above and carried down through the subcutaneous tissue, muscular fascia and peritoneum. Once inside the abdominal cavity, a low cervical transverse incision was made in the lower uterine segment after creating a bladder flap by both blunt and sharp dissection. With creation of the bladder flap, a transverse incision was made and the infant was delivered as a vertex. The placenta was removed and appeared normal w/3 vessel cord, cord blood was obtained. The infant was handed off to the nurses in attendance. The uterus was then exteriorized and brought out through the abdominal incision. We then closed the uterine incision in the usual manner with #1 Chromic suture in a running continuous manner. The bladder flap was inspected for hemostasis and closed with #2-0 Chromic in a running continuous manner as well. Number 0 Vicryl was used to close the fascia in a running continuous manner. The subcutaneous tissue and peritoneum were closed with #2-0 Vicryl suture in a running continuous manner. The skin was closed as noted above. Foley catheter inserted. Clear urine was noted. The sponge count was correct times 2. There were no complications. Estimated blood loss was 600 cc. Delivery of live male infant weighting 5 pounds 1oz having Apgar’s of 7 at one minute and 9 at five minutes. The patient was then awakened and taken to the Recovery Room in good condition CPT Procedure Coding considerations for the above include: A) Coding and Billing for the Emergency Department visit (99281 – 99285) B) Coding and Billing for the Cesarean and/or Global Delivery Care by OB (Depending on care delivered) a. 59510 Routine obstetric care including antepartum care, cesarean delivery, and postpartum care (Global Service) b. 59514 Cesarean delivery only; c. 59515 Cesarean delivery only; including postpartum care ICD10 pcs Procedure Coding Consideration 10D00Z1 Extraction, Products of Conception, Low Cervical cesarean section ICD-10 cm Diagnosis Coding considerations include: O14.13 Severe pre-eclampsia, third trimester O61.0 Failed medical or unspecified induction of labor, delivered, with or without mention of antepartum condition O09.513 Supervision of high-risk pregnancy with elderly primigravida third trimester N/A Z3A.36 36 Weeks gestation Z37.0 Single live birth Coding Wrap up In the clinical documentation by the provider, it was very well outlined and recorded to show the clinical diagnosis of severe pre-eclampsia. As per the ICD-10cm guidelines, In coding for obstetrics, if the trimesters are known, it is to be coded, in addition to the weeks of gestation. When coding for this scenario in CPT, the E&M of the emergency room physician is considered “separately identifiable” from the obstetricians’ charges. Therefore, it is appropriate to code and bill for the emergency room physician, based upon the documentation. When choosing the CPT code for the delivery, the coder will need to ascertain whether or not the delivery was performed as a “global” service. If the global service was performed by the OB provider, the entire spectrum of pregnancy care (which includes; antepartum, delivery care, and postpartum care services) should be billed. If the physician performed only the cesarean delivery and is not the global provider of service, then the cesarean only code should be billed. This also holds true if the provider performed the cesarean and is going to provide the postpartum care too. ICD-10pcs – the coder needs to know whether or not the cesarean was performed as a classical, low cervical, or extraperitoneal cesarean section. In the operative note, the physician noted this was a low transverse cesarean section. The ICD-10 tables bring us to the code 10D00Z1 Extraction, Products of Conception, Low Cervical cesarean section. If you are not seeing all the information you need in the clinical documentation to determine if the diagnosis is “hypertension” “pre-eclampsia” “Eclampsia” or “HELLP” syndrome, do not hesitate to query the provider and ask for additional clarification to be documented in the record. Lori-Lynne A. Webb, CPC, CCS-P, CCP, CHDA, COBGC and ICD10 cm/pcs Ambassador/trainer is an E&M, and Procedure based Coding, Compliance, Data Charge entry and HIPAA Privacy specialist, with over 20 years of experience. Lori-Lynne’s coding specialty is OB/GYN office & Hospitalist Services, Maternal Fetal Medicine, OB/GYN Oncology, Urology, and general surgical coding. She can be reached via e-mail at [email protected] or you can also find current coding information on her blog site: http://lori-lynnescodingcoachblog.blogspot.com/.	4	18	2	0	0	4	0.932395	6	2,939
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
November Is National COPD Awareness Month Chronic obstructive pulmonary disease (COPD) is a blanket term for progressive diseases of the lower respiratory tract — most commonly, chronic bronchitis and emphysema. Approximately 15.7 million men and women in the United States were reportedly diagnosed with COPD in 2015; millions more are believed to go undiagnosed every year, according to the Centers for Disease Control and Prevention (CDC). Everyone plays a role in battling COPD: Patients must be vigilant in receiving annual wellness exams; practitioners must educate patients on the causes and early signs of COPD; and medical coders must ensure patient diagnoses are reported with the utmost specificity. Chronic Lower Respiratory Diseases An individual diagnosed with chronic obstructive pulmonary disease (COPD) may have one or both of these chronic lower respiratory diseases: Emphysema – Walls of alveoli (air sacs in the lungs) are destroyed, reducing the exchange levels of oxygen and carbon dioxide. Chronic bronchitis – Inflamed bronchial tubes lose their shape and become clogged with mucus. Annual Wellness Exams Allow Early Detection Preliminary warning signs of COPD are easy to miss, but important to catch. Although there is no cure for this life-threatening disease, early diagnosis, education, and treatment can slow its progression. Global Initiative for Chronic Obstructive Lung Disease (GOLD) guidelines map out the different stages of COPD: Stage 1 – The patient has very mild COPD with an FEV1 (forced expiratory volume in one second) of 80 percent or higher of normal. Reduced airflow often goes unnoticed or is written off to “getting older.” Stage 2 – The patient has moderate COPD with an FEV1 between 50 and 80 percent of normal. The patient develops chronic coughing, wheezing, and shortness of breath. Stage 3 – The patient has severe emphysema with FEV1 between 30 and 50 percent of normal. Symptoms worsen and may impede normal activity. Stage 4 – The patient has very severe, or “end stage,” COPD with a lower FEV1 than stage 3, or has stage 3 FEV1 and low blood oxygen levels. Lung function has deteriorated to life-threatening levels. Education Is Key Although pulmonary function tests (PFT) can confirm COPD, even at stage 1, Medicare does not deem PFT reasonable and necessary when a diagnosis or evaluation can be made clinically, or when test results are not necessary to manage the patient’s disease. With or without PFT, the clinician should document any risk factors (e.g., age, race, gender, family history), symptoms (e.g., shortness of breath, wheezing, cough, abnormal sputum), and physical exam findings (e.g., hypoxia, muscle wasting, tachypnea, tachycardia, jugular vein distension). This documentation will help track the progression of the disease and prove future PFT and therapies reasonable and necessary. Clinicians also should educate patients about COPD and recommend smoking cessation counseling, if applicable. Influenza, pneumococcal, and Tdap vaccinations should be recommended, as well, to protect patients against acute upper and lower respiratory viral infections. Approximately 174,000 emergency room visits related to acute exacerbation of COPD occur every year, according to the CDC. Code COPD Clearly If a definitive diagnosis of COPD is not made, code only the symptoms. When COPD is confirmed, report J44.x Other chronic obstructive pulmonary disease, the type of asthma, if applicable (J45.x Asthma), as well as a Z code to identify any documented factors influencing the patient’s health status. Although smoking cigarettes is the leading cause of COPD, one out of six individuals diagnosed with COPD has never smoked. Exposure to environmental or occupational irritants (e.g., Z57.2 Occupational exposure to dust) or genetics can factor in, too. Coding Tip: Follow-up testing may be appropriate to monitor acute exacerbation of interstitial lung disease. Per ICD-10 guidelines, “If the same condition is described as both acute (subacute) and chronic, and separate subentries exist in the Alphabetic Index at the same indention level, code both and sequence the a Upon Diagnosis, There Is Treatment Although COPD causes irreversible lung damage, therapies beneficial in improving quality of life include (in order of severity): - Self-management and smoking cessation (if applicable) - Medication (e.g., inhaled corticosteroid) - Pulmonary rehabilitation - Medicare covers pulmonary rehabilitation for moderate to severe COPD. Consider Quality Reporting COPD: Long-acting Inhaled Bronchodilator Therapy is a quality measure (NQF 0102) in the Merit-based Incentive Payment System (MIPS). Although, it’s not a high-priority measure, it’s easy enough to fulfill submission requirements, especially if your practice sees a lot of Medicare patients. Simply report the percentage of symptomatic patients aged 18 years and older with a diagnosis of COPD who have an FEV1 less than 60 percent predicted and who were prescribed a long-acting inhaled bronchodilator. Clinicians who are ineligible to participate in MIPS may report this data voluntarily (without penalty). The ABCs of PFTs Pulmonary function tests (PFTs) measure the mechanical ability to move air in and out of the lungs and the effectiveness of getting oxygen in to, and carbon dioxide out of, the body. PFTs include: - Lung volume - Diffusion capacity - Lung compliance - Pulmonary studies during exercise testing Pulmonary diagnostic testing and therapy services are reported using CPT® codes 94010-94799, which include laboratory procedures and interpretation of the results. To support PFT services, the provider must document the presence or absence of lung dysfunction, suggested by history or clinically significant physical signs and symptoms, or by other diagnostic tests. There also must be a referral on file; and all studies require an interpretation with written report. Refer to your Medicare administrative contractor’s (MAC) local coverage determination (LCD) for PFT for a list of ICD-10 codes that support use of these procedure codes. Note: MACs have updated their LCDs for PFTs. Noridian Medicare, for example, has made the following changes to their LCD for PFT (L34247), effective Oct. 1, 2017: Added: I27.20-I27.24, I27.29, I27.83 Deleted from Group 1 codes that support medical necessity: I27.2 Revised: M33.01, M33.02, M33.09, M33.11, M33.12, M33.19 Summary Health Statistics: National Health Interview Survey, 2015, https://ftp.cdc.gov/pub/Health_Statistics/NCHS/NHIS/SHS/2015_SHS_Table_A-2.pdf Latest posts by Renee Dustman (see all) - HEALTHCON: Speaker Heather Greene, MBA, RHIA, CPC, CIC, CPMA, CDIP - January 18, 2019 - HEALTHCON: Speaker Michael Warner, DO, CPC, CPCO, CPMA, AAPC Fellow - January 18, 2019 - Complex Repair vs. Tissue Transfer, Rearrangement - January 18, 2019	4	9	2	0	0	4	0.902649	6	1,605
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. Hi folks. I wonder if anyone can help me. I have been covered in a rash for around 6 months and have tried loads of creams etc but nothing is working. The rash is mainly on my legs and arms and it...frances22039 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	0.589307	2	2,279
- freely available J. Dev. Biol. 2013, 1(1), 47-63; doi:10.3390/jdb1010047 Abstract: The epicardium is the outer skin of the mature vertebrate heart. Its embryonic origin and its possible roles in the developing and mature heart did not receive much recognition during the 19th and most of the 20th century. During the past 25 years, however, the epicardium came into the focus of developmental biology and regenerative medicine. Clinical researchers usually prefer genetically modified mouse models when they want to gain insight into developmental or pathological processes. The story of research on the embryonic epicardium, however, nicely demonstrates the value of non-mammalian species, namely avian species, for elucidating fundamental processes in embryonic and fetal development. Studies on chick and quail embryos have not only led to the identification of the primarily extracardiac source of the epicardium—presently called the proepicardium (PE)—they have also significantly contributed to our current knowledge about the developmental significance of the embryonic epicardium. In this review article, I describe three “classical” microsurgical experiments that have been developed for studying the developmental significance of the PE/epicardium in avian embryos (mechanical PE-blocking, PE-photoablation, orthotopic PE-grafting). Furthermore, I show how these microsurgical experiments have contributed to our current knowledge about the roles of the PE/epicardium in cardiac development. There are still some unsolved aspects in the physiology of the developing epicardium, which may be clarified with the aid of these “classical” microsurgical experiments. The mature heart of vertebrates is a hollow muscle—the myocardium—whereby the inner and outer surfaces are covered by skins called the endocardium and epicardium, respectively. Up to the end of the 20th century, research on the developing heart mainly focused on the origin and differentiation of the myocardium and endocardium. The epicardium did not receive much recognition. It was generally thought that the definitive myocardium and epicardium both derived from the outer epithelial wall of the early embryonic heart, which was consequently named the myoepicardium. We now know that the so-called myoepicardium consists exclusively of primary myocardial cells and that the epicardial mesothelium, the majority of the subepicardial and intramyocardial fibroblasts, and the coronary blood vessels derive from a primarily extracardiac source of progenitor cells called the proepicardium (PE). We also know that the epicardium and other PE-derived structures play important roles in normal development of the myocardial wall and in the repair of damaged myocardium (see other articles in this issue). Studies on chick embryos have significantly contributed to our current knowledge about the developmental biology of the PE/epicardium. The chick embryo was the first model organism enabling the German anatomist Remak to describe the accumulation of villous protrusions of the pericardial mesothelium, now known as the PE . The chick embryo was also the first model organism through which the Polish anatomist Kurkiewicz in 1909 was able to correctly identify these pericardial villi as the source of the embryonic epicardium . Finally, avian embryos (chicks, quails) were the first model organisms from which microsurgical experiments led to the identification of further derivatives of the PE. In this article, I describe three “classical” microsurgical experiments that have been developed and used for studying the developmental significance of the PE/epicardium in avian embryos. These are, firstly, the mechanical PE-blocking technique; secondly, the PE-photoablation technique; and thirdly, the orthotopic PE-grafting technique. These microsurgical experiments have significantly contributed to our current knowledge about the roles of the PE/epicardium in development of the vertebrate heart. 2. Mechanical PE-Blocking The PE-blocking technique is used in experiments that are conducted to see how hearts will develop in the absence or reduced amounts of PE-derived tissues (loss-of-PE-function experiments). It was developed in my lab in the early 1990s and first published in an original research paper in 1993 . The goal of the PE-blocking technique is to prevent the normal cell transfer from the PE to the heart. In mammalian species (e.g., mice, humans), as well as in some fish species (e.g., zebrafish), PE-derived cells reach the surface of the embryonic heart in the form of free-floating vesicles (for review see ). These vesicles are released into the fluid of the free pericardial cavity where they float passively to the surface of the heart. Such a PE-cell transfer cannot be blocked effectively by a simple microsurgical procedure. In avian, reptilian, and amphibian embryos, however, the PE-cell transfer is normally accomplished via a secondary tissue bridge, which is formed by the firm attachment of PE villi to the opposite surface of the developing ventricles (Figure 1). This PE-derived tissue bridge is called the sinu-ventricular ligament since it runs from the ventral wall of the sinus venosus to the dorsal wall of the ventricles . The goal of the PE-blocking technique, thus, is to prevent the formation of PE-derived tissue bridges to the heart. The reason for choosing a mechanical PE-blocking technique instead of a mechanical PE-excision technique is due to the avian PE occupying the thin and fragile wall of the right horn of the sinus venosus, so that any mechanical damage to the PE may cause lethal bleeding of the embryo. The prevention of damage to the sinus venosus is not the only advantage of this technique; it is also a technically simple and quick microsurgical procedure. A disadvantage of this technique, however, is the fact that it does not lead to a removal of the PE, so that the colonization of the embryonic heart with PE-derived cells is not completely prevented but only severely delayed [3,6]. The embryo is treated in ovo before the villous protrusions of the PE have established firm contacts to the dorsal wall of the embryonic ventricle (3rd incubation day, at stages 15–17, according to Hamburger and Hamilton ). The egg is windowed over the embryo according to Hara . The primitive pericardial cavity then is opened by cutting the vitelline and chorionic membranes. This is possible since, in avian species, the primitive pericardial cavity is not separated from the extra-embryonic coelomic cavity during the early phase of embryonic heart development . In order to prevent the formation of PE-derived tissue bridges to the heart, a small rectangular piece of eggshell membrane is introduced into the primitive pericardial cavity, where it is placed between the PE and the dorsal wall of the embryonic ventricle. Since the cardiac pumping activity can lead to an unwanted displacement of the membrane from this “blocking position,” the membrane must be mechanically anchored at two sides: (1) ventrally at the coelomic wall of the anterior intestinal portal; and (2) dorsally in the furrow between the primitive atrium and sinus venosus (Figure 2). Further contacts between the “blocking-membrane” and other embryonic or extra-embryonic sides should be avoided since such contacts will lead to the formation of unwanted adhesions between the heart, the implanted membrane, and embryonic or extra-embryonic tissues with the final consequences of development of severe malformations of the heart and body wall. After correct implantation of the blocking-membrane, the egg is closed again with an adhesive tape and returned to the incubator. For the correct interpretation of the results of PE-blocking experiments, it should be remembered that the goal of the procedure is to prevent the formation of PE-derived tissue bridges to the heart, and thereby to block the direct cell transfer from the PE to the developing heart. Therefore, the criterion for assigning an experimental embryo to the group of successful PE-blocking experiments is the complete lack of PE-derived tissue bridges to the heart, whereas the presence of such tissue bridges must be regarded as the criterion for non-successful operations. According to these criteria, it has been found that the correct implantation of the blocking-membrane principally can lead to the development of two different kinds of hearts, that have since been classified as group-A and group-B hearts . Group-A hearts represent the group of successful PE-blocking experiments, as they are characterized by the complete lack of any PE-derived tissue bridges to the heart. They only show a PE-derived tissue bridge, which connects the ventral wall of the sinus venosus with the blocking-membrane. This tissue bridge has been named the sinus cord (Figure 3). It serves as a pathway over which PE-derived cells colonize the blocking-membrane. Due to its adherence to the anterior intestinal portal, the blocking-membrane does not remain in its initial position behind the heart, but becomes displaced caudally concomitant with the descent of the anterior intestinal portal. The sinus cord subsequently becomes continuously elongated and finally traverses the pericardial and peritoneal cavities. Corresponding to group-A hearts, group-B hearts lack the sinu-ventricular ligament and posses a sinus cord. They show, however, dystopic PE-derived tissue bridges, which connect the sinus cord or the blocking membrane with the developing ventricles. The points of attachment of such dystopic cardiac ligaments to the ventricles usually lie caudal from the normal attachment point of the sinu-ventricular ligament. In some cases, for example, dystopic cardiac ligaments are attached to the apex of the heart (Figure 3C). Thus, it is likely that, in group-B hearts, the implantation of the blocking-membrane initially has successfully prevented the formation of the sinu-ventricular ligament (indicated by the lack of this ligament and the presence of a sinus cord). With prolonged re-incubation time, however, the PE-derived mesothelium covering the sinus cord or the blocking-membrane must have formed new villous protrusions, which became firmly attached to the dorsal wall of the developing ventricles. Due to the caudal displacement of the blocking-membrane, the points of attachment of these cardiac ligaments lie caudal to the normal attachment point of the sinu-ventricular ligament. Since its first publication in 1993, the PE-blocking technique was used in at least 12 further studies, which were conducted to clarify the roles of the epicardium and other PE-derived tissues in normal development of the avian embryonic heart. The observation that group-A hearts usually show defective formation of the epicardium and subepicardial mesenchyme (Figure 4), while group-B hearts do not develop substantial defects of their epicardial and subepicardial coverings, was the first experimental evidence supporting Kurkiewizc’s view that the epicardial mesothelium derives from a cauliflower-shaped accumulation of pericardial mesothelial villi, currently called the PE . Analyses of the development and phenotype of group-A hearts have disclosed that such hearts not only develop deficiencies of the epicardium and subepicardial mesenchyme, but additionally develop (1) defects in coronary vasculature [6,10,11,12,13]; (2) a growth defect of the outer compact layer of the ventricular myocardium [6,10,11,13,14,15]; (3) impaired maturation of the working myocardium ; (4) disturbed differentiation of the Purkinje-fiber system ; (5) impaired remodeling of the outflow tract myocardium [6,18]; (6) anomalies of the atrio-ventricular endocardial cushions [6,11]; and (7) defective formation of the fibrous heart skeleton causing persistent atrio-ventricular myocardial connection with electrical conduction anomalies [19,20]. It was also found that the lack of PE-derived epicardium is partially compensated by non-removed PE and pericardial mesothelium, which colonize the heart along its connections with the pericardial wall at the arterial and venous poles of the heart [3,6]. The formation of this compensatory epicardium led to a complete mesothelial covering of the heart in every group-A embryo surviving up to the ninth incubation day, which is a three-day delay compared with normal hearts. The PE-photoablation technique was developed in my lab in 2003 and first published in 2005 . The goal of PE-photoablation is the complete elimination of the PE. In contrast to the PE-blocking technique, which is a technically simple and quick microsurgical procedure (see above), PE-photoablation is a technically demanding and time-consuming microsurgical technique. Compared to PE-blocking or mechanical PE-excision techniques, however, it has the great advantage that it facilitates a complete and long-lasting elimination of the PE without significant damage to non-PE tissues. This reduces the amount of compensatory epicardium colonizing the heart via its venous pole. It, thus, seems to be the ideal technique for loss-of-PE-function experiments. Photoablation techniques use the combined application of two individually non-toxic agents to induce oxygen-dependent tissue damage: (1) a photoactivatable substance, the photosensitizer; and (2) light of a specific wavelength exciting the photosensitizer. In the presence of oxygen, the excitation of the photosensitizer produces reactive oxygen species, which damage the tissues in contact with the photosensitizer. For PE-photoablation, we use the reddish colored dye Rose Bengal (4,5,6,7-tetra-chloro-2',4',5',7'-tetraiodofluorescein sodium salt) as photosensitizer. Use of a colored photosensitizer facilitates selective and complete application of the photosensitizer to the PE under visual control. The embryo is treated in ovo at stage 16 according to Hamburger and Hamilton . At this stage, the PE normally has not made firm contact to the heart and its size is large enough to facilitate selective staining of all of its villi without unwanted staining of surrounding structures. The egg is windowed and the primitive pericardial cavity is opened as described above. After the opening of the primitive pericardial cavity, a small rectangular piece of eggshell membrane is placed in the “blocking-position” between the PE and the dorsal wall of the embryonic ventricle (Figure 5). This membrane protects the heart from staining with Rose Bengal. The PE then is externally stained with a 1% solution of Rose Bengal (dissolved in physiological saline) by means of a micropipette. Small boluses of the dye solution are repeatedly injected into the pericardial fluid surrounding the PE, where most of the dye is instantly bound to the PE. To prevent staining of non-PE tissues, any unbound dye solution is immediately sucked away by means of a second micropipette. After complete and intense staining of the PE, the protecting membrane is removed from the primitive pericardial cavity and the egg is closed again with an adhesive tape and returned to the incubator. Since Rose Bengal is photoactivated by visible light concomitantly with its application to the PE, there is no need for a specific light exposure phase subsequent to staining of the PE. The reason for developing the PE-photoablation technique was the fact that, due to the formation of a compensatory epicardium, firstly, from remnants of non-removed PE at the venous heart pole and, secondly, from pericardial mesothelium at the arterial heart pole [3,6], the PE-blocking technique was regarded as not suitable for studying long-term effects of epicardium deficiency. We have found that PE-photoablation is a powerful technique for complete and long-lasting removal of the PE. The loss-of-PE-function phenotype corresponds to that observed subsequent to PE-blocking, but the degree of the defects is more severe than in PE-blocking experiments. Although it is not possible to prevent the formation of a compensatory epicardium, its formation is markedly delayed in comparison with PE-blocking experiments. The growth defect of the compact myocardium is also more severe than the thin myocardium phenotype observed in PE-blocking experiments (Figure 6), and the thinned ventricular wall develops an anomaly not observed in PE-blocking experiments, namely an aneurysma-like outward bulging of those areas devoid of epicardium. The latter finding suggests that the embryonic epicardium may make important contributions to the passive mechanics of the ventricular wall . The use of PE-photoablation in future studies on avian embryos may help to clarify the hitherto unknown role of epicardial mechanics in cardiac development. 4. Orthotopic PE-Grafting PE-grafting experiments have been primarily used for PE-fate mapping in avian embryos. The production of chimeras by interspecies grafting of tissues from quail donor embryos into chick host embryos is a well-established experimental approach for clarifying the fate of embryonic structures in avian embryos since the 1970s (for reviews see [22,23]). It is based on the existence of structural and immunological differences between the cells of quail and chick embryos, which can be used for the detection of grafted quail cells in chick hosts. Avian embryos, therefore, were the first choice when embryologists and developmental biologists became interested in clarifying the fate of the PE in the 1990s. The microsurgical techniques used for the first PE-grafting experiments were relatively simple. A piece of quail PE-villi, or a piece of quail liver carrying the PE, was loosely placed into the primitive pericardial cavity of a chick host, without blocking or elimination of the host PE [24,25,26,27]. Although the hearts produced in such experiments receive only a limited contribution of grafted PE-cells, such grafting experiments have significantly contributed to our current knowledge about the PE, since they have shown that the PE not only provides epicardial mesothelial cells but, additionally, provides mesenchymal cells which invade the subepicardium and myocardium, where they differentiate into fibroblasts and cells of the coronary vasculature. A shortcoming of the early PE-grafting procedures, however, is the fact that the donor PE usually contributes only a minority of PE-cells to the host heart, while the host PE still contributes the majority of PE-cells to the host heart. Using such grafting procedures, it is, therefore, not possible to clarify the whole extent of PE-cell contributions to the developing heart. In view of this shortcoming, I developed a new PE-grafting technique called the “orthotopic PE-grafting” procedure, which is outlined in the following paragraphs . This grafting procedure facilitates an orthotopic attachment of the donor PE to the host heart combined with blocking of the attachment of the host PE to the host heart. The results of such experiments are chimeric hearts in which the tissues derived from the PE are almost completely of donor origin (Figure 7). The orthotopic PE-grafting procedure starts with the preparation of the host embryo, which is treated in ovo before PE-villi have established firm contacts to the heart (stages 16/17, according to Hamburger and Hamilton ). The egg is windowed and the primitive pericardial cavity is opened as described above. After the opening of the primitive pericardial cavity, a small rectangular piece of eggshell membrane is prepared for accurate placement in “blocking” position between the PE and the dorsal wall of the embryonic ventricle (see above). This membrane will serve two functions: (1) as a carrier for the PE-graft, keeping the graft in a position within the pericardial cavity of the host embryo, which facilitates an orthotopic attachment of donor PE-villi to the host heart; (2) as a blocking-membrane preventing the transfer of host PE-cells to the host heart. This “carrier-membrane” is then transferred to the donor embryo, where it will be mounted with a cuff of tissue from the venous heart pole carrying the PE. Preparation of the donor embryo (stages 16/17, according to Hamburger and Hamilton ) is carried out in a dish with sterile Locke’s solution outside of the egg (Figure 8A–C). The pericardial cavity is opened (see above) and the heart is removed by two cuts with fine scissors; the first along the sinu-atrial border, and the second through the outflow tract. The rest of the venous heart pole is then disconnected from the body veins, by cuts through the lateral mesocardia, and the right omphalomesenteric vein is opened by an incision into its ventral wall. This opening is used to introduce the “carrier-membrane” (see above) into the lumen of the omphalomesenteric vein and the rest of the venous heart pole. This tissue block is then excised together with the carrier-membrane. The carrier-membrane now is mounted with a cuff of donor tissue carrying the ventral wall of the sinus venosus with the PE. The graft is transferred to the pericardial cavity of the host embryo, where the carrier-membrane is placed in the PE “blocking-position” (Figure 8D). After correct implantation of the graft, the host egg is closed again with an adhesive tape and returned to the incubator. Since its first publication in 1999, the orthotopic PE-grafting technique or modifications of this procedure have been used in several studies on avian embryos [6,10,11,12,13,29,30,31,32,33,34]. The results of these experiments have confirmed and complemented the results of the earlier fate-mapping studies using “loose PE-grafting” techniques. The picture about the fate of the avian PE arising from these studies presents as follows: (1) the PE provides the epicardium and subepicardial mesenchyme of the heart, while the epicardium and subepicardial mesenchyme of the great arterial trunks derive from the pericardium at the arterial heart pole (Figure 7, Figure 9A) [28,32]. (2) PE- and epicardium-derived mesenchymal cells invade the subepicardial , myocardial, and endocardial layers of the heart. They also colonize the atrio-ventricular endocardial cushions but do not colonize the endocardial outflow ridges [11,25,28,33]. (3) PE- and epicardium-derived mesenchymal cells provide the endothelium, smooth muscle cells, and fibroblast of the coronary blood vessels (Figure 9) [11,12,25,26,28,29,31]. (4) PE- and epicardium-derived mesenchymal cells do not substantially contribute to the formation of the cardiac lymphatic system . (5) PE- and epicardium-derived mesenchymal cells provide the majority of the subepicardial and intramyocardial fibroblasts, and contribute to the formation of the fibrous heart skeleton [11,12,25,26,28,29,31]. (6) PE- and epicardium-derived mesenchymal cells do not contribute a substantial number of myocardioblasts to the developing avian heart (Figure 9B) . Due to the high reliability of interspecies quail–chick grafting experiments, the results of such fate-mapping studies may be used as “reference standards” for the evaluation of the outcome of fate-mapping studies made in other vertebrate model systems, such as the mouse or the zebrafish. With respect to the PE, it was found that the results of genetic PE-fate mapping largely corresponded to those made in the aforementioned avian PE-grafting experiments. There seemed to exist, however, two exceptions. The first exception was a possible contribution of PE/epicardium-derived cells to the myocardium. While avian PE-grafting experiments did not provide any evidence for the contribution of a substantial number of PE-derived myocardioblast to the developing heart , genetic PE-fate mapping of mouse PE/epicardium seemed to have identified PE/epicardium-derived cells as hitherto unrecognized myocardial progenitor cells in mouse embryos [35,36]. The divergent results have led to a discussion about the reliability of genetic PE-fate mapping [37,38,39]. It was shown, for example, that the “epicardium-specific” Cre-line (TBX18) used in one of two aforementioned mouse studies has a limited expression in the myocardium of the “naked” embryonic heart . It is, therefore, questionable whether mouse PE really contributes a substantial number of myocardioblasts to the developing heart. The data from classical fate-mapping studies on avian embryos, as well as recent data from genetic PE-fate mapping in the zebrafish , indicate that, in these species, the natural fates of PE-/epicardium-derived cells are limited to non-myocardial cell lineages. The second exception was the contribution of the PE to the endothelium of the coronary blood vessels. Most of the fate-mapping studies conducted on avian embryos provided evidence for a PE/epicardial origin of the coronary endothelium [28,31,41,42]. Genetic PE-fate mapping of mouse PE/epicardium using TBX18 and WT-1 Cre lines, on the other hand, did not provide any evidence for a significant contribution of PE-/epicardium-derived cells to the coronary endothelium [35,36]. Therefore, the origin of the coronary endothelium of the developing mouse heart has remained obscure until recently when marker analyses have shown that the mouse PE is a source of coronary endothelial cells [43,44]. 5. Future Perspectives During the initial phase of the research on the PE, the interest of researchers was mainly focused on classical themes of developmental biology, such as the fate or the functional significance of PE-derived cells. The easy accessibility and the possibility to carry out microsurgical experiments, such as mechanical PE-blocking or PE-grafting, made avian embryos attractive for use in studies addressing classical themes of developmental biology. During the past two decades, however, the focus of PE research has shifted from classical to molecular themes. This was accompanied by a shift in usage of model organisms from birds to mice and zebrafish. Although transgenic technologies are more advanced in the latter two systems at the present time, remarkable progress has been made in the development of methods for manipulating genetic and molecular functions in avian embryos during the past few years. Thus, chick and quail embryos are still regarded as attractive model systems for developmental biology, as well as regenerative medicine [45,46]. It is especially the possibility of combining modern molecular techniques, such as gene delivery via in vivo electroporation, siRNA technology or transgenic quails , with classical microsurgical techniques that make quail and chick embryos attractive for future research. With respect to the research on the PE and epicardium, it is, therefore, likely that the three aforementioned techniques for microsurgical manipulation of the avian PE may be used in future studies in combination with molecular techniques. The orthotopic PE-grafting technique, for example, can be used for the transplantation of PE from transgenic into wild type embryos or vice versa. Such in vivo experiments cannot be carried out in zebrafish or mice and may be very helpful for elucidating the function of genes involved in the dialog between the developing epicardium and myocardium, which controls the growth of the compact layer of ventricular myocardium (for reviews see [48,49]). Experiments in which the PE from green fluorescent protein-expressing transgenic chicken will be grafted into a wild-type host may facilitate the in vivo visualization of the initial phase of epicardial development. In the past, descriptive and experimental studies on avian embryos have significantly contributed to our current knowledge about the developmental significance of the PE. The possibility of combining modern molecular techniques with classical microsurgical experiments, such as mechanical PE-blocking, PE-photoablation, and orthotopic PE-grafting, make avian embryos attractive for use in future studies aimed to clarify hitherto neglected or unrecognized aspects in the developmental biology of PE-derived tissues. Two of these aspects are (1) the origin and functional significance of the (sub)-epicardial adipose tissue, which plays an important role in normal and pathological conditions of the mature human heart [51,52]; and (2) the mechanobiology of the embryonic epicardium, which seems to make an important contribution to the mechanical behavior of the ventricular wall . Parts of this work were supported by a grant from the Deutsche Forschungsgemeinschaft (MA 2377/4-1; MA 2377/4-2). The author thanks Kirsten Falk-Stietenroth and Hans-Georg Sydow for technical and photographical assistance. Conflict of Interest The author declares no conflict of interest. References and Notes - Remak, R. Über die Entwicklung des Hühnchens im Ei. Arch. Anat. Physiol. Wiss. Med. Jahrg. 1843, 478–484. [Google Scholar] - Kurkiewicz, T. O histogenezie miesna sercowego zwierzat kregowych—Zur Histogenese des Herzmuskels der Wirbeltiere. Bull. Int. Acad. Sci. Cracovie 1909, 148–191. [Google Scholar] - Männer, J. Experimental study on the formation of the epicardium in chick embryos. Anat. Embryol. (Berl). 1993, 187, 281–289. [Google Scholar] [CrossRef] - Männer, J.; Pérez–Pomares, J.M.; Macias, D.; Muñoz–Chápuli, R. The origin, formation, and developmental significance of the epicardium: A review. Cells Tissues Organs 2001, 169, 89–103. [Google Scholar] [CrossRef] - Männer, J. The development of pericardial villi in the chick embryos. Anat. Embryol. (Berl). 1992, 186, 397–385. [Google Scholar] [CrossRef] - Gittenberger–de Groot, A.C.; Vrancken Peeters, M.P.; Bergwerff, M.; Mentink, M.M.; Poelmann, R.E. Epicardial outgrowth inhibition leads to compensatory mesothelial outflow tract collar and abnormal cardiac septation and coronary formation. Circ. Res. 2000, 87, 969–971. [Google Scholar] [CrossRef] - Hamburger, V.; Hamilton, H.L. A series of normal stages in the development of the chick embryo. J. Morphol. 1951, 88, 49–92. [Google Scholar] - Hara, K. Micro-surgical operation on the chick embryo in ovo without vital staining. A modification of the intra-coelomic grafting technique. Mikroskopie 1971, 27, 267–270. [Google Scholar] - Steding, G.; Klemeyer, L. Die Entwicklung der Perikardfalte des Hühnerembryo. Z. Anat. Entwicklungsgesch. 1969, 129, 223–233. [Google Scholar] - Pérez–Pomares, J.M.; Phelps, A.; Muñoz–Chápuli, R.; Wessels, A. The contribution of the proepicardium to avian cardiovascular development. Int. J. Dev. Biol. 2001, 45, S155–S156. [Google Scholar] - Pérez–Pomares, J.M.; Phelps, A.; Sedmerova, M.; Carmona, R.; González–Iriarte, M.; Muñoz–Chápuli, R.; Wessels, A. Experimental studies on the spatiotemporal expression of WT1 and RALDH2 in the embryonic avian heart: a model for the regulation of myocardial and valvuloseptal development by epicardially derived cells (EPDCs). Dev. Biol. 2002, 247, 307–326. [Google Scholar] [CrossRef] - Gittenberger–de Groot, A.C.; Eralp, I.; Lie–Venema, H.; Bartelings, M.M.; Poelmann, R.E. Development of the coronary vasculature and its implications for coronary abnormalities in general and specifically in pulmonary atresia without ventricular septal defect. Acta Paediatr. Suppl. 2004, 93, 13–19. [Google Scholar] - Eralp, I.; Lie–Venema, H.; DeRuiter, M.C.; van den Akker, N.M.S.; Bogers, A.J.J.C.; Mentink, M.M.T.; Maas, S.; Poelmann, R.E.; Gittenberger–de Groot, A.C. Coronary artery and orifice development is associated with proper timing of epicardial outgrowth and correlated Fas ligand associated apoptosis patterns. Circ. Res. 2005, 96, 526–534. [Google Scholar] - Poelmann, R.E.; Lie–Venema, H.; Gittenberger–de Groot, A.C. The role of the epicardium and neural crest as extracardiac contributors to coronary vascular development. Tex. Heart Inst. J. 2002, 29, 255–261. [Google Scholar] - Pennisi, D.J.; Ballard, V.L.T.; Mikawa, T. Epicardium is required for the full rate of myocyte proliferation and levels of expression of myocyte mitogenic factors FGF2 and its receptor, FGFR–1, but not for transmural myocardial patterning in the chick embryo heart. Dev. Dyn. 2003, 228, 161–172. [Google Scholar] - Weeke–Klimp, A.; Bax, N.A.; Bellu, A.R.; Winter, E.M.; Vrolijk, J.; Plantinga, J.; Maas, S.; Brinker, M.; Mahtab, E.A.; Gittenberger–de Groot, A.C.; et al. Epicardium–derived cells enhance proliferation, cellular maturation and alignment of cardiomyocytes. J. Mol. Cell. Cardiol. 2010, 49, 606–616. [Google Scholar] - Eralp, I.; Lie–Venema, H.; Bax, N.A.; Wijffels, M.C.; van der Laarse, A.; DeRuiter, M.C.; Bogers, A.J.; van den Akker, N.M.; Gourdie, R.G.; Schalij, M.J.; et al. Epicardium–derived cells are important for correct development of the Purkinje fibers in the avian heart. Anat. Rec. A. 2006, 288, 1272–1280. [Google Scholar] - Rothenberg, F.; Hitomi, M.; Fisher, S.A.; Watanabe, M. Initiation of apotosis in the developing avian outflow tract myocardium. Dev. Dyn. 2002, 223, 469–482. [Google Scholar] [CrossRef] - Kolditz, D.P.; Wijffels, M.C.; Blom, N.A.; van der Laarse, A.; Hahurij, N.D.; Lie–Venema, H.; Markwald, R.R.; Poelmann, R.E.; Schalij, M.J.; Gittenberger–de Groot, A.C. Epicardium–derived cells in development of annulus fibrosis and persistence of accessory pathways. Circulation 2008, 117, 1508–1517. [Google Scholar] [CrossRef] - Lie–Venema, H.; Eralp, I.; Markwald, R.R.; van den Akker, N.M.; Wijffels, M.C.; Kolditz, D.P.; van der Laarse, A.; Schalij, M.J.; Poelmann, R.E.; Bogers, A.J.; et al. Periostin expression by epicardium–derived cells is involved in the development of the atrioventricular valves and fibrous heart skeleton. Differ. 2008, 76, 809–819. [Google Scholar] - Männer, J.; Schlueter, J.; Brand, T. Experimental analyses of the function of the proepicardium using a new microsurgical procedure to induce loss–of–proepicardial–function in chick embryos. Dev. Dyn. 2005, 233, 1454–1463. [Google Scholar] [CrossRef] - Le Douarin, N.M. Developmental patterning deciphered in avian chimeras. Dev. Growth Differ. 2008, 50, S11–S28. [Google Scholar] [CrossRef] - Le Douarin, N.M.; Dieterlen–Lièvre, F. How studies on the avian embryo have opened new avenues in the understanding of development: A view about the neural and hematopoietic systems. Dev. Growth Differ. 2013, 55, 1–14. [Google Scholar] [CrossRef] - Poelmann, R.E.; Gittenberger–de Groot, A.C.; Mentink, M.M.; Bökenkamp, R.; Hogers, B. Development of the cardiac coronary vascular endothelium, studied with antiendothelial antibodies, in chicken–quail chimeras. Circ. Res. 1993, 73, 559–568. [Google Scholar] [CrossRef] - Gittenberger–de Groot, A.C.; Vrancken Peeters, M.P.; Mentink, M.M.; Gourdie, R.G.; Poelmann, R.E. Epicardium–derived cells contribute a novel population to the myocardial wall and the atrioventricular cushions. Circ. Res. 1998, 82, 1043–1052. [Google Scholar] [CrossRef] - Pérez–Pomares, J.M.; Macías, D.; García–Garrido, L.; Muñoz–Chápuli, R. The origin of the subepicardial mesenchyme in the avian embryo: An immunohistochemical and quail–chick chimera study. Dev. Biol. 1998, 200, 57–68. [Google Scholar] [CrossRef] - Lie–Venema, H.; Eralp, I.; Maas, S.; Gittenberger–de Groot, A.C.; Poelmann, R.E.; DeRuiter, M.C. Myocardial heterogeneity in permissiveness for epicardium–derived cells and endothelial precursor cells along the developing heart tube at the onset of coronary vascularization. Anat. Rec. A. 2005, 282, 120–129. [Google Scholar] - Männer, J. Does the subepicardial mesenchyme contribute myocardioblasts to the myocardium of the chick embryo heart? A quail–chick chimera study tracing the fate of the epicardial primordium. Anat. Rec. 1999, 255, 212–226. [Google Scholar] [CrossRef] - Männer, J. Embryology of congenital ventriculo–coronary communications: a study on quail–chick chimeras. Cardiol. Young 2000, 10, 233–238. [Google Scholar] [CrossRef] - Lu, J.; Landerholm, T.E.; Wei, J.S.; Dong, X.R.; Wu, S.P.; Liu, X.; Nagata, K.; Inagaki, M.; Majesky, M.W. Coronary smooth muscle differentiation from proepicardial cells requires rhoA–mediated actin reorganization and p160 rho–kinase activity. Dev. Biol. 2001, 240, 404–418. [Google Scholar] [CrossRef] - Pérez–Pomares, J.M.; Carmona, R.; González–Iriarte, M.; Atencia, G.; Wessels, A.; Muñoz–Chápuli, R. Origin of coronary endothelial cells from epicardial mesothelium in avian embryos. Int. J. Dev. Biol. 2002, 46, 1005–1013. [Google Scholar] - Pérez–Pomares, J.M.; Phelps, A.; Sedmerova, M.; Wessels, A. Epicardial–like cells on the distal arterial end of the cardiac outflow tract do not derive from the proepicardium but are derivatives of the cephalic pericardium. Dev. Dyn. 2003, 227, 56–68. [Google Scholar] [CrossRef] - de Lange, F.J.; Moorman, A.F.; Anderson, R.H.; Männer, J.; Soufan, A.T.; de Gier–de Vries, C.; Schneider, M.D.; Webb, S.; van den Hoff, M.J.; Christoffels, V.M. Lineage and morphogenetic analysis of the cardiac valves. Circ. Res. 2004, 95, 645–654. [Google Scholar] - Wilting, J.; Buttler, K.; Schulte, I.; Papoutsi, M.; Schweigerer, L.; Männer, J. The proepicardium delivers hemangioblasts but not lymphangioblasts to the developing heart. Dev. Biol. 2007, 305, 451–459. [Google Scholar] - Cai, C.L.; Martin, J.C.; Sun, Y.; Cui, L.; Wang, L.; Ouyang, K.; Yang, L.; Bu, L.; Liang, X.; Zhang, X.; et al. A myocardial lineage derives from Tbx18 epicardial cells. Nature 2008, 454, 104–108. [Google Scholar] - Zhou, B.; Ma, Q.; Rajagopal, S.; Wu, S.M.; Domian, I.; Rivera–Feliciano, J.; Jiang, D.; von Gise, A.; Ikeda, S.; Chien, K.R.; Pu, W.T. Epicardial progenitors contribute to the cardiomyocyte lineage in the developing heart. Nat. 2008, 454, 109–113. [Google Scholar] - Christoffels, V.M.; Grieskamp, T.; Norden, J.; Mommersteeg, M.T.; Rudat, C.; Kispert, A. Tbx18 and the fate of epicardial progenitors. Nat. 2009, 458, E8–E9. [Google Scholar] - Rudat, C.; Kispert, A. Wt1 and epicardial fate mapping. Circ. Res. 2012, 111, 165–169. [Google Scholar] [CrossRef] - Zhou, B.; Pu, W.T. Genetic Cre–loxP assessment of epicardial cell fate using Wt1–driven Cre alleles. Circ. Res. 2012, 111, e276–e280. [Google Scholar] [CrossRef] - Kikuchi, K.; Gupta, V.; Wang, J.; Holdway, J.E.; Wills, A.A.; Fang, Y.; Poss, K.D. tcf21+ epicardial cells adopt non–myocardial fates during zebrafish heart development and regeneration. Dev. 2011, 138, 2895–2902. [Google Scholar] [CrossRef] - Mikawa, T.; Gourdie, R.G. Pericardial mesoderm generates a population of coronary smooth muscle cells migrating into the heart along with ingrowth of the epicardial organ. Dev. Biol. 1996, 174, 221–232. [Google Scholar] [CrossRef] - Guadix, J.A.; Carmona, R.; Muñoz–Chápuli, R.; Pérez–Pomares, J.M. In vivo and in vitro analysis of the vasculogenic potential of avian propeicardiial and epicardial cells. Dev. Dyn. 2006, 235, 1014–1026. [Google Scholar] [CrossRef] - Cosette, S.; Misra, R. The identification of different endothelial cell populations within the mouse proepicardium. Dev. Dyn. 2011, 240, 2344–2353. [Google Scholar] [CrossRef] - Katz, T.C.; Singh, M.K.; Degenhardt, K.; Rivera–Feliciano, J.; Johnson, R.L.; Epstein, J.A.; Tabin, C.J. Distinct compartments of the proepicardial organ give rise to coronary endothelial cells. Dev. Cell. 2012, 22, 639–650. [Google Scholar] [CrossRef] - Stern, C. The chick: a great model system becomes even greater. Dev. Cell. 2005, 8, 9–17. [Google Scholar] - Coleman, C.M. Chicken embryos as model for regenerative medicine. Birth Defects Res. Part C. 2008, 84, 245–256. [Google Scholar] [CrossRef] - Poynter, G.; Huss, D.; Lansford, R. Japanese quail: An efficient animal model for the production of transgenic avians. Cold Spring Harb. Protoc. 2009. [Google Scholar] [CrossRef] - Männer, J. Extracardiac tissues and the epigenetic control of myocardial development in vertebrate embryos. Ann. Anat. 2006, 188, 199–212. [Google Scholar] [CrossRef] - Smith, T.K.; Bader, D.M. Signals from both sides: Control of cardiac development by the endocardium and epicardium. Semin. Cell. Dev. Biol. 2007, 18, 84–89. [Google Scholar] [CrossRef] - Chapman, S.C.; Lawson, A.; Mac Arthur, W.C.; Wiese, R.J.; Loechel, R.H.; Burgos–Trinidad, M.; Wakefield, J.K.; Ramabhadran, R.; Mauch, T.J.; Schoenwolf, G.C. Ubiquitous GFP expression in transgenic chickens using a lentiviral vector. Dev. 2005, 132, 935–940. [Google Scholar] [CrossRef] - Iacobellis, G.; Malavazos, A.E.; Corsi, M.M. Epicardial fat: from the biomolecular aspects to the clinical practice. Int. J. Biochem. Cell. Biol. 2011, 43, 1651–1654. [Google Scholar] [CrossRef] - Sacks, H.S.; Fain, J.N. Human epicardial fat: What is new and what is missing? Clin. Exp. Pharmacol. Physiol. 2011, 38, 879–887. [Google Scholar] [CrossRef] © 2013 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution license (http://creativecommons.org/licenses/by/3.0/).	2	8	0	0	0	1	0.561532	1	10,508
Flashcards in Fiser ABSITE Ch. 36-37 Colorectal/Anal Deck (135): What embryologic layer does the gut derive from? In regards to for-, mid- and hindgut, what portion of the small bowel arises from each? Forgut: Ends at the second portion of the duodenum, uses the celiac artery as blood supply. Midgut: Duodenal ampulla to first two-thirds of the transverse colon, supplied by the SMA. Hindgut: distal transverse colon and descending colon, proximal rectum, supplied by the IMA. What is the distal rectum derived from? The cloaca (also gives rise to the urogenital tract), with branches of the internal iliac system supplying it. What does the anus derive from? Invagination of the ectodermal anal pit and fusion with the distal rectum at the dentate line. How does the longitudinal muscles of the colon and rectum differ? It completely encapsulates the rectum, but makes of teniae coli of the colon. (which are associated with haustra). Where is the end of the colon? Ends at the peritoneal reflection, roughly 15 cm from the anal verge. What is the largest diameter portion of the colon, and most prone to perforation? The cecum, with an average diameter of 7.5 cm. When attempting to identify the left ureter, what is a helpful landmark associated with the sigmoid colon? The intersigmoid fossa, a small recess formed by the mesosigmoid, where it attaches to the pelvic sidewall. At what level do the teniae coli broaden to begin the rectum? The sacral promontory (marks the start of the rectum). What lies just posterior to the rectum? Which layer contains lymphatics, that when dissected around, preserves them? Presacral fascia; The fascia propria is a distinct mesothelial layer that envelops the mesorectum and presacral fascia. Dissection in the plane between the fascia propria and sacrum preserves the lymphatics contained in the mesorectum. What ligament contains the middle rectal artery, and what are its origins? The fascia propria condenses anterolaterally into two rectal ligamentsthat contain the middle rectal artery and mixed autonomic nerves (injury can lead to impotence and bladder dysfunction). What region marks the beginning of the anus? What anatomic structures are found there? The dentate line, where mucosa forms longitudinal folds (Columns of Morgagni). In what order do the arterial branches come off the SMA? Middle colic, then right colic and finally the ileocolic (which branches into the appendiceal artery). What arteries supply the rectum? What are their origins? Superior rectal - off IMA; Middle rectal - from the internal iliac artery; Inferior rectal - arises from the internal pudendal artery What artery connects the proximal SMA and the IMA? The Arc of Riolan. What are the order of veins converging when creating the portal vein? The inferior mesenteric vein joins the splenic vein, then the superior mesenteric vein joins to create the portal vein. How does the inferior rectum's venous drainage vary from more superior parts of the large intestine? The distal rectum and anus are drained by the middle and inferior rectal veins into the internal iliac veins. When ligating the IMA, what nerve injury has been described and what are its consequences? The Hypogastric nerve, resulting in ejaculatory dysfunction in men. What are the overall physiologic roles of the colon? Extract water (roughly 90% recovered), sodium (90% recovered via active Na/K ATPases), short chain fatty acids (butyrate, propionate, acetate that are made by bacterial fermentation are the PRIMARY ENERGY SOURCE FOR COLONIC EPITHELIUM, also help create an osmotic gradient) and some vitamins. In regard to bacteria, what are the most common colonic anaerobe and aerobe? Anaerobe: Bacteroides; Aerobe: E. coli What are the three primary contractility patterns of the colon? Retrograde: begins at hepatic flexure down to cecum, allows increased stool mixing; Segmental contraction: short distances, further increases stool mixing; Mass movements: longer progression, with pressures rising up to 200 mmHg (highest in the sigmoid colon) What are the two nerve complexes of the colon? Auerbach (myenteric, located between the longitudinal and circular layers of the muscularis propria) and Meissner (submucosal, from nerve fibers that perforate the circular muscle layer). Why are diverticula of the colon pseudodiverticula? They only contain mucosa and submucosa, and not ALL the layers of the colonic wall. What percentage of patients who have an episode of uncomplicated diverticulitis experience a second episode? What is Hinchey Stage I diverticulitis? Small confined pericolic abscess (Less than 2 cm). Treated with antibiotic What is Hinchey Stage II diverticulitis? Larger, walled off abscess that is pericolic. Often percutaneously drained What is Hinchey Stage III diverticulitis? Generalized purulent peritonitis. What is Hinchey Stage IV diverticulitis? Generalized feculant peritonitits. What is the most common fistula to form following complicated diverticulitis (specify for men and women)? Men: Colovesicular fistulas, presenting with pneumaturia and recurrent UTIs. What are the two most common causes (in order) of LARGE bowel obstruction? Cancer if first. Benign stricture from diverticular disease is second. How does a competent ileocecal valve change a colonic obstruction? It creates a closed loop obstruction, leading to vascular compromise, pneumatosis and portal venous gas. What populations are at highest risk of sigmoid volvulus? Elderly (> 70 yo), including the debilitated and chronically constipated, and those on psychotropic medications are at highest risk. In these patients the sigmoid colon can lengthen by as much as 70%, predisposing to volvulus. On imaging they'll have a "bent inner tube on plain film, or a whorl sign on CT. What is the first treatment for sigmoid volvulus? Decompression with a rectal tube placed via proctoscopy or colonoscope. Roughly 80% reduce. They will then eventually need surgical resection of the their sigmoid (nearly 20-50% will recur). What is a cecal bascule (makes up 10% of all cecal volvulus)? A capacious and mobile cecum flips over and is trapped by the fixed ascending colon. What percentage of surgically reduced cecal volvulus cases will recur? At what size is colonic dilation considered an emergency due to likely vascular compromise? What infectious colitis affects roughly 10% of AIDS patients? Cytomegaloviral colitis. Treat with Gancyclovir. What two toxins are made by C. difficile, and what overall effect do they have? Enterotoxin A and Cytotoxin B, which cause mucosal damage, causing characteristic exudative pseudomembrane. On pathology you see PMN inflammation of mucosa and submucosa. What are some common pathologic findings in both UC and Crohn's disease? Pseudopolyps (clumps of regenerating mucosa), crypt abscesses (infammatory collections in the base of the Crypts of Lieberkuhn, which contain mucus-secreting Goblet Cells). What are some extra-intestinal manifestations of Ulcerative Colitis? Pyoderma gangrenosum: inflammatory ulcerative skin disease often found around ostomy sites and on arms/legs. Can be controlled once intestinal disease is controlled! Primary sclerosing cholangitis: obliterating inflammatory disease of the small and large bile ducts. Colectomy does NOT improve symptoms. Arthritides, including ankylosing spondylitis and sacroiliitis, linked to UC and HLA-B27. Not improved with colectomy. What is the overall risk of developing colorectal cancer when you have UC? During first 10 years of disease, risk is 2-3%. It rises 2% per year after that with a lifetime risk of 35%. Therefore, annual colonoscopy starts 10 years after age of diagnosis. What is pouchitis, a complication often seen following ileal pouch-anal anastomosis (IPAA)? Treatment? Idiopathic inflammation of the ileal pouch, seen in 50% of patients after an IPAA. Likely due to bacterial overgrowth. Diagnosis confirmed by sigmoidoscopy. Treated with Flagyl (Metronidizole). IF the patient has stump pouchitis from unused rectum, you treat with short chain fatty acid enemas. Salycilates, stool enemas and probiotics have been shown to help. What are indications for surgical intervention in Crohn's disease? 1) Failure of medical therapy 2) Obstruction (from inflammation, abscess or stricture) 3) Intra-abdominal abscess 4) Symptomatic fistulas 5) Severe perianal disease 6) Failure to thrive 7) Colorectal cancer What is sited as adequate margins when resecting Crohn's disease? What are the two major types of polyps found in the colon? Neoplastic (adenomatous) and benign (hyperplastic, juvenile, inflammatory). What conditions do you see hyperplastic polyps (benign) in the colon? The most common benign polyp, are usually small and have no malignant potential. What conditions do you see juvenile polyps (benign) in the colon? They are hamartomas, seen throughout the GI tract, often in patients with severe polyposis syndromes (aka Juvenile Polyposis syndrome, an AD disorder). Can often bleed. Not malignant, but patients at increased risk of colon cancer. What conditions do you see inflammatory polyps (benign) in the colon? Heaps of regenerative and inflammatory tissue, seen in BID and some erosive infections. What are the three pathological classifications of adenomatous polyps? Includes tubular (with branching glands, most common [75%] ), villous (with long fingerlike glands), or tubulovillous (mixed features). What are features of adenomatous polyps are harbingers of increased risk for cancer? Increased size (> 2 cm), villous features (40% risk), sessile appearance (flat, as opposed to pedunculated). What constitutes and adequate polypectomy when resecting a cancerous polyp? If malignancy is classified as 3 or below (invasion into the stalk, but NOT the submucosa of the bowel wall), is well or moderately differentiated, and margins are > 2 mm. These patients then require early repeat colonoscopy at 3 years out due to 40% risk of developing another cancerous polyp. Why are polyps classified as a Level 4 (invading colonic submucosa), or with poorly differentiated lesions, not adequately treated by polypectomy? Because risk of nodal metastasis is 10%, and therefore requires surgical resection. What is the most common mutation in Familial Adenomatous Polyposis (FAP)? Most often (80% of the time) due to autosomal dominant APC mutation (a tumor suppressor gene) on chromosome 5. * Remember, that there is a 100% chance of developing cancer by 40 y/o! These patients get total colectomies prophylactically at 20 years old (total abdominal colectomy with IPAA) What is Gardner Syndrome? Genetic disorder causing GI polyposis, ostemoas, sarcomas and epidermoid inclusion cysts. What is Turcot Syndrome? Genetic disorder with associated polyposis and brain tumors. At what age do patients with increased risk of colon cancer (FAP, Turcot/Gardner Syndrome) begin screening? 10-12 years old. They get annual sigmoidoscopy, stopping at age 40 IF NORMAL. What is the risk of developing cancer in an FAP patient who underwent total abdominal colectomy with ileorectal anastomosis? What is done about this? 25%. Therefore screening of the remaining rectum should be performed frequently. Why do FAP patients need to undergo upper endoscopy every 2 years? They are at risk of developing polyps in the stomach and near the duodenal papilla (periampullary cancer is the second leading cause of death in FAP patients after colon cancer)! What disease is the most common cause of inherited colon cancer, and what is the cause? Hereditary Non-Polyposis Colorectal Cancer (HNPCC, aka Lynch Syndrome), caused by errors in the DNA mismatch repair genes (many varying mutations identified). Associated with disease in many other organ systems (small bowel, ureter and renal pelvis, endometrium) What criteria are utilized for the diagnosis of Lynch Syndrome (aka HNPCC)? Amsterdam Criteria (Three, Two, One): Three affected relatives spanning two generations, with at least one family member diagnosed before 50 y/o. When diagnosed with colon cancer in patients with HNPCC, what is the suggested surgical treatment for men and women? Men: Total abdominal colectomy with ileorectal anastomosis (future rectal monitoring will be needed yearly). Women: Above + TAHBSO (these patients are at increased risk of ovarian, endometrial, bladder and stomach cancer) What genetic mutation characterizes Multiple Polyposis Coli? AD syndrome in the SMAD4 gene. Have juvenile polyps that can bleed or act as lead points for intussusception. Adenomatous elements found in 10% of polyps. Patients at increased risk for gastric, pancreatic and duodenal cancer. What genetic mutation characterizes Peutz-Jeghers Syndrome? AD disease with mutation in tumor suppressor gene STK11. Patients have multiple hamartomatous polyps, as well as hyperpigmentation of the lips and digits. There's a 2-10% risk of GI cancer as well as extra-intestinal malignancy (gonadal, breast, biliary). What are the screening recommendations for someone at moderate risk for colon cancer (cancer in first degree relative diagnosed > 60 y/o)? Screening every 10 years starting at 40 y/o. What are the screening recommendations for someone at moderate risk for colon cancer (cancer in first degree relative diagnosed > 60 y/o or > 2 relatives with diagnosis)? Every 5 years, starting 10 years before the youngest relative's age of diagnosis. What are the screening recommendations for someone at high risk with FAP? Start screening at 10 yo with sigmoidoscopy, and continue yearly at least until 40 years old. What are the screening recommendations for someone at high risk with HNPCC? Start screening at 20 y/o (or 10 years earlier than earliest diagnosed relative), then perform every 2 years until 35-40, then yearly. What are the screening recommendations for someone at high risk with inflammatory bowel disease? Begin screening 10 years after onset of symptoms of colitis, then continue every 1-2 years. What percentage of patients who have limited metastases with the diagnosis of colon cancer have curative resections? What is the minimally acceptable margins when resecting colon cancer? What criteria would allow transanal excision of a rectal tumor? - Must be less than 4 cm in size - Within 6 cm of anal verge - Involve less than 40% circumference of the bowel - Be T1 or T2 (invade the muscularis propria AT MOST) - Well differentiated without vascular/lymphatic involvement. Rate of recurrence is 8% (T1 lesions) or 20% (T2 lesions). When attempting to identify a GI bleed, what criteria are needed for tagged radionuclide red blood cell scan to be effective? Requires active bleeding at a rate of 1 mL/min. Because labeling of RBCs persists up to 24 hours, intermittent bleeds can identified with serial scans. Only allows regional localization of bleeding. Labeled colloid scans can identify bleeds at a rate of ≥ 0.1 mL/min. When attempting to identify a GI bleed, what criteria are needed for angiography to be effective? Requires active bleeding at a rate of 0.5 mL/min. What percentage of diverticular bleeds resolve spontaneously? 75%. It's the most common cause of lower GI bleeding. What causes the initial vague abdominal pain of appendicitis, as well as nausea and anorexia? Distention of the appendix and stimulation of visceral nerves. What causes the progressively focal pain of appendicitis? Irritation of the peritoneum locally as the inflammation progresses. Following non-operative management of perforated appendicitis, what is the rate of recurrence? 35% (Hence the argument for interval appendectomy). What is Goodsall's rule in regard to relationship between the internal and external openings of fistuals-in-ano? Openings in the skin anterior and within 3 cm of the verge track directly to the anal canal. Those posterior to the line or further than 3 cm will take a curved path and track to the posterior midline. What are the two types of rectovaginal fistula, and how does that change management? Either LOW (involving lower third of the rectum and lower half of the vagina) or HIGH (between middle third of the rectum and posterior vaginal fornix. Low lesions are amenable to transanal approaches (including myocutaneous advancement flap of the rectal mucosa and internal sphincter). High lesions require transabdominal repair. What causes anal fissures? Mucosal ischemia secondary to hyperplasia and hypertonicity of the internal anal sphincter. Ulcers will have an associated heaped up epidermis (sentinel pile). If fissures are away from the midline (particularly the posterior midline) worry about Crohn's disease. Once medical management has failed (including CCBs or nitroglycerin creams) for anal fissures, what surgical technique can be utilized? Lateral internal sphincterotomy. Recurrence after surgery is less than 10%. Incontinence is a RARE (less than 1%) complication. What are the locations of the highly vascularized, thickened submucosa that lines the anal canal, and can give rise to hemorrhoids? Left lateral, right anterior and posterior. Those ABOVE the dentate are internal and those below are external. Describe the following: First degree hemorrhoid Bleeding, without prolapse Describe the following: Second degree hemorrhoid Prolapse with strain, spontaneously reduces. Describe the following: Third degree hemorrhoid Prolapses with strain, requires manual reduction. Describe the following: Fourth degree hemorrhoid Prolapse and incarcerated. Can become strangulated and thrombosed. Why are no more than two hemorrhoids excised at a time? Decreases the risk of anal stenosis. What is the rate of recurrence of an incised external hemorrhoid if done within 72 hours? What has been linked to incontinence and rectal prolapse (procidentia)? Proximal pudendal nerve damage causing weakening of the pelvic floor. Can be due to diabetes, obstetric trauma, or iatrogenic injury. Over 50% have constipation, 35% are nullparous, 15% have diarrhea, 75% experience some incontinence. Before surgical intervention for prolapse (procidentia), what study must be performed? Colonoscopy, to rule out more severe pathology. What are the percentages of improvement in regards to incontinence, constipation and prolapse (procidentia) in patients who've undergone transabdominal resection rextopexy? Incontinence: 40%; Constipation: 50%; Prolapse: 98% What is the recurrence rate following an Altemeier procedure for rectal prolapse (procidentia)? What is the determining factor for T status in patients with anal cancer? Size (duh), with T1 less than 2 cm, T2 is 2 - 5 cm, T3 > 5 cm What disease is an indolent in situ squamous cell carcinoma of the anal verge? Bowen disease! Usually plaque-like, can present with pruritis and burning. What is the treatment for Bowen Disease (in situ squamous cell carcinoma? Wide local excision with minimal margins. Invasion is found in 5% of cases, and requires APR. What is a plaque-like anal lesion (similar in appearance to Bowen disease) that is more common in older patients, and is possibly originating from Langerhans cells or a met from underlying rectal carcinoma? Paget disease (adenocarcinoma in situ). Treated with wide local excision (if primary), or APR (if associated with underlying carcinoma). Adjuvant chemo/radiation is standard. What is the disease caused by HPV, and is the most common perianal STD? Condyloma acuminatum. It is STRONGLY associated with HIV infection. Requires anoscopy (some cases extend into the anal canal). What is the treatment for Condyloma Acuminatum? Fulguration with electrocautery and direct excision. What are Buschke-Lowenstein Tumors, and what are they associated with? Also called verrucous carcinomas, they are larger invasive derivatives of condyloma, and can become infected or fistulize. It is NOT uncommon for it to undergo transformation squamous cell carcinoma. Treat with WLE or APR, with addition of rad/chemo if carcinoma found. In regards to anorectal carcinoid tumors (4th most common site of presentation), what is the size determination in regards to treatment? Tumors less than 2 cm can be locally excised, > 2 cm require APR. What are the transverse bands that form the haustra of the colon? What major arteries does the Marginal Artery of Drummond connect? The SMA and IMA branches. In regards to the rectum, what do the lateral stalks contain? The middle rectal arteries, which supply the mid-portion of the rectum. Where does the inferior rectal artery branch off of? The internal pudendal artery, which is a branch of the internal iliac. What is the muscular origin of the external sphincter, and what nerves innervate it? Puborectalis (as a continuation of the levator ani muscle). Neural control comes from the inferior rectal branch of the internal pudendal nerve and perineal branch of S4. With regard to colonic polyps, what is another term for high grade dysplasia? Carcinoma in situ (the basement membrane is intact). It becomes invasive once it invades the submucosa (T1). What is the new surveillance in a patient who had the below, and subsequent interval screening: - Had resection of a single adenoma less than 1 cm? 5 years post-polypectomy. IF normal, can resume average-risk recommendations. What is the new surveillance in a patient who had the below, and subsequent interval screening: - Following resection ≥ 1 cm or high-risk adenoma? 3 years post-polypectomy. Then repeat again in 3 years, if normal can return to normal screening. What is the new surveillance in a patient who had the below, and subsequent interval screening: - Had curative resection of colorectal cancer? Within one year post-op. Repeat again in 3 years, then every 5 years. What is the new surveillance in a patient who had the below, and subsequent interval screening: - Inflammatory bowel disease? Within 8 years of diagnosis. Survey for dysplasia q1-2 years. In patients with colon cancer and liver metastases, if resectable what is the 5-year survival? What percentage of colorectal cancer patients have drop metastases to the ovaries? What is the significance of Batson's plexus in regards to colorectal cancer? Rectal cancer can metastasize directly to the the spine via this venous plexus. What are the primary chemotherapeutic agents utilized for Stage II (rectal) and III (colon or rectal) cancers? 5FU, Leukovorin and Oxaliplatin What are chemotherapeutic agents used for stage IV colon or rectal cancer? 5FU, Leuovorin. Can also use Avastin (Bevacizumab), a monoclonal Ab against VEGF OR Erbitux (Cetaximab), monoclonal Ab against epidermal growth factor. These increase survival but do not cure cancer. What criteria must be met for rectal cancer to get post-op XRT? Needs to be a T3 tumor or have positive nodes (stage II or higher) What is the most common site of injury following radiation treatment for rectal cancer? Rectum, causing vasculitis, thrombosis, ulcers or strictures. For colorectal cancer patients status post resection, what is the interval follow up for PE, CEA and stool guaiac? Every 6 months for 3 years, then annually. They should also get yearly LFTs, abdominal CT, colonoscopy and CXR. In addition to colon screening, what do WOMEN with HNPCC need to undergo? Endometrial biopsy every 3 years, annual pelvic exam, earlier mammograms. What medications have been shown to maintain remission in UC patients? In ulcerative colitis, what is the most likely site of perforation? In ulcerative colitis, what is the colon cancer risk? 1-2% per year starting 10 years after initial diagnosis. High grade dysplasia is an indication to operate. What is Ogilvie's Syndrome? Pseudo-obstruction of the colon causing massively dilated colon. Treat with colonoscopy and decompression with neostigmine (a reversible acetylcholinesterase inhibitor causing more ACh, leading to more nicotinic/muscarinic stimulation). What is Lymphogranuloma venereum? Caused by Chlamydia, mostly in homosexuals, it causes proctitis, tenesmus and bleeding. Treat with Doxycycline, Hydrocortisone. What is neutropenic typhlitis (enterocolitis)? Following chemotherapy when WBCs are low, it can mimic surgical disease (including pneumatosis), treat with antibiotics. What bacterial infection can mimic appendicitis? Yersinia. Treat with Tetracycline or Bactrim. What is Solitary Rectal Ulcer Syndrome? Chronic straining leads to internal intussusception, frictional damage. You treat with high fiber diets and defecation training. In regards to the anus, what is the venous drainage? Above the dentate line it is via the internal hemorrhoid plexus and below the external hemorrhoid plexus What is the most dreaded complication of internal closed hemorrhoidectomy? Pelvic sepsis (usually in immunocompromised patients), which presents with perianal pain, fever and urinary retention. Treat with IV antibiotics (cover GNR and anaerobes), EUA to rule out necrotizing soft tissue infection. Prior to surgical intervention, what are the various medical treatments for anal fissures? Sitz baths, bulk, lidocaine jelly, stool softeners (90% heal), also topical Diltiazem cream, Nitroglycerine paste or Bethanachol. The BEST non-operative intervention is botulinum injection In regards to anal canal cancers (above the dentate line), what is the primary treatment? This is similar to squamous, basaloid, cloacogenic, and transition cancers (all behave similarly). The Nigro protocol: 5-FU, Mitomycin, as well as radiation (not surgery). In regard to anal melanoma (third most common site after skin and eyes), what is the preferred treatment? APR vs. WLE (no survival benefit). What is the lymphatic drainage of the rectum and anus, which helps explain metastases via nodes in various cancers? Superior and middle rectum = IMA nodes; Lower rectum = primarily IMA nodes, but also internal iliac nodes; Upper 2/3 of anal canal = internal iliac and pelvic nodes; Lower 1/3 of anal canal = inguinal nodes In regards to radiation proctitis, what is the primary treatment? If that fails what is the fall back? Sucralfate enemas. If that fails use argon plasma coagulation or formalin injections.	2	16	0	0	0	1	0.395604	1	6,454
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. - Urinary Incontinence The symptoms and causes of urinary incontinence or urge incontinence. - COPD Get the facts about chronic obstructive pulmonary disease (COPD), including symptoms and complications. - Contraceptive Implants Facts about contraceptive implants; how they work, benefits & risks. - Diabetic Macular Edema Facts about diabetic macular edema, including the different types. - Endometriosis Facts about endometriosis, including symptoms. - Flu Facts about influenza (flu), including symptoms and vaccines. - GERD Get the facts about gastroesophageal reflux disease (GERD). - Hysterectomy Why a hysterectomy is performed and different surgeries available. - View All Care Guides Prepare for your next visit with our extensive library of Care Guides Dr. Bryan A Mejia has 2 locations 3555 Knickerbocker Rd San Angelo, TX 76904 Book Online Now Dr. Bryan A Mejia has the following 2 specialties - Obstetrics and Gynecology An obstetrician & gynecologist, or OB/GYN, is a physician who cares for women throughout their pregnancies, straight through to the delivery of their baby (obstetrician). They also specialize in annual care, as well as the diagnosis and treatment of disorders of the female reproductive system (gynecologist). Many physicians specialize in both of these fields in order to provide complete overall health services to women at every stage of life. - 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. Dr. Bryan A Mejia has the following 10 expertise - Pelvic Exam - Child Birth - Pap Smear - Menstrual Pain - Birth Control - Women's Health Dr. Bryan A Mejia is Board Certified in 2 specialties 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 2 of 2 Self-verified patient of Dr. Bryan A Mejia - Posted on December 11th, 2017 Self-verified patient of Dr. Bryan A Mejia - Posted on January 5th, 2016 Compassionate Doctor Recognition (2016, 2017, 2018) 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 (2016, 2018) 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. Patients' Choice Award (2016, 2017, 2018) 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. 11 Years Experience St Matthews University (Grand Cayman) Graduated in 2007 Medical Center Of Central Ga Dr. Bryan A Mejia Accepts the Following Insurance - Aetna Choice POS II - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO BCBS Blue Card - BCBS Blue Card PPO - BCBS TX Blue Advantage HMO - BCBS TX BlueChoice - BCBS TX HMO Blue Texas - First Health PPO - UHC Choice Plus POS - UHC Navigate HMO - UHC Navigate POS - UHC Options PPO Locations & Directions Dr. Bryan A Mejia is similar to the following 4 Doctors near San Angelo, TX.	2	6	1	0	0	0	0.955884	1	942
Australian Institute of Health and Welfare (2021) Heart, stroke and vascular disease—Australian facts., AIHW, Australian Government, accessed 09 December 2021 Australian Institute of Health and Welfare. (2021). Heart, stroke and vascular disease—Australian facts. Retrieved from https://www.aihw.gov.au/reports/heart-stroke-vascular-diseases/hsvd-facts Heart, stroke and vascular disease—Australian facts. Australian Institute of Health and Welfare, 29 September 2021, https://www.aihw.gov.au/reports/heart-stroke-vascular-diseases/hsvd-facts Australian Institute of Health and Welfare. Heart, stroke and vascular disease—Australian facts [Internet]. Canberra: Australian Institute of Health and Welfare, 2021 [cited 2021 Dec. 9]. Available from: https://www.aihw.gov.au/reports/heart-stroke-vascular-diseases/hsvd-facts Australian Institute of Health and Welfare (AIHW) 2021, Heart, stroke and vascular disease—Australian facts, viewed 9 December 2021, https://www.aihw.gov.au/reports/heart-stroke-vascular-diseases/hsvd-facts Get citations as an Endnote file: PDF \| 12.2Mb Australia uses the International Statistical Classification of Diseases and Related Health Conditions to code causes of death (WHO 2019). In this report, deaths between 1981 and 1996 were coded using the Ninth Revision (ICD-9), and deaths from 1997 using the Tenth Revision (ICD-10) (Table 1). Acute rheumatic fever and Rheumatic heart disease Coronary heart disease Acute myocardial infarction Atrial fibrillation and flutter Heart failure and cardiomyopathy 414.8, 428.0, 428.1, 428.9, 425.2, 425.4, 425.5, 425.7, 425.8, 425.9 I50, I25.5, I42.0, Peripheral arterial disease Atherosclerosis of peripheral arteries Abdominal aortic aneurysm Transient ischaemic attack Congenital heart disease Source: WHO 2019. The change in classification between ICD-9 and ICD-10 has resulted in a break in the underlying cause of death series between 1996 and 1997. Where available, comparability factors been applied to allow underlying cause of death data to be compared across this time period (Table 2). Heart failure & cardiomyopathy Acute rheumatic fever and rheumatic heart disease (a) Uses the comparability factor for Diseases of arteries, arterioles and capillaries (ICD–10 codes I70–I79). (b) Uses the comparability factor for Congenital malformations of the circulatory system (ICD–10 codes Q20–Q28). Source: ABS 2009. For hospital diagnoses and procedures, a classification modified for Australia is used. Hospital data to 1997–98 used the ICD-9-CM (International Classification of Diseases and Related Health Conditions, Ninth Revision, Clinical Modification) classification. After 1997–98, the ICD-10-AM classification (International Statistical Classification of Diseases and Related Health Conditions, Tenth Revision, Australian Modification) was used. Diagnosis and procedure data for 2018–19 were reported to the NHMD using the 11th edition of the ICD-10-AM (ACCD 2018a), incorporating the Australian Classification of Health Interventions (ACHI) (ACCD 2018b) (Tables 3 and 4). I00–I99, excluding I84 (haemorrhoids) Source: ACCD 2018a. Block no: 668 Block no: 1942 Heart valve repair / replacement Block no: 621–638 Block no: 650 Cardiac defibrillator implant Block no: 653 Block no: 660 Percutaneous coronary intervention Block no: 670, 671 Coronary artery bypass graft Block no: 672–679 Procedure code: 33500-00 Source: ACCD 2018b. Anatomical Therapeutic Chemical (ATC) codes are used in this report to classify medicines. This classification groups medicines according to the body organ or system they act upon, their therapeutic characteristics, and their chemical characteristics. A list of the medicine groups included in this report is shown in Table 5. More information on the ATC classification system can be found at the WHO Collaborating Centre for Drug Statistics Methodology. Renin-angiotensin system agents We'd love to know any feedback that you have about the AIHW website, its contents or reports. The browser you are using to browse this website is outdated and some features may not display properly or be accessible to you. Please use a more recent browser for the best user experience.	4	11	1	0	0	8	0.447115	9	1,056
CPR being administered during a simulation of cardiac arrest. \|Classification and external resources\| Cardiac arrest, also known as cardiopulmonary arrest or circulatory arrest, is a sudden stop in effective blood circulation due to the 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 myocardial infarction (also known as a heart attack), where blood flow to the muscle of the heart is impaired such that part or all of the heart tissue dies. 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 treatment is important. 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. A number of heart conditions and non-heart-related events can cause cardiac arrest; the most common cause is coronary artery disease. - 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 sometimes preceded by certain symptoms such as fainting, fatigue, blackouts, dizziness, chest pain, shortness of breath, weakness, and vomiting. The arrest may also occur with no warning. When the arrest occurs, the most obvious sign of its occurrence will be the lack of a palpable pulse in the person experiencing it (since the heart has ceased to contract, the usual indications of its contraction such as a pulse will no longer be detectable). Certain types of prompt intervention can often reverse a cardiac arrest, but without such intervention the event will almost always lead to death. In certain cases, it is an expected outcome of a serious illness where death is expected. Also, as a result of inadequate cerebral perfusion, the patient will quickly become 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 artery disease is the leading cause of sudden cardiac arrest. Many other cardiac and non-cardiac conditions also increase one's risk. Coronary artery disease Approximately 60–70% of SCD is related to coronary artery disease, also known as ischemic heart disease. Among adults, it is the predominant cause of arrest, with 30% of people at autopsy showing signs of recent myocardial infarction. Non-ischemic heart disease A number of non-ischemic cardiac abnormalities can increase the risk of SCD, including cardiomyopathy, cardiac rhythm disturbances, myocarditis, hypertensive heart disease, and congestive heart failure. 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 fivefold. 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 one of every 5000 to 7000 newborns and is estimated to be responsible for 3000 deaths each year compared to the approximately 300,000 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 and 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). Mnemonic for causes - 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. A Cochrane review published in 2016 found moderate-quality evidence to show that blood pressure-lowering drugs do not appear to reduce sudden cardiac death. 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 with 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 an 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 that 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's 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. Either a bag valve mask or an advanced airway may be used to help with breathing. High levels of oxygen are generally given during CPR. Tracheal intubation has not been found to improve survival rates in cardiac arrest and in the prehospital environment may worsen it. 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. Mechanical chest compressions (as performed by a machine) are no better than chest compressions performed by hand. 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 children 2 to 4 J/Kg is recommended. 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 not been shown to improve survival to hospital discharge following out-of-hospital cardiac arrest. This includes the use of epinephrine, atropine, lidocaine, and amiodarone. Epinephrine is generally recommended every five minutes. Vasopressin overall does not improve or worsen outcomes compared to epinephrine. Epinephrine does appear to improve short-term outcomes such as return of spontaneous circulation. Some of the lack of long-term benefit may be related to delays in epinephrine use. While evidence does not support its use in children guidelines state its use is reasonable. Lidocaine and amiodarone are also deemed reasonable in children with cardiac arrest who have a shockable rhythm. The general use of sodium bicarbonate or calcium is not recommended. The 2010 guidelines from the American Heart Association no longer contain the association's previous 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. Targeted temperature management Cooling adults after cardiac arrest who have a return of spontaneous circulation (ROSC) but no return of consciousness improves outcomes. This procedure is called targeted temperature management (previously known as therapeutic hypothermia). People are typically cooled for a 24-hour period, with a target temperature of 32–36 °C (90–97 °F). Death rates in the hypothermia group are 35% lower than in those with no temperature management. Complications are generally no greater in those who receive this therapy. Earlier versus later cooling may result in better outcomes. A trial that cooled in the ambulance, however, found no difference compared to starting cooling in-hospital. A registry database found poor neurological outcome increased by 8% with each five-minute delay in initiating TH and by 17% for every 30-minute delay in time to target temperature. In children it is unclear if cooling is beneficial however fever should be prevented. 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 organizations 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 although high quality evidence is lacking. It is recommended that it is done as soon as possible in those who have had a cardiac arrest with ST elevation due to underlying heart problems. 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 overall chance of survival among those who have cardiac arrest outside of a hospital is 7.6%. Among children rates of survival is 3 to 16% in North America. Prognosis is typically assessed 72 hours or more after cardiac arrest. Rates of survival are better in those who someone saw collapse, got bystander CPR, or had either ventricular tachycardia or ventricular fibrillation when assessed. Survival among those with Vfib or Vtach is 15 to 23%. Women are more likely to survive cardiac arrest and leave hospital than men. 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. 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 reflects 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% had major neurological disability. Of those who were discharged from hospital, 70% were still alive four years later. Based on death certificates, sudden cardiac death accounts for about 15% of all death in Western countries (330,000 per year in the United States). The lifetime risk is three times greater in men (12.3%) than women (4.2%) based on analysis of the Framingham Heart Study. However this gender difference disappeared beyond 85 years of age. - Jameson, J. N. St C.; Dennis L. Kasper; Harrison, Tinsley Randolph; Braunwald, Eugene; Fauci, Anthony S.; Hauser, Stephen L; Longo, Dan L. (2005). Harrison's principles of internal medicine. New York: McGraw-Hill Medical Publishing Division. ISBN 0-07-140235-7. - Mallinson, T (2010). "Myocardial infarction". Focus on First Aid (15): 15. Retrieved 2010-06-08. - Safar P (December 1986). "Cerebral resuscitation after cardiac arrest: a review". Circulation 74 (6 Pt 2): IV138–53. PMID 3536160. - Holzer M, Behringer W (April 2005). "Therapeutic hypothermia after cardiac arrest". Current Opinion in Anesthesiology 18 (2): 163–8. doi:10.1097/01.aco.0000162835.33474.a9. PMID 16534333. - Safar P, Xiao F, Radovsky A, et al. (January 1996). "Improved cerebral resuscitation from cardiac arrest in dogs with mild hypothermia plus blood flow promotion". Stroke 27 (1): 105–13. doi:10.1161/01.STR.27.1.105. PMID 8553385. - Rippe, James M.; Irwin, Richard S. (2003). Irwin and Rippe's intensive care medicine. Hagerstwon, MD: Lippincott Williams & Wilkins. ISBN 0-7817-3548-3. - "Resuscitation Council (UK) Guidelines 2005". - Jasmeet Soar, Gavin D. Perkins, Jerry Nolan., eds. (2012). ABC of resuscitation (6th ed.). Chichester, West Sussex: Wiley-Blackwell. p. 43. ISBN 9781118474853. - "Mount Sinai - Cardiac arrest". - Parnia, S; Spearpoint, K; Fenwick, PB (August 2007). "Near death experiences, cognitive function and psychological outcomes of surviving cardiac arrest.". Resuscitation 74 (2): 215–21. doi:10.1016/j.resuscitation.2007.01.020. PMID 17416449. - Zheng ZJ, Croft JB, Giles WH, Mensah GA (October 2001). "Sudden cardiac death in the United States, 1989 to 1998". Circulation 104 (18): 2158–63. doi:10.1161/hc4301.098254. PMID 11684624. - Centers for Disease Control and Prevention (CDC) (February 2002). "State-specific mortality from sudden cardiac death--United States, 1999". MMWR Morb. Mortal. Wkly. Rep. 51 (6): 123–6. PMID 11898927. - Eisenberg MS, Mengert TJ (April 2001). "Cardiac resuscitation". N. Engl. J. Med. 344 (17): 1304–13. doi:10.1056/NEJM200104263441707. PMID 11320390. - Kannel WB, Wilson PW, D'Agostino RB, Cobb J (August 1998). "Sudden coronary death in women". Am. Heart J. 136 (2): 205–12. doi:10.1053/hj.1998.v136.90226. PMID 9704680. - Eckart RE, Scoville SL, Campbell CL, et al. (December 2004). "Sudden death in young adults: a 25-year review of autopsies in military recruits". Annals of Internal Medicine 141 (11): 829–34. doi:10.7326/0003-4819-141-11-200412070-00005. PMID 15583223. - Sudden Cardiac Death - Kuisma M, Alaspää A (July 1997). "Out-of-hospital cardiac arrests of non-cardiac origin. Epidemiology and outcome". Eur. Heart J. 18 (7): 1122–8. doi:10.1093/oxfordjournals.eurheartj.a015407. PMID 9243146. - Friedlander Y, Siscovick DS, Weinmann S, et al. (January 1998). "Family history as a risk factor for primary cardiac arrest". Circulation 97 (2): 155–60. doi:10.1161/01.cir.97.2.155. PMID 9445167. - ECC Committee, Subcommittees and Task Forces of the American Heart Association (December 2005). "2005 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care". Circulation 112 (24 Suppl): IV1–203. doi:10.1161/CIRCULATIONAHA.105.166550. PMID 16314375. - Ochoa FJ, Ramalle-Gómara E, Carpintero JM, García A, Saralegui I (June 1998). "Competence of health professionals to check the carotid pulse". Resuscitation 37 (3): 173–5. doi:10.1016/S0300-9572(98)00055-0. PMID 9715777. - Bahr J, Klingler H, Panzer W, Rode H, Kettler D (August 1997). "Skills of lay people in checking the carotid pulse". Resuscitation 35 (1): 23–6. doi:10.1016/S0300-9572(96)01092-1. PMID 9259056. - British Red Cross; St Andrew's Ambulance Association; St John Ambulance (2006). First Aid Manual: The Authorised Manual of St. John Ambulance, St. Andrew's Ambulance Association, and the British Red Cross. Dorling Kindersley Publishers Ltd. ISBN 1-4053-1573-3. - Taverny, G; Mimouni, Y; LeDigarcher, A; Chevalier, P; Thijs, L; Wright, JM; Gueyffier, F (10 March 2016). "Antihypertensive pharmacotherapy for prevention of sudden cardiac death in hypertensive individuals.". The Cochrane database of systematic reviews 3: CD011745. doi:10.1002/14651858.CD011745.pub2. PMID 26961575. Retrieved 19 March 2016. - Kause J, Smith G, Prytherch D, Parr M, Flabouris A, Hillman K (September 2004). "A comparison of antecedents to cardiac arrests, deaths and emergency intensive care admissions in Australia and New Zealand, and the United Kingdom--the ACADEMIA study". Resuscitation 62 (3): 275–82. doi:10.1016/j.resuscitation.2004.05.016. PMID 15325446. - "Slow Codes, Show Codes and Death". New York Times (New York Times Company). 22 August 1987. Retrieved 2013-04-06. - "Decision-making for the End of Life". Physician Advisory Service. College of Physicians and Surgeons of Ontario. May 2006. Retrieved 2013-04-06. - DePalma, Judith A.; Miller, Scott; Ozanich, Evelyn; Yancich, Lynne M. (November 1999). "Slow" Code: Perspectives of a Physician and Critical Care Nurse. Critical Care Nursing Quarterly 22 (Lippincott Williams and Wilkins). pp. 89–99. ISSN 1550-5111. - Birnie, David H; Sambell, Christie; Johansen, Helen; Williams, Katherine; Lemery, Robert; Green, Martin S; Gollob, Michael H; Lee, Douglas S; Tang, Anthony SL (July 2007). "Use of implantable cardioverter defibrillators in Canadian and IS survivors of out-of-hospital cardiac arrest". Canadian Medical Association Journal 177 (1): 41–6. doi:10.1503/cmaj.060730. PMC 1896034. PMID 17606938. Retrieved 2007-07-29. - Simpson CS (July 2007). "Implantable cardioverter defibrillators work--so why aren't we using them?". CMAJ 177 (1): 49–51. doi:10.1503/cmaj.070470. PMC 1896028. PMID 17606939. - Moss AJ, Brown MW, Cannom DS, et al. (October 2005). "Multicenter automatic defibrillator implantation trial-cardiac resynchronization therapy (MADIT-CRT): design and clinical protocol". Ann Noninvasive Electrocardiol 10 (4 Suppl): 34–43. doi:10.1111/j.1542-474X.2005.00073.x. PMID 16274414. - American Heart, Association (May 2006). "2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric advanced life support". Pediatrics 117 (5): e1005–28. doi:10.1542/peds.2006-0346. PMID 16651281. - Mutchner L (January 2007). "The ABCs of CPR--again". Am J Nurs 107 (1): 60–9; quiz 69–70. doi:10.1097/00000446-200701000-00024. PMID 17200636. - Resuscitation Council (UK). "Pre-hospital cardiac arrest" (PDF). www.resus.org.uk. p. 41. Retrieved 3 September 2014. - Resuscitation Council (UK) (5 September 2012). "Comments on the duration of CPR following the publication of 'Duration of resuscitation efforts and survival after in-hospital cardiac arrest: an observational study' Goldberger ZD et al. Lancet.". Retrieved 3 September 2014. - Neumar, RW; Shuster, M; Callaway, CW; Gent, LM; Atkins, DL; Bhanji, F; Brooks, SC; de Caen, AR; Donnino, MW; Ferrer, JM; Kleinman, ME; Kronick, SL; Lavonas, EJ; Link, MS; Mancini, ME; Morrison, LJ; O'Connor, RE; Samson, RA; Schexnayder, SM; Singletary, EM; Sinz, EH; Travers, AH; Wyckoff, MH; Hazinski, MF (3 November 2015). "Part 1: Executive Summary: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation 132 (18 Suppl 2): S315–67. doi:10.1161/cir.0000000000000252. PMID 26472989. - Studnek JR, Thestrup L, Vandeventer S, et al. (September 2010). "The association between prehospital endotracheal intubation attempts and survival to hospital discharge among out-of-hospital cardiac arrest patients". Acad Emerg Med 17 (9): 918–25. doi:10.1111/j.1553-2712.2010.00827.x. PMID 20836771. - Yao, L; Wang, P; Zhou, L; Chen, M; Liu, Y; Wei, X; Huang, Z (Jun 2014). "Compression-only cardiopulmonary resuscitation vs standard cardiopulmonary resuscitation: an updated meta-analysis of observational studies.". The American journal of emergency medicine 32 (6): 517–23. doi:10.1016/j.ajem.2014.01.055. PMID 24661781. - Huang, Y; He, Q; Yang, LJ; Liu, GJ; Jones, A (Sep 12, 2014). "Cardiopulmonary resuscitation (CPR) plus delayed defibrillation versus immediate defibrillation for out-of-hospital cardiac arrest.". The Cochrane database of systematic reviews 9: CD009803. doi:10.1002/14651858.CD009803.pub2. PMID 25212112. - de Caen, AR; Berg, MD; Chameides, L; Gooden, CK; Hickey, RW; Scott, HF; Sutton, RM; Tijssen, JA; Topjian, A; van der Jagt, ÉW; Schexnayder, SM; Samson, RA (3 November 2015). "Part 12: Pediatric Advanced Life Support: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation 132 (18 Suppl 2): S526–42. doi:10.1161/cir.0000000000000266. PMID 26473000. - Zoll AED Plus - Lyon R.M; Cobbe S.M.; Bradley J.M.; Grubb N.R.; et al. (2004). "Surviving out of hospital cardiac arrest at home: a postcode lottery?". Emergency Medical Journal 21: 619–624. doi:10.1136/emj.2003.010363. - Olasveengen TM, Sunde K, Brunborg C, Thowsen J, Steen PA, Wik L (November 2009). "Intravenous drug administration during out-of-hospital cardiac arrest: a randomized trial". JAMA 302 (20): 2222–9. doi:10.1001/jama.2009.1729. PMID 19934423. - Lin, S; Callaway, CW; Shah, PS; Wagner, JD; Beyene, J; Ziegler, CP; Morrison, LJ (Mar 15, 2014). "Adrenaline for out-of-hospital cardiac arrest resuscitation: A systematic review and meta-analysis of randomized controlled trials.". Resuscitation 85 (6): 732–40. doi:10.1016/j.resuscitation.2014.03.008. PMID 24642404. - Morley, PT (June 2011). "Drugs during cardiopulmonary resuscitation.". Current Opinion in Critical Care 17 (3): 214–8. doi:10.1097/MCC.0b013e3283467ee0. PMID 21499094. - Attaran, RR; Ewy, GA (July 2010). "Epinephrine in resuscitation: curse or cure?". Future cardiology 6 (4): 473–82. doi:10.2217/fca.10.24. PMID 20608820. - Neumar, RW; Otto, CW; Link, MS; Kronick, SL; Shuster, M; Callaway, CW; Kudenchuk, PJ; Ornato, JP; McNally, B; Silvers, SM; Passman, RS; White, RD; Hess, EP; Tang, W; Davis, D; Sinz, E; Morrison, LJ (Nov 2, 2010). "Part 8: adult advanced cardiovascular life support: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation 122 (18 Suppl 3): S729–67. doi:10.1161/CIRCULATIONAHA.110.970988. PMID 20956224. - Ong, ME; Pellis, T; Link, MS (June 2011). "The use of antiarrhythmic drugs for adult cardiac arrest: a systematic review.". Resuscitation 82 (6): 665–70. doi:10.1016/j.resuscitation.2011.02.033. PMID 21444143. - Perrott, J; Henneberry, RJ; Zed, PJ (December 2010). "Thrombolytics for cardiac arrest: case report and systematic review of controlled trials.". Annals of Pharmacotherapy 44 (12): 2007–13. doi:10.1345/aph.1P364. PMID 21119096. - Xiao, G; Guo, Q; Shu, M; Xie, X; Deng, J; Zhu, Y; Wan, C (February 2013). "Safety profile and outcome of mild therapeutic hypothermia in patients following cardiac arrest: systematic review and meta-analysis.". Emergency medicine journal : EMJ 30 (2): 91–100. doi:10.1136/emermed-2012-201120. PMID 22660549. - Nielsen, Niklas; Wetterslev, Jørn; Cronberg, Tobias; Erlinge, David; Gasche, Yvan; Hassager, Christian; Horn, Janneke; Hovdenes, Jan; Kjaergaard, Jesper; Kuiper, Michael; Pellis, Tommaso; Stammet, Pascal; Wanscher, Michael; Wise, Matt P.; Åneman, Anders; Al-Subaie, Nawaf; Boesgaard, Søren; Bro-Jeppesen, John; Brunetti, Iole; Bugge, Jan Frederik; Hingston, Christopher D.; Juffermans, Nicole P.; Koopmans, Matty; Køber, Lars; Langørgen, Jørund; Lilja, Gisela; Møller, Jacob Eifer; Rundgren, Malin; Rylander, Christian; Smid, Ondrej; Werer, Christophe; Winkel, Per; Friberg, Hans (17 November 2013). "Targeted Temperature Management at 33°C versus 36°C after Cardiac Arrest". New England Journal of Medicine 369 (23): 131117131833001. doi:10.1056/NEJMoa1310519. PMID 24237006. - Arrich, J; Holzer, M; Havel, C; Müllner, M; Herkner, H (Sep 12, 2012). "Hypothermia for neuroprotection in adults after cardiopulmonary resuscitation.". Cochrane database of systematic reviews (Online) 9: CD004128. doi:10.1002/14651858.CD004128.pub3. PMID 22972067. - Stockmann, H; Krannich, A; Schroeder, T; Storm, C (November 2014). "Therapeutic temperature management after cardiac arrest and the risk of bleeding: Systematic review and meta-analysis.". Resuscitation 85 (11): 1494–1503. doi:10.1016/j.resuscitation.2014.07.018. PMID 25132475. - Dell'anna, AM; Scolletta, S; Donadello, K; Taccone, FS (June 2014). "Early neuroprotection after cardiac arrest.". Current opinion in critical care 20 (3): 250–8. doi:10.1097/mcc.0000000000000086. PMID 24717694. - Sendelbach, S; Hearst, MO; Johnson, PJ; Unger, BT; Mooney, MR (July 2012). "Effects of variation in temperature management on cerebral performance category scores in patients who received therapeutic hypothermia post cardiac arrest.". Resuscitation 83 (7): 829–34. doi:10.1016/j.resuscitation.2011.12.026. PMID 22230942. - Loertscher, L; Reed, DA; Bannon, MP; Mueller, PS (January 2010). "Cardiopulmonary resuscitation and do-not-resuscitate orders: a guide for clinicians". The American Journal of Medicine 123 (1): 4–9. doi:10.1016/j.amjmed.2009.05.029. PMID 20102982. - Knox, C; Vereb, JA (December 2005). "Allow natural death: a more humane approach to discussing end-of-life directives". Journal of Emergency Nursing 31 (6): 560–1. doi:10.1016/j.jen.2005.06.020. PMID 16308044. - Lehot, JJ; Long-Him-Nam, N; Bastien, O (December 2011). "[Extracorporeal life support for treating cardiac arrest].". Bulletin de l'Academie nationale de medecine 195 (9): 2025–33; discussion 2033–6. PMID 22930866. - Camuglia, AC.; Randhawa, VK.; Lavi, S.; Walters, DL. (Sep 2014). "Cardiac catheterization is associated with superior outcomes for survivors of out of hospital cardiac arrest: Review and meta-analysis.". Resuscitation 85: 1533–1540. doi:10.1016/j.resuscitation.2014.08.025. PMID 25195073. - Cave, DM; Gazmuri, RJ; Otto, CW; Nadkarni, VM; Cheng, A; Brooks, SC; Daya, M; Sutton, RM; Branson, R; Hazinski, MF (2010-11-02). "Part 7: CPR techniques and devices: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation 122 (18 Suppl 3): S720–8. doi:10.1161/CIRCULATIONAHA.110.970970. PMC 3741663. PMID 20956223. - Sasson, C; Rogers, MA; Dahl, J; Kellermann, AL (January 2010). "Predictors of survival from out-of-hospital cardiac arrest: a systematic review and meta-analysis.". Circulation. Cardiovascular quality and outcomes 3 (1): 63–81. doi:10.1161/circoutcomes.109.889576. PMID 20123673. - Bougouin, W; Mustafic, H; Marijon, E; Murad, MH; Dumas, F; Barbouttis, A; Jabre, P; Beganton, F; Empana, JP; Celermajer, DS; Cariou, A; Jouven, X (September 2015). "Gender and survival after sudden cardiac arrest: A systematic review and meta-analysis.". Resuscitation 94: 55–60. doi:10.1016/j.resuscitation.2015.06.018. PMID 26143159. - Ballew KA (May 1997). "Cardiopulmonary resuscitation". BMJ 314 (7092): 1462–5. doi:10.1136/bmj.314.7092.1462. PMC 2126720. PMID 9167565. - van Gijn, MS; Frijns, D; van de Glind, EM; C van Munster, B; Hamaker, ME (Jul 2014). "The chance of survival and the functional outcome after in-hospital cardiopulmonary resuscitation in older people: a systematic review.". Age and ageing 43 (4): 456–63. doi:10.1093/ageing/afu035. PMID 24760957. - Cobbe SM, Dalziel K, Ford I, Marsden AK (June 1996). "Survival of 1476 patients initially resuscitated from out of hospital cardiac arrest". BMJ 312 (7047): 1633–7. doi:10.1136/bmj.312.7047.1633. PMC 2351362. 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 at Wikipedia's sister projects \|Definitions from Wiktionary\| \|Media from Commons\| \|News from Wikinews\| \|Quotations from Wikiquote\| \|Texts from Wikisource\| \|Textbooks from Wikibooks\| \|Learning resources from Wikiversity\|	2	54	9	0	2	0	0.912338	11	9,599
International Statistical Classification of Diseases and Related Health Problems The International Statistical Classification of Diseases and Related Health Problems, usually called by the short-form name International Classification of Diseases (ICD), is the international "standard diagnostic tool for epidemiology, health management and clinical purposes". The ICD is maintained by the World Health Organization (WHO), the directing and coordinating authority for health within the United Nations System. The ICD is designed as a health care classification system, providing a system of diagnostic codes for classifying diseases, including nuanced classifications of a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or disease. This system is designed to map health conditions to corresponding generic categories together with specific variations, assigning for these a designated code, up to six characters long. Thus, major categories are designed to include a set of similar diseases. The ICD is published by the WHO and used worldwide for morbidity and mortality statistics, reimbursement systems, and automated decision support in health care. This system is designed to promote international comparability in the collection, processing, classification, and presentation of these statistics. As in the case of the analogous (but limited to mental and behavioral disorders) Diagnostic and Statistical Manual of Mental Disorders (DSM, currently in version 5), the ICD is a major project to statistically classify health disorders, and provide diagnostic assistance. The ICD is a core statistically based classificatory diagnostic system for health care related issues of the WHO Family of International Classifications (WHO-FIC). The ICD is revised periodically and is currently in its tenth revision. The ICD-10, as it is therefore known, was developed in 1992 to track health statistics. ICD-11 was planned for 2017, but has been pushed back to 2018. Annual minor updates and triennial major updates are published by the WHO. The ICD is part of a "family" of guides that can be used to complement each other, including also the International Classification of Functioning, Disability and Health which focuses on the domains of functioning (disability) associated with health conditions, from both medical and social perspectives. - 1 Historical synopsis - 2 Versions of ICD - 3 Usage and current topics - 4 See also - 5 References - 6 External links In 1860, during the international statistical congress held in London, Florence Nightingale made a proposal that was to result in the development of the first model of systemic collection of hospital data. In 1893, a French physician, Jacques Bertillon, introduced the Bertillon Classification of Causes of Death at a congress of the International Statistical Institute in Chicago.http://www.who.int/entity/classifications/icd/en/HistoryOfICD.pdf History of the development of the ICD.A number of countries and cities adopted Bertillon's system, which was based on the principle of distinguishing between general diseases and those localized to a particular organ or anatomical site, as used by the City of Paris for classifying deaths. Subsequent revisions represented a synthesis of English, German, and Swiss classifications, expanding from the original 44 titles to 161 titles. In 1898, the American Public Health Association (APHA) recommended that the registrars of Canada, Mexico, and the United States also adopt it. The APHA also recommended revising the system every ten years to ensure the system remained current with medical practice advances. As a result, the first international conference to revise the International Classification of Causes of Death took place in 1900, with revisions occurring every ten years thereafter. At that time, the classification system was contained in one book, which included an Alphabetic Index as well as a Tabular List. The book was small compared with current coding texts. The revisions that followed contained minor changes, until the sixth revision of the classification system. With the sixth revision, the classification system expanded to two volumes. The sixth revision included morbidity and mortality conditions, and its title was modified to reflect the changes: International Statistical Classification of Diseases, Injuries and Causes of Death (ICD). Prior to the sixth revision, responsibility for ICD revisions fell to the Mixed Commission, a group composed of representatives from the International Statistical Institute and the Health Organization of the League of Nations. In 1948, the WHO assumed responsibility for preparing and publishing the revisions to the ICD every ten years. WHO sponsored the seventh and eighth revisions in 1957 and 1968, respectively. It later become clear that the established ten year interval between revisions was too short. The ICD is currently the most widely used statistical classification system for diseases in the world. International health statistics using this system are available at the Global Health Observatory (GHO).WHOSIS. WHO Statistical Information System. In addition, some countries—including Australia, Canada, and the United States—have developed their own adaptations of ICD, with more procedure codes for classification of operative or diagnostic procedures. Versions of ICD The ICD-6, published in 1949, was the first to be shaped to become suitable for morbidity reporting. Accordingly, the name changed from International List of Causes of Death to International Statistical Classification of Diseases. The combined code section for injuries and their associated accidents was split into two, a chapter for injuries, and a chapter for their external causes. With use for morbidity there was a need for coding mental conditions, and for the first time a section on mental disorders was added. The International Conference for the Seventh Revision of the International Classification of Diseases was held in Paris under the auspices of WHO in February 1955. In accordance with a recommendation of the WHO Expert Committee on Health Statistics, this revision was limited to essential changes and amendments of errors and inconsistencies. The Eighth Revision Conference convened by WHO met in Geneva, from 6 to 12 July 1965. This revision was more radical than the Seventh but left unchanged the basic structure of the Classification and the general philosophy of classifying diseases, whenever possible, according to their etiology rather than a particular manifestation. During the years that the Seventh and Eighth Revisions of the ICD were in force, the use of the ICD for indexing hospital medical records increased rapidly and some countries prepared national adaptations which provided the additional detail needed for this application of the ICD. In the USA, a group of consultants was asked to study the 8th revision of ICD (ICD-8a) for its applicability to various users in the United States. This group recommended that further detail be provided for coding hospital and morbidity data. The American Hospital Association's "Advisory Committee to the Central Office on ICDA" developed the needed adaptation proposals, resulting in the publication of the International Classification of Diseases, Adapted (ICDA). In 1968, the United States Public Health Service published the International Classification of Diseases, Adapted, 8th Revision for use in the United States (ICDA-8a). Beginning in 1968, ICDA-8a served as the basis for coding diagnostic data for both official morbidity [and mortality] statistics in the United States. The International Conference for the Ninth Revision of the International Classification of Diseases, convened by WHO, met in Geneva from 30 September to 6 October 1975. In the discussions leading up to the conference, it had originally been intended that there should be little change other than updating of the classification. This was mainly because of the expense of adapting data processing systems each time the classification was revised. There had been an enormous growth of interest in the ICD and ways had to be found of responding to this, partly by modifying the classification itself and partly by introducing special coding provisions. A number of representations were made by specialist bodies which had become interested in using the ICD for their own statistics. Some subject areas in the classification were regarded as inappropriately arranged and there was considerable pressure for more detail and for adaptation of the classification to make it more relevant for the evaluation of medical care, by classifying conditions to the chapters concerned with the part of the body affected rather than to those dealing with the underlying generalized disease.History of ICD (PDF) At the other end of the scale, there were representations from countries and areas where a detailed and sophisticated classification was irrelevant, but which nevertheless needed a classification based on the ICD in order to assess their progress in health care and in the control of disease. A field test with a bi-axial classification approach—one axis (criterion) for anatomy, with another for etiology—showed the impracticability of such approach for routine use. The final proposals presented to and accepted by the Conference in 1978 retained the basic structure of the ICD, although with much additional detail at the level of the four digit subcategories, and some optional five digit subdivisions. For the benefit of users not requiring such detail, care was taken to ensure that the categories at the three digit level were appropriate. For the benefit of users wishing to produce statistics and indexes oriented towards medical care, the Ninth Revision included an optional alternative method of classifying diagnostic statements, including information about both an underlying general disease and a manifestation in a particular organ or site. This system became known as the dagger and asterisk system and is retained in the Tenth Revision. A number of other technical innovations were included in the Ninth Revision, aimed at increasing its flexibility for use in a variety of situations. It was eventually replaced by ICD-10, the version currently in use by the WHO and most countries. Given the widespread expansion in the tenth revision, it is not possible to convert ICD-9 data sets directly into ICD-10 data sets, although some tools are available to help guide users. Publication of ICD-9 without IP restrictions in a world with evolving electronic data systems led to a range of products based on ICD-9, such as MeDRA or the Read directory. When ICD-9 was published by the World Health Organization (WHO), the International Classification of Procedures in Medicine (ICPM) was also developed (1975) and published (1978). The ICPM surgical procedures fascicle was originally created by the United States, based on its adaptations of ICD (called ICDA), which had contained a procedure classification since 1962. ICPM is published separately from the ICD disease classification as a series of supplementary documents called fascicles (bundles or groups of items). Each fascicle contains a classification of modes of laboratory, radiology, surgery, therapy, and other diagnostic procedures. Many countries have adapted and translated the ICPM in parts or as a whole and are using it with amendments since then. International Classification of Diseases, Clinical Modification (ICD-9-CM) is an adaption created by the U.S. National Center for Health Statistics (NCHS) and used in assigning diagnostic and procedure codes associated with inpatient, outpatient, and physician office utilization in the United States. The ICD-9-CM is based on the ICD-9 but provides for additional morbidity detail. It is updated annually on October 1. It consists of two or three volumes: - Volumes 1 and 2 contain diagnosis codes. (Volume 1 is a tabular listing, and volume 2 is an index.) Extended for ICD-9-CM - Volume 3 contains procedure codes for surgical, diagnostic, and therapeutic procedures. ICD-9-CM only The NCHS and the Centers for Medicare and Medicaid Services are the U.S. governmental agencies responsible for overseeing all changes and modifications to the ICD-9-CM. Work on ICD-10 began in 1983, and the new revision was endorsed by the Forty-third World Health Assembly in May 1990. The latest version came into use in WHO Member States starting in 1994. The classification system allows more than 155,000 different codes and permits tracking of many new diagnoses and procedures, a significant expansion on the 17,000 codes available in ICD-9. Adoption was relatively swift in most of the world. Several materials are made available online by WHO to facilitate its use, including a manual, training guidelines, a browser, and files for download. Some countries have adapted the international standard, such as the "ICD-10-AM" published in Australia in 1998 (also used in New Zealand), and the "ICD-10-CA" introduced in Canada in 2000. Adoption of ICD-10-CM was slow in the United States. Since 1979, the USA had required ICD-9-CM codes for Medicare and Medicaid claims, and most of the rest of the American medical industry followed suit. On 1 January 1999 the ICD-10 (without clinical extensions) was adopted for reporting mortality, but ICD-9-CM was still used for morbidity. Meanwhile, NCHS received permission from the WHO to create a clinical modification of the ICD-10, and has production of all these systems: - ICD-10-CM, for diagnosis codes, replaces volumes 1 and 2. Annual updates are provided. - ICD-10-PCS, for procedure codes, replaces volume 3. Annual updates are provided. On August 21, 2008, the US Department of Health and Human Services (HHS) proposed new code sets to be used for reporting diagnoses and procedures on health care transactions. Under the proposal, the ICD-9-CM code sets would be replaced with the ICD-10-CM code sets, effective October 1, 2013. On April 17, 2012 the Department of Health and Human Services (HHS) published a proposed rule that would delay, from October 1, 2013 to October 1, 2014,the compliance date for the ICD-10-CM and PCS. Once again, Congress delayed implementation date to October 1, 2015, after it was inserted into "Doc Fix" Bill without debate over objections of many. Revisions to ICD-10-CM Include: - Relevant information for ambulatory and managed care encounter. - Expanded injury codes. - New combination codes for diagnosis/symptoms to reduce the number of codes needed to describe a problem fully. - Addition of sixth and seventh digit classification. - Classification specific to laterality. - Classification refinement for increased data granularity. ICD-10-CA is a clinical modification of ICD-10 developed by the Canadian Institute for Health Information for morbidity classification in Canada. ICD-10-CA applies beyond acute hospital care, and includes conditions and situations that are not diseases but represent risk factors to health, such as occupational and environmental factors, lifestyle and psycho-social circumstances. The World Health Organization is currently revising the International Classification of Diseases (ICD) towards the ICD-11. The development is taking place on an internet-based workspace, called iCAT (Collaborative Authoring Tool) Platform, somewhat similar to a wiki – yet it requires more structure and peer review process. The WHO collaborates through this platform with all interested parties. The final draft of the ICD-11 system is expected to be submitted to WHO's World Health Assembly (WHA) for official endorsement by 2017. The draft review was completed in April 2015 A final version for approval at the WHA is expected in 2018. In ICD-11 each disease entity will have definitions that give key descriptions and guidance on what the meaning of the entity/category is in human readable terms - to guide users. This is an advancement over ICD-10, which had only title headings. The Definitions have a standard structure according to a template with standard definition templates and further features exemplified in a "Content Model". The Content Model is a structured framework that captures the knowledge that underpins the definition of an ICD entity. The Content Model therefore allows computerization (with links to ontologies and SNOMED CT). Each ICD entity can be seen from different dimensions or "parameters". For example, there are currently 13 defined main parameters in the Content Model (see below) to describe a category in ICD. - ICD Entity Title - Fully Specified Name - Classification Properties - disease, disorder, injury, etc. - Textual Definitions - short standard description - Terms - synonyms, other inclusion and exclusions - Body System/Structure Description - anatomy and physiology - Temporal Properties - acute, chronic or other - Severity of Subtypes Properties - mild, moderate, severe, or other scales - Manifestation Properties - signs, symptoms - Causal Properties - etiology: infectious, external cause, etc. - Functioning Properties - impact on daily life: activities and participation - Specific Condition Properties - relates to pregnancy etc. - Treatment Properties - specific treatment considerations: e.g. resistance - Diagnostic Criteria - operational definitions for assessment ICD exists in 41 Languages in electronic versions and its expression in multiple languages will be systematically pursued in ICD11. Usage and current topics \|\|The examples and perspective in this article deal primarily with the United States and do not represent a worldwide view of the subject. (April 2012) (Learn how and when to remove this template message)\| History and usage in the United States In the United States, the U.S. Public Health Service published The International Classification of Diseases, Adapted for Indexing of Hospital Records and Operation Classification (ICDA), completed in 1962 and expanding the ICD-7 in a number of areas to more completely meet the indexing needs of hospitals. The U.S. Public Health Service later published the Eighth Revision, International Classification of Diseases, Adapted for Use in the United States, commonly referred to as ICDA-8, for official national morbidity and mortality statistics. This was followed by the ICD, 9th Revision, Clinical Modification, known as ICD-9-CM, published by the U.S. Department of Health and Human Services and used by hospitals and other healthcare facilities to better describe the clinical picture of the patient. The diagnosis component of ICD-9-CM is completely consistent with ICD-9 codes, and remains the data standard for reporting morbidity. National adaptations of the ICD-10 progressed to incorporate both clinical code (ICD-10-CM) and procedure code (ICD-10-PCS) with the revisions completed in 2003. In 2009, the U.S. Centers for Medicare and Medicaid Services announced that it would begin using ICD-10 on April 1, 2010, with full compliance by all involved parties by 2013. The years for which causes of death in the United States have been classified by each revision as follows: Cause of death on United States death certificates, statistically compiled by the Centers for Disease Control and Prevention (CDC), are coded in the ICD, which does not include codes for human and system factors commonly called medical errors. Mental and behavioral disorders The ICD includes a section classifying mental and behavioral disorders (Chapter V). This has developed alongside the American Psychiatric Association's Diagnostic and Statistical Manual of Mental Disorders (DSM) and the two manuals seek to use the same codes. There are significant differences, however, such as the ICD including personality disorders in the same way as other mental disorders. The WHO is revising their classifications in these sections as part the development of the ICD-11 (scheduled for 2017), and an "International Advisory Group" has been established to guide this. An international survey of psychiatrists in 66 countries comparing use of the ICD-10 and DSM-IV found that the former was more often used for clinical diagnosis while the latter was more valued for research. The ICD is actually the official system for the US, although many mental health professionals do not realize this due to the dominance of the DSM. A psychologist has stated: "Serious problems with the clinical utility of both the ICD and the DSM are widely acknowledged." - Clinical coder - Medical classifications - Medical diagnosis - Medical terminology - Current Procedural Terminology - MedDRA (Medical Dictionary for Regulatory Activities) - Systematized Nomenclature of Medicine Clinical Terms (SNOMED CT) - "International Classification of Diseases (ICD)". World Health Organization. Archived from the original on 12 February 2014. Retrieved 14 March 2014. - "About WHO". World Health Organization. Archived from the original on 9 February 2014. Retrieved 14 March 2014. - "The WHO Family of International Classifications". World Health Organization. Archived from the original on 22 December 2013. Retrieved 14 March 2014. - A beta-version of iCD-11 has been posted online. See "ICD-11 Beta Draft". World Health Organization. 13 March 2014. Retrieved 14 March 2014. - "The International Classification of Diseases 11th Revision is due by 2017". World Health Organization. Archived from the original on 21 February 2014. Retrieved 14 March 2014. - "ICD Revision Timelines". World Health Organization (WHO). World Health Organization (WHO). Retrieved 2015-09-18. - WHO. List of Official ICD-10 Updates. - Katsching, Heinz (February 2010). "Are psychiatrists an endangered species? Observations on internal and external challenges to the profession". World Psychiatry. World Psychiatric Association. 9 (1): 21–28. doi:10.1002/j.2051-5545.2010.tb00257.x. PMC . PMID 20148149. - ICD-10 Volume 2, online at http:// www.who.int/classifications - Scientific Data Documentation, International Classification of Diseases-9-CM - O'Malley KJ, Cook KF, Price MD, Wildes KR, Hurdle JF, Ashton CM (2005). "Measuring diagnoses: ICD code accuracy". Health Serv Res. 40: 1620–39. doi:10.1111/j.1475-6773.2005.00444.x. PMC . PMID 16178999. - World Health Organization. FAQ on ICD. Accessed 12 July 2011. - National Center for Health Statistics, CDC. ICD-9-CM Guidelines, Conversion Table, and Addenda. Classification of Diseases, Functioning, and Disability. Retrieved 2010-01-24. - InstaCode Institute. ICD-10 Demystified. - "ICD-9-CM Diagnosis Codes - International Classisfication of Diseases - Medical Diagnosis Codes". www.findacode.com. Retrieved 2015-12-30. - WHO. International Classification of Diseases (ICD). - CMS Office of Public Affairs (February 11, 2008). "HHS Proposes Adoption of ICD-10 Code Sets and Updated Electronic Transaction Standards" (Press release). U.S. Department of Health & Human Services. Retrieved 2009-02-11. - New Zealand Health Information Service. ICD-10-AM. Accessed 12 July 2011. - Canadian Institute for Health Information. ICD-10-CA. Accessed 12 July 2011. - International Classification Of Diseases - 9 - CM, (1979). Wonder.cdc.gov. Retrieved on 2014-06-20. - "Classification of Diseases, Functioning, and Disability". U.S. Centers for Disease Control. Retrieved 29 October 2010. - Initial WHO response to the report of the external review of the ICD-11 revision. WHO Department of Health Statistics and Information Systems (12 May 2015) Retrieved on 2015-10-26. - Makary, MA; Daniel, M (3 May 2016). "Medical error—the third leading cause of death in the US". BMJ: i2139. doi:10.1136/bmj.i2139. - Moriyama, IM; Loy, RM; Robb-Smith, AHT (2011). Rosenberg, HM; Hoyert, DL, eds. History of the Statistical Classification of Diseases and Causes of Death (PDF). Hyattsville, MD: U.S. Department of Health and Human Services, Centers for Disease Control and Prevention, National Center for Health Statistics. ISBN 978-0-8406-0644-0. - Who \| Mental Health Evidence And Research (Mer). Who.int. Retrieved on 2014-06-20. - Mezzich, Juan E. (2002). "International Surveys on the Use of ICD-10 and Related Diagnostic Systems" (guest editorial, abstract). Psychopathology. 35 (2–3): 72–75. doi:10.1159/000065122. PMID 12145487. Retrieved 2008-09-02. - Reed, Geoffrey M. (1 January 2010). "Toward ICD-11: Improving the clinical utility of WHO's International Classification of mental disorders" (PDF). Professional Psychology: Research and Practice. 41 (6): 457–464. doi:10.1037/a0021701. Note: since adoption of ICD-10 CM in the USA, several online tools have been mushrooming. They all refer to that particular modification and thus are not linked here.	3	26	1	0	0	0	0.629815	1	5,184
- Robinow syndrome Robinow syndrome Classification and external resources An infant exhibiting the facial features of Robinow syndrome. ICD-10 Q87.1 ICD-9 759.8 OMIM 180700 Robinow syndrome is an extremely rare genetic disorder characterized by short-limbed dwarfism, abnormalities in the head, face, and external genitalia, as well as vertebral segmentation. The disorder was first described in 1969 by human geneticist Meinhard Robinow, along with physicians Frederic N. Silverman and Hugo D. Smith, in the American Journal of Diseases of Children. By 2002, over 100 cases had been documented and introduced into medical literature. Two forms of the disorder exist, dominant and recessive, of which the former is more common. Patients with the dominant version often suffer moderately from the aforementioned symptoms. Recessive cases, on the other hand, are usually more physically marked, and individuals may exhibit more skeletal abnormalities. The recessive form is particularly frequent in Turkey. However, this can likely be explained by a common ancestor, as these patients' families can be traced to a single town in Eastern Turkey. Clusters of the autosomal recessive form have also been documented in Oman and Czechoslovakia. The syndrome is also known as Robinow-Silverman-Smith syndrome, Robinow dwarfism, fetal face, fetal face syndrome, fetal facies syndrome, acral dysostosis with facial and genital abnormalities, or mesomelic dwarfism-small genitalia syndrome. The recessive form was previously known as Covesdem syndrome. Signs and symptoms Robinow noted the resemblance of affected patients' faces to that of a fetus, using the term "fetal facies" to describe the appearance of a small face and widely spaced eyes. Clinical features also may include a short, upturned nose, a prominent forehead, and a flat nasal bridge. The upper lip may be "tented," exposing dental crowding, "tongue tie," or gum hypertrophy. Though the eyes do not protrude, abnormalities in the lower eyelid may give that impression. Surgery may be necessary if the eyes cannot close fully. In addition, the ears may be set low on the head or have a deformed pinna. Patients suffer from dwarfism, short lower arms, small feet, and small hands. Fingers and toes may also be abnormally short and laterally or medially bent. The thumb may be displaced and some patients, notably in Turkey, experience ectrodactyly. All patients often suffer from vertebral segmentation abnormalities. Those with the dominant variant have, at most, a single butterfly vertebra. Those with the recessive form, however, may suffer from hemivertebrae, vertebral fusion, and rib anomalies. Some cases resemble Jarcho-Levin syndrome or spondylocostal dysostosis. Genital defects characteristically seen in males include a micropenis with a normally developed scrotum and testes. Sometimes, testicles may be undescended, or the patient may suffer from hypospadias. Female genital defects may include a reduced size clitoris and underdeveloped labia minora. Infrequently, the labia majora may also be underdeveloped. Some research has shown that females may experience vaginal atresia or haematocolpos. The autosomal recessive form of the disorder tends to be much more severe. Examples of differences are summarized in the following table: Characteristic Autosomal dominant Autosomal recessive Stature More than 2 SD shorter Short or normal Arms Very short Slightly short Elbow Radial head dislocation No dislocation Upper lip very broad Tented Mortality rate Normal 10% Data on fertility and the development of secondary sex characteristics is relatively sparse. It has been reported that both male and female patients have had children. Males who have reproduced have all suffered from the autosomal dominant form of the disorder; the fertility of those with the recessive variant is unknown. Researchers have also reported abnormalities in the renal tract of affected patients. Hydronephrosis is a relatively common condition, and researchers have theorized that this may lead to urinary tract infections. In addition, a number of patients have suffered from cystic dysplasia of the kidney. A number of other conditions are often associated with Robinow syndrome. About 15% of reported patients suffer from congenital heart defects. Though there is no clear pattern, the most common conditions include pulmonary stenosis and atresia. In addition, though intelligence is generally normal, around 15% of patients show developmental problems. Genetic studies have linked the autosomal recessive form of the disorder to the ROR2 gene on position 9 of the long arm of chromosome 9. The gene is responsible for aspects of bone and cartilage growth. This same gene is involved in causing autosomal dominant brachydactyly B. The autosomal dominant form has not been linked to a specific gene, though those related to ROR2 are being studied. This form is often caused by new mutations. Alternatively, it may be passed from a parent who is so mildly affected by the disorder that he or she has not been diagnosed. A fetal ultrasound can offer prenatal diagnosis 19 weeks into pregnancy. However, the characteristics of a fetus suffering from the milder dominant form may not always be easy to differentiate from a more serious recessive case. Genetic counseling is an option given the availability of a family history. The disorder was first described in 1969 by human geneticist Meinhard Robinow, along with physicians Frederic N. Silverman and Hugo D. Smith, in the American Journal of Diseases of Children. By 2002, over 100 cases had been documented and introduced into medical literature. - ^ a b c d e f g h i j k l m n Patton M, Afzal A (2002). "Robinow syndrome.". Journal of Medical Genetics 39 (5): 305–310. doi:10.1136/jmg.39.5.305. PMC 1735132. PMID 12011143. http://jmg.bmjjournals.com/cgi/content/full/39/5/305. - ^ a b c d e f Robinow Syndrome Foundation. General Information. Accessed 19 May 2006. - ^ a b Balci S, Beksaç S, Haliloglu M, Ercis M, Eryilmaz M (1998). "Robinow syndrome, vaginal atresia, hematocolpos, and extra middle finger". American Journal of Medical Genetics 79 (1): 27–29. doi:10.1002/(SICI)1096-8628(19980827)79:1<27::AID-AJMG7>3.0.CO;2-F. PMID 9738864. - ^ van Bokhoven H, Celli J, Kayserili H, van Beusekom E, Balci S, Brussel W, Skovby F, Kerr B, Percin E, Akarsu N, Brunner H (2000). "Mutation of the gene encoding the ROR2 tyrosine kinase causes autosomal recessive Robinow syndrome.". Nature Genetics 25 (4): 423–426. doi:10.1038/78113. PMID 10932187. - ^ National Organization for Rare Disorders, Inc. Robinow Syndrome. Last modified 15 May 2006. Accessed 19 May 2006. - ^ Jablonski's Syndromes Database. Multiple Congenital Anomaly/Mental Retardation (MCA/MR) Syndromes. Accessed 20 May 2006. - ^ Robinow M (1993). "The Robinow (fetal face) syndrome: a continuing puzzle". Clin. Dysmorphol. 2 (3): 189–98. PMID 8287180. - ^ Shprintzen RJ, Goldberg RB, Saenger P, Sidoti EJ (1982). "Male-to-male transmission of Robinow's syndrome. Its occurrence in association with cleft lip and cleft palate". Am. J. Dis. Child. 136 (7): 594–7. PMID 7091086. - ^ Webber S, Wargowski D, Chitayat D, Sandor G (1990). "Congenital heart disease and Robinow syndrome: coincidence or an additional component of the syndrome?". Am J Med Genet 37 (4): 519–21. doi:10.1002/ajmg.1320370418. PMID 2260599. Congenital abnormality · multiple abnormalities (Q87, 759.7) Craniofacial Short stature Limbs Overgrowth Laurence-Moon-Bardet-BiedlBardet–Biedl syndrome · Laurence-Moon syndrome Combined/other, Genetic disorder, membrane: cell surface receptor deficiencies G protein-coupled receptor (including hormone)Class AClass BClass CCASR (Familial hypocalciuric hypercalcemia)Class FFZD4 (Familial exudative vitreoretinopathy 1) growth factor)ROR2 (Robinow syndrome) · FGFR1 (Pfeiffer syndrome, KAL2 Kallmann syndrome) · FGFR2 (Apert syndrome, Antley-Bixler syndrome, Pfeiffer syndrome, Crouzon syndrome, Jackson-Weiss syndrome) · FGFR3 (Achondroplasia, Hypochondroplasia, Thanatophoric dysplasia, Muenke syndrome) · INSR (Donohue syndrome · Rabson–Mendenhall syndrome) · NTRK1 (Congenital insensitivity to pain with anhidrosis) · KIT (KIT Piebaldism, Gastrointestinal stromal tumor) JAK-STAT TNF receptor Lipid receptor Other/ungroupedEDAR (EDAR Hypohidrotic ectodermal dysplasia) · PTCH1 (Nevoid basal cell carcinoma syndrome) · BMPR1A (BMPR1A Juvenile polyposis syndrome) · IL2RG (X-linked severe combined immunodeficiency) Wikimedia Foundation. 2010. Look at other dictionaries: Robinow syndrome — dwarfism associated with increased interorbital distance, malaligned teeth, bulging forehead, depressed nasal bridge, and short limbs. Called also Robinow dwarfism and fetal face s … Medical dictionary Robinow syndrome (dwarfism) — Rob·i·now syndrome (dwarfism) (robґĭ nou) [Meinhard Robinow, German born American physician, 1909â€“1997] see under syndrome … Medical dictionary Syndrome de char — Le syndrome de Char est l’association d’anomalies du cinquième doigt, d’une cardiopathie congénitale à type de persistance du canal artériel et d’un visage caractéristique. Sommaire 1 Autres noms de la maladie 2 Etiologie 3 Incidence … Wikipédia en Français Robinow-Silverman-Smith-Syndrom — Klassifikation nach ICD 10 Q87.1 Angeborene Fehlbildungssyndrome, die vorwiegend mit Kleinwuchs einhergehen Robinow (Silverman Smith )Syndrom … Deutsch Wikipedia Robinow-Syndrom — Klassifikation nach ICD 10 Q87.1 Angeborene Fehlbildungssyndrome, die vorwiegend mit Kleinwuchs einhergehen Robinow (Silverman Smith )Syndrom … Deutsch Wikipedia Robinow-Zwergwuchs — Klassifikation nach ICD 10 Q87.1 Angeborene Fehlbildungssyndrome, die vorwiegend mit Kleinwuchs einhergehen Robinow (Silverman Smith )Syndrom … Deutsch Wikipedia Syndrome de Char — Le syndrome de Char est l’association d’anomalies du cinquième doigt, d’une cardiopathie congénitale à type de persistance du canal artériel et d’un visage caractéristique. Sommaire 1 Autres noms de la maladie 2 Etiologie 3 Incidence … Wikipédia en Français Syndrome de Robinow (forme recessive) — Syndrome de Robinow (forme récessive) Le syndrome de Robinow est une ostéochondrodysplasie avec un faciès caractéristique et des anomalies des organes génitaux et urinaire. Cette pathologie est reconnue dès la naissance ou rapidement chez le… … Wikipédia en Français Syndrome de robinow (forme récessive) — Le syndrome de Robinow est une ostéochondrodysplasie avec un faciès caractéristique et des anomalies des organes génitaux et urinaire. Cette pathologie est reconnue dès la naissance ou rapidement chez le nourrisson. Sommaire 1 Autres noms 2… … Wikipédia en Français Robinow — Syndrome de Robinow (forme récessive) Le syndrome de Robinow est une ostéochondrodysplasie avec un faciès caractéristique et des anomalies des organes génitaux et urinaire. Cette pathologie est reconnue dès la naissance ou rapidement chez le… … Wikipédia en Français	2	13	1	0	0	5	0.608179	6	2,950
Basics Of Medical Billing Code Systems As a healthcare quality employee and certified Risk Adjustment coder (CRC), I know the basics of ICD-10 (International Statistical Classification of Disease and Related Health Problems) coding, CPT-II (Current Procedural Terminology) codes and HCPCS (Healthcare Common Procedures Coding System). The difference between CPT, CPT-II, CPT-III and HCPCS (which I have seen referred to as CPT-4) has eluded me. I am not 100 percent sure I have a clear understanding even now but here are the facts. I do know that accurate coding is essential to report claims and quality measures. ICD-10, CPT, and HCPCS codes identify: diagnosis or diagnoses certain devices, supplies, and equipment acquired for the client ICD-10 - International Statistical Classification of Diseases and Related Health Problems Implemented in October 2015, this revision and replacement of ICD-9 includes codes for diseases, symptoms, abnormal findings, conditions, circumstances and external causes of injury or diseases. ICD is the international standard for reporting diseases and health conditions including monitoring of diseases, observing reimbursement, strategizing resource allocation and keeping track of quality measures. ICD-10 is composed of two parts: ICD-10-CM (Clinical Modification) for diagnosis coding in all healthcare settings. Around 68,000 codes. ICD-10-PCS (Procedure Coding System) used only for coding hospital inpatient procedures. Around 76,000 codes. When most people talk about ICD-10, they are referring to ICD-10-CM. An example of an ICD-10 code is E11.21 – Type II Diabetes Mellitus with Diabetic Nephropathy. CPT - Current Procedural Terminology CPT codes are the codes used for reporting claims and getting paid. When a claim is filed with the CPT procedure code along with the appropriate ICD-10 diagnosis code, payment is made to the providing practitioner. Example: An insurance company won't pay just because the patient had a sore foot. CPT codes will let the payer know an office visit was performed, pain was assessed, medications were ordered, tests were ordered. The CPT system is maintained and copyrighted by the American Medical Association. Please refer to the AMA for specifics on these codes. There are three categories for CPT codes: CPT - used for reporting claims and getting paid. Example, reporting code 99201 will get you payment for an office visit. CPT II - used to track additional services attached to a health care visit. These codes are not payable but will help reduce the need for chart audits during HEDIS (Health Effectiveness Data and Information Set) season. Example, reporting 1170F will let the payer know that during the 99201 office visit above, a functional status assessment was also conducted. Unfortunately, since these codes are optional and non-money generating, they are often not reported. Category III - codes are not federally regulated and are fairly new to the healthcare industry. They are reported to help health facilities and government agencies track the efficacy of new, emergent medical techniques. Using Category III codes is an important part of keeping the medical community up to date, and supporting advancements in the medical community and healthcare technology. For more information on these, please refer to the American Medical Association.	4	3	3	0	0	2	0.340481	5	716
\|Classification and external resources\| Tolosa-Hunt syndrome describes episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but can relapse and remit. It was first described in 1954 by Tolosa. In 1961, Hunt reported 6 cases of painful ophthalmoplegia that rapidly resolved with steroids. Tolosa-Hunt syndrome was first classified by International Headache Society in 2004 and now is a part of Classification ICHD – II. ICD-10 for Tolosa Hunt Syndrome is G44.850 The diagnostic criteria for Tolosa-Hunt syndrome is as follows: - One or more episodes of unilateral orbital pain persisting for weeks if untreated. - Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of cavernous sinus granuloma by MRI or biopsy. - Paresis coincides with the onset of pain or follows it within 2 weeks. - Pain and paresis resolve within 72 hours when treated adequately with corticosteroids. - Other causes have been excluded by appropriate investigations (please see below for [[Differential Diagnosis\|differential diagnosis). Some reported cases of Tolosa-Hunt syndrome had additional involvement of the trigeminal nerve (commonly the first division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected. The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital fissure or orbit in some biopsied cases. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia. Tolosa-Hunt syndrome is idiopathic. A possible risk factor for Tolosa-Hunt syndrome is a recent viral infection. Tolosa-Hunt syndrome is idiopathic, sterile inflammation of the cavernous sinus. Its pathology is described as fibroblastic, lymphocytic, and plasmocytic infiltration of the cavernous sinus. Granulocytic and giant-cell infiltrations have also been described. Pathology may extend to involve the superior orbital fissure (sphenocavernous or parasellar syndrome) or orbital apex and affect the optic nerve. The patient may complain of double vision worse at distance, headaches, dizziness, nausea, neck stiffness, photophobia, blurred vision, and a “boring” pain may be associated with the headache. In addition to the standard ophthalmic examination of the patient including vision, IOP, pupil check for APD and nystagmus, slit-lamp and dilated fundus exam, a complete sensorimotor exam should be done. This includes oculomotor exam (to check for esotropia, exotropia, hypertropia or hypotropia), ductions, vergence, saccades, pursuit, and head tilt/turn. A common finding is abduction deficit associated with esodeviation that increases with gaze to the affected side. Lids should be checked for ptosis or lid retraction or any change in lid aperture during eye movements (to check for aberrant regeneration). Lid strength, fatigue or variability should be noted. Facial sensation should be checked. Stereopsis and color plates should also be evaluated. Involvement of multiple contiguous cranial nerves strongly suggest a lesion in the cavernous sinus or subarachnoid space. Only one nerve may be involved, most likely the sixth cranial nerve, which is the only one not protected within the dural wall of the cavernous sinus. In addition to the complete ophthalmic exam as described, the physician must closely look for Horner syndrome, facial hypoesthesia or engorgement of ocular surface vessels, orbital venous congestion, increased IOP or pulse pressure. All positive findings should be noted and the differential diagnoses listed below should be considered. The most appropriate imaging includes MRI /MRA (DWI series) which provides information about the cavernous sinus and orbital apex in greater detail than a CT. A CTA w/ and w/o contrast can also be obtained if an MRI/MRA is not available. A lumbar puncture is recommended to check for opening pressure and CSF should be evaluated for infection/ oligoclonal bands. Work up should include tests that can rule out the various diseases listed above given the history and context of the patient. This can include CBC w diff, RPR, FTA-ABS, ACE, ANA, p-ANCA, c-ANCA, Anti dsDNA, RF, myasthenia antibodies (binding/blocking/modulating antibodies and anti-MUSK antibodies), TFTs, and a fasting glucose. Tolosa-Hunt syndrome is considered a diagnosis of exclusion. Thus, the following entities must be considered and ruled out before a diagnosis of Tolosa-Hunt syndrome is made: - Ischemic disease: Hemorrhage, ischemic mononeuropathy - Infectious process: post viral syndrome, chronic inflammation of petrous bone (recurrent ear infections), syphilis, basal meningitis - Anatomical malformation: aneurysm, AVM, carotid-cavernous fistula, cavernous sinus thrombosis, pseudotumor cerebrii, Duane syndrome/Moebius syndrome, Chiari malformation - Inflammatory disease: Sarcoidosis, granulomatosis with polyangiitis (formerly Wegener's), Behcet’s disease - Autoimmune condition: myasthenia gravis, thyroid disease, lupus - Neoplastic disease: meningioma, neurogenic tumor, hemangioma, lymphoma/leukemia, schwannoma, pituitary adenoma, metastasis, CPA lesion, nasopharyngeal carcinoma, chordoma, chondrosarcoma, brain stem glioma in children - Demyelinating disease: MS - Others: Diabetes mellitus, Head trauma, BBPV, Meniere’s, ophthalmoplegic migraine Oral steroids are the mainstay of treatment. Both symptoms and physical exam findings (headache, ptosis, ophthalmoplegia, etc) can be expected to resolve rapidly with an oral steroid taper regimen over 3-4 months. The patient can be co-managed with the Neurology service to rule out other entities listed in the differential diagnosis of Tolosa-Hunt syndrome. Before starting steroid, fungal infection of the orbit with fungal sinusitis (mucormycosis in diabetic/immunocompromised) must be ruled out as in that case steroid will worsen the disease. A rare complication is aberrant regeneration of cranial nerves. Prognosis of Tolosa-Hunt syndrome is excellent. Full recovery is expected with steroid treatment. The disease may have a relapsing-remitting course. - Albert et al. Principles and Practice of Ophthalmology. Third Edition. © 2008. - Aligluo Z et al. Tolosa-Hunt syndrome: A case report. Journal of Neuroradiology. 1999. 26 (1): 68. - BCSC series. Neuro-Ophthalmology. Section 5. American Academy of Ophthalmology. - Dach F et al. Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria. Cephalalgia an international journal of headache. 2007. 27 (8): 960-961. - Duane’s Ophthalmology. Lipincott Williams & Wilkins. CD Rom 2006 Edition. www.oculist.net - Mendez et al. Painful ophthalmoplegia of the left eye in a 19-year-old female, with an emphasis in Tolosa-Hunt syndrome: a case report. Cases J. 2009; 2: 8271. - Takahashi Y et al. Tolosa-Hunt Syndrome with Atypical Intrasellar and Juxtasellar Lesions:Two Case Reports. The Kurume Medical Journal. 1996. 43: 165-174.	2	6	0	0	0	2	0.569151	2	1,749
Seasonal Affective Disorder Part 2- Turning in your final treatment plan and Analysis The final treatment plan will include the primary diagnosis, diagnostic testing recommended by National Guidelines. Medications, interventions, education, labs, follow up, referrals. After completing the treatment plan include the following sections in a large area called ANALYSIS: 1. Pathophysiology and Pharmacology: For the primary diagnoses in the case, write a brief summary of the underlying pathophysiology and tie pharmacological treatment chosen in the reversal or control of that pathology. 2. Additional analysis of the case: This includes national guidelines that were or should have been used to make diagnosis or treatment and review how they applied or how care was unique but based in guidelines. 3. Follow-up/Referrals: This means how the patient was doing when seen a second time if this applies. This would be their response to your plan of care. OR when Follow up will occur and what actions will be taken on the follow up visit. Referrals if indicated. 4. Quality: Include anything that should have been considered in hindsight or changes you would make in seeing similar patients in the future with the same complaint, history, exam, or diagnosis. Add anything you learned from discussion in the class that shed new light on this patient. 5. Coding and Billing. Any or all CPT and ICD-10 codes that should have been used (List them and name them only. Mental illness goes undiagnosed in so many individuals living in the United States. Studies have shown it to affect approximately one in every five (19 percent) of adults. The American Psychiatric Association (APA) (2019) defines mental illnesses as health conditions affecting deviations in emotion, thinking or behavior (or a combination of these). These illnesses are also coupled with distress and/or difficulties acting in social, work or family events. George is a 27-year-old, single, Caucasian male who came to the clinic seeking medication for his fatigue, lack of energy, reduced sex drive and withdrawn behavior. Which he states this feeling happens to him all the time, around this time of year(winter) for the past 2 years. G.A. 27-year-old Caucasian male CC (chief complaint): fatigue, lack of energy, reduced sex drive, and withdrawn behavior HPI: G.A. is a 27-year-old, single, Caucasian male who came to the clinic seeking medication that will help with his fatigue, lack of energy, reduced sex drive, and withdrawn behavior. He stated that his symptoms had been harsh this year around wintertime and is now wanting to seek help because it has affected his personal life. He reported that he started feeling this way 2 years ago, and is becoming more withdrawn from the things that he normally enjoys doing. He denied seeking help because he was afraid of being labeled. Current Medications: None. PMHx: Immunizations are current. Reports having no medical problems. Social Hx: Single Caucasian male. G.A. is an only child. He lives with his divorced mother. He denies drinking, smoking and illegal drug use. Fam Hx: Father was an alcoholic and is now deceased from a motor vehicle accident 5 years ago. Mother 65 is alive and suffers from depression. No other pertinent family hx reported. Constitutional: States he lost 10lbs in the last month. Negative for chills, fever, weakness, and night sweats HEENT: Eyes: No visual loss, PERRLA. Ears, Nose, Throat: No hearing loss, nasal congestion, or sore throat. Skin: Normal temperature, tone, texture, turgor and no rash or itching. Cardiovascular: No chest pain, discomfort, and palpitations. No edema. Respiratory: No shortness of breath. No cough or sputum. Gastrointestinal: No nausea, vomiting, diarrhea, or abdominal pain. Abdomen soft, non-tender, non-distended, bowel sounds active in all four quadrants. Genitourinary: No sign of dysuria or polyuria and voiding freely Neurological: No headache, dizziness, or syncope and numbness or tingling. Musculoskeletal: No back pain, muscle, and joint pain or stiffness. Hematologic: Negative for bleeding or bruising. Lymphatics: Negative for enlarged nodes. Psychiatric: Family hx of depression (mother) Endocrinologic: No polyuria or polydipsia. Vital signs: BP 110/60, Pulse 100, Temp 36.1 C, Resp 20, Height 6′ 1″, Weight 170lb. General: Flat affect, no eye contact, patient looks at floor while speaking. Speaks in a low tone HEENT: Head normocephalic and atraumatic. Hair evenly distributed throughout the scalp. Eyes: Sclera clear. Conjunctiva: white, PERRLA, EOMs intact bilateral. Ears: Tympanic membranes gray and intact and no discharge or erythema. Nose: Nares normal, septum midline, mucosal pink and moist and no drainage or sinus tenderness. Throat: Lips, mucosa and tongue normal, no lesions or exudate. Neck. Supple, trachea midline, no tenderness, no cervical lymphadenopathy or nodules. No carotid bruit or JVD. Thyroid midline: small and firm without palpable masses. Lungs: Lungs clear to auscultation bilaterally. Respirations even and unlabored. Cardiovascular: S1 and S2 normal, no murmur, rub or gallop. RRR, no displaced PMI. Peripheral pulses equal bilaterally, no peripheral edema. Abdomen: Soft, non-tender, non-distended, bowel sounds present in all four quadrants. No hernias and no masses. No organomegaly. Musculoskeletal: Moves all extremities, good ROM, no edema Skin: Warm to touch, normal tone, texture, turgor, no induration, no rash and no lesions. Neurologic: Negative for any deficits Psychiatric: Flat affect. American Psychiatric Association. (2019). What is mental illness? Retrieved from https://www. psychiatry.org/patients-families/what-is-mental-illness D.A. 32-year-old Caucasian male CC: fearful of being around loud noises, terrible anxiety, memories of an explosion and nightmares. HPI: D.A. is a 32-year-old, single, Caucasian male who came to the clinic seeking medication that will help with panic attacks, feeling fearful of being around loud noises, terrible anxiety, memories of an explosion and nightmares. He stated that his symptoms were harsh and that they started about one year after being around an explosion that took the lives of five men and women while he was in Baghdad two years prior. He reported serving two tours in the military. He reported that he started feeling this way a year now when he went out in public and heard bang noises from an old car muffler. He reported that any banging would trigger his feelings, and the symptoms would ease if he meditates and or take slow deep breaths. He reported the episode would last for twenty-four hours, and he reports becoming more withdrawn than usual. He denied seeking help because he was afraid of being labeled. Diagnosis: Post traumatic stress disorder, also called, PTSD. (F43.1) One must understand that PTSD is a type of anxiety disorder that arises after exposure to a distressing incident such as threat of death, serious physical injury to and threat to physical well-being (Domino, Baldor, Golding, & Stephens, 2019). Individuals affected must have a comprehensive management plan which are based on clinical practice guidelines, clinical judgment, patient preferences and the patient’s response to psychotherapy or psychopharmacology are completely vital factors in selecting the course of action or treatment for PTSD. The clinical practice guidelines that should have been applied to D.A. are psychotherapies such as cognitive processing therapy (CPT) and eye movement desensitization and reprocessing (EMDR) (American Psychological Association, 2017) which is a newer nontraditional way of therapy along with medications such as Sertraline (Zoloft) or Paroxetine (Paxil) which are the only approved medications by the Food and Drug Administration (FDA) for PTSD (American Psychological Association, 2019). The lessons learned from this discussion were the importance of thoroughly assessing one’s patients, being very attentive to details, listen to your patients and the need for making referrals beyond the scope of practice. Final Treatment Plan/Analysis: The research has shown that the pathophysiology of PTSD is unsure, however the evidence is suggestive of underlying methods include activation of the part of the brain that’s immersed in fear, volume loss of the hippocampus that’s involved in formation of memory (Lisieski, Eagle, Conti, Liberzon, & Perrine, 2018) therefore creating a need for medications such as SSRIs. These are known to play an active part in regulating mood and anxiety disorders. The action of this neurotransmitter in both the peripheral and central nervous systems can be controlled by SSRIs (APA, 2019). Paroxetine (Paxil) 20 mg daily Take one tablet by mouth once daily Educate the patient on the possibility of side effects from the medication. It will take some weeks before the medication starts to work, and therefore taking the medication as prescribed is important. Psychiatrist and Psychologist and PCP in 2 to 4 weeks to reassess medication effect Coding and Billing: ICD-10 code is F43.1- Post-traumatic stress disorder (PTSD) American Psychological Association. (2017). Clinical practice guideline for the treatment of posttraumatic stress disorder (PTSD) in adults. Guideline development panel for the treatment of PTSD in adults. Retrieved from https://www.apa.org/ptsd-guideline/ptsd.pdf American Psychological Association. (2019). Clinical practice guideline for the treatment of post traumatic stress disorder: medications for PTSD. Retrieved from https://www.apa.org/ptsd-guideline/treatments/medications Domino, F.J., Baldor, R.A., Golding, J., & Stephens, M.B. (2019). The 5-minute clinical consult 2019 (27th ed.). Philadelphia, PA: Wolters Kluwer Health/Lippincott Williams & Wilkins. Lisieski, M. J., Eagle, A. L., Conti, A. C., Liberzon, I., & Perrine, S. A. (2018). Single-prolonged stress: A review of two decades of progress in a rodent model of post-traumatic stress disorder. Frontier in Psychiatry. Doi.org/10.3389/fpsyt.2018.00196	2	5	1	0	0	3	0.933759	4	2,360
I'm a member You will be redirected to myBlue. Would you like to continue? Please wait while you are redirected. Please enter a username and password. Printer Friendly Version Several techniques have been developed to measure the thickness of the optic nerve/retinal nerve fiber layer (RNFL) as a method to diagnose and monitor glaucoma. Measurement of ocular blood flow is also being evaluated as a diagnostic and management tool for glaucoma. Glaucoma is a disease characterized by degeneration of the optic nerve (optic disc). Elevated intraocular pressure has long been thought to be the primary etiology, but the relationship between intraocular pressure and optic nerve damage varies among patients, suggesting a multifactorial origin. For example, some patients with clearly elevated intraocular pressure will show no damage to the optic nerve, while other patients with marginal or no pressure elevation will nonetheless show optic nerve damage. The association between glaucoma and other vascular disorders, such as diabetes or hypertension, suggests vascular factors may play a role in glaucoma. Specifically, it has been hypothesized that reductions in blood flow to the optic nerve may contribute to the visual field defects associated with glaucoma. A comprehensive ophthalmologic exam is required for the diagnosis of glaucoma, but no single test is adequate for establishing the diagnosis. A comprehensive ophthalmologic examination includes an examination of the optic nerve by fundoscopy, evaluation of visual fields, and measurement of ocular pressure. The presence of characteristic changes in the optic nerve or abnormalities in visual field, together with increased IOP, is sufficient for a definitive diagnosis. However, some patients will show ophthalmologic evidence of glaucoma with normal IOPs, therefore an elevated IOP is not essential for diagnosis. Conventional management of the patient with glaucoma involves drug therapy to control elevated intraocular pressures and serial evaluation of the optic nerve to follow disease progression. Standard methods of evaluation include careful direct examination of the optic nerve using the ophthalmoscopy or stereophotography, or evaluation of visual fields. There is interest in developing more objective, reproducible techniques both to document optic nerve damage and to detect early changes in the optic nerve and retinal nerve fiber layer (RNFL) before the development of permanent visual field deficits. Specifically, evaluating changes in the thickness of the retinal nerve fiber layer has been investigated as a technique to diagnose and monitor glaucoma. In addition, there is interest in measuring ocular blood flow as a diagnostic and management tool for glaucoma. A variety of new techniques have been developed, as described below: 1. Techniques to Evaluate the Optic Nerve/Retinal Nerve Fiber Layer (Note: This policy only addresses uses of these techniques related to glaucoma.) A. Confocal Scanning Laser Ophthalmoscopy Confocal scanning laser ophthalmoscopy (CSLO) is a laser-based image acquisition technique, which is intended to improve the quality of the examination compared to standard ophthalmologic examination. A laser is scanned across the retina along with a detector system. Only a single spot on the retina is illuminated at any time, resulting in a high-contrast image of great reproducibility that can be used to estimate the thickness of the RNFL. In addition, this technique does not require maximal mydriasis, which may be a problem in patients with glaucoma. The Heidelberg Retinal Tomography is probably the most common example of this technology. B. Scanning Laser Polarimetry The RNFL is birefringent, causing a change in the state of polarization of a laser beam as it passes. A 780-nm diode laser is used to illuminate the optic nerve. The polarization state of the light emerging from the eye is then evaluated and correlated with RNFL thickness. Unlike CSLO, scanning laser polarimetry (SLP) can directly measure the thickness of the RNFL. GDx® is a common example of a scanning laser polarimeter. GDx® contains a normative database and statistical software package to allow comparison to age-matched normal subjects of the same ethnic origin. The advantages of this system are that images can be obtained without pupil dilation, and evaluation can be done in about 10 minutes. Current instruments have added enhanced and variable corneal compensation technology to account for corneal polarization. C. Optical Coherence Tomography Optical coherence tomography (OCT) uses near-infrared light to provide direct cross-sectional measurement of the RNFL. The principles employed are similar to those used in B-mode ultrasound except light, not sound, is used to produce the 2-dimensional images. The light source can be directed into the eye through a conventional slit-lamp biomicroscope and focused onto the retina through a typical 78-diopter lens. This system requires dilation of the patient’s pupil. OCT® is an example of this technology. 2. Pulsatile Ocular Blood Flow The pulsatile variation in ocular pressure results from the flow of blood into the eye during cardiac systole. Pulsatile ocular blood flow can thus be detected by the continuous monitoring of intraocular pressure. The detected pressure pulse can then be converted into a volume measurement using the known relationship between ocular pressure and ocular volume. Pulsatile blood flow is primarily determined by the choroidal vessels, particularly relevant to patients with glaucoma, since the optic nerve is supplied in large part by the choroidal circulation. 3. Doppler Ultrasonography Color Doppler imaging has also been investigated as a technique to measure the blood velocity in the retinal and choroidal arteries. In 2012, The iExaminer™ (Welch Allyn) received marketing clearance from the U.S. Food and Drug Administration (FDA). The iExaminer consists of a hardware adapter and associated software (iPhone® App) to capture, store, send and retrieve images from the Welch Allyn PanOptic™ Ophthalmoscope using an iPhone®. POLICYAnalysis of the optic nerve (retinal nerve fiber layer) in the diagnosis and evaluation of patients with glaucoma or glaucoma suspects may be considered medically necessary when using scanning laser ophthalmoscopy, scanning laser polarimetry, and optical coherence tomography. The measurement of ocular blood flow, pulsatile ocular blood flow or blood flow velocity with Doppler ultrasonography is considered investigational in the diagnosis and follow-up of patients with glaucoma. POLICY EXCEPTIONSFederal Employee Program (FEP) may dictate that all FDA-approved devices, drugs or biologics may not be considered investigational and thus these devices may be assessed only on the basis of their medical necessity. The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language. Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Nervous/Mental Conditions, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D: A. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and B. appropriate with regard to standards of good medical practice; and C. not solely for the convenience of the Member, his or her Provider; and D. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient. For the definition of Medically Necessary, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary. Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting. POLICY HISTORY5/1998: Approved by Medical Policy Advisory Committee (MPAC) 4/12/2001: Managed Care Requirements deleted 5/2001: Reviewed by MPAC; investigational status remains 2/7/2002: Investigational definition added 5/2/2002: Type of Service and Place of Service deleted 5/29/2002: Code Reference section completed 11/2002: Reviewed by MPAC; Scanning Laser Polarimetry (SLP) changed to medically necessary 7/2003: Reviewed by MPAC; "Description" section revised to be consistent with BCBSA, scanning laser ophthalmoscopy and optical coherence tomography are medically necessary for high risk individuals, scanning laser ophthalmoscopy, optical coherence tomography and scanning laser polarimetry are considered investigational as a method of monitoring disease progression in patients with glaucoma and as a screening test for glaucoma in the general population, measurement of pulsatile ocular blood flow or blood flow velocity with doppler ultrasonography is considered investigational in the diagnosis and follow-up of patients with glaucoma, FEP exception added 11/1/2004: Code Reference section updated, CPT code 92135 moved to covered, ICD-9 procedure code 88.90 added covered codes, ICD-9 diagnosis code 250.00, 250.01, 250.02, 250.03, 250.10, 250.11, 250.12, 250.13, 250.20, 250.21, 250.22, 250.23, 250.30, 250.31, 250.32, 250.33, 250.40, 250.41, 250.42, 250.43, 250.50, 250.51, 250.52, 250.53, 250.60, 250.61, 250.62, 250.63, 250.70, 250.71, 250.72, 250.73, 250.80, 250.81, 250.82, 250.83, 250.90, 250.91, 250.92, 250.93, 360.21, 362.85, V19.0 added covered codes, ICD-9 diagnosis 365.00, 365.01, 365.02, 365.03, 365.04, 365.10, 365.11, 365.12, 365.13, 365.14, 365.15, 365.20, 365.21, 365.22, 365.23, 365.24, 365.31, 365.32, 365.41, 365.42, 365.43, 365.44, 365.51, 365.52, 365.59, 365.60, 365.61, 365.62, 365.63, 365.64, 365.65, 365.81, 365.82, 365.83, 365.89, 365.9 description revised and moved from non-covered to covered, CPT code 93875 added to non-covered codes 1/10/2005: Code Reference section updated, ICD-9 diagnosis code 362.01, 362.02, 368.40, 368.41, 368.42, 368.43, 368.44, 368.45, 368.46, 368.47 added covered codes, HCPCS S0820 deleted 11/16/2005: Code Reference section updated, ICD9 diagnosis codes 362.03 - 362.07 added 3/17/2006: Policy reviewed, no changes 12/19/2007: Coding updated per 2008 CPT/HCPCS revisions 1/18/2008: Policy reviewed, no changes 9/16/2008: Annual ICD-9 updates effective 10-1-2008 applied 12/31/2008: Code reference section updated per 2009 CPT/HCPCS revisions 08/03/2010: Policy Description revised to remove optic nerve head analyzers. Policy statement revised to remove optic nerve head analyzers and analysis of the optic nerve (retinal nerve fiber layer) in the diagnosis and evaluation of patients with glaucoma or glaucoma suspects may be considered medically necessary when using scanning laser ophthalmoscopy, scanning laser polarimetry, and optical coherence tomography. FEP verbiage revised in Policy Exceptions section. Code Reference section revised to add ICD-9 diagnosis codes to the Covered Codes Table: 115.02, 190.5, 190.6, 224.5, 224.6, 228.03, 360.11, 361.00 - 361.9, 362.00 - 362.9, 363.00 - 363.9, 364.21 - 364.24 and 368.15. A note was revised for CPT Code 33875 in the Non-Covered Codes Table. 03/07/2011: Policy statement updated to add ocular blood flow as investigational. Added new CPT codes 92132, 92133, and 92134 to the Code Reference section. 03/02/2012: Policy reviewed. Deleted outdated references from Sources section. 04/17/2013: Policy reviewed; no changes to policy statement. Added ICD-9 procedure code 38.25 to the Code Reference section and deleted 88.90 from the Code Reference section. 03/18/2014: Policy reviewed; description updated regarding FDA-approved device. Policy statement unchanged. 03/11/2015: Policy description updated with information regarding the comprehensive ophthalmologic exam. Policy statements unchanged. 08/31/2015: Code Reference section updated for ICD-10. Removed deleted CPT codes 0187T and 93875. 05/27/2016: Policy number added. Policy Guidelines updated to add medically necessary and investigative definitions. SOURCE(S)Blue Cross Blue Shield Association policy #9.03.06 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	8	8	0	2	2	0.735333	12	3,265
Flashcards in Second Part Of Ex 1 Deck (231): What is portal venous congestion leading to portal HTN? What is the order of primary salivary gland pathologies. ? 1. Parotid 2. Sublingual 3. Submandibular. Minor salivary glands ______% of leukoplakia can turn to what? 25, squamous cell carcinoma Where is cholera most common? Pseudopolyps are associated with what? What kind of hiatal hernia is separate portion of the stomach protrudes, and is prone to strangulation or obstruction ? Q Non axial ( rolling ) What is tenesmus and what condition is it associated with ? Sensation of inadequate BM, sigmoid diverticulitis Esophageal varices is secondary to what? What is twisting a loop of bowel ? External hemorrhoids =? Below the anorectal line If an acquired diverticulum in the Colon becomes infected what can happen? Perforation leading to hemorrhage What is excreted via salivary glands? >99% h20, IgA, enzymes What is multiple small shallow ulceration of the stomach/duodenum ? Acute peptic ulceration What makes up the majorly of all parotid gland tumors? When antibiotics disrupt intestinal flora, this allows what to happen? Clostridium diff to overgrow If herpes spreads to the brain what is it called? (Life threatening) Herpesviral encephalitis (usually HSV-1) Superficial inflammation =? What are symptoms of GERD? Dysphasia, heartburn, "sour brash" Where is the most common site of tumors in the small intestine? What kind of polyp makes up the majorly of gastric polyps.? Inflammatory and hyper plastic polyps (75%) How many children die each ear from infectious enterocolitis 12,000 ( 500 infants a day) What are the 3 top malabsorption problems in the US? Pancreatic insufficiency, celiac disease, crohn disease Do smaller or larger glands have higher cancer risk ? Smaller (but are less common) Oral cancer is about _____more common among males? Relapsing "attacks" with grossly bloody and mucoid stool is associated with what? What a treatment for sigmoid diverticulitis? Increase fiber, lifelong dietary modification What percent of cholera is lethal and in what amount of time? 70% in 24 hours What is a raised lesion that is firm with irregular boarders, that look whitish-grey or erythematous? Oral squamous cell carcinoma What is the most common location for angiodysplasia? Cecum, but also in ascending colon Environmental enteropathy is cycles of what? Mucosal injury, malnutrition, and inflammation Acute peptic ulceration is a complication of what 3 things? Severe physiological stress, high NSAIDS, inter-cranial disease. What constitutes diagnosis of travelers diarrhea? Three or more unformed loose stools in 24 hours What are other names for a canker sore ? Aphthous ulcer, recurrent aphthous stomatitis What is the actual problem causing barrett esophagus ? The lower esophageal sphincter doesn't close all the way. How common is pyloric stenosis ? What is lower pharyngeal outpouching superior to the UES? Are peptic ulcers more common in men or women ? Men !0, woman 4 Genital herpes is called what? What kind of gastric polyp makes up 15% of them? What kind of hiatal hernia is most common and is a "bell shaped" dilation ? What are risks for squamous cell carcinoma (oral) ? Alcohol, tobacco, >30, HPV-16 What part of the esophagus is where squamous cell carcinomas would be found? What do you see with the crypts with campylobacter? What percent of colon cancers are lethal? Villus flattening is a characteristic of what? What is complete rupture at the thoracic esophagus? Hammans sign? What is esophageal metaplasia at the distal esophagus? Where strat squamous Turing into columnar? What causes oral candidiasis? (Thrush) What is an early sign of appendicitis? What is a vascular lesion with tortuous and dilated submucosal and mucosal vessels? Are most hiatal hernias symptomatic ? What disease is when chronic gastritis leads to atrophy with antibodies against parietal cells? Autoimmune gastritis, pernicious anemia What are complications of barrett esophagus ? Esophageal adenocarcinoma, ulceration If someone has lost a lot of weight, has abdominal dissension, borborygmus, flatulence, anorexia? What kind of ulcer gets worse after eating? What is when a segment of intestine telescopes into a distal segment? Who is at risk for barrett esophagus ? White ppl (30-300) males (4x) 40-60 Campylobacter enterocolitis may initiate what? Reactive arthritis (HLA-B27) How do you diagnose adenomas? Screen, sessile or pedunculated, polyectomy In what condition do 100% of patients develop colon cancer, most commonly before 30? Familial adenomatous polyposis Giardiasis (never fever) = ? What kind of mineral deficiency is associated with celiac disease? How do you diagnose zenkers diverticulum? Barium swallow and video fluoroscopy Eating a highly processed diet increased your risk of colorectal adenocarcinoma ? What kind of esophagitis is most common after an ulcer and debilitated? What sign is achalasia associated with? Bird beak sign Flagellated protozoa is associated with what? What is the genetic mutation for someone with familial adenomatous polyposis ? APC gene on chromosome 5 What is the treatment for crohns disease? Probiotics, and immunosuppressive meds Chronic and relapsing abdominal pain, bloating, constipation and diarrhea are symptoms of what? Colorectal adenocarcinomas most commonly metastasize to where? How common is crohns disease? What is the cause of the ischemia in ischemic bowel disease? From hypotension or occlusion, mucosal infarction What kind of Esophageal lesion is associated with ectopic gastric mucosa, upper 1/3. Asymptomatic. Parasitic diseases affect how many people? >1/2 the worlds pop What causes viral gastroenteritis in children ? What is the most common cause of esophagitis ? What is the most common outpatient G.I. Complaint? What % of parotid gland tumors are malignant ? What kind of Esophageal lesion is associated with atresia, tracheal fistula, Stenosis, regurgitation? What is celiac disease? Immune mediated, reaction to gliadin IBD is most commonly seen in what type of control? Females, adolescence, white. Tumors of the small intestine account for what percent of all G.I. Malignancies ? Are adenomas age related? What kind of parotid gland tumor is more invasive, less mobile and malignant? Carcinoma Ex Pleomophic adenoma What is the most common malignancy of the G.I. Tract? What are symptoms of acute ischemic bowel disease? Sudden/severe abdominal pain, nausea. Vomiting, frank blood in stool Where do most intestinal obstructions occur? What is the cause of lynch syndrome? Inherited mutations in DNA mismatch repair system What deficiency is associated with colorectal adenocarcinoma ? Iron deficiency anemia What is the long term survival rate for someone with squamous cell carcinoma? <50% long term What are risks for chronic gastritis ? Heliobacter pylori infection, old age Skip lesions are seen in what condition? What are symptoms of dermatitis herpetiformis ? Blister on arms, legs, butt, itchy, seen in 10% of celiac disease patients What do Crohn's disease and ulcerative colitis have in common? IBD, Familial, females, risk of adenocarcinomas What is a benign smooth muscle tumor of the esophagus ? What parts are injured in viral gastroenteritis? To superficial cells of the stomach or SI What are the most common types of tumors of the small intestine? 50% adenocarcinoma, 50% carcinoid tumors What does ulcerative colitis? Canker sores are most common in what kind of people? Females, over 20, Celiac disase, IBD, Behcet disease Crohn disease effects what ? Entire GI tract, Most commonly in the ileum What mutation is common for oral cancer? Oralfacial herpes is called what? What are treatments for ischemic bowel disease? What condition is associated with a wall defect that leads to a blind need pouch and there are most commonly multiple ? What type of gastritis is transient mucosal inflammation (neutrophils), with possible erosion/ulceration ? If the appendix Rutgers there is what? High morbidity and mortality. When does zenkers diverticulum develop and what are sympotoms? After an increase in pharyngeal pressure. Halitosis, dysphasia, regurgitation What has a high (50%) risk of transitioning to CA has irregular boarder and is a red velvety area? What causes 10-20% initial HSV infections ? Acute herpetic gingivostomatitis What kind of Esophageal lesion is secondary to portal hypertension ? Are there granulomas or skip lesions seen in Ulcerative colitis? Is there inflammation associated with IBS? What are some treatments for canker sores? NSAIDS, B12 (cobalamin) What is the defect that causes hirschsprung disease? Neural crest cell migration What is treatment for IBS? Fiber, decrease carbs What does herpes virus lay dormant? What condition is a hemangioma on gingiva, caused by hormonal factors and or irritation that grows rapidly ? What % of he us have appendicitis? What is the most common acute abdominal condition ? What is the location of hemorrhoids ? Anal and perianal vessels Where are common locations for peptic ulcer? Prox duodenum (4x), gastric Antrim Co-morbidity impairs esophageal functioning. Inflammation near Auerbach plexus is what? What do you see with e coli crypt architecture What is the Most common esophageal cancer worldwide? Squamous cell carcinoma Are colorectal adenocarcinomas asymptomatic early? What makes up 90% of all stomach cancers? What is a raised white patch that cant be scraped off? With malabsorption what is defective? Fats,carbs, proteins, vit A D E K, mineral, electrolytes What percent of herpes simplex virus is asymptomatic? What percent of people over 70 have xerostomia? What is the most common type of oral cancer? Squamous cell carcinoma What are shared feathers between duodenal and gastric ulcers? Eating influences pain, relieved by vomiting or alkaline substance, worse at night What's is another name for sigmoid diverticulitis ? What are 2 treatments for achalasia Botulinum toxin, pneumatic dilation What percent of sublingual or minor salivary gland tumors are malignant? How common is Ulcerative colitis? 1 per 5,000 What causes viral gastroenteritis in adults? What is the most common cause of bacterial sialadenitis? What kind of esophageal laceration is a longitudinal tear from forceful vomiting? Mallory weiss tear 90% of all cirrhosis cases have what? What type of esophagitis is acute, self limiting, and due to tobacco, alcohol, pill induce? If you have perianal citing with bright red bloody (frank) stool, most are self limiting which is what? What are risks for gastric adenocarcinoma ?? H pylori, japan(20x) What are symptoms of chronic ischemic bowel disease?? Episodic bloody diarrhea, mimics IBD What is a blocked or rupture of a duct, found on the lower lip in the young and old? Mucocele (mucous cyst, Ranula) Who is most commonly effected by tropical sprue? Children 2-3 years old What condition is an hereditary cancer syndrome? What is the most common tumor of the appendix? Carcinoid, (rarely adenocarcinoma ) Hamartomas = what? Are adenomas considered malignant until proven otherwise? Is fibrosis seen in crohns or ulcerative colitis ? What are dietary risk factors for an adenocarcinoma ? Low veggie fiber, high amounts of carbs, high animal fat, decreased antioxidants. What kind of achalasia is most common? In HPV (16) associated squamous cell carcinoma where are the most common sites? Base of tongue, tonsillar crypts Who is at risk for celiac disease ? White people, 30-60 What condition is associated with barrett esophagus ? What is associated with projectile vomiting and the fluid is not bile stained. ? What makes up 20% of lower intestinal bleeds? What is a nodular mass, following chronic irritation also has Hyperplasia and fibrosis and is most common along the bite line? What is a solitary punched out lesion in the stomach ? Peptic ulcer disease Where does U.C. Always being ? What are the most common (3) locations of Crohn's disease ? Terminal ileum, ileocecal valve Who is at risk forr a squamous cell carcinoma in the esophagus ? 45^, males, African americans If caught early what's the 5 ear survival rate for gastric adenocarcinoma ? What causes cholera ? Vinbro cholera (gram -) Is crohns disease or ulcerative colitis malabsorptive ? What kind of pain is assocaited with peptic ulcers and when? Gnawing, burning, boring, at night 1-3 hours after eating When celiac disase manifests on the skin what is it called? What is inflammation of the gastric mucosa ? What kind of gastritis is less severe but prolonged? What relieves peptic ulcers? What arteries are associated with ischemic bowl disease? (Ischemic colitis) Superior and inferior mesenteric arteries, and celiac artery What are characteristics of hemorrhoid? Thin walled varices, swollen and tortuous What is responsible for 50% of esophageal cancers in the us? What causes celiac disease? B and T cells damage S.I. Causing villous atrophy What is the most common form of viral Sialadenitis? Mumps virus infection (pediatrics = self limiting. Adults, pancreatitis and orchitis) How does one diagnose lactose intolerance? Breath hydrogen test What is a major risk for adenomas and how much higher is the risk? Family history, 4x increased risk What is secondary to injury to esophageal mucosa? What syndrome is associated with salivary gland problems? Canker sores effect what percent of the population ??? Where is the problem with lactose intolerance ? S.I. Brush border What condition is associated with grey/white pseudomembranes that can be scraped off? What are symptoms of sigmoid diverticulitis ? Cramping, LLQ pain What is lost in celiac Disease? What is characterized by dry mouth, due to decreased saliva production? What is known as a pregnancy tumor? The ______the size of a adenoma the_______chance of cancer. What is it called when inflammation leads to fibrosis that creates adherent intestinal segments? What are signs of an acute appendicitis ? Anorexia, nausea, vomiting The cholera toxin opens what? CFTR which = a massive chloride ion secretion What is a major risk of esophageal varices? Hemorrhage. 50% of first time bleeds are lethal What is the triad for achalasia? Incomplete LES relaxation, increased LES tone, esophageal aperistalsis Internal hemorrhoids =? Above the anorectal line What is another name for environmental enteropathy ? What are late symptoms of appendicitis ? RLQ tenderness to deep/constant pain What is the most common bacterial enteric pathogen in the US? How many people in the us over have have adenomas? What is a main symptom of acute peptic ulceration ? Coffee ground mematemesis Transmural inflammation with fissures and Melena is associated with what? What things are aspirated with toxic Megacolon? Polyarthritis, sacoiliitis, AS, eye irritation Where is the most common site of mets for oral squamous cell carcinoma? Cervical lymph nodes What is the most common cancer associated with Lynch syndrome? Is chronic gastric mucosa symptomatic ? What kind of pain is associated with Crohn's disease? How does malabsorption manifest itself? What causes travelers diarrhea ? Fecal oral contamination What are 3 organisms that cause acute self limited colitis ? Campylobacter, shigella, E. coli, salmonella What are risks for pseudomembranous colitis? What is congenital outpouching of the small intestine called ? What kind of ulcer gets better after eating? What is another name for hirschsprung disease? Congenital aganglionic megacolon What is the most common vascular disorder of G.I. Tract ? What is the loss of inhibitory innervation to the LES? What are carries are salmonella? Snakes, lizards, gecko, salamanders q Thrush is seen in what percent of newborns? What disease is associated with lack of neurologic ganglia in the rectum that may involve the sigmoid colon? What is the most common cause of appendicitis ? What type of esophageal lesion is associated with achalasia, aperistalsis? What percent of submandibular gland tumors are malignant ? What is the main cause of travelers diarrhea? Enterotoxigenic E. Coli What organisms is associated with parasitic disease? How does one diagnose familial adenomatous polyposis ? 100 or more adenomas most commonly 500-2,500 What are treatments for pseudomembranous colitis ? Vancomycin or metronidazole What is a shallow painful ulceration in the oral mucosa and or pharynx usually less than 5mm? What is inhibitory for U.C.? Does location of herpes indicate type ? At what age are most people diagnosed with colorectal adenocarcinoma ? What are risk factors for pyloric stenosis ? White, male, turner syndrome	2	9	0	0	0	2	0.322304	2	4,428
Diagnosis Code N83.7 Information for Medical Professionals The following edits are applicable to this code: Diagnoses for females only - Diagnoses for females only. Diagnostic Related Groups The diagnosis code N83.7 is grouped in the following Diagnostic Related Group(s) (MS-DRG V35.0) - 742 - UTERINE AND ADNEXA PROCEDURES FOR NON-MALIGNANCY WITH CC/MCC - 743 - UTERINE AND ADNEXA PROCEDURES FOR NON-MALIGNANCY WITHOUT CC/MCC Convert to ICD-9 - 620.7 - Broad ligament hematoma - Hematoma of broad ligament Information for Patients Also called: Hematoma, Hemorrhage Bleeding is the loss of blood. It can happen outside or inside the body. You may bleed when you get a cut or other wound. Bleeding can also be due to an injury to internal organs. Sometimes bleeding can cause other problems. A bruise is bleeding under the skin. Some strokes are caused by bleeding in the brain. Other bleeding, such as gastrointestinal bleeding, coughing up blood, or vaginal bleeding, can be a symptom of a disease. Normally, when you bleed, your blood forms clots to stop the bleeding. Severe bleeding may require first aid or a trip to the emergency room. If you have a bleeding disorder, your blood does not form clots normally. - Bleeding (Medical Encyclopedia) - Bleeding gums (Medical Encyclopedia) - Bleeding into the skin (Medical Encyclopedia) - Intraventricular hemorrhage of the newborn (Medical Encyclopedia) - Subarachnoid hemorrhage (Medical Encyclopedia) The ovaries are part of the female reproductive system. Women have two ovaries, one on each side of the uterus. Each ovary is about the size and shape of an almond. The ovaries produce and store a woman's eggs. During ovulation, an ovary releases an egg. If that egg is fertilized by a sperm, a pregnancy can occur. Ovaries also make the female hormones estrogen and progesterone. When a woman goes through menopause, her ovaries stop making those hormones and releasing eggs. Problems with the ovaries include - Ovarian cancer - Ovarian cysts and polycystic ovary syndrome - Primary ovarian insufficiency - Ovarian torsion, a twisting of the ovary - Ovarian hyperstimulation syndrome (Medical Encyclopedia) - Ovarian overproduction of androgens (Medical Encyclopedia) The uterus, or womb, is the place where a baby grows when a woman is pregnant. The first sign of a problem with the uterus may be bleeding between periods or after sex. Causes can include hormones, thyroid problems, fibroids, polyps, cancer, infection, or pregnancy. Treatment depends on the cause. Sometimes birth control pills treat hormonal imbalances. If a thyroid problem is the cause, treating it may also stop the bleeding. If you have cancer or hyperplasia, an overgrowth of normal cells in the uterus, you may need surgery. With two other uterine problems, tissue that normally lines the uterus grows where it is not supposed to. In endometriosis, it grows outside the uterus. In adenomyosis, it grows in the uterus's outside walls. Pain medicine may help. Other treatments include hormones and surgery. - Adenomyosis (Medical Encyclopedia) - Asherman syndrome (Medical Encyclopedia) - D and C (Medical Encyclopedia) - Endometrial ablation (Medical Encyclopedia) - Endometrial polyps (Medical Encyclopedia) - Endometritis (Medical Encyclopedia) - Hysteroscopy (Medical Encyclopedia) - Retroversion of the uterus (Medical Encyclopedia) General Equivalence Map Definitions The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs. - Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent. - No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system. - Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system. Present on Admission The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.	4	2	0	0	0	4	0.89119	4	1,072
Vaginal hysterectomy is a surgical procedure to remove the uterus through the vagina. During a vaginal hysterectomy, the surgeon detaches the uterus from the ovaries, fallopian tubes and upper vagina, as well as from the blood vessels and connective tissue that support it, before removing the uterus . Your uterus — or womb — is where a baby grows if you're pregnant. A partial hysterectomy removes just the uterus, leaving the cervix intact. A total hysterectomy removes the uterus and the cervix What Is a Hysterectomy? A hysterectomy is a surgical procedure to remove a woman's uterus. The uterus, also known as the womb, is where a baby grows when a woman is pregnant. The uterine lining is.. . It ends menstruation and the ability to become pregnant. Depending on the reason for the surgery, a hysterectomy may also involve the removal of other organs and tissues, such as the ovaries and/or fallopian tubes A hysterectomy is the surgical removal of the uterus. Hysterectomies are performed for a wide variety of reasons. A hysterectomy is major surgery, but with new technological advances, the discomfort, risk of infection and recovery time has all been decreased A hysterectomy is the surgical removal of all or part of a woman's uterus. Hysterectomies are typically performed to treat cancer, chronic pain, or heavy bleeding that has not been controlled by less invasive methods. Because you cannot get pregnant after having a hysterectomy, other medications or procedures are sometimes tried beforehand A hysterectomy is surgery to remove your uterus. The uterus is the part of a woman's body where a baby grows. There are different ways to perform a hysterectomy. Depending on the reason for the.. A partial hysterectomy is surgical removal of the uterus alone, and a myomectomy is removal of only fibroids. A total hysterectomy removes the cervix as well as the uterus. In certain cancer cases,.. Each year, up to 500,000 women in the United States have a hysterectomy, meaning a surgical removal of the uterus and sometimes the cervix and supporting tissues. Once a woman has this procedure,.. Hysterectomy is a major surgical procedure that has risks and benefits, and affects the hormonal balance and overall health of patients. Because of this, hysterectomy is normally recommended as a last resort after pharmaceutical or other surgical options have been exhausted to remedy certain intractable and severe uterine/reproductive system conditions A hysterectomy is a surgical procedure whereby the uterus (womb) is removed. This surgery for women is the most common non-obstetrical surgical procedure in the United States. Approximately 300 out of every 100,000 women will undergo a hysterectomy Vaginal or laparoscopic assisted vaginal hysterectomy (LAVH). A vaginal hysterectomy is less surgically invasive than an abdominal procedure, and recovery can be as short as two weeks. Most women.. A hysterectomy is performed when you had a medical condition that makes the removal of the uterus, and cervix necessary. A hysterectomy can be performed in several ways, from a full abdominal surgery to minimally invasive laparoscopic or vaginal methods Radical Hysterectomy The surgeon removes your uterus, cervix, the top portion of your vagina, most of the tissue that surrounds the cervix, and sometimes the pelvic lymph nodes. Radical hysterectomy can be an option for treating cancer Hysterectomy is the second most common major surgical procedure performed in women worldwide and almost one in three women in the USA have undergone a hysterectomy by the age of 60 years A simple hysterectomy is a procedure where the uterus and cervix are removed while other surrounding structures, such as vagina, lymph nodes, ovaries and fallopian tubes, are left in place. A simple hysterectomy can be performed via an abdominal hysterectomy, a vaginal hysterectomy or a laparoscopic hysterectomy Hysterectomy is the surgical removal of the uterus. Learn about hysterectomy types and diagnosis A hysterectomy refers to the removal of all or part of a woman's uterus, effectively putting an end to periods and the ability to get pregnant. There are four different kinds of hysterectomy, each referring to how much of the female reproductive system is removed: A Partial or Subtotal Hysterectomy involves removing only the uterus, leaving. Hysterectomy may be recommended for several life-threatening conditions. If you have any of the following conditions, hysterectomy may save your life and also free you from significant pain and discomfort. Invasive cancer of the uterus, cervix, vagina, fallopian tubes, and/or ovaries. Only 8 to 12 percent of hysterectomies are performed to. A hysterectomy is a major, life-altering surgery, so it is not a procedure that should be taken lightly. Technically, any woman of legal age can consent to the procedure, but it should be medically justified A hysterectomy is an operation to remove the uterus (womb). Most hysterectomies are not emergencies. You have time to think about what you want to do. This booklet covers the benefits and risks - as well as options other than surgery. It is designed to help yo A hysterectomy is a surgical procedure to remove the womb (uterus). You'll no longer be able to get pregnant after the operation. If you have not already gone through the menopause, you'll no longer have periods, regardless of your age. Many women have a hysterectomy Hysterectomy is the second most common surgery for women in the United States. But that doesn't mean it's a breeze. Many women feel uneasy or sad about no longer having a major female organ, feel severe effects of hormonal fluctuations, or find that recovery takes longer than they expected During a total hysterectomy, your womb and cervix (neck of the womb) is removed. A total hysterectomy is usually the preferred option over a subtotal hysterectomy, as removing the cervix means there's no risk of you developing cervical cancer at a later date Hysterectomy is a surgery for the removal of the uterus. Depending on whether there is the removal of other organs or tissues, hysterectomy may be of different types. Radical hysterectomy is a surgery that involves the removal of the entire uterus along with its supporting tissues, cervix (the neck of the uterus), and the vagina A total hysterectomy removes the uterus and the cervix. A total hysterectomy is also called a simple hysterectomy. A subtotal hysterectomy removes the uterus but leaves the cervix in place. A radical hysterectomy removes the uterus, cervix, the uppermost part of the vagina next to the cervix and the nearby ligaments that support the uterus A hysterectomy is a type of surgical procedure in which a woman's uterus is removed. In a total hysterectomy, the cervix is removed as well. Laparoscopic surgery involves the creation of small puncture holes in the abdominal wall instead of the large incisions made with open surgery Radical hysterectomy - this hysterectomy is the most complete and is usually performed in cases of uterine, cervical, or other cancer. All portions of the female reproductive system are removed, including the uterus, cervix, ovaries, fallopian tubes, upper vagina, and nearby lymph nodes Expected recovery time following a hysterectomy can vary widely depending on the surgery type, your diagnosis, and other factors. Generally speaking, the more invasive the surgery, the longer the recovery can be; however, each woman will heal at her own rate A hysterectomy (the surgical removal of the uterus) is a major operation. In addition to a lengthy hospital stay and recovery period at home, there's a 30% complication rate and obvious issues regarding a woman's sexuality, fertility, and quality of life once the uterus is removed Hysterectomy is a surgery to remove the uterus and cervix. Abdominal is the surgical technique that will be used. This means the surgery will be done through an incision in your abdomen. A bilateral salpingo-oophorectomy is surgery to remove both of your ovaries and fallopian tubes. The hysterectomy and bilateral salpingo-oophorectomy. My OB-GYN suggested I consider a hysterectomy, unless I wanted more children. Once I recovered from the fit of laughter (two boys of my own and a stepson is plenty for me, thanks), I agreed it was time. I had done research on the laparoscopic hysterectomy, and everything I read was encouraging — less downtime, pain, and scarring Hysterectomy is the surgical removal of a woman's uterus. This surgery can be done through small incisions using a thin, lighted scope with a camera on the end (a laparoscope). This is called a laparoscopic hysterectomy. In robotic-assisted laparoscopic hysterectomy, the surgeon uses a computer to. A hysterectomy is a surgical procedure that completely or partially removes a person's uterus. The uterus, also known as the womb, is where a fetus grows during pregnancy What to Expect from a Robotic Hysterectomy. By Geoffrey Bowers, MD, Virtua Gynecologist/Robotic Surgeon Section Chief, Gynecology, and Medical Director, Robotic Surgery—Virtua South. If you have pain and heavy bleeding caused by uterine fibroids or endometriosis, a hysterectomy (surgical removal of the uterus) often is your only option for symptom relief A partial — or supracervical — hysterectomy involves removal of the upper part of the uterus but leaves the cervix intact. In a complete hysterectomy, both the uterus and cervix are removed. When the ovaries and fallopian tubes are also removed, it is called a hysterectomy with bilateral salpingo-oophorectomy Tests to find uterine cancer include a pelvic exam, imaging tests, and a biopsy. The most common treatment is a hysterectomy, which is surgery to remove the uterus. Sometimes the surgery also removes the ovaries and fallopian tubes. Other treatments include hormone therapy, radiation therapy, and chemotherapy A hysterectomy surgically removes a woman's uterus, where a baby grows during a pregnancy. It is the second most common surgery among women in the United States.. A hysterectomy may be used to treat cervical, ovarian and uterine cancers. The procedure may involve removing the ovaries and fallopian tubes in a surgery called a salpingo-oophorectomy.If you have not yet reached menopause, a. radical hysterectomy: [ his″tĕ-rek´to-me ] surgical removal of the uterus . Within the past decade this has become a common major surgery in the United States. Controversy continues over whether many hysterectomies are really necessary. In making the decision for hysterectomy, patients often seek a second medical opinion and are encouraged to. A hysterectomy is major surgery to remove your uterus, or womb. In a total hysterectomy, the cervix, which is the lower part of the uterus near the vagina, is also removed . Your ovaries, fallopian tubes, cervix, or part of your vagina may also need to be removed. The organs and tissue that will be removed depends on your medical condition Although a hysterectomy is often recommended as a way to treat uterine prolapse. However, even a surgical procedure that seeks to resolve one issue, may run the risk of presenting complications during or post recovery. Removing the uterus may put you at a higher risk pelvic organ prolapse As with most surgeries, after a hysterectomy, women will have to deal with pain. How much pain you will feel after the operation depends on what type of hysterectomy they performed and your individual susceptibility to pain. The degree of pain will vary depending upon the type of incision made. Some studies and research suggest that women have less pain after laparoscopically assisted vaginal. A vaginal cuff is a closure made at the top of the vagina in the place of the cervix after a person has a hysterectomy. In this article, we look at the risks involved with having a vaginal cuff. Surgery to remove the womb (radical hysterectomy) Surgery to remove the womb is quite a big operation, and may be necessary to make sure that all the cancer has gone. Doctors usually offer a hysterectomy to women with stage 1 or 2A cervical cancer. You have the operation while you are asleep (under general anaesthetic) Abdominal Hysterectomy - $9,995 to $18,880. Also known as an open hysterectomy, this invasive operation is the removal of the uterus by incising the patient's lower abdomen. Women's Health says it takes four to six weeks for the woman to recover completely. Laparoscopic Hysterectomy - $8,088 to $12,19 What is Hysterectomy? Hysterectomy is a therapeutic procedure in which a woman's uterus or womb is removed. Hysterectomy is commonly performed to treat uterine fibroids, abnormal uterine bleeding, or cancer FACTS FACT: Women report a loss of physical sexual sensation after hysterectomy. FACT: A woman's vagina is shortened, scarred and dislocated by hysterectomy. FACT: Hysterectomy's damage is life-long. Among its most common consequences, in addition to operative injuries are: heart disease loss of sexual desire, arousal, sensation and uterine orgasm weight gain osteoporosis bone, joint. A hysterectomy is a surgical operation to remove the womb. This is a major surgical operation and it can be associated with some complications. Many hysterectomies are performed each year for different reasons. This article will discuss the following important points: Reasons for having a hysterectomy. Different types of hysterectomies A hysterectomy is a surgical procedure which removes some or all of a woman's reproductive organs. These organs, which include the uterus, ovaries, cervix and fallopian tubes, are located in a woman's lower abdomen. The ovaries produce eggs and hormones, the cervix is at the lower end of the uterus, the fallopian tubes transport eggs from. Risks of a laparoscopic hysterectomy: The surgeon may need to change from a laparoscopic surgery to an open abdominal surgery. You will not be able to become pregnant after you have a hysterectomy. If your ovaries are removed during surgery, you will go through menopause. You may bleed more than expected or get an infection Hysterectomy (tangohanga kōpū) is an operation to remove your uterus (womb). There are many reasons you may need a hysterectomy. How the operation is done depends on these reasons, as well as your needs and wishes Vaginal hysterectomy - With this procedure, women may spend one night in the hospital or even go home the same day. It can take three to four weeks to recover. Laparoscopic or robotic hysterectomy - This procedure is the least invasive option and can take two to four weeks to recover . Hysterectomy is used to treat a number of conditions, including heavy or painful periods, fibroids and prolapse. The operation may be performed through the abdomen or the vagina. Apart from cancer, many gynaecological conditions can often be successfully. A total hysterectomy removes all of the uterus, including the cervix. The ovaries and the fallopian tubes may or may not be removed. This is the most common type of hysterectomy. A partial, also called subtotal or supracervical, hysterectomy removes just the upper part of the uterus. The cervix is left in place Hysterectomy is a radical surgery that involves the removal of the uterus. It may or may not involve removal of the ovaries and cervix. The surgery results in the elimination of organs and their function. A partial hysterectomy involves removing only the upper part of the uterus while the cervix is left intact An abdominal binder is a fitted elastic material that wraps around the tummy and fastens (usually with velcro). It is meant to fit snugly, but not tightly; similar to an ace bandage for a sprained ankle. An abdominal binder serves several functions in the early post-op stages of an abdominal or pelvic surgery, including a hysterectomy A hysterectomy is a procedure that typically removes some part of the uterus or the entire uterus. There are various types of hysterectomies, including a subtotal hysterectomy, total hysterectomy, and a radical hysterectomy. In a subtotal hysterectomy, only the upper portion of the uterus is removed. This keeps the cervix in place A laser hysterectomy may be performed laparoscopically. In LVAH procedures, the surgeon inserts several tools through the abdomen to access the uterus and the uterus is removed through the vagina. The laser hysterectomy procedure is less invasive than an abdominal hysterectomy, where a large incision is made over the pelvis to access the uterus and remove it . The uterus is the place where a baby grows when a woman is pregnant. After a hysterectomy, you no longer have menstrual periods and can't become pregnant Hysterectomy is used to treat many women's health conditions. Some of these conditions include. uterine fibroids (this is the most common reason for hysterectomy) . endometriosis. pelvic support problems (such as uterine prolapse). abnormal uterine bleedin A hysterectomy is the surgical removal of the uterus. Depending on the type of hysterectomy, other pelvic organs or tissues also may be removed. The types of hysterectomy include: Subtotal, supracervical or partial hysterectomy. The uterus is removed, but not the cervix A hysterectomy is the second most common surgery among women in the U.S. (C-section is the most common). A hysterectomy is a surgery to remove a women's uterus, often due to conditions including fibroids, uterine prolapse and others that cause pain and bleeding A hysterectomy is a surgical procedure to remove the uterus from a woman's body. There are many different types of hysterectomy surgeries that use various approaches, techniques, and technology, including a number of innovative, minimally invasive procedures A hysterectomy is the surgical removal of the uterus, otherwise known as the womb. Rarely is a hysterectomy an emergency surgery. There is usually considerable time to research and explore options for discussions with your personal surgeon. Description of Hysterectomy. During a hysterectomy the uterus is completely or partially removed Sex and Vaginal Dilators After a Hysterectomy Sexual problems for women occur for a variety of reasons. Sometimes it's the onset of menopause causing vaginal dryness, leading to painful penetration. For some, it's from the uterus, cervix, and part of the vagina being removed due to a radical hysterectomy as a treatmen A hysterectomy is a major life-changing procedure, but for thousands of women, it can provide relief and an effective treatment for issues such as heavy periods, prolapse and endometrial cancer.. However, a hysterectomy isn't necessarily the first line of treatment for certain conditions. We'll take a look at some of the common reasons for a hysterectomy, what you can expect when. Hysterectomy is a surgical procedure that involves removing a part of or the whole uterus. Some opt for it to treat excessive bleeding, others due to cancer, and a fraction aiming to stop their child-bearing capabilities Hysterectomy is surgical removal of the uterus and sometimes the cervix and supporting tissues. It is the most common non-pregnancy-related major surgery performed on women in the United States, with one in three women having a hysterectomy by age 60 Pain After Laparoscopic / Robotic Hysterectomy. While the approaches and types of surgery are different, the pain is not. Both laparoscopic and robotic hysterectomies can cause scar tissue that can restrict fascia and decrease blood flow to muscles and nerves. This is especially true in the pelvic, stomach and lower back regions Abdominal hysterectomy. Abdominal hysterectomy should only be performed when a vaginal, laparoscopic, or robotic approach is not safe or feasible. When compared to vaginal hysterectomy, abdominal hysterectomy has a longer hospital stay, increased pain, and slower return to normal activities A laparoscopic hysterectomy is surgery to take out the uterus. Your doctor put a lighted tube and surgical tools through small cuts in your belly to remove the uterus. You can expect to feel better and stronger each day. But you might need pain medicine for a week or two. It's normal to also have some shoulder or back pain A hysterectomy is the surgical removal of a woman's uterus, and when it's just the uterus being removed - no other organs, except perhaps the cervix and surrounding, supporting tissues - it's often referred to as a partial hysterectomy A hysterectomy, which is the surgical removal of the uterus, is the second most common type of surgery among women. A hysterectomy is most often recommended to treat cancerous and non-cancerous tumors, endometriosis, chronic pelvic pain, uterine prolapse, and prolonged abnormal uterine bleeding. The specifics of the procedure and your recovery. A hysterectomy is a procedure in which a woman's uterus is surgically removed, and there are many surgical options for a woman who must undergo this. In premenopausal, menopausal, perimenopausal, and postmenopausal women, hysterectomies have been a very common surgical option Hysterectomy is a surgery, which includes removal of uterus and its supporting structures, as required by the underlying condition. There are 4 types of hysterectomy surgeries and it is important to know about the procedure and its recovery time. For some conditions, alternatives to hysterectomy may be considered Three different types of hysterectomy (surgical removal of the uterus) surgeries are depicted. The different types of surgery include the abdominal, vaginal. • Radical hysterectomy - a total hysterectomy that also includes removal of structures around the uterus This procedure puts a woman into surgical menopause. Unlike natural menopause, surgically induced menopause causes an immediate decline in progesterone, estrogen, and testosterone production, rather than the natural, gradual decline that. Hysterectomy has been successfully combined with other gender affirming surgeries performed on the same day in the same operating suite including vaginectomy, mastectomy, and genital reconstruction including metoidioplasty and phalloplasty.[10,12] Hysterectomy itself does not largely differ, however some modifications in concurrent surgeries. A complete hysterectomy involves removal of the uterus and cervix. A complete hysterectomy, also referred to as a total hysterectomy, is a medical procedure during which a woman's cervix and uterus are removed. The surgeon might also take out a woman's ovaries and fallopian tubes in some cases. A complete hysterectomy is different from a. In a vaginal hysterectomy, theorgans are removed through an incision made in the top of the vagina. In both types of hysterectomy, sometimes the cervix of the uterus is left behind, and this is called a partial or supracervical hysterectomy. Once a vaginal procedure has begun, the doctor might decide to perform an abdominal hysterectomy instead. In a simple hysterectomy, the uterus is removed. In a radical hysterectomy, the uterus and some surrounding parts around the cervix are removed. The open radical hysterectomy is performed by removing the uterus through an incision (similar to the one made in a C-section) in the lower abdomen Hysterectomy is a surgical procedure for removing the uterus, which is a pelvic organ situated between the bladder and rectum in women. The uterus is the organ in which a fetus grows and develops. Dysfunctional uterine bleeding (DUB) is a common clinical condition that frequently leads to hysterectomy. Endometrial ablation --a minimally invasive surgical technique that removes or destroys the endometrial lining of the uterus -- is a conservative alternative to hysterectomy for DUB. While en From what I have read, hysterectomy is a quicker surgery so the surgeon is paid more per hour. Hysterectomy is the cash cow of gynecology which is why each resident must do at least 85 (recently increased from 70) but there is no requirement for myomectomy. That is why it can take some shopping around to find a myomectomy surgeon Hysterectomy. This is a surgery in which a woman's uterus is removed from her body. This operation is conducted by a professional surgeon. It may also involve the removal of fallopian tubes, ovaries, cervix, and other surrounding structures.Also Read The term hysterectomy means removal of the uterus. Hysterectomies are very common surgical procedures; one in three women will have one. There are many reasons why a woman may need to have her uterus removed. The most common reason is heavy bleeding due to fibroids, which are benign growths within the walls of the uterus When you remove the uterus and leave the ovaries in place during a partial hysterectomy, you are severing their major source of blood. As a result, the ovaries are more likely to malfunction or shut down all together. As you can see in this blog's photo, the major connecting piece is missing Size of uterine fibroids that require hysterectomy. Uterine fibroids of 4 inches (9-10 cm) or larger in size must be treated through surgery immediately. It is almost like the average size of a plum or an orange. According to gynecologists it is the largest size for a fibroid and requires immediate treatment A Hysterectomy is a procedure in which the uterus and cervix are removed. Depending on the goal of surgery, removal of the fallopian tubes and or removal of the ovaries can be considered but this is a separate decision. If the ovaries need to be removed, a process is undergone called an oophorectomy A study published in 2008 showed that these problems can develop up to at least 8 years after the ablation and increase the risk for hysterectomy (to resolve the pelvic pain). I recall a woman saying that a gynecologist at a renowned east coast institution told her the procedure should not be done on women under age 50 A hysterectomy is technically only the removal of the uterus—a.k.a. the womb, where a fetus develops and grows, and which plays a critical role in your menstrual cycle and fertility What is a chemical hysterectomy? - Answered by a verified OB GYN Doctor. Disclaimer: Information in questions, answers, and other posts on this site (Posts) comes from individual users, not JustAnswer; JustAnswer is not responsible for Posts After news spread of Lena Dunham's total hysterectomy to treat her endometriosis, a global conversation began to unfold: Is a hysterectomy an effective form of endometriosis treatment? There is no certainty that a hysterectomy will rid a patient of their endometriosis, Harry Reich, informally known as the father of endometriosis, tells The Blossom What is a Hysterectomy? For those who don't know, a hysterectomy is essentially the surgical removal of the uterus. There are typically three types of hysterectomies: Subtotal - the body of the uterus is removed, but the cervix remains; Total - both the body of the uterus and the cervix are remove A hysterectomy is an operation to remove a woman's uterus. A woman may have a hysterectomy for different reasons, including: Uterine fibroids that cause pain, bleeding, or other problems. Uterine prolapse, which is a sliding of the uterus from its normal position into the vaginal canal HYSTERECTOMY, LAPAROSCOPICALLY ASSISTED VAGINAL (LAVH), +/- BSO The following CPT codes require prior authorization: Code Description 58263 Vaginal hysterectomy, for uterus 250 grams or less, with removal of tube(s), and/or ovary(s), with repair of enterocel A total hysterectomy is the removal of the whole uterus, the fundus, and cervix. A subtotal, partial, or supracervical hysterectomy is the removal of the fundus or top portion of the uterus only, leaving the cervix in place. Radical hysterectomy includes the removal of the entire uterus and nearby tissue, the cervix, and the top part of the vagina Radical hysterectomy is the preferred treatment in the surgical management of patients with early-stage cervical cancer, and its cure rate (in the absence of indications for adjuvant therapy) is over 90%. 17,18 Radical hysterectomy offers several advantages over radiotherapy in that it preserves ovarian function in young patients, it allows for. A hysterectomy is a surgery to remove all or part of the uterus. A total hysterectomy means that the whole uterus and the cervix are removed. Removing the ovaries and fallopian tubes is a separate procedure—you and your doctor can discuss whether to combine this with your hysterectomy Hysterectomy is the surgical removal of the uterus.Hysterectomy may be advised for the treatment of many health conditions in women. The gynaecologist will decide the best approach to conduct a hysterectomy, depending upon the nature of the case and the medical condition of the patient Approach 1: An abdominal hysterectomy is an open surgery and is the most common approach to hysterectomy, accounting for about 54% for all benign disease. To perform an abdominal hysterectomy, a surgeon makes a 5- to 7-inch incision, either up-and-down or side-to-side, across the belly. The surgeon then removes the uterus through this incision Additionally, what happens after hysterectomy and prolapse repair? Most women recover well from this surgery in six weeks. Avoid high-impact exercise, sexual activity, and heavy lifting after surgery for at least six weeks. It is normal to notice spotting and discharge from your vagina	2	12	2	0	0	0	0.391926	2	6,794
- Hemolytic-Uremic Syndrome (HUS) is a very rare disorder that primarily affects young children between the ages of one and 10 years, particularly those under the age of four years. Symptoms of Hemolytic-Uremic Syndrome usually become apparent three. — “CIGNA - Typical Hemolytic Uremic Syndrome”, - Definition of uremic in the Medical Dictionary. uremic explanation. Information about uremic in Free online English dictionary. What is uremic? Meaning of uremic medical term. What does uremic mean?. — “uremic - definition of uremic in the Medical dictionary - by”, medical- - uremia also uraemia n. A toxic condition resulting from kidney disease in which there is retention in the bloodstream of waste products normally Some uremic signs are due to losses of kidney function that do not involve azotemia. — “uremia: Definition from ”, - Atypical hemolytic-uremic syndrome is a disease that primarily affects kidney function. This condition, which can occur at any age, causes abnormal blood clots (thrombi) to form in small blood vessels in the kidneys. Atypical hemolytic-uremic syndrome is characterized by three major. — “Atypical hemolytic-uremic syndrome - Genetics Home Reference”, m.nih.gov - Uremic. Lifestyle, fitness & health information about Uremic. What Are the Causes of Acute Renal Failure in Children?, Kidney Disease Symptoms in Adults, What Causes Hemolytic uremic syndrome is a rare disease that primarily affects children under the age of ten and leads to acute renal failure. — “Uremic \| ”, - Detailed information on hemolytic uremic syndrome, including cause, progression, treatment, and statistics. — “Hemolytic Uremic Syndrome”, - Early symptoms include anorexia and lethargy, and late symptoms can include decreased mental acuity and coma. Renal failure (Acute renal failure, Chronic renal failure) · Uremic pericarditis · Uremia. — “Uremia - Wikipedia, the free encyclopedia”, - Uremic pruritus. FOLLOW UP. Given the typical histological ﬁndings from the skin biopsy in Uremic pruritus is often considered a misnomer for the. following reasons: pruritus in. — “Uremic pruritus”, uphs.upenn.edu - Uremic Frost. The Nephrology Blog. Monday, November 22, 2010. NEJM: The Frequent hemodialysis, as compared with conventional hemodialysis, was associated with favorable results with respect to the. — “Uremic Frost”, - Explains how hemolytic uremic syndrome develops after E-coli infection in the digestive tract and describes the treatments available. — “Hemolytic Uremic Syndrome in Children”, kidney.niddk.nih.gov - Hemolytic Uremic Syndrome (HUS) is a serious disease that affects the kidneys and blood clotting system. It is a rare disease but is more common in children, especially younger than age 5. — “Hemolytic Uremic Syndrome - Medpedia”, - What are the Symptoms of Uremic Poisoning? Answer: The term uremia was first used in 1847 to describe the symptoms of kidney failure. Nonetheless, the terms uremia or uremic poisoning have persisted, and now mean the symptoms that patients feel when they have kidney failure. — “What are the Symptoms of Uremic Poisoning? (Renalife)”, - Uremic definition, pertaining to uremia. See more. — “Uremic \| Define Uremic at ”, - Hemolytic Uremic Syndrome cases mostly are due to food poisoning by E. coli strain O157:H7. Research has not yet found the causes for the Hemolytic Uremic Syndrome occurrence in most of the patients. — “” - Introduction Background Richard Bright first described uremic pericarditis 163 years ago. causing 341 illnesses, including hemolytic uremic syndrome (HUS). HUS is not well described in non-O157 E coli. — “Uremic”, - Signs and symptoms of hemolytic uremic syndrome may include: Fever Abdominal pain Pale Hemolytic uremic syndrome (HUS) In children, most cases of hemolytic uremic syndrome. — “Uremic - Kosmix : Reference, Videos, Images, News, Shopping”, - Overview: Uremic neuropathy is a distal sensorimotor polyneuropathy caused by uremic toxins. The severity of neuropathy is correlated strongly with the severity of the renal insufficiency. Uremic neuropathy is considered a dying-back neuropathy. — “Uremic Neuropathy: eMedicine Neurology”, - Because an incubation period of approximately one week occurs between the start of diarrhea and the onset of hemolytic uremic syndrome, physicians should maintain a high index of suspicion; early laboratory testing is important to diagnose and manage this syndrome. — “Hemolytic Uremic Syndrome: An Emerging Health Risk”, - List of disease causes of Uremic encephalopathy, patient stories, diagnostic guides. Diagnostic checklist, medical tests, doctor questions, and related signs or symptoms for Uremic encephalopathy. — “Uremic encephalopathy - ”, - Definition of uremic in the Online Dictionary. Meaning of uremic. Pronunciation of uremic. Translations of uremic. uremic synonyms, uremic antonyms. Information about uremic in the free online English dictionary and encyclopedia. — “uremic - definition of uremic by the Free Online Dictionary”, - Read about hemolytic uremic syndrome (HUS), blood clotting disease caused by E. Coli infection, birth control pills, pneumonia, medications, and more. Symptoms, diagnosis, treatment and prevention information is included. — “Hemolytic Uremic Syndrome (HUS) Causes, Symptoms, Diagnosis”, related videos for uremic - Gastritis Uremic 98180 - Food Safety Attorney Bill Marler Explains E. coli O157 and HUS Video www.about- Food Safety Attorney Bill Marler Explains E. coli O157 Infection E. coli O157:H7 produces a toxin when it's in the human body that is excreted from the bacteria and if it enters the bloodstream can cause severe, severe illness. The normal illness that can be ascribed to E. coli O157 is Hemolytic Uremic Syndrome, or HUS and that has caused untold deaths and severe illness in the United States and frankly, throughout the world. Where is E. coli O157:H7? It is everywhere. It used to be thought of -- primarily still is -- as a reservoir in cattle, but we've been finding it in buffalo, sheep, goats, deer -- they even found it in a trout in a lake. These are bacteria that are fecal in origin, that come from animals other than us, and in our body...our body doesn't tolerate them. E. coli O157:H7 outbreaks can kill. E. coli and other bacteria are especially dangerous to the young and the elderly. Because in the young their immune system is not fully developed, and in the elderly their immune system has been compromised. What are the symptoms? E. coli O157 symptoms are pretty classic. Three to four days after the ingestion of the bacteria it's cramping that'll then turn to bloody diarrhea. Bloody diarrhea is a hallmark of E. coli O157:H7 infection. Most people recover. Some ninety percent of the people within ten days will start to get better, but it's a very horrible experience, and very painful. Five to ten percent of people will go on to develop ... - Gastroduodenoscopy on the cat , uremic gastritis - DIC, TTP, HUS - The Microangiopathic Anemias - Quinine Dangers! Night time leg cramps Quinine (kwye' nine) Last revised: July 1, 2010. ISSUE: Due to continued reports of serious side effects in patients using quinine (Qualaquin) ''off-label'' for night time leg cramps, FDA has approved a risk management plan to warn against the use of this drug for such unapproved uses. Quinine should not be used for night time leg cramps. Quinine use may result in serious and life-threatening hematological reactions, including serious bleeding due to thrombocytopenia, and hemolytic-uremic syndrome/ thrombotic thrombocytopenic purpura, which in some cases may result in permanent kidney damage. In some patients, adverse reactions result in hospitalization and death. - say prayer audition by jewelry sa audi para sa mga friends ko n sobra kong mahal miss ko kau lagi ^_^I LOVE YOU ALL guys, radshie, raize, uremic, mhondz, wiloma, athru, min3, ate honey, hibana, at sa lahat lahat ^_^ - E. coli Lawyer Bill Marler Explains Bologna E. coli Outbreak and Recall E. coli attorney Bill Marler of the Marler Clark, the nation's largest food poisoning law firm explains the recent Seltzer's Lebanon bologna E. coli outbreak and recall. The CDC, about a week ago announced an outbreak of E. coli O157:H7 linked to what's known as Lebanon bologna, which is a sausage, salami type of product primarily made in Pennsylvania and has sickened about a dozen people in five states, mostly on the East coast. There doesn't to be anyone who has developed Hemolytic Uremic Syndrome or kidney failure but there has been a couple of people hospitalized and about a dozen people sick. The real risk is to the very young and very old. This bologna outbreak seems to be striking people in the middle age range, which is why I think we're not seeing a lot of people hospitalized which is obviously a good thing. Sausage, salami, and bologna have had a history of problems over the last two decades with dozens of outbreaks of Salmonella, E. coli, Listeria and others. It is not heat cured. It is a low heat and they tend to use a lot of spices and things to deal with the bacterial growth. Anybody who has this product in their homes should discard it or contact the department of health or contact the company and ask for a refund. This kind of product is generally safe, but there was clearly in this instance some sort of problem in the production process that has allowed fecal bacteria to remain in this product, to get in this product, to remain in this product and then ... - Kibow_Enteric_Toxin_Removal.wmv Kibow® Biotics metabolizes nitrogenous waste that has diffused from the bloodstream into the bowel. Nitrogenous wastes are utilized by Kibow® Biotics as nutrients. As probiotics grow and multiply, they consume more nitrogenous waste and therefore effectively help maintain healthy kidney function. When these waste products accumulate in high concentrations in the blood, they become highly toxic and can cause severe damage to many organ systems if they are not properly excreted. Due to the overloaded and impaired kidneys, a buildup of poisonous wastes occurs in the bloodstream. Certain probiotic microorganisms can utilize urea, uric acid and creatinine and other toxins as its nutrients for growth. They then multiply, thereby creating a greater diffusion of these uremic toxins from the circulating blood across the lining of the intestinal walls into the bowel. This increased microbial growth is excreted along with the feces (which is normally 50% microbes by weight). Enteric toxin reduction technology uses probiotic organisms to transform the colon into a blood cleansing agent, which, with the aid of microbes, indirectly removes toxic wastes and helps eliminate them as fecal matter. Consequently, it is possible to maintain a healthy kidney function with the oral use of Kibow® Biotics. The patented, proprietary probiotics in Kibow® Biotics have been clinically tested and shown to be safe, effective and free of serious side effects when taken for as long as six months. - Explaining Gastroenteritis with Food Safety Attorney Bill Marler Food poisoning attorney Bill Marler of Marler Clark, The Food Safety Law Firm explains gastroenteritis and its role in foodborne illness litigation. Gastroenteritis is the medical term for what we've come to know as food poisoning or foodborne illness. In essence, it's the vomiting, it's the diarrhea, it is sort of a catchall phrase before the stool cultures come back, or if the stool culture comes back negative and you're still having those symptoms it's so much easier for a doctor to say, "Oh that person has Gastroenteritis, it's inflammation of the stomach lining, inflammation of your bowels." It is what is most typically understood as the definite sign of a foodborne illness. Gastroenteritis is usually the first step, and for most people it's the only step, in a foodborne illness. The vast majority of people don't get sicker. They're very ill, they may be sick for five or six days, they could have E. coli or Salmonella, Campylobacter, Shigella, all of those bacteria have similar symptoms that a doctor could lump into Gastroenteritis. However as the new CDC statistics show, about 128000 people will go on to need hospitalization, and it may be for a Salmonella illness that has gone septic or E. coli illness that has developed into Hemolytic Uremic Syndrome which may require much more intervention and dialysis and then unfortunately, Gastroenteritis will lead to the deaths of some 3000 American every year. - Child with E coli HUS: Attorney Discusses Legal Rights Attorney Elliot Olsen discusses the legal rights of a child with E. coli HUS, hemolytic uremic syndrome, a severe illness that can cause kidney failure (renal failure), brain damage, stroke, blindness, heart failure, pancreatitis, hypertension and death. Elliot and the other E. coli attorneys at Pritzker Olsen law firm represent children and adults sickened by E. coli O157:H7 throughout the United States. They also represent families in wrongful death lawsuits. To contact Elliot for a free consultation, call 1-888-377-8900 (toll free). - Veronica Lake - Love Is Blue A tribute to the beautiful Veronica Lake - The peek a boo girl. She was the actress who personified the word femme fatale. During the 1940's Veronica was one of the most popular and sought-after actresses. The clips in this video are from the movies; The Blue Dahlia This Gun For Hire The Glass Key So Proudly We Hail! Sullivan's Travels Veronica Lake is one of the biggest,brightest,most beautiful stars in Hollywood history, but sadly one of the most tragic too. She was born Constance Frances Marie Ockleman on November 14,1922 in New York. During the 1940's she made her greatest movies (four with Alan Ladd) The public loved her. She quickly became one of the most popular stars, and pretty much all women of the world tried to copy her iconic hairstyle. Ronnie was a fantastic actress-she did it all- drama, film noir, comedy, and she starred in some of the greatest classics of all time. But in the 1950's her career collapsed, the reason why she didnt get any good roles anymore, and why Paramount didn't re-new her contract, was because nobody wanted to work with her- she was suffering from alcoholism and mental illness, and that made her extremely difficult to work with. In the 1960's she was at one point working as a barmaid at a hotel in Manhattan. (when former lover Marlon Brando found out about that, he sent her a check for a thousand dollars, but out of pride she never cashed it, but kept it framed in her living room.) Veronica published her autobiography in 1972, with ... - Enlarged Prostate -- Benign Prostate Hypertrophy Natural Remedies Part 1 One of the hot spots in the body. It is reddish brown, size of an English walnut. It can disturb one's sleep by requiring several trips to the bathroom each night or kill a man by producing uremic poisoning. In old age it can become a site for cancer too. The prostate is a gland which contributes greatly to one's *ual life. It is a principal storage depot for the seminal fluid, without which chances of pregnancy will be about zero. At each ejaculation, testicles provide over 150-200 million sperms cells. The prostate produces a fluid that dilutes them and this fluid contains proteins, enzymes, fats and sugars to nourish the fragile sperm, alkalinity to overcome the deadly acidity of the female tract and a watery medium in which the sperm can swim towards female egg. It is very astonishing fact to know that the condition BPH has been explained in Ayurvedic texts long back. The anatomical positon of the prostate gland, symptoms of BPH and its remedies are explained in Acharya Sushruta.The anatomical position of prostate gland is described in Ayurvedic classics as follows. In Yogaratnakara it has been described as -- "Naabheradhasthaatsanjaatha Sanchaari Yadi Vaachalaha Ashteelaavad Ghano Granthiroodhwar Maayata Unnataha" Which means "Below umbilicus (NAABHI), there is a hard gland which is little bit bulged and changes its place some times and some times stays stationary. This ... - Susan P Fisher Hoch: WITI Hall of Fame 2008 Induction Video - Women In Technology International Susan P. Fisher-Hoch MD, Professor of Epidemiology, The University of Texas School of Public Health Sue Fisher-Hoch was born in England in 1940. After completing High School she attended the Sorbonne in Paris, then continued linguistic and cultural studies in Rome. She gained admission to the Royal Free Hospital School of Medicine in 1970 at a time when women, particularly married women as she was, were not offered places. At the Royal Free she was awarded the Elizabeth Garrett Anderson fellowship, in memory of the first English woman doctor. She graduated First Class in 1975 with seven prizes for excellence. After internship with Dr. Sheila Sherlock at the Royal Free Hospital, she joined the Radcliffe Hospital, Oxford to train in virology. Between 1978 and 1982 she taught medical students, ran virology laboratories and conducted research, publishing several papers. By 1981 she had membership of the Royal College of Pathology in Virology, a Master's degree in Microbiology with distinction and a doctoral degree in epidemiology (MD) from London University. Her doctoral thesis findings were published in the Lancet and were the first identification of hot water systems as the source of outbreaks of Legionnaire's disease, as opposed to air conditioning. She was also central to the discovery that the parvovirus B19 was responsible for Fifth's Disease (Slapped Cheek Syndrome), and in the first identification of E.coli O157 as the cause of hemolytic uremic syndrome. In 1982 she ... - Aiden's Cross 2 year old Aiden Cross has an "ultra rare" disorder called Atypical Hemolytic Uremic Syndrome. The disease attacks Aiden's own little body when his immune system is stimulated. To date, Aiden has had 9 surgical procedures, the most recent being a bilateral kidney removal just 2 weeks ago. He relies on medications and daily dialysis (for 12 hours) to survive. You cannot get this little man down...he remains upbeat and a joy to be around. AHUS affects approximately 300 families in the US. There is little research and no cure. Please spread awareness by wearing Aiden's Cross! Please visit Aiden's site @ and the site for The Foundation for Children with Atypical HUS @ for more info. Thanks for watching! - E. coli and Hemolytic Uremic Syndrome (HUS) Attorney Fred Pritzker ( ) discusses hemolytic uremic syndrome (HUS) ( ), a severe illness caused by E. coli, usually E. coli O157:H7 ( ). Shiga toxins produced by the E. coli can affect the red blood cells, causing them to be misshapen. When this happens, those misshapen blood cells can get clogged in the glomeruli, tiny vessels in the kidneys. This can block the filtration system in the kidneys that allows waste products to be removed from the body. This can lead to kidney failure and a host of other life threatening complications, including stroke, heart attack and hypertension. To contact food safety attorney Fred Pritzker for a free consultation about a personal injury or wrongful death lawsuit, please call 1-888-377-8900 (toll free). - Stroke? Call 1-800-Vampire It's unfortionate that evolution takes so long, but then again survival of the fittest. Leviticus 17:11 "For the life of the flesh is in the blood...." A stroke -- sometimes called a brain attack -- happens when a blood clot prevents blood circulation in the brain. Unless the clot is quickly dissolved, the brain cells will die. There is currently only one approved drug to dissolve these clots, but it must be given within three hours of the stroke. Unfortunately, most stroke victims don't get to the hospital that quickly. A new drug called desmoteplase has now proven safe and effective when given up to nine hours after a stroke. This is a preliminary conclusion after limited human testing. Most amazingly, this drug is made from the saliva of vampire bats! These bats must feed on blood. Typically, they pierce the skin of a cow or some other mammal. The bat saliva contains the working chemical in desmoteplase and keeps the blood from coagulating while the bat is feeding; when it is finished, the blood clots in its host quite naturally. Not only is the critical time in which desmoteplase can be applied much greater than that of the man-made drug, but it appears to carry no risk of causing a hemorrhage. Again, man has gone to one of God's designs and found something that is better than anything man could devise. That this happens every day should send the message to everyone that we are the result of a wise Creator. Prayer: Thank You, Father, for all the gifts you have given ... - 2011 New Pioneers Awards - Dr. Mohamed Karmali (Science Award) Originally from Kenya, Dr. Mohamed Karmali arrived in Toronto in 1976, after completing his medical degree in Scotland and specializing in internal medicine and infectious diseases at the University of Glasgow Teaching Hospitals. Adjusting to life in Canada and to the Canadian medical system was a challenge. However, with determination, dedication, focus and his sights set high, he overcame many of the common obstacles faced by foreign medical professionals. His hard work paid off. In 1980 he became a Fellow of the Royal College of Physicians (FRCP) of Canada with Specialty Certification in Medical Microbiology. Immediately after obtaining his FRCP qualifications, Dr. Karmali joined the Hospital for Sick Children, Toronto as Staff Microbiologist. In 1989, he became the Head of the Microbiology Department; in this position, he applied his skills in persuasion, leadership and planning to develop a department that became highly respected internationally in pediatric medical microbiology. An internationally recognized physician-scientist, Dr. Karmali has made pioneering contributions in medical microbiology and infectious diseases. His first influential work was the discovery that a major cause for childhood enteritis, a common intestinal infection, was Campylobacter jejuni. He published the first study of the natural history of this infection and pioneered the diagnostic technique, conducted epidemiological studies and investigated its antimicrobial resistance. In fact, the ... - Meet Aly: Hemolytic Uremic Syndrome For most kids, after-prom means staying up all night bowling, dancing, watching movies or just hanging out. For 16-year-old Aly Reynolds, after-prom became an extended stay at Akron Children's Hospital, much of it spent in the Pediatric Intensive Care Unit, as a mysterious case of hemolytic uremic syndrome shut down her kidneys and led to more than a few scary moments. In this Children's Channel video podcast, Aly and her mom, Andrea Joliet, share the story behind her hospital stay, highlighting the ups and downs they experienced, as well as the team that helped save her life. - Dr KP Buteyko Swimming in Snow - Cold Shower Benefits Taking cold shower and cold adaptation have always been important parts of the Buteyko breathing technique. Medical research suggested numerous benefits of regular cold adaptation and cold shower use: - increase the blood level of beta-endorphin and noradrenaline and increase synaptic release of noradrenaline in the brain as well (Shevchuk, 2008) - activation the sympathetic nervous system (Shevchuk, 2008) - electrical stimulation of peripheral nerve endings to the brain, which could result in an anti-depressive effect (Shevchuk, 2008) - reduction in muscle soreness after running a marathon (Liang et al, 2001) - significant gesic effect without noticeable side effects or cause dependence (Shevchuk, 2008) - improved quality of sleep (Onen et al, 1994) - inhibition of purine metabolism (Brenke et al, 1994) - long-term antioxidative adaptation (Brenke et al, 1994) - improved tone of the skin and muscles (Mergeay et al, 1990) - decrease of uric acid level in blood plasma (Brenke et al, 1994) - reduction in uremic pruritus (a major problem for patients with end-stage renal disease) (Zucker et al, 2003) - increase in brown fat cells or brown adipose tissue that protects from aging, fight obesity, diabetes, heart disease, etc. (Kanzleiter et al,2005; Mattson, 2010). Additional and the main factors relate to improvements in breathing parameters: slower and lighter breathing and higher body oxygen content. For references and Cold Shower Benefits, Rules and RESTRICTIONS, visit ... - Thrombotic Thrombocytopenic Purpura...Yesterday, Today and Tomorrow Thrombotic Thrombocytopenic Purpura...Yesterday, Today and Tomorrow This ppt powerpoint presentation is uploaded by: Medical powerpoint search engine. - Disgorge-The Vile Sores In Urticariothrocisism Goulashed Decrepitance Artist:Disgorge Album:Chronic Corpora Infest Year:1998 Lyrics: Grotesquely pathologyc reabsorbed Artrosis by craps ventriculloyds Exfoliative erosive ganglium The turfs makes lupose Exantematic tifea Mist in furst acra Diseminosuture in my croup lunch The juices sacarates in arormalism My saliva exagerates one lordosism And makes the tummefaccied complicates Vilism And flicteaned sorigness Chomping on your safenectomy Decrepitate in engendrance adherent Somation by exterior endo spams Sub cutaneon rectalism goulashing Spiro chaeta morsus incarnis soma Saturates my sacrophagy sodomy On your sores my vulgar taste grows Fragments on varicose oblation Internous lidistrophia in crhonicism Uremic dolours on meningo cephallia Evolutive decrepitance incongenity Aperture insides malformed glaucoma In procedence of tracto aqueous Facial precognized neuralgy Ecto acridic nauseo transform Convulsive digesting Blists embrionary localized Oncogerms fenotype on stygma Clonal in general ventriculate Rabdosarcoma feast on fungoids Seccioning and transfixioning the carcasse Malformed of neonal emancipate Infeccionous acrid lessions Cavities now on genito urinary trastorn Fractured neoprovertebrous sericid Femoro cuteanized in cronaxium Vulgarism phido spasmic pleurasy Engorging flemonose disrupt Complex of incercioned enzimopathy Cavernose collesteatomized Exfoliative teratome in plasmose cava Micose ifostamidedenigrate In your coryo the flebitys engendred Tract dolours on your nephria ... - Tell Me a Story: Dad Gives Son a Precious Gift--a Kidney Preston Henson was a toddler when his parents realized his illness on a family vacation was more than a run-of-the-mill virus. It was E. coli food poisoning that quickly worsened into a disease that began destroying his kidneys. Doctors diagnosed Preston with hemolytic uremic syndrome — known as HUS. Without a kidney transplant, doctors said Preston would need to undergo dialysis for a lifetime. His dad, Gerald, made the decision to donate one of his kidneys, and the transplant was successful. Preston is now a healthy 4-year-old who takes two medications a day and is as active as any preschooler. In this "Tell Me a Story," listen to the Hensons talk about how the decision changed their lives. - Hemolytic Uremic Syndrome (HUS) Ludovivo Guarini MD Pediatric Hematology/ Oncology Residency: Beth Israel Medical Center New York Fellowship: College of Physician and Surgeons Columbia University Produced by - Concurrent CRRT and plasmapheresis This is a video that demonstrates the technique of concurrent continuous renal replacement therapy and therapeutic plasma exchange (CCRRT+TPE). CCRRT+TPE may be useful in the treatment of children with fulminant hepatic failure, hemolytic uremic syndrome or patients with immune-mediated acute renal failure. The video was filmed by Kim Yaeger,BSN in the Center for Advanced Pediatric Education (CAPE) at Stanford University. The video was presented by Dr Peter Yorgin at the 2006 CRRT Conference in San Diego. - frequent flyer 2 music-pal shazar/vid/sherry/ The Platinum Blonde The Blonde Bombshell : Jean Harlow died from uremic poisoning from renal failure and died of cerebral edema at age 26. the cost of using caustic carcinogenic chemicals via her hair coloring dyes for platinum and the indelible inks for drawn on high-brows are absorb daily into her pores. amazing how much she accomplished in her brief 26 years of life and also only 5'1" bless her heart. and bless pal shazar's heart for this song. - ImPACKD on Rogers Ottawa with Marie-Eve Chainey Marie-Eve Chainey is a third year nursing student at the university of Ottawa. Marie-Eve works as a fitness trainer and is a competitive high jumper. She spent her time volunteering for numerous organizations and has been on dialysis since she was 18. In the fall of 2001, Marie-Eve left her hometown of Kapuskasing, Ontario to attend an exchange program in Spain; to learn a new language and continue her high jump training. After four months abroad, Marie-Eve experienced a chain of severe headaches, dwindling energy and noticeable weight gain. After a series of tests, doctors concluded that Marie-Eve had dual kidney failure due to a rare blood disorder called Atypical Hemolytic Uremic Syndrome (AHUS). Although Marie-Eve is very lucky to have access to nocturnal hemodialysis at home, she still finds travelling very difficult and after five operations fears that her fistula will stop working. Like many CKD patients, independence is what Marie-Eve works towards everyday. "There are many issues surrounding kidney disease that are not being addressed and with the formation of ImPACKD we will to finally give a voice to the CKD community." - E. coli Lawsuit Attorney for Complications of E coli and HUS E. coli lawsuit information is provided by Brendan Flaherty, an attorney with PritzkerOlsen, PA ( ), a national food safety law firm. Mr. Flaherty works with Attorney Fred Pritzker ( http ), who speaks nationally at attorney seminars, food safety conferences and law schools about food litigation and building a winning case for a food poisoning victim. Mr. Flaherty and Mr. Pritzker represent E. coli victims throughout the United States, including those who have developed life threatening complications of E. coli: 1. hemolytic uremic syndrome (HUS) - 2. thrombotic thrombocytopenic purpura (TTP) - To contact attorneys Brendan Flaherty and Fred Pritzker about an E. coli lawsuit involving personal injury or wrongful death, call 1-888-377-8900 (toll free). - Jean Harlow -Falling Star(ronstadt)Happy 100th Birthday Harlow Centennial March 3 Beautiful Linda Ronstadt song Fallen Star tagged to pics of the ever beautiful ever young Jean Harlow,always in our hearts.March 3 marks the 100th year anniversery of the birth of the forever beloved Superstar,born March 3, 1911, as Harlean Carpentier in Kansas City. Originally wealthy, Harlean nicknamed the baby was doted on by her mother, Mama Jean. At 16, Harlean married the well off Charles MacGraw, but marriage ended when Mama Jean decided to move with the baby to Los Angeles, after the Senior Jean had failed at stardom. Harlean landed roles in a Laurel and Hardy and in the woman whom she would replace as Americas Goddess the gorgeous Clara Bow in The Saturday Night Kid. The elder star liked the youngster,seen unflatteringly in her bit role. After several extra background roles, Harlow was discovered when multimillionaire Howard Huges decided to make his then most expensive film ever, "Hell's Angels"into a talkie and German actress Greata Nissens voice was unsuitable for the role. Harlow's carefree uality actuality,gorgeous face and magnificent body made her the most famous woman in America. Five films in 1931 followed , all hits, including The Public Enemy with James Cagney,but the public felt Harlow couldnt act. Hughes sold her contract to MGM, and she made a promising first appearance with a total done over heavily made up look in the B, Beast of the City. Harlow's sensational teaming with Clark Gable in "Red Dust" made her a superstar, and she stole the ... - Hemolytic Uremic Syndrome (HUS): Tracking an Epidemic of E-coli Poisoning View this Lecture for FREE by signing up at Viewany number of our other 28000 sessions from over 280 conferences by going to Speaker(s) Nancy McAfee, MN, RN, CNN Hemolytic Uremic Syndrome (HUS): Tracking an Epidemic of E-coli Poisoning - American Nephrology Nurses Association National Symposium 2008 Nephrology Nursing and Evidence-Based Practice: In Harmony to Improve Patient OutcomesRemember, if you attended this meeting you have Free Access to this content! Click here for more information.All invited faculty members and planning committee members participating in an ANNA-sponsored program are required to disclose any real or apparent conflict(s) of interest that may have a direct bearing on the subject matter of the educational activity. To review speaker disclosures, click hereThe presence of any product, company, or corporation in any recording or content in no way signifies an endorsement of the product, company or corporation by ANCC Commission on Accreditation or the American Nephrology Nurses' Association (ANNA).Contact Hours available through: 6/1/2010Please note: Learners may earn contact hours (for recertification or relicensure) for each educational activity once only. If you attended a session at a live meeting (National Symposium, Fall Meeting, Audio Conference, etc.), you cannot repeat and claim credit for that session again. While the session is now offered online in a different format, the educational content is ... - Faith Healing Death - Oregon City Followers of Christ Church The death of a 16-year-old Oregon boy being treated by a faith healer could have easily been prevented according to a state medical examiner. The 16-year-old member of a faith healing church died of uremic heart failure caused by a stricture in his urinary tract, according to autopsy results. The condition could have been treated with a catheter in a routine outpatient procedure. Instead, 16-year-old Neal Beagley's urinary tract became inflamed and closed off. He was unable to urinate, which made his bladder and kidneys stop functioning, the autopsy showed. Toxins backed up into his blood stream. Beagley had started complaining about stomach aches and shortness of breath last week. The family told police he did not want medical attention. Gladstone police said relatives and church members told them the **ager refused treatment for the illness, as he was entitled to do under Oregon law. The family belongs to the Oregon City Followers of Christ Church. Like all members of the religious order, Beagley did not receive medical care. His condition worsened Sunday and members of the church gathered for prayer, Benton said. The church is a fundamentalist Christian denomination that recently made headlines after two members were arrested and accused of using prayer instead of medical care to try to cure their deathly ill daughter. In March, the boy's 15-month-old cousin, Ava Worthington, died at home from bronchial pneumonia and a blood infection. Her parents, Carl and Raylene ... - Have a Blythe Xmas! Blythe, everyone's favorite doll from the 70's with uremic leontiasis osse, helps Japanese department store Parco celebrate Christmas. Styled by the fabulous Gina Garan. Blogs & Forum blogs and forums about uremic “Hemolytic Uremic Syndrome Outbreak: Lawyer and lawsuit information. Pritzker Olsen Law Firm Food Safety Blog. Pritzker Olsen attorneys have appeared on CBS News, Fox news, and numerous local television stations throughout the country” — Hemolytic Uremic Syndrome Outbreak, “Uremic Frost. The Nephrology Blog. Tuesday, October 13, 2009. Kidney Public Policy Forum. The Renal Support Network has sponsored a forum dedicated to the CMS Proposed Rule for CKD / dialysis. Comments are due by November 16th. Please log on and contribute prior to this landmark ruling” — Uremic Frost: Kidney Public Policy Forum, “"It's just hard to put into words," says the grandmother, Sue Henderson, of a Corning, California girl sickened by E. coli O157:H7. "Yo Add this blog to your feeds or subscribe by email More Request Info. See our list of services or enter your information below to send us a quick message and we'll” — Northern California girl recovering from hemolytic uremic, “Hemolytic uremic syndrome is a condition in which the red blood cells disintegrate and lead to problems in the normal functioning of the kidneys and renal failure. Mostly affects children below the age of 10 years” — Hemolytic Uremic Syndrome in Children \| Symptoms \| Treatment, home-remedies-for- “Hemolytic Uremic Syndrome (HUS) induced kidney injury by Shiga-Toxin E. coli - Guest Blog - Bruce T. Clark, JD Uremic Syndrome (HUS) induced kidney injury by Shiga-Toxin E. coli - Guest Blog - Bruce” — Hemolytic Uremic Syndrome (HUS) induced kidney injury by, “hemolytic uremic syndrome. Post Reply. Author. 2 Posts. asmi. Forum Hero. Topics: 1049. Posts: 4, Similar forum topics. hemolytic uremic syndrome. Hemolytic jaundice” — hemolytic uremic syndrome, prep4 “Welcome to the Elaine Moore Graves' and Autoimmune Disease Education site for the support and education of patients with Graves' Disease and other autoimmune disorders. Here you'll learn about the autoimmune nature, symptoms, diagnosis and” — Elaine Moore Graves' and Autoimmune Disease Education > My, elaine- “Healthchannels forum, An online health community from , Inc. Discuss Hemolytic Uremic Syndrome with others. 6 Topics. Last post by TessaP. on Thu Aug 07, 2008 7:41 pm. Published Stories - Read Entries. Read published stories submitted by other browsers. 2 Topics. Last post” — Hemolytic Uremic Syndrome: - Nephrology Channel, related keywords for uremic - uremic poisoning - uremic frost - uremic syndrome - uremic encephalopathy - uremic pericarditis - uremic bleeding - uremic fetor - uremic breath - uremic pruritus - uremic toxins - uremic poisoning brought on by acute nephritis - uremic poisoning pregnancy - uremic poisoning acute nephritis - uremic poisoning in dogs - uremic poisoning cats - uremic poisoning jean harlow - uremic poisoning symptoms - uremic poisoning causes - uremic poisoning in cats - uremic poisoning death	2	18	2	0	0	1	0.703616	3	8,926
Breast and colon cancer are the leading causes of cancer deaths in the United States right after lung cancer. However, there are screening procedures available to diagnose these cancers in the earliest stages. This article provides instructions for coding the diagnoses and procedures for screening malignant neoplasm of the breast and the colon. Before reviewing these instructions, let's first review the official guidelines for coding the diagnoses for all screening procedures. Screening is the testing for diseases in seemingly well individuals so that early detection and treatment can be provided for those who test positive for the disease. If testing is performed because the patient has a sign or symptom, the sign or symptom code is used to explain the reason for the test, not the screening code. A screening code may be the first listed code if the reason for the visit is specifically the screening exam. It may also be used as an additional code if the screening is done during an encounter for other health problems. If a condition is discovered during the screening, the code for the condition may be assigned as an additional diagnosis. The following V code categories are reported to indicate that a screening exam is planned. A procedure code is also required to confirm that the screening was performed. V28 Antenatal screening V73-V82 Special screening examinations When the reason for performing a test is because the patient has had contact with, or exposure to, a communicable disease, the appropriate code from category V01, Contact with or exposure to communicable diseases, should be assigned, not a screening code. Every woman is at risk for developing breast cancer, and the risk increases with age. According to the Center of Disease Control and Prevention (CDC), approximately 94 percent of breast cancers are diagnosed in women older than age 40. Mammography is the best available way to detect breast cancer early, when it is most curable. Timely screening can reduce breast cancer mortality in women 40 and older by 17 percent to 30 percent. The National Cancer Institute (NCI) guidelines for screening mammography recommend that asymptomatic women 40 years or older be screened every 1 to 2 years and women aged 50 or older be screened every 1 to 2 years. NCI further recommends that younger women who are at higher risk for developing breast cancer consult with their physician regarding screening mammography and the frequency of such screenings. Diagnostic mammograms differ from screening mammograms. Screening mammograms are for patients without documented problems. Diagnostic mammograms are performed when there is a problem such as a breast mass, pain, discharge, etc. Code any positive findings found on the diagnostic mammogram as the first listed diagnoses. If there are no reported findings, assign the reason for the test. The diagnoses codes for encounters for screening mammogram are V76.11 and V76.12. V76.12, Special screening for malignant neoplasm, other screening mammography Example: A healthy 40-year-old woman presents to the radiology department for a screening mammogram. The patient has no symptoms or known risks for breast cancer. Assign code V76.12. V76.11, Special screening for malignant neoplasm, screening mammogram for high-risk patients. The following codes may be assigned 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 Example: A woman with no symptoms is referred to the hospital for screening mammogram. The patient is considered high risk for breast cancer secondary to family history of breast malignancy in the mother and sister. Assign code V76.11 followed by code V16.3. If a condition is found during the screening, then the code for the condition may be used as an additional diagnosis. The rationale for this is that even though a condition is found during the mammography, the visit is still considered a screening. For Medicare services, diagnosis codes V76.11 and V76.12 must be the first listed diagnosis on all encounters for screening mammography services. However, effective Oct. 1, 2006, this requirement will change to allow the reporting of any applicable diagnosis code as a primary diagnosis on claims containing other services in addition to a screening mammography. Continue reporting diagnosis codes V76.11 and V76.12 as the first listed diagnosis codes on claims that contain only screening mammography services. A mammogram is a low-dose X-ray of the breast that can find lumps that are too small to be felt during a breast examination. The breast is compressed firmly between two planes and pictures are taken. This spreads the tissue and allows for a lower X-ray dose. 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 then a diagnostic mammogram is performed and coded instead. A screening mammogram is inherently bilateral and is reported with codes 76092 and G0202. 76092, Screening mammography, bilateral (two view film study of each breast) G0202, Screening mammography, producing direct digital image, bilateral, all views Code 76083, Computer aided detection (CAD); screening mammography, may be assigned as an additional procedure code when it is performed in addition to the primary procedure. The additional CAD code indicates that a laser beam was used to scan the mammography film and then the image was converted to digital data for computer analysis. As mentioned earlier, if a condition is discovered during the screening, then the code for the condition may be used as an additional diagnosis. In this instance, if the radiologist performing the mammogram orders additional films based on the condition discovered during the screening mammogram, both may be coded. When a screening mammogram is converted to a diagnostic mammogram on the same day 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 that 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 which side the diagnostic mammogram was performed on. Colorectal cancer is one of the leading causes of cancer deaths in the United States. Approximately 56,290 people died from colorectal cancer, and 145,290 people were newly diagnosed with the disease in 2005. Colorectal cancer is usually found in people ages 50 and older. Therefore, screening for colorectal cancer for people ages 50 and older is strongly recommended. Colorectal cancer can be prevented and treated through routine screening and early detection. The ICD-9-CM diagnosis code for an average risk patient presenting for a screening colonoscopy is V76.51, Special screening for malignant neoplasm, colon. A screening colonoscopy may also be performed for patients considered to be a high risk for colon cancer. Code V76.51 should be the first listed diagnosis code if the reason for the visit is specifically for the screening exam. For high risk patients, the appropriate family or personal history V code identifying the risk should also be assigned. As with screening for malignant neoplasm of the breast, if a condition is discovered during the screening then the code for the condition may also be assigned as an additional diagnosis. If the colonoscopy is performed because the patient has a sign or symptom, the sign or symptom code is used to explain the reason for the test, not the screening code. However, if positive findings are discovered during the diagnostic colonoscopy assign the code for these findings instead. Colorectal cancer screening tests and procedures can be used alone or in various combinations and include fecal blood test, barium enema, flexible sigmoidoscopy and colonoscopy. Colonoscopy screening procedures are discussed here. A colonoscopy is considered the gold standard for colorectal cancer screening. After the patient's bowel has been prepped, the physician inserts the colonoscope-a long, thin, flexible lighted tube-through the anus and advances the scope through the colon past the splenic flexure. The lumen of the colon and rectum is visualized. Most polyps and some cancers can be removed during this procedure. The colonoscope is then withdrawn. HCPCS Level ll codes G0105 and G0121 should be reported for Medicare outpatients requiring screening colonoscopy for colorectal cancer. G0121, Colorectal cancer screening, colonoscopy on individual not meeting criteria for high risk G0105, Colorectal cancer screening, colonoscopy on individual at high risk According to Medicare, a patient is considered to be at high risk if he or she has any of the following risk factors: close relative (sibling, parent or child) who has had colorectal cancer or an adenomatous polyp; family history of familial adenomatous polyposis; family history of hereditary nonpolyposis colorectal cancer; personal history of adenomatous polyps; personal history of colorectal cancer; inflammatory bowel disease, including Crohn's disease and ulcerative colitis. Screening colonoscopy for non-Medicare patients is coded with 45378, Colonoscopy, flexible, proximal to splenic flexure, diagnostic. If an abnormality is found during a screening colonoscopy and results in a therapeutic procedure, then the appropriate diagnostic colonoscopy CPT code (45379-45392) is used instead of codes G0105, G0121 or 45378. Therapeutic procedures include biopsy, polypectomy, etc. Review the CCS Prep column titled, "Understanding How to Code Colonoscopies" for instructions on coding therapeutic procedures. Example: A patient is seen in the outpatient clinic for screening colonoscopy due to family history of colon cancer. The colonoscopy revealed a colonic polyp that was removed by snare technique. Assign CPT code 45385. For Medicare OPPS coding, when a screening colonoscopy is attempted but due to extenuating circumstances cannot be completed, code G0105 or G0121 should be reported with either modifier -73 or -74 as appropriate. 1) A patient with a family history of colon cancer is seen in the hospital outpatient department for a screening colonoscopy. During the procedure, two polyps are discovered and removed by electrocautery snare. How should the diagnoses and procedures for this case be coded? a. V76.51, V16.0 45385 b. V76.51, 211.3, V16.0, 45385 c. 211.3, V16.0, 45385 d. V76.51, 211.3, V16.0 G0105 2) An asymptomatic 65-year-old woman has 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, 76092 b. 611.72, 76092 c. V76.12, 76092 d. V76.11 611.72, 76091 3) 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, 76092 b. 611.72, 76092, 76083GG, G0206 c. V7611, 611.72, V16.3, 76083, G0206 d. V7611, 611.72, V16.3, 76083GGRT, G0206 This month's column has been prepared by Cheryl D'Amato, RHIT, CCS, director of HIM, and Melinda Stegman, MBA, CCS, manager of clinical HIM services at HSS, an Ingenix company (www.hssweb.com). HSS specializes in the development and use of 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. Answers to CCS Prep!: 1) b. Assign code V76.51, Special screening for malignant neoplasm, colon, as the first-listed diagnosis because this was a screening colonoscopy. V16.0 is assigned for the family history of colon cancer. Assign code 211.3, Benign neoplasm of colon as an additional diagnosis. Procedure code 45385 is reported instead of one of the screening colonoscopy codes because a definitive procedure, snare removal of polyp, was performed; 2) 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 X-ray finding of the breast mass. Procedure code 76092 is assigned to identify the performance of the screening mammography; 3) 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 76092 is reported for the bilateral screening mammogram. Procedure codes 76083 and G0206 are assigned for the diagnostic mammogram. Modifiers GG and RT are appended to code 76083 to indicate that the screening mammogram was converted to a diagnostic mammogram of the right breast.	4	14	16	0	13	21	0.814748	50	2,967
Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful. The ICD-10 (World Health Organization 1992) defines a personality disorder as: a severe disturbance in the characterological condition and behavioural tendencies of the individual, usually involving several areas of the personality and nearly always associated with considerable personal and social disruption. In the American Psychiatric Association's DSM 4th edition (DSM-IV), a personality disorder was defined as: an enduring pattern of inner experience and behaviour that differs markedly from the expectations of the individual's culture, is pervasive and inflexible, has an onset in adolescence or early adulthood, is stable over time and leads to distress or impairment. Personality disorders are a long-standing and maladaptive pattern of perceiving and responding to other people and to stressful circumstances. The DSM 5th edition (DSM-5) takes an entirely different approach to personality disorder and has stimulated debate about how the condition should be diagnosed and categorised. It acknowledges that such an approach may well be difficult to use in a clinical situation, which has led to proposals for hybrid and compromise systems. As mentioned above, this article relies mainly on the ICD-10 definition. The aetiology of personality disorders remains obscure. Traditional belief is that these behaviours result from a dysfunctional early environment that prevents the evolution of adaptive patterns of perception, response and defence. Factors in childhood which are postulated to be linked to personality disorder include: - Sexual abuse - Physical abuse - Emotional abuse - Being bullied Emotional or behavioural factors that might play a part include: - Bullying others. - Being expelled/suspended. - Running away from home. - Deliberate self-harm. - Prolonged periods of misery. The evidence base supporting a link between personality disorder and genetic factors is growing. People with personality disorders are at increased risk for many psychiatric disorders. Mood disorders are a particular risk across all personality diagnoses. Patients with depression and personality disorder have a more persistent condition than those who have depression alone.Some types of mental illnesses are more specific to particular personality disorders. It is unsurprising from the above that many people with personality disorders offend against the law. The ICD-10 gives nine categories of personality disorder. In DSM-IV there are ten personality disorders that are divided into three clusters, designated A, B, C. The ICD-10 classification, listed under the code F60, is as follows: F60 Specific personality disorders - F60.0 Paranoid personality disorder. - F60.1 Schizoid personality disorder. - F60.2 Dissocial personality disorder. - F60.3 Emotionally unstable personality disorder: - .30 Impulsive type - .31 Borderline type - F60.4 Histrionic personality disorder. - F60.5 Anankastic personality disorder. - F60.6 Anxious (avoidant) personality disorder. - F60.7 Dependent personality disorder. - F60.8 Other specific personality disorders including: - "Haltlose" type - F60.9 Personality disorder, unspecified - this encompasses character neurosis and pathological personality disorder. These are the main types, although ICD-10 subdivides some categories into further subtypes. ICD-10 also identifies an additional category, 'Unspecified personality disorder'. Studies estimate that personality disorder affects 4-11% of the UK population and between 60-70% of the prison population.This is so common as to be almost a variation of normal rather than pathological. Many of the features we can possibly recognise in ourselves or others but, often, several features are required to make a diagnosis. In the prison population there are probably comparatively few who do not have at least one of personality disorder, mental illness, learning difficulties and substance abuse. International figures must be viewed with caution, as the diagnosis is highly dependent upon culture. A UK study (albeit using the DSM-IV categories) reported that rates were highest among men, and separated and unemployed participants in urban locations. It identified a subgroup of people who tended not to use local health services but drifted from childcare services to the criminal justice system at an early age. A study of psychiatric patients managed by mental health teams showed that in total, 40% of all patients in secondary care had at least one personality disorder suggesting a significant degree of comorbidity with other psychopathology. Personality disorders should not normally be diagnosed in children and adolescents because the development of personality is incomplete and symptomatic traits may not persist into adulthood. However, trajectory studies suggest that some adults with personality disorder have traits which can be detected in childhood.It has been traditionally considered that the diagnosis of personality disorder in the elderly should be made with caution, due to the attenuation of symptoms, failure to comply with the old DSM criteria and the difficulties of separating the symptomatology of dementia. However, more recent meta-analyses suggest that personality disorder may be a valid diagnosis, particularly in some elderly patients with depression. Further research is needed. Personality disorders present with a wide range of problems in social relationships and regulation of mood. Such individuals have usually been like it throughout their adult lives. The patterns of perception, thought and response are fixed and inflexible, although their behaviour is often unpredictable. These patterns do not adhere to their own culture's expectations. The ICD-10 criteria for clinical diagnosis refer to conditions not directly attributable to gross brain damage or disease, or to another psychiatric disorder, which meet the following criteria: - Markedly disharmonious attitudes and behaviour, involving usually several areas of functioning - eg, affectivity, arousal, impulse control and relationships with others. - The abnormal behaviour pattern is persistent, lasts for a long time and is not limited to episodes of mental illness. - The abnormal behaviour pattern is widespread and obviously maladaptive to a broad range of personal and social situations. - The above manifestations always appear during childhood or adolescence and continue into adulthood. - The disorder leads to considerable personal distress but this may only become apparent at a later stage. - The disorder is usually, but not always, associated with significant difficulties with work and social relationships. Clinically significant distress or impairment must occur in all settings and not be limited to one area only. - Common presenting features are as follows: - Paranoid - they display pervasive distrust and suspicion. Common beliefs include: - Others are exploiting or deceiving them. - Friends and associates are untrustworthy. - Information confided to others will be used maliciously. - There is hidden meaning in remarks or events others perceive as benign. - The spouse or partner is unfaithful. Pathological jealousy is sometimes called the Othello syndrome. - Schizoid - this is characterised by withdrawal from affectional, social and other contacts. This type of person is isolated and has a limited capacity to experience pleasure and express feelings. - Dissocial - there is a tendency to act outside social norms, a disregard for the feelings of others and an inability to modify behaviour in response to adverse events (eg, punishment). A low threshold for violence and a tendency to blame others may be features. - Emotionally unstable - people with this personality disorder tend to be impulsive and unpredictable. They may act without appreciating the consequences. Outbursts of emotion and quarrelsome behaviour may be exhibited. Relationships tend to be unstable and there may be suicidal gestures and attempts. - Histrionic - this is characterised by shallow and labile affectivity and theatricality. There is lack of consideration for others and a tendency for egocentricity. People with this type of personality often crave excitement and attention. - Anankastic - this is characterised by feelings of doubt, perfectionism and excessive conscientiousness. There is a compulsion to check and a preoccupation with details. This personality type tends to be stubborn, cautious and rigid. Insistent and unwelcome thoughts may intrude or impulses that do not attain the severity of an obsessive-compulsive disorder. - Anxious (avoidant) - this is characterised by feelings of tension and apprehension, insecurity and inferiority. People with this type yearn to be liked and accepted, are sensitive to rejection. There is a tendency to exaggerate potential dangers and risks, leading to an avoidance of everyday activities. - Dependent - this is characterised by a reliance on others to take decisions and a fear of abandonment. There is an excessive reliance on authority figures and difficulty in acting independently. This can affect the capacity to deal with the intellectual and emotional demands of daily life. There are no physical abnormalities to help diagnose personality disorders but there may be findings related to the consequences of various personality disorders. - Those especially with emotionally unstable personality disorders may show signs of intentional self-harm or stigmata of substance abuse. There may be scars from self-inflicted wounds. - Substance abuse is common and may present the physical stigmata of alcoholism or drug abuse. - Patients with histrionic personality disorder may display 'la belle indifférence', a seemingly indifferent detachment, while describing dramatic physical symptoms. - A hostile attitude is typical of patients with dissocial personality disorder. - Patients with paranoid personality disorder voice persecutory ideation without the formal thought disorder observed in schizophrenia. - Patients with schizoid personality disorder speak with odd or idiosyncratic use of language. Psychological testing may support or direct the clinical diagnosis. - The Minnesota Multiphasic Personality Inventory (MMPI) is the best-known psychological test. - The Eysenck Personality Inventory and the Personality Diagnostic Questionnaire are also used. - A structured psychometric assessment - this is particularly relevant to dissocial and emotionally unstable personality disorders. The National Institute for Health and Care Excellence (NICE) has published guidance on the treatment, management and prevention of antisocial personality disorder and borderline personality disorder. This maps to the ICD-10 categories of dissocial and emotional unstable personality disorder respectively and so remains relevant. NICE has also published quality standards advice aimed at commissioners of mental health services.This provides guidance on a number of diagnostic and management issues. It used to be taught that psychopaths and personality disorders were untreatable. Psychotherapy is the basis of care for personality disorders. Personality disorders produce symptoms as a result of poor or limited coping skills. Therefore, psychotherapy aims to improve perceptions of and responses to social and environmental triggers.[12, 15] - Psychodynamic psychotherapy examines the ways that events are perceived. It states that perceptions are shaped by experiences in early life and therapy aims to identify perceptual distortions and their origin and to facilitate the development of more adaptive modes of perception and response. Treatment is usually prolonged over a course of several years at intervals from several times a week to once a month. It uses transference. - Cognitive and behavioural therapy (CBT) suggests that cognitive errors based on long-standing beliefs influence the meaning attached to interpersonal events. It explores how people think about their world and their perception of it. It is a very active form of therapy that identifies the distortions and engages the patient in efforts to reformulate perceptions and behaviours. This therapy is usually limited to episodes of 6 to 20 weeks at intervals of once a week. For personality disorders, therapy is repeated often over the course of years. - Interpersonal therapy (IPT) assumes that difficulties result from a limited range of interpersonal problems, including such issues as role definition and grief. Current problems are interpreted narrowly through the screen of these formulations and solutions are framed in interpersonal terms. Therapy is usually weekly for a period of 6 to 20 sessions. It is used mostly for anxiety and depression and is not widely practised. - Group psychotherapy allows interpersonal problems to be displayed among a peer group, whose feedback is used by the therapist to identify and correct maladaptive ideas, communication and behaviour. Sessions are usually once weekly over a course that may range from several months to years. The technique enables several people to be treated simultaneously, reducing cost per patient. - Dialectical behavioural therapy (DBT) is a skills-based therapy that can be used in both individual and group formats. It has been applied to borderline personality disorder. The emphasis is on the development of coping skills to improve affective stability and impulse control and on reducing self-harmful behaviour. This treatment is also being used with other cluster B personality disorders, to reduce impulsive behaviour. Drugs do not cure personality disorders but they may be useful as an adjunct so that the patient may productively engage in psychotherapy. The focus is on treatment of symptom clusters such as cognitive-perceptual symptoms, affective dysregulation and impulsive-behavioural dyscontrol. Such symptoms may complicate almost all personality disorders to some degree but all of them have been noted in borderline personality disorder. NICE recommends that antipsychotic or sedative medication such as benzodiazepines should only be used for short‑term crisis management or treatment of comorbid conditions in dissocial or emotionally unstable personality disorder. - A Cochrane systematic review found that mood stabilisers and second-generation antipsychotics may be helpful for specific symptoms in borderline personality disorder but that pharmacotherapy did not affect the overall severity of the condition. - Anticonvulsants help to stabilise the affective extremes in patients with bipolar disorder but are less effective for that purpose in patients with personality disorders. They have some benefit in suppressing impulsive and particularly aggressive behaviour in patients with personality disorder. - Some personality disorders, especially borderline personality disorder, produce transient psychotic periods, while others such as schizoid personality disorder show chronic idiosyncratic ideation bordering on psychosis. Response to antipsychotics is less dramatic than in true psychotic disorders but symptoms such as anxiety, hostility and sensitivity to rejection may be reduced. Antipsychotics are normally used for a short period while the symptoms are active. The atypical antipsychotics have almost completely replaced the older neuroleptics because of their margin of safety but neurological side-effects including tardive dyskinesia and neuroleptic malignant syndrome do sometimes occur. Risperidone, olanzapine and aripiprazole are often used. There is no evidence of superior efficacy of any product and each one may have advantages and disadvantages of adverse effects. Consult the patient's crisis plan (a plan devised to identify trigger factors, advise on self-help strategies and identify when the individual should seek professional help).Assess problem and risk - Maintain a calm and non-threatening attitude. - Try to understand the crisis from the person's point of view. - Explore the person's reasons for distress. - Use empathetic open questioning, including validating statements, to identify the onset and the course of the current problems. - Seek to stimulate reflection about solutions. - Avoid minimising the person's stated reasons for the crisis. - Wait for full clarification of the problems before offering solutions. - Explore other options before considering admission to a crisis unit or inpatient admission. - Offer appropriate follow-up within a timeframe agreed with the person. - Assess risk to self or to others. - Ask about previous episodes and effective management strategies used in the past. - Help to manage their anxiety by enhancing coping skills and helping them to focus on the current problems. - Encourage them to identify manageable changes that will enable them to deal with the current problems. - Offer a follow-up appointment at an agreed time. - Levels of distress and/or the risk of harm to self or to others is increasing. - Levels of distress and/or the risk of harm to self or to others has not subsided despite attempts to reduce anxiety and improve coping skills. - Patients request further help from specialist services. The following may occur more often than expected: Frequent enquiries about suicidal ideation are warranted, regardless of whether the patient spontaneously raises the subject.There is no risk of implanting the idea of suicide in a patient who is not already considering it. Enquiry about drugs and other available means of suicide may help prevention. Patients with personality disorder who have children should be asked frequently and in detail about their parenting practices. Their low frustration tolerance, externalisation of blame for psychological distress and impaired impulse control put the children of these patients at risk of neglect or abuse. The NICE guidance puts some emphasis on identification of individuals at risk of developing personality disorders.A variety of interventions is suggested to try to prevent some of the consequences of the personality disorders covered by this guidance. For example, NICE suggests that services should establish robust methods to identify children at risk of developing conduct problems and that vulnerable parents could be identified antenatally - for example, in antisocial personality, by identifying: - Parents with other mental health problems, or with significant drug or alcohol problems. - Mothers younger than 18 years, particularly those with a history of maltreatment in childhood. - Parents with a history of residential care. - Parents with significant previous or current contact with the criminal justice system. A wide variety of different interventions is then suggested, ranging from anger management to parenting classes. It is not uncommon for people with personality disorders to offend against the law and come into contact with the criminal justice system.They are often held to be untreatable. Treatment is prolonged, difficult and far from universally successful. When society is preoccupied with a punitive approach to offenders rather than the rehabilitation of offenders, the result is overcrowded prisons and recidivism amongst offenders. Jack Straw, when he was Home Secretary, wrote that one of the most important steps for the prevention of re-offending was that the person should secure a job. However, most employers enquire about criminal records and hold it against potential employees. The management of those with personality disorders, including those who have run foul of the law, is not easy and success is limited but the stakes are such that it is essential that society make the effort. The guidance from NICE poses challenges to the different agencies involved in the management and care of individuals with personality disorders.[13, 12]The NICE quality standards recommend that people with borderline or antisocial personality disorder should have their long‑term goals for education and employment identified in their care plan. Further reading and references Gask L, Evans M, Kessler D; Personality disorder. BMJ. 2013 Sep 10347:f5276. doi: 10.1136/bmj.f5276. Morey LC, Shea MT, Markowitz JC, et al; State effects of major depression on the assessment of personality and personality disorder. Am J Psychiatry. 2010 May167(5):528-35. doi: 10.1176/appi.ajp.2009.09071023. Epub 2010 Feb 16. Working with offenders with personality disorders - a practitioners guide; National Offender Management Service and NHS England (September 2015) Reichborn-Kjennerud T, Ystrom E, Neale MC, et al; Structure of genetic and environmental risk factors for symptoms of DSM-IV borderline personality disorder. JAMA Psychiatry. 2013 Nov70(11):1206-14. doi: 10.1001/jamapsychiatry.2013.1944. Skodol AE, Grilo CM, Keyes KM, et al; Relationship of personality disorders to the course of major depressive disorder in a nationally representative sample. Am J Psychiatry. 2011 Mar168(3):257-64. Epub 2011 Jan 18. Specific Personality Disorders - ICD-10 Version:2016; World Health Organization Coid J, Yang M, Tyrer P, et al; Prevalence and correlates of personality disorder in Great Britain. Br J Psychiatry. 2006 May188:423-31. Working with personality disordered offenders: A practitioners guide, 2011; Ministry of Justice Newton-Howes G, Tyrer P, Anagnostakis K, et al; The prevalence of personality disorder, its comorbidity with mental state disorders, and its clinical significance in community mental health teams. Soc Psychiatry Psychiatr Epidemiol. 2010 Apr45(4):453-60. doi: 10.1007/s00127-009-0084-7. Epub 2009 Jun 20. Vizard et al; The British Journal of Psychiatry May 2007, 190 (49) s27-s32. Rosowsky E et al; Personality Disorders in Older Adults: Emerging Issues in Diagnosis and Treatment, 2013. The ICD-10 Classification of Mental and Behavioural Disorders; World Health Organization Banerjee P et al; Assessment of personality disorder, Advances in psychiatric treatment (2009), vol. 15, 389–397. Borderline personality disorder: recognition and management; NICE Clinical Guideline (January 2009) Antisocial personality disorder; NICE Clinical Guideline (January 2009) Personality disorders: borderline and antisocial; NICE Quality Standard, June 2015 Kolly S, Kramer U, Maillard P, et al; Psychotherapy for Personality Disorders in a Natural Setting: A Pilot Study over Two Years of Treatment. J Nerv Ment Dis. 2015 Sep203(9):735-8. doi: 10.1097/NMD.0000000000000356. Lieb K, Vollm B, Rucker G, et al; Pharmacotherapy for borderline personality disorder: Cochrane systematic review of randomised trials. Br J Psychiatry. 2010 Jan196(1):4-12. Chesin MS, Jeglic EL, Stanley B; Pathways to high-lethality suicide attempts in individuals with borderline personality disorder. Arch Suicide Res. 2010 Oct14(4):342-62. hey. just started paroxetine today 10mg, just wondered if anyone that's taken it had any side effects? thanks.Sawyer9 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	8	1	0	0	11	0.702516	12	4,638
Purpose of Review Cardiac arrhythmias are known complications in patients with COVID-19 infection that may persist even after recovery from infection. A review of the spectrum of cardiac arrhythmias due to COVID-19 infection and current guidelines and assessment or risk and benefit of management considerations is necessary as the population of patients infected and covering from COVID-19 continues to grow. Cardiac arrhythmias such as atrial fibrillation, supraventricular tachycardia, complete heart block, and ventricular tachycardia occur in patients infected, recovering and recovered from COVID-19. Personalized care while balancing risk/benefit of medical or invasive therapy is necessary to improve care of patients with arrhythmias. Providers must provide thorough follow-up care and use necessary precaution while caring for COVID-19 patients. SARS-CoV-2, the severe acute respiratory syndrome coronavirus 2, responsible for COVID-19, has infected over 37 million people globally and almost 8 million people in the USA alone . While the primary symptoms of COVID-19 may be respiratory in nature for the vast majority of cases, several studies have pointed to extrapulmonary effects of the virus . This phenomenon is likely observed due to the cumulative effects of the hyperinflammatory response of the body and the omnipresence in major organs of the angiotensin-converting enzyme 2 (ACE2) cellular receptor that SARS-CoV-2 uses for cell entry . Of all systems which may be affected by the virus, possibly the most common extrapulmonary complications can be observed in the cardiovascular system, with these complications including myocardial injury, cardiomyopathy, acute coronary syndrome, cardiogenic shock, acute cor pulmonale, thrombotic complications, and arrhythmias [4••, 5]. Herein, we describe the signs, symptoms, and pathophysiology of cardiac arrhythmia in COVID-19 (Table 1). SARS-CoV-2 infection may cause deleterious cardiovascular effects manifested as cardiac enzyme release and a heightened systemic inflammatory response, which has noted to include elevations in ferritin, lactate dehydrogenase (LDH), C-reactive protein (CRP), and interleukin-6 (IL-6) . The mechanism of myocardial injury may be secondary to the immune response, elevated catecholamine, hypercoagulable state, and/or directly due to myocyte viral invasion [7, 8]. Indeed, post-mortem pathological studies have discovered myocardial tissue positive for SARS-CoV-2 by reverse transcription polymerase chain reaction (RT-PCR) and electron microscopy . However, there have been no detailed reports to date of post-mortem virus that have evaluated if those patients who died of a cardiac arrhythmia had viral infection in the cardiac conduction tissue such as the His-Purkinje system. The high incidence of arrhythmias in COVID-19 is thought to be multifactorial: hypoxemia due to acute respiratory distress, acute decline in cardiac hemodynamics, myocarditis, cardiac injury, prominent inflammatory response, direct viral invasion, and/or use of QT prolonging medications [10,11,12]. Early reports from Wuhan showed that 44% of patients admitted to the intensive care unit with COVID-19 had arrhythmias, raising initial suspicions of arrhythmias associated with COVID-19 . We discuss in this manuscript a perspective on the arrhythmia associated with COVID-19 and their management considerations (Fig. 1 and Table 2). Atrial arrhythmias are the most commonly reported arrhythmias in patients with COVID-19. Similarly, atrial fibrillation (AF)-related consultations were the most common electrophysiology consultations during the peak of the pandemic in New York City at Columbia University for COVID-positive patients (31%), with only 13% of these COVID-positive patients having a history of AF . In stark contrast with a typical AF population, none of these COVID-19 patients with new-onset atrial tachyarrhythmia had a history of cardiac surgery, ablation, cardioversion, or antiarrhythmic drug-use. The etiology of these atrial arrhythmias is yet to be fully understood, but proposed theories include alterations in ACE2-related signaling pathways, inflammation, direct viral endothelial damage, and metabolic derangements during the acute illness . Notably, AF has been associated with worse outcomes in patients who have acute respiratory disease. Prior to the COVID-19 era, patients with new-onset AF during acute respiratory syndrome (ARDS) and severe pneumonia had increased mortality compared to those ARDS patients without new AF . In Columbia University’s aforementioned consultation experience, 55% of patients in the small sample of nine expired by the end of the study, with the remaining hospitalized, reflecting the poor prognosis of patients with new onset of AF during COVID-19 infection . In another study, atrial arrhythmias were also more common among patients who needed mechanical ventilation (17.7% vs. 1.9% otherwise) . These studies highlight the importance of careful management considerations for these patients, especially those with new-onset AF. AF is typically treated with rate or rhythm control and also with anticoagulation in patients who meet criteria and do not have contraindications due to bleeding risk . For rate control, pharmacological management with diltiazem in patients with COVID-19 seemed to be common since there is concern that beta-blockers may cause bronchospasm during respiratory illness (Table 2). In COVID-19 patients with new-onset AF, Columbia University’s study reported the use of amiodarone in 29% of patients referred for electrophysiology consultation, with anticoagulant usage reported in 83% . It is unclear if early detection and treatment of COVID-19 may mitigate such arrhythmic cardiac complications. Furthermore, it is unclear if prophylactic use of antiarrhythmics in COVID-positive patients at risk of cardiac complications should be considered earlier on in the treatment, due to the increase in mortality in patients with arrhythmia . In contrast to atrial arrhythmias consisting of 31% of electrophysiology consults at the peak of the pandemic, ventricular arrhythmias made up a significantly smaller 7% of consults at the Columbia University Medical Center . Structural heart disease has historically been shown to be a risk factor for ventricular arrhythmias . We have previously reported ventricular tachycardia (VT) and ventricular fibrillation (VF) as the primary cause of death in COVID-19 patients without a prior history of structural heart disease . However, this could be due to the association of ventricular arrhythmias in patients who are critically ill . Several studies investigating cardiac arrhythmia most proximate to death for patients infected with COVID-19 describe VT and VF in 6% of deaths [24, 25]. Notably, the majority of COVID-19 deaths had asystole or pulseless electric activity (PEA) at time of death, likely due to respiratory failure or pulmonary embolism . VT/VF may also be due to cardiac ischemia or acute myocardial infarction as there have been studies suggesting increased thromboembolism in COVID-19 [26,27,28]. Furthermore, patients with myocarditis due to COVID-19 may have increased risk for sudden cardiac death although it is unclear how to risk stratify these patients [27,28,29]. The usage of antiarrhythmics for prophylaxis of VT/VF for patients during COVID infection is unclear, especially since it is still not known why some patient have cardiac arrhythmic complications and others do not. Usage of QT prolonging drugs, such as amiodarone, presents a possible risk of torsades de pointes (TDP) and nodal agents may cause bradycardia (Table 2) [30•]. Ventricular arrhythmias that arise in COVID-19 patients may lead to VT storm defined as 3 or more hemodynamically stable ventricular tachyarrhythmias in 24 h, VT recurring after termination of another episode, or sustained and nonsustained VT episodes exceeding normal beats within 24 h. Antiarrhythmic drug therapy may be effective using amiodarone and beta-blockers but may require deep sedation and hemodynamic support as well . For COVID-19 patients suffering from a VT storm, case studies of patients with new-onset ventricular arrhythmias have also shown the efficacy of substrate-based VT catheter ablation procedures if implantable cardioverter defibrillator (ICD) shocks prove futile. These case studies report patient recovery from COVID-19 without further ICD interventions . While the sample size is small, these ablation case studies may offer a promising alternative to the difficulties in applying proper drug-therapies to COVID-19 patients. Possible long-term effects of aggressive ablation strategy in a COVID-19 specific population are yet to be observed. Atrioventricular (AV) block may account for up to 12% of arrhythmias seen in patients with COVID-19 . While the mechanism behind this observation is not certain, heart block can be a manifestation of myocarditis , and myocarditis has been associated with the infection . Although several cases of COVID-19-related AV block have been reported in patients with preserved ventricular function and/or normal cardiac biomarkers [36,37,38], it is possible that these cases otherwise represent subclinical myocardial inflammation. In one such case, cardiac magnetic resonance imaging (MRI) revealed ventricular wall edema suggestive of myocarditis despite no evidence of myocardial injury . Moreover, AV block seen in the setting of acute infection can resolve spontaneously [39, 40]. Patients with COVID-19 and persistent high-grade AV block have been managed with standard-of-care pacemaker placement and outpatient follow-up (Table 2) [36,37,38]. However, the development of heart block in patients with COVID-19 has been suggested to be a poor prognostic sign, with many of the reported cases occurring in patients who ultimately succumbed to the disease [40,41,42,43]. Inappropriate Sinus Tachycardia The incidence of inappropriate sinus tachycardia (IST) in patients with COVID-19 is uncertain. By definition, IST is a diagnosis of exclusion. Therefore, it is very unlikely to be diagnosed in the setting of acute infection as patients with hypoxemia may be in sinus tachycardia. Persistent tachycardia after infection may represent as IST and has been shown in patients recovering from SARS, suggesting it may be seen in patients recovering from COVID-19 as well [44, 45]. The mechanism of IST is likely multifactorial including intrinsic sinus node hyperactivity, autonomic dysfunction, and a hyperadrenergic state [46•]. Inflammatory cytokines released by patients with COVID-19 may affect the function of myocardial ion channels and perpetuate tachyarrhythmia including sinus tachycardia . Ongoing symptomatic IST may be treated with beta-blockers and/or ivabradine (Table 2) , although treatment efficacy is unknown in patients with COVID-19. It is of note that ivabradine usage in IST is not FDA approved and is off label. Postural Orthostatic Tachycardia Syndrome Postural orthostatic tachycardia syndrome (POTS) is caused by autonomic dysfunction; the underlying mechanism of which may be related to peripheral neuropathy, increased serum norepinephrine, baroreceptor dysfunction, or hypovolemia [49, 50]. The syndrome has previously been reported to develop after acute stressors including viral illness and, therefore, may also develop in some patients recovering from COVID-19 [49,50,51]. Furthermore, dysautonomia including postural tachycardia have already been reported in COVID-19 patients [52, 53]. Nonpharmacologic management of POTS includes increasing salt and fluid intake, use of lower extremity compression garments to reduce venous pooling, and participation in regular exercise to prevent deconditioning . However, patients with COVID-19 and evidence of myocardial injury or inflammation should abstain from competitive sports or aerobic activity until resolution of imaging findings or normalization of cardiac biomarkers [55, 56••]. When these measures are ineffective, various pharmacologic therapies may be attempted depending on the specific etiology suspected. These therapies include fludrocortisone, midodrine, pyridostigmine, propranolol, ivabradine, and alpha-2 agonists . Ivabradine prescription for treatment of POTS is not FDA approved and is off label. Patients with COVID-19 may benefit from low-dose propranolol for lowering heart rate and reducing adrenergic activity (Table 2) . While immediate treatment-requiring arrhythmias in children are often extremely rare, pediatric patients most commonly report supraventricular tachycardias. The latter are often carefully treated with a combination of both pharmacotherapy such as amiodarone, ibutilide, beta-blockers, or cardioversion . The American Heart Association released guidelines in collaboration with the American Academy of Pediatrics as to treatment of children and infants with COVID-19. Within these guidelines, they suggest for pediatric patients suffering from life-threatening arrhythmias, early usage of endotracheal tubes/ventilation, and defibrillation . That said, apart from COVID-19 case studies which primarily report increased risk for pediatric patients with prior history of cardiac surgery, limited data are available for children with COVID-19 at this time of writing . Myocarditis is a leading cause for the development of ventricular arrhythmias . Myocarditis has also been presented as the most probable cause of myocardial injury and has been observed in 7.2–27.8% of COVID-19 patients . While it may not be possible to fully rule out prior silent ischemia in these patients, myocarditis may be a more likely culprit, due to direct viral infection of cardiomyocytes, hypoxia, or hyperimmune response . Autopsies on COVID-19 patients with myocarditis have shown direct invasion by the virus and inflammatory processes in the tissue without any presence of COVID-19 [61, 62]. Although the exact etiology of myocarditis remains in question, management of myocarditis for COVID-19 patients has shown relatively favorable outcomes in patients treated with glucocorticoid therapies . Furthermore, in accordance with possibilities of hyperimmune response and hypoxia, second-line agents such as IL-6 inhibitors and intravenous immunoglobulin (IVIG) were often administered with or without vasopressor support . Large-scale studies have yet to show the efficacy of glucocorticoid therapies on the heart for COVID-19 patients and their use remains controversial, with several studies suggesting these therapies have no or a harmful effect on patients . Optimal dosing guided by ARDS sub-phenotypes, biomarkers, and co-morbidities should be utilized before administration of glucocorticoids for COVID-19 patients with myocarditis . Cardiac Arrhythmias During and After Recovery Given the recent onset of the COVID-19 pandemic, data on long-term cardiovascular outcomes in patients who have recovered are lacking. Patients who developed overt cardiac disease should receive regular monitoring and reassessment. For those diagnosed with arrhythmias in the acute setting, it is plausible that some of these may resolve during convalescence. Therefore, the optimal duration of therapy should be personalized and made according to the patient and physician’s discretion. Risk stratification may be helpful to guide monitoring during recovery, with further testing indicated in those with cardiac involvement during infection. It has been proposed that patients with any evidence of possible myocardial injury should undergo follow-up transthoracic echocardiogram (TTE) and electrocardiogram (EKG) 2–6 months after COVID-19 diagnosis . Holter or event monitoring should be used as indicated by symptoms, but it may also be reasonable to consider their use in asymptomatic patients, given the risk of a variety of arrhythmias in patients with COVID-19 (Table 2). Abnormal findings on TTE, EKG, or cardiac monitoring should trigger additional investigation, which may include stress testing or cardiac MRI. The role of cardiac MRI after COVID-19 infection has been controversial as it is more sensitive than initial screening tests and may reveal findings of unclear clinical significance. In a study of 100 COVID-19-recovered patients, 60 had evidence of myocardial inflammation, 32 had myocardial late gadolinium enhancement (including 12 patterns suggestive of ischemia), and 22 had pericardial enhancement . Finally, three patients had severe abnormalities that were followed up with endomyocardial biopsy, ultimately revealing active lymphocytic inflammation. Due in part to evidence of subclinical myocarditis seen in these patients, there is concern that COVID-19 infection may declare itself as a risk factor for heart failure in the long term . As more data is obtained, cost-effectiveness analyses of testing in the recovery period may be considered, as well as randomized trials of prophylactic therapy for arrhythmias and/or myocardial dysfunction . Cardiac MRI may be helpful for risk stratification and to guide counseling on return to aerobic exercise or competitive sports. It has been proposed that COVID-19 patients who have ever had symptoms should rest for at least 2 weeks and undergo initial testing similar to that described above . Given the absence of COVID-19-specific data, patients with evidence of cardiac involvement should follow the guidelines for athletes with myocarditis and defer resumption of activity for 3–6 months [55, 56••, 69]. Management of patients after long-term COVID-19 recovery is even more uncertain. Patients who recovered from SARS were shown to have more hyperlipidemia, cardiovascular disorders, and impaired glucose metabolism at 12-years of follow-up compared to age-matched controls . Findings of the same study suggest that recovered SARS patients have altered lipid metabolism due to steroid use during their infection. It is plausible that similar findings may be seen in patients who are recovered from COVID-19, especially if they were also treated with steroids for severe pneumonia. At minimum, these patients should undergo age-appropriate cardiovascular disease screening in a primary care setting. However, more intensive screening may be considered. Monitoring of Corrected QT Interval (QTc) The use of QTc-prolonging drugs, including hydroxychloroquine (HCQ) and azithromycin (AZ), in the treatment of COVID-19 infection was forced into the limelight after a small, nonrandomized study of 36 patients suggested that HCQ alone or added to AZ aided clearance of a positive nasopharyngeal virus sample . This study was the foundation for the rapid adaptation of HCQ and/or AZ in worldwide clinical practice for COVID-19. Prolongation of the QT interval is a known risk factor for sudden cardiac death due to ventricular cardiac arrhythmias such as TDP [72, 73]. A previous study has reported significantly elevated risk of cardiac arrest in COVID-19 patients receiving HCQ + AZ, as well as in nonventilated COVID-19 patients receiving HCQ alone . The known effect of HCQ and AZ on prolongation of the QT interval has led to many subsequent studies on incidence of TDP and sudden cardiac death in COVID infection. Due to these risks and evidence that the regimen lacks efficacy [75,76,77], these drugs are no longer used to treat COVID-19. The direct causes of QT prolongation by HCQ and AZ are due to inhibition of the rapid delayed rectifier potassium current (IKr, or hERG) [78, 79]. However, indirect causes of QT prolongation in COVID infection may include inflammation, renal dysfunction, new onset of cardiac disease, electrolyte imbalance, and usage of other additional QTc-prolonging drugs. Previous studies have suggested that increase in cytokines, such as IL-6, may prolong QT in patients with viral infection. HIV-associated inflammation, causing elevated IL-6, has been shown to be independently associated with QT prolongation and prolonged repolarization represented as T wave onset-to-peak duration . HIV-positive patients had longer QTc intervals and QTc prolongation often > 500 ms, even after taking into account other QT prolonging drugs like methadone when compared to HIV negative patients . The true prevalence of cardiac events in patients with COVID-19 may not be fully appreciated. This review highlights the cardiac arrhythmias—such as AF, AV block, IST, POTS, and VT/VF—during and after COVID-19 infection (Fig. 1), underscoring the importance of careful cardiac management considerations in COVID-19 patients. Furthermore, given the breadth of cardiac arrhythmias involved in COVID-19 and the diversity of their etiologies, remote digital monitoring has emerged as a growing and necessary aspect of COVID-19 management for patients with cardiac complications or at risk of cardiac complications. With the onset of new therapeutics for COVID-19, further questions are raised as to how this will impact the management of arrhythmias. Studies will be needed to investigate the association between vaccination-status and risk of COVID-19-related cardiac arrhythmias. Furthermore, future research is needed to determine whether vaccination may be protective of cardiac injury and development of cardiac arrhythmias and which patients would most derive benefit. Similarly, with monoclonal antibody treatment recently gaining increased attention, it remains unknown what cardiac management changes would accompany widespread adoption of these types of treatment. Finally, with subsequent waves of COVID-19 approaching, or already starting in many parts of the country, little is known on how re-infection, a second course of infection, will affect previously infected individuals. The exact cardiac complications which may accompany a second COVID-19 infection are yet unknown. Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance Dong E, Du H, Gardner L. An interactive web-based dashboard to track COVID-19 in real time. Lancet Infect Dis. 2020;20(5):533–4. Zaim S, Chong JH, Sankaranarayanan V, Harky A. COVID-19 and multiorgan response. Curr Probl Cardiol. 2020;45(8):100618. Xu X, Chen P, Wang J, Feng J, Zhou H, Li X, et al. Evolution of the novel coronavirus from the ongoing Wuhan outbreak and modeling of its spike protein for risk of human transmission. Sci China Life Sci. 2020;63(3):457–60. Gupta A, et al. Extrapulmonary manifestations of COVID-19. Nat Med. 2020;26(7):1017–32 An important review of effects of COVID-19 on organ systems other than the lungs. Guo T, Fan Y, Chen M, Wu X, Zhang L, He T, et al. Cardiovascular implications of fatal outcomes of patients with coronavirus disease 2019 (COVID-19). JAMA Cardiol. 2020;5(7):811–8. Clerkin KJ, Fried JA, Raikhelkar J, Sayer G, Griffin JM, Masoumi A, et al. COVID-19 and cardiovascular disease. Circulation. 2020;141(20):1648–55. Fried JA, Ramasubbu K, Bhatt R, Topkara VK, Clerkin KJ, Horn E, et al. The variety of cardiovascular presentations of COVID-19. Circulation. 2020;141(23):1930–6. Madjid M, et al. Potential effects of coronaviruses on the cardiovascular system: a review. JAMA Cardiol. 2020. Tian S, Xiong Y, Liu H, Niu L, Guo J, Liao M, et al. Pathological study of the 2019 novel coronavirus disease (COVID-19) through postmortem core biopsies. Mod Pathol. 2020;33(6):1007–14. Wang C, Horby PW, Hayden FG, Gao GF. A novel coronavirus outbreak of global health concern. Lancet. 2020;395(10223):470–3. Driggin E, Madhavan MV, Bikdeli B, Chuich T, Laracy J, Biondi-Zoccai G, et al. Cardiovascular considerations for patients, health care workers, and health systems during the COVID-19 pandemic. J Am Coll Cardiol. 2020;75(18):2352–71. Kazi DS, Martin LM, Litmanovich D, Pinto DS, Clerkin KJ, Zimetbaum PJ, et al. Case 18-2020: a 73-year-old man with hypoxemic respiratory failure and cardiac dysfunction. N Engl J Med. 2020;382(24):2354–64. Wang D, Hu B, Hu C, Zhu F, Liu X, Zhang J, et al. Clinical characteristics of 138 hospitalized patients with 2019 novel coronavirus-infected pneumonia in Wuhan, China. JAMA. 2020;323(11):1061–9. Berman JP, Abrams MP, Kushnir A, Rubin GA, Ehlert F, Biviano A, et al. Cardiac electrophysiology consultative experience at the epicenter of the COVID-19 pandemic in the United States. Indian Pacing Electrophysiol J. 2020. Gawalko M, et al. COVID-19 associated atrial fibrillation: incidence, putative mechanisms and potential clinical implications. Int J Cardiol Heart Vasc. 2020;30:100631. Ambrus DB, Benjamin EJ, Bajwa EK, Hibbert KA, Walkey AJ. Risk factors and outcomes associated with new-onset atrial fibrillation during acute respiratory distress syndrome. J Crit Care. 2015;30(5):994–7. Goyal P, Choi JJ, Pinheiro LC, Schenck EJ, Chen R, Jabri A, et al. Clinical characteristics of Covid-19 in New York city. N Engl J Med. 2020;382(24):2372–4. January CT, Wann LS, Alpert JS, Calkins H, Cigarroa JE, Cleveland JC Jr, et al. 2014 AHA/ACC/HRS guideline for the management of patients with atrial fibrillation: a report of the American College of Cardiology/American Heart Association Task Force on practice guidelines and the Heart Rhythm Society. Circulation. 2014;130(23):e199–267. Bhatla A, Mayer MM, Adusumalli S, Hyman MC, Oh E, Tierney A, et al. COVID-19 and cardiac arrhythmias. Heart Rhythm. 2020;17(9):1439–44. Yamin M, Demili AU. Prevention of ventricular arrhythmia and sudden cardiac death in COVID-19 patients. Acta Med Indones. 2020;52(3):290–6. Proietti R, Joza J, Essebag V. Therapy for ventricular arrhythmias in structural heart disease: a multifaceted challenge. J Physiol. 2016;594(9):2431–43. Abrams MP, et al. Malignant ventricular arrhythmias in patients with severe acute respiratory distress syndrome due to COVID-19 without significant structural heart disease. Heart Rhythm Case Rep. 2020. Annane D, Sébille V, Duboc D, le Heuzey JY, Sadoul N, Bouvier E, et al. Incidence and prognosis of sustained arrhythmias in critically ill patients. Am J Respir Crit Care Med. 2008;178(1):20–5. Shao F, Xu S, Ma X, Xu Z, Lyu J, Ng M, et al. In-hospital cardiac arrest outcomes among patients with COVID-19 pneumonia in Wuhan, China. Resuscitation. 2020;151:18–23. Parish DC, Goyal H, Dane FC. Mechanism of death: there’s more to it than sudden cardiac arrest. J Thorac Dis. 2018;10(5):3081–7. Long B, Brady WJ, Koyfman A, Gottlieb M. Cardiovascular complications in COVID-19. Am J Emerg Med. 2020;38(7):1504–7. Iba T, Levy JH, Connors JM, Warkentin TE, Thachil J, Levi M. The unique characteristics of COVID-19 coagulopathy. Crit Care. 2020;24(1):360. Kashi M, Jacquin A, Dakhil B, Zaimi R, Mahé E, Tella E, et al. Severe arterial thrombosis associated with Covid-19 infection. Thromb Res. 2020;192:75–7. Giudicessi JR, Roden DM, Wilde AAM, Ackerman MJ. Genetic susceptibility for COVID-19-associated sudden cardiac death in African Americans. Heart Rhythm. 2020;17(9):1487–92. Rattanawong P, et al. Guidance on short-term management of atrial fibrillation in coronavirus disease 2019. J Am Heart Assoc. 2020;9(14):e017529 This manuscript summarizes considerations of antiarrhythmics usage for AF in the setting of acute COVID-19 infection. Sorajja D, Munger TM, Shen WK. Optimal antiarrhythmic drug therapy for electrical storm. J Biomed Res. 2015;29(1):20–34. Enriquez A, Liang J, Gentile J, Schaller RD, Supple GE, Frankel DS, et al. Outcomes of rescue cardiopulmonary support for periprocedural acute hemodynamic decompensation in patients undergoing catheter ablation of electrical storm. Heart Rhythm. 2018;15(1):75–80. Mitacchione G, et al. Ventricular tachycardia storm management in a COVID-19 patient: a case report. Eur Heart J Case Rep. 2020;4(FI1):1–6. Wang Y, Wang Z, Tse G, Zhang L, Wan EY, Guo Y, et al. Cardiac arrhythmias in patients with COVID-19. J Arrhythm. 2020;36(5):827–36. Cooper LT Jr, Blauwet LA. When should high-grade heart block trigger a search for a treatable cardiomyopathy? Circ Arrhythm Electrophysiol. 2011;4(3):260–1. Al-Assaf O, Mirza M, Musa A. Atypical presentation of COVID-19 as subclinical myocarditis with persistent high degree atrio-ventricular block treated with pacemaker implant. Heart Rhythm Case Rep. 2020. Haddadin FI, et al. A case of complete heart block in a COVID-19 infected patient. J Cardiol Cases. 2020. Gupta MD, Qamar A, MP G, Safal S, Batra V, Basia D, et al. Bradyarrhythmias in patients with COVID-19: a case series. Indian Pacing Electrophysiol J. 2020;20(5):211–2. Kir D, Mohan C, Sancassani R. Heart brake: an unusual cardiac manifestation of COVID-19. JACC Case Rep. 2020;2(9):1252–5. Eneizat Mahdawi T, Wang H, Haddadin FI, al-Qaysi D, Wylie JV. Heart block in patients with coronavirus disease 2019: a case series of 3 patients infected with SARS-CoV-2. Heart Rhythm Case Rep. 2020;6(9):652–6. Chinitz JS, Goyal R, Harding M, Veseli G, Gruberg L, Jadonath R, et al. Bradyarrhythmias in patients with COVID-19: marker of poor prognosis? Pacing Clin Electrophysiol. 2020;43(10):1199–204. Azarkish M, Laleh far V, Eslami M, Mollazadeh R. Transient complete heart block in a patient with critical COVID-19. Eur Heart J. 2020;41(22):2131. Kochav SM, Coromilas E, Nalbandian A, Ranard LS, Gupta A, Chung MK, et al. Cardiac arrhythmias in COVID-19 infection. Circ Arrhythm Electrophysiol. 2020;13(6):e008719. Lau ST, Yu WC, Mok NS, Tsui PT, Tong WL, Cheng SWC. Tachycardia amongst subjects recovering from severe acute respiratory syndrome (SARS). Int J Cardiol. 2005;100(1):167–9. Yu CM, Wong RS, Wu EB, Kong SL, Wong J, Yip GW, et al. Cardiovascular complications of severe acute respiratory syndrome. Postgrad Med J. 2006;82(964):140–4. Olshansky B, Sullivan RM. Inappropriate sinus tachycardia. J Am Coll Cardiol. 2013;61(8):793–801 This systemic review summarizes recommendations for treatment of IST. Lazzerini PE, Laghi-Pasini F, Boutjdir M, Capecchi PL. Cardioimmunology of arrhythmias: the role of autoimmune and inflammatory cardiac channelopathies. Nat Rev Immunol. 2019;19(1):63–4. Page RL, Joglar JA, Caldwell MA, Calkins H, Conti JB, Deal BJ, et al. 2015 ACC/AHA/HRS guideline for the management of adult patients with supraventricular tachycardia. Circulation. 2016;133(14):e506–74. Agarwal AK, Garg R, Ritch A, Sarkar P. Postural orthostatic tachycardia syndrome. Postgrad Med J. 2007;83(981):478–80. Raj SR. Postural tachycardia syndrome (POTS). Circulation. 2013;127(23):2336–42. Schondorf R, Low PA. Idiopathic postural orthostatic tachycardia syndrome: an attenuated form of acute pandysautonomia? Neurology. 1993;43(1):132–7. Umapathi T, Poh MQW, Fan BE, Li KFC, George J, Tan JYL. Acute hyperhidrosis and postural tachycardia in a COVID-19 patient. Clin Auton Res. 2020. Romero-Sanchez CM, et al. Neurologic manifestations in hospitalized patients with COVID-19: the ALBACOVID registry. Neurology. 2020;95(8):e1060–70. Sheldon RS, Grubb BP II, Olshansky B, Shen WK, Calkins H, Brignole M, et al. 2015 heart rhythm society expert consensus statement on the diagnosis and treatment of postural tachycardia syndrome, inappropriate sinus tachycardia, and vasovagal syncope. Heart Rhythm. 2015;12(6):e41–63. Maron BJ, Udelson JE, Bonow RO, Nishimura RA, Ackerman MJ, Estes NA 3rd, et al. Eligibility and disqualification recommendations for competitive athletes with cardiovascular abnormalities: task force 3: hypertrophic cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy and other cardiomyopathies, and myocarditis: a scientific statement from the American Heart Association and American College of Cardiology. Circulation. 2015;132(22):e273–80. Hendren NS, et al. Description and proposed management of the acute COVID-19 cardiovascular syndrome. Circulation. 2020;141(23):1903–14 This comprehensive manuscript proposes management considerations for treatment of cardiovascular disease in acute COVID-19 infection. Raj SR, Black BK, Biaggioni I, Paranjape SY, Ramirez M, Dupont WD, et al. Propranolol decreases tachycardia and improves symptoms in the postural tachycardia syndrome: less is more. Circulation. 2009;120(9):725–34. Hanash CR, Crosson JE. Emergency diagnosis and management of pediatric arrhythmias. J Emerg Trauma Shock. 2010;3(3):251–60. Edelson DP, Sasson C, Chan PS, Atkins DL, Aziz K, Becker LB, et al. Interim guidance for basic and advanced life support in adults, children, and neonates with suspected or confirmed COVID-19. Circulation. 2020;141(25):e933–43. Sanna G, Serrau G, Bassareo PP, Neroni P, Fanos V, Marcialis MA. Children’s heart and COVID-19: up-to-date evidence in the form of a systematic review. Eur J Pediatr. 2020;179(7):1079–87. Tavazzi G, Pellegrini C, Maurelli M, Belliato M, Sciutti F, Bottazzi A, et al. Myocardial localization of coronavirus in COVID-19 cardiogenic shock. Eur J Heart Fail. 2020;22(5):911–5. Kim IC, Kim JY, Kim HA, Han S. COVID-19-related myocarditis in a 21-year-old female patient. Eur Heart J. 2020;41(19):1859. Sawalha K, Abozenah M, Kadado AJ, Battisha A, al-Akchar M, Salerno C, et al. Systematic review of COVID-19 related myocarditis: insights on management and outcome. Cardiovasc Revasc Med. 2020. Lu S, et al. Effectiveness and safety of glucocorticoids to treat COVID-19: a rapid review and meta-analysis. Ann Transl Med. 2020;8(10):627. Prescott HC, Rice TW. Corticosteroids in COVID-19 ARDS: evidence and hope during the pandemic. JAMA. 2020;324:1292–5. Mitrani RD, Dabas N, Goldberger JJ. COVID-19 cardiac injury: implications for long-term surveillance and outcomes in survivors. Heart Rhythm. 2020;17:1984–90. Puntmann VO, et al. Outcomes of cardiovascular magnetic resonance imaging in patients recently recovered from coronavirus disease 2019 (COVID-19). JAMA Cardiol. 2020. Yancy CW, Fonarow GC. Coronavirus disease 2019 (COVID-19) and the heart-is heart failure the next chapter? JAMA Cardiol. 2020. Phelan D, Kim JH, Chung EH. A game plan for the resumption of sport and exercise after coronavirus disease 2019 (COVID-19) infection. JAMA Cardiol. 2020. Wu Q, Zhou L, Sun X, Yan Z, Hu C, Wu J, et al. Altered lipid metabolism in recovered SARS patients twelve years after infection. Sci Rep. 2017;7(1):9110. Gautret P, Lagier JC, Parola P, Hoang VT, Meddeb L, Mailhe M, et al. Hydroxychloroquine and azithromycin as a treatment of COVID-19: results of an open-label non-randomized clinical trial. Int J Antimicrob Agents. 2020;56:105949. Fuster V, Varieur Turco J. COVID-19: a lesson in humility and an opportunity for sagacity and hope. J Am Coll Cardiol. 2020;75(20):2625–6. Giudicessi JR, Noseworthy PA, Friedman PA, Ackerman MJ. Urgent guidance for navigating and circumventing the QTc-prolonging and torsadogenic potential of possible pharmacotherapies for coronavirus disease 19 (COVID-19). Mayo Clin Proc. 2020;95:1213–21. Rosenberg ES, Dufort EM, Udo T, Wilberschied LA, Kumar J, Tesoriero J, et al. Association of treatment with hydroxychloroquine or azithromycin with in-hospital mortality in patients with COVID-19 in New York state. JAMA. 2020;323:2493–502. Geleris J, Sun Y, Platt J, Zucker J, Baldwin M, Hripcsak G, et al. Observational study of hydroxychloroquine in hospitalized patients with Covid-19. N Engl J Med. 2020;382(25):2411–8. Cavalcanti AB, Zampieri FG, Rosa RG, Azevedo LCP, Veiga VC, Avezum A, et al. Hydroxychloroquine with or without azithromycin in mild-to-moderate Covid-19. N Engl J Med. 2020. Effect of hydroxychloroquine in hospitalized patients with Covid-19. N Engl J Med. 2020. Capel RA, Herring N, Kalla M, Yavari A, Mirams GR, Douglas G, et al. Hydroxychloroquine reduces heart rate by modulating the hyperpolarization-activated current if: novel electrophysiological insights and therapeutic potential. Heart Rhythm. 2015;12(10):2186–94. Zhang M, Xie M, Li S, Gao Y, Xue S, Huang H, et al. Electrophysiologic studies on the risks and potential mechanism underlying the proarrhythmic nature of azithromycin. Cardiovasc Toxicol. 2017;17(4):434–40. Wu KC, Zhang L, Haberlen SA, Ashikaga H, Brown TT, Budoff MJ, et al. Predictors of electrocardiographic QT interval prolongation in men with HIV. Heart. 2019;105(7):559–65. Wu KC, Bhondoekhan F, Haberlen SA, Ashikaga H, Brown TT, Budoff MJ, et al. Associations between QT interval subcomponents, HIV serostatus, and inflammation. Ann Noninvasive Electrocardiol. 2020;25(2):e12705. Myerson M, et al. Prolonged QTc in HIV-infected patients: a need for routine ECG screening. J Int Assoc Provid AIDS Care. 2019;18:2325958219833926. Romero J, Husain SA, Kelesidis I, Sanz J, Medina HM, Garcia MJ. Detection of left atrial appendage thrombus by cardiac computed tomography in patients with atrial fibrillation: a meta-analysis. Circ Cardiovasc Imaging. 2013;6(2):185–94. Lakkireddy DR, Chung MK, Gopinathannair R, Patton KK, Gluckman TJ, Turagam M, et al. Guidance for cardiac electrophysiology during the COVID-19 pandemic from the Heart Rhythm Society COVID-19 Task Force; Electrophysiology Section of the American College of Cardiology; and the Electrocardiography and Arrhythmias Committee of the Council on Clinical Cardiology. Am Heart Assoc Heart Rhythm. 2020;17(9):e233–41. E.Y.W. is supported by the NIH R01 HL152236, R03 HL146881, the Louis V. Gerstner, Jr. Scholar Program and the Esther Aboodi Professorship and the Wu Family Research Fund. Conflict of Interest The authors have no conflict of interest to declare. Human and Animal Rights and Informed Consent This article does not contain any studies with human or animal subjects performed by any of the authors. Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This article is part of the Topical Collection on Invasive Electrophysiology and Pacing About this article Cite this article Desai, A.D., Boursiquot, B.C., Melki, L. et al. Management of Arrhythmias Associated with COVID-19. Curr Cardiol Rep 23, 2 (2021). https://doi.org/10.1007/s11886-020-01434-7 - Cardiac arrhythmias	2	18	2	0	0	1	0.804214	3	9,772
From Wikipedia, the free encyclopedia Lady Windermere syndrome describes infection in the lungs due to Mycobacterium avium complex. It is named after a character in Oscar Wilde's play Lady Lady Windermere syndrome is a type of mycobacterial lung Patients with Lady Windermere syndrome experience chronic cough, shortness of breath, fatigue and other less specific symptoms. Mycobacterium avium complex is the most commonly found form of non-tuberculous mycobacteria. Immunodeficiency is not a requirement for Mycobacterium Mycobacterium avium Complex (MAC) usually affects patients with abnormal lungs or bronchi. However, Candace Baker, Jerome Reich and Richard Johnson describe a series of six patients with MAC infection of the right middle lobe or left lingula who did not have any predisposing lung The right middle lobe and left lingula of the lungs are served by that are oriented downward when a person is in the upright position. As a result, these areas of the lung may be more dependent upon vigorous voluntary expectoration (cough) for clearance of bacteria and Since the six patients in their retrospective case series were older females, Reich and Johnson and Baker propose that patients without a vigorous cough may develop right middle lobe or left lingular infection with MAC. They propose that this syndrome be named Lady Windermere syndrome, after the character Lady Windermere in Oscar Wilde's play Lady Windermere's Fan. The diagnosis requires consistent symptoms with two additional Lady Windermere syndrome is usually treated with a three-drug regimen of either clarithromycin or azithromycin, plus rifampicin and ethambutol. Treatment typically lasts at least 12 months. The original Chest article proposing the existence and pathophysiology of the Lady Windermere syndrome suggests that the character Lady Windermere in Oscar Wilde's Victorian-era play Lady Windermere's Fan is a good example of the fastidious behavior believed to cause the syndrome. The article states: - We offer the term, Lady Windermere's Syndrome, from the Victorian-era play, Lady Windermere's Fan, to convey the fastidious behavior hypothesized: "How do you do, Lord Darlington. No, I can't shake hands with you. My hands are all wet with the roses." Victorian women presumably believed that "Ladies don't spit," and consequently might have been predisposed to develop lung Shortly after the Lady Windermere syndrome was proposed, a librarian wrote a letter to the editor of Chest challenging the use of Lady Windermere as the eponymous ancestor of the proposed syndrome. In Lady Windermere's Fan, Lady Windermere is a vivacious young woman, married only 2 years, who never coughs or displays any other signs of illness. While her avoidance of shaking hands might be interpreted as "fastidiousness," two alternative explanations may be just as - 1) Lady Windermere actually is in the midst of arranging flowers and consequently cannot properly greet her - [LADY WINDERMERE is at table R., arranging roses in a blue - 2) Lady Windermere wishes to discourage the flirtatious advances of her would-be suitor Lord Darlington and cites her wet hands as an excuse to keep him from touching her: - LADY WINDERMERE. Lord Darlington, you annoyed me last night at the Foreign Office. I am afraid you are going to annoy me again. . . . - LORD DARLINGTON. [Takes chair and goes across L.C.] I am quite miserable, Lady Windermere. You must tell me what I did. [Sits down at table L.] - LADY WINDERMERE. Well, you kept paying me elaborate compliments the whole evening.] The scholars highlight the literary malapropism, but some in the medical community have adopted the term regardless, and peer-reviewed medical journals still sometimes mention the Lady In recent years, some have described the eponym as and some have noted that it would have been unlikely that Lady Windermere had the condition to which her name was assigned. - ^ Reich JM, Johnson RE (June 1992). "Mycobacterium avium complex pulmonary disease presenting as an isolated lingular or middle lobe pattern. The Lady Windermere syndrome". Chest 101 (6): 1605–9. doi:10.1378/chest.101.6.1605. PMID 1600780. http://www.chestjournal.org/cgi/reprint/101/6/1605. - ^ Wilde, Oscar (1940). The Importance of Being Earnest and Other Plays. Penguin. ISBN - ^ Subcommittee Of The Joint Tuberculosis Committee Of The British Thoracic Society, (March 2000). "Management of opportunist mycobacterial infections: Joint Tuberculosis Committee Guidelines 1999. Subcommittee of the Joint Tuberculosis Committee of the British Thoracic Society". Thorax 55 (3): 210–8. doi:10.1136/thorax.55.3.210. PMID 10679540. PMC 1745689. http://thorax.bmj.com/cgi/pmidlookup?view=long&pmid=10679540. Wickremasinghe M, Ozerovitch LJ, Davies G, et al. (December 2005). "Non-tuberculous mycobacteria in patients with bronchiectasis". Thorax 60 (12): 1045–51. doi:10.1136/thx.2005.046631. PMID 16227333. - ^ Martins AB, Matos ED, Lemos AC (April 2005). "Infection with the Mycobacterium avium complex in patients without predisposing conditions: a case report and literature review". Braz J Infect Dis 9 (2): 173–9. doi:/S1413-86702005000200009. PMID 16127595. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1413-86702005000200009&lng=en&nrm=iso&tlng=en. ^ "Chest -- eLetters for Reich and Johnson, 101 (6) 1605-1609". http://www.chestjournal.org/cgi/eletters/101/6/1605#85. b "Oscar Wilde: Lady Windermere's Fan: ACT I. Morning-room in Lord Windermere's house. - Free Online Library". http://wilde.thefreelibrary.com/Lady-Windermeres-Fan/1-1. - ^ Sexton P, Harrison AC (June 2008). "Susceptibility to nontuberculous mycobacterial lung disease". Eur. Respir. J. 31 (6): 1322–33. doi:10.1183/09031936.00140007. PMID 18515557. http://erj.ersjournals.com/cgi/pmidlookup?view=long&pmid=18515557. - ^ Kasthoori JJ, Liam CK, Wastie ML (February 2008). "Lady Windermere syndrome: an inappropriate eponym for an increasingly important condition" (PDF). Singapore Med J 49 (2): e47–9. PMID 18301826. http://smj.sma.org.sg/4902/4902cr6.pdf. - ^ Rubin BK (October 2006). "Did Lady Windermere have cystic fibrosis?". Chest 130 (4): 937–8. doi:10.1378/chest.130.4.937. PMID 17035420. http://www.chestjournal.org/cgi/pmidlookup?view=long&pmid=17035420. diseases · Bacterial diseases: G+ (primarily A00–A79, 001–041, 080–109)	2	10	0	0	2	1	0.520813	3	1,812
CONDITIONS ORIGINATING IN THE PERINATAL PERIOD (P00-P96) Certain conditions originating in the perinatal period (P00-P96) were identified as the underlying cause of 658 deaths registered in 2006. Of these, 363 (55%) were males, and 295 (45%) were females. The number of deaths due to these causes, as well as the proportion of males and females, has remained relatively stable over the past 10 years. Although the majority of deaths attributed to this cause occur in the neonatal period (within 28 days of birth), this is not always the case. In 2006, 95% of deaths due to Certain conditions originating in the perinatal period occurred in the perinatal period, while 5.3% of these deaths were in older age groups. Further information on deaths occurring during the perinatal period can be found in Chapter 4. Of note, deaths attributed to Other conditions originating in the perinatal period (P96) increased from 6 in 2005 to 48 in 2006. This is largely a residual code, and the increase reflects a decline in the specificity of certification. Similarly, Disorders related to short gestation and low birth weight, not elsewhere classified (P07) also increased from 92 in 2005 to 129 in 2006, continuing a gradual increase over the last 10 years. 2.16 Selected Underlying Causes, Conditions Originating in the Perinatal Period (P00-P96) - 2006 Proportion of all deaths \|Cause of death and ICD code \| \|CHAPTER XVI Certain conditions originating in the perinatal period (P00-P96) \| \|Foetus and newborn affected by maternal factors and by complications of pregnancy, labour, and delivery (P00-P04) \| \|Foetus and newborn affected by maternal conditions that may be unrelated to present pregnancy (P00) \| \|Foetus and newborn affected by maternal complications of pregnancy (P01) \| \|Foetus and newborn affected by complications of placenta, cord and membranes (P02) \| \|Disorders related to length of gestation and foetal growth (P05-P08) \| \|Disorders related to short gestation and low birth weight, not elsewhere classified (P07) \| \|Respiratory and cardiovascular disorders specific to the perinatal period (P20-P29) \| \|Birth asphyxia (P21) \| \|Infections specific to the perinatal period (P35-P39) \| \|Bacterial sepsis of newborn (P36) \| \|Haemorrhagic and haematological disorders of foetus and newborn (P50-P61) \| \|Intracranial nontraumatic haemorrhage of foetus and newborn (P52) \| \|Other disorders originating in the perinatal period (P90-P96) \| \|Other conditions originating in the perinatal period (P96) \| \|- nil or rounded to zero (including null cells) \|	4	8	0	0	0	6	0.56189	6	644
Medical Policy: 07.01.57 Original Effective Date: March 2013 Reviewed: February 2016 Revised: February 2016 Benefit determinations are based on the applicable contract language in effect at the time the services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary based on contract, and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable. This medical policy may not apply to FEP. Benefits are determined by the Federal Employee Program. This Medical Policy document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy will be reviewed regularly and be updated as scientific and medical literature becomes available. The benefit of gender reassignment surgery is only available to select employee groups that have chosen to add this to their benefit package. Biological sex is assigned at birth, depending on the appearance of the genitals. Gender identity is the gender that a person "identifies" with or feels themselves to be. While biological sex and gender identity are the same for most people, this is not the case for everyone. For example, some people may have the anatomy of a man, but identify themselves as a woman, while others may not feel they are definitively either male or female. This mismatch between sex and gender identity can lead to distressing and uncomfortable feelings that are called gender dysphoria. Gender dysphoria is a recognized medical condition, for which treatment is sometimes appropriate. The condition is also sometimes known as gender identity disorder (GID), gender incongruence or transgenderism. Some people with gender dysphoria have a strong and persistent desire to live according to their gender identity, rather than their biological sex. These people are sometimes called transsexual or trans people. Some trans people have treatment to make their physical appearance more consistent with their gender identity. Gender dysphoria is not the same as transvestism or cross-dressing and is not related to sexual orientation. People with the condition may identify as straight, gay, lesbian, bisexual or asexual, and this may change with treatment. According to the American Psychiatric Association, the Diagnostic and Statistical Manual of Mental Disorders (DSM-5) defines gender dysphoria as a condition where a person’s gender at birth is contrary to the one they identify with. This definition replaces the criteria for gender identity disorder which will no longer be used in DSM-5. However, ICD-9 and ICD-10 codes continue to use the term gender identity disorder. In 2010, the World Professional Association for Transgender Health (WPATH) released a statement noting that “the expression of gender characteristics, including identities that are not stereotypically associated with one’s assigned sex at birth is a common and culturally-diverse human phenomenon [that] should not be judged as inherently pathological or negative.” Accordingly, transsexual, transgender, and gender nonconforming persons are not intrinsically disordered. Rather, the distress of gender dysphoria, when present, is the matter that may be diagnosable and for which several therapeutic options are available. Therapeutic approaches include psychological interventions and gender reassignment therapy, including hormonal interventions that muscularize or feminize the body, and surgical interventions that change the genitalia and other sex characteristics. Gender identity disorders may manifest at childhood, adolescence, or adulthood. Treatment for gender dysphoria aims to help reduce or remove the distressing feelings of a mismatch between biological sex and gender identity. This can mean different things for different people. For some people, it can mean dressing and living as their preferred gender. For others, it can mean taking hormones or having surgery to change their physical appearance. Many trans people have treatment to change their body permanently, so that they are more consistent with their gender identity, and the vast majority are satisfied with the eventual results. The surgical procedures for male-to-female individuals, also known as “transwomen” may include orchiectomy, penectomy, vaginoplasty, clitoroplasty, and labioplasty. Techniques include penile skin inversion, pedicled colosigmoid transplant, and free skin grafts to line the neovagina. For female-to-male persons, also known as “transmen” surgery may include hysterectomy, ovariectomy, salpingo-oophorectomy, vaginectomy, metoidioplasty, scrotoplasty, urethroplasty, placement of testicular prostheses, and phalloplasty. Prior to surgery, patients typically undergo hormone replacement therapy for a period of at least 12 continuous months. Biological females are treated with testosterone to increase muscle and bone mass, decrease breast size, increase clitoris size, increase facial and body hair, arrest menses, and deepen the voice. Biological males are treated with anti-androgens and estrogens to increase percentage of body fat compared to muscle mass, decrease body hair, decrease testicular size, decrease erectile function, and increase breast size. Individuals diagnosed with gender dysphoria also must undertake real life experience living in the identity-congruent gender role. This provides sufficient opportunity for patients to experience and socially adjust in their desired role before undergoing irreversible surgery. During this experience, patients should present themselves consistently, on a day-to-day basis and across all life settings, in their desired gender role. This includes coming out to partners, family, friends and community members. Changing gender role can have profound personal and social consequences, and individuals must demonstrate an awareness of the challenges and the ability to function successfully in their gender role. Individuals considering gender reassignment treatment will need to consider their reproductive health and make decisions concerning fertility prior to starting hormone therapy or undergoing surgery. Prior Approval is required This is a group specific benefit, if chosen by the employer. Prior approval is only necessary for those members seeking coverage under that benefit. Qualifications for all gender reassignment treatments: - Single letter of referral from a qualified mental health professional; and - Persistent, well-documented gender dysphoria (per DSM V criteria below); and - Capacity to make a fully informed decision and to consent for treatment; and - 18 years of age or older; and - If significant medical or mental health concerns are present, they must be reasonably well controlled. DSM 5 Criteria for Gender Dysphoria in Adults and Adolescents: A. A marked incongruence between one’s experienced/expressed gender and assigned gender, of at least 6 months duration, as manifested by two or more of the following: - A marked incongruence between one’s experienced/expressed gender and primary and/or secondary sex characteristics (or, in young adolescents, the anticipated secondary sex characteristics) - A strong desire to be rid of one’s primary and/or secondary sex characteristics because of a marked incongruence with one’s experienced/expressed gender (or, in young adolescents, a desire to prevent the development of the anticipated secondary sex characteristics) - A strong desire for the primary and/or secondary sex characteristics of the other gender - A strong desire to be of the other gender (or some alternative gender different from one’s assigned gender) - A strong desire to be treated as the other gender (or some alternative gender different from one’s assigned gender) - A strong conviction that one has the typical feelings and reactions of the other gender (or some alternative gender different from one’s assigned gender) B. The condition is associated with clinically significant distress or impairment in social, occupational, or other important areas of functioning. The treatment for Gender Dysphoria involves some combination of hormone therapy, sex reassignment surgery and/or Real Life Experience (living for a period of time in accordance with your gender identity). Each patient must be evaluated on a case-by-case basis, with expert medical judgment required for both reaching a diagnosis and determining a course of treatment. There is no set formula for gender transition. Additional qualifications for Breast Surgery Male to Female (breast augmentation) Female to Male (breast mastectomy) Genital Reassignment Surgery Additional qualifications prior to genital reconstruction surgery - Living 12 months of continuous, full time real life experience in the desired gender - 12 months of continuous hormone therapy (unless contraindications exists) - Two referral letters from qualified mental health professionals. The professional should include the physician responsible for endocrine transition therapy and the mental health professional providing current treatment. Male to Female - vaginoplasty ( including colovaginoplasty, penectomy, labiaplasty, clitoroplasty, vulvoplasty, penile skin inversion, repair of introitus, construction of vagina with graft, coloproctostomy) Colovaginoplasty is best viewed as two separate operations taking place simultaneously. The first, a general surgery team, performs laparotomy on the abdomen, harvesting a 15 to 20 centimeter colon interposition flap. During this time, the reassignment team performs many of the duties also done in a penile inversion, such as removal of the gonads, and corpora cavernosae. (if either are present) At this point, the general surgery team will internally hand off the colon segment to the reassignment team and secure it in place in the perineal body. And finally, the urethra is put in place, external genitalia are formed, and the patient is prepared for recovery. Female to Male vaginectomy (including colpectomy, metoidioplasty, phalloplasty, urethroplasty, urethromeatoplasty) Non-Covered Procedures (due to being considered cosmetic in nature) - body contouring (waist liposuction) - brow lift - calf implants - cheek/malar implants - chin/nose implants - collagen injections - drugs for hair loss or growth - drugs for sexual performance after genital reconstruction - drugs for other cosmetic purposes - face/forehead lift - facial bone reduction - hair removal/hair transplantation - jaw shortening/sculpturing/facial bone reduction - lip reduction/enhancement - mastopexy/breast lift - neck tightening - nipple/areola reconstruction - nose implants - pectoral implants - penile prosthesis (noninflatable /inflatable) - removal of redundant skin - replacement of tissue expander with permanent prosthesis testicular insertion - resizing of the nipple-areola complex - skin resurfacing (e.g., dermabrasion, chemical peels) - surgical correction of hydraulic abnormality of inflatable (multi-component) prosthesis including pump and/or cylinders and/or reservoir - testicular expanders - testicular prostheses - thyroid chondroplasty - trachea shave/reduction thyroid chondroplasty - voice modification surgery - voice therapy The process of gender reassignment does not include procedures to assist with fertility. Including, but not limited to: - Storage and thawing of reproductive tissue (ie, ovaries, testicular tissue) - Procurement and cryopreservation or storage of embryos, sperm or oocytes At least one of the professionals submitting a letter must have a doctoral degree (Ph.D., M.D., Ed.D, D.Sc., D.S.W., or Psy.D) and be capable of adequately evaluating any comorbid psychiatric conditions. A single letter is sufficient if signed by two providers, one of whom has met the doctoral degree specifications, in addition to the other specifications listed. The AMA passed a resolution in 2008 recognizing “an established body of medical research” that “demonstrates the effectiveness and medical necessity of mental health care, hormone therapy, and sex reassignment surgery as forms of therapeutic treatment for many patients diagnosed with [Gender Dysphoria].” Procedure Codes and Billing Guidelines: To report provider services, use appropriate CPT codes, Alpha Numeric (HCPCS level 2) codes, Revenue codes, and/or diagnosis codes. - Center of Excellence for Transgender Health, University of California, San Francisco. 2011. Primary care protocol for transgender health care. Available at: http:// transhealth.ucsf.edu/trans?page=ab-00-00. Accessed August 14, 2012. - De Cuypere G, T’Sjoen G, Beerten R et al. Sexual and physical health after sex reassignment surgery. Arch Sex Behav. 2005 Dec; 34(6): 679-90. - Diamond M. Human intersexuality: Difference of disorder? Arch Sex Behav 2009 Apr; 38(2):172. - Selvaggi G, Ceulemans P, De Cuypere G et al. Gender identity disorder: general overview and surgical treatment for vaginoplasty in male-to-female transsexuals. Plast Reconstr Surg. 2005; 116(6):135e-145e. - American College of Obstetricians and Gynecologists (ACOG). Healthcare for transgender individuals. Committee Opinion. No 512. December 2011. Obstet Gynecol 2011; 118:1454-8. - Hembree WC, Cohen-Kettenis P, Delemarre-van de Waal HA et al. Endocrine treatment of transsexual persons: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2009 Sep; 94(9):3132-54. - Sutcliffe PA, Dixon S, Akehurst RL et al. Evaluation of surgical procedures for sex reassignment: a systematic review. J Plast Reconstr Aesthet Surg. 2009 Mar; 62(3):294-306; discussion 306-8. - American Psychiatric Association. (2013). Cautionary statement for forensic use of DSM-5. In Diagnostic and statistical manual of mental disorders (5th ed.). doi:10.1176/appi.books.9780890425596.744053 - American Psychiatric Association. (2013). Diagnostic and statistical manual of mental disorders (5th ed.). Arlington, VA: American Psychiatric Publishing. - Standards of Care for the Health of Transsexual, Transgender, and Gender-Nonconforming People, version 7 2012; World Professional Association for Transgender Health (WPATH) - Trans - a practical guide for the NHS; Dept of Health, October 2008 - Technical Note - Measuring gender identity; Equality and Human Rights Commission survey, 2012 - Guidance for GPs, other clinicians and health professionals on the care of gender variant people; NHS, 2008 - Gender dysphoria services. A guide for general practitioners and other healthcare staff; NHS, 2012 - Meriggiola MC, Berra M; Safety of hormonal treatment in transgenders. Curr Opin Endocrinol Diabetes Obes. 2013 Dec;20(6):565-9. doi: 10.1097/01.med.0000436187.95351.a9. - Hembree WC; Management of juvenile gender dysphoria. Curr Opin Endocrinol Diabetes Obes. 2013 Dec;20(6):559-64. doi: 10.1097/01.med.0000436193.33470.1f. - Dhejne C, Lichtenstein P, Boman M, et al; Long-term follow-up of transsexual persons undergoing sex reassignment surgery: cohort study in Sweden. PLoS One. 2011 Feb 22;6(2):e16885. doi: 10.1371/journal.pone.0016885. February 2016 - Annual Review, Policy Revised February 2015 - Annual Review, Policy Renewed March 2014 - Annual Review, Policy Renewed March 2013, New Policy Wellmark medical policies address the complex issue of technology assessment of new and emerging treatments, devices, drugs, etc. They are developed to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. Wellmark medical policies contain only a partial, general description of plan or program benefits and do not constitute a contract. Wellmark does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Wellmark or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. Our medical policies may be updated and therefore are subject to change without notice. *Current Procedural Terminology © 2012 American Medical Association. All Rights Reserved.	2	13	2	0	0	0	0.923088	2	3,540
Deep vein thrombosis \|Deep vein thrombosis\| DVT in the right leg with swelling and redness \|Classification and external resources\| \|Patient UK\|\|Deep vein thrombosis\| Deep vein thrombosis, or deep venous thrombosis, (DVT) is the formation of a blood clot (thrombus) within a deep vein,[a] predominantly in the legs. Nonspecific signs may include pain, swelling, redness, warmness, and engorged superficial veins. Pulmonary embolism, a potentially life-threatening complication, is caused by the detachment (embolization) of a clot that travels to the lungs. Together, DVT and pulmonary embolism constitute a single disease process known as venous thromboembolism. Post-thrombotic syndrome, another complication, significantly contributes to the health-care cost of DVT. Prevention options for at-risk individuals include early and frequent walking, calf exercises, anticoagulants, aspirin, graduated compression stockings, and intermittent pneumatic compression. In 1856, German pathologist Rudolf Virchow postulated the interplay of three processes resulting in venous thrombosis, now known as Virchow's triad: a decreased blood flow rate (venous stasis), increased tendency to clot (hypercoagulability), and changes to the blood vessel wall. DVT formation typically begins inside the valves of the calf veins, where the blood is relatively oxygen deprived, which activates certain biochemical pathways. Several medical conditions increase the risk for DVT, including cancer, trauma, and antiphospholipid syndrome. Other risk factors include older age, surgery, immobilization (as with bed rest, orthopedic casts, and sitting on long flights), combined oral contraceptives, pregnancy, the postnatal period, and genetic factors. Those genetic factors include deficiencies with antithrombin, protein C, and protein S, the mutation of factor V Leiden, and the property of having a non-O blood type. The rate of new DVTs increases dramatically from childhood to old age; in adulthood, about one in 1000 adults develops it annually. Individuals suspected of having DVT may be assessed using a clinical prediction rule such as the Wells score. A D-dimer test may also be used to assist with excluding the diagnosis (because of its high sensitivity) or to signal a need for further testing. Diagnosis is most commonly done with ultrasound of the suspected veins. Anticoagulation is the standard treatment; typical medications include a low-molecular-weight heparin and a vitamin K antagonist. Wearing graduated compression stockings appears to reduce the risk of post-thrombotic syndrome. - 1 Signs and symptoms - 2 Causes - 3 Pathophysiology - 4 Diagnosis - 5 Prevention - 6 Treatment - 7 Prognosis - 8 Epidemiology - 9 Economics - 10 History - 11 Research directions - 12 Notes - 13 References - 14 External links Signs and symptoms Common signs and symptoms of DVT include pain or tenderness, swelling, warmth, redness or discoloration, and distention of surface veins, although about half of those with the condition have no symptoms. Signs and symptoms alone are not sufficiently sensitive or specific to make a diagnosis, but when considered in conjunction with known risk factors, can help determine the likelihood of DVT. In most suspected cases, DVT is ruled out after evaluation, and symptoms are more often due to other causes, such as cellulitis, Baker's cyst, musculoskeletal injury, or lymphedema. Other differential diagnoses include hematoma, tumors, venous or arterial aneurysms, and connective tissue disorders. Phlegmasia cerulea dolens is a very large and dangerous type of DVT. It is characterized by an acute and almost total venous occlusion of the entire extremity outflow, including the iliac and femoral veins. The leg is usually painful, tinged blue in color, and swollen, which may result in venous gangrene. The three factors of Virchow's triad—venous stasis, hypercoagulability, and changes in the endothelial blood vessel lining (such as physical damage or endothelial activation)—contribute to DVT and are used to explain its formation. Other related causes include activation of immune system components, the state of microparticles in the blood, the concentration of oxygen, and possible platelet activation. Various risk factors contribute to DVT, though many at high risk never develop it. Acquired risk factors include the strong risk factor of older age, which alters blood composition to favor clotting. Other important acquired risk factors include major surgery and trauma, both of which may increase the risk because of tissue factor from outside the vascular system entering the blood. In orthopedic surgery, venous stasis may be temporarily provoked by a cessation of blood flow as part of the procedure. Cancer can grow in and around veins, causing venous stasis, and can also stimulate increased levels of tissue factor. Pregnancy causes blood to favor clotting, and in the postpartum, placental tearing releases substances that favor clotting. Oral contraceptives[b] and hormonal replacement therapy increase the risk through a variety of mechanisms, including altered blood coagulation protein levels and reduced fibrinolysis. The disease term venous thromboembolism (VTE) includes the development of either DVT or pulmonary embolism (PE). Genetic factors that increase the risk of VTE include deficiencies of three proteins that normally prevent blood from clotting—protein C, protein S, and antithrombin—in addition to non-O blood type and mutations in the factor V and prothrombin genes.Deficiencies in antithrombin, protein C, and protein S are rare but strong, or moderately strong, risk factors. These three thrombophilia[c] increase the risk of VTE by about 10 times. Factor V Leiden, which makes factor V resistant to inactivation by activated protein C, and the genetic variant prothrombin G20210A, which causes increased prothrombin levels, are predominantly expressed in Caucasians.[d] They moderately increase risk for VTE, by three to eight times for factor V Leiden and two to three times for prothrombin G20210A. Having a non-O blood type roughly doubles VTE risk. Non-O blood type is common in all races, making it an important risk factor. Individuals without O blood type have higher blood levels of von Willebrand factor and factor VIII than those with O blood type, increasing the likelihood of clotting. Some risk factors influence the location of DVT within the body. In isolated distal DVT, the profile of risk factors appears distinct from proximal DVT. Transient factors, such as surgery and immobilization, appear to dominate, whereas thrombophilias and age do not seem to increase risk. In upper-extremity DVT, the most important risk factor is having a central venous catheter, and thoracic outlet syndrome also increases risk. DVT often develops in the calf veins and "grows" in the direction of venous flow, towards the heart. When DVT does not grow, it can be cleared naturally and dissolved into the blood (fibrinolysis). Veins in the calf or thigh are most commonly affected, including the femoral vein, the popliteal vein, and the iliofemoral vein (as with May–Thurner syndrome). Extensive lower-extremity DVT can reach into the iliac vein of the pelvis or the inferior vena cava. Occasionally the veins of the arm are affected, as after central venous catheter placement and with the rare Paget–Schrötter disease. The mechanism behind arterial thrombosis, such as with heart attacks, is more established than the steps that cause venous thrombosis. With arterial thrombosis, blood vessel wall damage is required, as it initiates coagulation, but clotting in the veins mostly occurs without any such damage. The beginning of venous thrombosis is thought to be caused by tissue factor, which leads to conversion of prothrombin to thrombin, followed by fibrin deposition. Red blood cells and fibrin are the main components of venous thrombi, and the fibrin appears to attach to the blood vessel wall lining (endothelium), a surface that normally acts to prevent clotting. Platelets and white blood cells are also components. Platelets are not as prominent in venous clots as they are in arterial ones, but they may play a role. Inflammation is associated with VTE,[f] and white blood cells play a role in the formation and resolution of venous clots. Often, DVT begins in the valves of veins. The blood flow pattern in the valves can cause low oxygen concentrations in the blood (hypoxemia) of a valve sinus. Hypoxemia, which is worsened by venous stasis, activates pathways—ones that include hypoxia-inducible factor-1 and early-growth-response protein 1. Hypoxemia also results in the production of reactive oxygen species, which can activate these pathways, as well as nuclear factor-κB, which regulates hypoxia-inducible factor-1 transcription. Hypoxia-inducible factor-1 and early-growth-response protein 1 contribute to monocyte association with endothelial proteins, such as P-selectin, prompting monocytes to release tissue factor-filled microvesicles, which presumably begin clotting after binding to the endothelial surface. DVT diagnosis requires the use of imaging devices such as ultrasound. Clinical assessments, which predict DVT likelihood, can help determine if a D-dimer test is useful. In those not highly likely to have DVT, a normal D-dimer result[g] can rule out a diagnosis. Provoked DVTs occur in association with acquired risk factors, such as surgery, oral contraceptives, trauma, immobility, obesity, or cancer; cases without acquired states are called unprovoked or idiopathic. Acute DVT is characterized by pain and swelling and is usually occlusive, which means that it obstructs blood flow, whereas non-occlusive DVT is less symptomatic. The label "chronic" has been applied to symptomatic DVT that persists longer than 10 to 14 days. DVT that has no symptoms, but is found only by screening, is labeled asymptomatic or incidental. DVT in the legs is proximal (or iliofemoral) when above the knee and distal (or calf) when below the knee. DVT below the popliteal vein, a proximal vein behind the knee, is classified as distal and has limited clinical significance compared to proximal DVT. An initial episode of DVT is called incident and any subsequent DVT is termed recurrent. Bilateral DVT refers to clots in both legs while unilateral means that only a single leg is affected. Wells score or criteria: (possible score −2 to 9) - Active cancer (treatment within last 6 months or palliative): +1 point - Calf swelling ≥ 3 cm compared to asymptomatic calf (measured 10 cm below tibial tuberosity): +1 point - Swollen unilateral superficial veins (non-varicose, in symptomatic leg): +1 point - Unilateral pitting edema (in symptomatic leg): +1 point - Previous documented DVT: +1 point - Swelling of entire leg: +1 point - Localized tenderness along the deep venous system: +1 point - Paralysis, paresis, or recent cast immobilization of lower extremities: +1 point - Recently bedridden ≥ 3 days, or major surgery requiring regional or general anesthetic in the past 12 weeks: +1 point - Alternative diagnosis at least as likely: −2 points Those with Wells scores of two or more have a 28% chance of having DVT, those with a lower score have 6% odds. Alternatively, Wells scores can be categorized as high if greater than two, moderate if one or two, and low if less than one, with likelihoods of 53%, 17%, and 5%, respectively. D-dimers are a fibrin degradation product, and an elevated level can result from plasmin dissolving a clot—or other conditions. Hospitalized patients often have elevated levels for multiple reasons. When individuals are at a high-probability of having DVT, diagnostic imaging is preferred to a D-dimer test. For those with a low or moderate probability of DVT, a D-dimer level might be obtained, which excludes a diagnosis if results are normal. An elevated level requires further investigation with diagnostic imaging to confirm or exclude the diagnosis. For a suspected first leg DVT in a low-probability situation, the American College of Chest Physicians recommends testing either D-dimer levels with moderate or high sensitivity or compression ultrasound of the proximal veins. These options are suggested over whole-leg ultrasound, and D-dimer testing is the suggested preference overall. The UK National Institute for Health and Care Excellence (NICE) recommends D-dimer testing prior to proximal vein ultrasound. For a suspected first leg DVT in a moderate-probability scenario, a high-sensitivity D-dimer is suggested as a recommended option over ultrasound imaging, with both whole-leg and compression ultrasound possible. The NICE guideline uses a two-point Wells score and does not refer to a moderate probability group. Imaging tests of the veins are used in the diagnosis of DVT, most commonly either proximal compression ultrasound or whole-leg ultrasound. Each technique has drawbacks: a single proximal scan may miss a distal DVT, while whole-leg scanning can lead to distal DVT overtreatment. Doppler ultrasound, CT scan venography, MRI venography, or MRI of the thrombus are also possibilities. The gold standard for judging imaging methods is contrast venography, which involves injecting a peripheral vein of the affected limb with a contrast agent and taking X-rays, to reveal whether the venous supply has been obstructed. Because of its cost, invasiveness, availability, and other limitations, this test is rarely performed. Depending upon the risk for DVT, different preventive measures are used. Walking and calf exercises reduce venous stasis because leg muscle contractions compress the veins and pump blood up towards the heart. In immobile individuals, physical compression methods improve blood flow. Anticoagulation, which increases the risk of bleeding, might be used in high-risk scenarios. The risk of major bleeding with long-term anticoagulation is about 3% per year, and the point where annual VTE risk is thought to warrant long-term anticoagulation is estimated to be between 3 and 9%. Usually, only when individuals exceed a 9% annual VTE risk is long-term anticoagulation a common consideration. Antithrombin deficiency, a strong or moderately strong risk factor, carries an annual risk of VTE of only 0.8–1.5%; as such, asymptomatic individuals with thrombophilia do not warrant long-term anticoagulation. Aside from anticoagulation, the antiplatelet drug aspirin might be used in some people following orthopedic surgery and in those with a previous VTE. Statins might decrease the risk for people who are otherwise healthy, but the evidence is not clear. Following the completion of warfarin long term aspirin is useful to prevent re occurrence. In 2011, the American College of Physicians (ACP) issued a clinical practice guideline making three strong recommendations based on moderate-quality evidence: that hospitalized patients be assessed for their risk of thromboembolism and bleeding before prophylaxis is started; that heparin or a related drug be used if potential benefits are thought to outweigh potential harms; and that graduated compression stockings not be used. The ACP also drew attention to a lack of support for any performance measures encouraging physicians to apply universal prophylaxis without regard to the risks. A 2014 Cochrane review found that using heparin in medical patients did not change the risk of death or pulmonary embolism. While its use decreased people's risks of DVTs, it also increased people's risks of major bleeding. The review thus recommended the need to balance risks and benefits. The 2012 ACCP guidelines for nonsurgical patients[h] recommend anticoagulation for the acutely ill in cases of elevated risk when neither bleeding nor a high risk of bleeding exists. Mechanical prophylaxis is suggested when risks for bleeding and thrombosis are elevated. For the critically ill, either pharmacological or mechanical prophylaxis is suggested depending upon the risk. Heparin is suggested in outpatients with cancer who have solid tumors and additional risk factors for VTE—listed as "previous venous thrombosis, immobilization, hormonal therapy, angiogenesis inhibitors, thalidomide, and lenalidomide"—and a low risk of bleeding. Major orthopedic surgery—total hip replacement, total knee replacement, or hip fracture surgery—has a high risk of causing VTE. If prophylaxis is not used after these surgeries, symptomatic VTE has about a 4% chance of developing within 35 days. Options for VTE prevention in people follow nonorthopedic surgery include early walking, mechanical prophylaxis (intermittent pneumatic compression or graduated compression stockings), and drugs (low-molecular-weight heparin and low-dose-unfractionated heparin) depending upon the risk of VTE, risk of major bleeding, and person's preferences. Following major orthopedic surgery, the ACCP recommends treatment with drugs that reduce the risk of clots (such as fondaparinux and aspirin) with low-molecular-weight heparin (LMWH) suggested as a preference. Intermittent pneumatic compression is also an option. Graduated compression stockings are effective after both general and orthopedic surgery. The risk of VTE is increased in pregnancy by about five times because of a more hypercoagulable state, a likely adaptation against fatal postpartum hemorrhage. Additionally, pregnant women with genetic risk factors are subject to a roughly three to 30 times increased risk for VTE. Preventative treatments for pregnancy-related VTE in hypercoagulable women were suggested by the ACCP. Homozygous carriers of factor V Leiden or prothrombin G20210A with a family history of VTE were suggested for antepartum LMWH and either LMWH or a vitamin K antagonist (VKA) for the six weeks following childbirth. Those with another thrombophilia and a family history but no previous VTE were suggested for watchful waiting during pregnancy and LMWH or—for those without protein C or S deficiency—a VKA. Homozygous carriers of factor V Leiden or prothrombin G20210A with no personal or family history of VTE were suggested for watchful waiting during pregnancy and LMWH or a VKA for six weeks after childbirth. Those with another thrombophilia but no family or personal history of VTE were suggested for watchful waiting only. Warfarin, a common VKA, can cause harm to the fetus and is not used for VTE prevention during pregnancy. The 2012 ACCP guidelines offered weak recommendations. For at-risk long-haul travelers—those with "previous VTE, recent surgery or trauma, active malignancy, pregnancy, estrogen use, advanced age, limited mobility, severe obesity, or known thrombophilic disorder"—suggestions included calf exercises, frequent walking, and aisle seating in airplanes to ease walking. The use of graduated compression stockings that fit below the knee and give 15–30 mm Hg of pressure to the ankle was suggested, while aspirin or anticoagulants were not. Compression stockings have sharply reduced the levels of asymptomatic DVT in airline passengers, but the effect on symptomatic VTE is unknown, as none of the individuals studied developed symptomatic VTE. Anticoagulation, which prevents further coagulation, but does not act directly on existing clots, is the standard treatment for DVT.[i] Balancing risk vs. benefit is important in determining the duration of anticoagulation, and three months is generally the standard length of treatment. In those with an annual risk of VTE in excess of 9%, as after an unprovoked episode, extended anticoagulation is a possibility. Those who finish VKA treatment after idiopathic VTE with an elevated D-dimer level show an increased risk of recurrent VTE (about 9% vs about 4% for normal results), and this result might be used in clinical decision-making. Thrombophilia test results rarely play a role in the length of treatment. For acute cases in the leg, the ACCP recommended a parenteral anticoagulant (such as LMWH, fondaparinux, or unfractionated heparin) for at least five days[j] and a VKA, the oral anticoagulant, the same day. LMWH and fondaparinux are suggested over unfractionated heparin, but both are retained in those with compromised kidney function, unlike unfractionated heparin. The VKA is generally taken for a minimum of three months to maintain an international normalized ratio of 2.0–3.0, with 2.5 as the target. The benefit of taking a VKA declines as the duration of treatment extends,[needs update] and the risk of bleeding increases with age. The ACCP recommended treatment for three months in those with proximal DVT provoked by surgery. A three-month course is also recommended for those with proximal DVT provoked by a transient risk factor, and three months is suggested over lengthened treatment when bleeding risk is low to moderate. Unprovoked DVT patients should have at least three months of anticoagulation and be considered for extended treatment. Those whose first VTE is an unprovoked proximal DVT are suggested for anticoagulation longer than three months unless there is a high risk of bleeding. In that case, three months is sufficient. Those with a second unprovoked VTE are recommended for extended treatment when bleeding risk is low, suggested for extended treatment when bleeding risk is moderate, and suggested for three months of anticoagulation in high-risk scenarios. Home treatment, stockings, walking, and repeat imaging The ACCP recommended initial home treatment instead of hospital treatment for those with acute leg DVT. This applies as long as individuals feel ready for it, and those with severe leg symptoms or comorbidities would not qualify. An appropriate home environment is expected: one that can provide a quick return to the hospital if necessary, support from family or friends, and phone access. In addition to anticoagulation, the ACCP suggested graduated compression stockings—which apply higher pressure (30–40 mm Hg) at the ankles and a lower pressure around the knees—for those with symptomatic DVT. Use should begin as soon as possible after anticoagulation. Evidence however does not support that these stockings reduce the risk of post-thrombotic syndrome nor do they indicate a reduction in recurrent VTE. Use is suggested for two years, though inconvenience and discomfort can reduce compliance. Walking is also suggested for those without severe pain or edema. Unless a person has medical problems preventing movement, after a person starts anti-coagulation therapy bed rest should not be used to treat acute deep vein thrombosis. There are clinical benefits associated with walking and no evidence that walking is harmful, but people with DVT are harmed by bed rest except when it is medically necessary. Instead of anticoagulation, a follow-up imaging test (typically ultrasound) about one-week post-diagnosis is an option for those with an acute isolated distal DVT without a high risk for extension; if the clot does not grow, the ACCP does not recommend anticoagulation. This technique can benefit those at a high risk for bleeding. Patients may choose anticoagulation over serial imaging, however, to avoid the inconvenience of another scan if concerns about the risk of bleeding are insignificant. When applied to symptomatic patients with a negative initial ultrasound result, serial testing is inefficient and not cost effective. IVC filters, thrombolysis, and thrombectomy Inferior vena cava filters (IVC filters) are used on the presumption that they reduce PE, although their effectiveness and safety profile are not well established. In general, they are only recommended in some high risk scenarios. The ACCP recommended them for those with a contraindication to anticoagulant treatment but not in addition to anticoagulation, unless an individual with an IVC filter but without a risk for bleeding develops acute proximal DVT. In this case, both anticoagulation and an IVC filter are suggested. NICE recommends caval filters in settings where someone with an acute proximal DVT or PE cannot receive anticoagulation, and that the filter is removed when anticoagulation can be safely started. While IVC filters themselves are associated with a long-term risk of DVT, they are not reason enough to maintain extended anticoagulation. Thrombolysis is the administration of an enzyme (intravenous or directly into the affected vein through a catheter), which acts to enzymatically break up clots. This may reduce the risk of post-thrombotic syndrome by a third, and possibly reduce the risk of leg ulcers, but is associated with an increased risk of bleeding. The ACCP currently suggests anticoagulation rather than thrombolysis, but patients may choose thrombolysis if prevention of post-thrombotic syndrome outweighs concerns over the complexity, bleeding risk, and cost of the procedure. NICE recommends that thrombolysis is considered in those who have had symptoms for less than two weeks, are normally well, have a good life expectancy and a low risk of bleeding. A mechanical thrombectomy device can remove venous clots, although the ACCP considers it an option only when the following conditions apply: "iliofemoral DVT, symptoms for < 7 days (criterion used in the single randomized trial), good functional status, life expectancy of ≥ 1 year, and both resources and expertise are available." Anticoagulation alone is suggested over thrombectomy. The most frequent complication of proximal DVT is post-thrombotic syndrome, which is caused by a reduction in the return of venous blood to the heart. Some symptoms of post-thrombotic syndrome are pain, edema, paresthesia, and in severe cases, leg ulcers. An estimated 20–50% of those with DVT will develop it, and 5–10% will develop the severe form. PE is the most serious complication of proximal DVT, and the risk of PE is higher when clots are present in the thigh and pelvis. Distal DVT itself is hardly if ever associated with post-thrombotic syndrome or PE. Untreated lower extremity DVT has a 3% PE-related mortality rate, while deaths associated with upper extremity DVT are extremely rare. The presence of a remaining thrombus after a DVT frequently occurs in a minority of people, and it increases the risk of recurrence, though to a lesser extent than an elevated D-dimer. In the 10 years following a VTE, approximately a third of individuals will have a recurrent episode. About 1 in 1000 adults per year has DVT, but as of 2011, available data is dominated by North American and European populations. VTE is rare in children, with an incidence of about 1 in 100,000 a year. From childhood to old age, incidence increases by a factor of about 1000, with almost 1% of the elderly experiencing VTE yearly. During pregnancy and after childbirth, acute VTE occurs about once per 1000 deliveries. After surgery with preventative treatment, VTE develops in about 10 of 1000 people after total or partial knee replacement, and in about 5 of 1000 after total or partial hip replacement. About 300,000–600,000 Americans develop VTE each year, with about 60,000–100,000 deaths attributable to PE. In England, an estimated 25,000 a year die from hospital-related VTE. For unclear reasons, people of Asian descent have a lower VTE risk than whites. In North American and European populations, around 4–8% of people have a thrombophilia, most commonly factor V leiden and prothrombin G20210A. For populations in China, Japan, and Thailand, deficiences in protein S, protein C, and antithrombin predominate. Non-O blood type is present in around 50% of the general population and varies with ethnicity, and it is present in about 70% of those with VTE. Altogether, global data is incomplete. Initial DVT costs for an average hospitalized patient in the U.S. are around $7,700–$10,800. VTE follow-up costs at three months, six months, and a year are about $5,000, $10,000, and $33,000 respectively; in Europe, the three and six-month figures are about €1,800 and €3,200. Post-thrombotic syndrome is a significant contributor to DVT follow-up costs. Annual DVT costs in the U.S. are an estimated $5 billion or in excess of $8 billion, and the average annual cost per treated individual is thought to be about $20,000. As an example, if 300,000 symptomatic DVT patients were treated at costs averaging $20,000 annually, that would cost $6 billion a year. The earliest case of DVT was described by Sushruta in his book Sushruta Samhita around 600–900 BC. Another documented case is thought to have occurred in the 13th century, in the leg of a 20-year-old male. At some point, the increased incidence of DVT in women after childbirth was noticed, and in the late 1700s, a public health recommendation was issued to encourage women to breastfeed as a means to prevent this phenomenon; the DVT was called "milk leg", as it was thought to result from milk building up in the leg. In 1856, German physician and pathologist Rudolf Virchow published what is referred to as Virchow's triad, the three major causes of thrombosis. The triad provides the theoretical framework for the current explanation of venous thrombosis, although it was focused on the effect of a foreign body in the venous system and the conditions required for clot propagation. Multiple pharmacological therapies for DVT were introduced in the 20th century: oral anticoagulants in the 1940s, subcutaneous LDUH in 1962 and subcutaneous LMWH in 1982. Diagnoses were commonly performed by impedance plethysmography in the 1970s and 1980s, but the use of Doppler ultrasound techniques, with their increased sensitivity and specificity, largely superseded this method. As of 2011, three large randomized controlled trials—the Norwegian CaVent trial, the North American ATTRACT trial, and the Dutch CAVA trial—are studying the effectiveness and safety of catheter-directed thrombolysis. In 2012, two studies found a clinical benefit in taking aspirin to prevent recurrent VTE. - Thrombosis associated with the abdominal organs (viscera)—such as portal vein thrombosis, renal vein thrombosis, and Budd–Chiari syndrome—are separate diseases excluded from the scope of this definition. - Third-generation combined oral contraceptives (COCs) have an approximate two to three times higher risk than second-generation COCs. Progestogen-only pill use is not associated with increased VTE risk. - The term 'thrombophilia' as used here applies to the five inherited abnormalities of antithrombin, protein C, protein S, factor V, and prothrombin, as is done elsewhere. - Factor V Leiden and prothrombin G20210A are present in about 3–5% and 1–3%, respectively, of people of European descent. - Factor V Leiden increases the risk of DVT more than it does for PE, a phenomenon referred to as the factor V Leiden paradox. - VTE might cause the observed inflammation. - An elevated level is greater than 250 ng/mL D-dimer units (DDU) or greater than 0.5 μg/mL fibrinogen equivalent units (FEU). A normal level is below these values. - Page e197S of Kahn et al. specifies that the guideline does not apply to those with "trauma and spinal cord injury" nor does it apply to those "with ischemic and hemorrhagic stroke." - Evidence for anticoagulation comes from studies other than definitive randomized controlled trials that demonstrate efficacy and safety for anticoagulation vs. placebo or using NSAIDs. - The international normalized ratio should be ≥ 2.0 for 24 hours minimum, but if the ratio is > 3.0, then the parenteral anticoagulant is not needed for five days. - "What are the signs and symptoms of deep vein thrombosis?". National Heart, Lung, and Blood Institute. 28 October 2011. Retrieved 15 April 2012. - Scarvelis D, Wells P (2006). "Diagnosis and treatment of deep-vein thrombosis". CMAJ. 175 (9): 1087–92. doi:10.1503/cmaj.060366. PMC . PMID 17060659. - Bates SM, Jaeschke R, Stevens SM, et al. (2012). "Diagnosis of DVT: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e351S–e418S. doi:10.1378/chest.11-2299. PMC . PMID 22315267. - Hargett CW, Tapson VF; Tapson (2008). "Clinical probability and D-dimer testing: How should we use them in clinical practice?". Semin Respir Crit Care Med. 29 (1): 15–24. doi:10.1055/s-2008-1047559. PMID 18302083. - Arumilli BR, Lenin Babu V, Paul AS; Lenin Babu; Paul (2008). "Painful swollen leg – think beyond deep vein thrombosis or Baker's cyst". World J Surg Oncol. 6: 6. doi:10.1186/1477-7819-6-6. PMC . PMID 18205917. - Chinsakchai, K; Ten Duis, K; Moll, FL; de Borst, GJ (January 2011). "Trends in management of phlegmasia cerulea dolens.". Vascular and Endovascular Surgery. 45 (1): 5–14. doi:10.1177/1538574410388309. PMID 21193462. - Owings JT (2005). "Management of venous thromboembolism". ACS Surgery. American College of Surgeons. Retrieved 16 January 2012. - Barham K, Shah T; Shah (2007). "Images in clinical medicine: Phlegmasia cerulea dolens". N Engl J Med. 356 (3): e3. doi:10.1056/NEJMicm054730. PMID 17229945. - Martinelli I, Bucciarelli P, Mannucci PM (2010). "Thrombotic risk factors: Basic pathophysiology". Crit Care Med. 38 (suppl 2): S3–S9. doi:10.1097/CCM.0b013e3181c9cbd9. PMID 20083911. - Bovill EG, van der Vliet A (2011). "Venous valvular stasis-associated hypoxia and thrombosis: What is the link?". Annu Rev Physiol. 73: 527–45. doi:10.1146/annurev-physiol-012110-142305. PMID 21034220. - Reitsma PH, Versteeg HH, Middeldorp S (2012). "Mechanistic view of risk factors for venous thromboembolism". Arterioscler Thromb Vasc Biol. 32 (3): 563–8. doi:10.1161/ATVBAHA.111.242818. PMID 22345594. - Lijfering WM, Rosendaal FR, Cannegieter SC (2010). "Risk factors for venous thrombosis – current understanding from an epidemiological point of view". Br J Haematol. 149 (6): 824–33. doi:10.1111/j.1365-2141.2010.08206.x. PMID 20456358. - Wong P, Baglin T (2012). "Epidemiology, risk factors and sequelae of venous thromboembolism". Phlebology. 27 (suppl 2): 2–11. doi:10.1258/phleb.2012.012S31. PMID 22457300. - Mantha, S.; Karp, R.; Raghavan, V.; et al. (2012). "Assessing the risk of venous thromboembolic events in women taking progestin-only contraception: A meta-analysis". BMJ. 345: e4944. doi:10.1136/bmj.e4944. PMC . PMID 22872710. - de Jong PG, Coppens M, Middeldorp S; Coppens; Middeldorp (2012). "Duration of anticoagulant therapy for venous thromboembolism: Balancing benefits and harms on the long term". British Journal of Haematology. 158 (4): 433–41. doi:10.1111/j.1365-2141.2012.09196.x. PMID 22734929. - Qaseem A, Chou R, Humphrey LL, et al. (2011). "Venous thromboembolism prophylaxis in hospitalized patients: A clinical practice guideline from the American College of Physicians". Annals of Internal Medicine. 155 (9): 625–32. doi:10.7326/0003-4819-155-9-201111010-00011. PMID 22041951. - Varga EA, Kujovich JL; Kujovich (2012). "Management of inherited thrombophilia: Guide for genetics professionals". Clin Genet. 81 (1): 7–17. doi:10.1111/j.1399-0004.2011.01746.x. PMID 21707594. - Middeldorp S (2011). "Is thrombophilia testing useful?" (PDF). Hematology Am Soc Hematol Educ Program. 2011 (1): 150–5. doi:10.1182/asheducation-2011.1.150. PMID 22160027. - Shaheen K, Alraies MC, Alraiyes AH, et al. (2012). "Factor V Leiden: How great is the risk of venous thromboembolism?" (PDF). Cleve Clin J Med. 79 (4): 265–72. doi:10.3949/ccjm.79a.11072. PMID 22473726. - Rosendaal FR, Reitsma PH (2009). "Genetics of venous thrombosis". J Thromb Haemost. 7 (suppl 1): 301–4. doi:10.1111/j.1538-7836.2009.03394.x. PMID 19630821. - Dentali F, Sironi AP, Ageno W, et al. (2012). "Non-O blood type is the commonest genetic risk factor for VTE: Results from a meta-analysis of the literature". Semin Thromb Hemost. 38 (5): 535–48. doi:10.1055/s-0032-1315758. PMID 22740183. - Palareti G, Schellong S; Schellong (2012). "Isolated distal deep vein thrombosis: What we know and what we are doing". J Thromb Haemost. 10 (1): 11–9. doi:10.1111/j.1538-7836.2011.04564.x. PMID 22082302. - Jackson E, Curtis KM, Gaffield ME (2011). "Risk of venous thromboembolism during the postpartum period: A systematic review". Obstet Gynecol. 117 (3): 691–703. doi:10.1097/AOG.0b013e31820ce2db. PMID 21343773. - Baglin T (2012). "Inherited and acquired risk factors for venous thromboembolism". Semin Respir Crit Care Med. 33 (2): 127–37. doi:10.1055/s-0032-1311791. PMID 22648484. - Turpie AGG (March 2008). "Deep venous thrombosis". The Merck's Manuals Online Medical Library. Merck. - Mai C, Hunt D (2011). "Upper-extremity deep venous thrombosis: A review". Am J Med. 124 (5): 402–7. doi:10.1016/j.amjmed.2010.11.022. PMID 21531227. - Tichelaar YI, Kluin-Nelemans HJ, Meijer K (2012). "Infections and inflammatory diseases as risk factors for venous thrombosis. A systematic review". Thromb Haemost. 107 (5): 827–37. doi:10.1160/TH11-09-0611. PMID 22437808. - Zöller B, Li X, Sundquist J, et al. (2012). "Risk of pulmonary embolism in patients with autoimmune disorders: A nationwide follow-up study from Sweden". Lancet. 379 (9812): 244–9. doi:10.1016/S0140-6736(11)61306-8. PMID 22119579. - Stephens, MB (Feb 1, 1997). "Deep venous thrombosis of the upper extremity.". American family physician. 55 (2): 533–9. PMID 9054222. - Brown, Jeremy (2008). Oxford American Handbook of Emergency Medicine. p. 453.9. ISBN 9780199779482. - van Langevelde K, Flinterman LE, van Hylckama Vlieg A, et al. (2012). "Broadening the factor V Leiden paradox: Pulmonary embolism and deep-vein thrombosis as 2 sides of the spectrum". Blood. 120 (5): 933–46. doi:10.1182/blood-2012-02-407551. PMID 22496157. - Jenkins PV, Rawley O, Smith OP, et al. (2012). "Elevated factor VIII levels and risk of venous thrombosis". Br J Haematol. 157 (6): 653–63. doi:10.1111/j.1365-2141.2012.09134.x. PMID 22530883. - Chan WS, Spencer FA, Ginsbergm JS (2010). "Anatomic distribution of deep vein thrombosis in pregnancy". CMAJ. 182 (7): 657–60. doi:10.1503/cmaj.091692. PMC . PMID 20351121. - Saha P, Humphries J, Modarai B, et al. (2011). "Leukocytes and the natural history of deep vein thrombosis: Current concepts and future directions". Arterioscler Thromb Vasc Biol. 31 (3): 506–12. doi:10.1161/ATVBAHA.110.213405. PMC . PMID 21325673. - "What is deep vein thrombosis?". National Heart, Lung, and Blood Institute. 28 October 2011. Retrieved 26 July 2012. - Kim ES, Bartholomew JR. "Venous thromboembolism". Disease Management Project. Cleveland Clinic. Retrieved 15 February 2011. - López JA, Chen J (2009). "Pathophysiology of venous thrombosis". Thromb Res. 123 (suppl 4): S30–S34. doi:10.1016/S0049-3848(09)70140-9. PMID 19303501. - "DDI/9290 clinical: D-dimer, plasma". Mayo Medical Laboratories. Retrieved 27 August 2012. - Iorio A, Kearon C, Filippucci E, et al. (2010). "Risk of recurrence after a first episode of symptomatic venous thromboembolism provoked by a transient risk factor: A systematic review". Arch Intern Med. 170 (19): 1710–6. doi:10.1001/archinternmed.2010.367. PMID 20975016. - Conklin P, Soares GM, Dubel GJ, et al. (2009). "Acute deep vein thrombosis (DVT): Evolving treatment strategies and endovascular therapy" (PDF). Med Health R I. 92 (12): 394–7. PMID 20066826. - Rao AS, Konig G, Leers SA, et al. (2009). "Pharmacomechanical thrombectomy for iliofemoral deep vein thrombosis: An alternative in patients with contraindications to thrombolysis". J Vasc Surg. 50 (5): 1092–8. doi:10.1016/j.jvs.2009.06.050. PMID 19782528. - Lloyd NS, Douketis JD, Moinuddin I, et al. (2008). "Anticoagulant prophylaxis to prevent asymptomatic deep vein thrombosis in hospitalized medical patients: A systematic review and meta-analysis". J Thromb Haemost. 6 (3): 405–14. doi:10.1111/j.1538-7836.2007.02847.x. PMID 18031292. - Font C, Farrús B, Vidal L, et al. (2011). "Incidental versus symptomatic venous thrombosis in cancer: A prospective observational study of 340 consecutive patients". Annals of Oncology. 22 (9): 2101–6. doi:10.1093/annonc/mdq720. PMID 21325446. - Hofmann LV, Kuo WT (2012). "Catheter-directed thrombolysis for acute DVT". Lancet. 379 (9810): 3–4. doi:10.1016/S0140-6736(11)61875-8. PMID 22172245. - Johnson SA, Stevens SM, Woller SC, et al. (2010). "Risk of deep vein thrombosis following a single negative whole-leg compression ultrasound: A systematic review and meta-analysis". JAMA. 303 (5): 438–45. doi:10.1001/jama.2010.43. PMID 20124539. - Welch 2010, p. 2. - Galanaud JP, Bosson JL, Quéré I (2011). "Risk factors and early outcomes of patients with symptomatic distal vs. proximal deep-vein thrombosis". Current Opinion in Pulmonary Medicine. 17 (5): 387–91. doi:10.1097/MCP.0b013e328349a9e3. PMID 21832920. - Heit JA, Mohr DN, Silverstein MD, et al. (2000). "Predictors of recurrence after deep vein thrombosis and pulmonary embolism: A population-based cohort study". Arch Intern Med. 160 (6): 761–8. doi:10.1001/archinte.160.6.761. PMID 10737275. - Spencer FA, Emery C, Lessard D, et al. (2006). "The Worcester venous thromboembolism study: A population-based study of the clinical epidemiology of venous thromboembolism". J Gen Intern Med. 21 (7): 722–7. doi:10.1111/j.1525-1497.2006.00458.x. PMC . PMID 16808773. - Casella IB, Bosch MA, Sabbag CR (2009). "Incidence and risk factors for bilateral deep venous thrombosis of the lower limbs". Angiology. 60 (1): 99–103. doi:10.1177/0003319708316897. PMID 18504268. - Guyatt et al. 2012, p. 16S: 3.1. - National Institute for Health and Clinical Excellence. Clinical guideline 144: Venous thromboembolic diseases: the management of venous thromboembolic diseases and the role of thrombophilia testing. London, 2012. - Geersing GJ, Zuithoff NP, Kearon C, Anderson DR, Ten Cate-Hoek AJ, Elf JL, et al. (2014). "Exclusion of deep vein thrombosis using the Wells rule in clinically important subgroups: individual patient data meta-analysis.". BMJ. 348: g1340. doi:10.1136/bmj.g1340. PMC . PMID 24615063. - Adam SS, Key NS, Greenberg CS (2009). "D-dimer antigen: Current concepts and future prospects". Blood. 113 (13): 2878–87. doi:10.1182/blood-2008-06-165845. PMID 19008457. - Guyatt et al. 2012, p. 17S: 3.4. - Guyatt et al. 2012, p. 16S: 3.2. - Guyatt et al. 2012, p. 16S: 3.3. - Guyatt et al. 2012, p. 19S: 5.3. & 6.1. - Hecht 2010, p. 47. - Keeling D, Baglin T, Tait C, et al. (2011). "Guidelines on oral anticoagulation with warfarin – fourth edition" (PDF). Br J Haematol. 154 (3): 311–24. doi:10.1111/j.1365-2141.2011.08753.x. PMID 21671894. - Guyatt et al. 2012, p. 11S: 7.1. - Stewart DW, Freshour JE (2013). "Aspirin for the Prophylaxis of Venous Thromboembolic Events in Orthopedic Surgery Patients: A Comparison of the AAOS and ACCP Guidelines with Review of the Evidence". Annals of Pharmacotherapy. 47 (1): 63–74. doi:10.1345/aph.1R331. PMID 23324504. - Warkentin TE (2012). "Aspirin for dual prevention of venous and arterial thrombosis". N Engl J Med. 367 (21): 2039–41. doi:10.1056/NEJMe1211480. PMID 23121404. - Li, L; Zhang, P; Tian, JH; Yang, K (18 December 2014). "Statins for primary prevention of venous thromboembolism.". The Cochrane database of systematic reviews. 12: CD008203. doi:10.1002/14651858.CD008203.pub3. PMID 25518837. - Simes, J; Becattini, C; Agnelli, G; Eikelboom, JW; Kirby, AC; Mister, R; Prandoni, P; Brighton, TA; INSPIRE Study Investigators (International Collaboration of Aspirin Trials for Recurrent Venous, Thromboembolism) (23 September 2014). "Aspirin for the prevention of recurrent venous thromboembolism: the INSPIRE collaboration.". Circulation. 130 (13): 1062–71. doi:10.1161/circulationaha.114.008828. PMID 25156992. - Lederle FA, Zylla D, MacDonald R, et al. (2011). "Venous thromboembolism prophylaxis in hospitalized medical patients and those with stroke: A background review for an American College of Physicians clinical practice guideline". Annals of Internal Medicine. 155 (9): 602–15. doi:10.1059/0003-4819-155-9-201111010-00008. PMID 22041949. - Alikhan, R; Bedenis, R; Cohen, AT (7 May 2014). "Heparin for the prevention of venous thromboembolism in acutely ill medical patients (excluding stroke and myocardial infarction).". The Cochrane database of systematic reviews. 5: CD003747. doi:10.1002/14651858.CD003747.pub4. PMID 24804622. - Kahn SR, Lim W, Dunn AS, et al. (2012). "Prevention of VTE in nonsurgical patients: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e195S–e226S. doi:10.1378/chest.11-2296. PMC . PMID 22315261. - Guyatt et al. 2012, p. 10S: 2.3., 2.4., & 2.7.1. - Guyatt et al. 2012, p. 10S: 2.7.2. - Guyatt et al. 2012, pp. 10S–11S: 3.4.3. & 3.4.4. - Guyatt et al. 2012, p. 11S: 4.2.2. - Sobieraj DM, Lee S, Coleman CI, et al. (2012). "Prolonged versus standard duration venous thromboprophylaxis in major orthopedic surgery: A systematic review". Annals of Internal Medicine. 156 (10): 720–7. doi:10.1059/0003-4819-156-10-201205150-00423. PMID 22412039. - Falck-Ytter Y, Francis CW, Johanson NA, et al. (2012). "Prevention of VTE in orthopedic surgery patients: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e278S–e325S. doi:10.1378/chest.11-2404. PMC . PMID 22315265. - Gould MK, Garcia DA, Wren SM, et al. (2012). "Prevention of VTE in nonorthopedic surgical patients: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e227S–e277S. doi:10.1378/chest.11-2297. PMC . PMID 22315263. - Kakkos SK, Caprini JA, Geroulakos G, et al. (2008). Kakkos SK, ed. "Combined intermittent pneumatic leg compression and pharmacological prophylaxis for prevention of venous thromboembolism in high-risk patients". Cochrane Database Syst Rev (4): CD005258. doi:10.1002/14651858.CD005258.pub2. PMID 18843686. - Sachdeva, A; Dalton, M; Amaragiri, SV; Lees, T (17 December 2014). "Graduated compression stockings for prevention of deep vein thrombosis.". The Cochrane database of systematic reviews. 12: CD001484. doi:10.1002/14651858.CD001484.pub3. PMID 25517473. - Marik PE, Plante LA (2008). "Venous thromboembolic disease and pregnancy". N Engl J Med. 359 (19): 2025–33. doi:10.1056/NEJMra0707993. PMID 18987370. - Bates SM, Greer IA, Middeldorp S, et al. (2012). "VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e691S–e736S. doi:10.1378/chest.11-2300. PMC . PMID 22315276. - Guyatt et al. 2012, pp. 39S–40S: 3.0. - Guyatt et al. 2012, p. 38S: 3.0.1. - Guyatt et al. 2012, p. 11S: 6.1.1. & 6.1.2. - "New DVT guidelines: No evidence to support "economy-class syndrome"; oral contraceptives, sitting in a window seat, advanced age, and pregnancy increase DVT risk in long-distance travelers". American College of Chest Physicians. 7 February 2012. Retrieved 10 February 2012. - Guyatt et al. 2012, p. 11S: 6.1.2. & 6.1.3. - Clarke M, Hopewell S, Juszczak E, et al. (2006). Clarke MJ, ed. "Compression stockings for preventing deep vein thrombosis in airline passengers". Cochrane Database Syst Rev (2): CD004002. doi:10.1002/14651858.CD004002.pub2. PMID 16625594. - "Deep venous thrombosis". A.D.A.M. Medical Encyclopedia. PubMed Health. 19 February 2012. Retrieved 2 July 2012. - Cundiff DK, Manyemba J, Pezzullo JC (2006). Cundiff, David K, ed. "Anticoagulants versus non-steroidal anti-inflammatories or placebo for treatment of venous thromboembolism". Cochrane Database Syst Rev (1): CD003746. doi:10.1002/14651858.CD003746.pub2. PMID 16437461. - Douketis J, Tosetto A, Marcucci M, et al. (2010). "Patient-level meta-analysis: Effect of measurement timing, threshold, and patient age on ability of D-dimer testing to assess recurrence risk after unprovoked venous thromboembolism". Annals of Internal Medicine. 153 (8): 523–31. doi:10.1059/0003-4819-153-8-201010190-00009. PMID 20956709. - Guyatt et al. 2012, p. 20S: 2.4. - Kearon C, Akl EA, Comerota AJ, et al. (2012). "Antithrombotic therapy for VTE disease: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): e419S–e494S. doi:10.1378/chest.11-2301. PMC . PMID 22315268. - Guyatt et al. 2012, p. 20S: 2.5.1. - Guyatt et al. 2012, pp. 20S–21S: 3.1. - Strijkers RH, Cate-Hoek AJ, Bukkems SF, et al. (2011). "Management of deep vein thrombosis and prevention of post-thrombotic syndrome". BMJ. 343: d5916. doi:10.1136/bmj.d5916. PMID 22042752. - Guyatt et al. 2012, p. 22S: 3.2. - Hutten BA, Prins MH (2006). Prins, Martin H, ed. "Duration of treatment with vitamin K antagonists in symptomatic venous thromboembolism". Cochrane Database Syst Rev (1): CD001367. doi:10.1002/14651858.CD001367.pub2. PMID 16437432. - Guyatt et al. 2012, p. 21S: 3.1.1. - Guyatt et al. 2012, p. 21S: 3.1.2. - Guyatt et al. 2012, p. 22S: 3.1.4. - Guyatt et al. 2012, p. 22S: 126.96.36.199. - Guyatt et al. 2012, p. 22S: 188.8.131.52. - Guyatt et al. 2012, p. 22S: 184.108.40.206. - Guyatt et al. 2012, p. 22S: 220.127.116.11. - Guyatt et al. 2012, p. 21S: 2.7. - Guyatt et al. 2012, p. 23S: 4.1. - Berntsen, CF; Kristiansen, A; Akl, EA; Sandset, PM; Jacobsen, EM; Guyatt, G; Vandvik, PO (April 2016). "Compression Stockings for Preventing the Postthrombotic Syndrome in Patients with Deep Vein Thrombosis.". The American Journal of Medicine. 129 (4): 447.e1–447.e20. doi:10.1016/j.amjmed.2015.11.031. PMID 26747198. - Guyatt et al. 2012, p. 21S: 2.14. - American Physical Therapy Association (15 September 2014), "Five Things Physicians and Patients Should Question", Choosing Wisely: an initiative of the ABIM Foundation, American Physical Therapy Association, retrieved 15 September 2014, which cites - Aissaoui, Nadia; Martins, Edith; Mouly, Stéphane; Weber, Simon; Meune, Christophe (2009). "A meta-analysis of bed rest versus early ambulation in the management of pulmonary embolism, deep vein thrombosis, or both". International Journal of Cardiology. 137 (1): 37–41. doi:10.1016/j.ijcard.2008.06.020. ISSN 0167-5273. - Anderson, Cathy M.; Overend, Tom J.; Godwin, Julie; Sealy, Christina; Sunderji, Aisha (2009). "Ambulation after Deep Vein Thrombosis: A Systematic Review". Physiotherapy Canada. 61 (3): 133–140. doi:10.3138/physio.61.3.133. ISSN 0300-0508. - Guyatt et al. 2012, p. 20S: 2.3. - Young T, Tang H, Hughes R (2010). Young, Tim, ed. "Vena caval filters for the prevention of pulmonary embolism". Cochrane Database Syst Rev (2): CD006212. doi:10.1002/14651858.CD006212.pub4. PMID 20166079. - Guyatt et al. 2012, p. 21S: 2.13.1.–3. - Guyatt et al. 2012, p. 21S: 2.13. - Watson L, Broderick C, Armon MP (23 Jan 2014). "Thrombolysis for acute deep vein thrombosis". Cochrane Database Syst Rev. 1: CD002783. doi:10.1002/14651858.CD002783.pub3. PMID 24452314. - Guyatt et al. 2012, p. 21S: 2.9 & 2.10. - Guyatt et al. 2012, p. 21S: 2.11. - Kahn SR (2009). "How I treat postthrombotic syndrome". Blood. 114 (21): 4624–31. doi:10.1182/blood-2009-07-199174. PMID 19741190. - "Deep vein thrombosis/pulmonary embolism (DVT/PE)". Centers for Disease Control and Prevention. 8 June 2012. Retrieved 5 July 2012. - Severinsen MT, Johnsen SP, Tjønneland A, et al. (2010). "Body height and sex-related differences in incidence of venous thromboembolism: A Danish follow-up study". Eur J Intern Med. 21 (4): 268–72. doi:10.1016/j.ejim.2010.03.013. PMID 20603033. - Zakai NA, McClure LA (2011). "Racial differences in venous thromboembolism". J Thromb Haemost. 9 (10): 1877–82. doi:10.1111/j.1538-7836.2011.04443.x. PMID 21797965. - Rosendaal 2009, p. 5. - Januel JM, Chen G, Ruffieux C, et al. (2012). "Symptomatic in-hospital deep vein thrombosis and pulmonary embolism following hip and knee arthroplasty among patients receiving recommended prophylaxis: A systematic review". JAMA. 307 (3): 294–303. doi:10.1001/jama.2011.2029. PMID 22253396. - Young A, Chapman O, Connor C, et al. (2012). "Thrombosis and cancer". Nature Reviews Clinical Oncology. 9 (8): 437–49. doi:10.1038/nrclinonc.2012.106. PMID 22777060. - Margaglione M, Grandone E (2011). "Population genetics of venous thromboembolism: A narrative review". Thromb Haemost. 105 (2): 221–31. doi:10.1160/TH10-08-0510. PMID 20941456. - "Blood types". American Red Cross. Retrieved 15 August 2012. - Hamasaki N (2012). "Unmasking Asian thrombophilia: is APC dysfunction the real culprit?". J Thromb Haemost. 10 (10): 2016–8. doi:10.1111/j.1538-7836.2012.04893.x. PMID 22905992. - Dobesh PP (2009). "Economic burden of venous thromboembolism in hospitalized patients". Pharmacotherapy. 29 (8): 943–53. doi:10.1592/phco.29.8.943. PMID 19637948. - Ruppert A, Steinle T, Lees M (2011). "Economic burden of venous thromboembolism: A systematic review". J Med Econ. 14 (1): 65–74. doi:10.3111/13696998.2010.546465. PMID 21222564. - Grosse SD (2012). "Incidence-based cost estimates require population-based incidence data. A critique of Mahan et al." (PDF). Thromb Haemost. 107 (1): 192–3. doi:10.1160/TH11-09-0666. PMID 22159589. - Mahan CE, Holdsworth MT, Welch SM, et al. (2011). "Deep-vein thrombosis: A United States cost model for a preventable and costly adverse event". Thromb Haemost. 106 (3): 405–15. doi:10.1160/TH11-02-0132. PMID 21833446. - Mahan CE, Holdsworth MT, Welch SM, et al. (2012). "Long-term attack rates, as compared with incidence rates, may provide improved cost-estimates in venous thromboembolism. A reply to S. D. Grosse". Thromb Haemost. 107 (1): 194–5. doi:10.1160/TH11-11-0802. - Goodman, LR (Oct 2013). "In search of venous thromboembolism: the first 2913 years.". AJR. American journal of roentgenology. 201 (4): W576–81. doi:10.2214/AJR.13.10604. PMID 24059395. - Rosendaal 2009, p. 3. - Bagot CN, Arya R (2008). "Virchow and his triad: A question of attribution". Br J Haematol. 143 (2): 180–9. doi:10.1111/j.1365-2141.2008.07323.x. PMID 18783400. - Dalen 2003, p. 2. - Dalen 2003, p. 3. - Becattini C, Agnelli G, Schenone A, et al. (2012). "Aspirin for preventing the recurrence of venous thromboembolism". N Engl J Med. 366 (21): 1959–67. doi:10.1056/NEJMoa1114238. PMID 22621626. - Brighton TA, Eikelboom JW, Mann K, et al. (2012). "Low-dose aspirin for preventing recurrent venous thromboembolism". N Engl J Med. 367 (21): 1979–87. doi:10.1056/NEJMoa1210384. PMID 23121403. - Dalen, James E. (2003). Venous thromboembolism. CRC Press. ISBN 978-0-8247-5645-1. - Guyatt, G. H.; Akl, E. A.; Crowther, M.; et al. (2012). "Executive Summary: Antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical practice guidelines". Chest. 141 (suppl 2): 7S–47S. doi:10.1378/chest.1412S3. PMC . PMID 22315257. - Hecht, M. E. (2010). A practical guide to hip surgery: From pre-op to recovery. Sunrise River Press. ISBN 978-1-934716-12-0. - Rosendaal, Frits R. (2009). van Beek, Edwin J. R.; Büller, Harry R.; Oudkerk, Mathijs, eds. Deep vein thrombosis and pulmonary embolism. John Wiley & Sons. ISBN 978-0-470-74499-4. - Welch, Ellen (2010). Venous thromboembolism: A nurse's guide to prevention and management. John Wiley & Sons. ISBN 978-0-470-51189-3.	2	80	23	0	3	1	0.901828	27	15,692
The Department of Health today published statistics relating to medical abortions and terminations of pregnancy during 2017/18. The statistical bulletin presents information on the number of medical abortions and terminations of pregnancy during 2017/18. It details information on the number of terminations of pregnancy by HSC Trust, by country of residence and by age band. The information release is published on the Department of Health website. - There were 12 terminations of pregnancy in HSC hospitals in Northern Ireland during 2017/18. This was one less than the previous year (Table 1). It should be noted that due to small numbers, relatively large fluctuations may be expected from year to year. - All of the 12 terminations of pregnancy during 2017/18 were carried out on women normally resident in Northern Ireland (Table 3). - During 2017/18 there were 8 terminations of pregnancy carried out on women aged 30 years and over, 4 termination of pregnancy carried out on a woman aged 25 to 29, and none on women aged 24 and under (Table 4). Notes to editors: - The source of this information is the HSC Trusts in Northern Ireland. - The structure of the terminology used within the UK National Clinical Coding Standards ICD-10 4th Edition (2013) Reference Book is as follows: Abortion: The term abortion means the expulsion or extraction of all (complete) or any part (incomplete) of the placenta or membranes (products of conception) without an identifiable foetus or without a liveborn infant, or stillborn, before the 24th completed week of gestation. This covers a range of codes within the ICD-10 structure and includes ‘missed miscarriages’ (the retention of a dead foetus before 24 completed weeks of gestation, with no signs of abortion), spontaneous abortions (known miscarriages) and terminations of pregnancy. Medical Abortion: The term medical abortion within the confines of the use of the ICD-10 code O04 – Medical Abortion equates to the interruption of a live pregnancy for legally acceptable, medically approved conditions. It also includes re-admission with retained products of conception following a previous termination of pregnancy only. Termination of Pregnancy: This is defined as any patient who has a live pregnancy terminated for Northern Ireland legally acceptable, medically approved conditions. This is a subset of the term ‘medical abortion’. - Following a public consultation carried out by the Health and Social Care Information Centre, the UK National Clinical Coding Standards were revised for 2013. As a result, the definition of ‘medical abortion’ has been significantly altered. The revised definition (provided in point 2) has been applied throughout the UK from 1st April 2013. The change in definition of medical abortion has resulted in a break in the time series of medical abortions; consequently, figures from 2013/14 onwards are not comparable to figures for before then. There has been no change to the definition of a termination of pregnancy. Further information on Northern Ireland Terminations of Pregnancy is available from: Hospital Information Branch Department of Health Annexe 2, Castle Buildings Telephone: 028 90 522914 - For media enquiries please contact the Department of Health Press Office team on 028 9052 0575 or by email. For out of hours please contact the Duty Press Officer on 028 9037 8110. - Follow us on twitter @healthdpt. - Inquiry into Hyponatraemia-Related Deaths – Duty of Candour update 14 February 2019 - Further evaluation of the impact of the registration of NI social care workers published 28 January 2019 - Emergency Care Waiting Time Statistics (October - December 2018) 24 January 2019 - 23 January 2019	3	13	0	0	0	2	0.360203	2	766
Nature Communications have such a database. Human symptoms–disease network XueZhong Zhou, Jörg Menche, Albert-László Barabási & Amitabh Sharma Published: 26 June 2014 They count the number of times a disease and a symptom keywords both appear on the same PudMed article, and assign a TF-IDF value to each pair. In their words: Acquisition of symptom and ... I think the closest thing to a "standardization of drugs" are the pharmacopeia: (just some examples) As you will notice, these are not global standards, but each one defines the parameters by which a drug is of adequate quality and purity to be deemed that drug. To generate a global standard, it would be ... The idea that the International Statistical Classification of Diseases and Related Health Problems (ICD) forms an international standard on health problems is contentious within some groups of people when there is the Diagnostic and Statistical Manual of Mental Disorders (DSM), which cross-references the ICD and there can sometimes be conflicting criteria ... What you're looking for is used by AI or Bayesian networks for online diagnosis. These databases are commercially sensitive and likely only accessible by API rather than raw data. For example, ISABEL, has been around for 20 years 12-4-2020 84279 - 78891 = 5388 11-4-2020 78891 - 70272 = 8619 10-4-2020 70272 - 65077 = 5195 It looks like the PHE data is a day ahead of the ECDC data The USDA database does provide detailed information about fatty acids in foods. Too detailed actually: for each food, you can get the amount of fatty acids with each number of carbon atoms. So in your specific case, you would have to get this data (for instance as .csv), and sum up the respective amounts of short- and medium-chain fatty acids (until 12 ... According to the little number on the lower left, this is from 2012. The situation is worse than I expected in Europe. Also, there is a map from the states: It will be so cool to ...	2	6	1	0	0	0	0.56156	1	460
AED Devices \| Heart Defibrillator Machines & Accessories (On Sale Now) Defibrillators, also know as AED Machine (Automated External Defibrillators), are medical devices designed to stop heart fibrillation or irregular heartbeats. Essentially, they de-fibrillate the heart. This treatment intervention emits a high energy electrical pulse of 50 to 360 joules. The energy pulse stimulates the heart back to normal operation. This heart resuscitation treatment restores an uneven, too fast or too slow heart pattern to its normal, healthy heartbeat. An irregular heartbeat or arrhythmia places the patient at risk of cardiac arrest. Cardiac arrest interrupts the flow of oxygen and blood throughout the body. The lack of oxygen and blood produces organ failure and death. Cardiac defibrillators jump-start the heart to transition back to a regular heartbeat and proper function. Click for more information on How to Select the Best Defibrillator Starting at: $19.64 Starting at: $26.82 Starting at: $4,178.50 Starting at: $747.50 Out of stock Starting at: $676.00 Out of stock Starting at: $637.00 Out of stock Starting at: $35.10 Out of stock - What Are Arrhythmias? - What Are the Components of a Defibrillator? - How Do They Work? - What Are the Types of Defibrillators? - AED Defibrillator - Defibrillator Comparison Chart - Top Ranking Manufacturers - Medical Studies What are Arrhythmias? Arrhythmias are erratic heartbeats. There are two types of arrhythmias that are particularly dangerous. The first is ventricular fibrillation (VF). This is when the heart experiences very rapid beating or an abnormal rhythm. This condition causes the heart to quiver uselessly instead of pumping blood throughout your body. The reduced blood supply results in organ failure. The other is ventricular tachycardia (VT). VT is when the heart speeds up more than 100 beats per minute with at least 3 irregular heartbeats in a row. This condition does not allow the heart to fill with blood before it contracts; thereby, reducing the blood supply. Either condition may lead to cardiac arrest. What Are the Components of a Defibrillator? The AED Machines for sale basically consist of a power source, an inductor, a capacitor, a rectifier, a trans-former and electrodes. These components draw energy from the power source, store it, modulate it, and deliver the energy pulse to provide pulmonary resuscitation. The electrodes are placed on the chest of the patient, transferring the charge to their chest cavity. An automated defibrillator machine has the addition of a monitoring device, algorithm and a series of prompts for the user. These supplemental components determine the current condition of the arrhythmia and the amount of energy required to resuscitate. An automated defibrillator may be operated by almost anyone. One study found that 11 to 12 year olds could follow the prompts and successfully administer life-saving defibrillation. Key AED Optional Features An AED with a rechargeable battery makes sense for devices that are going to be used often. Non-rechargeable batteries have a service life of around four years, upon which, the batteries must be replaced. Rechargeable batteries need to be replaced every 2 years and have a rechargeable time of 2 to 15 hours. Users that employ rechargeable batteries should have extra batteries available for when recharging is taking place to keep their AED device always ready for an emergency service. Semi-automatic AED device notify the user when to press a button to administer the electrical pulse. Fully-automatic AED devices, instead, do not require the user to press a button to administer the jolt. The automatic AED electronically senses the heartbeat pattern of the patient and auto adjusts the energy pulse to meet the needs of the patient. This pulse is fired automatically without the need of the user to activate the pulse manually. The automatic versions warn the user to not touch the patient and counts-down to the pulse to ensure the safety of the user. AED’s that have an ECG display allow the user to visualize the ECG waveform on an LCD screen. The user can then interpret for themselves the condition of the patient, allowing them to override the AED’s assessment and deliver a manual pulse to the patient. This function is usually reserved for professional devices. Defibrillator Procedure - How Do Defibrillators Work? Defibrillators work through a biphasic truncated waveform (BTE) or electrical shock that depolarizes the heart muscles. Depolarizing allows the heart’s normal electrical impulse to re-engage itself. This brief electric impulse interrupts the heart fibrillation and allows the heart muscles to return to their normal sequence and operational pattern. What Are the Types of Defibrillators For Sale There are three types of defibrillators. First are public access defibrillators that are simple and easy to use. They are commonly referred to as Automated External Defibrillators (AED). AED's do not require a prescription and have been found usable by children down to 11 to 12 years old. That's right, an 11-year old can use an AED device to save someone experiencing cardiac arrhythmia. Second are personal devices that are either internally implanted in the patient or worn externally by the patient. These devices are called Implantable Cardioverter Defibrillators (ICD) or Wearable Cardioverter Defibrillators (WCD). Lastly, there are professional devices more commonly used by hospitals, clinics, or first responders called Advanced Life Support (ALS). AED Defibrillator (Home Defibrillator) This heart stimulator is portable and automatically detects arrhythmia and then calculates the amount of electrical charge needed to stimulate the heart to regain normal operation. An automated external defibrillator works by attaching the adhesive electrodes to the patient’s chest. The AED then senses the heartrate and determines if defibrillation treatment is necessary. If necessary, it automatically calculates the amount of electrical charge required and prompts the user to initiate the pulse to the patient. Most AED for sale use audio prompts to guide the user step-by-step through the heart resuscitation process. The use of defibrillator is for anyone who can follow simple prompts. This type of defibrillator implants in the patient’s chest or abdomen. It is with the patient 24/7 as a heart monitor and emergency defibrillator. When the device detects a fibrillation, it automatically initiates a treatment pulse to restore normal heart rhythm. Modern ICD’s act as both a defibrillator and pacemaker. Wearable cardioverter defibrillators are similar to ICD’s but are external and non-invasive. Instead, the patient simply wears this personal defibrillator. ALS devices constitute the next step after an external defibrillator fails to treat an arrhythmia attack. When connected to the patient, it monitors their heart rhythm and allows medical personnel to manually induce an electrical charge. The device analyzes the patient and recommends an electrical dosage to sufficiently stimulate the patient’s heart. These devices are used only by professionals in hospitals, clinics or ambulances. How to Choose the Best Defibrillator Health practitioners usually select the ICD’s, WCD’s or ALS’s for the patient. However, AED selection usually takes place by someone in the company, business, facility or home. Choosing the best AED for home use is the same as for a business or facility in terms of selection criteria. Below is a selection continuum to graphically see where the different features for AED defibrillators lie followed by a list of purchase considerations and to help you buy defibrillators on sale. Selecting the Best Defibrillator - Continuum of Product Features Key Selection Criteria - Self-test, self-diagnostics - Voice prompts - Text prompts - LED prompts - Automated functions - Battery life - Battery recharging - CPR coaching - CPR feedback - ECG data - Pediatric adaptability - Interchangeable electrode pads - Auto-escalating electrical shock therapy - FDA-approved defibrillator device - Warranty length and coverage Defibrillator Comparison Chart Top Ranking Manufacturers Below is a alphabetical list of the top six manufacturers of cardiac treatment devices. - Cardiac Science - Philips Healthcare - Deakin, Charles D., and Jerry P. Nolan. "European Resuscitation Council Guidelines for Resuscitation 2005: Section 3. Electrical therapies: Automated external defibrillators, defibrillation, cardioversion and pacing." Resuscitation 67 (2005): S25-S37. - Verbeek, P. Richard, et al. "Derivation of a termination‐of‐resuscitation guideline for emergency medical technicians using automated external defibrillators." Academic Emergency Medicine 9.7 (2002): 671-678. - Drezner, Jonathan A., et al. "Use of automated external defibrillators at NCAA Division I universities." Medicine and science in sports and exercise 37.9 (2005): 1487-1492. - Swor, Robert, et al. "Cardiac arrests in schools: assessing use of automated external defibrillators (AED) on school campuses." Resuscitation 84.4 (2013): 426-429. - Rothmier, Justin D., Jonathan A. Drezner, and Kimberly G. Harmon. "Automated external defibrillators in Washington State high schools." British journal of sports medicine (2007). - Descatha, Alexis, and Michel Baer. "Automated external defibrillators in the workplace." BMJ/BMJ (CLINICAL RESEARCH ED); Br Med J; British Medical Journal; Brit Med J 337 (2008): a1816. - Marenco, John P., et al. "Improving survival from sudden cardiac arrest: the role of the automated external defibrillator." Jama 285.9 (2001): 1193-1200. - van Alem, Anouk P., et al. "Use of automated external defibrillator by first responders in out of hospital cardiac arrest: prospective controlled trial." Bmj 327.7427 (2003): 1312. - Cummins, Richard O, et al. "Sensitivity, accuracy, and safety of an automatic external defibrillator: report of a field evaluation." The Lancet 324.8398 (1984): 318-320. - Weaver, W. Douglas, et al. "Use of the automatic external defibrillator in the management of out-of-hospital cardiac arrest." New England Journal of Medicine 319.11 (1988): 661-666. - Klein, Helmut U., et al. "Bridging a temporary high risk of sudden arrhythmic death. Experience with the wearable cardioverter defibrillator (WCD)." Pacing and Clinical Electrophysiology 33.3 (2010): 353-367. - Kao, Andrew C., et al. "Wearable defibrillator use in heart failure (WIF): results of a prospective registry." BMC cardiovascular disorders 12.1 (2012): 123. - Dillon, Katie A., Steven J. Szymkiewicz, and Thomas E. Kaib. "Evaluation of the effectiveness of a wearable cardioverter defibrillator detection algorithm." Journal of electrocardiology43.1 (2010): 63-67. - White, Roger D., and James K. Russell. "Refibrillation, resuscitation and survival in out-of-hospital sudden cardiac arrest victims treated with biphasic automated external defibrillators." Resuscitation 55.1 (2002): 17-23. - Callaham, Michael, and Christopher D. Madsen. "Relationship of timeliness of paramedic advanced life support interventions to outcome in out-of-hospital cardiac arrest treated by first responders with defibrillators." Annals of emergency medicine27.5 (1996): 638-648.	2	9	0	0	0	1	0.838631	1	2,619
I'm a member You will be redirected to myBlue. Would you like to continue? Please wait while you are redirected. Please enter a username and password. Printer Friendly Version Portable devices have been developed to provide point-of-care (POC) nerve conductions studies (NCSs). These devices have computational algorithms that are able to drive stimulus delivery, measure and analyze the response, and provide a report of study results. Automated nerve conduction could be used in various settings, including primary care, without the need for specialized training or equipment. Nerve conductions studies (NCS) and needle electromyography (EMG), when properly performed by a trained practitioner, are considered the criterion standard of electrodiagnostic testing. However, the need for specialized equipment and personnel may limit the availability of electrodiagnostic testing for some patients. One proposed use of automated nerve conduction devices is to assist in the diagnosis of carpal tunnel syndrome (CTS). CTS is a pressure-induced entrapment neuropathy of the median nerve as it passes through the carpal tunnel, resulting in sensorimotor disturbances. This syndrome is defined by its characteristic clinical symptoms, which may include pain, subjective feelings of swelling, and nocturnal paresthesia. A variety of simple diagnostic tools are available, and a positive response to conservative management (steroid injection, splints, modification of activity) can confirm the clinical diagnosis. Electrodiagnostic studies may also be used to confirm the presence or absence of a median neuropathy at the wrist, assess the severity of the neuropathy, and assess alternate associated diagnoses. Nerve conduction is typically assessed before surgical release of the carpal tunnel, but the use of EMG in the diagnosis of CTS is controversial. Point-of-care nerve conduction testing has also been proposed for the diagnosis of peripheral neuropathy and, in particular, for detecting neuropathy in patients with diabetes. Peripheral neuropathy is relatively common in patients with diabetes mellitus, and the diagnosis is often made clinically through the physical examination. Diabetic peripheral neuropathy can lead to important morbidity including pain, foot deformity, and foot ulceration. Clinical practice guidelines recommend using simple sensory tools such as the 10-g Semmes-Weinstein monofilament or the 128-Hz vibration tuning fork for diagnosis. These simple tests predict the presence of neuropathy defined by electrophysiologic criteria with a high level of accuracy. Electrophysiologic testing may be used in research studies and may be required in cases with an atypical presentation. NC-stat® by NeuroMetrix is a portable nerve conduction test device designed to be used at the point-of-care. The system comprises a biosensor array, an electronic monitor, and a remote report generation system. The biosensor is a single use, preconfigured array consisting of a stimulation anode and cathode, skin surface digital thermometer, and response sensor. Biosensor arrays are available for assessment of sensory and motor nerves of the wrist (median and ulnar), and for the foot (peroneal, posterior tibial, and sural). A chip imbedded in the biosensor panel measures skin surface temperature, the analysis algorithm adjusts for differences in temperature from 30º C, or if skin surface temperature is less than 23º C, the monitor will indicate that limb warming is necessary. Data are sent to a remote computer via a modem in the docking station, and the remote computer generates a report based on the average of 6 responses that is sent back by fax or email. In addition to the automated stimulus delivery and reporting, NC-stat® analysis adjusts the calculation for body temperature, height, and weight, and uses the average of 6 responses. Sensitivity of the device for sensory nerve amplitude potentials is 2.1 µV; values lower than this are analyzed as zero, and responses with artifact are automatically eliminated from the analysis. The NeuroMetrix ADVANCE™ is a point-of-care test that includes the ability to perform needle EMG in addition to surface electrodes for the performance of nerve conduction studies. The NC-stat® DPN-Check™ is a handheld device that is applied to the skin posterior to the lateral malleolus in the area that overlies the distribution of the sural nerve. The device includes a biosensor pad, metal stimulating probes, and a thermometer that corrects for skin temperature. NC-stat® DPN-Check™ is designed specifically for NCS of the sural nerve for the assessment of diabetic peripheral neuropathy. The Axon-II™ (PainDx) is an automated system that is being marketed for the detection of various sensory neurologic impairments caused by various pathologic conditions or toxic substance exposures, including signs of sympathetic dysfunction and detection of down-regulated A-delta function to locate injured nerve(s). The Axon-II software works with the Neural-Scan™ system (Neuro Diagnostics) and lists 7 automated studies (Cervical, Thoracic, Lumbar, Upper Extremities, Lower Extremities, Neuroma, Trigeminal), as well as a custom study. The Neural-Scan™ is a voltage-actuated sensory nerve conduction test device, which measures the voltage amplitude necessary to cause a discernible nerve impulse. Results are adjusted and compared with population means; the most severe hypoesthesia is considered the primary lesion. Several devices are now being marketed for point-of-care neural conduction testing. NeuroMetrix received specific clearance to market NC-stat® via the U.S. Food and Drug Administration's (FDA) 510(k) process in 1998, listing as predicate devices the TECA model-10 electromyograph and the Neurometer® by Neurotron, which measures vibration threshold. The FDA-listed intended use was "to measure neromuscular signals that are useful in diagnosing and evaluating systemic and entrapment neuropathies." In addition, the approved application stated that "The NC-stat is intended to be used as an adjunct to and not a replacement for conventional electrodiagnostic measurements." The 2004 510(k) added a sural biosensor for use in diagnosing neuropathies affecting the sural nerve (K041320). NeuroMetrix also received FDA clearance to market models with biosensors and engineering changes that enable the NC-stat to be used for motor and sensory nerves of the wrist (median, ulnar) and foot (peroneal, tibial, and sural). The intended use as listed on the 510(k) approval from 2006 (K060584) is "to stimulate and measure neuromuscular signals that are useful in diagnosing and evaluating systemic and entrapment neuropathies." The original NC-stat is no longer being produced. The current NeuroMetrix device, NC-stat® DPN-Check™, assesses the sural nerve. The NeuroMetrix ADVANCE™ system received marketing clearance in 2008 (K070109). It is intended to perform nerve conduction studies using disposable surface electrodes (similar to NC-stat) with an additional module for invasive needle EMG. NeuroMetrix lists NC-stat as a predicate device for the ADVANCE™ system. Brevio® from Neurotron Medical received marketing clearance from FDA in 2001. The Brevio® is intended “for use for the measurement of nerve response latency and amplitude in the diagnosis and monitoring of peripheral neuropathies.” The XLTek Neuropath (Excel- Tech) received clearance for marketing through the FDA’s 510(k) process in 2006; the indications are the same as those for NC-stat®. POLICYAutomated nerve conduction tests are considered investigational. POLICY EXCEPTIONSFederal Employee Program (FEP) may dictate that all FDA-approved devices, drugs or biologics may not be considered investigational and thus these devices may be assessed only on the basis of their medical necessity. POLICY GUIDELINESNerve conduction studies performed without needle electromyography studies, unless contraindicated (i.e., an individual receiving anticoagulant therapy) are considered investigational. Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting. The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language. POLICY HISTORY3/2/2007: Policy added 3/22/2007: Reviewed and approved by Medical Policy Advisory Committee (MPAC) 6/14/2007: Code Reference section updated per quarterly HCPCS and Category III revisions 10/11/2007: Code Reference section reviewed. CPT 95900, 95903, and 95904 removed from policy as non-covered for an automated point of care nerve conduction test (Note: Standard nerve conduction tests may be covered with these Copts); a specific HCPCS code for an automated point of care nerve conduction test became effective 7-1-2007 7/6/2009: Policy reviewed, description updated, policy statement unchanged 11/03/2010: Policy description section revised to provide a list of devices and research findings regarding portable automated nerve conduction tests compared to standard testing. Policy statement unchanged. FEP verbiage added to the Policy Exceptions section. Added the following statement to the Policy Guidelines: Nerve conduction studies performed without needle electromyography studies, unless contraindicated (i.e., an individual receiving anticoagulant therapy) are considered investigational. Code Reference section revised to add 95905, 95999, and G0255 to the Non-Covered Codes Table. A note was also added to state that CPT Codes 95900, 95903 and 95904 should not be used to bill for automated nerve conduction testing. 08/02/2011: Policy reviewed; no changes. 07/17/2012: Policy reviewed; no changes. 09/03/2013: Policy reviewed; no changes. 08/01/2014: Policy reviewed; description updated regarding devices. Policy statement unchanged. 07/08/2015: Code Reference section updated for ICD-10. 10/28/2015: Policy description updated regarding devices. Policy statement unchanged. Investigative definition updated in the policy guidelines section. SOURCE(S)Blue Cross Blue Shield Association Policy # 2.01.77 CODE REFERENCEThis is may not be a comprehensive list of procedure codes applicable to this policy.	4	7	6	0	2	0	0.41619	8	2,399
What is Telehealth? Telehealth is the use of technology for the delivery of health care, health information, or health education at a distance. Although there are several applications used for telehealth, the most popular applications are teleradiology, continuing professional education, and home monitoring. Teleradiology is used to send test results to another facility for diagnosis; continuing professional education consists of presentations given by specialists to general practitioners; and home monitoring is provided by nurses as a supplement to home visits. Telehealth consists of two general types of applications. One is referred to as real-time communication, and the other is called store-and-forward. Real-time is when a patient and nurse practitioner consult with a specialist by way of an audio-visual link, or when a doctor and patient communicate in an exam room via an interpreter through the phone or webcam. Another example of real-time communication would be if, say, a cardiologist holds a teleconference with internists about new best practices in treating angina. Store-and-forward is when digital images, such as in radiology or dermatology, are transmitted for diagnosis. Telehealth – What Is It and What Changes Are Coming In 2015? It is important to note that all telehealth applications require health information technology (IT), but not all uses of health IT are considered telehealth. Examples of non-telehealth applications would be such stand-alone systems as electronic health records (EHRs) and computerized decision support (CDS). In summary, telehealth is a way to increase the communication between a patient and the health system, and it can also bring more expertise to consult on a case, reaching out to patients when they live far from the facility. This technology not only eliminates the time it takes to travel, but it also reduces the expense for both physicians and patients, and is beneficial to improving preventative medicine and the treatment of chronic conditions. What Changes Are Coming In Telehealth? As of January 1, 2015, there will be some major changes that will take effect in telehealth services, according to the Physician Fee Schedule Final Rule released by CMS. Reimbursement rates will increase with regard to telehealth services to telehealth originating locations by 0.8 percent. Also, there are seven new reimbursement codes for 2015 related to telehealth. They include: Psychotherapy services (CPT codes 90845, 90846, and 90847) Prolonged services in the office (CPT codes 99354 and 99355) Annual wellness visits (HCPCS codes G0438 and G0239) As far as billing is concerned, the originating site fee will be $24.83. There is also a new CPT code 99490, which permits doctors to bill Medicare for management of chronic care when a patient has two or more chronic conditions, including heart failure and diabetes. The monthly, unadjusted, non-facility fee will be $42.60. Since 99490 is a physician service, it is available across the country and is not just for rural telehealth. Therefore, patients are not expected to be present. CPT code 99490 can be bundled with 99091 for the collection and interpretation of physiologic data, but CMS still does not allow any further reimbursements for this service. They do, however, allow providers to count the data review time invested toward the monthly minimum time for billing for chronic care management. CMS will also allow two valuable service codes: 99090 – Analysis of clinical data stored in computers (eg, ECGs, blood pressures, hematologic data). 99091 – Collection and interpretation of physiologic data (eg, ECG, blood pressure, glucose monitoring) digitally stored and/or transmitted by the patient and/or caregiver to the physician or other qualified health care professional, qualified by education, training, licensure/regulation (when applicable) requiring a minimum of 30 minutes of time. Remote patient monitoring of chronic conditions will be paid by Medicare with a monthly, unadjusted, non-facility fee of $56.92 when using CPT code 99091. Previously they only covered services when they were bundled with evaluation and management codes. Traditionally, Medicare has provided little coverage for telehealth services. However, this new CMS rule is a big step and will allow physicians to improve costs and quality of healthcare by using telehealth technology. Therefore, it is imperative that medical coders and billers understand how to accurately bill for these services. By Debbie Jones, CPC-A, CCA	4	7	12	0	2	0	0.223822	14	945
May 21, 2012 Coding for Seizures and Epilepsy For The Record Vol. 24 No. 10 P. 28 Seizures are episodes of abnormal electrical brain activity that cause changes in attention or behavior. The term “convulsions” may be used interchangeably with seizures, but during a convulsion, the body rapidly and uncontrollably shakes. Epilepsy is a brain disorder in which a person has repeated seizures. Also called a seizure disorder, epilepsy may be diagnosed when the patient has two or more unprovoked seizures. A seizure episode is classified to ICD-9-CM code 780.39, Other convulsions. This code also includes convulsive disorder not otherwise specified (NOS), fit NOS, and recurrent convulsions NOS. Basically, code 780.39 is for the single episode of a seizure. Subcategory 780.3, Convulsions, includes the following codes: • 780.31, Febrile convulsions (simple), unspecified; • 780.32, Complex febrile convulsions; and • 780.33, Posttraumatic seizures. Epilepsy and recurrent seizures are classified to category 345. Subcategory 345.9 includes the following diagnoses: • Epileptic convulsions, fits, or seizures NOS; • Seizure disorder NOS; and • Recurrent seizure disorder NOS. Types of Epilepsy There are two main categories of epilepsy: partial (also called local or focal) and generalized. Partial seizures occur in only one part of the brain. The following are two common types of partial epilepsy: • Simple focal seizure is a type of partial epilepsy where the awareness is retained and does not result in loss of consciousness. It may alter emotions or change the patient’s senses, such as taste or smell. This type of epilepsy is classified to subcategory 345.5, Localization-related (focal) (partial) epilepsy and epileptic syndromes with simple partial seizures. • Complex focal seizure (subcategory 345.4) alters consciousness resulting in staring or nonpurposeful movements such as hand rubbing, chewing, lip smacking, and walking in circles. Generalized seizures involve all parts of the brain. The following are the six types of generalized seizures: • Absence seizures (petit mal): characterized by blank staring and subtle body movements that begin and end abruptly. It may cause a brief loss of consciousness. • Tonic seizures: causes stiffening of the muscles and may cause the patient to fall to the ground. • Clonic seizures: characterized by rhythmic, jerking muscle contractions that affect both sides of the body at the same time. • Myoclonic seizures: associated with sudden brief jerks or twitches on both sides of the body. • Atonic seizures: causes patients to lose muscle tone, so they subsequently collapse. • Tonic-clonic seizures (grand mal): most intense type of epilepsy causing loss of consciousness, muscle rigidity, and convulsions. Subcategory 345.0, Generalized nonconvulsive epilepsy, includes atonic and typical absences, minor and pykino-epilepsy, petit mal, and akinetic and atonic seizures. Subcategory 345.1, Generalized convulsive epilepsy, includes clonic, myoclonic, tonic, and tonic-clonic epileptic seizures; grand mal; major and progressive myoclonic epilepsy; and Unverricht-Lundborg disease. Notice that petit mal is classified to subcategory 345.0, and grand mal is classified to subcategory 345.1. However, there are also codes available for petit mal status (345.2) and grand mal status (345.3). Status epilepticus indicates a state of continuous seizure activity lasting for a significant amount of time or having frequent recurrent seizures without regaining full consciousness in between them. This is a life-threatening condition because the brain cannot get enough oxygen to survive. Most of the codes in category 345 require a fifth digit subclassification to complete the code. Fifth digit 0 is for without mention of intractable epilepsy. Fifth digit 1 is with intractable epilepsy and includes the terms “pharmaco-resistant,” “poorly controlled,” “refractory,” or “treatment resistant.” Codes 345.2, Petit mal status, and 345.3, Grand mal status, do not require fifth digit subclassification. Seizures documented as the late effect of stroke are classified to code 438.89, Other late effects of cerebrovascular disease. An additional code may be assigned for the specific type of seizure/seizure disorder (code 780.39 or category 345) documented. Coding and sequencing for seizures and epilepsy are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding. — This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447. ICD-10-CM Coding for Seizures and Epilepsy Coding for seizures and epilepsy in ICD-10-CM is similar to ICD-9-CM. ICD-10-CM category G40 is titled “Epilepsy and recurrent seizures.” The following are the fourth character subcategories for epilepsy: • G40.0, Localization-related (focal) (partial) idiopathic epilepsy and epileptic syndromes with seizures of localized onset; • G40.1, Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with simple partial seizures; • G40.2, Localization-related (focal) (partial) symptomatic epilepsy and epileptic syndromes with complex partial seizures; • 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; • G40.5, Epileptic seizures related to external causes; • G40.8, Other epilepsy and recurrent seizures; and • G40.9, Epilepsy, unspecified. Notice that the above subcategories are mainly separated by localized vs. generalized. In addition, there are separate codes if the epilepsy is idiopathic vs. symptomatic. The fifth and sixth characters will identify the presence or absence of status epilepticus and intractable epilepsy. Convulsions, not elsewhere classified are classified to category R56 and includes the following subcategories: • R56.0, Febrile convulsions; • R56.1, Posttraumatic seizures; and • R56.9, Unspecified convulsions (which includes seizures NOS).	4	7	0	0	0	5	0.793455	5	1,595
- Open Access Ellis-Van Creveld syndrome Orphanet Journal of Rare Diseases volume 2, Article number: 27 (2007) Ellis-van Creveld syndrome (EVC) is a chondral and ectodermal dysplasia characterized by short ribs, polydactyly, growth retardation, and ectodermal and heart defects. It is a rare disease with approximately 150 cases reported worldwide. The exact prevalence is unknown, but the syndrome seems more common among the Amish community. Prenatal abnormalities (that may be detected by ultrasound examination) include narrow thorax, shortening of long bones, hexadactyly and cardiac defects. After birth, cardinal features are short stature, short ribs, polydactyly, and dysplastic fingernails and teeth. Heart defects, especially abnormalities of atrial septation, occur in about 60% of cases. Cognitive and motor development is normal. This rare condition is inherited as an autosomal recessive trait with variable expression. Mutations of the EVC1 and EVC2 genes, located in a head to head configuration on chromosome 4p16, have been identified as causative. EVC belongs to the short rib-polydactyly group (SRP) and these SRPs, especially type III (Verma-Naumoff syndrome), are discussed in the prenatal differential diagnosis. Postnatally, the essential differential diagnoses include Jeune dystrophy, McKusick-Kaufman syndrome and Weyers syndrome. The management of EVC is multidisciplinary. Management during the neonatal period is mostly symptomatic, involving treatment of the respiratory distress due to narrow chest and heart failure. Orthopedic follow-up is required to manage the bones deformities. Professional dental care should be considered for management of the oral manifestations. Prognosis is linked to the respiratory difficulties in the first months of life due to thoracic narrowness and possible heart defects. Prognosis of the final body height is difficult to predict. Ellis-van Creveld syndrome (MIM 225500) Ellis van Creveld syndrome (EVC) is an autosomal recessive skeletal dysplasia, with inter- and intra-familial variability, characterized by short ribs, short limbs, postaxial polydactyly, and dysplastic teeth and nails. Congenital heart defects occur in 60% of the individuals. Mutations in the EVC1 and EVC2 genes are associated with this syndrome. EVC is a rare disease. The exact prevalence remains unknown. About 100 cases have been reported between the first full description of the syndrome in 1940, by R. Ellis and S. Van Crefeld , and 1968 . Since 1968, approximately 50 other cases have been reported in the literature. EVC is found with an increased frequency among the Amish community in Lancaster Country, Pennsylvania, US, where the largest pedigree has been described: 52 cases in 30 sib ships . Prenatal abnormalities may be early discovered, after the 18th gestation week; they include narrow thorax, marked shortening of the long bones, hexadactyly of hands and feet, and cardiac defect [11, 12], leading to discussing the diagnosis of short rib-polydactyly syndromes (cf. below). Increased first-trimester fetal nuchal translucency thickness in association with ECV has been described at 13th week of gestation . After birth, the cardinal features usually present are: 1) disproportionate small stature whith increasing severity from the proximal to distal portions of the limbs, and shortening of the middle and distal phalanges (Figure 1); 2) polydactyly affecting hands (uni [exceptional: ] – or, usual, bilateral) and, occasionally, the feet (Figure 2); 3) hidrotic ectodermal dysplasia mainly affecting the nails, hair and teeth; 4) congenital heart malformations occurring in about 50–60% of cases and comprising of single atrium, defects of the mitral and tricuspid valves, patent ductus, ventricular septal defect, atrial septal defect and hypoplastic left heart syndrome. The presence of congenital heart disease may support the diagnosis of the EVC syndrome and appears to be the main determinant of longevity [6, 14]. The oral manifestations spectrum is wide, including malocclusion, labiogingival adherences and gingival hypertrophy, labiogingival frenulum hypertrophy, accessory labiogingival frenula, serrated incisal margins, dental transposition, diastrema, conical teeth, enamel hypoplasia and hypodontia (Figure 3). Teeth may be prematurely erupted, at birth, or exfolliate prematurely . Several inconstant additional clinical findings are described, including strabismus, epi- and hypospadias, cryptorchidism , and thoracic wall and pulmonary malformations . Renal abnormalities are found in very rare cases with agenesis, dysplasia, megaureter and nephrocalcinosis. Lethal nephronophthisis has been reported only once, in a patient with short limbs, short ribs, abnormal teeth and polydactyly (considered as EVC) , but this diagnosis may be discussed. Hematologic abnormalities have also been rarely reported: one case with dyserythropoiesis and another associated with perinatal myeloblastic leukemia . Head circumference and mental developement in EVC are normal. Because of the overlap between EVC and other short ribs-chondrodysplasias, and because molecular studies are only recently available and non-constantly performed, some old reports of EVC associated with exceptional features should be carefully read, since they may be mixed with publications that evidently do not deal with the EVC syndrome. The possibility of manifestations in heterozygous carriers has been discussed for a long time. From the observation of the large Amish kindred, McKusick concluded that there is no heterozygous manifestation of EVC . However, other authors described polydactyly in relatives of four unrelated EVC families [20, 21]. Spranger and Tariverdian described a father of an EVC patient with fingers and teeth abnormalities, and then reviewed other reports of possible heterozygous manifestations. The Weyers acrofacial dysostosis, an autosomal dominant disorder described in 1952, is characterized by variable extremities and facial features. This condition has been found to be associated with EVC and EVC2 mutations that, finally, confirmed that Weyers dysostosis represents the heterozygous expression of the mutation, which, in homozygous form, causes the autosomal recessive disorder EVC [23, 24]. A variety of radiological skeletal features may be observed, including retarded bone maturation, fusion of the hamate and capitate bones of the wrist, defect of the lateral aspect of the proximal part of the tibia (knock-knees), cubitus valgus, hypoplastic cubitus, supernumerary carpal bone centre, clinodactyly of the 5th finger, fusion of the 5th and 6th metacarpals, disturbance in bone modeling of the metacarpals and/or phalanges. Bone age is usually retarded. The EVC gene was previously localized by linkage analysis to the distal short arm of chromosome 4 [25, 26], in an area proximal to other chondrodystrophias. Mutations in this gene have been identified in EVC individuals from the Amish cases and from other pedigrees (Mexico, Equador and Brazil) . But the screening of the 21 EVC coding exons in 58 patients with EVC permitted to identify only 13 patients with homozygous mutations. Among the remaining 45 cases, no mutation in one or two allele was identified . The cDNA analysis of fibroblast RNA from three of these patients was also normal. These observations had raised the possibility of genetic heterogeneity. A second gene, EVC2, was identified in an Ashkenasi child, immediately adjacent to EVC (named EVC1 or EVC), in a head to head configuration . Expression of the gene is found in heart, placenta, lung, liver and skeletal muscle. EVC2 spans 166.4 kb and shares a common promoter region with EVC. The transcriptional start sites of EVC and EVC2 are separated by only 1643 bp. There is no significant sequence homology between EVC and EVC2 at either protein or nucleic levels. The EVC2 gene encodes a protein predicted to have one transmembrane segment, three coiled-coil regions, and one RhoGEF domain (SMART). The EVC2 protein has significant sequence homology with the tail domains of class IX non muscle myosins (BLAST). Mutations in the LIMBIN gene, a bovine orthologue of EVC2, are associated with bovine chondrodysplastic dwarfism . Affected EVC individuals with mutations in EVC or EVC2 are phenotypically indistinguishable . An interesting recent work with sequencing EVC and EVC2 in a series of 65 EVC patients identified EVC mutations in 20 families and EVC2 mutations in 25 families . No mutation has been identified in 20/65 families but as none of these individuals had consanguineous parents (even if it was possible that some of these cases were misdiagnosed), this has lead to evidence for a genetic heterogeneity in EVC syndrome. As previously mentioned, heterozygous mutations in the EVC or EVC2 genes also cause Weyers acrofacial dysostosis, an allelic disorder, showing autosomal dominant inheritance. The clinical diagnosis is based on observation of the symptoms and manifestations described above and supported by the skeletal survey. The definitive diagnosis is molecular, based on homozygosity for a mutation in the EVC and EVC2 genes by direct sequencing. EVC belongs to the short rib-polydactyly group (SRP). These SRPs are all autosomal recessive disorders that have been classified into types (Saldino-Noonan syndrome, type I; Majewski syndrome, type II; Verma-Naumoff syndrome, type III; Beemer-Langer syndrome, type IV; and Jeune Dystrophy). They are characterized by hypoplastic thorax due to short ribs, short limbs, frequent polydactyly and visceral abnormalities, and are discussed prenatally. Radiographically and histologically, SRP III (Verma-Naumoff syndrome, OMIM 263510) most resembles some forms of EVC [30, 31]. The question of SRP being due to mutation in the EVC1 gene was excluded by Takamine et al. . Postnatally, the essential differential diagnoses include Jeune dystrophy, McKusick-Kaufman syndrome and Weyers syndrome. Jeune dystrophy (MIM 208500) is characterized by thoracic dystrophy, shortening of the extremities and generalized bone dysplasia. Similarities and differences of patients with EVC and Jeune dystrophy have been stressed [33, 34]. There are no specific constant features to confirm the diagnosis of presumptive EVC but some features, including congenital heart disease, supernumerary digits and ectodermal dysplasia will mostly support the diagnosis of EVC syndrome than Jeune dystrophy. EVC and McKusick-Kaufman syndrome (MKK, MIM 236700), both recessively inherited disorders, share postaxial polydactyly and congenital heart defect. Distinguishing characteristics are the osteochondrodysplasia and ectodermal anomalies in EVC syndrome, and hydro metrocolpos in MKK syndrome. MKK is caused by mutations in a gene on chromosome 20p12, encoding a protein similar to members of the chaperonin family. Mutation in the same gene causes Bardet-Biedl syndrome-6 . Weyers acrodental dysostosis (OMIM 193530) is, as mentioned above, the heterozygotous manifestation of the EVC gene. Disproportionate dwarfism, heart defect and thoracic dysplasia are not present in this autosomal dominant condition. EVC syndrome is an autosomal recessive disorder, with a mendelian risk of 25% for subsequent pregnancies. EVC may be detected prenatally by ultrasound examination. The association of several structural fetal defects in the late first trimester, including narrow thorax, short and bowed long bones, rounded metaphyses, postaxial polydactyly, and cardiac defect may suggest the diagnosis ECV. Increased nuchal translucency thickness has been described associated with ECV . In case of potential recurrence, prenatal diagnosis using molecular genetic techniques is, in theory, feasible, on DNA extracted from chorionic villus samples. The management of EVC is multidisciplinary. Symptomatic management is mostly required in the neonatal period, including treatment of the respiratory distress due both to narrow chest and heart failure. Neonatal teeth should be removed because they may impair the feeding. In infancy and early adulthood, general and specialized pediatric follow-up are also required: the short stature is considered resulting of chondrodysplasia of the legs and the possible treatment with growth hormone is considered ineffective. It is important to notice, however, that the association of growth hormone deficiency and ECV has been reported in one patient and, in this case, the growth hormone treatment had a favorable effect on growth . The possibility of bones deformity, especially knee valgus with depression of the lateral tibial plateau and dislocation of the patella , needs regular orthopedic follow-up. Dentists play an important role in control of dental and oral manifestations. Dental treatment must be performed under prophylactic antibiotic coverage with consideration for the high incidence of cardiac defects in EVC patients. In addition, as in other chondrodysplasias, rare narrowing of the spinal canal can occur and justify a regular follow-up during adult life. Although there is no systematic follow-up EVC series reported, the prognosis is linked to the respiratory difficulties in the first months of life and these difficulties are related to thoracic narrowness and possible heart defect. We insist on the fact that the cognitive development in EVC syndrome is normal. Prognosis of the final body height in individual patients with EVC is difficult to predict, as the rare publications of adult EVC cases report a variable final stature, from 119 cm to 161 cm . The clinical spectrum of EVC syndrome is not well delineated at present. There are no specific constant features in EVC syndrome: some of them are usually present but their absence does not exclude the diagnosis. Depending on the diagnostic criteria, some patients may be recognized as EVC and other patients not. In the literature, there are many cases described as EVC syndrome in which the diagnosis is uncertain, and some of them definitely represent other entities. The reported data are limited to small series. Careful collection of case history, genotype-phenotype correlation with the two known genes and patient follow-up will provide supplementary information and further delineation of the EVC syndrome, and distinction from the other short rib-polydactyly syndromes. ECV is associated with a genetic heterogeneity and EVC1 and EVC2 do not account for the totality of EVC cases. Further studies are needed to elucidate other genes involved in EVC manifestations. They could also contribute to unraveling specific molecular processes that lead to the phenotypic manifestations of ECV. Ellis RW, van Crefeld S: A syndrome characterized by ectodermaldysplasia, polydactyly, chondrodysplasia and congenital morbus cardia. Arch Dis Child. 1940, 15: 65. Lynch JI, Perry LW, Takakvwa T, Scott LP: Congenital heart disease and chondroextodermal dysplasia. Am J Dis Child. 1968, 115: 80-87. Mac Kusick V: Ellis-van Crefeld syndrome and the Amish. Nature Genet. 2000, 24: 203-204. 10.1038/73389. Oliveira da Siva E, Janovitz D, Cavalcanti de Albuquerque S: Ellis-van Creveld syndrome: report of 15 cases in an inbred kindred. J Med Genet. 1980, 17: 349-356. Waldrigues A, Grohmann L, Takahashi T, Reis H: Ellis van Creveld. An inbred kindred with 5 cases. Rev Bras de Pesquisas Med e Biol. 1977, 10: 193-198. Blackburn M, Belliveau R: Ellis-vanCreveld syndrome: a report of previously undescribed anomalies in two siblings. Am J Dis Child. 1971, 122: 267-270. Prabhu S, Daftary D, Dholakia H: Chondroectodermal dysplasia (Ellis-van Creveld syndrome): report of 2 cases. J Oral Surgery. 1978, 36: 631-638. Renier JC, Larget-Piet L, Boasson M, Berthelot J, Fouillet L: Dysplasie chondroépidermique d'Ellis-van Creveld: deux cas dans une même fratrie. Revue du rhumatisme. 1975, 42: 417-422. Goor M, Farriaux J, Dupuis C, François P, Fontaine G: Le syndrome d'Ellis-van Creveld: étude d'une observation familiale. La revue de Pédiatrie. 1970, 4: 233-236. Kushnick T, Paya K, Namunes P: Chondroectodermal Dysplasia. Am J Dis Child. 1962, 103: 77-80. Horigome H, Hamada H, Sohda S, Oyake Y, Kurosaki Y: Prenatal ultrasonic diagnosis of a case of Ellis-van Creveld syndrome with a single atrium. Pediatr Radiol. 1997, 27: 942-944. 10.1007/s002470050277. Venkat-Raman N, Sebire N, Murphy K: Increased first-trimester fetal nuchal translucenty thickness in association with chondroectodermal dysplasia (Ellis-van Creveld). Ultrasound Obstet Gynecol. 2005, 25: 412-414. 10.1002/uog.1849. Engle M, Ehlers K: Ellis-van Creveld syndrome with asymmetric polydactyly and successful surgical correction of common atrium. Birth defects Orig Art Ser. 1969, V: 65-67. Digilio M, Marino B, Ammirati A, Borgaza U, Giannotti A, Dallapiccola B: Cardiac malformations in patients with oral-facial-skeletal syndromes: clinical similarities with heterotaxia. Am J Med Genet. 1999, 84: 350-356. 10.1002/(SICI)1096-8628(19990604)84:4<350::AID-AJMG8>3.0.CO;2-E. Winter G, Geddes M: Oral manifestations of chondroectodermal dysplasia (Ellis-Van Creveld Syndrome). Report of a case. Br Dent J. 1967, 122: 103-107. Moore T: Chondroectodermal dysplasia (Ellis-van Creveld syndrome) with bronchial malformation and neonatal tension lobar emphysema. J Thoracic and Cardiovas Surg. 1963, 46: 1-10. Moudgil A, Bagga A, Kamil ES, Rimoin DL, Lachman RS, Cohen AH, Jordan SC: Nephronophtisis associated with Ellis-van Creveld syndrome. Pediatr Nephrol. 1998, 12: 20-22. 10.1007/s004670050395. Scurlock D, Ostler D, Nguyen A, Wahed A: Ellis-van Creveld syndrome and dyserythropoiesis. Arch Pathol Lab Med. 2005, 129: 680-682. Miller D, Newstead G, Young L: Perinatal leukemia with a possible variant of the Ellis-van Crefeld. J Pediatr. 1969, 74: 300-303. 10.1016/S0022-3476(69)80081-8. Fryns JP: Postaxial polydactyly as heterozygote manifestation in Ellis-van Creveld syndrome? [letter]. Am J Med Genet. 1991, 39: 500-10.1002/ajmg.1320390430. Goldblatt J, Minutillo C, Pemberton P, Hurst J: Ellis-van Creveld syndrome in a western Australian Aboriginal community: postaxial polydactyly as a heterozygous manifestation?. Med J Aust. 1992, 157: 271. Spranger S, Tariverdian G: Symtomatic heterozygozygosity in the Ellis-van Creveld syndrome?. Clin Genet. 1995, 47: 217-220. Ruiz-Perez VL, Ide SE, Strom TM, Lorenz B, Wilson D, Woods K, King L, Francomano C, Freisinger P, Spranger S, Marino B, Dallapiccola B, Wright M, Meitinger T, Polymeropoulos MH, Goodship J: Mutations in a new gene in Ellis-van Creveld syndrome and Weyers acrodental dysostosis. Nature Genet. 2000, 24: 283-286. 10.1038/73508. Ye X, Song G, Fan M, Shi L, Jabs EW, Huang S, Guo R, Bian Z: A novel heterozygous deletion in the EVC2 gene causes Weyers acrofacial dysostosis. Hum Genet. 2006, 119: 199-205. 10.1007/s00439-005-0129-2. Francomano C, Ortez deLuna R, Ide S, Pyeritz R, Wright M, Polymeropoulos M: The gene for the Ellis-van Creveld syndrome maps to chromosome 4p16. Am J Hum Genet. 1995, 57 (suppl): A191. Polymeropoulos M, Ide S, Wright M, Goodship J, Weissenbach J, Pyeritz R, Da Silva E, Ortiz De Luna R, Francomano CA: The gene for the Ellis-van Creveld syndrome is located on chromosome 4p16. Genomics. 1996, 35: 1-5. 10.1006/geno.1996.0315. Ruiz-Perez V, Tompson S, Blair H, Espinoza-Valdez C, Lapunzina P, Silva E, Hamel B, Gibbs J, Young I, Wright M, Goodship J: Mutations in two nonhomologous genes in a head-to-head configuration cause Ellis-van Creveld syndrome. Am J Hum Genet. 2003, 72: 728-732. 10.1086/368063. Galdzicka M, Patnala S, Hirshman M, Cai J, Nitowsky , Egeland J, Ginns E: A new gene, EVC2, is mutated in Ellis-van Creveld syndrome. Molec Genet Metab. 2002, 77: 291-295. 10.1016/S1096-7192(02)00178-6. Tompson SW, Ruiz-Perez VL, Blair HJ, Barton S, Navarro V, Robson JL, Wright MJ, Goodship JA: Sequencing EVC and EVC2 identifies mutations in two-thirds of Ellis-van Creveld syndrome patients. Hum Genet. 2007, 120: 663-670. 10.1007/s00439-006-0237-7. Yang S, Langer L, Cacciarelli A, Dahms B, Unger E, Roskamp J: Three conditions in neonatal asphyxiating thoracic dysplasia (Jeune) and short rib-polydactyly syndrome spectrum: a clinicopathologic study. Am J Med Genet. 1987, 3 (suppl): 191-207. 10.1002/ajmg.1320280523. Elcioglu N, Hall C: Diagnostic dilemmas in the short rib-polydactyly syndrome group. Am J Med Genet. 2002, 111: 392-400. 10.1002/ajmg.10562. Takamine Y, Krejci P, Wilcox W: Mutations in the EVC1 gene are not a common finding in the Ellis-van Creveld and Short-Rib-polydactyly type III syndromes. Am J Med Genet. 2004, 130A: 96-97. 10.1002/ajmg.a.20579. Maroteaux P, Savart P: La dystrophie thoracique asphyxiante. Etude radiologique et rapports avec le syndrome d'Ellis-van Creveld. Ann Radiol. 1964, 7: 332-338. Kolowski K, Szmgiel C, Barylak A, Stopyrowa M: Difficulties in differentiation between chondroectodermal dysplasia (Ellis-van Creveld syndrome) and Asphyxiating Thoracic Dystrophy. Aust Radiol. 1972, 16: 401-410. Stone D, Slavotinek A, Bouffard G, Banerjee-Basu S, Baxevanis A, Barr M, Biesecker L: Mutation of a gene encoding a putative chaperonin causes McKusick-Kaufman syndrome. Nature Genet. 2000, 25: 79-82. 10.1038/75637. Pirazzoli P, Mazzanti L, Mandini M, Cau M, Ravagli L, Cacciari E: GH-deficiency in Ellis-van-Crefeld Syndrome: response to remplacement therapy. Growth Abnormalities. 1989, 56: 391-394. Shibata T, Kawabata H, Yasui N, Nakahara H, Hirabayashi S, Nakase T: Correction of knee deformity in patients with Ellis-van Creveld syndrome. J Pediatr Orthop B. 1999, 8: 282-284. About this article Cite this article Baujat, G., Le Merrer, M. Ellis-Van Creveld syndrome. Orphanet J Rare Dis 2, 27 (2007). https://doi.org/10.1186/1750-1172-2-27 - Hypoplastic Left Heart Syndrome - Ectodermal Dysplasia - Oral Manifestation	2	21	7	0	2	0	0.867251	9	5,843
\|Publication number\|\|US6795733 B1\| \|Application number\|\|US 10/103,605\| \|Publication date\|\|Sep 21, 2004\| \|Filing date\|\|Mar 21, 2002\| \|Priority date\|\|Mar 21, 2002\| \|Publication number\|\|10103605, 103605, US 6795733 B1, US 6795733B1, US-B1-6795733, US6795733 B1, US6795733B1\| \|Original Assignee\|\|Pacesetter, Inc.\| \|Export Citation\|\|BiBTeX, EndNote, RefMan\| \|Patent Citations (3), Referenced by (31), Classifications (18), Legal Events (4)\| \|External Links: USPTO, USPTO Assignment, Espacenet\| This invention relates to methods and apparatus for measuring a patient's transthoracic impedance, and more particularly, to techniques for measuring transthoracic impedance for use by a rate-responsive stimulation device. Stimulation devices such as pacemakers are used to treat a variety of cardiac conditions. Some stimulation devices simply provide pacing pulses to a patient's heart at a fixed rate. More sophisticated devices contain sensing circuitry that allows the stimulation devices to monitor a patient's intrinsic signals. For example, some stimulation devices can monitor a patient's atrial heartbeat signals and provide corresponding ventricular pacing pulses, which allows the patient's cardiac output to be adjusted depending on the patient's intrinsic atrial heart rate. However, there are situations when the heart is not able to regulate its rate appropriately in response to physiological stress. This is known as chronotropic incompetence. Physiologically, the cardiac need of a patient varies depending on the patient's physical activity level. Because of this, so-called rate-responsive stimulation devices have been developed that provide pacing pulses at a rate based on the patient's level of exercise. Some rate-responsive stimulation devices contain accelerometer-based activity sensors, which assess a patient's level of physical activity by measuring the patient's body movements. When the measured frequency and intensity of a patient's movements are high, the patient's heart is paced at a correspondingly high rate. Although this approach is generally satisfactory, many rate-responsive stimulation devices that use activity sensors are unable to clearly differentiate between body movements due to physical activity and body movements due to external sources (e.g., body movements experienced during an automobile ride). Other rate-responsive stimulation devices use oxygen sensors to measure a patient's blood-oxygen level. Rate-responsive stimulation devices that use oxygen sensors adjust the pacing rate to maintain a suitable oxygen level. However, oxygen sensors require the use of a special lead. Another approach that has been used to assess a patient's need for cardiac output is to attempt to determine the amount of air being inhaled and exhaled by the patient. Taking breaths deeply and frequently, for example when climbing the stairs, indicates that there is a high need for cardiac output. Accordingly, if a measure of a patient's air usage can be provided, it can be used for rate-responsive pacing. One measure of a patient's air usage is termed “minute ventilation”. Minute ventilation is the total volume of air moved in and out of the lungs in a minute. Transthoracic impedance is measured to calculate minute ventilation and is defined as a measure of the impedance across the chest cavity. More specifically, lungs that are filled with air have a higher impedance than lungs which are empty. Upon inhalation, impedance increases. Upon exhalation, impedance decreases. Minute ventilation is calculated based upon the formula: A rate-responsive device measures minute ventilation using the transthoracic impedance, computes a minute ventilation signal, and then compares the current minute ventilation with a long-term average of “change in minute ventilation” to arrive at a required rate. Consider, for example, FIG. 1 which is a graph of a parameter known as a rate response factor (RRF). The rate response factor can be used to set the expected change in pacing rate in response to increasing changes in minute ventilation during exercise. Each patient has a resting minute ventilation measurement and a maximum minute ventilation measurement. These are respectively indicated on the x-axis as “Resting” and “Peak”. Three lines are graphed in FIG. 1 and represent different RRFs relative to a particular patient to whom the graph corresponds. For example, the top line indicates a RRF that is too high for this patient because a maximum metabolic indicated rate is reached before the peak minute ventilation. The bottom line indicates a RRF that is too low for this patient because the maximum metabolic indicated rate is never reached. The middle line indicates an appropriate RRF for this patient because the maximum metabolic indicated rate is reached at the peak minute ventilation. Before a minute ventilation sensor in a stimulation device can be activated, a baseline impedance measurement for a particular patient needs to be established. This is because the baseline impedance measurement is associated with a minimum pacing rate for that particular patient. The minimum pacing rate for the patient whose graph is shown in FIG. 1 is indicated as the minimum metabolic indicated rate. The minimum pacing rate when the patient is at rest might be around 60 ppm. The maximum pacing rate when, for example, the patient is exercising might be around 150 ppm. These two points are determined, respectively, by the baseline impedance signal (i.e. the signal when the patient is at rest) and the impedance signal when the patient is at maximum exercise. The line between these two points is determined by the rate responsive factor programmed into the stimulation device discussed above. An advantage of monitoring the impedance of the chest cavity to assess cardiac need is that the stimulation device is less likely to be affected by body movements due to external sources and does not require the use of special leads. One way for the stimulation device to measure body impedance is to apply a current signal of a known magnitude and waveform across the patient's chest. The resulting voltage signal across the body can be measured by sensing circuitry. The impedance is calculated based on the known magnitude of the applied current signal and the measured magnitude of the voltage signal. For example, many stimulation devices utilize a ventricular bipolar lead. A ventricular bipolar lead is a lead that is implantable in the ventricle. The lead is bipolar because it is fitted with two electrodes. One of the electrodes is used as the anode, and the other of the electrodes is used as the cathode. In these types of leads, a current signal for measuring impedance can be injected between a ring or coil electrode and the case or “can”. A corresponding voltage signal can then be measured between a tip electrode and the case or can, as will be appreciated and understood by those of skill in the art. One of the problems associated with minute ventilation sensors is that the lead or leads from which impedance measurements are ascertained can sometimes fail to operate as intended. This means that the impedance measurements that are utilized to provide rate-responsiveness can no longer be used to provide this functionality. Accordingly, this invention arose out of concerns associated with providing improved stimulation devices and methods that provide improved minute ventilation sensors. Methods and systems for measuring the transthoracic impedance of patient are described. Various embodiments provide stimulation devices and methods that can automatically adapt to different minute ventilation electrode configurations. This, in turn, permits minute ventilation functionality to continue, e.g. rate-responsive pacing, in spite of the fact that an electrode configuration has changed. Accordingly, minute ventilation functionality can automatically continue in an adaptive manner when a previously-available electrode configuration is no longer available for minute ventilation functionality. Further features and advantages of the claimed embodiments can be more readily understood by reference to the following description taken in conjunction with the accompanying drawings, in which: FIG. 1 is a graph that is useful in understanding aspects of the described embodiments. FIG. 2 is a simplified diagram illustrating an implantable stimulation device in electrical communication with at least three leads implanted into a patient's heart for delivering multi-chamber stimulation and shock therapy. FIG. 3 is a functional block diagram of a multi-chamber implantable stimulation device illustrating the basic elements of a stimulation device which can provide cardioversion, defibrillation and pacing stimulation in four chambers of the heart. FIG. 4 is a diagram that shows one exemplary minute ventilation electrode configuration. FIG. 5 is a diagram that shows one exemplary minute ventilation electrode configuration. FIG. 6 is a diagram that shows one exemplary minute ventilation electrode configuration. FIG. 7 is a flow diagram that describes steps in a method in accordance with one embodiment. FIG. 8 is a block diagram of an exemplary table in accordance with one embodiment. FIG. 9 is a flow diagram that describes steps in a method in accordance with one embodiment. FIG. 10 is a flow diagram that describes steps in a method in accordance with one embodiment. FIG. 11 is a block diagram that illustrates exemplary minute ventilation accumulators in accordance with one embodiment. The following description is of the best mode presently contemplated for practicing the described embodiments. This description is not to be taken in a limiting sense, but rather is made merely for the purpose of describing the general principles of the inventive embodiments. The scope of the described embodiments should be ascertained with reference to the issued claims. In the description of the embodiments that follow, like numerals or reference designators will be used to refer to like parts or elements throughout. The methods and systems described below provide stimulation devices and methods that can automatically adapt to different minute ventilation electrode configurations. This, in turn, permits minute ventilation functionality to continue, e.g. rate-responsive pacing, in spite of the fact that an electrode configuration has changed. Accordingly, minute ventilation functionality can automatically continue in an adaptive manner when a previously-available electrode configuration is no longer available for minute ventilation functionality. The techniques that are described below are intended to be implemented in connection with a stimulation device that is configured or configurable to stimulate or shock a patient's heart. FIG. 2 shows an exemplary stimulation device 10 in electrical communication with a patient's heart 12 by way of three leads, 20, 24 and 30, suitable for delivering multi-chamber stimulation and shock therapy. To sense atrial cardiac signals and to provide right atrial chamber stimulation therapy, stimulation device 10 is coupled to an implantable right atrial lead 20 having at least an atrial tip electrode 22, which typically is implanted in the patient's right atrial appendage. The right atrial lead can also have a ring electrode 23 positioned above the atrial tip electrode 22. To sense left atrial and ventricular cardiac signals and to provide left chamber pacing therapy, stimulation device 10 is coupled to a “coronary sinus” lead 24 designed for placement in the “coronary sinus region” via the coronary sinus os for positioning a distal electrode adjacent to the left ventricle and/or additional electrode(s) adjacent to the left atrium. As used herein, the phrase “coronary sinus region” refers to the vasculature of the left ventricle, including any portion of the coronary sinus, great cardiac vein, left marginal vein, left posterior ventricular vein, middle cardiac vein, and/or small cardiac vein or any other cardiac vein accessible by the coronary sinus. Accordingly, an exemplary coronary sinus lead 24 is designed to receive atrial and ventricular cardiac signals and to deliver left ventricular pacing therapy using at least a left ventricular tip electrode 26, left atrial pacing therapy using at least a left atrial ring electrode 27, and shocking therapy using at least a left atrial coil electrode 28. Stimulation device 10 is also shown in electrical communication with the patient's heart 12 by way of an implantable right ventricular lead 30 having, in this embodiment, a right ventricular tip electrode 32, a right ventricular ring electrode 34, a right ventricular (RV) coil electrode 36, and an SVC coil electrode 38. Typically, the right ventricular lead 30 is transvenously inserted into the heart 12 so as to place the right ventricular tip electrode 32 in the right ventricular apex so that the RV coil electrode will be positioned in the right ventricle and the SVC coil electrode 38 will be positioned in the superior vena cava. Accordingly, the right ventricular lead 30 is capable of receiving cardiac signals, and delivering stimulation in the form of pacing and shock therapy to the right ventricle. FIG. 3 shows an exemplary, simplified block diagram depicting various components of stimulation device 10. The stimulation device can be capable of treating both fast and slow arrhythmias with stimulation therapy, including cardioversion, defibrillation, and pacing stimulation. While a particular multi-chamber device is shown, it is to be appreciated and understood that this is done for illustration purposes only. Thus, the inventive techniques and methods described below can be implemented in connection with any suitably configured or configurable stimulation device. Accordingly, one of skill in the art could readily duplicate, eliminate or disable the appropriate circuitry in any desired combination to provide a device capable of treating the appropriate chamber(s) with cardioversion, defibrillation and pacing stimulation. Housing 40 for stimulation device 10 is often referred to as the “can”, “case” or “case electrode”, and may be programmably selected to act as the return electrode for all “unipolar” modes. There may be multiple electrode positioned on or otherwise supported by the housing. The multiple electrodes can be used for impedance measurements, as will become apparent below. Housing 40 may further be used as a return electrode alone or in combination with one or more of the coil electrodes, 28, 36 and 38, for shocking purposes. Housing 40 further includes a connector (not shown) having a plurality of terminals 42, 44, 46, 48, 52, 54, 56, and 58 (shown schematically and, for convenience, the names of the electrodes to which they are connected are shown next to the terminals). As such, to achieve right atrial sensing and pacing, the connector includes at least a right atrial tip terminal (AR TIP) 42 adapted for connection to the atrial tip electrode 22. To achieve left chamber sensing, pacing and shocking, the connector includes at least a left ventricular tip terminal (VL TIP) 44, a left atrial ring terminal (AL RING) 46, and a left atrial shocking terminal (AL COIL) 48, which are adapted for connection to the left ventricular ring electrode 26, the left atrial tip electrode 27, and the left atrial coil electrode 28, respectively. To support right chamber sensing, pacing and shocking, the connector further includes a right ventricular tip terminal (VR TIP) 52, a right ventricular ring terminal (VR RING) 54, a right ventricular shocking terminal (RV COIL) 56, and an SVC shocking terminal (SVC COIL) 58, which are adapted for connection to the right ventricular tip electrode 32, right ventricular ring electrode 34, the RV coil electrode 36, and the SVC coil electrode 38, respectively. At the core of the stimulation device 10 is a programmable microcontroller 60 which controls the various modes of stimulation therapy. As is well known in the art, microcontroller 60 typically includes a microprocessor, or equivalent control circuitry, designed specifically for controlling the delivery of stimulation therapy, and may further include RAM or ROM memory, logic and timing circuitry, state machine circuitry, and I/O circuitry. Typically, microcontroller 60 includes the ability to process or monitor input signals (data) as controlled by a program code stored in a designated block of memory. The details of the design and operation of the microcontroller 60 are not critical to the described embodiments. Rather, any suitable microcontroller 60 may be used that carries out the functions described herein. The use of microprocessor-based control circuits for performing timing and data analysis functions are well known in the art. FIG. 3 also shows an atrial pulse generator 70 and a ventricular pulse generator 72 which generate pacing stimulation pulses for delivery by the right atrial lead 20, the right ventricular lead 30, and/or the coronary sinus lead 24 via an electrode configuration switch 74. It is understood that in order to provide stimulation therapy in each of the four chambers of the heart, the atrial and ventricular pulse generators, 70 and 72, may include dedicated, independent pulse generators, multiplexed pulse generators, or shared pulse generators. The pulse generators, 70 and 72, are controlled by the microcontroller 60 via appropriate control signals, 76 and 78, respectively, to trigger or inhibit the stimulation pulses. Microcontroller 60 further includes timing control circuitry 79 which is used to control the timing of such stimulation pulses (e.g., pacing rate, atrio-ventricular (AV) delay, atrial interconduction (A-A) delay, or ventricular interconduction (V-V) delay, etc.) as well as to keep track of the timing of refractory periods, blanking intervals, noise detection windows, evoked response windows, alert intervals, marker channel timing, etc., which is well known in the art. Microcontroller 60 further includes one or more minute ventilation reconfiguration algorithms 81 that can be utilized by the stimulation device 10 for automatically reconfiguring various electrode configurations that are used to ascertain impedance which, in turn, is used to calculate a patient's minute ventilation. A switch 74 includes a plurality of switches for connecting the desired electrodes to the appropriate I/O circuits, thereby providing complete electrode programmability. Accordingly, switch 74, in response to a control signal 80 from the microcontroller 60, determines the polarity of the stimulation pulses (e.g., unipolar, bipolar, combipolar, etc.) by selectively closing the appropriate combination of switches (not shown). The switch 74 can also be used by the microcontroller 60 to reconfigure electrode configurations that are used for determining minute ventilation responsive to the minute ventilation reconfiguration algorithms 81. Atrial sensing circuits 82 and ventricular sensing circuits 84 may also be selectively coupled to the right atrial lead 20, coronary sinus lead 24, and the right ventricular lead 30, through the switch 74 for detecting the presence of cardiac activity in each of the four chambers of the heart. Accordingly, the atrial (ATR. SENSE) and ventricular (VTR. SENSE) sensing circuits, 82 and 84, may include dedicated sense amplifiers, multiplexed amplifiers, or shared amplifiers. Switch 74 determines the “sensing polarity” of the cardiac signal by selectively closing the appropriate switches, as is also known in the art. In this way, the clinician may program the sensing polarity independent of the stimulation polarity. Each sensing circuit, 82 and 84, preferably employs one or more low power, precision amplifiers with programmable gain and/or automatic gain control, bandpass filtering, and a threshold detection circuit, as known in the art, to selectively sense the cardiac signal of interest. The automatic gain control enables the device 10 to deal effectively with the difficult problem of sensing the low amplitude signal characteristics of atrial or ventricular fibrillation. The outputs of the atrial and ventricular sensing circuits, 82 and 84, are connected to the microcontroller 60 which, in turn, are able to trigger or inhibit the atrial and ventricular pulse generators, 70 and 72, respectively, in a demand fashion in response to the absence or presence of cardiac activity in the appropriate chambers of the heart. The sensing circuits, 82 and 84, in turn, receive control signals over signal lines, 86 and 88, from the microcontroller 60 for purposes of controlling the gain, threshold, polarization charge removal circuitry (not shown), and the timing of any blocking circuitry (not shown) coupled to the inputs of the sensing circuits, 82 and 86, as is known in the art. For arrhythmia detection, the device 10 utilizes the atrial and ventricular sensing circuits, 82 and 84, to sense cardiac signals to determine whether a rhythm is physiologic or pathologic. As used herein “sensing” is reserved for the noting of an electrical signal, and “detection” is the processing of these sensed signals and noting the presence of an arrhythmia. The timing intervals between sensed events (e.g., P-waves, R-waves, and depolarization signals associated with fibrillation which are sometimes referred to as “F-waves” or “Fib-waves”) are then classified by the microcontroller 60 by comparing them to a predefined rate zone limit (i.e., bradycardia, normal, low rate VT, high rate VT, and fibrillation rate zones) and various other characteristics (e.g., sudden onset, stability, physiologic sensors, and morphology, etc.) in order to determine the type of remedial therapy that is needed (e.g., bradycardia pacing, anti-tachycardia pacing, cardioversion shocks or defibrillation shocks, collectively referred to as “tiered therapy”). Cardiac signals are also applied to the inputs of an analog-to-digital (A/D) data acquisition system 90. The data acquisition system 90 is configured to acquire intracardiac electrogram signals, convert the raw analog data into a digital signal, and store the digital signals for later processing and/or telemetric transmission to an external device 102. The data acquisition system 90 is coupled to the right atrial lead 20, the coronary sinus lead 24, and the right ventricular lead 30 through the switch 74 to sample cardiac signals across any pair of desired electrodes. Advantageously, the data acquisition system 90 may be coupled to the microcontroller, or other detection circuitry, for detecting an evoked response from the heart 12 in response to an applied stimulus, thereby aiding in the detection of “capture”. Capture occurs when an electrical stimulus applied to the heart is of sufficient energy to depolarize the cardiac tissue, thereby causing the heart muscle to contract. The microcontroller 60 detects a depolarization signal during a window following a stimulation pulse, the presence of which indicates that capture has occurred. The microcontroller 60 enables capture detection by triggering the ventricular pulse generator 72 to generate a stimulation pulse, starting a capture detection window using the timing control circuitry 79 within the microcontroller 60, and enabling the data acquisition system 90 via control signal 92 to sample the cardiac signal that falls in the capture detection window and, based on the amplitude, determines if capture has occurred. Capture detection may occur on a beat-by-beat basis or on a sampled basis. A capture threshold search can desirably be performed once a day during at least the acute phase (e.g., the first 30 days) and less frequently thereafter. A capture threshold search would begin at a desired starting point (either a high energy level or the level at which capture is currently occurring) and decrease the energy level until capture is lost. The value at which capture is lost is known as the capture threshold. Thereafter, a safety margin is added to the capture threshold. The microcontroller 60 is further coupled to a memory 94 by a suitable data/address bus 96, wherein the programmable operating parameters used by the microcontroller 60 are stored and modified, as required, in order to customize the operation of the stimulation device 10 to suit the needs of a particular patient. Such operating parameters define, for example, pacing pulse amplitude, pulse duration, electrode polarity, rate, sensitivity, automatic features, arrhythmia detection criteria, and the amplitude, waveshape and vector of each shocking pulse to be delivered to the patient's heart 12 within each respective tier of therapy. One feature of the described embodiments is the ability to sense and store a relatively large amount of data (e.g., from the data acquisition system 90), which data may then be used for subsequent analysis to guide the programming of the device. Advantageously, the operating parameters of the implantable device 10 may be non-invasively programmed into the memory 94 through a telemetry circuit 100 in telemetric communication with the external device 102, such as a programmer, transtelephonic transceiver, or a diagnostic system analyzer. The telemetry circuit 100 is activated by the microcontroller by a control signal 106. The telemetry circuit 100 advantageously allows intracardiac electrograms and status information relating to the operation of the device 10 (as contained in the microcontroller 60 or memory 94) to be sent to the external device 102 through an established communication link 104. The stimulation device 10 can further include a physiologic sensor 108, commonly referred to as a “rate-responsive” sensor because it is typically used to adjust pacing stimulation rate according to the exercise state of the patient. However, the physiological sensor 108 may further be used to detect changes in cardiac output, changes in the physiological condition of the heart, or diurnal changes in activity (e.g., detecting sleep and wake states). Accordingly, the microcontroller 60 responds by adjusting the various pacing parameters (such as rate, AV Delay, V-V Delay, etc.) at which the atrial and ventricular pulse generators, 70 and 72, generate stimulation pulses. While shown as being included within the stimulation device 10, it is to be understood that the physiologic sensor 108 may also be external to the stimulation device 10, yet still be implanted within or carried by the patient. A common type of rate responsive sensor is an activity sensor, such as an accelerometer (e.g. a three-dimensional accelerometer) or a piezoelectric crystal, which is mounted within the housing 40 of the stimulation device 10. Other types of physiologic sensors are also known, for example, sensors which sense the oxygen content of blood, respiration rate and/or minute ventilation, pH of blood, ventricular gradient, etc. However, any sensor may be used which is capable of sensing a physiological parameter which corresponds to the exercise state of the patient. The type of sensor used is not critical to the described embodiments and is shown only for completeness. The described embodiments can utilize a “sleep state” or diurnal sensor that can detect sleep, rest, and wake states. One such sensor is known as “activity variance” wherein an activity sensor is monitored diurnally to detect the low variance in the measurement corresponding to the sleep state. The stimulation device additionally includes a battery 110 that provides operating power to all of the circuits shown in FIG. 3. For the stimulation device 10, which employs shocking therapy, the battery 110 is capable of operating at low current drains for long periods of time (e.g. preferably less than 10 μA), and is capable of providing high-current pulses (for capacitor charging) when the patient requires a shock pulse (e.g. preferably, in excess of 2 A, at voltages above 2 V, for periods of 10 seconds or more). The battery 110 also desirably has a predictable discharge characteristic so that elective replacement time can be detected. Accordingly, the device 10 preferably employs lithium/silver vanadium oxide batteries, as is true for most (if not all) current devices. The stimulation device 10 can further include magnet detection circuitry (not shown), coupled to the microcontroller 60. It is the purpose of the magnet detection circuitry to detect when a magnet is placed over the stimulation device 10, which magnet may be used by a clinician to perform various test functions of the stimulation device 10 and/or to signal the microcontroller 60 that the external programmer 102 is in place to receive or transmit data to the microcontroller 60 through the telemetry circuits 100. FIG. 3 also shows an impedance measuring circuit 112 which is enabled by the microcontroller 60 via a control signal 114. Uses for an impedance measuring circuit 120 can include, but are not limited to, lead impedance surveillance during the acute and chronic phases for proper lead positioning or dislodgement; detecting operable electrodes and automatically switching to an operable pair if dislodgement occurs; measuring respiration or minute ventilation; measuring thoracic impedance for determining shock thresholds; detecting when the device has been implanted; measuring stroke volume; and detecting the opening of heart valves, etc. In the embodiments described below, the impedance measuring circuit 112 is additionally used to ascertain when a particular electrode configuration that is used for ascertaining minute ventilation (i.e. ascertaining impedance which is then used to ascertain minute ventilation as described above) is no longer able to reliably function in this capacity. The impedance measuring circuit 120 is advantageously coupled to the switch 74 so that any desired electrode may be used. In the case where the stimulation device 10 is intended to operate as an implantable cardioverter/defibrillator (ICD) device, it detects the occurrence of an arrhythmia, and automatically applies an appropriate electrical shock therapy to the heart aimed at terminating the detected arrhythmia. To this end, the microcontroller 60 further controls a shocking circuit 116 by way of a control signal 118. The shocking circuit 116 generates shocking pulses of low (up to 0.5 Joules), moderate (0.5-10 Joules), or high energy (11 to 40 Joules), as controlled by the microcontroller 60. Such shocking pulses are applied to the patient's heart through at least two shocking electrodes, and as shown in this embodiment, selected from the left atrial coil electrode 28, the RV coil electrode 36, and/or the SVC coil electrode 38. As noted above, the housing 40 may act as an active electrode in combination with the RV electrode 36, or as part of a split electrical vector using the SVC coil electrode 38 or the left atrial coil electrode 28 (i.e., using the RV electrode as a common electrode). Cardioversion shocks are generally considered to be of low to moderate energy level (so as to minimize pain felt by the patient), and/or synchronized with an R-wave and/or pertaining to the treatment of tachycardia. Defibrillation shocks are generally of moderate to high energy level (i.e., corresponding to thresholds in the range of 5-40 Joules), delivered asynchronously (since R-waves may be too disorganized), and pertaining exclusively to the treatment of fibrillation. Accordingly, the microcontroller 60 is capable of controlling the synchronous or asynchronous delivery of the shocking pulses. As noted above, to ascertain a patient's minute ventilation the thoracic or transthoracic impedance is determined. There are various electrode configurations that can be utilized to ascertain this impedance measure. As will be understood by those of skill in the art, the transthoracic impedance is different from the lead impedance associated with determining lead electrode integrity. FIG. 4 shows one electrode configuration referred to as a “ventricular bipolar configuration”. In this configuration, a ventricular bipolar lead is used. Electrical current signals are injected between ring electrode 34 and the can or case. Corresponding voltage signals are measured between the tip electrode 32 and the can or case. The impedance can then be calculated from the measured signals. From the impedance measure, the minute ventilation can be derived. FIG. 5 shows another electrode configuration referred to as an “atrial bipolar configuration”. In this configuration, an atrial bipolar lead is used. Electrical current signals are injected between the ring electrode 23 and the can or case. Corresponding voltage signals are measured between the tip electrode 22 and the can or case. The impedance can then be calculated from these two signals. From the impedance measure, the minute ventilation can be derived. FIG. 6 shows another electrode configuration referred to as a “dual unipolar configuration”. In this configuration, the atrial and ventricular leads are unipolar leads. Electrical current signals are injected between the atrial tip electrode 22 and the can or case. Corresponding voltage signals are measured between the ventricular tip electrode 32 and the can or case. The impedance can then be calculated from these two signals. From the impedance measure, the minute ventilation can be derived. Other minute ventilation electrode configurations can, of course be used. For example, multiple electrodes (e.g. dot electrodes) can be provided on the can or case so that a single unipolar ventricular or atrial lead can be used to inject current signals, and can serve as an electrode relative to which a voltage signal is measured from the can or case. An example of such an electrode configuration is shown and described in U.S. patent application Ser. No. 09/326,939, entitled “Minute Volume Pacemakers that Require Only a Single Distal Electrode”, filed on Jun. 7, 1999, naming Richard Lu as inventor, the disclosure of which is incorporated by reference herein. FIGS. 4-6 and the discussion in this section are intended to illustrate exemplary non-limiting electrode configurations that can be used for ascertaining minute ventilation. It is to be appreciated and understood that other electrode configurations could be used. For example, other configurations can include shock electrodes in an ICD system. In addition, it may be likely that in the future additional electrode configurations (including new and/or additional electrodes) for use in determining minute ventilation will be conceived and developed. It should be readily apparent that the principles about to be described below can be applied to these other additional electrode configurations. A key consideration in such other configurations is to have the two electrodes (for current injection or impedance measurements) span across the lung. FIG. 7 shows a flow diagram that describes steps in a method in accordance with one embodiment. The method can be implemented in any suitable hardware, software, firmware, or combination thereof. In the illustrated embodiment, the method is implemented in both hardware and software. Step 700 establishes a first minute ventilation electrode configuration. Any electrode configuration that is suitable for use in determining minute ventilation can be used. The electrode configuration is desirably programmatically established by a suitably programmed stimulation device. Several examples of minute ventilation electrode configurations are given above. One example of a stimulation device is given above. Other stimulation devices can, of course, be utilized to establish the first minute ventilation electrode configuration. Step 702 then ascertains minute ventilation using the minute ventilation electrode configuration. This step can be implemented in any suitable way. In the examples given above, the electrode configurations are used to ascertain a thoracic impedance measurement which, in turn, can be used to determine a tidal volume meter and a respiration rate parameter. These two parameters can then be to derive minute ventilation. Step 704 monitors the integrity of the minute ventilation electrode configuration. Any suitable techniques can be utilized to monitor the integrity of the configuration. For example, known lead supervision techniques can be used to monitor the impedance of the leads. Step 706 determines whether there is a problem with the integrity of the monitored electrode configuration. For example, if lead supervision techniques are used, then this step can be implemented by determining whether the impedance of the current injection electrodes (e.g. ring to case) and the impedance measurement electrodes (e.g. tip to case) fall outside a range of normal operating values. Alternately, this step can be implemented by ascertaining whether the impedances of the electrodes involved change by a pre-determined amount or percentage (thus possibly indicating a faulty lead). If there are no perceived integrity problems with the current electrode configuration, the method branches back to step 704 and continues to monitor the configuration integrity. If, on the other hand, step 706 determines that there is an integrity problem with the current electrode configuration, then step 708 switches to a different minute ventilation electrode configuration. This step is desirably automatically implemented by the stimulation device. Several different minute ventilation electrode configurations are described above. Step 708 then branches back to step 702 which then ascertains the minute ventilation using the new minute ventilation electrode configuration. Step 702 can involve some baseline calibration operations which are described in more detail below in a section entitled “Re-establishing the Baseline”. In the FIG. 3 example given above, one or more minute ventilation reconfiguration algorithms 81 reside in the microcontroller 60. These algorithms can receive input from the impedance measuring circuit 112. Recall that the impedance measuring circuit can continuously measure the impedance of the leads that are implanted in the patient. If the minute ventilation algorithm(s) determine that the measured impedance of one or more of the leads is problematic, the algorithms 81 can cause microcontroller 60 to provide an input to switch 74 via line 80. This input can cause the switch 74 to reconfigure the minute ventilation electrode configuration. In some embodiments, a stimulation device can be configured such that multiple different alternate minute ventilation electrode configurations are possible. That is, in addition to a first configuration, there can be additional different configurations that are possible for selection in the event that the first configuration has integrity problems. As an example, consider FIG. 8. There, a minute ventilation (MV) configuration table 800 is shown. The MV configuration table 800 can be maintained on the stimulation device so that the stimulation device can use it if the need arises. Table 800 includes two columns 802, 804. Column 802 is a priority number column and contains a value that is associated with the priority of a particular MV electrode configuration. Column 804 contains an entry that corresponds to a particular MV electrode configuration. In this specific example, the stimulation device is configured so that it can implement, if necessary, the following configurations: ventricular bipolar, atrial bipolar, dual unipolar, and one or more “other” configurations. Each of these particular configurations carries with it a priority. Specifically, the ventricular bipolar configuration has the highest priority followed respectively by the atrial bipolar configuration, the dual unipolar configuration and the “other” configuration(s). If the next configuration is available, a check can be made to ensure that the configuration is operable. An impedance measurement can be used to check the operability. In operation (in this example), minute ventilation will be ascertained using first, the ventricular bipolar configuration. If an integrity problem develops with this configuration, then the electrode configuration will be switched to the next-in-line configuration which, in this case, is the atrial bipolar configuration, and so on. Each stimulation device is desirably knowledgeable of the leads and lead types that it has in place, and can thus be programmed with a table that has the established priorities. If the stimulation device does not have a particular lead configuration that permits a particular MV electrode configuration in the table, then that particular configuration can be removed or otherwise nullified from the table. The table can also be automatically generated at implant and periodically confirmed for availability, or it can be specifically programmed by, for example, a physician who desires to implement a specific combination of electrode configurations that, for whatever reason, are desirable for a particular patient. FIG. 9 shows a flow diagram that describes steps in a method in accordance with one embodiment. The method can be implemented in any suitable hardware, software, firmware, or combination thereof. In the illustrated embodiment, the method is implemented in both hardware and software. Step 900 defines priorities between multiple different minute ventilation electrode configurations. This step can be implemented in several different ways. For example, individual stimulation devices can be pre-programmed to support many of a potentially large number of electrode configurations that can be utilized to ascertain minute ventilation. The stimulation devices can then, at some later time, ascertain which of the leads they are equipped with and then pare down the possible configurations to only those that are specifically supported by the lead arrangement connected to the stimulation device. The priorities and their associated configurations can be maintained in any suitable manner, such as with a table like the one described in FIG. 8. Alternately, stimulation devices can be programmed to support only a desired number of configurations which might be less then the total number of possible configurations that the devices can support. Step 902 establishes a first minute ventilation electrode configuration in accordance with the defined priorities. Any electrode configuration that is suitable for use in determining minute ventilation can be used. Examples of minute ventilation electrode configurations that can be established in accordance with defined priorities are given in FIG. 8. Step 904 then ascertains minute ventilation using the established minute ventilation electrode configuration. This step can be implemented in any suitable way. In the examples given above, the electrode configurations are used to ascertain a thoracic impedance measurement which, in turn, can be used to determine a tidal volume parameter and a respiration rate parameter. These two parameters can then be used to derive minute ventilation. Step 906 monitors the integrity of the minute ventilation electrode configuration. Any suitable techniques can be utilized to monitor the integrity of the configuration, with examples being given above. Step 908 determines whether there is a problem with the integrity of the monitored electrode configuration. If there are no perceived integrity problems with the current electrode configuration, the method branches back to step 906 and continues to monitor the configuration integrity. If, on the other hand, step 908 determines that there is an integrity problem with the current electrode configuration, then step 910 switches to a lower priority minute ventilation electrode configuration. This step is desirably automatically implemented by the stimulation device. Step 910 then branches back to step 904 which then ascertains the minute ventilation using the new minute ventilation electrode configuration. Step 904 can involve some baseline calibration operations which are described in more detail below in a section entitled “Re-establishing the Baseline”. Recall that a baseline impedance measurement is utilized to establish a rate response factor (RRF) that is used to rate-responsively pace a patient. When the minute ventilation electrode configuration is changed, the baseline impedance measurement that was previously used is no longer valid. Accordingly, the baseline impedance is re-calculated or re-established so that it can be used with the new electrode configuration. FIG. 10 is a flow diagram that describes steps in a method in accordance with one embodiment. The method can be implemented in any suitable hardware, software, firmware, or combination thereof. In the illustrated embodiment, the method is implemented in both hardware and software. The method about to be described can take place typically when the stimulation device ascertains that there is an integrity problem with the current minute ventilation electrode configuration. This corresponds to the “Yes” branches of step 706 in FIG. 7 and step 908 in FIG. 9. When an integrity problem with a current minute ventilation electrode configuration is detected, part of the processing that can take place is that step 1000 can stop the rate-responsive pacing that is based on minute ventilation. At this point, the device can be placed in a non-rate responsive mode to pace at a fixed rate delivered through a selected pacing configuration and in a mode where it can operate to measure the patient's transthoracic impedance. Step 1002 then switches to a different minute ventilation electrode configuration. This step can be implemented in any of the ways described above. The device can now be used in its impedance measuring mode to measure the patient's transthoracic impedance using the new electrode configuration. Step 1004 re-establishes the baseline transthoracic impedance and calculates the baseline minute ventilation (see FIG. 1). This step can be implemented in a couple of different ways. For example, a minute ventilation sensor can typically require a baseline acquisition while the patient is at rest for a pre-determined period of time, e.g. 3 minutes. The minute ventilation sensor will not typically reinitiate rate-responsive pacing until this is done. Hence, in one embodiment, a patient may be required to remain physically at rest for the pre-determined period of time. In yet other embodiments, baseline impedance values can be re-established by using one or more activity sensors that are onboard the stimulation device. Specifically, if an activity sensor is available, the stimulation device can monitor for a time period during which the patient's activity levels have not exceeded a certain threshold. The device can then use transthoracic impedance measurements taken during this time period for establishing the baseline impedance. In yet other embodiments, an activity sensor may not be available for use. In this case, the stimulation device can use an adaptive approach to re-establishing the baseline impedance. For example, the stimulation device can adapt for a pre-determined period of time, e.g. one hour, before the baseline impedance values are written into the storage devices that are used for minute ventilation. After the baseline impedance measure is re-established by step 1004, step 1006 re-starts rate-responsive pacing using the new electrode configuration to determine minute ventilation. As a practical matter of how the new baseline impedance measurements can be calculated, in but one example, consider FIG. 11. There, minute ventilation accumulators 1100 comprises a short term accumulator 1102 and a long term accumulator 1104. Accumulators 1100 can be implemented as storage devices, i.e. computer-readable media, on board the stimulation device. Short term accumulator 1102 contains short term data that corresponds to measurements of an impedance signal over the short or near term. Long term accumulator 1104 contains data that is the baseline impedance data that is associated with a particular minute ventilation electrode configuration. Essentially, the impedance signals are measured or calculated and stored in the accumulators. The short term accumulator 1102 will eventually get copied into the long term accumulator 1104. The difference between the two accumulators is that they are associated with different time constants. To do rate response pacing, a difference between data in the short term accumulator and the long term accumulator is calculated to determine a rate at which to pace the patient. Accordingly, the assumption in this example is that long term accumulator 1104 holds the base line impedance data and the short term accumulator 1102 holds data that is associated with current patient activity, e.g. when the patient starts exercising. As an example of how the above methods and systems can be used to automatically continue rate responsive pacing using minute ventilation when, for example, a polarity switch occurs on the leads of a stimulation device, consider the following. In this example, a lead supervision feature on the stimulation device continuously monitors the integrity of the leads. This is desirably done by monitoring the impedance of the leads and determining whether the lead impedance remains within an operable range. When the lead impedance extends outside the operable range, it is desirable to change the polarity of the leads. This may entail changing from a bipolar configuration to a unipolar configuration. If the patient is receiving rate-responsive pacing, it is desirable to continue the rate-responsiveness. As noted above, however, the transthoracic impedance measurements that serve to define the impedance baseline for the minute ventilation sensor will likely need to be re-calculated as a result of the polarity change. Assume now that the lead supervision feature indicates that a polarity switch criteria are met (i.e. there is another polarity to which the device can be switched), the current ventricular pacing configuration is bipolar, and the current minute ventilation configuration is also ventricular bipolar. In this case, the following procedure can be followed. First, the minute ventilation sensor can be temporarily turned off. This can entail turning off the rate-responsive functionality associated with the sensor, but allowing the sensor to operate in a mode that permits the transthoracic impedance to be determined. The stimulation device can now select an alternative minute ventilation electrode configuration if there is one available. In this example, assume that the patient has an atrial bipolar lead. Hence, the atrial bipolar minute ventilation electrode configuration can be selected. If the atrium has a unipolar lead, the next alternate configuration after the atrial bipolar configuration would be a dual unipolar configuration. In high voltage or multiple chamber (i.e. more than two) devices, other alternatives are also available. For example, current can be injected through the right ventricle shock electrode to the case. Transthoracic impedance measurements can be made through the ventricular tip electrode to the case. Alternately, current injection can occur through the atrial shock electrode to the case, and impedance measurements can be made between the ventricle shock electrode and the case. Once the new configuration has been selected, impedance measurements can now be performed to ascertain the new impedance baseline. Non-limiting examples of ways that this can be done are given above. Once the baseline impedance measurements are established, the minute ventilation sensors can be returned to a mode in which it can be used for rate-responsive pacing. In the event that the minute ventilation sensor is the only rate responsive sensor in the stimulation device and an alternative minute ventilation electrode configuration is not available (e.g. perhaps only a ventricular lead is implanted and a single unipolar configuration for minute ventilation measurements is not available), the patient can be notified that the rate-responsive function is not enabled. Notification can take place, for example, through a slight electrical stimulation that causes a muscle twitch in the patient. In this case, the stimulation device can fallback to a fixed-rate. Notification can also take place through the use of an audio alarm. The various embodiments described above provide stimulation devices that can automatically adapt to different electrode configurations that permit minute ventilation-dependent rate-responsive pacing to continue in spite of the fact that an electrode configuration has changed. This is advantageous from the standpoint of being able to continue minute ventilation functionality when a previously-available electrode configuration is no longer available for minute ventilation functionality. In addition, the configuration change can be recorded in the device so that the clinician can be informed as soon as possible (e.g. at the next follow up or through other means such as a remote monitor). Although the invention has been described in language specific to structural features and/or methodological steps, it is to be understood that the invention defined in the appended claims is not necessarily limited to the specific features or steps described. Rather, the specific features and steps are disclosed as preferred forms of implementing the claimed invention. \|Cited Patent\|\|Filing date\|\|Publication date\|\|Applicant\|\|Title\| \|US5282840 \|\|Mar 26, 1992\|\|Feb 1, 1994\|\|Medtronic, Inc.\|\|Multiple frequency impedance measurement system\| \|US5707398\|\|Nov 12, 1996\|\|Jan 13, 1998\|\|Pacesetter, Inc.\|\|Automatic determination of optimum electrode configuration for a cardiac stimulator\| \|US20020147475 \|\|Apr 10, 2001\|\|Oct 10, 2002\|\|Avram Scheiner\|\|Cardiac rhythm management system for hypotension\| \|Citing Patent\|\|Filing date\|\|Publication date\|\|Applicant\|\|Title\| \|US7010347 \|\|Jun 4, 2004\|\|Mar 7, 2006\|\|Pacesetter, Inc.\|\|Optimization of impedance signals for closed loop programming of cardiac resynchronization therapy devices\| \|US7272442 \|\|Dec 12, 2003\|\|Sep 18, 2007\|\|Cardiac Pacemakers, Inc.\|\|Automatically configurable minute ventilation sensor\| \|US7329226 \|\|Jul 6, 2004\|\|Feb 12, 2008\|\|Cardiac Pacemakers, Inc.\|\|System and method for assessing pulmonary performance through transthoracic impedance monitoring\| \|US7603170 \|\|Oct 13, 2009\|\|Cardiac Pacemakers, Inc.\|\|Calibration of impedance monitoring of respiratory volumes using thoracic D.C. impedance\| \|US7653436\|\|Apr 10, 2007\|\|Jan 26, 2010\|\|Pacesetter, Inc.\|\|Global cardiac performance\| \|US7881781\|\|Feb 1, 2011\|\|Cardiac Pacemakers, Inc.\|\|Thoracic impedance detection with blood resistivity compensation\| \|US7937150 \|\|Jan 31, 2006\|\|May 3, 2011\|\|Medtronic, Inc.\|\|Lead-carried proximal electrode for quadripolar transthoracic impedance monitoring\| \|US8050764\|\|Oct 29, 2003\|\|Nov 1, 2011\|\|Cardiac Pacemakers, Inc.\|\|Cross-checking of transthoracic impedance and acceleration signals\| \|US8200326\|\|Jun 12, 2012\|\|Cardiac Pacemakers, Inc.\|\|Calibration of impedance monitoring of respiratory volumes using thoracic D.C. impedance\| \|US8209011\|\|Jun 26, 2012\|\|Cardiac Pacemakers, Inc.\|\|Automatically configurable minute ventilation sensor\| \|US8306621\|\|Nov 6, 2012\|\|Cardiac Pacemakers, Inc.\|\|Cardiac cycle synchronized sampling of impedance signal\| \|US8423142\|\|Oct 31, 2011\|\|Apr 16, 2013\|\|Cardiac Pacemakers, Inc.\|\|Cross-checking of transthoracic impedance and acceleration signals\| \|US8442633\|\|Oct 30, 2012\|\|May 14, 2013\|\|Cardiac Pacemakers, Inc.\|\|Cardiac cycle synchronized sampling of impedance signal\| \|US8521286\|\|May 3, 2011\|\|Aug 27, 2013\|\|Medtronic, Inc.\|\|Lead-carried proximal electrode for quadripolar transthoracic impedance monitoring\| \|US8688214\|\|May 10, 2013\|\|Apr 1, 2014\|\|Cardiac Pacemakers. Inc.\|\|Cardiac cycle synchronized sampling of impedance signal\| \|US8750981 \|\|Aug 25, 2011\|\|Jun 10, 2014\|\|Pacesetter, Inc.\|\|Systems and methods for assessing heart failure and controlling cardiac resynchronization therapy using hybrid impedance measurement configurations\| \|US8781847\|\|May 3, 2005\|\|Jul 15, 2014\|\|Cardiac Pacemakers, Inc.\|\|System and method for managing alert notifications in an automated patient management system\| \|US8880171\|\|Feb 13, 2014\|\|Nov 4, 2014\|\|Cardiac Pacemakers, Inc.\|\|Cardiac cycle synchronized sampling of impedance signal\| \|US9132275\|\|Nov 7, 2011\|\|Sep 15, 2015\|\|Cardiac Pacemakers, Inc.\|\|Automatic determination of chronotropic incompetence using atrial pacing at rest\| \|US9138590\|\|Jun 27, 2012\|\|Sep 22, 2015\|\|Cardiac Pacemakers, Inc.\|\|Implantable medical device sensing and selecting wireless ECG and intracardiac electrogram\| \|US20040186526 \|\|Dec 12, 2003\|\|Sep 23, 2004\|\|Scott Freeberg\|\|Automatically configurable minute ventilation sensor\| \|US20050096704 \|\|Oct 29, 2003\|\|May 5, 2005\|\|Scott Freeberg\|\|Cross-checking of transthoracic impedence and acceleration signals\| \|US20050182447 \|\|Jun 4, 2004\|\|Aug 18, 2005\|\|Schecter Stuart O.\|\|Optimization of impedance signals for closed loop programming of cardiac resynchronization therapy devices\| \|US20060241513 \|\|Apr 26, 2005\|\|Oct 26, 2006\|\|Cardiac Pacemakers, Inc.\|\|Calibration of impedance monitoring of respiratory volumes using thoracic D.C. impedance\| \|US20070179390 \|\|Apr 10, 2007\|\|Aug 2, 2007\|\|Pacesettler, Inc.\|\|Global cardiac performance\| \|US20070179544 \|\|Jan 31, 2006\|\|Aug 2, 2007\|\|Kuehn Kevin P\|\|Lead-carried proximal electrode for quadripolar transthoracic impedance monitoring\| \|US20070191901 \|\|Apr 25, 2007\|\|Aug 16, 2007\|\|Pacesetter, Inc.\|\|Quantifying systolic and diastolic cardiac performance from dynamic impedance waveforms\| \|US20090326408 \|\|Dec 31, 2009\|\|Loell Boyce Moon\|\|Providing Impedance Plethysmography Electrodes\| \|US20110208262 \|\|Aug 25, 2011\|\|Medtronic, Inc.\|\|Lead-carried proximal electrode for quadripolar transthoracic impedance monitoring\| \|US20120150060 \|\|Jun 14, 2012\|\|Pacesetter, Inc.\|\|Method and system to estimate impedance of a pseudo sensing vector\| \|US20130053912 *\|\|Aug 25, 2011\|\|Feb 28, 2013\|\|Pacesetter, Inc.\|\|Systems and methods for assessing heart failure and controlling cardiac resynchronization therapy using hybrid impedance measurement configurations\| \|U.S. Classification\|\|607/17, 600/508, 600/547\| \|International Classification\|\|A61N1/05, A61B5/053, A61N1/365, A61B5/08\| \|Cooperative Classification\|\|A61B5/0535, A61N1/05, A61N1/36185, A61B5/0809, A61N1/3686, A61B5/0538, A61N1/36521\| \|European Classification\|\|A61B5/053N, A61N1/368R, A61N1/36Z5P3A, A61B5/08J\| \|Mar 21, 2002\|\|AS\|\|Assignment\| Owner name: PACESETTER, INC., CALIFORNIA Free format text: ASSIGNMENT OF ASSIGNORS INTEREST;ASSIGNOR:LU, RICHARD;REEL/FRAME:012723/0058 Effective date: 20020314 \|Mar 21, 2008\|\|FPAY\|\|Fee payment\| Year of fee payment: 4 \|Mar 21, 2012\|\|FPAY\|\|Fee payment\| Year of fee payment: 8 \|Mar 21, 2016\|\|FPAY\|\|Fee payment\| Year of fee payment: 12	2	65	1	0	0	0	0.603199	1	12,050
What is the acronym for Health Maintenance Organization? There are two types of medical billing: Institutional and _______________________. An __________ is a statement sent by a health insurance company to covered individuals explaining what medical treatments and/or services were paid for on their behalf. What type of claim form is used by hospitals? Another name for a Health Information Specialist is a _________________________ Specialist. An _____________________________ is a digital version of the traditional paper-based medical record for an individual. The EMR represents a medical record within a single facility, such as a doctor's office or a clinic. What is the amount you pay for health care services before your health insurance begins to pay? A __________ is a fixed amount you pay for a health care service? A primary care physician is also known as a _______________________. What is the abbreviation for out-of-network? What is the abbreviation for in-network? What type of managed care plan lets you choose between an HMO or a PPO each time you need care? How many parts does Medicare have? A 1996 federal law that is sometimes called the "privacy rule", outlining how certain entities can use or disclose personal health information. The reason for the visit or surgery that defines the disease process or injury. M48.1 is an example of what type of code? What does UB stand for in UB-04? The healthcare system, funded by the U.S. Department of Defense, that active and retired military and their dependents use. The entity that reimburses the provider for services. Insurance companies, Medicare, Medicaid, and third-party administrators are all payers in the healthcare industry. Any health care plan, provider, or service that transmits health care information in an electronic form and is thereby governed by laws and regulations in the handling of such data. It's called a covered _______________? Make your own Crossword Create your own Crossword, and print copies for an entire class. All in 5 minutes.	3	2	0	0	0	1	0.714857	1	431
Four Types of Diagnostic Codes and How to Transcribe Them What It Is This discussion is regarding ICD-10 codes taken from the 10th revision of the International Statistical Classification of Diseases and Related Health Problems, a medical classification list by the World Health Organization. International Classification of Diseases (ICD) codes are found on patient paperwork, including hospital records, medical charts, visit summaries, correspondence, and bills. These codes ensure that patients receive proper treatment and are charged appropriately for any medical services received. Types of Coding The Certified Professional Coder certification (CPC®), is the established standard for medical coding in physician office settings and hospitals. Certified professionals help maintain compliance and profitability within healthcare practices through accurate medical coding and documentation. CPCs have demonstrated mastery of all medical code sets, evaluation and management principles, surgical coding, pathology coding, dentistry coding with adherence to documentation and coding guidelines through rigorous examination and experience. Before the CPCs can do their jobs, however, the diagnostic codes must be dictated and transcribed by a Medical Transcriptionist/Healthcare Documentation Specialist (MT/HDS). When transcribing, the codes are typed adjacent to the diagnosis for which they are intended (see codes below). Tab the codes over so they are easy to recognize and the report looks neat. Do not bury the codes within a diagnosis paragraph. Make sure they are positioned so that they are easy to spot for the coder. Medical coding is transcribed in arabic numerals with letters, decimal points, colons, brackets, and parentheses sometimes used. The more specific the dictated diagnoses, the better the coding can be. Codes that describe symptoms and signs, as opposed to diagnoses, are acceptable for reporting purposes when a related definitive diagnosis has not been established (confirmed) by the provider. The subcategory describes the digit that comes after the decimal point. This digit further describes the nature of the illness or injury and gives additional information as to the location or manifestation of the illness or condition. Examples of Diagnostic Codes Below are five samples, transcribed in different styles, that show exactly how coding is typed along with the exact meanings of each individual diagnostic code (see bolded exemplary remarks). - Undisplaced fracture involving the left clavicle with abrasions and contusions. S42.001A S42.001A Fracture of unspecified part of right clavicle, initial encounter for closed fracture - Fracture involving the mandible. S02.600A S02.600A Fracture of unspecified part of body of mandible, unspecified side, initial encounter for closed fracture - Chronic cholecystitis K81.1 K81.1 Chronic cholecystitis - No evidence of malignancy is seen in these sections. (No code exists for this comment.) DIAGNOSTIC IMPRESSION: Mild to moderate osteoarthritis of the right knee, stable for viscosupplementation with Synvisc injection #2. M17.11 Because the above diagnosis is not specified as to whether the patient’s osteoarthritis is primary or post-traumatic, either of the following codes might be correct. If the code is dictated, then transcribe as dictated. M17.11 Unilateral primary osteoarthritis, right knee M17.31 Unilateral post-traumatic osteoarthritis, right knee - Otitis externa, infective. H60.393 H60.393 Other infective otitis externa, unspecified ear - Bronchitis J40 J40 Bronchitis, not specified as acute or chronic ASSESSMENT: Rule out appendicitis. K35.80 If this is an outpatient, this assessment has no diagnostic code; however, if it is an inpatient, all “rule-outs” are coded as the dictated condition, so the diagnostic code would be: K35.80 Unspecified acute appendicitis Thanks to Jennifer Della’Zanna, CPC, CGSC, CEHRS, CHDS, AAPC Fellow, for her help with the above diagnostic codes and their specific meanings in ICD-10. As for medical coding acronyms and abbreviations, here are the top 25 you are sure to come up against: ATD – Applied to Deductible The funds owed to the provider, as determined and fixed by the agreed insurance policy. AOB – Assignment of Benefits Funds paid directly to the medical provider. BCBS – Blue Cross Blue Shield COB – Coordination of Benefits Essentially which insurance agency is the primary provider, and which is the secondary when a patient has more than one policy. DOS – Date of Service DCI – Duplicate Coverage Inquiry When an insurance provider contacts another to see if they’re currently providing specific coverage. EDI – Electronic Data Interchange The electronic network that collects information before delving it out to particular individual insurance providers. EFT – Electronic Funds Transfer Transferring money electronically. A credit or debit charge or transfer must take place. EMR – Electronic Medical Records Digitally formatted health records: the complete record of a patient that is sent to a healthcare provider and/or insurance agency. EOB – Explanation of Benefits An explanation of what the insurance company provides, usually consisting of covered charges, payment methods, deductibles, patient responsibility and potential write-offs. FDCPA – Fair Debt Collection Practices Act Law explaining the guidelines for creditors and collections agencies trying to collect from delinquent accounts. FI – Fiscal Intermediary The Medicare official that handles Medicare claims and cases. HIPAA – Health Insurance Portability & Accountability Act HMO – Health Maintenance Organization A healthcare policy that requires a gatekeeper or primary care physician. If a situation calls for further action, this gatekeeper will refer the patient to a different specialist. INN – In-Network A medical care provider that is contracted with the specific insurance provider used by a patient. IPA – Independent Practice Association The group of medical care providers contracted with an HMO plan. N/C – Non-Covered Charge A healthcare service that is not covered by the insurance policy. NEC – Not Elsewhere Classifiable The abbreviation used on ICD forms when the information given does not permit a more refined assignment. NOS — Not otherwise specified OON – Out of Network A medical service provider that does not currently work with the specific insurance agency. PPO – Preferred Provider Organization A network of medical care providers that patients are allowed to visit, as determined by the insurance agency. PHI – Protected Health Information Basic patient information that remains classified, usually consists of name, date of birth, social security number, insurance ID, medical records and telephone numbers. TIN – Tax Identification Number The specific number assigned to an individual for tax filing and tracking purposes. TOP – Triple Option Plan The cafeteria-style insurance plan that offers a choice of HMP, PPO or traditional insurance policies. UCR – Usual Customary & Reasonable The coverage limitations set in place by an insurance patient. Limits the maximum amount of funding a company will pay for a service. WC – Workers’ Compensation Work-related injury insurance claim Written by Patricia A. Ireland, CMT, AHDI-F Medical Transcription Instructor, Consultant	4	1	0	0	0	8	0.346941	8	1,551
Ghost Cell Glaucoma Summary[edit \| edit source] Ghost cell glaucoma is a secondary open-angle glaucoma caused by degenerated red blood cells (ghost cells) blocking the trabecular meshwork. Disease Entity[edit \| edit source] Ghost cell glaucoma ICD 365.63 Disease[edit \| edit source] Following a vitreous hemorrhage episode, blood breakdown products may accumulate in the trabecular meshwork. Hemolyzed erythrocytes may obstruct aqeous outflow and lead to a secondary open-angle glaucoma known as ghost cell glaucoma. Etiology[edit \| edit source] Ghost cell glaucoma may occur after vitreous hemorrhage. Causes of ghost cell glaucoma include ocular trauma, systemic diseases such as diabetes or sickle cell disease/trait, iritis (Fuchs heterochromic iridocyclitis, herpes simplex, herpes zoster, etc.), intraocular tumors (retinoblastoma, malignant melanoma, etc.), uveitis glaucoma hyphema syndrome, rubeosis iridis, iris varices, papillary microhemangiomas, and ocular surgery including but not limited to cataract extraction, laser trabeculoplasty and iridotomy. One case report of ghost cell glaucoma after a snake bite has been reported. Ghost cell glaucoma has also been reported to occur spontaneously. Risk Factors[edit \| edit source] Risk factors for posttraumatic glaucoma include advancing age, visual acuity on presentation worse than 20/200, iris injury, lens injury, hyphema, and angle recession. Risk factors for ghost cell glaucoma include vitreous hemorrhage. Angle recession is not seen in ghost cell glaucoma. Pathophysiology[edit \| edit source] A constellation of histopathologic findings may develop in the vitreous following vitreous hemorrhage. After 3-10 days, red blood cell (RBC) clots undergo fibrinolysis and red blood cells may diffuse throughout the vitreous cavity. At this time, breakdown of red blood cells also occurs. The hemoglobin that remains within the cell denatures and forms clumps called Heinz bodies, which adhere to the inner surface of the plasma membrane. The extracellular hemoglobin also becomes denatured and clumped, often forming small to large accumulations that tend to adhere to vitreous strands. The adherence to and the entrapment within vitreous strands prevents these extracellular clumps of hemoglobin from moving freely and from passing into the anterior chamber. Loss of hemoglobin from the red blood cells produces ghost cells and hemoglobin spherules. During the conversion to the ghost cell form, intracellular hemoglobin is lost, presumably through leaky membranes, into the extracellular vitreous spaces. Ghost cells appear as small, spherical, khaki-colored cells and do not adhere to each other or to the vitreous strands and are free to move anteriorly. They gain access to the anterior chamber through a disrupted anterior hyaloid face, which can occur from previous surgery (pars plana vitrectomy, cataract extraction, or capsulotomy), trauma or spontaneous disruption. Ghost cells are generally 4 to 7 micrometers in size and less pliable than normal RBCs. As a result of their loss of pliability, ghost cells remain longer in the anterior chamber because their rigidity makes it difficult for them to escape through the trabecular meshwork. This causes obstruction of the trabecular meshwork and secondary glaucoma. The cells develop within 1-3 months of a vitreous hemorrhage. It is important to note that the presence of ghost cells does not necessarily lead to development of ghost cell glaucoma. Diagnosis[edit \| edit source] Ghost cell glaucoma is a clinical diagnosis. Diagnostic findings include presence of heme in the vitreous, ghost cells in the anterior chamber, delayed onset of increased intraocular pressure, an open angle on gonioscopy with possible presence of ghost cells layering over the trabecular meshwork inferiorly due to gravity , and often a disrupted anterior hyaloid face. Historically, the diagnosis of ghost cell glaucoma was made by phase-contrast microscopy* of anterior chamber (AC) aspirate, paraffin embedding after centrifugation of AC aspirate, or staining of the sample with 1 % methyl violet. Heinz bodies, spherical erythrocytes with denaturized hemoglobin granules bound to the internal surface of the cell membrane, are observed with H&E staining. History[edit \| edit source] Ghost cell glaucoma was originally described by Campbell et al in 1976. Once it was also termed “hemophthalmitis” which was a misnomer as usually there is no evidence of active inflammation and this term has been since abandoned. Physical Exam[edit \| edit source] Clinically, patients present with intraocular pressure (IOP) spikes (as high as 60 to 70 mmHg) and a history of vitreous hemorrhage resulting from trauma, surgery, or preexisting retinal disease 1-3 months prior to presentation. The IOP may be markedly elevated, causing corneal edema. The anterior chamber is filled with small, circulating, tan colored cells. If fresh red blood cells exist, two or more different layers of cells are seen, with the lighter khaki-colored layer of ghost cells appearing on top of a heavier, red blood cell layer, imparting a candy-striped appearance. The cellular reaction appears out of proportion to the aqeous flare. The conjunctiva tends not be inflamed unless the IOP is markedly elevated or there is a history of previous surgery. Gonioscopy reveals either a normal appearing open angle; an open angle covered by a fine layer of khaki-colored cells, which have slightly to moderately discolored the trabecular meshwork; or a heavy layer filling the angle, generally inferiorly, with cells composing an early pseudohypopyon. The vitreous has the appearance of an old hemorrhage, with characteristic khaki coloration of RBCs and clumps of extracellular pigmentation from degenerated hemoglobin. Symptoms[edit \| edit source] The symptoms relate to the etiology. Pain secondary to trauma or surgery may be experienced by the patient. However, less pain is reported than expected from a severely elevated IOP. Patients with high IOP may also present with blurry vision, headache, brow ache, nausea and/or vomiting. Diagnostic Procedures[edit \| edit source] The diagnosis of ghost cell glaucoma is usually clinical. The history and paracentesis with phase-contrast microscopy of the aspirate would confirm this diagnosis. Laboratory Tests[edit \| edit source] For spontaneous hemorrhages, complete blood count with coagulation profile and sickle cell prep in African American patients are warranted. Differential Diagnosis[edit \| edit source] Differential Diagnosis of Ghost cell glaucoma includes Angle Recession glaucoma, Hemolytic glaucoma, Hemosiderotic glaucoma, Uveitic glaucoma, Neovascular glaucoma and Acute angle closure (Mechanical secondary to trauma). Hemosiderotic Glaucoma is a late onset glaucoma following intraocular hemorrhage with iron deposition in and damage to the trabecular meshwork. This extremely rare glaucoma is more chronic, does not have ghost cells in the anterior chamber, and is characteristically associated with a slight discoloration of the meshwork. It occurs many years after the original injury, in contrast to the ghost cell glaucoma, which occurs within weeks to months after the original injury. Hemolytic Glaucoma is a type of secondary glaucoma where red blood cell debris and macrophages block the trabecular meshwork after a vitreous hemorrhage. In ghost cell glaucoma, little to no red blood cell debris and few to no macrophages are found in the trabecular meshwork. Neovascular glaucoma is differentiated from ghost cell glaucoma by the absence of ghost cells within the anterior chamber and the presence of neovascularization at the papillary margin and in the angle. The history of vitreous hemorrhage, disruption of the hyaloid face, a multitude of tiny khaki-colored cells, a relatively non inflamed conjunctiva, and an absence of keratic precipitates differentiates ghost cell glaucoma from uveitis and endophthalmitis. Management[edit \| edit source] Ghost cell glaucoma usually resolves once the vitreous hemorrhage has cleared. Medical therapy with aqeous suppressants is the preferred initial approach. Surgical intervention is often necessary because of a persistently elevated intraocular pressure despite maximum medical therapy. Medical Therapy[edit \| edit source] Aqeous suppressants are the first line approach. Monotherapy or a combination of topical alpha adrenergic agonists, beta adrenergic blockers, parasympathomimmetics, prostaglandin analogues, and carbonic anhydrase inhibitors may be used. An oral carbonic anyhdrase inhibitor may be added. Intravenous Mannitol or Diamox may be used for extremely high IOPs in acute settings. This may occur once cells reaccumulate in the trabecular meshwork after initial clearing. Surgery[edit \| edit source] Surgery might be required to clear the cell load from the trabecular meshwork. This can be accomplished by AC paracentesis and irrigation, pars plana vitrectomy (PPV), and/or a trabeculectomy. If AC washout lowers the intraocular pressure successfully but the pressure rises again because of the further entrance of ghost cells from the vitreous, a washout of the AC can be repeated. If this relatively simple and safe procedure is unsuccessful, vitrectomy to remove the contents of the vitreous cavity may be required. For refractory glaucoma caused by chronic obstruction of trabecular meshwork by ghost cells, trabeculectomy or usage of glaucoma drainage device is warranted. Complications[edit \| edit source] If the IOP is uncontrolled, this may lead to optic nerve damage, however given the number of treatment options that are available, this is rare. Also, ghost cell glaucoma usually resolves once the hemorrhage clears. Prognosis[edit \| edit source] Prognosis of ghost cell glaucoma is usually excellent as the condition is typically transient, although it may last many months. Eventually, the supply of erythrocyte ghosts in the vitreous cavity become exhausted and cells stop passing forward into the anterior chamber. If ghost cells linger, surgical treatment options are available as discussed above. References[edit \| edit source] Albert DM & Miller JW. Principles and Practice of Opthalmology. Third Edition. Philadelphia, PA. W.B Saunders Company © 2000 and Elsevier, Inc © 20008. Campbell DG, Simmons RJ, & Grant WM. Ghost cells as a cause of glaucoma. Am J Ophthalmol. 1976; 81:441-440. Cioffi GA, Durcan FJ, Girkin CA, Gross RL, Netland PA, Samples JR, Samuelson TW, O’Connell SS & Barton K. Glaucoma. Last major revision 2008-2009. San Francisco, CA. American Academy of Ophthalmology. Copyright 2010. Girkin CA, McGwin G, Cherie L, Robert M & Ferenc K. Glaucoma after ocular contusion: A cohort study of the United States eye injury registry. Journal of Glaucoma. 2005; 14(6): 470-473. Ritch R, Shields MB & Krupin T. The Glaucomas. Volume 2. St. Louis, MI. C.V. Mosby Company. © 1989. Rojas L, Ortiz G, Gutierrez M & Corredor, S. Ghost Cell Glaucoma Related to Snake Poisoning. Arch Ophthalmol. 2001; 119 (8): 1212-1213. Shetlar DJ, Chevez-Barrios P, Dubovy S, Rosa RH, Syed N, Wilson MW, Pelton RW & Pe’er J. Ophthalmic Pathology and Intraocular Tumors. Last major revision 2007-2008. San Francisco, CA. American Academy of Ophthalmology. Copyright 2010. Spraul CW & Grossniklaus HE. Vitreous Hemorrhage. Surv Ophthalmol. 1997; 42(1): 3-39. Mansour AM, Chess J, Starita R. Nontraumatic ghost cell glaucoma- a case report. Ophthalmic Surg. 1986;17:34-36. - Phase contrast microscopy imparts contrast to unstained biological material by transforming phase differences of light caused by differences in refractive index between cellular components into differences in amplitude of light, i.e., light and dark areas, which can be observed.	2	6	1	0	0	0	0.673244	1	2,872
Acute respiratory distress syndrome This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages)(Learn how and when to remove this template message) \|Acute respiratory distress syndrome\| Chest x-ray of patient with ARDS \|Classification and external resources\| \|Specialty\|\|Critical care medicine\| \|Patient UK\|\|Acute respiratory distress syndrome\| Acute respiratory distress syndrome (ARDS), previously known as respiratory distress syndrome (RDS), adult respiratory distress syndrome, or shock lung, is a medical condition occurring in critically ill patients characterized by widespread inflammation in the lungs. ARDS is not a particular disease, rather it is a clinical phenotype which may be triggered by various pathologies such as trauma, pneumonia and sepsis. The hallmark of ARDS is diffuse injury to cells which form the alveolar barrier, surfactant dysfunction, activation of the innate immune response, and abnormal coagulation. In effect, ARDS results in impaired gas exchange within the lungs at the level of the microscopic alveoli. The syndrome is associated with a high mortality rate between 20 and 50%. The mortality rate with ARDS varies widely based on severity, the patient's age, and the presence of other underlying medical conditions. Although the terminology of "adult respiratory distress syndrome" has at times been used to differentiate ARDS from "infant respiratory distress syndrome" in neonates, international consensus is that "acute respiratory distress syndrome" is the best moniker because ARDS can affect those of all ages. - 1 Signs and symptoms - 2 Causes - 3 Diagnosis - 4 Pathophysiology - 5 Treatment - 6 Complications - 7 Epidemiology - 8 History - 9 See also - 10 References - 11 Further reading - 12 External links Signs and symptoms The signs and symptoms of ARDS often begin within two hours of an inciting event, but can occur after 1–3 days. Signs and symptoms may include shortness of breath, fast breathing, and a low oxygen level in the blood. A chest x-ray frequently demonstrates generalized infiltrates or opacities in both lungs, which represent fluid accumulation in the lungs.Other signs and symptoms that occur in people with ARDS may be associated with the underlying disease process. For example, those with ARDS from sepsis may have low blood pressure and fever, while a person with pneumonia may have a cough. The predisposing factors of ARDS are numerous and assorted. Common causes of ARDS include sepsis, pneumonia, trauma, multiple blood transfusions, babesiosis, lung contusion, aspiration of stomach contents, and drug abuse or overdose. Other causes of ARDS include burns, pancreatitis, near drowning, or the inhalation of chemical irritants such as smoke, phosgene, or chlorine gas.Some cases of ARDS are linked to large volumes of fluid used during post-trauma resuscitation. Diagnostic criteria for ARDS have evolved over time, in step with an increasing understanding of both the pathophysiology and the limits of diagnostic capability in general practice. The international consensus criteria for ARDS were most recently updated in 2012 and are known as the "Berlin definition". In addition to generally broadening the diagnostic thresholds, other notable changes from the prior 1994 consensus criteria include discouraging the term "acute lung injury," and defining grades of ARDS severity according to degree of hypoxemia (see history). According to the 2012 Berlin definition, ARDS is characterized by: - lung injury of acute onset, within 1 week of an apparent clinical insult and with progression of respiratory symptoms - bilateral opacities on chest imaging (chest radiograph or CT) not explained by other lung pathology (e.g. effusion, pneumothorax, or nodules) - respiratory failure not explained by heart failure or volume overload - decreased PaO 2 ratio (a decreased PaO 2 ratio indicates reduced arterial oxygenation from the available inhaled gas): - mild ARDS: 201 - 300 mmHg (≤ 39.9 kPa) - moderate ARDS: 101 - 200 mmHg (≤ 26.6 kPa) - severe ARDS: ≤ 100 mmHg (≤ 13.3 kPa) - Note that the Berlin definition requires a minimum positive end expiratory pressure (PEEP) of 5 cmH 2O for consideration of the PaO 2 ratio. This degree of PEEP may be delivered noninvasively with CPAP to diagnose mild ARDS. - acute onset - bilateral infiltrates on chest radiograph sparing costophrenic angles - pulmonary artery wedge pressure < 18 mmHg (obtained by pulmonary artery catheterization), if this information is available; if unavailable, then lack of clinical evidence of left atrial hypertension - if PaO 2 < 300 mmHg (40 kPa) acute lung injury (ALI) is considered to be present - if PaO 2 < 200 mmHg (26.7 kPa) acute respiratory distress syndrome (ARDS) is considered to be present With these 1994 criteria, arterial blood gas analysis and chest X-ray were required for formal diagnosis. Although severe hypoxemia is generally included, the appropriate threshold defining abnormal PaO 2 has never been systematically studied. A severe oxygenation defect is not synonymous with ventilatory support. Any PaO 2 below 100 (generally saturation less than 100%) on a supplemental oxygen fraction of 50% meets criteria for ARDS. This can easily be achieved by high flow oxygen supplementation without ventilatory support. Medical imaging is key to diagnosis. While a chest x-ray is often ordered as a first-line test, CT scanning showing a bilateral infiltrative process may also be helpful. Lung ultrasound, performed at the point of care, is becoming increasingly adopted in critical care medicine and aids in the diagnosis of ARDS as well, accurately distinguishing among the various causes of acute respiratory failure. Ultrasound findings suggestive of ARDS, as opposed to cardiogenic pulmonary edema, include: - Anterior subpleural consolidations - Absence or reduction of lung sliding - “Spared areas” of normal parenchyma - Pleural line abnormalities (irregular thickened fragmented pleural line) - Nonhomogeneous distribution of B-lines [characteristic ultrasonographic findings suggestive of edema] ARDS is a form of noncardiogenic pulmonary edema provoked by an acute injury to the lungs that results in flooding of the lungs' microscopic air sacs responsible for the exchange of gases such as oxygen and carbon dioxide with capillaries in the lungs. Additional common findings in ARDS include partial collapse of the lungs (atelectasis) and low levels of oxygen in the blood (hypoxemia). However, in ARDS, these changes are not due to heart failure. The clinical syndrome is associated with pathological findings including pneumonia, eosinophilic pneumonia, cryptogenic organizing pneumonia, acute fibrinous organizing pneumonia, and diffuse alveolar damage (DAD). Of these, the pathology most commonly associated with ARDS is DAD, which is characterized by a diffuse inflammation of lung tissue. The triggering insult to the tissue usually results in an initial release of chemical signals and other inflammatory mediators secreted by local epithelial and endothelial cells. Neutrophils and some T-lymphocytes quickly migrate into the inflamed lung tissue and contribute in the amplification of the phenomenon. Typical histological presentation involves diffuse alveolar damage and hyaline membrane formation in alveolar walls. Although the triggering mechanisms are not completely understood, recent research has examined the role of inflammation and mechanical stress. Inflammation, such as that caused by sepsis, causes endothelial dysfunction, fluid leakage from the capillaries and impaired drainage of fluid from the lungs. Elevated inspired oxygen concentration often becomes necessary at this stage, and may facilitate a 'respiratory burst' in immune cells. In a secondary phase, endothelial dysfunction causes cells and inflammatory exudate to enter the alveoli. This pulmonary edema increases the thickness of the alveolocapillary space, increasing the distance the oxygen must diffuse to reach the blood, which impairs gas exchange leading to hypoxia, increases the work of breathing and eventually induces fibrosis of the airspace. Edema and decreased surfactant production by type II pneumocytes may cause whole alveoli to collapse or to completely flood. This loss of aeration contributes further to the right-to-left shunt in ARDS. Similar to a traditional right-to-left shunt which refers to blood passing from the right side of the heart to the left side, thus bypassing oxygenation, lung right-to-left shunting occurs within the lungs. As the alveoli contain progressively less gas, the blood flowing through the alveolar capillaries is progressively less oxygenated, resulting in massive intrapulmonary shunting. The collapse of alveoli and small airways interferes with the process of normal gas exchange. It is common to see patients with a PaO 2 of 60 mmHg (8.0 kPa) despite mechanical ventilation with 100% inspired oxygen. The loss of aeration may follow different patterns depending upon the nature of the underlying disease and other factors. These are usually distributed to the lower lobes of the lungs, in their posterior segments, and they roughly correspond to the initial infected area. In sepsis or trauma-induced ARDS, infiltrates are usually more patchy and diffuse. The posterior and basal segments are always more affected, but the distribution is even less homogeneous. Loss of aeration also causes important changes in lung mechanical properties that are fundamental in the process of inflammation amplification and progression to ARDS in mechanically ventilated patients. Mechanical ventilation is an essential part of the treatment of ARDS. As the loss of aeration and the underlying disease progress, the end tidal volume grows to a level incompatible with life. Thus, mechanical ventilation is initiated to relieve respiratory muscles of their work and to protect the usually obtunded patient's airways. However, mechanical ventilation may constitute a risk factor for the development—or the worsening—of ARDS. Aside from the infectious complications arising from invasive ventilation with tracheal intubation, positive-pressure ventilation directly alters lung mechanics during ARDS. When these techniques are used the result is higher mortality through barotrauma. In 1998, Amato et al. published a paper showing substantial improvement in the outcome of patients ventilated with lower tidal volumes (Vt) (6 mL·kg−1). This result was confirmed in a 2000 study sponsored by the NIH. Both studies were widely criticized for several reasons, and the authors were not the first to experiment with lower-volume ventilation, but they increased the understanding of the relationship between mechanical ventilation and ARDS. This form of stress is thought to be applied by the transpulmonary pressure (gradient) (Pl) generated by the ventilator or, better, its cyclical variations. The better outcome obtained in patients ventilated with lower Vt may be interpreted as a beneficial effect of the lower Pl. The way Pl is applied on alveolar surface determines the shear stress to which lung units are exposed. ARDS is characterized by a usually inhomogeneous reduction of the airspace, and thus by a tendency towards higher Pl at the same Vt, and towards higher stress on less diseased units. The inhomogeneity of alveoli at different stages of disease is further increased by the gravitational gradient to which they are exposed and the different perfusion pressures at which blood flows through them. The different mechanical properties of alveoli in ARDS may be interpreted as having varying time constants—the product of alveolar compliance × resistance.Slow alveoli are said to be "kept open" using PEEP, a feature of modern ventilators which maintains a positive airway pressure throughout the whole respiratory cycle. A higher mean pressure cycle-wide slows the collapse of diseased units, but it has to be weighed against the corresponding elevation in Pl/plateau pressure. Newer ventilatory approaches attempt to maximize mean airway pressure for its ability to "recruit" collapsed lung units while minimizing the shear stress caused by frequent openings and closings of aerated units. Mechanical ventilation can exacerbate the inflammatory response in patients with ARDS by including cyclic tidal alveolar hyperinflation and/or recruiting/derecruiting. Stress index is measured during constant-flow assist-control mechanical ventilation without changing the baseline ventilatory pattern. Identifying the steadiest portion of the inspiratory flow (F) waveform fit the corresponding portion of the airway pressure (Paw) waveform in the following power equation: Paw = a × tb + c where the coefficient b—the Stress Index—describes the shape of the curve. The Stress Index depict a constant compliance if the value is around 1, an increasing compliance during the inspiration if the value is below 1, and a decreasing compliance if the value is above 1. Ranieri, Grasso, et al. set a strategy guided by the stress index with the following rules: - Stress Index below 0.9, PEEP was increased - Stress Index between 0.9 and 1.1, no change was made - Stress Index above 1.1 PEEP was decreased. Alveolar hyperinflation in patients with focal ARDS ventilated with the ARDSnet protocol is attenuated by a physiologic approach to PEEP setting based on the stress index measurement. If the underlying disease or injurious factor is not removed, the quantity of inflammatory mediators released by the lungs in ARDS may result in a systemic inflammatory response syndrome or sepsis if there is lung infection. The evolution towards shock or multiple organ dysfunction syndrome follows paths analogous to the pathophysiology of sepsis. This leads to the impaired oxygenation which is the central problem of ARDS, as well as to respiratory acidosis—which is often caused by ventilation techniques such as permissive hypercapnia, which attempt to limit ventilator-induced lung injury in ARDS. The result is a critical illness in which the 'endothelial disease' of severe sepsis or SIRS is worsened by the pulmonary dysfunction, which further impairs oxygen delivery. Acute respiratory distress syndrome is usually treated with mechanical ventilation in the intensive care unit (ICU). Mechanical ventilation is usually delivered through orotracheal intubation, or by tracheostomy whenever prolonged ventilation (≥2 weeks) is deemed inevitable. The possibilities of non-invasive ventilation are limited to the very early period of the disease or to prevention in individuals with atypical pneumonias, lung contusion, or major surgery patients, who are at risk of developing ARDS. Treatment of the underlying cause is imperative. Appropriate antibiotic therapy must be administered as soon as microbiological culture results are available. Empirical therapy may be appropriate if local microbiological surveillance is efficient. The origin of infection, when surgically treatable, must be operated on. When sepsis is diagnosed, appropriate local protocols should be enacted. Commonly used supportive therapy includes particular techniques of mechanical ventilation and pharmacological agents whose effectiveness with respect to the outcome has not yet been proven. The overall goal is to maintain acceptable gas exchange and to minimize adverse effects in its application. The parameters PEEP (positive end-expiratory pressure, to maintain maximal recruitment of alveolar units), mean airway pressure (to promote recruitment and predictor of hemodynamic effects) and plateau pressure (best predictor of alveolar overdistention) are used. Conventional therapy aimed at tidal volumes (Vt) of 12–15 ml/kg (where the weight is ideal body weight rather than actual weight). Recent studies have shown that high tidal volumes can overstretch alveoli resulting in volutrauma (secondary lung injury). The ARDS Clinical Network, or ARDSNet, completed a trial that showed improved mortality when ventilated with a tidal volume of 6 ml/kg compared to the traditional 12 ml/kg. Low tidal volumes (Vt) may cause hypercapnia and atelectasis because of their inherent tendency to increase physiologic shunt. Physiologic dead space cannot change as it is ventilation without perfusion. A shunt is perfusion without ventilation. Low tidal volume ventilation was the primary independent variable associated with reduced mortality in the NIH-sponsored ARDSnet trial of tidal volume in ARDS. Plateau pressure less than 30 cm H 2O was a secondary goal, and subsequent analyses of the data from the ARDSnet trial and other experimental data demonstrate that there appears to be no safe upper limit to plateau pressure; regardless of plateau pressure, patients fare better with low tidal volumes. Airway pressure release ventilation No particular ventilator mode is known to improve mortality in Acute respiratory distress syndrome (ARDS). Some practitioners favor airway pressure release ventilation when treating ARDS. Well documented advantages to APRV ventilation include: decreased airway pressures, decreased minute ventilation, decreased dead-space ventilation, promotion of spontaneous breathing, almost 24-hour-a-day alveolar recruitment, decreased use of sedation, near elimination of neuromuscular blockade, optimized arterial blood gas results, mechanical restoration of FRC (functional residual capacity), a positive effect on cardiac output (due to the negative inflection from the elevated baseline with each spontaneous breath), increased organ and tissue perfusion and potential for increased urine output secondary to increased kidney perfusion. A patient with ARDS, on average, spends between 8 and 11 days on a mechanical ventilator; APRV may reduce this time significantly and conserve valuable resources. Positive end-expiratory pressure Positive end-expiratory pressure (PEEP) is used in mechanically ventilated patients with ARDS to improve oxygenation. In ARDS, three populations of alveoli can be distinguished. There are normal alveoli which are always inflated and engaging in gas exchange, flooded alveoli which can never, under any ventilatory regime, be used for gas exchange, and atelectatic or partially flooded alveoli that can be "recruited" to participate in gas exchange under certain ventilatory regimens. The recruitable alveoli represent a continuous population, some of which can be recruited with minimal PEEP, and others which can only be recruited with high levels of PEEP. An additional complication is that some alveoli can only be opened with higher airway pressures than are needed to keep them open, hence the justification for maneuvers where PEEP is increased to very high levels for seconds to minutes before dropping the PEEP to a lower level. PEEP can be harmful; high PEEP necessarily increases mean airway pressure and alveolar pressure, which can damage normal alveoli by overdistension resulting in DAD. A compromise between the beneficial and adverse effects of PEEP is inevitable. The 'best PEEP' used to be defined as 'some' cmH 2O above the lower inflection point (LIP) in the sigmoidal pressure-volume relationship curve of the lung. Recent research has shown that the LIP-point pressure is no better than any pressure above it, as recruitment of collapsed alveoli—and, more importantly, the overdistension of aerated units—occur throughout the whole inflation. Despite the awkwardness of most procedures used to trace the pressure-volume curve, it is still used by some[who?] to define the minimum PEEP to be applied to their patients. Some new ventilators can automatically plot a pressure-volume curve. PEEP may also be set empirically. Some authors[who?] suggest performing a 'recruiting maneuver'—a short time at a very high continuous positive airway pressure, such as 50 cmH 2O (4.9 kPa)—to recruit or open collapsed units with a high distending pressure before restoring previous ventilation. The final PEEP level should be the one just before the drop in PaO 2 or peripheral blood oxygen saturation during a step-down trial. Intrinsic PEEP (iPEEP) or auto-PEEP—first described by John Marini of St. Paul Regions Hospital—is a potentially unrecognized contributor to PEEP in patients. When ventilating at high frequencies, its contribution can be substantial, particularly in patients with obstructive lung disease. iPEEP has been measured in very few formal studies on ventilation in ARDS patients, and its contribution is largely unknown. Its measurement is recommended in the treatment of ARDS patients, especially when using high-frequency (oscillatory/jet) ventilation. of lung infiltrates in acute respiratory distress syndrome is non-uniform. Repositioning into the prone position (face down) might improve oxygenation by relieving atelectasis and improving perfusion. If this is done early in the treatment of severe ARDS, it confers a mortality benefit of 26% compared to supine ventilation. An NIH-sponsored multicenter ARDSnet study of corticosteroids that ran from August 1997 to November 2003 titled LaSRS for ARDS demonstrated that despite an improvement in cardiovascular physiology, methylprednisone is not efficacious in treatment for ARDS. Inhaled nitric oxide (NO) potentially acts as selective pulmonary vasodilator which allows for more gas exchange. However, despite evidence of increased oxygenation status, there is no evidence that inhaled nitric oxide decreases morbidity and mortality in ARDS patients. Furthermore, nitric oxide may cause kidney damage and is not recommended as therapy for ARDS regardless of severity. Extracorporeal membrane oxygenation (ECMO) Extracorporeal membrane oxygenation is mechanically applied prolonged cardiopulmonary support. There are two types of ECMO: Venovenous which provides respiratory support and venoarterial which provides respiratory and hemodynamic support. Patients with ARDS who do not require cardiac support typically undergo venovenous ECMO. Multiple studies have shown the effectiveness of ECMO in acute respiratory failure. Specifically, the CESAR (Conventional ventilatory support versus Extracorporeal membrane oxygenation for Severe Acute Respiratory failure) trial demonstrated that a group referred to an ECMO center demonstrated significantly increased survival compared to conventional management (63% to 47%). Since ARDS is an extremely serious condition which requires invasive forms of therapy it is not without risk. Complications to be considered are: - Pulmonary: barotrauma (volutrauma), pulmonary embolism (PE), pulmonary fibrosis, ventilator-associated pneumonia (VAP) - Gastrointestinal: bleeding (ulcer), dysmotility, pneumoperitoneum, bacterial translocation - Cardiac: abnormal heart rhythms, myocardial dysfunction - Kidney: acute kidney failure, positive fluid balance - Mechanical: vascular injury, pneumothorax (by placing pulmonary artery catheter), tracheal injury/stenosis (result of intubation and/or irritation by endotracheal tube - Nutritional: malnutrition (catabolic state), electrolyte deficiency. The annual incidence of ARDS is 13-23 people per 100,000 in the general population. Its incidence in the mechanically ventilated population in intensive care units is much higher. According to Brun-Buisson et al (2004), there is a prevalence of acute lung injury (ALI) of 16.1% percent in ventilated patients admitted for more than 4 hours. Worldwide, severe sepsis is the most common trigger causing ARDS. Other triggers include mechanical ventilation, sepsis, pneumonia, Gilchrist's disease, circulatory shock, aspiration, trauma—especially pulmonary contusion—major surgery, massive blood transfusions, smoke inhalation, drug reaction or overdose, fat emboli and reperfusion pulmonary edema after lung transplantation or pulmonary embolectomy. Pneumonia and sepsis are the most common triggers, and pneumonia is present in up to 60% of patients and may be either causes or complications of ARDS. Alcohol excess appears to increase the risk of ARDS. Diabetes was originally thought to decrease the risk of ARDS, but this has shown to be due to an increase in the risk of pulmonary edema. Elevated abdominal pressure of any cause is also probably a risk factor for the development of ARDS, particularly during mechanical ventilation. Acute respiratory distress syndrome was first described in 1967 by Ashbaugh et al. Initially there was no clearly established definition, which resulted in controversy regarding the incidence and death of ARDS. In 1988, an expanded definition was proposed, which quantified physiologic respiratory impairment. 1994 American-European Consensus Conference on ARDS In 1994, a new definition was recommended by the American-European Consensus Conference Committee (AECC). It is simple to use and it recognizes that severity of pulmonary injury varies. ARDS was defined as the ratio of arterial partial oxygen tension (PaO 2) as fraction of inspired oxygen (FiO 2) below 200 mmHg in the presence of bilateral infiltrates on the chest x-ray. These infiltrates may appear similar to those of left ventricular failure, but the cardiac silhouette appears normal in ARDS. Also, the pulmonary capillary wedge pressure is normal (less than 18 mmHg) in ARDS, but raised in left ventricular failure. A PaO 2 ratio less than 300 mmHg with bilateral infiltrates indicates acute lung injury (ALI). Although formally considered different from ARDS, ALI is usually a precursor to ARDS. 2012 Berlin Definition In 2012, the Berlin Definition of ARDS was devised by the European Society of Intensive Care Medicine, and was endorsed by the American Thoracic Society and the Society of Critical Care Medicine. These recommendations were an effort to both update classification criteria in order to improve clinical usefulness, and to clarify terminology. Notably, the Berlin guidelines discourage the use of the term "acute lung injury" or ALI, as the term was commonly being misused to characterize a less severe degree of lung injury. Instead, the committee proposes a classification of ARDS severity as mild, moderate or severe according to arterial oxygen saturation. The Berlin definitions represent the current international consensus guidelines for both clinical and research classification of ARDS. - Fanelli, Vito; Ranieri, V. Marco (2015-03-01). "Mechanisms and clinical consequences of acute lung injury". Annals of the American Thoracic Society. 12 Suppl 1: S3–8. doi:10.1513/AnnalsATS.201407-340MG. ISSN 2325-6621. PMID 25830831. - Rubenfeld, Gordon D.; Caldwell, Ellen; Peabody, Eve; Weaver, Jim; Martin, Diane P.; Neff, Margaret; Stern, Eric J.; Hudson, Leonard D. (20 October 2005). "Incidence and Outcomes of Acute Lung Injury". New England Journal of Medicine 353 (16): 1685–1693. doi:10.1056/NEJMoa050333. PMID 16236739. - Bernard G, Artigas A, Brigham K, Carlet J, Falke K, Hudson L, Lamy M, Legall J, Morris A, Spragg R (1994). "The American-European Consensus Conference on ARDS. Definitions, mechanisms, relevant outcomes, and clinical trial coordination". Am J Respir Crit Care Med 149 (3 Pt 1): 818–24. doi:10.1164/ajrccm.149.3.7509706. PMID 7509706. - Bakowitz, Magdalena (August 2012). "Acute lung injury and the acute respiratory distress syndrome in the injured patient". Scandinavian Journal of Trauma, Resuscitation and Emergency Medicine 20: 54. doi:10.1186/1757-7241-20-54. - Marino (2006), pp 435 - Melmed 2011, pp. 636 - Cherkas, David (Nov 2011). "Traumatic Hemorrhagic Shock: Advances In Fluid Management". Emergency Medicine Practice 13 (11). - Ranieri VM, Rubenfeld GD, Thompson BT, Ferguson ND, Caldwell E, Fan E, Camporota L, Slutsky AS (Jun 2012). "Acute respiratory distress syndrome: the Berlin Definition. ARDS Definition Task Force". JAMA. 307 (23): 2526–33. doi:10.1001/jama.2012.5669. PMID 22797452. - Ferguson ND, Fan E, Camporota L, Antonelli M, Anzueto A, Beale R, Brochard L, Brower R, Esteban A, et al. (Oct 2012). "The Berlin definition of ARDS: an expanded rationale, justification, and supplementary material". Intensive Care Med. 38 (10): 1573–82. doi:10.1007/s00134-012-2682-1. PMID 22926653. Erratum in: Intensive Care Med. 2012 Oct;38(10):1731-2. PMID 22926653 - Irwin RS, Rippe JM (2003). Irwin and Rippe's Intensive Care Medicine (5th ed.). Lippincott Williams & Wilkins. ISBN 0-7817-3548-3. - Volpicelli, Giovanni; Elbarbary, Mahmoud; Blaivas, Michael; Lichtenstein, Daniel A.; Mathis, Gebhard; Kirkpatrick, Andrew W.; Melniker, Lawrence; Gargani, Luna; Noble, Vicki E. (2012-04-01). "International evidence-based recommendations for point-of-care lung ultrasound". Intensive Care Medicine 38 (4): 577–591. doi:10.1007/s00134-012-2513-4. ISSN 1432-1238. PMID 22392031. - Boyle, AJ; Mac Sweeney, R; McAuley, DF (August 2013). "Pharmacological treatments in ARDS; a state-of-the-art update". BMC Med 11: 166. doi:10.1186/1741-7015-11-166. PMC 3765621. PMID 23957905. - Amato M, Barbas C, Medeiros D, Magaldi R, Schettino G, Lorenzi-Filho G, Kairalla R, Deheinzelin D, Munoz C, Oliveira R, Takagaki T, Carvalho C (1998). "Effect of a protective-ventilation strategy on mortality in the acute respiratory distress syndrome". N Engl J Med 338 (6): 347–54. doi:10.1056/NEJM199802053380602. PMID 9449727. - MacIntyre N (2000). "Mechanical ventilation strategies for lung protection". Semin Respir Crit Care Med 21 (3): 215–22. doi:10.1055/s-2000-9850. PMID 16088734. - Slutsky AS (May 2005). "Ventilator-induced lung injury: from barotrauma to biotrauma" (PDF). Respir Care 50 (5): 646–59. PMID 15912625. - Grasso S, Stripoli T, De Michele M, et al. (October 2007). "ARDSnet ventilatory protocol and alveolar hyperinflation: role of positive end-expiratory pressure". Am. J. Respir. Crit. Care Med. 176 (8): 761–7. doi:10.1164/rccm.200702-193OC. PMID 17656676. - Malhotra A (2007). "Low-tidal-volume ventilation in the acute respiratory distress syndrome". N Engl J Med 357 (11): 1113–20. doi:10.1056/NEJMct074213. PMC 2287190. PMID 17855672. - Hager et al., American Journal of Respiratory and Critical Care Medicine, 2005 - Frawley P. Milo; Habashi Nader M. (2001). "Airway Pressure Release Ventilation: Theory and Practice" (PDF). AACN Clinical Issues 12 (2): 234–246. doi:10.1097/00044067-200105000-00007. PMID 11759551. Archived from the original (PDF) on March 5, 2009. (archived from the original on 2009-03-05) - a positive effect on cardiac output - Sud S, Friedrich JO, Adhikari NK, et al. (8 Jul 2014). "Effect of prone positioning during mechanical ventilation on mortality among patients with acute respiratory distress syndrome: a systematic review and meta-analysis". CMAJ 186 (10): E381–90. doi:10.1503/cmaj.140081. PMC 4081236. PMID 24863923. - Steinberg, KP; Hudson, LD; Goodman, RB; Hough, CL; Lanken, PN; Hyzy, R; Thompson, BT; Ancukiewicz, M (20 April 2006). National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS) Clinical Trials. "Efficacy and safety of corticosteroids for persistent acute respiratory distress syndrome.". The New England Journal of Medicine 354 (16): 1671–84. doi:10.1056/nejmoa051693. PMID 16625008. \|last9=in Authors list (help) - Hough, CL (December 2014). "Steroids for acute respiratory distress syndrome?". Clinics in chest medicine 35 (4): 781–95. doi:10.1016/j.ccm.2014.08.014. PMC 4297198. PMID 25453425. - Adhikari, NK; Burns, KE; Friedrich, JO; Granton, JT; Cook, DJ; Meade, MO (14 April 2007). "Effect of nitric oxide on oxygenation and mortality in acute lung injury: systematic review and meta-analysis". BMJ (Clinical research ed.) 334 (7597): 779. doi:10.1136/bmj.39139.716794.55. PMC 1852043. PMID 17383982. - Adhikari, NK; Dellinger, RP; Lundin, S; Payen, D; Vallet, B; Gerlach, H; Park, KJ; Mehta, S; Slutsky, AS; Friedrich, JO (February 2014). "Inhaled nitric oxide does not reduce mortality in patients with acute respiratory distress syndrome regardless of severity: systematic review and meta-analysis". Critical Care Medicine (Systematic Review & Meta-Analysis) 42 (2): 404–12. doi:10.1097/CCM.0b013e3182a27909. PMID 24132038. - Makdisi, G; Wang, IW (July 2015). "Extra Corporeal Membrane Oxygenation (ECMO) review of a lifesaving technology". Journal of thoracic disease 7 (7): E166–76. doi:10.3978/j.issn.2072-1439.2015.07.17 (inactive 2016-07-24). PMC 4522501. PMID 26380745. - Hemmila, MR; Rowe, SA; Boules, TN; Miskulin, J; McGillicuddy, JW; Schuerer, DJ; Haft, JW; Swaniker, F; Arbabi, S; Hirschl, RB; Bartlett, RH (October 2004). "Extracorporeal life support for severe acute respiratory distress syndrome in adults.". Annals of Surgery 240 (4): 595–605; discussion 605–7. doi:10.1097/01.sla.0000141159.90676.2d. PMC 1356461. PMID 15383787. - Brogan, TV; Thiagarajan, RR; Rycus, PT; Bartlett, RH; Bratton, SL (December 2009). "Extracorporeal membrane oxygenation in adults with severe respiratory failure: a multi-center database.". Intensive Care Medicine 35 (12): 2105–14. doi:10.1007/s00134-009-1661-7. PMID 19768656. - Peek, GJ; Mugford, M; Tiruvoipati, R; Wilson, A; Allen, E; Thalanany, MM; Hibbert, CL; Truesdale, A; Clemens, F; Cooper, N; Firmin, RK; Elbourne, D; CESAR trial, collaboration (17 October 2009). "Efficacy and economic assessment of conventional ventilatory support versus extracorporeal membrane oxygenation for severe adult respiratory failure (CESAR): a multicentre randomised controlled trial.". Lancet (London, England) 374 (9698): 1351–63. doi:10.1016/S0140-6736(09)61069-2. PMID 19762075. - editors, Fabio Sangalli, Nicolò Patroniti, Antonio Pesenti, (2014). ECMO-- extracorporeal life support in adults. ISBN 978-88-470-5427-1. - Lewandowski K, Lewandowski M (2006). "Epidemiology of ARDS". Minerva Anestesiol 72 (6): 473–7. PMID 16682918. - Goldman, Lee (2011). Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. p. 635. ISBN 1437727883. - Vlaar AP, Binnekade JM, Prins D, et al. (2010). "Risk factors and outcome of transfusion-related acute lung injury in the critically ill: a nested case-control study". Crit Care Med 38 (3): 771–8. doi:10.1097/CCM.0b013e3181cc4d4b. PMID 20035217. - Moss M, Bucher B, Moore FA, Moore EE, Parsons PE (1996). "The role of chronic alcohol abuse in the development of acute respiratory distress syndrome in adults". JAMA 275 (1): 50–4. doi:10.1001/jama.1996.03530250054027. - Moss M, Guidot DM, Steinberg KP, et al. (2000). "Diabetic patients have a decreased incidence of acute respiratory distress syndrome". Crit Care Med 28 (7): 2187–92. - Koh GC, Vlaar AP, Hofstra JJ, et al. (2012). "In the critically ill patient, diabetes predicts mortality independent of statin therapy but is not associated with acute lung injury: A cohort study". Crit Care Med 40 (6): 1835–1843. doi:10.1097/CCM.0b013e31824e1696. PMC 3379571. PMID 22488007. - The Acute Respiratory Distress Syndrome Network (May 2000). "Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome". N Engl J Med 342 (18): 1301–8. doi:10.1056/NEJM200005043421801. PMID 10793162. - Wiedemann HP, Wheeler AP, Bernard GR, et al. (2006). "; National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS) Clinical Trials Network. Comparison of two fluid-management strategies in acute lung injury". N Engl J Med 354 (24): 2564–2575. doi:10.1056/nejmoa062200. PMID 16714767. - Wheeler AP, Bernard GR, Thompson BT, et al. (2006). "; National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome (ARDS) Clinical Trials Network. Pulmonary-artery versus central venous catheter to guide treatment of acute lung injury". N Engl J Med 354 (21): 2213–2224. doi:10.1056/nejmoa061895. PMID 16714768. - Brun-Buisson C, Minelli C, Bertolini G, et al. (2004). "; ALIVE Study Group. Epidemiology and outcome of acute lung injury in European intensive care units. Results from the ALIVE study". Intensive Care Med. 30 (1): 51–61. doi:10.1007/s00134-003-2022-6. PMID 14569423. - Ashbaugh D, Bigelow D, Petty T, Levine B (1967). "Acute respiratory distress in adults". Lancet 2 (7511): 319–23. doi:10.1016/S0140-6736(67)90168-7. PMID 4143721. - Ware L, Matthay M (2000). "The acute respiratory distress syndrome". N Engl J Med 342 (18): 1334–49. doi:10.1056/NEJM200005043421806. PMID 10793167. - Marino, Paul (2006). The ICU book. Baltimore: Williams & Wilkins. ISBN 978-0781748025. - Martin GS, Moss M, Wheeler AP, Mealer M, Morris JA, Bernard GR (2005). "A randomized, controlled trial of furosemide with or without albumin in hypoproteinemic patients with acute lung injury". Crit. Care Med. 33 (8): 1681–7. doi:10.1097/01.CCM.0000171539.47006.02. PMID 16096441. - Jackson WL, Shorr AF (2005). "Blood transfusion and the development of acute respiratory distress syndrome: more evidence that blood transfusion in the intensive care unit may not be benign". Crit. Care Med. 33 (6): 1420–1. doi:10.1097/01.CCM.0000167073.99222.50. PMID 15942365. - Mortelliti MP, Manning HL (May 2002). "Acute respiratory distress syndrome". Am Fam Physician 65 (9): 1823–30. PMID 12018805. - Metnitz PG, Bartens C, Fischer M, Fridrich P, Steltzer H, Druml W (February 1999). "Antioxidant status in patients with acute respiratory distress syndrome". Intensive Care Med 25 (2): 180–5. doi:10.1007/s001340050813. PMID 10193545. \|The Wikibook Intensive Care Medicine has a page on the topic of: ARDS\|	2	51	7	0	2	0	0.814943	9	9,114
This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages)(Learn how and when to remove this template message) In human–computer interaction, computer accessibility (also known as accessible computing) refers to the accessibility of a computer system to all people, regardless of disability type or severity of impairment. The term "accessibility" is most often used in reference to specialized hardware or software, or a combination of both, designed to enable use of a computer by a person with a disability or impairment. Specific technologies may be referred to as assistive technology. There are many disabilities or impairments that can be a barrier to effective computer use. These impairments, which can be acquired from disease, trauma, or may be congenital, include but are not limited to: - Cognitive impairments (head injury, autism, developmental disabilities), and learning disabilities, such as dyslexia, dyscalculia or ADHD. - Visual impairment such as low-vision, complete or partial blindness, and color blindness. - Hearing-related disabilities including deafness, being hard of hearing, or hyperacusis. - Motor or dexterity impairment such as paralysis, cerebral palsy, dyspraxia, carpal tunnel syndrome and repetitive strain injury. Accessibility is often abbreviated as the numeronym a11y, where the number 11 refers to the number of letters omitted. This parallels the abbreviations of internationalization and localization as i18n and l10n respectively. - 1 Special-needs assessment - 2 Considerations for specific impairments - 3 Software accessibility - 4 Open Accessibility Framework - 5 Standards and Regulations - 6 See also - 7 References - 8 External links People wishing to overcome an impairment in order to use a computer comfortably and productively may require a "special needs assessment" by an assistive technology consultant (such as an occupational therapist, a rehabilitation engineering technologist, or an educational technologist) to help them identify and configure appropriate assistive technologies to meet individual needs. Even those who are unable to leave their own home or who live far from assessment providers may be assessed (and assisted) remotely using remote desktop software and a web cam. For example, the assessor logs on to the client's computer via a broadband Internet connection, observes the users computer skills, and then remotely makes accessibility adjustments to the client's computer where necessary. Considerations for specific impairments Cognitive impairments and illiteracy The biggest challenge in computer accessibility is to make resources accessible to people with cognitive disabilities - particularly those with poor communication and reading skills. As an example, people with learning disabilities may rely on proprietary symbols and thus identify particular products via the product's symbols or icons. Unfortunately copyright laws can limit icon or symbol release to web-based programs and websites by owners who are unwilling to release them to the public. In these situations, an alternative approach for users who want to access public computer based terminals in libraries, ATMs, and information kiosks is for the user to present a token to the computer terminal, such as a smart card, that has configuration information to adjust the computer speed, text size, etcetera to their particular needs. The concept is encompassed by the CEN EN 1332-4 Identification Card Systems - Man-Machine Interface. This development of this standard has been supported in Europe by SNAPI and has been successfully incorporated into the Local Authority Smartcards Standards e-Organisation (LASSeO) specifications. Since computer interfaces often solicit visual input and provide visual feedback, another significant challenge in computer accessibility involves making software usable by people with visual impairments. For individuals with mild to medium vision impairment, it is helpful to use large fonts, high DPI displays, high-contrast themes and icons supplemented with auditory feedback, and screen magnifying software. In the case of severe vision impairment such as blindness, screen reader software that provides feedback via text to speech or a refreshable braille display is a necessary accommodation for interaction with a computer. About 8% of people suffer from some form of color-blindness. The main color combinations that might be confused by people with visual deficiency include red/green and blue/green. However, in a well-designed user interface, color will not be the primary way to distinguish between different pieces of information. Motor and dexterity impairments Some people may not be able to use a conventional input device, such as the mouse or the keyboard, therefore, it is important for software functions to be accessible using both devices. Ideally, software will use a generic input API that permits the use even of highly specialized devices unheard of at the time of software's initial development. Keyboard shortcuts and mouse gestures are ways to achieve this access, as are more specialized solutions, including on-screen software keyboards and alternate input devices (switches, joysticks and trackballs). Speech recognition technology is also a compelling and suitable alternative to conventional keyboard and mouse input as it simply requires a commonly available audio headset. The astrophysicist Stephen Hawking's use of assistive technology is an example of a person with severe motor and physical limitations who uses technology to support activities of daily living. He uses a switch, combined with special software, that allows him to control his wheelchair-mounted computer using his limited and small movement ability. This personalized system allows him to remain mobile, do research, produce his written work. Mr. Hawking also uses augmentative and alternative communication technology to speak and an environmental control device to access equipment independently. A small amount of modern research indicates that utilizing a standard computer mouse device improves fine-motor skills. While sound user interfaces have a secondary role in common desktop computing, these interfaces are usually limited to using system sounds such as feedback. Some software producers take into account people who can't hear due to hearing impairments, silence requirements or lack of sound producing software. System sounds like beeps can be substituted or supplemented with visual notifications and captioned text (akin to closed captioning). Closed captions are a very popular means of relaying information for the Deaf and hearing impaired communities. Accessibility Application Programming Interfaces (APIs) - Java Accessibility and the Java Access Bridge for Java software (being standardized as ISO/IEC TR 13066-6); - Assistive Technology Service Provider Interface (AT-SPI) on UNIX and Linux (being standardized as ISO/IEC PDTR 13066-4 ); - Microsoft Active Accessibility (MSAA) on Microsoft Windows; - IAccessible2 on Microsoft Windows, a competitor of Microsoft UI Automation also replacing MSAA by Free Standards Group (standardized as ISO/IEC 13066-3:2012); - Mac OS X Accessibility; - Microsoft UI Automation on Microsoft Windows, replacing MSAA. Accessibility features in mainstream software Accessibility software can also make input devices easier to access at the user level: - Keyboard shortcuts and MouseKeys allow the user to substitute keyboarding for mouse actions. Macro recorders can greatly extend the range and sophistication of keyboard shortcuts. - Sticky keys allows characters or commands to be typed without having to hold down a modifier key (Shift, Ctrl, Alt) while pressing a second key. Similarly, ClickLock is a Microsoft Windows feature that remembers a mouse button is down so that items can be highlighted or dragged without holding the mouse button down while scrolling. - Customization of mouse or mouse alternatives' responsiveness to movement, double-clicking, and so forth. - ToggleKeys is a feature of Microsoft Windows 95 onwards. A high sound is heard when the CAPS LOCK, SCROLL LOCK, or NUMBER LOCK key is switched on while a low sound is heard when any of those keys is switched off. - Customization of pointer appearance, such as size, color and shape. - Predictive text - Spell checkers and grammar checkers Support for learning disabilities Other approaches that may be particularly relevant to users with a learning disability include: - Cause and effect software - Switch accessible software - Hand–eye coordination skills software - Diagnostic assessment software - Mind mapping software - Study skills software - Symbol-based software - Touch typing software Enabling access to Web content for all users is the concern of the Web accessibility movement, which strives to create accessible websites via conformance to certain design principles. For example, screen readers are of limited use when reading text from websites designed without consideration to accessibility. Sometimes these limitations are due to the differences between spoken and written language and the complexity of text, but it is often caused by poor page design practices. The tendency to indicate semantic meaning using methods that are purely presentational (e.g. larger or smaller font sizes, using different font colors, embedded images, or multimedia to provide information) restricts meaningful access to some users. Therefore, designing sites in accordance with Web accessibility principles helps enable meaningful access for all users. Open Accessibility Framework The Open Accessibility Framework (OAF) provides an outline of the steps that must be in place in order for any computing platform to be considered accessible. These steps are analogous to those necessary to make a physical or built environment accessible. The OAF divides the required steps into two categories: creation and use. The “creation” steps describe the precursors and building blocks required for technology developers to create accessible applications and products. They are as follows: - Define what “accessible” means for the identified use of the platform. It must be clear what is meant by “accessible” as this will differ according to the modality and capabilities of each platform. Accessibility features may include tabbing navigation, theming, and an accessibility API. - Provide accessible stock user interface elements. Pre-built “stock” user interface elements, used by application developers and authoring tools, must be implemented to make use of the accessibility features of a platform. - Provide authoring tools that support accessibility. Application developers and content authors should be encouraged to implement tools that will improve the accessibility features of a platform. Using these tools can support accessible stock user interface elements, prompt for information required to properly implement an accessibility API, and identify accessibility evaluation and repair tools. The “use” steps describe what is necessary in the computing environment in which these accessible applications will run. They are as follows: - Provide platform supports. Computing platforms must properly implement the accessibility features that are specified in their accessibility definition. For example, the accessibility API definitions must be implemented correctly in the program code. - Provide accessible application software. Accessible applications must be available for the platform and they must support the accessibility features of the platform. This may be achieved by simply engaging the accessible stock elements and authoring tools that support accessibility. - Provide assistive technologies. Assistive technologies (e.g. screen readers, screen magnifiers, voice input, adapted keyboards) must actually be available for the platform so that the users can effectively interface with the technology. The following examples show that the OAF can be applied to different types of platforms: desktop operating systems, web applications and the mobile platform. A more complete list can be found in the Open Source Accessibility Repository by the Open Accessibility Everywhere Group (OAEG). - Accessibility APIs include the Assistive Technology Service Provider Interface and UI Automation on the desktop, WAI-ARIA in web applications, and the Blackberry Accessibility API on the Blackberry operating system. - Other APIs are keyboard access and theming in widget libraries like Java Swing for desktop applications, the jQuery UI and Fluid Infusion for Web applications, and the Lightweight User Interface Toolkit (LWUIT) for mobile applications. - Support for accessible development can be effective by using Glade (for the GTK+ toolkit), the DIAS plugin for NetBeans IDE, Xcode IDE for iOS applications. Accessibility inspection tools like Accerciser (for AT-SPI) and support for accessible authoring with the AccessODF plugin for LibreOffice and Apache OpenOffice also fit into this step. - Support for UI Automation on Microsoft Windows, support for ATK and AT-SPI in Linux GNOME, WAI-ARIA support in Firefox, and the MIDP LWUIT mobile runtime (or the MIDP LCDUI mobile runtime) that is available on mobile phones with Java are examples of APIs. - The DAISY player AMIS on the Microsoft Windows desktop and the AEGIS Contact Manager for phones with Java ME are designed for accessibility. - The GNOME Shell Magnifier and Orca on the GNOME desktop, GNOME's ATK (Accessibility Toolkit), the web-based screen reader WebAnywhere, and the alternative text-entry system Dasher for Linux, iOS and Android are examples of assistive technologies. The goal of the listed tools is to embed accessibility into various mainstream technologies. Standards and Regulations ISO 9241-171: Ergonomics of human-system interaction - Guidance on software accessibility Compiled from independent standards experts, this document is the most comprehensive and technical standard for designing accessible features for software, covering all disabilities and all aspects of software. It provides examples of two priority levels ('Required' and 'Recommended') and offers a handy checklist designed to help with recording software testing results. The only trouble is that because of its complexity and technical nature, and with upwards of 150 individual statements, ISO 9241-172 is difficult to interpret and apply. Luckily, not every statement is relevant to every situation, therefore it may be advisable to identify a subset of statements that are tailored to the particular software environment, making the use of this document much more achievable. - AccessApps - a collection of accessibility software which fits on a USB drive - Assistive Technology - Augmentative and alternative communication - Fire Vox - Game accessibility - Section 508 Amendment to the Rehabilitation Act of 1973 - Web accessibility - CEN: Personal identification, electronic signature and cards and their related systems and operations - Structure. - "Draft EN 1332-4 Identification Card Systems - Man-Machine Interface - Part 4 : Coding of user requirements for people with special needs". Tiresias.org. 2009-11-20. Retrieved 2013-07-28. - LASSeO: Feasibility Studies - Final Report. August 2011. - Bohannon, John (December 19, 2013). "Click here to improve your motor skills". Science. Retrieved 23 December 2013. - Oracle: Java Accessibility - Oracle: Java SE Desktop Accessibility (page containing a link to the Java Access Bridge). - ISO: ISO/IEC PRF TR 13066-6: Information technology -- Interoperability with Assistive Technology (AT) -- Part 6: Java accessibility application programming interface (API). - ISO: ISO/IEC PDTR 13066-4: Information Technology - Interoperability with Assistive Technology (AT) -- Part 4: Linux/UNIX graphical environments accessibility API. - ISO: ISO/IEC TR 13066-3:2012: Information technology -- Interoperability with assistive technology (AT) -- Part 3: IAccessible2 accessibility application programming interface (API). - Richard Hodgkinson: 7th Report on International ICT Accessibility Standards Proposed, Being Developed and Recently Published. 3 October 2008. - Richard Hodgkinson: 10th Report on International ICT Accessibility Standards Proposed, Being Developed and Recently Published. 26 June 2009. - Microsoft: Using ClickLock - Microsoft: To turn on ToggleKeys. Windows XP Professional Product Documentation. - Bates, Roger; Jones, Melanie (2003). "Using Computer Software To Develop Switch Skills". 2003 [Technology and Persons with Disabilities] Conference Proceedings. Retrieved 2007-02-08. - Hawes, Paul; Blenkhorn, Paul (2002). "Bridging the Gap between Aspiration and Capability for Aphasic and Brain Injured People". 2002 [Technology and Persons with Disabilities] Conference Proceedings. Retrieved 2007-02-08. - AEGIS Consortium: AEGIS OAF and high-level architecture. Accessed 2013-01-17. - AEGIS Consortium: AEGIS Architecture Definition. Accessed 2013-01-17. - Open Accessibility Everywhere Group (OAEG): Open Source Accessibility Repository. Accessed 2013-01-17. - Research in Motion (RIM): Package net.rim.device.api.ui.accessibility. BlackBerry JDE 6.0.0 API Reference. Accessed 2013-01-17. - Fluid Infusion. Accessed 2013-01-17. - Glade - A User Interface Designer. Accessed 2013-01-17. - DIAS Netbeans IDE plugin & Standalone. Accessed 2013-01-17. - Apple Inc.: Xcode 4. Accessed 2013-01-17. - AccessODF. Accessed 2013-01-17. - Microsoft Developer Network: Accessibility (.NET Framework 4.5). Accessed 2013-01-17. - Microsoft Windows Dev Center: UI Automation (Windows). Accessed 2013-01-17. - GNOME Dev Center: Introducing ATK, AT-SPI, GAIL and GTK+. Accessed 2013-01-17. - Access Mozilla. Accessed 2013-01-17. - Access Firefox: Firefox Accessibility Features. Accessed 2013-01-17. - AEGIS Consortium: LWUIT - Mobile Accessibility. Accessed 2013-01-21. - DAISY Consortium: AMIS: DAISY 2.02 & DAISY 3 Playback Software. Accessed 2013-01-17. - AEGIS Contact Manager. Accessed 2013-01-17. - WebInSight: WebAnywhere: A Screen reader on the go. Accessed 2013-01-17. - Inference Group (University of Cambridge): Mobile Dasher. Accessed 2013-01-17. - Dasher for iOS on iTunes. Accessed 2013-01-17. - Iosif Klironomos, Julio Abascal, Ilse Bierhoff: D3.1 Report with background material needed to support the SDDP-2 Meeting: An Introduction to the Key Issues Relating to Accessible User Interfaces. Accessed 2013-01-17. \|Wikimedia Commons has media related to Computer accessibility.\| - The annual ERCIM Workshop on 'User Interfaces for All' emphasising accessibility - Better Living Through Technology - contains guides on accessibility options and information about specialist assistive hardware and software - HP Accessibility - AbilityNet - provides information on Accessibility, Assistive Technology and Remote Assessment - C4EA Consortium For E-learning Accessibility - W3C Web Accessibility Initiative (WAI) - Accessibility in the Opera web browser - Mozilla Accessibility Project - Open Office Accessibility Project - EU Project GUIDE: Multimodal user interfaces for elderly people with mild impairments	2	17	4	0	0	0	0.296267	4	3,961
- Author: Tomislav Dragovich, MD, PhD; Chief Editor: N Joseph Espat, MD, MS, FACS more... Pancreatic cancer is the fourth leading cause of cancer deaths, being responsible for 7% of all cancer-related deaths in both men and women. Approximately 75% of all pancreatic carcinomas occur within the head or neck of the pancreas, 15-20% occur in the body of the pancreas, and 5-10% occur in the tail. See the image below. Signs and symptoms The initial symptoms of pancreatic cancer are often quite nonspecific and subtle in onset. Patients typically report the gradual onset of nonspecific symptoms such as anorexia, malaise, nausea, fatigue, and midepigastric or back pain. Patients with pancreatic cancer may present with the following signs and symptoms: Significant weight loss: Characteristic feature of pancreatic cancer Midepigastric pain: Common symptom of pancreatic cancer, sometimes with radiation of the pain to the midback or lower-back region Often, unrelenting pain: Nighttime pain often a predominant complaint Onset of diabetes mellitus within the previous year Painless obstructive jaundice: Most characteristic sign of cancer of head of the pancreas Pruritus: Often the patient's most distressing symptom Migratory thrombophlebitis (ie, Trousseau sign) and venous thrombosis: May be the first presentation Palpable gallbladder (ie, Courvoisier sign) Developing, advanced intra-abdominal disease: Presence of ascites, a palpable abdominal mass, hepatomegaly from liver metastases, or splenomegaly from portal vein obstruction Advanced disease: Paraumbilical subcutaneous metastases (or Sister Mary Joseph nodule or nodules) Possible presence of palpable metastatic mass in the rectal pouch (Blumer's shelf) Possible presence of palpable metastatic cervical nodes: Nodes may be palpable behind the medial end of the left clavicle (Virchow's node) and other areas in the cervical region See Clinical Presentation for more detail. Pancreatic cancer is notoriously difficult to diagnose in its early stages. The laboratory findings in patients with pancreatic cancer are usually nonspecific. Patients with advanced pancreatic cancers and weight loss may have general laboratory evidence of malnutrition (eg, low serum albumin or cholesterol level). Potentially useful tests in patients with suspected pancreatic cancer include the following: Hepatobiliary tests: Patients with obstructive jaundice show significant elevations in bilirubin (conjugated and total), ALP, GGT, and, to a lesser extent, AST and ALT Serum amylase and/or lipase levels: Elevated in less than 50% of patients with resectable pancreatic cancers and in only 25% of patients with unresectable tumors Tumor markers such as CA 19-9 antigen and CEA: 75-85% have elevated CA 19-9 levels; 40-45% have elevated CEA levels Imaging studies that aid in the diagnosis of pancreatic cancer include the following: Magnetic resonance imaging Endoscopic retrograde cholangiopancreatography Positron emission tomography scanning See Workup for more detail. See also Pancreatic Adenocarcinoma Imaging: What You Need to Know, a Critical Images slideshow, to help identify which imaging studies to use to identify and evaluate this disease. Surgery is the primary mode of treatment for pancreatic cancer. However, an important role exists for chemotherapy and/or radiation therapy. Curative resection options include the following: Pancreaticoduodenectomy (Whipple Procedure), with/without sparing of the pylorus Antineoplastic agents and combinations of agents used in managing pancreatic carcinoma include the following: Gemcitabine monotherapy: For symptomatic patients with metastatic or locally advanced unresectable disease with poor performance status GTX regimen (gemcitabine, docetaxel and capecitabine) Gemcitabine and albumin-bound paclitaxel FOLFIRINOX (LV5-FU [leucovorin/5-fluorouracil] plus oxaliplatin plus irinotecan): National Comprehensive Cancer Network recommends as first-line treatment for patients with metastatic or locally advanced unresectable disease with good performance status [1, 2] Paclitaxel protein bound 125 mg/m 2 plus gemcitabine 1000 mg/m 2 IV over 30-40 min on Days 1, 8, and 15 of each 28-day cycle [3, 4] Erlotinib plus gemcitabine Capecitabine monotherapy or capecitabine plus erlotinib: May provide second-line therapy benefit in patient's refractory to gemcitabine Adjuvant therapy with gemcitabine is accepted as standard therapy for surgically resected pancreatic cancer. The use of chemotherapy and/or radiation therapy in the neoadjuvant setting has been a source of controversy. The rationale for using neoadjuvant therapy includes the assertions that (1) pancreatic cancer is a systemic disease and should be treated systemically from the start, (2) patients will be able to tolerate the toxic effects of chemotherapy more readily before undergoing major pancreatic resection than after, and (3) the tumor will shrink with neoadjuvant therapy, and the resection will be less cumbersome, leading to an improved overall survival. Palliative therapy may be administered for the following conditions associated with pancreatic cancer: Pain: Pain relief is crucial for patients not undergoing resection for pancreatic cancer; narcotic analgesics should be used early and in adequate dosages Jaundice: Obstructive jaundice warrants palliation if the patient has pruritus or right upper quadrant pain or has developed cholangitis Duodenal obstruction secondary to pancreatic carcinoma: Can be palliated operatively with a gastrojejunostomy or an endoscopic procedure Although pancreatic cancer accounts for only about 3% of all cancers in the United States, it is the fourth leading cause of cancer deaths in both men and women, being responsible for 7% of all cancer-related deaths. The average lifetime risk of developing pancreatic cancer is about 1 in 67. (See Epidemiology.) Pancreatic cancer is notoriously difficult to diagnose in its early stages. At the time of diagnosis, 52% of all patients have distant disease and 26% have regional spread. The relative 1-year survival rate for pancreatic cancer is only 28%, and the overall 5-year survival is 7%. (See Prognosis and Workup.) Types of pancreatic cancer Of all pancreatic cancers, 80% are adenocarcinomas of the ductal epithelium. Only 2% of tumors of the exocrine pancreas are benign. (See Etiology and Histologic Findings.) Less common histologic appearances of exocrine pancreatic cancers include giant cell carcinoma, adenosquamous carcinoma, microglandular adenocarcinoma, mucinous carcinoma, cystadenocarcinoma, papillary cystic carcinoma, acinar cystadenocarcinoma, and acinar cell cystadenocarcinoma. Very rarely, primary connective tissue cancers of the pancreas can occur. The most common of these is primary pancreatic lymphoma. An adenocarcinoma of the pancreas is seen below. (See Histologic Findings.) Typically, pancreatic cancer first metastasizes to regional lymph nodes, then to the liver and, less commonly, to the lungs. It can also directly invade surrounding visceral organs such as the duodenum, stomach, and colon, or it can metastasize to any surface in the abdominal cavity via peritoneal spread. Ascites may result, and this has an ominous prognosis. Pancreatic cancer may spread to the skin as painful nodular metastases. Metastasis to bone is uncommon. Pancreatic cancer rarely spreads to the brain, but it can produce meningeal carcinomatosis. Pancreatic cancers can arise from the exocrine and endocrine portions of the pancreas, but 95% of them develop from the exocrine portion, including the ductal epithelium, acinar cells, connective tissue, and lymphatic tissue. Approximately 75% of all pancreatic carcinomas occur within the head or neck of the pancreas, 15-20% occur in the body of the pancreas, and 5-10% occur in the tail. Estimates indicate that 40% of pancreatic cancer cases are sporadic in nature. Another 30% are related to smoking, and 20% may be associated with dietary factors. Only 5-10% are hereditary in nature. Diabetes mellitus may be associated with a 2-fold increase in the risk of developing pancreatic cancer. Less than 5% of all pancreatic cancers are related to underlying chronic pancreatitis. Alcohol consumption does not appear to be an independent risk factor for pancreatic cancer unless it is associated with chronic pancreatitis. The risk factors for pancreatic cancer are discussed in more detail below. Smoking is the most common environmental risk factor for pancreatic carcinoma. Estimates indicate that smoking accounts for up to 30% of cases of pancreatic cancer. People who smoke have at least a 2-fold greater risk for pancreatic cancer than do nonsmokers. Current smokers with over a 40 pack-year history of smoking may have up to a 5-fold risk greater risk for the disease. Smokeless tobacco also increases the risk of pancreatic cancer. It takes 5-10 years of discontinued smoking to reduce the increased risk of smoking to approximately that of nonsmokers. Obesity and dietary factors In a number of studies, obesity, especially central, has been associated with a higher incidence of pancreatic cancer. For example, Li et al found that being overweight or obese during early adulthood was associated with a greater risk of pancreatic cancer and a younger age of disease onset, while obesity at an older age was associated with lower overall survival. Several other studies have supported a link between early obesity and the risk of pancreatic cancer.[11, 12] The incidence of pancreatic cancer is lower in persons with a diet rich in fresh fruits and vegetables. Fruits and vegetables rich in folate and lycopenes (such as tomatoes) may be especially good at reducing the risk of pancreatic cancer.[13, 14] Consumption of red meat, especially of the processed kinds, is associated with a higher risk of pancreatic cancer. Poultry and dairy product consumption does not increase the risk of this disease. Despite early reports to the contrary, coffee consumption is not associated with an increased risk of pancreatic cancer. Numerous studies have examined the relative risk of pancreatic cancer in persons with diabetes mellitus. A systematic review of 30 studies concluded that patients with diabetes mellitus of at least 5-years' duration have a 2-fold increased risk of developing pancreatic carcinoma. Pancreatic cancer may follow 18-36 months after a diagnosis of diabetes mellitus in elderly patients with no family history of diabetes mellitus. The National Comprehensive Cancer Network (NCCN) acknowledges long-standing diabetes mellitus as a risk factor for pancreatic cancer. The NCCN also notes an association between sudden onset of type II diabetes mellitus in an adult older than 50 years and a new diagnosis of pancreatic cancer, although in those cases the diabetes is thought to be caused by the cancer. Long-standing, chronic pancreatitis is a substantial risk factor for the development of pancreatic cancer. A multicenter study of more than 2000 patients with chronic pancreatitis showed a 26-fold increase in the risk of developing pancreatic cancer. This risk increased linearly with time, with 4% of patients who had chronic pancreatitis for 20 years' duration developing pancreatic cancer. The risk of pancreatic cancer is even higher in patients with hereditary pancreatitis. The mean age of development of pancreatic cancer in these patients is approximately 57 years. The relative risk of pancreatic cancer in hereditary pancreatitis is increased more than 50-fold, and the cumulative risk rate of pancreatic cancer by age 70 years is 40%. This cumulative risk increases to 75% in persons whose family has a paternal inheritance pattern. Chronic pancreatitis from alcohol consumption is also associated a much higher incidence and an earlier age of onset of pancreatic carcinoma. Approximately 5-10% of patients with pancreatic carcinoma have some genetic predisposition to developing the disease. The molecular genetics of pancreatic adenocarcinoma have been well studied.[21, 22, 23] Of these tumors, 80-95% have mutations in the KRAS2 gene; 85-98% have mutations, deletions, or hypermethylation in the CDKN2 gene; 50% have mutations in p53; and about 55% have homozygous deletions or mutations in Smad4. Some of these mutations can also be found in high-risk precursors of pancreatic cancer. For example, in chronic pancreatitis, 30% of patients have detectable mutations in p16 and 10% have K-ras mutations. Families with BRCA-2 mutations, which are associated with a high risk of breast cancer, also have an excess of pancreatic cancer. Assaying pancreatic juice for the genetic mutations associated with pancreatic adenocarcinoma is invasive, but it may be useful for the early diagnosis of the disease. However, this approach is problematic, because genetic mutations in the pancreatic juice may be found in patients with inflammatory pancreatic disease. Certain precursor lesions have been associated with pancreatic tumors arising from the ductal epithelium of the pancreas. The main morphologic form associated with ductal adenocarcinoma of the pancreas is pancreatic intraepithelial neoplasia (PIN). These lesions arise from specific genetic mutations and cellular alterations that contribute to the development of invasive ductal adenocarcinoma. The initial alterations appear to be related to KRAS2 gene mutations and telomere shortening. Thereafter, p16/CDKN2A is inactivated. Finally, the inactivation of TP53 and MAD4/DPC4 occur. These mutations have been correlated with increasing development of dysplasia and thus with the development of ductal carcinoma of the exocrine pancreas. Based on more recent data from sequencing of human tumors, pancreatic cancer is a genetically complex and heterogeneous disease. This is confounded by considerable variability in terms of the genetic malformations and pathways involved between individual tumors. In addition, the long time from early to clinically manifested disease (21.2 y on average) allows for an accumulation of complex genetic changes, which probably explains the fact that it is often resistant to chemotherapy and radiation therapy.[28, 29] The inherited disorders that increase the risk of pancreatic cancer include hereditary pancreatitis, multiple endocrine neoplasia (MEN), hereditary nonpolyposis rectal cancer (HNPCC), familial adenomatous polyposis (FAP) and Gardner syndrome, familial atypical multiple mole melanoma (FAMMM) syndrome, von Hippel-Lindau syndrome (VHL), and germline mutations in the BRCA1 and BRCA2 genes. Hereditary pancreatitis has been associated with a 40% cumulative risk of developing pancreatic cancer at 40%. MEN-1 and VHL are other genetic syndromes associated with pancreatic endocrine tumor development. Patients with MEN-1 develop symptomatic pancreatic endocrine tumors about 50% of the time, and these pancreatic tumors are noted to be the leading cause of disease-specific mortality. Von Hippel-Lindau syndrome has been associated with malignancy in 17% of masses found in the pancreas in people with this syndrome. Syndromes associated with an increased risk of the development of colon cancer, such as HNPCC and FAP (and Gardner syndrome), have also shown an increased correlation with existence of pancreatic cancer, but the statistics have not been impressive. In a cohort study of 1391 patients with FAP, only 4 developed pancreatic adenocarcinoma. No statistics are available to show the incidence of pancreatic cancer in patients with HNPCC. FAMMM has been shown to increase relative risk of developing pancreatic cancer by 13- to 22-fold and the incidence in sporadic cases to be 98%. The above disorders have specific genetic abnormalities associated with the noted increased risk of pancreatic cancer. Pancreatic cancer in hereditary pancreatitis is associated with a mutation in the PRSS1 gene. Pancreatic cancer appearing in FAP and HNPCC has been associated with a mutation in the APC gene and MSH2 and MLH1 genes respectively. FAMMM and pancreatic cancer has been associated with a mutation in CDKN2A. Endocrine tumors of the pancreas associated with VHL are thought to develop by way of the inactivation of the VHL tumor suppressor gene. Germline mutations in BRCA1 and BRCA2 have been shown to moderately increase the risk of developing pancreatic cancer by 2.3- to 3.6-fold, but BRCA2 has been associated more commonly with pancreatic cancer, at an incidence of 7%. Black males in the United States have the highest incidence rate of pancreatic cancer. (See Epidemiology, below.) The reasons for the higher incidence of pancreatic cancer in African Americans are unclear. Certainly, differences in risk factors for pancreatic cancer, such as dietary habits, obesity, and the frequency of cigarette smoking, are recognized among different population groups and may contribute to the higher incidence of this disease among blacks. However, Arnold et al found that excess pancreatic cancer in blacks cannot be attributed to currently known risk factors, suggesting that as-yet undetermined factors play a role in the disease process. One possibility is a difference in the underlying frequency of predisposing genetic mutations for pancreatic cancer. Incidence in the United States The American Cancer Society estimates that in the United States in 2015, about 48,960 new cases of pancreatic cancer (24,840 in men and 24,120 in women) will be diagnosed. The overall incidence rate of pancreatic cancer was stable from 2007 to 2011, after increasing slowly over the previous decade. The incidence rate in men has been stable since 1993. In women, however, the incidence has been increasing by 0.6% per year since 1994. . These trends probably represent the effect of changing smoking rates for men and women. Worldwide, pancreatic cancer ranks 13th in incidence but 8th as a cause of cancer death. Most other countries have incidence rates of 8-12 cases per 100,000 persons per year. In some areas of the world, pancreatic cancer is quite infrequent; for example, the incidence in India is less than 2 cases per 100,000 persons per year. The highest incidence rate of pancreatic cancer is 16.2 cases per 100,000 persons per year, in black males in the United States. The incidence for black females in the United States was 12.4 cases per 100,000 persons per year from 2001 to 2005. For white males in the United States from 2001 to 2005, the incidence was 12.1 cases per 100,000 persons per year, and for white females, the incidence was 9.1 cases per 100,000 persons per year. Native Hawaiian males and men of Korean, Czech, Latvian, and New Zealand Maori ancestry also have high incidence rates: 11 cases per 100,000 persons per year. In the absence of predisposing conditions, such as familial pancreatic cancer and chronic pancreatitis, pancreatic cancer is unusual in persons younger than 45 years. After age 50 years, the frequency of pancreatic cancer increases linearly. The median age at diagnosis is 69 years in whites and 65 years in blacks; some single-institution data reported from large cancer centers suggest that the median age at diagnosis in both sexes has fallen to 63 years of age. Although pancreatic cancer constitutes only about 3% of all cancers in the United States, it is the fourth leading cause of cancer deaths in both men and women, being responsible for 7% of all cancer-related deaths. The death rate from the disease rose from 5 per 100,000 population in 1930 to more than 10 per 100,000 in 2003. The American Cancer Society estimates that in the United States in 2014, about 39,590 people (20,170 men and 19,420 women) will die of pancreatic cancer. Pancreatic carcinoma is unfortunately usually a fatal disease. The collective median survival time for all patients is 4-6 months. The relative 1-year survival rate for patients with pancreatic cancer is only 28%, and the overall 5-year survival rate is 7%, having increased from the 3% rate as calculated between 1975 and 1977. (However, patients with neuroendocrine and cystic neoplasms of the pancreas, such as mucinous cystadenocarcinomas or intraductal papillary mucinous neoplasms [IPMN], have much better survival rates than do patients with pancreatic adenocarcinoma.) A 5-year survival in pancreatic cancer is no guarantee of cure; patients who survive for 5 years after successful surgery may still die of recurrent disease years after the 5-year survival point. The occasional patient with metastatic disease or locally advanced disease who survives beyond 2-3 years may die of complications of local spread, such as bleeding esophageal varices. In patients able to undergo a successful curative resection (about 20% of patients), median survival time ranges from 12-19 months, and the 5-year survival rate is 15-20%. The best predictors of long-term survival after surgery are a tumor diameter of less than 3 cm, no nodal involvement, negative resection margins, and diploid tumor deoxyribonucleic acid (DNA) content. The median survival for patients who undergo successful resection (only 20% of patients) is approximately 12-19 months, with a 5-year survival rate of 15-20%. Smoking is the most significant reversible risk factor for pancreatic cancer. Alcohol consumption does not increase the risk of pancreatic cancer unless it leads to chronic pancreatitis. A multicenter study of more than 2000 patients with chronic pancreatitis showed a 26-fold increase in the risk of developing pancreatic cancer. [Guideline] National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology. Pancreatic Adenocarcinoma, v.2.2015. Available at http://www.nccn.org/professionals/physician_gls/pdf/pancreatic.pdf. Accessed: October 20, 2015. Conroy T, Desseigne F, Ychou M, et al. FOLFIRINOX versus gemcitabine for metastatic pancreatic cancer. N Engl J Med. 2011 May 12. 364(19):1817-25. [Medline]. Von Hoff DD, Arena FP, Chiorean EG, Infante JR, Moore MJ, Seay TE, et al. Randomized phase III study of weekly nab-paclitaxel plus gemcitabine versus gemcitabine alone in patients with metastatic adenocarcinoma of the pancreas (MPACT). J Clin Oncol 30: 2012 (suppl 34; abstr LBA148), Presented January 25, 2013 at the 2013 Gastrointestinal Cancers Symposium, San Francisco, CA. Mulcahy N. FDA Approves Nab-Paclitaxel for Pancreatic Cancer. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/810564. Accessed: September 16, 2013. Kulke MH, Blaszkowsky LS, Ryan DP, Clark JW, Meyerhardt JA, Zhu AX, et al. Capecitabine plus erlotinib in gemcitabine-refractory advanced pancreatic cancer. J Clin Oncol. 2007 Oct 20. 25(30):4787-92. [Medline]. Neuhaus P, Riess H, Post S. CONKO-001:Final results of the randomized, prospective multicenter phase III trial of adjuvant chemotherapy with gemcitabine versus observation in patients with resected pancratic cancer. J Clin Oncol. 2008. 26(15S):204s(abstract LBA4504). American Cancer Society. Pancreatic Cancer. American Cancer Society. Available at http://www.cancer.org/cancer/pancreaticcancer/. Accessed: October 20, 2015. Cancer Facts & Figures 2015. American Cancer Society. Available at http://www.cancer.org/acs/groups/content/@editorial/documents/document/acspc-044552.pdf. Accessed: October 20, 2015. Raimondi S, Maisonneuve P, Lowenfels AB. Epidemiology of pancreatic cancer: an overview. Nat Rev Gastroenterol Hepatol. 2009 Dec. 6(12):699-708. [Medline]. Li D, Morris JS, Liu J, Hassan MM, Day RS, Bondy ML, et al. Body mass index and risk, age of onset, and survival in patients with pancreatic cancer. JAMA. 2009 Jun 24. 301(24):2553-62. [Medline]. [Full Text]. Genkinger JM, Spiegelman D, Anderson KE, et al. A pooled analysis of 14 cohort studies of anthropometric factors and pancreatic cancer risk. Int J Cancer. 2011 Oct 1. 129(7):1708-17. [Medline]. [Full Text]. Urayama KY, Holcatova I, Janout V, et al. Body mass index and body size in early adulthood and risk of pancreatic cancer in a central European multicenter case-control study. Int J Cancer. 2011 Dec 15. 129(12):2875-84. [Medline]. [Full Text]. Nkondjock A, Ghadirian P, Johnson KC, Krewski D. Dietary intake of lycopene is associated with reduced pancreatic cancer risk. J Nutr. 2005 Mar. 135(3):592-7. [Medline]. Risch HA. Etiology of pancreatic cancer, with a hypothesis concerning the role of N-nitroso compounds and excess gastric acidity. J Natl Cancer Inst. 2003 Jul 2. 95(13):948-60. [Medline]. Nöthlings U, Wilkens LR, Murphy SP, Hankin JH, Henderson BE, Kolonel LN. Meat and fat intake as risk factors for pancreatic cancer: the multiethnic cohort study. J Natl Cancer Inst. 2005 Oct 5. 97(19):1458-65. [Medline]. Lin Y, Tamakoshi A, Kawamura T, et al. Risk of pancreatic cancer in relation to alcohol drinking, coffee consumption and medical history: findings from the Japan collaborative cohort study for evaluation of cancer risk. Int J Cancer. 2002 Jun 10. 99(5):742-6. [Medline]. Lowenfels AB, Maisonneuve P, Cavallini G, Ammann RW, Lankisch PG, Andersen JR, et al. Pancreatitis and the risk of pancreatic cancer. International Pancreatitis Study Group. N Engl J Med. 1993 May 20. 328(20):1433-7. [Medline]. Cowgill SM, Muscarella P. The genetics of pancreatic cancer. Am J Surg. 2003 Sep. 186(3):279-86. [Medline]. Whitcomb DC. Genetics and alcohol: a lethal combination in pancreatic disease?. Alcohol Clin Exp Res. 2011 May. 35(5):838-42. [Medline]. Greer JB, Whitcomb DC, Brand RE. Genetic predisposition to pancreatic cancer: a brief review. Am J Gastroenterol. 2007 Nov. 102(11):2564-9. [Medline]. Soto JL, Barbera VM, Saceda M, Carrato A. Molecular biology of exocrine pancreatic cancer. Clin Transl Oncol. May 2006. 8:306-12. [Medline]. Hahn SA, Kern SE. Molecular genetics of exocrine pancreatic neoplasms. Surg Clin North Am. 1995 Oct. 75(5):857-69. [Medline]. Shi C, Daniels JA, Hruban RH. Molecular characterization of pancreatic neoplasms. Adv Anat Pathol. 2008 Jul. 15(4):185-95. [Medline]. Goggins M, Schutte M, Lu J, et al. Germline BRCA2 gene mutations in patients with apparently sporadic pancreatic carcinomas. Cancer Res. 1996 Dec 1. 56(23):5360-4. [Medline]. Yan L, McFaul C, Howes N, Leslie J, Lancaster G, Wong T, et al. Molecular analysis to detect pancreatic ductal adenocarcinoma in high-risk groups. Gastroenterology. June 2005. 128:2124-30. [Medline]. Kojima K, Vickers SM, Adsay NV, et al. Inactivation of Smad4 accelerates Kras(G12D)-mediated pancreatic neoplasia. Cancer Res. 2007 Sep 1. 67(17):8121-30. [Medline]. Yachida S, Jones S, Bozic I, et al. Distant metastasis occurs late during the genetic evolution of pancreatic cancer. Nature. 2010 Oct 28. 467(7319):1114-7. [Medline]. Campbell PJ, Yachida S, Mudie LJ, et al. The patterns and dynamics of genomic instability in metastatic pancreatic cancer. Nature. 2010 Oct 28. 467(7319):1109-13. [Medline]. Kouvaraki MA, Shapiro SE, Cote GJ, Lee JE, Yao JC, Waguespack SG, et al. Management of pancreatic endocrine tumors in multiple endocrine neoplasia type 1. World J Surg. 2006 May. 30(5):643-53. [Medline]. Blansfield JA, Choyke L, Morita SY, Choyke PL, Pingpank JF, Alexander HR, et al. Clinical, genetic and radiographic analysis of 108 patients with von Hippel-Lindau disease (VHL) manifested by pancreatic neuroendocrine neoplasms (PNETs). Surgery. 2007 Dec. 142(6):814-8; discussion 818.e1-2. [Medline]. Groen EJ, Roos A, Muntinghe FL, Enting RH, de Vries J, Kleibeuker JH, et al. Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol. 2008 Sep. 15(9):2439-50. [Medline]. Lynch HT, Fusaro RM, Lynch JF, Brand R. Pancreatic cancer and the FAMMM syndrome. Fam Cancer. 2008. 7(1):103-12. [Medline]. American Cancer Society. Cancer facts and figures for African Americans 2009-2010. Available at http://www.acsevents.org/downloads/STT/cffaa_2009-2010.pdf. Accessed: February 5, 2010. Arnold LD, Patel AV, Yan Y, Jacobs EJ, Thun MJ, Calle EE, et al. Are racial disparities in pancreatic cancer explained by smoking and overweight/obesity?. Cancer Epidemiol Biomarkers Prev. 2009 Sep. 18(9):2397-405. [Medline]. Anderson KE, Mack T, Silverman D. Cancer of the pancreas. Schottenfeld D, Fraumeni JF Jr. Cancer Epidemiology and Prevention. 3rd Ed. New York: Oxford University Press; 2006. Chari ST, Leibson CL, Rabe KG, Ransom J, de Andrade M, Petersen GM. Probability of pancreatic cancer following diabetes: a population-based study. Gastroenterology. Aug 2005. 129:504-11. [Medline]. Turaga KK, Malafa MP, Jacobsen PB, Schell MJ, Sarr MG. Suicide in patients with pancreatic cancer. Cancer. 2011 Feb 1. 117(3):642-7. [Medline]. [Guideline] Locker GY, Hamilton S, Harris J, Jessup JM, Kemeny N, Macdonald JS, et al. ASCO 2006 update of recommendations for the use of tumor markers in gastrointestinal cancer. J Clin Oncol. 2006 Nov 20. 24(33):5313-27. [Medline]. Fujioka S, Misawa T, Okamoto T, Gocho T, Futagawa Y, Ishida Y, et al. Preoperative serum carcinoembryonic antigen and carbohydrate antigen 19-9 levels for the evaluation of curability and resectability in patients with pancreatic adenocarcinoma. J Hepatobiliary Pancreat Surg. 2007. 14(6):539-44. [Medline]. Kang CM, Kim JY, Choi GH, Kim KS, Choi JS, Lee WJ. The use of adjusted preoperative CA 19-9 to predict the recurrence of resectable pancreatic cancer. J Surg Res. 2007 Jun 1. 140(1):31-5. [Medline]. Horton KM, Fishman EK. Multidetector CT angiography of pancreatic carcinoma: part I, evaluation of arterial involvement. AJR Am J Roentgenol. 2002 Apr. 178(4):827-31. [Medline]. Horton KM, Fishman EK. Adenocarcinoma of the pancreas: CT imaging. Radiol Clin North Am. 2002 Dec. 40(6):1263-72. [Medline]. Kauhanen SP, Komar G, Seppänen MP, Dean KI, Minn HR, Kajander SA, et al. A prospective diagnostic accuracy study of 18F-fluorodeoxyglucose positron emission tomography/computed tomography, multidetector row computed tomography, and magnetic resonance imaging in primary diagnosis and staging of pancreatic cancer. Ann Surg. 2009 Dec. 250(6):957-63. [Medline]. Farma JM, Santillan AA, Melis M, Walters J, Belinc D, Chen DT, et al. PET/CT fusion scan enhances CT staging in patients with pancreatic neoplasms. Ann Surg Oncol. 2008 Sep. 15(9):2465-71. [Medline]. Itani KM, Taylor TV, Green LK. Needle biopsy for suspicious lesions of the head of the pancreas: pitfalls and implications for therapy. J Gastrointest Surg. 1997 Jul-Aug. 1(4):337-41. [Medline]. Turner BG, Cizginer S, Agarwal D, Yang J, Pitman MB, Brugge WR. Diagnosis of pancreatic neoplasia with EUS and FNA: a report of accuracy. Gastrointest Endosc. 2010 Jan. 71(1):91-8. [Medline]. Micames C, Jowell PS, White R, Paulson E, Nelson R, Morse M, et al. Lower frequency of peritoneal carcinomatosis in patients with pancreatic cancer diagnosed by EUS-guided FNA vs. percutaneous FNA. Gastrointest Endosc. 2003 Nov. 58(5):690-5. [Medline]. Louden K. New risk factors proposed for pancreatic cancer. Medscape Medical News. September 26, 2013. [Full Text]. [Guideline] National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology. Neuroendocrine Tumors, v.1.2015. Available at http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Accessed: October 21, 2015. Katz MH, Hwang R, Fleming JB, Evans DB. Tumor-node-metastasis staging of pancreatic adenocarcinoma. CA Cancer J Clin. 2008 Mar-Apr. 58(2):111-25. [Medline]. Callery MP, Strasberg SM, Doherty GM, Soper NJ, Norton JA. Staging laparoscopy with laparoscopic ultrasonography: optimizing resectability in hepatobiliary and pancreatic malignancy. J Am Coll Surg. 1997 Jul. 185(1):33-9. [Medline]. Vollmer CM, Drebin JA, Middleton WD, Teefey SA, Linehan DC, Soper NJ. Utility of staging laparoscopy in subsets of peripancreatic and biliary malignancies. Ann Surg. 2002 Jan. 235(1):1-7. [Medline]. Jarnagin WR, Bodniewicz J, Dougherty E, Conlon K, Blumgart LH, Fong Y. A prospective analysis of staging laparoscopy in patients with primary and secondary hepatobiliary malignancies. J Gastrointest Surg. 2000 Jan-Feb. 4(1):34-43. [Medline]. Al-Haddad M, Martin JK, Nguyen J, Pungpapong S, Raimondo M, Woodward T. Vascular resection and reconstruction for pancreatic malignancy: a single center survival study. J Gastrointest Surg. 2007 Sep. 11(9):1168-74. [Medline]. [Guideline] Ducreux M, Cuhna AS, Caramella C, Hollebecque A, Burtin P, Goéré D, et al. Cancer of the pancreas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up†. Ann Oncol. 2015 Sep. 26 Suppl 5:v56-v68. [Medline]. [Full Text]. Rombouts SJ, Vogel JA, van Santvoort HC, van Lienden KP, van Hillegersberg R, Busch OR, et al. Systematic review of innovative ablative therapies for the treatment of locally advanced pancreatic cancer. Br J Surg. 2015 Feb. 102 (3):182-93. [Medline]. Vervenne W, Bennouna J, Humblett Y. A randomized double-blind, placebo (P) controlled, multicenter phase III trial to evaluate the efficacy and safety of adding bevacizumab (B) to erlotinib (E) and gemcitabine (G) in patients (pts) with metastatic pancreatic cancer. J Clin Oncol. 2008. 26(15S):214s(abstract 4507). Loehrer P, Powell M, Cardenes H. A randomized phase III study of gemcitabine in combination with radiation therapy versus gemcitabine alone in patients with localized, unresectable pancreatic cancer:E4201. J Clin Oncol. 2008. 26(15S):214(abstract 4506). Bernhard J, Dietrich D, Scheithauer W, Gerber D, Bodoky G, Ruhstaller T, et al. Clinical benefit and quality of life in patients with advanced pancreatic cancer receiving gemcitabine plus capecitabine versus gemcitabine alone: a randomized multicenter phase III clinical trial--SAKK 44/00-CECOG/PAN.1.3.001. J Clin Oncol. 2008 Aug 1. 26(22):3695-701. [Medline]. Cunningham D, Chau I, Stocken DD, Valle JW, Smith D, Steward W, et al. Phase III randomized comparison of gemcitabine versus gemcitabine plus capecitabine in patients with advanced pancreatic cancer. J Clin Oncol. 2009 Nov 20. 27(33):5513-8. [Medline]. Kalser MH, Ellenberg SS. Pancreatic cancer. Adjuvant combined radiation and chemotherapy following curative resection. Arch Surg. 1985 Aug. 120(8):899-903. [Medline]. Neoptolemos JP, Stocken DD, Friess H, Bassi C, Dunn JA, Hickey H. A randomized trial of chemoradiotherapy and chemotherapy after resection of pancreatic cancer. N Engl J Med. 2004 Mar 18. 350(12):1200-10. [Medline]. Yang R, Cheung MC, Byrne MM, Jin X, Montero AJ, Jones C, et al. Survival effects of adjuvant chemoradiotherapy after resection for pancreatic carcinoma. Arch Surg. 2010 Jan. 145(1):49-56. [Medline]. Oettle H, Post S, Neuhaus P, et al. Adjuvant chemotherapy with gemcitabine vs observation in patients undergoing curative-intent resection of pancreatic cancer: a randomized controlled trial. JAMA. 2007 Jan 17. 297(3):267-77. [Medline]. Mulcahy N. Incredibly Promising' Adjuvant Agent for Pancreatic Cancer. Available at http://www.medscape.com/viewarticle/778018. Accessed: February 20, 2013. Pisters PW, Abbruzzese JL, Janjan NA, Cleary KR, Charnsangavej C, Goswitz MS. Rapid-fractionation preoperative chemoradiation, pancreaticoduodenectomy, and intraoperative radiation therapy for resectable pancreatic adenocarcinoma. J Clin Oncol. 1998 Dec. 16(12):3843-50. [Medline]. Pisters PW, Wolff RA, Janjan NA, Cleary KR, Charnsangavej C, Crane CN. Preoperative paclitaxel and concurrent rapid-fractionation radiation for resectable pancreatic adenocarcinoma: toxicities, histologic response rates, and event-free outcome. J Clin Oncol. 2002 May 15. 20(10):2537-44. [Medline]. Kadera BE, Sunjaya DB, Isacoff WH, Li L, Hines OJ, Tomlinson JS, et al. Locally Advanced Pancreatic Cancer: Association Between Prolonged Preoperative Treatment and Lymph-Node Negativity and Overall Survival. JAMA Surg. 2013 Dec 4. [Medline]. Boggs W. Preop Chemo for Locally Advanced Pancreatic Cancer Associated With 'Excellent' Survival. Medscape [serial online]. Available at http://www.medscape.com/viewarticle/817801. Accessed: December 22, 2013. Loehrer PJ Sr, Feng Y, Cardenes H, et al. Gemcitabine alone versus gemcitabine plus radiotherapy in patients with locally advanced pancreatic cancer: an eastern cooperative oncology group trial. J Clin Oncol. 2011 Nov 1. 29(31):4105-12. [Medline]. McPhee JT, Hill JS, Whalen GF, Zayaruzny M, Litwin DE, Sullivan ME. Perioperative mortality for pancreatectomy: a national perspective. Ann Surg. 2007 Aug. 246(2):246-53. [Medline]. Wente MN, Bassi C, Dervenis C, Fingerhut A, Gouma DJ, Izbicki JR, et al. Delayed gastric emptying (DGE) after pancreatic surgery: a suggested definition by the International Study Group of Pancreatic Surgery (ISGPS). Surgery. 2007 Nov. 142(5):761-8. [Medline]. van der Gaag NA, Rauws EA, van Eijck CH, Bruno MJ, van der Harst E, Kubben FJ, et al. Preoperative biliary drainage for cancer of the head of the pancreas. N Engl J Med. 2010 Jan 14. 362(2):129-37. [Medline]. Limongelli P, Pai M, Bansi D, Thiallinagram A, Tait P, Jackson J. Correlation between preoperative biliary drainage, bile duct contamination, and postoperative outcomes for pancreatic surgery. Surgery. 2007 Sep. 142(3):313-8. [Medline]. Pawlik TM, Gleisner AL, Cameron JL, Winter JM, Assumpcao L, Lillemoe KD. Prognostic relevance of lymph node ratio following pancreaticoduodenectomy for pancreatic cancer. Surgery. 2007 May. 141(5):610-8. [Medline]. House MG, Gonen M, Jarnagin WR, DAngelica M, DeMatteo RP, Fong Y. Prognostic significance of pathologic nodal status in patients with resected pancreatic cancer. J Gastrointest Surg. 2007 Nov. 11(11):1549-55. [Medline]. Gallagher S, Zervos E, Murr M. Distal Pancreatectomy. Von Hoff, Evans, Hruban. Pancreatic Cancer. Sudbury, Mass: Jones and Bartlett; 2005. 20. Muller MW, Friess H, Kleeff J, Dahmen R, Wagner M, Hinz U, et al. Is there still a role for total pancreatectomy?. Ann Surg. 2007 Dec. 246(6):966-74; discussion 974-5. [Medline]. Rothwell PM, Fowkes GR, Belch JF, Ogawa H, Warlow CP, Meade TW. Effect of daily aspirin on long-term risk of death due to cancer: analysis of individual patient data from randomized trials. Lancet. Dec 7/2010; Early online publication. [Full Text]. Bekaii-Saab T, Phelps MA, Li X, et al. Multi-institutional phase II study of selumetinib in patients with metastatic biliary cancers. J Clin Oncol. 2011 Jun 10. 29(17):2357-63. [Medline]. [Full Text]. Asbun HJ, Conlon K, Fernandez-Cruz L, et al. When to perform a pancreatoduodenectomy in the absence of positive histology? A consensus statement by the International Study Group of Pancreatic Surgery (ISGPS). Surgery. 2014 Jan 6. [Epub ahead of print]. Barone JE. When is it OK to do a pancreaticoduodenectomy without a histologic diagnosis of cancer?. Medscape Medical News. January 22, 2014. [Full Text]. Girelli R, Frigerio I, Giardino A, Regi P, Gobbo S, Malleo G, et al. Results of 100 pancreatic radiofrequency ablations in the context of a multimodal strategy for stage III ductal adenocarcinoma. Langenbecks Arch Surg. 2013 Jan. 398(1):63-9. [Medline]. Wang-Gillam A, Li CP, Bodoky G, Dean A, Shan YS, Jameson G, et al. Nanoliposomal irinotecan with fluorouracil and folinic acid in metastatic pancreatic cancer after previous gemcitabine-based therapy (NAPOLI-1): a global, randomised, open-label, phase 3 trial. Lancet. 2015 Nov 29. [Medline].	2	40	0	0	1	0	0.735351	1	10,330
Posted by Anonymous on Saturday, July 7, 2012 at 10:17am. Which of the following code categories should be chosen over codes from other chapters for the same condition? A. Complications of pregnancy C. Blood disorders D. Metabolic and nutritional diseases 2. Pyuria or bacteria in the urine should be coded to A. 790.7. C. 599.0. B. 038.8. D. 112.5. 3. A patient returns to learn the results of an HIV test, which are negative. Which code is listed as the reason for the encounter? A. V65.44 C. 042 B. 795.71 D. V08 4. A patient has a condition wherein the body fails to produce insulin. She requires daily insulin shots for control that seem to stabilize the condition. She isnít experiencing any significant health issues. This condition is coded as A. 250.01. C. 250.02. B. 250.00. D. 250.03. 5. A patient is experiencing diabetic nephropathy with hypertensive renal disease and renal failure. How many codes would be assigned for this patient? A. 1 C. 3 B. 2 D. 4 6. Hypopotassemia is coded as A. 266.5. C. 276.8. B. 244.0. D. 251.2. 7. Conditions that have a decrease in hemoglobin levels in the blood can be coded to Chapter D. Need more information 8. When should acute blood loss anemia following surgery be coded as a complication of the A. Whenever thereís a large amount of blood loss following a surgery B. When the physician states that the large amount of blood loss is due to the surgery and causing the anemia C. When anemia follows surgery and hemoglobin levels are elevated beyond the normal D. Never. Anemia is never considered a complication; instead, itís considered a disease 9. Which of the following should be used as a guideline when coding diabetes as uncontrolled A. Blood glucose levels outside of the normal range as documented in the patientís medical B. Physician documentation stating uncontrolled or controlled C. The need for daily insulin injections D. Any of the above 10. When coding infectious and parasitic diseases, A. a second code is assigned to indicate the causative organism. B. fourth digits or additional codes may indicate the causative organism(s). C. code categories 041�079 as principal, with a fourth digit indicating the causative D. optional E codes are used to indicate the causative organism. - medical coding - YECqRAmzUKZqp, Monday, March 4, 2013 at 8:47pm Happy and excited for new exrecienpes. They look so cute in their outfits. I am the lucky one to have cute and smart grand-daughters, and of course their mother is too! Speaking of smart and cute, Marc and Carson are right up there with the rest of them. What an awesome family!! - medical coding - Anonymous, Monday, March 3, 2014 at 5:51pm Conditions that have a decrease in hemoglobin levels in the blood can be coded to Chapter A. 2. C. 4. B. 3. D. Need more information - medical coding - Anonymous, Thursday, May 8, 2014 at 11:29pm Conditions that have a decrease i hemoglobin levels in the blood can be coded to Chapter A) 2 B) 3 C) 4 D) Need more information Answer This Question More Related Questions - Medical coding - Which of the following would be coded within the HCPCS Level II... - medical coding - what is the corresponding HCPCS Level II code for HCPCS Level I... - Medical Coding 1 - I need help with these last 3 questions. 1. Your asked to ... - medical billing and coding - you are a medical office manager who wants to make ... - college medical coding please help! - Discuss coding for obstetrics, including ... - Medical Coding ICD-9-CM - A 75-year-old male presented to the ambulatory ... - Medical Billing - Hi. I am working on my Final Project for my medical billing ... - Medical Coding - What is the ICD-9 Code for full-thickness defect, skin of right... - Medical coding - Which of the following is true about the ICD-10-CM? A. An ... - medical billing and coding - When using ICD-9-CM codes, there are four main ...	4	8	0	0	0	1	0.871536	1	997
Intellectual disability - Related Links Open Access Articles- Top Results for Intellectual disability Journal of Genetic Syndromes & Gene TherapyA Mosaic Ring Chromosome 21 in a Patient with Mild Intellectual Disability not Evidenced by Array-Cgh Journal of Aging ScienceWe need to Empower Health Prevention of Aged People with Intellectual Disability Journal of Psychiatry: Open AccessSub-specialties in psychiatry in Africa ? Intellectual disability Journal of Psychiatry: Open AccessDementia in intellectual disability: a review of diagnostic challenges A child runs through the finishing line\| Children with intellectual disability or other developmental conditions can compete in the Special Olympics. \|Classification and external resources\| \|Patient UK\|\|Intellectual disability\| Intellectual disability (ID), also called intellectual development disorder (IDD) or general learning disability (UK and Ireland), and formerly known as mental retardation (MR), is a generalized neurodevelopmental disorder characterized by significantly impaired intellectual and adaptive functioning. It is defined by an IQ score below 70 in addition to deficits in two or more adaptive behaviors that affect everyday, general living. Once focused almost entirely on cognition, the definition now includes both a component relating to mental functioning and one relating to individuals' functional skills in their environments. As a result of this focus on the person's abilities in practice, a person with an unusually low IQ may not be considered intellectually disabled. Intellectual disability is subdivided into syndromic intellectual disability, in which intellectual deficits associated with other medical and behavioral signs and symptoms are present, and non-syndromic intellectual disability, in which intellectual deficits appear without other abnormalities. Down syndrome and Fragile X syndrome are examples of syndromic intellectual disabilities. The terms used for this condition are subject to a process called the euphemism treadmill. This means that whatever term is chosen for this condition, it eventually becomes perceived as an insult. The terms mental retardation and mentally retarded were invented in the middle of the 20th century to replace the previous set of terms, which were deemed to have become offensive. By the end of the 20th century, these terms themselves have come to be widely seen as disparaging, politically incorrect, and in need of replacement. The term intellectual disability is now preferred by most advocates and researchers in most English-speaking countries. As of 2013[update], the term "mental retardation" is still used by the World Health Organization in the ICD-10 codes, which have a section titled "Mental Retardation" (codes F70–F79). In the next revision, the ICD-11 is expected to replace the term "mental retardation" with "intellectual disability" or "intellectual developmental disorder," which the DSM-5 already uses. Because of its specificity and lack of confusion with other conditions, the term "mental retardation" is still sometimes used in professional medical settings around the world, such as formal scientific research and health insurance paperwork. - 1 Signs and symptoms - 2 Cause - 3 Diagnosis - 4 Management - 5 Epidemiology - 6 History - 7 Society and culture - 8 Health disparities - 9 See also - 10 References - 11 External links Signs and symptoms The signs and symptoms of intellectual disability are all behavioral. Most people with intellectual disability do not look like they are afflicted with such, especially if the disability is caused by environmental factors such as malnutrition or lead poisoning. The so-called typical appearance ascribed to people with intellectual disability is only present in a minority of cases, all of which are syndromic. Children with intellectual disability may learn to sit up, to crawl, or to walk later than other children, or they may learn to talk later. Both adults and children with intellectual disability may also exhibit some or all of the following characteristics: - Delays in oral language development - Deficits in memory skills - Difficulty learning social rules - Difficulty with problem solving skills - Delays in the development of adaptive behaviors such as self-help or self-care skills - Lack of social inhibitors Children with intellectual disability learn more slowly than a typical child. Children may take longer to learn language, develop social skills, and take care of their personal needs, such as dressing or eating. Learning will take them longer, require more repetition, and skills may need to be adapted to their learning levels. Nevertheless, virtually every child is able to learn, develop and become a participating member of the community. In early childhood, mild intellectual disability (IQ 50–69) may not be obvious, and may not be identified until children begin school. Even when poor academic performance is recognized, it may take expert assessment to distinguish mild intellectual disability from learning disability or emotional/behavioral disorders. People with mild intellectual disability are capable of learning reading and mathematics skills to approximately the level of a typical child aged nine to twelve. They can learn self-care and practical skills, such as cooking or using the local mass transit system. As individuals with intellectual disability reach adulthood, many learn to live independently and maintain gainful employment. Moderate intellectual disability (IQ 35–49) is nearly always apparent within the first years of life. Speech delays are particularly common signs of moderate ID. People with moderate intellectual disability need considerable supports in school, at home, and in the community in order to participate fully. While their academic potential is limited, they can learn simple health and safety skills and to participate in simple activities. As adults they may live with their parents, in a supportive group home, or even semi-independently with significant supportive services to help them, for example, manage their finances. As adults, they may work in a sheltered workshop. People with severe or profound intellectual disability need more intensive support and supervision their entire lives. They may learn some activities of daily living. Some require full-time care by an attendant. Among children, the cause is unknown for one-third to one-half of cases. Down syndrome, velocariofacial syndrome, and fetal alcohol syndrome are the three most common inborn causes. However, doctors have found many other causes. The most common are: - Genetic conditions. Sometimes disability is caused by abnormal genes inherited from parents, errors when genes combine, or other reasons. The most prevalent genetic conditions include Down syndrome, Klinefelter's syndrome, Fragile X syndrome (common among boys), Neurofibromatosis, congenital hypothyroidism, Williams syndrome, Phenylketonuria (PKU), and Prader-Willi syndrome. Other genetic conditions include Phelan-McDermid syndrome (22q13del), Mowat-Wilson syndrome, genetic ciliopathy, and Siderius type X-linked intellectual disability (OMIM 300263) as caused by mutations in the PHF8 gene (OMIM 300560). In the rarest of cases, abnormalities with the X or Y chromosome may also cause disability. 48, XXXX and 49, XXXXX syndrome affect a small number of girls worldwide, while boys may be affected by 47, XYY, 49, XXXXY, or 49, XYYYY. - Problems during pregnancy. Intellectual disability can result when the fetus does not develop properly. For example, there may be a problem with the way the fetus' cells divide as it grows. A pregnant person who drinks alcohol (see fetal alcohol syndrome) or gets an infection like rubella during pregnancy may also have a baby with intellectual disability. - Problems at birth. If a baby has problems during labor and birth, such as not getting enough oxygen, he or she may have developmental disability due to brain damage. - Exposure to certain types of disease or toxins. Diseases like whooping cough, measles, or meningitis can cause intellectual disability if medical care is delayed or inadequate. Exposure to poisons like lead or mercury may also affect mental ability. - Iodine deficiency, affecting approximately 2 billion people worldwide, is the leading preventable cause of intellectual disability in areas of the developing world where iodine deficiency is endemic. Iodine deficiency also causes goiter, an enlargement of the thyroid gland. More common than full-fledged cretinism, as intellectual disability caused by severe iodine deficiency is called, is mild impairment of intelligence. Certain areas of the world due to natural deficiency and governmental inaction are severely affected. India is the most outstanding, with 500 million suffering from deficiency, 54 million from goiter, and 2 million from cretinism. Among other nations affected by iodine deficiency, China and Kazakhstan have instituted widespread iodization programs, whereas, as of 2006, Russia had not. - Malnutrition is a common cause of reduced intelligence in parts of the world affected by famine, such as Ethiopia. - Absence of the arcuate fasciculus. According to the fifth edition of the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV), three criteria must be met for a diagnosis of intellectual disability: deficits in general mental abilities, significant limitations in one or more areas of adaptive behavior across multiple environments (as measured by an adaptive behavior rating scale, i.e. communication, self-help skills, interpersonal skills, and more), and evidence that the limitations became apparent in childhood or adolescence. In general, people with intellectual disability have an IQ below 70, but clinical discretion may be necessary for individuals who have a somewhat higher IQ but severe impairment in adaptive functioning. It is formally diagnosed by an assessment of IQ and adaptive behavior. A third condition requiring onset in childhood is used to distinguish intellectual disability from dementia such as Alzheimer's disease or due to traumatic brain injuries. The first English-language IQ test, the Stanford-Binet Intelligence Scales, was adapted from a test battery designed for school placement by Alfred Binet in France. Lewis Terman adapted Binet's test and promoted it as a test measuring "general intelligence." Terman's test was the first widely used mental test to report scores in "intelligence quotient" form ("mental age" divided by chronological age, multiplied by 100). Current tests are scored in "deviation IQ" form, with a performance level by a test-taker two standard deviations below the median score for the test-taker's age group defined as IQ 70. Until the most recent revision of diagnostic standards, an IQ of 70 or below was a primary factor for intellectual disability diagnosis, and IQ scores were used to categorize degrees of intellectual disability. Since current diagnosis of intellectual disability is not based on IQ scores alone, but must also take into consideration a person's adaptive functioning, the diagnosis is not made rigidly. It encompasses intellectual scores, adaptive functioning scores from an adaptive behavior rating scale based on descriptions of known abilities provided by someone familiar with the person, and also the observations of the assessment examiner who is able to find out directly from the person what he or she can understand, communicate, and such like. IQ assessment must be based on a current test. This enables diagnosis to avoid the pitfall of the Flynn Effect, which is a consequence of changes in population IQ test performance changing IQ test norms over time. Distinction from other disabilities Clinically, intellectual disability is a subtype of cognitive deficit or disabilities affecting intellectual abilities, which is a broader concept and includes intellectual deficits that are too mild to properly qualify as intellectual disability, or too specific (as in specific learning disability), or acquired later in life through acquired brain injuries or neurodegenerative diseases like dementia. Cognitive deficits may appear at any age. Developmental disability is any disability that is due to problems with growth and development. This term encompasses many congenital medical conditions that have no mental or intellectual components, although it, too, is sometimes used as a euphemism for intellectual disability. Limitations in more than one area Adaptive behavior, or adaptive functioning, refers to the skills needed to live independently (or at the minimally acceptable level for age). To assess adaptive behavior, professionals compare the functional abilities of a child to those of other children of similar age. To measure adaptive behavior, professionals use structured interviews, with which they systematically elicit information about persons' functioning in the community from people who know them well. There are many adaptive behavior scales, and accurate assessment of the quality of someone's adaptive behavior requires clinical judgment as well. Certain skills are important to adaptive behavior, such as: - Daily living skills, such as getting dressed, using the bathroom, and feeding oneself - Communication skills, such as understanding what is said and being able to answer - Social skills with peers, family members, spouses, adults, and others By most definitions intellectual disability is more accurately considered a disability rather than a disease. Intellectual disability can be distinguished in many ways from mental illness, such as schizophrenia or depression. Currently, there is no "cure" for an established disability, though with appropriate support and teaching, most individuals can learn to do many things. There are thousands of agencies around the world that provide assistance for people with developmental disabilities. They include state-run, for-profit, and non-profit, privately run agencies. Within one agency there could be departments that include fully staffed residential homes, day rehabilitation programs that approximate schools, workshops wherein people with disabilities can obtain jobs, programs that assist people with developmental disabilities in obtaining jobs in the community, programs that provide support for people with developmental disabilities who have their own apartments, programs that assist them with raising their children, and many more. There are also many agencies and programs for parents of children with developmental disabilities. Beyond that there are specific programs that people with developmental disabilities can take part in wherein they learn basic life skills. These "goals" may take a much longer amount of time for them to accomplish, but the ultimate goal is independence. This may be anything from independence in tooth brushing to an independent residence. People with developmental disabilities learn throughout their lives and can obtain many new skills even late in life with the help of their families, caregivers, clinicians and the people who coordinate the efforts of all of these people. There are four broad areas of intervention that allow for active participation from caregivers, community members, clinicians, and of course, the individual(s) with an intellectual disability. These include psychosocial treatments, behavioral treatments, cognitive-behavioral treatments, and family-oriented strategies Psychosocial treatments are intended primarily for children before and during the preschool years as this is the optimum time for intervention. This early intervention should include encouragement of exploration, mentoring in basic skills, celebration of developmental advances, guided rehearsal and extension of newly acquired skills, protection from harmful displays of disapproval, teasing, or punishment, and exposure to a rich and responsive language environment. A great example of a successful intervention is the Carolina Abecedarian Project that was conducted with over 100 children from low SES families beginning in infancy through pre-school years. Results indicated that by age 2, the children provided the intervention had higher test scores than control group children, and they remained approximately 5 points higher 10 years after the end of the program. By young adulthood, children from the intervention group had better educational attainment, employment opportunities, and fewer behavioral problems than their control-group counterparts. Core components of behavioral treatments include language and social skills acquisition. Typically, one-to-one training is offered in which a therapist uses a shaping procedure in combination with positive reinforcements to help the child pronounce syllables until words are completed. Sometimes involving pictures and visual aids, therapists aim at improving speech capacity so that short sentences about important daily tasks (e.g. bathroom use, eating, etc.) can be effectively communicated by the child. In a similar fashion, older children benefit from this type of training as they learn to sharpen their social skills such as sharing, taking turns, following instruction, and smiling. At the same time, a movement known as social inclusion attempts to increase valuable interactions between children with an intellectual disability and their non-disabled peers. Cognitive-behavioral treatments, a combination of the previous two treatment types, involves a strategical-metastrategical learning technique[clarification needed] that teaches children math, language, and other basic skills pertaining to memory and learning. The first goal of the training is to teach the child to be a strategical thinker through making cognitive connections and plans. Then, the therapist teaches the child to be metastrategical by teaching them to discriminate among different tasks and determine which plan or strategy suits each task. Finally, family-oriented strategies delve into empowering the family with the skill set they need to support and encourage their child or children with an intellectual disability. In general, this includes teaching assertiveness skills or behavior management techniques as well as how to ask for help from neighbors, extended family, or day-care staff. As the child ages, parents are then taught how to approach topics such as housing/residential care, employment, and relationships. The ultimate goal for every intervention or technique is to give the child autonomy and a sense of independence using the acquired skills he/she has. Although there is no specific medication for intellectual disability, many people with developmental disabilities have further medical complications and may be prescribed several medications. For example autistic children with developmental delay may be prescribed antipsychotics or mood stabilizers to help with their behavior. Use of psychotropic medications such as benzodiazepines in people with intellectual disability requires monitoring and vigilance as side effects occur commonly and are often misdiagnosed as behavioral and psychiatric problems. Intellectual disabilities of all kinds have been documented under a variety of names throughout history. Throughout much of human history, society was unkind to those with any type of disability, and people with intellectual disabilities were commonly viewed as burdens on their families. Greek and Roman philosophers, who valued reasoning abilities, disparaged people with intellectual disabilities as barely human. The oldest physiological view of intellectual disability is in the writings of Hippocrates in the late fifth century BCE, who believed that it was caused by an imbalance in the four humors in the brain. Until the Enlightenment in Europe, care and asylum was provided by families and the church (in monasteries and other religious communities), focusing on the provision of basic physical needs such as food, shelter and clothing. Negative stereotypes were prominent in social attitudes of the time. In the 13th century, England declared people with intellectual disabilities to be incapable of making decisions or managing their affairs. Guardianships were created to take over their financial affairs. In the 17th century, Thomas Willis provided the first description of intellectual disabilities as a disease. He believed that it was caused by structural problems in the brain. According to Willis, the anatomical problems could be either an inborn condition or acquired later in life. In the 18th and 19th centuries, housing and care moved away from families and towards an asylum model. People were placed by, or removed from, their families (usually in infancy) and housed in large professional institutions, many of which were self-sufficient through the labor of the residents. Some of these institutions provided a very basic level of education (such as differentiation between colors and basic word recognition and numeracy), but most continued to focus solely on the provision of basic needs of food, clothing, and shelter. Conditions in such institutions varied widely, but the support provided was generally non-individualized, with aberrant behavior and low levels of economic productivity regarded as a burden to society. Individuals of higher wealth were often able to afford higher degrees of care such as home care or private asylums. Heavy tranquilization and assembly line methods of support were the norm, and the medical model of disability prevailed. Services were provided based on the relative ease to the provider, not based on the needs of the individual. A survey taken in 1891 in Cape Town, South Africa shows the distribution between different facilities. Out of 2046 persons surveyed, 1,281 were in private dwellings, 120 in jails, and 645 in asylums, with men representing nearly two thirds of the number surveyed. In situations of scarcity of accommodation, preference was given to white men and black men (whose insanity threatened white society by disrupting employment relations and the tabooed sexual contact with white women). In the late 19th century, in response to Charles Darwin's On the Origin of Species, Francis Galton proposed selective breeding of humans to reduce intellectual disabilities. Early in the 20th century the eugenics movement became popular throughout the world. This led to forced sterilization and prohibition of marriage in most of the developed world and was later used by Adolf Hitler as a rationale for the mass murder of intellectually challenged individuals during the holocaust. Eugenics was later abandoned as an evil violation of human rights, and the practice of forced sterilization and prohibition from marriage was discontinued by most of the developed world by the mid-20th century. Although ancient Roman law had declared people with intellectual disability to be incapable of the deliberate intent to harm that was necessary for a person to commit a crime, during the 1920s, Western society believed they were morally degenerate. Ignoring the prevailing attitude, Civitans adopted service to people with developmental disabilities as a major organizational emphasis in 1952. Their earliest efforts included workshops for special education teachers and daycamps for children with disabilities, all at a time when such training and programs were almost nonexistent. The segregation of people with developmental disabilities was not widely questioned by academics or policy-makers until the 1969 publication of Wolf Wolfensberger's seminal work "The Origin and Nature of Our Institutional Models", drawing on some of the ideas proposed by SG Howe 100 years earlier. This book posited that society characterizes people with disabilities as deviant, sub-human and burdens of charity, resulting in the adoption of that "deviant" role. Wolfensberger argued that this dehumanization, and the segregated institutions that result from it, ignored the potential productive contributions that all people can make to society. He pushed for a shift in policy and practice that recognized the human needs of those with intellectual disability and provided the same basic human rights as for the rest of the population. The publication of this book may be regarded as the first move towards the widespread adoption of the social model of disability in regard to these types of disabilities, and was the impetus for the development of government strategies for desegregation. Successful lawsuits against governments and an increasing awareness of human rights and self-advocacy also contributed to this process, resulting in the passing in the U.S. of the Civil Rights of Institutionalized Persons Act in 1980. From the 1960s to the present, most states have moved towards the elimination of segregated institutions. Normalization and deinstitutionalization are dominant. Along with the work of Wolfensberger and others including Gunnar and Rosemary Dybwad, a number of scandalous revelations around the horrific conditions within state institutions created public outrage that led to change to a more community-based method of providing services. By the mid-1970s, most governments had committed to de-institutionalization, and had started preparing for the wholesale movement of people into the general community, in line with the principles of normalization. In most countries, this was essentially complete by the late 1990s, although the debate over whether or not to close institutions persists in some states, including Massachusetts. In the past, lead poisoning and infectious diseases were significant causes of intellectual disabilities. Some causes of intellectual disability are decreasing, as medical advances, such as vaccination, increase. Other causes are increasing as a proportion of cases, perhaps due to rising maternal age, which is associated with several syndromic forms of intellectual disability. Along with the changes in terminology, and the downward drift in acceptability of the old terms, institutions of all kinds have had to repeatedly change their names. This affects the names of schools, hospitals, societies, government departments, and academic journals. For example, the Midlands Institute of Mental Subnormality became the British Institute of Mental Handicap and is now the British Institute of Learning Disability. This phenomenon is shared with mental health and motor disabilities, and seen to a lesser degree in sensory disabilities. Terms that denote mental deficiency have been subjected to the euphemism treadmill. The several traditional terms that long predate psychiatry are simple forms of abuse in common usage today; they are often encountered in such old documents as books, academic papers, and census forms (for example, the British census of 1901 has a column heading including the terms imbecile and feeble-minded). Negative connotations associated with these numerous terms for intellectual disability reflect society's attitude about the condition. Some elements of society seek neutral medical terms, while others want to use such terms as weapons of abuse. Today, new words like special or challenged are replacing the term retarded. The term developmental delay is popular among caretakers and parents of individuals with intellectual disability because delay suggests that a person is slowly reaching his or her full potential rather than being disabled. Usage has changed over the years and differed from country to country. For example, mental retardation in some contexts covers the whole field but previously applied to what is now the mild MR group. Feeble-minded used to mean mild MR in the UK, and once applied in the US to the whole field. "Borderline intellectual functioning" is not currently defined, but the term may be used to apply to people with IQs in the 70s. People with IQs of 70 to 85 used to be eligible for special consideration in the US public education system on grounds of intellectual disability. - Cretin is the oldest and comes from a dialectal French word for Christian. The implication was that people with significant intellectual or developmental disabilities were "still human" (or "still Christian") and deserved to be treated with basic human dignity. Individuals with the condition were considered to be incapable of sinning, thus "christ-like" in their disposition. This term has not been used in scientific endeavors since the middle of the 20th century and is generally considered a term of abuse. Although cretin is no longer in use, the term cretinism is still used to refer to the mental and physical disability resulting from untreated congenital hypothyroidism. - Amentia has a long history, mostly associated with dementia. The difference between amentia and dementia was originally defined by time of onset. Amentia was the term used to denote an individual who developed deficits in mental functioning early in life, while dementia included individuals who develop mental deficiencies as adults. During the 1890s, amentia meant someone who was born with mental deficiencies. By 1912, ament was a classification lumping "idiots, imbeciles, and feeble minded" individuals in a category separate from a dement classification, in which the onset is later in life. - Idiot indicated the greatest degree of intellectual disability, where the mental age is two years or less, and the person cannot guard himself or herself against common physical dangers. The term was gradually replaced by the term profound mental retardation (which has itself since been replaced by other terms). - Imbecile indicated an intellectual disability less extreme than idiocy and not necessarily inherited. It is now usually subdivided into two categories, known as severe intellectual disability and moderate intellectual disability. - Moron was defined by the American Association for the Study of the Feeble-minded in 1910, following work by Henry H. Goddard, as the term for an adult with a mental age between eight and twelve; mild intellectual disability is now the term for this condition. Alternative definitions of these terms based on IQ were also used. This group was known in UK law from 1911 to 1959/60 as feeble-minded. - Mongolism and Mongoloid idiot were medical terms used to identify someone with Down syndrome, as the doctor who first described the syndrome, John Langdon Down, believed that children with Down syndrome shared facial similarities with Blumenbach's "Mongolian race." The Mongolian People's Republic requested that the medical community cease use of the term as a referent to intellectual disability. Their request was granted in the 1960s, when the World Health Organization agreed that the term should cease being used within the medical community. - In the field of special education, educable (or "educable intellectual disability") refers to ID students with IQs of approximately 50–75 who can progress academically to a late elementary level. Trainable (or "trainable intellectual disability") refers to students whose IQs fall below 50 but who are still capable of learning personal hygiene and other living skills in a sheltered setting, such as a group home. In many areas, these terms have been replaced by use of "moderate" and "severe" intellectual disability. While the names change, the meaning stays roughly the same in practice. - Retarded comes from the Latin retardare, "to make slow, delay, keep back, or hinder," so mental retardation meant the same as mentally delayed. The term was recorded in 1426 as a "fact or action of making slower in movement or time." The first record of retarded in relation to being mentally slow was in 1895. The term mentally retarded was used to replace terms like idiot, moron, and imbecile because retarded was not then a derogatory term. By the 1960s, however, the term had taken on a partially derogatory meaning as well. The noun retard is particularly seen as pejorative; a BBC survey in 2003 ranked it as the most offensive disability-related word, ahead of terms such as spastic (or its abbreviation spaz) and mong. The terms mentally retarded and mental retardation are still fairly common, but currently the Special Olympics, Best Buddies and over 100 other organizations are striving to eliminate their by referring to the word retard and its variants as the "r-word", in an effort to equate it to the word nigger and the associated euphemism "n-word", in everyday conversation. These efforts have resulted in federal legislation, sometimes known as "Rosa's Law", to replace the term mentally retarded with the term intellectual disability in some federal statutes. The term mental retardation was a diagnostic term denoting the group of disconnected categories of mental functioning such as idiot, imbecile, and moron derived from early IQ tests, which acquired pejorative connotations in popular discourse. It acquired negative and shameful connotations over the last few decades due to the use of the words retarded and retard as insults. This may have contributed to its replacement with euphemisms such as mentally challenged or intellectually disabled. While developmental disability includes many other disorders (see below), developmental disability and developmental delay (for people under the age of 18), are generally considered more polite terms than mental retardation. - In North America intellectual disability is subsumed into the broader term developmental disability, which also includes epilepsy, autism, cerebral palsy and other disorders that develop during the developmental period (birth to age 18). Because service provision is tied to the designation 'developmental disability', it is used by many parents, direct support professionals, and physicians. In the United States, however, in school-based settings, the more specific term mental retardation or, more recently (and preferably), intellectual disability, is still typically used, and is one of 13 categories of disability under which children may be identified for special education services under Public Law 108-446. - The phrase intellectual disability is increasingly being used as a synonym for people with significantly below-average cognitive ability. These terms are sometimes used as a means of separating general intellectual limitations from specific, limited deficits as well as indicating that it is not an emotional or psychological disability. Intellectual disability may also refer to the outcome of traumatic brain injury, lead poisoning, or dementing conditions such as Alzheimer's disease. It is not specific to congenital disorders such as Down syndrome. The "American Association on Mental Retardation" continued to use the term mental retardation until 2006. In June 2006 its members voted to change the name of the organization to the "American Association on Intellectual and Developmental Disabilities," rejecting the options to become the AAID or AADD. Part of the rationale for the double name was that many members worked with people with pervasive developmental disorders, most of whom do not have intellectual disability. In the UK, mental handicap had become the common medical term, replacing mental subnormality in Scotland and mental deficiency in England and Wales, until Stephen Dorrell, Secretary of State for Health for the United Kingdom from 1995–97, changed the NHS's designation to learning disability. The new term is not yet widely understood, and is often taken to refer to problems affecting schoolwork (the American usage), which are known in the UK as "learning difficulties." British social workers may use "learning difficulty" to refer to both people with intellectual disability and those with conditions such as dyslexia. In education, "learning difficulties" is applied to a wide range of conditions: "specific learning difficulty" may refer to dyslexia, dyscalculia or developmental coordination disorder, while "moderate learning difficulties", "severe learning difficulties" and "profound learning difficulties" refer to more significant impairments. In England and Wales between 1983 and 2008 the Mental Health Act 1983 defined "mental impairment" and "severe mental impairment" as "...a state of arrested or incomplete development of mind which includes significant/severe impairment of intelligence and social functioning and is associated with abnormally aggressive or seriously irresponsible conduct on the part of the person concerned." As behavior was involved, these were not necessarily permanent conditions: they were defined for the purpose of authorizing detention in hospital or guardianship. The term mental impairment was removed from the Act in November 2008, but the grounds for detention remained. However, English statute law uses mental impairment elsewhere in a less well-defined manner—e.g. to allow exemption from taxes—implying that intellectual disability without any behavioral problems is what is meant. A BBC poll conducted in the United Kingdom came to the conclusion that 'retard' was the most offensive disability-related word. On the reverse side of that, when a contestant on Celebrity Big Brother live used the phrase "walking like a retard", despite complaints from the public and the charity Mencap, the communications regulator Ofcom did not uphold the complaint saying "it was not used in an offensive context [...] and had been used light-heartedly". It was however noted that two previous similar complaints from other shows were upheld. In the past, Australia has used British and American terms interchangeably, including "mental retardation" and "mental handicap". Today, "intellectual disability" is the preferred and more commonly used descriptor. Society and culture People with intellectual disabilities are often not seen as full citizens of society. Person-centered planning and approaches are seen as methods of addressing the continued labeling and exclusion of socially devalued people, such as people with disabilities, encouraging a focus on the person as someone with capacities and gifts as well as support needs. The self-advocacy movement promotes the right of self-determination and self-direction by people with intellectually disabilities, which means allowing them to make decisions about their own lives. Until the middle of the 20th century, people with intellectual disabilities were routinely excluded from public education, or educated away from other typically developing children. Compared to peers who were segregated in special schools, students who are mainstreamed or included in regular classrooms report similar levels of stigma and social self-conception, but more ambitious plans for employment. As adults they may live independently, with family members, or in different types of institutions organized to support people with disabilities. About 8% currently live in an institution or a group home. In the United States, the average lifetime cost of a person with an intellectual disability amounts to $1,014,000 per person, in 2003 US dollars. This is slightly more than the costs associated with cerebral palsy, and double that associated with serious vision or hearing impairments. About 14% is due to increased medical expenses (not including what is normally incurred by the typical person), 10% is due to direct non-medical expenses, such as the excess cost of special education compared to standard schooling, and 76% is indirect costs accounting for reduced productivity and shortened lifespans. Some expenses, such as costs associated with being a family caregiver or living in a group home, were excluded from this calculation. People with intellectual disabilities as a group have higher rates of adverse health conditions such as epilepsy and neurological disorders, gastrointestinal disorders and behavioral/psychiatric problems compared to people without disabilities. Adults also have a higher prevalence of poor social determinants of health, behavioral risk factors, depression, diabetes, poor or fair health status than adults without intellectual disability. - Wilmshurst, Linda (2012). "general+learning+disability" Clinical and Educational Child Psychology an Ecological-Transactional Approach to Understanding Child Problems and Interventions. Hoboken: Wiley. p. 168. ISBN 9781118439982. - Tidy, Colin (25 January 2013). "General Learning Disability". Patient.co.uk. The term general learning disability has now been recommended in the UK to replace terms such as mental handicap or mental retardation. - "Rosa's Law" (PDF). Washington, D.C. : U.S. G.P.O. 2010. Retrieved 13 September 2013. - Ansberry, Clare (20 November 2010). "Erasing a Hurtful Label From the Books". New York: Wall Street Journal. Retrieved 4 December 2010. Decades-long quest by disabilities advocates finally persuades state, federal governments to end official use of 'retarded' - Cummings, Nicholas A.; Rogers H. Wright (2005). "Chapter 1, Psychology's surrender to political correctness". Destructive trends in mental health: the well-intentioned path to harm. New York: Routledge. ISBN 0-415-95086-4. - American Psychiatric Association (2013). Diagnostic and Statistical Manual of Mental Disorders (Fifth ed.). Arlington, VA: American Psychiatric Publishing. ISBN 978-0-89042-555-8. Lay summary (15 July 2013). - Salvador-Carulla L, Reed GM, Vaez-Azizi LM et al. (October 2011). "Intellectual developmental disorders: towards a new name, definition and framework for "mental retardation/intellectual disability" in ICD-11". World Psychiatry 3 (10): 175–180. PMC 3188762. PMID 21991267. - John Cook (5 July 2001). "The "R" Word". Slate Magazine. - Daily DK, Ardinger HH, Holmes GE (February 2000). "Identification and evaluation of mental retardation". Am Fam Physician 61 (4): 1059–67, 1070. PMID 10706158. - Badano, Jose L.; Norimasa Mitsuma; Phil L. Beales; Nicholas Katsanis (September 2006). "The Ciliopathies : An Emerging Class of Human Genetic Disorders". Annual Review of Genomics and Human Genetics 7: 125–148. PMID 16722803. doi:10.1146/annurev.genom.7.080505.115610. Retrieved 2008-06-15. - Siderius LE, Hamel BC, van Bokhoven H et al. (2000). "X-linked mental retardation associated with cleft lip/palate maps to Xp11.3-q21.3". Am. J. Med. Genet. 85 (3): 216–220. PMID 10398231. doi:10.1002/(SICI)1096-8628(19990730)85:3<216::AID-AJMG6>3.0.CO;2-X. - Laumonnier F, Holbert S, Ronce N et al. (2005). "Mutations in PHF8 are associated with X linked mental retardation and cleft lip/cleft palate". J. Med. Genet. 42 (10): 780–786. PMC 1735927. PMID 16199551. doi:10.1136/jmg.2004.029439. - McNeil, Donald G., Jr. (2006-12-16). "In Raising the World's I.Q., the Secret's in the Salt". The New York Times. Retrieved 2009-07-21. - Wines, Michael (2006-12-28). "Malnutrition Is Cheating Its Survivors, and Africa's Future". The New York Times. Retrieved 2009-07-21. - Sundaram, SK; Sivaswamy, L; Makki, MI; Behen, ME; Chugani, HT. (2008). "Absence of arcuate fasciculus in children with global developmental delay of unknown etiology: a diffusion tensor imaging study". J Pediatr 152 (2): 250–5. PMID 18206698. doi:10.1016/j.jpeds.2007.06.037. - Lawyer, Liz (2010-11-26). "Rosa's Law to remove stigmatized language from law books". Ithaca, New York: The Ithaca Journal. Retrieved 2010-12-04. The resolution ... urges a change from the old term to "developmental disability" - Mash, E., & Wolfe, D. (2013). Abnormal child psychology. (5th ed., pp. 308-313). Wadsworth Cengage Learning. - Hodapp, R.M., & Burack, J.A. (2006). Developmental approaches to children with mental retardation: A second generation? In D. Cicchetti & D. J. Cohen (Eds.), Developmental psychopathology, Vol. 3: Risk, disorder, and adaptation (2nd ed., pp. 235-267). Hoboken, NJ: Wiley. - Ramey, S.L., & Ramey, C.T. (1992). Early educational intervention with disadvantaged children—To what effect? Applied and Preventive Psychology, 1, 131-140. - Campbell, F.A., Ramey, C.T., Pungello, E., Sparling, J., & Miller-Johnson, S. (2002). Early childhood education: Young adult outcomes from the Abecedarian Project. Applied Developmental Science, 6, 42-57. - Matson J.L., Matson M.L., Rivet T.T. (2007). Social-skills treatments for children with autism spectrum disorders: an overview. Behavior Modification. 31(5), pp. 682-707. - Van der Schuit M, Segers E, van Balkom H, Verhoeven L. (2011). Early language intervention for children with intellectual disabilities: a neurocognitive perspective. Research in Developmental Disabilities 32(2):705-12. - Kemp, C., & Carter, M. (2002). The social skills and social status of mainstreamed students with intellectual disabilities. Educational Psychology, 22, 391-411. - Siperstein, G.N., & Glick, G.C., & Parker, R. (2009). The social inclusion of children with intellectual disabilities in an out of school recreational setting. Intellectual and Developmental Diasbilities. 47(2), 97-107. - Hay, I., Elias, G., Fielding-Barnsley, R., Homel, R., & Freiberg, K. (2007) Language delays, reading delays and learning difficulties: Interactive elements requiring multidimensional programming. Journal of Learning Disabilities, 40 (5), 400-409 - Bagner, D.M., & Eyberg, S.M. (2007). Parent-child interaction therapy for disruptive behavior in children with mental retardation: A randomized controlled trial. Journal of Clinical Child and Adolescent Psychology, 36, 418-429. - Kalachnik, JE.; Hanzel, TE.; Sevenich, R.; Harder, SR. (Sep 2002). "Benzodiazepine behavioral side effects: review and implications for individuals with mental retardation". Am J Ment Retard 107 (5): 376–410. ISSN 0895-8017. PMID 12186578. doi:10.1352/0895-8017(2002)107<0376:BBSERA>2.0.CO;2. - Wickham, Parnell. Encyclopedia of Children and Childhood in History and Society. Retrieved 8 October 2010. - Roy Porter; David Wright (7 August 2003). The Confinement of the Insane: International Perspectives, 1800-1965. Cambridge University Press. ISBN 978-0-521-80206-2. Retrieved 11 August 2012. - Armbrester, Margaret E. (1992). The Civitan Story. Birmingham, AL: Ebsco Media. pp. 74–75. - Wolf Wolfensberger (January 10, 1969). "The Origin and Nature of Our Institutional Models". Changing Patterns in Residential Services for the Mentally Retarded. President's Committee on Mental Retardation, Washington, D.C. - "The ARC Highlights — Beyond Affliction: Beyond Affliction Document". Disabilitymuseum.org. Retrieved 2010-06-29. - "Christmas in Purgatory & Willowbrook". Arcmass.org. Retrieved 2010-06-29. - "Fernald School Closing and RICCI Class". Arcmass.org. Retrieved 2010-06-29. - "cretin". The American Heritage Dictionary of the English Language, Fourth Edition. Houghton Mifflin Company. 2006. Retrieved 2008-08-04. - Howard-Jones, Norman (1979). "On the diagnostic term "Down's disease"". Medical History 23 (1): 102–04. PMC 1082401. PMID 153994. doi:10.1017/s0025727300051048. - "Worst Word Vote". Ouch. BBC. 2003. Archived from the original on 2007-03-20. Retrieved 2007-08-17. - "SpecialOlympics.org". SpecialOlympics.org. Retrieved 2010-06-29. - "R-Word.org". R-Word.org. 2010-06-18. Retrieved 2010-06-29. - "Frequently Asked Questions on Intellectual Disability". American Association on Intellectual and Developmental Disabilities (AAIDD). Retrieved 12 September 2013. The term intellectual disability covers the same population of individuals who were diagnosed previously with mental retardation in number, kind, level, type, duration of disability, and the need of people with this disability for individualized services and supports. - Chakrabarti S, Fombonne E (2001). "Pervasive developmental disorders in preschool children". JAMA 285 (24): 3093–9. PMID 11427137. doi:10.1001/jama.285.24.3093. - "mencap". Retrieved 2010-12-07. Website of the UK's leading learning disability charity, which uses that term throughout. - "Learning Disabilities: Prevalence". Social Work, Alcohol & Drugs. University of Bedfordshire. Retrieved 2014-10-18. - "Special Educational Needs and Disability: A. Cognition and Learning Needs". teachernet. Archived from the original on 2010-05-01. Retrieved 2010-12-08. - Vickerman, Philip (2009-07-08). "Severe Learning Difficulties". Teacher Training Resource Bank. Retrieved 2014-10-19. Extensive further references. - "Draft Illustrative Code of Practice" (PDF). Retrieved 2007-08-23. - Rohrer, Finlo (2008-09-22). "UK \| Magazine \| The path from cinema to playground". BBC News. Retrieved 2010-06-29. - Beckford, Martin (2010-03-11). "Ofcom says TV channels have 'human right' to broadcast offensive material". Telegraph. Retrieved 2010-06-29. - "Australian Psychological Society : Psychologists and intellectual disability". Psychology.org.au. Retrieved 2010-06-29. - Cooney G, Jahoda A, Gumley A, Knott F (June 2006). "Young people with intellectual disabilities attending mainstream and segregated schooling: perceived stigma, social comparison and future aspirations". J Intellect Disabil Res 50 (Pt 6): 432–44. PMID 16672037. doi:10.1111/j.1365-2788.2006.00789.x. - Centers for Disease Control and Prevention (CDC) (January 2004). "Economic costs associated with mental retardation, cerebral palsy, hearing loss, and vision impairment--United States, 2003". MMWR Morb. Mortal. Wkly. Rep. 53 (3): 57–9. PMID 14749614. - Krahn GL, Fox MH. (2013). "Health disparities of adults with intellectual disabilities: what do we know? What do we do?". Journal of Applied Research in Intellectual Disability 27 (5): 431–446. doi:10.1111/jar.12067. - Haider SI, Ansari Z, Vaughan L, Matters H, Emerson E. (2013). "Health and wellbeing of Victorian adults with intellectual disability compared to the general Victorian population". Research in Developmental Disabilities 34 (11): 4034–4042. PMID 24036484. doi:10.1016/j.ridd.2013.08.017. \|40x40px\|\|Wikimedia Commons has media related to Intellectual disability.\| - FAQ on intellectual disabilities from the US Centers for Disease Control's National Center on Birth Defects and Developmental Disabilities Lua error in Module:Authority_control at line 346: attempt to index field 'wikibase' (a nil value).	2	39	5	0	0	2	0.799234	7	10,309
March 1, 2010 Coding for Multiple Myeloma For The Record Vol. 22 No. 4 P. 28 Multiple myeloma is a plasma cell cancer that involves a type of white blood cell in bone marrow. It results in bone erosion and malfunctioning of the bone marrow and the immune system, which may lead to anemia and infections. Multiple myeloma is classified to ICD-9-CM code 203.0. A fifth digit is required to identify whether the condition is in remission, in relapse, or without mention of having achieved remission. Code V10.79 identifies a patient with a personal history of multiple myeloma. A diagnostic statement of multiple myeloma with bone metastasis is assigned to code 203.00 only. No additional code for the bone metastasis is necessary since bone involvement is a part of the disease process (AHA Coding Clinic for ICD-9-CM, 1989, fourth quarter, page 10). In addition, malignant neoplasms classifiable to categories 200 to 208, Lymphomas and Hematopoietic Tissue Malignancies, and stated as secondary or metastatic remain within this category range and are not coded to category 196 (AHA Coding Clinic for ICD-9-CM, 1985, May-June, page 4). Signs and Symptoms Symptoms may not be present in the early disease stages but may occur as the disease progresses. Some common signs and symptoms include bone pain, usually in the back, pelvis, ribs, and skull; the presence of abnormal proteins (produced by myeloma cells) in blood or urine called monoclonal or M-proteins (If found in urine, may be referred to as Bence Jones proteins.); a high level of calcium in the blood that may cause excessive thirst and urination, mental confusion, constipation, and nausea; anemia that causes fatigue; pathological or nontraumatic bone fractures; repeat infections such as pneumonia, bladder or kidney infections, sinusitis, or shingles; weight loss; and weakness or numbness in the legs. If the physician suspects the patient has multiple myeloma, these tests may be performed: blood tests to measure blood cell counts and levels of calcium, uric acid, creatinine, beta-2 microglobulin, and the percentage of plasma cells; x-rays to show bone lesions; MRI; a CT or PET scan; and/or a bone marrow biopsy to check for myeloma cells. A bone marrow biopsy (41.31) should not be confused with a bone biopsy (77.4x). It is rare to perform both types of biopsies during the same procedure since each one is a diagnostic tool for different illnesses. Before assigning a code for a bone biopsy, review the record for medical intent and a signed consent stating that a bone biopsy will be performed. Also review the pathology report for bone core, cortical bone analysis, or core specimen demineralization. However, the pathology report may indicate that a bone core examination took place when the procedure was actually a bone marrow biopsy. To ensure appropriate procedure code assignment in this situation, it is necessary to review physician intent and reason for the procedure. A patient with multiple myeloma may experience complications, including impaired immunity, osteoporosis, fractures, kidney function problems, or anemia. If a patient with multiple myeloma experiences a pathological or stress fracture, assign the appropriate code for the fracture and for the multiple myeloma. Sequencing will depend on the circumstances of admission. “Although manifestations of the disease are expected, they are not always inherent in the disease process” (AHA Coding Clinic for ICD-9-CM, 1996, first quarter, page 16). Treatment will help relieve pain, control complications, stabilize the condition, and slow the disease progression and may include the following: • medications such as bortezomib (Velcade), thalidomide (Thalomid), and lenalidomide (Revlimid); • chemotherapy (99.25) taken orally or intravenously to kill myeloma cells; • radiation therapy (92.2x) that targets myeloma cells and kills them; and/or • bone marrow transplantation (41.0x) that replaces bone marrow with healthy bone marrow either from a donor (allogeneic) or from the patient (autologous). Coding and sequencing for multiple myeloma are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding. — This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 4,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447.	4	6	0	0	0	1	0.847574	1	1,105
Skip to 0 minutes and 13 secondsSPEAKER: By the end of this presentation, you should be able to describe the World Health Organization's classification of visual impairment. This classification is based on Section H54 of the ICD-10, the International Classification of Diseases. ICD-10 is the standard diagnostic tool for epidemiology, health management, and clinical purposes. It was endorsed by the 43rd World Health Assembly in May 1990, and it came into use in WHO member states in 1994. Visual acuity is an objective, quantitative measure of visual function, and it is one of the first clinical examinations in eye care. It can be carried out in various settings and conditions using a range of standardised charts, distances, and illumination. Skip to 0 minutes and 59 secondsVarious charts are used across the world. The most commonly used are the Snellen chart, Landolt C, E chart, and the LogMAR chart. The characters on the charts, known as optotypes, are arranged in a standardised way to test distance vision. The most recent arrangements is a LogMAR chart. This is based on proportional spacing, geometric progression, Sans-serif letters, and five letters to each line. Visual acuity measurements made using one chart can be converted to other chart measurements. For example, a visually acuity of 6/60 on a Snellen chart is 0.1 on a Decimal scale and 1.0 on a LogMAR scale. In order to understand the epidemiology of visual impairment, we need to use an agreed definition of visual acuity. Skip to 1 minute and 49 secondsThis will allow us to compare studies and also to measure impact. The World Health Organization has agreed on the use of four levels of visual function, as described in the ICD-10. These four levels are mild or no visual impairment, moderate visual impairment, severe visual impairment, and blindness. Each of these four levels has a clear, quantitative measure. Moderate visual impairment and severe visual impairment are grouped together under the term low vision. And when low vision is taken together with blindness, it represents all visual impairment. Skip to 2 minutes and 27 secondsMeasurement of visual acuity is based on distance vision, or visual field assessment. A Snellen or equivalent chart is used at a distance of six metres or 20 feet in a well-illuminated environment. The patient reads across and down the chart as far as they can. Each eye is tested independently and recorded. WHO categories are based on the visual acuity of the better eye in presenting vision. Pinhole is not used when using this method. Presenting visual acuity provides an understanding of refractive need, and is important, particularly for epidemiological surveys and for planning. The WHO's four levels of visual acuity are categorised and measured as follows. Category 0 is mild or no visual impairment. Skip to 3 minutes and 13 secondsIn this category, vision is equal to or better than 6/18 using Snellen chart measurements. Category 1 is moderate vision impairment. In this category, vision is worse than 6/18 but equal to or better than 6/60. Category 2 is severe visual impairment. In this category, vision is worse than 6/60, but equal to or better than 3/60. The final category is Category 3, blindness. In this category, vision is worse than 3/60. Skip to 3 minutes and 45 secondsIt's important to remember that visual status categories are based on visual acuity as measured in the better eye. Blindness is also used if the visual field is less than 10 degrees from the point of fixation. Skip to 3 minutes and 59 secondsIn summary, visual acuity management is an objective measure of visual function, and it is used to categorise and define vision impairment in epidemiological research, public health planning, and screening programmes. Finally, by using the ICD-10 standard definition and categorisation of visual impairment, we are able to compare data and understand the epidemiology of visual impairment. Defining visual impairment In order to understand the epidemiology of visual impairment we need to use an agreed definition of visual acuity. This will allow us to compare studies from different locations and also measure impact. In this step we look at the World Health Organization’s (WHO) classification of visual impairment, which will be used throughout the course. How can this classification be applied to your own work? Visual acuity conversion chart Throughout the course we use the Snellen (metre) measurement of visual acuity. In other contexts Decimal or LogMAR measurement systems are preferred. The chart shown below allows you to convert from one system to another. © London School of Hygiene & Tropical Medicine	2	10	0	0	0	2	0.739086	2	964
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. Kevin T Harley MD Dr. Kevin T Harley, MD is a Doctor primarily located in Orange, CA, with other offices in Abingdon, VA and Fairfax, VA . He has 13 years of experience. His specialties include Nephrology and Internal Medicine. He speaks English. Dr. Kevin T Harley has 3 locations 16000 Johnston Memorial Dr Abingdon, VA 24211 Book Online Now Dr. Kevin T Harley 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. Dr. Kevin T Harley has the following 8 expertise - Chronic Renal Disease - Kidney Biopsy - End Stage Renal Disease (ESRD) - Peritoneal Dialysis - Kidney Disease Showing 1 of 1 Self-verified patient of Dr. Kevin T Harley - Posted on August 14th, 2018 I have been a dialysis patient for nearly 13 years and never have I had better care then from Dr. Harley since he left UCI my health has had such a decline and I have never received again at UCI the care, attention to detail and a man who is confident with his patience. I now am planning my funeral since Dr. Harley left from doctors who clearly can't find that connection to help their parents. Since Doctor Harley I have lost 5o pounds in a wheelchair and can't eat. I was in perfect health with Doctor Harley..I do miss him and I feel my life is at a decline since his departure. I'm happy for all the future patients who have the honor of having him for their doctor :) Thank you Doctor Harley 13 Years Experience New York Medical College Graduated in 2005 Dr. Kevin T Harley Accepts the Following Insurance - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO Blue Cross California - Blue Cross CA PPO Prudent Buyer Small Group - Blue Cross CA Advantage PPO Preferred DirectAccess Plus - Blue Cross CA PPO Prudent Buyer Individual - Blue Cross CA PPO Prudent Buyer Large Group - Blue Cross CA Pathway X PPO Blue Shield California - Blue Shield CA PPO - UHC Choice Plus POS - UHC Options PPO Locations & Directions Dr. Kevin T Harley is similar to the following 4 Doctors near Orange, CA.	2	8	1	0	0	0	0.902486	1	907
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. Beverly A Stoudemire-Howlett MD Dr. Beverly A Stoudemire-Howlett, MD is a Doctor primarily located in Montgomery, AL, with another office in Montgomery, AL. She has 34 years of experience. Her specialties include Cardiovascular Disease and Internal Medicine. Dr. Stoudemire-Howlett is affiliated with Jackson Hospital Physicians Partners. She speaks English. Dr. Beverly A Stoudemire-Howlett has 2 locations 273 Winton M Blount Loop Montgomery, AL 36117 Book Online Now Dr. Beverly A Stoudemire-Howlett 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. Dr. Beverly A Stoudemire-Howlett has the following 12 expertise - Heart Diseases - Heart Failure - Heart Block - High Cholesterol - Heart Attack - Blood Clot - Unstable Angina - Acute Coronary Syndrome (ACS) Showing 4 of 4 Do not go to this doctor. Self-verified patient of Dr. Beverly A Stoudemire-Howlett - Posted on January 17th, 2019 Self-verified patient of Dr. Beverly A Stoudemire-Howlett - Posted on August 25th, 2017 I met Dr Stoudemire when my father had to have a heart cath. She quickly amazed me by her mannerism, bedside manners, and friendliness. She was very thorough and straight forward (no chaser :) The small talk made it easy to talk to her and yet her seriousness allowed me to be comfortable with her providing care to my father and trusting her expertise. Self-verified patient of Dr. Beverly A Stoudemire-Howlett - Posted on October 28th, 2014 Self-verified patient of Dr. Beverly A Stoudemire-Howlett - Posted on March 16th, 2013 This Doctor truly cares about her patients and takes the time to discuss all problems and explain the procedures she suggest. She has the most patience with the elderly I have seen in a doctor in some time. I would recommend her to anybody. She has treated both my mother and myself with dignity and real concern. Castle Connolly 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™. On-Time Doctor Award (2017, 2018) 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. Patients' Choice Award (2018, 2014, 2017) 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. Compassionate Doctor Recognition (2014, 2017, 2018) 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. Dr. Stoudemire-Howlett is affiliated (can practice and admit patients) with the following hospital(s). 34 Years Experience University Of South Alabama College Of Medicine Graduated in 1984 University Of South Alabama Health System Dr. Beverly A Stoudemire-Howlett Accepts the Following Insurance - Aetna Choice POS II - Aetna Signature Administrators PPO - BCBS AL PPO BCBS Blue Card - BCBS Blue Card PPO - CIGNA Open Access Plus - CIGNA PPO - First Health PPO - Humana Choice POS - Humana ChoiceCare Network PPO - UHC Choice Plus POS - UHC Navigate HMO - UHC Navigate POS - UHC Options PPO Locations & Directions Take a minute to learn about Dr. Beverly A Stoudemire-Howlett, MD - Cardiovascular Disease in Montgomery, AL, in this video. Dr. Beverly A Stoudemire-Howlett is similar to the following 4 Doctors near Montgomery, AL.	2	8	1	0	0	0	0.944153	1	1,296
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. - 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. Markian R Bochan MD Dr. Markian R Bochan, MD is a Doctor primarily located in Carmel, IN. He has 21 years of experience. His specialties include Infectious Disease and Internal Medicine. Dr. Bochan is affiliated with St Vincent Heart Center Of Indiana. He speaks English. Book Online Now Dr. Markian R Bochan 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.ttcqeayetrwczwezdueawsrsbdyr Dr. Markian R Bochan has the following 7 expertise - Human Immunodeficiency Virus (HIV/AIDS) - AIDS/HIV (Acquired Immunodeficiency Syndrome) - Hepatitis C Showing 2 of 2 Self-verified patient of Dr. Markian R Bochan - Posted on September 21st, 2014 Dr. Bochan is an excellent diagnostician and very professional. He was able to see me on short notice and was very straight forward concerning diagnosis and treatment. He came highly recommended by the medical personnel that were treating my symptoms. If one is seeking a professional in Infectious Disease, I would highly recommend him without any hesitation. Self-verified patient of Dr. Markian R Bochan - Posted on April 1st, 2011 I've been recieving treatment for HIV since 1995 and have had experience with 6 HIV Specialists and Dr. Bochan has been the most excellent physican I've ever had. I feel blessed to be in his care. If you are searching for a Infectious Disease doctor, this is the man and the practice to go to. Patients' Choice Award (2013) 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. Compassionate Doctor Recognition (2014) 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. Castle Connolly 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. Bochan is affiliated (can practice and admit patients) with the following hospital(s). 21 Years Experience Indiana University School Of Medicine Graduated in 1997 Vanderbilt University Medical Center Dr. Markian R Bochan Accepts the Following Insurance - Aetna Choice POS II - Aetna HMO - Anthem Blue Access PPO - Anthem Blue Preferred HMO - Anthem IN Pathway X Bronze Direct CACA HIX BCBS Blue Card - BCBS Blue Card PPO - BCBS IL PPO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO Encore Health Network - Encore Health Network - Humana Choice POS - Humana ChoiceCare Network PPO - Multiplan PPO - PHCS PPO PHP Northern IN - PHP Select with Encircle SIHO Insurance Services - SIHO Network - Sagamore Plus - UHC Choice Plus POS - UHC Navigate HMO - UHC Navigate POS - UHC Options PPO Locations & DirectionsINFECTIOUS DISEASE OF INDIANA, 11455 N Meridian St Ste 200, Carmel, IN Dr. Markian R Bochan is similar to the following 4 Doctors near Carmel, IN.	2	8	1	0	0	0	0.84014	1	1,250
Age-related macular degeneration is a general term for any type of acquired maculopahy that affects people over 50-years-old. The most common type of age-related macular degeneration (AMD) is an atrophic form of the disease known as dry age-related macular degeneration. Dry age-related macular degeneration (ARMD) is characterized by the following clinical features: - Gradual breakdown of retinal pigment epithelial cells in the macula - Gradual blurring of central vision - Appearance, enlargement or coalescense of retinal drusen in the macula - In advanced stages, development or progression of geographic atrophy - A 72-year-old white woman presented with a chief complaint of decreased vision - Case history described a subjective decrease in vision over the past year - The patient had not been examined in two years and wanted to know if new eyeglasses would help to improve her vision Gradual atrophy of retinal pigment epithelial cells with resultant accumulation of metabolic debris and loss of photoreceptor function is the main cause of vision loss in dry age-related macular degeneration. Progression of atrophy like that seen in this patient is the culmination of prolonged, progressive changes in the retina. The most advanced presentation of this progressive atrophy can result in total loss of central vision known as geographic atrophy. History of Present Illness - Associated signs and symptoms: none - Location: vision is worse in the left eye - Duration: past one year - Quality: n/a - Context: decreased vision is more noticeable when reading - Severity: mild decrease in vision - Timing: vision seems to be getting worse over time - Modifiers: none Review of Systems The patient reported that she was in good health and taking no medications. Past, Family and Social History Uncorrected Distance Visual Acuity - 20/20 in the right eye - 20/40 in the left eye Normal Examination Findings - Mental status - General medical observation - Gross visual fields - Basic sensorimotor examination - External examination - Adnexal examination - External ocular examination with biomicroscopy Intraocular Pressure Measurements - 15 mm Hg in the right eye - 16 mm Hg in the left eye - Normal fundus appearance in the right eye - Focal geographic atrophy in the left macula The clinical diagnosis is a determination based on the knowledge obtained from the patient’s medical history and from the results of the eye examination alone, without the benefit of diagnostic tests or procedures. The patient’s clinical diagnosis is dry age-related macular degeneration based on the following clinical findings: - Focal atrophy of retinal pigment cells in the left macula - Decreased visual acuity in the left eye To gather the information required to treat macular degeneration, a diagnostic and treatment program is initiated. - Determination of different types of diagnoses - Selection of one or more treatment options Several macular diseases have clinical signs that mimic the appearance of dry, age-related macular degeneration. - Drusen usually outside macular region - Drusen usually form circular pattern around inner posterior pole - Rarely affects vision - Only in high axial myopia - Drusen rarely present - Usually has associated peripheral retinal changes - Variable presentation of chorioretinal atrophy that may involve macula - Associated inflammatory signs (vitreous cells) Most patients with dry macular degeneration do not experience significant vision loss (90% less than 20/50). ARMD typically has an asymmetric presentation with the fellow eye showing clinical changes about one-third of the time within two years of the intial presentation in the first eye. There are multiple risk factors for ARMD but the most important are age, history of smoking, family history, poor vascular health, obesity, and nutritional factors. While the risk of progression to the most severe form of the disease (wet or exudative macular degeneration) is very low, monitoring for potential conversion to the wet form is the highest clinical concern. Ordering Diagnostic Tests The following diagnostic tests can help determine the severity of dry age-related macular degeneration and help monitor progression. - 10-2 threshold visual field examination - Retinal laser scan with spectral-domain OCT - Extended color vision examination - Dark adaptation testing Some authorities also recommend additional testing including genetic testing and macular pigment density testing, both discussed later. - Measuring visual acuity is a method of evaluating functional vision loss - Age-related macular degeneration can produce central visual field defects which result in a loss of visual acuity - There was no improvement in the left eye’s visual acuity after a subjective refraction was performed or by pinhole testing Retinal Laser Scan — Macula - Measuring the thickness of the macula along with a detailed analysis of retinal structure provides an overall assessment of retinal health - The procedure can be accomplished by using the Cirrus OCT manufactured by Carl Zeiss Meditec Visual Field Examination Automated threshold perimeters measure the visual field by plotting the threshold luminance value of the patient in various locations in the visual field. The luminance of the light stimulus is represented by non-specific units of measurement called decibels (dB). Clinically significant macular degeneration can produce a central scotoma in the visual field — even subtle macular degeneration can cause a decrease in the threshold values in the central points that loosely correlate to an expected degree of Snellen acuity. - 32+dB — expect normal Snellen acuity - High teens to low thirties — one or two lines loss - Mid to high teens — three to four lines loss - Low to mid teens — five to eight lines loss Automated threshold perimeters characterize specific parameters of the overall visual field status by the use of numbers called Global Indices. Two of the indices, Mean Deviation (MD) and Pattern Standard Deviation (PSD) express the raw data generated by the instrument. The Pattern Standard Deviation is a measure of focal loss of visual field taking into account any generalized depression in the hill of vision. A visual field defect can be classified as mild, moderate, or advanced based upon an abnormal Mean Deviation. - Mild visual field defect = 0 through -5.99 dB - Moderate visual field defect = -6.00 dB through -11.99 dB - Advanced visual field defect = anything above -12.00 dB - No loss of retinal sensitivity - Mild loss of retinal sensitivity - Central scotoma at the point of fixation superiorly - Paracentral scotomas temporally - Clinically significant asymmetry - Visual field test results correlate with OCT tests, visual acuity and the clinical examination The results of the examination revealed central and paracentral scotomas in the left eye. These visual field defects are seen in macular degeneration and other retinal lesions. Mid-to-high threshold values would correspond to the acuity loss in the 20/40 Snellen range. Because the central scotoma in the left eye agrees with measurable, observable structural damage to the left eye’s macula, the clinical diagnosis of age-related macular degeneration is confirmed. While there is little doubt that genetics play a significant role in the pathogenesis of ARMD, current testing is still suspect for clinically significant sensitivity and specificity. At best, they may help indicate which patients who already have the disease may progress more rapidly. No currently available test can accurately predict who might develop ARMD. Macular Pigment Density Tests Once again, there is little doubt of the importance carotenoids play in maintaining macular health. Exactly how they relate to ARMD is still the subject of debate. Of more concern is the lack of standards in evaluating macular pigment density, lack of knowledge regarding what is normal or abnormal in the individual patient presentation and how the measurement can or does relate the rate of vision decline. As explained in the summary on MPOD tests in Archives of Clinical and Experimental Ophthalmology 2011, clinicians should exercise caution in considering MPOD tests as an accurate predictor of ARMD onset or progression. All of the diagnostic test results confirmed the initial diagnosis of subclinical macular degeneration in the right eye and mild-to-moderate macular degeneration in the left eye. According to Current Procedural Terminology, when eye doctors perform ophthalmological examinations, the complexity of medical decision-making is not separated from the examining techniques used. As a guideline to assist eye doctors in enhancing their medical decision-making skills, consider that the complexity of medical decision-making involves three components. The first component concerns the number of possible diagnoses and treatment options that must be considered. Determination of the diagnosis of macular degeneration involves the determination of structural damage to the retina with associated functional loss of vision. The second component concerns the amount and complexity of medical records and diagnostic tests that have to be obtained, reviewed and analyzed. In addition to an eye examination, this visit required the review and analysis of a subjective refraction, a retinal laser scan, and a threshold visual field examination. Third, the complexity of medical decision-making is affected by the risk of significant complications and/or morbidity associated with macular degeneration and the risks involved in any treatment options. This patient’s retinal condition was classified as a significant problem where the risk of significant central vision loss without treatment was possible. In addition, the treatment plan will involve continued monitoring of her retinal condition. Dry macular degeneration is generally considered to be an irreversible process, even if detected early. Multiple studies, starting with the 1991 AREDS study, have shown variable benefit in the role of antioxidant therapy through macro-nutrient support. All the studies have random, somewhat arbitrary, and differing analysis of available nutrient supplements with highly variable claims of benefit in slowing progression of the disease. While the results could be considered somewhat less than exciting, nutritional support can still be considered the frontline therapy for at risk or confirmed ARMD patients. At this time, the most positive results support the use of the carotenoids lutein and zeazanthine as well as omega-3 fatty acids. Patients with almost any stage of ARMD should be counseled regarding home monitoring of their disease. At a minimum, this should include paying close attention to the quality of their detailed vision. Amsler grids have been advocated for years. While very insensitive in detecting change, to the point of many authorities doubting their clinical value, they may help a small percentage of patients better detect qualitative changes in their vision. There is no local (retinal) treatment for early, dry age-related macular degeneration so no referral to a retinal specialist is warranted. Most importantly, those at risk or diagnosed with ARMD should be counseled regarding the deleterious effects of smoking and obesity. The patient was counseled regarding the nature of her disease and given an Amsler grid for home monitoring. It was recommended she begin nutritional supplementation in the the form of lutein 30mg/day, zeazanthine 30mg/day, and omega-3 supplements containing at least 2000mg/day of EPA/DHA. Next visit scheduled for 6 months. This case demonstrates several key aspects of monitoring the current knowledge base in ocular disease. It is important to accurately follow patients with ARMD with the greatest concern in watching for conversion to the more serious wet or exudative form of the disease. While accurate detection of ARMD is possible with normal ophthalmoscopic examination, additional diagnostic testing can be very beneficial in confirming the diagnosis and monitoring for disease progression. As the changes with advancing disease are often suble, progression is best monitored by SD OCT technology with RPE change analysis and or autofluorescence capability. Although sometimes beneficial, visual field testing and Amsler grid monitoring are subject to extremely poor sensitivity in detecting change. Treatment of dry ARMD is at this time limited to lifestyle changes and supplementation with antioxidants. An evaluation of the complete visual system was performed. - Perform the eye examination that is medically necessary - Provide the diagnostic tests or services that are medically necessary - Properly document the services provided - Code from the documentation - Report the services to the payor Physicians Quality Reporting System PQRS Measure 14: Age-Related Macular Degeneration: Dilated Macular Examination – CPT code 2019F. This measure applies to patients 50 years and older diagnosed with age-related macular degenertion. CPT code 2019F is reported when these patients receive a dilated macular examination. The measure should be reported on the date of the dilated examination and on all subsequent eye examinations during the next 12 months – even if a dilated macular examination was not performed on the follow-up examinations. Remember that you may be required to report this measure more than once since the reporting period covers 12 months. PQRS Measure 40: Age-Related Macular Degeneration: Counseling on Antioxidant Supplement – CPT code 4177F This measure applies to patients 50 years and older diagnosed with age-related macular degeneration. CPT code 4177F is reported when these patients receive counseling on antioxidant supplementation (e.g., per AREDS recommendations). The measure should be reported on the date of the initial diagnosis and on all subsequent eye examinations during the next twelve months – even if counseling was not performed on the follow-up examinations. Remember that you may be required to report this measure more than once since the reporting period covers 12 months. PQRS Measure 226: Patient screened for tobacco use and identified as a non-user of tobacco – CPT code 1036F. This measure applies to patients 18 years and older. The measure should be reported on the day of the examination and can be used with any diagnosis code. PQRS Measure 130: Current Medications with Name, Dosage, Frequency and Route Documented – CPT code G8427. Multiple Procedure Payment Reduction Effective January 1, 2013, there is a small reduction in payment from Medicare if certain multiple procedures are billed on the same day. The fee for the technical component of the diagnostic test for the second and subsequent tests will be reduced by 20%. The second diagnostic test and subsequent tests should be reported with a -51 modifier. Professional services such as gonioscopy, extended ophthalmoscopy and provocative glaucoma testing are excluded from this policy. Visual evoked potential testing is excluded from this policy. This modifier is used to identify the secondary procedure or when multiple procedures are performed on the same day by the same provider. List the major primary procedure first and append the modifier to the subsequent procedure. The primary procedure is the one with the highest dollar value. \|Diagnosis Code\|\|Procedure Code\|\|Modifier\|\|Quantity\|\|Payor\|\|Amount Allowed\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|92014 - Medical eye examination\|\|1\|\|Medicare\|\|127.36\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|92083 - Visual field examination\|\|1\|\|Medicare\|\|58.45\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|92134 - Retinal laser scan\|\|51\|\|1\|\|Medicare\|\|42.19\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|2019F - Dilated macular examination\|\|Medicare\|\|0.00\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|4177F - Counseling on antioxidant supplement\|\|Medicare\|\|0.00\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|1036F - Patient screened for tobacco use\|\|Medicare\|\|0.00\| \|H35.31 - Nonexudative senile macular degeneration of retina\|\|G8427 - Current medications documented\|\|Medicare\|\|0.00\|	2	9	1	0	3	1	0.625004	5	3,414
Lung cancer staging In medicine, lung cancer staging is the assessment of the extent to which a lung cancer has spread from its original source. As with most cancers, staging is an important determinant of treatment and prognosis. In general, more advanced stages of cancer are less amenable to treatment and have a worse prognosis. The initial evaluation of non-small cell lung cancer staging uses the TNM classification. This is based on the size of the primary tumor, lymph node involvement, and distant metastasis. After this, using the TNM descriptors, a group is assigned, ranging from occult cancer, through stage 0, IA (one-A), IB, IIA, IIB, IIIA, IIIB to IV (four). This stage group assists with the choice of treatment and estimate of prognosis. There are several methods by which this assessment is made. They are broadly classified into non-invasive techniques, which generally involve medical imaging of the lungs such as computer tomography (CT) scans and PET scans, and invasive techniques such as biopsy and surgery. Invasive techniques provide additional information because tissue samples can be seen microscopically to confirm presence of cancer cells (as opposed to enlargement of tissues due to other causes such as infection) and to determine the type of lung cancer and its grade. Staging may also be categorized as either clinical or as pathological/surgical staging. Clinical staging is performed prior to definitive surgery. It is based on the results of imaging studies (such as CT scans ) and biopsy results (i.e. clinical staging does include the results of biopsy, an "invasive technique.") Pathological staging is also called surgical staging and reflects not only the results of non-surgical biopsy, but is evaluated either intra- or post-operatively and is based on the combined results of surgical and clinical findings, including surgical sampling of thoracic lymph nodes. - 1 Lung cancer - 2 Pattern of progression - 3 Staging - 4 Staging modalities - 5 References - 6 External links Lung cancer is responsible for 1.3 million deaths worldwide annually, and is the most common cause of cancer-related death in men and the second most common in women. The most common cause of lung cancer is long-term exposure to tobacco smoke. Lung cancer in non-smokers, who account for approximately 15% of cases, is often attributed to a combination of genetic factors, radon gas, asbestos, and air pollution. The main types of lung cancer are non-small cell lung carcinoma and small cell lung carcinoma, the two being distinguished histologically as well as by how they are treated; non-small cell lung carcinoma is primarily treated with surgery if feasible, while small cell lung carcinoma is more frequently treated with chemotherapy and radiation. The diagnosis of lung cancer is based on chest radiograph and computer tomography (CT) scans, and is confirmed by biopsy. Biopsy is usually performed via bronchoscopy or CT-guided biopsy. Treatment and prognosis depend upon the histological type of cancer, the stage, and the patient's performance status. Treatments include surgery, chemotherapy, and radiotherapy. Pattern of progression Lung cancer can start in various portions of the lung. From there it spreads in fairly predictable pattern. Typically, if lung cancer spreads, it first goes to close-by lymph nodes, followed by lymph nodes further away located between the lungs in a space called the mediastinum. In the mediastinum, the lung cancer tends at first to stay on the side where the original tumor started. Once it crosses the mediastinal midline, it denotes more advanced, surgically unresectable disease. Lung cancer can also spread to distant organs, for example, the liver or adrenal glands, which constitutes the most advanced stage of the disease called stage IV. Staging is the process of determining how much cancer there is in the body and where it is located. Staging of lung cancer is of paramount importance as treatment choices are often highly complex, even for physicians with much experience in the field, and the options largely depend on the stage of the disease. The underlying purpose is to describe the extent or severity of an individual's cancer, and to bring together cancers that have similar prognosis and treatment. Staging information which is obtained prior to surgery, for example by x-rays and endoscopic ultrasound, is called clinical staging and staging by surgery is known as pathological staging. Clinical staging is done by a combination of imaging and sampling (biopsies), or stated differently, non-invasive (radiological) and invasive (biopsy) methods. Pathologic staging is more accurate than clinical staging, but clinical staging is the first and sometimes the only staging type. For example, if clinical staging reveals stage IIIB or IV disease, surgery is not helpful and no pathological staging information will be obtained (appropriately). Lung cancer biopsies can be taken for two different reasons: - Diagnosis: To find out whether an abnormality seen on a chest x-ray or CT scan is indeed lung cancer, and what histological type it is (small cell or non-small cell). - Staging: To find out whether a structure, such as a lymph node in the mediastinum, has already been invaded by cancer or not. However, it is often possible, with proper planning, to obtain both diagnostic and staging information with a single biopsy procedure. There is an extensive array of staging methods available, each with advantages and disadvantages. Many cancer treatment centers review newly diagnosed patients at an inter-disciplinary chest tumor board where radiologists, oncologists, surgeons, pulmonologists, pathologists and EUS specialists (endosonographers) discuss the relative merits of the available modalities and make a choice by consensus. - T Tumor - T1a: Primary tumor is ≤2 cm in greatest dimension. - T1b: Primary tumor is >2 but ≤3 cm in greatest dimension. - T2a: Primary tumor is >3 and ≤5 cm in greatest dimension. - T2b: Primary tumor is >5 and ≤7 cm in greatest dimension. - T3 size: Primary tumor is >7 cm in greatest dimension; - T3 inv: Primary tumor invades the chest wall, diaphragm, phrenic nerve, mediastinal pleura, or pericardium; - T3 centr: Primary tumor is <2 cm to the carina or there is atelectasis of the entire lung; - T3 satell: Primary tumor is associated with separate tumor nodule(s) in same pulmonary lobe; - T4 inv: Tumor invading the heart, great vessels, trachea, recurrent laryngeal nerve, esophagus, or spine; - T4 ipis: Tumor of any size with additional tumor nodule(s) in a different ipsilateral lobe; - N Lymph node - N1 : Nodal metastasis in ipsilateral pulmonary or hilar lymph nodes; - N2 : Nodal metastasis in ipsilateral mediastinal/subcarinal lymph nodes; \|AJCC edition\|\|published\|\|went into effect\|\|Lung cancer link(s) and page numbers in the original \|7\|\|2009\|\|2010\|\|AJCC, NCI, and NCCN\| \|6\|\|2002\|\|2003\|\|AJCC; original pages 167-177\| \|5\|\|1997\|\|1998\|\|AJCC; original pages 127-137\| \|4\|\|1992\|\|1993\|\|AJCC; original pages 115-122\| \|3\|\|1988\|\|1989\|\|AJCC; original pages 114-121\| \|2\|\|1983\|\|1984\|\|AJCC; original pages 99–105\| \|1\|\|1977\|\|1978\|\|AJCC; original pages 59–64\| The American Joint Committee on Cancer (AJCC) as well as the International Union Against Cancer (UICC) recommend TNM staging, which is a two step procedure. Their TNM system, which they now develop jointly, first classifies cancer by several factors, T for tumor, N for nodes, M for metastasis, and then groups these TNM factors into overall stages as shown in their table. AJCC has provided web accessible poster versions of these copyrighted TNM descriptors and stage groups, to which readers are directed for accurate, detailed, up to date information; additionally, both the National Cancer Institute (NCI) and the National Comprehensive Cancer Network (NCCN) reprint these descriptors and grouping tables with AJCC permission, and extensively discuss staging modalities and treatment options, providing expert clear assistance in decisions about the best treatments. The NCCN pathways "outline the step-by-step treatment decisions from diagnosis through all phases of treatment and survivorship." Although TNM classification is an internationally agreed system, it has gradually evolved through its different editions; the dates of publication and of adoption for use of AJCC editions is summarized in the table in this article; past editions are available from AJCC for web download. Therefore, it is important when reviewing reports of treatment or prognosis to be aware that the criteria used in the TNM system have varied over time, sometimes fairly substantially, according to the different editions. Literature reports usually reflect the staging that was in use while the study was initiated and performed, rather than when it was eventually published. The dates of publication and adoption for use of AJCC editions is summarized here to assist readers in understanding which era is being used by the literature. in the same lobe (local invasion of certain structures) in a different ipsilateral (same side) lobe As in each edition of the TNM staging system, that used from 2010 January 1 (7 edition) made significant changes to the schema that is used for non-small cell lung carcinoma, small-cell lung carcinoma and broncho-pulmonary carcinoid tumors. The revisions were based on a detailed analysis and consensus process by AJCC and UICC that looked at the overall survival of 81,015 patients. The changes have been reviewed in detail, including an extensive presentation (with multiple tables and detailed discussion) of prognostic data for both 6th and 7th edition, looking at both individual T, N and M descriptors, and at overall stage groups. The table uses limited excerpts from the copyrighted TNM system to demonstrate the concept that there are changes, as well as similarilities, between editions. As a result, a given stage may have quite a different prognosis depending on which staging edition is used, independent of any changes in diagnostic methods or treatments, an effect that has been termed "stage migration." The technologies used to assign patients to particular categories have changed also, and it can be seen that increasingly sensitive methods tend to cause individual cancers to be reassigned to higher stages, making it improper to compare that cancer's prognosis to the historical expectations for that stage. Finally, of course, a further important consideration is the effect of improving treatments over time as well. Of considerable historical importance, Dr. Clifton Mountain, a surgeon in Texas, made significant contributions to the TNM staging system, particularly in non-small cell lung carcinoma, and the literature thus often refers to "Mountain" staging. In small-cell lung carcinoma, the TNM classification is often used along with an additional categorization, the Veterans Administration Lung Cancer Study Group system. The VA scheme has two stages. Limited-stage disease is confined to an area that is tolerably treated by one radiotherapy area ("port"), but excludes cancers with pleural and pericardial effusions. All other small-cell lung cancers are extensive-stage in this scheme. There has been some drift over time in what this definition means. CT and PET scans The mainstay of non-invasive staging is a CT scan of the chest followed by metabolic imaging with a PET scan. The CT scan shows abnormalities such as a lung nodule or enlarged lymph nodes whereas the PET scan reveals increased metabolism such as occurs in structures which contain rapidly growing cancer cells. PET/CT combined the benefits of functional assessment with PET and anatomic assessment with CT. PET/CT represents a significant advance for staging of patient's with lung cancer with management impact in the order of 40% and discordant findings compared with conventional imaging in half of patients. PET/CT also has high inter- and intra-reporter agreement. The main benefit of PET is to identify distant metastatic disease, thereby indicating futility of locoregional approaches such as surgery or curative intent radiotherapy. According to the American College of Chest Physicians (ACCP) Non-Invasive Staging Guidelines for Lung Cancer (2007), the pooled sensitivity and specificity of CT scanning for identifying mediastinal lymph node metastasis are 51% and 85%, respectively and for PET scanning 74% (95% CI, 69 to 79%) and 85% (95% CI, 82 to 88%), respectively. In other words, were one to rely on the results of non-invasive staging alone, between 21 and 31 percent of patients would be understaged (the cancer is more advanced than it seems) and between 12 and 18 percent of patients would be overstaged ( the cancer is in fact in an earlier stage than it seems). In selected clinical situations confirmation of the results of the status of the mediastinal nodes by sampling will therefore be necessary. Pulmonary function tests (PFT) are not a formal part of staging but can be useful in treatment decisions. Patients with lung cancer resulting from air pollution (occupational or cigarette smoking-related) are more likely to have other lung disorders such as COPD, which limit their respiratory reserve. Patients with limited respiratory reserve are at higher risk for postoperative complications should surgical treatment be pursued; they may not be able to tolerate the diminished lung capacity remaining after the removal of a portion of the lung. PET scans need to be specifically modulated to detect metastases within the brain, which otherwise shows fairly uniformly active uptake from normal brain activity. In practice, CT or MRI scans are used to detect brain metastases. Although MRI has a modestly higher detection rate and can find smaller metastases, contrast-enhanced CT scan can be a suitable choice due to many factors such as MRI-incompatible metal prosthetics, claustrophobia, or noise-intolerance. Nearly half of lung cancers have mediastinal disease at diagnosis. If cancer involves any of the mediastinal lymph nodes, these lymph nodes are designated N2 if they are on the same side as the original tumor, and N3 if they are on the other. N2, and particularly N3 lymph nodes, affect the clinical stage very significantly. The American Thoracic Society has standardized the nomenclature of lymph nodes in the chest. There are fourteen numbered nodal stations. Lymph nodes considered to be in the mediastinum are stations 1–9, which are thus potential N2 or N3 locations, while stations 10-14 are hilar and peripheral nodes, and thus potential N1 locations. There are numerous modalities which allow staging of mediastinal lymph nodes. In the following table they are arranged from the most to the least invasive. \|Thoracotomy (surgical opening of the chest)\|\|Allows the most thorough inspection and sampling of lymph node stations, may be followed by resection of tumor, if feasible\|\|Most invasive approach, not indicated for staging alone, significant risk of procedure-related morbidity\| \|Extended cervical mediastinoscopy combined with a Chamberlain procedure, which is also called a left parasternal mediastinotomy, or anterior mediastinotomy\|\|Permits evaluation of the aortopulmonary window lymph nodes\|\|More invasive; false-negative rate approximately 10%.\| \|Chamberlain procedure\|\|Access to station 5 (aortopulmonary window lymph node)\|\|Limited applications, invasive.\| \|Cervical mediastinoscopy\|\|Still considered the gold standard (usual comparitor) by many, excellent for 2RL 4RL.\|\|Does not cover all medastinal lymph node stations, particularly subcarinal lymph nodes (station 7), paraesophageal and pulmonary ligament lymph nodes (stations 8 and 9), the aortopulmonary window lymph nodes (station 5), and the anterior mediastinal lymph nodes (station 6); false-negative rate approximately 20%; invasive\| \|Video-assisted thoracoscopy\|\|Good for inferior mediastinum, station 5 and 6 lymph nodes\|\|Invasive, does not cover superior anterior mediastinum\| \|Transthoracic percutaneous fine needle aspiration (FNA) under CT guidance\|\|More widely available than some other methods\|\|Traverses a lot of lung tissue, therefore high pneumothorax risk, some lymph node stations inaccessible\| \|Bronchoscopy with blind transbronchial FNA (Wang needle)\|\|Less invasive than above methods\|\|Relatively low yield, not widely practiced, bleeding risk\| \|Endobronchial ultrasound (EBUS)\|\|Direct visualization of lymph node stations. Complements EUS: covers lymph node stations 2R and 4R which are difficult to access by EUS; lower false-negative rate than with blind transbronchial FNA and fewer complications\|\|More invasive than EUS, few practitioners, but rapidly growing in popularity\| \|Endoscopic ultrasound (EUS)\|\|Least invasive modality, uses the esophagus to access mediastinal lymph nodes, excellent for station 5, 7, 8 lymph nodes. Useful for station 2L and 4L, L adrenal, celiac lymph node\|\|Cannot reliably access right sided paratracheal lymph node stations 2 R and 4R; accurate discrimination of primary hilar tumors and involved lymph nodes is important\| This section focuses on the emerging role various types of endoscopic ultrasound and biopsy are playing in the diagnosis and staging of lung cancer, with an emphasis on the most common type of lung cancer, non-small cell lung cancer (NSCLC). These techniques have been reviewed extensively and have attained substantial consensus in guidelines such as from the NCCN. Endoscopic ultrasound (EUS) is an endoscopic technique where a miniaturized ultrasound probe is passed though the mouth into the upper gastrointestinal tract to investigate organs and structures close to the esophagus, stomach, or duodenum, such as the lung. In 1993, Wiersema published the first description of EUS to diagnose and stage lung cancer, done by advancing a fine needle through the esophagus into adjacent lymph nodes. Numerous subsequent studies have shown this general methodology to be effective, very safe, minimally invasive, and very well tolerated. Given these advantages, many authorities think that EUS together with endobronchial ultrasound (EBUS) enhances lung cancer diagnosis and staging. Endoscopic ultrasound (EUS) A metaanalysis published in 2007, based on 1,201 cancers in 18 high-quality clinical trials carefully selected by predefined criteria from the literature, systematically examined the performance of EUS-guided FNA in NSCLC staging. Two scenarios were considered: the setting of enlarged lymph nodes on CT (suggestive but not diagnostic of cancer), and the obverse scenario of an absence of lymph node enlargement on CT (suggestive but not diagnostic of no cancer). Overall, in both settings, minor complications were reported in 0.8% of procedures; no major complications were recorded. EUS-FNA in enlarged discrete mediastinal lymph nodes had an excellent pooled sensitivity (8 studies) of 90% (95% CI, 84 to 94%) and specificity of 97% (95% CI, 95 to 98%). EUS-FNA in the setting of no enlarged mediastinal lymph nodes on CT had a pooled sensitivity (4 studies) of 58% (95% CI, 39 to 75%) and specificity of 98% (95% CI, 96 to 99%). Although this sensitivity (58% in CT-negative disease) might on first consideration seem disappointing, if EUS is performed as a staging test it can help avoid more invasive staging procedures, or surgery, if positive (for the presence of cancer). In other words, an EUS that has a positive result (shows cancer) will avoid further needless surgery, whereas a result not showing cancer may be false-negative, and probably requires an excisional biopsy technique for confirmation, such as VATS or mediastinoscopy. The ACCP guidelines recommend invasive staging for patients with or without mediastinal lymph node enlargement on CT regardless of the PET scan findings. If needle techniques are used (such as EUS-NA, TBNA, EBUS-NA, or TTNA) a non-malignant result should be further confirmed by mediastinoscopy as explained above. EUS can reliably reach the lymph node stations 5, 7, 8 and 9. In the superior mediastinum the trachea is somewhat to the right of the esophagus which makes it often possible to reach left-sided area 2 and 4 lymph nodes and, less often, right sided paratracheal lymph nodes. In general, EUS is most appropriate for evaluation of the posterior inferior mediastinum while mediastinoscopy or EBUS are best for the anterior superior mediastinum. The feasibility of EUS-FNA of aorto-pulmonary window (subaortic) lymph nodes (station 5) is a major advantage of EUS. Evaluation of this station has traditionally required a paramedian mediastinotomy (Chamberlain procedure). EUS can easily sample celiac lymph nodes, which cannot be reached by the other mediastinal staging methods. In one recent study an unexpectedly high incidence of celiac lymph node metastasis (11%) was noted. EUS can also be used to biopsy potential left adrenal metastases, whereas the right adrenal gland is mostly inaccessible. The potential utility of EUS-FNA in restaging of the mediastinum in patients who have undergone chemotherapy and radiotherapy for N2 or N3 disease is under investigation. The underlying idea is that initially advanced cancers, previously too extensive for surgery, may have responded to chemotherapy and radiation so much that they now may be operative candidates. Rather than immediately proceeding to thoracotomy based on CT or PET results, which could lead to an “open and close” thorax surgery, restaging, including invasive staging, may deselect non-responders, missed on imaging tests alone. If the initial mediastinal staging included a mediastinoscopy, most surgeons try to avoid a repeat mediastinoscopy after radiation treatment because of scarring. Although restaging by PET and CT scanning may help to provide targets for biopsies, the concept is that even PET-negative mediastinums need to be sampled. In N2 disease, EUS-FNA and EBUS-FNA appear to offer the best risk-benefit ratio in these patients. Endobronchial ultrasound (EBUS) As mentioned in the table, EUS cannot reliably access right sided paratracheal lymph node stations 2R and 4R and may not be optimal for the left sided paratracheal lymph node stations either. An adaptation of the endoscopic ultrasound scope originally designed for the gastrointestinal tract is known as endobronchial ultrasound (EBUS). The instrument is inserted into the trachea rather than the esophagus. There are two types of EBUS bronchoscopes available: radial catheter probe and convex probe EBUS (CP-EBUS), but only the latter concerns us here. Early experience with mediastinal staging by CP-EBUS appears very promising with sensitivities ranging from 92 to 96 percent in 4 series comprising 70 to 502 patients. Many patients will, if given a choice, prefer an instrument which is inserted into the esophagus (EUS) over one which is inserted into the trachea (EBUS). Furthermore, many patients with suspected lung cancer will have other smoking related illnesses, such as emphysema or COPD, which makes a bronchoscopy-like procedure (EBUS) a higher risk than an upper endoscopy through the esophagus (EUS). An area of active and emerging research concerns the value of combining EUS and EBUS in a single session, one specialist following the other, or -even more convenient- a dual trained operator doing one or the other – or both- as needed. EUS-FNA and EBUS-FNA are complementary techniques. EUS has the highest yield in the posterior inferior mediastinum, and EBUS is strongest for the superior anterior mediastinum. Some lymph node stations can only be accessed by one method and not the other (for example, station 2 and 4 L and 3 are hard or impossible to see by EUS, stations 5 and 8 cannot be biopsied by EBUS). Together, EBUS and EUS cover the entire mediastinum (except possibly station 6) and complete mediastinal staging should be possible with a combination of these two procedures. This combination could conceptually eliminate the need for most surgical mediastinoscopies and in fact be more comprehensive. When combined, this approach has been termed “complete medical mediastinoscopy.” EUS-FNA with EBUS may allow near-complete, minimally invasive mediastinal staging in patients with suspected lung cancer - Lu Charles, Onn A, Vaporciyan AA et al. Holland-Frei Cancer Medicine. 8th edition. Chapter 78: Cancer of the Lung. People's Medical Publishing House, USA (2010) ISBN 9781607950141 - What is Cancer Staging? American Joint Committee on Cancer 2010 May 5.http://www.cancerstaging.org/mission/whatis.html - Rami-Porta, R; Crowley JJ; Goldstraw P (February 2009). "The revised TNM staging system for lung cancer" (PDF). Annals of Thoracic and Cardiovascular Surgery 15 (1): 4–9. PMID 19262443. - American Joint Committee on Cancer. Past Editions of the AJCC Cancer Staging Manual available at http://www.cancerstaging.org/products/pasteditions.html - AJCC Cancer Staging Manual, 7th edition, updated Cancer Staging Posters. http://www.cancerstaging.org/staging/posters/lung8.5x11.pdf - NCCN Guidelines for patients: non-smallcell lung cancer; 2010. http://www.nccn.com/images/patient-guidelines/pdf/nsclc.pdf - AJCC Cancer Staging Manual 6th edition; Chapter 19; Lung - original pages 167-177http://www.cancerstaging.org/products/csmanual6ed-4.pdf - AJCC Cancer Staging Manual 5th edition; Chapter 19; Lung - original pages 127-137http://www.cancerstaging.org/products/csmanual5ed_3.pdf - AJCC Cancer Staging Manual 4th edition; Chapter 19; Lung - original pages 115-122 http://www.cancerstaging.org/products/csmanual4ed.pdf - AJCC Cancer Staging Manual 3rd edition; Chapter 18; Lung - original pages 114-121http://www.cancerstaging.org/products/csmanual3ed_2.pdf - AJCC Cancer Staging Manual 2nd edition; Chapter 16; Lung - original pages 99-105http://www.cancerstaging.org/products/csmanual2ed.pdf - AJCC Cancer Staging Manual 1977; Chapter 6; Lung - original pages 59-64http://www.cancerstaging.org/products/csmanual1ed.pdf - A. De la Guerra. New TNM Classification for Lung Cancer - Part I: The changes. Doctors Lounge Website. http://www.doctorslounge.com/index.php/articles/page/340 - A. De la Guerra. New TNM Classification for Lung Cancer. Part II: A review. Doctors Lounge Website. http://www.doctorslounge.com/index.php/articles/page/342 - Feinstein AR, Sosin DM, Wells CK (June 1985). "The Will Rogers phenomenon. Stage migration and new diagnostic techniques as a source of misleading statistics for survival in cancer". N. Engl. J. Med. 312 (25): 1604–8. doi:10.1056/NEJM198506203122504. PMID 4000199. - Chee KG, Nguyen DV, Brown M, Gandara DR, Wun T, Lara PN (July 2008). "Positron emission tomography and improved survival in patients with lung cancer: the Will Rogers phenomenon revisited". Arch. Intern. Med. 168 (14): 1541–9. doi:10.1001/archinte.168.14.1541. PMID 18663166. - Mountain, Clifton F; Herman I Libshitz; Kay E Hermes. A Handbook for Staging, Imaging, and Lymph Node Classification. Charles P Young Company. - Mountain CF (June 1997). "Revisions in the International System for Staging Lung Cancer". Chest 111 (6): 1710–7. doi:10.1378/chest.111.6.1710. PMID 9187198. - Gregory, D. L.; Hicks, R. J.; Hogg, A.; Binns, D. S.; Shum, P. L.; Milner, A.; Link, E.; Ball, D. L.; Mac Manus, M. P. (2012). "Effect of PET/CT on Management of Patients with Non-Small Cell Lung Cancer: Results of a Prospective Study with 5-Year Survival Data". Journal of Nuclear Medicine 53 (7): 1007–1015. doi:10.2967/jnumed.111.099713. PMID 22677701. - Hofman, M. S.; Smeeton, N. C.; Rankin, S. C.; Nunan, T.; O'Doherty, M. J. (2008). "Observer variation in FDG PET-CT for staging of non-small-cell lung carcinoma". European Journal of Nuclear Medicine and Molecular Imaging 36 (2): 194–199. doi:10.1007/s00259-008-0946-3. PMID 18828012. - Silvestri G et al. Noninvasive staging of non-small cell lung cancer: ACCP evidenced-based clinical practice guidelines (2nd edition). Chest 2007, 132(3 Suppl):178S-201S - Detterbeck F et al. Invasive mediastinal staging of lung cancer: ACCP evidence-based clinical practice guidelines (2nd edition). Chest 2007, 132(3 Suppl):202S-220S. - Micames CG, McCrory DC, Pavey DA, Jowell PS, Gress FG (February 2007). "Endoscopic ultrasound-guided fine-needle aspiration for non-small cell lung cancer staging: A systematic review and metaanalysis". Chest 131 (2): 539–48. doi:10.1378/chest.06-1437. PMID 17296659. - "Lymph Node Map for EUS -EusWiki". Retrieved 2009-03-11. - Kim ES, Bosquée L (June 2007). "The importance of accurate lymph node staging in early and locally advanced non-small cell lung cancer: an update on available techniques". J Thorac Oncol. 2 Suppl 2: S59–67. doi:10.1097/01.JTO.0000269738.13586.fd. PMID 17589301. - Wiersema M et al. Real-time endoscopic ultrasound-guided fine-needle aspiration of a mediastinal lymph node. Gastrointest Endosc 1993, 39(3):429-431 - Annema J, Rabe K: State of the art lecture: EUS and EBUS in pulmonary medicine. Endoscopy 2006, 38 Suppl 1:S118-122 - Larsen S et al. Endoscopic ultrasound guided biopsy versus mediastinoscopy for analysis of paratracheal and subcarinal lymph nodes in lung cancer staging. Lung Cancer 2005, 48(1):85-92. - Singh P et al. Endoscopic ultrasound as a first test for diagnosis and staging of lung cancer: a prospective study. Am J Respir Crit Care Med 2007, 175(4):345-354. - DeWitt J et al. Endoscopic ultrasound-guided fine-needle aspiration of left adrenal gland masses. Endoscopy 2007, 39(1):65-71. - Cerfolio R, Bryant A, Ojha B: Restaging patients with N2 (stage IIIa) non-small cell lung cancer after neoadjuvant chemoradiotherapy: a prospective study. J Thorac Cardiovasc Surg 2006, 131(6):1229-1235 - Yasufuku K et al. Endobronchial ultrasonography: current status and future directions. J Thorac Oncol 2007, 2(10):970-979. - Yasufuku K et al. Real-time endobronchial ultrasound-guided transbronchial needle aspiration of mediastinal and hilar lymph nodes. Chest 2004, 126(1):122-128 - Yasufuku K et al. Endobronchial ultrasound guided transbronchial needle aspiration for staging of lung cancer. Lung Cancer 2005, 50(3):347-354 - Herth F et al.: Real-time endobronchial ultrasound guided transbronchial needle aspiration for sampling mediastinal lymph nodes. Thorax 2006, 61(9):795-798. - Herth F et al.: Endobronchial ultrasound-guided transbronchial needle aspiration of lymph nodes in the radiologically normal mediastinum. Eur Respir J 2006, 28(5):910-914 - Wallace MB, Pascual JM, Raimondo M, et al. (February 2008). "Minimally invasive endoscopic staging of suspected lung cancer". JAMA 299 (5): 540–6. doi:10.1001/jama.299.5.540. PMID 18252884. - Vilmann P, Larsen SS. Endoscopic ultrasound-guided biopsy in the chest: little to lose, much to gain.Eur Respir J. 2005 Mar;25(3):400-1. Review - Endoscopic Ultrasound Resources	2	29	1	0	0	1	0.55414	2	7,343
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. David L Arluck MD Dr. David L Arluck, MD is a Doctor primarily located in Somers Point, NJ, with other offices in Somers Point, NJ and Somers Point, NJ . He has 43 years of experience. His specialties include Internal Medicine and Cardiovascular Disease. Dr. Arluck is affiliated with Shore Medical Center. He speaks English. Dr. David L Arluck has 3 locations 155 Medical Center Way Fl 2 Somers Point, NJ 08244 Book Online Now Dr. David L Arluck 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. - Cardiovascular Disease Dr. David L Arluck has the following 13 expertise - Heart Block - Heart Failure - Acute Coronary Syndrome (ACS) - Unstable Angina - Heart Attack - High Blood Pressure (Hypertension) - Heart Diseases - Blood Clot - High Cholesterol Dr. David L Arluck is Board Certified in 2 specialties 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 4 of 4 Self-verified patient of Dr. David L Arluck - Posted on September 14th, 2018 Dr. Arluck is very thorough. He takes the time to answer any questions. He has made recommendations for me to follow that will promote better health . He is approachable and gets to the point. I was happy that he ordered the right test that I needed to bring my problems to light which in turn were addressed. I am healthier after a few appointments & procedures. I would highly recommend him . Dolores Gill Very good heart doctor Self-verified patient of Dr. David L Arluck - Posted on April 30th, 2013 Self-verified patient of Dr. David L Arluck - Posted on October 28th, 2011 He Believed Me Self-verified patient of Dr. David L Arluck - Posted on April 9th, 2011 Dr. Arluck is one of the rare doctors...He believes a fast heart rate, shortness of breath, and chest pain in a 38 year old woman can be a sign of cardiac problems and not just panic attacks. It was so reassuring to find a doctor who didn't think it was all in my head. Only two weeks and one angioplasty after my very first visit, I was "fixed" Dr. Arluck is affiliated (can practice and admit patients) with the following hospital(s). 43 Years Experience University Of Pennsylvania School Of Medicine Graduated in 1975 University Of Virginia Health System Dr. David L Arluck Accepts the Following Insurance - Aetna Choice POS Open Access - Aetna Managed Choice POS Open Access - Aetna Signature Administrators PPO BCBS Blue Card - BCBS Blue Card PPO - CIGNA HMO - CIGNA Open Access Plus - CIGNA PPO - First Health PPO Geisinger Health Plan - Geisinger Health Plan HIP of NY - Emblem HIP Select PPO - Humana Choice POS - Humana ChoiceCare Network PPO - Oxford Health Garden State - QualCare HMO - QualCare PPO - UHC Choice Plus POS - UHC Navigate HMO - UHC Navigate POS - UHC Options PPO More about Dr. David L Arluck Dr. David Arluck, MD is in Cardiology and Internal Medicine (1981). He attended medical school at the University of Pennsylvania of Medicine, did his fellowship at the University of Virginia and completed internship and residency at the Long Island Jewish Medical Center. Dr. Arluck has been affiliated with AtlantiCare since 1982. Locations & Directions Dr. David L Arluck is similar to the following 4 Doctors near Somers Point, NJ.	2	10	1	0	0	0	0.939301	1	1,178
Whenever a patient sees a healthcare provider, the provider documents their visit, reviews the complaint and medical history, makes an expert assessment of the condition, and proposes treatment. This process involves the use of medical codes. Medical codes are a term referring to diseases, symptoms, diagnoses, prescriptions, treatments, services, and other medical procedures a doctor or healthcare provider performs on a patient. These codes tell the patients, medical workers, insurances, and other involved participants more about the diagnoses, provided services, and how much they cost. Different categories of medical codes There are two primary groups of medical codes: 1. CPT Codes ‘Current Procedural Terminology’ or CPT refers to standard codes used in most contemporary medical settings. These codes describe each type of service a healthcare provider carries out on a patient, including evaluations, tests, surgeries, and so on. They are mainly used to enable reimbursement by submitting to insurance, Medicare, or another payer. Patients can also use them to better understand the services they received, check for any issues in their bills, or to try to (re)negotiate the prices for the services. 2. ICD Codes Short for ‘International Classification of Diseases and Related Health Problems’, ICD refers to a medical classification list whose publication is authorized by the World Health Organization (WHO). The list serves for classifying diseases, illnesses, injuries, health encounters, and inpatient procedures in morbidity settings. Primary users of ICD codes are healthcare professionals, like physicians and nurses, but also medical coders who assign ICD codes to diagnosis or procedure information. The codes are used for various purposes, including for billing and claims reimbursement, as well as in statistics. Secondary users of ICD codes are people who already use coded data from hospitals, healthcare providers, or health plans to carry out research and monitoring activities. Public health is one example of a secondary user of ICD codes. Other groups of medical codes include: 3. HCPCS Codes The lesser-known category of medical codes, used by Medicare, is Healthcare Common Procedure Coding System or HCPCS. This system draws on CPT codes and is useful to patients using Medicare, especially those in need of ambulance services or other resources outside of their doctor’s office. There are two levels of HCPCS codes: - Level I: these codes mirror CPT codes and denote medical procedures and services a physician or another licensed professional has ordered. - Level II: these are alphanumeric and denote non-physician services such as ambulance rides, walkers, wheelchairs, and other kinds of durable medical equipment, as well as other medical services that Level I doesn’t cover. 4. ICF Codes Short for the ‘International Classification of Functioning, Disability, and Health’, ICF codes measure and describe the level of health and disability in connection to a certain health condition. While the ICD codes classify an illness, the ICF codes measure how the affected person is functional in their environment. 5. DRG Codes The ‘diagnostic-related group’ or DRG system ‘groups together’ different medical codes for hospital billing purposes. This system categorizes hospital services based on a diagnosis, treatment, and other relevant criteria. It allows hospitals to get paid for inpatient services on the basis of a patient’s assigned DRG profile at a fixed rate, regardless of the actual cost of the hospital stay or how much the hospital bills the insurance company or Medicare. There are around 500 different DRGs, assuming the same patient profile requires roughly the same treatment. 6. NDC Codes The National Drug Code or NDC indicates nonprescription (OTC) and prescription medications. It is a 10-digit numeric code that is visible on all medication packages in the US and includes three parts: - identifying the medication labeler (manufacturer, repackager, distributor, or marketer) - identifying the medication (strength, formulation, and dosage form) - identifying the size and type of the medication package Although the US Food and Drug Administration (FDA) manages and updates the NDC Directory daily, the medications on the list aren’t necessarily approved by it. 7. CDT Codes Like its name indicates, the Code on Dental Procedures and Nomenclature (CDT) has a role in oral health and related dentistry services, allowing dental professionals to have a set of procedural alphanumeric codes for their work. The American Dental Association (ADA) annually publishes the CDT reference manual, categorizing the codes according to the type of service: diagnostic, restorative, preventive, periodontics, endodontics, removable prosthodontics, maxillofacial prosthetics, implant services, fixed prosthodontics, implant services, maxillofacial surgery, orthodontics, and adjunctive general services. 8. DSM Codes The American Psychiatric Association (APA) publishes and maintains the Diagnostic and Statistical Manual of Mental Disorders. The codes in this manual (the latest edition is DSM-5) are used by mental health professionals and other relevant entities to diagnose, describe, and classify psychiatric illnesses. That said, the newest edition of the manual recommends using ICD-10 codes for psychiatric conditions instead. The existing patient records may still include the DSM codes. Now, let’s inspect the two major categories – CPT and ICD codes. What are CPT codes Developed by the AMA, the Current Procedural Terminology (CPT) codes are vital in billing medical services, as well as the procedures for their reimbursement. Providers of medical services (physicians, hospitals, laboratories, outpatient facilities, non-physician practitioners, and allied health professionals) use these codes to describe and report services and procedures to private and federal payers. Through its CPT Editorial Panel, the American Medical Association (AMA) maintains and annually updates the list of CPT codes. These codes include five characters which are typically numeric, but some of them include a fifth alpha character. There are three categories of CPT codes: - Category I: Five-digit codes that include descriptions of a procedure or service (most CPT codes are in this category) - Category II: Supplemental alphanumeric tracking codes referring to performance evaluation or clinical services with no relative value - Category III: Temporary codes assigned to tracking the efficacy of emerging and experimental technologies, procedures, and services Category I CPT codes are divided into six main sections: - Evaluation and Management: 99201 – 99499 - Anesthesia: 00100 – 01999; 99100 – 99140 - Surgery: 10021 – 69990 - Radiology: 70010 – 79999 - Pathology and Laboratory: 80047 – 89398 - Medicine/Medical Services and Procedures: 90281 – 99199; 99500 – 99607 Each of these sections further includes its own subfields. Category II of CPT codes is not associated with any relative value, so the services in this group are billed with a $0.00 billable charge amount. The Performance Measures Advisory Group (PMAG), which is an advisory body to the CPT Editorial Committee and the CPT/HCPAC Advisory Committee, reviews the codes in accordance with the medical and additional expertise. This category includes 10 sections: - Composite Measures: 0001F – 0015F - Patient Management: 0500F – 0584F - Patient History: 1000F – 1505F - Physical Examination: 2000F – 2060F - Diagnostic/Screening Processes or Results: 3006F – 3776F - Therapeutic, Preventive, or Other Interventions: 4000F – 4563F - Follow-up or Other Outcomes: 5005F – 5250F - Patient Safety: 6005F – 6150F - Structural Measures: 7010F – 7025F - Non-measure Listing: 9001F – 9007F Since it covers emerging technologies only, Category III contains temporary CPT codes (which stay there for up to five years). They range from 0016T-0207T and these codes may become Category I codes if the CPT Editorial Panel approves them. The AMA releases on its website new or revised codes from Category III semi-annually, while it publishes deletions annually, along with the full listing of temporary codes. The most common CPT codes Some of the CPT codes occur more frequently than the others, for instance: - 99214 – established patient office or other outpatient services - 47350 – simple repair of liver hemorrhage - 47360 – complex liver repair procedure, with or without hepatic artery ligation - 62000 – elevation of a simple, extradural depressed skull fracture - 62005 – elevation of a compound or commuted, extradural depressed skull fracture - 3008F – Body Mass Index (BMI), documented - 0001F – heart failure assessed - 0503F – postpartum care visit - 1030F – pneumococcus immunization status assessed - 2014F – mental status assessed - 3006F – chest X-ray documented and reviewed - 4037F – influenza immunization ordered or administered - 5005F – patient directed to perform self-examination for new or changing moles - 6015F – patient received or is allowed to receive foods, fluids, or medication by mouth - 7025F – patient data input in the reminder system with a date for the next mammogram - 0123T – fistulization of sclera for glaucoma, through the ciliary body The most common psychiatric CPT codes Some CPT codes appear regularly in psychiatry, such as: - 90791 – psychiatric diagnostic evaluation without medical services - 90792 – psychiatric diagnostic evaluation with medical services - 90832 – psychotherapy, 30 minutes - 90833 – evaluation and management with 30 minutes psychotherapy - 90846 – family psychotherapy, without patient present - 90847 – family psychotherapy, with patient present - 90839 – psychotherapy in crisis - 99203 – new patient, outpatient, in-office services, 30 minutes - 99215 – established patient, outpatient, in-office services, 40 minutes - 99242 – new or established patient, outpatient, consultation, 30 minutes - 99252 – inpatient consultation, 40 minutes - 90865 – narcosynthesis - 90870 – electroconvulsive therapy (ECT) - 90885 – psychiatric evaluation of records For the full list of CPT codes check this link. What are ICD codes The International Classification of Diseases and Related Health Problems (ICD) is an international diagnostic tool for health management, epidemiology, and clinical purposes. The list of ICD codes changes over time. Its most up-to-date version, first released in 1992, is ICD-10, which is an upgrade from the previous one – ICD-9 and includes more codes and classifications for updated conditions and diagnoses than its predecessor. ICD-10 contains over 70,000 disease codes. The 11th revision will officially come into effect on January 1, 2022, after every WHO member endorsed it at the organization’s 72nd World Health Assembly (WHA) on May 25, 2019. In between revisions, the ICD manual goes through annual minor updates and triennial major updates. Although WHO is the manager and publisher of the base ICD, some of its member states have modified it in accordance with their specific needs. Currently, the US is using two types of ICD-10 systems (both updated annually): - ICD-10-CM (Clinical Modification), which includes diagnosis codes - ICD-10-PCS (Procedure Coding System), which includes procedure codes ICD codes can have between three to seven alphanumeric characters and classify a wide array of symptoms, signs, complaints, abnormal findings, social circumstances, as well as external causes of disease or injury. Hence, larger categories include similar diseases. The first three characters in the code mark the category and can stand alone if the category has no further subdivision. The first alpha letter groups diseases together to specify a specific condition, organ system, or a condition characteristic. For example, ‘A’ is for ‘Infectious and parasitic diseases’, ‘G’ for ‘Nervous system’, ‘Q’ for ‘Congenital and chromosomal abnormalities’, and ‘Z’ for ‘Factors influencing health status and contact with health services’. The next three characters refer to the related etiology, severity, anatomic location, or another important clinical detail. Appearing only rarely, the seventh character is the extension that provides information about the characteristic of the encounter. For injuries, these character extensions include: initial encounter – ‘A’, subsequent encounter – ‘D’, and sequela – ‘S’. It always stands in seventh place. Hence, if a code has fewer than six characters, the fields before the extension need to contain a placeholder X. The most common ICD codes for family practice Some ICD-10 codes appear more often than others in family practice, such as: - Z00.00 – General adult medical examination without abnormal findings - Z12.4 – Screening for malignant neoplasm of cervix - Z12.5 – Screening for malignant neoplasm of prostate - Z79.01 – Long-term (current) use of anticoagulants - Z79.891 – Long-term (current) use of opiate analgesic - Z01.411 Gynecological exam (general, routine) with abnormal findings - Z01.419 Gynecological exam (general, routine) without abnormal findings - Z34.80 – Supervision of normal pregnancy, unspecified trimester - I10 – Essential (primary) hypertension - I48.91 – Unspecified atrial fibrillation - J02.9 – Acute pharyngitis, unspecified - E11.9 – Type 2 diabetes mellitus without complications - E11.65 – Type 2 diabetes mellitus with hyperglycemia - G93.3 – Postviral fatigue syndrome - R10.84 – Generalized abdominal pain - R63.5 – Abnormal weight gain - R51 – Headache - R50.9 – Fever, unspecified - R53.1 – Weakness - R31.9 – Hematuria - R19.7 – Diarrhea, unspecified - K52.2 – Allergic and dietetic gastroenteritis and colitis - R30.0 – Dysuria - R30.9 – Painful micturition, unspecified - R79.0 – Abnormal level of blood mineral - R60.0 – Localized edema - R60.1 – Generalized edema - R05 – Cough - R42 – Dizziness and giddiness - N39.0 – Urinary tract infection, site not specified - N30.00 – Acute cystitis without hematuria - N30.01 – Acute cystitis with hematuria - E78.5 – Hyperlipidemia, unspecified - E78.2 – Mixed hyperlipidemia - E78.0 – Pure hypocholesterolemia - E55.9 – Vitamin D deficiency, unspecified - E03.9 – Hypothyroidism, unspecified - D64.9 – Anemia, unspecified - D50.9 – Iron deficiency anemia, unspecified - L03.90 – Cellulitis, unspecified - L03.91 – Acute lymphangitis, unspecified The most common ICD codes for mental disorders Practitioners treating mental disorders will commonly encounter these ICD-10 codes: - Part F40-48 – Anxiety, dissociative, stress-related, somatoform and other nonpsychotic mental disorders, for example: - F40.01 – Agoraphobia with panic disorder - F40.11 – Social phobia, generalized - F40.9 – Phobic anxiety disorder, unspecified - F41.1 – Generalized anxiety disorder - F41.9 – Anxiety disorder, unspecified - F42 – Obsessive-compulsive disorder - F43.10 – Post-traumatic stress disorder, unspecified - F43.20 – Adjustment disorder, unspecified - F43.21 – Adjustment disorder with depressed mood - F44.4 – Conversion disorder with motor symptom or deficit - F44.9 – Dissociative and conversion disorder, unspecified - Part F60-F69 – Disorders of adult personality and behavior, including: - F60.3 – Borderline personality disorder - F64.2 – Gender identity disorder of childhood - Part F50-F59 – Behavioral syndromes associated with psychological disturbances and physical factors: - F50.00 – Anorexia nervosa, unspecified - F50.2 – Bulimia nervosa - F51.01 – Primary insomnia - F51.03 – Paradoxical insomnia - F51.04 – Psychophysiologic insomnia - F51.05 – Insomnia due to other mental disorder - F51.09 – Other insomnia not due to a substance or known physiological condition - Part F80-F89 – Pervasive and specific developmental disorders, most often: - F84.0 – Autistic disorder - F84.2 – Rett’s syndrome - F84.5 – Asperger’s syndrome - Part F30-F39 – Mood [affective] disorders, like: - F31.0 – Bipolar disorder, current episode hypomanic - F31.10 – Bipolar disorder, current episode manic without psychotic features, unspecified - F31.5 – Bipolar disorder, current episode depressed, severe, with psychotic features - F31.60 – Bipolar disorder, current episode mixed, unspecified - F31.70 – Bipolar disorder, currently in remission, most recent episode unspecified - F31.81 – Bipolar II disorder - F31.9 – Bipolar disorder, unspecified - F32.1 – Major depressive disorder, single episode, severe without psychotic features - F33.1 – Major depressive disorder, recurrent, moderate - F34.1 – Dysthymic disorder - F39 – Unspecified mood [affective] disorder - Part F20-F29 – Schizophrenia, schizotypal, delusional, and other non-mood psychotic disorders, such as: - F20.9 – Schizophrenia, unspecified - F25.1 – Schizoaffective disorder, depressive type - F25.9 – Schizoaffective disorder, unspecified - Part F90-F98 – Behavioral and emotional disorders with onset usually occurring in childhood and adolescence. Examples include: - F90.0 – Attention-deficit hyperactivity disorder, predominantly inattentive type - F90.1 – Attention-deficit hyperactivity disorder, predominantly hyperactive type - F90.9 – Attention-deficit hyperactivity disorder, unspecified type - Part F10-F19 – Mental and behavioral disorders due to psychoactive substance use: - F10.27 – Alcohol dependence with alcohol-induced persisting dementia - F11.20 – Opioid dependence, uncomplicated - F11.221 – Opioid dependence with intoxication delirium - F11.23 – Opioid dependence with withdrawal - F13.26 – Sedative, hypnotic or anxiolytic dependence with sedative, hypnotic, or anxiolytic-induced persisting amnestic disorder - Part F01-F09 – Mental disorders due to known psychological conditions: - F02.80 – Dementia in other diseases classified elsewhere without behavioral disturbance - F03.90 – Unspecified dementia without behavioral disturbance - F05 – Delirium due to known physiological condition - F06.32 – Mood disorder due to known physiological condition with major depressive-like episode - Part G30-G32 (within Chapter 6 – Diseases of the nervous system) – Other generative diseases of the nervous system, such as: - G30.0 – Alzheimer’s disease with early onset - G30.1 – Alzheimer’s disease with late onset - G30.9 – Alzheimer’s disease, unspecified For the full list of ICD codes, click here.	4	66	4	0	0	4	0.768539	8	4,407
Individual differences \| Methods \| Statistics \| Clinical \| Educational \| Industrial \| Professional items \| World psychology \| The classification of all headaches, including migraines, is organized by the International Headache Society, and published in the International Classification of Headache Disorders (ICHD). The current version, the ICHD-2, was published in 2004. The first category within the ICHD is Migraine. Migraines in general are considered to be a neurological syndrome. It is estimated that 11% (303 million) of the global population, including 43 million Europeans and 28 million Americans, experience migraines. Organization of migraine subclasses Edit The ICHD-2 categorization includes 6 subclasses of migraine (formerly 7), most of which are further subdivided. The following table outlines these classes and their ICHD-1, -2, and ICD-10 codes. \|1.1\|\|1.1\|\|G430\|\|Migraine without aura\| \|1.2\|\|1.2\|\|G431\|\|Migraine with aura\| \|1.3\|\|1.5\|\|G4382\|\|Childhood periodic syndromes that are commonly precursors of migraine\| \|1.5\|\|1.6\|\|G433\|\|Complications of migraine\| \|n/a\|\|1.7\|\|Migrainous disorder not fulfilling above criteria\| Migraine without auraEdit —International Headache Society Migraine without aura also referred to as a common migraine, (previously known as hemicrania simplex) is a specific neurological disorder characterized by recurrent, throbbing headaches that often affect one side of the head (i.e., it is unilateral), are of at least moderate intensity, and may cause nausea, phonophobia or photophobia. One defining characteristic of the common migraine is a lack of the visual disturbances known as an aura. The exact International Classification of Headache Disorders diagnostic criteria appear to the right. Because migraine without aura can be hard to distinguish from an infrequent episodic tension-type headache, 5 attacks must have been experienced to meet the diagnosis. When migraine without aura is likely, but 5 attacks have not occurred, a diagnosis of probable migraine without aura (ICHD-2: 1.6.1) is warranted. For children, the criteria are slightly less strict. For a pediatric diagnosis of migraine without aura, each attack need only last 1 hour to qualify. Also, pediatric migraines are frequently bilateral (on both sides of the head); unilaterality is not the typical pattern for migraineurs until late adolescence. Note also that migraine without aura can be diagnosed even if a patient has before experienced an aura. One popular theory in migraine pathophysiology is the depolarization theory, which centres around the phenomenon of cortical spreading depression. However, it appears that this theory can not account for migraine without aura. Blood flow imaging has revealed no evidence of this phenomenon, though it has noted some changes in blood flow that are secondary to pain activation, particularly in the brainstem. Research has revealed that nitric oxide (NO) and calcitonin gene-related peptide (CGRP) do have roles in the pathogenesis of a migraine without aura attack. Several studies have shown that migraines without aura develop in most subjects after the infusion of glyceryl trinitrate (GTN, well known as nitroglycerin), which is known to transport NO to tissues, but only in patients who are migraine sufferers. As well, inhibition of the nitric oxide synthase enzymes (NOS) by L-nitromonomethylarginine (L-NMMA) successfully reduced pain severity (in contrast with a placebo) in spontaneous attacks of migraine without aura. In general, migraine without aura is more common than migraine with aura, with more frequent and more disabling attacks. —International Headache Society —International Headache Society It is well documented that migraine occurs nearly 3 times as often in women than in men, and is one of the top 5 most common disabling conditions in women. In over half these women, their headaches are strictly related to their menstrual cycle. A clinical epidemiological study of women with migraine without aura in Parma and Pavia, Italy, revealed that 60% of those women experienced their attacks almost exclusively while menstruating, that 10.7% of their migraines first began at menarche (their very first "period", at puberty), and that 67% of them no longer had migraines while pregnant (and thus not menstruating). This relationship was noted by the IHS in both versions of the ICHD, and particularly that this disorder fell under "migraine without aura". The ICHD-1 referred to this as menstrual migraine, noting that there were no strict guidelines for this diagnosis, but that at least 90% of a woman's attacks should occur within 2 days of the beginning or end of menstruation. When the ICHD-2 was published, explicit guidelines for a diagnosis of 2 distinct types of menstruation-related migraine were released, and appear to the right. However, because the nature of the relationship is still unclear, and because the IHS was still uncertain as to whether these were a subset of migraine without aura or a distinct class of migraine, the criteria were delegated to an appendix, while anticipating that they would appear within the main text in the next revision. The ICHD-2 specifies 2 different forms of the previously-dubbed "menstrual migraine": pure menstrual migraine without aura and menstrually-related migraine without aura. The sole difference between these diagnoses is the occurrence of headache attacks outside of the 5-day period described in the diagnostic criteria. If a woman experiences no attacks outside of this 5-day period, she may be diagnosed with pure menstrual migraine with aura; if she does experience other attacks, however, she may suffer from menstrually-related migraine without aura. This distinction is made solely for treatment purposes; a woman who only experiences migraines in that 5-day period is likely to benefit more from hormone therapy than a traditional migraine medication such as a triptan. One defining characteristic of these menstrual migraines is that the woman does not experience an aura. Clinical research has shown migraine with aura to be unrelated to the menstrual cycle, and, in women who have headaches sometimes with aura and sometimes without, the presence or absence of aura does not appear to be related to the menstrual cycle. As well as being split into 2 classes, menstrual migraines may have 2 different pathophysiologies, based on whether or not a woman is taking any oral contraceptives or another form of cyclical hormone replacement therapy. When these medications are being used, the regular hormonal changes that take place and result in ovulation and other events in the menstrual cycle are suppressed, and menstruation is instead the result of withdrawal from abnormal progestogen concentrations. Menstrual migraines may also be linked to oestrogen withdrawal. Under the category of headache attributed to a substance or its withdrawal, the ICHD specifies the diagnostic criteria for oestrogen-withdrawal headache (8.4.3, G4483 and Y424), and suggests that both that diagnosis and one of the menstrual migraine diagnoses be used in case of migraines related to oestrogen withdrawal occurring mainly at menstruation. Migraine with auraEdit The second-most common form of migraine headache: the patient primarily suffers migraine with aura, and might also suffer migraine without aura. The International Classification of Headache Disorders definition is: Description: Recurrent disorder manifesting in attacks of reversible focal neurological symptoms that usually develop gradually over 5–20 minutes and last for less than 60 minutes. Headache with the features of "migraine without aura" usually follows the aura symptoms. Less commonly, headache lacks migrainous feature or is completely absent [i.e., the aura may occur without any subsequent headache]. A. At least two attacks fulfilling criterion B B. Migraine aura fulfilling criteria [described below] C. Not attributed to another disorder. ...[Criteria for "Typical aura":] Aura consisting of at least one of the following, but no motor weakness: 1. Fully reversible visual symptoms including positive features (e.g. flickering lights, spots or lines) and/or negative features (i.e., loss of vision) 2. Fully reversible sensory symptoms including positive features (i.e., pins and needles) and/or negative features (i.e., numbness) 3. Fully reversible dysphasic speech disturbance [Aura also has] at least two of the following: 1. Homonymous visual symptoms [i.e., affecting just one side of the field of vision] and/or unilateral sensory symptoms [i.e., affecting just one side of the body] 2. At least one aura symptom develops gradually over [at least] 5 minutes and/or different aura symptoms occur [one after the other] over [at least] 5 minutes 3. Each symptom lasts [from] 5 [to] 60 minutes ...[Other potential aura criteria:] - Fully reversible motor weakness... - Each aura symptom lasts [from] 5 minutes [to] 24 hours... - [In the case of a "Basilar-type" migraine], Dysarthria [difficulty speaking], vertigo [dizziness], tinnitus [ringing in the ears], [and other symptoms]. — International Classification of Headache Disorders</div> Basilar type migraineEdit Basilar type migraine (BTM) (previously basilar artery migraine [BAM] and basilar migraine [BM]) is an uncommon, complicated migraine with symptoms caused by brainstem dysfunction. Serious episodes of BTM can lead to stroke, coma, and death. Using triptans and other vasoconstrictors as abortive treatments for BTM is contraindicated. Abortive treatments for BTM address vasodilation and restoration of normal blood flow to the vertebrobasilar territory to restore normal brainstem function. Familial and sporadic hemiplegic migraineEdit - Main article: Familial hemiplegic migraine Familial hemiplegic migraine (FHM) is migraine with a possible polygenetic cause—in fact, FHM can only be diagnosed when at least one close relative has it too. The patient experiences typical migraine with aura headache either preceded or accompanied with one-sided, reversible limb weakness and/or sensory difficulties and/or speech difficulties. FHM is associated with ion channel mutations. There also exists the "sporadic hemiplegic migraine" (SHM), which is the same as FHM but with no close family members showing the symptoms. Effecting a differential diagnosis between basilar migraine and hemiplegic migraine is difficult. Often, the decisive symptom is either motor weakness or unilateral paralysis, which occur in FHM and SHM. Basilar migraine can present tingling and numbness, but true motor weakness and paralysis occur only in hemiplegic migraine. Abdominal migraine Edit Abdominal migraine is a recurrent disorder of unknown origin, principally affecting children. Sometimes early on, it can be misdiagnosed in an ER setting as appendicitis. Episodes feature nausea, vomiting, and moderate-to-severe central, abdominal pain. The child is well between episodes. The International Classification of Headache Disorders definition is: - A. At least 5 attacks fulfilling criteria B-D. - B. Attacks of abdominal pain lasting 1-72 hours (untreated or unsuccessfully treated) - C. Abdominal pain has all of the following characteristics: - 1. midline location, periumbilical or poorly localized - 2. dull or "just sore" quality - 3. moderate or severe intensity - D. During abdominal pain at least 2 of the following: - 1. anorexia - 2. nausea - 3. vomiting - 4. pallor - E. Not attributed to another disorder — International Classification of Headache Disorders</div> Most children with abdominal migraines will develop migraine headache in adult life; the two propensities might co-exist during the child's adolescence. Retinal migraine Edit - Main article: Retinal migraine Retinal migraines are a subclass of optical migraines. Sufferers will experience a scotoma—a patch of vision loss in one eye surrounded by normal vision—for less than one hour before vision returns to normal. Retinal migraines may be accompanied by a throbbing unilateral headache, nausea, or photophobia. Not classified in the ICHD-2 under migraineEdit Acephalgic migraine Edit - Main article: Acephalgic migraine Acephalgic migraine is a neurological syndrome. It is a variant of migraine in which the patient may experience aura symptoms such as scintillating scotoma, nausea, photophobia, hemiparesis and other migraine symptoms but does not experience headache. Acephalgic migraine is also referred to as amigrainous migraine, ocular migraine, ophthalmic migraine or optical migraine, last three being misnomers. Sufferers of acephalgic migraine are more likely than the general population to develop classical migraine with headache. The prevention and treatment of acephalgic migraine is broadly the same as for classical migraine. However, because of the absence of "headache", diagnosis of acephalic migraine is apt to be significantly delayed and the risk of misdiagnosis significantly increased. Visual snow might be a form of acephalgic migraine. - ↑ 1.0 1.1 DOI:10.1016/j.cpr.2009.05.002 - ↑ Leonardi, Matilde (2000). Global burden of migraine in the Year 2000: summary of methods and data sources. Global Burden of Disease 2000. World Health Organization. URL accessed on 4 September 2009. - ↑ includeonly>"The Global Burden of Disease: A response to the need for comprehensive, consistent and comparable global information on diseases and injuries", Epidemiology and Burden of Disease, World Health Organization. Retrieved on 4 September 2009. - ↑ DOI:10.1111/j.1468-2982.2008.01837.x - ↑ 5.0 5.1 DOI:10.1212/WNL.0b013e3181b7c1d8 - ↑ 6.00 6.01 6.02 6.03 6.04 6.05 6.06 6.07 6.08 6.09 6.10 6.11 6.12 6.13 6.14 6.15 6.16 6.17 6.18 6.19 6.20 6.21 Headache Classification Subcommittee of the International Headache Society (2004). The International Classification of Headache Disorders, 2nd Edition. Cephalagia 24 (Supplement 1). - ↑ (1988). ICHD-1. International Headache Society. URL accessed on 4 September 2009. - ↑ (2007). G43. International Statistical Classification of Diseases and Related Health Problems 10th Revision Version for 2007. World Health Organization & the German Institute of Medical Documentation and Information. URL accessed on 4 September 2009. - ↑ 9.0 9.1 9.2 DOI:10.1016/j.pharmthera.2008.08.003 - ↑ PMID 8241457 (PMID 8241457) - ↑ Thomsen, L.L., Kruuse, C.; Iversen, H.K.; Olesen, J. (1994). A nitric oxide donor (nitroglycerin) triggers genuine migraine attacks. European Journal of Neurology 1 (1): 73–80. - ↑ 12.0 12.1 DOI:10.1046/j.1468-2982.2000.00064.x - ↑ DOI:10.1046/j.1468-2982.1998.1801027.x - ↑ DOI:10.1111/j.1468-2982.2007.01288.x - ↑ PMID 8376100 (PMID 8376100) - ↑ Ask the Clinician with Dr. John Claude Krusz and Teri Robert for About Headaches and Migraine: Question and Answer #1 for 12/22/03 - ↑ What Is Abdominal Migraine? from About Headaches and Migraine - ↑ amitriptyline: Definition from Answers.com - ↑ What Are Abdominal Migraines in Children and Adults? - ↑ Topiramate (Topomax) Side Effects, Dosage - MedicineNet - ↑ Cyclic Vomiting Syndrome - National Digestive Diseases Information Clearinghouse - ↑ What is abdominal migraine? Find the definition for abdominal migraine at WebMD This page uses Creative Commons Licensed content from Wikipedia (view authors).	2	10	2	0	3	1	0.653963	6	3,554
Last month, we discussed OB/GYN coding as part of our ICD-10 Quick Tips blog series. This week, we will continue our discussion of OB/GYN coding and focus on coding of multiple gestations. In our past life (ICD-9!) we did not have too many options to capture this data, but ICD-10 certainly took care of that for us! We now have new coding concepts to address and apply, so let’s take a look at one of the biggest changes involving multiple gestation coding. Once again, I want to start with some basic information which is critical for understanding this concept of coding. The first thing we must understand is the th ree different types of multiple gestations: - Monoamniotic/monochromic (mo/mo): Mo/mo twins share the same amniotic sac and share the same placenta within the uterus. These multiples are always identical, but have two separate umbilical cords. - Monoamniotic/diamniotic (mono/di): Mono/di twins generally have two amniotic sacs (a fetus in each sac) yet share the same placenta and have separate umbilical cords. These multiples are also identical. - Dichorionic/diamniotic (di/di): This is the most common form of multiples. These type of twins usually have two amniotic sacs and two placentas. Di/di twins are commonly referred to as fraternal twins. This type of multiple rarely produces identicals. To identify the fetus in a multiple gestation that is affected by the condition being coded. These are the applicable seventh characters: - The seventh character 0 is for single gestations and multiple gestations where the affected fetus is unspecified. - Seventh characters 1 through 9 are for cases of multiple gestations to identify the fetus for which the code applies. - 0 – not applicable or unspecified (also used for single pregnancies) - 1 – Fetus 1 - 2 – Fetus 2 - 3 – Fetus 3 - 4 – Fetus 4 - 5 – Fetus 5 - 9 – Other Fetus - A code from category O30, Multiple gestation must also be assigned when assigning these codes Therefore, if the physician is caring for a pregnant woman with the baby in breech presentation, you would report the appropriate seventh character from 1 through 9 to specify fetus 1, fetus 2, etc. - For example - Mary, pregnant with twins, is close to her due date and the physician noticed that fetus 2 is in breech position. Report code 032.1xx2 (maternal care for breech presentation, fetus 2). - Another example - Joan, pregnant with her first baby (single gestation), is ready to deliver but the baby is in breech position. Report 032.1xx0 (maternal care for breech presentation, not applicable). Physicians often document twins as fetus A and fetus B. However, the fetal extensions in chapter 15, Pregnancy, childbirth and the puerperium, for codes related to complications of multiple gestation (e.g., O31, O32, etc.) refer to fetus 1, fetus 2, and so on. For the purposes of selecting the seventh character for these codes, it is appropriate to assume that fetus A is fetus 1 and B is 2, etc. There you have it folks! Hope you found this short and sweet summary of coding multiple gestations in ICD-10!	4	7	0	0	0	2	0.692129	2	735
I. Sick Sinus Syndrome: What every physician needs to know. Sick sinus syndrome (SSS), more well-known as sinus node dysfunction (SND), is a term that includes a variety of cardiac arrhythmias that have as their basis an abnormality of initiation or propagation of the sinus node impulse. SND may be categorized as intrinsic or extrinsic. Intrinsic SND is due to either abnormalities of the sinus node or contiguous atrial tissue. The etiology is usually degeneration with scarring of the sinus node and perinodal atrial tissue. SND may also arise from other conditions associated with degeneration or destruction of these structures, including myocardial infarction, restrictive or congestive cardiomyopathies, and some cardiac surgeries most notably for correction of congenital heart disease. Extrinsic SND results from other causes (thyroid disorders, drugs or drug interactions, disorders of or affecting the autonomic nervous system, or extreme body temperatures). II. Diagnostic Confirmation: Are you sure your patient has Sick Sinus Syndrome? The diagnosis is made electrocardiographically. SND may manifest with atrial bradyarrhythmias, tachyarrhythmias, or a combination of the two. It is commonly accepted that ambulatory sinus pauses of greater than 3 seconds in duration are rare and may indicate SND. Sinus pauses may be due to either sinus arrest or sinus exit block. Other rhythms associated with or indicative of SND are severe sinus bradycardia, paroxysmal atrial tachyarrhythmias (classically with associated bradyarrhythmias with or without posttachyarrhythmia conversion pauses, the so-called tach-brady syndrome), and chronic atrial fibrillation with slow ventricular rates in the absence of AV nodal blocking pharmacologic therapy). Chronotropic incompetence is another manifestation of SND. It is the inability to increase heart rate with exercise. There exist varying quantitative criteria for defining chronotropic incompetence. One is the inability to achieve a maximal exercise heart rate of 100 bpm. Another is the inability to achieve 80% of the predicted maximal heart rate when adjusted for age. SND may first manifest after initiation of antiarrhythmic drugs. Sometimes the SND is solely due to the drug but other times may unmask or exacerbate intrinsic SND. If the first manifestation of the SND is with pharmacologic therapy that may be discontinued or there exists an alternative drug, cessation of the drug and observation with prolonged monitoring may be appropriate to differentiate between intrinsic and extrinsic SND. Usually there is underlying (intrinsic) physiologic substrate for SND if pharmacologic therapy causes SND, though it might not be clinically apparent or significant. Symptoms may or may not be present. The absence or presence of symptoms is not necessary for the diagnosis of SND, though it is the determinant of therapeutic recommendations. Extrinsic causes are addressed with assessment of medications, blood tests (for thyroid abnormalities), and evaluation for associated autonomic dysfunction, if suspected. A. History Part I: Pattern Recognition: The typical patient with SND presents with arrhythmias detected via electrocardiogram (ECG), ambulatory monitoring 24-hour recording, prolonged electrocardiographic event monitoring, or outpatient telemetry recordings (see diagnostic tests below). The recordings may have been prompted as a part of the routine exam (ECG), or in the case of monitoring, prompted by findings found on a 12-lead ECG or by symptoms. Symptoms are diverse, corresponding to a broad range of electrocardiographic manifestations. They may include palpitations (due to PACs, paroxysmal atrial tachycardias, or atrial fibrillation), fatigue (from bradycardia or chronotropic incompetence), light-headedness, or syncope (from pauses). More unusual symptoms may include cognitive abnormalities, periodic polyuria (related to atrial distention with release of atrial natriuretic peptide experienced with or immediately after paroxysmal atrial tachycardia or atrial fibrillation), and thromboembolic phenomena (from bradycardia-induced hypoperfusion of a stenotic vessel or embolic related to atrial fibrillation). As many times symptoms are present without electrocardiographic findings and nonspecific electrocardiographic findings present without symptoms, the correlation of electrocardiographic abnormalities with symptoms is paramount in establishing a causal relationship to best determine the most appropriate therapeutic approach. B. History Part 2: Prevalence: The prevalence of SND is not known because so many patients are asymptomatic. In addition, the criteria for defining a heart rate or pause as abnormally slow are more dependent on the association of symptoms than an arbitrary number. SND is usually diagnosed in the seventh and eighth decades of life, with an average age of 65 in a few studies. There are rare familial cases of SND that may manifest in younger patients, some of which may be associated with long Q–T syndrome. C. History Part 3: Competing diagnoses that can mimic Sick Sinus Syndrome. The differential diagnosis of SND includes chronically high vagal tone [usually manifest as chronic sinus bradycardia, which may be marked, or sinus pauses during sleep, which may be accompanied by varying degrees of second-degree atrioventricular (AV) block (with or without AV dissociation)]. These patients are asymptomatic, are often young, and are usually in excellent physical condition and athletic. Thyroid disease must be ruled out because it requires specific treatment, which generally results in resolution of the arrhythmia problem. D. Physical Examination Findings. Physical examination is rarely helpful in establishing a diagnosis. A resting bradycardia on examination is usually not associated with symptoms in patients with SND. E. What diagnostic tests should be performed? Correlation of diagnostic electrocardiographic abnormalities is what is necessary to best guide management decisions. The resting electrocardiogram is usually unrevealing or demonstrates nondiagnostic abnormalities without symptoms. The next step should be to obtain ambulatory monitoring. This may be a 24-hour test (which has a very low yield but may be mandated by the insurer prior to longer term monitoring). Interestingly, up to 15% of patients will experience symptoms without arrhythmias, providing equally useful information. If the 24-hour monitoring is unrevealing, or if symptoms occur on a less frequent than daily basis, then a prolonged monitor with an event recorder or outpatient telemetry is warranted. Cardiac event recorders in a well-defined population (recurrent syncope and/or presyncope within a 1-month period) may be very useful. The yield may be as high as 25%. Symptoms may be associated with either tachyarrhythmias, bradyarrhythmias, or both. If symptoms are less frequent or the test unrevealing, an implantable loop recorder is a diagnostic option. The device is activated to store recordings either automatically for detected significant brady or tachyarrhythmias, or patient activated with symptoms. If the presenting symptom is syncope in a patient who is suspected of having SND with an otherwise unrevealing workup, then consideration should be given to invasive testing with an intracardiac electrophysiology test. This is an insensitive but specific test. The sinus node recovery time (SNRT) is usually determined by pacing the atrium at decremental cycle lengths from 600 to 350 msec and measuring the return interval, which is then corrected for the patient’s resting heart rate. Corrected values under 525 msec are regarded as normal and greater than 1 second as markedly abnormal. A secondary pause (pause occurring after the first return sinus complex) is a more sensitive indicator of SND. The positive yield of EP testing is approximately 5%. The test is highly insensitive, and a negative test does not rule out sinus node disease. Rarely induction of clinically relevant tachyarrhythmias is seen. 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? Laboratory studies indicated in patients presenting with symptoms and suspected of possibly having SND (with or without electrocardiographic abnormalities at the time of initial visit) should include a complete blood count, electrolytes, renal function tests, liver function tests, and serum thyroid studies. These tests are performed to diagnose secondary causes of SND or causes of the presenting symptoms, rather than making a diagnosis of intrinsic SND. 2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted? In patients with significant neurologic symptoms, a radiologic test of the head (CT or MRI) may be indicated, as intracranial processes resulting in increased intracranial pressure may rarely be a cause of bradycardia. An echocardiogram may be useful in determining if there is presence of structural heart disease. This will be useful information if institution of antiarrhythmic drug therapy is considered. The presence of structural heart disease increases the risk of proarrhythmia and will be important in determining which drug is safest. In addition, arrhythmias associated with SND, especially atrial fibrillation, may result in atrial remodeling, leading to dilatation, which may facilitate the formation of an arrhythmia itself. Left ventricular function should be assessed. Rarely, extrinsic pressure on the heart by either an intracardiac or mediastinal mass found on echocardiography may result in arrhythmias thought to be due to SND. Management of the patient is determined by the results of diagnostic testing and more importantly the correlation of results with the presence or absence of symptoms. Patients with symptomatic SND and bradyarrhythmias usually are indicated for permanent pacemaker therapy. Dual-chamber pacing versus single ventricular pacing has been shown to result in less atrial fibrillation, stroke, and congestive heart failure, but no difference in mortality. Ventricular pacing even in the DDDR mode can promote heart failure, and newer pacemakers employ algorithms that favor maximizing atrial pacing, with back-up dual chamber pacing. These algorithms should not be programmed in patients with preexisting AV nodal disease (approximately 15% of patients with SND). An exception to the requirement for symptoms is the patient with concomitant severe bradyarrhythmias, who has tachyarrhythmias requiring treatment with drugs that might worsen the bradyarrhythmias. Another exception to the requirement for symptoms is the patient with a history of myocardial infarction requiring beta-blocker therapy as part of post-MI care, who has sinus bradycardia. Patients with atrial tachyarrhythmias and rapid ventricular responses should receive AV nodal blocking therapy with a beta-blocker, or if contraindicated, verapamil or diltiazem. Antiarrhythmic drug therapy for atrial fibrillation, if indicated, would be determined according to established clinical guidelines. A. Immediate management. Immediate management of SND is rarely required. Intravenous atropine or beta-agonist for symptomatic severe sinus node dysfunction may be warranted. In a hospital or other health care facility setting, external pacing pads may be employed until more definitive treatment is administered (temporary or permanent transvenous pacing). Discontinuation of any possibly offensive cardioactive drug is warranted until pacing therapy is instituted. B. Physical Examination Tips to Guide Management. Patients who receive permanent pacemakers should be monitored for signs and symptoms of congestive heart failure. If heart failure develops, they should then have the pacemaker, if possible (no AV nodal disease present) programmed to minimize ventricular pacing. If left ventricular dysfunction develops as a cause of necessary ventricular pacing, consideration should be given to upgrade the pacemaker to a cardiac resynchronization device. For patients with atrial tachyarrhythmias, one should monitor for signs or symptoms of congestive heart failure or coronary artery disease, which may be related to the tachyarrhythmias or inappropriate ventricular tracking of such arrhythmias in patients with AV block. The pacemaker diagnostic data (giving duration and ventricular rates of tachyarrhythmias) should be used to guide antiarrhythmic drug therapy. C. Laboratory Tests to Monitor Response To, and Adjustments in, Management. If the SND is extrinsic and due to thyroid disease, periodic assessment of thyroid function tests is indicated. Regular periodic measurement of INR indicated for those patients with atrial fibrillation/flutter indicated warfarin for thromboemboli prophylaxis. INR should be 2.0 to 3.0 in patients without mechanical valves and 2.5 to 3.5 in those with mechanical valves. D. Long-term management. The patient with clinically significant bradyarrhythmias, tachyarrhythmias, or both, regardless of having received a permanent pacemaker requires long-term follow-up. If a pacemaker is not implanted, clinical reevaluation is warranted to check for progression of bradycardia and/or symptoms, progression of sinus node disease to involve AV nodal or infranodal disease (which might signal a change of medications or pacemaker prescriptive therapy). In addition, patients with SND often have concomitant (or develop later) hypertension and/or coronary artery disease, and if present would be diagnoses that also require long-term follow-up. Pacemaker evaluation should include interrogation of arrhythmia logs (looking for atrial and ventricular tachyarrhythmias), percentage of ventricular pacing, rate histograms, and automatic threshold checks performed by the pacemaker. Patients indicated for long-term antithrombotic therapy for atrial tachyarrhythmias should be evaluated for signs and symptoms of bleeding, and have regular INR checks if taking warfarin. E. Common Pitfalls and Side-Effects of Management The most common pitfall in patients being evaluated for SND is treating bradycardia during sleep, asymptomatic sinus bradycardia unassociated with atrial tachyarrhythmias not requiring antiarrhythmic drug therapy, bradycardia associated with high vagal tone, or vagally mediated events with pacemaker therapy. The correlation of symptoms with arrhythmias is a cornerstone of evaluation in the vast majority of patients. Side effects of antiarrhythmic drug therapy may include worsening of sinus node dysfunction or conduction below the sinus node, ventricular arrhythmias in patients with structural heart disease or noncardiac side effects and toxicities related to the specific drug. Pacemaker therapy may be complicated by heart failure with ventricular pacing (see above). Pacemaker function needs to be checked as per established guidelines. IV. Management with Co-Morbidities Patients with concomitant sinus node dysfunction and significant aortic valve stenosis who receive pacemakers should have their pacemakers programmed carefully to avoid elevated heart rates via the rate response mode to avoid heart failure, angina, syncope, or severe ventricular arrhythmias related to hemodynamic causes (personal experience). Patients with coronary artery disease who require beta-blocker therapy may have had their drug discontinued when SND was diagnosed. If this was the case and a pacemaker is implanted, the drug should be reinstituted. This is almost always possible to do. If a patient is on a class 1C antiarrhythmic drug and develops coronary artery disease, the drug should be discontinued. If the patient is on a type 1C drug or dronedarone and develops atrial fibrillation that is deemed to be chronic, or congestive heart failure, it should be discontinued. Patients on amiodarone should have liver and thyroid function tests, a chest radiograph, pulmonary function tests with diffusion capacity, and eye examinations as per established guidelines. V. Patient Safety and Quality Measures A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. Patients experiencing presyncope, syncope, chest pain, and/or shortness of breath should be instructed to alert their physician, as these symptoms may represent worsening arrhythmia status, the development of concomitant coronary artery disease, or congestive heart failure, or adverse effects from medication or pacemaker programmed parameters. B. What's the Evidence for specific management and treatment recommendations? “ACC/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities”. Circulation. vol. 117. 2008. pp. e350 “2011 ACCF/ACC/HRS focused update of the management of patients with atrial fibrillation”. C. DRG Codes and Expected Length of Stay. sick sinus syndrome: 149.5 other specified cardiac arrhythmias: 149.8 supraventricular tachycardia: 147.1 atrial fibrillation: 148.0 atrial flutter: 148.1 sinus bradycardia: R00.1 tachycardia unspecified: R00.0 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. Sick Sinus Syndrome: What every physician needs to know. - II. Diagnostic Confirmation: Are you sure your patient has Sick Sinus Syndrome? - A. History Part I: Pattern Recognition: - B. History Part 2: Prevalence: - C. History Part 3: Competing diagnoses that can mimic Sick Sinus Syndrome. - 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	8	0	0	0	2	0.899784	2	4,034
Embryonic stem cell Embryonic stem cells (ES cells or ESCs) are pluripotent stem cells derived from the inner cell mass of a blastocyst, an early-stage pre-implantation embryo. Human embryos reach the blastocyst stage 4–5 days post fertilization, at which time they consist of 50–150 cells. Isolating the embryoblast, or inner cell mass (ICM) results in destruction of the blastocyst, a process which raises ethical issues, including whether or not embryos at the pre-implantation stage should have the same moral considerations as embryos in the post-implantation stage of development. Researchers are currently focusing heavily on the therapeutic potential of embryonic stem cells, with clinical use being the goal for many labs. Potential uses include the treatment of diabetes and heart disease. The cells are being studied to be used as clinical therapies, models of genetic disorders, and cellular/DNA repair. However, adverse effects in the research and clinical processes such as tumours and unwanted immune responses have also been reported. - 1 Properties - 2 Utilizations - 3 Concern and controversy - 4 History - 5 Techniques and conditions for derivation and culture - 6 See also - 7 References - 8 External links Embryonic stem cells (ESCs), derived from the blastocyst stage of early mammalian embryos, are distinguished by their ability to differentiate into any cell type and by their ability to propagate. It is these traits that makes them valuable in the scientific and medical fields. ESCs are also described as having a normal karyotype, maintaining high telomerase activity, and exhibiting remarkable long-term proliferative potential. Embryonic stem cells of the inner cell mass are pluripotent, meaning they are able to differentiate to generate primitive ectoderm, which ultimately differentiates during gastrulation into all derivatives of the three primary germ layers: ectoderm, endoderm, and mesoderm. These include each of the more than 220 cell types in the adult human body. Pluripotency distinguishes embryonic stem cells from adult stem cells, which are multipotent and can only produce a limited number of cell types. Under defined conditions, embryonic stem cells are capable of propagating indefinitely in an undifferentiated state. Conditions must either prevent the cells from clumping, or maintain an environment that supports an unspecialized state. While being able to remain undifferentiated, ESCs also have the capacity, when provided with the appropriate signals, to differentiate (presumably via the initial formation of precursor cells) into nearly all mature cell phenotypes. Due to their plasticity and potentially unlimited capacity for self-renewal, embryonic stem cell therapies have been proposed for regenerative medicine and tissue replacement after injury or disease. Pluripotent stem cells have shown potential in treating a number of varying conditions, including but not limited to: spinal cord injuries, age related macular degeneration, diabetes, neurodegenerative disorders (such as Parkinson's disease), AIDS, etc. In addition to their potential in regenerative medicine, embryonic stem cells provide an alternative source of tissue/organs which serves as a possible solution to the donor shortage dilemma. Not only that, but tissue/organs derived from ESCs can be made immunocompatible with the recipient. Aside from these uses, embryonic stem cells can also serve as tools for the investigation of early human development, study of genetic disease and as in vitro systems for toxicology testing. According to a 2002 article in PNAS, "Human embryonic stem cells have the potential to differentiate into various cell types, and, thus, may be useful as a source of cells for transplantation or tissue engineering." However, embryonic stem cells are not limited to cell/tissue engineering. Cell replacement therapies Current research focuses on differentiating ESCs into a variety of cell types for eventual use as cell replacement therapies (CRTs). Some of the cell types that have or are currently being developed include cardiomyocytes (CM), neurons, hepatocytes, bone marrow cells, islet cells and endothelial cells. However, the derivation of such cell types from ESCs is not without obstacles, therefore current research is focused on overcoming these barriers. For example, studies are underway to differentiate ESCs in to tissue specific CMs and to eradicate their immature properties that distinguish them from adult CMs. - Researchers have differentiated ESCs into dopamine-producing cells with the hope that these neurons could be used in the treatment of Parkinson’s disease. - ESCs have been differentiated to natural killer (NK) cells and bone tissue. - Studies involving ESCs are underway to provide an alternative treatment for diabetes. For example, D’Amour et al. were able to differentiate ESCs into insulin producing cells and researchers at Harvard University were able to produce large quantities of pancreatic beta cells from ES. - An article published in the European Heart Journal describes a translational process of generating human embryonic stem cell-derived cardiac progenitor cells to be used in clinical trials of patients with severe heart failure. Besides becoming an important alternative to organ transplants, ESCs are also being used in field of toxicology and as cellular screens to uncover new chemical entities (NCEs) that can be developed as small molecule drugs. Studies have shown that cardiomyocytes derived from ESCs are validated in vitro models to test drug responses and predict toxicity profiles. ES derived cardiomyocytes have been shown to respond to pharmacological stimuli and hence can be used to assess cardiotoxicity like Torsades de Pointes. ESC-derived hepatocytes are also useful models that could be used in the preclinical stages of drug discovery. However, the development of hepatocytes from ESCs has proven to be challenging and this hinders the ability to test drug metabolism. Therefore, current research is focusing on establishing fully functional ESC-derived hepatocytes with stable phase I and II enzyme activity. Models of genetic disorder Several new studies have started to address the concept of modeling genetic disorders with embryonic stem cells. Either by genetically manipulating the cells, or more recently, by deriving diseased cell lines identified by prenatal genetic diagnosis (PGD), modeling genetic disorders is something that has been accomplished with stem cells. This approach may very well prove valuable at studying disorders such as Fragile-X syndrome, Cystic fibrosis, and other genetic maladies that have no reliable model system. Yury Verlinsky, a Russian-American medical researcher who specialized in embryo and cellular genetics (genetic cytology), developed prenatal diagnosis testing methods to determine genetic and chromosomal disorders a month and a half earlier than standard amniocentesis. The techniques are now used by many pregnant women and prospective parents, especially couples who have a history of genetic abnormalities or where the woman is over the age of 35 (when the risk of genetically related disorders is higher). In addition, by allowing parents to select an embryo without genetic disorders, they have the potential of saving the lives of siblings that already had similar disorders and diseases using cells from the disease free offspring. Repair of DNA damage Differentiated somatic cells and ES cells use different strategies for dealing with DNA damage. For instance, human foreskin fibroblasts, one type of somatic cell, use non-homologous end joining (NHEJ), an error prone DNA repair process, as the primary pathway for repairing double-strand breaks (DSBs) during all cell cycle stages. Because of its error-prone nature, NHEJ tends to produce mutations in a cell’s clonal descendants. ES cells use a different strategy to deal with DSBs. Because ES cells give rise to all of the cell types of an organism including the cells of the germ line, mutations arising in ES cells due to faulty DNA repair are a more serious problem than in differentiated somatic cells. Consequently, robust mechanisms are needed in ES cells to repair DNA damages accurately, and if repair fails, to remove those cells with un-repaired DNA damages. Thus, mouse ES cells predominantly use high fidelity homologous recombinational repair (HRR) to repair DSBs. This type of repair depends on the interaction of the two sister chromosomes formed during S phase and present together during the G2 phase of the cell cycle. HRR can accurately repair DSBs in one sister chromosome by using intact information from the other sister chromosome. Cells in the G1 phase of the cell cycle (i.e. after metaphase/cell division but prior the next round of replication) have only one copy of each chromosome (i.e. sister chromosomes aren’t present). Mouse ES cells lack a G1 checkpoint and do not undergo cell cycle arrest upon acquiring DNA damage. Rather they undergo programmed cell death (apoptosis) in response to DNA damage. Apoptosis can be used as a fail-safe strategy to remove cells with un-repaired DNA damages in order to avoid mutation and progression to cancer. Consistent with this strategy, mouse ES stem cells have a mutation frequency about 100-fold lower than that of isogenic mouse somatic cells. On January 23, 2009, Phase I clinical trials for transplantation of oligodendrocytes (a cell type of the brain and spinal cord) derived from human ES cells into spinal cord-injured individuals received approval from the U.S. Food and Drug Administration (FDA), marking it the world's first human ES cell human trial. The study leading to this scientific advancement was conducted by Hans Keirstead and colleagues at the University of California, Irvine and supported by Geron Corporation of Menlo Park, CA, founded by Michael D. West, PhD. A previous experiment had shown an improvement in locomotor recovery in spinal cord-injured rats after a 7-day delayed transplantation of human ES cells that had been pushed into an oligodendrocytic lineage. The phase I clinical study was designed to enroll about eight to ten paraplegics who have had their injuries no longer than two weeks before the trial begins, since the cells must be injected before scar tissue is able to form. The researchers emphasized that the injections were not expected to fully cure the patients and restore all mobility. Based on the results of the rodent trials, researchers speculated that restoration of myelin sheathes and an increase in mobility might occur. This first trial was primarily designed to test the safety of these procedures and if everything went well, it was hoped that it would lead to future studies that involve people with more severe disabilities. The trial was put on hold in August 2009 due to FDA concerns regarding a small number of microscopic cysts found in several treated rat models but the hold was lifted on July 30, 2010. In October 2010 researchers enrolled and administered ESTs to the first patient at Shepherd Center in Atlanta. The makers of the stem cell therapy, Geron Corporation, estimated that it would take several months for the stem cells to replicate and for the GRNOPC1 therapy to be evaluated for success or failure. In November 2011 Geron announced it was halting the trial and dropping out of stem cell research for financial reasons, but would continue to monitor existing patients, and was attempting to find a partner that could continue their research. In 2013 BioTime, led by CEO Dr. Michael D. West, acquired all of Geron's stem cell assets, with the stated intention of restarting Geron's embryonic stem cell-based clinical trial for spinal cord injury research. BioTime company Asterias Biotherapeutics (NYSE MKT: AST) was granted a $14.3 million Strategic Partnership Award by the California Institute for Regenerative Medicine (CIRM) to re-initiate the world’s first embryonic stem cell-based human clinical trial, for spinal cord injury. Supported by California public funds, CIRM is the largest funder of stem cell-related research and development in the world. The award provides funding for Asterias to reinitiate clinical development of AST-OPC1 in subjects with spinal cord injury and to expand clinical testing of escalating doses in the target population intended for future pivotal trials. AST-OPC1 is a population of cells derived from human embryonic stem cells (hESCs) that contains oligodendrocyte progenitor cells (OPCs). OPCs and their mature derivatives called oligodendrocytes provide critical functional support for nerve cells in the spinal cord and brain. Asterias recently presented the results from phase 1 clinical trial testing of a low dose of AST-OPC1 in patients with neurologically-complete thoracic spinal cord injury. The results showed that AST-OPC1 was successfully delivered to the injured spinal cord site. Patients followed 2–3 years after AST-OPC1 administration showed no evidence of serious adverse events associated with the cells in detailed follow-up assessments including frequent neurological exams and MRIs. Immune monitoring of subjects through one year post-transplantation showed no evidence of antibody-based or cellular immune responses to AST-OPC1. In four of the five subjects, serial MRI scans performed throughout the 2–3 year follow-up period indicate that reduced spinal cord cavitation may have occurred and that AST-OPC1 may have had some positive effects in reducing spinal cord tissue deterioration. There was no unexpected neurological degeneration or improvement in the five subjects in the trial as evaluated by the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI) exam. The Strategic Partnership III grant from CIRM will provide funding to Asterias to support the next clinical trial of AST-OPC1 in subjects with spinal cord injury, and for Asterias’ product development efforts to refine and scale manufacturing methods to support later-stage trials and eventually commercialization. CIRM funding will be conditional on FDA approval for the trial, completion of a definitive agreement between Asterias and CIRM, and Asterias’ continued progress toward the achievement of certain pre-defined project milestones. Concern and controversy The major concern with the possible transplantation of ESC into patients as therapies is their ability to form tumors including teratoma. Safety issues prompted the FDA to place a hold on the first ESC clinical trial, however no tumors were observed. The main strategy to enhance the safety of ESC for potential clinical use is to differentiate the ESC into specific cell types (e.g. neurons, muscle, liver cells) that have reduced or eliminated ability to cause tumors. Following differentiation, the cells are subjected to sorting by flow cytometry for further purification. ESC are predicted to be inherently safer than IPS cells created with genetically-integrating viral vectors because they are not genetically modified with genes such as c-Myc that are linked to cancer. Nonetheless, ESC express very high levels of the iPS inducing genes and these genes including Myc are essential for ESC self-renewal and pluripotency, and potential strategies to improve safety by eliminating c-Myc expression are unlikely to preserve the cells' "stemness". However, N-myc and L-myc have been identified to induce iPS cells instead of c-myc with similar efficiency. More recent protocols to induce pluripotency bypass these problems completely by using non-integrating RNA viral vectors such as sendai virus or mRNA transfection. Due to the nature of embryonic stem cell research, there are a lot of controversial opinions on the topic. Since harvesting embryonic stem cells necessitates destroying the embryo from which those cells are obtained, the moral status of the embryo comes into question. Some people argue that the 5-day old mass of cells is too young to achieve personhood or that the embryo, if donated from an IVF clinic (which is where labs typically acquire embryos from), would otherwise go to medical waste anyway. Opponents of ESC research counter that any embryo has the potential to become a human, therefore destroying it is murder and the embryo must be protected under the same ethical view as a developed human being. - 1964: Lewis Kleinsmith and G. Barry Pierce Jr. isolated a single type of cell from a teratocarcinoma, a tumor now known to be derived from a germ cell. These cells isolated from the teratocarcinoma replicated and grew in cell culture as a stem cell and are now known as embryonal carcinoma (EC) cells. Although similarities in morphology and differentiating potential (pluripotency) led to the use of EC cells as the in vitro model for early mouse development, EC cells harbor genetic mutations and often abnormal karyotypes that accumulated during the development of the teratocarcinoma. These genetic aberrations further emphasized the need to be able to culture pluripotent cells directly from the inner cell mass. - 1981: Embryonic stem cells (ES cells) were independently first derived from mouse embryos by two groups. Martin Evans and Matthew Kaufman from the Department of Genetics, University of Cambridge published first in July, revealing a new technique for culturing the mouse embryos in the uterus to allow for an increase in cell number, allowing for the derivation of ES cells from these embryos. Gail R. Martin, from the Department of Anatomy, University of California, San Francisco, published her paper in December and coined the term “Embryonic Stem Cell”. She showed that embryos could be cultured in vitro and that ES cells could be derived from these embryos. - 1989: Mario R. Cappechi, Martin J. Evans, and Oliver Smithies publish their research which details their isolation and genetic modifications of embryonic stem cells, creating the first "knockout mice". In creating knockout mice, this publication provided scientists with an entirely new way to study disease. - 1998: A paper titled "Embryonic Stem Cell Lines Derived From Human Blastocysts" is published by a team from the University of Wisconsin, Madison. The researchers behind this study not only create the first embryonic stem cells, but recognize their pluripotency, as well as their capacity for self-renewal. The abstract of the paper notes the significance of the discovery with regards to the fields of developmental biology and drug discovery. - 2001: President George W. Bush allows federal funding to support research on roughly 60—at this time, already existing—lines of embryonic stem cells. Seeing as the limited lines that Bush allowed research on had already been established, this law supported embryonic stem cell research without raising any ethical questions that could arise with the creation of new lines under federal budget. - 2006: Japanese scientists Shinya Yamanaka and Kazutoshi Takashi publish a paper describing the induction of pluripotent stem cells from cultures of adult mouse fibroblasts. Induced pluripotent stem cells (iPSCs) are a huge discovery, as they are seemingly identical to embryonic stem cells and could be used without sparking the same moral controversy. - January, 2009: The US Food and Drug Administration (FDA) provides approval for Geron Corporation's phase I trial of their human embryonic stem cell-derived treatment for spinal cord injuries. The announcement was met with excitement from the scientific community, but also with wariness from stem cell opposers. The treatment cells were, however, derived from the cell lines approved under George W. Bush's ESC policy. - March, 2009: Executive Order 13505 is signed by President Barack Obama, removing the restrictions put in place on federal funding for human stem cells by the previous presidential administration. This would allow the National Institutes of Health (NIH) to provide funding for hESC research. The document also states that the NIH must provide revised federal funding guidelines within 120 days of the order's signing. Techniques and conditions for derivation and culture Derivation from humans In vitro fertilization generates multiple embryos. The surplus of embryos is not clinically used or is unsuitable for implantation into the patient, and therefore may be donated by the donor with consent. Human embryonic stem cells can be derived from these donated embryos or additionally they can also be extracted from cloned embryos using a cell from a patient and a donated egg. The inner cell mass (cells of interest), from the blastocyst stage of the embryo, is separated from the trophectoderm, the cells that would differentiate into extra-embryonic tissue. Immunosurgery, the process in which antibodies are bound to the trophectoderm and removed by another solution, and mechanical dissection are performed to achieve separation. The resulting inner cell mass cells are plated onto cells that will supply support. The inner cell mass cells attach and expand further to form a human embryonic cell line, which are undifferentiated. These cells are fed daily and are enzymatically or mechanically separated every four to seven days. For differentiation to occur, the human embryonic stem cell line is removed from the supporting cells to form embryoid bodies, is co-cultured with a serum containing necessary signals, or is grafted in a three-dimensional scaffold to result. Derivation from other animals Embryonic stem cells are derived from the inner cell mass of the early embryo, which are harvested from the donor mother animal. Martin Evans and Matthew Kaufman reported a technique that delays embryo implantation, allowing the inner cell mass to increase. This process includes removing the donor mother's ovaries and dosing her with progesterone, changing the hormone environment, which causes the embryos to remain free in the uterus. After 4–6 days of this intrauterine culture, the embryos are harvested and grown in in vitro culture until the inner cell mass forms “egg cylinder-like structures,” which are dissociated into single cells, and plated on fibroblasts treated with mitomycin-c (to prevent fibroblast mitosis). Clonal cell lines are created by growing up a single cell. Evans and Kaufman showed that the cells grown out from these cultures could form teratomas and embryoid bodies, and differentiate in vitro, all of which indicating that the cells are pluripotent. Gail Martin derived and cultured her ES cells differently. She removed the embryos from the donor mother at approximately 76 hours after copulation and cultured them overnight in a medium containing serum. The following day, she removed the inner cell mass from the late blastocyst using microsurgery. The extracted inner cell mass was cultured on fibroblasts treated with mitomycin-c in a medium containing serum and conditioned by ES cells. After approximately one week, colonies of cells grew out. These cells grew in culture and demonstrated pluripotent characteristics, as demonstrated by the ability to form teratomas, differentiate in vitro, and form embryoid bodies. Martin referred to these cells as ES cells. It is now known that the feeder cells provide leukemia inhibitory factor (LIF) and serum provides bone morphogenetic proteins (BMPs) that are necessary to prevent ES cells from differentiating. These factors are extremely important for the efficiency of deriving ES cells. Furthermore, it has been demonstrated that different mouse strains have different efficiencies for isolating ES cells. Current uses for mouse ES cells include the generation of transgenic mice, including knockout mice. For human treatment, there is a need for patient specific pluripotent cells. Generation of human ES cells is more difficult and faces ethical issues. So, in addition to human ES cell research, many groups are focused on the generation of induced pluripotent stem cells (iPS cells). Potential method for new cell line derivation On August 23, 2006, the online edition of Nature scientific journal published a letter by Dr. Robert Lanza (medical director of Advanced Cell Technology in Worcester, MA) stating that his team had found a way to extract embryonic stem cells without destroying the actual embryo. This technical achievement would potentially enable scientists to work with new lines of embryonic stem cells derived using public funding in the USA, where federal funding was at the time limited to research using embryonic stem cell lines derived prior to August 2001. In March, 2009, the limitation was lifted. Induced pluripotent stem cells The iPSC technology was pioneered by Shinya Yamanaka’s lab in Kyoto, Japan, who showed in 2006 that the introduction of four specific genes encoding transcription factors could convert adult cells into pluripotent stem cells. He was awarded the 2012 Nobel Prize along with Sir John Gurdon "for the discovery that mature cells can be reprogrammed to become pluripotent." In 2007 it was shown that pluripotent stem cells highly similar to embryonic stem cells can be generated by the delivery of three genes (Oct4, Sox2, and Klf4) to differentiated cells. The delivery of these genes "reprograms" differentiated cells into pluripotent stem cells, allowing for the generation of pluripotent stem cells without the embryo. Because ethical concerns regarding embryonic stem cells typically are about their derivation from terminated embryos, it is believed that reprogramming to these "induced pluripotent stem cells" (iPS cells) may be less controversial. Both human and mouse cells can be reprogrammed by this methodology, generating both human pluripotent stem cells and mouse pluripotent stem cells without an embryo. This may enable the generation of patient specific ES cell lines that could potentially be used for cell replacement therapies. In addition, this will allow the generation of ES cell lines from patients with a variety of genetic diseases and will provide invaluable models to study those diseases. However, as a first indication that the induced pluripotent stem cell (iPS) cell technology can in rapid succession lead to new cures, it was used by a research team headed by Rudolf Jaenisch of the Whitehead Institute for Biomedical Research in Cambridge, Massachusetts, to cure mice of sickle cell anemia, as reported by Science journal's online edition on December 6, 2007. On January 16, 2008, a California-based company, Stemagen, announced that they had created the first mature cloned human embryos from single skin cells taken from adults. These embryos can be harvested for patient matching embryonic stem cells. Contamination by reagents used in cell culture The online edition of Nature Medicine published a study on January 24, 2005, which stated that the human embryonic stem cells available for federally funded research are contaminated with non-human molecules from the culture medium used to grow the cells. It is a common technique to use mouse cells and other animal cells to maintain the pluripotency of actively dividing stem cells. The problem was discovered when non-human sialic acid in the growth medium was found to compromise the potential uses of the embryonic stem cells in humans, according to scientists at the University of California, San Diego. However, a study published in the online edition of Lancet Medical Journal on March 8, 2005 detailed information about a new stem cell line that was derived from human embryos under completely cell- and serum-free conditions. After more than 6 months of undifferentiated proliferation, these cells demonstrated the potential to form derivatives of all three embryonic germ layers both in vitro and in teratomas. These properties were also successfully maintained (for more than 30 passages) with the established stem cell lines. - Embryoid body - Embryonic Stem Cell Research Oversight Committees - Fetal tissue implant - Stem cell controversy - Induced stem cells - Thomson; Itskovitz-Eldor, J; Shapiro, SS; Waknitz, MA; Swiergiel, JJ; Marshall, VS; Jones, JM (1998). "Blastocysts Embryonic Stem Cell Lines Derived from Human". Science. 282 (5391): 1145–1147. Bibcode:1998Sci...282.1145T. doi:10.1126/science.282.5391.1145. PMID 9804556. - "NIH Stem Cell Basics. What are embryonic stem cells?". - Baldwing A (2009). "Morality and human embryo research. Introduction to the Talking Point on morality and human embryo research". EMBO Reports. 10 (4): 299–300. doi:10.1038/embor.2009.37. PMC 2672902. PMID 19337297. - Nakaya, Andrea C. (August 1, 2011). Biomedical ethics. San Diego, CA: ReferencePoint Press. p. 96. ISBN 978-1601521576. - "Introduction: What are stem cells, and why are they important?". National Institutes of Health. Retrieved 28 October 2018. - Carla A Herberts; Marcel SG Kwa; Harm PH Hermsen (2011). "Risk factors in the development of stem cell therapy". Journal of Translational Medicine. 9 (29): 29. doi:10.1186/1479-5876-9-29. PMC 3070641. PMID 21418664. - Thomson, J. A.; Itskovitz-Eldor, J; Shapiro, S. S.; Waknitz, M. A.; Swiergiel, J. J.; Marshall, V. S.; Jones, J. M. (1998). "Embryonic Stem Cell Lines Derived from Human Blastocysts". Science. 282 (5391): 1145–7. Bibcode:1998Sci...282.1145T. doi:10.1126/science.282.5391.1145. PMID 9804556. - Ying; Nichols, J; Chambers, I; Smith, A (2003). "BMP Induction of Id Proteins Suppresses Differentiation and Sustains Embryonic Stem Cell Self-Renewal in Collaboration with STAT3". Cell. 115 (3): 281–292. doi:10.1016/S0092-8674(03)00847-X. PMID 14636556. - Mahla, Ranjeet (July 19, 2016). "Stem Cell Applications in Regenerative Medicine and Disease Therapeutics". International Journal of Cell Biology. 2016: 6940283. doi:10.1155/2016/6940283. PMC 4969512. PMID 27516776. - Levenberg, S. (2002). "Endothelial cells derived from human embryonic stem cells". Proceedings of the National Academy of Sciences. 99 (7): 4391–4396. Bibcode:2002PNAS...99.4391L. doi:10.1073/pnas.032074999. PMC 123658. PMID 11917100. - Davila, JC; Cezar, GG; Thiede, M; Strom, S; Miki, T; Trosko, J (2004). "Use and application of stem cells in toxicology". Toxicological Sciences. 79 (2): 214–23. doi:10.1093/toxsci/kfh100. PMID 15014205. - Siu, CW; Moore, JC; Li, RA (2007). "Human embryonic stem cell-derived cardiomyocytes for heart therapies". Cardiovascular & Hematological Disorders Drug Targets. 7 (2): 145–52. doi:10.2174/187152907780830851. PMID 17584049. - Perrier, A. L. (2004). "Derivation of midbrain dopamine neurons from human embryonic stem cells". Proceedings of the National Academy of Sciences. 101 (34): 12543–12548. Bibcode:2004PNAS..10112543P. doi:10.1073/pnas.0404700101. PMC 515094. PMID 15310843. - Parish, CL; Arenas, E (2007). "Stem-cell-based strategies for the treatment of Parkinson's disease". Neuro-Degenerative Diseases. 4 (4): 339–47. doi:10.1159/000101892. PMID 17627139. - Waese, EY; Kandel, RA; Stanford, WL (2008). "Application of stem cells in bone repair". Skeletal Radiology. 37 (7): 601–8. doi:10.1007/s00256-007-0438-8. PMID 18193216. - d'Amour, KA; Bang, AG; Eliazer, S; Kelly, OG; Agulnick, AD; Smart, NG; Moorman, MA; Kroon, E; Carpenter, MK; Baetge, EE (2006). "Production of pancreatic hormone-expressing endocrine cells from human embryonic stem cells". Nature Biotechnology. 24 (11): 1392–401. doi:10.1038/nbt1259. PMID 17053790. - Colen, B.D. (9 October 2014) Giant leap against diabetes The Harvard Gazette, Retrieved 24 November 2014 - Menasché, Phillip; Vanneaux, Valérie; Fabreguettes, Jean-Roch; Bel, Alain; Tosca, Lucie; Garcia, Sylvie (21 March 2015). "Towards a clinical use of human embryonic stem cell derived-cardiac progenitors: a translational experience". European Heart Journal. 36 (12): 743–750. doi:10.1093/eurheartj/ehu192. PMID 24835485. - Jensen, J; Hyllner, J; Björquist, P (2009). "Human embryonic stem cell technologies and drug discovery". Journal of Cellular Physiology. 219 (3): 513–9. doi:10.1002/jcp.21732. PMID 19277978. - Söderdahl, T; Küppers-Munther, B; Heins, N; Edsbagge, J; Björquist, P; Cotgreave, I; Jernström, B (2007). "Glutathione transferases in hepatocyte-like cells derived from human embryonic stem cells". Toxicology in Vitro. 21 (5): 929–37. doi:10.1016/j.tiv.2007.01.021. PMID 17346923. - "Dr. Yury Verlinsky, 1943–2009: Expert in reproductive technology" Chicago Tribune, July 20, 2009 - Mao Z, Bozzella M, Seluanov A, Gorbunova V (September 2008). "DNA repair by nonhomologous end joining and homologous recombination during cell cycle in human cells". Cell Cycle. 7 (18): 2902–6. doi:10.4161/cc.7.18.6679. PMC 2754209. PMID 18769152. - Tichy ED, Pillai R, Deng L, et al. (November 2010). "Mouse embryonic stem cells, but not somatic cells, predominantly use homologous recombination to repair double-strand DNA breaks". Stem Cells Dev. 19 (11): 1699–711. doi:10.1089/scd.2010.0058. PMC 3128311. PMID 20446816. - Hong Y, Stambrook PJ (October 2004). "Restoration of an absent G1 arrest and protection from apoptosis in embryonic stem cells after ionizing radiation". Proc. Natl. Acad. Sci. U.S.A. 101 (40): 14443–8. Bibcode:2004PNAS..10114443H. doi:10.1073/pnas.0401346101. PMC 521944. PMID 15452351. - Aladjem MI, Spike BT, Rodewald LW, et al. (January 1998). "ES cells do not activate p53-dependent stress responses and undergo p53-independent apoptosis in response to DNA damage". Curr. Biol. 8 (3): 145–55. doi:10.1016/S0960-9822(98)70061-2. PMID 9443911. - Bernstein C, Bernstein H, Payne CM, Garewal H (June 2002). "DNA repair/pro-apoptotic dual-role proteins in five major DNA repair pathways: fail-safe protection against carcinogenesis". Mutat. Res. 511 (2): 145–78. doi:10.1016/S1383-5742(02)00009-1. PMID 12052432. - Cervantes RB, Stringer JR, Shao C, Tischfield JA, Stambrook PJ (March 2002). "Embryonic stem cells and somatic cells differ in mutation frequency and type". Proc. Natl. Acad. Sci. U.S.A. 99 (6): 3586–90. Bibcode:2002PNAS...99.3586C. doi:10.1073/pnas.062527199. PMC 122567. PMID 11891338. - "FDA approves human embryonic stem cell study - CNN.com". January 23, 2009. Retrieved May 1, 2010. - Keirstead HS, Nistor G, Bernal G, et al. (2005). "Human embryonic stem cell-derived oligodendrocyte progenitor cell transplants remyelinate and restore locomotion after spinal cord injury". J. Neurosci. 25 (19): 4694–705. doi:10.1523/JNEUROSCI.0311-05.2005. PMID 15888645. - Reinberg, Steven (2009-01-23) FDA OKs 1st Embryonic Stem Cell Trial. The Washington Post - Geron comments on FDA hold on spinal cord injury trial. geron.com (August 27, 2009) - Vergano, Dan (11 October 2010). "Embryonic stem cells used on patient for first time". USA Today. Retrieved 12 October 2010. - Brown, Eryn (November 15, 2011). "Geron exits stem cell research". LA Times. Retrieved 2011-11-15. - "Great news: BioTime Subsidiary Asterias Acquires Geron Embryonic Stem Cell Program". iPScell.com. October 1, 2013. - California Institute of Regenerative Medicine. BioTime, Inc. - Knoepfler, Paul S. (2009). "Deconstructing Stem Cell Tumorigenicity: A Roadmap to Safe Regenerative Medicine". Stem Cells. 27 (5): 1050–6. doi:10.1002/stem.37. PMC 2733374. PMID 19415771. - Varlakhanova, Natalia V.; Cotterman, Rebecca F.; Devries, Wilhelmine N.; Morgan, Judy; Donahue, Leah Rae; Murray, Stephen; Knowles, Barbara B.; Knoepfler, Paul S. (2010). "Myc maintains embryonic stem cell pluripotency and self-renewal". Differentiation. 80 (1): 9–19. doi:10.1016/j.diff.2010.05.001. PMC 2916696. PMID 20537458. - Wernig, Marius; Meissner, Alexander; Cassady, John P; Jaenisch, Rudolf (2008). "C-Myc is Dispensable for Direct Reprogramming of Mouse Fibroblasts". Cell Stem Cell. 2 (1): 10–2. doi:10.1016/j.stem.2007.12.001. PMID 18371415. - King, Nancy; Perrin, Jacob (July 7, 2014). "Ethical issues in stem cell research and therapy". Stem Cell Research and Therapy. 5 (4): 85. doi:10.1186/scrt474. - Kleinsmith LJ, Pierce GB Jr (1964). "Multipotentiality of Single Embryoncal Carcinoma Cells". Cancer Res. 24: 1544–51. PMID 14234000. - Andrews P, Matin M, Bahrami A, Damjanov I, Gokhale P, Draper J (2005). "Embryonic stem (ES) cells and embryonal carcinoma (EC) cells: opposite sides of the same coin" (PDF). Biochem Soc Trans. 33 (Pt 6): 1526–30. doi:10.1042/BST20051526. PMID 16246161. - Martin GR (1980). "Teratocarcinomas and mammalian embryogenesis". Science. 209 (4458): 768–76. Bibcode:1980Sci...209..768M. doi:10.1126/science.6250214. PMID 6250214. - Evans M, Kaufman M (1981). "Establishment in culture of pluripotent cells from mouse embryos". Nature. 292 (5819): 154–6. Bibcode:1981Natur.292..154E. doi:10.1038/292154a0. PMID 7242681. - Martin G (1981). "Isolation of a pluripotent cell line from early mouse embryos cultured in medium conditioned by teratocarcinoma stem cells". Proc Natl Acad Sci USA. 78 (12): 7634–8. Bibcode:1981PNAS...78.7634M. doi:10.1073/pnas.78.12.7634. PMC 349323. PMID 6950406. - "The 2007 Nobel Prize in Physiology or Medicine - Advanced Information". Nobel Prize. Nobel Media. - Thompson, James A.; Itskovitz-Eldor, Joseph; Shapiro, Sander S.; Waknitz, Michelle A.; Swiergiel, Jennifer J.; Marshall, Vivienne S.; Jones, Jeffrey M. (6 November 1998). "Embryonic Stem Cell Lines Derived From Human Blastocyst". Science. 282 (5391): 1145–1147. Bibcode:1998Sci...282.1145T. doi:10.1126/science.282.5391.1145. - "President George W. Bush's address on stem cell research". CNN Inside Politics. CNN. Aug 9, 2001. - Yamanaka, Shinya; Takahashi, Kazutoshi (25 Aug 2006). "Induction of Pluripotent Stem Cells From Mouse Embryonic and Adult Fibroblast Cultures by Defined Factors". Cell. 126 (4): 663–676. doi:10.1016/j.cell.2006.07.024. PMID 16904174. - Wadman, Meredith (27 January 2009). "Stem cells ready for primetime". Nature. Nature. 457 (7229): 516. doi:10.1038/457516a. PMID 19177087. - "Executive Order 13505—Removing Barriers To Responsible Scientific Research Involving Human Stem Cells" (PDF). Federal Register: Presidential Documents. 74 (46). 11 March 2009. - Mountford, JC (2008). "Human embryonic stem cells: origins, characteristics and potential for regenerative therapy". Transfus Med. 18 (1): 1–12. doi:10.1111/j.1365-3148.2007.00807.x. PMID 18279188. - Thomson JA, Itskovitz-Eldor J, Shapiro SS, Waknitz MA, Swiergiel JJ, Marshall VS, Jones JM (1998). "Embryonic stem cell lines derived from human blastocysts". Science. 282 (5391): 1145–1147. Bibcode:1998Sci...282.1145T. doi:10.1126/science.282.5391.1145. PMID 9804556. - Smith AG, Heath JK, Donaldson DD, Wong GG, Moreau J, Stahl M, Rogers D (1988). "Inhibition of pluripotential embryonic stem cell differentiation by purified polypeptides". Nature. 336 (6200): 688–690. Bibcode:1988Natur.336..688S. doi:10.1038/336688a0. PMID 3143917. - Williams RL, Hilton DJ, Pease S, Willson TA, Stewart CL, Gearing DP, Wagner EF, Metcalf D, Nicola NA, Gough NM (1988). "Myeloid leukaemia inhibitory factor maintains the developmental potential of embryonic stem cells". Nature. 336 (6200): 684–687. Bibcode:1988Natur.336..684W. doi:10.1038/336684a0. PMID 3143916. - Ledermann B, Bürki K (1991). "Establishment of a germ-line competent C57BL/6 embryonic stem cell line". Exp Cell Res. 197 (2): 254–258. doi:10.1016/0014-4827(91)90430-3. PMID 1959560. - Takahashi K, Tanabe K, Ohnuki M, Narita M, Ichisaka T, Tomoda K, Yamanaka S (2007). "Induction of pluripotent stem cells from adult human fibroblasts by defined factors". Cell. 131 (5): 861–872. doi:10.1016/j.cell.2007.11.019. PMID 18035408. - Klimanskaya I, Chung Y, Becker S, Lu SJ, Lanza R (2006). "Human embryonic stem cell lines derived from single blastomeres". Nature. 444 (7118): 481–5. Bibcode:2006Natur.444..481K. doi:10.1038/nature05142. PMID 16929302. - US scientists relieved as Obama lifts ban on stem cell research, The Guardian, 10 March 2009 - Takahashi, K; Yamanaka, S (2006). "Induction of pluripotent stem cells from mouse embryonic and adult fibroblast cultures by defined factors". Cell. 126 (4): 663–76. doi:10.1016/j.cell.2006.07.024. PMID 16904174. - "The Nobel Prize in Physiology or Medicine – 2012 Press Release". Nobel Media AB. 8 October 2012. - Wernig, Marius; Meissner, Alexander; Foreman, Ruth; Brambrink, Tobias; Ku, Manching; Hochedlinger, Konrad; Bernstein, Bradley E.; Jaenisch, Rudolf (2007-07-19). "In vitro reprogramming of fibroblasts into a pluripotent ES-cell-like state". Nature. 448 (7151): 318–324. Bibcode:2007Natur.448..318W. doi:10.1038/nature05944. ISSN 1476-4687. PMID 17554336. - "Embryonic stem cells made without embryos". Reuters. 2007-11-21. - Weiss, Rick (2007-12-07). "Scientists Cure Mice Of Sickle Cell Using Stem Cell Technique: New Approach Is From Skin, Not Embryos". The Washington Post. pp. A02. - Hanna, J.; Wernig, M.; Markoulaki, S.; Sun, C.-W.; Meissner, A.; Cassady, J. P.; Beard, C.; Brambrink, T.; Wu, L.-C.; Townes, T. M.; Jaenisch, R. (2007). "Treatment of Sickle Cell Anemia Mouse Model with iPS Cells Generated from Autologous Skin". Science. 318 (5858): 1920–3. Bibcode:2007Sci...318.1920H. doi:10.1126/science.1152092. PMID 18063756. - Helen Briggs (2008-01-17). "US team makes embryo clone of men". BBC. pp. A01. - Ebert, Jessica (24 January 2005). "Human stem cells trigger immune attack". Nature News. London: Nature Publishing Group. doi:10.1038/news050124-1. Archived from the original on 2010-09-24. Retrieved 2009-02-27. - Martin MJ, Muotri A, Gage F, Varki A (2005). "Human embryonic stem cells express an immunogenic nonhuman sialic acid". Nat. Med. 11 (2): 228–32. doi:10.1038/nm1181. PMID 15685172. - Klimanskaya I, Chung Y, Meisner L, Johnson J, West MD, Lanza R (2005). "Human embryonic stem cells derived without feeder cells". Lancet. 365 (9471): 1636–41. doi:10.1016/S0140-6736(05)66473-2. PMID 15885296. \|Wikimedia Commons has media related to Embryonic stem cells.\| - Understanding Stem Cells: A View of the Science and Issues from the National Academies - National Institutes of Health - University of Oxford practical workshop on pluripotent stem cell technology - Fact sheet on embryonic stem cells - Fact sheet on ethical issues in embryonic stem cell research - Information & Alternatives to Embryonic Stem Cell Research - A blog focusing specifically on ES cells and iPS cells including research, biotech, and patient-oriented issues	2	55	11	0	4	2	0.352422	17	10,395
Read medical definition of Dactylitis Dactylitis Dactylitis, or “sausage digits,” refers to inflammation/swelling of an entire finger or toe. It occurs due to inflammation of the small joints and enthesitis of the surrounding tendons. Dactylitis is another distinguishing indicator of psoriatic arthritis. Usually Introduction: This information shows the various causes of Dactylitis, and how common these diseases or conditions are in the general population.This is not a direct indication as to how commonly these diseases are the actual cause of Dactylitis, but gives a relative idea as to how frequent these diseases are seen overall. People with psoriatic arthritis can have swollen fingers or toes. This is known as dactylitis (dak-till-eye-tus), or sausage digit, to describe how whole fingers or toes swell up. It most commonly affects one or two fingers or toes at a time. It can also cause Alphabetical Index Use the alphabetical index for the main term dactylitis to review the available sub terms and properly select the ICD-10 code with the highest degree of specificity. Instructional notations will guide the coder with information such as “see”, “see Objective Biologic agents with different mechanisms of actions (inhibitors of TNF-α, interleukin-12/23 and interleukin-17) showed efficacy in randomized controlled trials in the treatment of psoriatic arthritis. We aimed to conduct a pooled meta-analysis of those Swollen, sausage-like fingers and toes, called dactylitis, are a hallmark of psoriatic arthritis. Unlike other types of arthritis, psoriatic arthritis tends to make your entire finger or toe Dactylitis (the sausage-like swelling of the toes and fingers) Reduced range of motion of the low back, spine, and pelvis Psoriasis Chronic fatigue Osteoporosis Endocarditis (inflammation of the heart valve) Uveitis (inflammation of the pigmented part of the eye) People with USpA have symptoms and disease features consistent with spondyloarthritis, but their disease doesn’t fit into another category of SpA. For example, an adult may have iritis, heel pain (enthesitis), and knee swelling, WITHOUT back pain, psoriasis, a Sickle cell disease is an inherited disease caused by defects, called mutations, in the beta globin gene that helps make hemoglobin.Normally, hemoglobin in red blood cells takes up oxygen in the lungs and carries it through the arteries to all the cells in the tissues Synonyme: Dactylitis, Fingerentzündung, Zehenentzündung Englisch: dactylitis Definition Als Daktylitis bezeichnet man die Entzündung eines Fingers oder einer Zehe. Sie ist ein typisches Symptom der seronegativen Spondylarthritiden (z.B. Psoriasis-Arthritis, ) Dactylitis – Symptoms, Causes, Treatment, Pictures What is Dactylitis? This disorder is also known or denoted as “sausage digit”. This is a disorder where the toes or fingers become inflamed. Tissue swelling also accompanies the inflammation. The name of The medical name for swollen, puffy fingers is dactylitis and the condition is also referred to as sausage fingers. Because we use our fingers so much during the day, they are prone to injury, infection, or strain. This can result in your fingers becoming bloated and Dactylitis or ‘sausage-like’ digit is a characteristic and highly specific manifestation of SpA (Figs 1 and 2) .It totally differs from other digit diseases called dactylitis .Even though more frequent in PsA [3, 4], SpA dactylitis has been observed in all types of SpA, including the undifferentiated ones .]. Jeffrey R. Carlson, MD Dactylitis is a symptom that is most often seen in patients who have inflammatory Psoriatic or Rheumatoid arthritis, which are auto-immune diseases. It is also known as “Sausage Finger” or “Sausage Toe” because of the localized, painful Summary: Dactylitis is reported only by a few people who take Vitamin B12. We study 57,122 people who have side effects while taking Vitamin b12 from Food and Drug Administration (FDA). Among them, 1 has Dactylitis. Find out below who they are, when they Having dactylitis or “sausage fingers” can be both embarrassing and painful. The dactylitis definition is simply the inflammation or swelling of the fingers or toes. It can also have a debilitating effect as you may not be able to bend or move your digits as Background/Purpose: Dactylitis is one of the most commonly reported features in spondyloarthritis. It has been hypothesized that dactylitis is a functional enthesitis at the proximal interphalangeal joints, resulting in synovitis, tenosynovitis, bone and soft tissue In 2018, thalidomide was started for unsightly cutaneous sarcoids on the face and hands. This treatment was discontinued in March 2019 due to hepatitis. The cutaneous sarcoids became painful, especially on the fingers with progressive dactylitis (Figure 1). Treating Dactylitis The treatment of Dactylitis mostly depends on the underlying condition causing it. Your doctor may recommend medications based on the underlying conditions such as people suffering from any type of arthritis may require NSAIDs, DMARDs, corticosteroids to ease pain and inflammation as well as prevent further joint damage. Related information Helpline Call our helpline – 0800 5200 520. Our advisors aim to bring all of the information and advice about arthritis to provide tailored support for you. Call us for free today (Monday-Friday, 9am-8pm). Order our booklets Order or download from dactylitis (uncountable) English Wikipedia has an article on: dactylitis Wikipedia () Inflammation of an entire digit (a finger or toe). Synonyms sausage digit Derived terms tuberculous dactylitis Related terms dactylitic Retrieved from “https://en.wiktionary” : Dactylitis is associated with joint erosions. Enthesitis is associated with worse disease & radiographic damage in psoriatic arthritis. We diagnosed her with PsA on the basis of inflammatory joint pain, a current history of psoriasis, nail dystrophy typical of Strep and Staph can cause numerous infections in children, several of them with skin manifestations. Blistering distal dactylitis is one. Infectious diseases seem to be omnipresent in the Ped ED and, thus, have their own dedicated Category of the Blistering dactylitis is treated with oral antibiotics. If dog bites are involved, the chosen antibiotic should have coverage against components of dogs’ oral flora, which can usually be achieved by β-lactams, or amoxicillin-clavulanic acid. A rabies vaccination is also 乾癬性關節炎（英語：psoriatic arthritis 或簡稱為乾癬關節炎英語：PsA）是一種長期慢性的發炎性關節炎（英语：inflammatory arthritis），發生在患有乾癬（一種自體免疫性疾病）的病人身上。乾癬關節炎的典型特徵是整個手指和腳趾腫脹（英语：dactylitis Case A man, 74 years of age, presented with dactylitis on the first, second and fourth digits of his left hand, characterised by glossy erythema, oedema, pustules and yellowish scales. Additionally, there was onychodystrophy, subungual hyperkeratosis, almost Dactylitis and diagnosis A woman I know is an educated patient who has worked hard to make certain she is diagnosed and treated appropriately. Recently, she showed pictures of swollen fingers to her rheumatologist and had her diagnosis changed again. Her · PDF 檔案 Clinical and Experimental Rheumatology 2018 881 Dactylitis in early spondyloarthritis / M.I. TévarSnchez et al. SpA, and a good response to non-steroi-dal anti-inflammatory drugs (NSAIDs). The complementary examinations that were included are lab tests of C dactylitis : German – English translations and synonyms (BEOLINGUS Online dictionary, TU Chemnitz) dactylitis : Deutsch – Englisch Übersetzungen und Synonyme (BEOLINGUS Online-Dictionary, TU Chemnitz) A service provided by TU Chemnitz IBS and . Find all the synonyms and alternative words for dactylitis at Synonyms.com, the largest free online thesaurus, antonyms, definitions and translations resource on the web. The ASL fingerspelling provided here is most commonly used for proper names of people and places; it is also used in some languages for concepts for which no sign is available at that moment. 11/3/2020 · Enthesitis, dactylitis, and nail disease are highly prevalent in patients with psoriatic arthritis (PsA), according to results of a systematic review and meta-analysis published in Rheumatology. Extra-articular manifestations in PsA may help inform disease diagnosis, classification, and management Tuberculous dactylitis is an unusual form of osteoarticular tuberculosis involving the short tubular bones of hands and feet, which is uncommon beyond six years of age. We report the case of a fifteen-year-old adolescent boy who was diagnosed with tuberculous Arthritis is commonly seen in the joints of the fingers. The knuckle joints of the fingers are frequently affected in patients with osteoarthritis. Anti-Inflammatory Medications: These medications can help treat the pain of finger arthritis, and also help decrease 24/7/2017 · This video is about Sausage Fingers Nikole Hannah-Jones – Reframing the Legacy of Slavery with “The 1619 Project” \| The Daily Show – Duration: 9:53. The Daily Show with Trevor Noah Recommended 作者: Mackenzie Danielson Some people with PsA develop dactylitis, which can make your fingers or toes swell and give them a sausage-like appearance. In some cases, your eyes can become red and inflamed, or you may have a In patients with psoriatic arthritis (PsA), tumor necrosis factor alpha (TNF-α), anti-interleukin (IL)-12/23, and anti-IL-17 agents demonstrated comparable efficacies in terms of enthesitis and dactylitis resolution,, according to a study published in The Journal of. · PDF 檔案 condition at its inception. PsA has several unique characteristics such as enthesopathy, dactylitis, and abnormal bone remodeling. In particular, dactylitis occurs on the easily observed sites such as Among patients with psoriatic arthritis with pre-existing enthesitis or dactylitis, 90-mg treatments of ixekizumab, administered either every 2 or 4 weeks, significantly improved both conditions, Polyarticular psoriatic arthritis distinguished from rheumatoid arthritis by the presence of dactylitis and the absence of anticyclic citrullinated peptide antibodies. Non-steroidal anti-inflammatory drugs (NSAIDs) usually sufficient to treat limited disease. Patients with Spondylitis may also be associated with other infectious diseases, such as brucellosis, in which the intervertebral disks and the vertebrae are affected and sometimes destroyed; this is one cause of ankylosing spondylitis, a particularly serious variety. A systematic review has shown that TNF inhibitors (TNFi) are equaled in efficacy by other biologic agents (ustekinumab, secukinumab, and ixekizumab) in psoriatic arthritis (PsA) patients with in dactylitis and enthesitis. The literature review analyzed datafrom Sarcoid dactylitis is due to typical non‐caseating granulomas invading the phalanges and the adjacent soft tissue. Blistering distal dactylitis is an infection of the anterior fat pad on the volar surface of the distal portion of a single finger or more rarely a toe, mostly Nail changes due to psoriatic arthritis are very common. Researchers have found that 80 to 90 percent of people with plaque psoriasis experience changes in their nails, and that nail changes are The Leeds Dactylometer provides a means to measure psoriatic disease or other similar dermatological and rheumatological factors. Simple and quick to use. Measures joint circumference. Dactylitis has been defined as “uniform swelling such that the soft Comment dire dactylitis Anglais? Prononciation de dactylitis à 1 prononciation audio, 4 traductions, et de plus pour dactylitis. No single test can confirm a diagnosis of psoriatic arthritis. But some types of tests can rule out other causes of joint pain, such as rheumatoid arthritis or gout. Imaging tests X-rays. Plain X-rays can help pinpoint changes in the joints that occur in psoriatic arthritis · PDF 檔案 dactylitis count of 0, indicating the absence of dactylitis on all 20 digits. statistical analysis Data from PALACE 1, 2 and 3 were pooled in a prespec-ified analysis, permitting a robust analysis of data for all randomised patients with pre-existing enthesitis (base 4/1/2019 · Dactylitis is diffuse swelling of the digits that is usually related to an underlying inflammatory or infiltrative disorder. Psoriatic arthritis (PsA) is the most common severe disease thought to	2	13	0	0	0	1	0.52418	1	3,191
In medicine, myopathy is a disease of the muscle in which the muscle fibers do not function properly. This results in muscular weakness. Myopathy means muscle disease (Greek : myo- muscle + patheia -pathy : suffering). This meaning implies that the primary defect is within the muscle, as opposed to the nerves ("neuropathies" or "neurogenic" disorders) or elsewhere (e.g., the brain). Muscle cramps, stiffness, and spasm can also be associated with myopathy. Signs and symptoms Myopathies in systemic disease results from several different disease processes including endocrine, inflammatory, paraneoplastic, infectious, drug- and toxin-induced, critical illness myopathy, metabolic, collagen related, and myopathies with other systemic disorders. Patients with systemic myopathies often present acutely or sub acutely. On the other hand, familial myopathies or dystrophies generally present in a chronic fashion with exceptions of metabolic myopathies where symptoms on occasion can be precipitated acutely. Most of the inflammatory myopathies can have a chance association with malignant lesion; the incidence appears to be specifically increased only in patients with dermatomyositis. There are many types of myopathy. ICD-10 codes are provided here where available. - (G71.0) Dystrophies (or muscular dystrophies) are a subgroup of myopathies characterized by muscle degeneration and regeneration. Clinically, muscular dystrophies are typically progressive, because the muscles' ability to regenerate is eventually lost, leading to progressive weakness, often leading to use of a wheelchair, and eventually death, usually related to respiratory weakness. - (G71.1) Myotonia - (G71.2) The congenital myopathies do not show evidence for either a progressive dystrophic process (i.e., muscle death) or inflammation, but instead characteristic microscopic changes are seen in association with reduced contractile ability of the muscles. Congenital myopathies include, but are not limited to: - (G71.2) nemaline myopathy (characterized by presence of "nemaline rods" in the muscle), - (G71.2) multi/minicore myopathy (characterized by multiple small "cores" or areas of disruption in the muscle fibers), - (G71.2) centronuclear myopathy (or myotubular myopathy) (in which the nuclei are abnormally found in the center of the muscle fibers), a rare muscle wasting disorder - (G71.3) Mitochondrial myopathies, which are due to defects in mitochondria, which provide a critical source of energy for muscle - (G72.3) Familial periodic paralysis - (G72.4) Inflammatory myopathies, which are caused by problems with the immune system attacking components of the muscle, leading to signs of inflammation in the muscle - (G73.6) Metabolic myopathies, which result from defects in biochemical metabolism that primarily affect muscle - (G72.89) Other myopathies - (G72.0 - G72.2) External substance induced myopathy - (G72.0) Drug-induced myopathy - (G72.1) Alcoholic myopathy - (G72.2) Myopathy due to other toxic agents - including atypical myopathy in horses caused by toxins in sycamore seeds and seedlings. - Dermatomyositis produces muscle weakness and skin changes. The skin rash is reddish and most commonly occurs on the face, especially around the eyes, and over the knuckles and elbows. Ragged nail folds with visible capillaries can be present. It can often be treated by drugs like corticosteroids or immunosuppressants. (M33.2) - Polymyositis produces muscle weakness. It can often be treated by drugs like corticosteroids or immunosuppressants. - Inclusion body myositis is a slowly progressive disease that produces weakness of hand grip and straightening of the knees. No effective treatment is known. - (M61) Myositis ossificans - (M62.89) Rhabdomyolysis and (R82.1) myoglobinurias The Food and Drug Administration is recommending that physicians restrict prescribing high-dose Simvastatin (Zocor, Merck) to patients, given an increased risk of muscle damage. The FDA drug safety communication stated that physicians should limit using the 80-mg dose unless the patient has already been taking the drug for 12 months and there is no evidence of myopathy. "Simvastatin 80 mg should not be started in new patients, including patients already taking lower doses of the drug," the agency states. - (I40) Acute myocarditis - (I41) Myocarditis in diseases classified elsewhere - (I42) Cardiomyopathy - (I42.0) Dilated cardiomyopathy - (I42.1) Obstructive hypertrophy cardiomyopathy - (I42.2) Other hypertrophic cardiomyopathy - (I42.3) Endomyocardial (eosinophilic) disease - (I42.4) Endocardial fibroelastosis - (I42.5) Other restrictive cardiomyopathy - (I42.6) Alcoholic cardiomyopathy - (I42.8) Other cardiomyopathies - (I43) Cardiomyopathy in diseases classified elsewhere - None as systemic causes; mainly hereditary Onset in childhood - Inflammatory myopathies – dermatomyositis, polymyositis (rarely) - Infectious myopathies - Endocrine and metabolic disorders – hypokalemia, hypocalcemia, hypercalcemia Onset in adulthood - Inflammatory myopathies – polymyositis, dermatomyositis, inclusion body myositis, viral (HIV) - Infectious myopathies - Endocrine myopathies – thyroid, parathyroid, adrenal, pituitary disorders - Toxic myopathies – alcohol, corticosteroids, narcotics, colchicines, chloroquine - Critical illness myopathy - Metabolic myopathies - Paraneoplastic myopathy Because different types of myopathies are caused by many different pathways, there is no single treatment for myopathy. Treatments range from treatment of the symptoms to very specific cause-targeting treatments. Drug therapy, physical therapy, bracing for support, surgery, and massage are all current treatments for a variety of myopathies. - "Myopathy - Definition from the Merriam-Webster Online Dictionary". - Voermans NC, van Alfen N, Pillen S, Lammens M, Schalkwijk J, Zwarts MJ, van Rooij IA, Hamel BC, van Engelen BG (June 2009). "Neuromuscular involvement in various types of Ehlers-Danlos syndrome". Ann. Neurol. 65 (6): 687–97. doi:10.1002/ana.21643. PMID 19557868. S2CID 22600065. - Chawla J (2011). "Stepwise approach to myopathy in systemic disease". Front Neurol. 2: 49. doi:10.3389/fneur.2011.00049. PMC 3153853. PMID 21886637. - Seene T (July 1994). "Turnover of skeletal muscle contractile proteins in glucocorticoid myopathy". J. Steroid Biochem. Mol. Biol. 50 (1–2): 1–4. doi:10.1016/0960-0760(94)90165-1. PMID 8049126. S2CID 27814895. - "Information On Sycamore Poisoning". Rainbow Equine Hospital. Retrieved 16 May 2017. - "Equine Atypical Myopathy toxin and biochemical tests and tree sample testing available at the RVC". Royal Veterinary college - University of London. 13 February 2017. Retrieved 16 May 2017. - "2019 ICD-10-CM Diagnosis Code I42.9: Cardiomyopathy, unspecified". The Web's Free 2019 ICD-10-CM/PCS Medical Coding Reference. 2018-10-01. Retrieved 2019-02-05. - GeneReviews/NCBI/NIH/UW entry on Myopathy with Deficiency of ISCU - See http://neuromuscular.wustl.edu/ for medical descriptions.	4	6	3	0	0	9	0.911737	12	1,858
National Provider Identifier (NPI): The National Provider Identifier (NPI) is a HIPAA Administrative Simplification Standard for the Health Insurance Portability and Accountability Act. UPINs, Medicare PINs, state Medicaid numbers, provider identities provided by thousands of health insurers, and other identifiers were all needed to disambiguate a specific healthcare provider across the healthcare system. This new system replaces the fragmented system of identifiers. To address this issue, the NPI was created. HIPAA requires all health plans, clearinghouses, and covered healthcare providers to use NPIs in administrative and financial transactions. The NPI is a ten-position numeric identification that lacks intelligence (10-digit number). Hence, no other information about healthcare practitioners, such as their place of residence or medical specialty, is included in the data. In HIPAA standards transactions, the NPI must replace legacy provider identities. Providers covered by the 1996 Health Insurance Portability and Accountability Act are obligated to share their NPI for billing purposes with others, health plans, clearinghouses, and other institutions (HIPAA). Providers are required to apply for their NPI number (also known as “enumeration”) through a CMS online application, which is offered by the Department of Health and Human Services’ Centers for Medicare and Medicaid Services (CMS) (HHS). The provider’s data is stored in the NPPES database. The data includes detailed contact information, practice or facility specifics, and identifiers like Medicare and Medicaid numbers, as well as the deprecated UPIN. Who Can Get A National Provider Identifier? An NPI is required for any physician who wants to bill Medicare or Medicaid for their services. Only lawful permanent residents and students are allowed to apply, according to the regulations. Residents who write prescriptions and order home health services expect pharmacists and durable equipment companies to require a physician NPI on orders to ensure they are reimbursed for their services. Healthcare providers who require NPIs to submit claims or undertake HIPAA-specified activities will be given them. Healthcare providers are those who provide medical or other health services or supplies to the general public. Included in this are the following: - physician/practitioner groups - nursing homes - dental clinics - and pharmacies are all examples of institutions that provide health care services. Are National Provider Identifiers (Npis) Required For Medical Students, Interns, And Residents? NPIs are available to all healthcare practitioners that qualify and wish to apply. NPIs are available to medical students, interns, residents, and fellows because they are health care providers. As long as they don’t transmit any health information as part of the transaction, they are exempt from the NPI Final Rule’s requirement to obtain NPIs. These providers are now classified as covered health care providers and must acquire NPIs. To ensure that health plans are adequately reimbursed, pharmacies and other health care providers will need to identify interns and residents who prescribed medications for patients whose prescriptions were filled by those providers, referred patients to those providers, or ordered tests from those providers in the claims transactions they submitted to health plans. To identify the prescriber, the referral provider, and the ordering provider, health plans may ask for the NPI to be used. As a result, while the NPI Final Rule does not mandate that these providers obtain NPIs, they may need to be reimbursed by health plans for services provided to pharmacies and other providers. Students and unlicensed physicians (PG 1 residents) can and should apply for an NPI number, so please be aware of this. Physicians who are not licensed will have to use a taxonomy code that says they are students. (This code is updated as a physician’s license or specialty changes during their career.) Because students can get an NPI number, we’ll demand that all new residents do so before beginning their studies. Upon receiving the NPI notification letter, residents should have a copy sent to their program coordinator to begin the enrollment process. Employees in the health care industry who support the provision of health care, such as admissions and billing employees, housekeeping staff, and orderlies, are not eligible for NPI. When Applying For National Provider Identifiers, Which Healthcare Provider Taxonomy Codes Should Medical Students, Interns, Residents, And Fellows Use? When applying for a National Provider Identifier, a healthcare provider must select a Healthcare Provider Taxonomy Code (NPI). NPIs are available to all covered health care professionals who wish to apply for one. Those in the health care professions such as doctors and nurses are also eligible to apply for NPIs. A standard for health data transactions was developed by the Department of Health and Human Services secretary. Unless they transmit health data as part of those transactions, they are not “covered” health care providers under HIPAA and do not need to obtain NPIs. - Health care providers who aren’t licensed can be classified as students or health care interns under the Healthcare Provider Taxonomy Code: (390200000X). The following is an explanation of what the code means: Enrolled in a health care provider training program that leads to a degree, certification, registration, or license. Doctors who are not licensed to practice medicine, interns, and residents should use the Student Health Care code to apply for an NPI. - According to the American Medical Association, a physician who is licensed as an allopathic or osteopathic physician should modify the Healthcare Provider Taxonomy Code to reflect the change in status from medical student to the physician in the National Plan and Provider Enumeration System, which is used to track medical students and physicians (NPPES). It is necessary to notify the NPPES of any changes in “covered” healthcare providers within thirty days of the change taking effect in order to avoid a fine. - It is the responsibility of physicians who have been assigned NPIs to keep their NPPES data up-to-date with any modifications or additions to their specialty (i.e. healthcare Provider Taxonomy Codes can be used to indicate changes or additions to a database). Those that fall under the category of “covered health care providers” are obliged to update their contact information with the NPPES within 30 days of making any changes to that information. Will A Health Care Provider’s NPI Ever Change? Even if a healthcare provider changes their name, address, provider taxonomy, or other information provided as part of the initial NPI application process, their National Provider Identifier (NPI) is supposed to be a permanent identifier. However, in rare cases, an NPI can be altered, such as when health care provider organizations determine they require a new NPI due to, for example, ownership transfers, purchase conditions, or the subpart strategies of a new owner. It’s also possible that a new NPI is required if the old one was fraudulently obtained. To Apply For An NPI, A Health Care Practitioner Must Submit The Following Information: To obtain an NPI, a healthcare practitioner has three options: - Start the application process by submitting a web-based form. This site’s URL is https://nppes.cms.hhs.gov/#/, for those who are interested in learning more about it. - Permit an Electronic File Interchange Organization (EFIO) to submit the application data on behalf of the healthcare provider if requested (i.e., through a bulk enumeration process). A healthcare provider agreeing to let an EFIO apply for the NPI will give the EFIO guidelines on what information is needed to complete the procedure. - Fill up a paper application form and send it to the NPI Enumerator by postal mail. NPI Enumerator is located at 7125 Ambassador Rd, Suite 100, Windsor Mill, MD 21244-2751, where they will enter the application data into the NPPES. If you are a health care provider, you can get a copy of the paper NPI Application/Update Form (CMS-10114). You can also request this form from the NPI Enumerator Contact Center. To get a copy of this form from the NPI Enumerator, health care providers have the following options: Phone: (800) 465-3203, TTY: (800) 692-2326 E-mail: ([email protected]) Contact Information.	3	3	2	0	0	0	0.364393	2	1,746
\|Classification and external resources\| \|OMIM\|\|603342 608923 603175 192430\| Psychosis refers to an abnormal condition of the mind described as involving a "loss of contact with reality". People with psychosis are described as psychotic. People experiencing psychosis may exhibit some personality changes and thought disorder. Depending on its severity, this may be accompanied by unusual or bizarre behavior, as well as difficulty with social interaction and impairment in carrying out daily life activities. Psychosis (as a medical sign of a psychiatric disorder) is a diagnosis of exclusion. That is, a new-onset episode of psychosis is not considered a symptom of a psychiatric disorder until other relevant and known causes of psychosis are properly excluded. Medical and biological laboratory tests should exclude central nervous system diseases and injuries, diseases and injuries of other organs, psychoactive substances, and toxins as causes of symptoms of psychosis before any psychiatric illness can be diagnosed. In medical training, psychosis as a sign of illness is often compared to fever since both can have multiple causes that are not readily apparent. The term "psychosis" is very broad and can mean anything from relatively normal aberrant experiences through to the complex and catatonic expressions of schizophrenia and bipolar type 1 disorder. In properly diagnosed psychiatric disorders (where other causes have been excluded by extensive medical and biological laboratory tests), psychosis is a descriptive term for the hallucinations, delusions and impaired insight that may occur. Psychosis is generally the term given to noticeable deficits in normal behavior (negative signs) and more commonly to diverse types of hallucinations or delusional beliefs, particularly with regard to the relation between self and others as in grandiosity and pronoia or paranoia. The first-line treatment for many psychotic disorders is antipsychotic medication. Meta-analyses of these drugs show either no difference in effects, or a moderate effect size, suggesting that the mechanism of psychosis is more complex than an overactive dopamine system. - 1 Signs and symptoms - 2 Causes - 3 Pathophysiology - 4 Diagnosis - 5 Prevention - 6 Treatment - 7 History - 8 References - 9 Further reading - 10 External links Signs and symptoms A hallucination is defined as sensory perception in the absence of external stimuli. Hallucinations are different from illusions, or perceptual distortions, which are the misperception of external stimuli. Hallucinations may occur in any of the senses and take on almost any form, which may include simple sensations (such as lights, colors, tastes, and smells) to experiences such as seeing and interacting with fully formed animals and people, hearing voices, and having complex tactile sensations. Auditory hallucinations, particularly experiences of hearing voices, are the most common and often prominent feature of psychosis. Hallucinated voices may talk about, or to, the person, and may involve several speakers with distinct personalities. Auditory hallucinations tend to be particularly distressing when they are derogatory, commanding or preoccupying. However, the experience of hearing voices need not always be a negative one. One research study has shown that the majority of people who hear voices are not in need of psychiatric help. The Hearing Voices Movement has subsequently been created to support voice hearers, regardless of whether they are considered to have a mental disorder or not. Psychosis may involve delusional beliefs, some of which are paranoid in nature. Put simply, delusions are false beliefs that a person holds on to, without adequate evidence. It can be difficult to change the belief, even with evidence to the contrary. Common themes of delusions are persecutory (person believes that others are out to harm them), grandiose (person believing that they have special powers or skills), etc. Persons with Ekbom syndrome may have delusional beliefs of an imaginary parasite infestation, whereas depressed persons might have delusions consistent with their low mood (e.g., delusions that they have sinned, or have contracted serious illness, etc.). Karl Jaspers has classified psychotic delusions into primary and secondary types. Primary delusions are defined as arising suddenly and not being comprehensible in terms of normal mental processes, whereas secondary delusions are typically understood as being influenced by the person's background or current situation (e.g., ethnicity; also religious, superstitious, or political beliefs). Catatonia describes a profoundly agitated state in which the experience of reality is generally considered impaired. There are two primary manifestations of catatonic behavior. The classic presentation is a person who does not move or interact with the world in any way while awake. This type of catatonia presents with waxy flexibility. Waxy flexibility is when someone physically moves part of a catatonic person's body and the person stays in the position even if it is bizarre and otherwise nonfunctional (such as moving a person's arm straight up in the air and the arm staying there). The other type of catatonia is more of an outward presentation of the profoundly agitated state described above. It involves excessive and purposeless motor behaviour, as well as extreme mental preoccupation that prevents an intact experience of reality. An example is someone walking very fast in circles to the exclusion of anything else with a level of mental preoccupation (meaning not focused on anything relevant to the situation) that was not typical of the person prior to the symptom onset. In both types of catatonia there is generally no reaction to anything that happens outside of them. It is important to distinguish catatonic agitation from severe bipolar mania, although someone could have both. Thought disorder describes an underlying disturbance to conscious thought and is classified largely by its effects on speech and writing. Affected persons show loosening of associations, that is, a disconnection and disorganization of the semantic content of speech and writing. In the severe form speech becomes incomprehensible and it is known as "word salad". Many causes of schizophrenia are also causes of psychosis. There is some evidence that trauma during childhood increases the risk of developing psychosis. From a diagnostic standpoint, organic disorders were those believed caused by physical illness affecting the brain (that is, psychiatric disorders secondary to other conditions), while functional disorders were considered disorders of the functioning of the mind in the absence of physical disorders (that is, primary psychological or psychiatric disorders). The materialistic or naturalistic (i.e. scientific) view of the mind–body problem holds that mental disorders arise from physical processes; in this view, the distinction between brain and mind, and therefore between organic and functional disease, is an artificial one. Subtle physical abnormalities have been found in illnesses traditionally considered functional, such as schizophrenia. The DSM-IV-TR avoids the functional/organic distinction, and instead lists traditional psychotic illnesses, psychosis due to general medical conditions, and substance-induced psychosis. - schizophrenia and schizophreniform disorder - affective (mood) disorders, including severe depression, and severe depression or mania in bipolar disorder (manic depression). People experiencing a psychotic episode in the context of depression may experience persecutory or self-blaming delusions or hallucinations, while people experiencing a psychotic episode in the context of mania may form grandiose delusions. - schizoaffective disorder, involving symptoms of both schizophrenia and mood disorders - brief psychotic disorder, or acute/transient psychotic disorder - delusional disorder (persistent delusional disorder) - chronic hallucinatory psychosis Psychotic symptoms may also be seen in - schizotypal disorder - certain personality disorders at times of stress (including paranoid personality disorder, schizoid personality disorder, and borderline personality disorder) - major depressive disorder in its severe form, although it is possible and more likely to have severe depression without psychosis - bipolar disorder in the manic and mixed episodes of bipolar I disorder and depressive episodes of both bipolar I and bipolar II; however, it is possible to experience such states without psychotic symptoms. - post-traumatic stress disorder - induced delusional disorder - Sometimes in obsessive-compulsive disorder - Dissociative disorders, due to many overlapping symptoms, careful differential diagnosis includes especially dissociative identity disorder. Stress is known to contribute to and trigger psychotic states. A history of psychologically traumatic events, and the recent experience of a stressful event, can both contribute to the development of psychosis. Short-lived psychosis triggered by stress is known as brief reactive psychosis, and patients may spontaneously recover normal functioning within two weeks. In some rare cases, individuals may remain in a state of full-blown psychosis for many years, or perhaps have attenuated psychotic symptoms (such as low intensity hallucinations) present at most times. Brief hallucinations are not uncommon in those without any psychiatric disease. Causes or triggers include: - Falling asleep and waking: hypnagogic and hypnopompic hallucinations, which are entirely normal - Bereavement, in which hallucinations of a deceased loved one are common - Severe sleep deprivation - Sensory deprivation and sensory impairment - Caffeine intoxication - An extremely stressful event Subtypes of psychosis include: - Menstrual psychosis, including circa-mensual (approximately monthly) periodicity, in rhythm with the menstrual cycle. - Postpartum psychosis, occurring recently after childbirth - Monothematic delusions - Myxedematous psychosis - Occupational psychosis - Stimulant psychosis - Tardive psychosis - Shared psychosis Cycloid psychosis is psychosis that progresses from normal to full-blown, usually within a few hours, not related to drug intake or brain injury. In addition, diagnostic criteria include at least four of the following symptoms: - Mood-incongruent delusions - Pan-anxiety, a severe anxiety not bound to particular situations or circumstances - Happiness or ecstasy of high degree - Motility disturbances of akinetic or hyperkinetic type - Concern with death - Mood swings to some degree, but less than what is needed for diagnosis of an affective disorder Cycloid psychosis occurs in people of generally 15–50 years of age. \|\|This section needs more medical references for verification or relies too heavily on primary sources, specifically: Case reports should be replaced with review articles or meta-analyses.. (September 2014)\| A very large number of medical conditions can cause psychosis, sometimes called secondary psychosis. Examples include: - disorders causing delirium (toxic psychosis), in which consciousness is disturbed - neurodevelopmental disorders and chromosomal abnormalities, including velocardiofacial syndrome - neurodegenerative disorders, such as Alzheimer's disease, dementia with Lewy bodies, and Parkinson's disease - focal neurological disease, such as stroke, brain tumors, multiple sclerosis, and some forms of epilepsy - malignancy (typically via masses in the brain, paraneoplastic syndromes, or drugs used to treat cancer) - infectious and postinfectious syndromes, including infections causing delirium, viral encephalitis, HIV/AIDS, malaria, Lyme disease, syphilis - endocrine disease, such as hypothyroidism, hyperthyroidism, adrenal failure, Cushing's syndrome, hypoparathyroidism and hyperparathyroidism; sex hormones also affect psychotic symptoms and sometimes childbirth can provoke psychosis, termed puerperal psychosis - inborn errors of metabolism, such as Succinic semialdehyde dehydrogenase deficiency, porphyria and metachromatic leukodystrophy - nutritional deficiency, such as vitamin B12 deficiency - other acquired metabolic disorders, including electrolyte disturbances such as hypocalcemia, hypernatremia, hyponatremia, hypokalemia, hypomagnesemia, hypermagnesemia, hypercalcemia, and hypophosphatemia, but also hypoglycemia, hypoxia, and failure of the liver or kidneys - autoimmune and related disorders, such as systemic lupus erythematosus (lupus, SLE), sarcoidosis, Hashimoto's encephalopathy, and anti-NMDA-receptor encephalitis - poisoning, by therapeutic drugs (see below), recreational drugs (see below), and a range of plants, fungi, metals, organic compounds, and a few animal toxins - some sleep disorders, including hallucinations in narcolepsy (in which REM sleep intrudes into wakefulness) Various psychoactive substances (both legal and illegal) have been implicated in causing, exacerbating, and/or precipitating psychotic states and/or disorders in users. This may be upon intoxication, for a more prolonged period after use, or upon withdrawal. Individuals who have a substance induced psychosis tend to have a greater awareness of their psychosis and tend to have higher levels of suicidal thinking compared to individuals who have a primary psychotic illness. Drugs that can induce psychotic symptoms include alcohol, cannabis, cocaine, amphetamines, cathinones, psychedelic drugs (such as LSD and psilocybin), κ-opioid receptor agonists (such as enadoline and salvinorin A) and NMDA receptor antagonists (such as phencyclidine and ketamine). Approximately three percent of people who are suffering from alcoholism experience psychosis during acute intoxication or withdrawal. Alcohol related psychosis may manifest itself through a kindling mechanism. The mechanism of alcohol-related psychosis is due to the long-term effects of alcohol resulting in distortions to neuronal membranes, gene expression, as well as thiamin deficiency. It is possible in some cases that alcohol abuse via a kindling mechanism can cause the development of a chronic substance induced psychotic disorder, i.e. schizophrenia. The effects of an alcohol-related psychosis include an increased risk of depression and suicide as well as causing psychosocial impairments. According to some studies, the more often cannabis is used the more likely a person is to develop a psychotic illness, with frequent use being correlated with twice the risk of psychosis and schizophrenia. While cannabis use is accepted as a contributory cause of schizophrenia by some, it remains controversial, with pre-existing vulnerability to psychosis emerging as the key factor that influences the link between cannabis use and psychosis. Some studies indicate that the effects of two active compounds in cannabis, tetrahydrocannabinol (THC) and cannabidiol (CBD), have opposite effects with respect to psychosis. While THC can induce psychotic symptoms in healthy individuals, CBD may reduce the symptoms caused by cannabis. Cannabis use has increased dramatically over the past few decades whereas the rate of psychosis has not increased. Together, these findings suggest that cannabis use may hasten the onset of psychosis in those who may already be predisposed to psychosis. High-potency cannabis use indeed seems to accelerate the onset of psychosis in predisposed patients. A 2012 study concluded that cannabis plays an important role in the development of psychosis in vulnerable individuals, and that cannabis use in early adolescence should be discouraged. Methamphetamine induces a psychosis in 26-46 percent of heavy users. Some of these people develop a long-lasting psychosis that can persist for longer than six months. Those who have had a short-lived psychosis from methamphetamine can have a relapse of the methamphetamine psychosis years later after a stress event such as severe insomnia or a period of heavy alcohol abuse despite not relapsing back to methamphetamine. Individuals who have long history of methamphetamine abuse and who have experienced psychosis in the past from methamphetamine abuse are highly likely to rapidly relapse back into a methamphetamine psychosis within a week or so of going back onto methamphetamine. Administration, or sometimes withdrawal, of a large number of medications may provoke psychotic symptoms. Drugs that can induce psychosis experimentally and/or in a significant proportion of people include amphetamine and other sympathomimetics, dopamine agonists, ketamine, corticosteroids (often with mood changes in addition), and some anticonvulsants such as vigabatrin. Stimulants that may cause this include lisdexamfetamine. A 2014 study found no evidence that familial risk accounts for associations between childhood physical abuse and psychotic disorder, or that it substantially increases the odds of psychosis among individuals reporting abuse. A meta-analysis showed that high neuroticism is an independent predictor of the development of psychotic symptoms and psychosis. The first brain image of an individual with psychosis was completed as far back as 1935 using a technique called pneumoencephalography (a painful and now obsolete procedure where cerebrospinal fluid is drained from around the brain and replaced with air to allow the structure of the brain to show up more clearly on an X-ray picture). The purpose of the brain is to collect information from the body (pain, hunger, etc.), and from the outside world, interpret it to a coherent world view, and produce a meaningful response. The information from the senses enter the brain in the primary sensory areas. They process the information and send it to the secondary areas where the information is interpreted. Spontaneous activity in the primary sensory areas may produce hallucinations, which the secondary areas misinterpret as information from the real world. For example, a PET or fMRI scan of a person who claims they hear voices may show activation in the primary auditory cortex, or parts of the brain involved in the perception and understanding of speech. Tertiary brain cortex collects the interpretations from the secondary cortexes and creates a coherent world view of it. A study investigating structural changes in the brains of people with psychosis showed there was significant grey matter reduction in the right medial temporal, lateral temporal, and inferior frontal gyrus, and in the cingulate cortex bilaterally of people before and after they became psychotic. Findings such as these have led to debate about whether psychosis itself causes excitotoxic brain damage and whether potentially damaging changes to the brain are related to the length of psychotic episode. Recent research has suggested that this is not the case although further investigation is still ongoing. Studies with sensory deprivation have shown that the brain is dependent on signals from the outer world to function properly. If the spontaneous activity in the brain is not counterbalanced with information from the senses, loss from reality and psychosis may occur after some hours. A similar phenomenon is paranoia in the elderly, when poor eyesight, hearing and memory make the person abnormally suspicious of the environment. On the other hand, loss from reality may also occur if the spontaneous cortical activity is increased so that it is no longer counterbalanced with information from the senses. The 5-HT2A receptor seems to be important for this, since psychedelic drugs that activate them produce hallucinations. However, the main feature of psychosis is not hallucinations, but the inability to distinguish between internal and external stimuli. Close relatives to psychotic patients may hear voices, but since they are aware that they are unreal they can ignore them, so that the hallucinations do not affect their reality perception. Hence they are not considered psychotic. Psychosis has been traditionally linked to the neurotransmitter dopamine. In particular, the dopamine hypothesis of psychosis has been influential and states that psychosis results from an overactivity of dopamine function in the brain, particularly in the mesolimbic pathway. The two major sources of evidence given to support this theory are that dopamine receptor D2 blocking drugs (i.e., antipsychotics) tend to reduce the intensity of psychotic symptoms, and that drugs that boost dopamine activity (such as amphetamines and cocaine) can trigger psychosis in some people (see amphetamine psychosis). However, increasing evidence in recent times has pointed to a possible dysfunction of the excitory neurotransmitter glutamate, in particular, with the activity of the NMDA receptor. This theory is reinforced by the fact that dissociative NMDA receptor antagonists such as ketamine, PCP and dextromethorphan (at large overdoses) induce a psychotic state more readily than dopaminergic stimulants, even at "normal" recreational doses. The symptoms of dissociative intoxication are also considered to mirror the symptoms of schizophrenia, including negative psychotic symptoms, more closely than amphetamine psychosis. Dissociative induced psychosis happens on a more reliable and predictable basis than amphetamine psychosis, which usually only occurs in cases of overdose, prolonged use or with sleep deprivation, which can independently produce psychosis. New antipsychotic drugs that act on glutamate and its receptors are currently undergoing clinical trials. The connection between dopamine and psychosis is generally believed complex. While dopamine receptor D2 suppresses adenylate cyclase activity, the D1 receptor increases it. If D2-blocking drugs are administered the blocked dopamine spills over to the D1 receptors. The increased adenylate cyclase activity affects genetic expression in the nerve cell, which takes time. Hence antipsychotic drugs take a week or two to reduce the symptoms of psychosis. Moreover, newer and equally effective antipsychotic drugs actually block slightly less dopamine in the brain than older drugs whilst also blocking 5-HT2A receptors, suggesting the 'dopamine hypothesis' may be oversimplified. Soyka and colleagues found no evidence of dopaminergic dysfunction in people with alcohol-induced psychosis and Zoldan et al. reported moderately successful use of ondansetron, a 5-HT3 receptor antagonist, in the treatment of levodopa psychosis in Parkinson's disease patients. Psychiatrist David Healy has criticised pharmaceutical companies for promoting simplified biological theories of mental illness that seem to imply the primacy of pharmaceutical treatments while ignoring social and developmental factors that are known important influences in the aetiology of psychosis. Some theories believe many psychotic symptoms are a problem with the perception of ownership of internally generated thoughts and experiences. For example, the hallucination of hearing voices may arise from internally generated speech that is mislabeled by the psychotic person as coming from an external source. It has been suggested that persons with bipolar disorder may have increased activity of the left hemisphere compared to the right hemisphere of the brain, while persons with schizophrenia have increased activity in the right hemisphere. Increased level of right hemisphere activation has also been found in people who have high levels of paranormal beliefs and in people who report mystical experiences. It also seems that people who are more creative are also more likely to show a similar pattern of brain activation. Some researchers have been quick to point out that this in no way suggests that paranormal, mystical or creative experiences are in any way by themselves a symptom of mental illness, as it is still not clear what makes some such experiences beneficial and others distressing. In otherwise normal individuals, exogenous ligands can produce psychotic symptoms. NMDA receptor antagonists, such as ketamine, can produce a similar psychosis to that experienced in schizophrenia.[non-primary source needed] Prolonged or high dose use of psychostimulants can alter normal functioning, making it similar to the manic phase of bipolar disorder. NMDA antagonists replicate some of the so-called "negative" symptoms like thought disorder in subanesthetic doses (doses insufficient to induce anesthesia), and catatonia in high doses. Psychostimulants, especially in one already prone to psychotic thinking, can cause some "positive" symptoms, such as delusional beliefs, particularly those persecutory in nature. Psychosis is first and foremost a diagnosis of exclusion. So a new-onset episode of psychosis cannot be considered a symptom of a psychiatric disorder until other relevant and known causes of psychosis are properly excluded, or ruled out. Many clinicians improperly perform, or entirely miss this step, introducing avoidable diagnostic error and misdiagnosis. An initial assessment includes a comprehensive history and physical examination by a physician, psychiatrist, psychiatric nurse practitioner or psychiatric physician assistant. Biological tests should be performed to exclude psychosis associated with or caused by substance use, medication, toxins, surgical complications, or other medical illnesses. Delirium should be ruled out, which can be distinguished by visual hallucinations, acute onset and fluctuating level of consciousness, indicating other underlying factors, including medical illnesses. Excluding medical illnesses associated with psychosis is performed by using blood tests to measure: - Thyroid-stimulating hormone to exclude hypo- or hyperthyroidism, - Basic electrolytes and serum calcium to rule out a metabolic disturbance, - Full blood count including ESR to rule out a systemic infection or chronic disease, and - Serology to exclude syphilis or HIV infection. Other investigations include: Because psychosis may be precipitated or exacerbated by common classes of medications, medication-induced psychosis should be ruled out, particularly for first-episode psychosis. Both substance- and medication-induced psychosis can be excluded to a high level of certainty, using a - Urinalysis and a - Full serum toxicology screening. Because some dietary supplements may also induce psychosis or mania, but cannot be ruled out with laboratory tests, a psychotic individual's family, partner, or friends should be asked whether the patient is currently taking any dietary supplements. Common mistakes made when diagnosing people who are psychotic include: - Not properly excluding delirium, - Not appreciating medical abnormalities (e.g., vital signs), - Not obtaining a medical history and family history, - Indiscriminate screening without an organizing framework, - Missing a toxic psychosis by not screening for substances and medications - Not asking family or others about dietary supplements, - Premature diagnostic closure, and - Not revisiting or questioning the initial diagnostic impression of primary psychiatric disorder. Only after relevant and known causes of psychosis are excluded, a mental health clinician may make a psychiatric differential diagnosis using a person's family history, incorporating information from the person with psychosis, and information from family, friends, or significant others. Types of psychosis in psychiatric disorders may be established by formal rating scales. The Brief Psychiatric Rating Scale (BPRS) assesses the level of 18 symptom constructs of psychosis such as hostility, suspicion, hallucination, and grandiosity. It is based on the clinician's interview with the patient and observations of the patient's behavior over the previous 2–3 days. The patient's family can also answer questions on the behavior report. During the initial assessment and the follow-up, both positive and negative symptoms of psychosis can be assessed using the 30 item Positive and Negative Symptom Scale (PANSS). The evidence for the effectiveness of early interventions to prevent psychosis appeared inconclusive. Whilst early intervention in those with a psychotic episode might improve short term outcomes, little benefit was seen from these measures after five years. However, there is evidence that cognitive behavioral therapy (CBT) may reduce the risk of becoming psychotic in those at high risk, and in 2014 the UK National Institute for Health and Care Excellence (NICE) recommended preventive CBT for people at risk of psychosis. The treatment of psychosis depends on the specific diagnosis (such as schizophrenia, bipolar disorder or substance intoxication). The first-line psychiatric treatment for many psychotic disorders is antipsychotic medication, which can reduce the positive symptoms of psychosis in about 7 to 14 days. The choice of which antipsychotic to use is based on benefits, risks, and costs. It is debatable whether, as a class, typical or atypical antipsychotics are better. Tentative evidence supports that amisulpride, olanzapine, risperidone and clozapine may be more effective for postive symptoms but result in more side effects. Typical antipsychotics have equal drop-out and symptom relapse rates to atypicals when used at low to moderate dosages. There is a good response in 40–50%, a partial response in 30–40%, and treatment resistance (failure of symptoms to respond satisfactorily after six weeks to two or three different antipsychotics) in 20% of people. Clozapine is an effective treatment for those who respond poorly to other drugs ("treatment-resistant" or "refractory" schizophrenia), but it has the potentially serious side effect of agranulocytosis (lowered white blood cell count) in less than 4% of people. Most people on antipsychotics get side effects. People on typical antipsychotics tend to have a higher rate of extrapyramidal side effects while some atypicals are associated with considerable weight gain, diabetes and risk of metabolic syndrome; this is most pronounced with olanzapine, while risperidone and quetiapine are also associated with weight gain. Risperidone has a similar rate of extrapyramidal symptoms to haloperidol. Early intervention in psychosis is based on the observation that identifying and treating someone in the early stages of a psychosis can improve their longer term outcome. This approach advocates the use of an intensive multi-disciplinary approach during what is known as the critical period, where intervention is the most effective, and prevents the long term morbidity associated with chronic psychotic illness. The word psychosis was introduced to the psychiatric literature in 1841 by Karl Friedrich Canstatt in his work Handbuch der Medizinischen Klinik. He used it as a shorthand for 'psychic neurosis'. At that time neurosis meant any disease of the nervous system, and Canstatt was thus referring to what was considered a psychological manifestation of brain disease. Ernst von Feuchtersleben is also widely credited as introducing the term in 1845, as an alternative to insanity and mania. The term stems from Modern Latin psychosis, "a giving soul or life to, animating, quickening" and that from Ancient Greek ψυχή (psyche), "soul" and the suffix -ωσις (-osis), in this case "abnormal condition". The word was also used to distinguish a condition considered a disorder of the mind, as opposed to neurosis, which was considered a disorder of the nervous system. The psychoses thus became the modern equivalent of the old notion of madness, and hence there was much debate on whether there was only one (unitary) or many forms of the new disease. One type of broad usage would later be narrowed down by Koch in 1891 to the 'psychopathic inferiorities' - later renamed abnormal personalities by Schneider. The division of the major psychoses into manic depressive illness (now called bipolar disorder) and dementia praecox (now called schizophrenia) was made by Emil Kraepelin, who attempted to create a synthesis of the various mental disorders identified by 19th century psychiatrists, by grouping diseases together based on classification of common symptoms. Kraepelin used the term 'manic depressive insanity' to describe the whole spectrum of mood disorders, in a far wider sense than it is usually used today. In Kraepelin's classification this would include 'unipolar' clinical depression, as well as bipolar disorder and other mood disorders such as cyclothymia. These are characterised by problems with mood control and the psychotic episodes appear associated with disturbances in mood, and patients often have periods of normal functioning between psychotic episodes even without medication. Schizophrenia is characterized by psychotic episodes that appear unrelated to disturbances in mood, and most non-medicated patients show signs of disturbance between psychotic episodes. Early civilizations considered madness a supernaturally inflicted phenomenon. Archaeologists have unearthed skulls with clearly visible drillings, some datable back to 5000 BC suggesting that trepanning was a common treatment for psychosis in ancient times. Written record of supernatural causes and resultant treatments can be traced back to the New Testament. Mark 5:8-13 describes a man displaying what would today be described as psychotic symptoms. Christ cured this "demonic madness" by casting out the demons and hurling them into a herd of swine. Exorcism, is still utilized in some religious circles as a treatment for psychosis presumed to be demonic possession. A research study of out-patients in psychiatric clinics found that 30 percent of religious patients attributed the cause of their psychotic symptoms to evil spirits. Many of these patients underwent exorcistic healing rituals that, though largely regarded as positive experiences by the patients, had no effect on symptomology. Results did, however, show a significant worsening of psychotic symptoms associated with exclusion of medical treatment for coercive forms of exorcism. The medical teachings of the fourth-century philosopher and physician, Hippocrates of Cos, proposed a natural, rather than supernatural, cause of human illness. In Hippocrates' work, the Hippocratic corpus, a holistic explanation for health and disease was developed to include madness and other "diseases of the mind." Hippocrates writes: Men ought to know that from the brain, and from the brain only, arise our pleasures, joys, laughter, and jests, as well as our sorrows, pains, griefs and tears. Through it, in particular, we think, see, hear, and distinguish the ugly from the beautiful, the bad from the good, the pleasant from the unpleasant…. It is the same thing which makes us mad or delirious, inspires us with dread and fear, whether by night or by day, brings sleeplessness, inopportune mistakes, aimless anxieties, absentmindedness, and acts that are contrary to habit.— Hippocrates of Cos, the Hippocratic corpus Hippocrates espoused a theory of humoralism wherein disease is resultant of a shifting balance in bodily fluids including blood, phlegm, black bile, and yellow bile. According to humoralism, each fluid or "humour" has temperamental or behavioral correlates. In the case of psychosis, symptoms are thought to be caused by an excess of both blood and yellow bile. Thus, the proposed surgical intervention for psychotic or manic behavior was bloodletting. 18th century physician, educator, and widely considered "founder of American psychiatry", Benjamin Rush, also prescribed bloodletting as a first-line treatment for psychosis. Although not a proponent of humoralism, Rush believed that active purging and bloodletting were efficacious corrections for disruptions in the circulatory system, a complication he believed was the primary cause of "insanity". Although Rush's treatment modalities are now considered antiquated and brutish, his contributions to psychiatry, namely the biological underpinnings of psychiatric phenomenon including psychosis, have been invaluable to the field. In honor of such contributions, Benjamin Rush's image is in the official seal of the American Psychiatric Association. Early 20th century treatments for severe and persisting psychosis were characterized by an emphasis on shocking the nervous system. Such therapies include insulin shock therapy, cardiazol shock therapy, and electroconvulsive therapy. Despite considerable risk, shock therapy was considered highly efficacious in the treatment of psychosis including schizophrenia. The acceptance of high-risk treatments led to more invasive medical interventions including psychosurgery. In 1888, Swiss psychiatrist, Gottlieb Burckhardt, performed the first medically sanctioned psychosurgery in which the cerebral cortex was excised. Although some patients showed improvement of symptoms and became more subdued, one patient died and several developed aphasia and/or seizure disorders. Burckhardt would go on to publish his clinical outcomes in a scholarly paper. This procedure was met with criticism from the medical community and his academic and surgical endeavors were largely ignored. In the late 1930s, Egas Moniz conceived the leucotomy (AKA prefrontal lobotomy) in which the fibers connecting the frontal lobes to the rest of the brain were severed. Moniz’s primary inspiration stemmed from a demonstration by neuroscientists, John Fulton and Carlyle’s 1935 experiment in which two chimpanzees were given leucotomies and pre and post surgical behavior was compared. Prior to the leucotomy, the chimps engaged in typical behavior including throwing feces and fighting. After the procedure, both chimps were pacified and less violent. During the Q&A, Moniz asked if such a procedure could be extended to human subjects, a question that Fulton admitted was quite startling. Moniz would go on to extend the controversial practice to humans suffering from various psychotic disorders, an endeavor for which he received a Nobel Prize in 1949. Between the late 1930s and early 1970s, the leucotomy was a widely accepted practice, often performed in non-sterile environments such as small outpatient clinics and patient homes. Psychosurgery remained standard practice until the discovery of antipsychotic pharmacology in the 1950s. The first clinical trial of antipsychotics (also commonly known as neuroleptics) for the treatment of psychosis took place in 1952. Chlorpromazine (brand name: Thorazine) passed clinical trials and became the first antipsychotic medication approved for the treatment of both acute and chronic psychosis. Although the mechanism of action was not discovered until 1963, the administration of chlorpromazine marked the advent of the dopamine antagonist, or first generation antipsychotic. While clinical trials showed a high response rate for both acute psychosis and disorders with psychotic features, the side-effects were particularly harsh, which included high rates of often irreversible Parkinsonian symptoms such as tardive dyskinesia. With the advent of atypical antipsychotics (also known as second generation antipsychotics) came a dopamine antagonist with a comparable response rate but a far different, though still extensive, side-effect profile that included a lower risk of Parkinsonian symptoms but a higher risk of cardiovascular disease. Atypical antipsychotics remain the first-line treatment for psychosis associated with various psychiatric and neurological disorders including schizophrenia, bipolar disorder, major depressive disorder, anxiety disorders, dementia, and some autism spectrum disorders. It is now known that dopamine is the primary neurotransmitter implicated in psychotic symptomology. Thus, blocking dopamine receptors (namely, the dopamine D2 receptors) and decreasing dopaminergic activity continues to be an effective but highly unrefined pharmacologic goal of antipsychotics. Recent pharmacological research suggests that the decrease in dopaminergic activity does not eradicate psychotic delusions or hallucinations, but rather attenuates the reward mechanisms involved in the development of delusional thinking; that is, connecting or finding meaningful relationships between unrelated stimuli or ideas. The author of this research paper acknowledges the importance of future investigation: The model presented here is based on incomplete knowledge related to dopamine, schizophrenia, and antipsychotics—and as such will need to evolve as more is known about these.— Shitij Kapur, From dopamine to salience to psychosis—linking biology, pharmacology and phenomenology of psychosis - Kelly, Evelyn B. (2001). Coping with schizophrenia (1st ed.). New York: Rosen Pub. p. 25. ISBN 9780823928538. - "Schizophrenia, schizotypal and delusional disorders (F20-F29)". ICD-10 Version:2010. World Health Organization. - Freudenreich, Oliver (3 December 2012). "Differential Diagnosis of Psychotic Symptoms: Medical "Mimics"". Psychiatric Times. UBM Medica. Retrieved October 2013. Check date values in: - American Psychiatric Association, 1994 The Diagnostic and Statistical Manual Revision IV (DSM-IV) - Gelder, Michael G; Mayou, Richard; Geddes, John (2005). Psychiatry. New York: Oxford University Press. p. 12. ISBN 978-0-19-852863-0. - Yuhas, Daisy. "Throughout History, Defining Schizophrenia Has Remained a Challenge (Timeline)". Scientific American Mind (March 2013). Retrieved 2 March 2013. - Murray, Robin; Hill, P. D. (Peter David); McGuffin, P. (Peter) (1997). The essentials of postgraduate psychiatr. Cambridge ; New York, NY, USA: Cambridge University Press. p. 231. ISBN 978-0-521-57801-1. - National Collaborating Centre for Mental Health (25 March 2009). "Schizophrenia: Full national clinical guideline on core interventions in primary and secondary care" (PDF). Retrieved 25 November 2009. - Leucht, S; D Arbter; RR Engel; W Kissling; JM Davis (April 2009). "How effective are second-generation antipsychotic drugs? A meta-analysis of placebo-controlled trials" (PDF). Molecular Psychiatry. 14 (4): 429–447. doi:10.1038/sj.mp.4002136. PMID 18180760. Retrieved 24 March 2013. - Rattehalli, R. D.; Jayaram, M. B.; Smith, M. (5 April 2010). "Risperidone Versus Placebo for Schizophrenia". Schizophrenia Bulletin. 36 (3): 448–449. doi:10.1093/schbul/sbq030. PMC . PMID 20368309. - Fusar-Poli, P.; Deste, G.; Smieskova, R.; Barlati, S.; Yung, AR.; Howes, O.; Stieglitz, RD.; Vita, A.; McGuire, P.; Borgwardt, S (Jun 2012). "Cognitive functioning in prodromal psychosis: a meta-analysis". Arch Gen Psychiatry. 69 (6): 562–71. doi:10.1001/archgenpsychiatry.2011.1592. PMID 22664547. - Brown, EC.; Tas, C.; Brüne, M. (Jan 2012). "Potential therapeutic avenues to tackle social cognition problems in schizophrenia". Expert Rev Neurother. 12 (1): 71–81. doi:10.1586/ern.11.183. PMID 22149657. - Harper, Douglas (November 2001). "hallucinate". Online Etymology Dictionary. Retrieved October 15, 2006. - Honig A, Romme MA, Ensink BJ, Escher SD, Pennings MH, deVries MW; Romme; Ensink; Escher; Pennings; Devries (October 1998). "Auditory hallucinations: a comparison between patients and nonpatients". J. Nerv. Ment. Dis. 186 (10): 646–51. doi:10.1097/00005053-199810000-00009. PMID 9788642. - Susannah Cahalan. Brain on Fire-My Month of Madness, New York: Simon & Schuster, 2013. - Jaspers, Karl (1997-11-27) . Allgemeine Psychopathologie (General Psychopathology). Translated by J. Hoenig & M.W. Hamilton from German (Reprint ed.). Baltimore, Maryland: Johns Hopkins University Press. ISBN 0-8018-5775-9. - Mizrahi, R (February 2016). "Social Stress and Psychosis Risk: Common Neurochemical Substrates?". Neuropsychopharmacology. 41 (3): 666–74. doi:10.1038/npp.2015.274. PMID 26346639. - World Health Organization, The ICD-10 Classification of Mental and Behavioural Disorders: Clinical descriptions and diagnostic guidelines (CDDG), 1992. - American Psychiatric Association, Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revision (DSM-IV-TR), American Psychiatric Association, 2000. - Cardinal, R.N. & Bullmore, E.T., The Diagnosis of Psychosis, Cambridge University Press, 2011, ISBN 978-0-521-16484-9 - Shibayama M (2011). "Differential diagnosis between dissociative disorders and schizophrenia". Seishin shinkeigaku zasshi=Psychiatria et neurologia Japonica. 113 (9): 906–911. PMID 22117396. - Jauch, D. A.; William T. Carpenter, Jr. (February 1988). "Reactive psychosis. I. Does the pre-DSM-III concept define a third psychosis?". Journal of Nervous and Mental Disease. 176 (2): 72–81. doi:10.1097/00005053-198802000-00002. PMID 3276813. - Ohayon, M. M.; R. G. Priest; M. Caulet; C. Guilleminault (October 1996). "Hypnagogic and hypnopompic hallucinations: pathological phenomena?". British Journal of Psychiatry. 169 (4): 459–67. doi:10.1192/bjp.169.4.459. PMID 8894197. Retrieved 2006-10-21. - Sharma, Verinder; Dwight Mazmanian (April 2003). "Sleep loss and postpartum psychosis". Bipolar Disorders. 5 (2): 98–105. doi:10.1034/j.1399-5618.2003.00015.x. PMID 12680898. Retrieved 2006-09-27. - Chan-Ob, T.; V. Boonyanaruthee (September 1999). "Meditation in association with psychosis". Journal of the Medical Association of Thailand. 82 (9): 925–930. PMID 10561951. - Devillieres, P.; M. Opitz; P. Clervoy; J. Stephany (May–June 1996). "[Delusion and sleep deprivation]". L'Encéphale. 22 (3): 229–31. - Pillmann, Frank; Marneros, Andreas (2004). Acute and transient psychoses. Cambridge, UK: Cambridge University Press. p. 188. ISBN 0-521-83518-6. OCLC 144618418. - Lesser JM, Hughes S; Hughes (December 2006). "Psychosis-related disturbances. Psychosis, agitation, and disinhibition in Alzheimer's disease: definitions and treatment options". Geriatrics. 61 (12): 14–20. PMID 17184138. - McKeith, Ian G. (February 2002). "Dementia with Lewy bodies". British Journal of Psychiatry. 180 (2): 144–7. doi:10.1192/bjp.180.2.144. PMID 11823325. - Wedekind S (June 2005). "[Depressive syndrome, psychoses, dementia: frequent manifestations in Parkinson disease]". MMW Fortschr Med (in German). 147 (22): 11. PMID 15977623. - Lisanby, S. H.; C. Kohler; C. L. Swanson; R. E. Gur (January 1998). "Psychosis Secondary to Brain Tumor". Seminars in clinical neuropsychiatry. 3 (1): 12–22. PMID 10085187. - Rodriguez Gomez, Diego; Elvira Gonzalez Vazquez and Óscar Perez Carral (August 16–31, 2005). "Psicosis aguda como inicio de esclerosis multiple" [Acute psychosis as the presenting symptom of multiple sclerosis]. Revista de Neurología (in Spanish). 41 (4): 255–6. PMID 16075405. Retrieved 2006-09-27. - Evans, Dwight L.; Karen I. Mason, Jane Leserman, Russell Bauer And John Petitto (2002-02-01). "Chapter 90: Neuropsychiatric Manifestations of HIV-1 Infection and AIDS". In Kenneth L Davis, Dennis Charney, Joseph T Coyle, Charles Nemeroff. Neuropsychopharmacology: The Fifth Generation of Progress (5th ed.). Philadelphia: Lippincott Williams & Wilkins. pp. 1281–1301. ISBN 0-7817-2837-1. Retrieved 2006-10-16. - Tilluckdharry, C. C.; D. D. Chaddee; R. Doon; J. Nehall (March 1996). "A case of vivax malaria presenting with psychosis". West Indian Medical Journal. 45 (1): 39–40. PMID 8693739. - Fallon BA, Nields JA; Nields (November 1994). "Lyme disease: a neuropsychiatric illness". Am J Psychiatry. 151 (11): 1571–83. doi:10.1176/ajp.151.11.1571. PMID 7943444. (subscription required) - Hess, A; et al. (March 1999). "Borrelia burgdorferi central nervous system infection presenting as an organic schizophrenialike disorder". Biol. Psychiatry. 45 (6): 795. doi:10.1016/S0006-3223(98)00277-7. PMID 10188012. - van den Bergen, HA; Smith, JP; van der Zwan, A (October 1993). "[Lyme psychosis]". Ned Tijdschr Geneeskd (in Dutch). 137 (41): 2098–100. PMID 8413733. - Kararizou E, Mitsonis C, Dimopoulos N, Gkiatas K, Markou I, Kalfakis N; Mitsonis; Dimopoulos; Gkiatas; Markou; Kalfakis (May–Jun 2006). "Psychosis or simply a new manifestation of neurosyphilis?". J. Int. Med. Res. 34 (3): 335–7. doi:10.1177/147323000603400314. PMID 16866029. - Brooke D, Jamie P, Slack R, Sulaiman M, Tyrer P; Jamie; Slack; Sulaiman; Tyrer (October 1987). "Neurosyphilis—a treatable psychosis". Br J Psychiatry. 151 (4): 556. doi:10.1192/bjp.151.4.556. PMID 3447677. - Hermle L, Becker FW, Egan PJ, Kolb G, Wesiack B, Spitzer M; Becker; Egan; Kolb; Wesiack; Spitzer (1997). "[Metachromatic leukodystrophy simulating schizophrenia-like psychosis]". Der Nervenarzt (in German). 68 (9): 754–8. doi:10.1007/s001150050191. PMID 9411279. - Black DN, Taber KH, Hurley RA; Taber; Hurley (2003). "Metachromatic leukodystrophy: a model for the study of psychosis". The Journal of Neuropsychiatry and Clinical Neurosciences. 15 (3): 289–93. doi:10.1176/appi.neuropsych.15.3.289. PMID 12928504. free full text - Kumperscak HG, Paschke E, Gradisnik P, Vidmar J, Bradac SU; Paschke; Gradisnik; Vidmar; Bradac (2005). "Adult metachromatic leukodystrophy: disorganized schizophrenia-like symptoms and postpartum depression in 2 sisters". Journal of psychiatry & neuroscience : JPN. 30 (1): 33–6. PMC . PMID 15644995. - Sethi NK, Robilotti E, Sadan Y (2005). "Neurological Manifestations Of Vitamin B-12 Deficiency". The Internet Journal of Nutrition and Wellness. 2 (1). doi:10.5580/5a9. - Masalha R, Chudakov B, Muhamad M, Rudoy I, Volkov I, Wirguin I; Chudakov; Muhamad; Rudoy; Volkov; Wirguin (September 2001). "Cobalamin-responsive psychosis as the sole manifestation of vitamin B12 deficiency". Isr. Med. Assoc. J. 3 (9): 701–3. PMID 11574992. - Rossman, Phillip L.; Robert M. Vock (September 1956). "Postpartum Tetany and Psychosis Due to Hypocalcemia". California Medicine. 85 (3): 190–3. PMC . PMID 13356186. - Jana, D. K.; L. Romano-Jana (October 1973). "Hypernatremic psychosis in the elderly: case reports". Journal of the American Geriatrics Society. 21 (10): 473–7. PMID 4729012. - Haensch, C. A.; G. Hennen and J. Jorg (April 1996). "[Reversible exogenous psychosis in thiazide-induced hyponatremia of 97 mmol/l]". Der Nervenarzt. 67 (4): 319–22. PMID 8684511. - Hafez, H.; J. S. Strauss; M. D. Aronson; C. Holt (June 1984). "Hypokalemia-induced psychosis in a chronic schizophrenic patient". Journal of Clinical Psychiatry. 45 (6): 277–9. PMID 6725222. - Konstantakos, Anastasios K.; Enrique Grisoni (May 25, 2006). "Hypomagnesemia". eMedicine. WebMD. Retrieved October 16, 2006. - Velasco, P. Joel; Manoochehr Manshadi; Kevin Breen; Steven Lippmann (1 December 1999). "Psychiatric Aspects of Parathyroid Disease". Psychosomatics. 40 (6): 486–90. doi:10.1016/S0033-3182(99)71186-2. PMID 10581976. Retrieved 2006-10-17. - Rosenthal, M.; I. Gil and B. Habot (1997). "Primary hyperparathyroidism: neuropsychiatric manifestations and case report". Israel Journal of Psychiatry and Related Sciences. 34 (2): 122–125. PMID 9231574. - Nanji, A. A. (November 1984). "The psychiatric aspect of hypophosphatemia". Canadian Journal of Psychiatry. 29 (7): 599–600. PMID 6391648. - Padder, Tanveer; Aparna Udyawar; Nouman Azhar; Kamil Jaghab (December 2005). "Acute Hypoglycemia Presenting as Acute Psychosis". Psychiatry online. Retrieved 2006-09-27. - Robert, M.; R. Sunitha; N. K. Thulaseedharan (1 March 2006). "Neuropsychiatric manifestations systemic lupus erythematosus: A study from South India". Neurology India. 54 (1): 75–7. doi:10.4103/0028-3886.24713. PMID 16679649. Retrieved 2006-09-29. - Bona, Joseph R.; Sondralyn M. Fackler, Morris J. Fendley and Charles B. Nemeroff (1 August 1998). "Neurosarcoidosis as a Cause of Refractory Psychosis: A Complicated Case Report". American Journal of Psychiatry. 155 (8): 1106–8. PMID 9699702. Retrieved 2006-09-29. - Wilcox RA, To T, Koukourou A, Frasca J; To; Koukourou; Frasca (November 2008). "Hashimoto's encephalopathy masquerading as acute psychosis". J Clin Neurosci. 15 (11): 1301–4. doi:10.1016/j.jocn.2006.10.019. PMID 18313925. - Gómez-Bernal GJ, Reboreda A, Romero F, Bernal MM, Gómez F; Reboreda; Romero; Bernal; Gómez (2007). "A Case of Hashimoto's Encephalopathy Manifesting as Psychosis". Prim Care Companion J Clin Psychiatry. 9 (4): 318–9. doi:10.4088/PCC.v09n0411f. PMC . PMID 17934563. - Ray M, Kothur K, Padhy SK, Saran P; Kothur; Padhy; Saran (May 2007). "Hashimoto's encephalopathy in an adolescent boy". Indian J Pediatr. 74 (5): 492–4. doi:10.1007/s12098-007-0084-0. PMID 17526963. - Nasky KM, Knittel DR, Manos GH (August 2008). "Psychosis associated with anti-N-methyl-D-aspartate receptor antibodies". CNS Spectr. 13 (8): 699–703. - Steinberg, D.; S. R. Hirsch; S. D. Marston; K. Reynolds; R. N. Sutton (May 1972). "Influenza infection causing manic psychosis". British Journal of Psychiatry. 120 (558): 531–535. doi:10.1192/bjp.120.558.531. PMID 5041533. - Maurizi, C. P. (February 1985). "Influenza and mania: a possible connection with the locus ceruleus". Southern Medical Journal. 78 (2): 207–209. doi:10.1097/00007611-198502000-00025. PMID 3975719. - Keddie, K. M. (August 1965). "Toxic psychosis following mumps". British Journal of Psychiatry. 111 (477): 691–696. doi:10.1192/bjp.111.477.691. PMID 14337417. - Grant KM, LeVan TD, Wells SM, et al. (March 2012). "Methamphetamine-associated psychosis". J Neuroimmune Pharmacol. 7 (1): 113–39. doi:10.1007/s11481-011-9288-1. PMC . PMID 21728034. - Krebs, TS; Johansen, PO (August 2013). "Psychedelics and mental health: a population study.". PLOS ONE. 8 (8): e63972. doi:10.1371/journal.pone.0063972. PMC . PMID 23976938. - Alcohol-Related Psychosis at eMedicine - Moore THM, Zammit S, Lingford-Hughes A et al.. Cannabis use and risk of psychotic or affective mental health outcomes: a systematic review. Lancet. 2007;370(9584):319–328. doi:10.1016/S0140-6736(07)61162-3. PMID 17662880. - Leweke FM, Koethe D. Cannabis and psychiatric disorders: it is not only addiction. Addict Biol. June 2008;13(2):264–75. doi:10.1111/j.1369-1600.2008.00106.x. PMID 18482435. - Sewell RA, Ranganathan M, D'Souza DC. Cannabinoids and psychosis. International review of psychiatry (Abingdon, England). 2009 Apr;21(2):152–62. doi:10.1080/09540260902782802. PMID 19367509. - Henquet C, Di Forti M, Morrison P, Kuepper R, Murray RM. Gene-environment interplay between cannabis and psychosis. Schizophr Bull. November 2008;34(6):1111–21. doi:10.1093/schbul/sbn108. PMID 18723841. - McLaren JA, Silins E, Hutchinson D, Mattick RP, Hall W. Assessing evidence for a causal link between cannabis and psychosis: a review of cohort studies. Int. J. Drug Policy. January 2010;21(1):10–9. doi:10.1016/j.drugpo.2009.09.001. PMID 19783132. - Ben Amar M, Potvin S. Cannabis and psychosis: what is the link?. Journal of Psychoactive Drugs. 2007 Jun;39(2):131–42. doi:10.1080/02791072.2007.10399871. PMID 17703707. - Bhattacharyya, S.; et al. (February 2010). "Opposite Effects of D-9-Tetrahydrocannabinol and Cannabidiol on Human Brain Function and Psychopathology". Neuropsychopharmacology. 35 (3): 764–774. doi:10.1038/npp.2009.184. PMC . PMID 19924114. - Degenhardt L, Hall W, Lynskey M (2001). "Comorbidity between cannabis use and psychosis: Modelling some possible relationships" (PDF). Technical Report No. 121. Sydney: National Drug and Alcohol Research Centre. Retrieved 2006-08-19. - Marta Di Forti (17 December 2013). "Daily Use, Especially of High-Potency Cannabis, Drives the Earlier Onset of Psychosis in Cannabis Users". - Dragt, S; Nieman, DH; Schultze-Lutter, F; van der Meer, F; Becker, H; de Haan, L; Dingemans, PM; Birchwood, M; Patterson, P; Salokangas, RKR; Heinimaa, M; Heinz, A; Juckel, G; Graf von Reventlow, H; French, P; Stevens, H; Ruhrmann, S; Klosterkötter, J; Linszen, DH (January 2012). "Cannabis use and age at onset of symptoms in subjects at clinical high risk for psychosis". Acta Psychiatrica Scandinavica. 125 (1): 45–53. doi:10.1111/j.1600-0447.2011.01763.x. - Sander JW, Hart YM, Trimble MR, Shorvon SD (1991). "Vigabatrin and psychosis". Journal of Neurology, Neurosurgery, and Psychiatry. 54 (5): 435–9. doi:10.1136/jnnp.54.5.435. PMC . PMID 1865207. - "Adderall XR Prescribing Information" (PDF). United States Food and Drug Administration. December 2013. pp. 4–6. Retrieved 30 December 2013. - Fisher, Helen L.; McGuffin, Peter; Boydell, Jane; Fearon, Paul; Craig, Thomas K.; Dazzan, Paola; Morgan, Kevin; Doody, Gillian A.; Jones, Peter B.; Leff, Julian; Murray, Robin M.; Morgan, Craig (January 7, 2014). "Interplay Between Childhood Physical Abuse and Familial Risk in the Onset of Psychotic Disorders". Schizophr Bull. In press (6): 1443–51. doi:10.1093/schbul/sbt201. PMID 24399191. - Jeronimus B.F.; Kotov, R.; Riese, H.; Ormel, J. (2016). "Neuroticism's prospective association with mental disorders halves after adjustment for baseline symptoms and psychiatric history, but the adjusted association hardly decays with time: a meta-analysis on 59 longitudinal/prospective studies with 443 313 participants". Psychological Medicine: 1–24. doi:10.1017/S0033291716001653. PMID 27523506. - Moore, M T; Nathan D; Elliot AR; Laubach C (1935). "Encephalographic studies in mental disease". American Journal of Psychiatry. 92 (1): 43–67. - Copolov DL, Seal ML, Maruff P, et al. (April 2003). "Cortical activation associated with the experience of auditory hallucinations and perception of human speech in schizophrenia: a PET correlation study". Psychiatry Res. 122 (3): 139–52. doi:10.1016/S0925-4927(02)00121-X. PMID 12694889. - Pantelis, C; Velakoulis D, McGorry PD, Wood SJ, Suckling J, Phillips, LJ, Yung AR, Bullmore ET, Brewer W, Soulsby B, Desmond, P, McGuire PK (2003). "Neuroanatomical abnormalities before and after onset of psychosis: a cross-sectional and longitudinal MRI comparison". Lancet. 25 (361 (9354)): 281–8. doi:10.1016/S0140-6736(03)12323-9. PMID 12559861. - Ho, BC; Alicata D; Ward J; Moser DJ; O'Leary DS; Arndt S; Andreasen NC (2003). "Untreated initial psychosis: relation to cognitive deficits and brain morphology in first-episode schizophrenia". American Journal of Psychiatry. 160 (1): 142–8. doi:10.1176/appi.ajp.160.1.142. PMID 12505813. - Kapur, Shitij; Mizrahi Rominia; Li Ming (November 2005). "From dopamine to salience to psychosis—linking biology, pharmacology and phenomenology of psychosis". Schizophrenia Research. 79 (1): 59–68. doi:10.1016/j.schres.2005.01.003. PMID 16005191. - Jones HM, Pilowsky LS (October 2002). "Dopamine and antipsychotic drug action revisited". Br J Psychiatry. 181 (4): 271–5. doi:10.1192/bjp.181.4.271. PMID 12356650. - Soyka, Michael; Thomas Zetzsche; Stefan Dresel; Klaus Tatsch (May 2000). "FDG-PET and IBZM-SPECT Suggest Reduced Thalamic Activity but No Dopaminergic Dysfunction in Chronic Alcohol Hallucinosis". Journal of Neuropsychiatry & Clinical Neurosciences. 12 (2): 287–288. doi:10.1176/appi.neuropsych.12.2.287. PMID 11001615. - Zoldan, J.; G. Friedberg, M. Livneh, and E. Melamed. (July 1995). "Psychosis in advanced Parkinson's disease: treatment with ondansetron, a 5-HT3 receptor antagonist". Neurology. 45 (7): 1305–1308. doi:10.1212/WNL.45.7.1305. PMID 7617188. - Healy, David (2002). The Creation of Psychopharmacology. Cambridge: Harvard University Press. ISBN 0-674-00619-4. - Blakemore, SJ; Smith J; Steel R; Johnstone CE; Frith CD (2000). "The perception of self-produced sensory stimuli in patients with auditory hallucinations and passivity experiences: evidence for a breakdown in self-monitoring". Psychological Medicine. 30 (5): 1131–9. doi:10.1017/S0033291799002676. PMID 12027049. - Lohr, JB; Caligiuri MP (1997). "Lateralized hemispheric dysfunction in the major psychotic disorders: historical perspectives and findings from a study of motor asymmetry in older patients". Schizophrophrenia Research. 30 (27 (2–3)): 191–8. doi:10.1016/S0920-9964(97)00062-5. PMID 9416648. - Pizaagalli, D; Lehmann D; Gianotti L; Koenig T; Tanaka H; Wackermann J; Brugger P. (2000). "Brain electric correlates of strong belief in paranormal phenomena: intracerebral EEG source and regional Omega complexity analyses". Psychiatry Research. 100 (3): 139–154. doi:10.1016/S0925-4927(00)00070-6. PMID 11120441. - Makarec, K; Persinger, MA (1985). "Temporal lobe signs: electroencephalographic validity and enhanced scores in special populations". Perceptual and Motor Skills. 60 (3): 831–42. doi:10.2466/pms.19126.96.36.1991. PMID 3927256. - Weinstein, S; Graves RE (2002). "Are creativity and schizotypy products of a right hemisphere bias?". Brain and Cognition. 49 (1): 138–51. doi:10.1006/brcg.2001.1493. PMID 12027399. Retrieved 2006-08-19. - Krystal, JH; Karper, LP; Seibyl, JP; Freeman, GK; Delaney, R; Bremner, JD; Heninger, GR; Bowers, MB, Jr; Charney, DS (March 1994). "Subanesthetic Effects of the Noncompetitive NMDA Antagonist, Ketamine, in Humans: Psychotomimetic, perceptual, cognitive, and neuroendocrine responses". Arch Gen Psychiatry. 51 (3): 199–214. doi:10.1001/archpsyc.1994.03950030035004. PMID 8122957. - Curran, Catherine; Byrappa, Neetha; McBride, Andrew (September 2004). "Stimulant psychosis: systematic review". British Journal of Psychiatry. 185 (3): 196–204. doi:10.1192/bjp.185.3.196. PMID 15339823. - Nordqvist, Christian (2 Jun 2010). "What Is Schizoaffective Disorder? What Causes Schizoaffective Disorder?". Medical News Today. Retrieved March 2013. Check date values in: - Food and Drug Administration (11 February 2004). "Final rule declaring dietary supplements containing ephedrine alkaloids adulterated because they present an unreasonable risk". Federal Register. 69 (28): 6787–854. PMID 14968803. (69 FR 6814 and 69 FR 6818) - Overall JE, Gorham DR. The Brief Psychiatric Rating Scale. Psychol Rep. 1962;10:799-812 - Kay, S.R., Fiszbien, A., & Opler, L.A. 1987. The Positive and Negative Symptom Scale (PANNS) for schizophrenia" Schizophrenia Bulletin 13, pp261-276 - Marshall M, Rathbone J. Early intervention for psychosis. Cochrane Database Syst Rev. 2006;(4):CD004718. doi:10.1002/14651858.CD004718.pub2. PMID 17054213. - van Os J, Kapur S. Schizophrenia. Lancet. August 2009;374(9690):635–45. doi:10.1016/S0140-6736(09)60995-8. PMID 19700006. - Stafford, MR; Jackson, H; Mayo-Wilson, E; Morrison, AP; Kendall, T (Jan 18, 2013). "Early interventions to prevent psychosis: systematic review and meta-analysis". BMJ (Clinical research ed.). 346: f185. doi:10.1136/bmj.f185. PMC . PMID 23335473. - "Offer talking therapies to people at risk of psychosis and schizophrenia". Nice.org.uk. 2014-02-12. Retrieved 2014-04-15. - "Psychosis and schizophrenia in adults". Nice.org.uk. 2014-03-31. Retrieved 2014-04-15. - Kane JM, Correll CU; Correll (2010). "Pharmacologic treatment of schizophrenia". Dialogues Clin Neurosci. 12 (3): 345–57. PMC . PMID 20954430. - Hartling L, Abou-Setta AM, Dursun S, et al. (14 August 2012). "Antipsychotics in Adults With Schizophrenia: Comparative Effectiveness of First-generation versus second-generation medications: a systematic review and meta-analysis". Annals of Internal Medicine. 157 (7): 498–511. doi:10.7326/0003-4819-157-7-201210020-00525. PMID 22893011. - Barry SJE, Gaughan TM, Hunter R; Gaughan, T. M.; Hunter, R (2012). "Schizophrenia". BMJ Clinical Evidence. 2012. PMC . PMID 23870705. - Schultz SH, North SW, Shields CG; North; Shields (June 2007). "Schizophrenia: a review". Am Fam Physician. 75 (12): 1821–9. PMID 17619525. - Smith T, Weston C, Lieberman J; Weston; Lieberman (August 2010). "Schizophrenia (maintenance treatment)". Am Fam Physician. 82 (4): 338–9. PMID 20704164. - Taylor DM; Taylor; Duncan-Mcconnell (2000). "Refractory schizophrenia and atypical antipsychotics". J Psychopharmacol. 14 (4): 409–418. doi:10.1177/026988110001400411. PMID 11198061. - Picchioni MM, Murray RM. Schizophrenia. BMJ. 2007;335(7610):91–5. doi:10.1136/bmj.39227.616447.BE. PMID 17626963. - Essali A, Al-Haj Haasan N, Li C, Rathbone J; Al-Haj Haasan; Li; Rathbone (2009). "Clozapine versus typical neuroleptic medication for schizophrenia". Cochrane Database of Systematic Reviews (1): CD000059. doi:10.1002/14651858.CD000059.pub2. PMID 19160174. - Ost, LG (October 2014). "The efficacy of Acceptance and Commitment Therapy: an updated systematic review and meta-analysis.". Behaviour research and therapy. 61: 105–21. doi:10.1016/j.brat.2014.07.018. PMID 25193001. - Birchwood, M; P. Todd; C. Jackson (1998). "Early Intervention in Psychosis: The Critical Period Hypothesis". British Journal of Psychiatry. 172 (33): 53–59. PMID 9764127. - Burgy, M. (20 August 2008). "The Concept of Psychosis: Historical and Phenomenological Aspects" (PDF). Schizophrenia Bulletin. 34 (6): 1200–1210. doi:10.1093/schbul/sbm136. PMC . PMID 18174608. - Beer, M D (1995). "Psychosis: from mental disorder to disease concept". Hist Psychiatry. 6 (22(II)): 177–200. doi:10.1177/0957154X9500602204. PMID 11639691. - "Psychosis, Henry George Liddell, Robert Scott, A Greek-English Lexicon, at Perseus". Perseus.tufts.edu. Retrieved 2011-06-11. - "Online Etymology Dictionary". Douglas Harper. 2001. Retrieved 2006-08-19. - Berrios G E (1987). "Historical Aspects of Psychoses: 19th Century Issues". British Medical Bulletin. 43 (3): 484–498. PMID 3322481. - Berrios G E, Beer D; Beer (1994). "The notion of Unitary Psychosis: a conceptual history". History of Psychiatry. 5 (17 Pt 1): 13–36. doi:10.1177/0957154X9400501702. PMID 11639278. - Porter, Roy (2003). Madness: A Brief History. USA: Oxford University Press. p. 10. ISBN 0192802674. - Vlachos, I.O; Beratis, Hartocollis (1997). "Magico religious beliefs and psychosis". Psychopathology. 30 (2): 93–99. doi:10.1159/000285035. PMID 9168565. - Pfeifer, S (September 1994). "Belief in demons and exorcism in psychiatric patients in Switzerland". British Journal of Medical Psychology. 67 (3): 247–258. doi:10.1111/j.2044-8341.1994.tb01794.x. - Bennet, S. (2008). "Mind and madness in classical antiquity". History of Psychiatry and Medical Psychology: 175–197. doi:10.1007/978-0-387-34708-0_3. ISBN 978-0-387-34707-3. - Spring, B; L. Weinstein; M. Lemon; A. Haskell (1991). "Schizophrenia from Hippocrates to Kraepelin". Clinical Psychology: 259–277. doi:10.1007/978-1-4757-9715-2_10. ISBN 978-1-4757-9717-6. - Rush, M.D., Benjamin (1830). Medical Inquiries and Observations upon Diseases of the Mind. Philadelphia. pp. 98–190. ISBN 978-0559921674. - Shorter, Edward (1998). A History of Psychiatry: From the Era of the Asylum to the Age of Prozac. Hoboken, New Jersey: John Wiley & Sons. ISBN 0471245313. - Stone, J.L. (March 2010). "Dr. Gottlieb Burckhardt--the pioneer of psychosurgery". Journal of the History of the Neurosciences. 10 (1): 79–92. doi:10.1076/jhin.10.1.79.5634. PMID 11446267. - Gross, D.; G. Schâfer (April 2011). "Egas Moniz and the "invention" of modern psychosurgery: A historical and ethical reanalysis under special consideration of portuguese original sources". Neurosurgical Focus. 30 (2): 8–10. doi:10.3171/2011.3.FOCUS10214a. - Pressman, Jack David (1998). Last Resort: Psychosurgery and the Limits of Medicine. Cambridge Studies in the History of Medicine. Cambridge, UK: Cambridge University Press. pp. 18–40. ISBN 978-0521353717. OCLC 36729044. - Berrios, G.E. (March 1997). "The origins of psychosurgery: Shaw, burckhardt and moniz". Hist. Psychiatry. 8 (29): 61–81. doi:10.1177/0957154X9700802905. PMID 11619209. - Mashour, George; Erin E. Walker; Robert L. Martuza (7 September 2005). "Psychosurgery: past, present, and future". Brain Research Reviews. 48 (3): 409–419. doi:10.1016/j.brainresrev.2004.09.002. PMID 15914249. - Stip, Emmanuel (22 January 2002). "Happy birthday neuroleptics! 50 year later: la folie du doute". European Psychiatry. 17 (3): 115–119. doi:10.1016/S0924-9338(02)00639-9. PMID 12052571. - Crossley, Nicolas; Miguel Constante (2010). "Efficacy of atypical v. typical antipsychotics in the treatment of early psychosis: meta-analysis". The British Journal of Psychiatry. 196 (6): 434–439. doi:10.1192/bjp.bp.109.066217. PMC . PMID 20513851. - Maher, Alicia; Margaret Maglione, Steven Bagley, Marika Suttorp, Jian-Hui Hu, Brett Ewing, Zhen Wang, Martha Timmer, David Sultzer, Paul Shekelle (28 September 2011). "Efficacy and Comparative Effectiveness of Atypical Antipsychotic Medications for Off-Label Uses in Adults A Systematic Review and Meta-analysis". The Journal of the American Medical Association. 306 (12): 1359–1369. doi:10.1001/jama.2011.1360. PMID 21954480. - Sims, A. (2002). Symptoms in the mind: An introduction to descriptive psychopathology (3rd edition). Edinburgh: Elsevier Science Ltd. ISBN 0-7020-2627-1 - Murray ED, Buttner N, Price BH. (2012). Depression and Psychosis in Neurological Practice. In: Neurology in Clinical Practice, 6th Edition. Bradley WG, Daroff RB, Fenichel GM, Jankovic J (eds.) Butterworth Heinemann. April 12, 2012. ISBN 1437704344 \| ISBN 978-1437704341 - Williams, Paris (2012). Rethinking Madness: Towards a Paradigm Shift In Our Understanding and Treatment of Psychosis, Sky’s Edge Publishing. - Personal accounts - Dick, P.K. (1981) VALIS. London: Gollancz. [Semi-autobiographical] ISBN 0-679-73446-5 - Jamison, K.R. (1995) An Unquiet Mind: A Memoir of Moods and Madness. London: Picador. - Schreber, Daniel Paul (2000) Memoirs of My Nervous Illness. New York: New York Review of Books. ISBN 0-940322-20-X - Hinshaw, S.P. (2002) The Years of Silence are Past: My Father's Life with Bipolar Disorder. Cambridge: Cambridge University Press. - McLean, R (2003) Recovered Not Cured: A Journey Through Schizophrenia. Allen & Unwin. Australia. ISBN 1-86508-974-5 - The Eden Express by Mark Vonnegut - James Tilly Matthews - Saks, Elyn R. (2007) The Center Cannot Hold—My Journey Through Madness. New York: Hyperion. ISBN 978-1-4013-0138-5	2	74	27	0	9	4	0.640178	40	17,243
In the mental health field, many psychiatric clinicians use the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV), and, since May 19, 2013, the updated DSM-5 for diagnosing mental disorders. Psychiatric specialists have knowledge and a comfort level with the DSM for clinical decision-making. When planning for ICD-10, it is important to understand CMS policy about the intended purpose of ICD-10 and the DSM. As Benjamin F. Miller, PsyD and Assistant Professor from the University of Colorado School of Medicine notes, the DSM-IV addressed diagnostic codes only for the purpose of describing mental disorders; but ICD-10-CM provides for both physical and mental disorders. “It is important to note that there are other differences between the DSM-V and ICD-10. For example, the DSM was produced by a single national professional association for psychiatrists (the American Psychiatric Association) while the ICD was produced by a global health agency with a public health mission to help countries reduce the disease burden including mental disorders; the DSM generates revenue for the American Psychiatric Association while the ICD is free through the internet; the DSM was developed primarily by psychiatrists with the ICD’s development being multidisciplinary; and, the DSM as a manual is approved by the American Psychiatric Association while the ICD is approved by the World Health Assembly.” See http://www.ncvhs.hhs.gov/wp-content/uploads/2014/05/130618p15a.pdf sourced February 22, 2014. It is not surprising that I have noted some conflicting information on the American Psychological Association (APA) bulletins on the use of the DSM and ICD-10. Going forward, CMS has made a clear statement about the intended use of ICD-10 with respect to reimbursement for Behavioral health providers. According to CMS, “…neither the DSM-IV nor the DSM-5 is a HIPAA Compliant code set. Therefore Behavioral Health specialists must use ICD-9 CM codes and after October 1, 2015 the appropriate ICD-10 CM diagnosis code.” Additionally, for inpatient procedures, ICD-10 PCS must also be used, and for ambulatory procedures, ICD-10 CM diagnosis must be paired with CPT codes. Do Fewer Codes for Behavioral Health Mean Less Complexity and an “out” from doing a proper ICD-10 Assessment? No. Here’s Why. There are relatively few increases in ICD-10 codes in behavioral health relative to high impact specialties such as Orthopedics. For example, there are 27 ICD-9 codes for Anxiety Disorders and 47 codes under ICD-10. Schizophrenia codes are reduced from 56 in ICD-9 to 11 in ICD-10. Therefore, some behavioral health providers assume in error that because there are fewer codes that the ICD-10 transition has little impact on their organization, which is not true. Because DSM is used for clinicians and ICD-10 for reimbursement, there is an increased level of complexity. Local and state requirements regarding the use of DSM and ICD-10 vary, which then causes you to consider: Can you eliminate DSM if not required in your state? Should you map or crosswalk from DSM-IV to DSM-5 to ICD-10? If this is your strategy you may consider: - This approach will require an understanding of changes in medical concepts between the DSV-IV and DSM-5, and whether you have DSM or ICD codes dependencies in your systems. - Education opportunities including helping behavioral health organizations appreciate the nuances of various reimbursement acronyms and standards that are inter-related. For example, a Behavioral specialty provider recently stated, “…we don’t use DRGs we use Inpatient Psychiatric Facility Prospective Payment System (IPF PPS).” In fact the facility is using DRGs. According to CMS …IPF PPS, Federal per diem rates include inpatient operating and capital-related costs (including routine and ancillary services) and are determined based on: - Geographic factors: - A hospital wage index value is assigned to account for geographic differences in wage - The non-labor-related portion accounts for higher cost of living for IPFs located in Alaska and Hawaii; - Patient characteristics: - Medicare Severity-Diagnosis Related Group (MS-DRG) classification; - Presence of specified comorbidities; and - Length of stay; and - Facility characteristics: - A 17 percent payment adjustment for rural facilities due to their higher costs; and - Teaching hospitals receive payment to account for indirect medical education costs… How to Successfully Transition Behavioral Health Organizations to ICD-10 - Ensure that you hire or retain consulting expertise knowledgeable in both DSM-5 and ICD-10, and the purpose and usage of these codes throughout the mental health specialty organization and physician group. - Include psychiatric clinicians with knowledge of the DSM on your team. - Ensure proper knowledge transfer to your coding, billing, clinical and operations staff. - Consider patient referral documentation and its impact on your reimbursement, and ensure that non-employed physicians as a source of referral are engaged as much as possible. - Identify those departments that are using DSM codes. - Determine if the codes being used are DSM-4 or DSM-5. - Evaluate patient intake systems, reporting, and more for DSM-4 or ICD-9 specific dependencies - Consider payor contracting factors. Even if your payor contracts are per-diem or if you have self-pay clients, you must consider the reporting and potential audit risk if you do not use the HIPAA required ICD-10 codes. Additional Factors – Affordable Care Act Quality Measures for Behavioral Health and ICD-10 Section 1886(s)(4)(C) of the Social Security Act, amended by sections 3401(f) and 10322(a) of the Affordable Care Act requires IPFs to report quality data for 6 quality measures starting in fiscal 2013 for Medicare beneficiaries. Beginning in FY 2015, two quality measures are added. Failure to comply with these regulations will result in a 2 percent reduction to the applicable base rate for IPFs. (Source: Medicare Learning Network publication Inpatient Psychiatric Facility Prospective Payment System).Why does this matter in the context of ICD-10? The quality measures will be based on diagnosis and procedures coded in ICD-10 beginning October 1, 2015. How to establish whether DSM and ICD-10 requirements impact your IT systems and processes - Consider the specificity of clinical documentation that your HIM systems support. If you use an Electronic Medical Record, determine whether it supports not only the proper level of specificity in the DSM (if it is required by your State or local laws) but also the specificity to support ICD-10. - Determine what impact these changes have on your revenue cycle management systems and processes. - In a recent engagement with a regional freestanding Behavioral health provider, we determined that at least forty (40) processes related to RCM are impacted. - Ensure that your systems support both the entry, storage, and reporting of both ICD-10 and the IPF quality measure reporting as above. Otherwise even Federal per diem rate facilities who might otherwise believe that ICD-10 has no impact may risk substantial reductions in their Medicare reimbursement, as well as other possible regulatory consequences. - Determine whether your analytic systems also need to be re-tooled based on ICD-10 and the resulting quality reporting requirements mentioned above. - Develop a test plan that comprehends all of these issues. Without testing the entire end-to-end process from patient chart to coding, billing, claim submission and reimbursement, your assumption that you will be ready on October 1, 2014 without a reimbursement or compliance risk will only be theoretical.	4	10	2	0	0	0	0.754132	2	1,644
Imagine, you visit your doctor after a long wait as it took a lot of time to get an appointment and you forgot your reports at home. You don’t want to miss this one chance of meeting your doctor as getting another appointment would mean a wait for another couple of days. What will you do in that case? Now think if the entire record of your disease is encrypted in one single code that you can get from the clinics health directory that provides you with the platform where you can get all the relevant information about the disease or the injury you are suffering from. This is now possible with the advancements in the medical standard associated with WHO under ICD-10 codes. Key facts associated with ICD Emphasizing on the US, ICD-10 is split into two systems: - ICD-10-CM: This is used for the diagnostic coding. - ICD-10-PCS: This is implemented for inpatient hospital procedure coding. - ICD is widely used in researches and clinical care to give an idea about the diseases and study their pattern. It also helps in managing the healthcare, calculate the effective outcomes and then allocate the resources. - ICD gives a standard idea about the mortality and morbidity rates. - The mortality data generated by the ICD denotes the health status of the country. It also keeps a check on the number of deaths and the disease rates. Purpose of ICD: - ICD is an international standard for reporting the health and disease conditions. It provides the world a platform to share as well as compare health information using a common language. - ICD holds all the information about the diseases, any health condition as well as the injuries. All the information is listed in such a comprehensive way that everything gets covered. - It gives a platform for sharing the health information among the hospitals situated in different locations and countries. - ICD helps in keeping a count of the diseases and deaths, their reasons and influences on general health status. Who can avail ICD-10 benefits? The main areas where ICD is taken into practice includes the research areas. General physicians, insurance policy makers, health program and information managers can also avail the benefits of ICD-10. ICD-10 Code structure: ICD-10 CM diagnosis have codes ranging between 3 to 7 characters. - The initial three characters of an ICD-10 code tells about the category of diagnosis. For example if we consider S86.011.D; the letter S tells us about the diagnosis related to “injuries, poisoning or any certain causes that are related to single body regions externally.” S along with 8 and 6 tells that it indicates an “injury of tendon or muscle in the lower portion of the leg. - The next two characters “0”, “1”, “1” specifies the diagnosis of the ” strain in the right Achilles tendon.” - The last character that is the seventh character tells us about the basic difference between ICD-0 and the ICD-10 as ICD-9 did not include the seventh character. The seventh character tells about the treatment and care. Here in this example, the seventh character deals with the injury. The character D specifies the phase when the patient is receiving routine care during the healing time of recovery. Why should you go for ICD-10 procedures? The reason to go for ICD-10 can be divided into three categories: - It gives an elaborate idea about the patient’s condition and helps in giving improved healthcare teams. - It targets on the capital investments to meet the needs of the specific patient’s condition. - This provides the basic data for comparison and utilization benchmarking. - Reduces the risks associated with an audit by implementing the diagnosis codes with high specificity that is supported by the clinical research documentation. - Since, better data is collected and evaluated, it helps in taking better decisions. - Gives a broader platform for research and clinical trials.	4	4	0	0	0	2	0.751784	2	842
Diagnosis Code F32.81 Information for Medical Professionals The following edits are applicable to this code: Diagnoses for females only - Diagnoses for females only. Convert to ICD-9 - 625.4 - Premenstrual tension (Approximate Flag) - Premenstrual Dysphoric Disorder: A condition in which a woman suffers from severe depression, irritability, and tension before MENSTRUATION. Premenstrual dysphoric disorder (PMDD) may involve a wide range of physical or emotional symptoms, which are more severe and debilitating than those seen with premenstrual syndrome (PMS), and which include at least one mood-related symptom. Symptoms usually stop when, or shortly after, menstruation begins. - Minor depressive disorder - Premenstrual dysphoric disorder - Premenstrual dysphoric disorder in remission Index to Diseases and Injuries References found for the code F32.81 in the Index to Diseases and Injuries: Tabular List of Diseases and Injuries References found for the code F32.81 in the Tabular List of Diseases and Injuries: - Type 1 Excludes Notes: - premenstrual tension syndrome (N94.3) This code replaces the following previously assigned ICD-10 code(s) listed below: - F32.8 - Other depressive episodes Information for Patients Also called: Clinical depression, Dysthymic disorder, Major depressive disorder, Unipolar depression Depression is a serious medical illness. It's more than just a feeling of being sad or "blue" for a few days. If you are one of the more than 19 million teens and adults in the United States who have depression, the feelings do not go away. They persist and interfere with your everyday life. Symptoms can include - Feeling sad or "empty" - Loss of interest in favorite activities - Overeating, or not wanting to eat at all - Not being able to sleep, or sleeping too much - Feeling very tired - Feeling hopeless, irritable, anxious, or guilty - Aches or pains, headaches, cramps, or digestive problems - Thoughts of death or suicide Depression is a disorder of the brain. There are a variety of causes, including genetic, biological, environmental, and psychological factors. Depression can happen at any age, but it often begins in teens and young adults. It is much more common in women. Women can also get postpartum depression after the birth of a baby. Some people get seasonal affective disorder in the winter. Depression is one part of bipolar disorder. There are effective treatments for depression, including antidepressants, talk therapy, or both. NIH: National Institute of Mental Health - Depression (Medical Encyclopedia) - Depression - elderly (Medical Encyclopedia) - Depression - stopping your medicines (Medical Encyclopedia) - Dysthymia (Medical Encyclopedia) - Heart disease and depression (Medical Encyclopedia) - Learning about depression (Medical Encyclopedia) - Major depression (Medical Encyclopedia) - Major depression with psychotic features (Medical Encyclopedia) Also called: PMS Premenstrual syndrome, or PMS, is a group of symptoms that start one to two weeks before your period. Most women have at least some symptoms of PMS, and the symptoms go away after their periods start. For some women, the symptoms are severe enough to interfere with their lives. They have a type of PMS called premenstrual dysphoric disorder, or PMDD. Common PMS symptoms include - Breast swelling and tenderness - Bloating and weight gain - Pain - headache or joint pain - Food cravings - Irritability, mood swings, crying spells, depression No one knows what causes PMS, but hormonal changes trigger the symptoms. No single PMS treatment works for everyone. Over-the-counter pain relievers such as ibuprofen, aspirin or naproxen may help ease cramps, headaches, backaches and breast tenderness. Exercising, getting enough sleep, and avoiding salt, caffeine, and alcohol can also help. Dept. of Health and Human Services Office on Women's Health - Breast - premenstrual tenderness and swelling (Medical Encyclopedia) - Premenstrual dysphoric disorder (Medical Encyclopedia) - Premenstrual syndrome (Medical Encyclopedia) - Premenstrual syndrome - self-care (Medical Encyclopedia) Depression Depression (also known as major depression or major depressive disorder) is a psychiatric disorder that affects mood, behavior, and overall health. It causes prolonged feelings of sadness, emptiness, or hopelessness, and a loss of interest in activities that were once enjoyed. People with depression may also have changes in appetite (leading to overeating or not eating enough), changes in sleeping patterns (sleeping too much or not being able to sleep), loss of energy, and difficulty concentrating. Although depression is considered primarily a mental health disorder, it can also have physical features including headaches, other unexplained aches and pains, unusually slow or fast movements, and digestive problems. To be diagnosed with depression, an individual must have signs and symptoms nearly every day for at least 2 weeks. However, the features of this condition vary widely.Depression most commonly begins in late adolescence or early adulthood, although it can appear at any age. If untreated, episodes of depression can last for weeks, months, or years, and can go away and come back (recur). Affected individuals may have difficulty functioning in their daily lives, including at school or work. People with depression have a higher risk of substance abuse problems and dying by suicide than the general population.Several health conditions are closely related to depression or have depression as a characteristic feature. These include dysthymia (which has long-lasting signs and symptoms that are similar to, but not as severe as, those of depression), perinatal or postpartum depression (which occurs around or following the birth of a child), seasonal affective disorder (which is triggered by the changing of the seasons), bipolar disorder (which can include both "highs," or manic episodes, and depressive episodes), and generalized anxiety disorder. In people with schizoaffective disorder, depression or another mood disorder occurs together with features of schizophrenia (a brain disorder that affects a person's thinking, sense of self, and perceptions). General Equivalence Map Definitions The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs. - Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent. - No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system. - Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system. Index of Diseases and Injuries Definitions - And - The word "and" should be interpreted to mean either "and" or "or" when it appears in a title. - Code also note - A "code also" note instructs that two codes may be required to fully describe a condition, but this note does not provide sequencing direction. - Code first - Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions, the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists, there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation. - Type 1 Excludes Notes - A type 1 Excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition. - Type 2 Excludes Notes - A type 2 Excludes note represents "Not included here". An excludes2 note indicates that the condition excluded is not part of the condition represented by the code, but a patient may have both conditions at the same time. When an Excludes2 note appears under a code, it is acceptable to use both the code and the excluded code together, when appropriate. - Includes Notes - This note appears immediately under a three character code title to further define, or give examples of, the content of the category. - Inclusion terms - List of terms is included under some codes. These terms are the conditions for which that code is to be used. The terms may be synonyms of the code title, or, in the case of "other specified" codes, the terms are a list of the various conditions assigned to that code. The inclusion terms are not necessarily exhaustive. Additional terms found only in the Alphabetic Index may also be assigned to a code. - NEC "Not elsewhere classifiable" - This abbreviation in the Alphabetic Index represents "other specified". When a specific code is not available for a condition, the Alphabetic Index directs the coder to the "other specified” code in the Tabular List. - NOS "Not otherwise specified" - This abbreviation is the equivalent of unspecified. - See - The "see" instruction following a main term in the Alphabetic Index indicates that another term should be referenced. It is necessary to go to the main term referenced with the "see" note to locate the correct code. - See Also - A "see also" instruction following a main term in the Alphabetic Index instructs that there is another main term that may also be referenced that may provide additional Alphabetic Index entries that may be useful. It is not necessary to follow the "see also" note when the original main term provides the necessary code. - 7th Characters - Certain ICD-10-CM categories have applicable 7th characters. The applicable 7th character is required for all codes within the category, or as the notes in the Tabular List instruct. The 7th character must always be the 7th character in the data field. If a code that requires a 7th character is not 6 characters, a placeholder X must be used to fill in the empty characters. - With - The word "with" should be interpreted to mean "associated with" or "due to" when it appears in a code title, the Alphabetic Index, or an instructional note in the Tabular List. The word "with" in the Alphabetic Index is sequenced immediately following the main term, not in alphabetical order. Present on Admission The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.	4	4	0	0	0	4	0.979309	4	2,401
There are many health facilities and medical practices who often find it difficult to implement the specific ICD-10 codes for abdominal pain. Most are stumped as to why medical coders use the code for unspecified abdominal pain i.e. R10.9 instead of using a more specific code. Well, the answer is simple: it all depends on the documentation. If the healthcare provider identifies that the reason for the exam is only abdominal pain, then the unspecified code for abdominal pain can be used. However, when documenting cases of abdominal pain, it is important to not only identify the pain location e.g. right upper quadrant or left upper quadrant, but also the type of pain that is being experienced by the patient which can include colic, rebound, tenderness or chronic pain. For instance, if a radiologist writes an MRI test for the abdomen, the prescription should clearly mention the specific location of the abdomen where the MRI has to be performed since the information is then later used by the coders to code the specific diagnosis. If any information is missing, the coding staff should query the ordering physician to get full details. The adoption of the ICD 10 code set in 2015 meant that radiology coders have to assign the correct and most appropriate codes for their cases. In this article, we briefly look at how pain is classified in the ICD 10, the rules when coding for pain in the abdomen and focus on the ICD 10 code for RUQ pain (right upper quadrant). Classification of Pain in the ICD 10 Codes related to pain are classified in three ways in the ICD 10 manual, which are: – Pain that is the result from a disorder is found in the body system chapters. For example, testicular pain is classified under the Genitourinary System (N50.8) Chapter. – Some specific types of pain which are not elsewhere classified are found in the category G89 in the chapter about Nervous System – Pain that cannot be traced to a specific body system is classified under the Symptoms and Signs Chapter, including ICD 10 RUQ pain. What is Abdominal Pain? Abdominal pain is described as having discomfort or ache in the belly, from the ribs to the pelvis. Abdominal pain and stomach pain are terms that are used interchangeably, even though pain in the abdomen can be caused by issues in a number of organs besides the stomach. To specify the pain, the abdomen is divided into further sections to easily diagnose and treat the underlying cause. The sections include right upper quadrant, left upper quadrant, right lower quadrant and left lower quadrant. A brief episode of pain is described as acute abdomen pain. Pain that has been there for more than three months is described as being chronic. Abdominal pain is found in many forms, ranging from dull aches to sudden stabs and cramps in the abdomen area and organs. Even a mild pain can be an early sign of a serious issue and should be monitored and treated as soon as possible. Since this article is regarding ICD 10 RUQ pain (right upper quadrant), describing all the structures and organs of the abdominal would exceed the scope. Instead, we will be focusing entirely on the right upper quadrant. The RUQ includes the gallbladder, biliary system and the liver, and each of these organs can cause pain in the abdomen. The gallbladder can be a source of abdominal pain if the patient has stones which leads to biliary colic. Liver issues that can cause pain include inflammation of the liver, hepatic abscess and cirrhosis. Types of Abdominal Pain in the ICD 10 The ICD 10 has numerous codes pertaining to the R10 category for both pelvic and abdominal pain. Apart from the codes for the different location in which the pain is found in the abdomen, there are different types of pains including: Abdominal tenderness: Tenderness means to have an abnormal sensitivity to touch. Pain is identified as the patient’s symptoms and tenderness is the reaction that the physician observes. Rebound abdominal tenderness: Simple tenderness is the discomforting reaction when pressure is applied to the abdomen. On the other hand, rebound tenderness happens when pressure is released from the abdomen. Acute abdomen: Acute abdomen is characterized by severe pain in the abdomen usually followed by rigidity in the area as well. Patients that have this type of pain often have to undergo surgery to feel better, such as peritonitis and acute appendicitis. Colic: This is a pain in the abdomen that comes in waves, most often caused by contractions in the ureter or the intestines. Flank Pain: Flank is the area that falls below the ribs, and is usually caused by kidney stones. When coding for flank pain, it is often advised to use the unspecified abdominal pain code i.e R10.9, unless there is additional information provided by the health physician, such as the upper or lower quadrant. Pelvic Pain: R10.2 is used to classify pain in the pelvic for both males and females. Perinea pain is also included in this classification, which is the pain in the area of a woman’s vulva or anus, or the area between a man’s scrotum and anus. About the ICD 10 The International Classification of Diseases, tenth revision is a clinical system applied by healthcare providers and physicians to code and classify the diseases, diagnoses, symptoms and procedures that are recorded during health care provided. The ICD 10 is important to compile diagnostic specificity and morbidity data in the US. The International Classification of Diseases is published by the World Health Organization, used by physicians, health information managers, coders, nurses and other professionals associated with the healthcare sector for storage and retrieval of information relating to diagnosis. In the bigger picture, the data is compiled to provide national morbidity and mortality statistics. The ICD 10 comprises known disease and health problems listed in a systematic way, and uses unique alphanumeric codes that correspond to each disease and condition to make identification easy. After the ICD 10 was formally regulated in the US health system, all HIPAA (Health Insurance Portability and Accountability Act) compliant/covered entities are required to adhere to the ICD 10 code sets. Coding for ICD 10 Abdominal Pain When coding for abdominal pain or more specifically like ICD 10 RUQ abd pain, it is important to keep in mind the following qualifiers: 1) All codes for abdominal pain begin with R10 2) Up to three numbers can be added to R10 to provide a more specific diagnosis. 3) Do not record codes for renal colic along with abdominal pain codes. 4) Codes for abdominal pain do not cover flatulence or dorsalgia related conditions. R10 is used to define the category for abdominal and pelvic pain. Below is a list of most commonly used codes for the ICD 10 RUQ pain and tenderness. R10.11 – RUQ pain ICD 10 R10.811 – RUQ tenderness ICD 10 Guidelines for Coding Abdominal Pain ICD 10 It is important to remember the codes in the ICD 10 for abdominal pain are used to describe symptoms, not the specific diagnoses. This means that the codes should be used in those cases when a concrete diagnosis has not yet been reached by the healthcare provider. The codes can also be used in the cases when the abdominal pain symptoms occur alongside another symptom that is not associated with the condition, but the code for the main symptoms should be identified and documented first. Subsequently, unspecified codes should only be used strictly when there is insufficient information from the provider to allow for a more specific assignment of code. It is required to be as accurate as possible when recording with the ICD 10. For instance, a case where the location is specified for the pain in the right upper quadrant should be assigned the corresponding abdominal pain RUQ ICD 10. If the pain occurs in two or more places, separate codes need to be assigned for each location. The ICD 10 is now in its sixth year since the US healthcare system completely adopted the code set. If you think that you need to take your coding skills to the next level, or fell that your radiology practice lacks the knowledge when it comes to coding in the ICD 10, we invite you to discuss with the experts at UControl Billing where we have specialized manuals and staff for radiology ICD 10 coding. Additionally, if you have a staff to train, a UControl Billing staff can deliver the required training at your location. We make sure that we help find the best medical billing and coding solution for your organization.	4	2	0	0	0	16	0.823959	16	1,817
- ICD-9 379.23 - ICD-10 H43.1 Vitreous Hemorrhage is a relatively common cause of acute vision loss, having an incidence of approximately 7 cases per 100,000, 4.8 per 10000 in Taiwan, and may vary according to population characteristic, geography, and other factors. It is therefore frequently encountered by ophthalmologists and Emergency Room professionals alike due to its often rapid onset which causes painless, but substantial vision loss. Although the diagnosis of vitreous hemorrhage is often straightforward to make on funduscopic examination or ultrasonography, further investigation may be required to determine the underlying etiology. The frequency of the etiologies of vitreous hemorrhage is variable according to the characteristics of the study population. The three most common causes include - proliferative diabetic retinopathy (PDR), - posterior vitreous detachment (PVD) with or without retinal tear, and - ocular trauma, which account for 59-88.5% of all cases. Less common causes of vitreous hemorrhage include - retinal vein occlusion, - retinal vasculitis, - proliferative sickle cell retinopathy, - retinal arterial macroanuerysm, - subarachnoid hemorrhage (Terson syndrome), - Valsalva retiniopathy - other disorders including X-linked retinochisis, retinopathy of prematurity, familial exudative vitreoretinopathy, intermediate uveitis, - Blood dyscrasias/coagulation disorders, and - neovascular age-related macular degeneration (See Differential Diagnosis, below). The population at risk for vitreous hemorrhage will have the demographic and clinical characteristics according to its causes. For example, poorly controlled diabetics with end-organ damage such as proliferative diabetic retinopathy are at high risk. People younger than 40 with vitreous hemorrhage often have a history of recent ocular trauma whereas older, non-diabetic populations with vitreous hemorrhage often suffered an acute PVD and/or retinal tear. Although anticoagulants and antiplatelet agents do not likely cause spontaneous vitreous hemorrhage, they may enhance bleeding from pathology. Notably, however, the Early Treatment of Diabetic Retinopathy Study did not show increased risk of vitreous hemorrhage among aspirin users. Patients with systemic coagulation disorders and blood dyscrasias such as leukemia and thrombocytopenia may have an increased risk of vitreous hemorrhage, but these cases are rare. Extravasation of blood into the vitreous cavity is generally caused by two basic mechanisms: - Rupture of normal vessels through mechanical force: - Closed globe injury from blunt trauma: Compression of the globe in an anterior-posterior direction causes the equator of the globe to bulge in a coronal plane. Especially in a young patient with formed vitreous and strong adherence of the vitreous to the retina, this bulging in the coronal plane causes inward-directed traction exerted by the vitreous on the retina. The fact that the vitreous base, an area of especially strong vitreous attachment, is near the equator contributes to the tractional force the vitreous exerts on the retina in this area. This inward-directed tractional force on the retina by the vitreous can cause a retinal dialysis, but it can also result in retinal tears and vitreous hemorrhage as retinal vessels may be ruptured. Blunt trauma may also rupture blood vessels associated with the iris and ciliary body, causing hyphema and spillover hemorrhage into vitreous. - Open globe injury: Blunt or sharp trauma causing a full-thickness defect in the eyewall may cause hemorrhage in all layers of the eye, including vitreous hemorrhage. - Shaken baby syndrome: The trauma associated with shaken baby syndrome may cause hemorrhage in all ocular layers, including vitreous hemorrhage. It has been suggested that vitreous hemorrhage in this context may portend a worse prognosis. - Acute PVD: Vitreous hemorrhage in the setting of acute PVD is associated with a retinal tear or break in 70-95% of cases and should invoke an exhaustive search for retinal breaks. - Terson syndrome: Terson syndrome is a rare cause of vitreous hemorrhage associated with subarachnoid hemorrhage. The vitreous hemorrhage is not a direct extension of subarachnoid hemorrhage into the eye via the optic nerve sheath. Rather, increased intracranial pressure causes increased pressure in retinal venules, causing them to rupture. Sub-internal limiting membrane (sub-ILM) hemorrhage is noted and intraoperatively a break may be noted in the ILM through which the blood is thought to reach the vitreus gel from the sub-ILM cavity. - Hemorrhage from pathologic structures: - Rupture of retinal neovascularization: Retinal ischemia caused by conditions such as diabetic retinopathy, retinal vein occlusion, sickle cell retinopathy, and retinopathy of prematurity promote the growth of new vessels (neovascularization) via the elaboration of angiogenic factors such as vascular endothelial growth factor (VEGF), basic fibroblast growth factor, and insulinlike growth factor. The neovascular vessels grow into the vitreous and are fragile. Normal eye movements, acute PVD, and fibrovascular contraction of the neovascularization can all cause these vessels to bleed. - Retinal macroaneurysms and acutely occluded retinal venules in retinal vein occlusion may rupture, causing vitreous hemorrhage. - Choroidal tumors, or choroidal neovascularization secondary to conditions such as age-related macular degeneration can cause "break-through" bleeding, through the retina and into the vitreous. Hemorrhage into the vitreous body results in rapid clot formation and clears at a rate of approximately 1% per day . Erythrocytes exit through the trabecular meshwork, undergo hemolysis, phagocytosis, or persist within the vitreous for many months. The cellular response to erythrocytes in the vitreous is unusual because there is no early polymorphonuclear cell response and the ensuing inflammatory response is instead more similar to a "low-turnover" granuloma. The muted inflammatory response in the immunologically privileged eye serves to mitigate ocular tissue damage and promote clarity of the visual axis. Primary prevention should be directed at controlling risk factors for systemic vascular disease such as diabetes, hypertension, and smoking. Frequent dilated fundus exams can reveal advanced retinopathy in high risk populations and provide the opportunity for therapeutic intervention. Proper eye protection should be worn during activities likely to cause eye trauma (e.g. hammering or grinding metal, using firearms, playing sports with high-speed balls such as racquetball). Evidence of vitreous hemorrhage is often seen on physical exam and inferred through history. Systemic past medical and ocular history can help lead to diagnosis. Sudden, painless visual loss or haze is a common presentation. Patients may describe a red hue to their vision. Patients may describe new onset floaters, shadows, or "cobwebs". Symptoms may be worse in the morning if blood settles on the macula during the night. History of diabetes, hypertension, sickle cell disease, trauma, previous retinal conditions or ocular surgery may help lead to the diagnosis. Visual acuity is variable based on the location, size, and degree of vitreous hemorrhage. In severe cases, patients may have dramatically reduced visual acuity and/or visual field defects. Slit lamp examination usually reveals red blood cells in the anterior vitreous. Presence of iris rubeosis and intraocular pressure should be noted. Gonioscopy should be used to detect neovascularization of the angle. Dilated fundus exam may reveal hemorrhage diffusely spread throughout the vitreous cavity, or the blood may conform to the anatomy of the vitreous. For example, hemorrhage in the subhyaloid space may result in a scaphoid (boat-shaped) hemorrhage. Occasionally, a detached posterior hyaloid face will have blood adherent to its posterior surface. If vitreous hemorrhage is associated with acute PVD, retinal tear or detachment should be ruled out using scleral depression. It is important to thoroughly examine the fellow eye, as it will often reveal clues as to the etiology. In chronic vitreous hemorrhage, the red blood cells become dehemoglobinized and the hemorrhage takes on a khaki color or even yellow or white color. If the vitreous hemorrhage obscures a complete view to the retina, B-scan ultrasonography can detect vitreous hemorrhage, PVD, retinal tears, retinal detachment, tractional membranes, intraocular tumors, and foreign bodies. Fluorescein angiography may be useful in the setting of mild to moderate vitreous hemorrhage to help identify neovascularization. If an open globe injury is suspected, orbital CT scan is indicated to characterize the orbital bony structures, assess the integrity of posterior the eyewall (though clinically its presence is denoted by hypotony and other features), and rule out intraocular foreign body. Blood pressure should also be checked. If familial exudative vitreoretinopathy is suspected, examination of family members is helpful. Laboratory testing is used according to clinical suspicion to diagnose diabetes, sickle cell disease, leukemia, thrombocytopenia, and other hematologic abnormalities. - Posterior vitreous detachment with or without retinal tear, with or without rhegmatogenous retinal detachment - With neovascularization - Proliferative diabetic retinopathy - Retinal vein occlusion - Hypertensive retinopathy - Sickle retinopathy - Radiation retinopathy - Retinopathy of prematurity - Familial exudative vitreoretinopathy - Nonperfusion from retinal vasculitis (e.g. sarcoidosis) - Without neovascularization - Retinal vein occlusion - Familial retinal arteriolar tortuosity - With neovascularization - Choroidal neovascularization secondary to: - Age-related macular degeneration - Peripheral exudative hemorrhagic chorioretinopathy - Pathologic myopia - Inflammatory/infectious choroidopathies (e.g. sarcoidosis, punctate inner choroidopathy, presumed ocular histoplasmosis syndrome, etc) - Choroidal tumors (e.g. uveal melanoma) - Choroidal neovascularization secondary to: - Shaken baby syndrome - Closed globe injury - Open globe injury - Avulsion of prepapillary loop - Valsalva maneuver - Blood disorders (thrombocytopenia, leukemia, hemoglobinopathies, etc.) - Terson syndrome - Inflammatory/infectious conditions causing vitritis mimicking chronic vitreous hemorrhage (multifocal choroiditis with panuveitis, endophthalmitis, etc) - Primary central nervous system lymphoma masquerading as chronic vitreous hemorrhage Treat the underlying etiology as soon as possible. If the retina can be adequately visualized and the etiology of the vitreous hemorrhage can be determined, treatment of the underlying cause may be attempted, if needed. If the retina can be adequately visualized and the etiology of the vitreous hemorrhage can be determined, but the vitreous hemorrhage does not permit safe treatment of the underlying etiology, pars plana vitrectomy is indicated. A short period of cautious observation for vitreous clearing may be reasonable. For example, occasionally a retinal tear associated with vitreous hemorrhage can be seen with a bright indirect ophthalmoscope, but adequate uptake of laser spots to the posterior margin of the break cannot be achieved. Red laser may be tried in such cases as it has better penetration. Cryotherapy can be considered in these cases, but the dual risk of cryotherapy and vitreous hemorrhage potentially leading to proliferative vitreoretinopathy should be considered compared to the risk of pars plana vitrectomy with endolaser. If the retina cannot be adequately visualized in 360 degrees and the etiology of the vitreous hemorrhage is unknown, prompt pars plana vitrectomy is indicated. Again, a short period of cautious observation for vitreous clearing may be reasonable. For example, in a case of a superior retinal tear with dense vitreous hemorrhage, adequate treatment of the superior retinal tear may be achieved. However, undetected inferior retinal breaks may be present. Between office visits, retinal detachment may progress underneath vitreous hemorrhage without a change in the patient’s symptoms. Therefore, if the view is not clearing briskly, pars plana vitrectomy is indicated. Neovascularization of the iris or angle in the setting of new dense vitreous hemorrhage would prompt earlier surgical intervention. Diabetics frequently present with vitreous hemorrhage, and in general the same principles apply as outlined above. Of note, new vitreous hemorrhage in diabetics cannot always be assumed to be secondary to diabetic retinopathy if there is an inadequate view; diabetics are susceptible to retinal tears and detachments like the general population. The severity of the fellow eye may give a clue as to the etiology of the vitreous hemorrhage, but asymmetric levels of retinopathy are relatively common. In any case, diabetic patients with proliferative diabetic retinopathy, new vitreous hemorrhage prohibiting adequate panretinal photocoagulation, and no history of panretinal photocoagulation benefit from pars plana vitrectomy with intra-operative panretinal photocoagulation or intravitreal anti-vascular endothelial growth factor (anti-VEGF) injection. In this scenario, if the vitreous hemorrhage does not clear in about one month, many surgeons will perform pars plana vitrectomy. However, in the case of an established patient with known proliferative diabetic retinopathy and known panretinal photocoagulation, a new or recurrent vitreous hemorrhage is far more likely to be secondary to diabetes than a retinal tear. Longer periods of observation (3 to 6 months) before considering pars plana vitrectomy may be reasonable in these situations. As always, individual situations, patient wishes, and surgeon judgment are paramount. Patients are instructed to minimize strenuous activity, as an increase in blood pressure may disrupt the newly formed clot and cause new active bleeding. Patients are also instructed to keep their head of bed elevated to allow settling of the blood, improving their vision and permitting more complete fundoscopic examination. Bilateral patching and bedrest may facilitate settling of blood. However, the patches must be removed immediately before examination or treatment, as normal eye movements quickly disperse the hemorrhage again. For this reason and its inconvenience to patients, bilateral patching is infrequently attempted. Neovascularization from proliferative retinopathy, associated with diabetes or otherwise, is often treated with panretinal photocoagulation if the view is adequate. This will cause regression of neovascularization and help reduce the risk of further hemorrhage. Intravitreal injection of anti-VEGF agents may be used to cause regression of neovascularization in proliferative retinopathies, particularly if there is no view to perform panretinal photocoagulation. Bhavsar et al. compared the use of ranibizumab with saline injected intravitreally for patients with proliferative diabetic retinopathy-associated vitreous hemorrhage in a randomized trial, and found that there seemed to be no difference in vitrectomy rates at 16 weeks between the two groups. Of note, the recent Protocol S data from the Diabetic Retinopathy Clinical Research Network (DRCR.net) compared the use of panretinal photocoagulation versus intravitreal ranibizumab for proliferative diabetic retinopathy; at five years, the rates of vitreous hemorrhage were similar (nearly 50%) in both groups. Additionally, there is anecdotal evidence that anti-VEGF injection may worsen tractional retinal detachment as neovascular membranes contract, so the the potential risks and benefits should be considered in this setting. Recently, the initial results from Protocol AB from the Diabetic Retinopathy Clinical Research Retina Network evaluated 100 patients with vitreous hemorrhage from proliferative diabetic retinopathy initially treated with aflibercept vs. 105 patients with vitreous hemorrhage initially treated with pars plana vitrectomy with laser photocoagulation. At 24 weeks, there was no statistically difference in the primary outcome of mean visual acuity letter score between the vitrectomy and aflibercept cohorts, although the vitrectomy group showed a faster visual recovery. Approximately one-third of patients in the aflibercept cohort required vitrectomy during follow-up compared with 8% of the patients in the vitrectomy/laser cohort. Many surgeons use pre-operative anti-VEGF agents 1 to 7 days before pars plana vitrectomy for vitreous hemorrhage in diabetics, as regression of neovascular membranes reduces intra- and post-operative bleeding and dissection of tissue may become easier. Several small studies support this belief, although other small studies refute it. There is concern, however, that these patients frequently fail their pre-anesthesia testing and that their surgery may be cancelled after the anti-VEGF agent has been given, potentially exacerbating tractional retinal detachment. For this reason, many surgeons wait until the patient is medically cleared for surgery before giving the anti-VEGF agent. Intravitreal injection of an anti-VEGF agent is usually indicated when the cause of vitreous hemorrhage is neovascular age-related macular degeneration. The Early Treatment of Diabetic Retinopathy Study showed that aspirin did not increase risk of vitreous hemorrhage, and no anticoagulant has been definitively shown to increase risk of vitreous hemorrhage. One report showed that patients taking aspirin, clopidogrel, or warfarin who develop an acute PVD are more likely to develop a vitreous hemorrhage, although the difference was small. Most clinicians do not recommend discontinuation of anticoagulation with the goal of resolving a vitreous hemorrhage, especially when the anticoagulation is medically indicated. Medical follow up Patients with systemic causes of vitreous hemorrhage are followed closely by an internist or endocrinologist in addition to close ophthalmology follow up. Pars plana vitrectomy is indicated for vitreous hemorrhage accompanied by retinal detachment or break seen on B-scan, nonclearing vitreous hemorrhage, many cases of intraocular foreign body, vitreous hemorrhage with iris neovascularization or associated with hemolytic or ghost-cell glaucoma. Pars plana vitrectomy is also indicated in cases of dense vitreous hemorrhage of unknown etiology. In these cases, pars plana vitrectomy can be both diagnostic and therapeutic. Depending on the etiology of vitreous hemorrhage, endolaser may also be placed intraoperatively. Some surgeons will also perform a concurrent air-fluid exchange after the vitrectomy or may even inject intravitreal anti-VEGF at the conclusion of case, but there is no substantial evidence that this prevents post-operative recurrent vitreous hemorrhage, a frustrating occurrence seen in some patients, especially diabetic patients. In cases of open globe injury with vitreous hemorrhage but without intraocular foreign body, most surgeons close the eyewall first and address the vitreous hemorrhage as a second stage procedure. Surgical follow up Frequency of follow up is specific to the surgical indication. The prognosis is variable according to the etiology, status of optic nerve, and macular involvement. For example, patients with vitreous hemorrhage secondary to proliferative diabetic retinopathy or age-related macular degeneration will have a more guarded prognosis compared to those with vitreous hemorrhage resulting from posterior vitreous detachment. Thus, it is important to counsel the patient pre-operatively that prognosis might be guarded, especially since a thorough assessment is precluded while the vitreous hemorrhage is present. - Spraul, CW and Grossniklaus, HE. Vitreous Hemorrhage. Surv Ophthalmol. 42:3-39, 1997. - Wang CY, Cheang WM, Hwang DK, Lin CH. Vitreous haemorrhage: a population-based study of the incidence and risk factors in Taiwan. Int J Ophthalmol. 2017;10(3):461‐466. Published 2017 Mar 18. doi:10.18240/ijo.2017.03.21 - Witmer MT, Cohen SM. Oral anticoagulation and the risk of vitreous hemorrhage and retinal tears in eyes with acute posterior vitreous detachment. Retina. 2013 Mar;33(3):621-6. - Early Treatment Diabetic Retinopathy Study design and baseline patient characteristics. ETDRS report number 7. Ophthalmology. 1991 May;98(5Suppl):741-56. - Matthews GP, Das A. Dense vitreous hemorrhages predict poor visual and neurological prognosis in infants with shaken baby syndrome. J Pediatr Ophthalmol Strabismus. 1996 Jul-Aug;33(4):260-5. - Rinaldi C, Bhatnagar P, Yannuzzi LA. Familial retinal arteriolar tortuosity associated with retinal and vitreous hemorrhages. Retin Cases Brief Rep. 2011 Spring;5(2):157-9. - Wickham L, Bunce C, Wong D, Charteris DG. Retinal detachment repair by vitrectomy: simplified formulae to estimate the risk of failure. Br J Ophthalmol. 2011 Sep;95(9):1239-44. - Ahmadieh H, Shoeibi N, Entezari M, Monshizadeh R. Intravitreal bevacizumab for prevention of early postvitrectomy hemorrhage in diabetic patients: a randomized clinical trial. Ophthalmology. 2009 Oct;116(10):1943-8. - Bhavsar AR, Torres K, Beck, RW, et al.; Diabetic Retinopathy Clinical Research Network Study Investigators. Randomized Clinical Trial Evaluating Intravitreal Ranibizumab or Saline for Vitreous Hemorrhage from Proliferative Diabetic Retinopathy. JAMA Ophthalmol. 2013 Mar; 131(3): 283–293. - Gross JG, Glassman AR, Liu D, et al.;Diabetic Retinopathy Clinical Research Network. Five-Year Outcomes of Panretinal Photocoagulation vs Intravitreous Ranibizumab for Proliferative Diabetic Retinopathy: A Randomized Clinical Trial. JAMA Ophthalmol. 2018 Oct 1;136(10):1138-1148. - Antoszyk AN, Glassman AR, Beaulieu WT, et al, for the DRCR Retina Network. Effect of intravitreous aflibercept vs vitrectomy with panretinal photocoagulation on visual acuity in patients with vitreous hemorrhage from proliferative diabetic retinopathy: a randomized clinical trial. JAMA. 2020;324:2383-2395. - Manabe A, Shimada H, Hattori T, Nakashizuka H, Yuzawa M. RANDOMIZED CONTROLLED STUDY OF INTRAVITREAL BEVACIZUMAB 0.16 MG INJECTED ONE DAY BEFORE SURGERY FOR PROLIFERATIVE DIABETIC RETINOPATHY. Retina. 2015 Apr 29. [Epub ahead of print] - di Lauro R, De Ruggiero P, di Lauro R, di Lauro MT, Romano MR. Intravitreal bevacizumab for surgical treatment of severe proliferative diabetic retinopathy. Graefes Arch Clin Exp Ophthalmol. 2010 Jun;248(6):785-91. - Modarres M, Nazari H, Falavarjani KG, Naseripour M, Hashemi M, Parvaresh MM. Intravitreal injection of bevacizumab before vitrectomy for proliferative diabetic retinopathy. Eur J Ophthalmol. 2009 Sep-Oct;19(5):848-52. - Ahn J, Woo SJ, Chung H, Park KH. The effect of adjunctive intravitreal bevacizumab for preventing postvitrectomy hemorrhage in proliferative diabetic retinopathy. Ophthalmology. 2011 Nov;118(11):2218-26. - Farahvash MS, Majidi AR, Roohipoor R, Ghassemi F. Preoperative injection of intravitreal bevacizumab in dense diabetic vitreous hemorrhage. Retina. 2011 Jul-Aug;31(7):1254-60.	2	17	3	0	0	2	0.712546	5	5,503
Read your latest personalised notifications No account yet? Start here Don't miss out Ok, got it Dr. Francesca Menichetti Dr. Maria Grazia Bongiorni Over time, pacemaker (PM) leads can develop several complications, often necessitating their risky removal and replacement. At present, a self-contained leadless pacemaker can be placed directly into the heart but can be used only for single-chamber ventricular pacing. Reports from recent non-randomized studies have shown encouraging short-term results that hold the promise of leadless pacing and suggest the potential value of the next generation of devices in the future of cardiovascular implantable electronic devices (CIEDs). This will include multi-chamber leadless pacing systems, left ventricular resynchronisation systems and a combination with a subcutaneous defibrillator. CIEDs cardiovascular implantable electronic devices CRT cardiac resynchronisation therapy ICD implantable cardioverter-defibrillator LCP leadless cardiac pacemakers LV left ventricle RAO right anterior oblique s-ICD subcutaneous implantable cardioverter-defibrillator TPS transcatheter pacing system Pacemakers were developed in the 1950s, primarily to treat life-threatening heart block. They were large external devices connected to epicardial electrodes placed surgically. Although life-sustaining, these devices were plagued by many problems including short battery life, lead failures, and infections, due in part to externalisation of part of the system . With advances in technology, size was reduced significantly to allow the first implantable pacemaker; however, it is now more than 50 years since transvenous leads were developed and first used with implantable devices . These early pacemakers had very limited functionality and only performed asynchronous pacing. Rapid advances have been made over the past five decades, including dual-chamber pacing, rate response algorithms, remote control devices, improved battery technology and cardiac resynchronisation therapy. Pacemaker leads, which connect the chest wall of a pacemaker to the pacing electrode in the heart, are the weakest part of pacing systems, often necessitating their risky removal and replacement. In addition, transvenous leads provide a portal into the vascular space, which increases the risk of infection. Patients with traditional pacemakers are also susceptible to haematomas and pocket infections in the chest wall where the generators lie. Thus, a self-contained leadless pacemaker which can be placed directly into the heart is an appealing prospect. Reports of two recent non-randomised, industry-sponsored studies of leadless cardiac pacemakers (LCP) have now been published and have evaluated the Nanostim device (St. Jude Medical, St. Paul, MN, USA) , and the Micra device (Medtronic, Minneapolis, MN, USA) (Figure 1). Figure 1. A) The volume of each LCP is approximately 1 cm3. Each attaches to the right ventricle — the Micra (on the left) with tines, the Nanostim (on the right) with a helical wire screw. B) Example of chest X-ray after LCP implant (Micra) in the right anterior oblique (RAO) projection. Both devices are implanted in the same setting as traditional pacemakers (i.e., in a cardiac catheterisation laboratory or operating room), using sedative medications and local anesthesia. However, the entire procedure for both mechanisms is faster (20 to 45 minutes, depending on the experience of the specialist) compared to the 60-minute procedure for the insertion of traditional pacemakers. After placing a sheath in the femoral vein in the groin, the pacemaker is delivered to the apex of the right ventricle using a steerable catheter. The procedure is guided by fluoroscopy (Figure 2). If the position is suboptimal, both leadless pacemakers are repositionable and retrievable in the short term using a specific catheter. Moreover, a steroid-eluting tip is incorporated to reduce inflammation. After pacemaker implantation, a closing bandage is used for the access site in the groin. Figure 2. Leadless cardiac pacemaker (LCP) implantation steps. A) A venogram may be performed (optional). B) The introducer is placed up to the heart through the venous system. C) The LCP is positioned into the RV by deflecting the delivery system and placed in the right ventricle. The protective cover is pulled back to expose the LCP. D) The pacemaker is undocked from the delivery catheter while a tethered connection is maintained. In case the position is suboptimal, the LCP can be re-engaged and repositioned. E) & F) The LCP is released (E) in the final position. In this LCP (Micra device; Medtronic) its active fixation mechanism is made by tines. Both systems offer pacing features similar to conventional VVIR pacemakers including rate response algorithms, thus atrioventricular synchrony is not allowed. Although these devices are significantly smaller than conventional pacing systems, the predicted longevity is 10 years. This is comparable to the longevity of a standard pacemaker and is made possible by a design optimising both energy consumption and the electrode/tissue interface. Both of these studies met their primary safety and efficacy goals. The short-term performance of the leadless pacemakers (at six months), including the persistence of good thresholds and sensing, proved to be excellent in both studies. There are a number of questions that remain to be answered regarding this new technology. Will the device continue to perform at a high level in the long term and match the reliability of current pacemaker pulse generators? Will there be any long-term thrombogenic complications associated with an indwelling right ventricular pacemaker? Moreover, the rates of dislodgement of leadless devices over time are unknown and are of potential concern. In the event of battery depletion, removal of the leadless pacemaker may be unnecessary, as an option may be simply to implant an additional device. Abandoning the device and implanting a second unit is probably a viable option for many patients at elective replacement, given the small size of the unit, 10-year battery life, and mean age of 70 years for initial implantation. However, how this will affect cardiac function, and how many additional devices may be implanted, remains to be determined. Because of its design, it is expected that retrieval of the LCP will be a rare event. However, it is anticipated that there may be some circumstances in which the LCP may need to be retrieved, for example high thresholds shortly after implant or infection, although these new devices will probably be associated with a lower overall risk of infection. To facilitate retrieval, the LCP is designed with a feature on the proximal end of the device to allow a snare to capture the device. Animal studies and case reports have shown that it is possible to retrieve the device up to 28 months after implantation, using a custom sheath combined with market-released tools [5,6]. However, it is not known if it is possible to retrieve the device safely after it has been in place for more years. Device follow-up will clarify the long-term LCP performance and the related necessity of transvenous removal. To date, these newer devices can be used only for single-chamber ventricular pacing. However, the next generation of leadless devices is briefly taken in account next. Further studies are required to reach a definitive conclusion on the safety and the performance of these new systems. There is a growing interest in left ventricular endocardial pacing, which has been discovered to be better than traditional epicardial stimulation in the setting of cardiac resynchronisation therapy (CRT) . In some studies, permanent left ventricular endocardial pacing has been achieved by using a conventional lead placed in the left ventricle (LV) via a transseptal puncture [8,9]. More recently, a novel leadless pacing technology has been developed, which may address some of the issues associated with the current CRT delivery as well as potentially overcoming the safety limitations in the chronic use of conventional leads implanted in the LV . The Wireless Endocardial Stimulation (WiSE) Technology (EBR Systems Inc., Sunnyvale, CA, USA), which is not available at the time of publication of this article in the United States, consists of a small electrode implanted in the LV that paces the heart via electrical energy. The electrode generates electricity by converting an ultrasound signal received from a small transmitter placed between the ribs. Another advantage of leadless pacemakers is their small size, which makes them easier to implant than a transcatheter pacing system (TPS). Thus, chronic left ventricular endocardial pacing may address the limitations in the selection of an LV pacing site and provide improvements in CRT effectiveness. In fact, two independent randomised controlled trials, TARGET (Targeted Left Ventricular Lead Placement to Guide Cardiac Resynchronisation Therapy) and STARTER (Speckle Tracking Assisted Resynchronisation Therapy for Electrode Region), have demonstrated that better targeting of the left ventricular pacing site (at the site of latest contraction or ventricular activation) leads to improvements in clinical response, including freedom from heart failure, hospitalisation, and mortality [11,12]. The feasibility of providing an endocardial stimulation for CRT with leadless technology was successfully demonstrated in the WiSE CRT study . This study demonstrated a significant subjective improvement in the patients’ functional status and a significant increase of LV ejection fraction. However, the study was terminated prematurely for safety reasons, and so the delivery system was redesigned. Then the SELECT-LV study suggest that the delivery system modifications permit atraumatic implantation of the receiver electrode onto the left ventricular endocardial surface (Presented at the Heart Rhythm Society Annual Scientific Sessions, May 2016 in San Francisco, CA, USA. Reddy VY et al. Leadless LV Endocardial Stimulation for CRT: Final Outcomes of the Safety and Performance of Electrodes Implanted in the Left Ventricle [SELECT-LV] Study. Abstract AB17-02). There remain several technology-specific concerns that require consideration. First, although the left ventricular receiver component of this system is indeed leadless, the system does require the concomitant conventional implantable right ventricular pacing devices. Second, it is theoretically possible that long-term ultrasound energy exposure to subcutaneous or myocardial tissue in humans may have unintended adverse consequences. Third, there may be untoward effects of external (environmental) interference and changes in the acoustic window on the system’s sensing or pacing performance. In recent years, major advances have been achieved in entirely subcutaneous ICD (s-ICD) systems that obviate intracardiac leads and hence carry the potential to reduce associated complications. The s-ICD is establishing its role as a viable alternative, particularly as primary prevention in non-pacing-dependent younger patients. Unlike standard implantable cardioverter-defibrillators (ICD), the s-ICD is devoid of anti-bradycardia pacing capabilities. Nevertheless, certain patients may benefit from leadless devices with both pacing and ICD functions. Recently, the case of the first patient in whom the leadless pacemaker and s-ICD were used in combination has been described . This patient had been implanted with an s-ICD system and after some time presented a complete atrioventricular block, but he had no vascular access for transvenous lead insertion, so a leadless pacemaker was inserted. When these two technologies are used together, pacemakers must be programmed to stimulate in a bipolar mode in order to avoid double counting by the s-ICD. Although VF was not re-induced, an 80 J shock was delivered by the s-ICD on QRS timing, which did not result in mode reversion, shutdown, or dislodgment of the pacemaker. Moreover, no noise was perceived by the s-ICD during pacemaker interrogation and programming, even when programmed to maximum output stimulation. This strategy carries the potential of pacing and defibrillation for patients in whom standard transvenous approaches are not feasible or desirable. The leadless devices may ultimately obviate conventional pacing technologies and enhance arrhythmia sensing by the s-ICD which will be able to incorporate intracardiac electrogram data to confirm ventricular arrhythmias, reducing the oversensing of T waves, myopotentials, or non-biologic noise associated with such ICDs. Further development of both leadless pacemakers for multi-chamber pacing may occur pending positive results from ongoing studies. Multi-device systems can introduce unique challenges for implementing such multi-chamber therapy. In multi-device systems, two separate devices may be responsible for sensing cardiac events in different chambers and delivering electrical stimulation to the different chambers. In some instances, each of the devices may be able to detect and/or deliver electrical stimulation to one chamber of the heart. The multiple devices of such systems may be configured to communicate sensed cardiac events and other information to the other devices in order to deliver electrical stimulation to the various chambers safely and effectively. It is anticipated that leadless pacemakers will be more expensive than a transcatheter pacing system (TPS). In Europe, the estimated cost for the Nanostim pacemaker and implantation procedure is €11,500. The cost of the retrieval of the pacemaker (if necessary) shortly after implantation is €6,000 . However, savings generated from the shorter procedure time and fewer healthcare resources used to manage lead and chest pocket complications could help offset the additional cost. Another economic consideration is the battery life comparison. Based on the International Organisation for Standardisation (ISO) standard (2.5 V, 0.4 ms, 600 U, 60 beats/min, and 100% pacing), the battery longevity of a TPS is approximately one half that of the LCP (4.7 vs. 9.8 years). Even considering the use of capture management to minimise battery drain, the projected battery life for the LCP at nominal settings (1.5 V, 0.24 ms, 60 beats/min with an impedance load of 500 ohms and 100% pacing) would be longer than the TPS one (14.7 vs. 9.6 years) . A longer battery life means less need for replacement for LCP compared with TPS. This would save healthcare resources from the additional cost for a new device and patients from the infection risk related to the procedure. A complete study comparing the costs of LCP and TPS is still missing, but we can postulate an economic return of LCP both for hospital resources and patient health. Predicting the role of this device in pacemaker therapy is a challenge, as it is for most new devices. In the United States of America and most European countries, ~20% to 30% of patients receive ventricular pacemakers primarily for chronic atrial fibrillation, and this was largely the population implanted in the current trial. However, in less developed countries, this proportion is often ≥50% . In addition, pacemaker use is limited in many areas of the world, in part because of the lack of implantation expertise. The steep learning curve for the leadless pacemaker implantation and the more common skill set of femoral vascular access and sheath manipulation suggest that this approach could fill an unmet need. Consequently, the adoption of this therapy in such countries could be high, particularly if the cost is competitive with traditional pacing systems. Future developments of leadless pacing make it easy to envisage its important role in future cardiovascular implantable electronic devices (CIEDs). The ability to communicate with a second device placed in the right atrium would result in dual-chamber pacing capabilities. Similarly, communication with a subcutaneous ICD would allow both bradycardia and anti-tachycardia pacing, and would probably improve rhythm discrimination. The potential safety of placing leads in the left ventricle in the absence of anticoagulation is unclear, but endocardial stimulation with a leadless technology could be a novel developing tool for cardiac resynchronisation therapy. All of these possibilities point towards a bright future for leadless pacing with the likely possibility that the devices of the future will be largely devoid of intravascular leads. As such, leadless cardiac pacemakers could become the future of pacing in many types of device. Francesca Menichetti1,2, MD; Maria Grazia Bongiorni1, MD 1. Cardiothoracic and Vascular Department, University Hospital of Pisa, Pisa, Italy; 2. Sant'Anna School of Advanced Studies, Pisa, Italy Address for correspondence: Dr Francesca Menichetti, Cardiovascular Department, Cisanello Hospital, UO MCVII, (Building 10, second floor), Via Paradisa, 56100 Pisa, Italy. Tel. +39 050992343 Fax +39 050995335 Conflict of interest: The authors have no conflicts to declare. Our mission: To reduce the burden of cardiovascular disease © 2018 European Society of Cardiology. All rights reserved	2	12	1	0	0	0	0.430737	1	3,637
\|Synonyms\|\|cardiopulmonary arrest, circulatory arrest, sudden cardiac arrest (SCA), sudden cardiac death (SCD)\| \|CPR being administered during a simulation of cardiac arrest.\| \|Classification and external resources\| Cardiac arrest is a sudden stop in effective blood flow due to the failure of the heart to contract effectively. Symptoms include loss of consciousness and abnormal or absent breathing. Some people may have chest pain, shortness of breath, or nausea before this occurs. If not treated within minutes, death usually occurs. The most common cause of cardiac arrest is coronary artery disease. Less common causes include major blood loss, lack of oxygen, very low potassium, heart failure, and intense physical exercise. A number of inherited disorders may also increase the risk including long QT syndrome. The initial heart rhythm is most often ventricular fibrillation. The diagnosis is confirmed by finding no pulse. While a cardiac arrest may be caused by heart attack or heart failure these are not the same. Prevention includes not smoking, physical activity, and maintaining a healthy weight. Treatment for cardiac arrest is immediate cardiopulmonary resuscitation (CPR) and if a shockable rhythm is present defibrillation. Among those who survive targeted temperature management may improve outcomes. An implantable cardiac defibrillator may be placed to reduce the chance of death from recurrence. In the United States cardiac arrest outside of hospital occurs in about 13 per 10,000 people per year (326,000 cases). In hospital cardiac arrest occurs in an additional 209,000 Cardiac arrest becomes more common with age. It affects males more often than females. The percentage of people that survive with treatment is about 8%. Many who survive have significant disability. Many U.S. television shows, however, have portrayed unrealistically high survival rates of 67%. Signs and symptoms Cardiac arrest is sometimes preceded by certain symptoms such as fainting, fatigue, blackouts, dizziness, chest pain, shortness of breath, weakness, and vomiting. The arrest may also occur with no warning. When the arrest occurs, the most obvious sign of its occurrence will be the lack of a palpable pulse in the person experiencing it (since the heart has ceased to contract, the usual indications of its contraction such as a pulse will no longer be detectable). Certain types of prompt intervention can often reverse a cardiac arrest, but without such intervention the event will almost always lead to death. In certain cases, it is an expected outcome of a serious illness where death is expected. Also, as a result of inadequate cerebral perfusion, the patient will quickly become 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 artery disease is the leading cause of sudden cardiac arrest. Many other cardiac and non-cardiac conditions also increase one's risk. Coronary artery disease often results in coronary ischemia and ventricular fibrillation (v-fib). Cases have shown that the most common finding at postmortem examination of SCD is chronic high-grade stenosis of at least one segment of a major coronary artery, the arteries that supply the heart muscle with its blood supply. Left ventricular hypertrophy is thought to be the leading cause of sudden cardiac death in the adult population. This is most commonly the result of longstanding high blood pressure which has caused secondary damage to the wall of the main pumping chamber of the heart, the left ventricle. - Coronary heart disease - Physical stress - Inherited disorders - Enlarged heart due to increased blood pressure - Commotio cordis Coronary artery disease Approximately 60–70% of SCD is related to coronary artery disease, also known as ischemic heart disease. Among adults, it is the predominant cause of arrest, with 30% of people at autopsy showing signs of recent myocardial infarction. Non-ischemic heart disease A number of non-ischemic cardiac abnormalities can increase the risk of SCD, including cardiomyopathy, cardiac rhythm disturbances, myocarditis, hypertensive heart disease, and congestive heart failure. 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 fivefold. 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 one of every 5000 to 7000 newborns and is estimated to be responsible for 3000 deaths each year compared to the approximately 300,000 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 and 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). Mnemonic for causes - 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 The mechanism of death in the majority of people dying of sudden cardiac death is ventricular fibrillation. Structural changes in the diseased heart as a result of inherited factors (mutations in ion-channel coding genes for example) cannot explain the suddenness of SCD. Also, sudden cardiac death could be the consequence of electric-mechanical disjunction and bradyarrhythmias. 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. 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. 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. A Cochrane review published in 2016 found moderate-quality evidence to show that blood pressure-lowering drugs do not appear to reduce sudden cardiac death. 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". People 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 with 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 people in the acute phase of their illness in order to prevent a cardiac arrest. These teams have been found to decrease the rates of in hospital cardiac arrest and improve survival. - 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 person in cardiac arrest, a practice known as "slow code", or may fake the response altogether for the sake of the person's family, a practice known as "show code". This is generally done for people 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. Marine-derived omega-3 polyunsaturated fatty acids (PUFAs) has been promoted for the prevention of sudden cardiac death due to its postulated ability to lower triglyceride levels, prevent arrhythmias, decrease platelet aggregation, and lower blood pressure. However, according to a recent systematic review, omega-3 PUFA supplementation are not been associated with a lower risk of sudden cardiac death. 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 an 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 that 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's 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. Bystander CPR, by the lay public, before the arrival of EMS also improves outcomes. Either a bag valve mask or an advanced airway may be used to help with breathing. High levels of oxygen are generally given during CPR. Tracheal intubation has not been found to improve survival rates in cardiac arrest and in the prehospital environment may worsen it. 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. Mechanical chest compressions (as performed by a machine) are no better than chest compressions performed by hand. It is unclear if a few minutes of CPR before defibrillation results in different outcomes than immediate defibrillation. If cardiac arrest occurs after 20 weeks of pregnancy someone should pull or push the uterus to the left during CPR. If a pulse has not returned by four minutes emergency Cesarean section is recommended. 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 not been shown to improve survival to hospital discharge following out-of-hospital cardiac arrest. This includes the use of epinephrine, atropine, lidocaine, and amiodarone. Epinephrine is generally recommended every five minutes. Vasopressin overall does not improve or worsen outcomes compared to epinephrine. Epinephrine does appear to improve short-term outcomes such as return of spontaneous circulation. Some of the lack of long-term benefit may be related to delays in epinephrine use. While evidence does not support its use in children guidelines state its use is reasonable. Lidocaine and amiodarone are also deemed reasonable in children with cardiac arrest who have a shockable rhythm. The general use of sodium bicarbonate or calcium is not recommended. The 2010 guidelines from the American Heart Association no longer contain the recommendation for using atropine in pulseless electrical activity and asystole due to the lack of evidence for its use. Neither lidocaine nor amiodarone, in those who continue in ventricular tachycardia or ventricular fibrillation despite defibrillation, improves survival to hospital discharge but both equally improve survival to hospital admission. Thrombolytics when used generally may cause harm but may be of benefit in those with a confirmed pulmonary embolism as the cause of arrest. Evidence for use of naloxone in those with cardiac arrest due to opioids is unclear but it may still be used. In those with cardiac arrest due to local anesthetic lipid emulsion may be used. Targeted temperature management Cooling adults after cardiac arrest who have a return of spontaneous circulation (ROSC) but no return of consciousness improves outcomes. This procedure is called targeted temperature management (previously known as therapeutic hypothermia). People are typically cooled for a 24-hour period, with a target temperature of 32–36 °C (90–97 °F). Death rates in the hypothermia group are 35% lower than in those with no temperature management. Complications are generally no greater in those who receive this therapy. Earlier versus later cooling may result in better outcomes. A trial that cooled in the ambulance, however, found no difference compared to starting cooling in-hospital. A registry database found poor neurological outcome increased by 8% with each five-minute delay in initiating TH and by 17% for every 30-minute delay in time to target temperature. In children it is unclear if cooling is beneficial however fever should be prevented. 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 organizations 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 which may include percutaneous coronary intervention 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 although high quality evidence is lacking. It is recommended that it is done as soon as possible in those who have had a cardiac arrest with ST elevation due to underlying heart problems. 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 overall chance of survival among those who have cardiac arrest outside of a hospital is 7.6%. Among children rates of survival is 3 to 16% in North America. For in hospital cardiac arrest survival to discharge is around 22% with many having a good neurological outcome. Prognosis is typically assessed 72 hours or more after cardiac arrest. Rates of survival are better in those who someone saw collapse, got bystander CPR, or had either ventricular tachycardia or ventricular fibrillation when assessed. Survival among those with Vfib or Vtach is 15 to 23%. Women are more likely to survive cardiac arrest and leave hospital than men. 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. 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 reflects 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% had major neurological disability. Of those who were discharged from hospital, 70% were still alive four years later. Based on death certificates, sudden cardiac death accounts for about 15% of all death in Western countries. In the United States 326,000 cases of out of hospital and 209,000 cases of in hospital cardiac arrest occur among adults a year. The lifetime risk is three times greater in men (12.3%) than women (4.2%) based on analysis of the Framingham Heart Study. However this gender difference disappeared beyond 85 years of age. In many publications the stated or implicit meaning of "sudden cardiac death" is sudden death from cardiac causes. However, sometimes physicians call cardiac arrest "sudden cardiac death" even if the person survives. Thus one can hear mentions of "prior episodes of sudden cardiac death" in a living person, which sounds odd as judged by the sense of the term that involves death but is understood as invoking the other sense. - Field, John M. (2009). The Textbook of Emergency Cardiovascular Care and CPR. Lippincott Williams & Wilkins. p. 11. ISBN 9780781788991. - "What Is Sudden Cardiac Arrest?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - "What Are the Signs and Symptoms of Sudden Cardiac Arrest?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - "What Causes Sudden Cardiac Arrest?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - "How Can Death Due to Sudden Cardiac Arrest Be Prevented?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - "How Is Sudden Cardiac Arrest Treated?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - Schenone, AL; Cohen, A; Patarroyo, G; Harper, L; Wang, X; Shishehbor, MH; Menon, V; Duggal, A (10 August 2016). "Therapeutic hypothermia after cardiac arrest: a systematic review/meta-analysis exploring the impact of expanded criteria and targeted temperature.". Resuscitation. PMID 27521472. - Kronick, SL; Kurz, MC; Lin, S; Edelson, DP; Berg, RA; Billi, JE; Cabanas, JG; Cone, DC; Diercks, DB; Foster, JJ; Meeks, RA; Travers, AH; Welsford, M (3 November 2015). "Part 4: Systems of Care and Continuous Quality Improvement: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 132 (18 Suppl 2): S397–413. PMID 26472992. - "Who Is at Risk for Sudden Cardiac Arrest?". NHLBI. June 22, 2016. Retrieved 16 August 2016. - Adams, James G. (2012). Emergency Medicine: Clinical Essentials (Expert Consult -- Online). Elsevier Health Sciences. p. 1771. ISBN 1455733946. - "What Are the Signs and Symptoms of Sudden Cardiac Arrest?". National Heart, Lung and Blood Institute. 1 April 2011. Retrieved 2015-06-21. - Jameson, J. N. St C.; Dennis L. Kasper; Harrison, Tinsley Randolph; Braunwald, Eugene; Fauci, Anthony S.; Hauser, Stephen L; Longo, Dan L. (2005). Harrison's principles of internal medicine. New York: McGraw-Hill Medical Publishing Division. ISBN 0-07-140235-7. - "Mount Sinai - Cardiac arrest". - Parnia, S; Spearpoint, K; Fenwick, PB (August 2007). "Near death experiences, cognitive function and psychological outcomes of surviving cardiac arrest.". Resuscitation. 74 (2): 215–21. doi:10.1016/j.resuscitation.2007.01.020. PMID 17416449. - Koplan, Bruce A.; Stevenson, William G. (2009). "Ventricular Tachycardia and Sudden Cardiac Death". Mayo Clinic Proceedings. 84 (3): 289–297. doi:10.4065/84.3.289. ISSN 0025-6196. PMC 2664600. PMID 19252119. - Fuster, Valentin; Topol, Eric J.; Nabel, Elizabeth G. (2005). Atherothrombosis and Coronary Artery Disease. Lippincott Williams & Wilkins. ISBN 9780781735834. - Stevens, Steven M.; Reinier, Kyndaron; Chugh, Sumeet S. (2013). "Increased Left Ventricular Mass as a Predictor of Sudden Cardiac Death Is it Time to Put it to The Test?". Circulation: Arrhythmia and Electrophysiology. 6 (1): 212–217. doi:10.1161/CIRCEP.112.974931. ISSN 1941-3149. PMID 23424223. - Katholi RE, Couri DM (2011). "Left Ventricular Hypertrophy: Major Risk Factor in Patients with Hypertension: Update and Practical Clinical Applications". Int J Hypertens.: 495349. doi:10.4061/2011/495349. PMC 3132610. PMID 21755036. - "What Causes Sudden Cardiac Arrest?". National Heart, Lung and Blood Institute. 1 April 2011. Retrieved 2015-06-21. - Zheng ZJ, Croft JB, Giles WH, Mensah GA (October 2001). "Sudden cardiac death in the United States, 1989 to 1998". Circulation. 104 (18): 2158–63. doi:10.1161/hc4301.098254. PMID 11684624. - Centers for Disease Control and Prevention (CDC) (February 2002). "State-specific mortality from sudden cardiac death--United States, 1999". MMWR Morb. Mortal. Wkly. Rep. 51 (6): 123–6. PMID 11898927. - Eisenberg MS, Mengert TJ (April 2001). "Cardiac resuscitation". N. Engl. J. Med. 344 (17): 1304–13. doi:10.1056/NEJM200104263441707. PMID 11320390. - Kannel WB, Wilson PW, D'Agostino RB, Cobb J (August 1998). "Sudden coronary death in women". Am. Heart J. 136 (2): 205–12. doi:10.1053/hj.1998.v136.90226. PMID 9704680. - Eckart RE, Scoville SL, Campbell CL, et al. (December 2004). "Sudden death in young adults: a 25-year review of autopsies in military recruits". Annals of Internal Medicine. 141 (11): 829–34. doi:10.7326/0003-4819-141-11-200412070-00005. PMID 15583223. - Sudden Cardiac Death - Kuisma M, Alaspää A (July 1997). "Out-of-hospital cardiac arrests of non-cardiac origin. Epidemiology and outcome". Eur. Heart J. 18 (7): 1122–8. doi:10.1093/oxfordjournals.eurheartj.a015407. PMID 9243146. - Friedlander Y, Siscovick DS, Weinmann S, et al. (January 1998). "Family history as a risk factor for primary cardiac arrest". Circulation. 97 (2): 155–60. doi:10.1161/01.cir.97.2.155. PMID 9445167. - "Resuscitation Council (UK) Guidelines 2005". - ECC Committee, Subcommittees and Task Forces of the American Heart Association (December 2005). "2005 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care". Circulation. 112 (24 Suppl): IV1–203. doi:10.1161/CIRCULATIONAHA.105.166550. PMID 16314375. - Rubart, Michael; Zipes, Douglas P. (2005). "Mechanisms of sudden cardiac death". Journal of Clinical Investigation. 115 (9): 2305–2315. doi:10.1172/JCI26381. ISSN 0021-9738. PMC 1193893. PMID 16138184. - Bunch, T. Jared; Hohnloser, Stefan H.; Gersh, Bernard J. (2007). "Mechanisms of Sudden Cardiac Death in Myocardial Infarction Survivors Insights From the Randomized Trials of Implantable Cardioverter-Defibrillators". Circulation. 115 (18): 2451–2457. doi:10.1161/CIRCULATIONAHA.106.683235. ISSN 0009-7322. PMID 17485594. - "Types of Arrhythmia". National Heart, Lung and Blood Institute. 1 April 2011. Retrieved 2015-06-21. - Ochoa FJ, Ramalle-Gómara E, Carpintero JM, García A, Saralegui I (June 1998). "Competence of health professionals to check the carotid pulse". Resuscitation. 37 (3): 173–5. doi:10.1016/S0300-9572(98)00055-0. PMID 9715777. - Bahr J, Klingler H, Panzer W, Rode H, Kettler D (August 1997). "Skills of lay people in checking the carotid pulse". Resuscitation. 35 (1): 23–6. doi:10.1016/S0300-9572(96)01092-1. PMID 9259056. - British Red Cross; St Andrew's Ambulance Association; St John Ambulance (2006). First Aid Manual: The Authorised Manual of St. John Ambulance, St. Andrew's Ambulance Association, and the British Red Cross. Dorling Kindersley Publishers Ltd. ISBN 1-4053-1573-3. - Jasmeet Soar, Gavin D. Perkins, Jerry Nolan., eds. (2012). ABC of resuscitation (6th ed.). Chichester, West Sussex: Wiley-Blackwell. p. 43. ISBN 9781118474853. - Taverny, G; Mimouni, Y; LeDigarcher, A; Chevalier, P; Thijs, L; Wright, JM; Gueyffier, F (10 March 2016). "Antihypertensive pharmacotherapy for prevention of sudden cardiac death in hypertensive individuals.". The Cochrane database of systematic reviews. 3: CD011745. doi:10.1002/14651858.CD011745.pub2. PMID 26961575. Retrieved 19 March 2016. - Kause J, Smith G, Prytherch D, Parr M, Flabouris A, Hillman K (September 2004). "A comparison of antecedents to cardiac arrests, deaths and emergency intensive care admissions in Australia and New Zealand, and the United Kingdom--the ACADEMIA study". Resuscitation. 62 (3): 275–82. doi:10.1016/j.resuscitation.2004.05.016. PMID 15325446. - "Slow Codes, Show Codes and Death". New York Times. New York Times Company. 22 August 1987. Retrieved 2013-04-06. - "Decision-making for the End of Life". Physician Advisory Service. College of Physicians and Surgeons of Ontario. May 2006. Retrieved 2013-04-06. - DePalma, Judith A.; Miller, Scott; Ozanich, Evelyn; Yancich, Lynne M. (November 1999). "Slow" Code: Perspectives of a Physician and Critical Care Nurse. Critical Care Nursing Quarterly. 22. Lippincott Williams and Wilkins. pp. 89–99. ISSN 1550-5111. - Birnie, David H; Sambell, Christie; Johansen, Helen; Williams, Katherine; Lemery, Robert; Green, Martin S; Gollob, Michael H; Lee, Douglas S; Tang, Anthony SL (July 2007). "Use of implantable cardioverter defibrillators in Canadian and IS survivors of out-of-hospital cardiac arrest". Canadian Medical Association Journal. 177 (1): 41–6. doi:10.1503/cmaj.060730. PMC 1896034. PMID 17606938. Retrieved 2007-07-29. - Simpson CS (July 2007). "Implantable cardioverter defibrillators work--so why aren't we using them?". CMAJ. 177 (1): 49–51. doi:10.1503/cmaj.070470. PMC 1896028. PMID 17606939. - Moss AJ, Brown MW, Cannom DS, et al. (October 2005). "Multicenter automatic defibrillator implantation trial-cardiac resynchronization therapy (MADIT-CRT): design and clinical protocol". Ann Noninvasive Electrocardiol. 10 (4 Suppl): 34–43. doi:10.1111/j.1542-474X.2005.00073.x. PMID 16274414. - Neil K. Kaneshiro (2 August 2011). "Omega-3 fatty acids". MedlinePlus Medical Encyclopedia. Retrieved 2015-06-21. - Evangelos C. Rizos; Evangelia E. Ntzani; Eftychia Bika; Michael S. Kostapanos; Moses S. Elisaf (September 2012). "Association Between Omega-3 Fatty Acid Supplementation and Risk of Major Cardiovascular Disease Events A Systematic Review and Meta-analysis". JAMA. 308 (10): 1024–1033. doi:10.1001/2012.jama.11374. PMID 22968891. - American Heart, Association (May 2006). "2005 American Heart Association (AHA) guidelines for cardiopulmonary resuscitation (CPR) and emergency cardiovascular care (ECC) of pediatric and neonatal patients: pediatric advanced life support". Pediatrics. 117 (5): e1005–28. doi:10.1542/peds.2006-0346. PMID 16651281. - Mutchner L (January 2007). "The ABCs of CPR--again". Am J Nurs. 107 (1): 60–9; quiz 69–70. doi:10.1097/00000446-200701000-00024. PMID 17200636. - Resuscitation Council (UK). "Pre-hospital cardiac arrest" (PDF). www.resus.org.uk. p. 41. Retrieved 3 September 2014. - Resuscitation Council (UK) (5 September 2012). "Comments on the duration of CPR following the publication of 'Duration of resuscitation efforts and survival after in-hospital cardiac arrest: an observational study' Goldberger ZD et al. Lancet.". Retrieved 3 September 2014. - Neumar, RW; Shuster, M; Callaway, CW; Gent, LM; Atkins, DL; Bhanji, F; Brooks, SC; de Caen, AR; Donnino, MW; Ferrer, JM; Kleinman, ME; Kronick, SL; Lavonas, EJ; Link, MS; Mancini, ME; Morrison, LJ; O'Connor, RE; Samson, RA; Schexnayder, SM; Singletary, EM; Sinz, EH; Travers, AH; Wyckoff, MH; Hazinski, MF (3 November 2015). "Part 1: Executive Summary: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 132 (18 Suppl 2): S315–67. doi:10.1161/cir.0000000000000252. PMID 26472989. - Studnek JR, Thestrup L, Vandeventer S, et al. (September 2010). "The association between prehospital endotracheal intubation attempts and survival to hospital discharge among out-of-hospital cardiac arrest patients". Acad Emerg Med. 17 (9): 918–25. doi:10.1111/j.1553-2712.2010.00827.x. PMID 20836771. - Yao, L; Wang, P; Zhou, L; Chen, M; Liu, Y; Wei, X; Huang, Z (Jun 2014). "Compression-only cardiopulmonary resuscitation vs standard cardiopulmonary resuscitation: an updated meta-analysis of observational studies.". The American journal of emergency medicine. 32 (6): 517–23. doi:10.1016/j.ajem.2014.01.055. PMID 24661781. - Huang, Y; He, Q; Yang, LJ; Liu, GJ; Jones, A (Sep 12, 2014). "Cardiopulmonary resuscitation (CPR) plus delayed defibrillation versus immediate defibrillation for out-of-hospital cardiac arrest.". The Cochrane database of systematic reviews. 9: CD009803. doi:10.1002/14651858.CD009803.pub2. PMID 25212112. - Lavonas, EJ; Drennan, IR; Gabrielli, A; Heffner, AC; Hoyte, CO; Orkin, AM; Sawyer, KN; Donnino, MW (3 November 2015). "Part 10: Special Circumstances of Resuscitation: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 132 (18 Suppl 2): S501–18. PMID 26472998. - de Caen, AR; Berg, MD; Chameides, L; Gooden, CK; Hickey, RW; Scott, HF; Sutton, RM; Tijssen, JA; Topjian, A; van der Jagt, ÉW; Schexnayder, SM; Samson, RA (3 November 2015). "Part 12: Pediatric Advanced Life Support: 2015 American Heart Association Guidelines Update for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 132 (18 Suppl 2): S526–42. doi:10.1161/cir.0000000000000266. PMID 26473000. - Zoll AED Plus - Lyon R.M; Cobbe S.M.; Bradley J.M.; Grubb N.R.; et al. (2004). "Surviving out of hospital cardiac arrest at home: a postcode lottery?". Emergency Medical Journal. 21: 619–624. doi:10.1136/emj.2003.010363. - Olasveengen TM, Sunde K, Brunborg C, Thowsen J, Steen PA, Wik L (November 2009). "Intravenous drug administration during out-of-hospital cardiac arrest: a randomized trial". JAMA. 302 (20): 2222–9. doi:10.1001/jama.2009.1729. PMID 19934423. - Lin, S; Callaway, CW; Shah, PS; Wagner, JD; Beyene, J; Ziegler, CP; Morrison, LJ (Mar 15, 2014). "Adrenaline for out-of-hospital cardiac arrest resuscitation: A systematic review and meta-analysis of randomized controlled trials.". Resuscitation. 85 (6): 732–40. doi:10.1016/j.resuscitation.2014.03.008. PMID 24642404. - Laina, A; Karlis, G; Liakos, A; Georgiopoulos, G; Oikonomou, D; Kouskouni, E; Chalkias, A; Xanthos, T (9 July 2016). "Amiodarone and cardiac arrest: Systematic review and meta-analysis.". International journal of cardiology. 221: 780–788. PMID 27434349. - Morley, PT (June 2011). "Drugs during cardiopulmonary resuscitation.". Current Opinion in Critical Care. 17 (3): 214–8. doi:10.1097/MCC.0b013e3283467ee0. PMID 21499094. - Attaran, RR; Ewy, GA (July 2010). "Epinephrine in resuscitation: curse or cure?". Future cardiology. 6 (4): 473–82. doi:10.2217/fca.10.24. PMID 20608820. - Velissaris, D; Karamouzos, V; Pierrakos, C; Koniari, I; Apostolopoulou, C; Karanikolas, M (April 2016). "Use of Sodium Bicarbonate in Cardiac Arrest: Current Guidelines and Literature Review.". Journal of clinical medicine research. 8 (4): 277–83. PMID 26985247. - Neumar, RW; Otto, CW; Link, MS; Kronick, SL; Shuster, M; Callaway, CW; Kudenchuk, PJ; Ornato, JP; McNally, B; Silvers, SM; Passman, RS; White, RD; Hess, EP; Tang, W; Davis, D; Sinz, E; Morrison, LJ (Nov 2, 2010). "Part 8: adult advanced cardiovascular life support: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 122 (18 Suppl 3): S729–67. doi:10.1161/CIRCULATIONAHA.110.970988. PMID 20956224. - Sanfilippo, F; Corredor, C; Santonocito, C; Panarello, G; Arcadipane, A; Ristagno, G; Pellis, T (October 2016). "Amiodarone or lidocaine for cardiac arrest: A systematic review and meta-analysis.". Resuscitation. 107: 31–7. PMID 27496262. - Perrott, J; Henneberry, RJ; Zed, PJ (December 2010). "Thrombolytics for cardiac arrest: case report and systematic review of controlled trials.". Annals of Pharmacotherapy. 44 (12): 2007–13. doi:10.1345/aph.1P364. PMID 21119096. - Xiao, G; Guo, Q; Shu, M; Xie, X; Deng, J; Zhu, Y; Wan, C (February 2013). "Safety profile and outcome of mild therapeutic hypothermia in patients following cardiac arrest: systematic review and meta-analysis.". Emergency medicine journal : EMJ. 30 (2): 91–100. doi:10.1136/emermed-2012-201120. PMID 22660549. - Nielsen, Niklas; Wetterslev, Jørn; Cronberg, Tobias; Erlinge, David; Gasche, Yvan; Hassager, Christian; Horn, Janneke; Hovdenes, Jan; Kjaergaard, Jesper; Kuiper, Michael; Pellis, Tommaso; Stammet, Pascal; Wanscher, Michael; Wise, Matt P.; Åneman, Anders; Al-Subaie, Nawaf; Boesgaard, Søren; Bro-Jeppesen, John; Brunetti, Iole; Bugge, Jan Frederik; Hingston, Christopher D.; Juffermans, Nicole P.; Koopmans, Matty; Køber, Lars; Langørgen, Jørund; Lilja, Gisela; Møller, Jacob Eifer; Rundgren, Malin; Rylander, Christian; Smid, Ondrej; Werer, Christophe; Winkel, Per; Friberg, Hans (17 November 2013). "Targeted Temperature Management at 33°C versus 36°C after Cardiac Arrest". New England Journal of Medicine. 369 (23): 131117131833001. doi:10.1056/NEJMoa1310519. PMID 24237006. - Arrich, J; Holzer, M; Havel, C; Müllner, M; Herkner, H (Sep 12, 2012). "Hypothermia for neuroprotection in adults after cardiopulmonary resuscitation.". Cochrane database of systematic reviews (Online). 9: CD004128. doi:10.1002/14651858.CD004128.pub3. PMID 22972067. - Stockmann, H; Krannich, A; Schroeder, T; Storm, C (November 2014). "Therapeutic temperature management after cardiac arrest and the risk of bleeding: Systematic review and meta-analysis.". Resuscitation. 85 (11): 1494–1503. doi:10.1016/j.resuscitation.2014.07.018. PMID 25132475. - Dell'anna, AM; Scolletta, S; Donadello, K; Taccone, FS (June 2014). "Early neuroprotection after cardiac arrest.". Current opinion in critical care. 20 (3): 250–8. doi:10.1097/mcc.0000000000000086. PMID 24717694. - Sendelbach, S; Hearst, MO; Johnson, PJ; Unger, BT; Mooney, MR (July 2012). "Effects of variation in temperature management on cerebral performance category scores in patients who received therapeutic hypothermia post cardiac arrest.". Resuscitation. 83 (7): 829–34. doi:10.1016/j.resuscitation.2011.12.026. PMID 22230942. - Loertscher, L; Reed, DA; Bannon, MP; Mueller, PS (January 2010). "Cardiopulmonary resuscitation and do-not-resuscitate orders: a guide for clinicians". The American Journal of Medicine. 123 (1): 4–9. doi:10.1016/j.amjmed.2009.05.029. PMID 20102982. - Knox, C; Vereb, JA (December 2005). "Allow natural death: a more humane approach to discussing end-of-life directives". Journal of Emergency Nursing. 31 (6): 560–1. doi:10.1016/j.jen.2005.06.020. PMID 16308044. - Millin, MG; Comer, AC; Nable, JV; Johnston, PV; Lawner, BJ; Woltman, N; Levy, MJ; Seaman, KG; Hirshon, JM (15 September 2016). "Patients without ST elevation after return of spontaneous circulation may benefit from emergent percutaneous intervention: A systematic review and meta-analysis.". Resuscitation. 108: 54–60. PMID 27640933. - Lehot, JJ; Long-Him-Nam, N; Bastien, O (December 2011). "[Extracorporeal life support for treating cardiac arrest].". Bulletin de l'Academie nationale de medecine. 195 (9): 2025–33; discussion 2033–6. PMID 22930866. - Camuglia, AC.; Randhawa, VK.; Lavi, S.; Walters, DL. (Sep 2014). "Cardiac catheterization is associated with superior outcomes for survivors of out of hospital cardiac arrest: Review and meta-analysis.". Resuscitation. 85: 1533–1540. doi:10.1016/j.resuscitation.2014.08.025. PMID 25195073. - Cave, DM; Gazmuri, RJ; Otto, CW; Nadkarni, VM; Cheng, A; Brooks, SC; Daya, M; Sutton, RM; Branson, R; Hazinski, MF (2010-11-02). "Part 7: CPR techniques and devices: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care.". Circulation. 122 (18 Suppl 3): S720–8. doi:10.1161/CIRCULATIONAHA.110.970970. PMC 3741663. PMID 20956223. - Sasson, C; Rogers, MA; Dahl, J; Kellermann, AL (January 2010). "Predictors of survival from out-of-hospital cardiac arrest: a systematic review and meta-analysis.". Circulation. Cardiovascular quality and outcomes. 3 (1): 63–81. doi:10.1161/circoutcomes.109.889576. PMID 20123673. - Bougouin, W; Mustafic, H; Marijon, E; Murad, MH; Dumas, F; Barbouttis, A; Jabre, P; Beganton, F; Empana, JP; Celermajer, DS; Cariou, A; Jouven, X (September 2015). "Gender and survival after sudden cardiac arrest: A systematic review and meta-analysis.". Resuscitation. 94: 55–60. doi:10.1016/j.resuscitation.2015.06.018. PMID 26143159. - Ballew KA (May 1997). "Cardiopulmonary resuscitation". BMJ. 314 (7092): 1462–5. doi:10.1136/bmj.314.7092.1462. PMC 2126720. PMID 9167565. - van Gijn, MS; Frijns, D; van de Glind, EM; C van Munster, B; Hamaker, ME (Jul 2014). "The chance of survival and the functional outcome after in-hospital cardiopulmonary resuscitation in older people: a systematic review.". Age and ageing. 43 (4): 456–63. doi:10.1093/ageing/afu035. PMID 24760957. - Cobbe SM, Dalziel K, Ford I, Marsden AK (June 1996). "Survival of 1476 patients initially resuscitated from out of hospital cardiac arrest". BMJ. 312 (7047): 1633–7. doi:10.1136/bmj.312.7047.1633. PMC 2351362. 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". - Elsevier, Dorland's Illustrated Medical Dictionary, Elsevier. - Porter, I; Vacek, J (2008), "Single ventricle with persistent truncus arteriosus as two rare entities in an adult patient: a case report", J Med Case Reports, 2: 184, doi:10.1186/1752-1947-2-184, PMC 2424060, PMID 18513397.	2	52	9	0	2	0	0.842861	11	11,473
The CDC announced that SYNGAP1 has been assigned an ICD-10 code in the US, effective on October 1, 2021 along with 159 other new codes. The SYNGAP1 diagnosis code, F78.A1, falls under the F category of diseases representing mental, behavioral and neurodevelopmental disorders. Having an ICD-10 code will help our patients get access to services, medicines and attention more easily. As a SYNGAP1 family member or caregiver, you need to make your clinician aware and ask them to add this diagnosis code to the medical record. The International Classification of Disease (ICD) coding system is the global standard for classifying and coding patient diagnoses, symptoms, and procedures. Per Wikipedia, it is “the code used for the purpose of documenting a person's medical condition. An ICD code is needed for a person's medical records—it is important for health insurance reimbursement, administration, epidemiology, and research.” The ICD system is governed jointly by the World Health Organization (WHO), the Centers for Medicare and Medicaid Services (CMS), and the Centers for Disease Control and Prevention (CDC). First introduced in 1948 and updated on a regular basis, it is now in its 10th edition. Until now, SYNGAP1 has not had a code. Instead, our patients have accumulated codes for their symptoms: epilepsy, hypotonia, developmental delay, etc. Those codes are adequate for insurance billing and to obtain some services, but they don’t provide a way to accurately track or learn from SYNGAP1 patients and their experiences across healthcare systems. There hasn’t been a way to indicate in a medical record in any measurable way, “This patient has SYNGAP1.” Having a unique ICD code for SYNGAP1 will provide valuable insights into the actual number and demographics of patients, the range and severity of their symptoms, the types of treatments, testing, and services they are receiving, and the impact that interventions are having. The code could help simplify and streamline the insurance approval process for patients, making it easier to receive the most appropriate and beneficial care. And further, being able to report accurately on the high costs associated in caring for SYNGAP1 patients could motivate and foster more research, quickening the development of new therapies and treatments. Once tangible numbers are available, the urgency we feel as the SynGAP community will spread. We ask families and caregivers to share the news and the new code with all their healthcare providers. Ask every doctor, specialist, therapist, and hospital to add the code F78.A1 to patient records and insurance claims. Let’s get this code into the medical record of every SynGAP patient! It was Hans Schlecht, MD, MMSc, a SynGAP parent and a Director of the Syngap Research Fund, who first submitted a proposal for the creation of a new SYNGAP1 code & has diligently advocated for it ever since. Dr. Schlecht is a tireless advocate for ICD coding for rare genetic disease as evidenced by his submission to the ASGH about a new approach for the same. Indeed, the Everylife Foundation wrote a case study on his work in the recent ICD-10 Roadmap. After his effective engagement with the CDC, SYNGAP1 was chosen for consideration at the March 2020 review meeting. Connie Smith-Hicks, MD, PhD of Kennedy Krieger at Johns Hopkins presented the proposal at the six-hour virtual meeting! SRF is very grateful for this decision and the potential for enhanced patient care and research progress it represents. We’re also extremely grateful to Dr. Schlecht for his tireless and determined advocacy, Dr. Smith-Hicks for the investment of her time and expertise, and all involved who made this happen. Good question. ICD codes are published by the WHO and then adopted by countries. ICD-10 was first endorsed by the WHO in 1990, but no adopted in the US until 2015. New diseases are created and codes needed each year, so after adopting ICD-10, individual countries do updates annually. F78.A1 is a US update to our version of the ICD-10 codes. We encourage advocates in other countries to use this example as a way to request the same code in your country. The way we get one code worldwide is by working with the WHO on including SYNGAP1 in ICD-11. Don't worry, Hans has been working on that for months... stay tuned. Here is a screen shot of Hans' account with the WHO ICD-11 Maintenance Platform.	3	2	0	0	0	5	0.653954	5	966
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	0.711575	10	1,077
March is national Deep-Vein Thrombosis (DVT) Awareness Month. This public health initiative aims to raise awareness about this widely prevalent medical condition and its potentially life-threatening complication, pulmonary embolism (PE). The American Medical Association estimates that about 2 million people are affected by DVT each year, more than the numbers affected by heart attack or stroke annually. Billing and coding for these conditions is complex. An experienced medical billing and coding service provider would have proper knowledge of important diagnostic norms and documentation requirements to help physicians assign appropriate diagnoses and procedure codes on claims. What is DVT? DVT occurs when a blood clot (thrombus) forms in one or more veins located deep inside the body. This serious condition occurs when the blood moves too slowly through the veins due to long periods of immobility or reduced mobility or other factors. DVT typically occurs in the lower leg, thigh, or pelvis, but can also occur in other parts of the body. If the blood clot breaks off and travels through the bloodstream, it can block a blood vessel of the lung. This causes the fatal condition known as a pulmonary embolism. DVT Symptoms and Risk Factors Symptoms of DVT can include - Swelling in the foot, ankle, or leg - Cramping and pain - swollen veins - Unusually hot skin in the affected area If DVT occurs with no identifiable risk factor, the condition is called unprovoked DVT. There are many risk factors for DVT. The most common risk factors include prior DVT, obesity, smoking, stroke, pregnancy, hormone therapy, varicose veins, being over the age of 65, and prolonged immobility because of bed rest or travel. The problem with diagnosing DVT is that in most cases, symptoms are minimal and sometimes absent. The presence of certain conditions such as muscle strain, skin inflection, and inflammation of veins (phlebitis) can also make DVT difficult to diagnose. Methods use to diagnose DVT or check for a blood clot include: - Venous Ultrasound: This standard test for DVT involves using sound waves to display how blood flows through the veins. - Venography: This x-ray test involves injecting a dye into a large vein in the foot or ankle to detect clots. - D-Dimer Blood Test: People with severe DVT have high levels of D dimer in the blood. - Impedance Plethysmography: Also known as compression ultrasonography, this highly sensitive test detects DVT using electrodes and a blood pressure cuff placed on the calf and thigh. The recently released guidelines of the European Society for Vascular Surgery (ESVS) recommend using ultrasonography as the first modality in a setting of suspected DVT. - Magnetic Resonance Imaging (MRI): An MRI scan may be used to diagnose DVT in veins of the abdomen. The aim of DVT treatment is to stop the blood clot from increasing in size or breaking off and moving toward the lungs as well as reducing the patient’s risks of another episode. Therapies include: - Anticoagulant Medications: Usually, heparin is prescribed first to prevent further clotting, which may be followed by warfarin to prevent another blood clot forming. - Inferior Vena Cava (IVC) Filter: This small, cone-shaped filter is inserted into the inferior vena cava, the largest vein in your body. The filter can prevent a large clot from reaching the lungs. - Compression Stockings: These special stocking fit in a way to prevent blood from pooling in the veins and ease symptoms. Documenting and Coding DVT Correct ICD-10 coding for DVT requires documentation to the highest level of specificity. Key points to note for proper coding: - Distinguishing Between DVT and Venous Thrombosis with Phlebitis: The term “DVT” indicates venous thrombosis without accompanying phlebitis or inflammation of a vein. “Thrombophlebitis” is the term for venous thrombosis with phlebitis. Thrombophlebitis is due to one or more blood clots in a vein that cause inflammation. DVT, deep vein thrombophlebitis, or central vein thrombosis are the the most common causes of PE. - Documenting to the Highest Level of Specificity: Providers should clearly document whether DVT is acute, chronic or historical. A diagnosis of acute thrombosis is assigned when a new, symptomatic thrombosis is detected and anticoagulation therapy is being started; chronic is assigned when the condition is established, and the patient needs lifelong anticoagulation therapy; the condition is historical when patient no longer has thrombosis but is on anticoagulant therapy as a preventive measure. - Specifying Laterality, Location, and Vein: The documentation should indicate: - Laterality – side of the body, i.e., left or right - The specific location – upper or lower extremity - The specific vein such as femoral, iliac or tibial The physician should also document the diagnostic method used, the anticoagulants administered and duration of treatment. The treatment and its goals should be linked to the diagnosis. ICD 10 Codes for DVT of Lower Extremities and Upper Extremities – Acute and Chronic Acute embolism and thrombosis of lower extremity I82.41 femoral vein 182.42 Iliac vein I82.43 Popliteal vein I82.44 Tibial vein I82.45 Peroneal vein I82.46 Calf muscular vein I82.49 Other specified deep vein of lower extremity Chronic embolism and thrombosis of lower extremity 182.52 Iliac vein I82.53 Popliteal vein I82.54 Tibial vein I82.55 Peroneal vein I82.56 Calf muscular vein I82.59 Other specified deep vein of lower extremity Acute embolism and thrombosis of upper extremity I82.61 Superficial veins of upper extremity I82.62 deep veins of upper extremity I82.A1 Axillary vein I82.B1 Subclavian vein I82.C1 Internal jugular vein Chronic embolism and thrombosis of upper extremity I82.71 Superficial veins of upper extremity I82.72 Deep veins of upper extremity 182.A2 Axillary vein I82.B2 Subclavian vein I82.C2 Internal jugular vein The personal history codes for “history of” DVT, thrombophlebitis, and PE are Z86.718, Z86.72, and Z86.711, respectively. As a secondary diagnosis, all codes for acute and chronic DVT are categorized as complications/comorbidities (CCs) and almost all of them are included in HCC 108, vascular disease without complication (ACP Hospitalist). As the nation observes DVT Awareness Month, leading medical, public health and patient advocacy groups are working towards educating the public about the signs, symptoms and risk factors of this serious medical condition. Though DVT can develop without any obvious reason and is not entirely preventable, taking appropriate preventative measures can reduce the risk of formation of a blood clot. Preventive measures to maintain good circulation include: - Living an active life with regular exercise - Maintaining a healthy weight - Avoiding long periods of inactivity or immobility - Moving legs when on a long flight - Wearing compression stockings if at risk for DVT Health professionals should focus on early diagnosis and treatment of DVT to minimize the risk of complications. Ensuring clear and comprehensive documentation is crucial for medical coding service providers to use the right codes to represent the diagnosis accurately.	4	2	0	0	0	2	0.971706	2	1,713
What is ICD code? The full form of ICD is the International Classification of Diseases. WHO created International Statistical Classification of Diseases and Related Health Problems (ICD), a medical classification list of codes to help develop a universal code for doctors and insurance companies worldwide. It contains codes for diseases and causes of injuries and deaths. The universal codes help doctors, and insurance companies keep a record of patients. What is the latest version of ICD? The latest version of ICD is the ICS-11 which WHO released a preview in 2019 with 55,000 codes for causes of death, diseases, and injuries. The new revision was designed to simplify the coding structure, so healthcare providers can more easily record medical conditions. This edition is also the first to be completely electronic, so it can integrate with electronic data sources and is free to download online for personal use. The ICD-11 will come into effect on 1 January 2022. The new revision will also include new chapters on sexual health and traditional medicine. Get a dose of health tips delivered for free ICD-10 code for Erectile Dysfunction N52.9 is the code for Erectile Dysfunction in 1CD-10. The ICD-10-CM code N52.9 might also be used to specify conditions or terms like arteriopathy impotence, cannot get an erection, cannot sustain an erection, complaining of erectile dysfunction, delayed erection, endocrine impotence, etc The code N52 is applicable to adult patients aged 15 through 124 years inclusive. It is clinically and virtually impossible to use this code on a patient outside the stated age range. In N52 there are different codes specifying different Erectile Dysfunctions based on their causes \|N52.0\|\|Vasculogenic erectile dysfunction\| \|N52.01\|\|Erectile dysfunction due to arterial insufficiency\| \|N52.02\|\|Corporo-venous occlusive erectile dysfunction\| \|N52.03\|\|Combined arterial insufficiency and corporo-venous occlusive erectile dysfunction\| \|N52.1\|\|Erectile dysfunction due to diseases classified elsewhere\| \|N52.2\|\|Drug-induced erectile dysfunction\| \|N52.3\|\|Postprocedural erectile dysfunction\| \|N52.31\|\|Erectile dysfunction following radical prostatectomy\| \|N52.32\|\|Erectile dysfunction following radical cystectomy\| \|N52.33\|\|Erectile dysfunction following urethral surgery\| \|N52.34\|\|Erectile dysfunction following simple prostatectomy\| \|N52.35\|\|Erectile dysfunction following radiation therapy\| \|N52.37\|\|Erectile dysfunction following prostate ablative therapy\| \|N52.8\|\|Other male erectile dysfunction\| \|N52.9\|\|Male erectile dysfunction, unspecified\| - Erectile dysfunction - Erectile dysfunction, organic - Erectile dysfunction, vasculopathic - The impotence of organic origin - Male erectile disorder - Vasculopathic erectile dysfunction - A disorder characterized by the persistent or recurrent inability to achieve or to maintain an erection during sexual activity. - An inability to have an erection of the penis adequate for sexual intercourse. - Erectile dysfunction (ed) is when a man has trouble getting or keeping an erection. Ed becomes more common as you get older. But male sexual dysfunction is not a natural part of aging. Some people have trouble speaking with their doctors about sex. But if you have ed, you should tell your doctor. Ed can be a sign of health problems. It may mean your blood vessels are clogged. It may mean you have nerve damage from diabetes. If you don’t see your doctor, these problems will go untreated. Your doctor can offer several new treatments for ed. For many men, the answer is as simple as taking a pill. Getting more exercise, losing weight, or stopping smoking may also help. - Inability to perform sexual intercourse. - The inability in the male to have a penile erection due to psychological or organ dysfunction. ICD-11 code and description for Erectile Dysfunction HA01.1 is the code for erectile dysfunction in ICD-11 Male erectile dysfunction is characterized by the inability or marked reduction in the ability in men to attain or sustain a penile erection of sufficient duration or rigidity to allow for sexual activity. The pattern of erectile difficulty occurs despite the desire for sexual activity and adequate sexual stimulation, has occurred episodically or persistently over a period of at least several months, and is associated with clinically significant distress. \|1\|\|HA40\|\|Aetiological considerations in sexual dysfunctions and sexual pain disorders\| \|2\|\|HA40.1\|\|Associated with psychological or behavioral factors, including mental disorders\| \|3\|\|HA40.2\|\|Associated with the use of psychoactive substance or medication\| \|4\|\|HA40.3\|\|Associated with lack of knowledge or experience\| \|5\|\|HA40.4\|\|Associated with relationship factors\| Associated with cultural factors Other specified aetiological considerations in sexual dysfunctions and sexual pain disorders Risk factors for Erectile dysfunction mentioned in ICD-11 There are many risk factors for Erectile dysfunction and those include - Heart disease ICD-10: I25.10 - Atherosclerosis ICD-10: I70.90 - High cholesterol ICD-10: E78.5 - High blood pressure ICD-10: R03.0 - Diabetes ICD-10: E11.9 - Obesity ICD-10: E66	4	2	0	0	0	7	0.861072	7	1,206
Nerve conduction studies (NCS) measure how fast electrical impulses travel along the nerves. A nerve conduction test is usually performed in conjunction with electromyography (EMG) which evaluates the electrical functioning of muscles. These electrodiagnostic (EDX) tests are used to help in the diagnosis of a variety of nerve and muscle disorders. Neurology medical billing and coding can be complex as there are specific codes for every diagnostic service and treatment. Up-to-date professional billing and coding guidance can be crucial for the submission of claims for reimbursement of covered services. Here are 4 key tips to help with nerve conduction study coding: - Select the correct code based on the number of nerves tested: Choosing the correct code depends on the number of nerve conduction studies performed. The CPT codes for NCS are: 95905 Motor and/or sensory nerve conduction, using preconfigured electrode array(s), amplitude and latency/velocity study, each limb, includes F-wave study when performed, with interpretation and report 95907 1-2 nerves 95908 3-4 nerves 95909 5-6 nerves 95910 7-8 nerves 95911 9-10 nerves 95912 11-12 nerves 95913 13 or more nerves CPT defines a single conduction study is: “a sensory conduction test, a motor conduction test with or without an F wave test, or an H-reflex test.” CPT lists individual nerves for billing NCS. The list identifies each individual sensory, motor, or mixed nerve, or nerve segment that can be counted separately toward the NCS total. Each list of nerves refers to a different nerve and should be counted as an individual unit. For e.g., the 4 different nerve segments of the ulnar motor nerve can each be counted separately toward the total. - Each type of study is counted as a distinct study. - If the provider performs stimulation or recording of multiple sites on a single nerve during a single study, this should be still counted as a single study. For e.g., testing the ulnar nerve at wrist, forearm, below elbow, above elbow, axilla and supraclavicular regions are all considered as a single nerve. - Multiple NCS CPT codes cannot be billed for a given patient on a single day The American Association of Neuromuscular & Electrodiagnostic Medicine (AANEM) recommends that physicians test the fewest number of nerves needed to assess or diagnose a medical issue when performing an NCS. The physician should provide supplementary documentation to justify any additional testing and its medical necessity. - Report EMG when performed: Electrodiagnostic tests (EDX) cover a range of specialized tests, including nerve conduction studies (NCS) and needle electromyography (EMG).Individual tests should be reported using the NCS code as the primary code and EMG codes as add-on codes. The add-on codes for the EMG are: 95885 Needle electromyography, each extremity, with related paraspinal areas, when performed, done with nerve conduction, amplitude and latency/velocity study; limited (List separately in addition to code for primary procedure) 95886 Needle electromyography, each extremity, with related paraspinal areas, when performed, done with nerve conduction, amplitude and latency/velocity study; complete, five or more muscles studied, innervated by three or more nerves or four or more spinal levels (List separately in addition to code for primary procedure) 95887 Needle electromyography, non-extremity (cranial nerve supplied or axial) muscle(s) done with nerve conduction, amplitude and latency/velocity study (List separately in addition to code for primary procedure). Checking payer policies in important to know which neurology diagnoses support coverage for testing. - Adhere to payer guidelines: To bill and code nerve conduction studies correctly, providers need to be aware of the latest codes as well as the reimbursement policies of governmental and private payers. For instance, the Centers for Medicare & Medicaid Service (CMS) guidelines on CPT code 95905 are as follows: - Nerve conduction studies done using preconfigured electrode array(s) using automated devices, such as NC- stat® System, cannot support testing of other locations and other nerves as needed depending on the concurrent results of testing and must not be billed with any CPT code other than 95905. - When the beneficiary has a high pre-test or a priori probability for a Carpal Tunnel Syndrome diagnosis, the automated devices alone will be allowed, one service per arm, using code 95905 along with diagnosis codes G56.00-G56.03 only. - Code 95905 is payable only once per limb studied and cannot be used in conjunction with any other nerve conduction codes. - Code 95905 is not allowed to be billed by physical therapists. - Document medical necessity: To avoid having claims denied, providers should report diagnoses to inform the payer why a service was performed and support medical necessity of the nerve conduction study and needle electromyography. Every CPT code should be paired with an ICD code that demonstrates the medical necessity. Government and commercial health insurance payers state the criteria that nerve conduction studies and needle electromyography need to meet to be considered medically necessary, and which procedures are considered unproven and medically unnecessary. AANEM clarifies: - Nerve Conduction Studies EDX testing with automated, noninvasive nerve conduction testing devices is considered investigational and not medically necessary for all indications. - Screening testing for polyneuropathy of diabetes or end stage renal disease (ESRD) is NOT covered. Testing for the sole purpose of monitoring disease intensity or treatment efficacy in these two conditions is not covered. - Psychophysical measurements (electrical, vibratory or thermal perceptions) are not covered. Current Perception Threshold/Sensory Nerve Conduction Threshold Test (sNCT) is investigational and not covered. The patient’s health plan and the regulations laid out by the payer determine whether a procedure is billable. Reliable neurology medical billing company have teams of expert coders and billing specialists. Coders will report the correct diagnostic code for each diagnostic test ordered and the billing team will review the codes reported to ensure that the procedures coded are billable. With their knowledge of the latest codes and coverage policies of each payer, neurology medical billing support can help practitioners avoid claim denials.	4	13	9	0	0	2	0.233766	11	1,382
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. 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: Environmental conditions often erroneously thought to affect ICDs include: 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 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.	3	8	2	0	0	0	0.717374	2	2,520
Textbook of Psychiatry/Psychotic Disorders< Textbook of Psychiatry Schizophrenia and Related Psychotic Disorders Psychosis, a syndrome with many causes, traditionally refers to an impaired ability to distinguish between false and real perceptions and beliefs. Schizophrenia is the prototypical psychotic disorder. The most common psychotic symptoms are positive symptoms such as abnormal perceptions (including illusions and hallucinations), false beliefs, including a wide variety of delusional thoughts (e.g., paranoid delusions, delusions of reference, grandiose, somatic, etc.), and disorganized thinking. In addition, patients with schizophrenia might have prominent negative symptoms such as affective flattening, alogia (decreased thought/speech production), and avolition, together with amotivation, anhedonia and social isolation. Disorganized or bizarre behavior is a separate symptom dimension of the disorder. Affective symptoms can also be present and cognitive and social deficits are common. This chapter focuses on primary psychotic disorders, as illustrated by schizophrenia, meaning that the clinical picture of psychosis is not deemed to be secondary to other processes. It is important to note that in addition to the primary psychoses a number of psychiatric and somatic conditions affecting the brain homeostasis can produce psychotic symptoms. Patients with personality disorders (PDs) can present with overt psychotic symptoms in response to stress (e.g., paranoid PD, schizotypal PD, borderline PD). Schizoid PD is considered a risk factor and might precede Schizophrenia and Delusional Disorder. With regards to mood disorders, severe psychotic depression can present with mood congruent (e.g., nihilistic delusions, delusional guilt) and/or auditory hallucinations making critical and negative comments. At the opposite end of the spectrum, severe mania can present with grandiose and religious delusions, delusions of special powers, and auditory hallucinations (God’s or angelic voices). Late life psychosis can be present in the later stages of dementia disorders. Conditions that affect the brain structure, either acutely [e.g., rapidly growing brain tumors, traumatic brain injury, strokes, infectious/inflammatory processes such as tertiary syphilis, multiple sclerosis or systemic lupus erythematosus (SLE)], or chronically [e.g., nutrient and vitamin deficiencies such as B12, niacin deficiency (pellagra), etc.] can present with a variety of psychotic symptoms. Last but not least, a number of drugs (prescribed and illicit) can be associated with psychotic symptoms either during treatment/intoxication or withdrawal. This chapter will first review the definitions of the different types of psychotic symptoms, as the basis for the discussion about the approach (including initial assessment as well as short and long-term treatment plans) to a patient with a generic psychotic syndrome. For the remainder of the chapter schizophrenia is used as the foundation for the discussion of clinical diagnosis, differential diagnosis, epidemiology, pathophysiology, genetics and treatment. Pertinent details of schizophrenia-related disorders will be discussed (compared and contrasted whenever the case) within the confines of the broader schizophrenia mainframe. Clinical Manifestations and Definition of TermsEdit - Positive Symptoms are thought of as an excess of normal function. Overvalued misperceptions that become illusions and hallucinations and overvalued ideas that become delusions (fixed ideas) are classical examples of positive symptoms. - Negative Symptoms refer to a lack of what is considered to be normal function. Normally, a degree of volitional ability is expected; therefore decreased or absent volition (avolition) is a negative symptom. Similarly, a lack of motivation (amotivation), a lack of ability to enjoy things (anhedonia), or decreased ability to engage in social activities (social isolation) are other classical negative symptoms. - Catatonia refers to two extreme (and fundamentally opposite) states. Agitated catatonia refers to a state of excessive, extreme behavioral agitation (not in response to internal stimuli), while catatonic immobility refers to extreme negativism (the patient actively resists any attempts to have his extremities or whole body moved) or catalepsy (waxy flexibility). Other catatonic symptoms include posturing (assuming strange body postures), grimacing, mannerisms, stereotyped movements, echolalia (where the patient repeats in parrot-like fashion the words of another person), and echopraxia (where the patient imitates in mirror-like fashion the movements of another person). - Disorganized thinking (formal thought disorder) refers to an alteration in the thought process. Normally the flow of thinking is coherent, linear and goal directed. In psychotic patients the associations may be loose to the point of being non-existent. The psychotic patient’s thought form may present with tangentiality (ideas are only marginally connected) or circumstantiality (the patient responds to questions moving in gradually more focused, concentric circles until eventually reaching the answer). In extreme cases, even the structure of the sentence might be lost which results in word salad. - Disorganized behavior refers to the patient difficulty to complete most goal oriented activities. A range of behaviors have been described: actively responding to inner stimuli (e.g., talking to oneself or shouting for no apparent reason), aimless, repetitive movements and activities, poor ability to maintain one’s basic hygiene and perform routine actives of daily living (which often results in a disheveled appearance, and poor grooming and hygiene), or uncensored public sexual activity (being naked, or masturbating in public). - Active phase refers to a period of time when a combination of the above symptoms are prominently manifested. - Prodromal and residual phases refer to periods of time of attenuated symptoms that either precede (prodromal) or follow (residual) the active phase period. - Cognitive Symptoms: Memory (more specifically working memory), attention, concentration, processing speed, problem solving (executive functioning), and social cognition are a few of the many cognitive domains shown to be impaired in schizophrenia. - Insight is a multidimensional concept referring to awareness of illness, specific symptoms and their consequences, as well as need for treatment. Insight refers to the patient’s ability to understand that some of his or her non-reality based experiences (usually hallucinatory experiences and delusional representations) are secondary to having schizophrenia rather than reality. Awareness and attribution of both current and past symptoms represent specific aspects of insight. Additional dimensions of insight include a more global understanding of the diagnosis and need for treatment. Approach to the Patient with Acute PsychosisEdit The following major issues should be kept in the forefront: - What is the most accurate diagnosis? - Is there a treatable or reversible component to the psychosis? - Is the patient safe? - Can the physician help to alleviate the positive symptoms? - Can the physician help to alleviate the negative, cognitive symptoms and insight deficits to improve social/functional outcomes? The history should clarify the onset (acute versus gradual), tempo (slow/protracted versus rapid), chronology, course (persistent versus episodic), and type of symptoms. Onset and tempo An acute or subacute onset of psychosis may represent delirium, psychosis due to a general medical condition, or a substance induced psychosis and should trigger the search for intoxication, infection, or metabolic derangement. According to the Diagnostic and Statistical Manual of Mental Disorders IV Text Revision (DSM-IV-TR) a diagnosis of schizophrenia requires the presence of a combination of prominent positive, negative, disorganized thinking (formal thought disorder), catatonia, or behavior type of symptoms for at least a month (active phase), with a total duration of the episode (including active phase, and some type of prodromal or residual symptoms) for at least 6 months and resulting in social and occupational dysfunction. A schizophrenia-like presentation that lasts more than a month but less than 6 months would be more appropriately diagnosed as schizophreniform disorder. Brief psychotic disorder should be diagnosed when the total duration of symptoms is shorter than a month. Schizoaffective disorder trumps schizophrenia if in addition to stand alone episodes of psychotic symptoms there is also a long history of affective symptoms, and the affective symptoms occurred for a longer time than the psychotic symptoms. Refers to the temporal rapport between the different symptoms. Clarifying what started and what followed are essential in ruling out phenomenologically overlapping disorders. If it is determined that the psychotic symptoms followed a medical condition or drug (prescribed or illicit) psychotic disorder due to a general medical condition, substance induced psychotic disorder, or delirium need to be considered first. Mood disorder with psychotic symptoms is diagnosed if the history shows that psychotic symptoms always occurred in the context of already present, and most often severe affective (depressive and manic) symptoms. A clearly episodic course is most times indicative of a primary affective disorder. Unfortunately, schizophrenia tends to be chronic, with some level of residual symptoms following the active phase for most patients. However, for schizophrenia, after one year since the onset of the acute phase symptoms, DSM allows for a number of course based specifiers including: single episode with partial/total remission, episodic with/without inter-episode residual symptoms, and continuous. Physical and Neurological ExaminationEdit A thorough general and neurological examination is recommended. General physical examination Is recommended to first rule out a systemic disease that may be responsible for the psychotic syndrome. A number of non-specific physical abnormalities including an arched palate, narrow or wide–set eyes or subtle ear malformations are more frequently reported in patients with schizophrenia than in the general population. For patients treated with antipsychotics a physical exam will document the general state of health and is important to exclude side effects of medication. Side effects include orthostatic hypotension, hypersalivaton (secondary to clozapine), anticholinergic syndrome (dry mouth, and tachycardia secondary to anticholinergics), hyperprolactinemia (lactation secondary to D2 antagonism), and metabolic syndrome (most common with clozapine and olanzapine). Is recommended to rule out neurological conditions that may present with psychotic manifestations; of note, abnormal focal neurological signs are not typically found in primary psychotic disorders. Such findings should prompt the clinician to do a more extensive neurological work-up. In addition, a neurological exam is necessary to exclude the presence of soft neurological signs and abnormal involuntary movements. Soft (neurological) signs, while not pathognomonic, are frequently seen in schizophrenia, where "soft" denotes the absence of a clearly localized ("hard") central nervous pathology that can explain the observed deficits. They include: - Sensory function integration abnormalities include poor audio—visual integration, astereognosis (the inability to identify an object by touch without visual input), and agraphaesthesia (the inability to recognize writing on the skin purely by the sensation of touch). - Motor function integration abnormalities might include balance and gait abnormalities, poor coordination, intention tremor, finger—thumb opposition difficulties. In addition, a number of abnormal involuntary movements have been classically described in chronic schizophrenia (before the neuroleptic age) but have been much more prevalent since the introduction of antipsychotic dopamine antagonist drugs. These include: - Akathisia, which refers to low amplitude, high frequency movement typically involving the lower extremities. The patient reports a feeling of intolerable restlessness, specifically manifested as a need to continuously move one’s feet. The patient cannot stop pacing (paces in place when asked to sit or stand without walking), - Dystonia, which refers to a high amplitude, low frequency, spastic type of movement, typically involving an isolated muscle group, e.g., oculopharogyric crisis (eyes turned upwards), torticollis (neck turned sideways), laryngeal spasm (rare but serious as it might result in asphyxia), opisthotonus (arched back, rare, painful) - Dyskinesia, which refers to low amplitude, repetitive, moderate frequency, pseudo-parkinsonian movements that may involve any muscle group but most typically involve the fingers, hands, toes, feet, lips and lower face muscles (including perioral and mandibular muscles) - Tremor, which refers to a low amplitude, high frequency, repetitive movement. Tremor of the hands and fingers can be spontaneous or can be elicited by asking the patient to put his arms in a horizontal position and stretch his fingers. In addition, a parkinsonian pill rolling tremor may also be observed. In patients taking lithium a fine tremor(very low amplitude, very high frequency) may be seen. Mental Status Examination (MSE)Edit - Appearance: disheveled or bizarre appearance may be a clue to underlying psychosis. Impaired reality testing commonly results in poor grooming and hygiene. - Attitude: paranoid patients may be unwilling to co-operate during an interview, while very psychotic patients my be unable to engage with the interviewer. - Motor behavior: posturing, repetitive gestures, extreme psychomotor agitation (without any apparent precipitants or retardation) can indicate a catatonic presentation. Alternatively, the patient may present with psychomotor agitation in response to overwhelming internal stimuli (e.g., loud, demeaning voices or threatening visions) or because of severe paranoid ideation. - Mood: patient’s reported mood can vary from good to depressed or afraid. - Affect: paranoid patients present with guarded affect, eyes scanning the room, and a closed up body language. - Speech/thought process: can be vague, circumstantial or overtly disorganized. At times nonsensical neologisms, word salad, clang (rhyming, nonsensical associations) are present. - Thought content: may be positive for delusional ideation (most common ideas of references and paranoid delusions). In addition, the patient may harbor suicidal and violent thoughts due to his persistent psychotic symptoms or, at times, related to concomitant depressive symptoms. - Perceptual disturbances: auditory hallucinations can be commanding and order the patient to kill himself or other people. When visual hallucinations are present they tend to be unpleasant as a rule and are often overtly terrifying. - Insight and Judgement: judgement is mostly impaired and the patient has very limited, if any, insight. - Cognition: with the possible exception of decreased attention, other cognitive deficits may not be obvious during a cursory MSE. In schizophrenia neuropsychological testing routinely reveals deficits in working memory, executive functioning, social functioning, processing speed, verbal fluency, and/or reaction time abnormalities. Unfortunately, the ability to test for these deficits routinely in clinical practice is limited by the lack of good, time efficient screening cognitive instruments for schizophrenia and related disorders. There are no tests that can rule in a diagnosis of schizophrenia or related disorders. The role of laboratory investigations are to rule out substance induced disorders and general medical conditions that can present with a psychotic syndrome; to establish a baseline and monitor physiological functions that can be affected by, or can affect the metabolism of psychotropic medications; and monitor drug levels when necessary. Investigations to exclude a substance induced disorder or general medical condition: - urine or blood toxicology screen: should be performed routinely in all patients presenting with new onset or exacerbated psychotic symptoms, as a number of illicit drugs can cause/worsen psychosis (e.g., hallucinogens, cocaine, stimulants, marijuana). - Complete blood cell count (CBC): blood dyscrasias can point to an underlying vitamin deficit that may manifest with psychosis (e.g., pernicious or megaloblastic anaemia as a sign of vitamin B12/folate deficits) - Rapid plasma reagin (RPR): done to rule out (tertiary) syphilis - Thyroid panel: indicated when there is a clinical suspicion for hypo or hyperthyroidism - Brain Imaging: - Structural brain imaging (CT or MRI) is indicated to rule out other brain pathologies (e.g., multiple strokes, demyelination, masses). Neuroimaging studies do not show a pattern of findings specific for schizophrenia or related disorders and may be normal early in the course of the disease. As schizophrenia progresses, enlarged ventricles and diffuse cortical atrophy becomes apparent. MRI scans may also show atrophy of the parahippocampal gyrus, dorsolateral prefrontal cortex, mesolimbic system, the anterior cingulate cortex, and planum temporalis asymmetry reversal or generalized reductions in grey and white matter. - Functional brain imaging studies (PET and functional MRI) demonstrate abnormalities in the same regions. However, none of these changes are pathognomonic for schizophrenia or related disorders. A liver function panel and chemistry panel (to document renal function) are recommended to establish a baseline for physiological functions that can affect the metabolism of psychotropic medications. Other tests that may be indicated to monitor side effects of psychotropic medication include a blood glucose level, a lipid panel, and an ECG (as some antipsychotics have the potential of prolonging the QTc interval). A prolactin level should only be measured when prolactinemia is suspected on clinical grounds. The following drug levels need monitoring: lithium (0.7 to 1.2 mEq/L), carbamazepine (5 to 12 mcg/mL), and valproic acid (50 to 100 mcg/mL). A clozapine level above 350 ng/mL is recommended to establish compliance and has been shown to correlate with improved efficacy for refractory schizophrenia. There is no clear evidence of a therapeutic range for other antipsychotics. Specific Types of Primary Psychotic DisordersEdit General Considerations and Differential DiagnosisEdit When a patient presents with a psychotic syndrome the first order of business is to establish if the presenting symptoms are due to another psychiatric or somatic condition. In other words, a psychotic syndrome is classified as "primary psychosis" only after other possible underlying pathologies have been ruled out. In terms of somatic contributors, the main suspects should include processes that may affect the brain either acutely or chronically, in which case a diagnosis of psychotic disorder due to a general medical condition is appropriate. A substance induced psychotic disorder should be diagnosed if there is a likely cause and effect relationship between a substance (including medication, OTC products or illicit drugs) and the psychotic presentation. Psychiatric underlying pathologies include severe depressive and bipolar disorder, which may present with mood congruent psychotic features. As discussed, under stress, some personality disorders may present with transient psychotic symptoms. The differential diagnosis between different primary psychotic disorders should take into account the type and duration of symptoms. Virtually identical symptoms are seen in schizophrenia, brief psychotic disorder, and schizophreniform disorder. The symptom duration differentiates brief psychotic disorder (1 day to <1 month) from schizophreniform disorder (1 month <6 months) and schizophrenia (>6 months). Delusional disorder is differentiated from schizophrenia based on prominent, non-bizarre delusions without any other associated symptoms. When distinct psychotic episodes are present but affective symptoms account for the majority of the clinical presentation a diagnosis of schizoaffective disorder should be considered. Conceptual History and Diagnostic ClassificationEdit - 1853: Morel’s curious cases of Démence Précoce: Bénédict Morel introduces the concept of Démence Précoce, literally "early dementia", described a distinct syndrome affecting teenagers and young adults. The syndrome is characterized by bizarre behavior and mental function, withdrawal and self neglect starting in adolescence. - 1868: Kahlbaum’s Katatonie: Karl Ludwig Kahlbaum and Ewald Hecker publish Die Gruppierung der psychischen Krankheiten (The Classification of Psychiatric Diseases). By considering the longitudinal course of psychiatric symptoms in addition to the clinical presentation Kahlbaum and Hecker were the first to describe and name a number of psychiatric syndromes including cyclothymia, dysthymia, paranoia, catatonia, and hebephrenia. Kahlbaum’s Katatonie was characterized by stereotyped movements, outbursts of excitement and stupor. - 1870: Ewald Hecker’s hebephrenia and cyclothymia: Hecker differentiates between hebephrenia, a disorder that begins in adolescence with erratic behavior followed by a rapid decline of all mental functions, and cyclothymia, a cyclical mood disorder. - 1891: Arnold Pick reports on a case of a psychotic disorder which he calls Dementia Praecox - 1893: Emil Kraepelin’s Dementia Praecox: Kraepelin new classification of mental disorders distinguishes between dementia praecox and mood disorder (termed manic depression and including both unipolar and bipolar depression). - Dementia Praecox: A "sub-acute development of a peculiar simple condition of mental weakness occurring at a youthful age." - Distinct from catatonia and dementia paranoides. - Kraepelin’s concept relied heavily on course (chronic versus episodic) and prognosis - 1899: hebefrenia, catatonia and dementia paranoides as subtypes of dementia praecox. - 1919: Kraepelin writes that "it is becoming increasingly clear that we cannot distinguish satisfactorily between these two illnesses and this brings home the suspicion that our formulation of the problem may be incorrect." - 1908: Eugen Bleuler’s Schizophrenia gk. skhizein "to split"+ phren (gen. phrenos) "diaphragm, heart, mind", where "split mind" referred to being split off from reality and unable to distinguish what is real from what is not real. Of note, Bleuler never implied that people with schizophrenia have split personalities; he proposed the term of schizophrenia to describe the separation of function between personality, thinking, memory, and perception. - Bleuler 4 A's: flattened Affect, Autism, impaired Association of ideas and Ambivalence. - Bleuler proposal for a new name also stemmed from his dissatisfaction with the implications of dementia praecox label. Bleuler noted that schizophrenia was NOT a dementia, as some of his patients improved. - 1887 – 1967: Kurt Schneider described the first rank symptoms (FRS), thought to be specific for schizophrenia psychosis. He included thought insertion/broadcast/withdrawal, made feelings/impulses/actions/somatic sensations (a type of delusion), third person auditory hallucinations (running commentary or arguments), delusional perception, and thought echo (echo de la pensee or gendankenlautwerden) – a type of hallucination. Only 58% of patients with a diagnosis of schizophrenia experience at least one FRS, while 20% never experience FRS. Furthermore, 10% of patients with a diagnosis of schizophrenia experience FRS. - Modern positive and negative symptoms based classification systems: - Positive symptoms include distortions or excesses of normal functioning such as, hallucinations, delusions, disorganized thinking and speech, and inappropriate affect. Frequently hallucinations are auditory in nature; rarely they may be visual, tactile or olfactory. Delusions are fixed false beliefs held despite negative evidence, and are not consistent with cultural norms. Types include persecutory, referential, somatic, grandiose, etc. Positive symptoms are generally more responsive to treatment than negative symptoms. - Negative symptoms involve a decrease or absence of normal behavior. They include affective flattening, impoverishment of speech and language, avolition, amotivation, lack of interest, anhedonia, and social isolation. - Modern classifications: - Andreasen's Positive and Negative Symptoms Type - Crow Type I and II: - Type I – positive symptoms, good response to treatment, relatively better outcome - Type II – negative symptoms, poorer response to treatment, relatively poor outcome, MRI changes. Current classification – ICD 10/ DSM-IV-TREdit Common ICD/DSM types: - Paranoid schizophrenia: - Prominent delusions, auditory hallucinations - Usually minimal thought disorder or negative symptoms - Catatonic schizophrenia is characterized by prominent psychomotor symptoms e.g., violent excitement, posturing, waxy flexibility, automatic obedience, perseveration, stupor. - Residual schizophrenia or "defect state", when positive symptoms give way to negative symptoms. - Simple schizophrenia refers to insidious development of negative symptoms without positive symptoms DSM IV only: - Disorganized schizophrenia: mainly thought disorder, and negative symptoms, without prominent positive or affective symptoms. ICD 10 only: - Hebephrenic schizophrenia: affective abnormality, thought disorder, mannerisms. May have chronic course. Epidemiology and Risk FactorsEdit The life time prevalence of schizophrenia is between 0.5-1.5% in the general population and is one of the ten leading causes of disability worldwide. Of note, this 1 in 100 rate has been shown to be remarkably constant across different historical periods and across different cultures. The annual incidence is reported to be in the range of 0.5 to 5 per 10,000. The onset of schizophrenia is usually between the ages of 20-45. Most times, the course of the disorder is chronic and characterized by a gradual, progressive deterioration. However partial or complete recovery is reported to occur for 30-60% of patients following a first episode of schizophrenia.* About 20-40% of patients with schizophrenia attempt suicide at least once during their lifetime, and about 10-15% die of suicide. The prevalence in males and females is equal. The following risk factors have been reported for schizophrenia: - Men tend to be diagnosed earlier than women (males age 15-25 years, females age 25 – 35 years) - Seasonality: winter birth excess - Schizoid and schizotypal personality disorders - A family history of schizophrenia or major affective disorders - A family with a high level of expressed emotions (EE) - Schizophrenia tends to be more frequent in urban areas and in developed countries - Lower socioeconomic status - Schizophrenia is more frequent in recent immigrants (deprivation, stress of immigration may increase risk) The rate of schizophrenia is increased in families with affected members. Mode of Transmission is unknown and likely to be multi-factorial, possibly polygenic. 70% of the heritability of schizophrenia is genetic. Adoption studies have shown an increased incidence of schizophrenia spectrum disorders among adopted offspring of schizophrenic parents. When one parent has schizophrenia there is a twelve fold increase in the risk of developing the disorder; with one affected sibling there is a 9 fold increase in risk; for monozygotic, identical twins the rate of concordance is around 50%. Working memory appears to be heritable and showed significant associations with DISC1, reelin, and AKT1 in schizophrenia. While there are no structural or functional brain changes specific to schizophrenia or other psychotic disorders a number of abnormalities are reported. Enlarged ventricles, deep cortical sulci, diffuse gray and white matter loss, increased neuronal density, decreased synapse density, and an overall decrease in brain size have been reported in schizophrenia studies using structural brain imaging (CT, structural and diffusion sensor MRI studies) or postmortem observations. Smaller frontal and temporal lobes, lower volume hippocampus, thalamus, corpus callosum, and anterior cingulate, as well as larger caudate and putamen have been reported in schizophrenia. Decreased activation in dorsolateral prefrontal cortex (during working memory task), and increased activation of the superior temporal gyrus (during auditory hallucinations) have been also reported in functional brain imaging (fMRI and PET) studies. Impaired fetal or neonatal brain development may sow the seeds for the onset of psychotic symptoms in later life. Patients with schizophrenia have a lower than average IQ, and often subtle/soft neurological signs. A number of parental risk factors have been reported including multiparity, maternal bleeding during pregnancy, small baby size for gestational age, increased paternal age, and severe stress to mother during first trimester. In addition, the following environmental risk factors have been associated with increased risk of developing psychotic illness later in life: late winter birth, prenatal exposure to famine, in-utero exposure to analgesics, and cannabis use. - P50 sensory gating deficits: following an auditory stimulus schizophrenia patients fail to gate a subsequent stimulus that follows closely (within the normal 50 msec suppression). - Reduced P300 evoked response potential (ERP) [oddball deficit paradigm]: schizophrenia patients fail to respond to an odd ball stimulus administered during a series of otherwise identical stimuli. - Prepulse Inhibition (PPI) Paradigm. - Hypothesis: excessive DA activity in mesolimbic and cortical brain regions. Schizophrenia is the result of a dopaminergic hyper-salient state - Supporting evidence: - Postmortem studies: increase DA receptors in schizophrenia - HVA (dopamine metabolite) in plasma, CSF and severity of psychosis/response to neuroleptics - DA Agonists - Amphetamines release DA at synapses and cause positive symptoms (in people who do not have schizophrenia) - L-dopa increases central DA concentrations and causes positive symptoms - DA Antagonists: All effective antipsychotics are D2 receptor antagonists; efficacy correlates with D2 occupancy - Amphetamines and L-dopa do not produce negative symptoms - Antipsychotics are ineffective in 30% of patients - Antipsychotics block D2 receptors instantly but antipsychotic effect not evident for days - Hypothesis: serotonin excess - LSD and psilocybin are potent 5HT receptor agonists and cause positive symptoms of schizophrenia (in people who do not have schizophrenia) - Atypical antipsychotics are potent 5HT2 receptor antagonists - Limitations: LSD produces visual hallucinations which are uncommon in schizophrenia Excitatory amino acids (EAAs): glutamate and aspartate - Hypothesis: EAAs deficit - Phenylcyclidine (PCP), which antagonizes EAA receptors, can produce positive and negative symptoms in people without schizophrenia - Glutamate agonists (e.g., glycine), may be modestly therapeutic in schizophrenia - Freud: delusions as a way of making sense of a disturbed internal world ("I need to respond with aggression to protect myself as everyone is attacking me"). - Klein: failure to resolve the paranoid/schizoid position - Cameron: loss of conceptual boundaries - Goldstein: concrete thinking - Difficulties in filtering sensory input (see also electrophysiological findings) - Probably more important in precipitating schizophrenia than causing it - Lidz’s marital schism/marital skew - Bateson’s double bind theory - High expressed emotion - Social adversity in childhood and fetal life associated with risk of developing schizophrenia and other psychoses later in life - Risk factors for psychoses later in life (in developed countries): - households receiving social welfare benefits - single-parent households - low socioeconomic status - rented apartments According to DSM-IV Schizophrenia is diagnosed when the patient presents with a combination of positive (delusions and hallucinations) and negative symptoms, which have been present for at least 6 months and have resulted in significant dysfunction. It is also accepted that disorganized speech/behavior and/or catatonic symptoms, when combined with other positive or negative symptoms, can count toward a diagnosis of schizophrenia. Schizophrenia is a diagnosis of exclusion; in other words, it is required that there are no other medical, psychiatric, or substance-induced conditions that would explain the patient’s diagnosis better than schizophrenia. Early in the disease course, other etiologies of psychosis should be excluded. These include treatable conditions such as tertiary syphilis, vitamin deficiencies, brain tumor, drug and medication intoxication, chronic infection, and mood disorders. While neuroimaging studies (CT and MRI) do not show a single specific pattern with schizophrenia or related disorders and may be normal early in the course of the disease a screening CT is recommended for patients with a first episode of primary psychosis, especially for late or acute onsets. An affective psychosis (mania or depression with psychotic features) should be ruled out if affective symptoms preceded psychotic symptoms or are dominating the clinical picture at the time of presentation. A diagnosis of schizoaffective disorder is appropriate if historically the course has been dominated by affective symptoms and there are at least some episodes of "pure" psychosis i.e., independent of the affective background. Symptom duration will separate brief psychotic disorder (<1 month), schizophereniform disorder (<6 months), and schizophrenia (>6 months). Hospitalization is recommended if the acute psychotic symptoms result in danger to self or others or significant impairment. Traditionally, dopamine 2 (D2) antagonists (blockers), most often labelled as first generation (typical) neuroleptics, have been the pillar of schizophrenia treatment. - D2 blockers, by decreasing the presumably excessive mesolimbic dopamine, have established efficacy for positive psychotic symptoms; however, due to concomitant blockade of the frontostriatal dopamine pathway, where dopamine is presumably decreased all along in schizophrenia, they do not improve (and in some cases can worsen) negative, cognitive symptoms, and/or functional/social outcomes. - Due to an alteration of the physiological dopamine/acetylcholine ratio in the basal ganglia these drugs also have a number of extra-pyramidal adverse effects (EPSEs) both short term (acute dystonia, dyskinesia, akathisia) and long term (parkinsonism and tardive diskinesia). - Finally, following a dopamine blockade in the tuberoinfundibular system, there is a prolactin increase with common sexual side effects, including decreased sexual interest, sexual difficulties, lactation and (in men) gynaecomastia. - The side effects of typical neuroleptics can be stigmatizing and are a major reason for non-adherence to treatment. Some of the above issues have been resolved with the advent of the second generation antipsychotics (SGA) or atypical neuroleptics, a drug class that tends to share the mechanism of D2 and 5HT2 (serotonin) antagonism. We say "tends to share" rather than "share the characteristic" as the second generation drugs show a number of differences in terms of both receptor profile and affinities. To illustrate, the prototypical atypical neuroleptic is clozapine, a drug that has strong D4 and 5HT2A antagonism but only partial D2 antagonism. - SGAs have fewer EPSEs and tend to be better for negative symptoms than typicals (not increasing negative symptoms). - Some of the atypicals (e.g., olanzapine, clozapine) increase the risk for metabolic adverse effects including significant weight gain, diabetes and dyslipidemia. - Clozapine is recommended for treatment resistant schizophrenia. - Generally SGAs, with the exception of olanzapine and clozapine, are first line treatments. This preference is based more on better tolerability (less EPSEs and cognitive adverse effects) than greater efficacy. On a case by case basis first generation antipsychotics (FGAs) may represent a reasonable alternative. Perphenazine and molindone efficacy and overall tolerability has been shown to be similar to SGAs. General prescribing principles: - Initial management may include use of sedative medication such as lorazepam. - IM medication may be required in a very disturbed, involuntary patient. - Depot (long-acting) neuroleptics are indicated when treatment adherence is problematic. - Polypharmacy is common yet not supported by evidence. - The goal of treatment is stability on monotherapy at the lowest effective dose. Psychological (Individual and Family Interventions)Edit - Good evidence: - Education of patient and carers - Reduction of high expressed emotion: shown to affect relapse rates - Supportive, solution oriented psychotherapy - Unclear benefit: - Cognitive behavioral therapy - Cognitive and functional rehabilitation - Self–help unclear - Good evidence: - Regular intensive case management - Unclear benefit: - As needed case management - Consumer based organizations 15-25% of patients diagnosed with schizophrenia have one episode and no residual impairment. 25-40% have recurrent episodes and no residual impairment. 5-10% have recurrent episodes and develop significant non-progressive impairment. 30-40% have recurrent episodes and develop significant progressive impairment. Therefore, the majority of patients do not recover fully BUT DO NOT have a chronic unremitting course. There is little evidence that antipsychotics have altered the course of illness for most patients. However, evidence suggests that prolonged psychosis which is untreated has a bad prognosis. Suicide rate is up to 15%. Good prognostic factors: - Female gender - Older age of onset - Higher socioeconomic status - Living in a developing (as opposed to developed) country - Good premorbid personality - No previous psych history - Good education and employment record - Acute onset, affective symptoms, good adherence to medication. Predicting risk of suicide: - Acute exacerbation of psychosis - Depressive symptoms - History of attempted suicide - Male gender - Command auditory hallucinations Clinical Manifestations and Diagnosic ConsiderationsEdit The clinical presentation is identical to schizophrenia, however impairment in function is not a requirement. The required duration of symptoms is of at least a month but less than 6 months. If symptoms persist for longer than 6 months it is appropriate to change the diagnosis to schizophrenia. The diagnosis requires for other pathologies that may be responsible for the clinical manifestations (e.g., medical and drug use) to be ruled out before a diagnosis of schizophreniform disorder is made. It is not clear if schizophreniform disorder is a different disorder or just a more acute, better prognosis type of schizophrenia. With good prognostic features: - Good premorbid level of function - Abrupt onset - Absence of flat affect Without good prognostic features: when less than 2 of the above features are present The prevalence is low overall. There may be differences between developed countries (estimated around 0.2%) and developing countries (estimated around 1%). - Hospitalization is recommended if the acute psychotic symptoms result in danger to self or others or significant impairment. - Acute psychosis should be treated with antipsychotics. Second generation antipsychotics, with the exception of olanzapine, are preferred first line. - Treatment should be continued for one year and reassessed after. - Supportive and solution oriented psychotherapy is beneficial. About one third of the patients recover. The rest of the patients initially diagnosed with schizophreniform disorder progress to schizophrenia or schizoaffective disorder. Brief Psychotic DisorderEdit Clinical Manifestations and Diagnosic ConsiderationsEdit Phenomenologically there is no difference between brief psychotic disorder (BPD), schizophreniform disorder, and schizophrenia. The difference between these three diagnoses is based on symptom duration. As indicated by its name, the duration of symptoms in BPD are brief: more than 1 day but less than 1 month. When the symptoms last longer than a month but less than 6 months the diagnosis changes to schizophreniform disorder. The psychotic symptoms should not be part of a pre-existing medical, drug induced, or primary psychiatric condition (including other psychotic or mood disorders). DSM-IV-TR specifiers include: - With marked stressor(s) (brief reactive psychosis) - Without marked stressor(s) - With postpartum onset: when onset of symptoms is within 4 weeks postpartum Rare overall but more frequent in developing countries compared to developed countries. Hospitalization is recommended if the acute psychotic symptoms result in danger to self or others or significant impairment. Neuroleptics for short term treatment should be considered on a case by case basis. By definition full remission of symptoms and return to prior level of functioning is expected within a month. Clinical Manifestations and Diagnostic ConsiderationsEdit The patient presents with symptoms of schizophrenia, mania, depression or a combination of mood and psychotic symptoms. The history is significant for at least one distinct episode of psychosis not overlapping with mood symptoms and a relative temporal predominance of mood symptoms. Differential diagnoses should include drug induced and medical conditions with secondary psychotic symptoms. While patients with schizophrenia can experience mood symptoms their duration is relatively short relative to the total duration of illness. When the psychotic symptoms represent a culmination of a severe mood episode a diagnosis of mood disorders (i.e., bipolar and major depression) with psychotic features should also be included in the differential. Unclear but possibly less common than schizophrenia. Hospitalization is recommended if the acute psychotic symptoms result in danger to self or others or significant impairment. Antipsychotics are recommended for acute psychotic symptoms. Second generation antipsychotics (SGA), excluding olanzepine, should be considered as first line. Mood stabilizers including lithium, valproic acid, and carbamazepine, or SGA are recommended for acute manic symptoms. A neuroleptic-mood stabilizer combination may work better than either agent alone, and augmenting a neuroleptic with lithium or valproic acid should be considered as an augmentation strategy in cases of poor response to neuroleptic monotherapy. Antidepressants should be used conservatively for depressive symptoms. Close monitoring is required as an antidepressant can precipitate a manic switch in a patient with schizoaffective disorder. Better than schizophrenia but not as good as mood disorders. Clinical Manifestations and Diagnostic ConsiderationsEdit The patient presents with non-bizarre delusional beliefs but most often the mental status examination is otherwise fairly normal. The delusional ideas are restricted to a specific subject and do not contaminate other mental processes. Other psychotic symptoms may include olfactory/gustatory hallucinations, which may be prominent and are closely related to the main delusional themes. If prominent auditory/visual hallucinations are present a diagnosis of schizophrenia rather than delusional disorder may be more appropriate. Associated symptoms are rare but may include mood or anxiety symptoms. When present, such symptoms are often secondary to the delusional beliefs (e.g., "of course I feel anxious with the NSA following me around the clock"). Other conditions (medical, drug induced, other primary psychiatric disorders, including other psychotic or mood disorders) cannot better explain the clinical picture. - Erotomanic type: the patient erroneously believes that another person is in love with him/her - Grandiose type: the patient erroneously believes that he/she possesses enormous wealth, power, authority, knowledge, or has a special relationship to a deity or famous person - Jealous type: the patient erroneously believes that his/her partner is unfaithful - Persecutory type: the patient erroneously that he/she is targeted for punishment or retaliation - Somatic type: the patient erroneously believes that he/she has a medical condition or body deformity that is overlooked or misdiagnosed - Mixed type: delusions characteristic of more than one of the above types but without any one dominating theme - Unspecified type Rare. According to DSM-IV-TR estimated around 0.03% in the general population; 1-2% of all inpatient psychiatric admissions. The most common subtype is the persecutory type. Variable: the jealous type may wane and wax or remit; the persecutory type is often chronic. Clinical Manifestations and Diagnostic ConsiderationsEdit Mental status examination is significant for non-bizarre delusions but otherwise is within normal limits. There are minimal associated mood or anxiety symptoms; if present such symptoms appear secondary to the tenaciously held delusional beliefs. History is significant for a close relationship with another person who presents with similar delusional beliefs and meets criteria for a psychotic disorder. The patient who first presents with delusional symptoms is designated as the "primary," the "secondary" follows. Also, usually, the primary is dominant in his/her relationship with the secondary, who acts as a more passive recipient. For example, a parent with schizophrenia and chronic paranoid delusions about FBI surveillance may be the primary while his/her child, who only recently started to believe that indeed there are FBI cameras hidden on their property, is the secondary. Other diagnoses, including medical or drug induced disorders as well as other psychotic or mood disorders, should be excluded if folie à deux is to be diagnosed. Rare overall but statistics may be misleading due to under-reporting. Preliminary data suggest an increased prevalence in women. Hospitalization is recommended if the acute psychotic symptoms result in danger to self or others or significant impairment. Usually removing the secondary from the primary's environment is sufficient to promote complete remission of symptoms. In addition, the primary's condition should be treated as indicated. Interestingly, a remission of the primary's symptoms is followed by the remission of the secondary's delusional beliefs. When the secondary is separated from the primary the prognosis is good. - Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR. PsychiatryOnline.com Online ISBN 0-89042-334-2. Accessed 03/01/2011 - Kapur S.Psychosis as a state of aberrant salience: a framework linking biology, phenomenology, and pharmacology in schizophrenia.Am J Psychiatry. 2003 Jan;160(1):13-23 - Wicks S, Hjern A, Gunnell D, et. Social adversity in childhood and the risk of developing psychosis: a national cohort study. Am J Psychiatry. 2005 Sep;162(9):1652-7 - APA Practice Guidelines Guideline Watch (September 2009): Practice Guideline for the Treatment of Patients With Schizophrenia.PsychiatryOnline.com Online ISBN 0-89042-336-9. Accessed 03/01/2011	2	15	2	0	0	3	0.896984	5	9,675
Diagnosis Code B44.81 Information for Medical Professionals The diagnosis code B44.81 is grouped in the following Diagnostic Related Group(s) (MS-DRG V35.0) - 196 - INTERSTITIAL LUNG DISEASE WITH MCC - 197 - INTERSTITIAL LUNG DISEASE WITH CC - 198 - INTERSTITIAL LUNG DISEASE WITHOUT CC/MCC Convert to ICD-9 - 518.6 - Alrgc brncpul asprglosis - Allergic bronchopulmonary aspergillosis - Aspergillus bronchitis - Pulmonary aspergillosis - Tracheobronchitis due to Aspergillus Index to Diseases and Injuries References found for the code B44.81 in the Index to Diseases and Injuries: - - Aspergillosis (with pneumonia) - B44.9 Information for Patients Aspergillosis is a disease caused by a fungus (or mold) called Aspergillus. The fungus is very common in both indoors and outdoors. Most people breathe in the spores of the fungus every day without being affected. But some people get the disease. It usually occurs in people with lung diseases or weakened immune systems. There are different kinds of aspergillosis. One kind is allergic bronchopulmonary aspergillosis (also called ABPA). Symptoms of ABPA include wheezing and coughing. ABPA can affect healthy people but it is most common in people with asthma or cystic fibrosis. Another kind is invasive aspergillosis, which damages tissues in the body. It usually affects the lungs. Sometimes it can also cause infection in other organs and spread throughout the body. It affects people who have immune system problems, such as people who have had a transplant, are taking high doses of steroids, or getting chemotherapy for some cancers. Your doctor might do a variety of tests to make the diagnosis, including a chest x-ray, CT scan of the lungs, and an examination of tissues for signs of the fungus. Treatment is with antifungal drugs. If you have ABPA, you may also take steroids. Centers for Disease Control and Prevention - Aspergillosis (Medical Encyclopedia) - Aspergillosis precipitin (Medical Encyclopedia) - Pulmonary aspergilloma (Medical Encyclopedia) General Equivalence Map Definitions The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs. - Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent. - No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system. - Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system. Present on Admission The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.	4	3	0	0	0	2	0.68237	2	781
October 24, 2011 Coding for Diabetes Mellitus For The Record Vol. 23 No. 19 P. 27 In type 1 diabetes mellitus (DM), beta cells are destroyed by an autoimmune process that usually leads to a complete loss of insulin production. The majority of patients who develop type 1 DM will do so prior to age 25, with an increased prevalence due to heredity or in patients with other autoimmune diseases. Type 1 DM patients are dependent on insulin. In type 2 DM, the pancreas continues to produce insulin but doesn’t produce enough and doesn’t utilize it properly (insulin resistance). Secondary diabetes is diabetes or glucose intolerance that develops from disorders or conditions other than type 1 or type 2 diabetes or gestational diabetes. Secondary diabetes may bring out primary diabetes in people who are predisposed to developing primary diabetes. Common causes of secondary diabetes include but are not limited to pancreatitis, pancreatectomy; malnutrition, endocrinopathies, and drugs, chemical agents, and toxins. DM is assigned to ICD-9-CM category 250. Secondary diabetes is classified to category 249. When the physician documents DM, additional documentation is necessary to completely classify the condition: type 1 vs. type 2, uncontrolled vs. controlled, and manifestations associated with the condition, if any. The fourth-digit subcategory identifies any condition or manifestation associated with diabetes. The fifth-digit subclassification refers to type 1 or type 2 DM and whether it is controlled or uncontrolled. DM defaults to type 2 if not specifically documented, as this is the most common type. The fact that the patient receives insulin during the hospital stay has no effect on diabetes classification (AHA Coding Clinic for ICD-9-CM, 2005, first quarter, page 44). Uncontrolled diabetes is a nonspecific term indicating a patient’s glucose levels are not within acceptable levels in relation to the current treatment regime. There can be a variety of reasons for this, including noncompliance, insulin resistance, dietary indiscretion, and current illness. Uncontrolled diabetes should not be reported unless the physician’s documentation supports the diagnosis and should not be reported based on blood glucose levels. Uncontrolled diabetes should be coded only when the physician documents uncontrolled diabetes or out-of-control diabetes (AHA Coding Clinic for ICD-9-CM, 1993, fourth quarter, page 19). However, documentation of “poorly controlled” requires additional clarification from the physician to determine whether this is uncontrolled or controlled diabetes (AHA Coding Clinic for ICD-9-CM, 2002, second quarter, page 13). Hemoglobin A1c (HbA1c) levels measure overall blood glucose concentrations during the previous two to three months. When there is excess circulating glucose in the bloodstream, the glucose attaches itself to hemoglobin (glycosylation). This is measured in three subsets of hemoglobin (A1a, A1b, and A1c) as the percentage of red blood cells that are glycosylized. Since a red blood cell has a half-life of approximately 120 days, it is possible to measure average glucose concentrations (hence diabetic control) over a two- to three-month period. If the patient is admitted with a diabetic condition or has a condition due to diabetes, the diabetic code from category 250 must be sequenced as the principal diagnosis followed by the manifestations as secondary diagnoses. Conditions are coded in this manner even though the ICD-9-CM alphabetic index may not indicate dual coding (AHA Coding Clinic for ICD-9-CM, 1991, third quarter, page 8). In other words, the physician must state a cause-and-effect relationship between the manifestation and the diabetes before it can be coded as a diabetic condition. Two exceptions to this rule are gangrene and osteomyelitis. If a diabetic patient is admitted with gangrene or osteomyelitis with no other documented causes of those conditions, then it is automatically reported as a diabetic condition without a cause-and-effect relationship established by the physician (AHA Coding Clinic for ICD-9-CM, 2004, first quarter, pages 14-15). If a patient is admitted with uncontrolled diabetes and there are no other diabetic manifestations documented, then assign code 250.02 or 250.03. However, if the patient has a documented diabetic manifestation (eg, diabetic neuropathy) with the uncontrolled diabetes, assign the diabetic code related to the manifestation (eg, 250.62 or 250.63). This is the case even though the manifestation was not the reason for the admission. A patient may have diabetic complications in more than one body system. Report as many diabetic manifestations as are needed to fully describe the patient’s condition. Sequence the diabetic condition that necessitated the admission as the principal diagnosis. If treatment was directed toward all conditions equally, any diabetic condition may be sequenced as the principal diagnosis (AHA Coding Clinic for ICD-9-CM, 2005, first quarter, page 45). Coding and sequencing for DM are dependent on the physician documentation in the medical record and application of the Official Coding Guidelines for inpatient care. Also, use specific AHA Coding Clinic for ICD-9-CM and American Medical Association CPT Assistant references to ensure complete and accurate coding. — This information was prepared by Audrey Howard, RHIA, of 3M Consulting Services. 3M Consulting Services is a business of 3M Health Information Systems, a supplier of coding and classification systems to more than 5,000 healthcare providers. The company and its representatives do not assume any responsibility for reimbursement decisions or claims denials made by providers or payers as the result of the misuse of this coding information. More information about 3M Health Information Systems is available at www.3mhis.com or by calling 800-367-2447. ICD-10-CM Coding for Diabetes Mellitus There are five categories for diabetes mellitus (DM) in ICD-10-CM: • Diabetes mellitus due to underlying condition (E08); • Drug- or chemical-induced diabetes mellitus (E09); • Type 1 diabetes mellitus (E10); • Type 2 diabetes mellitus (E11); and • Other specified diabetes mellitus (E13). If DM is due to an underlying condition, drug induced, or chemical induced, then that condition is sequenced first. In ICD-10-CM, DM is indicated in combination with the type of diabetes, the body system affected, and the complications affecting the body system rather than requiring two or more codes as in ICD-9-CM. For example, type 2 DM with mild nonproliferative diabetic retinopathy with macular edema is completely classified with one ICD-10-CM code (E11.321). ICD-10-CM does not classify DM by control status. However, inadequately controlled, out-of-control, or poorly controlled diabetes are coded to diabetes, by type, with hyperglycemia. — Audrey Howard	4	8	0	0	0	4	0.909054	4	1,474
Coding Tip: Coding Changes for Pulmonary Hypertension in FY2018 This Coding Tip was updated on 12/4/2018 Kim Carrier RHIT, CDIP, CCS, CCS-P Director of Coding Quality Assurance AHIMA Approved ICD-10-CM/PCS Trainer For FY2018, coding for pulmonary hypertension has changed. There are new codes and expanded codes for the Subcategory I27.2. What is Pulmonary Hypertension? Pulmonary hypertension is a certain type of high blood pressure that increases the pressure in the pulmonary arteries/lungs and the right side of the heart. Some of the forms of pulmonary hypertension can be serious and over time even fatal. Some forms of pulmonary hypertension are not curable but there are medications that can help lessen the symptoms and improve quality of life. Pulmonary hypertension is different than systemic high blood pressure/hypertension. Pulmonary blood pressure reflects the pressure the heart exerts to pump blood from the heart through the lung arteries. So, this is pressure that is focused on the blood flow in the lungs. Secondary PH is always caused by something else or due to another condition. Symptoms of Pulmonary Hypertension Early stages of pulmonary hypertension may have minimal or no symptoms. As the disease progresses, the symptoms become worse. Common symptoms of Pulmonary Hypertension - Dyspnea/Shortness of breath - Dizziness/syncopal episodes/feeling of being lightheaded - Chest pain or pressure - Swelling of ankles, legs and eventually the abdomen - Cyanosis/bluish color to lips and/or skin - Palpitation of the heart or racing pulse - Decreased appetite - Upper right side abdominal pain Groups of Pulmonary Hypertension Pulmonary hypertension is classified into five groups, depending on the cause of the disease. Group 1: Pulmonary arterial hypertension: This is the most recognized category of pulmonary hypertension. This type includes both primary and secondary causes. This can be idiopathic (cause unknown) or due to a variety of other conditions/factors. This group can be inherited, drug or toxin induced, caused by connective tissue disease, HIV, liver disease, sickle cell disease, congenital heart disease or by conditions that affect the veins and small blood vessels of the lungs to name a few. There are two ICD-10-CM codes to report for this type of pulmonary hypertension. I27.0 (primary pulmonary hypertension) and I27.21 (secondary pulmonary arterial hypertension). Group 2: Pulmonary hypertension due to left heart disease: This category of pulmonary hypertension is caused by failure of the left ventricle or left sided valvular heart disease (mitral and/or aortic valve disease). The most common cause of pulmonary hypertension is left heart disease. ICD-10-CM code I27.22 (pulmonary hypertension due to left heart disease) is reported for this type. Group 3: Pulmonary hypertension occurring secondary to lung disease and/or hypoxia: This category of pulmonary hypertension is caused by COPD/emphysema, pulmonary fibrosis, sleep apnea or other sleep disorders and long term exposure to high altitudes. ICD-10-CM code I27.23 (pulmonary hypertension due to lung disease and hypoxia) is reported for this type. Group 4: Chronic thromboembolic hypertension: This category is caused by clotting disorders or blood clots in the lung also called pulmonary emboli. ICD-10-CM code I27.24 (chronic thromboembolic pulmonary hypertension) is reported for this type. Group 5: Other secondary pulmonary hypertension: This would include causes such as polycythemia vera, essential thrombocytopenia, sarcoidosis, vasculitis, thyroid or glycogen storage disease, kidney disease, anything that presses on the pulmonary artery (like a tumor) or multifactorial. ICD-10-CM code I27.29 (other secondary pulmonary hypertension) is reported for this type. Unspecified pulmonary hypertension is reported with ICD-9-CM code I27.20. Sequencing is based on the circumstances of admission or reason for the encounter. When reporting a code from subcategory I27.2, be sure and also report the associated conditions or the adverse effect of the drug/toxin. As stated above, there is no cure for the disease pulmonary hypertension. But medication/treatment can lessen the symptoms and improve quality of life. Here are a few life style changes that can improve the symptoms: - Discuss with your MD before taking over the counter medications - Quit smoking or don’t start to smoke - Follow a healthy diet-low in fat, cholesterol, sodium and sugar - Record your weight. If there is a rapid weight gain it may be a sign of worsening - Stay active - Avoid the hot tub/sauna AHA Coding Clinic, Fourth Quarter 2017 Pages: 14-15 The information contained in this coding advice is valid at the time of posting. Viewers are encouraged to research subsequent official guidance in the areas associated with the topic as they can change rapidly. Last week, we looked at tidbits for reporting the ICD-10-CM codes for pregnancy/obstetric records. Now we will look at some for the ICD-10-PCS reporting of these records. In reporting the appropriate ICD-10-PCS codes a coder must know what is included in the terminology of products of conception (POC). Chances are, we all know someone affected by heart disease and stroke, because about 2,300 Americans die of cardiovascular disease each day, an average of 1 death every 38 seconds. But together we can change that. There was a time when coding delivery records was considered simple. Many times, these types of records were given to the newer coders. However, as coding becomes more complex, this is no longer the case. With the implementation of ICD-10-CM came more codes for very detailed and specific issues that occur during pregnancy, childbirth and the puerperium. When it comes to coding and documentation, finding your own rhythm can lead to positive results. For our series, Find Your Routine, we interviewed our most productive coders and asked them what steps they take to find a rhythm that works for them. This week, we talked with Allison Curry, RHIT, CCS, Coding Specialist at Health Information Associates, about the steps she takes to find her routine. One way to shorten a lengthy query is by avoiding repetition in the supporting documentation. Does the same diagnosis really need to be mentioned multiple times in the clinical indicators? Is it necessary to list the results of a chest x-ray twice? Does listing the same documentation multiple times give further specification or explanation to the query? Tobacco use can lead to tobacco/nicotine dependence and serious health problems. Quitting smoking greatly reduces the risk of developing smoking-related diseases. Tobacco/nicotine dependence is a condition that often requires repeated treatments, but there are helpful treatments and resources for quitting. This is Part 5 of a five part series on the new 2019 CPT codes. For the remaining areas we will just briefly summarize the section. Due to the intricate nature of these sections in CPT, it is recommended that the coder read the entire section notes associated with the new codes. This is Part 4 of a five part series on the new 2019 CPT codes. In this series we will explore the CPT changes for FY 2019 and include examples to help the coder understand the new codes. There is 1 new lymphatic code, 2 new digestive system codes with 3 deletions, 3 new urinary system codes with one deletion and 7 deleted nervous system codes with 2 revisions. This is Part 3 of a five part series on the new 2019 CPT codes. In this series we will explore the CPT changes for FY2019 and include examples to help the coder understand the new codes. There are 9 new cardiovascular CPT codes added with 2 deletions and 3 revisions. When it comes to coding and documentation, finding your own rhythm can lead to positive results. For our series, Find Your Routine, we interviewed our most productive coders and asked them what steps they take to find a rhythm that works for them. This week, we talked with Tilina Sablan, RHIT, CCS, Coding Specialist with Health Information Associates, about the steps she takes to find her routine. This is Part 2 of a five part series on the new 2019 CPT codes. In this series we will explore the CPT changes for FY2019 and include examples to help the coder understand the new codes. There are 4 new musculoskeletal CPT codes added with 2 deletions and 0 revisions. This is Part 1 of a five part series on the new 2019 CPT codes. In this series we will explore the CPT changes for FY2019 and include examples to help the coder understand the new codes. There were 15 new integumentary CPT codes added with 3 deletions and 1 revision. In part 5 of our series, we look at DRG 64—Intracranial hemorrhage or cerebral infarction with MCC. For this DRG recommendation the majority (almost all) were recommended to DRG 65 (Intracranial hemorrhage or cerebral infarction with CC) with deletion of the reported MCC. The majority of the recommendations from DRG 190 (Chronic obstructive pulmonary disease w/MCC) was to DRG 189 (Pulmonary edema and respiratory failure) with re-sequencing of respiratory failure as the PDX or adding as a new code and sequenced as PDX. The majority of the recommendations from DRG 853 (Infectious & parasitic disease with O.R. procedure with MCC) were to DRG 871 (Septicemia w/o MV 96+ hours with MCC) with deletion or revision of the PCS code. Some of these required physician query. The majority of the recommendations from DRG 872 (Septicemia w/o mechanical ventilation 96+ hours w/o MCC) were to DRG 871 (Septicemia w/o mechanical ventilation 96+ hours with MCC) with the addition of an MCC to the account. Not all of these required a physician query and were present in the medical record documentation without any clarification needed prior to coding. The majority of the recommendations from DRG 871 (Septicemia w/o MV 96+ hours with MCC) were to DRG 872 (Septicemia w/o MV 96+ hours w/o MCC) with the recommendation to delete the reported MCC or query for clarification to support the MCC that had been reported. Every year, we make plans to live a healthier, more organized, and balanced life. For some of us, we end up falling short of those expectations. This year, to keep us on track with our New Year’s goals, we have put together a few of the most common New Year’s resolutions along with their ICD-10 diagnoses codes. Check out our tips and tricks for a healthy 2019! Top 5 ProFee diagnosis changes found in recent HIA reviews: 1. I10 – Essential (Primary) Hypertension; 2. E11.9 – Type 2 Diabetes Mellitus Without Complications; 3. K29.60 – Other Gastritis Without Bleeding; 4. R13.19 – Other Dysphagia; 5. I25.10 – Atherosclerotic Heart Disease of Native Coronary Artery Without Angina Pectoris. What is the principal procedure? The procedure that is performed for definitive treatment or is taking care of a complication is the principal procedure. Procedures for diagnostic or exploratory purposes that are performed in addition to a procedure being performed for definitive treatment, would be reported in addition to the principal procedure. A query question that is directive in nature, indicating what the provider should document, rather than asking for his/her professional determination of clinical facts, constitutes a leading query. The provider should not be made to feel obligated to document anything. When I start coding a chart, I enter all account information in log and do any abstracting—disposition, admitting, and attending—take care of all of that first. ED, H&P, consult, progress reports, and discharge summary. Some Speed Reading Tips: Once you start reading, don’t stop! Read the text straight through. If you have any question after you have completed reading the material, go back and reread the relevant sections. Reread the marked sections of the text (the items you indicated that you didn’t quite understand). Write a small summary at the beginning of the chapter – consisting about 3-4 sentences. In HIA quality reviews we are finding that some coders are reporting Z41.2—Encounter for routine and ritual circumcision, during the male newborn birth admission, when circumcision is performed prior to discharge. The cause/etiology of GI bleeding is not always easily determined. During procedures, to work the bleeding up, there are often multiple potential sources of bleeding found but not identified as the culprit. Many of these findings have “with” or “in” in the main or subterms. On December 1, 2018, the HIA team based at our headquarters in Pawleys Island, South Carolina received a visit from a surprise guest – meet Otis, HIA’s very own Elf on a Shelf. Otis will be sticking around until Christmas to keep an eye on all of us. We have a feeling he may get into some trouble! Check back daily to see what Otis is up to. #OtisOnOtisDrive When it comes to coding and documentation, finding your own rhythm can lead to positive results. For our new series, Find Your Routine, we interviewed our most productive coders and asked them what steps they take to find a rhythm that works for them. This week, we talked with Crystal Junkins, CCS, CPC, Coding Specialist with Health Information Associates, about the steps she takes to find her routine. COPD is a respiratory condition where there is chronic obstruction to airflow in the lungs. Air is breathed into the lungs but a patient with COPD has trouble emptying air out of the lungs. This can also cause patients with COPD to have CO2 retention. COPD is an irreversible and progressive disease in which the lung function worsens as time goes on. Tissue findings interpreted by a pathologist are not equivalent to the attending physician’s medical diagnosis based on the patient’s clinical condition. If the attending physician has not indicated the significance of an abnormal finding within a pathology report…	4	15	0	12	0	21	0.947074	33	3,117
Congestive heart failure (CHF) \|Integrative Therapy Quick Links:\| - Amyloidosis, anemia, angioplasty, aortic valve, aquapheresis, arrhythmias, arteriosclerosis, atherosclerosis, BNP, B-type natriuretic peptide, CABG, CAD, calcium, cardiac catheterization, cardiomyopathy, cholesterol, coronary artery bypass graft, coronary artery disease, C-reactive protein, CRP, ECG, echocardiogram, edema, EKG, electrocardiogram, electrolyte, emphysema, fatigue, heart attack, heart failure, hemochromatosis, homocysteine, hypertension, hyperthyroidism, hypothyroidism, IABP, ICD, idiopathic, implantable cardiac defibrillator, intra-aortic balloon pump, Keshan disease, left atrium, left ventricle, left ventricular assist device, left ventricular hypertrophy, LVAD, murmer, myocardial infarction, myocarditis, nuclear stress test, pacemaker, pneumonia, post-pericardiotomy syndrome, potassium, pulmonary edema, pulmonary valve, radionuclide ventriculography, right atrium, right ventricle, sleep apnea, sodium, stethoscope, tress test, tricuspid valve, valve, x-ray. - Congestive heart failure (CHF), or heart failure, is a condition in which the heart is unable to adequately pump blood throughout the body and/or unable to prevent blood from accumulating, or "backing up," into the lungs. - In most cases, CHF is a process that occurs over time, when an underlying condition damages the heart or makes it work too hard, weakening the organ. Health conditions that may lead to CHF include: coronary artery disease, hypertension (high blood pressure), or arrhythmias. - Symptoms of heart failure include shortness of breath and abnormal fluid retention, which usually results in swelling in the feet and legs. - According to the American Heart Association (AHA), nearly five million individuals experience heart failure and about 550,000 new cases are diagnosed each year in the United States. Heart failure becomes more prevalent with age and the number of cases is expected to grow as the overall age of the population increases. Signs and symptoms - Congestive heart failure (CHF) is chronic (long-term) and generally occurs slowly. Congestion, or the backing up of blood, occurs in the liver, abdomen, lower extremities, and lungs. The backing up of blood causes symptoms such as shortness of breath, fatigue, and swelling (particularly in the legs and feet). Other symptoms develop as the body tries to compensate for the heart's reduced pumping ability. The heart beats faster, its muscle thickens, and the ventricles may stretch to accommodate more blood. Damage to the ventricles may cause them to pump out of sync, further reducing the efficient delivery of blood to the body. Symptoms of CHF include: a dry, hacking cough, especially when lying down; confusion, sleepiness, and disorientation may occur in older individuals; dizziness, fainting, fatigue, or weakness; fluid buildup, especially in the legs, ankles, and feet; increased urination at night; nausea; abdominal swelling, tenderness, or pain; weight gain due to fluid buildup; weight loss as nausea causes a loss of appetite and as the body fails to absorb food well; rapid breathing, bluish skin, and feelings of restlessness, anxiety, and suffocation; shortness of breath and lung congestion as the blood backs up in the lungs; and wheezing and spasms of the airways similar to asthma. - Physical examination and medical history: During a physical examination, a doctor will look for underlying causes of the problem and assess heart function. A stethoscope is used to detect murmurs (abnormal heart sounds) that may indicate a leaky or narrowed valve, and to detect fluid accumulation in the lungs. The doctor also looks for enlarged veins in the neck and for edema (swelling) in the legs, particularly the ankles, feet, and/or the abdomen. - A patient history may include information about risk factors, such as family medical history, past surgeries and medications, history of chest pain, high blood pressure (including treatments), heart attack, recent viral illness, or recent pregnancy. - Blood tests: Blood tests may include: blood cell counts to test for conditions such as anemia (low red blood cells); electrolyte levels, including sodium, potassium, and calcium; nutrient levels, such as vitamins and trace minerals; tests for kidney function, including blood urea nitrogen (BUN) and creatinine levels; and testing for homocysteine and/or C-reactive protein (CRP), both markers of inflammation and heart disease. The diagnostic test marker for CHF is N-terminal prohormone brain natriuretic peptide or NT-proBNP. Brain natriuretic peptide (BNP) is a hormone produced at higher levels by the failing heart muscle. - Electrocardiogram: An electrocardiogram (ECG or EKG) is a noninvasive test used to measure electrical activity in the heart. Electrical sensors called leads are attached to predetermined positions on the arms, legs, and chest to record electrical activity and help assess heart function. The heart's rhythm of contraction is controlled by the sinoatrial node (SA node), often called the pacemaker. Electrical impulses generated from the SA node spread through the heart via a nodal tissue pathway that coordinates the events leading to heart beat. The conduction system initiates and coordinates the muscular activity of the heart. - Echocardiogram: Echocardiogram, or echo, is an ultrasound examination of the heart that produces detailed images of the organ. It can be used to detect abnormalities in the structure of the heart and to measure the amount of blood ejected from the heart. During an echocardiogram, a microphone-like device (transducer) is used to transmit and receive ultrasonic waves that travel through the chest wall to the heart and are reflected back to the transducer. The reflected sound waves are translated into images of the heart, including the valves, chambers, and walls. - Echocardiogram also is used to measure the pressure change (gradient) between the left ventricle and the aorta, to assess thickening of the walls of the heart, to evaluate pumping function, and to measure the amount of dilation (increased diameter) of the left ventricle. - Chest x-ray: A chest x-ray is used in identifying the buildup of fluid in the lungs. Also, the heart usually enlarges in CHF, which may be visible on the x-ray film. - Cardiac catheterization: Cardiac catheterization may be performed in individuals with angina and in those with a history of heart attack to determine if coronary heart disease (CHD) is causing heart failure. Cardiac catheterization produces angiograms (such as x-ray images) of the coronary arteries and the left ventricle, and can be used to monitor heart function. - Cardiac catheterization involves injecting a small amount of radioactive dye, called a contrast agent, into the left ventricle through a catheter (a thin flexible tube). A special camera is then used to determine how much of the dye is ejected from the heart with each beat. The infusion of dye typically produces a characteristic "hot flash" sensation throughout the body that lasts 10-15 seconds. - Stress test: In some individuals, a less invasive procedure called a stress test is used to assess the possibility of coronary heart disease. If the results of this procedure suggest the presence of coronary artery disease, a subsequent referral for cardiac catheterization is likely. - Several types of stress tests may be used by doctors to access heart function. In some cases, the individual simply walks on a treadmill while connected to an electrocardiogram (ECG). Another type uses intravenous (IV, or in the veins) medication, usually dipyridamole (Persantine®), which reproduces the stress of exercise on the heart. - Nuclear stress test: Nuclear stress tests involve injecting a radioactive substance, most commonly technetium or Tc-99m sestamibi (Cardiolite®), into a vein. A special camera is then used to obtain images of the heart during rest and immediately following exercise on a treadmill as the radioactivity flows through the heart. The radioactivity levels used are not harmful. - A nuclear test called a radionuclide ventriculography or multiple gated acquisition (MUGA) scanning allows doctors to see how much blood the heart pumps with each beat, called the ejection fraction. The MUGA scan gives an accurate and reproducible means of measuring and monitoring the actual amount of blood ejected from the heart. The tests use a small amount of radioactive material injected into the veins. A special camera detects the radioactive material as it flows through the heart. Individuals with an allergy to iodine or shellfish have special considerations and may not be able to have this test because the dye contains iodine. The use of medications, including the antihistamine diphenhydramine (Benadryl®) and/or prednisone (Delatasone®), prior to the administration of the dyes (contrast media), may help to prevent or decrease the chance of an allergic reaction. - Classifying heart failure: Results of these tests help doctors determine the cause of CHF and develop a program to treat the heart. To determine the best course of treatment, doctors may classify heart failure using one of two scales. The New York Heart Association scale classifies heart failure in categories from one to four. In Class I heart failure, the mildest form, individuals can perform everyday activities and not feel winded or fatigued. Individuals with class II have slight limitation of physical activity and ordinary physical activity may result in fatigue, palpitation, shortness of breath, or chest pain. Those with class III have marked limitation of physical activity and less than ordinary activity causes fatigue, palpitation, shortness of breath, or chest pain. Class IV is the most severe, and individuals are short of breath even at rest. The American College of Cardiology scale uses letters A-D. The system includes a category for individuals who are at risk of developing heart failure. Early stage heart failure includes stage A (individuals are at risk for developing heart failure without evidence of heart dysfunction) and stage B (there is evidence of heart dysfunction without symptoms). Advanced stage heart failure includes stage C (there is evidence of heart dysfunction with symptoms) and stage D (there are symptoms of heart failure despite maximal therapy). Doctors can use these classifications to identify the risk factors and begin early, more aggressive treatment to help prevent or delay heart failure. - Congestive heart failure can affect many organs of the body. For example, the weakened heart muscles may not be able to supply enough blood to the kidneys, which then begin to lose their normal ability to excrete salt (sodium) and water. This diminished kidney function can cause the body to retain more fluid. The lungs may become congested with fluid (pulmonary edema) and the person's ability to exercise is decreased. Fluid may likewise accumulate in the liver, thereby impairing its ability to rid the body of toxins and produce essential proteins. The intestines may become less efficient in absorbing nutrients and medicines. Over time, untreated congestive heart failure will affect virtually every organ in the body. The lack of blood flow to the heart can lead to irreversible damage to the heart muscle. - Chest pain (angina) is an indicator of a heart attack (myocardial infarction). A heart attack can cause sudden death. - Treatment for congestive heart failure (CHF) varies and involves reducing symptoms, treating the underlying cause of the condition when possible, and using medications to prevent further deterioration of heart function. - Lifestyle changes: - Lifestyle changes can help reduce symptoms such as fatigue, shortness of breath, and edema (swelling). These modifications may include dietary changes (such as a restricted salt intake of less than 2,000 mg daily), abstaining from alcohol, stopping smoking, and exercising regularly (under the supervision of a doctor). - A combination of medications is used to treat CHF. Depending on the symptoms, individuals with CHF may take one, two, or more of these drugs. Several types of medications have proved useful in the treatment of heart failure including: angiotensin-converting enzyme (ACE) inhibitors, angiotensin receptor blockers (ARBS), beta-blockers, digoxin, diuretics, and aldosterone antagonists. - Angiotensin-converting enzyme (ACE) inhibitors: Angiotensin-converting enzyme (ACE) inhibitors are a medication that dilates or widens blood vessels to lower blood pressure, improve blood flow, and decrease the workload on the heart. ACE inhibitors include enalapril (Vasotec®), lisinopril (Prinivil®, Zestril®), and captopril (Capoten®). - Side effects of ACE inhibitors include chronic, nonproductive cough (occurs in about 10% of patients), dizziness or weakness (caused by low blood pressure), increased potassium levels, skin rashes, and sudden swelling of the lips, face, and cheeks (if this occurs, the patient must seek medical attention immediately). - Angiotensin II (A-II) receptor blockers (ARBs): Angiotensin receptor blockers, or ARBS, have many of the beneficial effects of ACE inhibitors, but they do not cause a persistent cough. They may be an alternative for individuals who cannot tolerate ACE inhibitors. ARBs include losartan (Cozaar®) and valsartan (Diovan®). - Digoxin (Lanoxin®): Digoxin (Lanoxin®) increases the strength of the heart muscle contractions. Digoxin also tends to slow the heartbeat. Digoxin reduces heart failure symptoms and improves the individual's ability to live with the CHF. Side effects are rare, but may include blurred vision, cardiac problems (such as arrhythmias or heart block), diarrhea, headaches, loss of appetite, hypotension (low blood pressure), and nausea and vomiting. - Beta blockers: Beta blockers are a class of drugs that slows the heart rate and reduces blood pressure. Beta blockers include carvedilol (Coreg®), metoprolol (Lopressor®), and propranolol (Inderal®). These medicines also reduce the risk of some abnormal heart rhythms. Beta blockers may reduce signs and symptoms of heart failure and improve heart function. Beta-blockers are started at low doses that are gradually increased over a period of several months. During the first several weeks of treatment, some patients experience worsening symptoms due to a decrease in oxygen circulation in the body. Other side effects include low blood pressure, difficulty breathing, sexual dysfunction, nausea, and weakness with exertion. - Diuretics: Diuretics, or water pills, make individuals urinate more frequently and keep fluid from collecting in the body. Commonly prescribed diuretics for heart failure include hydrochlorothiazide (Diuril®) and furosemide (Lasix®). Diuretics also decrease fluid in the lungs, helping individuals breathe more easily. Side effects include frequent urination and low potassium blood levels. Because of this, blood tests are performed periodically and a potassium supplement is prescribed if blood levels are low. Individuals may be asked to eat more fruits high in potassium, such as bananas and oranges, while on diuretic therapy. - Aldosterone antagonists: Aldosterone antagonists are primarily potassium-sparing diuretics, but they have additional properties that help the heart work better, may reverse scarring of the heart, and may help individuals with severe heart failure live longer. Aldosterone antagonists include spironolactone (Aldactone®) and eplerenone (Inspra®). Unlike other diuretics, spironolactone can raise the level of potassium in the blood to dangerous levels. Healthcare professionals recommend eliminating high-potassium foods, such as bananas, lentils, nuts, peaches, potatoes, salmon, tomatoes, and watermelon while taking aldosterone antagonists. - Others: A medication called BiDil® is a single pill that combines hydralazine and isosorbide dinitrate, both of which dilate and relax the blood vessels. BiDil® increases survival when added to standard therapy in African American individuals with advanced heart failure. This is the first drug studied and approved for a specific racial group. Further studies will be necessary to determine if this combination medicine will be helpful for others with heart failure. Side effects may include blurred vision, dry mouth, irregular heartbeat, blood in the urine or stools, numbness or tingling in the arms or legs, and fainting. - Doctors often prescribe other heart medications, such as HMG-CoA reductase inhibitors (statin) drugs, for cholesterol reduction. Statin drugs include atorvastatin (Lipitor®) or lovastatin (Mevacor®). They may cause liver problems or muscle pain. Anti-arrhythmic drugs may also be prescribed to control irregular heart beats, including diltiazem (Cardizem®, Cardizem CR®) and verapamil (Calan®, Calan SR®). - Individuals may be hospitalized for a few days if complications arise as a result of CHF symptoms. While in the hospital, individuals may receive additional medications such as intravenous (IV, or into the veins) dobutamine (Dobutrex®), milrinone, (Primacor®), and nitroglycerin. These drugs work quickly to help the heart pump better and relieve symptoms. Individuals may also receive supplemental oxygen through a mask or small tubes placed in the nose. If severe heart failure is present, the individual may need to use supplemental oxygen long term. - Individuals hospitalized with severe CHF may be given an intravenous drug called nesiritide (Natrecor®). Nesiritide is a synthetic version of a naturally occurring hormone in the body called brain natriuretic peptide (BNP). BNP is secreted in high levels by the heart in response to a failing heart. However, it is not clear if nesiritide is better than other intravenous medications for severe heart failure. Studies are ongoing to evaluate the safety and effectiveness of nesiritide in heart failure. - Other Treatments: - Aquapheresis: In some cases, heart failure persists or worsens in spite of treatment. An ultrafiltration process called aquapheresis, which uses a mechanical system called the Aquadex FlexFlowT, may be used to remove excess fluids and salt in CHF individuals who do not respond to lifestyle modifications and medication. In this treatment, blood is withdrawn using catheters (small tubes) inserted into veins in the arm, leg, or neck. The blood is then passed through a filter that removes excess fluid and is returned to the body. Studies have reported that ultrafiltration can remove more fluid at a faster rate than medication. The length of each treatment depends on the rate at which fluid can be removed from the body and the amount that must be removed. - Angioplasty: CHF caused by reduced blood flow in the heart as a result of blockages (plaques) in one or more coronary arteries may be treated using coronary angioplasty. In this procedure, a hollow tube (catheter) is inserted through an artery (usually the femoral artery in the groin), into the coronary artery, and to the blockage. A small balloon is then inserted through the catheter and is inflated to open the blocked artery. There is a slight risk for damage to the artery during angioplasty, but heart failure symptoms usually improve following the procedure. Stenting is used along with balloon angioplasty. Stenting involves placing a mesh-like metal device into an artery at a site narrowed by plaque. The stent is mounted on a balloon-tipped catheter, threaded through an artery, and positioned at the blockage. The balloon is then inflated, opening the stent. Then, the catheter and deflated balloon are removed, leaving the stent in place. The opened stent keeps the vessel open and stops the artery from collapsing. Re-closure may occur with both balloon angioplasty and stenting. Doctors will prescribe blood thinning medications to help keep the arteries open, including aspirin, warfarin (Coumadin®), and clopidogrel (Plavix®). - Coronary artery bypass graft surgery (CABG): A coronary artery bypass surgery (CABG) may be recommended if the individual has severe coronary artery disease in addition to CHF. This may improve the blood supply to the heart. Coronary artery bypass graft (CABG) surgery uses blood vessel grafts, which usually come from the patient's own arteries and veins located in the chest, leg, or arm. The graft goes around the clogged artery to create new pathways for oxygen-rich blood to flow to the heart. Some problems associated with CABG include a heart attack (occurs in 5% of patients), stroke (occurs in 5%, with the risk greatest in those over 70 years old), blood clots, death (occurs in 1 - 2% of individuals), and wound infection (occurs in 1 - 4%). Infection is most often associated with obesity, diabetes, or having had a previous CABG. In about 30% of patients, post-pericardiotomy syndrome can occur anywhere from a few days to six months after surgery. The symptoms of this syndrome are fever and chest pain. Symptoms can be treated with medications, including antibiotics (for infection), nitroglycerin, and anti-inflammatory drugs. The incision in the chest or the graft site (if the graft was from the leg or arm) can be itchy, sore, numb, or bruised. Some individuals report memory loss, loss of mental clarity, or "fuzzy thinking" following a CABG. - Implantable cardiac defibrillator: An implantable cardiac defibrillator (ICD) may be used to treat severe heart failure. An ICD is a small electronic device that is surgically implanted under the skin in the chest to monitor heart rhythm. When an abnormal rhythm is detected, the defibrillator delivers an electrical "shock" to the heart to restore normal heart rhythm. - Intra-aortic balloon pump: An intra-aortic balloon pump (IABP) is a device that is inserted through an artery in the groin (femoral artery) and then placed within the main artery (aorta). An IABP is an inflatable balloon that expands and deflates in coordination with each heartbeat. It can be left in place for days to weeks, and decreases the strain on the heart and increases blood flow throughout the body. - Valve replacement surgery: Individuals with heart failure caused by an abnormal heart valve may require valve repair or valve replacement surgery. These are open-heart procedures in which an abnormal valve is repaired or replaced with a porcine valve (from pig tissue), a mechanical valve (made of synthetic material), or a homograft valve (from a human donor). Complications include bleeding, blood clots, infection, kidney failure, stroke, heart attack, and death. A homograft valve is preferred, as these valves are not associated with a significant risk for blood clot formation and, thus, do not require blood thinner therapy. Most individuals remain in the hospital for a week after surgery and recovery takes approximately three to four weeks, after which most patients can resume leisure activities and many return to work. Approximately 60% of individuals who have valve replacement have a ten-year post-surgery survival rate. - Left ventricular assist device: A left ventricular assist device (LVAD) is a mechanical pump that is surgically implanted in the upper abdomen to bypass the left ventricle and pump blood throughout the body. This device may be used in patients with end-stage heart failure who are awaiting heart transplantation. Long-term use of the device in patients with severe heart failure is being explored and defined. - Pacemaker: If individuals with CHF experience arrhythmias that will not respond to medication therapy, the arrhythmias may be corrected with a pacemaker. A pacemaker is a small, battery-powered device that is usually implanted near the collarbone. Pacemakers can be surgically placed into the chest (a permanent pacemaker) through a small incision, or they can be worn outside the body (a temporary pacemaker) and attached to the heart through a wire that is threaded through a neck vein. Temporary pacemakers are used only while an individual is in the hospital. - The surgery needed to implant a permanent pacemaker is considered a minor surgical procedure. The procedure may take one to two hours to complete. The area where the pacemaker will be inserted will be numbed with an injection of an anesthetic such as lidocaine (Xylocaine®). The individual should not feel any pain during the procedure, and should inform the doctor or staff if they are having pain so that more anesthetic medication may be given. One or more electrode-tipped wires run from the pacemaker through the blood vessels to the inner heart. If the heart rate is too slow or if it stops, the pacemaker sends out electrical impulses that stimulate the heart to beat at a steady, proper rate. The more advanced pacemakers can monitor and pace either the atria or ventricles (or both) in proper sequence to maximize the amount of blood being pumped from the heart. The pacemaker's batteries may need to be changed every five to ten years. It is recommended by the American Heart Association to limit exposure to devices that may interfere with pulse generators such as cellular phones, CB radios, electric blankets, and microwaves. - It is normal for the surgical wound to be somewhat painful and swollen for a few days after the procedure. This can usually be controlled with medications, such as ultram (Tramadol®) or ibuprofen (Motrin®). The wound may also appear mildly red for a few days; however, if the area of redness enlarges, a doctor should be notified due to the potential for a serious infection. If there are no other problems, most individuals who have a permanent pacemaker surgically implanted can go home the next day. They can usually return to normal activities within six weeks. For several weeks after having a pacemaker implanted, the individual may be asked not to lift more than five pounds or raise the affected arm over their shoulder. - Heart transplant: In some cases, despite the use of optimal therapies as described above, the individual's condition continues to deteriorate due to progressive CHF. In selected individuals, heart transplantation is a viable treatment option. Candidates for a heart transplant are generally under age 70, do not smoke, and do not have severe or irreversible diseases affecting the other organs. Additionally, a transplant is done only when it is clear that the individual's prognosis on continued medical treatment is poor. Transplant patients require close medical follow-up while taking necessary drugs that suppress the immune system and because of the risk of rejection of the transplanted heart. They must even be monitored for possible development of coronary artery disease in the transplanted heart. - Although there are thousands of patients on waiting lists for a heart transplant at any given time, the number of operations performed each year is limited by the number of available donor organs. For these reasons, heart transplantation is a realistic option in only a small subset of the large numbers of patients with congestive heart failure. - Strong scientific evidence: - Hawthorn: Hawthorn (Crataegus sp.), a flowering shrub of the rose family has an extensive history of use in cardiovascular disease dating back to the 1st Century. Increased blood flow to the heart and heart performance has been observed in animals when given hawthorn supplements. Extracts of the leaves and flowers of hawthorn have been reported as effective in the treatment of mild-to-moderate congestive heart failure (CHF), improving exercise capacity and reducing symptoms of cardiac insufficiency. However, whether hawthorn is as effective as drugs considered standard-of-care for heart failure (such as angiotensin converting enzyme inhibitors, diuretics, or beta-adrenergic receptor blockers) is unclear, as is the effect of the combined use of hawthorn with these drugs. Nonetheless, hawthorn is a potentially beneficial treatment for patients who cannot or will not take prescription drugs and may offer additive benefits to established therapies. Further study is warranted. - Avoid if allergic to hawthorn or to members of the Crataegus species. Avoid with a history of low blood pressure, irregular heartbeat, asthma, low blood pressure when standing or insomnia. Use cautiously in elderly patients. Avoid if pregnant or breastfeeding. - Good scientific evidence: - Arginine: Studies of arginine in patients with chronic heart failure have shown mixed results. Some studies report improved exercise tolerance. Additional studies are needed to confirm these findings. - Avoid if allergic to arginine, or with a history of stroke, or liver or kidney disease. Avoid if pregnant or breastfeeding. Use caution if taking blood-thinning drugs (like warfarin or Coumadin®) and blood pressure drugs or herbs or supplements with similar effects. Blood potassium levels should be monitored. L-arginine may worsen symptoms of sickle cell disease. Caution is advised in patients taking prescription drugs to control blood sugar levels. - Berberine: Berberine is a bitter-tasting, yellow, plant alkaloid with a long history of medicinal use in Chinese and Ayurvedic medicine. Berberine is present in the roots, rhizomes, and stem bark of various plants including Hydrastis canadensis (goldenseal), Coptis chinensis (coptis or goldenthread), Berberis aquifolium (Oregon grape), Berberis vulgaris (barberry), and Berberis aristata (tree turmeric). Preliminary clinical research suggests that berberine, in addition to a standard prescription drug regimen for chronic congestive heart failure (CHF), may improve quality of life, heart function, and mortality. Further research is necessary. - Berberine has been reported to cause nausea, vomiting, hypertension (high blood pressure), respiratory failure, and paresthesias (abnormal sensations such as numbness or tingling). Use cautiously in patients with diabetes. Avoid if allergic or hypersensitive to berberine, to plants that contain berberine [Hydrastis canadensis (goldenseal), Coptis chinensis (coptis or goldenthread), Berberis aquifolium (Oregon grape), Berberis vulgaris (barberry), and Berberis aristata (tree turmeric)], or to members of the Berberidaceae family. Avoid in newborns due to potential for increase in free bilirubin, jaundice, and development of kernicterus. Use cautiously with cardiovascular disease, gastrointestinal disorders, hematologic disorders, leucopenia, kidney disease, liver disease, respiratory disorders, cancer, hypertyraminemia, diabetes, or hypotension. Use cautiously in children due to lack of safety information. Use cautiously in individuals with high exposure to sunlight or artificial light. Use cautiously for longer than eight weeks due to theoretical changes in bacterial gut flora. Use cautiously if taking anticoagulants, antihypertensives, sedatives, anti-inflammatories, medications metabolized by CYP P450 3A4 including cyclosporin, or any prescription medications. Avoid if pregnant or breastfeeding. - Coleus: Coleus species have been used in the Asian traditional medicine for several indications. Since the 1970s, research was predominantly concentrated on forskolin, a root extract of Coleus forskohlii. A small number of studies suggest that forskolin may improve cardiovascular function in patients with cardiomyopathy. However, these trials are small and of poor quality. Larger studies are needed. - Coleus is generally regarded as safe, although long-term safety data are lacking. Avoid with a known allergy or hypersensitivity to Coleus forskohlii and related species. Rash may occur in sensitive individuals. Inhalation of forskolin may cause sore throat, upper respiratory tract irritation, mild to moderate cough, tremor, or restlessness. Coleus eye drops may produce a milky covering over the eyes. Use cautiously in patients with heart disease, asthma, thyroid disorders, diabetes, a history of bleeding, hemostatic disorders or drug-related hemostatic problems, low blood pressure, or in patients at risk for hypotension. Discontinue use in patients at least two weeks prior to surgical or dental procedures, due to risk of bleeding. Avoid in patients with active bleeding. Avoid during pregnancy. - Creatine: Creatine is naturally synthesized in the human body from amino acids primarily in the kidney and liver, and transported in the blood for use by muscles. Cardiac creatine levels have been reported as depressed in patients with chronic congestive heart failure. Several studies report that creatine supplementation is associated with improved heart muscle strength, body weight, and endurance in patients with heart failure. However, it is not clear what dose may be safe or effective. Supplementation is also reported to increase creatine in skeletal muscle in these patients, helping to increase strength and endurance. Well-designed studies comparing creatine with drugs used to treat heart failure are needed. - Avoid if allergic to creatine or with diuretics (like hydrochlorothiazide, furosemide (Lasix®)). Use caution in asthma, diabetes, gout, kidney, liver, or muscle problems, stroke or a history of these conditions. Avoid dehydration. Avoid if pregnant or breastfeeding. - Selenium: Selenium is a trace metal that has been reported to have antioxidant properties. Prophylactic administration of sodium selenite has been shown to significantly decrease the incidence of Keshan disease, which is cardiomyopathy (heart disease) restricted to areas of China in people having an extremely low selenium status. - Avoid if allergic or sensitive to products containing selenium. Avoid with a history of nonmelanoma skin cancer. Selenium is generally regarded as safe for pregnant or breastfeeding women. However, animal research reports that large doses of selenium may lead to birth defects. - Unclear or conflicting scientific evidence: - Aconite: The toxic effects associated with aconite limit its ability to be used to treat heart failure, including reno-cardiovascular disease and left ventricular function. Further study is needed. - Aconite is highly toxic and is not safe for human consumption. Avoid with heart disease, heart dysfunction, irregular heartbeat, hemodynamic instability (abnormal blood flow), gastrointestinal disorders, ulcers, reflux esophagitis, ulcerative colitis, spastic colitis, and diverticulosis. Use caution with diabetes and suicidal tendencies. Avoid if younger than 18 years old. Avoid if pregnant or breastfeeding. - Astragalus: Astragalus (Astragalus membranaceus) is used in combination with other herbs in Chinese medicine to treat various heart diseases. There is some evidence that astragalus may offer symptomatic improvement for chronic heart failure. Recommendations cannot be made until well-designed clinical trials have been conducted. - Avoid if allergic to astragalus, peas, or any related plants or with a history of Quillaja bark-induced asthma. Avoid with aspirin or aspirin products or herbs or supplements with similar effects. Avoid with inflammation (swelling) or fever, stroke, transplant or autoimmune diseases (like HIV/AIDS). Stop use two weeks before surgery/dental/diagnostic procedures with a risk of bleeding and avoid use immediately after these procedures. Use cautiously with bleeding disorders, diabetes, high blood pressure, lipid disorders or kidney disorders. Use cautiously with blood-thinners, blood sugar drugs, or diuretics or herbs and supplements with similar effects. Avoid if pregnant or breastfeeding. - Ayurveda: Ayurveda, which originated in ancient India over 5,000 years ago, is probably the world's oldest system of natural medicine. Preliminary evidence suggests that sodium nimbidinate, made from the traditional Ayurvedic herb Nimba/Neem/Arishta (Azadirachta indica), may be an effective diuretic in patients with congestive heart failure. More studies are needed to confirm this effect. - Ayurvedic herbs should be used cautiously because they are potent and some constituents can be potentially toxic if taken in large amounts or for a long time. Some herbs imported from India have been reported to contain high levels of toxic metals. Ayurvedic herbs can interact with other herbs, foods and drugs. A qualified healthcare professional should be consulted before taking. - Coenzyme Q10 (CoQ10): CoQ10 is produced by the human body and is necessary for the basic functioning of cells. The evidence for CoQ10 in the treatment of heart failure is controversial and remains unclear. Different levels of disease severity have been studied (New York Heart Association classes I through IV). Better research is needed in this area, studying effects on quality of life, hospitalization, and death rates. There is also conflicting evidence from research on the use of CoQ10 in patients with dilated or hypertrophic cardiomyopathy. - CoQ10 is generally safe in recommended dosages, but further studies are needed. - Allergy associated with Coenzyme Q10 supplements has not been reported, although rash and itching have been reported rarely. Stop use two weeks before surgery/dental/diagnostic procedures with bleeding risk and do not use immediately after these procedures. Use caution with a history of blood clots, diabetes, high blood pressure, heart attack, or stroke, or with anticoagulants (blood thinners), antiplatelet drugs (like aspirin, warfarin, clopidogrel (like Plavix®), or blood pressure, blood sugar, cholesterol or thyroid drugs. Avoid if pregnant or breastfeeding. - Ginseng: A clinical study on the effect of Panax ginseng on congestive heart failure did not show a clear benefit of combining digoxin with ginseng. The relatively small study size and the use of a drug instead of a standardized extract limit the value of the evidence. Additional research is needed. - Ginseng may also lower blood pressure. Caution is used when taking ginseng supplements, as adverse effects including drug interactions are possible. Ginseng supplements are not used if pregnant or breastfeeding unless otherwise directed by a doctor. - Goldenseal: Limited available study suggests that berberine in addition to a standard prescription drug regimen for chronic congestive heart failure (CHF) may improve quality of life and decrease ventricular premature complexes (VPCs) and mortality. Further research is needed to confirm these results. - Use cautiously in patients with gastrointestinal disorders, cardiovascular disease, bleeding disorders or in those taking anticoagulants, diabetes or in those taking antidiabetic agents. Use cautiously in infants with increased bilirubin levels or individuals with glucose-6-phosphate deficiency. Use cautiously in pregnancy. - Hawthorn: Herbal combinations containing hawthorn have been found effective in the treatment of functional cardiovascular disorders. However, due to a lack of information on the use of hawthorn alone, there is not enough evidence to recommend for or against this use of hawthorn. - Avoid if allergic to hawthorn or to members of the Crataegus species. Avoid with a history of low blood pressure, irregular heartbeat, asthma, low blood pressure when standing or insomnia. Use cautiously in elderly patients. Avoid if pregnant or breastfeeding. - L-carnitine: L-carnitine, carnitine, or acetyl-L-carnitine, is an amino acid found in the body. Although preliminary results are promising, there is insufficient available clinical evidence for the use of L-carnitine in congestive heart failure. - Avoid with a known allergy or hypersensitivity to carnitine. Use cautiously with peripheral vascular disease, hypertension (high blood pressure), alcohol-induced liver cirrhosis, and diabetes. Use cautiously in low birth weight infants and individuals on hemodialysis. Use cautiously if taking anticoagulants (blood thinners), beta-blockers, or calcium channel blockers. Avoid if pregnant or breastfeeding. - Meditation: Meditation may improve quality of life in elderly patients, and may potentially reduce the risk for congestive heart failure. However, there is not enough evidence to make a conclusion. - Use cautiously with underlying mental illnesses. People with psychiatric disorders should consult with their primary mental healthcare professional(s) before starting a program of meditation, and should explore how meditation may or may not fit in with their current treatment plan. Avoid with risk of seizures. The practice of meditation should not delay the time to diagnosis or treatment with more proven techniques or therapies, and should not be used as the sole approach to illnesses. - Oleander: The term "oleander" refers to two plants: Nerium oleander (common oleander) and Thevetia peruviana (yellow oleander). Both plants contain heart-active "cardiac glycoside" chemicals (similar to the prescription drug digoxin) and have been associated with serious side effects in humans, including death. The plants have been used to treat congestive heart failure in China and Russia for decades, but scientific evidence supporting this use is limited to small, poorly designed studies. Human research began in the 1930s, but was largely abandoned due to serious gastrointestinal and heart toxicity. - All parts of the oleander plant, including flowers, leaves, and nectar are considered toxic and may cause death. Avoid if allergic to oleander or other cardiac glycosides such as digoxin. Avoid with a history of irregular heartbeat (arrhythmia), seizures, liver or kidney disease, depression, or asthma. Avoid if pregnant or breastfeeding. - Passionflower: An extract containing passionflower and hawthorn has been studied for potential enhancement of exercise capacity in congestive heart failure patients. Individuals using this combination of herbs have experienced improvements in symptoms; however, any positive effects may have resulted from hawthorn, which is more commonly used for congestive heart failure. High quality human research of passion flower alone and compared to prescription drugs used for this condition is needed. - Avoid if allergic to passion flower or any of its constituents. Avoid consuming raw Passiflora fruit (Passiflora adenopoda), due to possible cyanide constituents. Passionflower extracts may cause drowsiness in sensitive individuals. Avoid driving or operating heavy machinery while taking passion flower. Use cautiously with low blood pressure. Avoid if pregnant or breastfeeding. - Physical therapy: Both supervised and home-based exercise training may enhance exercise capacity in patients with chronic heart failure. However, consensus has not been obtained regarding a standard rehabilitation program for these patients and the literature often suggests individually-tailored programs. Due to the lack of standardization, duration of treatment, and various outcomes measures, more study is needed before a conclusion can be made. - Not all physical therapy programs are suited for everyone, and patients should discuss their medical history with a qualified healthcare professional before beginning any treatments. Physical therapy may aggravate pre-existing conditions. Persistent pain and fractures of unknown origin have been reported. Physical therapy may increase the duration of pain or cause limitation of motion. Pain and anxiety may occur during the rehabilitation of patients with burns. Both morning stiffness and bone erosion have been reported in the literature although causality is unclear. Erectile dysfunction has also been reported. Physical therapy has been used in pregnancy and although reports of major adverse effects are lacking the available literature, caution is advised nonetheless. All therapies during pregnancy and breastfeeding should be discussed with a licensed obstetrician/gynecologist before initiation. - Relaxation therapy: Early studies suggest that progressive muscle relaxation training may benefit patients with heart failure when used as an adjunct to standard care. - Avoid with psychiatric disorders like schizophrenia/psychosis. Jacobson relaxation (flexing specific muscles, holding that position, then relaxing the muscles) should be used cautiously with illnesses like heart disease, high blood pressure, or musculoskeletal injury. Relaxation therapy is not recommended as the sole treatment approach for potentially serious medical conditions, and should not delay the time to diagnosis or treatment with more proven techniques. - Selenium: Low selenium levels have been associated with the development of cardiomyopathy, and selenium supplementation is likely of benefit in such cases (for example in Keshan disease and Chagas' disease). However, most cases of cardiomyopathy are not due to low selenium levels and therefore selenium may not be helpful. It has been suggested that low selenium levels may be a risk factor for coronary heart disease, although this remains unclear. - Avoid if allergic or sensitive to products containing selenium. Avoid with a history of nonmelanoma skin cancer. Selenium is generally regarded as safe for pregnant or breastfeeding women. However, animal research reports that large doses of selenium may lead to birth defects. - Taurine: Taurine is a nonessential amino acid-like compound, found in high abundance in the tissues of many animals, especially sea animals, and in much lower concentrations in plants, fungi, and some bacteria. Preliminary study suggests that taurine may be beneficial as an adjunct to traditional medications for symptoms of congestive heart failure (CHF). Further study is warranted to confirm these findings. - Taurine appears to be safe in recommended dosages. As an amino acid, it is unlikely that there are allergies related to this constituent. However, allergies may occur from multi-ingredient products that contain taurine. Use cautiously in patients with high cholesterol, low blood pressure, coagulation disorders, potential for mania, or epilepsy. Avoid alcohol or exercise after consumption of energy drinks containing taurine, caffeine, glucuronolactone, B vitamins, and other ingredients. Use cautiously if pregnant or breastfeeding; taurine is a natural component of breast milk - Thiamin: Thiamin (also spelled "thiamine") is a water-soluble B-complex vitamin, previously known as vitamin B1 or aneurine. Thiamin was isolated and characterized in the 1920s, and thus was one of the first organic compounds to be recognized as a vitamin. Chronic severe thiamin deficiency can cause heart failure (wet beriberi), a condition that merits thiamin supplementation. Currently, it is not clear if thiamin supplementation is beneficial in patients with heart failure due to other causes. However, it is reasonable for patients with heart failure to take a daily multivitamin including thiamin, because some of these individuals may be thiamin deficient. Diuretics may lower thiamin levels. Since diuretics are commonly administered to patients with heart failure, patients taking diuretics are at an increased risk of thiamin deficiency. This area remains controversial, and further evidence is necessary before a conclusion can be reached. - Avoid if allergic or hypersensitive to thiamin. Rare hypersensitivity/allergic reactions have occurred with thiamin supplementation. Skin irritation, burning, or itching may rarely occur at injection sites. Large doses may cause drowsiness or muscle relaxation. Use cautiously if pregnant or breastfeeding with doses higher than the U.S. Recommended Daily Allowance (RDA). - Thymus extract: The thymus is a lobular gland located under the breastbone near the thyroid gland. It reaches its maximum size during early childhood and plays a large role in immune function. Preliminary evidence suggests that thymus extract may increase left ventricular function, exercise tolerance, and survival in patients with cardiomyopathy. Additional research is needed to confirm these results. - It is important to use high quality thymus gland supplements. Avoid if allergic or hypersensitive to thymus extracts. Use bovine thymus extract supplements cautiously due to potential for exposure to the virus that causes "mad cow disease." Avoid use with an organ transplant or other forms of allografts or xenografts. Avoid if receiving immunosuppressive therapy, with thymic tumors, myasthenia gravis (neuromuscular disorder), untreated hypothyroidism, or if taking hormonal therapy. Avoid if pregnant or breastfeeding; thymic extract increases human sperm motility and progression. - Traditional Chinese medicine (TCM): Many studies of TCM herbs have focused on treatment of congestive heart failure. Further research of better design is needed before recommendations can be made. - Chinese herbs can be potent and may interact with other herbs, foods or drugs. Consult a qualified healthcare professional before taking. There have been reports of manufactured or processed Chinese herbal products being tainted with toxins or heavy metals or not containing the listed ingredients. Herbal products should be purchased from reliable sources. Avoid ma huang, which is the active ingredient in ephedra. Avoid ginseng if pregnant or breastfeeding. - Fair negative scientific evidence: - Guided imagery: Therapeutic guided imagery may be used to help individuals relax and focus on images associated with personal issues they are confronting. Preliminary human research does not report benefits of guided imagery in congestive heart failure. - Guided imagery is usually intended to supplement medical care, not to replace it, and guided imagery should not be relied on as the sole therapy for a medical problem. Contact a qualified health care provider if mental or physical health is unstable or fragile. Never use guided imagery techniques while driving or doing any other activity that requires strict attention. Use cautiously with physical symptoms that can be brought about by stress, anxiety or emotional upset because imagery may trigger these symptoms. If feeling unusually anxious while practicing guided imagery, or with a history of trauma or abuse, speak with a qualified health care provider before practicing guided imagery. - Smoking cessation: Smoking damages blood vessels, reduces the amount of oxygen in the blood, and makes the heart beat faster. If an individual smokes, a doctor can help recommend a program to help them quit. Individuals are not considered for a heart transplant if smoking is continued. - Weight control: It is recommended that individuals weigh themselves each morning after urination, but before breakfast. Notify a doctor if there is a weight gain of three or more pounds in a day. Weight gain may indicate fluid build-up. - Being overweight contributes to other risk factors for stroke, such as high blood pressure, cardiovascular disease, and diabetes. Weight loss of as little as ten pounds may lower blood pressure and improve cholesterol levels. - Exercise can lower blood pressure, increase the level of high density lipoprotein (HDL cholesterol or good cholesterol), and improve the overall health of blood vessels and heart. It also helps control weight, control diabetes, and reduce stress. Cardiac rehabilitation programs exist for individuals recovering from heart surgery. Cardiac rehabilitation is a medically supervised program to help heart patients recover quickly and improve their overall physical, mental, and social functioning. The goal is to stabilize, slow, or even reverse the progression of cardiovascular disease, thereby reducing the risk of heart disease, another cardiac event, or death. Cardiac rehabilitation programs include: counseling so the individual can understand and manage the disease process; an exercise program; counseling on nutrition; helping the patient modify risk factors such as high blood pressure, smoking, high blood cholesterol, physical inactivity, obesity, and diabetes; providing vocational guidance to enable the patient to return to work; information on physical limitations; lending emotional support; and counseling on appropriate use of prescribed medications. A doctor can help initiate an exercise program and cardiac rehabilitation tailored to the individual with congestive heart failure (CHF). - Salt restriction: Too much sodium (from salt) contributes to water retention, which makes the heart work harder. Excess sodium may causes shortness of breath and swollen legs, ankles, and feet. For individuals with heart failure the recommended sodium intake is no more than 2,000 mg daily. Some substitutes or "lite" salts contain a mixture of salt and other compounds. To get that familiar salty taste, individuals may use too much of the substitute and actually not reduce sodium intake. In addition, many salt substitutes contain potassium chloride. Too much potassium can be harmful. A dietitian can help outline a healthy, low-salt diet. - Stress management: Stress can cause an increase in blood pressure along with increasing the blood's tendency to clot. Managing stress can be vital to keeping a heart healthy. - Diet modification: Eating healthy foods is important. A heart-healthy diet should include five or more daily servings of fruits and vegetables, foods rich in soluble fiber (such as oatmeal and beans), foods rich in calcium (dairy products, spinach), soy products (such as tempeh, miso, tofu, and soy milk), and foods rich in omega-3 fatty acids, including cold-water fish, such as salmon, mackerel, and tuna. Pregnant women and women who plan to become pregnant in the next several years should limit their weekly intake of cold-water fish because of the potential for mercury contamination. Limiting red meats and high fat foods (such as doughnuts, cookies, and chips) is recommended by healthcare professionals. - Alcohol: Excessive use of alcohol may weaken the heart muscle or increase the risk of abnormal heart rhythms, further worsening existing heart failure. Alcohol may also interact with some medications used to treat heart conditions. One glass of red wine daily may be beneficial for heart health. - Swelling: Leg, ankle, and foot edema can be improved by elevating the legs above heart level for 30 minutes three or four times per day. Leg elevation alone may be sufficient therapy for patients with mild venous insufficiency, but is usually not adequate for more severe cases. In addition, it may not be practical for those who work to elevate their legs several times per day. - Leg edema (swelling) can also be prevented and treated with the use of compression stockings. Many types are available, including knee-high, thigh-high, and pantyhose. Knee-high stockings are sufficient for most individuals; thigh-high stockings are less desirable because they tend to provide too much pressure behind the knees, reducing blood flow in the veins, and causing discomfort. The stockings should be put on as early as possible in the morning when edema is minimal. Healthcare professionals can help with choosing the right compression stocking for each individual. - Sleep: It is recommended that individuals with CHF who have shortness of breath sleep with their head propped up at a 45 degree angle using a pillow or a wedge. - This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com). - Allard ML, Jeejeebhoy KN, Sole MJ. The management of conditioned nutritional requirements in heart failure. Heart Fail Rev. 2006;11(1):75-82. . - American Heart Association. . - Boekholdt SM, Sandhu MS, Day NE, et al. Physical activity, C-reactive protein levels and the risk of future coronary artery disease in apparently healthy men and women: the EPIC-Norfolk prospective population study. Eur J Cardiovasc Prev Rehabil. 2006;13(6):970-6. . - Dauchet L, Amouyel P, Hercberg S, et al. Fruit and vegetable consumption and risk of coronary heart disease: a meta-analysis of cohort studies. J Nutr. 2006;136(10):2588-93. . - Jiang W, Kuchibhatla M, Clary GL, et al. Relationship between depressive symptoms and long-term mortality in patients with heart failure. Am Heart J. 2007;154(1):102-8. . - National Institutes of Health. . - Natural Standard: The Authority on Integrative Medicine. . - Riegel B, Moser DK, Powell M, et al. Nonpharmacologic care by heart failure experts. J Card Fail. 2006;12(2):149-153. . - U.S. Food and Drug Administration. . - von Haehling S, Doehner W, Anker SD. Nutrition, metabolism, and the complex pathophysiology of cachexia in chronic heart failure. Cardiovasc Res. 2007;73(2):298-309. . Causes and risk factors - The heart consists of four chambers: the right atrium, the left atrium, the right ventricle, and the left ventricle. The heart also has four major valves: the mitral valve, the tricuspid valve, the aortic valve, and the pulmonary valve. Atria are relatively thin-walled chambers that receive blood from the circulatory system and from the lungs. Ventricles are muscular chambers that pump blood into the circulatory system and into the lungs. - Blood passes from the atria into the ventricles through two processes. During the resting phase, when the ventricles are not contracting, the tricuspid and mitral valves open and allow some of the blood that has accumulated in the atria to flow passively through the valves into the ventricles. Then, the atria contract and actively pump blood out through the valves and into the ventricles. Once the ventricles fill with blood, they contract, pumping blood to the lungs and the rest of the body. - When the left ventricle cannot adequately pump blood out of the left atrium, or when one or more of the heart valves becomes leaky or narrowed, blood can back up into the lungs, causing left-sided heart failure. When this occurs, the lungs become congested with fluid (called pulmonary edema), causing difficulty breathing and interfering with the movement of oxygen from the lungs into the bloodstream, causing fatigue. - When an abnormality or condition affects the flow of blood through the right ventricle, pressure in the blood vessels increases and fluid is forced from the blood vessels into body tissues. This right-sided heart failure causes swelling (edema), usually in the feet and legs, and sometimes, in the abdomen. - Coronary artery disease and heart attack: Coronary artery disease is the most common form of heart disease and the most common cause of heart failure. Over time, arteries that supply blood to the heart muscle narrow from a buildup of fatty deposits, a process called atherosclerosis. The buildup containing fats and clotting factors is called a plaque. Blood moves slowly through narrowed arteries, leaving some areas of the heart muscle chronically deprived of oxygen-rich blood. These areas of the heart may become weak from the lack of oxygen and pump less vigorously. In many cases, the blood flow to the muscle is just enough to keep the muscle alive but not functioning well. A heart attack occurs if an unstable plaque ruptures, causing a blood clot to completely block blood flow to an area of the heart muscle. A heart attack results in the death of heart muscle, which can quickly weaken the heart's pumping ability. Sometimes coronary artery disease is limited to the small coronary arteries. If these arteries become blocked, this won't cause a heart attack, but over time, it can gradually weaken the heart. - Hypertension (high blood pressure): Blood pressure is the force of blood pumped by the heart through the arteries. If blood pressure is high, the heart has to work harder than it should to circulate blood throughout the body. Over time, the heart muscle may become thicker to compensate for the extra work it must perform. In some cases, the heart will enlarge. Eventually, the heart muscle may become either too stiff or too weak to effectively pump blood. - Defective heart valves: The four valves of the heart keep blood flowing in the proper direction through the heart. A damaged valve forces the heart to work harder to keep blood flowing as it should. Over time, this extra work can weaken the heart. - Cardiomyopathy: Cardiomyopathy is a serious disease in which the heart muscle becomes inflamed and does not work as well as it should. There may be multiple causes including viral infections, alcohol abuse, and the toxic effect of drugs such as cocaine or doxorubicin (Adriamycin®) used for chemotherapy. In addition, whole-body diseases that may lead to inflammation, such as lupus or thyroid problems, can also damage heart muscle. - Myocarditis: Myocarditis is an inflammation of the heart muscle. Myocarditis is most commonly caused by a virus and can lead to left-sided heart failure. The virus most often associated with myocarditis is coxsackievirus B, but other viruses may include adenovirus, parvovirus B19, echovirus, influenza virus, Epstein-Barr virus, and rubella virus. Human immunodeficiency virus (HIV) can directly infiltrate the heart muscle and cause myocarditis. Other causes of myocarditis include bacteria (such as Staphylococcus aureus), parasites (such as Trypanosoma cruzi and toxoplasma), fungi (such as Candida albicans), exposure to certain chemicals such as arsenic and hydrocarbons, medications that may cause allergic or toxic reactions (such as cocaine or penicillin), and systemic diseases causing inflammation (including lupus). - Congenital heart defects: Congenital heart defects are structural problems with the heart present at birth. Genetic defects contribute to the risk of certain types of heart diseases (such as arrythmias or valve problems), which in turn may lead to heart failure. Estimates suggest that about one million Americans have a congenital heart defect. Approximately 35,000 babies in the United States are born with a heart defect each year. - Arrhythmias: Arrhythmias, or abnormal heart rhythms, may cause the heart to beat too fast. This creates extra work for the heart. Over time, the heart may weaken leading to heart failure. - Other conditions: Health conditions, such as diabetes, severe anemia (lack of red blood cells to carry oxygen to tissues), hyperthyroidism (high thyroid hormone levels), hypothyroidism (low thyroid hormone levels), emphysema (lung disease that involves damage to the air sacs in the lungs), pulmonary edema (fluid in the lungs), hemochromatosis (an inherited disease in which too much iron builds up in the body), and amyloidosis (a group of diseases in which one or more organ systems in the body accumulate deposits of abnormal proteins such as C-reactive protein), may also contribute to heart failure. Kidney disease can contribute to heart failure due to an increase in high blood pressure and fluid retention. - Heart failure may develop during the last the months of pregnancy or several months after pregnancy. The cause of this is not well understood, but it may be due to an abnormal immune system response. - Viral infections may cause idiopathic heart failure or heart failure in which there is never an identified cause. Bacterial infections, such as pneumonia, may lead to CHF. - Exposure to toxins, such as alcohol or cocaine, may also lead to CHF. An example of drug-induced CHF is with encainide hydrochloride (Enkaid®). Encainide is a drug used for arrhythmias (irregular heart beats), but in susceptible individuals, its use may lead to CHF. - Obesity may also lead to CHF. Obesity promotes diabetes, hypertension (high blood pressure), and dyslipidemia (high cholesterol levels). These conditions are risk factors for developing coronary artery disease and ultimately, CHF. - Sleep apnea, or the inability to breathe properly at night, results in low blood oxygen levels and increased risk of abnormal heart rhythms. Both of these problems can weaken the heart and lead to CHF. - The condition affects 1% of people aged 50 years and older and about 5% of those aged 75 years and older. African Americans experience heart failure twice as often as Caucasians. About 10% of patients diagnosed with heart failure die within one year, and about 50% die within five years of diagnosis. - CHF is equally frequent in men and women, and annual incidence approaches ten per 1,000 population after 65 years of age. Survival following diagnosis of congestive heart failure is worse in men than women, but even in women, only about 20% survive much longer than eight to 12 years. Copyright © 2011 Natural Standard (www.naturalstandard.com) The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.	2	10	0	0	0	3	0.951418	3	13,749
\|Classification and external resources\| Hypochondriasis, also known as hypochondria, health anxiety or illness anxiety disorder, refers to worry about having a serious illness. This debilitating condition is the result of an inaccurate perception of the condition of body or mind despite the absence of an actual medical condition. An individual suffering from hypochondriasis is known as a hypochondriac. Hypochondriacs become unduly alarmed about any physical or psychological symptoms they detect, no matter how minor the symptom may be, and are convinced that they have, or are about to be diagnosed with, a serious illness. Often, hypochondria persists even after a physician has evaluated a person and reassured them that their concerns about symptoms do not have an underlying medical basis or, if there is a medical illness, their concerns are far in excess of what is appropriate for the level of disease. Many hypochondriacs focus on a particular symptom as the catalyst of their worrying, such as gastro-intestinal problems, palpitations, or muscle fatigue. To qualify for the diagnosis of hypochondria the symptoms must have been experienced for at least 6 months. The DSM-IV-TR defines this disorder, "Hypochondriasis", as a somatoform disorder and one study has shown it to affect about 3% of the visitors to primary care settings. The newly published DSM-5 replaces the diagnosis of hypochondriasis with the diagnoses of "Somatic Symptom Disorder" and "Illness Anxiety Disorder". Hypochondria is often characterized by fears that minor bodily or mental symptoms may indicate a serious illness, constant self-examination and self-diagnosis, and a preoccupation with one's body. Many individuals with hypochondriasis express doubt and disbelief in the doctors' diagnosis, and report that doctors’ reassurance about an absence of a serious medical condition is unconvincing, or short-lasting. Additionally, many hypochondriacs experience elevated blood pressure, stress, and anxiety in the presence of doctors or while occupying a medical facility, a condition known as "white coat syndrome". Many hypochondriacs require constant reassurance, either from doctors, family, or friends, and the disorder can become a disabling torment for the individual with hypochondriasis, as well as his or her family and friends. Some hypochondriacal individuals completely avoid any reminder of illness, whereas others frequently visit medical facilities, sometimes obsessively. Other victims of this disease will never speak about it. Hypochondriasis is categorized as a somatic amplification disorder—a disorder of "perception and cognition"—that involves a hyper-vigilance of situation of the body or mind and a tendency to react to the initial perceptions in a negative manner that is further debilitating. Hypochondriasis manifests in many ways. Some people have numerous intrusive thoughts and physical sensations that push them to check with family, friends, and physicians. For example, a person who has a minor cough may think that they have tuberculosis. Or sounds produced by organs in the body, such as those made by the intestines, might be seen as a sign of a very serious illness to patients dealing with hypochondriasis. Other people are so afraid of any reminder of illness that they will avoid medical professionals for a seemingly minor problem, sometimes to the point of becoming neglectful of their health when a serious condition may exist and go undiagnosed. Yet others live in despair and depression, certain that they have a life-threatening disease and no physician can help them. Some consider the disease as a punishment for past misdeeds. Hypochondriasis is often accompanied by other psychological disorders. Bipolar disorder, clinical depression, obsessive-compulsive disorder (OCD), phobias, and somatization disorder are the most common accompanying conditions in people with hypochondriasis, as well as a generalized anxiety disorder diagnosis at some point in their life. Many people with hypochondriasis experience a cycle of intrusive thoughts followed by compulsive checking, which is very similar to the symptoms of obsessive-compulsive disorder. However, while people with hypochondriasis are afraid of having an illness, patients with OCD worry about getting an illness or of transmitting an illness to others. Although some people might have both, these are distinct conditions. Patients with hypochondriasis often are not aware that depression and anxiety produce their own physical symptoms, and mistake these symptoms for manifestations of another mental or physical disorder or disease. For example, people with depression often experience changes in appetite and weight fluctuation, fatigue, decreased interest in sex and motivation in life overall. Intense anxiety is associated with rapid heartbeat, palpitations, sweating, muscle tension, stomach discomfort, dizziness, and numbness or tingling in certain parts of the body (hands, forehead, etc.). If a person is ill with a medical disease such as diabetes or arthritis, there will often be psychological consequences, such as depression. Some even report being suicidal. In the same way, someone with psychological issues such as depression or anxiety will sometimes experience physical manifestations of these affective fluctuations, often in the form of medically unexplained symptoms. Common symptoms include headaches; abdominal, back, joint, rectal, or urinary pain; nausea; fever and/or night sweats; itching; diarrhea; dizziness; or balance problems. Many people with hypochondriasis accompanied by medically unexplained symptoms feel they are not understood by their physicians, and are frustrated by their doctors’ repeated failure to provide symptom relief. The ICD-10 defines hypochondriasis as follows: - A. Either one of the following: - A persistent belief, of at least six months' duration, of the presence of a maximum of two serious physical diseases (of which at least one must be specifically named by the patient). - A persistent preoccupation with a presumed deformity or disfigurement (body dysmorphic disorder). - B. Preoccupation with the belief and the symptoms causes persistent distress or interference with personal functioning in daily living, and leads the patient to seek medical treatment or investigations (or equivalent help from local healers). - C. Persistent refusal to accept medical advice that there is no adequate physical cause for the symptoms or physical abnormality, except for short periods of up to a few weeks at a time immediately after or during medical investigations. - D. Most commonly used exclusion criteria: not occurring only during any of the schizophrenia and related disorders (F20-F29, particularly F22) or any of the mood disorders (F30-F39). The DSM-IV defines hypochondriasis according to the following criteria: A. Preoccupation with fears of having, or the idea that one has, a serious disease based on the person's misinterpretation of bodily symptoms. B. The preoccupation persists despite appropriate medical evaluation and reassurance. C. The belief in Criterion A is not of delusional intensity (as in Delusional Disorder, Somatic Type) and is not restricted to a circumscribed concern about appearance (as in Body Dysmorphic Disorder). D. The preoccupation causes clinically significant distress or impairment in social, occupational, or other important areas of functioning. E. The duration of the disturbance is at least 6 months. F. The preoccupation is not better accounted for by Generalized Anxiety Disorder, Obsessive-Compulsive Disorder, Panic Disorder, a Major Depressive Episode, Separation Anxiety, or another Somatoform Disorder. The newly published DSM-5 replaces the diagnosis of hypochondriasis with "illness anxiety disorder". Hypochondria is currently considered a psychosomatic disorder, as in a mental illness with physical symptoms. Cyberchondria is a colloquial term for hypochondria in individuals who have researched medical conditions on the Internet. The media and the Internet often contribute to hypochondria, as articles, TV shows and advertisements regarding serious illnesses such as cancer and multiple sclerosis often portray these diseases as being random, obscure and somewhat inevitable. Inaccurate portrayal of risk and the identification of non-specific symptoms as signs of serious illness contribute to exacerbating the hypochondriac’s fear that they actually have that illness. Major disease outbreaks or predicted pandemics can also contribute to hypochondria. Statistics regarding certain illnesses, such as cancer, will give hypochondriacs the illusion that they are more likely to develop the disease. It is common for serious illnesses or deaths of family members or friends to trigger hypochondria in certain individuals. Similarly, when approaching the age of a parent's premature death from disease, many otherwise healthy, happy individuals fall prey to hypochondria. These individuals believe they are suffering from the same disease that caused their parent's death, sometimes causing panic attacks with corresponding symptoms. Family studies of hypochondriasis do not show a genetic transmission of the disorder. Among relatives of people suffering from hypochondriasis only somatization disorder and generalized anxiety disorder were more common than in average families. Other studies have shown that the first degree relatives of patients with OCD have a higher than expected frequency of a somatoform disorder (either hypochondriasis or body dysmorphic disorder). Most research indicates that cognitive behavioral therapy (CBT) is an effective treatment for hypochondriasis. Much of this research is limited by methodological issues. A small amount of evidence suggests that selective serotonin reuptake inhibitors can also reduce symptoms, but further research is needed. Among the regions of the abdomen, the hypochondrium is the uppermost part. The word derives from the Greek term ὑποχόνδριος hypokhondrios, meaning "of the soft parts between the ribs and navel" from hypo ("under") and khondros, or cartilage (of the sternum). Hypochondria in Late Latin meant "the abdomen". The term hypochondriasis for a state of disease without real cause reflected the ancient belief that the viscera of the hypochondria were the seat of melancholy and sources of the vapor that caused morbid feelings. Until the early 18th century, the term referred to a "physical disease caused by imbalances in the region that was below your rib cage" (i.e., of the stomach or digestive system). For example, Robert Burton's The Anatomy of Melancholy (1621) blamed it "for everything from 'too much spittle' to 'rumbling in the guts'". Immanuel Kant discussed hypochondria in his 1798 book, Anthropology like this: The disease of the hypochondriac consists in this: that certain bodily sensations do not so much indicate a really existing disease in the body as rather merely excite apprehensions of its existence: and human nature is so constituted – a trait which the animal lacks – that it is able to strengthen or make permanent local impressions simply by paying attention to them, whereas an abstraction – whether produced on purpose or by other diverting occupations – lessen these impressions, or even effaces them altogether. - Munchausen syndrome - Psychosomatic medicine - Sickness behavior - Somatoform disorder - Somatosensory amplification - Medical students' disease - Man flu - The Imaginary Invalid - Avia, M. D.; Ruiz, M. A. (2005). "Recommendations for the Treatment of Hypochondriac Patients". Journal of Contemporary Psychotherapy. 35 (3): 301–13. doi:10.1007/s10879-005-4322-3. - Kring, Ann M.; Davison, Gerald C.; Neale, John M.; Johnson, Sheri L. (2007). Abnormal Psychology with Cases (10th ed.). Wiley. ISBN 978-0-471-71260-2. - Goldberg, Richard J. (2007). Practical Guide to the Care of the Psychiatric Patient. ISBN 978-0-323-03683-2. - American Psychiatric Association: Diagnostic and Statistical Manual of Mental Disorders, 4th ed., text revised, Washington, DC, APA, 2000. - Escobar, Javier I; Gara, Michael; Waitzkin, Howard; Silver, Roxanne Cohen; Holman, Alison; Compton, Wilson (1998). "DSM-IV Hypochondriasis in Primary Care". General Hospital Psychiatry. 20 (3): 155–9. doi:10.1016/S0163-8343(98)00018-8. PMID 9650033. - Olatunji, Bunmi O.; Etzel, Erin N.; Tomarken, Andrew J.; Ciesielski, Bethany G.; Deacon, Brett (2011). "The effects of safety behaviors on health anxiety: An experimental investigation". Behaviour Research and Therapy. 49 (11): 719–28. doi:10.1016/j.brat.2011.07.008. PMID 21839987. - Daniel L. Schacter, Daniel T. Gilbert, Daniel M. Wegner.(2011).Generalized Anxiety Disorder.Psychology second edition. - Fallon, Brian A.; Qureshi, Altamash I.; Laje, Gonzalo; Klein, Brian (2000). "Hypochondriasis and its relationship to obsessive-compulsive disorder". The Psychiatric Clinics of North America. 23 (3): 605–16. doi:10.1016/S0193-953X(05)70183-0. PMID 10986730. - Barsky, AJ (1992). "Hypochondriasis and obsessive compulsive disorder". The Psychiatric Clinics of North America. 15 (4): 791–801. PMID 1461796. - Wenning, Michael; Davy, Leigh; Catalano, Glenn; Catalano, Maria (2003). "Atypical Antipsychotics in the Treatment of Delusional Parasitosis". Annals of Clinical Psychiatry. 15 (3-4): 233–9. doi:10.3109/10401230309085693. PMID 14971869. - Harth, Wolfgang; Gieler, Uwe; Kusnir, Daniel; Tausk, Francisco A. (2008). "Hypochondriacal Delusions". Clinical Management in Psychodermatology. Springer. p. 36. ISBN 978-3-540-34718-7. - Ford, Allison. "Hypochondria: Can You Worry Yourself Sick?". divine caroline. divine caroline. Retrieved 19 November 2012. - "Hypochondriasis." CareNotes. Thomson Healthcare, Inc., 2011. Health Reference Center Academic. Retrieved April 5, 2012. - Bienvenu, O.Joseph; Samuels, Jack F; Riddle, Mark A; Hoehn-Saric, Rudolf; Liang, Kung-Yee; Cullen, Bernadette A.M; Grados, Marco A; Nestadt, Gerald (2000). "The relationship of obsessive–compulsive disorder to possible spectrum disorders: results from a family study". Biological Psychiatry. 48 (4): 287–93. doi:10.1016/S0006-3223(00)00831-3. PMID 10960159. - Olatunji, Bunmi O.; Kauffman, Brooke Y.; Meltzer, Sari; Davis, Michelle L.; Smits, Jasper A.J.; Powers, Mark B. (2014). "Cognitive-behavioral therapy for hypochondriasis/health anxiety: A meta-analysis of treatment outcome and moderators". Behaviour Research and Therapy. 58: 65–74. doi:10.1016/j.brat.2014.05.002. PMID 24954212. - Bouman, Theo K. (February 2014). "Psychological Treatments for Hypochondriasis: A Narrative Review". Current Psychiatry Reviews. 10 (1): 58–69. doi:10.2174/1573400509666131119010612. - Louw, Kerry-Ann; Hoare, Jacqueline; Stein, Dan J (February 2014). "Pharmacological Treatments for Hypochondriasis: A Review". Current Psychiatry Reviews. 10 (1): 70–4. doi:10.2174/1573400509666131119004750. - "hypochondria (n.)". Etymonline. Retrieved 14 April 2015. - Susan Harvey (February 21, 2010). "Hypochondria". The Virtual Linguist. - Leslie Mann (July 11, 2012). "New book tries to explain the roots of hypochondria". Chicago Tribune. \|Look up hypochondriasis in Wiktionary, the free dictionary.\| - Belling, Catherine. 2012. "A Condition of Doubt: The Meanings of Hypochondria." New York: Oxford University Press. ISBN 978-0199892365.	2	8	6	0	3	1	0.981775	10	3,545
Study sets, textbooks, questions Upgrade to remove ads Terms in this set (137) AAMI Chemical Analysis of water used for hemodialysis is tested... A fistula connects... an artery to a vein A marker used to determine the middle molecule clearance of a dialyzer is A mixed-bed DI tank contains cations and anions A nephron is made up of... a glomerulus and a tubule system A patient who is to receive their first dialysis treatment is prone to... dialysis disequilibrium syndrome What is a food high in potassium? A slow pulse of 54 beats/minute would indicate the patient has A reason why a dialysis technician is to be certified is It's a CMS requirement A rise in transmembrane pressure in a low flux dialyzer during hemodialysis is indicative of... clotting of the extracorporeal circuit A true aneurysm can be dangerous because it... may rupture and cause the patient to bleed profusely A patients lab results indicate that the pre-dialysis BUN level was lower than the post dialysis BUN level. When interpreting these findings the technician would seek the answer to which question? Was the post dialysis sample drawn correctly? A rapid pulse of 104 beats/minute would indicate that the patient has AAMI standard for conventional dialysate requires the total microbial count be less than... Abnormal levels of this electrolyte can cause cardiac arrhythmias and even death... Acceptable interdialytic weight gains are 3-5% of TW The AAMI standard for endotoxin levels in conventional dialysate is... less than 0.50 EU/mL Albumin is an important test that tells about the patients' ________ status All of the following are phosphate binders EXCEPT... Vitamin B12 (other options: TUMS, Phos-Lo, Renagel) All of the following may be signs and symptoms of an infected access EXCEPT... Numbness and tingling (other options: fever and swelling of the access, redness and warmth, tenderness and drainage) Asepsis is defined as... the absence of disease producing organisms Dialysate that is too hot will cause... Target weight is defined as the... weight at which the patient has no edema or fluid in the lungs and BP is normal Low venous pressure alarm is most likely caused by... disconnection of the bloodline If three hours of time has actually passed but the machine is showing that only two and a half hours have passed, the technician should understand that... the machine was in bypass for 30 minutes Biohazardous waste includes any material exposed to... Decreasing the patients prescribed blood flow rate affects the rate of... After an infiltration of the venous needle, the technician should FIRST... STOP THE BLOOD PUMP Dialysate pH should be... between 6.9 and 7.6 If the air detector alarm is going off and foam is visible in the venous drip chamber, the dialysis technician should immediately... clamp the venous line What question should a technician ask during pre-dialysis data collection? How have you been since your last dialysis treatment? If a patient complains of being chilled, which action should the technician take? take the patient's temperature When setting up the machine, if the conductivity test fails, what action should be taken? notify the equipment technician ESRD specific tests such as BUN, Creatinine, and URR are... indicators of adequacy of the renal replacement therapy Excess fluid is forced out of the blood and into the dialysate during dialysis by which principle? Feed conductivity minus product conductivity divided by feed conductivity X 100 is the formula for calculating... Dizziness and nausea experienced towards the end of the hemodialysis treatment are most likely due to... Fluid to be removed divided by the treatment time tells us the ultrafiltration rate Germicide rebound occurs due to inadequate dialyzer priming A semipermeable membrane is a porous barrier that... allows only certain sized particles to cross Hives, itching, and tightness of the chest and respiratory distress may be signs and symptoms of anaphylaxis (severe allergic reaction) Hyperkalemia can lead to... If a patient develops an infection post transplant, the largest contributing factor would most likely be... If the extracorporeal circuit clots, it would be most important to monitor the patient's serum level of... During dialyzer reprocessing, the blood component is tested to... check dialyzer for total cell volume Lidocaine is administered... A 58-year old patient who is blind comes to treatment accompanied by his wife. Which of these approaches should the dialysis technician use when interacting with the patient? describe the activity in the immediate enviornment Low albumin levels in the dialysis patient have been linked with: higher hospitalization and death rates A medication that dialyzes off easily is... Mr. Jones demonstrates how to wash his access. This is an example of which adult learning principle? Mr. Jones suffers from constipation. He goes to the drug store to buy a laxative. Mr. Jones should avoid which medication? Milk of Magnesia Mr. Roberts verbalizes how to hand crank his blood pump in the event of an emergency. This is an example of which adult principle? Mrs. Pruitt's temperature is elevated post-hemodialysis treatment. Which of these actions should the dialysis technician take? notify the nurse Mrs. Smith arrives for dialysis treatment complaining of shortness of breath. Her pre-dialysis weight is 4.7 kilograms above the last post-treatment weight. The dialysis technician reweighed and verified the weight. Which of the actions should the dialysis technician take next? notify the nurse of need for an assessment Ms. Foster complains of shortness of breath. While calling for the nurse the dialysis technician notices there is air in the extracorporeal circuit she/he should place Ms. Foster in: trendelenburg position on the left side One reason that normal saline is used to prime and perform recirculation for the extracorporeal circuit prior to treatment is to remove: Normal body pH is... 7.35 - 7.45 One disadvantage of a fistula is an increase in.. time to mature One cause of aneurysms and pseudoaneurysms is: poor rotation of sites The air detector alarms during treatment. If the machine is working properly, the dialysis technician should expect which of these events to have occurred? blood pump has stopped Pain at the venous needle site, along with respiratory difficulty and chest pain could be an indicator of: Patient exposure to chloramines during the hemodialysis treatment may cause: One sign and symptom of fluid overload is Solutions that have a higher osmolarity than body fluids are called? Sam's TW is 70 kilograms (kg). He arrives for treatment today at a weight of 70 kg. He complains of shortness of breath and you notice his ankles are swollen. You notify the charge nurse. What do you think will be the plan for Sam's treatment today? the nurse will get an order to decrease the TW Symptoms of hyperkalemia may include... numbness and tingling The accuracy of lab results depends on adherence to proper lab draw technique. You must ensure that labs are drawn: at the right time, right site, and right order Short frequent hemodialysis treatments in patients with high BUN (blood urea nitrogen) are performed to prevent: dialysis disequilibrium syndrome The medication used to treat anemia is: The most important monitor in the dialysis setting is: The extracorporeal circuit consists of the blood lines and the dialyzer The normal range of resting respirations in an adult is: 12-20 breaths per minute The dextrose used in the Dialysate for peritoneal dialysis can cause: The leading cause of chronic kidney disease for adults in the United States: The organization that has as a goal to 'improve the care and outcomes of all individuals with kidney disease' is called: The dialysis technician's role in patient education is to: reinforce educational information provided The most important intervention to prevent the spread of infection is: performing hand hygiene The movement of fluid from the tissues toward a higher concentration of sodium in the blood is an example of The functional unit of the kidney, or the structure in the kidney that does the work, is the: The bruit in an AV fistula or graft is detected by listening with a stethoscope The National standard, based on the KDOQI (Kidney Disease Outcomes Quality Initiative) guidelines, for the Urea Reduction Rate (URR) is a ratio greater than: The most important member of the patient care team is the: The medication used to improve calcium absorption is: How do you know if a patient can safely disconnect themselves in an emergency? let the patient show you after a treatment 'Dwell time' in peritoneal dialysis is defined as: the amount of time it takes to drain the peritoneal fluid Dialysate conductivity measures: the total electrical charge of a solution Name the two compartments of the dialyzer: Which statement is true about kidney transplantation? there is a shortage of available kidney donors The usual fluid allowance for a person with kidney failure is 1.0 to 1.5 liters per day The scale says a patient weights 70 kg. This is the same as weighing how many pounds (lb)? The organization that sets the standards for dialysis water quality is called: Total cell volume (TCV) is an indirect measure of the: performance of the dialyzer Total chlorine levels in the water are tested before each patient shift and every 4 hours The purpose of using counter-current flow is to increase the rate of waste removal Water moves across a membrane from an area of lower solute concentration to an area of higher solute concentration by which principle The water softener operates on the principle of ion exchange What are the typical signs of fluid overload? edema and shortness of breath The usual heparin sodium concentration for anticoagulation during hemodialysis is: To determine the patency of an arteriovenous vascular access, the dialysis technician should initially feel for the presence of Ultrafiltration is influenced by Waste products are removed during dialysis by which principle The water softener removes calcium and magnesium The reprocessed dialyzer is carefully rinsed prior to patient use to remove: The part of the cell wall of bacteria that, when released, can cause fever in hemodialysis patients is: The venous needle in an AV fistula should be placed antegrade The total pressure that exists across the dialyzer membrane is called The substance that may be added to the municipal drinking water to make it clearer is: Which of the following is the most accurate measure of dialysis adequacy? Which component in dialysate is used to correct metabolic acidosis? Which of the following is most likely to cause a high venous pressure alarm? kinking of the venous blood line What lab tests are used to monitor anemia in the ESKD patient? Which learning domain is knowledge based? What is the desired outcome when the patient performs fistula exercises? enlargement of the vein Which of the following is the classic sign of hemolysis? cranberry juice colored blood in the venous line Which food below is highest in phosphorus? When fluid replacement is necessary during the hemodialysis treatment, which of the following solutions is used? 0.9% normal saline Which of the following is NOT a symptom of uremia? Which of the following is a cause of hypotension during and after dialysis treatments? removing too much fluid Which of the following is NOT a possible cause of an increased temperature pre dialysis? the patient has fluid overload Which of the following is considered to be safe water for dialysis? dialysis quality water Which of the following is the largest gauge? Which of the following actions would increase diffusion during dialysis? using a bath that will provide a greater concentration gradient Which of the following diffuses from the patient's blood during dialysis? What is the most important activity that the dialysis technician can perform to prevent the spread of infectious disease in the dialysis clinic? Which learning domain is behavioral based? When cannulating, the tips of the needles should be at least 1.5 inches apart Which of the following symptoms would indicate a need to increase the target weight? hypotension and cramping Your patient is to receive 2000 units of heparin. The vial you are using is 1000u/ml heparin. How many mLs will you draw up for this patient? Which of these types of vascular accesses has the fewest complications? an arteriovenous fistula Which of the following is used to prevent air in the bloodlines? tighten all connections Which organization establishes the standards and recommendations for dialyzer reprocessing? Which of the following would cause a high venous pressure alarm? venous needle is against the wall of the vessel Which of the following might indicate a need to decrease the heparin dose? patient just had surgery yesterday Which of the following might indicate the need to increase heparin? clotting in the extracorporeal circuit Which of these technician activities contributes to data collection used to measure quality outcomes? ensuring accurate documentation You have documented incorrectly on a patient's chart. How do you correct this? draw line through wrong entry, write error, initial/date entry A decrease in transmembrane pressure in a high flux dialyzer during hemodialysis is indicative of: clotting of the extracorporeal circuit Sets with similar terms Clinical Annual Competency Exam - Fresenius Clinical Annual Competency Exam - Fresenius Other sets by this creator Developmental Psych Test 3 Developmental Psych Test 2 Biology Test 2 Biology Evolution and Phylogeny Other Quizlet sets DYE MODULE from the Spinnning & Fiber Ye… GI for midunit Chapter 8 Study Guide Questions	2	9	0	0	0	1	0.691752	1	3,131
With a mission to raise awareness about endometriosis – a disease which affects an estimated 176 million women worldwide – the month of March is observed as “National Endometriosis Awareness Month” in the United States. Sponsored by the Endometriosis Association, this month-long campaign aims to bring together women, families and medical communities and make them aware about the symptoms of this painful hormone and immune-system disease. Endometriosis can have a devastating effect on the quality of life due to its very painful symptoms. Reports suggest that this hormonal disorder affects five million women in the United States and is most common among women in the age group of 30-40. If left untreated, this condition can lead to several complications like – infertility, uterine abnormalities and ovarian cancer. Treatment for endometriosis usually involves medications or surgery (to remove the endometriosis implants). Obstetrics and Gynecology medical billing and coding may involve several challenges. OB-GYN (obstetrician-gynecologist) who provides treatment for endometriosis need to ensure that the medical billing and coding for the same is done appropriately on the medical claims. Opting for billing services from a reliable and established medical billing company would be a practical solution. The major goal behind the endometriosis month is to educate the general public and medical professionals about endometriosis; to find a cure and develop non-invasive diagnostic testing and to improve health screenings among young girls and women in public schools. Regarded as one of the biggest causes of infertility in women, endometriosis occurs when the endometrium (the tissue that normally lines the inside of your uterus) grows outside your uterus. The condition most commonly affects your ovaries, fallopian tubes and the tissue lining your pelvis. In some rare cases, endometrial tissue may spread beyond pelvic organs as well. This “misplaced” tissue can cause severe pain, painful/heavy periods (dysmenorrhea), pain during intercourse/ bowel movements or urination, excessive bleeding and other symptoms. The pain usually occurs in the abdomen, lower back or pelvic areas. The 2019 monthly campaign is a perfect platform to generate awareness about endometriosis and underline the need for early diagnosis of symptoms for effective treatment. Diagnosis of this condition may generally begin with a detailed physical examination wherein the physicians will study the symptoms of the patient, including the location of the pain and when it occurs. A wide range of diagnostic tests like pelvic exam, ultrasound, Magnetic resonance imaging (MRI) and laparoscopy will be conducted to identify physical clues of endometriosis. These tests will help identify the location, extent and size of the endometrial implants in order to determine the best treatment options. Treatment for endometriosis usually involves medication or surgery and this again will depend on the severity of symptoms and whether a woman is planning to become pregnant. Pain medications, supplemental hormone therapy and surgery to remove endometriosis implants while preserving the uterus and ovaries will be opted as the initial part of the treatment. Women with endometriosis can have trouble conceiving. In such cases, fertility treatment (supervised by a fertility specialist) ranging from stimulating ovaries to make more eggs to in vitro fertilization will be opted. The diagnosis, screening tests and other treatment procedures performed by obstetrician-gynecologists must be carefully documented using the correct medical billing codes. Medical billing and coding services offered by experienced providers ensure this so that accurate claims submission is guaranteed. ICD-10 codes used for diagnosing endometriosis include – ICD – 10 Codes - N80 – Endometriosis - N80.0 – Endometriosis of uterus - N80.1 – Endometriosis of ovary - N80.2 – Endometriosis of fallopian tube - N80.3 – Endometriosis of pelvic peritoneum - N80.4 – Endometriosis of rectovaginal septum and vagina - N80.5 – Endometriosis of intestine - N80.6 – Endometriosis in cutaneous scar - N80.8 – Other endometriosis - N80.9 – Endometriosis, unspecified Yellow is the official color for the event. The monthly observance of “Endometriosis Awareness” first began in the year 1993. The event was started by Mary Lou Ballweg (one among the eight women founders) who founded an Endometriosis Awareness Week during The Endometriosis Association’s roundtable in Milwaukee. Those eight founders soon grew to 22, and shortly after that, the week-long observance was extended to a month-long celebration – in order to recognize the pain and sufferings of women who suffer from endometriosis. People throughout the country can participate in National Endometriosis Month celebration in different ways like – talking or engaging in conversation with others about endometriosis (whether that be family, friends, or coworkers), sharing facts about the disease through several social media platforms, participating in different events and sharing survivor stories (by publishing blogs, or through guest posting on others’ blogs or social media accounts). Join the 2019 National Endometriosis Month celebrations in March! Educate women about the need for identifying this hidden and often unrecognized hormonal condition at an early age.	3	2	0	0	0	2	0.952088	2	1,126
DSM V and ICD 11 - Concept and Evolution of ICD 10 Diagnostic and Statistical Manual of Mental Disorder (DSM) & International Classification of Diseases are repositories of classification codes in an alphanumeric format that is useful for correctly identifying diseases and other disorders. This HIPAA-approved system of classification is used by healthcare professionals, physiatrists, researchers, clinicians, and nurses to efficiently store, retrieve, and share diagnostic information. However, the disease classification handbook from ICD and DSM does not include information on treatment of any disorders or diseases. DSM was first published in 1952 while development on ICD began as early as 1893. The DSM is strictly confined to mental disorders while the ICD system is considered a global classification. Both the system is subject to expansion every 10 years. Therefore, DSM V and ICD 11 are the newest classifications to streamline diagnosis of illness. What are DSM and ICD? DSM and ICD are standardized criteria for classifying diseases. ICD is a classification approved by WHO for its member nations, while DSM is finalized by American Psychiatry Association (APA). The DSM is exclusive to psychiatric conditions, but ICD consists of codes for all illness and conditions. DSM is called a statistical manual because its antecedents were relied upon for collection of mortality data from hospitals. Here is a listicle of facts that broadly distinguishes DSM and ICD classifications - ICD is the WHO's official classification recognized internationally ICD can benefit all medical practitioners of WHO's member nations Special attention is given to primary care as well as low and middle-income countries Mainly aimed at clinical utility with a reduced number of diagnostics ICD does not provide operational criteria. However, it provides guidance and diagnostic descriptions DSM is a classification provided by APA. Although it is officially used in the USA, DSM is used by many countries worldwide Most commonly used by psychiatrists for diagnosis and treatment of mental disorders DSM is beneficial for high-income countries to assist in the secondary psychiatric care With each new revision, the number of diagnostics tends to increase the need for more diagnoses The diagnostic system uses the polythetic system to treat most conditions depending on operational criteria The Concept of ICD 10 World Health Organization (WHO) is the governing body behind the ICD codes. The code classifications are periodically updated to help the global healthcare industry to have a common language in understanding illness. The 10th revision of ICD came into effect on October 2015 after facing an extensive delay in its implementation. The WHO planned to release minor updates annually while major ones are slated to be rolled out once in three years. Purpose of ICD-10 and Its Usage 155,000 ICD-10 codes are widely used by healthcare industry professionals, coders, insurance companies, and other agencies to keep track of diseases and to speed up reimbursement. ICD-10 provides an accurate mapping from a multilingual classification of clinical terminology called SNOMED CT. The mapping from SNOMED CT to ICD-10 can facilitate computerized coding which is fast and accurate. The procedure codes in ICD-10 provides a detailed information of the procedure performed by a healthcare provider along with the medical device used in the procedure. Countries like New Zealand, Australia, and Canada uses a customized version of the ICD-10 that is adapted to their healthcare system. A newer version of ICD called ICD-11 was planned for endorsement in 2017, however, the presentation at the World Health Assembly (WHA) was rescheduled to May 2019. In June 2018, a preview of ICD-11 was released for adoption by WHO's member states. It is expected for the latest classification, which is a user-friendly and electronic format to come into effect by January 2022. This new classification is a quantum leap over ICD-10 because it covers the latest developments in medicine and includes an updated understanding of recent illnesses. When Can DSM-5 Be Used for Insurance Purposes? DSM-5 meshes perfectly with ICD-9-CM. Since the latter is a HIPAA compliant coding system it is used by insurance companies. However, some delay can occur while updating claims form because of the format change in DSM IV-TR as well as the slightly different reporting procedure to facilitate the changes in DSM V. What is the Importance of ICD-10? How do you benchmark the safety, efficiency, and quality of healthcare service? The answer is ICD-10 code set. It is a code repository that provides a detailed information for reimbursement, gauging the clinical outcome, claims processing, payment capture, and disease management. If you wish to do away with the risk of claims rejection, fraudulent claims, and non-payment then ICD-10 is the way to go. ICD-10 was recognized as a handy tool for entities that are covered and not covered by HIPAA regulations. The broadened understanding could streamline worker compensation and injury classification for benefit coordination. What is the Importance of DSM? DSM, currently in its 5th edition, is considered as a comprehensive handbook with the latest description, symptoms, and other information to help clinicians to classify and interpret psychological disorders. The current version is accorded by practitioners, healthcare experts, researchers, and other key professionals on the latest understanding of psychological disorders. It provides a common language and it is consistent throughout the manual. This would enable researchers and physicians to collaborate and discuss patient cases for accurate diagnosis. Future revisions (if any) can be made effortlessly by harnessing the database of treatment and diagnosis insights. Transition to ICD-10 The transition to ICD-10 from ICD-9 wasn't an easy one. The move faced stiff opposition from lobbyists, politicians, et al. Leading the opposition were physicians along with American Medical Association who feared that they would lose backing from other critical areas in order to make up for the cost-intensive process of ICD-10 implementation. ICD-11 and DSM V Being a reliable source of clinical documentation for record future diagnosis and completion of insurance formalities, there are similarities and differences of ICD-11 and DSM V. Similarities between ICD -11 and DSM V ICD and DSM Systems are similar in the following areas - Common Codes - Although the specificity of definition varies, the ICD and DSM have commonalities in codes. For instance, the social pragmatic communication disorder and alcohol use disorder are two conditions with the same codes in ICD and DSM classifications Code Classification - To process insurance and diagnose illness with maximum efficiency, ICD and DSM provide almost identical code sets. However, attenuated psychotic symptoms are not included in the classification of mental disorder Crosswalking in Code - The DSM and ICD classifications allow code crosswalking from the older format to present one. This is a process where old code set isn't entirely replaced but rather expanded to broaden the documentation Consensus Method - The ICD and DSM criteria are not concorded on the merits of clinical evidence, but a group of medical experts will collaborate and approve after reaching an agreement. Both systems take dimensional symptom assessment. DSM V and ICD-11 has brought genetic factors into a classification of psychological disorders Differences between ICD-11 and DSM V Despite having commonalities in many aspects, the ICD and DSM code sets differ in many ways. The difference between ICD and DSM are as follows - ICD codes are considered to be less accurate than DSM because it provides diagnostic criteria without operational criteria It is widely regarded as accurate due to clarity in description and operational criteria along with the clinical significance In ICD classification the diagnostic criteria are not based on independent validation. Hence, it slightly lags slightly behind the DSM code set With the introduction of operational criteria, it is considered by healthcare professionals as the most accurate classification ICD is used by member nations of WHO and it is widely used because of its advantages in diagnoses DSM classification is used by healthcare providers in the USA. Due to its practicality, it is adopted by psychologists of many other countries for research ICD code classification covers a wide range of illnesses including mental health disorders DSM is a statistical manual with code modifications that cover only the phycological conditions and illnesses WHO's member nations across low to middle-income bracket are beneficiaries of this code system DSM code system is adopted by countries that fall under a high-income category ICD-11 to Offer a Mountain of Positives in a Few Years In the view of active developments in the field of healthcare, a greater need is ever-present to update the system so that the diagnoses and treatment approach can be accelerated and streamlined to the present standards. WHO is gearing up to introduce ICD-11 in 2022 ergo, but all eyes are set on May 2019 because ICD-11 classification will be presented at World Health Assembly after being deferred multiple times. This new classification is believed to be medically focussed and capable of speeding up the evaluation process along with claims management. However, there are a few who firmly stand by the opinion that ICD-11 could face a string of challenge and delays before efficient adaptation because it took a decade for ICD-10 to develop clinical modifications. Outsource Medical Billing & Coding Services to Flatworld Solutions - The Leader in Healthcare BPO Flatworld Solutions is an ISO 9001: 2015 certified company with 15 years of experience in healthcare BPO solutions. We take ISO/IEC 27001:2013 certified security measures to ensure the confidentiality of the project and your client's clinical data. We handle an array of healthcare medical, coding, translation, and transcription projects with care and precision. We exploit the latest developments in coding classifications to speed up diagnosis, patient care, and insurance process. Our cost-effective services also come with quality support on a 24x7x365 basis. We have highly skilled coding and billing specialists who are adept at HIPAA compliant process and coding classifications. The services we provide include medical transcription, medical billing & coding, healthcare claims adjudication, and more. Contact Us to outsource your medical coding requirements. Our team will follow-up with a customized quote within 24 hours.	3	5	1	0	0	0	0.595179	1	2,108
Capnography During Sedation The line of demarcation between conscious sedation and general anesthesia is sometimes very thin. It is possible during conscious sedation, intravenous sedatives and narcotics administered to allay apprehension can result in the loss of consciousness and respiratory obstruction. If this is not recognized immediately, the patient can become hypoxic. In the majority of cases, the respiratory obstruction occurs well before the onset of hypoxia. Therefore monitoring of respiratory status is essential to enable corrective measures before the occurrence of hypoxia. Following illustration explains reinforces this concept . Pulse oximetry is a monitor of oxygenation Supplemental oxygen can mask apnea or hypoventilation A Preponderance of evidence suggests that procedural sedation is associated with undetected apnea or hypoventilation that can result in oxygen desaturation. Hence it is obvious that some type of ventilation monitoring is required to safeguard against hypoxia during sedation procedures outside of the operating room. To detect apnea or hypoventilation during procedural sedation, a specific monitor of ventilation is required. The value of capnography is well appreciated during anesthesia as an airway and ventilation monitor so much so that it has become a standard of practice in the operating room. It is only a matter of time that capnography will find its way to becoming incorporated as a standard of practice for monitoring ventilation during sedation procedures. Capnography has evolved into a standard of monitoring during anesthesia because it has proven itself to be a valuable tool in recognizing ventilatory and circulatory events that could potentially lead to deleterious effects. Hypoxia is our primary concern during anesthesia, and therefore, by all means, any situation that results in hypoxia is avoided. One of the greatest assets of capnography is that it can identify situations that can potentially result in hypoxia. Capnography serves as a warning device by instantly drawing the anesthesiologist's attention to the events that could potentially lead to hypoxia, if uncorrected. Because of this, care- providers have been encouraged to extend the benefit of capnography to areas beyond the domain of anesthesiologists. Examples include the use of capnography in the EMT / ambulance services for monitoring ventilation, and emergency medical rooms for procedures and sedation. Presently, several procedures are being performed under sedation outside of the operating room. Although the aim of the conscious sedation nurse/personnel is to provide conscious sedation, the line between conscious sedation and sleep is very narrow and the patient drifts quite often into an unconscious state. During this state of sleep, airway obstruction or hypoventilation may occur that may not be detected until hypoxia occurs as is indicated by pulse oximetry. Occasionally endoscopies performed via the upper airway can also result in airway obstruction. The delayed identification of airway problems leads to a delayed intervention. It is not uncommon for anesthesiologists to be called in urgently to intervene under these circumstances. Capnography could provide early warning in identifying such respiratory or airway problems in advance so that corrective intervention can be undertaken before hypoxia ensues. Policymakers who understand the potential benefits and strides made in the operating room by capnography, logically recommend and extend its use to areas beyond the operating room to enhance the safety of patients undergoing sedation procedures. Capnography is routinely used in patients undergoing cardiac electrophysiology studies and ablation, ICD placement and testing at our institution. Others look for evidence to determine if capnography has truly any beneficial role to play in sedation procedures outside of the operating room. The value of capnography was recognized in the operating room settings prior to being adopted as standard of monitoring during anesthesia procedures. Similarly, several recent studies have shown the benefit of capnography in identifying events that can potentially lead to hypoxia during procedural sedation. This could be a precursor for capnography being accepted as standard of care during sedation procedures in the near future. Vargo et al studied forty-nine patients undergoing therapeutic therapeutic upper endoscopy, who were monitored with standard methods including pulse oximetry, automated blood pressure measurement, and visual assessment. In addition, graphic assessment of respiratory activity with side-stream capnography was performed in all patients. Endoscopy personnel were blinded to capnography data. Episodes of apnea (cessation of respiration for 30 or more seconds) or disordered respiration (45 second interval that contained at least 30 seconds of cumulative apneic activity) detected by capnography were documented and compared with the occurrences of hypoxemia, hypercapnea, hypotension, and the recognition of abnormal respiratory activity by endoscopy personnel. Capnography identified 54 events of apnea or disordered respiratory events during the endoscopic procedure, whereas, pulse oximetry picked up only 27 events (50%) where the respiratory events that were detected by capnography progressed to result in hypoxia (as defined by oxygen saturation below 90%). Hypoxemia occurred approximately 45.6 seconds (15-120 seconds) after the capnographic detection of respiratory events. Visual inspection of the patient by the care providers detected none of the 54 events detected by capnography. Capnography also detected hypoventilation as shown by a 25% increase from baseline end expiratory carbon dioxide values. Minor et al prospectively studied whether end-tidal carbon dioxide (ETCO2) monitors can detect respiratory depression (RD) and the level of sedation in emergency department (ED) patients undergoing procedural sedation. This was a prospective observational study conducted in an urban county hospital of adult patients undergoing procedural sedation. Patients were monitored for vital signs, depth of sedation per the physician by the Observer's Assessment of Alertness/Sedation scale (OAA/S), pulse oximetry, and nasal-sample ETCO2CO2 during procedural sedation. Respiratory depression was defined as an oxygen saturation <90%, an ETCO2 >50 mm Hg, or an absent ETCO2 waveform at any time during the procedure. The physician also determined whether protective airway reflexes were lost during the procedure and assisted ventilation was required, or whether there were any other complications. Seventy-four patients were enrolled in the study. Forty (54.1%) received methohexital, 21 (28.4%) received propofol, ten (13.5%) received fentanyl and midazolam, and three (4.1%) received etomidate. Respiratory depression was seen in 33 (44.6%) patients, including 47.5% of patients receiving methohexital, 19% receiving propofol (p = 0.008), 80% receiving fentanyl and midazolam, and 66.6% receiving etomidate. No correlation between OAA/S and ETCO2 was detected. Eleven (14.9%) patients required assisted ventilation at some point during the procedure, all of whom met the criteria for RD. Pulse oximetry detected 11 of the 33 patients with RD. Using the criteria of an ETCO2 >50 mm Hg, an absolute change >10 mm Hg, or an absent waveform may detect subclinical RD not detected by pulse oximetry alone. The authors concluded that the ETCO2 may add to the safety of procedural safety by quickly detecting hypoventilation during sedation in the Emergency department. McQuillen and steele described ETCO2 changes associated with different sedation strategies. This was a prospective, observational patient series in an urban pediatric emergency department (PED). Participants included 106 children with a mean age of 6.8 years. (range 1.2-16.6 years). Sedation/analgesia was given for fracture reduction (55%), laceration repair (37%), abscess incision and drainage (4%), and lumbar puncture (LP) (4%). Medications included fentanyl, morphine, ketamine, and midazolam. Continuous ETCO2 waveforms were recorded via a Capnogard ETCO2 Monitor. Oxygen saturation was recorded using a Nelcor N-200 pulse oximeter. Recording began prior to sedation and continued until the patient was awake or when it was necessary to remove the patient from the monitor for further medical care. Each record was analyzed for peak ETCO2 and averaged over five consecutive breaths, before and after the administration of medications. The main outcome measure was the change in ETCO2 levels. The mean increase in ETCO2 was 6.7 mmHg (P is included in, 0.00001; range: +0.16 to +22.3). ETCO2 increased by 3.2 mmHg (95% CI = 2.2-4.2) for midazolam alone, 5.4 mmHg (95% CI = 4.5-6.4) for midazolam and ketamine, and 8.8 mmHg (95% CI = 7.4-10.2) for midazolam and opiate. Two patients had transient SpO2 desaturations below 93%, which corrected with stimulation. The authors concluded that commonly used agents for pediatric sedation result in significant increases in ETCO2. ETCO2 is a useful adjunct in assessing ventilation and may serve as an objective research tool for assessing different sedation strategies. Tobias in his study used both end-tidal carbon dioxide (ETCO2) monitoring and pulse oximetry to evaluate the respiratory effects of revised midazolam and ketamine protocol. Fifty children who required sedation during invasive procedures formed the cohort for the study. During sedation, ETCO2 was sampled from nasal cannulae of spontaneously breathing patients and measured by a side-stream aspirating infrared device. During the procedure, O2 saturation decreased by 3% or more in three patients. Supplemental oxygen at 2 liters per minute was administered to these patients. The lowest oxygen saturation was 84%. During the total of 767 minutes of monitoring, there were 3068 ETCO2 values recorded. The high ETCO2 values ranged from 37 to 53 mmHg (40.5 +/- 3.3 mmHg). Ninety percent, or 2760, of the values were 40 mmHg or less, 7% or 214 were between 41 and 45 mmHg, 3% or 92 were between 46 and 49 mmHg, and 2 isolated values were greater than 50 mmHg. One episode of airway obstruction was identified by noting cessation of the ETCO2 waveform. This was relieved by repositioning the patient's airway. The three episodes of O2 desaturation, two ETCO2 values greater than 50 mmHg, and the episode of upper airway obstruction all occurred in three patients. Two of these patients had trisomy 21 with macroglossia, and the third had had a recent upper respiratory infection and a history of tonsillar hypertrophy. The incidence of adverse cardiorespiratory events associated with the current sedation regimen of midazolam-ketamine is lower than that reported with other commonly used regimens. The addition of ETCO2 monitoring provides an additional monitor to allow for early detection of airway obstruction or subclinical degrees of respiratory depression. Hart et al state that many studies have evaluated conscious sedation regimens commonly used in pediatric patients and recent advances in capnography equipment now enable physicians to assess respiratory parameters, specifically end-tidal CO2 (et-CO2), more accurately in spontaneously breathing sedated children than was possible in the earlier studies. They designed the study to: 1) compare the safety and efficacy of intravenous fentanyl, intravenous fentanyl combined with midazolam, and intramuscular meperidine-promethazine-chlorpromazine (MPC) compound when used for painful emergency department (ED) procedures: and 2) to determine whether the addition of et-CO2 monitoring enabled earlier identification of respiratory depression in this population. Forty-two children requiring analgesia and sedation for painful ED procedures were randomly assigned to receive either fentanyl, fentanyl-midazolam, or MPC compound. Vital signs, oxygen saturation, and et-CO2 were monitored continuously. Pain, anxiety, and sedation scores were recorded every five minutes. Respiratory depression (O2 saturation < or = 90% for over the minute or any et-CO2 > or = 50) occurred in 20% of fentanyl, 23% of fentanyl-midazolam, and 11% of MPC patients (P = NS). Of those patients manifesting respiratory depression, 6/8 were detected by increased et-CO2 only. MPC patients required significantly longer periods of time to meet discharge criteria than fentanyl and fentanyl-midazolam patients (P < 0.05). No differences were noted in peak pain, anxiety, or sedation scores. The authors concluded that Fentanyl, fentanyl-midazolam, and MPC produced a high incidence of subclinical respiratory depression. End-tidal CO2 monitoring provided an earlier indication of respiratory depression than pulse oximetry and respiratory rate alone. MPC administration resulted in a significantly delayed discharge from the ED. All the above studies are unanimous in that airway compromise and hypoventilation events do occur during procedural sedation and capnography identifies these events earlier than pulse oximetry. Thus, capnography serves as an early warning device of an impending hypoxia. If anesthesiologists are involved in laying out guidelines for non-operating-room locations, they are more likely to include capnography to monitor ventilation. This is probably because, by nature, anesthesiologists are trained to think about airway and ventilation at all times. This is also the reason why our cardiac electrophysiology/ablation rooms and minor Obstetric procedure rooms are provided with capnography as we oversee the sedation and anesthesia care here. The American Society of Anesthesiology in their document on "Practice Guidelines for Sedation and Analegesia by Non-Anesthesiologists" under the section 'Pulmonary ventilation' (http://www.asahq.org/publicationsAndServices/practiceparam.htm) states as follows: "It is the opinion of the Task Force that the primary causes of morbidity associated with sedation/analgesia are drug-induced respiratory depression and airway obstruction. For both moderate and deep sedation, the literature is insufficient to evaluate the benefit of monitoring ventilatory function by observation or auscultation. However, the consultants strongly agree that monitoring of ventilatory function by observation or auscultation reduces the risk of adverse outcomes associated with sedation/analgesia. The consultants were equivocal regarding the ability of capnography to decrease risks during moderate sedation, while agreeing that it may decrease risks during deep sedation. In circumstances where patients are physically separated from the caregiver, the Task Force believes that automated apnea monitoring (by detection of exhaled CO2 or other means) may decrease risks during both moderate and deep sedation, while cautioning practitioners that impedance plethysmography may fail to detect airway obstruction. The Task Force emphasizes that because ventilation and oxygenation are separate though related physiological processes, monitoring oxygenation by pulse oximetry is not a substitute for monitoring ventilation." Therefore, there are recommendations to monitor ventilation during procedural sedation 'particularly' if deep, but not a standard. Yet, it is up to the care-providers to decide how and what method is best for their practicing environment. Certainly capnography provides an early warning device to draw attention to abnormal ventilation. No doubt, capnography has limitations, particularly in nasal sampling. This limitation should be understood. However, there are devices to improve sampling of carbon dioxide in expired air and thus decrease artifacts. I generally get a baseline waveform and ETCO2 values, and focus attention to any changes from the baseline values. Thinking, always in my mind that any changes in ETCO2 from the baseline are due to depression of ventilation or airway obstruction until otherwise proven by close examination. In my opinion, a decrease in pulse oximetry is a late finding, and I would use other methods to identify events before they have had a chance to produce hypoxia. In conclusion, capnography seems to be a logical device to monitor ventilation during procedural sedation. This is because (a) The general consensus among anesthesiologists is that airway problems are primary causes of morbidity associated with sedation/analgesia are drug-induced respiratory depression and airway obstruction; (b) Available studies on procedural sedation confirm that respiratory events precede hypoxia, and capnography is a valuable monitoring device to detect respiratory events that could culminate in hypoxia; (c) Sedation procedures are being performed more often by non-anesthesiologists at remote locations from the 'OR' (operating room). They may not be as well experienced and adept as anesthesiologists in recognizing airway obstruction, apnea and hypoventilation; (d) Endoscopic physicians are preferring to use hypnotic agents such as propofol for quick recovery and discharge to reduce health care cost. These agents can result in respiratory depression and apnea. Just like the evolution of capnography in the operating room from a guideline status to the status of standard of practice, it is only a question of time that capnography will be accepted as a standard of care to enhance patient safety during procedural sedation. 1. Vargo JJ, Zuccaro G, Dumont JA, Conwell DL, Morrow JB, Shay SS. Automated graphic assessment of respiratory activity is superior to pulse oximetry and visual assessment of the detection of early respiratory depression during therapeutic upper endoscopy. Gastrointestinal Endoscopy 2002;55:826-31. 2. Miner JR, Heegaard W, Plummer D. End-tidal carbon dioxide monitoring during procedural sedation. Acad Emerg Med 2002;9:275-80. 3. McQuillen KK, Steele DW. Capnography during sedation/analgesia in the pediatric emergency department. Pediatr Emerg Care 2000;16:401-4. 4. Tobias JD. End-tidal carbon dioxide monitoring during sedation with a combination of midazolam and ketamine for children undergoing painful, invasive procedures. Pediatr Emerg Care 1999;15:173-5. 5. Hart LS, Berns SD, Houck CS, Boenning DA. The value of end-tidal CO2 monitoring when comparing three methods of conscious sedation for children undergoing painful procedures in the emergency department. Pediatr Emeg Care 1997;13:189-93. 6. Practice Guidelines For Sedation And Analgesia By Non-Anesthesiologists (Approved by the House of Delegates on October 25, 1995, and last amended on October 17, 2001).	2	19	1	0	0	0	0.827896	1	3,996
Diagnosis Code D3A.024 Information for Medical Professionals The diagnosis code D3A.024 is grouped in the following Diagnostic Related Group(s) (MS-DRG V35.0) - 393 - OTHER DIGESTIVE SYSTEM DIAGNOSES WITH MCC - 394 - OTHER DIGESTIVE SYSTEM DIAGNOSES WITH CC - 395 - OTHER DIGESTIVE SYSTEM DIAGNOSES WITHOUT CC/MCC Convert to ICD-9 - 209.55 - Ben carcinoid desc colon - Benign carcinoid tumor of descending colon - Benign neoplasm of descending colon Index to Diseases and Injuries References found for the code D3A.024 in the Index to Diseases and Injuries: - - Tumor - See Also: Neoplasm, unspecified behavior, by site; Information for Patients Also called: Benign cancer, Benign neoplasms, Noncancerous tumors Tumors are abnormal growths in your body. They can be either benign or malignant. Benign tumors aren't cancer. Malignant ones are. Benign tumors grow only in one place. They cannot spread or invade other parts of your body. Even so, they can be dangerous if they press on vital organs, such as your brain. Tumors are made up of extra cells. Normally, cells grow and divide to form new cells as your body needs them. When cells grow old, they die, and new cells take their place. Sometimes, this process goes wrong. New cells form when your body does not need them, and old cells do not die when they should. These extra cells can divide without stopping and may form tumor. Treatment often involves surgery. Benign tumors usually don't grow back. NIH: National Cancer Institute - Biopsy - polyps (Medical Encyclopedia) - Cherry angioma (Medical Encyclopedia) Carcinoid tumors are rare, slow-growing cancers. They usually start in the lining of the digestive tract or in the lungs. They grow slowly and don't produce symptoms in the early stages. As a result, the average age of people diagnosed with digestive or lung carcinoids is about 60. In later stages the tumors sometimes produce hormones that can cause carcinoid syndrome. The syndrome causes flushing of the face and upper chest, diarrhea, and trouble breathing. Surgery is the main treatment for carcinoid tumors. If they haven't spread to other parts of the body, surgery can cure the cancer. - 5-HIAA (Medical Encyclopedia) - Carcinoid syndrome (Medical Encyclopedia) - Serum serotonin level (Medical Encyclopedia) Also called: Large intestine diseases Your colon, also known as the large intestine, is part of your digestive system. It's a long, hollow tube at the end of your digestive tract where your body makes and stores stool. Many disorders affect the colon's ability to work properly. Some of these include - Colorectal cancer - Colonic polyps - extra tissue growing in the colon that can become cancerous - Ulcerative colitis - ulcers of the colon and rectum - Diverticulitis - inflammation or infection of pouches in the colon - Irritable bowel syndrome - an uncomfortable condition causing abdominal cramping and other symptoms Treatment for colonic diseases varies greatly depending on the disease and its severity. Treatment may involve diet, medicines and in some cases, surgery. NIH: National Institute of Diabetes and Digestive and Kidney Diseases - Angiodysplasia of the colon (Medical Encyclopedia) - Colitis (Medical Encyclopedia) - Colonoscopy (Medical Encyclopedia) - Hirschsprung disease (Medical Encyclopedia) - Intestinal ischemia and infarction (Medical Encyclopedia) - Large bowel resection (Medical Encyclopedia) - Lower GI Series - NIH (National Institute of Diabetes and Digestive and Kidney Diseases) General Equivalence Map Definitions The ICD-10 and ICD-9 GEMs are used to facilitate linking between the diagnosis codes in ICD-9-CM and the new ICD-10-CM code set. The GEMs are the raw material from which providers, health information vendors and payers can derive specific applied mappings to meet their needs. - Approximate Flag - The approximate flag is on, indicating that the relationship between the code in the source system and the code in the target system is an approximate equivalent. - No Map Flag - The no map flag indicates that a code in the source system is not linked to any code in the target system. - Combination Flag - The combination flag indicates that more than one code in the target system is required to satisfy the full equivalent meaning of a code in the source system. Present on Admission The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement.	4	3	0	0	0	2	0.407395	2	1,093
Heart Disease Icd 10! Never Underestimate! But why? Is it a serious problem? Let us know in this article in an easy way. We here will know what is a heart disease icd 10? How does it work? Who needs? What is heart disease icd 10? Also known as implantable cardioverter-defibrillator, and icd 10 is a small battery-powered device that is placed inside your chest to monitor your heart rhythm and detect irregular heartbeats. It can deliver electric shocks via one or more wires connected to your heart to fix an abnormal heart rhythm. You will be needing an ICD 10 if you have an uncontrollably fast heartbeat or also known as ventricular tachycardia that keeps your heart from supplying enough blood to the rest of your body or this phenomenon known as ventricular fibrillation. Ventricles are present in the lower chambers of your heart. Must read, which blood lipid is linked most directly to heart disease? For a heart disease icd 10 conditions, there are different problems related like atrial fibrillation and flutter, cardiac arrhythmias, chest pain, heart failure, hypertension, etc. How it works for icd 10? When you have a rapidly fast heartbeat, the wires present in your heart to the device transmit signals to the ICD 10, which then sends electrical pulses to regulate your heartbeat. ICD 10 is programmed for: It may be seen that patients may feel nothing or a painless fluttering in their chest, then the ICD 10 responds to mild disruptions in your heartbeat. A higher-energy shock: For having more serious heart rhythm problems, the ICD 10 may deliver a higher-energy shock which can be painful. The pain usually lasts only a second, and there shouldn’t be discomfort after the shock ends. Usually, only one shock is needed to restore a normal heartbeat but sometimes, you might have two or more shocks during a 24-hour period. The process of having three or more shocks in a short time period is known as an electrical or arrhythmia storm. If you are experiencing an electrical storm, you should seek emergency care to see if your ICD 10 is working properly and check if you are having a problem that’s making your heartbeat abnormally. Who need as icd 10? You are a victim of a valvular heart disease icd 10 if you’ve had experienced ventricular tachycardia, survived a cardiac arrest, or even fainted from a ventricular arrhythmia. You may also experience an ICD 10 if you have- - A history of coronary artery disease and heart attack has lead to the weakening of your heart. - A heart condition that involves abnormal heart muscle, like enlarged or thickened heart muscle. - An inherited heart defect that makes the heartbeat abnormally. - Other rare conditions that affect your heart rhythm. How to prepare for valvular heart disease icd 10? To determine whether you need a hypertensive heart disease icd 10, your doctor might perform a variety of diagnostic tests which includes- This test uses sensor pads with wires attached or sometimes electrodes placed on your body to measure your heart’s electrical impulses and then the heart’s beating pattern gives an idea of the defect. This test uses harmless sound waves that allow the doctor to see the insides of the heart without making cuts. The images show how well your heart is functioning and the basic information like the size and thickness of your heart muscle. Electrophysiology study (EPS)- In this method, the electrodes are guided through blood vessels to the heart and used to test the functioning of the heart’s electrical system. This can identify whether someone might have developed heart rhythm problems. Sometimes doctors might ask to wear a pager-sized device that records the activities of the heart for more than 24 hours. Unlike a Holter monitor, it doesn’t operate continuously, rather you have to turn it on when you feel your heart is beating abnormally. icd 10 family history of heart disease If we talk about icd 10 family history of heart disease, Z82.49 is a billable code used to indicate a clinical conclusion of the family background of heart disease. The code is legitimate for the financial year 2021 for the accommodation of HIPAA-covered exchanges. The ICD-10-CM code Z82.49 may likewise be utilized to determine conditions or terms like the family background of an intense clinical problem, icd 10 family history of heart disease. The code is excluded from the present on confirmation revealing for inpatient admissions to general intense consideration medical clinics. Read also, carcinoid heart disease, All you need to know! The code Z82.49 portrays a situation that impacts the patient’s wellbeing status yet not a current ailment or injury. The code is unsatisfactory as a chief analysis. icd 10 code for hypertensive heart disease According to ICD 10 data, icd 10 code for hypertensive heart disease are as follows: - I11.0 is a billable/explicit ICD-10-CM code that can be utilized to demonstrate an analysis for repayment purposes. - The 2021 release of ICD-10-CM I11.0 got viable on October 1, 2020. - This is the American ICD-10-CM rendition of I11.0 – other worldwide adaptations of ICD-10 I11.0 may vary. It is applicable to hypertensive heart disease icd 10. Mainly icd 10 code is used to identify heart failure type. Below are the icd 10 code for hypertensive heart disease code history: - 2016 (effective 10- January-2015): New code (first year of non-draft ICD-10-CM) - 2017 (effective 10-January-2016): No change - 2018 (effective 10-January-2017): No change - 2019 (effective 10-January-2018): No change - 2020 (effective 10-January-2019): No change - 2021 (effective 10-January-2020): No change Thanks for reading this article. You can also read SOME PREVENTIVE MEASURES TO KEEP OUR HEART HEALTHY.	4	4	0	0	0	11	0.941437	11	1,309
Sepsis is a life-threatening condition that arises when the body's response to infection causes injury to its own tissues and organs. This initial stage is followed by suppression of the immune system. Common signs and symptoms include fever, increased heart rate, increased breathing rate, and confusion. There may also be symptoms related to a specific infection, such as a cough with pneumonia, or painful urination with a kidney infection. The very young, old, and people with a weakened immune system may have no symptoms of a specific infection, and the body temperature may be low or normal instead of having a fever. Severe sepsis causes poor organ function or blood flow. The presence of low blood pressure, high blood lactate, or low urine output may suggest poor blood flow. Septic shock is low blood pressure due to sepsis that does not improve after fluid replacement. Sepsis is caused by many organisms including bacteria, viruses and fungi. Common locations for the primary infection include the lungs, brain, urinary tract, skin, and abdominal organs. Risk factors include being very young or old, a weakened immune system from conditions such as cancer or diabetes, major trauma, and burns. Previously, a sepsis diagnosis required the presence of at least two systemic inflammatory response syndrome (SIRS) criteria in the setting of presumed infection. In 2016, a shortened sequential organ failure assessment score (SOFA score), known as the quick SOFA score (qSOFA), replaced the SIRS system of diagnosis. qSOFA criteria for sepsis include at least two of the following three: increased breathing rate, change in the level of consciousness, and low blood pressure. Sepsis guidelines recommend obtaining blood cultures before starting antibiotics; however, the diagnosis does not require the blood to be infected. Medical imaging is helpful when looking for the possible location of the infection. Other potential causes of similar signs and symptoms include anaphylaxis, adrenal insufficiency, low blood volume, heart failure, and pulmonary embolism. Sepsis requires immediate treatment with intravenous fluids and antimicrobials. Ongoing care often continues in an intensive care unit. If an adequate trial of fluid replacement is not enough to maintain blood pressure, then the use of medications that raise blood pressure becomes necessary. Mechanical ventilation and dialysis may be needed to support the function of the lungs and kidneys, respectively. A central venous catheter and an arterial catheter may be placed for access to the bloodstream and to guide treatment. Other helpful measurements include cardiac output and superior vena cava oxygen saturation. People with sepsis need preventive measures for deep vein thrombosis, stress ulcers, and pressure ulcers unless other conditions prevent such interventions. Some people might benefit from tight control of blood sugar levels with insulin. The use of corticosteroids is controversial, with some reviews finding benefit, and others not. Disease severity partly determines the outcome. The risk of death from sepsis is as high as 30%, while for severe sepsis it is as high as 50%, and septic shock 80%. Sepsis affected about 49 million people in 2017, with 11 million deaths (1 in 5 deaths worldwide). In the developed world, approximately 0.2 to 3 people per 1000 are affected by sepsis yearly, resulting in about a million cases per year in the United States. Rates of disease have been increasing. Sepsis is more common among males than females. However, other data show a greater prevalence of the disease among women. Descriptions of sepsis date back to the time of Hippocrates. The terms "septicemia" and "blood poisoning" have been used in various ways and are no longer recommended. Signs and symptoms In addition to symptoms related to the actual cause, people with sepsis may have a fever, low body temperature, rapid breathing, a fast heart rate, confusion, and edema. Early signs include a rapid heart rate, decreased urination, and high blood sugar. Signs of established sepsis include confusion, metabolic acidosis (which may be accompanied by a faster breathing rate that leads to respiratory alkalosis), low blood pressure due to decreased systemic vascular resistance, higher cardiac output, and disorders in blood-clotting that may lead to organ failure. Fever is the most common presenting symptom in sepsis, but fever may be absent in some people such as the elderly or those who are immunocompromised. Oxidative stress is observed in septic shock, with circulating levels of copper and vitamin C being decreased. Infections leading to sepsis are usually bacterial but may be fungal, parasitic or viral. Gram-positive bacteria were the primary cause of sepsis before the introduction of antibiotics in the 1950s. After the introduction of antibiotics, gram-negative bacteria became the predominant cause of sepsis from the 1960s to the 1980s. After the 1980s, gram-positive bacteria, most commonly staphylococci, are thought to cause more than 50% of cases of sepsis. Other commonly implicated bacteria include Streptococcus pyogenes, Escherichia coli, Pseudomonas aeruginosa, and Klebsiella species. Fungal sepsis accounts for approximately 5% of severe sepsis and septic shock cases; the most common cause of fungal sepsis is an infection by Candida species of yeast, a frequent hospital-acquired infection. The most common causes for parasitic sepsis are Plasmodium (which leads to malaria), Schistosoma and Echinococcus. The most common sites of infection resulting in severe sepsis are the lungs, the abdomen, and the urinary tract. Typically, 50% of all sepsis cases start as an infection in the lungs. In one-third to one-half of cases, the source of infection is unclear. Early diagnosis is necessary to properly manage sepsis, as the initiation of rapid therapy is key to reducing deaths from severe sepsis. Some hospitals use alerts generated from electronic health records to bring attention to potential cases as early as possible. Within the first three hours of suspected sepsis, diagnostic studies should include white blood cell counts, measuring serum lactate, and obtaining appropriate cultures before starting antibiotics, so long as this does not delay their use by more than 45 minutes. To identify the causative organism(s), at least two sets of blood cultures using bottles with media for aerobic and anaerobic organisms are necessary. At least one should be drawn through the skin and one through each vascular access device (such as an IV catheter) that has been in place more than 48 hours. Bacteria are present in the blood in only about 30% of cases. Another possible method of detection is by polymerase chain reaction. If other sources of infection are suspected, cultures of these sources, such as urine, cerebrospinal fluid, wounds, or respiratory secretions, also should be obtained, as long as this does not delay the use of antibiotics. Within six hours, if blood pressure remains low despite initial fluid resuscitation of 30 ml/kg, or if initial lactate is ≥ four mmol/l (36 mg/dl), central venous pressure and central venous oxygen saturation should be measured. Lactate should be re-measured if the initial lactate was elevated. Evidence for point of care lactate measurement over usual methods of measurement, however, is poor. Within twelve hours, it is essential to diagnose or exclude any source of infection that would require emergent source control, such as a necrotizing soft tissue infection, an infection causing inflammation of the abdominal cavity lining, an infection of the bile duct, or an intestinal infarction. A pierced internal organ (free air on an abdominal X-ray or CT scan), an abnormal chest X-ray consistent with pneumonia (with focal opacification), or petechiae, purpura, or purpura fulminans may indicate the presence of an infection. \|Temperature\|\|<36 °C (96.8 °F) or >38 °C (100.4 °F)\| \|Respiratory rate\|\|>20/min or PaCO2<32 mmHg (4.3 kPa)\| \|WBC\|\|<4x109/L (<4000/mm3), >12x109/L (>12,000/mm3), or ≥10% bands\| Previously, SIRS criteria had been used to define sepsis. If the SIRS criteria are negative, it is very unlikely the person has sepsis; if it is positive, there is just a moderate probability that the person has sepsis. According to SIRS, there were different levels of sepsis: sepsis, severe sepsis, and septic shock. The definition of SIRS is shown below: - SIRS is the presence of two or more of the following: abnormal body temperature, heart rate, respiratory rate, or blood gas, and white blood cell count. - Sepsis is defined as SIRS in response to an infectious process. - Severe sepsis is defined as sepsis with sepsis-induced organ dysfunction or tissue hypoperfusion (manifesting as hypotension, elevated lactate, or decreased urine output). Severe sepsis is an infectious disease state associated with multiple organ dysfunction syndrome (MODS) - Septic shock is severe sepsis plus persistently low blood pressure, despite the administration of intravenous fluids. In 2016 a new consensus was reached to replace screening by systemic inflammatory response syndrome (SIRS) with the sequential organ failure assessment (SOFA score) and the abbreviated version (qSOFA). The three criteria for the qSOFA score include a respiratory rate greater than or equal to 22 breaths per minute, systolic blood pressure 100 mmHg or less and altered mental status. Sepsis is suspected when 2 of the qSOFA criteria are met. The SOFA score was intended to be used in the intensive care unit (ICU) where it is administered upon admission to the ICU and then repeated every 48 hours, whereas the qSOFA could be used outside the ICU. Some advantages of the qSOFA score are that it can be administered quickly and does not require labs. However, the American College of Chest Physicians (CHEST) raised concerns that qSOFA and SOFA criteria may lead to delayed diagnosis of serious infection, leading to delayed treatment. Although SIRS criteria can be too sensitive and not specific enough in identifying sepsis, SOFA also has its limitations and is not intended to replace the SIRS definition. qSOFA has also been found to be poorly sensitive though decently specific for the risk of death with SIRS possibly better for screening. - Lungs: acute respiratory distress syndrome (ARDS) (PaO2/FiO2 ratio < 300), different ratio in pediatric acute respiratory distress syndrome - Brain: encephalopathy symptoms including agitation, confusion, coma; causes may include ischemia, bleeding, formation of blood clots in small blood vessels, microabscesses, multifocal necrotizing leukoencephalopathy - Liver: disruption of protein synthetic function manifests acutely as progressive disruption of blood clotting due to an inability to synthesize clotting factors and disruption of metabolic functions leads to impaired bilirubin metabolism, resulting in elevated unconjugated serum bilirubin levels - Kidney: low urine output or no urine output, electrolyte abnormalities, or volume overload - Heart: systolic and diastolic heart failure, likely due to chemical signals that depress myocyte function, cellular damage, manifest as a troponin leak (although not necessarily ischemic in nature) More specific definitions of end-organ dysfunction exist for SIRS in pediatrics. - Cardiovascular dysfunction (after fluid resuscitation with at least 40 ml/kg of crystalloid) - hypotension with blood pressure < 5th percentile for age or systolic blood pressure < 2 standard deviations below normal for age, or - vasopressor requirement, or - two of the following criteria: - Respiratory dysfunction (in the absence of a cyanotic heart defect or a known chronic respiratory disease) - the ratio of the arterial partial-pressure of oxygen to the fraction of oxygen in the gases inspired (PaO2/FiO2) < 300 (the definition of acute lung injury), or - arterial partial-pressure of carbon dioxide (PaCO2) > 65 torr (20 mmHg) over baseline PaCO2 (evidence of hypercapnic respiratory failure), or - supplemental oxygen requirement of greater than FiO2 0.5 to maintain oxygen saturation ≥ 92% - Neurologic dysfunction - Hematologic dysfunction - Kidney dysfunction - Liver dysfunction (only applicable to infants > 1 month) Consensus definitions, however, continue to evolve, with the latest expanding the list of signs and symptoms of sepsis to reflect clinical bedside experience. Biomarkers can help diagnosis because they can point to the presence or severity of sepsis, although their exact role in the management of sepsis remains undefined. A 2013 review concluded moderate-quality evidence exists to support the use of the procalcitonin level as a method to distinguish sepsis from non-infectious causes of SIRS. The same review found the sensitivity of the test to be 77% and the specificity to be 79%. The authors suggested that procalcitonin may serve as a helpful diagnostic marker for sepsis, but cautioned that its level alone does not definitively make the diagnosis. A 2012 systematic review found that soluble urokinase-type plasminogen activator receptor (SuPAR) is a nonspecific marker of inflammation and does not accurately diagnose sepsis. This same review concluded, however, that SuPAR has prognostic value, as higher SuPAR levels are associated with an increased rate of death in those with sepsis. Serial measurement of lactate levels (approximately every 4 to 6 hours) may guide treatment and is associated with lower mortality in sepsis. The differential diagnosis for sepsis is broad and has to examine (to exclude) the non-infectious conditions that may cause the systemic signs of SIRS: alcohol withdrawal, acute pancreatitis, burns, pulmonary embolism, thyrotoxicosis, anaphylaxis, adrenal insufficiency, and neurogenic shock. Hyperinflammatory syndromes such as hemophagocytic lymphohistiocytosis (HLH) may have similar symptoms and are on the differential diagnosis. In common clinical usage, neonatal sepsis refers to a bacterial blood stream infection in the first month of life, such as meningitis, pneumonia, pyelonephritis, or gastroenteritis, but neonatal sepsis also may be due to infection with fungi, viruses, or parasites. Criteria with regard to hemodynamic compromise or respiratory failure are not useful because they present too late for intervention. This section needs expansion with: Viral sepsis. You can help by adding to it. (March 2020) Sepsis is caused by a combination of factors related to the particular invading pathogen(s) and to the status of the immune system of the host. The early phase of sepsis characterized by excessive inflammation (sometimes resulting in a cytokine storm) may be followed by a prolonged period of decreased functioning of the immune system. Either of these phases may prove fatal. On the other hand, systemic inflammatory response syndrome (SIRS) occurs in people without the presence of infection, for example, in those with burns, polytrauma, or the initial state in pancreatitis and chemical pneumonitis. However, sepsis also causes similar response to SIRS. Bacterial virulence factors, such as glycocalyx and various adhesins, allow colonization, immune evasion, and establishment of disease in the host. Sepsis caused by gram-negative bacteria is thought to be largely due to a response by the host to the lipid A component of lipopolysaccharide, also called endotoxin. Sepsis caused by gram-positive bacteria may result from an immunological response to cell wall lipoteichoic acid. Bacterial exotoxins that act as superantigens also may cause sepsis. Superantigens simultaneously bind major histocompatibility complex and T-cell receptors in the absence of antigen presentation. This forced receptor interaction induces the production of pro-inflammatory chemical signals (cytokines) by T-cells. There are a number of microbial factors that may cause the typical septic inflammatory cascade. An invading pathogen is recognized by its pathogen-associated molecular patterns (PAMPs). Examples of PAMPs include lipopolysaccharides and flagellin in gram-negative bacteria, muramyl dipeptide in the peptidoglycan of the gram-positive bacterial cell wall, and CpG bacterial DNA. These PAMPs are recognized by the pattern recognition receptors (PRRs) of the innate immune system, which may be membrane-bound or cytosolic. There are four families of PRRs: the toll-like receptors, the C-type lectin receptors, the NOD-like receptors, and the RIG-I-like receptors. Invariably, the association of a PAMP and a PRR will cause a series of intracellular signalling cascades. Consequentially, transcription factors such as nuclear factor-kappa B and activator protein-1, will up-regulate the expression of pro-inflammatory and anti-inflammatory cytokines. Upon detection of microbial antigens, the host systemic immune system is activated. Immune cells not only recognise pathogen-associated molecular patterns but also damage-associated molecular patterns from damaged tissues. An uncontrolled immune response is then activated because leukocytes are not recruited to the specific site of infection, but instead they are recruited all over the body. Then, an immunosuppression state ensues when the proinflammatory T helper cell 1 (TH1) is shifted to TH2, mediated by interleukin 10, which is known as "compensatory anti-inflammatory response syndrome". The apoptosis (cell death) of lymphocytes further worsens the immunosuppression. Neutrophils, monocytes, macrophages, dendritic cells, CD4+ T cells, and B cells all undergo apoptosis, whereas regulatory T cells are more apoptosis resistant. Subsequently, multiple organ failure ensues because tissues are unable to use oxygen efficiently due to inhibition of cytochrome c oxidase. Inflammatory responses cause multiple organ dysfunction syndrome through various mechanisms as described below. Increased permeability of the lung vessels causes leaking of fluids into alveoli, which results in pulmonary edema and acute respiratory distress syndrome (ARDS). Impaired utilization of oxygen in the liver impairs bile salt transport, causing jaundice (yellowish discoloration of the skin). In kidneys, inadequate oxygenation results in tubular epithelial cell injury (of the cells lining the kidney tubules), and thus causes acute kidney injury (AKI). Meanwhile, in the heart, impaired calcium transport, and low production of adenosine triphosphate (ATP), can cause myocardial depression, reducing cardiac contractility and causing heart failure. In the gastrointestinal tract, increased permeability of the mucosa alters the microflora, causing mucosal bleeding and paralytic ileus. In the central nervous system, direct damage of the brain cells and disturbances of neurotransmissions causes altered mental status. Cytokines such as tumor necrosis factor, interleukin 1, and interleukin 6 may activate procoagulation factors in the cells lining blood vessels, leading to endothelial damage. The damaged endothelial surface inhibits anticoagulant properties as well as increases antifibrinolysis, which may lead to intravascular clotting, the formation of blood clots in small blood vessels, and multiple organ failure. The low blood pressure seen in those with sepsis is the result of various processes, including excessive production of chemicals that dilate blood vessels such as nitric oxide, a deficiency of chemicals that constrict blood vessels such as vasopressin, and activation of ATP-sensitive potassium channels. In those with severe sepsis and septic shock, this sequence of events leads to a type of circulatory shock known as distributive shock. Early recognition and focused management may improve the outcomes in sepsis. Current professional recommendations include a number of actions ("bundles") to be followed as soon as possible after diagnosis. Within the first three hours, someone with sepsis should have received antibiotics and, intravenous fluids if there is evidence of either low blood pressure or other evidence for inadequate blood supply to organs (as evidenced by a raised level of lactate); blood cultures also should be obtained within this time period. After six hours the blood pressure should be adequate, close monitoring of blood pressure and blood supply to organs should be in place, and the lactate should be measured again if initially it was raised. A related bundle, the "Sepsis Six", is in widespread use in the United Kingdom; this requires the administration of antibiotics within an hour of recognition, blood cultures, lactate, and hemoglobin determination, urine output monitoring, high-flow oxygen, and intravenous fluids. Apart from the timely administration of fluids and antibiotics, the management of sepsis also involves surgical drainage of infected fluid collections and appropriate support for organ dysfunction. This may include hemodialysis in kidney failure, mechanical ventilation in lung dysfunction, transfusion of blood products, and drug and fluid therapy for circulatory failure. Ensuring adequate nutrition—preferably by enteral feeding, but if necessary, by parenteral nutrition—is important during prolonged illness. Medication to prevent deep vein thrombosis and gastric ulcers also may be used. Two sets of blood cultures (aerobic and anaerobic) are recommended without delaying the initiation of antibiotics. Cultures from other sites such as respiratory secretions, urine, wounds, cerebrospinal fluid, and catheter insertion sites (in-situ more than 48 hours) are recommended if infections from these sites are suspected. In severe sepsis and septic shock, broad-spectrum antibiotics (usually two, a β-lactam antibiotic with broad coverage, or broad-spectrum carbapenem combined with fluoroquinolones, macrolides, or aminoglycosides) are recommended. The choice of antibiotics is important in determining the survival of the person. Some recommend they be given within one hour of making the diagnosis, stating that for every hour of delay in the administration of antibiotics, there is an associated 6% rise in mortality. Others did not find a benefit with early administration. Several factors determine the most appropriate choice for the initial antibiotic regimen. These factors include local patterns of bacterial sensitivity to antibiotics, whether the infection is thought to be a hospital or community-acquired infection, and which organ systems are thought to be infected. Antibiotic regimens should be reassessed daily and narrowed if appropriate. Treatment duration is typically 7–10 days with the type of antibiotic used directed by the results of cultures. If the culture result is negative, antibiotics should be de-escalated according to the person's clinical response or stopped altogether if an infection is not present to decrease the chances that the person is infected with multiple drug resistance organisms. In case of people having a high risk of being infected with multiple drug resistant organisms such as Pseudomonas aeruginosa, Acinetobacter baumannii, the addition of an antibiotic specific to the gram-negative organism is recommended. For Methicillin-resistant Staphylococcus aureus (MRSA), vancomycin or teicoplanin is recommended. For Legionella infection, addition of macrolide or fluoroquinolone is chosen. If fungal infection is suspected, an echinocandin, such as caspofungin or micafungin, is chosen for people with severe sepsis, followed by triazole (fluconazole and itraconazole) for less ill people. Prolonged antibiotic prophylaxis is not recommended in people who has SIRS without any infectious origin such as acute pancreatitis and burns unless sepsis is suspected. Once-daily dosing of aminoglycoside is sufficient to achieve peak plasma concentration for a clinical response without kidney toxicity. Meanwhile, for antibiotics with low volume distribution (vancomycin, teicoplanin, colistin), a loading dose is required to achieve an adequate therapeutic level to fight infections. Frequent infusions of beta-lactam antibiotics without exceeding total daily dose would help to keep the antibiotics level above minimum inhibitory concentration (MIC), thus providing a better clinical response. Giving beta-lactam antibiotics continuously may be better than giving them intermittently. Access to therapeutic drug monitoring is important to ensure adequate drug therapeutic level while at the same time preventing the drug from reaching toxic level. The Surviving Sepsis Campaign has recommended 30 ml/kg of fluid to be given in adults in the first three hours followed by fluid titration according to blood pressure, urine output, respiratory rate, and oxygen saturation with a target mean arterial pressure (MAP) of 65 mmHg. In children an initial amount of 20 ml/kg is reasonable in shock. In cases of severe sepsis and septic shock where a central venous catheter is used to measure blood pressures dynamically, fluids should be administered until the central venous pressure reaches 8–12 mmHg. Once these goals are met, the central venous oxygen saturation (ScvO2), i.e., the oxygen saturation of venous blood as it returns to the heart as measured at the vena cava, is optimized. If the ScvO2 is less than 70%, blood may be given to reach a hemoglobin of 10 g/dL and then inotropes are added until the ScvO2 is optimized. In those with acute respiratory distress syndrome (ARDS) and sufficient tissue blood fluid, more fluids should be given carefully. Crystalloid solution is recommended as the fluid of choice for resuscitation. Albumin can be used if a large amount of crystalloid is required for resuscitation. Crystalloid solutions shows little difference with hydroxyethyl starch in terms of risk of death. Starches also carry an increased risk of acute kidney injury, and need for blood transfusion. Various colloid solutions (such as modified gelatin) carry no advantage over crystalloid. Albumin also appears to be of no benefit over crystalloids. The Surviving Sepsis Campaign recommended packed red blood cells transfusion for hemoglobin levels below 70 g/L if there is no myocardial ischemia, hypoxemia, or acute bleeding. In a 2014 trial, blood transfusions to keep target hemoglobin above 70 or 90 g/L did not make any difference to survival rates; meanwhile, those with a lower threshold of transfusion received fewer transfusions in total. Erythropoietin is not recommended in the treatment of anemia with septic shock because it may precipitate blood clotting events. Fresh frozen plasma transfusion usually does not correct the underlying clotting abnormalities before a planned surgical procedure. However, platelet transfusion is suggested for platelet counts below (10 × 109/L) without any risk of bleeding, or (20 × 109/L) with high risk of bleeding, or (50 × 109/L) with active bleeding, before a planned surgery or an invasive procedure. IV immunoglobulin is not recommended because its beneficial effects are uncertain. Monoclonal and polyclonal preparations of intravenous immunoglobulin (IVIG) do not lower the rate of death in newborns and adults with sepsis. Evidence for the use of IgM-enriched polyclonal preparations of IVIG is inconsistent. On the other hand, the use of antithrombin to treat disseminated intravascular coagulation is also not useful. Meanwhile, the blood purification technique (such as hemoperfusion, plasma filtration, and coupled plasma filtration adsorption) to remove inflammatory mediators and bacterial toxins from the blood also does not demonstrate any survival benefit for septic shock. If the person has been sufficiently fluid resuscitated but the mean arterial pressure is not greater than 65 mmHg, vasopressors are recommended. Norepinephrine (noradrenaline) is recommended as the initial choice. Delaying initiation of vasopressor therapy during septic shock is associated with increased mortality. Norepinephrine is often used as a first-line treatment for hypotensive septic shock because evidence shows that there is a relative deficiency of vasopressin when shock continues for 24 to 48 hours. Norepinephrine raises blood pressure through a vasoconstriction effect, with little effect on stroke volume and heart rate. In some people, the required dose of vasopressor needed to increase the mean arterial pressure can become exceedingly high that it becomes toxic. In order to reduce the required dose of vasopressor, epinephrine may be added. Epinephrine is not often used as a first-line treatment for hypotensive shock because it reduces blood flow to the abdominal organs and increases lactate levels. Vasopressin can be used in septic shock because studies have shown that there is a relative deficiency of vasopressin when shock continues for 24 to 48 hours. However, vasopressin reduces blood flow to the heart, finger/toes, and abdominal organs, resulting in a lack of oxygen supply to these tissues. Dopamine is typically not recommended. Although dopamine is useful to increase the stroke volume of the heart, it causes more abnormal heart rhythms than norepinephrine and also has an immunosuppressive effect. Dopamine is not proven to have protective properties on the kidneys. Dobutamine can also be used in hypotensive septic shock to increase cardiac output and correct blood flow to the tissues. Dobutamine is not used as often as epinephrine due to its associated side effects, which include reducing blood flow to the gut. Additionally, dobutamine increases the cardiac output by abnormally increasing the heart rate. The use of steroids in sepsis is controversial. Studies do not give a clear picture as to whether and when glucocorticoids should be used. The 2016 Surviving Sepsis Campaign recommends low dose hydrocortisone only if both intravenous fluids and vasopressors are not able to adequately treat septic shock. A 2019 Cochrane review found low-quality evidence of benefit, as did two 2019 reviews. During critical illness, a state of adrenal insufficiency and tissue resistance to corticosteroids may occur. This has been termed critical illness–related corticosteroid insufficiency. Treatment with corticosteroids might be most beneficial in those with septic shock and early severe ARDS, whereas its role in others such as those with pancreatitis or severe pneumonia is unclear. However, the exact way of determining corticosteroid insufficiency remains problematic. It should be suspected in those poorly responding to resuscitation with fluids and vasopressors. Neither ACTH stimulation testing nor random cortisol levels are recommended to confirm the diagnosis. The method of stopping glucocorticoid drugs is variable, and it is unclear whether they should be slowly decreased or simply abruptly stopped. However, the 2016 Surviving Sepsis Campaign recommended to taper steroids when vasopressors are no longer needed. A target tidal volume of 6 mL/kg of predicted body weight (PBW) and a plateau pressure less than 30 cm H2O is recommended for those who require ventilation due to sepsis-induced severe ARDS. High positive end expiratory pressure (PEEP) is recommended for moderate to severe ARDS in sepsis as it opens more lung units for oxygen exchange. Predicted body weight is calculated based on sex and height, and tools for this are available. Recruitment maneuvers may be necessary for severe ARDS by briefly raising the transpulmonary pressure. It is recommended that the head of the bed be raised if possible to improve ventilation. However, β2 adrenergic receptor agonists are not recommended to treat ARDS because it may reduce survival rates and precipitate abnormal heart rhythms. A spontaneous breathing trial using continuous positive airway pressure (CPAP), T piece, or inspiratory pressure augmentation can be helpful in reducing the duration of ventilation. Minimizing intermittent or continuous sedation is helpful in reducing the duration of mechanical ventilation. General anesthesia is recommended for people with sepsis who require surgical procedures to remove the infective source. Usually, inhalational and intravenous anesthetics are used. Requirements for anesthetics may be reduced in sepsis. Inhalational anesthetics can reduce the level of proinflammatory cytokines, altering leukocyte adhesion and proliferation, inducing apoptosis (cell death) of the lymphocytes, possibly with a toxic effect on mitochondrial function. Although etomidate has a minimal effect on the cardiovascular system, it is often not recommended as a medication to help with intubation in this situation due to concerns it may lead to poor adrenal function and an increased risk of death. The small amount of evidence there is, however, has not found a change in the risk of death with etomidate. Paralytic agents are not suggested for use in sepsis cases in the absence of ARDS, as a growing body of evidence points to reduced durations of mechanical ventilation, ICU and hospital stays. However, paralytic use in ARDS cases remains controversial. When appropriately used, paralytics may aid successful mechanical ventilation, however, evidence has also suggested that mechanical ventilation in severe sepsis does not improve oxygen consumption and delivery. Source control refers to physical interventions to control a focus of infection and reduce conditions favorable to microorganism growth or host defense impairment, such as drainage of pus from an abscess. It is one of the oldest procedures for control of infections, giving rise to the Latin phrase Ubi pus, ibi evacua, and remains important despite the emergence of more modern treatments. Early goal directed therapy Early goal directed therapy (EGDT) is an approach to the management of severe sepsis during the initial 6 hours after diagnosis. It is a step-wise approach, with the physiologic goal of optimizing cardiac preload, afterload, and contractility. It includes giving early antibiotics. EGDT also involves monitoring of hemodynamic parameters and specific interventions to achieve key resuscitation targets which include maintaining a central venous pressure between 8–12 mmHg, a mean arterial pressure of between 65 and 90 mmHg, a central venous oxygen saturation (ScvO2) greater than 70% and a urine output of greater than 0.5 ml/kg/hour. The goal is to optimize oxygen delivery to tissues and achieve a balance between systemic oxygen delivery and demand. An appropriate decrease in serum lactate may be equivalent to ScvO2 and easier to obtain. In the original trial, early goal-directed therapy was found to reduce mortality from 46.5% to 30.5% in those with sepsis, and the Surviving Sepsis Campaign has been recommending its use. However, three more recent large randomized control trials (ProCESS, ARISE, and ProMISe), did not demonstrate a 90-day mortality benefit of early goal-directed therapy when compared to standard therapy in severe sepsis. It is likely that some parts of EGDT are more important than others. Following these trials the use of EGDT is still considered reasonable. Neonatal sepsis can be difficult to diagnose as newborns may be asymptomatic. If a newborn shows signs and symptoms suggestive of sepsis, antibiotics are immediately started and are either changed to target a specific organism identified by diagnostic testing or discontinued after an infectious cause for the symptoms has been ruled out. Despite early intervention, death occurs in 13% of children who develop septic shock, with the risk partly based on other health problems. Those without multiple organ system failures or who require only one inotropic agent mortality is low. Treating fever in sepsis, including people in septic shock, has not been associated with any improvement in mortality over a period of 28 days. Treatment of fever still occurs for other reasons. Recombinant activated protein C (drotrecogin alpha) was originally introduced for severe sepsis (as identified by a high APACHE II score), where it was thought to confer a survival benefit. However, subsequent studies showed that it increased adverse events—bleeding risk in particular—and did not decrease mortality. It was removed from sale in 2011. Another medication known as eritoran also has not shown benefit. In those with high blood sugar levels, insulin to bring it down to 7.8–10 mmol/L (140–180 mg/dL) is recommended with lower levels potentially worsening outcomes. Glucose levels taken from capillary blood should be interpreted with care because such measurements may not be accurate. If a person has an arterial catheter, arterial blood is recommended for blood glucose testing. Intermittent or continuous renal replacement therapy may be used if indicated. However, sodium bicarbonate is not recommended for a person with lactic acidosis secondary to hypoperfusion. Low-molecular-weight heparin (LMWH), unfractionated heparin (UFH), and mechanical prophylaxis with intermittent pneumatic compression devices are recommended for any person with sepsis at moderate to high risk of venous thromboembolism. Stress ulcer prevention with proton-pump inhibitor (PPI) and H2 antagonist are useful in a person with risk factors of developing upper gastrointestinal bleeding (UGIB) such as on mechanical ventilation for more than 48 hours, coagulation disorders, liver disease, and renal replacement therapy. Achieving partial or full enteral feeding (delivery of nutrients through a feeding tube) is chosen as the best approach to provide nutrition for a person who is contraindicated for oral intake or unable to tolerate orally in the first seven days of sepsis when compared to intravenous nutrition. However, omega-3 fatty acids are not recommended as immune supplements for a person with sepsis or septic shock. The usage of prokinetic agents such as metoclopramide, domperidone, and erythromycin are recommended for those who are septic and unable to tolerate enteral feeding. However, these agents may precipitate prolongation of the QT interval and consequently provoke a ventricular arrhythmia such as torsades de pointes. The usage of prokinetic agents should be reassessed daily and stopped if no longer indicated. Sepsis will prove fatal in approximately 24.4% of people, and septic shock will prove fatal in 34.7% of people within 30 days (32.2% and 38.5% after 90 days). Lactate is a useful method of determining prognosis, with those who have a level greater than 4 mmol/L having a mortality of 40% and those with a level of less than 2 mmol/L having a mortality of less than 15%. There are a number of prognostic stratification systems, such as APACHE II and Mortality in Emergency Department Sepsis. APACHE II factors in the person's age, underlying condition, and various physiologic variables to yield estimates of the risk of dying of severe sepsis. Of the individual covariates, the severity of the underlying disease most strongly influences the risk of death. Septic shock is also a strong predictor of short- and long-term mortality. Case-fatality rates are similar for culture-positive and culture-negative severe sepsis. The Mortality in Emergency Department Sepsis (MEDS) score is simpler and useful in the emergency department environment. Some people may experience severe long-term cognitive decline following an episode of severe sepsis, but the absence of baseline neuropsychological data in most people with sepsis makes the incidence of this difficult to quantify or to study. Sepsis causes millions of deaths globally each year and is the most common cause of death in people who have been hospitalized. The number of new cases worldwide of sepsis is estimated to be 18 million cases per year. In the United States sepsis affects approximately 3 in 1,000 people, and severe sepsis contributes to more than 200,000 deaths per year. Sepsis occurs in 1–2% of all hospitalizations and accounts for as much as 25% of ICU bed utilization. Due to it rarely being reported as a primary diagnosis (often being a complication of cancer or other illness), the incidence, mortality, and morbidity rates of sepsis are likely underestimated. A study of U.S. states found approximately 651 hospital stays per 100,000 population with a sepsis diagnosis in 2010. It is the second-leading cause of death in non-coronary intensive care unit (ICU) and the tenth-most-common cause of death overall (the first being heart disease). Children under 12 months of age and elderly people have the highest incidence of severe sepsis. Among people from the U.S. who had multiple sepsis hospital admissions in 2010, those who were discharged to a skilled nursing facility or long-term care following the initial hospitalization were more likely to be readmitted than those discharged to another form of care. A study of 18 U.S. states found that, amongst people with Medicare in 2011, sepsis was the second most common principal reason for readmission within 30 days. Several medical conditions increase a person's susceptibility to infection and developing sepsis. Common sepsis risk factors include age (especially the very young and old); conditions that weaken the immune system such as cancer, diabetes, or the absence of a spleen; and major trauma and burns. From 1979 to 2000, data from the United States National Hospital Discharge Survey showed that the incidence of sepsis increased fourfold, to 240 cases per 100,000 population, with a higher incidence in men when compared to women. However, the global prevalence of sepsis has been estimated to be higher in women. During the same time frame, the in-hospital case fatality rate was reduced from 28% to 18%. However, according to the nationwide inpatient sample from the United States, the incidence of severe sepsis increased from 200 per 10,000 population in 2003 to 300 cases in 2007 for population aged more than 18 years. The incidence rate is particularly high among infants, with an incidence of 500 cases per 100,000 population. Mortality related to sepsis increases with age, from less than 10% in the age group of 3 to 5 years to 60% by sixth decade of life. The increase in the average age of the population, alongside the presence of more people with chronic diseases or on immunosuppressive medications, and also the increase in the number of invasive procedures being performed, has led to an increased rate of sepsis. The term "σήψις" (sepsis) was introduced by Hippocrates in the fourth century BC, and it meant the process of decay or decomposition of organic matter. In the eleventh century, Avicenna used the term "blood rot" for diseases linked to severe purulent process. Though severe systemic toxicity had already been observed, it was only in the 19th century that the specific term – sepsis – was used for this condition. The terms "septicemia", also spelled "septicaemia", and "blood poisoning" referred to the microorganisms or their toxins in the blood. The International Statistical Classification of Diseases and Related Health Problems (ICD) version 9, which was in use in the US until 2013, used the term septicemia with numerous modifiers for different diagnoses, such as "Streptococcal septicemia". All those diagnoses have been converted to sepsis, again with modifiers, in ICD-10, such as "Sepsis due to streptococcus". The current terms are dependent on the microorganism that is present: bacteremia if bacteria are present in the blood at abnormal levels and are the causative issue, viremia for viruses, and fungemia for a fungus. By the end of the 19th century, it was widely believed that microbes produced substances that could injure the mammalian host and that soluble toxins released during infection caused the fever and shock that were commonplace during severe infections. Pfeiffer coined the term endotoxin at the beginning of the 20th century to denote the pyrogenic principle associated with Vibrio cholerae. It was soon realized that endotoxins were expressed by most and perhaps all gram-negative bacteria. The lipopolysaccharide character of enteric endotoxins was elucidated in 1944 by Shear. The molecular character of this material was determined by Luderitz et al. in 1973. It was discovered in 1965 that a strain of C3H/HeJ mouse was immune to the endotoxin-induced shock. The genetic locus for this effect was dubbed Lps. These mice were also found to be hyper susceptible to infection by gram-negative bacteria. These observations were finally linked in 1998 by the discovery of the toll-like receptor gene 4 (TLR 4). Genetic mapping work, performed over a period of five years, showed that TLR4 was the sole candidate locus within the Lps critical region; this strongly implied that a mutation within TLR4 must account for the lipopolysaccharide resistance phenotype. The defect in the TLR4 gene that led to the endotoxin resistant phenotype was discovered to be due to a mutation in the cytoplasm. Controversy occurred in the scientific community over the use of mouse models in research into sepsis in 2013 when scientists published a review of the mouse immune system compared to the human immune system and showed that on a systems level, the two worked very differently; the authors noted that as of the date of their article over 150 clinical trials of sepsis had been conducted in humans, almost all of them supported by promising data in mice and that all of them had failed. The authors called for abandoning the use of mouse models in sepsis research; others rejected that but called for more caution in interpreting the results of mouse studies, and more careful design of preclinical studies. One approach is to rely more on studying biopsies and clinical data from people who have had sepsis, to try to identify biomarkers and drug targets for intervention. Society and culture Sepsis was the most expensive condition treated in United States' hospital stays in 2013, at an aggregate cost of $23.6 billion for nearly 1.3 million hospitalizations. Costs for sepsis hospital stays more than quadrupled since 1997 with an 11.5 percent annual increase. By payer, it was the most costly condition billed to Medicare and the uninsured, the second-most costly billed to Medicaid, and the fourth-most costly billed to private insurance. A large international collaboration entitled the "Surviving Sepsis Campaign" was established in 2002 to educate people about sepsis and to improve outcomes with sepsis. The Campaign has published an evidence-based review of management strategies for severe sepsis, with the aim to publish a complete set of guidelines in subsequent years. The guidelines were updated in 2016 and again in 2021. Some authors suggest that initiating sepsis by the normally mutualistic (or neutral) members of the microbiome may not always be an accidental side effect of the deteriorating host immune system. Rather it is often an adaptive microbial response to a sudden decline of host survival chances. Under this scenario, the microbe species provoking sepsis benefit from monopolizing the future cadaver, utilizing its biomass as decomposers, and then transmitted through soil or water to establish mutualistic relations with new individuals. The bacteria Streptococcus pneumoniae, Escherichia coli, Proteus spp., Pseudomonas aeruginosa, Staphylococcus aureus, Klebsiella spp., Clostridium spp., Lactobacillus spp., Bacteroides spp. and the fungi Candida spp. are all capable of such a high level of phenotypic plasticity. Evidently, not all cases of sepsis arise through such adaptive microbial strategy switches. Paul E. Marik's "Marik protocol", also known as the "HAT" protocol, proposed a combination of hydrocortisone, vitamin C, and thiamine as a treatment for preventing sepsis for people in intensive care. Marik's own initial research, published in 2017, showed a dramatic evidence of benefit, leading to the protocol becoming popular among intensive care physicians, especially after the protocol received attention on social media and National Public Radio, leading to criticism of science by press conference from the wider medical community. Subsequent independent research failed to replicate Marik's positive results, indicating the possibility that they had been compromised by bias. A systematic review of trials in 2021 found that the claimed benefits of the protocol could not be confirmed. Another more recent review found that "HAT therapy significantly reduced the duration of vasopressor use and improved the SOFA score but appeared not to have significant benefits in other outcomes for patients with sepsis." - "Sepsis Questions and Answers". cdc.gov. Centers for Disease Control and Prevention (CDC). 22 May 2014. Archived from the original on 4 December 2014. Retrieved 28 November 2014. - Jui J, et al. (American College of Emergency Physicians) (2011). "Ch. 146: Septic Shock". In Tintinalli JE, et al. (eds.). Tintinalli's Emergency Medicine: A Comprehensive Study Guide (7th ed.). New York: McGraw-Hill. pp. 1003–14. ISBN 9780071484800. - Deutschman CS, Tracey KJ (April 2014). "Sepsis: Current dogma and new perspectives". Immunity. 40 (4): 463–75. doi:10.1016/j.immuni.2014.04.001. PMID 24745331. - Singer M, Deutschman CS, et al. (February 2016). "The Third International Consensus Definitions for Sepsis and Septic Shock (Sepsis-3)". JAMA. 315 (8): 801–10. doi:10.1001/jama.2016.0287. PMC 4968574. PMID 26903338. - Rhodes A, Evans LE, et al. (March 2017). "Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock: 2016". Intensive Care Medicine. 43 (3): 304–377. doi:10.1007/s00134-017-4683-6. PMID 28101605. - Jawad I, Lukšić I, et al. (June 2012). "Assessing available information on the burden of sepsis: Global estimates of incidence, prevalence and mortality". Journal of Global Health. 2 (1): 010404. doi:10.7189/jogh.01.010404. PMC 3484761. PMID 23198133. - Martin GS (June 2012). "Sepsis, severe sepsis and septic shock: Changes in incidence, pathogens and outcomes". Expert Review of Anti-infective Therapy. 10 (6): 701–6. doi:10.1586/eri.12.50. PMC 3488423. PMID 22734959. - Chao C, Muming Y, Yanfen C (2019). "Pathological Alteration and Therapeutic Implications of Sepsis-Induced Immune Cell Apoptosis". Cell Death & Disease. 10 (10): 782. doi:10.1038/s41419-019-2015-1. PMC 6791888. PMID 31611560. - Dellinger RP, Levy MM, et al. (February 2013). "Surviving Sepsis Campaign: International Guidelines for Management of Severe Sepsis and Septic Shock: 2012". Critical Care Medicine. 41 (2): 580–637. doi:10.1097/CCM.0b013e31827e83af. PMID 23353941. - Sehgal, Mukul; Ladd, Hugh J.; Totapally, Balagangadhar (1 December 2020). "Trends in Epidemiology and Microbiology of Severe Sepsis and Septic Shock in Children". Hospital Pediatrics. 10 (12): 1021–1030. doi:10.1542/hpeds.2020-0174. ISSN 2154-1663. PMID 33208389. S2CID 227067133. - Annane, D; Bellissant, E; Bollaert, PE; Briegel, J; Keh, D; Kupfer, Y; Pirracchio, R; Rochwerg, B (6 December 2019). "Corticosteroids for treating sepsis in children and adults". The Cochrane Database of Systematic Reviews. 2019 (12): CD002243. doi:10.1002/14651858.CD002243.pub4. PMC 6953403. PMID 31808551. - Fang F, Zhang Y, et al. (February 2019). "Association of corticosteroid treatment with outcomes in adult patients with sepsis: A systematic review and meta-analysis". JAMA Internal Medicine. 179 (2): 213–223. doi:10.1001/jamainternmed.2018.5849. PMC 6439648. PMID 30575845. - Long B, Koyfman A (November 2017). "Controversies in corticosteroid use for sepsis". The Journal of Emergency Medicine. 53 (5): 653–661. doi:10.1016/j.jemermed.2017.05.024. PMID 28916121. - Rudd, Kristina E; Johnson, Sarah Charlotte; Agesa, Kareha M; Shackelford, Katya Anne; Tsoi, Derrick; Kievlan, Daniel Rhodes; Colombara, Danny V; Ikuta, Kevin S; Kissoon, Niranjan; Finfer, Simon; Fleischmann-Struzek, Carolin; Machado, Flavia R; Reinhart, Konrad K; Rowan, Kathryn; Seymour, Christopher W; Watson, R Scott; West, T Eoin; Marinho, Fatima; Hay, Simon I; Lozano, Rafael; Lopez, Alan D; Angus, Derek C; Murray, Christopher J L; Naghavi, Mohsen (January 2020). "Global, regional, and national sepsis incidence and mortality, 1990–2017: analysis for the Global Burden of Disease Study". The Lancet. 395 (10219): 200–211. doi:10.1016/S0140-6736(19)32989-7. PMC 6970225. PMID 31954465. - Rudd, Kristina E.; Johnson, Sarah Charlotte; Agesa, Kareha M.; Shackelford, Katya Anne; Tsoi, Derrick; Kievlan, Daniel Rhodes; Colombara, Danny V.; Ikuta, Kevin S.; Kissoon, Niranjan; Finfer, Simon; Fleischmann-Struzek, Carolin (18 January 2020). "Global, regional, and national sepsis incidence and mortality, 1990–2017: analysis for the Global Burden of Disease Study". The Lancet. 395 (10219): 200–211. doi:10.1016/S0140-6736(19)32989-7. hdl:11343/273829. ISSN 0140-6736. PMC 6970225. PMID 31954465. - Angus DC, van der Poll T (August 2013). "Severe sepsis and septic shock". The New England Journal of Medicine. 369 (9): 840–51. doi:10.1056/NEJMra1208623. PMID 23984731. Lay summary (30 August 2013). - Bone RC, Balk RA, et al. (The ACCP/SCCM Consensus Conference Committee. American College of Chest Physicians/Society of Critical Care Medicine) (June 1992). "Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis". Chest. 101 (6): 1644–55. doi:10.1378/chest.101.6.1644. PMID 1303622. Septicemia... has been used... in a variety of ways... We therefore suggest that this term be eliminated from current usage. - Levy MM, Fink MP, et al. (April 2003). "2001 SCCM/ESICM/ACCP/ATS/SIS International Sepsis Definitions Conference" (PDF). Critical Care Medicine. 31 (4): 1250–6. doi:10.1097/01.CCM.0000050454.01978.3B. PMID 12682500. S2CID 19605781. Archived (PDF) from the original on 24 September 2015. - Felner K, Smith RL (2012). "Ch. 138: Sepsis". In McKean S, et al. (eds.). Principles and Practice of Hospital Medicine. New York: McGraw-Hill. pp. 1099–109. ISBN 978-0071603898. - Gauer, RL (1 July 2013). "Early recognition and management of sepsis in adults: the first six hours". American Family Physician. 88 (1): 44–53. PMID 23939605. - MedlinePlus Encyclopedia: Sepsis. Retrieved 29 November 2014. - Singer, Pierre; Blaser, Annika Reintam; Berger, Mette M.; Alhazzani, Waleed; Calder, Philip C.; Casaer, Michael P.; Hiesmayr, Michael; Mayer, Konstantin; Montejo, Juan Carlos; Pichard, Claude; Preiser, Jean-Charles; van Zanten, Arthur R.H.; Oczkowski, Simon; Szczeklik, Wojciech; Bischoff, Stephan C. (February 2019). "ESPEN guideline on clinical nutrition in the intensive care unit". Clinical Nutrition. 38 (1): 48–79. doi:10.1016/j.clnu.2018.08.037. PMID 30348463. S2CID 53036546. - Munford RS, Suffredini AF (2014). "Ch. 75: Sepsis, Severe Sepsis and Septic Shock". In Bennett JE, et al. (eds.). Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases (8th ed.). Philadelphia: Elsevier Health Sciences. pp. 914–34. ISBN 9780323263733. - Polat G, Ugan RA, et al. (February 2017). "Sepsis and septic shock: Current treatment strategies and new approaches". The Eurasian Journal of Medicine. 49 (1): 53–8. doi:10.5152/eurasianjmed.2017.17062. PMC 5389495. PMID 28416934. - Bloch KC (2009). "Ch. 4: Infectious Diseases". In McPhee SJ, et al. (eds.). Pathophysiology of Disease (6th ed.). New York: McGraw-Hill. ISBN 9780071621670. - Ramachandran G (January 2014). "Gram-positive and gram-negative bacterial toxins in sepsis: A brief review". Virulence. 5 (1): 213–8. doi:10.4161/viru.27024. PMC 3916377. PMID 24193365. - Delaloye J, Calandra T (January 2014). "Invasive candidiasis as a cause of sepsis in the critically ill patient". Virulence. 5 (1): 161–9. doi:10.4161/viru.26187. PMC 3916370. PMID 24157707. - Harris R (22 February 2018). "Synergy Between Nurses And Automation Could Be Key To Finding Sepsis Early". All Things Considered. NPR. Archived from the original on 27 February 2018. Retrieved 26 February 2018. - "Blood Culture Collection" (PDF). WVUH Laboratories. 7 April 2012. Retrieved 23 March 2020. - Wacker C, Prkno A, et al. (May 2013). "Procalcitonin as a diagnostic marker for sepsis: A systematic review and meta-analysis". The Lancet Infectious Diseases. 13 (5): 426–35. doi:10.1016/S1473-3099(12)70323-7. PMID 23375419. - Morris E, McCartney D, et al. (December 2017). "Point-of-care lactate testing for sepsis at presentation to health care: A systematic review of patient outcomes". The British Journal of General Practice. 67 (665): e859–70. doi:10.3399/bjgp17X693665. PMC 5697556. PMID 29158243. - Bone RC, Balk RA, et al. (The ACCP/SCCM Consensus Conference Committee. American College of Chest Physicians/Society of Critical Care Medicine) (June 1992). "Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis". Chest. 101 (6): 1644–55. doi:10.1378/chest.101.6.1644. PMID 1303622. - Soong J, Soni N (June 2012). "Sepsis: Recognition and treatment". Clinical Medicine. 12 (3): 276–80. doi:10.7861/clinmedicine.12-3-276. PMC 4953494. PMID 22783783. - Simpson SQ (May 2016). "New sepsis criteria: A change we should not make". Chest. 149 (5): 1117–8. doi:10.1016/j.chest.2016.02.653. PMID 26927525. We believe that adopting a more restrictive definition that requires further progression along the sepsis pathway may delay intervention in this highly time-dependent condition, with additional risk to patients. - Vincent JL, Martin GS, et al. (July 2016). "qSOFA does not replace SIRS in the definition of sepsis". Critical Care. 20 (1): 210. doi:10.1186/s13054-016-1389-z. PMC 4947518. PMID 27423462. We hope this editorial will clarify that the qSOFA is meant to be used to raise suspicion of sepsis and prompt further action—it is not a replacement for SIRS and is not part of the definition of sepsis. - Fernando SM, Tran A, et al. (February 2018). "Prognostic accuracy of the quick Sequential Organ Failure Assessment for mortality in patients with suspected infection: A systematic review and meta-analysis". Annals of Internal Medicine. 168 (4): 266–75. doi:10.7326/M17-2820. PMID 29404582. S2CID 3441582. - Abraham E, Singer M (October 2007). "Mechanisms of sepsis-induced organ dysfunction". Critical Care Medicine. 35 (10): 2408–16. doi:10.1097/01.CCM.0000282072.56245.91. PMID 17948334. S2CID 12657048. - Goldstein B, Giroir B, et al. (January 2005). "International pediatric sepsis consensus conference: Definitions for sepsis and organ dysfunction in pediatrics". Pediatric Critical Care Medicine. 6 (1): 2–8. doi:10.1097/01.PCC.0000149131.72248.E6. PMID 15636651. S2CID 8190072. - Pierrakos, Charalampos; Vincent, Jean-Louis (2010). "Sepsis biomarkers: a review". Critical Care. 14 (1): R15. doi:10.1186/cc8872. PMC 2875530. PMID 20144219. - Backes Y, van der Sluijs KF, et al. (September 2012). "Usefulness of suPAR as a biological marker in patients with systemic inflammation or infection: A systematic review". Intensive Care Medicine. 38 (9): 1418–28. doi:10.1007/s00134-012-2613-1. PMC 3423568. PMID 22706919. - Mayr FB, Yende S, et al. (January 2014). "Epidemiology of severe sepsis". Virulence. 5 (1): 4–11. doi:10.4161/viru.27372. PMC 3916382. PMID 24335434. - Machowicz R, Janka G, et al. (June 2017). "Similar but not the same: Differential diagnosis of HLH and sepsis". Critical Reviews in Oncology/Hematology. 114: 1–12. doi:10.1016/j.critrevonc.2017.03.023. PMID 28477737. - Satar M, Ozlü F (September 2012). "Neonatal sepsis: A continuing disease burden" (PDF). The Turkish Journal of Pediatrics. 54 (5): 449–57. PMID 23427506. Archived from the original (PDF) on 19 December 2014. - Ely EW, Goyette RE (2005). "Ch. 46: Sepsis with Acute Organ Dysfunction". In Hall JB, et al. (eds.). Principles of Critical Care (3rd ed.). New York: McGraw-Hill Medical. ISBN 978-0071416405. - Shukla P, Rao GM, et al. (November 2014). "Therapeutic interventions in sepsis: Current and anticipated pharmacological agents". British Journal of Pharmacology. 171 (22): 5011–31. doi:10.1111/bph.12829. PMC 4253453. PMID 24977655. - Park BS, Lee JO (December 2013). "Recognition of lipopolysaccharide pattern by TLR4 complexes". Experimental & Molecular Medicine. 45 (12): e66. doi:10.1038/emm.2013.97. PMC 3880462. PMID 24310172. - Cross AS (January 2014). "Anti-endotoxin vaccines: Back to the future". Virulence. 5 (1): 219–25. doi:10.4161/viru.25965. PMC 3916378. PMID 23974910. - Fournier B, Philpott DJ (July 2005). "Recognition of Staphylococcus aureus by the innate immune system". Clinical Microbiology Reviews. 18 (3): 521–40. doi:10.1128/CMR.18.3.521-540.2005. PMC 1195972. PMID 16020688. - Leentjens J, Kox M, et al. (June 2013). "Immunotherapy for the adjunctive treatment of sepsis: From immunosuppression to immunostimulation. Time for a paradigm change?". American Journal of Respiratory and Critical Care Medicine. 187 (12): 1287–93. doi:10.1164/rccm.201301-0036CP. PMID 23590272. - Antonopoulou A, Giamarellos-Bourboulis EJ (January 2011). "Immunomodulation in sepsis: State of the art and future perspective". Immunotherapy. 3 (1): 117–28. doi:10.2217/imt.10.82. PMID 21174562. - Yuki K, Murakami N (6 January 2016). "Sepsis pathophysiology and anesthetic consideration". Cardiovascular & Hematological Disorders Drug Targets. 15 (1): 57–69. doi:10.2174/1871529x15666150108114810. PMC 4704087. PMID 25567335. - Fujishima S (1 November 2016). "Organ dysfunction as a new standard for defining sepsis". Inflammation and Regeneration. 36 (24): 24. doi:10.1186/s41232-016-0029-y. PMC 5725936. PMID 29259697. - Nimah M, Brilli RJ (July 2003). "Coagulation dysfunction in sepsis and multiple organ system failure". Critical Care Clinics. 19 (3): 441–58. doi:10.1016/s0749-0704(03)00008-3. PMID 12848314. - Marik PE (June 2014). "Iatrogenic salt water drowning and the hazards of a high central venous pressure". Annals of Intensive Care. 4: 21. doi:10.1186/s13613-014-0021-0. PMC 4122823. PMID 25110606. - Marik PE (June 2014). "Early management of severe sepsis: Concepts and controversies". Chest. 145 (6): 1407–18. CiteSeerX 10.1.1.661.7518. doi:10.1378/chest.13-2104. PMID 24889440. - Daniels R (April 2011). "Surviving the first hours in sepsis: Getting the basics right (an intensivist's perspective)" (PDF). Journal of Antimicrobial Chemotherapy. 66 (Suppl 2): ii11–23. doi:10.1093/jac/dkq515. PMID 21398303. - Scottish Intercollegiate Guidelines Network (SIGN) (May 2014). Care of Deteriorating Patients (PDF). Guideline 139. Edinburgh: SIGN. ISBN 978-1-909103-26-9. Archived from the original (PDF) on 11 August 2014. Retrieved 6 December 2014. - Sterling SA, Miller WR, et al. (September 2015). "The impact of timing of antibiotics on outcomes in severe sepsis and septic shock: A systematic review and meta-Analysis". Critical Care Medicine. 43 (9): 1907–15. doi:10.1097/CCM.0000000000001142. PMC 4597314. PMID 26121073. - Roberts JA, Abdul-Aziz MH, et al. (September 2016). "Continuous versus intermittent β-Lactam infusion in severe sepsis. A meta-analysis of individual patient data from randomized trials". American Journal of Respiratory and Critical Care Medicine. 194 (6): 681–91. doi:10.1164/rccm.201601-0024oc. PMID 26974879. - de Caen AR, Berg MD, et al. (November 2015). "Part 12: Pediatric Advanced Life Support: 2015 American Heart Association guidelines update for cardiopulmonary resuscitation and emergency cardiovascular care". Circulation. 132 (18 Suppl 2): S526–42. doi:10.1161/cir.0000000000000266. PMC 6191296. PMID 26473000. - Lewis SR, Pritchard MW, et al. (August 2018). "Colloids versus crystalloids for fluid resuscitation in critically ill people". The Cochrane Database of Systematic Reviews. 8: CD000567. doi:10.1002/14651858.CD000567.pub7. PMC 6513027. PMID 30073665. - Zarychanski R, Abou-Setta AM, et al. (February 2013). "Association of hydroxyethyl starch administration with mortality and acute kidney injury in critically ill patients requiring volume resuscitation: A systematic review and meta-analysis". JAMA. 309 (7): 678–88. doi:10.1001/jama.2013.430. PMID 23423413. - Haase N, Perner A, et al. (February 2013). "Hydroxyethyl starch 130/0.38-0.45 versus crystalloid or albumin in patients with sepsis: Systematic review with meta-analysis and trial sequential analysis". BMJ. 346: f839. doi:10.1136/bmj.f839. PMC 3573769. PMID 23418281. - Serpa Neto A, Veelo DP, et al. (February 2014). "Fluid resuscitation with hydroxyethyl starches in patients with sepsis is associated with an increased incidence of acute kidney injury and use of renal replacement therapy: A systematic review and meta-analysis of the literature". Journal of Critical Care. 29 (1): 185.e1–7. doi:10.1016/j.jcrc.2013.09.031. PMID 24262273. - Patel A, Laffan MA, et al. (July 2014). "Randomised trials of human albumin for adults with sepsis: Systematic review and meta-analysis with trial sequential analysis of all-cause mortality". BMJ. 349: g4561. doi:10.1136/bmj.g4561. PMC 4106199. PMID 25099709. - Holst LB, Haase N, et al. (October 2014). "Lower versus higher hemoglobin threshold for transfusion in septic shock". The New England Journal of Medicine. 371 (15): 1381–91. doi:10.1056/NEJMoa1406617. PMID 25270275. S2CID 16280618. - Alejandria MM, Lansang MA, et al. (September 2013). "Intravenous immunoglobulin for treating sepsis, severe sepsis and septic shock". The Cochrane Database of Systematic Reviews. 9 (9): CD001090. doi:10.1002/14651858.CD001090.pub2. PMC 6516813. PMID 24043371. - Bai, X; Yu, W; Ji, W; Lin, Z; Tan, S; Duan, K; Dong, Y; Xu, L; Li, N (3 October 2014). "Early versus delayed administration of norepinephrine in patients with septic shock". Critical Care. 18 (5): 532. doi:10.1186/s13054-014-0532-y. PMC 4194405. PMID 25277635. - Avni T, Lador A, et al. (2015). "Vasopressors for the treatment of septic shock: Systematic review and meta-analysis". PLOS One. 10 (8): e0129305. Bibcode:2015PLoSO..1029305A. doi:10.1371/journal.pone.0129305. PMC 4523170. PMID 26237037. - Hamzaoui O, Scheeren TW, et al. (August 2017). "Norepinephrine in septic shock: When and how much?". Current Opinion in Critical Care. 23 (4): 342–7. doi:10.1097/mcc.0000000000000418. PMID 28509668. S2CID 2078670. - Dubin A, Lattanzio B, et al. (2017). "The spectrum of cardiovascular effects of dobutamine - from healthy subjects to septic shock patients". Revista Brasileira de Terapia Intensiva. 29 (4): 490–8. doi:10.5935/0103-507x.20170068. PMC 5764562. PMID 29340539. - Patel GP, Balk RA (January 2012). "Systemic steroids in severe sepsis and septic shock". American Journal of Respiratory and Critical Care Medicine. 185 (2): 133–9. doi:10.1164/rccm.201011-1897CI. PMID 21680949. - Volbeda M, Wetterslev J, et al. (July 2015). "Glucocorticosteroids for sepsis: Systematic review with meta-analysis and trial sequential analysis". Intensive Care Medicine. 41 (7): 1220–34. doi:10.1007/s00134-015-3899-6. PMC 4483251. PMID 26100123. - Ni YN, Liu YM, et al. (September 2019). "Can corticosteroids reduce the mortality of patients with severe sepsis? A systematic review and meta-analysis". The American Journal of Emergency Medicine. 37 (9): 1657–64. doi:10.1016/j.ajem.2018.11.040. PMID 30522935. S2CID 54537066. - Marik PE, Pastores SM, et al. (June 2008). "Recommendations for the diagnosis and management of corticosteroid insufficiency in critically ill adult patients: Consensus statements from an international task force by the American College of Critical Care Medicine". Critical Care Medicine. 36 (6): 1937–49. doi:10.1097/CCM.0b013e31817603ba. PMID 18496365. S2CID 7861625. - NHLBI–NIH ARDS Network (2014). "Tools". ardsnet.org. National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH). Archived from the original on 3 December 2019. - Cherfan AJ, Arabi YM, et al. (May 2012). "Advantages and disadvantages of etomidate use for intubation of patients with sepsis". Pharmacotherapy. 32 (5): 475–82. doi:10.1002/j.1875-9114.2012.01027.x. PMID 22488264. S2CID 6406983. - Chan CM, Mitchell AL, et al. (November 2012). "Etomidate is associated with mortality and adrenal insufficiency in sepsis: A meta-analysis*". Critical Care Medicine. 40 (11): 2945–53. doi:10.1097/CCM.0b013e31825fec26. PMID 22971586. S2CID 916535. - Gu WJ, Wang F, et al. (February 2015). "Single-dose etomidate does not increase mortality in patients with sepsis: A systematic review and meta-analysis of randomized controlled trials and observational studies". Chest. 147 (2): 335–46. doi:10.1378/chest.14-1012. PMID 25255427. S2CID 22739840. - Lagunes, Leonel; Encina, Belen; Ramirez-Estrada, Sergio (September 2016). "Current understanding in source control management in septic shock patients: a review". Annals of Translational Medicine. 4 (17): 330. doi:10.21037/atm.2016.09.02. ISSN 2305-5839. PMC 5050189. PMID 27713888. - De Waele, J. J.; Malbrain, M. M. L. G.; De Laet, I. E. (2009), Vincent, Jean-Louis (ed.), "Source Control in the ICU", Yearbook of Intensive Care and Emergency Medicine, Springer Berlin Heidelberg, pp. 93–101, doi:10.1007/978-3-540-92276-6_9, ISBN 978-3-540-92275-9 - Dellinger RP, Levy MM, et al. (January 2008). "Surviving Sepsis Campaign: International Guidelines for Management of Severe Sepsis and Septic Shock: 2008". Intensive Care Medicine. 34 (1): 17–60. doi:10.1007/s00134-007-0934-2. PMC 2249616. PMID 18058085. - Rivers E, Nguyen B, et al. (November 2001). "Early goal-directed therapy in the treatment of severe sepsis and septic shock". The New England Journal of Medicine. 345 (19): 1368–77. doi:10.1056/NEJMoa010307. PMID 11794169. - Fuller BM, Dellinger RP (June 2012). "Lactate as a hemodynamic marker in the critically ill". Current Opinion in Critical Care. 18 (3): 267–72. doi:10.1097/MCC.0b013e3283532b8a. PMC 3608508. PMID 22517402. - Dell'Anna AM, Taccone FS (October 2015). "Early-goal directed therapy for septic shock: Is it the end?". Minerva Anestesiologica. 81 (10): 1138–43. PMID 26091011. - Rusconi AM, Bossi I, et al. (September 2015). "Early goal-directed therapy vs usual care in the treatment of severe sepsis and septic shock: A systematic review and meta-analysis". Internal and Emergency Medicine. 10 (6): 731–43. doi:10.1007/s11739-015-1248-y. PMID 25982917. S2CID 207311061. - Shane AL, Stoll BJ (January 2014). "Neonatal sepsis: Progress towards improved outcomes". Journal of Infection. 68 (Suppl 1): S24–32. doi:10.1016/j.jinf.2013.09.011. PMID 24140138. - Camacho-Gonzalez A, Spearman PW, et al. (April 2013). "Neonatal infectious diseases: Evaluation of neonatal sepsis". Pediatric Clinics of North America. 60 (2): 367–89. doi:10.1016/j.pcl.2012.12.003. PMC 4405627. PMID 23481106. - Kutko MC, Calarco MP, et al. (July 2003). "Mortality rates in pediatric septic shock with and without multiple organ system failure". Pediatric Critical Care Medicine. 4 (3): 333–7. doi:10.1097/01.PCC.0000074266.10576.9B. PMID 12831416. S2CID 18089789. - Drewry AM, Ablordeppey EA, et al. (May 2017). "Antipyretic therapy in critically ill septic patients: A systematic review and meta-analysis". Critical Care Medicine. 45 (5): 806–13. doi:10.1097/CCM.0000000000002285. PMC 5389594. PMID 28221185. - Niven DJ, Laupland KB, et al. (December 2013). "Diagnosis and management of temperature abnormality in ICUs: A EUROBACT investigators' survey". Critical Care. 17 (6): R289. doi:10.1186/cc13153. PMC 4057370. PMID 24326145. - Launey Y, Nesseler N, et al. (2011). "Clinical Review: Fever in septic ICU patients--friend or foe?". Critical Care. 15 (3): 222. doi:10.1186/cc10097. PMC 3218963. PMID 21672276. - Szakmany T, Hauser B, et al. (September 2012). "N-acetylcysteine for sepsis and systemic inflammatory response in adults". The Cochrane Database of Systematic Reviews. 9 (9): CD006616. doi:10.1002/14651858.CD006616.pub2. PMC 6517277. PMID 22972094. - Martí-Carvajal AJ, Solà I, et al. (December 2012). "Human recombinant protein C for severe sepsis and septic shock in adult and paediatric patients". The Cochrane Database of Systematic Reviews. 12: CD004388. doi:10.1002/14651858.CD004388.pub6. PMC 6464614. PMID 23235609. - Fink MP, Warren HS (October 2014). "Strategies to improve drug development for sepsis". Nature Reviews Drug Discovery. 13 (10): 741–58. doi:10.1038/nrd4368. PMID 25190187. S2CID 20904332. - Hirasawa H, Oda S, et al. (September 2009). "Blood glucose control in patients with severe sepsis and septic shock". World Journal of Gastroenterology. 15 (33): 4132–6. doi:10.3748/wjg.15.4132. PMC 2738808. PMID 19725146. - Bauer M, Gerlach H, Vogelmann T, Preissing F, Stiefel J, Adam D (May 2020). "Mortality in sepsis and septic shock in Europe, North America and Australia between 2009 and 2019- results from a systematic review and meta-analysis". Critical Care (London, England). 24 (1): 239. doi:10.1186/s13054-020-02950-2. PMC 7236499. PMID 32430052. - Carpenter CR, Keim SM, et al. (October 2009). "Risk stratification of the potentially septic patient in the emergency department: The Mortality in the Emergency Department Sepsis (MEDS) score". The Journal of Emergency Medicine. 37 (3): 319–27. doi:10.1016/j.jemermed.2009.03.016. PMID 19427752. - Jackson JC, Hopkins RO, et al. (November 2009). "Acute respiratory distress syndrome, sepsis, and cognitive decline: A review and case study". Southern Medical Journal. 102 (11): 1150–7. doi:10.1097/SMJ.0b013e3181b6a592. PMC 3776422. PMID 19864995. - Lyle NH, Pena OM, et al. (September 2014). "Barriers to the effective treatment of sepsis: Antimicrobial agents, sepsis definitions, and host-directed therapies". Annals of the New York Academy of Sciences. 1323 (2014): 101–14. Bibcode:2014NYASA1323..101L. doi:10.1111/nyas.12444. PMID 24797961. S2CID 5089865. - Munford RS (2011). "Ch. 271: Severe Sepsis and Septic Shock". In Longo DL, et al. (eds.). Harrison's Principles of Internal Medicine (18th ed.). New York: McGraw-Hill. pp. 2223–31. ISBN 9780071748896. - Sutton JP, Friedman B (September 2013). "Trends in Septicemia Hospitalizations and Readmissions in Selected HCUP States, 2005 and 2010". Healthcare Cost and Utilization Project. Statistical Brief #161. United States National Library of Medicine. PMID 24228290. Archived from the original on 6 September 2017. - Martin GS, Mannino DM, et al. (April 2003). "The epidemiology of sepsis in the United States from 1979 through 2000". The New England Journal of Medicine. 348 (16): 1546–54. doi:10.1056/NEJMoa022139. PMID 12700374. - Hines AL, Barrett ML, et al. (April 2014). "Conditions with the Largest Number of Adult Hospital Readmissions by Payer, 2011". Healthcare Cost and Utilization Project. Statistical Brief #172. Agency for Healthcare Research and Quality: United States National Library of Medicine. PMID 24901179. Archived from the original on 4 March 2016. - Koh GC, Peacock SJ, et al. (April 2012). "The impact of diabetes on the pathogenesis of sepsis". European Journal of Clinical Microbiology & Infectious Diseases. 31 (4): 379–88. doi:10.1007/s10096-011-1337-4. PMC 3303037. PMID 21805196. - Rubin LG, Schaffner W (July 2014). "Clinical Practice: Care of the asplenic patient". The New England Journal of Medicine. 371 (4): 349–56. doi:10.1056/NEJMcp1314291. PMID 25054718. - Geroulanos S, Douka ET (December 2006). "Historical perspective of the word "sepsis"". Intensive Care Medicine. 32 (12): 2077. doi:10.1007/s00134-006-0392-2. PMID 17131165. S2CID 37084190. - Vincent J (2008). "Ch. 1: Definition of Sepsis and Non-infectious SIRS". In Cavaillon J, et al. (eds.). Sepsis and Non-infectious Systemic Inflammation: From Biology to Critical Care. John Wiley & Sons. p. 3. ISBN 9783527319350. - Marshall JC (March 2008). "Sepsis: Rethinking the approach to clinical research". Journal of Leukocyte Biology. 83 (3): 471–82. CiteSeerX 10.1.1.492.7774. doi:10.1189/jlb.0607380. PMID 18171697. S2CID 24332955. - Stewart, Cynthia (8 April 2011). "Understand How ICD-10 Expands Sepsis Coding – AAPC Knowledge Center". AAPC. Retrieved 6 February 2020. - "Bacteremia". The Merck Manual—Home Health Handbook. Merck & Co. Archived from the original on 28 July 2017. Retrieved 25 November 2017. - Shear MJ (1944). "Chemical treatment of tumors, IX: Reactions of mice with primary subcutaneous tumors to injection of a hemorrhage-producing bacterial polysaccharide". Journal of the National Cancer Institute. 4 (5): 461–76. doi:10.1093/jnci/4.5.461. - Lüderitz O, Galanos C, et al. (1973). "Lipid A: Chemical structure and biological activity". The Journal of Infectious Diseases. 128: S17–S29. doi:10.1093/infdis/128.Supplement_1.S17. JSTOR 30106029. PMID 4352586. - Heppner G, Weiss DW (September 1965). "High susceptibility of Strain A mice to endotoxin and endotoxin-red blood cell mixtures". Journal of Bacteriology. 90 (3): 696–703. doi:10.1128/JB.90.3.696-703.1965. PMC 315712. PMID 16562068. - O'Brien AD, Rosenstreich DL, et al. (January 1980). "Genetic control of susceptibility to Salmonella typhimurium in mice: Role of the LPS gene". Journal of Immunology. 124 (1): 20–4. PMID 6985638. - Poltorak A, Smirnova I, et al. (September 1998). "Genetic and physical mapping of the Lps locus: Identification of the toll-4 receptor as a candidate gene in the critical region". Blood Cells, Molecules and Diseases. 24 (3): 340–55. doi:10.1006/bcmd.1998.0201. PMID 10087992. - Poltorak A, He X, et al. (December 1998). "Defective LPS signaling in C3H/HeJ and C57BL/10ScCr mice: Mutations in Tlr4 gene". Science. 282 (5396): 2085–8. Bibcode:1998Sci...282.2085P. doi:10.1126/science.282.5396.2085. PMID 9851930. - Korneev, K. V. (18 October 2019). "Mouse Models of Sepsis and Septic Shock". Molecular Biology. 53 (5): 704–717. doi:10.1134/S0026893319050108. PMID 31661479. S2CID 204758015. - Lewis AJ, Seymour CW, et al. (August 2016). "Current murine models of sepsis". Surgical Infections. 17 (4): 385–93. doi:10.1089/sur.2016.021. PMC 4960474. PMID 27305321. - Mills M, Estes MK (September 2016). "Physiologically relevant human tissue models for infectious diseases". Drug Discovery Today. 21 (9): 1540–52. doi:10.1016/j.drudis.2016.06.020. PMC 5365153. PMID 27352632. - Engber D (13 February 2013). "Septic Shock". Slate. Archived from the original on 9 April 2017. - Seok J, Warren HS, et al. (February 2013). "Genomic responses in mouse models poorly mimic human inflammatory diseases". Proceedings of the National Academy of Sciences of the United States of America. 110 (9): 3507–12. Bibcode:2013PNAS..110.3507S. doi:10.1073/pnas.1222878110. PMC 3587220. PMID 23401516. - Hazeldine J, Hampson P, et al. (2016). "The diagnostic and prognostic value of systems biology research in major traumatic and thermal injury: A review". Burns & Trauma. 4: 33. doi:10.1186/s41038-016-0059-3. PMC 5030723. PMID 27672669. - Torio CM, Moore BJ (1 January 2006). "National Inpatient Hospital Costs: The Most Expensive Conditions by Payer, 2013". Healthcare Cost and Utilization Project. Statistical Brief #204. Agency for Healthcare Research and Quality, National Library of Medicine. PMID 27359025. Archived from the original on 6 September 2017. - Pfuntner A, Wier LM, et al. (December 2013). "Costs for Hospital Stays in the United States, 2011". Healthcare Cost and Utilization Project. Statistical Brief #168. National Library of Medicine. PMID 24455786. - "History". Surviving Sepsis Campaign. Society of Critical Care Medicine. Archived from the original on 4 March 2014. Retrieved 24 February 2014. - Rhodes, Andrew; Evans, Laura E.; Alhazzani, Waleed; Levy, Mitchell M.; Antonelli, Massimo; Ferrer, Ricard; Kumar, Anand; Sevransky, Jonathan E.; Sprung, Charles L.; Nunnally, Mark E.; Rochwerg, Bram (2016). "Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock". Critical Care Medicine. 45 (3): 486–552. doi:10.1097/CCM.0000000000002255. ISSN 0090-3493. PMID 28098591. S2CID 52827184. - Evans, Laura; Rhodes, Andrew; Alhazzani, Waleed; Antonelli, Massimo; Coopersmith, Craig M.; French, Craig; Machado, Flávia R.; Mcintyre, Lauralyn; Ostermann, Marlies; Prescott, Hallie C.; Schorr, Christa (4 October 2021). "Surviving Sepsis Campaign: International Guidelines for Management of Sepsis and Septic Shock 2021". Critical Care Medicine. Publish Ahead of Print (11): e1063–e1143. doi:10.1097/CCM.0000000000005337. ISSN 0090-3493. PMID 34605781. S2CID 238257608. - Sepsis Alliance. "About Us". sepsis.org. Archived from the original on 8 September 2015. Retrieved 8 October 2015. - Rozsa L, Apari P, et al. (2017). "The evolutionary logic of sepsis". Infection, Genetics and Evolution. 55: 135–41. doi:10.1016/j.meegid.2017.09.006. PMID 28899789. - Rubin R (July 2019). "Wide Interest in a Vitamin C Drug Cocktail for Sepsis Despite Lagging Evidence". JAMA. 322 (4): 291–293. doi:10.1001/jama.2019.7936. PMID 31268477. S2CID 195788169. - Lee YR, Vo K, Varughese JT (May 2021). "Benefits of combination therapy of hydrocortisone, ascorbic acid and thiamine in sepsis and septic shock: A systematic review". Nutr Health: 2601060211018371. doi:10.1177/02601060211018371. PMID 34039089. S2CID 235215735. - Somagutta, Manoj Kumar Reddy; Pormento, Maria Kezia Lourdes; Khan, Muhammad Adnan; Hamdan, Alaa; Hange, Namrata; Kc, Manish; Pagad, Sukrut; Jain, Molly Sanjay; Lingarajah, Sivasthikka; Sharma, Vishal; Kaur, Jaspreet; Emuze, Bernard; Batti, Erkan; Iloeje, Obumneme Jude (31 August 2021). "The Efficacy of vitamin C, thiamine, and corticosteroid therapy in adult sepsis patients: a systematic review and meta-analysis". Acute and Critical Care. 36 (3): 185–200. doi:10.4266/acc.2021.00108. PMC 8435446. PMID 34185986. - Li YR, Zhu H (September 2021). "Vitamin C for sepsis intervention: from redox biochemistry to clinical medicine". Mol Cell Biochem. 476 (12): 4449–4460. doi:10.1007/s11010-021-04240-z. PMC 8413356. PMID 34478032. \|Wikimedia Commons has media related to Sepsis.\| - Sepsis at Curlie - SIRS, Sepsis, and Septic Shock Criteria - "Sepsis". MedlinePlus. U.S. National Library of Medicine.	2	93	24	0	3	5	0.956588	32	21,181
WHAT IS MEDICARE? What is Medicare? Medicare is health insurance for people age 65 and older as well as for those under age 65 with certain disabilities. Medicare was implemented in 1965 and was initially designed to limit the cost of medically necessary health care for seniors. Coverage was eventually expanded to younger people who met Social Security’s definition of disability. In the beginning, Medicare did not pay for preventative services or prescription drugs. Medicare gradually added coverage for many preventative services such as pap smears, mammography, and colonoscopies. The Medicare Modernization Act of 2003 introduced Part D prescription drug coverage. Just as Original Medicare Parts A and B have premiums, coinsurance, and deductibles, Part D Plans also have out-of-pocket costs including a coverage gap or “donut hole.” Whether you are soon turning 65, a person with a disability, or a caregiver, knowing the facts about Medicare will benefit you and your loved ones along the way. Explore this website to learn more, and you may always call HICAP at 1-800-434-0222 for more guidance and direction. Important: Medicare was never designed to cover all of your health costs. What are the different parts of Medicare? Medicare has different “Parts” that cover specific services. The Medicare Parts are: Medicare Part A (Hospital Insurance): helps cover inpatient care in hospitals and skilled nursing facilities, as well as some home health care, and hospice. Medicare Part B (Medical Insurance for Outpatient Services): helps cover physician services, x-rays, lab services, physical therapy, speech therapy, occupational therapy, outpatient procedures, and preventive services. Medicare Part C (Medicare Advantage): a health plan option offered by private insurance companies that contract with Medicare. Medicare Advantage Plans provide Part A and Part B coverage plus additional benefits and cost protections through a specific network of health providers. Some Part C plans also provide Pard D drug coverage. Medicare Advantage Plans are HMOs, PPOs, Private Fee for Service Plans (PFFS), Medicare Savings Plans, and Special Needs Plans. NOTE: if you are enrolled in a Medicare Advantage plan, you receive your health benefits through that plan rather than through Original Medicare. Medicare Part D (Prescription Drug Coverage): helps cover the cost of prescription drugs and can be included in a Medicare Advantage plan or in a stand-alone plan offered by private insurance companies that contract with Medicare. What is Medigap (supplemental) insurance? Although supplemental insurance is not considered one of the 4 parts of Medicare, supplemental plans can be helpful in keeping your health care costs manageable. Medigap is private health insurance that covers the deductibles and copayments associated with Original Medicare Parts A & B. Depending on the Medigap policy, it is possible you will have no out-of-pocket expenses for hospital or outpatient care apart from the plan’s premium. Note: the Medigap plans currently offered for sale do not include prescription coverage. You will need to purchase a stand-alone Part D plan. What do Medigap policies cover? A Medigap policy helps pay Medicare’s the deductibles, coinsurance, and copayments. The policy you purchase will determine which deductibles, coinsurance, or copays are paid. Medicare combined with a Medigap policy may be used nationally as long as the provider or facility accepts Medicare. If you have both Medicare and a Medigap policy, you do not have to go to a primary care physician to obtain an authorization for a specialist. With a Medigap, you may self-refer to any doctor who accepts Medicare. What are the basic benefits of Medigap policies? Medigap policies must follow federal and state laws designed to protect you when purchasing coverage. The policy must state that it is a Medicare Supplement Insurance. Insurance companies sell only standardized plans that are identified in most states by the letters A through N as of June 2010. Each individual, standardized Medigap policy must offer the same basic coverage no matter which company is selling it, though companies have the option of adding benefits to a policy. Cost is usually the only difference between specific Medigap policies sold by different companies. A Medigap policy will be your secondary insurance to Original Medicare. If you have a Private Fee-for-Service (PFFS) plan, or a Medicare HMO or PPO under the Medicare Advantage program, it is illegal to purchase a Medigap policy unless you are switching back to Original Medicare. NOTE: A Medigap policy will not cover the copays, coinsurance, or deductibles of a Medicare Advantage plan. What are the costs of Medigap policies? Although Medigap policies are standardized, premiums vary from company to company. Companies use three rating methods to determine the premium: ♦ Issue Age — Premiums and future increases are based on the age of the beneficiary when the policy is issued. ♦ Attained Age — Premium increases are based on the beneficiary’s age at the time of the increase. ♦ Community Age — Premiums and future increases are based on the average age of all beneficiaries in the plan. Once a Medigap policy is sold, it can only be terminated for failure to pay premiums. Companies may increase premiums, however, due to rising health care costs, inflation, and increasing age of beneficiaries. Companies may charge a higher premium to a Medicare beneficiary under 65 who has a disability than to a person 65 years or older applying for the same policy. It is recommended to compare quotes from a minimum of three companies. Go to the California Department of Insurance website by clicking below or contact HICAP at 1-800-434-0222 to schedule a one-on-one appointment. What should I consider when purchasing a Medigap policy? ♦ You must have Medicare Parts A and B to buy a plan. ♦ You pay a monthly premium to the private insurer. ♦ A Medigap policy does not cover more than one Medicare beneficiary. ♦ There are certain enrollment and guaranteed issue rights for Medicare-eligible people. See Enrollment Periods/Guaranteed Rights for more details. ♦ If you have a Medicare Advantage Plan, it is illegal for anyone to sell you a Medigap policy unless you are switching back to Original Medicare. ♦ If you have an older Medigap policy that includes prescription coverage, you cannot purchase a Medicare Part D prescription plan. ♦ Employer, union, or retiree group plans may offer coverage similar to or better than a Medigap. See Employer Group and Retiree Health Plans for more information. ♦ An insurance agent or broker cannot sell you a Medigap policy if you have Medi-Cal/Medicaid with no Share of Cost (SOC). What is the Medigap birthday rule? If you already have a Medigap policy, you may replace your current policy with one that has the same or fewer benefits for 30 days starting on your birthday without being denied coverage for pre-existing conditions. I am on Medi-Cal with a Share of Cost (SOC). Should I purchase a Medigap policy? If you have Medi-Cal with a Share of Cost (SOC) or monthly deductible, the purchase of a Medigap plan may help you lower or eliminate your SOC. If you have full Medi-Cal with no Share of Cost (no SOC), you cannot purchase a Medigap policy. Call HICAP at 1-800-434-0222 for more information. What are the enrollment requirements for Medicare coverage? Medicare Part A There is no monthly Part A premium for people who have at least 40 quarters of Social Security credits. People who have contributed less than 40 quarters to Social Security and meet the citizenship and residency requirements may be eligible to purchase Part A for a monthly premium. You will be automatically enrolled in Part A the month of your 65th birthday if you are receiving retirement benefits from Social Security or the Railroad Retirement Board. If you are not receiving retirement benefits from Social Security (1-800-772-1213) or from the Railroad Retirement Board (1-877-772-5772), you will need to contact these agencies three months before you turn 65 in order to enroll in Medicare Part A. If you are a person with a disability, you will usually be enrolled in Part A effective the 25th month of receiving Social Security Disability Insurance (SSDI). People diagnosed with End-Stage Renal Disease (ESRD) or Amyotrophic Lateral Sclerosis (ALS) will be eligible sooner. Contact Social Security (1-800-772-1213) for your enrollment benefits. Medicare Part B Part B has a monthly premium based on your income. Like Part A, you will automatically be enrolled in Part B at age 65 if you are already collecting Social Security or Railroad Retirement benefits. If you are not receiving Social Security (1-800-772-1213) or Railroad Retirement (1-877-772-5772), you will need to contact these agencies three months before you turn 65 in order to enroll in Medicare Part B. If you are a person with a disability, Part B enrollment is effective the 25th month of receiving Social Security Disability Insurance (SSDI) unless you are diagnosed with End-Stage Renal Disease (ESRD) or Amyotrophic Lateral Sclerosis (ALS), in which case you will be eligible for Medicare sooner. You may be able to defer enrollment into Part B if you are covered by the group health plan of a large employer (more than 20 employees) based on your own active employment or that of your spouse. It is best you consult your employer benefits administrator for advice. Contact HICAP at 1-800-434-0222 for more information. Medicare Part C (Medicare Advantage) It is optional to enroll in a Medicare Advantage Plan or Part C. If you want to enroll in one of these plans, you must be entitled to both Parts A and B. The Initial Coverage Election Period (ICEP) can be 3 months or 7 months depending on when the enrollment in Part B took place. NOTE: If you have End-Stage Renal Disease you will not be able to enroll in a Medicare Advantage Plan. You may also enroll in a Medicare Advantage Plan during the Annual Election Period (AEP) that runs from October 15th through December 7th each year, for an effective date of Medicare Prescription Part D If you are newly eligible for Medicare, the Initial Enrollment Period (IEP) for Part D is the same as the IEP for Parts A and B. To enroll in Part D, you must be enrolled in either Part A or Part B or both. People who have Medicare due to a disability have another IEP for Part D when they turn 65. If you don’t enroll in Medicare Part D during this Initial Enrollment Period and you are not eligible for Low-Income Subsidy (LIS), you can only enroll during the Annual Election Period (AEP) that runs from October 15th through December 7th or during a Special Election Period (SEP) due to certain circumstances. Contact HICAP at 1-800-434-0222 to learn more about Special Election Periods. Medigap (Supplemental) Insurance To enroll in a Medigap policy, you must have Medicare Parts A and B. If you are turning 65 or older, your 6-month Open Enrollment period for a Medigap policy begins the date your Part B becomes effective. Plans are required to sell you a policy and charge you their lowest premium for your age group regardless of pre-existing health conditions. You may apply for a policy prior to the effective date of your Part B and request your benefits begin the same date as your Medicare to avoid a break in coverage. In California, if you become eligible for Medicare due to a disability other than End-Stage Renal Disease (ESRD), you also have a 6-month Open Enrollment period once your Part B becomes effective. If you are notified retroactively of your Medicare eligibility, your Open Enrollment period begins the date you receive notice from Social Security of your right to receive Medicare benefits. NOTE: plans have the right to charge you a higher premium based on your Medicare status. If you were eligible for Medicare prior to your 65th birthday due to a disability, you are also entitled to an Open Enrollment period at age 65 regardless of pre-existing health conditions including End-Stage Renal Disease (ESRD). What happens if I do not enroll in Medicare or a Medigap when I first become eligible? Medicare Part A If you do not enroll in Part A when you are first eligible, you will have to wait until the General Enrollment Period (GEP) to do so and your benefits will not begin until the following July. You will be assessed a 10 percent penalty of the amount of the Part A premium at the time of enrollment. The penalty is assessed for twice the number of years you delayed enrolling in Medicare. Medicare Part B If you do not enroll in Part B when you are first eligible and do not qualify for a Special Election Period (SEP), you will have to wait until the General Enrollment Period (GEP) to do so and your benefits will not begin until the following July. You will be assessed a 10 percent penalty for each, full 12-month period that you qualified for coverage but did not have it. The penalty will be added to your monthly premium. Contact Social Security at 1-800-772-1213 for more detailed information. Medicare Part D If you do not join a Part D Plan when initially eligible, do not qualify for Low-Income Subsidy (LIS) or a Special Election Period (SEP), and do not have creditable prescription coverage, you will not be permitted to enroll until the Annual Election Period (AEP). You may also incur a late enrollment penalty. The penalty is 1% of the average national premium for every month you were eligible and did not sign up. This amount is added to your drug plan premium and you pay it as long as you are in a Part D plan or MA plan with prescription drug coverage. For more information, contact HICAP at 1-800-434-0222. What are “Guaranteed Issue Rights” under Medicare? Guaranteed Issue Rights, also called “Medigap Protections,” are rights you have in certain situations when insurance companies are required by law to sell or offer you a Medigap policy. In these situations, an insurance company can’t deny you a policy, or place conditions on a policy, such as exclusions for pre-existing conditions, and can’t charge you more for a policy because of past or present health problems. Examples of circumstances in which a beneficiary has Guaranteed Issue Rights to purchase a Medigap include: ♦ The 6-month Open Enrollment period at age 65 or older that begins on the effective date for Part B ♦ Your employer-sponsored retiree health plan supplementing Medicare involuntarily terminates ♦ You move out of the service area of your Medicare Advantage Plan ♦ Your Medicare Advantage plan terminates coverage in your service area NOTE: Depending on your circumstances, the duration of your Guaranteed Issue Rights vary. For more information, schedule a one-on-one appointment with HICAP at 1-800-434-0222. Medicare Premiums & Deductibles Medicare premiums and deductibles are legislated by the US Congress and change every year. Premiums are based on the individual’s or couple’s incomes. For more information, contact HICAP at 1-800-434-0222 to schedule a one-on-one appointment. Medicare for People with Disabilities I’m disabled, how do I become eligible for Medicare? If you qualify for Social Security Disability Insurance (SSDI), you may be eligible for Medicare even if you have not reached age 65. Medicare benefits generally will not be effective, however, until 24 months after the date Social Security determines you were disabled. People diagnosed with ALS (Lou Gehrig’s Disease) or End-Stage Renal Disease (ESRD) are eligible for Medicare sooner. For more information, contact Social Security at 1-800-772-1213 or www.ssa.gov For more general information regarding your benefits, please explore the pages of this website that explain Medicare coverage and options, or call HICAP at 1-800-434-0222 to schedule a confidential appointment. What other health care coverage is available? Depending on your circumstances, you may be eligible for health benefits through: ♦ Your employer ♦ Your spouse’s or parent’s employer ♦ Medi-Cal (Medicaid) ♦ Medicare Savings Programs ♦ Workers Compensation ♦ Veteran’s Administration ♦ Tricare (for retired military) For more information, call HICAP at 1-800-434-0222 to schedule a confidential appointment. What happens to my Medicare coverage when I turn 65? People who have Medicare due to a disability have another Initial Enrollment Period (IEP) for Medicare Advantage Part C and Part D Prescription Coverage when they turn 65. People diagnosed with End-Stage Renal Disease (ESRD), however, may not enroll in Medicare Advantage Part C. If you were eligible for Medicare prior to your 65th birthday due to a disability, you are entitled to an Open Enrollment period for Medigap (Medicare Supplemental Insurance) at age 65 regardless of pre-existing health conditions including End-Stage Renal Disease (ESRD). For more information, call HICAP at 1-800-434-0222 to schedule a confidential appointment. Medicare Fraud & Identity Theft What is Identity Theft? Identity theft is a serious crime that occurs when someone steals another’s personal, identifying information for the purpose of using that information fraudulently. Personal identifying information includes Social Security numbers, Medicare and Medi-Cal ID numbers, credit card and bank account numbers, patient records, and computer usernames and passwords. Fraudulent uses of this information can include opening new credit accounts, taking out loans in the victim’s name, stealing money from financial accounts, billing Medicare for services that you didn’t get, or using available credit. Sometimes identity theft is the result of a computer or institutional security breach. Other times, family members, friends, or acquaintances improperly access and use someone’s information for their own benefit. In other instances, strangers rely on the trusting nature of victims to solicit personal information to use fraudulently. How do I protect myself from Identity Theft? Do not inadvertently contribute to identity theft! Be protective of your personal information. Beware of unsolicited calls, e-mails, and visitors requesting personal, identifying information. It is possible they are fraudulently impersonating financial, medical or insurance organizations. Before you trust a stranger with your information, verify they are who they say they are. Let’s see some examples: 1. Your telephone rings and you answer it. The person on the other line calls you by name and says, “Good morning. I am calling from the Nevada Social Security office and I need to update your personal information for our files.” You proceed to give the caller the requested information. A few months later, you try to use your prescription Part D card at the pharmacy and are told you have no coverage. Why? The call you received a few months ago was not, in fact, from Social Security but from a private insurer that switched you to their plan. 2. A family member uses your personal information to fraudulently impersonate you without your knowledge. Soon after they leave town, you discover they have maxed out a credit card in your name and emptied your bank accounts. You find yourself penniless and working with Adult Protective Services and the police to clean up your credit report and get back on your feet. Don’t let these scenarios happen to you! What is Medicare Fraud and where would I see it? Medicare Fraud occurs when someone INTENTIONALLY misrepresents services or equipment, resulting in payment of an UNAUTHORIZED benefit to a person or an organization. These fraudulent expenses ultimately cost the Medicare program and you, the beneficiary. Examples of Medicare Fraud include: ♦ Fraudulent billings from nursing homes, hospitals, and hospices (Medicare Part A) ♦ Fraudulent billings for providers’ services (Medicare Part B) ♦ Fraudulent billings for Durable Medical Equipment (DME) such as wheelchairs, walkers, electric scooters, and incontinence supplies ♦ Pressure sales, usually door-to-door or over the telephone, offering a free prescription plan (Medicare Part D) or saying you have to change your Medicare coverage because your Medicare benefits have decreased. How can I protect myself from being a victim of Medicare Fraud? You can protect yourself from Medicare Fraud by doing the following: ♦ Review your Medicare Summary Notice to ensure you did, in fact, receive the services, supplies or equipment for which Medicare has paid. ♦ Sign up with “My Medicare” to see your Medicare Summary Notices online at www.MyMedicare.gov. ♦ Protect your Medicare and Social Security number. ♦ If someone is trying to convince you that you need a certain service, piece of equipment, or to see a doctor that you, in fact, do not need, DECLINE. ♦ Ask questions regarding what services you are receiving and why. BE INFORMED about your Medicare-billed care. ♦ NEVER purchase or accept medical supplies from door-to-door salespeople. ♦ Do not give out any of your personal information such as your Medicare number, Social Security number, banking information, and credit card numbers to anyone you do not know or whose business you did not solicit. What do I do if I suspect Medicare Fraud? If you suspect Medicare Fraud, you can take some of the following steps. ♦ Call HICAP at 1-800-434-0222. This HICAP belongs to the Senior Medicare Patrol (SMP) for reporting fraud. ♦ Call Medicare at 1-800-MEDICARE. ♦ Call the Inspector General’s Medicare Fraud Hotline at 1-800-477-8477. What other healthcare issues can HICAP help you with? Long Term Care Considerations: Long-Term Care (LTC) insurance only pays for long-term care. Long-term care usually means supervision or assistance with everyday tasks like bathing and dressing and does not require a licensed person to provide those services. Some of these policies only pay for care in institutional settings such as a nursing home or an assisted living facility; some only pay for home care; while other more comprehensive policies pay for care in a nursing home, assisted living facility, at home, or in community settings like adult day care. Some life insurance policies and annuities also contain benefits for long-term care. If you are considering buying LTC insurance, you will need to do some research to find the best set of benefits for your particular circumstances, and the best company for those benefits. You will also need to consult your accountant or tax advisor to understand any tax issues that might affect you. Counselors from the California Health Insurance Counseling and Advocacy Program (HICAP) can help you: ♦ Sort through some of these issues ♦ Understand how these benefits work ♦ Compare benefits and features of several policies ♦ Compare policies from more than one company To make an appointment for HICAP counseling, call 1-800-434-0222. 25 Main Street Chico, CA 95929 Tel // 530-898-5923	2	12	1	0	0	0	0.883979	1	5,010
- 1 What is Lennox Gastaut Syndrome? - 2 Lennox Gastaut Syndrome Symptoms - 3 Complications - 4 What Causes Lennox Gastaut Syndrome - 5 Lennox Gastaut Syndrome Tests and Diagnosis - 6 Treatment and Management What is Lennox Gastaut Syndrome? Lennox Gastaut Syndrome (LGS), also called childhood epileptic encephalopathy, is a rare severe form of progressive epilepsy, characterized by multiple types of seizures that are most prominent during infancy or early childhood (age 3-8 years), but may persist through adolescence to adult years. Affected children may also have intellectual functioning impaired as well as face behavioral problems and delayed development. The disorder, first discovered by Tissot in 1770, can be classified into two types on the basis of the cause of the symptoms: - Symptomatic LGS - Cryptogenic or Idiopathic LGS Lennox Gastaut Syndrome Symptoms Symptoms consist of different types of seizures and neurological malfunctions. Amidst the seizures, the more common are the tonic and atonic or “drop” seizures. Tonic Seizures, affecting about 74-90% of children with LGS are brief, occur mostly at night and can be triggered by noise, contact or movement. They may cause: - A slight bending of the body inward due to muscle contractions - Brief holding of breath - The opening of mouth and rolling of eyes - Abnormal rise of upper arms and legs much like a ballet dancer - Sudden collapse of the affected individual due to short loss of consciousness Atonic Seizures, also lasting for a minute time are characterized by: - Dropping of eyelids and nodding of head - Sudden falls - A tendency to collapse forward or backward particularly at the waist - Brief consciousness loss - Jerking muscle movements Atypical Absence Seizures, which are abrupt, are characterized by: - A span of unconsciousness - Unresponsive staring - Twitching around eyes or mouth - Stiffening of neck - Drooping of head and body Less common forms of seizures occurring in the condition include myoclonic, tonic-clonic, status epilepticus and nonconvulsive status epilepticus seizures. The less frequent neurological symptoms that generally develop with time involve: - Difficulty in sitting, crawling or walking suggesting delayed motor development - Loss of previously learned skills - Hyperactivity, aggressiveness and a tendency to seek attention suggesting behavioral problems - A slowing down of thought with reduced physical movement indicating psychomotor regression - Drop seizures replaced by partial seizures and secondarily generalized convulsions - Acute psychotic episodes - Chronic psychosis - Mental retardation - Cognitive incapability involving slowness in response and information processing - Head injuries and even death from fall or seizure - Renal, cardiac or metabolic complications - Cerebral palsy - Hearing loss What Causes Lennox Gastaut Syndrome The reasons behind Symptomatic Lennox-Gastaut syndrome, which accounts for nearly 70-80% of cases, are: - Internal injuries of brain resulted from problems before, at or during birth such as prematurity, insufficient supply of oxygen called perinatal hypoxia and low birth weight - Abnormal brain cortex development suggesting cortical dysplasia - Severe brain infections such as encephalitis or meningitis - A rare genetic disorder known as tuberous sclerosis - Degenerative or metabolic nervous system disorders Cryptogenic LGS which does not have an identifiable cause may have a later onset. ** Although in most cases, the causes are identifiable, yet the exact mechanisms that bring about the seizures are not known, and more researches need to be done to derive a genetic basis of the syndrome. - Family history of epilepsy - West syndrome Lennox Gastaut Syndrome Tests and Diagnosis The diagnosis of LGS is based on the identification of the clinical triad that comprises of multiple seizures, an abnormal EEG with a significant spike-wave pattern of the brain and some degree of cognitive and behavioral dysfunction. An advanced imaging technique such as MRI is also done to get three-dimensional images of the brain anatomy. - Early onset childhood absence epilepsy - Dravet syndrome - Atypical benign partial epilepsy - Myoclonic astatic epilepsy Treatment and Management Administration of Anti-Epileptic Drugs (AEDs) Treatment may start with medications, which often include: - Valproate, which may be given alone or in combination with other drugs like lamotrigine, topiramate or clobazam. Though valproate is beneficial for all types of seizures, yet it has side effects such as tremors or reduction in platelet counts. - Another drug of choice, topiramate may help lessen both drop attacks and multiple seizures but an increased dose can have side effects like sleepiness and ataxia. - Clonazepam, the most commonly used representative of the benzodiazepine class of drugs may have partial effect in myoclonic seizures. - Lamotrigine seems to be efficient in multiple seizures as well as a mood stabilizer. - Felbamate may help promote alertness, but it too can induce aplastic anemia. - FDA has approved Levetiracetam for partial seizures, but it may prove efficient in other seizures too. - Rufinamide (Benzel) is used as an adjunctive therapy for adults and children aged one or above having seizures. Occupational therapy, speech and physical therapy are the rehabilitation procedures that may help children suffering from the syndrome in reducing the levels of disability. The Ketogenic Diet However, since the above mentioned drugs may lose their effect with the passage of time, the condition may be treated with the help of another approach called the ketogenic diet. This strict high fat, low carbohydrate diet is initiated after a 24-hour fasting, intending to raise the ketone level in the brain. By restricting an intake of carbohydrates, this diet forces the body to burn fat to get energy instead of sugar. Followed under the strict supervision of a doctor and dietician, it does not prevent the use of medications. Vitamins and minerals may be supplemented to avoid a deficiency. Though proving effective in a minority of patients, it may have side effects like dehydration, constipation, kidney stones and vomiting. Surgical Therapies – Lennox Gastaut Syndrome Patients not responding to either medications or dietary therapy undergo a surgical procedure called corpus callosotomy. This surgery, accomplished by cutting the large bundle of nerve fibers called the corpus callosum that connects the two hemispheres is done to reduce the severity of drop attacks, tonic, and tonic-clonic seizures. According to several studies, in 50- 70% of cases, partial callosotomy is beneficial whereas complete callosotomy may result in the decrease of drop attacks in about 80-90% of cases. Vagus Nerve Stimulator This surgery lasting for about an hour and usually conducted under anesthesia, involves the insertion of a device called a pulse generator into the chest to transmit mild, electrical impulses to the brain through the vagus nerve. This process, intending to reduce the frequency of the seizures may have side effects such as voice hoarseness, tickling in the throat and coughing. The other surgical procedures are RNS stimulator, deep brain stimulation, and trigeminal nerve stimulation. Treatment Through Nonpharmacological Means Include - Intravenous Immunoglobulin (IVIG): IVIS is a blood product that can be given intravenously in the treatment of LGS. - Medical Marijuana/CBD (Cannabidiol): A compound called cannabidiol found in cannabis might reduce chemical as well as electrical activity that occurs in the brain and thus may be used therapeutically for patients living with LGS. The annual incidence of children having LGS is about 2 per 1000,000 children. Nearly 1-2% of all childhood epilepsies are represented by LGS. Amidst this, 75-90% children suffer from mental retardation which generally begins within a few years of the onset of the syndrome. Prognosis and Life Expectancy The prognosis of this progressive syndrome varies with individuals. Seizures continue in more than 80% of patients. MRIs and other clinical exams may occasionally be repeated to look for any change in the brain. The mortality rate of the syndrome ranges from 3% to 7% with most of the deaths occurring from accidents. The degree of disability may be lessened with early and useful treatment. Recovery also depends on the combined efforts of health care providers such as social workers and neuropsychologists. ICD Code- 9 and ICD Code- 10 The icd-9 code for LGS is 345.0 and the ICD- 10 Code is G40.814.	2	8	0	0	0	2	0.687876	2	1,872
I. Pulmonary Arterial Hypertension: What Every Physician Needs To Know. Pulmonary arterial hypertension (PAH) is a disorder specific to the pulmonary arteries, resulting in an increase in pulmonary artery pressure (PAP), and pulmonary vascular resistance (PVR), leading to right ventricular (RV) dysfunction, right heart failure, and death. Pulmonary hypertension (PH) refers to elevated pressure in the pulmonary vasculature that can result from a wide range of conditions. It is critical to go through the appropriate diagnostic tests to determine whether your patient has PAH versus PH related to other underlying systemic disorders since treatment approaches are vastly different. Right heart catheterization (RHC) is the only method of diagnosing PAH (see section II: Right Heart Catheterization: Necessary to Diagnose PAH). There are currently nine PAH-specific treatments approved by the U.S. Food and Drug Administration (FDA). It is imperative to note that these treatments are not approved or indicated for PH, and can be harmful when used inappropriately (see Management section). Pulmonary hypertension is defined as a resting mean pulmonary artery pressure (mPAP) greater than or equal to 25 mm Hg. Pulmonary arterial hypertension is defined as a resting mean pulmonary artery pressure (mPAP) greater than or equal to 25 mm Hg, and a pulmonary capillary wedge pressure (PCWP) less than or equal to 15 mm Hg. The American College of Cardiology Foundation and the American Heart Association definition of PAH also includes pulmonary vascular resistance greater than 3 Wood units. The current classification comes from the 4th World Symposium on Pulmonary Hypertension held in 2008 at Dana Point. PH is classified into five groups listed below. In essence, PAH includes the idiopathic PAH (IPAH) and associated conditions that affect pulmonary arteries, with similar presentations and responses to PAH-specific medical therapies. It should be noted that all clinical trials performed resulting in approval of therapies have been done on the Group I PAH population. Group 1: Pulmonary arterial hypertension (PAH) Drug- and toxin-induced PAH Connective tissue diseases Congenital heart diseases Chronic hemolytic anemia Persistent pulmonary hypertension of the newborn Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH) Group 2: Pulmonary hypertension owing to left heart disease Group 3: Pulmonary hypertension owing to lung diseases and/or hypoxia Chronic obstructive pulmonary disease Interstitial lung disease Other pulmonary diseases with a mixed restrictive and obstructive pattern Alveolar hypoventilation disorders Chronic exposure to high altitude Group 4: Chronic thromboembolic PH (CTEPH) Group 5: PH with unclear multifactorial mechanisms Hematologic disorders: myeloproliferative disorders, splenectomy Systemic disorders: sarcoidosis; pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, neurofibromatosis, vasculitis Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on dialysis 1. Primary pulmonary hypertension or “PPH”; this term is still frequently used in the clinical setting; officially no longer supported in literature. It has been replaced by the term idiopathic pulmonary arterial hypertension or IPAH. 2. Secondary pulmonary hypertension; similarly this term is no longer used in literature. It was used to denote PH of all types, except for PPH. 3. Precapillary PH; this is a hemodynamic terminology, referring to pulmonary arterial hypertension (elevated pulmonary pressure stemming from precapillary region [i.e., normal PCWP]). 4. Postcapillary PH; this is a hemodynamic term, referring to pulmonary venous hypertension (PVH) (elevated pulmonary pressure stemming from the postcapillary region; that is, elevated PCWP). PAH results from restriction of blood flow through pulmonary arteries that leads to increase in PVR and consequently resulting in right heart failure. The increase in PVR is related to various mechanisms including: vasoconstriction, obstructive remodeling of the pulmonary vessel wall, inflammation, and thrombosis. Though the process by which PAH is initiated and progresses is heterogeneous, it is thought that PAH is the result of an interaction of a predisposing state plus one or more stimuli, a concept termed “multiple-hit hypothesis.” It involves an individual rendered susceptible due to genetic abnormality or substrate. The second “hit” may be either a systemic disorder (i.e., collagen vascular disease or HIV), an environmental trigger (i.e., hypoxia or anorexigen), or additional genetic conditions (i.e., mutation or polymorphism). Since the absolute risk of PAH (or those with known risk factors) is generally low, it is believed that individual susceptibility or genetic predisposition likely plays a significant role in the disease initiation and progression. This is thought to be an early component of the pulmonary hypertensive process. Significant vasoconstriction has been linked to abnormal function or expression of potassium channels in the smooth muscle cells and to endothelial dysfunction. B. Endothelial Dysfunction Mediated Processes Currently Targeted for PAH Treatments The PA endothelium tightly regulates the production of vasodilators and vasoconstrictors to maintain a low-pressured state. In PAH, an imbalance of the mediators occur resulting in overproduction of vasoconstricting agents and diminished level of vasodilators (Figure 1). Prostacyclin and Thromboxane A2 – these are metabolites of arachidonic acid. Prostacyclin is a potent vasodilator, inhibitor of platelet activation, and exerts antiproliferative effects; thromboxane A2 is a potent vasoconstrictor and promotes platelet activation. In PAH, there is a decreased prostacyclin synthase and an increased production of thromboxane A2 leading to decreased prostacyclin. Nitric oxide (NO) – this produces vasodilatation, inhibits platelet activation and vascular smooth muscle cell proliferation. The effects of NO are mediated through its second messenger, cyclic guanosine monophosphate (cGMP), which is rapidly degraded by phosphodiesterase (PDE). NO in the pulmonary circulation is degraded by PDE-5 isoenzymes, which is present in abundance in the lung tissue. This is the basis of using PDE-5 inhibition in PAH. Endothelin-1 (ET-1) – this is a potent vasoconstrictor and stimulator of PA smooth muscle cell proliferation. The plasma level of ET-1 is increased in PAH and its level has been shown to be inversely proportional to the magnitude of the pulmonary blood flow and cardiac output. ET-1 exerts its effects through two receptors – ETA and ETB. Clearance of ET-1 in the pulmonary vasculature is reduced in PAH. The rationales of current therapeutic approaches are directed at correcting the imbalance of the mediators by: 1. Augmenting actions of prostacyclins and increasing NO-induced activity 2. Blocking the ET-1 mediated processes C. Additional mechanisms 1. Serotonin (5-hydroxytryptamine) – is a vasoconstrictor that promotes smooth muscle cell hypertrophy and hyperplasia. Elevated plasma serotonin and reduced content of serotonin in platelets have been reported in IPAH and PAH associated with ingestion of dexfenfluramine, which increases the release of serotonin from platelets and inhibits its reuptake. Furthermore, mutations in the serotonin transporter (5-HTT) and its receptor 5-HT2B have been described in PAH patients. However, it is not certain if elevated serotonin levels are implicated in PAH since selective serotonin-reuptake inhibitors (SSRI) are not associated with an increased incidence of PH and remains unclear (see below). 2. Potassium channels – Inhibition of voltage-dependent potassium channels (Kv) have been linked to factors which promote PAH, such as hypoxia and fenfluramine derivatives. 3. Abnormalities of the coagulant cascade – Including increased levels of von Willebrand factor, plasminogen activator inhibitor-1, and plasma fibrinopeptide have been reported in PAH patients. 4. Inflammatory factors – Proinflammatory cytokines and autoantibodies have been implicated in PAH. D. Genetic Substrates Mutations in the gene encoding bone morphogenetic protein receptor type 2 (BMPR2) have been found to be associated with heritable PAH. BMPR2 is a component of the heteromeric vascular smooth muscle cell BMPR receptor, a member of the transforming growth factor ß (TGFß) signaling pathway. These mutations result in cellular proliferation. Activin-like kinase (ALK1), a less common mutation also in the TGFß family, is associated with hereditary hemorrhagic telangiectasia and PAH. In PAH, it has been observed that there is a collection of abnormalities that favor a decreased apoptosis/proliferation ratio in PA smooth muscle cells. These abnormal factors include inappropriate activation of transcription factors HIF-1α and NFAT, decreased expression of certain K+ channels (i.e., Kv1.5 and Kv2.1) and de novo expression of the antiapoptotic proteins. Histologic changes include: 1. Medial hypertrophy – due to both hypertrophy and hyperplasia of smooth muscle fibers, as well as an increase in connective tissue matrix; results in increase in the cross-sectional area of the media 2. Intimal thickening – occurring either as concentric or eccentric patterns, immunohistochemical stains show features of fibroblasts, myofibroblasts, and smooth muscle cells. 3. Adventitial thickening – seen in most cases of PAH 4. Plexiform lesions – end-stage lesions formed by a focal proliferation of endothelial channels lined by myofibroblasts, smooth muscle cells, and connective tissue matrix. Arteritis may be associated with plexiform lesions. Although it is the pulmonary arterial vasculature where the pathologic processes take place in PAH, the factor which determines symptoms and survival rests on the ability of the RV to function under the increased pressure and resistance. The RV is a thin-walled, compliant, crescent-shaped structure, formed by the RV free wall and the interventricular septum. Due to the low resistance of the pulmonary vasculature, the compliant RV is designed to pump the same stroke volume as the LV with 1/6 of the work. The determinant factor rests on how well the RV adapts to the increased afterload in PAH. Normal RV is coupled to low pressure in the pulmonary vascular system. In pulmonary hypertension, RV becomes uncoupled, challenged with elevated afterload in the pulmonary vasculature. The RV demonstrates a heightened sensitivity to changes in afterload and the RV stroke volume decreases proportionately to acute increases in afterload. The initial response is usually RV hypertrophy, although early studies in postacute pulmonary embolus demonstrated that previously normal RV is incapable of acutely generating mPAP >40 mm Hg. This hypertrophic process can be followed by contractile dysfunction and/or RV dilatation for further compensatory maneuver in order to maintain cardiac output. Continued remodeling of the RV soon causes alterations in RV shape from crescent to concentric, which in turn flattens out the septum. Ventricular interdependence refers to the concept that the size, shape, and compliance of one ventricle may affect the same factors in the neighboring ventricle. In the presence of RV volume or pressure overload, the interventricular septum shifts toward the left and limits LV filling and output. The consequences of RV remodeling include: (1) Decreased coronary perfusion pressure in the setting of increased oxygen demand; (2) due to interventricular dependence, RV remodeling results in LV diastolic dysfunction and a decrease in LV end diastolic volume. These changes result in RV-PA uncoupling, a term coined to describe the inability of the RV to work in concert with the high afterload of the PA. The end result is further decline in stroke volume and deterioration of end organ perfusion. Interestingly, the development of RV failure due to PH is quite variable. It is unclear why some RVs can compensate maintaining adequate cardiac output for prolonged periods while others immediately dilate and progress into right heart failure. Several mechanisms have been proposed including: (1) Retention of the “fetal” genotype, which is believed to be a contributory factor resulting in favorable outcome for PAH associated with congenital heart disease (CHD); (2) polymorphisms in genes related to the renin-angiotensin-aldosterone system; (3) differences in the degree of ischemia and apoptosis. Although the past two decades have witnessed an explosion of knowledge in PAH, culminating in nine FDA therapies for this “orphan” disease, the reality is that there is still a considerable delay in the recognition and diagnosis of PAH. The ongoing Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) database is the largest collection of information dedicated to PAH patients with 54 centers participating in the U.S. One of the most revealing findings is the fact that despite our advances in PAH, there is still a significant delay between symptom onset to diagnosis, with the median time reported to be 13.6 months (34.1 +/- 1.2 months). When one compares this duration to the measurement assessed with the original NIH PPH Registry conducted in the 1980s when very limited information was known about this condition, it was 2.0 years. Thus there has been some improvement, but the delay is still significant. Why diagnosing PAH is challenging: Relative rarity of the disease (diagnosis not considered by most physicians). Nonspecific nature of the presenting symptoms, such as shortness of breath and fatigue. Common misdiagnoses include asthma, deconditioned or being “out of shape,” weight gain and obesity, and depression. Routine outpatient tests (labs, electrocardiography, chest radiograph) are not sensitive nor specific to detect until condition is advanced. No easily identifiable physical examination characteristics (see section IV: Physical Examination). In order to evaluate and manage a patient with PAH, the physician must: Recognize predisposing factors and comorbidities that place certain patients at risk. Acknowledge that a high degree of suspicion is necessary to proceed to screening for PAH with an echocardiogram due to the nonspecific nature of the presenting symptoms. Know the usefulness and pitfalls of echocardiography as a screening tool. Understand how to complete an evaluation for PAH to determine the type of PH. Know that right heart catheterization (RHC) is the only method of diagnosing PAH. Regarding acute vasoreactivity testing, know when it is indicated and how to interpret the data. Perform risk assessment to determine appropriate therapy. Know the pros and cons of each therapy. Know the goals of treatment. Know how to follow patients long-term to determine treatment response and efficacy. Know how to manage PAH crisis/right ventricular failure. Know what the indications are to refer for lung transplantation. II. Diagnostic Confirmation: are you sure your patient has Pulmonary Arterial Hypertension? If PH is suspected (based on history, risk factor assessment, and physical examination), an echocardiogram is the next step. The Doppler echocardiogram provides the following: Estimation of RV systolic pressure Done by using the modified Bernoulli equation 4v2 v is the velocity of the tricuspid regurgitation (TR) jet in m/s. Right ventricular systolic pressure (RVSP) is derived by adding the right atrial pressure (RAP) to the gradient (RVSP = 4v2 + RAP). The RAP is either a standardized value or an estimated value based on the echocardiographic characteristics of the inferior vena cava, or the vertical height of the jugular venous pulse on physical examination. Degree of correlation between RVSP derived from TR jet and hemodynamics from RHCs with marked various results from poor to significant correlations. Several factors attribute to the various results: Spectral Doppler profile of TR is too weak or insufficient to measure the RV to RA pressure gradient in about 10% to 25% patients referred for PH evaluation. The signal can be enhanced by intravenous bolus administration of a small amount of agitated saline contrast or with commercially available encapsulated microbubble contrast agents which are used to enhance imaging quality. Very small amounts are needed to define TR jet, but the encapsulated agents need to be used with caution in patients with significant pulmonary vascular disease/pulmonary disorder. Patients with comorbidities (obesity, pulmonary disorder) have demonstrated worse correlation, mostly related to difficulty in obtaining adequate acoustic windows. Thus it is important to note the technical quality of the study to determine how reliable the echo is to indicate possible presence of PAH. Lack of accuracy of RAP estimation can significantly influence the RVSP result, especially in “borderline” elevated pressure. Doppler echocardiography may underestimate RVSP in patients with severe PH due to inadequate jet visualization and overestimate in patients with near normal pressures usually as a result of inaccurate RAP estimation. Evaluation of RV size and function to assess functional and morphologic cardiac consequences due to PAH Size and function of the RV have been shown to be critical determinants in the outcome of patients with PAH. Presence of enlarged right atrium and RV, septal flattening, and reduced RV systolic function highly correlate with significant PH and are well recognized markers of poor prognosis. Methods of assessing quantitative measures of RV function: Visual estimation – most often used but highly subjective. RV fractional area change done in apical four chamber view. Though this method incorporates both longitudinal and transverse RV shortening into a single measurement, it can be limited by incomplete endocardial definition leading to high variability. TAPSE (tricuspid annular plane systolic excursion) – simple to perform and has shown to be reproducible in PAH. This measures the total longitudinal systolic displacement of the RV base toward the RV apex, performed using the 2-D echocardiography or M-mode. Normal TAPSE is 2.4-2.7 cm. Ranges of RV dysfunctions are: mild (1.9-2.2 cm), moderate (1.5-1.8 cm), and severe (<1.5 cm). Presence any pericardial effusion (even trivial) has been found to be associated with worse prognosis. To rule out left heart disease (LHD) as a cause of PH since diagnosis of PAH mandates the absence of clinically significant LHD. Assessment of LV size, structure and function is important to exclude significant LV systolic and diastolic dysfunction, enlargement, and hypertrophy. Close scrutiny for diastolic abnormalities is becoming more relevant since older patients with multiple cardiac risk factors are being diagnosed with elevated pulmonary pressure (see Competing Diagnoses That Can Mimic Pulmonary Hypertension section). Presence of left atrial enlargement, even in the absence of obvious LV dysfunction, should raise the suspicion of elevated left-sided pressure and a diagnosis of pulmonary venous hypertension (PVH) rather than PAH. In these patients, it is critical to obtain a reliable pulmonary capillary wedge pressure (PCWP) during right heart catheterization; if a satisfactory PCWP cannot be obtained, left heart catheterization may need to be performed to accurately measure the left ventricular end diastolic pressure to definitively rule out PVH. PAH-specific therapies can worsen PVH by precipitating pulmonary edema. It has been suggested that LA size can be viewed for PH as HgbA1c for DM. It is a marker of duration, chronicity, and severity of left-sided pressure elevation Assess for valvular abnormality and congenital heart disease, which can precede or coincide with the diagnosis of PH. An echocardiographic contrast (“bubble”) study using agitated saline solution can screen for presence of intracardiac shunting. Transesophageal echocardiography (TEE) can add more refined structural information in these cases and may better define atypically situated atrial septal defects of the sinus venosus type (Table 2). Right Heart Catheterization: Necessary To Diagnose PAH Right heart catheterization is needed in PAH to: Establish the diagnosis of PAH by confirming that pulmonary artery pressures are elevated with normal left sided filling pressure. Perform acute vasodilator testing for patients with a new diagnosis of PAH of idiopathic, hereditary, or anorexigen-associated conditions. Assess prognosis of PAH based on RAP and cardiac output measurements. Guide treatment of the patient The normal PAP is systolic 15-30 mm Hg, diastolic 4-12 mm Hg, and mean 9-18 mm Hg. The normal PA pulse pressure is approximately 15 mm Hg. The upstroke of the PAP waveform marks the onset of RV ejection followed by systolic peak and pressure decay. The dicrotic notch is due to pulmonic valve closure. The peak of the PAP wave occurs within the T wave of surface electrocardiography. A gradient should not exist between the RV and PA systolic measurements, unless there is pulmonary valvular or pulmonary artery stenosis (Table 3). PAP waveform is affected by respiratory changes similar to other right-sided pressure. The pressure changes upon cardiac chambers due to respiration are negligible under normal physiology. Important exceptions to this are seen for patients on mechanical ventilatory support, respiratory distress, marked pulmonary disease, or morbid obesity, where a significant variation in intrathoracic pressure can be seen resulting in marked differences in PAPs with the respiratory cycle. A reading at the end-expiration should be obtained since this is the phase where intrathoracic pressure is closest to zero. Pulmonary Artery Pressure Measurements in PAH The PA diastolic pressure is close (within 2-4 mm Hg) to both the mean PCWP and left ventricular end-diastolic pressure (LVEDP) in normal individuals (normal pulmonary vascular resistance and in the absence of mitral valve disease). The PA diastolic pressure does not correlate well with the mean PCWP in the presence of pulmonary vascular disease where the PA diastolic pressure overestimates the mean PCWP. The lack of correlation is also seen in mitral regurgitation with large v wave where the PA diastolic pressure underestimates the mean PCWP. The normal mean PCWP is 2-12 mm Hg. The PCWP is used as a measurement of left-sided filling pressure and under normal physiology, a close correlation exists between the PCWP, left atrial pressure, and LVEDP. Conditions where the PCWP does not correlate with LVEDP include in the presence of following conditions: Severe mitral regurgitation or aortic regurgitation Pulmonary venous obstruction Significant LV noncompliance Accurate measurement of left heart filling pressure is critical for the correct diagnosis of PAH: Definition of PAH requires both elevation of mPAP (≥ 25 mm Hg) and normal PCWP or LVEDP (≤ 15 mm Hg). The difference between these two measurements calculates the transpulmonary gradient (TPG = mPAP – PCWP in mm Hg). Accurate PCWP is critical in calculating PVR (PVR = TPG/CO in Wood units). Elevated PCWP is characteristic of PH in the setting of chronically elevated left-sided cardiac filling pressure, termed pulmonary venous hypertension (PVH), and is classified as WHO Group 2 PH (see Clinical Classification section). PVH usually results from systolic and/or diastolic cardiac dysfunction or valvular heart diseases. Therefore, therapeutic decisions can be significantly different based on the left-sided filling pressure measurement. PAH is characterized by elevated PAPs, normal PCWP, and elevated TPG. PVH is characterized by elevated PAPs, elevated PCWP, and normal TPG. Common Pitfalls in Obtaining Accurate PCWP: Underwedging or Hybrid Waveform “Underwedging” typically occurs with incomplete advancement of the PA catheter resulting in a hybrid tracing of PAP and PCWP. This usually results in a falsely elevated PCWP, leading to misdiagnosing a patient as PVH. If an operator is suspicious that the PCWP being measured is greater than expected based on clinical assessment, an oxygen saturation measurement can be done from the distal port with the catheter in the wedge position. It should be equal or close to the systemic arterial oxygen saturation (usually >90%) done by pulse oximetry. If lower, the catheter is most likely underwedged. Placing the catheter in the correct PCWP position can be very difficult in a patient with significantly elevated PAPs, especially if PA is markedly dilated. One helpful maneuver is deflating the balloon, allowing the catheter to migrate distally, and carefully reinflating the balloon following the pressure tracings closely. Usually with this approach, optimal placement is obtained with balloon partially inflated. An intraluminal guide wire can also aid in advancing the catheter to a more distal position. All these maneuvers must be performed very cautiously and under direct fluoroscopic visualization; it must be remembered that patients with PAH are at increased risk of PA rupture, a likely fatal event. Common Pitfalls in Obtaining Accurate PCWP: Overwedged Waveform “Overwedged” tracing occurs due to excessive inflation of the balloon relative to the size of the vessel. The result is a characteristic pressure tracing, which is usually easily detected. This should be avoided not only due to inaccurate pressure measurement but due to the increase in risk of vessel rupture. In bedside catheter measurements, the potential for PA catheter migration also needs to be kept in mind. The balloon should be slowly inflated at every measurement with close monitoring of the pressure tracings, with inflation stopped when a PCWP tracing is obtained. Common Pitfalls in Obtaining Accurate PCWP: The “V” Wave The v wave is a normal finding on the wedge tracing and normally higher than the a wave so as to what measurement constitutes a “large” v wave can be subjective. Common causes of a large v wave include mitral regurgitation (MR), though the height of the v wave is neither sensitive nor a specific indicator of the degree of MR. Other causes include any situations that increase the volume or flow into a noncompliant left atrium, such as ventricular septal defect, mitral stenosis, cardiomyopathy of any etiology, or postoperative surgical conditions. The presence of a large v wave distorts the correlation between the PA diastolic pressure and PCWP so PA diastolic pressure now underestimates the mean PCWP. A large v wave causes the PCWP to overestimate the LVEDP. The best point to estimate the LV filling pressure in the presence of a large v wave is to measure the wedge pressure at end diastole, which coincides with end of a wedge pressure a wave. In PAH where determination of an accurate wedge pressure is critical to guide therapy, if there is any question regarding accuracy or reliability of the measurement, obtaining LVEDP is recommended. Characteristics of a Physiologically Reliable PCWP Distinct a and v waves should be present. An exception is noted in atrial fibrillation where an a wave will be absent. Need to wait for steady state in PCWP tracing to occur (not immediately after the balloon is inflated) and record at end-expiratory phase. A distinct immediate rise in pressure when balloon is deflated out of the wedge position. Catheter tip should be stable in PA when viewed under fluoroscopy with the balloon inflated (not moving back and forth). An oxygen saturation measured in PCWP >90%. Multiple measurements of PCWP with similar results. If these maneuvers fail to obtain a reliable PCWP, a left heart catheterization should be performed to measure LVEDP. Normal cardiac output (CO) at rest is 5-8 L/min and the normal cardiac index is 2.6-4.2 L/min/m2 (see Table 3). Accurate cardiac output measurement is critical in calculating PVR and assessing prognosis in PAH. The total pulmonary resistance (TPR) calculates the relationship between the mPAP and CO: TPR = mPAP × 80/CO; the normal TRP is 100-300 dynes-sec-cm-5. The PVR measures the resistance to flow imposed by the pulmonary vasculature without the influence of the left-sided filling pressure: PVR = (mPAP – PCWP) × 80/CO or PVR = TPG × 80/CO; the normal PVR is 20-130 dynes-sec-cm-5 (PVR = TPG/CO in Wood units) CO assessment is a critical measure of RV performance in the presence of elevated PAPs and decreased cardiac index (<2.2 or 2.5 L/min/m2, pending sources) is a marker of poor prognosis. Fick Cardiac Output The Fick output equation is based upon the principle that in the absence of a significant intracardiac shunt, the pulmonary blood flow and the systemic blood flow are equal. CO = oxygen consumption/arteriovenous oxygen difference, or CO = oxygen consumption/(arterial saturation – mixed venous saturation) × (Hgb) × 13.6. The measurement of the arteriovenous oxygen (AV O2) difference provides an estimate of the cardiac output. The arterial oxygen saturation is the constant factor for most individuals. Thus changes in AV O2 difference reflect the increase or decrease in the mixed venous oxygen saturation. A decrease in the CO is compensated by an increase in tissue oxygen extraction, which results in the decrease in the mixed venous saturation. The correlation between cardiac output and changes in mixed venous oxygen saturation applies in the setting of constant hemoglobin, arterial oxygen saturation, and oxygen consumption. In most hemodynamic laboratories, due to the impracticality of obtaining the exact oxygen consumption which requires collecting the patient’s exhaled air over several minutes, “assumed Fick” is used, which is derived from oxygen consumption based on the patient’s age, gender, and body surface area. Potential sources of error include incorrect measurement of venous or arterial blood samples, presence of mitral or aortic regurgitation, and intracardiac shunting or peripheral shunting at the tissue level, such as in septic shock. Thermodilution Cardiac Output Thermodilution cardiac output is derived by injecting a 10-ml bolus of saline over 2-4 seconds into the RA via the proximal port. This is done with the distal catheter tip in a stable position in the PA and not in a “wedge” position. The cardiac output measurements are obtained in triplicates, and the variances between the measurements should be less than 10%. Thermodilution method can potentially overestimate CO in low flow states when compared with Fick method. The thermodilution method is known to be inaccurate in the presence of severe TR, intracardiac shunts, low output states, and marked respiratory variation. With significant TR, a portion of the indicator warms during its prolonged stay within the RA and RV, which produces a characteristic curve with a slow decay to baseline temperature. Although the Fick method is used often in the presence of TR, studies have demonstrated conflicting results regarding the accuracy of both techniques. Evaluation for Intracardiac Shunting Chronic left-to-right intracardiac shunting can produce PAH. Echocardiogram with agitated saline contrast can detect right-to-left shunt but can fail to detect left-to-right shunts. Multiple measurements of oxygen saturations from superior and inferior vena cava, right atrium, and PA can detect and quantify shunts. This step is a crucial part of right heart catheterization in a patient with clinical or echocardiographic suspicion of intracardiac shunting. The risks associated with RHC in PAH has been studied. A recent multicenter study, which included 15 PAH centers over a 5-year period with more than 7,000 procedures evaluated the safety and risks of performing RHC in the PAH population. The overall incidence of serious adverse events was 1.1%. The most frequent complications were related to obtaining venous access; others included arrhythmia and hypotension due to vagal reactions or pulmonary vasoreactivity testing. When performed in experienced centers, RHC in PAH patients are safe and associated with low morbidity rates. Maneuvers To Enhance Safety RHC can be performed via femoral or jugular approach and is often determined by operator’s preference. Accessing from an internal jugular approach allows use of an ultrasound device to visualize the size and depth of the vein, which can enhance safety. PAH patients often have dilated right-sided chambers, which can make maneuvering the catheter difficult, especially under high pressure systems and under significant tricuspid valvular regurgitation. Performing the procedure under fluoroscopy reduces the risks of catheter “coiling” and inducing arrhythmia. Direct visualization also assists in placing the catheter in the safe and optimal “wedge” position to avoid PA rupture, “overwedging” and migration of the catheter. Fluoroscopy is also necessary in patients with intracardiac devices. Obtaining peripheral IV access in patients prior to starting the procedure is recommended to promptly deliver treatment in the event of vagal episodes, which can lead to significant clinical deterioration in PAH patients. The purpose of evaluating PAH patients with a short-acting vasodilator is to determine the degree in which the pulmonary vasoconstriction is contributing to the elevated PAPs. Vasodilator responsiveness identifies patients with a better prognosis and those who are more likely to have a sustained beneficial response to calcium channel blockers (CCB). Before PAH-directed therapies were available, initial reports of improved outcome upon treatment with high doses of oral nifedipine or diltiazem led to a flurry of treatment with CCBs. However, only a small number of patients (approximately 6%) were found to be “responders” in large series retrospective analysis. For the majority of patients who are not responders, treatment with high dose CCBs can result in clinical deterioration (hypotension, arrhythmia, syncope, or death), which can be worsened by the long half-life of the agents. Reports of adverse outcomes associated with vasodilator testing with CCBs led to the use of short-acting agents to assess responsiveness to identify those patient who may be considered treating with CCBs. Intravenous epoprostenol and IV adenosine have both been studied as acute vasodilators. Both are short-acting, potent vasodilators and investigators have reported different degrees of responsiveness depending on the criteria used. However, because both agents have the potential to cause systemic hypotension and side effects, using inhaled nitric oxide (NO) has emerged as the vasodilator of choice due to its pulmonary selectivity, short half-life, and lack of systemic side effects. However, it is expensive and requires trained respiratory personnel to administer (Table 4). The definition of what constitutes acute vasodilator “responder” has undergone changes over the years. Initial studies used a composite decrease in mPAP and PVR of 20%-30% to define responsiveness. A recent analysis by the European group has demonstrated that the number of patients who remained stable long term (>1 year) on CCB monotherapy (initially placed using the previous definition of 20% reduction in PAP and PVR) was smaller than previously reported (6.8%). A comparison of the initial vasoreactivity hemodynamics between patients who remained stable on CCB alone versus those who decompensated on CCBs led the European Society of Cardiology to define a positive acute vasodilator responder in an IPAH patient as: A fall in mPAP of at least 10 mm Hg to less than or equal to 40 mm Hg and Increased or unchanged CO For the small number of patients who demonstrate significant hemodynamic response and meet the criteria, initiation of treatment with CCBs can be considered. Such treatment requires very close vigilant follow-up to monitor for both efficacy and safety. For patients who fail to improve by clinical and objective criteria (reach FC II symptoms, exercise, and hemodynamic parameters) within the initial 3 months, treatment with PAH-directed therapies need to be implemented. The majority of patients who fail to achieve this acute response are not likely to respond to CCBs. Contraindications for CCB treatment (and acute vasodilator testing) include patients with advanced disease defined as: Functional class IV symptoms Overt clinical right heart failure Hemodynamic markers of advanced process (high RAP and/or reduced CO, systemic hypotension) Patients with such an advanced state should not undergo acute vasodilator testing since these patients need prompt treatment with PAH-approved therapies. It needs to be emphasized that all the CCB testing and treatments were performed in IPAH patients. For patients with PAH associated with other underlying processes (i.e., scleroderma), the number of patients who had acute vasodilatory response is even less, and the net benefit of vasodilator testing is at best weak if used for the sole purpose of determining candidates for CCB therapies. The development of acute pulmonary edema during vasodilator testing should raise the suspicion of veno-occlusive disease or pulmonary capillary hemangiomatosis, in which therapy with pulmonary vasodilator is contraindicated. Assessment of Prognosis Since PAH is a disease manifested by an increase in afterload of pulmonary arteries leading to progressive right ventricular dysfunction and failure, hemodynamic markers are considered to be the gold standard for indicating prognosis. This was first demonstrated in the NIH PPH Registry in the 1980s where the investigators concluded that “mortality was most closely associated with right ventricular hemodynamic function and can be characterized by means of an equation using three variables: mean pulmonary artery pressure, mean right atrial pressure, and cardiac index.” Specifically, RAP greater than or equal to 20 mm Hg, mPAP greater than or equal to 85 mm Hg, and CI less than 2 L/min/m2 were shown to be associated with an increased risk of death. The data obtained were the basis of formulating the regression equation to calculate survival based on hemodynamics, which was validated in a prospective study. Subsequent studies have corroborated the importance of elevated RAP (>12 or 15 mm Hg, pending sources) and low cardiac index (<2.2 or 2.5 L/min/m2, pending sources) as determinants of poor outcome. The relevance of mPAP on prognosis has been variable. In the retrospective study among patients treated with epoprostenol, patients with lower mPAP correlated with poor outcome, which may indicate that mPAP per se is not a reliable surrogate for RV function but needs to be assessed as part of pulmonary vascular resistance. A. History Part 1: Pattern Recognition Diagnosing PAH can be challenging primarily due to the nonspecific nature of the presenting symptoms. Here is a list of common baseline demographics and presenting symptoms of PAH patients: From the REVEAL Registry, which reports current PAH patient population from U.S. centers, the mean age is reported to be 53 ± 14 years and majority (80%) to be females. From the NIH PPH Registry, the most common presenting symptoms were the following: Dyspnea (98%), fatigue (73%), chest pain (47%), near syncope (41%), syncope (36%), leg edema (37%), and palpitations (33%). The quality of dyspnea has been described as progressive and “relentless,” different from seasonal or allergic-related phenomena. Dyspnea is usually exacerbated by activity, particularly going up an incline or climbing steps are usually reported to be difficult. Other activities patients describe being problematic include bending down (to pick up objects off the floor), squatting (such as to tie shoes), vacuuming, carrying any weight up the stairs, and lifting any objects overhead. It is not uncommon for these activities to produce dizziness and/or chest pain; in patients with severe PAH, they can provoke syncope. The difficulties faced in performing these activities reflect hemodynamic changes that increase pulmonary vascular flow and/or resistance. Young patient presenting with syncope: need to at least consider PAH. Although PAH is a rare disorder, it should be considered in the differential for a young patient presenting with syncope associated with exertion (i.e., related to sports activity). PAH in a young patient can be difficult to recognize because young individuals can compensate well during initial stages of PAH and the condition can progress rapidly (especially if related to a genetic mutation). B. History Part 2: Prevalence: These are updated risk factors for PAH as outlined from the Dana Point World Symposium in 2008. Risk factors for PAH include “any factor or condition that is suspected to play a predisposing or facilitating role in the development of the disease.” Definite association – defined as an epidemic or from a large, multicenter epidemiologic study aminorex, fenfluramine, dexfenfluramine, toxic rapeseed oil Possible association – defined as drugs with similar mechanisms of action as those in the “definite” or “likely” categories but which have not been studied cocaine, phenylpropanolamine, St. John’s Wort, chemotherapeutic agents, SSRI Likely association – defined as a single-center, case-control study demonstrating an association or a multiple-case series amphetamines, L-tryptophan, methamphetamines Recent observational studies have reported methamphetamine abuse significantly increases the risk of developing PAH Unlikely association – defined as one in which a drug has been studied in epidemiologic studies and an association with PAH has not been demonstrated Oral contraceptives, estrogen, cigarette smoking A. Idiopathic PAH (IPAH) – corresponds to sporadic disease in which there is neither a family history of PAH nor an identified risk factor. Recent epidemiologic studies show that the prevalence for PAH is 15 cases per million; for IPAH, 5.9 cases per million. A trend for older age range of patients being diagnosed has shown to be increasing (patients >70 years of age). REVEAL registry has shown that the majority are females (80%). B. Heritable PAH – when PAH occurs with a family history. Mutations in the BMPR2 gene can be detected in approximately 70% of the cases that are transmitted in autosomal dominant pattern. U.S. Registries among PH centers report prevalence of PAH associated with positive family history ranging from 10% to 13%. Characteristics of genetic transmission include: Variable penetrance – there can be “skipped” generations as far as phenotypic manifestations. Genetic anticipation – the disease can manifest at an earlier age with more aggressive features in subsequent generations. Recent studies have suggested that patients with PAH associated with BMPR2 mutations most likely represent a subgroup of patients with more severe disease and are less likely to demonstrate vasoreactivity than those with IPAH without BMPR2 mutations. This stresses the need to understand the indications for performing acute vasoreactivity testing during right heart catheterization (see Right Heart Catheterization, Acute Vasodilator Testing). It should be noted that all patients with BMPR2 mutations have heritable disease, regardless of family history. BMPR2 mutations have been identified in 11% to 40% of IPAH patients with no family history. C. Drug and toxin-induced PAH – the most recent update of identified drugs and toxins associated with PAH are listed above in Risk Factors in PAH. A few recent findings include: Serotonin produces vasoconstriction, promotes PA smooth muscle cell hypertrophy and hyperplasia, and has been implicated in PAH. However, serotonin level alone per se is probably not likely a determinant of PAH since serotonin-reuptake inhibitors have not been associated with an increased incidence of PAH. However, a recent case control study that brought this line of thought into question regarding the use of selective serotonin reuptake inhibitors in pregnant women after 20 weeks of gestation was found to have a possible association with an increased risk in offspring developing persistent PH of the newborn. The role of serotonin with PAH in the setting of pregnancy needs further evaluation. A significant association between methamphetamine use, whether inhaled, smoked, taken orally or intravenously, and the development of IPAH has been reported. D. PAH associated with connective tissue disease (CTD) – among all CTDs, scleroderma patients represent the largest subset of patients at risk for developing PAH. The prevalence of PAH has been well studied only for the systemic sclerosis population. Recent studies using echocardiography as screening and RHC for confirmation found a prevalence of PAH between 7% and 12%. The most recent analysis from the REVEAL Registry demonstrates that patients with CTD-associated PAH had a worse overall outcome compared with IPAH patients (1-year survival rate and freedom from hospitalization, 85% for IPAH and 67% for CTD-associated PAH ). This is particularly true for patients with PAH associated with scleroderma. Thus CTD patients represent the largest group at risk and for those who develop PAH and the worst prognosis (even with PAH-specific therapies). It is recommended for scleroderma patients to have a baseline echocardiogram and PFTs [International and European guidelines recommend annual Doppler echocardiography]. Obtain a follow-up test with development of any symptoms. It is most challenging to detect if any pulmonary vascular disease is progressing since dyspnea and/or fatigue can occur due to the underlying disease or treatments. It is recommended that if patients have these symptoms, which cannot be explained by other reasons, PAH should be considered and an echocardiogram evaluated. With findings consistent with PAH, RHC needs to be performed. A recently published report suggests that improved outcome can be seen with patients diagnosed due to active screening, though the effects of lead time bias need to be considered. Patients with systemic sclerosis can have other processes that can cause elevated PAPs other than PAH, such as lung fibrosis, diastolic dysfunction, and primary cardiac involvement due to scleroderma. This stresses the critical importance of RHC to confirm the elevated PAPs and to classify its etiology, whether PAH or pulmonary venous hypertension, to determine appropriate therapy. E. PAH associated with HIV infection – shown to have clinical, hemodynamic, and histologic characteristics similar to those seen in IPAH Recent prevalence reported to be 0.46%. Mechanism remains unclear. Indirect action of the virus through secondary messengers (cytokines, growth factors, endothelin, and viral proteins) suspected. Small studies have shown a benefit of PAH-directed therapy in patients with HIV (bosentan, epoprostenol). Current recommendations include monitoring and optimal treatment with both antiretroviral and PAH-specific therapies. F. PAH associated with liver disease – portopulmonary hypertension is development of PAH in association with elevated pressure in the portal circulation. It is the presence of portal hypertension, rather than underlying liver disease, that is the main risk factor. All candidates for liver transplantation need to undergo echocardiography to screen for portopulmonary hypertension. If the echocardiogram shows elevated pulmonary pressure, RHC must be performed to confirm the diagnosis and assess risk/candidacy for liver transplant. Overall about 8% of candidates for liver transplantation have portopulmonary hypertension and are at risk of its complications. A recent study identified female sex and autoimmune hepatitis as risk factors for development of portopulmonary hypertension (hepatitis C associated with decreased risk in this study). Pathologic changes in the small arteries in patients with portopulmonary hypertension appear identical to those seen in IPAH. RHC is absolutely critical for diagnosis of portopulmonary hypertension because there are several other factors that can elevate PAPs with advanced liver disease, such as high CO associated with the hyperdynamic circulatory state and elevated PCWP due to fluid overload and/or diastolic dysfunction. G. PAH associated with congenital heart disease (CHD) – with the number of patients with adult CHD increasing, long-term complications, which include PH and right heart failure, pose serious risks for this group of patients. Patients with hemodynamically significant systemic-to-pulmonary shunts will develop PAH if left untreated. This can result in Eisenmenger syndrome, which is defined as CHD with an initial large systemic-to-pulmonary shunt that initiates and propagates pulmonary vascular changes due to the persistent presence of increased blood flow and pressure, which over time increases PVR that results in a reversal of the shunt and cyanosis. Eisenmenger syndrome represents the most advanced form of PAH associated with CHD. Prevalence of PAH associated with congenital systemic-to-pulmonary shunts in Europe and North America has been estimated to range between 1.6 and 12.5 cases per million adults; 25% to 50% of this population have been shown to be affected by Eisenmenger syndrome. H. PAH associated with schistosomiasis – most likely due to multifactorial mechanisms, including portopulmonary hypertension (which is a common complication of schistosomiasis) and vascular inflammation (as a result of impacted Schistosoma eggs). This entity has been shown to have a similar clinical presentation to IPAH and histologic findings, including plexiform lesions. It is estimated that more than 200 million people are infected with Schistosoma and that 4% to 8% will develop hepatosplenic disease. Recent hemodynamic data demonstrate prevalence of PAH to be 4.6% of those individuals with hepatosplenic disease. Pulmonary venous hypertension is also common (3%), which addresses the need for invasive hemodynamic studies. I. PAH associated with chronic hemolytic anemia – PH has been most well described in patients with sickle cell disease (SCD). Prevalence of PAH in SCD is not clearly established. The largest study reported 32% of patients had PH; however, PH was defined echocardiographically. There is very limited data with hemodynamic confirmation and a large proportion of patients with SCD have pulmonary venous hypertension. Furthermore, a hyperdynamic state leading to high CO can also result in moderate elevation of PAPs with normal PVR. The mechanism of PAH in SCD remains unclear. It has been proposed that chronic hemolysis results in high rates of nitric oxide consumption and produces a state of resistance of nitric oxide bioactivity. A. Patients with hereditary PAH – genetic mutations predisposing to PAH (i.e., BMPR2) identified Echocardiogram yearly; RHC if echo demonstrates evidence of PAH (high RVSP or right heart chamber enlargement) B. First degree relative of a patient with BMPR2 mutation (or within pedigree of two or more patients with a diagnosis of PAH) Genetic counseling regarding BMPR2 genotyping Echocardiogram yearly; RHC if echo demonstrates evidence of PAH (high RVSP or right heart chamber enlargement) C. Patients with systemic sclerosis Response to approved PAH treatment not as favorable compared to IPAH Early detection, accurate diagnosis, and appropriate therapy imperative Baseline echocardiogram and yearly thereafter; RHC if echo demonstrates evidence of PAH (high RVSP or right heart chamber enlargement) D. HIV infection Echocardiogram if symptoms or signs are suggestive of PAH; RHC if echo demonstrates evidence of PAH (high RVSP or right heart chamber enlargement) E. Patients with portal hypertension prior to liver transplantation All candidates for liver transplantation need to undergo echocardiogram RHC is necessary for full hemodynamic measurements; increased PAP associated with significant risk of perioperative mortality and contraindication to transplantation F. Prior appetite suppressant use Echocardiogram only if symptomatic G. Patients with congenital systemic to pulmonary shunts Echocardiogram and RHC at the time of diagnosis Consider repair of defect if significant L-R shunt present (need to consider RV function, degree of hypoxia, and PVR) High probability of PAH developing if unrepaired shunts (Eisenmenger syndrome) H. Recent acute pulmonary embolism VQ scan 3 months after event if symptomatic, and echocardiogram pending clinical course Pulmonary angiogram if results positive G. Sickle cell disease Echocardiogram yearly; RHC if echo demonstrates evidence of PAH C. History Part 3: Competing diagnoses that can mimic Pulmonary Arterial Hypertension Diastolic dysfunction leading to diastolic heart failure (DHF; aka HFpEF: heart failure with preserved ejection fraction) refers to a clinical syndrome in which patients present with heart failure symptoms with preserved left ventricular (LV) systolic function. Epidemiologic studies have shown high prevalence of DHF (approximately 40%-70%) among symptomatic patients and the risk factors have been well elucidated (age >65 years, hypertension, elevated pulse pressure, obesity, coronary artery disease, diabetes mellitus, and atrial fibrillation). The predominant underlying structural abnormalities in diastolic heart failure are concentric remodeling and hypertrophy of the LV caused by chronic pressure overload, usually due to systemic hypertension. These alterations produce abnormalities in both relaxation and filling, which can be a precursor to LV systolic dysfunction or the main structural abnormality, producing symptoms and signs of heart failure. Patients presenting with diastolic dysfunction and PH is a common clinical dilemma and can be very challenging to distinguish from PAH. Up to 70% of patients with LV diastolic dysfunction may develop PH, the presence of which is associated with a poor prognosis. The presentations are similar to PAH and include dyspnea, fatigue, and/or signs and symptoms of heart failure. An echocardiogram can be misleading, for at “first glance” it typically shows normal LV systolic function with some degree of elevated pulmonary pressure, which can be misinterpreted as a presentation representing pulmonary vascular disease. It is crucial to carefully scrutinize the echocardiogram to specifically focus on findings suggestive of LV diastolic dysfunction in all patients (left atrial enlargement, LV hypertrophy, and elevated LV filling pressure [grade II to IV diastolic dysfunction]), but especially those with risk factors for diastolic dysfunction. Conversely, it is imperative to have a reliable assessment of RV size and systolic function as well, because in a patient with hemodynamically significant PAH, the RV will be enlarged and/or dysfunctional, whereas in cases of DHF, RV usually demonstrates normal size and function (see Table 2). At this juncture, it is critical to perform RHC to measure the left-sided filling pressure and calculate the TPG and PVR. It needs to be emphasized that attention must be paid to the quality of the PCWP tracing in order for the correct diagnosis to be made. Misinterpretation of either “underwedged” or hybrid tracing as true PCWP (thereby misdiagnosing as diastolic dysfunction due to falsely elevated PCWP) or recorded measurements from improper placement of the catheter can lead to a wrong diagnosis (see Diagnostic Confirmation section) The hemodynamic assessment can possibly fall into one of the following three categories: 1. The PCWP is normal (<15 mm Hg) and the TPG and PVR are elevated (≥ 3 Wood units); the patient has PAH and treatment needs to be considered after full evaluation. The diagnosis cannot be fully dependent on the single measurement of PCWP alone, because if the patient has just undergone diuresis, the PCWP can be “normal.” If the patient has clinical risk factors and/or echo findings suggestive of diastolic dysfunction, additional hemodynamic maneuvers including fluid challenge or an exercise RHC may be needed to assess response as a measure of the LV compliance. Although there are no definite standardizations, the recently published ACCF/AHA Expert Consensus Document on Pulmonary Hypertension and reports from the 4th World Symposium on Pulmonary Hypertension outline consensus-based recommendations for evaluation of patients presenting with Group II PH. 2. The PCWP is elevated (>15 mm Hg), the PVR is less than 3 Wood Units, and the TPG is normal; the patient has diastolic dysfunction, and therapy should be aimed at optimizing volume status, heart rate, and systemic blood pressure. 3. The PCWP and the PVR are both elevated (the TPG can be normal or elevated); careful evaluation and intervention need to be made to determine if the elevated PVR is passive (due to elevated filling pressure and thus responsive to diuretics and/or a systemic vasodilator) or fixed (remains elevated despite normalizing the PCWP and systemic blood pressure). If the PCWP and PVR both decrease (TPG normal) with optimal heart failure therapy, then the patient needs to be treated aggressively with these regimens. If the PCWP is normalized but the PVR remains elevated (elevated TPG with variable CO), this may be indicative of pulmonary arteriopathy being the dominant disorder with structural changes in pulmonary vasculature along with diastolic dysfunction. No PAH-specific therapies have been shown to be beneficial in heart failure to date; recent small studies with phosphodiesterase-5 inhibitor (PDE-5 inhibitor) sildenafil have shown promise in a few small studies. Treatment with epoprostenol and endothelin receptor antagonists (ERAs) have failed to show beneficial effects, though these trials did not specifically target patients with heart failure and PH. Studies using sildenafil have shown improvement in LV systolic and diastolic function, as well as systemic vasoreactivity in animal models of heart failure. Recent short-term studies evaluating patients with chronic systolic heart failure and PH using sildenafil have demonstrated improvement in exercise capacity and quality of life. However, data from a well-designed trial studying long-term benefits is necessary before any recommendations can be made in regards to use of sildenafil in patients with heart failure and PH. D. Physical Examination Findings. Accentuated P2 – high pulmonary pressure increases the force of pulmonic valve closure; heard best at apex Left parasternal heave and right-sided S4 – presence of high right ventricular pressure and hypertrophy Holosystolic murmur increasing with inspiration and increased jugular v waves – tricuspid regurgitation Diastolic murmur – pulmonary regurgitation RV lift – due to enlargement and/or hypertrophy of RV Right ventricular S3 – right ventricular dysfunction Marked distended jugular veins, hepatojugular reflux, hepatomegaly, peripheral edema, and ascites – right ventricular dysfunction and tricuspid regurgitation Low blood pressure, diminished pulse pressure, and cool extremities – low CO due to right ventricular failure Central cyanosis – intrapulmonary shunt, hypoxemia, pulmonary to systemic shunt Clubbing – congenital heart disease Rales and decreased breath sounds – pulmonary congestion (left heart failure), effusion Fine rales or crackles, accessory muscle use, wheezing, and productive cough – pulmonary parenchymal disorder Sclerodactyly, arthritis, telangiectasia, Raynaud phenomenon, and rash – connective tissue disease Peripheral venous insufficiency or obstruction – venous thrombosis Splenomegaly, spider angiomata, palmar erythema, icterus, caput medusae, and ascites – portal hypertension E. What diagnostic tests should be performed? Diagnosing PAH and determining etiology of disease requires the following steps: 1. The recognition of predisposing conditions and genetic susceptibilities placing certain patients at greater risk (see History Part B: Prevalence – Risk Factors for PAH). 2. Knowing the nonspecific nature of clinical presentation and having a high index of suspicion to screen for PAH. 3. Understanding the usefulness and pitfalls of echocardiography as a screening modality. 4. Undergoing the complete evaluation to classify the type of PH. 5. Performing the invasive hemodynamic assessment and acute vasodilator challenge, when indicated, to confirm a PAH diagnosis. 6. Obtaining risk stratification to determine therapy, including assessment for exercise capacity. List of tests to perform to diagnose PAH (see Figure 2. ). 1. Screening evaluations – history and physical, CXR, ECG, and echocardiogram 2. Determine associated conditions for PAH – serologies (HIV, ANA [and other CTD labs, such as RF, Scl-70], and LFTs [hepatitis serologies]) 3. To evaluate for significant LHD pathology – echocardiogram (TEE and coronary angiography as indicated) 4. To evaluate for significant pulmonary pathology – PFTs and sleep study (CT of chest as indicated) 5. To evaluate for thromboembolic disease – VQ scan (CT angiography and/or pulmonary angiogram as indicated) 6. To assess exercise capacity/functional status – 6 MWT (CPET in some centers) 7. For diagnosis – right heart catheterization 1. What laboratory studies (if any) should be ordered to help establish the diagnosis? Diagnostic Tests A: Laboratory Evaluations in the Diagnosis for PAH There is no one laboratory marker of value that indicates presence of, or diagnostic for, PAH. The laboratory tests listed below are necessary to assess for presence of associated systemic diseases and to determine degree of right heart failure. Laboratory assessments to determine the etiology of PH Connective tissue disease evaluation ANA, rheumatoid factor Rheumatologist referral for further characterization of any positive CTD screening or high clinical suspicion of CTD Hepatitis B and C Other laboratory evaluations Routine comprehensive laboratory evaluations (CBC, BMP, LFTs) BNP (baseline and periodic evaluations with treatment recommended) TSH (as clinically indicated) The measurement of distance walked in 6 minutes has become the test most frequently used to assess baseline exercise capacity and response to therapy in PAH. Indeed, the difference in the 6-minute walk distance (6MWD) has been used as the primary objective for almost all clinical trials for PAH treatments. The advantage of 6MWD is that it is noninvasive, has been reasonably validated and standardized among patients with PAH, and is simple to perform, inexpensive, and well tolerated in most patients with PAH. The disadvantage of this modality includes a learning effect after repeated testing, comorbid conditions affecting test performance, and other activities of the day influencing results. Increases in 6MWD have been shown to correlate with hemodynamics, quality of life, and survival. While there is no “set” distance that determines outcome, most experts agree that 6MWD is a useful tool to follow patients on therapy. From risk standpoint, 380 m is considered one marker that has been shown to correlate with improved clinical outcome. In interpreting 6MWD, there is varied opinion as to which parameter is most indicative of RV function (i.e., absolute distance, % predicted based on age and gender, heart rate recovery, Borg score). Most consider that achieving 15%-20% improvement in 6MWD in the first 3-4 months of initiating a PAH-specific therapy should be a goal. 2. What imaging studies (if any) should be ordered to help establish the diagnosis? Diagnostic Tests B: Radiographic Studies for Evaluation of PAH Usually performed as initial evaluation for complaints of dyspnea. Findings on chest radiograph are neither sensitive nor specific for PAH (i.e., normal chest radiograph is a common finding) Findings indicative of PH (usually seen in advanced stages) include: Prominent hilar pulmonary arteries Peripheral hypovascularity (pruning) RV enlargement (seen in lateral view) Electrocardiograms are usually neither sensitive nor specific for PH in the initial nonadvanced stage Changes consistent with PAH (usually indicative of advanced PAH with right-sided chamber dilatation) include: Right axis deviation Right atrial enlargement Thorough pulmonary evaluation is needed to determine if PH is associated with a significant degree of parenchymal and/or airway related disorders. Full pulmonary function test (PFT) with diffusion capacity (DLCo) is required. Chest CT – to determine parenchymal lung disease Sleep study – as clinically indicated Full evaluation for chronic thromboembolic disease is essential. It has been reported that about 3%-4% of patients who survive acute pulmonary embolus (PE) do not fully resolve the thrombus burden despite anticoagulation and can develop chronic thromboembolic pulmonary hypertension (CTEPH). Furthermore, it can be present in the absence of a clear history of PE in up to 50% of the patients. CTEPH is associated with poor prognosis (30% probability of survival at 5 years with mPAP >40 mmHg). Screening using a ventilation perfusion (VQ) scan is the test of choice: A normal or very low probability scan essentially excludes CTEPH, whereas a high probability scan warrants further evaluation with a pulmonary angiogram. It is necessary to rule out CTEPH even in those patients with an identifiable underlying risk factor or cause of PAH, such as scleroderma, for therapeutic implications of the diagnosis is significant. Clinical judgment is required for those with nondiagnostic or intermediate scans; need to account for suspicion of underlying parenchymal lung disease. CT angiogram, while excellent for excluding acute PE, is less sensitive than the perfusion scan to exclude CTEPH. In patients with parenchymal lung disease where a perfusion scan may be difficult to interpret, a CT angiogram may be useful. Pulmonary angiography is the “gold standard” to fully assess location and extent of thrombus burden and to determine if a patient is a candidate for thromboendarterectomy Surgical intervention (thromboendarterectomy) can reverse pulmonary hypertension and potentially cure the patient. cMRI is being explored as a potential tool in PAH since RV function is the ultimate determinant regarding outcome and prognosis. Possible utilities of cMRI in PAH include: Anatomic evaluation (RV volume and mass) Noninvasive hemodynamics (curvature ratio, delayed contrast enhancement, and PA flow velocity and area) Marker of RV response and remodeling (prognostic information, response to treatment). Studies have shown that baseline cMRI RV size and function serve to predict long-term survival while conventional prognostic markers (FC and 6MWT) do not. Clinically, there are barriers in using cMRI in the PAH population which include: Test and interpretation not widely available Difficulty for unstable patients to tolerate the test (length of the study, need to lie supine, claustrophobia, need to time with respiratory cycle and breathholding, and/or the need for contrast agent) For patients on intravenous epoprostenol, need to arrange for long tubing (the cassette must be kept out of the cMRI area) Cost of the study and insurance coverage Significant advances in developing PAH targeted therapies have been accomplished in the past two decades. There are currently nine FDA approved treatments for PAH (Table 5). These treatments aim to restore the imbalances in the endothelial system that are present in PAH patients: augmenting prostacyclin and nitric oxide pathways and blocking the endothelin pathways (see Section I: Pulmonary Arterial Hypertension – Pathobiology). Clinical Research Highlights Intravenous epoprostenol, the first PAH-specific therapy to be approved by the FDA in 1995, was shown to improve functional class (FC), exercise capacity, hemodynamics, and survival in IPAH. The landmark pivotal trial enrolled 81 functional class III and IV IPAH patients in a 1:1 randomization comparing IV epoprostenol to a conventional treatment (diuretics, digoxin, warfarin, calcium channel blockers). There were eight deaths during the 12-week trial period, all which occurred among patients who were randomized to conventional therapy that resulted in a survival benefit (P = .003). This was soon followed by a clinical trial evaluating epoprostenol in PAH associated with connective tissue disease that demonstrated marked improvements in 6MWD and hemodynamics but no effect on mortality in a 12-week, open-label randomized trial. Two longer-term observational studies have confirmed the chronic benefits of IV epoprostenol in PAH patients, specifically improvements in survival compared with historical controls, FC, 6MWD, and hemodynamics. Intravenous epoprostenol is a challenging treatment to implement due to its short half-life (<6 min) and the need for continuous IV infusion via a tunneled catheter. Each patient must learn the techniques of sterile preparation of the medication, operation of the ambulatory infusion pump, and care of the central venous catheter. Incidences of sepsis and catheter-related infections are not negligible (0.1 to 0.6 case per patient-year) and can cause significant morbidity. Mastering the sterile techniques required to take care of the indwelling catheter is essential. Any interruption of the drug infusion can be potentially life threatening due to the short half-life of epoprostenol and potential for rebound pulmonary hypertension. Intravenous epoprostenol is commonly started in the hospital at a dose of 1-2 ng/kg/min and titrated on the basis of PAH symptoms and side effects. Most experts consider an optimal dose of chronic therapy to be between 25 to 40 ng/kg/min. Chronic overdose can lead to high CO failure and worsening of heart failure symptoms. Periodic follow-up RHC is recommended to obtain full hemodynamics, including CO measurements, for patients on chronic intravenous epoprostenol treatment. Common side effects include headache, jaw pain, diarrhea, nausea, flushing, rash, musculoskeletal pain, and thrombocytopenia. Jaw pain (usually with first bite) rarely completely resolves. Over-the-counter prophylaxes are used for diarrhea, which is usually a chronic problem. Nausea and flushing usually subsides and resolves after a period of time (usually days to weeks). A “Flolan” rash can appear in some people (on face, trunk, or limbs). Thrombocytopenia can occur with long-term use so there is a need to follow platelet counts. This can be dose dependent. Intravenous epoprostenol is also unstable at room temperature so the cassette needs to be kept cold (usually with ice packs). Recently, temperature-stable epoprostenol (Veletri®) has been approved by the FDA. Its properties are reported to be generally similar except for the difference in the temperature-related stability. A registry of patients being treated is being collected. Due to the complexity of administering this therapy, epoprostenol use should be limited to experienced centers. Authors’ Preferred Method of Treatment/Recommendation Intravenous epoprostenol is recommended as the therapy of choice for the following: Young patients presenting with IPAH/advanced and/or worrisome clinical features/possible family history of PAH Syncope, symptoms or signs indicating advanced disease/RV dysfunction Echocardiogram showing enlarged right-sided chambers, RV dysfunction, pericardial effusion Hemodynamics showing elevated RAP (>12 mm Hg) and/or decreased CI (<2.5 L/min) Patients progressing/not improving on other therapies (usually in combination treatments) As a “bridge” to lung transplant Generally recommended for patients who are rapidly progressing, advanced stage upon initial diagnosis, high risk markers Initiate on intravenous epoprostenol as lung transplant evaluation takes place If patient responds well and improves, listing for transplant can be delayed and patient followed on therapy For patients with portopulmonary hypertension Initiate therapy to assess response on intravenous epoprostenol as “bridge to candidacy” for liver transplant Epoprostenol is considered the “gold standard” in PAH-specific therapy and recommended for advanced PAH (especially patients presenting in FC IV) and PAH with right heart failure, cardiogenic shock from RV failure, and/or requiring rescue therapy. Clinical Research Highlights Treprostinil is a prostacyclin analogue with a half-life of 4 hours, which was studied as a continuous subcutaneous infusion in a 12-week, placebo-controlled, randomized trial of four hundred seventy patients with FC II, III, or IV PAH. There was a modest but statistically significant median increase of 16 meters in 6MWD; the improvement was dose related and patients in the highest dose quartile reported close to a 40-m improvement. However, the major obstacle of using subcutaneous treprostinil is the pain and erythema at the infusion site, which was reported by 85% of the patients, and curtailed titration of the infusion. It is now recognized that site pain is not dose related, and that for most patients the initial site pain diminishes after proper dose escalation, which helps them improve their PAH symptoms. Due to the limitations imposed by the subcutaneous route of delivery, IV treprostinil was studied in a 12-week open-label trial of 16 patients. It demonstrated improvements in 6MWD (82 m) and hemodynamics. In another open-label trial, 31 FC II and III PAH patients on IV epoprostenol were transitioned to IV treprostinil. Twenty-seven patients completed the transition, but four needed to be transitioned back to epoprostenol. 6MWD measurements were maintained among patients who completed the transition; however, there was a modest increase in mPAP and decrease in cardiac index. Noteworthy is that the dose of IV treprostinil at the end of the study period was more than twice the dose of epoprostenol at the start of the study. Pain at the site of administration (site pain) is the most commonly encountered difficulty in using subcutaneous (SC) treprostinil. Usually the pain is most troublesome at the beginning of the therapy. It is not dose related; rather, the discomfort tends to get less as the dose is increased and PAH symptoms improve. Most patients, with adequate support can tolerate this treatment and gain benefit. Patients need to be told what can be expected at the onset and be educated as to what can be done. Several remedies are available ranging from topical ointments, creams, antiinflammatory agents, or different techniques that can help with the site pain. The advantage is that patients can receive the benefit of continuous prostacyclin treatment without the risk of tunneled catheter. For those patients who cannot tolerate the site pain, treprostinil can be given intravenously. The need for a tunneled intravenous catheter and managing the pump for patients receiving intravenous treprostinil are the same as intravenous epoprostenol. The side effect profile is also similar. The major difference lies in that treprostinil’s longer half-life (4-5 minutes for epoprostenol versus 4-5 hours for treprostinil), potentially renders it “safer” in the event of line malfunction and/or therapy interruption. Transition from intravenous epoprostenol to intravenous treprostinil has been shown to be overall safe and well tolerated, though the dose of treprostinil is typically higher, ranging from 1.5 to 2x the original epoprostenol dose. Another major difference is regarding management of line-related infections. A Centers for Disease Control and Prevention report has raised concerns regarding possible increased incidence of blood stream infections, particularly with gram-negative organisms, in patients receiving intravenous treprostinil. It is unclear if the rates of infections are truly different, but the increased incidence of gram-negative organisms needs to be noted. If there is concern for line infection or bacteremia, initiate appropriate antibiotics to cover both gram-positive and gram-negative organisms after appropriate cultures have been drawn. Authors’ Preferred Method of Treatment/Recommendation For patients who understand the benefits of the SC method, starting with this approach is recommended. Intravenous treprostinil may be used instead of epoprostenol for added safety related to longer half-life and convenience (cassette change every other day instead of every day with epoprostenol; also room temperature stable). For patients with family support concerns, or for those who live at a farther distance from the PH center where accessing emergent treatment may be a concern, treprostinil provides a safer approach of providing intravenous prostacyclin infusion. Given the complexity of administering and following patients receiving these treatments, administration should be limited to centers with experience. Clinical Research Highlights and Clinical Considerations Iloprost is a stable prostacyclin analogue that is delivered via an aerosolized device for 6 to 9 treatments per day. It was studied in a 12-week, multicenter, placebo-controlled, randomized trial of 207 FC III and IV patients. The unique aspect of this study was that patients enrolled were comprised not only of PAH patients but also of patients with PH related to inoperable chronic thromboembolic disease. This study also used a novel composite end point (improvement in FC by at least 1 level and increase in 6MWD by at least 10% in the absence of clinical deterioration), which demonstrated treatment benefit (16.8% vs. 4.9%, treated vs. placebo, P = .007). It is available in a 2.5 mcg test dose followed by 5.0 mcg and 20 mcg doses. The average inhalation times on the 20 mcg dose ranges from 5-8 minutes per session. It is generally well tolerated with coughing, headache, and flushing occurring as the most common side effects. Assessing for compliance is important when prescribing inhaled iloprost. When combined with oral therapies, some prescribers have recommended four to five treatments a day. Clinical Research Highlights and Clinical Considerations Inhaled treprostinil was studied in a 12-week, placebo, randomized study in patients with PAH added to background therapies of bosentan or sildenafil as assessed by improvements in the 6WMT (median placebo-corrected treatment effect of 20 m). Treprostinil is taken four times a day via inhalation using a specialized nebulizer. The initial therapy is started with three breaths four times a day, with increased breaths as tolerated. The maximal dose is nine breaths four times a day. It is generally well tolerated with cough and hypotension as the most common side effects. The usual practice is to use as combination treatment with oral therapies. Clinical Research Highlights and Clinical Considerations Bosentan is a nonselective endothelin receptor blocker (ETA and ETB). It is the first orally available therapy approved for PAH. The pivotal study, named BREATHE-1, was conducted in a 12-week placebo-controlled study among 213 patients with group I PAH. Treatment with bosentan improved the primary end point of 6MWD by 36 m, whereas placebo patients deteriorated by 8 m (P = .0002). Bosentan also improved the composite end point of time to clinical worsening (defined as death, initiation of IV epoprostenol, hospitalization for worsening PAH, lung transplantation, or atrial septostomy). Long-term observational findings in survival of patients treated with bosentan as first line therapy have shown improved survival compared with expected outcome based on the NIH registry equation. Bosentan was shown to be effective in mildly symptomatic patients in a landmark trial called the EARLY study. This was a placebo controlled trial of 6 months duration that enrolled 168 FC II PAH patients. The baseline 6MWD of this cohort was 438 m, which is higher than all the other studies in PAH (approximately 330-350 m). The results demonstrated a significant decrease in PVR, which was the primary end point to evaluate treatment effects on vascular remodeling, and a significant delay in clinical worsening. Bosentan is available in 62.5 mg and 125 mg. It is recommended to be started with 62.5 mg twice a day for 4 weeks; if LFTs are stable, increase to 125 mg twice a day. Bosentan is mainly metabolized through the hepatic P450 enzymes, and an increase in hepatic transaminases greater than 3 times the upper limit of normal has been reported in 10%-12% of the clinical trial population. It is dose related and transaminase abnormalities resolve upon dose reduction or discontinuation of the treatment. Bosentan is teratogenic and may decrease the efficacy of hormonal contraception, so women of childbearing age must be counseled to use dual contraception for birth control. Other side effects include headache, flushing, lower-extremity edema, and anemia. Treatment with bosentan requires monitoring of liver function tests on a monthly basis, and pregnancy tests on women of childbearing potential on a monthly basis, and hemoglobin/hematocrit on a quarterly basis. Patients should be counseled regarding potential for lower extremity edema, especially in the initial weeks of therapy, and the possible need for diuretic adjustments. Glyburide and cyclosporine A are contraindicated with bosentan due to significant drug-drug interactions. Authors’ Preferred Method of Treatment/Recommendation Bosentan is most effective among PAH patients who are diagnosed early (as shown by the EARLY trial). PAH patients that are FC II or early III (IIIA), and those with CTD-associated PAH (ERAs demonstrate antifibrotic properties). Patients who respond to bosentan tend to do well and the efficacy tends to remain long term. Patients for whom bosentan would not be recommended include: patients who are diagnosed late in the course of disease (i.e., FC IV or right heart failure) and patients with hepatic dysfunction. It must be stressed that ERAs are not effective as rescue therapy. Special attention must be paid to those patients who present with overt right heart failure, because ERAs can make the heart failure worse. One approach is to diurese and stabilize the patient with other PAH-directed therapy and use bosentan after right heart failure has been fully treated. Clinical Research Highlights and Clinical Considerations Ambrisentan is a selective ETA receptor antagonist studied in two placebo-controlled, randomized, 12-week studies of WHO Group I patients (ARIES-1 and ARIES-2), which were conducted in the U.S. and Europe/South America, respectively, in approximately 400 patients. The treatment resulted in a significant improvement in 6MWD and delay in time to clinical worsening in all treatment groups. The 2-year open label extension results from the ARIES clinical studies was recently published, showing that improvements in exercise capacity and functional class were sustained with a low risk of clinical worsening. Ambrisentan is available in 5 mg and 10 mg oral tablets taken once a day. The incidence of hepatic transaminase elevation greater than 3 times the upper limit of normal was low at 0.8% in clinical trials. After reviewing the post marketing safety data, the FDA has removed the monthly LFT monitoring requirement. It is agreed upon by most experts that LFT monitoring is recommended on a scheduled periodic basis with other laboratory evaluations. Peripheral edema, a known side effect of the ERA-class, was reported as mild to moderate in the clinical trials. Reports of significant edema during post marketing use followed. Analysis of reported events showed clinically significant fluid retention to be most notable among elderly patients and has prompted the FDA to issue a warning to be placed in the package insert. Though the precise mechanisms behind the fluid retention is not clear, retention occurs most commonly in patients with clinical diastolic features (i.e., elderly, hypertensive, diabetic, obese, and those with echocardiographic findings to support diastolic dysfunction). Ambrisentan is also teratogenic, so a monthly pregnancy test is required for women of childbearing age. Ambrisentan also can decrease hemoglobin so periodic monitoring of laboratory checks are required. Authors’ Preferred Method of Treatment/Recommendation Ambrisentan is also best used for patients who are diagnosed early in the course of the disease (FC II and early III PAH), including CTD-associated PAH. It is best to not initiate in patients who are in right heart failure, FC IV, or with evidence of advanced disease. A special note for elderly patients who are diagnosed with PAH: if the patient has clinical evidence of right heart failure, peripheral edema, or echocardiogram findings of diastolic dysfunction (especially enlarged left atrium), it is recommended not to use ambrisentan (or at least ensure euvolemic state), for heart failure can significantly worsen. Clinical Research Highlights and Clinical Considerations Sildenafil was studied in a 12-week randomized placebo-controlled study of 278 symptomatic PAH patients. The primary end point of 6MWD improved by 45, 46, and 50 m in the 20, 40, and 80 mg groups, respectively (P <.001). There was no change in the time to clinical worsening at week 12. The result of 222 patients who completed 1 year of treatment demonstrated that the 6MWD improvement was maintained; however, nearly all patients were titrated up to a dose of 80 mg three times a day. Side effects included headache, flushing, dyspepsia, and epistaxis. Visual and hearing impairments have been reported; patients at risk (i.e., elderly or diabetics) should be counseled and evaluations performed as clinically indicated. Sildenafil is contraindicated with nitrates for risk of inducing hypotension. Authors’ Preferred Method of Treatment/Recommendation Considerable variation in the use of sildenafil exists regarding dose and type of patients. Some studies have shown a higher dose to be more effective (used as titration) and clinicians have advocated using higher doses off-label. However, obtaining financial approval can, for higher doses, can be difficult. Some patients have further improvement with a higher dose but this is not seen in all patients. Use of sildenafil for “secondary” PH (i.e., those with predominant PH but with some features of left heart disease and lung disease) has gained considerable interest. Among all PAH therapies available, sildenafil seems to be most well suited for this group of patients. However, it must be emphasized that consideration for treating these patients needs to be done with complete evaluation, including a RHC that demonstrates PAH (or PH “out of proportion” of the underlying condition). The primary underlying pathology must be in the pulmonary vascular component for this therapy to have a chance to be effective. To date, there are no large randomized trials, though one trial studying patients with diastolic dysfunction and PH is underway. Sildenafil is most likely the agent of choice for patients who are mildly symptomatic. Note, this treatment is not effective as a “rescue” per se, but can be used along with diuretics for moderately symptomatic patients and features of right heart failure. Hypotension can be an issue, so it needs to be monitored carefully. The benefit of sildenafil can be seen fairly quickly upon initiation of treatment; however, it can also “wane” with chronic use (patient variability). It is imperative to follow patients closely for maintenance of efficacy. Clinical Research Highlights and Clinical Considerations Tadalafil, a PDE-5 inhibitor with a longer half-life than sildenafil, was recently studied in a 16-week, double-blind, placebo-controlled trial among 405 PAH patients using 2.5, 10, 20, and 40 mg tablets once a day. The highest dose of tadalafil demonstrated a 41 m increase in 6MWD compared with 9 m for a placebo (P <.001). There was also a delay in the time to clinical worsening (defined as death, hospitalization, initiation of new PAH therapy, and worsening WHO FC). Side effects include headache, diarrhea, nausea, back pain, dizziness, dyspepsia, and flushing. Authors Preferred Method of Treatment/Recommendation The above comments regarding sildenafil also applies to tadalafil. The notable difference would be since tadalafil has a longer half-life, it may not be the agent of choice for unstable patients, for effects of hypotension can last longer. CCBs are recommended for patients who demonstrate responsiveness during an acute vasodilator testing (see Diagnostic Confirmation section). Patients with IPAH who meet the criteria may be considered for treatment with CCBs. Long-acting nifedipine, diltiazem, or amlodipine is suggested. Verapamil should be avoided due to its potential negative inotropic effects. Patients need to be followed closely for efficacy and safety on CCBs. If a patient does not improve to FC I or II with CCBs, the patient should not be considered a chronic responder and PAH-specific treatment should be initiated. Anticoagulation has been studied in two small uncontrolled trials in IPAH patients. Based on these studies, most experts recommend warfarin anticoagulation. The recommended targeted to international normalized ratio for PAH is 1.5-2.0. In patients with APAH, anticoagulation is controversial with few data to support its use. In CTD, CHD, and portopulmonary patients, the risk of gastrointestinal bleeding may be increased. Most experts recommend warfarin anticoagulation in APAH patients to be considered in patients with advanced disease on intravenous prostanoids after careful assessment for any contraindicating factors. Hypoxemia is a potent pulmonary vasoconstrictor and thus can contribute to the progression of PAH. It is recommended that patients with PAH maintain oxygen saturation greater than 90% at all times; the exception would be CHD patients and those with Eisenmenger physiology, which need to be assessed case by case. Diuretics are used to treat volume overload due to right heart failure. For diuretic naïve patients, slow initiation and monitoring of renal function are recommended with a goal of attaining near-normal intravascular volume. In acute decompensated right heart failure and/or in presence of diuretic resistance, IV diuretics are needed. Although digoxin has not been well studied in patients with PAH, it is used with careful monitoring in low doses in the setting of refractory right heart failure and/or atrial arrhythmia. With the approval of therapies targeting different pathways, combining treatments to attain improved outcome has been the natural progression in the treatment approach. The potential to increase efficacy by using combination therapy must be measured against possible toxicity and drug-drug interactions. Most studies are an add-on combination approach. One study used an upfront combination using bosentan versus a placebo in FC III or IV patients receiving intravenous epoprostenol (BREATHE-2). The study failed to show benefit, though the study was underpowered. Two studies evaluated adding inhaled iloprost to bosentan therapy in a randomized, double-blind, placebo-controlled design. The STEP study enrolled 67 patients in a 12 week study that demonstrated safety as well as improvement in 6MWD (26 m, placebo corrected, P = .051); the COMBI study, which evaluated 40 patients, failed to demonstrate benefit and the study was terminated. The largest completed combination trial in PAH to date is the PACES study, which added sildenafil as an add-on therapy to intravenous epoprostenol. This 16-week, multinational, double-blind, placebo-controlled study enrolled 267 patients who were stable on epoprostenol therapy. Patients were randomized to receive 20 mg three times a day, titrated to 40 mg and 80 mg tid, at 4-week intervals, or a corresponding placebo. At the end of 16 weeks, more than 80% of patients had reached the 80 mg tid dosing level. The primary endpoint was changed in 6MWD and there was a placebo-adjusted increase of 26 m in the subjects who received sildenafil. There were 7 deaths in the placebo group and none in patients receiving sildenafil. Clinical worsening events, defined as death, transplant, hospitalization, or an increase in epoprostenol dose, were significantly different in favor or the treated group. Several large studies are currently underway evaluating the effect of combining different classes of oral regimen, including the COMPASS-2 trial (Effects of combination of bosentan and sildenafil versus sildenafil monotherapy on morbidity and mortality in symptomatic patients with pulmonary arterial hypertension), which is the first morbidity/mortality driven trial focusing on combination therapy in PAH. Lung transplantation as a potential therapeutic option needs to be considered and discussed at the time of diagnosis. Optimal timing of referral can be challenging; local practices and organ availability need to be considered: Patients who are good candidates with suboptimal response/progression on treatment need to be referred without delay Note that under the current prioritizing system used by the United Network of Organ Sharing (UNOS), PAH patients may be assigned lung allocation score below that of patients with other common diagnosis (i.e., pulmonary fibrosis, COPD). The thoracic committee is working on the system and is reviewing exceptions based on hemodynamic criteria to elevate the score to be more consistent with the degree of the patients’ clinical status Although no “absolute” indications for lung transplant referral exists, it is recommended that for patients who are on optimal medical therapy (which usually includes a systemic prostacyclin) and have evidence of hemodynamic instability (elevated right atrial pressure and/or decreased cardiac output), proceeding with completing lung transplant evaluation needs to take place. With an increasing number of options available, the decision regarding when and how to use such treatments have become more complex. It is important to realize that head-to-head comparative trials of agents have not been performed. A useful tool to guide clinicians in making therapeutic decisions is outlined in the ACCF/AHA recommendations for PAH, which uses a list of clinically relevant parameters to make a risk assessment of patients (Table 6). Patients with more advanced and symptomatic diseases are recommended to receive continuous infusion prostacyclins (Figure 3). Additionally, for those patients who are assessed as functional class II or III, either class of oral drugs can be initiated, but frequent reassessment is critical (Table 1). (See section on Long-term Management.) A. Immediate management. Pulmonary arterial hypertension usually manifests itself as a gradual, progressive development of dyspnea, therefore generally an outpatient presentation and evaluation. There are cases in which PAH can present acutely: Young patients presenting with syncope: Although PAH is a rare disorder, it should be considered in the differential for a young patient presenting with syncope associated with exertion (i.e., related to sports activity, acute onset). PAH in a young patient can be difficult to recognize for they can compensate well during initial stages and the condition can progress rapidly (especially if related to a genetic disorder Patients with PAH (not diagnosed or on therapy): Presenting with systemic disorder (most common presentations include upper respiratory infection/pneumonia, atrial arrhythmias, dietary indiscretion leading to right heart failure) As discussed above, the consequences of pulmonary vascular remodeling are an increase in pulmonary vascular resistance (PVR) and impedance of flow. These changes cause RV strain that impairs filling and causes RV volume and pressure overload. The RV then either hypertrophies and/or dilates, encroaching on the LV. Due to ventricular interdependence (note that two ventricles share the same shared bundle of fibers and that they move in “series”), RV hypertrophy and dilatation results in decreasing LV preload, cardiac output, and coronary perfusion. Increased RV wall stress results in RV ischemia. Tricuspic regurgitation develops as a result of RV dilatation resulting in chronic elevation of central venous pressure (CVP), which increases renal vein pressure leading to renal dysfunction; peripheral edema; visceral organ edema, resulting in hepatic dysfunction; and gut edema, causing decreased absorption of medications and nutrients. Another consequence of RV failure is the opening of the foramen ovale and development of right to left shunting that can augment cardiac output at the expense of oxygenation, causing or worsening hypoxemia. Definition of RV Failure Essentially, it is the inability of the RV to maintain adequate circulation through the pulmonary vascular bed at normal central venous pressure Goals for Management of RV Failure 1. Optimize RV preload 2. Maintain systemic blood pressure (keep PVR less than SVR) to avoid RV ischemia 3. Augment cardiac output 4. Reduce PVR A. Optimal Preload Critical in RV Failure Within physiologic limits, RV preload does improve contractility (approximately CVP 12-15 mm Hg) Excessive RV preload has the potential to overdistend the RV and cause impaired LV filling and decrease cardiac output via ventricular interdependence. Essential to avoid fluid bolus without careful assessment Diuretic use in RV failure Consider effects of ineffective GI absorption in RV failure Intravenous diuretics are more effective to “unload” the gut edema and initiate diuresis process If inadequate response, consider continuous intravenous diuretic drip Use combination treatments with Spironolactone (relatively preserved renal function and electrolytes; effective in aiding treatment of ascites) Intravenous Diuril in conjunction with loop diuretics Ultrafiltration when diuretics are not sufficient to unload the RV B. Use of Pressors in the Management of Acute RV Failure Essential goal of treating decompensated RV failure is to maintain systemic blood pressure above pulmonary artery pressure to preserve right coronary blood flow Perfusion of RCA occurs throughout the cardiac cycle, dominating in systole. As PVR approaches SVR, coronary perfusion decreases. Judicious use of vasopressors can ameliorate RV ischemia. Need to balance with direct effects on pulmonary circulation (effect on PVR and HR) Key points regarding pressors (Table 7) Use of Pressors in Pulmonary Hypertension and Right Heart Failure Norepinephrine (Equipotent ß1 and α1 receptor agonist) Low doses decrease PVR/SVR ratio Probably the best first-line agent for PH/RHF with hypotension Improves RV function by improving SVR and increasing cardiac output Potential to increase PVR at higher doses Phenylephrine (Direct α1 agonist) Increases PAP and PVR, decreases cardiac output, and worsens RV function In PH and RVF, should be avoided Epinephrine (Potent ß1 and α1 agonists) Often agent of last resort Not much data in PH and RVF Isoproterenol (ß1 and ß2 adrenergic agonist) Use limited by tachyarrhythmia Arginine vasopressin (V1 receptor agonist) Decreases PVR/SVR ratio Less tachyarrhythmias than norepinephrine Useful in PH/RVF and hypotension refractory to/or as first-line agent Used as rescue therapy in PH crisis, RV failure with hypotension after cardiac surgery, sepsis with PH and RV failure C. Optimizing Cardiac Output Animal models of PH Doses up to 5 mcg/kg/min, reduce PVR while increasing CO Doses 5-10 mcg/kg/min, significant tachycardia without improving PVR Canine model of acute RVF Dobutamine superior to NE in promoting RV/PA coupling Clinical uses for both acute and chronic PH Doses should be maintained <5 mcg/kg/min Combine with pulmonary vasodilator when feasible May cause systemic hypotension due to peripheral -adrenergic effects and may need NE or vasopressin for BP support Animal models of PH Significantly reduce PVR and improve RV function In pediatric population, demonstrated additive pulmonary vasodilatation when combined with inhaled nitric oxide One comparison study with PDE-5 inhibitor demonstrated inferior pulmonary selectivity and more systemic hypotension Clinical use in PH patients Systemic hypotension is the most common limiting factor. In a patient with stable BP, milrinone can augment cardiac output and provide both arterial and venous dilatation . Low dose recommended and better tolerated (0.25-0.375 mcg/kg/min) Dose adjustment needed for renal impairment (relatively contraindicated for clinically relevant renal insufficiency) Unclear benefit due to tachyarrhythmia effects, which is commonly seen with dopamine. A major drawback since increase in heart rate can worsen demand ischemia. Increase in heart rate can be seen in low doses (so called “renal dose”). Shown to increase PVR/SVR ratio Levosimendan (available in Europe) Acts as a vasodilator and has been shown to improve diastolic function and myocardial contractility without increasing oxygen consumption. It has been shown to reduce PVR and improve PA-RV coupling in experimental acute RV failure D. Optimizing PVR – Pulmonary Vasodilators Pulmonary vasodilators in acute RVF setting – desirable characteristics Selective for pulmonary vascular bed with little or no systemic effects Administered via intravenous or inhaled route Easily titratable and short half-life No toxic metabolites/side effects Cost benefit ratio favorable Inhaled Nitric Oxide Highly selective for pulmonary vascular bed with no systemic BP effects, thus ideal agent for PH/RV crisis Can be given via facemask or endotracheal tubing for a ventilated patient Not widely available in all institutions; high cost associated with use Intravenous epoprostenol can be challenging to initiate in the setting of acute RVF due to systemic hypotensive effects Inhaled epoprostenol has been shown to decrease PAPs, and improve cardiac output without effect on systemic BP after CT surgery. Also shown to improve oxygenation. Significant cost savings reported compared with inhaled nitric oxide Refractory PAH to medical therapy is characterized by Progression of RV failure and end-organ damage Worsening hypoxia and oxygenation requirement Spectrum of mechanical circulatory support for the failing RV include the following Right ventricular assist device (RVAD), which can be surgically or percutaneously implanted For PAH patients, complications associated with increased PVR makes this not an ideal supportive device Extracorporeal Membrane Oxygenation (ECMO) ECMO – routinely used to completely bypass the failing heart and provide gas exchange VA ECMO (Veno-Arterial) – used when both circulatory and pulmonary support required, as in decompensated PAH VV ECMO (Veno-veno) – used for respiratory support Novalung – pumpless device serving as parallel circuit to the lung This can unload the RV by providing right to left shunt and provide oxygenation without centrifugal pump Most experiences reported from group in Toronto, as well as a few in Europe Patients presenting with line related problems with intravenous prostacyclin infusions For patients receiving intravenous prostacyclins (epoprostenol or treprostinil) with complaints of irritation/discomfort, drainage, fever, malaise, complications of line infection need to be managed immediately which include: Full evaluation including comprehensive laboratory evaluations, cultures Prompt initiation of intravenous antibiotics Gram-positive coverage (i.e., Vancomycin) for patients on epoprostenol Gram-positive and negative coverage (i.e., Vancomycin and Piperacillin) for patients on treprostinil Critical points to remember for emergent treatments: PROSTACYCLIN INFUSIONS CANNOT BE INTERRUPTED If the tunneled line cannot be used (purulent drains, dislodged, line malfunction, etc), epoprostenol and treprostinil can be given via peripheral catheter temporarily For epoprostenol, this diluent is not compatible with any other drugs/infusions. There must be a dedicated line solely for the epoprostenol infusion In the case of sepsis with hemodynamic instability (hypotension), a dose decrease may be necessary. This is best done with the PH specialist managing the patient. Vasopressor support may also be needed (see Immediate Management – Pressors in the Management of RV Failure) A. Physical Examination Tips to Guide Management. Signs of Refractory / Worsening RV Failure Blood pressure – decreasing/low blood pressure ominous sign for further progression of RV failure (see section: Immediate Management) Heart rate – maintaining optimal heart rate essential to optimize cardiac output (CO = SV × HR). Insufficient heart rate – with clinical evidence of low output state, may need to adjust therapies to assist achieving optimal rate. If patient is on AV nodal blocking medications or beta blockers, decrease the dose. If in context with systemic hypotension, initiation of vasopressors and/or inotropes would be of clinical benefit. Tachycardia – usually a sign of decompensated state and best not to slow it down pharmacologically but to treat underlying problem (i.e., optimize volume status and cardiac output). Atrial arrhythmia – atrial fibrillation uncommon in PAH. If present, should carefully scrutinize for evidence of LHD. Atrial flutter can be seen as well as atrial tachycardia. All these arrhythmias poorly tolerated in PAH. Need to assess to restore sinus rhythm whenever possible. Oxygenation – maintaining stable and adequate oxygenation is critical in the treatment of PAH for hypoxemia/acidosis further aggravates vasoconstriction and pulmonary vascular dysfunction. Support with appropriate modalities (facemask, BIPAP, CPAP, Vapotherm) need to be done as expediently as possible. Mechanical Ventilatory Support – patients with advanced PAH generally do not tolerate the measures associated with intubation well. The effects of anesthesia used for intubation can result in hemodynamic/circulatory compromise. Furthermore, weaning the patient off the ventilatory support usually becomes very difficult process. Due to high morbidity (as well as mortality) associated with mechanical ventilatory support in these patients, every effort should be placed to use pharmacologic and respiratory support, as well as treating underlying conditions (sepsis, pneumonia, acid/base imbalance). However, patients with PAH do not tolerate hypoxemia well either. These are very challenging patients to manage. A referral to a PAH center is highly advisable. Signs of volume overload due to RVF – increase in JVP, hepatomegaly, ascites, hepatojugular reflux, peripheral edema. Signs of inadequate perfusion – low systolic pressure, low pulse pressure, decreased mentation (especially in elderly), cool extremities, unintentional weight loss, loss of appetite, nausea with oral intake, presyncope, and syncope. B. Laboratory Tests to Monitor Response To, and Adjustments in, Management Renal function assessment BUN, creatinine, urine output – essential to follow renal function parameters and response to diuretic treatments. If there is an inadequate urine output response, then consider providing more inotropic support and/or intensify diuretic regimen. Ultrafiltration or renal replacement therapy (continuous versus intermittent) may need to be considered. Markers of RV Function / Perfusion Total bilirubin (T. bili) – elevated T. bili can be a marker of hepatic congestion due to RV failure. BNP – marker of cardiac “stretch and stress.” Useful to compare with a baseline (if available) to determine the extent and follow with treatment. C. Long-term management. It is critical to have scheduled close follow-up of PAH patients on treatment(s). Three major reasons include: Patients usually have variable responses to PAH treatments. It is imperative to reassess patients within 1-3 months (depending on severity/therapy used) to assess for clinical changes. The effects of therapy can wane and change over the course of time. Even patients who did well initially can have recurrence of symptoms and worsen precipitously Side effects of PAH medications are diverse and varied. Close follow-up of these parameters are needed to ensure safety and optimal care of patients. In order to assess patient’s prognosis and progress on treatment, several surrogate markers have been studied as discussed below. A risk based approach has been recommended to determine appropriate treatments (see Medical Treatment). A list of surrogate markers and associated risk parameters are listed in Table 6. On treatment goals for PAH patients are as follows: To reach functional Class I or II Achieve 6MWD greater than 380 m (there is considerable amount of debate and controversy regarding use of 6MWD in PAH. While the set distance “number” is not agreed upon, it is accepted that a walk distance of greater than 400 m is associated with a better outcome. To improve hemodynamics with normalization of cardiac index (>2.2 L/min/m2) and RAP (<8 mm Hg) On echo, to normalize RV size and function and absence of any pericardial effusion In following BNP as a surrogate for RV function, for BNP to decrease (studies have shown that a decrease in BNP by 33-50% in the initial 3 months of starting a therapy is associated with favorable 1-year survival) and normalize. Functional class (FC) assessment has proven to be a reliable indicator of severity of disease at baseline and as a marker to determine response to therapy. Functional assessment classification modified from New York Heart Association (NYHA) functional classification has been adopted for PAH (Table 8). The most notable difference between the two systems is in its definition of class IV, which included patients with signs of right heart failure and syncope, highlighting the importance of right ventricular dysfunction as a significant clinical marker of poor outcome. Extensive studies have explored correlation between FC at baseline and outcome. Patients with IPAH in the NIH study, the risk of death was higher for patients in NYHA-FC III or IV than among those in NYHA-FC I or II. For patients in NYHA-FC I or II, the median survival was almost 6 years, while it was 2.5 years for patients in NYHA-FC III and 6 months for NYHA-FC IV. Baseline functional assessment was also shown to be highly predictive of outcome among patients treated with long-term epoprostenol. In one large retrospective study among IPAH patients treated with epoprostenol, survival after 3 and 5 years was 81% and 70%, respectively, for those patients who were in NYHA-FC III at presentation whereas for patients who were in functional class IV at baseline, the survival rates at the same time points were 47% and 27%. These earlier studies highlighted the importance of initiating treatments early in the course of PAH. Assessments to determine correlation between FC on therapy and outcome have been done as well. In the same retrospective study as above among IPAH patients treated with epoprostenol, patients who improved to FC I or II by first follow-up period (17 ± 15 months) had 3- and 5-year survival rates of 89% and 73%, respectively, compared with 62% and 35% for patients who were FC III. Patients who were FC IV at the same time period suffered the worst outcome with 42% survival at 2 years and 0% at 3 years. Current recommendations states that FC assessment is recommended to be made early after initiation of therapy with the goal of attaining FC I to II. For patients who demonstrate clinical improvement achieving FC I or II within the initial months after therapy, they should be carefully followed. For patients who deteriorate or remain in functional class IV, evaluation for lung transplantation should be pursued. Six-Minute Walk Test The distance covered during a 6 minute hall walk test (6MWD) has been the primary end point for almost all the major pivotal clinical trials in PAH. However, significant debates and studies are ongoing that explore which parameter(s) best serves as a surrogate of RV function with activity in PAH patients (i.e., absolute distance covered, % predicted achieved, heart rate recovery, or combination of these factors). It is generally agreed that patients who can achieve greater than 400 m have a favorable clinical outcome. Right Heart Catheterization Follow-up RHC is necessary in patients who are on continuous systemic prostanoid therapy, who demonstrate clinical deterioration, or a suboptimal response to treatment(s). Specifically, RHC is needed to ensure that continuous prostanoid dose is optimal by adjusting based on the cardiac index rather than increasing as clinically, which can result in high CO failure. Repeat RHC is highly variable among PH centers. Echocardiographic changes have been noted following therapies. In the prospective randomized trial with epoprostenol, the 12-week infusion of prostacyclin had beneficial effects on right ventricular size, curvature of the interventricular septum, and maximal tricuspid regurgitant jet velocity. Similarly, comparison of patients receiving treatment with the nonselective endothelin-1 antagonist bosentan demonstrated significant differences in changes in ventricular morphology, the minimum diameter of the inferior vena cava, and Doppler measurements, including right ventricular ejection time and mitral valve peak velocity. The echocardiographic finding that tends to be the focus by most physicians when evaluating for PAH—the estimation of PA systolic pressure measurement—has not been found to be predictive of outcome. Furthermore, studies performed to assess correlation between echocardiographic assessment of PASP and right heart measurement have demonstrated variable findings, depending on the population studied, the time interval between the two measurements, and the method of estimating the right atrial pressure, among others. Biomarker: B-type Natriuretic Peptide Brain natriuretic peptide (BNP) is produced mainly in the ventricles in response to myocyte stretch and stress. Considering the effect PAH has on the right ventricle, measuring BNP level has biologic plausibility. BNP levels have been shown to correlate with hemodynamics and survival in following patients on therapy. Furthermore, follow-up measurements after 3 months of epoprostenol therapy indicated that changes in plasma BNP levels correlated closely with changes in hemodynamics and demonstrated to be an independent predictor of survival. NT-proBNP among PAH patients of various etiology demonstrated good correlation between plasma levels of NT-proBNP and hemodynamics and survival. Using plasma BNP measurements as surrogate markers appears attractive since it is relatively easy to obtain, and provides an objective data that lends to serial comparative evaluations over time. The weakness lies in its variability, both in defining a range for the disease state and intrasubject variability. Furthermore, the potential for comorbidities and concurrent therapies, which can affect BNP levels makes it difficult to interpret findings. However, it provides yet another way of assessing the state of the right ventricle and studies are being conducted to further elucidate potential use of BNP as a marker of disease severity in PAH. Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) is a U.S. database of over 3,000 patients used to assess outcomes in those receiving PAH-directed therapy. It is the largest database being collected for PAH. The clinical risk factors for mortality are as follows: PVR more than 32 Wood units PAH associated with portal hypertension Functional Class IV Men older than 60 years of age Family history of PAH Other factors predictive of mortality PAH associated with connective tissue disease Functional Class III Mean RAP more than 20 mm Hg Resting systolic BP less than 100 mm Hg and HR more than 92 bpm 6MWD less than 165 ms BNP greater than 180 pg/ml Percent predicted carbon monoxide diffusing capacity less than or equal to 32% Pericardial effusion on echocardiogram Functional Class I 6MWD more than or equal to 440 ms BNP less than 50 pg/ml Percent predicted carbon monoxide diffusion capacity more than or equal to 80% E. Common Pitfalls and Side-Effects of Management Patients on Intravenous Prostacyclins Tunneled catheter care Patient and at least one family member must be fully knowledgeable on the management of the catheter and the pump. Patient must be educated to call if any discomfort is felt around the line, drains, any elevation in temps, or alarms from the pump. Patient should have the number to their specialty pharmacy that provides the intravenous medications since they provide 24/7 nursing support. Patients are best advised to alert their local hospital ED department about their diagnosis, treatment, and potential need for emergency care. Patients on Endothelin receptor antagonists The development of edema and increasing symptoms can be seen. Patients must be counseled to monitor their weight and to call if they see increase in peripheral edema, weight gain, or worsening dyspnea. It is not unusual to need increase in diuretics during the initiation of ERA treatments. For most individuals, after the appropriate adjustment period, their symptoms improve and the need for diuretics resolve. If this period goes untreated, patients can present with marked exacerbation of right heart failure, hypoxia, and decline in renal function. Patients on PDE-5 Inhibitors Patients need to be followed closely to see if their clinical improvement is maintaining. Similar to other therapies in this class (i.e., nitrates), the initial efficacy can diminish. Patients need to be educated regarding contraindication with all forms of nitrates and to let their emergency health care providers know that they are on “Viagra” (Revatio not known to most physicians). IV. Management with Co-Morbidities Portopulmonary hypertension is one of several pulmonary complications of liver disease, where PAPs are due to increased resistance to blood flow in patients with portal hypertension. ALL candidates for liver transplantation must undergo echocardiography to screen for portopulmonary hypertension. If elevated PAPs are detected, RHC must be performed. The definition of portopulmonary hypertension by RHC is as follows (same as PAH hemodynamic definition): Elevated mPAP more than 25 mm Hg Increased PVR more than 240 dynes.s.cm-5 (>3 Wood units) Normal PCWP less than 15 mm Hg or an elevated transpulmonary gradient (TPG) (mPAP-PCWP; abnormal is >12 mm Hg) In portopulmonary hypertension, it is critical to determine what is causing the PAPs to be elevated: High CO state – in general, patients with liver disease have high CO, which can elevate PAPs Increased blood volume due to fluid shifts (elevated PCWP) In portopulmonary hypertension, the PAPs and PVR are elevated. Thus in a patient with mPAP of 45 mm Hg, PCWP 14 mm Hg (TPG = 31 mm Hg) and CO of 6 L/min, the PVR (TPG/CO) is 5.2 Wood units, this patient has portopulmonary hypertension. However, if the patient has mPAP of 45 mm Hg and PCWP 18 (TPG 27) and CO 6 L/min, though the PCWP is above the defined limit of 15 mm Hg the PVR is elevated at 4.5 Wood units so you would not exclude the diagnosis. If the same patient had CO 9 L/min, then the PVR would be 3 Wood units, which would suggest that elevated PAPs are due to a high output state. Acute vasodilator studies are not very helpful in portopulmonary hypertension. These patients generally do not tolerate calcium channel blockers since they can exacerbate edema and portal hypertension. Also a positive vasodilator response does not predict survival with or without liver transplantation. General recommendations regarding portopulmonary hypertension and liver transplantation: mPAP less than 35 mm Hg: usual risk; no treatment for PH needed mPAP 35-50 mm Hg: increased risk; consider PAH-directed therapy to determine response Risk during induction of anesthesia, during and after graft reperfusion, and in the immediate post op period Hemodynamic perturbations are due to an increase in blood flow following reperfusion or due to fluid boluses needed, which can exacerbate pulmonary hypertension and RV failure Mortality rates reported range from 36% to 92% for patients who underwent liver transplant with an mPAP greater than 35 mm Hg mPAP greater than 50 mm Hg: high risk; most likely not eligible for liver transplant; consider medical therapy Scleroderma population represents the largest group of patients potentially at risk for developing PAH The most at-risk are patients with limited scleroderma (especially after several years of diagnosis). Patients with scleroderma complicated by PAH do not respond well to PAH treatments compared to IPAH patients. Scleroderma, the diffuse form in particular, is associated with other organ involvements, which can cause significant complications: GI complications – this is common with scleroderma and can be problematic since many of the PAH therapies can exacerbate these problems (i.e., reflux and diarrhea). Other GI problems include difficulty swallowing, malabsorption, bleeding, and weight loss. Pulmonary disease – most common and deadly manifestation. The most often seen are pulmonary fibrosis or interstitial lung disease. V. Patient Safety and Quality Measures A. Appropriate Prophylaxis and Other Measures to Prevent Readmission. It needs to be stressed that the outcome of patients with PAH have improved significantly with advances in medical therapy. PAH has evolved from a fatal disease to a chronically managed condition. Prognosis is dependent on the following: Functional Class upon presentation – worse for those who present at FC IV Associated conditions – scleroderma-associated PAH have worse prognosis Demographics – male gender, older population Presence of other organ dysfunction – renal insufficiency Salt and Fluid Management Sodium restricted diet (<2,000 mg/day) is advised and is especially critical for patients with RV dysfunction in order to manage volume status. For advanced disease state with RV failure, fluid restriction (<2 L/day) is recommended. Monitoring daily weights should be encouraged. Yearly influenza vaccinations are advised, as well as being up to date with pneumococcal vaccinations. Hemodynamic fluctuations of pregnancy, labor, delivery, and postpartum period are poorly tolerated in PAH patients and can result with devastating consequences. Though not much data is present, some series report up to a 50% maternal mortality rate. Current guidelines recommend that pregnancy be avoided or terminated early in women with PAH. It is imperative to have discussions regarding methods of birth control with patients. There is a great deal of controversy as to what is the preferred method. Estrogen-containing contraceptives may increase the risk of venous thromboembolism; however, currently available lower-dose preparations with concurrent warfarin anticoagulation may be an option. Some have advocated surgical sterilization and barrier methods. Low-level graded aerobic exercise (i.e., walking) recommended as tolerated. A study among 30 PAH patients who were stable on PAH treatments demonstrated improvements in 6-minute walk distance (6MWD), quality of life, functional class, and peak oxygen consumption. It is best to avoid heavy physical exertion or isometric exercise (i.e., straining against a fixed resistance) as this can cause exertional syncope. Exposure to high altitudes may contribute to hypoxic pulmonary vasoconstriction and is not well tolerated. For air travel, it is recommended that for patients with preflight pulse oximetry less than 92% to receive supplemental oxygen. Sodium restricted diet (<2,000 mg/day) is advised and is especially critical for patients with RV dysfunction to manage volume status. For advanced disease state with RV failure, fluid restriction (<2 L/day) is recommended. Monitoring daily weights should be encouraged. Yearly influenza vaccinations are advised as well as being up to date with pneumococcal vaccinations. Hemodynamic fluctuations of pregnancy, labor, delivery and postpartum period are poorly tolerated in PAH patients and can result with devastating consequences. Though not much data is present, some series report up to 50% maternal mortality rate. Current guidelines recommend that pregnancy be avoided or terminated early in women with PAH. It is imperative to have discussions regarding methods of birth control with patients. There is a great deal of controversy as to what is the preferred method. Estrogen-containing contraceptives may increase risk of venous thromboembolism; however, currently available lower-dose preparations with concurrent warfarin anticoagulation may be an option. Some have advocated surgical sterilization and barrier methods. B. What's the Evidence for specific management and treatment recommendations? McLaughlin, VV, Archer, SL, Badesch, DB. “ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents”. J Am Coll Cardiol. vol. 53. 2009. pp. 1573-1619. (Comprehensive up-to-date recommendations on background, evaluation, and management of PAH.) D’Alonzo, GE, Barst, RJ, Ayres, SM. “Survival in patients with primary pulmonary hypertension: results from a national prospective registry”. Ann Intern Med. vol. 115. 1991. pp. 343-9. (Epidemiology and risk assessments from the first NIH PPH Registry data from 1980s, prior to PAH-specific treatments were available. Only publications with natural history and outcome of PAH.) Simonneau, G, Robbins, IM, Beghetti, M. “Updated clinical classification of pulmonary hypertension”. J Am Coll Cardiol. vol. 54. 2009. pp. S43-54. (Lists clinical classification for PH from the 5th Dana Point World Symposium Meeting) Barst, RJ, Gibbs, SR, Hossein, A. ” Updated evidence-based treatment algorithm in pulmonary arterial hypertension”. J Am Coll Cardiol. vol. 54. 2009. pp. S78-84. (Discusses updated treatment recommendations from the 5th Dana Point World Symposium Meeting) Badesch, DB, Champion, HC, Sanchez, MA. “Diagnosis and assessment of pulmonary arterial hypertension”. J Am Coll Cardiol. vol. 54. 2009. pp. S55-66. (Discusses the evaluation recommendations from the 5th Dana Point World Symposium Meeting) de Perrot, M. J Heart Lung Transplantation. vol. 30. 2011. pp. 997(Study demonstrating Novalung experience from the Toronto Group) C. DRG Codes and Expected Length of Stay. Despite changes in nomenclature in medical research and in published literature, billing codes still use the old terminologies “Primary Pulmonary Hypertension” (PPH) and “Secondary Pulmonary Hypertension” (SPH). Primary Pulmonary Hypertension (416.0) Secondary Pulmonary Hypertension (416.8) Other relevant DRGs Dyspnea/shortness of breath (786.05) Scleroderma (710.1, 701.0) Diastolic heart failure, unspecified (428.30) Diastolic heart failure, acute (428.31) Diastolic heart failure, chronic (428.32) Diastolic heart failure, acute on chronic (428.33) Right heart failure (428.0) Length of Stay (LOS) This varies depending on clinical situation 1. Initial admission for evaluation; full workup, including RHC; and possible initiation of treatment. LOS can vary mostly depending on how unstable patient is upon presentation. Key workups include echocardiogram (with bubble if not done), VQ scan, PFTs, 6MWT (if able), serologies, and RHC. 2. For patients with advanced PAH (FC IIIB or IV, or decompensated RHF) and require intravenous therapy, usually LOS 3-5 days. Recommend transfer to PH Center. 3. For decompensated right heart failure admission requiring intravenous diuretics with or without inotropes, usually 3-5 days but can vary pending other underlying comorbidities and conditions. 4. For central access-related admissions, 1-3 days (or longer) depending if related to malfunction or infectious or both. 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. Pulmonary Arterial Hypertension: What Every Physician Needs To Know. - II. Diagnostic Confirmation: are you sure your patient has Pulmonary Arterial Hypertension? - A. History Part 1: Pattern Recognition - B. History Part 2: Prevalence: - C. History Part 3: Competing diagnoses that can mimic Pulmonary Arterial Hypertension - 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? - 2. What imaging studies (if any) should be ordered to help establish the diagnosis? - III. Management - A. Immediate management. - A. Physical Examination Tips to Guide Management. - B. Laboratory Tests to Monitor Response To, and Adjustments in, Management - C. 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	53	0	0	0	3	0.985055	3	28,692
Cavernous lymphangiomas are generally present at birth, but may appear later in the child's life. These bulging masses occur deep under the skin, typically on the neck, tongue and lips, and vary widely in size, ranging from as small as a centimeter in diameter to several centimeters wide Histologically, a lymphangioma can be a well-circumscribed lesion composed of 1 or multiple large cysts, which can interconnect. These are typically called a cavernous lymphangioma. A lymphangioma can also be composed of microscopic cysts producing an ill-defined, compressible, spongelike lesion, known as a cystic lymphangioma.[2 Cavernous lymphangiomas are usually identified in infants and children with the majority of lesions found around the head and neck, trunk or extremities. Tumours affecting the intra-abdominal organs are rare She was diagnosed with cavernous lymphangioma of the jejunum with histopathological examination, and cured by surgical resection. With rapid development in advanced endoscopic techniques, including enteroscopy, jejunal lymphangioma has become less rare than before. As a result, there is an unmet need for an algorithm for identification and. Cavernous Lymphangioma It can occur in any region of the body, including the tongue and is composed of dilated lymphatic vessels. It appears as a skin colored, red or bluish rubbery inflammation under the skin Cavernous hemangioma, also called cavernous angioma, cavernoma, or cerebral cavernoma (CCM) (when referring to presence in the brain) is a type of benign vascular tumor or hemangioma, where a collection of dilated blood vessels form a lesion.The abnormal tissue causes a slowing of blood flow through the cavities, or caverns. The blood vessels do not form the necessary junctions with. Cavernous lymphangioma Typically, during infancy, a solitary rubbery nodule with no skin changes becomes evident in a single location, such as the face, trunk, or extremity. These lesions often.. Cavernous lymphangioma forms an ill-defined, spongy, and compressible mass and is found most commonly in the tongue, cheek, floor of mouth, and lips; it is uncommon in the soft tissues. In contrast, capillary lymphangioma is usually confined to the skin and is clinically the least significant of the three types. Pathologic feature • Cavernous lymphangioma • Soft, spongy masses of variable size • Progressive lymphangioma • Reasonably circumscribed, pinkish red cutaneous macule • Progressive enlargment over years • May reach considerable size (>10 cm) Prevalence • ~5% of vascular tumors • No discernable predilection for any race or sex Age • Progressive lymphangioma Cavernous venous malformation (cavernous hemangioma) is the most common benign neoplasm of the orbit. It is considered a congenital abnormality. There is no evidence to suggest a heritance pattern. It is not a neoplasm in the usual sense, as it is not derived from a single cell, proliferating cell Cavernous, arteriovenous, and mixed hemangioma-lymphangioma of the rectosigmoid: rare causes of rectal bleeding--case series and review of the literature Int J Colorectal Dis. 2008 Jul;23(7):653-8. doi: 10.1007/s00384-008-0466-4. Epub 2008 Mar 11. Authors Patricia Sylla 1. Cavernous lymphangiomas are centered in the reticular dermis and/or subcutis and are composed of numerous ectatic lymphatic channels (Fig. 13-84). There may be an associated lymphocytic infiltrate. The vascular lumens of both tumors are lined by cytologically bland endothelial cells. View chapter Purchase boo Cavernous lymphangioma typically presents during infancy as a painless, ill-defined subcutaneous swelling with no changes of the overlying skin that can be several centimeters in size. Rarely, an entire extremity may be affected. Patients may report tenderness upon deep palpation of the area Most described cases are cystic / cavernous Skin tumors are called lymphangioma circumscriptum Clinical features. Benign See also discussion in these chapters: Lymph nodes, Mediastinum, Skin - nonmelanocytic tumors, Spleen. Case reports Cavernous lymphangioma is a benign congenital lesion that usually appears in childhood. It rarely presents in the adult but may be diagnosed at this late stage due to the slow growing nature of the tumour. It is rarely found i n the salivary glands and when it does occur, the gland is usually incorporated by lymphangioma of surrounding tissue Cavernous Lymphangioma: This type of lymphangioma can occur anywhere in the body, including the tongue. The skin appears red or bluish, rubbery, and inflamed. Clear fluid can be seen within the vessels in infants. Lymphangioma Circumscriptum: This presents as a cluster of small abscesses filled with lymph fluid, ranging in colors from pink. Cavernous lymphangioma is also considered a macrocystic lesion. It is a a bluish or red rubbery swelling under the skin that can affect any area of the body, including the tongue. Lymphangioma circumscriptum is a microcystic lymphatic malformation. It appears as a cluster of small, firm blisters most commonly on the shoulders, neck, underarm. Cavernous lymphanioma and cystic lymphangioma (also called cystic hygroma, familial nuchal bleb, fetal cystic hygroma (FCH), or hygroma colli) do not affect the skinís surface like lymphangioma circumscriptum does, but instead result in a bulge or thickening of the skin. Cavernous lymphaniomas vary widely in size, ranging from as small as a. Lymphangiomas have been classified into lymphangioma simplex (capillary lymphangioma), cavernous lymphangioma, and cystic lymphangioma (cystic hygroma). Hemolymphangioma is another variant with features showing both a vascular and a lymphatic component. The head and the neck, followed by the proximal extremities, the buttocks, and the trunk are. Orthopedics \| ANSWER PLEASEFig 1.Fig 2.A 15-month-old boy presents with a slowly enlarging soft tissue mass on the dorsum of his right hand. The parents first noticed the mass when the patient was. Cavernous Lymphangioma Neck Excisio cavernous lymphangioma FREE subscriptions for doctors and students... click here You have 3 open access pages. This is a congenital lesion comprised of lymph-filled spaces which arise from an embryonic remnant of the jugular lymph sac. It is not a true cyst but rather a lymphatic hamartoma which forms multilocular cyst-like spaces Lymphangioma is located majorly around the head and neck regions, axilla, Microcystic (formerly cavernous lymphangioma) and Mixed. These classifications are based on the lesions type, size or location. Advertisement . Is Lymphangioma a Cancer? Lymphangiomas is a noncancerous rare tumors of the lymphatic system The cutaneous lymphangiomas are divided into superficial lymphangioma circumscriptum and deep lymphangioma cavernosum. No specific histologic criteria could be found to differentiate lymphangioma from bloodless hemangioma, primary from secondary lymphangioma (lymphangiectasia), or cystic cavernous lymphangoma from cystic hygroma . They also may be called cystic hygromas or cavernous lymphangiomas. They sometimes appear as bluish-reddish,.. Lymphangiomas are generally either congenital or acquired. The congenital form is due to an improper connection of lymphatic channels to the main lymphatic draining duct. Acquired lymphangiomas can occur following the interruption of lymphatic drainage through various mechanisms including surgery, trauma, malignancy, or radiation therapy Most described cases are cystic / cavernous Skin tumors are called lymphangioma circumscriptu Cavernous lymphangioma; Lymphangioma circumscriptum; Cavernous lymphangioma. Cavernous lymphangioma can affect any site on the body, including the tongue. Cavernous lymphangioma typically presents during infancy as a painless, ill-defined subcutaneous swelling with no changes of the overlying skin that can be several centimeters in size A cystic hygroma is an often congenital multiloculated lymphatic lesion that can arise anywhere, but is classically found in the left posterior triangle of the neck and armpits. This is the most common form of lymphangioma. It contains large cyst-like cavities containing lymph. It is most commonly seen with Turner syndrome Cavernous lymphangioma: Introduction. Cavernous lymphangioma: A birth disorder characterized by a lymphatic swelling under the skin that can be present at birth or occur later. More detailed information about the symptoms, causes, and treatments of Cavernous lymphangioma is available below.. Symptoms of Cavernous lymphangioma . July 2013; Indian Dermatology Online Journal 4(3):210-2; DOI: 10.4103/2229-5178.11552 Cavernous mesenteric lymphangioma (2006: 4b) Cavernous mesenteric lymphangioma (2006: 4b) Jeong, Woo; Kim, Yongsoo; Song, Soon-Young; Heo, Jung; Park, Choong 2006-05-13 00:00:00 We describe a case of mesenteric lymphangioma, a very rare disease in adults that is considered to be a developmental abnormality. Since treatment involves complete excision of the mass to prevent recurrence, it is. Lymphangiomas are congenital malformation of the lymphatic system that involve the skin and subcutaneous tissues. We are reporting two cases of cavernous lymphangioma. These cases are presented for their rarity. Keywords: Cavernous lymphangioma, congenital malformations, lymphatic malformatio Cavernous lymphangioma; Lymphangioma circumscriptum; Cystic hygroma. The cystic hygroma (also called 'cystic lymphangioma' and 'lymphangioma cysticum') is a ' macrocytic ' lymphatic malformation, and is composed of large fluid-filled spaces. It appears as a skin coloured, red or bluish, somewhat transparent, swelling under the skin Video Lymphangioma. Classification. Lymphangiomas have traditionally been classified into three subtypes: capillary and cavernous lymphangiomas and cystic hygroma. This classification is based on their microscopic characteristics. A fourth subtype, the hemangiolymphangioma is also recognized Introduction: We report an unusual case of unilateral leg swelling secondary to cavernous lymphangioma (cystic hygroma), which normally affects the head and neck regions. Case report: A 25 year gentleman presented to our department with a 13-year history of gradually increasing unilateral leg swelling and recurrent infections. Investigations showed appearances consistent with cavernous lymphangioma, and partial excision of the lesion led to resolution of symptoms Pediatricsubmental cavernous lymphangioma Lorraine Smith MD, MPH, FACS; Jason S. Hamilton, MD Figure J. Sagittal MRJ shows the large multiloculated cystic mass. A 6-year-old girl presented with a 3-week history of a painless, rapidly enlarging right submental swelling that had become noticeable following an upper respiratory infection Cavernous lymphangioma first appears during infancy, when a rubbery nodule with no skin changes becomes obvious in the face, trunk, or extremity. These lesions often grow at a rapid pace, similar to that of raised hemangiomas. No family history of prior lymphangiomas is described lymphangioma circumscriptum, cavernous lymphangioma, cystic hygroma, or acquired lymphangiomas (also known as lymphangiectasia), based on their depth and etiology. Results: A literature review revealed only 30 cases of penile lymphangioma between 1947 and March 30, 2018 Cavernous lymphangioma is a rare mediastinal benign tumor. A 43-year-old woman presented with cough and dyspnea for 1 month. Computed tomography of the chest showed a 3-cm well-circumscribed cystic mass in the posterior mediastinum. At thoracotomy, a cystic tumor in the mediastinum that was adherent to the descending aorta and esophagus was removed completely. The tumor, the cystic space of. Cavernous lymphangioma. Edit. Classic editor History Comments Share. Contents . Instructions for Filling in this Page Edit. Editing this page. To edit this page you will need to find the edit button located at the top right corner of this page. It´s just like word processing like you normally do at your desktop word processor, the main. lymphangioma [lim-fan″je-o´mah] a benign tumor composed of newly formed lymph spaces and channels. adj., adj lymphangio´matous. lymphangioma caverno´sum (cavernous lymphangioma) 1. a deeply situated lymphangioma, composed of cavernous lymphatic spaces, and always occurring in the neck or axilla. 2. cystic hygroma. lymphangioma circumscrip´tum a. Download PDF: Sorry, we are unable to provide the full text but you may find it at the following location(s): https://wjso.biomedcentral.com... (external link) http. Cavernous lymphangioma, H&E stain. Irregular, dilated spaces are visible in dermis. Lymphangiomas have traditionally been classified into three subtypes: capillary and cavernous lymphangiomas and cystic hygroma. This classification is based on their microscopic characteristics. A fourth subtype, the hemangiolymphangioma is also recognized. [8 Cavernous lymphangioma is a benign congenital lesion that usually appears in childhood. It rarely presents in the adult but may be diagnosed at this late stage due to the slow growing nature of the tumour. It is rarely found in the salivary glands and when it does occur, the gland is usually incorporated by lymphangioma of surrounding tissue A Case of Cavernous Lymphangioma of the Small Bowel Mesentery In Taik Hong, Jae Myung Cha, Joung Il Lee, Kwang Ro Joo, Il Hyun Baek, Hyun Phil Shin, Jung Won Jeon and Jun Uk Lim Department of Internal Medicine, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul, Korea Cavernous lymphangioma: deep, not involve the skin. Excise of the skin is not needed. Cystic hygroma: a kind of cavernous lymphangioma. Contain large cyst. They account for 4% of all vascular tumors and approximately 25% of all benign vascular tumors in children. It can become evident at any age, but the greatest incidence occurs at birth or. Cases of lymphangioma are diagnosed by histopathologic inspection. In prenatal cases, cystic lymphangioma is diagnosed using an ultrasound; when confirmed amniocentesis may be recommended to check for associated genetic disorders.. Classification. Lymphangiomas have traditionally been classified into three subtypes: capillary and cavernous lymphangiomas and cystic hygroma Lymphangioma in the maxillary sinus is rare. Herein, the authors report an unusual case of cavernous lymphangioma, in the maxillary sinus, with imaging examinations. Clinicians should be aware of the possibility of lymphangioma in the maxillary sinus, and be capable of distinguishing it, from other benign cystic lesions . However, depending on their location, they can cause breathing problems by choking the windpipe, or. LM: channels lined by benign endothelium containing RBCs: Subtypes: soft tissue (capillary, cavernous, arteriovenous, venous, intramuscular, synovial), childhood (tufted, microvenular hemangioma, glomeruloid hemangioma, epithelioid hemangioma (see angiolymphoid hyperplasia with eosinophilia), targetoid hemosideric hemangioma, infantile hemangioma): LM DD Radiology Gamuts Ontology -- differential diagnosis information about Cavernous lymphangioma Classification and external resources ICD 10 D18 (ILDS D18.100) DiseasesDB 7665 eMedicin Cavernous lymphangiomas present as spongy, vermiform lesions. Extensive involvement may be complicated by elephantiasis of the eyelid. Cystic hygroma is the most massive form of lymphangioma and usually involves the neck or extremities, and while seldom directly affecting the eyelid it may be associated with separate vascular lid abnormalities Cystic Lymphangioma:Happens around the neck,groin region and head.. Cavernous Lymphangioma:Arises around the groin region, head and neck, and moreover, are found inside the torso, mouth, abdomen and limbs.. Lymphangiomas are infrequent and appear as fluid-filled,painless,swollen masses that are not hateful Cavernous lymphangioma first appears during infancy, when a rubbery nodule with no skin changes becomes obvious in the face, trunk, or extremity. Surgical removal of the tumor is the typical treatment provided, with the understanding that additional removal procedures will most likely be required as the lymphangioma grows Find all the evidence you need on Lymphangioma via the Trip Database. Helping you find trustworthy answers on Lymphangioma \| Latest evidence made eas INTRODUCTION. Lymphangiomas are congenital malformations of the lymphatic system that involve the skin and subcutaneous tissues. Lymphangiomas have been classified into lymphangioma simplex (capillary lymphangioma), cavernous lymphangioma, and cystic lymphangioma (cystic hygroma). Hemolymphangioma is another variant with features showing both a vascular and a lymphatic component Cavernous lymphangioma of the tongue 52 expands to form the lymphatic system in the head and neck area. The second theory proposes that the lymphatic system is formed by mesenchymal clefts in the venous plexus reticulum that spread toward the center of the jugular sac (10). Differently than the infant lymphangioma; the etiology of lymphangioma cavernous Iymphangioma is a slow growing benign tumor with near absence of malignant symtoms and signs. Therefore one must incIude the possibil ity of cavernous lymphangioma when mainly solid mass is detected in ultrasonography and CT in the - 465 Learn more about Cavernous Lymphangioma from related diseases, pathways, genes and PTMs with the Novus Bioinformatics Tool Background: Lymphangiomas are benign lymphatic vessel hamartomas typically found in the skin or subcutaneous tissue of the head and neck. Although mostly seen in a congenital context, acquired forms have been reported. By contrast, cavernous hemangiomas are benign hamartomas of endothelial origin. They can arise anywhere in the body, but are. lymphangioma cavernosum cavernous lymphangioma 1. a deeply situated lymphangioma, composed of cavernous lymphatic spaces, and always occurring in the neck or axilla. See also vascular nevus, under nevus. 2. cystic hygroma. Symptoms of cavernous lymphangioma include a bulge or mass on the neck, lips or tongue. The mass can be small or large and is often present at birth. The mass can also be many different colors including white, pink, or black. Cystic hygoma are generally softer than the other types of lymphangioma and appear to be swollen. 75% of these are found. Mentioning: 2 - Chyloptysis is a relatively rare embodiment of disease that encompasses a lengthy differential and provides many diagnostic and therapeutic challenges. Presented here is the case of a young woman with massive chyloptysis due to a thoracic cavernous lymphangioma arising in the peripartum period. The severity of her condition mandated the use of cardiopulmonary bypass to resect. Cavernous lymphangioma and cystic hygroma have a lot of similarities that some doctors consider them to be too similar to merit separate categories. Lymphangioma Circumscriptum Lymphangioma circumscriptum is a 'microcytic' lymphatic malformation. It appears as a cluster of small firm blisters filled with lymph fluid, resembling frogspawn Synonyms for cavernous lymphangioma in Free Thesaurus. Antonyms for cavernous lymphangioma. 1 word related to lymphangioma: angioma. What are synonyms for cavernous lymphangioma Cavernous lymphangioma is a rare lesion in the breast of adults. Only a few cases have been documented in literature. We describe a 38-year-old woman who presented with a palpable breast lump, which measured 5 × 4 cm. A local excision of the lump was performed and a diagnosis of cavernous lymphangioma was made Lymphangioma of the pancreas is a very rare benign tumor, and it is thought to be caused by obstruction of the lymphatic tract. Most lymphangiomas of the pancreas reported previously were polycystic tumors, so their differential diagnosis were mostly cystic tumors of the pancreas Cavernous lymphangiomas are characterized by penetration through the subcutaneous areas between the muscular septa and represent rare variants of the more common superficial lymphangioma. Although frequently described in the fetus when involving the posterior aspect of the neck (i.e. cystic hygroma), involvement of the craniofacial region is rare Lymphangioma circumscription can be healed when treated with a flashlamp pulsed dye laser, although this can cause port-wine stains and other vascular lesions. Prognosis. The prognosis for lymphangioma circumscriptum and cavernous lymphangioma is generally excellent A superficial microcystic variant (lymphangioma circumscriptum) and a deep macrocystic variant (cavernous lymphangioma) can be recognized. Lymphangioma circumscriptum may exceptionally occur in the penoscrotal region, while cystic lymphangioma may arise in the kidney and urinary tract, including the urethra and the bladder Histopathol- ogy confirmed a benign cavernous lymphangioma of the transverse mesocolon, measuring 9 ⫻ 5 ⫻ 4 cm (Fig. 3). Mesenteric lymphagiomas are rare benign tumors, ac- counting for 70% of all intra-abdominal lymphangiomas This page includes the following topics and synonyms: Cystic Hygroma, Hygroma Cysticum, Cystic Lymphangioma, Cavernous Lymphangioma, Lymphangioma Cavernosum, Lymphangioma Cysticum Proptosis Surgery \| Big Eyes \| Cavernous Hemangioma \| Orbital Lymphoma \| Oculoplastic Surgery India Exophthalmos, also called proptosis, is a bulging of the eye anteriorly out of the orbit (bulging eyes or big eyes). Exophthalmos can be either bilateral (as is often seen in Graves' disease or Thyroi Lymphangioma Lymphangioma is rare, and constitutes [ Benign tumors of the tonsils occur infrequently. Lymphangiomas are rare congenital tumors of the lymphatic system, and tonsillar lymphangioma is an extremely rare occurrence. Its pathogenesis is uncertain, but history, clinical examination, and histological examination should establish the diagnosis. We present a 17-year-old white male with lymphangioma of the right tonsil Lymphangioma is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus, MeSH (Medical Subject Headings).Descriptors are arranged in a hierarchical structure, which enables searching at various levels of specificity	2	13	1	0	0	2	0.370686	3	5,302
SNOMED and ICD-10 are two of several sets of codes used to describe diagnoses and treatments in healthcare. Why do multiple sets of codes exist? Because each addresses a different set of use cases within healthcare. This post will give an overview of the SNOMED terminologies and ICD-10 classifications as we look at ways to normalize data between the two sets. Accurately normalizing data across these disparate code sets is important to providers, patients, and payers. Incorrect coding or coding that is not specific enough can result in claim denials. Accurate mapping would be a challenge even if these code sets never changed. A number of factors cause them to, however, including: - New diagnoses, such as “contact with and (suspected) exposure to SARS-CoV-2” - The upcoming version 11 of ICD - Changing interoperability requirements as mandated by Federal agencies SNOMED CT – Diagnosis and Treatment SNOMED CT stands for Systematized Nomenclature of Medicine – Clinical Terms. It’s the terminology for the diagnostic and treatment side of healthcare. SNOMED CT and its predecessors have been in use in the US since 1965. As of January 2020, the terminology set documented 352,567 concepts. These concepts are hierarchical, organized in parent/child relationships. Assigning a SNOMED code is usually automated and not as visible to end users as ICD-10. The following multi-generational references are ours. The number next to each is the concept code. You can see in this example that “Hyperglycemia due to type 2 diabetes mellitus (disorder)” is a great great great grandchild of “Clinical finding (finding).” - Parent: Clinical finding (finding) – 404684003 - Child: Disease (disorder) – 64572001 - Grandchild: Complication (disorder) – 116223007 - Great Grandchild: Complication due to diabetes mellitus (disorder) – 74627003 - Great Great Grandchild: Disorder due to type 2 diabetes mellitus (disorder) – 422014003 - Great Great Great Grandchild: Hyperglycemia due to type 2 diabetes mellitus (disorder) – 368051000119109 SNOMED is not just a coding system of diagnosis—it covers other types of clinical findings like signs and symptoms and includes tens of thousands of surgical, therapeutic, and diagnostic procedures in addition to observables, such as heart rate. According to SNOMED, “it also includes concepts representing body structures, organisms, substances, pharmaceutical products, physical objects, physical forces, specimens and many other types of information that may need to be recorded in or around a health record.” ICD-10 – Reimbursements and Analytics ICD stands for International Classification of Diseases. It is the standardized terminology for billing codes and has existed in one form or another since 1900. Its use in the United States began in 1968 and is managed by the World Health Organization. The current version, ICD-10, has over 70,000 codes. Those codes are assigned by professional medical coders, not an automated system as with SNOMED. ICD has two major groupings, ICD-10-CM and ICD-10-PCS. /ICD-10-CM is the code set for diagnoses. ICD-10-PCS is used in hospital inpatient settings for inpatient procedure coding. As evidence of the constant change referenced above, 577 codes were added to the 2021 ICD-10-PCS code set. ICD-10 was introduced in 1992. According to SoftwareAdvice, it took the US 23 years to completely transition to it. Now, only a few years after the completion, version 11 is scheduled to take effect in January 2022. Codes will differ substantially between versions 10 and 11. Take Alzheimer’s disease, for instance. In version 10, the code is G30. In version 11, it’s 8A20. The new version of ICD is a digital, multilingual product. It contains six major extensions called chapters. As with the transition from ICD-9 to ICD-10, payers will have to be ready to process claims with ICD-11 codes for medical diagnoses and inpatient procedures. Why Is Coding So Complex? One way to look at the root of the complexity is the fact that there are multiple dimensions, each with its own set of sub-dimensions. Above, we followed just one example of a SNOMED hierarchical path for diabetes. The disease has many dimensions, however, including: - Multiple origins (genetics, lifestyle, other medical conditions) - Multiple symptoms or impacts (infections, nerve pain in feet and legs, blurred vision) - Multiple treatments (drugs, devices, procedures, operations) The dimensions create a large number of branches in each hierarchy. Healthcare Software Systems Codes have to be mapped among IT systems at different points along the care continuum. Systems include: - Data warehouses - Other systems Codes within patient records need to be communicated among many different HIE participants, magnifying the complexity of mapping across IT systems. Such participants include: - Hospitals & health systems - Community health providers - Imaging centers - Long term care providers - Behavioral health providers These dimensions and sub-dimensions add up to many data consistency challenges. Compliance with Federal Mandates The U.S. Centers for Medicare and Medicaid Services (CMS) has mandated a July 2021 deadline for health insurers to make electronic patient data available through an application for their members, such as a smartphone app. Tied to this interoperability mandate is the need to normalize the terminologies and classifications that are used across different functional areas within a health system. SNOMED to ICD Mapping Options For many organizations, performing the data mapping between ICD and SNOMED has historically been done in one of two ways. One approach has been using large spreadsheets—thousands of columns and tens of thousands of cells. These files have multiple owners, and there are complex and maintenance and update procedures. It is labor-intensive to manage and update. Human error, in addition, can produce a negative ripple effect. Just one deleted cell can throw off thousands of spreadsheet rows. Furthermore, because traditional Excel spreadsheets are non-collaborative documents that are passed around from person to person, it’s easy for someone to end up with an outdated or incorrect version of the document. Spreadsheet alternatives such as Google Sheets and Smartsheet solve the collaboration problem, but they don’t solve ripple-effect issues. The other approach for SNOMED to ICD mapping has been custom on-premises solutions. These require dedicated IT resources to implement and manage. They are also expensive to purchase. Fortunately, there’s an easier and less expensive solution. Terminology Management for SNOMED-CT and ICD-10 Managed Terminology provides a fully managed environment in the cloud for maintaining current libraries of multiple medical coding systems like SNOMED CT and ICD and the translation between them. With a cloud environment, there is a single, consistent source of information across the enterprise. It is a more economical and efficient alternative to managing large spreadsheets or using custom, on-premise solutions. J2 Interactive offers managed terminology services, powered by HealthTerm. Have a single source of truth for clinical terminology across all of your applications.	3	5	0	0	0	1	0.518899	1	1,599
What do we mean that ICD-10 is more specific? While there are dozens of minor distinctions between ICD-9 and ICD-10 the three fundamental changes are: - There is expanded detail for many conditions (e.g., viral hepatitis has been expanded from ICD-9 070, a single 3-digit category, to ICD-10 B15-B19, five 3-digit categories - Conditions were transferred around the classification (e.g., hemorrhage has been moved from the circulatory chapter to the symptoms and signs chapter) - ICD codes used for morbidity and mortality were forked (technically the ICD-10 that most care practitioners are interested in is the ICD-10-CM) While most healthcare providers have familiarized themselves with the transfer classifications those who aren’t responsible for claims processing don’t really understand what the distinctions were in terms of complexity. Let’s take an easy example of burns which helps to make the distinction more specific. In ICD-9 there were a few codes associated with burns. 940) Burn confined to eye and adnexa 941) Burn of face, head, and neck 942) Burn of trunk 943) Burn of upper limb, except wrist and hand 944) Burn of wrist(s) and hand(s) 945) Burn of lower limb(s) 946) Burns of multiple specified sites 947) Burn of internal organs 948) Burns classified according to extent of body surface involved 949) Burn, unspecified Then the burn was subsequently secondarily coded using the rule of 9s. The rule of 9’s cut the body 12 sections 11 were weighted 9% each (head, chest, abdomen, upper back, lower back + buttocks, left arm, right arm, front left leg, rear left leg, front right leg, read right left ): and the perineum weighted the final 1%. Given ICD-9 codes a health provider, insurance company or a government agency could determine the degree of burns and over a rough percentage of the body but that was about it. With ICD-10 quite literally there are hundreds of codes used for burns. The system doesn’t use secondary coding and each burn on each part is classified individually. For example: T24.121D Burn of first degree of right knee, subsequent encounter? T24.121S Burn of first degree of right knee, sequela T24.122D Burn of first degree of left knee, subsequent encounter T24.122S Burn of first degree of left knee, sequela and when even more specificity is needed because of internal external problems ICD-10 introduces even more coding: T27.0XXD Burn of larynx and trachea, subsequent encounter T27.0XXS Burn of larynx and trachea, sequela T27.1XXD Burn involving larynx and trachea with lung, subsequent encounter T27.1XXS Burn involving larynx and trachea with lung, sequela T27.2XXD Burn of other parts of respiratory tract, subsequent encounter T27.2XXS Burn of other parts of respiratory tract, sequela T27.3XXD Burn of respiratory tract, part unspecified, subsequent encounter T27.3XXS Burn of respiratory tract, part unspecified, sequela This kind of coding is only practical because EMR are directly translating medical notations rather than having human coders / billing people translating notes for billing. The core of MU2 is removing the need for expert / human judgment from the seams between subsystems in the EMR. Architecting the systems that handle these seams has been the core of what companies like Nalashaa have been doing to move providers and ISVs across the country towards MU2 and ICD-10. Latest posts by Jeff Bolden (see all) - Blue Button+: A guide for EMR/EHR/PHR ISV’s (Part 2) - May 8, 2014 - Blue Button+: A guide for EMR/EHR/PHR ISVs (part 1) - April 25, 2014 - What do we mean that ICD-10 is more specific? - March 25, 2014	4	3	0	0	0	6	0.488058	6	910
OVERVIEW: What every practitioner needs to know Are you sure your patient with human immunodeficiency virus has pneumonia? What should you expect to find? Patients with pneumonia typically present with a number of cardinal symptoms and signs, which are listed below. The presentation may vary depending on the degree of immunosuppression or the organism causing the pneumonia; some organisms may even present with a normal physical exam. Cough—Cough is one of the most common indicators of pneumonia and may be productive or nonproductive, depending on the organism. Dyspnea—Dyspnea is frequently present in pneumonia, ranging from mild exertional shortness of breath to severe dyspnea at rest. The onset is typically progressive and may be subacute and insidious in the case of fungal pneumonia or Pneumocystis pneumonia (PCP). Acute onset of severe dyspnea should raise concern for another process, such as myocardial infarction, pulmonary embolus, or cardiogenic edema. Chest pain—Pleuritic chest pain may accompany pneumonia, particularly in the case of a necrotizing process or an associated pleural effusion or empyema. Musculoskeletal chest pain may develop in the setting of relentless cough. Severe chest pain in the absence of radiographic abnormalities should raise concern for pulmonary embolus or cardiac process. Fever—Fever typically accompanies pneumonia, but may not be present in a patient with severe immunosuppression. Abnormal lung exam—Patients with bacterial pneumonia typically have physical findings of lobar consolidation, including rhonchi, bronchial breath sounds, and egophany. Dullness to percussion in the absence of egophany suggests an associated pleural effusion. Patients with fungal pneumonia or PCP may have inspiratory crackles; however, the lung exam in PCP may be completely normal. Hypoxemia—Oxygen saturation may be assessed rapidly with pulse oximetry; decreased saturations correlate with more severe disease. (A minor point regarding assessment of saturation is that patients who are using dapsone for PCP prophylaxis may rarely have falsely decreased pulse oximetry values secondary to methemoglobinemia; this will be readily apparent on arterial blood gas.) Extrapulmonary signs and symptoms may coincide with and suggest particular infections, though they are not present in every case of disseminated disease (see below for a review of extrapulmonary signs and symptoms). Typical presentations of respiratory pathogens Bacterial pneumonia is usually associated with a cough productive of purulent sputum, and possibly pleuritic chest pain. The duration of symptoms prior to presentation is usually 3 to 5 days. Physical exam findings may include rhonchi and bronchial breath sounds. In the case of associated pleural effusion, there will be dullness to percussion. PCP usually presents with a subacute course, with symptoms present for 2 to 4 weeks prior to presentation. Cough is nonproductive, patients are usually dyspneic, and fever may be present. The most common lung exam finding is inspiratory crackles, but the lung exam may be normal. Influenza pneumonia is most commonly encountered during the winter season in temperate climates. Patients typically present with a rapid onset of a “flu-like syndrome” consisting of dry cough, myalgias, headache, and high fever. Pneumonia, when it develops, follows the initial symptoms by 12 to 36 hours; either crackles or lobar findings may be present. New hemoptysis, pleuritic pain, or clinical worsening that develop during the course of influenza pneumonia may signify bacterial superinfection (particularly with Staphylococcus aureus). Cytomegalovirus (CMV) pneumonitis presents with a subacute course (2–4 week symptom duration) and complaints of nonproductive cough, dyspnea, and fever. The lung exam may demonstrate crackles, but may be normal. CMV pneumonitis is rarely found in isolation; it typically presents in the setting of disseminated disease, with the most common signs and symptoms related to retinitis, colitis, or esophagitis. Fungal pneumonia other than PCP (histoplasmosis, blastomycosis, coccidiomycosis, penicillosis) may present subacutely with cough and dyspnea or may follow a more fulminant course with symptoms similar to acute bacterial pneumonia. Pleuritic chest pain may be present. Lung exam may demonstrate crackles. Disease is frequently disseminated, and therefore may be accompanied by constitutional symptoms (weight loss, lethargy, fever) or symptoms in other organ systems, namely the central nervous system, skin, and liver. Invasive aspergillosis presents similarly to bacterial pneumonia, with high fever, cough, and dyspnea. Pleuritic chest pain may be present. Hemoptysis (occasionally massive) may be present, particularly with angioinvasive disease. Tuberculosis has protean manifestations and may present at any cluster of differentiation 4 (CD4) count. In patients with preserved CD4 counts, tuberculosis (TB) has a similar presentation to what would be expected in an immunocompetent person (fever, chills, night sweats, anorexia, productive cough, and occasional hemoptysis). Persons with advanced human immunodeficiency virus (HIV) are more likely to present with symptoms consistent with primary infection or with disseminated disease (lymphadenopathy, central nervous system involvement). Mycobacterium avium very rarely presents as isolated pulmonary infection in HIV-positive persons; however, M. kansasii may present with symptoms and signs very similar to TB. Extrapulmonary signs and symptoms of certain pathogens The extrapulmonary signs and symptoms of certain pathogens are summarized in Table I. \|Mycobacterium avium complex\|\|Constitutional symptoms (fever, weight loss, anorexia)Hepatosplenomegaly, lymphadenopathyAbdominal pain, chronic diarrheaCytopenias may be present secondary to bone marrow involvement\| \|Cryptococcosis\|\|Meningeal signsMultiple, polymorphic skin lesions (papules, pustules, nodules, plaques) found most commonly on the head and neck\| \|Histoplasmosis (endemic region: Mississippi, Ohio, and St Lawrence river valleys)\|\|Consitutional symptoms (fever, weight loss)Lymphadenopathy, hepatomegalyOral mucosal ulcersErythema nodosum or erythema multiformeMultiple skin lesions (pustules, crusted papules)Cytopenias may be present secondary to bone marrow involvement\| \|Coccidiomycosis (endemic region: southwestern United States, west Texas, southern California, northern Mexico)\|\|Constitutional symptoms (fever, weight loss)Meningeal signsLymphadenopathyPolymorphic skin lesions (papules, pustules, erythematous nodules, plaques)\| \|Blastomycosis (endemic region: Mississippi and Ohio river valleys, Great Lakes regions)\|\|Bone lesionsSkin lesions resembling neoplasmsGenitourinary involvement, particularly in males\| \|Toxoplasmosis\|\|Meningeal signsLocalizing neurologic exam\| \|Cytomegalovirus\|\|RetinitisColitis, esophagitis, or abdominal pain\| \|Aspergillosis\|\|Sinus symptoms and nasal dischargeMucosal hemorrhage (including the gastrointestinal tract) may complicate angioinvasive disease\| Which individuals are of greater risk of developing pneumonia? The risk of developing pneumonia from various organisms will vary with the CD4 count and with the use of appropriate prophylactic therapy and antiretroviral therapy (ART). Bacterial pneumonia may develop at any CD4 count, though the risk is lower when the patient is on combination ART and increases with degree of immune suppression. Similarly, as the CD4 cell count declines, the incidence of TB increases, but TB can present at any CD4 count. The diseases that are associated with various CD4 counts are summarized in Table II. \|ANY CD4 COUNT\|\|Mycobacterium tuberculosisBacterial pneumoniaSeasonal influenza\| \|<200\|\|Pneumocystis jirovecii pneumonia (particularly in absence of prophylaxis)Cryptococcal pneumoniaExtrapulmonary tuberculosisBlastomycosis\| \|<50–100\|\|Toxoplasmosis (particularly in absence of TMP-SMX prophylaxis)Histoplasmosis, coccidiomycosis, penicillosisCytomegalovirusMycobacterium avium, Mycobacterium kansasiiInvasive aspergillosisRhodococcus equi\| CD, cluster of differentiation; SMX, sulfamethoxazole; TMP, trimethoprim. A general principle is that prior infection with any opportunistic infection is associated with increased risk of recurrence. Injection drug use and cigarette smoking increase the risk of bacterial pneumonia. Emerging data suggest that risk of methicillin-resistant S. aureus (MRSA) colonization is increased in HIV-positive individuals, particularly intravenous drug users, men who have sex with men, and those with low CD4 cell counts. MRSA may be a pathogen in pneumonia. Chronic corticosteroid use increases the risk of PCP. Broad-spectrum antibiotics, steroids, and neutropenia predispose to disseminated aspergillosis. Marijuana smoking is also a risk factor for aspergillosis. Exposure to domestic cats or eating undercooked meats increases risk for toxoplasmosis. Prior Mycobacterium avium complex (MAC) colonization increases the risk of disseminated MAC. Residence or travel in certain endemic regions increases the risk for fungal pathogens, see Table III. \|Fungal pathogen\|\|Endemic area\| \|Histoplasma capsulatum\|\|Ohio, Mississippi, and St Lawrence river valleys\| \|Coccidioides immitis\|\|Southwestern United States, west Texas, southern California, northern Mexico\| \|Blastomyces dermatitidis\|\|Ohio and Mississippi river valleys and regions around the Great Lakes\| Beware: there are other diseases that can mimic pneumonia The differential diagnosis of respiratory symptoms in an HIV-positive patient includes several conditions that are seen in the general population, as follows: Pulmonary embolus (PE): The clinical manifestations of PE are highly nonspecific. The classic presentation includes acute onset of dyspnea, hypoxemia, pleuritic chest pain, and sinus tachycardia with or without hemoptysis. Pleural effusion is common. Massive embolus may precipitate acute hypoxemic respiratory failure or hemodynamic collapse. Risk factors include immobility, recent surgery, thrombophilia, or underlying malignancy. Cardiogenic pulmonary edema: Pulmonary edema presents with dyspnea and occasionally cough. The primary physical exam findings are pulmonary crackles in concert with other signs of heart failure, including lower extremity edema, an elevated jugular venous pressure, an S3 or S4, or a cardiac murmur. The chest radiograph may show ground glass opacities, Kerley B and A lines, and pleural effusions. Chronic obstructive pulmonary disease (COPD): HIV infection is an independent risk factor for COPD, and this association persists in the era of combination ART. A primary risk factor for COPD, as in the general population, is smoking. The prevalence of smoking is higher in the HIV-positive population than the general population. COPD exacerbation typically presents with cough and dyspnea. Physical exam findings include diminished air entry, prolonged expiratory phase, and expiratory wheeze. Chest radiograph may demonstrate increased lung volumes if emphysema is present, but may be normal. Pulmonary hypertension: The prevalence of pulmonary arterial hypertension (PAH) is increased in the HIV-positive population, particularly in those who acquired HIV through intravenous drug use. PAH may present at any degree of immunosuppression, although highly active ART may have a favorable effect. The classic presentation of PAH includes subacute onset of dyspnea with signs of right heart failure, in the absence of left-heart failure. Physical exam may demonstrate pedal edema, an elevated jugular venous pressure, a loud pulmonic S2, a tricuspid murmur, or a right-sided S3 or S4. The chest radiograph is usually clear, but the pulmonary arteries may be pronounced in advanced disease. While the diagnosis may be supported by elevated pulmonary arterial systolic pressure on echocardiogram, pulmonary hypertension can neither be confirmed or ruled out without a right heart catheterization. HIV is specifically associated with a number of noninfectious pulmonary conditions, as follows: Immune reconstitution inflammatory syndrome (see also the separate chapter on IRIS): IRIS is a syndrome that develops in the context of recovery of CD4 cell counts following the initiation of ART. IRIS is believed to represent an excessive inflammatory response to an antigen, most frequently a subclinical or recently treated opportunistic infection. It is most commonly seen in conjunction with such infections as TB, PCP, MAC, and Cryptococcus, but can also occur in association with malignancy (Kaposi sarcoma) or sarcoidosis. Manifestations include fevers, lymphadenopathy, meningeal symptoms, and pulmonary infiltrates. Most cases occur in the first few weeks after starting ART, though cases of IRIS have been reported up to 12 months following the initiation of therapy. Risk factors include low CD4 count prior to initiation of ART and opportunistic infection treatment proximal in time to ART initiation. There are unfortunately no definitive diagnostic criteria for IRIS, which remains a diagnosis of exclusion. A high index of suspicion is imperative in the appropriate clinical setting, as treatment with corticosteroids can be life-saving in severe cases. ART should be continued during IRIS if at all possible. Interstitial lung diseases (ILDs): Nonspecific interstitial pneumonitis (NSIP) is an inflammatory condition characterized by a lymphocytic infiltrate in the peribronchial and perivascular tissue and interlobular septae. It is relatively commonly found in HIV-positive persons. NSIP may be asymptomatic or may present with fever, cough, and dyspnea. It can radiographically resemble PCP, with presence of a reticular pattern and ground glass opacities seen on a chest computed tomography (CT) scan. Lymphocytic interstitial pneumonia (LIP) is a lymphoproliferative interstitial lung disease characterized by infiltration of the pulmonary parenchyma with lymphocytes and plasma cells. Its presentation is similar to NSIP, and radiographic findings are similar, though LIP more frequently presents with poorly defined small nodules in a peribronchial distribution. Both ILDs require tissue for diagnosis. Transbronchial diagnosis can frequently identify LIP, but surgical lung biopsy may be required. Kaposi sarcoma (KS) is an angioproliferative malignancy that was very common in HIV patients prior to ART. Although the incidence of KS has declined dramatically in the current era, it is still encountered clinically. The primary risk factor is low CD4 count (particular <50, very rare with CD4 >200), and incidence is higher in men who have sex with men. Although most patients with pulmonary KS have concomitant mucocutaneous lesions, up to 15% may have isolated pulmonary disease. Presenting symptoms of pulmonary KS include dyspnea, cough, and fever; hemoptysis may be present. Characteristic radiographic findings include bilateral central or perihilar nodular or linear opacities, thickening of bronchovascular bundles, or thickening of the interlobular septa. Pleural effusions are common. KS frequently occurs with concomitant opportunistic pulmonary infection (particularly PCP), for which a careful evaluation should be undertaken. Non-Hodgkin lymphoma (NHL) is also associated with HIV and has declined in the ART era. The median CD4 cell count at diagnosis is 100. Most patients have B-cell subtype and present with disseminated nodal and extranodal disease, of which the thorax is one affected site. Pulmonary symptoms include cough, dyspnea, and pleuritic chest pain. Thoracic imaging findings most commonly include single or multiple parenchymal nodules, lobar infiltrates, and pleural effusion. Recent studies have suggested an increase in the incidence of Hodgkin lymphoma in the HIV-positive population following the introduction of ART. Symptoms and radiographic findings are similar to NHL. Mediastinal lymphadenopathy is almost invariably present in pulmonary Hodgkin lymphoma. Substance abuse, which remains common in the HIV-positive population, may predispose the patient to a number of pulmonary conditions. A careful substance history may suggest one of the following conditions: Noncardiogenic pulmonary edema can be seen in association with almost any opiate and also occasionally with cocaine use. Typical features include acute dyspnea, pulmonary crackles on lung exam, and bilateral ground glass opacities. The presentation is identical to cardiogenic pulmonary edema, but occurs in the absence of cardiac dysfunction. Septic emboli secondary to right-sided endocarditis or peripheral thrombophlebitis may present with symptoms suspicious for pneumonia, including fever, dyspnea, and cough. Chest radiograph may show multiple cavities or peripheral shaggy nodules. Both embolic foci and pulmonary infarcts may be confused with lobar or patchy pneumonia on plain imaging of the chest. Sepsis at presentation, a new cardiac murmur, or evidence of peripheral thrombophlebitis should raise the index of suspicion. Crack lung is an acute alveolar syndrome characterized by eosinophilic inflammation that develops in the setting of inhaled cocaine use. The syndrome usually presents with hypoxemia, diffuse alveolar infiltrates, and pulmonary obstruction. Alveolar hemorrhage is common and may manifest as hemoptysis. Crack lung may rapidly progress to respiratory failure. Pulmonary talcosis, a granulomatous reaction to foreign bodies, may be seen when pills are pulverized and either inhaled or peripherally injected. The radiographic findings typically include a fine nodular infiltrate, but may also include centrilobular nodules or ground glass opacities. What laboratory studies should you order and what should you expect to find? Results consistent with the diagnosis Peripheral white blood cell count with differential: an acute infectious pneumonia will usually present with a leukocytosis (and occasionally leukopenia). Bacterial pnuemonia frequently causes a left shift, with or without bandemia. Profound bandemia should raise suspicion for concomitant bacteremia or pleural space infection. The total white blood cell count should be considered relative to the patient’s baseline; a “normal” count may be falsely reassuring in a patient who is chronically leukopenic. A recent CD4 count should be obtained and reviewed, as the range of the CD4 count is associated with a risk for certain pathogens. An arterial blood gas should be obtained in all patients who have evidence of hypoxemia on pulse oximetry, who have apparent respiratory distress, or have signs of sepsis. A reduction in the arterial pH (<7.35) correlates with increased disease severity. Severe hypoxemia or respiratory alkalosis or acidosis are signals of impending respiratory failure and should prompt consideration for intensive care unit admission. Results that confirm the diagnosis Sputum culture: A sputum Gram stain and culture should be obtained on all patients who can expectorate sputum (or obtained from deep respiratory suctioning through an endotracheal tube), ideally before the initiation of antibiotics. An important caution is that empiric therapy should not be narrowed on the basis of a sputum Gram stain alone. Sputum fungal cultures can be diagnostic of Cryptococcus or Coccidioides but are of less value for Histoplasma and Blastomyces, which are more reliably diagnosed by bronchoscopy. Induced sputum: Induced sputum samples should be obtained on any patient for whom PCP is being considered and should be sent to cytology for histologic or immunofluorescent staining. Induced sputum has a broad range of sensitivity (from <50 to >90%) for PCP identification, depending on the adequacy of the sample and the experience of the laboratory. Spontaneously expectorated sputum has a low sensitivity for organism identification and should not be used to rule out the diagnosis. Bronchoscopy: Many patients with HIV will require bronchoscopy to make a definitive diagnosis. It is prudent to perform bronchoscopy with bronchoalveolar lavage (BAL) in patients requiring intubation and mechanical ventilation. Bronchoscopic evaluation is also appropriate when empiric therapy is failing, when the diagnosis remains unknown despite less invasive testing, or when specific diagnoses are being sought. While BAL is sufficient for diagnosis of many infections, including PCP, transbronchial biopsy is necessary to confirm a diagnosis of invasive aspergillosis, CMV pneumonitis, or noninfectious disorders such as non-Hodgkin lymphoma or sarcoidosis. Sputum acid-fast bacillus (AFB) smears: AFB smears and culture in patients with suspected pulmonary TB should ideally be obtained on three separate days. If three smears are negative and the index of suspicion remains high (or if sputum cannot be obtained), bronchoscopy with BAL is required. Nucleic acid amplification tests (NAATs) are available for the identification of mycobacteria from respiratory samples. They are more sensitive than AFB staining. When positive, the result is more likely to represent TB than a nontuberculous mycobacterial infection. Influenza nasopharyngeal reverse transcription polymerase chain reaction swab is >90% sensitive for the diagnosis of influenza and should be obtained during flu season when the virus is suspected. Blood culture: Patients with bacterial pneumonia and a CD4 count of less than 200 frequently have bacteremia, and the organism may be identified on blood cultures. Mycobacterial blood cultures should be obtained in patients with suspected TB or MAC and a CD4 count of less than 200. All positive cultures should be submitted for susceptibility testing. Fungal blood cultures should be obtained in patients with suspected fungal disease. The slow growth of fungal cultures limits diagnostic utility, but susceptibility testing may be used to guide therapy. Urine antigens (pneumococcal antigen and Legionella antigen) may be obtained in patients with suspected community-acquired bacterial pneumonia. Serum cryptococcal antigen should be obtained when cryptococcosis is suspected, but patients with isolated pulmonary disease may have a negative result. Histoplasma polysaccharide antigen (HPA)testing of the blood or urine may diagnose disseminated histoplasmosis; HPA testing of BAL fluid can rapidly diagnose pulmonary histoplasmosis. Skin biopsy of new skin lesions can be diagnostic of disseminated fungal disease or extrapulmonary TB. Lymph node biopsy may be diagnostic of disseminated fungal or mycobacterial disease when widespread peripheral lymphadenopathy is present. Bone marrow biopsy may be diagnostic of disseminated fungal or mycobacterial disease when signs of marrow involvement (i.e. cytopenias) are present. Pleural fluid studies: HIV-positive patients presenting with pleural effusion should undergo diagnostic thoracentesis, as the incidence of complicated parapneumonic effusion and empyema in association with bacterial pneumonia is higher in the HIV-positive population than in the general population. Pleural fluid should be sent for Gram stain, culture, cell count and differential, lactate dehydrogenase (LDH), protein, glucose, and pH. The presence of a transudate (pleural fluid LDH/serum LDH <0.6, pleural fluid protein/serum protein <0.5, AND pleural fluid LDH <2/3 normal serum value) argues against infectious cause of pleural effusion. Pleural fluid pH less than 7.3 and glucose less than 60 suggest infectious process. Pleural fluid pH less than 7.2 suggests empyema. Purulent fluid = empyema. Lymphocyte predominance on cell count highly is suggestive of TB. TB may be supported by pleural fluid adenosine deaminase level greater than 40. Emerging diagnostic methods for PCP The following three tests are research tools and not widely available to clinicians: Polymerase chain reaction (PCR): Assays detect Pneumocystis DNA in BAL fluid, induced sputum, and oral washes and has potential advantages, i.e. rapid turn-around time and ability to reliably detect PCP using a noninvasive method (e.g. oral wash). Sensitivities are reported from 88 to 98%, however, specificities are lower as the assay detects Pneumocystis colonization in patients without clinical pneumonia. Therefore, clinical utility is limited. 1→3-β-D-glucan (β-glucan): β-glucan is a polysaccharide component of the walls of most fungi, including Pneumocystis, and has been studied as a possible noninvasive biomarker of PCP. In studies of HIV-infected patients, median β-glucan levels are significantly higher in patients with PCP than in non-PCP controls. Because β-glucan may also be increased in other fungal infections, and possibly in some bacterial infections, it is best used as an ancillary test. S-Adenosylmethionine: S-Adenosylmethionine is an intermediate molecule important for methylation reactions and polyamine synthesis. Pneumocystis cannot synthesize this molecule and therefore must scavenge it from the host. Investigators have hypothesized that decreased plasma levels of S-adenosylmethionine may correlate with Pneumocystis infection, but studies have shown varying results. Selected studies may be found in the references section. What imaging studies will be helpful in making or excluding the diagnosis of pneumonia? All patients with suspected pneumonia should have a posteroanterior and lateral chest X-ray (CXR) scan, if possible. Many patients will benefit from noncontrasted chest computed tomography (CT)—particularly if there is no focal abnormality on the CXR scan and the patient appears ill. CXR findings (CD4 >200): Alveolar pattern with acute onset of symptoms suggests bacterial pneumonia. Alveolar pattern with subacute onset of symptoms (>5 days) suggests atypical pneumonia, fungal pneumonia, or mycobacterial infection (including TB). Cavitary infiltrate is strongly suggestive of TB; patient should be placed in respiratory isolation (Figure 1). Nodules or masses are associated with an alternative diagnosis, such as malignancy, and a CT chest scan should be obtained. CXR findings (CD4 <200): A normal CXR does NOT rule out pulmonary disease, particularly PCP; high-resolution CT is warranted for further evaluation. Alveolar, reticular, or interstitial pattern with acute onset (3–5 days) suggests bacterial pneumonia. Alveolar pattern with subacute onset may suggest PCP, fungal pneumonia, TB, or a noninfectious process. Reticular or interstitial pattern with subacute onset may suggest PCP or other fungal pneumonia, particularly Cryptococcus. Nodular pattern may suggest fungal or bacterial pneumonia, TB (nodules <1cm), or neoplasm (nodules >1cm). Most common radiographic manifestations of specific opportunistic infections The most common radiographic patterns associated with opportunistic infections are described in Table IV, but it should be noted that the radiographic findings are protean; no particular pattern should be considered pathognomonic for any disease. \|Organism\|\|Radiographic manifestations (most common)\| \|Pneumocystis\|\|Bilateral, symmetric ground glass opacitiesReticulation and thickening of interlobular septae (“crazy paving”)Thin-walled cysts (pneumatoceles), present in 10 to 35% of cases (see Figure 2, Figure 3)\| \|Cryptococcus\|\|Diffuse bilateral infiltrateReticular or reticulonodular interstitial patternDiscrete nodules\| \|Histoplasma\|\|Diffuse small nodular or irregular linear opacitiesPatchy consolidation\| \|Coccidioides\|\|Focal or diffuse areas of alveolar consolidationNodules (may be cavitary or noncavitating), lymphadenopathy\| \|Blastomyces\|\|Focal consolidationDiffuse interstitial or miliary patternNodules, cavitation, lymphadenopathy\| \|Aspergillus (invasive)\|\|Cavitary lung disease, upper lobe predominantBilateral alveolar or interstitial opacities\| \|Cytomegalovirus\|\|Ground glass opacities\| What consult service or services would be helpful for making the diagnosis and assisting with treatment? An HIV specialist should be consulted for most patients with suspected opportunistic infections, particularly when tuberculosis is suspected, when disseminated or invasive fungal disease is being treated, when treatment failure is encountered, and/or when the initiation of combination ART is being entertained. A pulmonary consult is necessary if bronchoscopy is being considered. If you decide the patient has pneumonia, what therapies should you initiate immediately? As with pneumonia in the general population, the cornerstones of therapy include prompt initiation of appropriate antibiotics and assurance of adequate oxygen delivery. Oxygen should be administered to maintain saturations greater than 92%, and patients should be intubated if necessary to maintain either oxygenation or a patent airway. Empiric antimicrobial therapy should be selected based on the patient’s clinical history, with particular attention given to the recent CD4 count, if available. Patients with a presentation consistent with TB should be placed in respiratory isolation until the diagnosis can be definitively ruled out. If I am not sure what pathogen is causing the infection, what type of anti-infective should I order? The most likely diagnoses, as determined based on the constellation of history/physical finding, laboratory test results, and radiographic findings, should be covered empirically pending the results of confirmatory tests. Strong consideration should be given to the empiric coverage of TB in the appropriate clinical setting. Coverage for bacterial pneumonia and TB can be initiated simultaneously if strong clinical suspicion exists for both. Similarly, if PCP is suspected, therapy should be started empirically rather than waiting for confirmation of the organism. Empiric coverage of community-acquired bacterial pneumonia The most frequently encountered community-acquired bacterial pathogens in the HIV-positive population are the same as in the general population. Streptococcus pneumoniae is the most frequently identified organism, followed by Haemophilus influenzae. Similar to the general population, macrolide-resistant S. pneumoniae is increasingly encountered, and the frequency of macrolide-resistant organisms is higher in the HIV-positive population. Additionally, there have been several studies which have suggested that trimethoprim-sulfamethoxazole (TMP-SMX) prophylaxis may predispose to the development of TMP-SMX resistant S. pneumonia in the HIV-positive population. HIV patients are also at increased risk for community-acquired Pseudomonas aeruginosa and S. aureus, including MRSA. Pseudomonas is particularly common in patients with a CD4 count greater than 100, structural lung disease, neutropenia, or corticosteroid use. Additional risk factors for S. aureus/ MRSA include recent viral infection, history of injection drug use, or men who have sex with men. Cavitating disease or severe necrotizing pneumonia should raise the index of suspicious for Psuedomonas or Staphylococcus. Atypical pathogens, including Legionella pneumophila, Mycoplasma pneumoniae, and Chlamydophila, occur with the same frequency as in the general population. Two important caveats: Do not use an empiric macrolide alone in an HIV-positive patient, as there is increased risk of macrolide-resistant S. pneumoniae in the HIV population. Only use a fluoroquinolone alone if the presentation strongly suggests bacterial pneumonia, and not TB (to avoid inadvertant monotherapy in the case of tuberculosis). Treatment options are summarized in Table V. \|Treatment setting\|\|Preferred regimen\|\|Alternative regimen (allergy OR received β-lactam within last 90 days)\| \|Outpatient\|\|Oral β-lactam (amoxicillin 1g orally three times a day or amoxicillin-clavulanate 875mg orally twice a day) AND azithromycin 500mg orally daily\|\|Moxifloxacin* 400mg orally daily or levofloxacin* 750mg orally daily orgemifloxacin* 320 mg PO dailyMacrolide allergy: oral β-lactam AND doxycycline 100mg orally twice a day\| \|Inpatient (non-ICU)\|\|Intravenous β-lactam (ceftriaxone 1g IV every 24 hours, cefotaxime 1g IV every 8 hours, or ampicillin-sulbactam 3g IV every 6 hours) AND azithromycin 500mg IV every 24 hours for two doses, then 500mg orally every 24 hours\|\|Moxifloxacin* 40mg IV every 24 hours or levofloxacin* 750mg IV every 24 hoursMacrolide allergy: intravenous β-lactam AND doxycycline 100mg orally twice a day\| \|ICU\|\|Intravenous β-lactam (ceftriaxone 1g IV every 24 hours, cefotaxime 1g IV every 8 hours, or ampicillin-sulbactam 3g IV every 6 hours) AND azithromycin 500mg IV every 24 hours for two doses, then 500mg orally every 24 hours Intravenous β-lactam (ceftriaxone 1g IV every 24 hours, cefotaxime 1g IV every 8 hours, or ampicillin-sulbactam 3g IV every 6 hours) AND moxifloxacin 400mg IV every 24 hours or levofloxacin 750mg IV every 24 hours Consider therapy of Pseudomonas and Staphylococcus\|\|Aztreonam 2g IV every 8 hours AND intravenous fluoroquinolone (moxifloxacin 400mg IV very 24 hours or levofloxacin 750mg IV every 24 hours)\| \|Suspicion for Pseudomonas (for both non-ICU and ICU)\|\|Intravenous extended-spectrum β-lactam (piperacillin-tazobactam 4.5g IV every 8 hours, cefepime 1g IV every 8 hours, imipenem 500mg IV every 6 hours, or meropenem 1g IV every 8 hours) AND ciprofloxacin 400mg IV every 12 hours or levofloxacin 750 mg IV every 24 hoursAddition of aminoglycoside may be necessary, depending on the regional antibiogram\|\|Aztreonam 2g IV every 8 hours AND intravenous fluoroquinolone (moxifloxacin 400mg IV every 24 hours or levofloxacin 750mg IV every 24 hours)\| \|Suspicion for Staphylococcus aureus (for both non-ICU and ICU)\|\|Vancomycin 15–20mg/kg every 12 hours (in addition to standard regimen)\|\|Linezolid 600mg IV every 24 hours (in addition to standard regimen)\| DO NOT use quinolone alone if tuberculosis is in the differential. ICU, intensive care unit; IV, intravenously. Empiric coverage of health care associated pneumonia or hospital-acquired pneumonia When health care associated pneumonia (HCAP)/hospital-acquired pneumonia (HAP) is suspected, it should be treated with empiric broad-spectrum coverage because of the increased risk for extended spectrum pathogens. These include aerobic gram-negative rods (particularly resistant Pseudomonas, Acinetobacter, and extended-spectrum β-lactamase-producing Klebsiella) and MRSA. The factors that define HCAP and HAP are reviewed below. Treatment options are summarized in Table VI. \|If HCAP/HAP is suspected, antibiotic regimen should include:\| \|One of these\|\|Antipseudomonal cephalosporin: • cefepime 1–2g IV every 8 to 12 hours • ceftazidime 2g IV every 8 hours\| \|Carbapenem: • imipenem 500mg IV every 6 hours • meropenem 1g IV every 8 hours\| \|Piperacillin-tazobactam 4.5g IV every 6 hours\| \|AND one of these\|\|Antipseudomonal fluoroquinolone: • levofloxacin 750mg IV every 24 hours • ciprofloxacin 400mg IV every 8 hours\| \|Aminoglycoside: • gentamicin 7mg/kg IV every 24 hours (trough <1mcg/mL) • tobramycin 7mg/kg IV every 24 hours (trough <1mcg/mL) • amikacin 20mg/kg IV every 24 hours (trough <4–5mcg/mL)\| \|AND one of these\|\|Vancomycin 15mg/kg every 12 hours (trough 15–20mcg/mL)\| \|Linezolid 600mg IV every 12 hours\| The choice of antibiotic from each category will depend on patient factors (allergy or contraindication) and local resistance data/antibiogram. Therapy should be narrowed to monotherapy as soon as possible following the identification of the culprit organism. Risk factors for healthcare associated pneumonia or hospital-acquired pneumonia Antibiotic therapy in the preceding 90 days Hospitalization for 2 days or more in the preceding 90 days Residence in nursing home or extended care facility Chronic hemodialysis within the preceding 30 days Home wound care Home infusion therapy Family member/household contact with known multidrug-resistant pathogen Current hospitalization for 5 days or more Coverage of Pneumocystis pneumonia If PCP is suspected, therapy should be started empirically rather than waiting for confirmation of the organism. Diagnostic yield of bronchoscopy will not be reduced by immediately initiating therapy, as BAL will remain positive for Pneumocystis for at least several days after starting TMP-SMX. TMP-SMX is first-line therapy for PCP (intravenous, dosed as trimethoprim at 15–20mg/kg daily, divided every 6–8 hours) Patients with a sulfa allergy or other intolerance to TMP-SMX can be treated with intravenous pentamidine (3–4 mg/kg every 24 hours) If PCP is suspected and the patient is hypoxemic (A-a gradient greater than or equal to 35mm Hg or PaO2 less than or equal to 70mm Hg on room air), adjunctive corticosteroids should be started at the same time as Pneumocystis antimicrobials regardless of confirmation of diagnosis. Treatment options are summarized in Table VII. \|Pneumocystis pneumonia adjunctive corticosteroids\|\|Days 1–5\|\|Days 6–10\|\|Days 11–21\| \|Prednisone (if tolerating orally)\|\|40mg orally every 12 hours\|\|40mg orally every 24 hours\|\|20mg orally every 24 hours\| \|Methylprednisolone (if not tolerating orally or concern for malabsorption)\|\|30mg intravenously every 12 hours\|\|30mg intravenously every 24 hours\|\|15mg intravenously every 24 hours\| Coverage of tuberculosis Patients with suspected TB should receive standard four-drug therapy (isoniazid, rifampicin or rifabutin, pyrazinamide, and ethambutol) until the organism’s sensitivities are available. Pyridoxine should always be given in conjunction with isoniazid. If there is clinical suspicion for M. avium, clarithromycin or azithromycin may be added to the regimen. Therapy may be narrowed when culture results are available. As there may be interactions with antiretroviral therapy, consultation with an HIV specialist should be sought if patients are receiving TB therapy and ART simultaneously. Initial treatment for specific pathogens All treatment decisions should be made with attention to possible drug interactions (with ART regimens and other medications). Hepatic and renal impairment may require alteration of dosing regimen or drug choice. Many antimicrobials are contraindicated in pregnancy. The antimicrobials listed are considered initial therapy; TB and most fungi will require extended periods of “consolidation” therapy following initial treatment. Alternative regimens must be initiated in the event of either drug resistance or treatment failure. Treatment options are summarized in \|Pneumocystis\|\|Mild disease (room air PaO2 >70 and A-a gradient <35): oral TMP-SMX two double-strength tablets (160mg TMP/800mg SMX) every 8 hoursModerate to severe disease: intravenous TMP-SMX (15–20mg/kg TMP daily, divided every 6 to 8 hours)\|\|Mild disease: TMP 15–20mg/kg daily, divided every 6 to 8 hours with dapsone 100mg orally dailyClindamycin 1,800mg daily, divided every 6 to 8 hours AND primaquine 30mg orally dailyAtovaquone 750mg orally every 8 hoursModerate to severe disease: Pentamidine 3–4mg/kg intravenously every 24 hoursClindamycin 600–900mg intravenously every 8 hours AND primaquine 30mg orally daily\| \|Mycobacterium avium\|\|Clarithromycin 500mg orally twice a day AND ethambutol 15mg/kg/day*the addition of a third (rifabutin 300–600mg orally daily) or fourth (streptomycin 15mg/kg intramuscularly three times weekly or amikacin 15mg/kg three times weekly) agent may be considered in cases with advanced immunosuppression and high mycobacterial loads\|\|Azithromycin 500mg orally daily may be used in place of clarithromycin\| \|Cytomegalovirus\|\|Ganciclovir 5mg/kg intravenously every 12 hours\|\|Foscarnet 90mg/kg intravenously every 12 hours\| \|Influenza\|\|Oseltamavir 75mg orally twice a day\|\|Zanamavir 10mg inhaled twice daily (not for use in patients with underlying structural lung disease or asthma)\| \|Toxoplasma\|\|Pyrimethamine 200mg loading then 50mg orally every 24 hours/folinic acid 20mg orally every 24 hours AND sulfadiazine 1.5g orally every 6 hours\|\|Clindamycin 1,200mg (orally or intravenously) every 6 hours may be given in place of sulfadiazine\| \|Histoplasma\|\|Amphotericin B (liposomal) 3–5mg/kg intravenously every 24 hours if disseminatedItraconazole 300mg orally twice a day for 3 days, then 200mg orally twice a day may be used when lung is only site of infection\| \|Coccidioides\|\|Amphotericin B (deoxycholate) 1mg/kg intravenously daily if disseminatedfluconazole 400mg orally daily or itraconazole 200mg orally twice a day may be used when lung is only site of infection\| \|Cryptococcus\|\|Amphotericin B (deoxycholate 0.7mg intravenously every 24 hours or liposomal 3–5mg/kg every 24 hours) AND flucytosine 100mg/kg daily in divided dosesFluconazole 400mg orally daily plus flucytosine if lung is confirmed to be the only site of infection\| \|Aspergillus (invasive)\|\|Voriconazole 6mg/kg intravenousy every 12 hours for two doses, then 200mg orally twice a day\|\|Amphotericin B deoxycholate 1mg/kg daily or caspofungin 50mg daily\| SMX, sulfamethoxazole; TMP, trimethoprim. What other therapies are helpful for reducing complications? Adjunctive steroid therapy is indicated in the treatment of PCP with hypoxemia (room air PaO2 <70 or A-a gradient >35); steroids should be initiated in conjunction with antimicrobials. Pneumonia from any source may progress to respiratory failure, and in severe cases, development of acute lung injury (ALI) or the acute respiratory distress syndrome (ARDS). When ALI or ARDS is suspected (PaO2/FiO2 ratio <300 or <200, respectively, diffuse bilateral airspace disease, no evidence of elevated left ventricular filling pressures), lung protective ventilation strategies should be followed according to ARDS Network guidelines. Control of plateau pressures is particularly important in patients with PCP and pneumatoceles, who are at increased risk for iatrogenic pneumothorax. When complicated parapneumonic effusion or empyema is confirmed, the collection should be drained with an appropriately sized pleural drainage catheter or chest tube. Intravenous fluids should be used with caution in patients with PCP, in whom alveolar transudate should be minimized. The initiation or continuation of combination ART during hospitalization in patients who are diagnosed with opportunistic infections has been found in several studies to improve survival. Starting ART in critically ill patients with opportunistic pneumonias remains controversial and is guided by expert opinion. A recent retrospective study (Croda et al) has suggested a potential benefit of ART, but prospective data are lacking. What complications could arise as a consequence of pneumonia? Respiratory failure, including ARDS, is a potential complication of any pneumonia in an HIV-positive patient. Pneumothorax is a potential complication of PCP due to the frequent presence of pneumatoceles. Pleural effusion or empyema may develop in conjunction with bacterial, fungal, or mycobacterial pneumonia. Sepsis may complicate pneumonia in the HIV-positive patient, particularly when there is associated bacteremia or disseminated disease. What should you tell the family about the patient's prognosis? Bacterial pneumonia is associated with increased mortality in HIV-positive patients, with case fatality rates reported as high as 20%. Radiographic progression of disease, a low baseline CD4 count, and presence of shock are independent predictors of mortality. Mortality in PCP has been reported from 15 to 50% in the current era, with risk factors for higher mortality including poor baseline functional and nutritional status, high A-a gradient at presentation, pneumothorax, and requirement for mechanical ventilation. Fungal disease localized to the lung has a relatively good prognosis, but disseminated disease has a very high mortality rate. Invasive aspergillosis in HIV also has a poor prognosis, with at least 50% mortality. What if the patient is not improving? There are a number of reasons why a patient may worsen or fail to improve on initial therapy. Alternative diagnosis: Is there a chance your patient does not have pneumonia? The mimics of pneumonia should be reviewed in light of the patient’s clinical scenario, and possible differential diagnoses (as covered above) should be explored. Inadequate treatment: Is there a chance the culprit organism is not covered? A patient who was initially treated with narrow therapy for community-acquired bacterial pneumonia may fail to respond because they are harboring community-acquired Pseudomonas or MRSA. TB should be reconsidered in patients who have failed to respond to adequate therapy for suspected bacterial pneumonia. Concomitant infection: Is there a coinfection which has not been treated? For instance, patients with PCP are frequently coinfected with another organism (20% prevalence), including bacteria, CMV, and TB. New infection: Has your patient contracted a hospital-acquired pathogen? Patients who have been hospitalized for 5 days or greater are at risk for hospital-acquired organisms, which have broad resistance patterns and may not be covered by initial empiric therapy for community-acquired pneumonia. Treatment failure: Treatment failure is a consideration with a number of conditions, including PCP and TB. In the case of PCP, treatment failure should not be considered until at least 8 days into the antibiotic course, as clinical response may not be apparent until days 4 to 8. If the patient has worsening respiratory function after that time, the drug class may be changed to pentamidine or clindamycin/primaquine. Coinfection and new infection must also be investigated, ideally via bronchoscopy. Bronchoscopy will remain positive for Pneumocystis throughout treatment and detection on BAL does not indicate treatment failure. In the case of TB, treatment failure is secondary to drug resistance and should be managed in conjunction with an HIV specialist. IRIS: Did your patient recently initiate ART? As previously stated, there are no definitive diagnostic criteria for IRIS, which is therefore a diagnosis of exclusion. If other processes have been ruled out, a trial of corticosteroids may be warranted. How can pneumonia be prevented in HIV-positive patients? The 23-valent polysacharrhide pneumococcal vaccine decreases the risk of pneumococcal pneumonia, and also reduces the risk of pneumococcal bacteremia in HIV patients who do develop pneumonia. All patients with a CD4 count greater than 200 should be given the vaccine, and it should be repeated every 5 years. Patients with a CD4 count less than 200 may have a less robust vaccine response secondary to immunosuppression, but the vaccine can still be offered. Revaccination in this group may be offered if the CD4 count rises above 200 after initiation of ART. Pneumococcal conjugate vaccines, including Prevnar-7 and Prevnar-13, are also available, but the use in HIV-infected adults is controversial. Ongoing studies may be useful for informing future recommendations. All patients with HIV should receive the seasonal influenza vaccine on a yearly basis. Live attenuated influenza vaccine is contraindicated in HIV-infected persons. Primary PCP prophylaxis should be initiated in any patient with a CD4 count less than 200 or a history of oral thrush (regardless of CD4 count). Prophylaxis may also be considered in patients with a CD4 cell percentage of less than 14% or with a history of any acquired immunodeficiency syndrome-defining illness. The preferred prophylactic agent is TMP-SMX, one double strength tablet daily. TMP-SMX has the additional benefit of offering protection from Toxoplasma. Patients with a CD4 count less than 50 should receive primary prophylaxis for Mycobacterium avium. Preferred prophylactic agents are azithromycin or clarithromycin. Patients with latent TB should be treated with isoniazid to prevent progression to active TB. Prophylaxis for fungi or CMV are not routinely recommended. Respiratory isolation of any patient with suspected TB should be performed to prevent spread both to staff and to other patients. Although there is some suggestion of person-to-person transmission of PCP, current guidelines do not recommend placing patients in isolation. WHAT'S THE EVIDENCE for specific management and treatment recommendations? Crothers, K, Morris, A, Huang, L, Mason, R, Broaddus, VC, Martin, TR, King, TE, Shraufnagel, DE, Murray, JF, Nadel, JA. “Pulmonary complications of human immunodeficiency virus infection.”. Murray and Nadel's textbook of respiratory medicine. 2010. pp. 1914-49. Kaplan, JE, Benson, C, Holmes, KH, Brooks, JT, Pau, A, Masur, H. “Guidelines for prevention and treatment of opportunistic infections in HIV-infected adults and adolescents: recommendations from CDC, the National Institutes of Health, and the HIV Medicine Association of the Infectious Diseases Society of America”. MMWR Recomm Rep. vol. 58. 2009. pp. 1-207. Huang, L, Crothers, K. “HIV-associated opportunistic pneumonias”. Respirology. vol. 14. 2009. pp. 474-85. Mandell, LA, Wunderink, RG, Anzueto, A. “Infectious Diseases Society of America/American Thoracic Society consensus guidelines on the management of community-acquired pneumonia in adults”. Clin Infect Dis. vol. 44. 2007. pp. S27-72. “American Thoracic Society, Infectious Diseases Society of America. Guidelines for the management of adults with hospital-acquired, ventilator-associated, and health care-associated pneumonia”. Am J Respir Crit Care Med. vol. 171. 2005. pp. 388-416. “Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome”. N Engl J Med. vol. 342. 2000. pp. 1301-8. Huang, L, Schnapp, LM, Gruden, JF, Hopewell, PC, Stansell, JD. “Presentation of AIDS-related pulmonary Kaposi's sarcoma diagnosed by bronchoscopy”. Am J Respir Crit Care Med. vol. 153. 1996. pp. 1385-90. Croda, J, Croda, MG, Neves, A, De Sousa dos Santos, S. “Benefit of antiretroviral therapy on survival of human immunodeficiency virus-infected patients admitted to an intensive care unit”. Crit Care Med. vol. 37. 2009. pp. 1605-11. De Boer, MG, Gelinck, LB, van Zelst, BD. “β-D-glucan and S-adenosylmethionine serum levels for the diagnosis of pneumonia in HIV-negative patients: a prospective study”. J Infect. vol. 62. 2011. pp. 93-100. Held, J, Koch, M, Reischl, U, Danner, T, Serr, A. “Serum (1–>3)-β-D-glucan measurement as an early indicator of pneumonia and evaluation of its prognostic value”. Clin Microbiol Infect. vol. 17. 2010. pp. 595-602. Larsen, HH, Huang, L, Kovacs, JA. “A prospective, blinded study of quantitative touch-down polymerase chain reaction using oral-wash samples for diagnosis of pneumonia in HIV-infected patients”. J Inf Dis. vol. 189. 2004. pp. 1679-83. Janda, S, Quon, BS, Swiston, J. “HIV and pulmonary arterial hypertension: a systematic review”. HIV Med.. vol. 11. 2010. pp. 620-34. 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. - OVERVIEW: What every practitioner needs to know - Are you sure your patient with human immunodeficiency virus has pneumonia? What should you expect to find? - Which individuals are of greater risk of developing pneumonia? - Beware: there are other diseases that can mimic pneumonia - What laboratory studies should you order and what should you expect to find? - What imaging studies will be helpful in making or excluding the diagnosis of pneumonia? - What consult service or services would be helpful for making the diagnosis and assisting with treatment? - If I am not sure what pathogen is causing the infection, what type of anti-infective should I order? - Empiric coverage of community-acquired bacterial pneumonia - Empiric coverage of health care associated pneumonia or hospital-acquired pneumonia - Risk factors for healthcare associated pneumonia or hospital-acquired pneumonia - Coverage of Pneumocystis pneumonia - Coverage of tuberculosis - Initial treatment for specific pathogens - What other therapies are helpful for reducing complications? - What complications could arise as a consequence of pneumonia? - What should you tell the family about the patient's prognosis? - What if the patient is not improving? - How can pneumonia be prevented in HIV-positive patients? - WHAT'S THE EVIDENCE for specific management and treatment recommendations? 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General Information And Response Options General Information On COVID-19 COVID-19 is the infectious disease caused by the most recently discovered coronavirus. Coronaviruses are a large family of viruses which may cause illness in animals or humans. In humans, several coronaviruses are known to cause respiratory infections ranging from the common cold to more severe diseases such as Middle East Respiratory Syndrome (MERS) and Severe Acute Respiratory Syndrome (SARS). The most recently discovered coronavirus causes COVID-19. This new virus was unknown before the outbreak was discovered in Wuhan, China, in December 2019. The most common symptoms of COVID-19 are: ● Most common symptoms: ● fever. ● dry cough. ● tiredness. Less common symptoms: ● aches and pains. ● sore throat. ● diarrhoea. ● conjunctivitis. ● headache. ● loss of taste or smell. ● a rash on skin, or discolouration of fingers or toes. ● difficulty breathing or shortness of breath. ● chest pain or pressure. ● loss of speech or movement These symptoms are mild in 40% of cases, and begin gradually. Some people become infected but don’t develop any symptoms and don't feel unwell. Most people (about 80%) recover from the disease without needing special treatment. Older people, and those with underlying medical problems like cardiovascular disease, diabetes, chronic respiratory disease, and cancer are more likely to develop serious illness. People can catch COVID-19 from others who have the virus. The disease can spread from person to person through small droplets from the nose or mouth which are spread when a person with COVID-19 coughs or exhales. These droplets land on objects and surfaces around the person. Other people then catch COVID-19 by touching these objects or surfaces, then touching their eyes, nose or mouth. People can also catch COVID-19 if they breathe in droplets from a person with COVID-19 who coughs out or exhales droplets. This is why it is important to stay 2 metres (6 feet) away from a person who is sick. It is important to mention some studies have shown that, in a poor ventilated area, the droplets can remain suspended in air for up to 3 hours. The most basic prevention measures in order to remain protected from COVID-19 are as follows: 1. Regularly and thoroughly clean your hands with an alcohol-based hand rub or wash them with soap and water. This will kill viruses that may be on your hands. 2. Maintain 2 metres (6 feet) distance between yourself and anyone. 3. Avoid touching eyes, nose and mouth, as once contaminated with the virus, hands can transfer the virus to your eyes, nose or mouth. From there, the virus can enter your body and can make you sick. 4. Cover your mouth and nose with your bent elbow or tissue when you cough or sneeze. Then dispose of the used tissue immediately. 5. Wearing of masks in public in line with WHO/MOH recommendation. 6. Stay home if you feel unwell. If you have a fever and cough or difficulty breathing, seek medical attention in accordance with your local health authority’s advice. Follow the directions of your local health authority. Calling in advance will allow your health care provider to quickly direct you to the right health or testing facility. This will also protect you and help prevent spread of viruses and other infections. 7. Regularly keep informed about the outbreak as strategies and response activities will constantly improve as new information becomes available about the disease. It is important to follow trusted and evidence-based sources such as the World Health Organization and your local health authorities for the latest guidance and updates on health information. Handwashing with soap is one of the key measures in breaking the transmission. Our hands have natural moisture and oils on them. We know germs can stay on the skin for a long time. ● What does soap do? The lather from the soap attaches to dirt and natural oils on the hands. When you wash the soap off the hands with water it picks up all of the dirt and oil and germs and washes them away. Soap deactivates the germs by breaking down the outside fatty cover of the virus. When soap comes in contact with the germ it basically dissolves it hence destroying it. This is why soap is such a good effective intervention to fight against the Coronavirus disease. ● What else is important when asking people to wash their hands with soap? Germs can live in the web between the fingers and in cracks of the skin so it is very important to lather the soap and wash hands for a minimum of 20 seconds ensuring each finger, thumb, palm and back of hand and wrist are washed so the germs can be destroyed. ● Can ash or sand be used if I don’t have soap? Ash and sand work the same way primarily through friction. We are not sure if it is as effective as soap but the friction does help remove the germs so it is better than just water alone. ● Is alcohol based hand rub better than soap? Soap and water remain the recommended method to remove the germs from hands. Alcohol hand rub is another effective method but it is more expensive than soap and most likely less available to many people. For the alcohol hand rub to be effective it must have at least 60% alcohol in it. ● Should we use chlorine to wash our hands? Soap and water is a highly effective way to kill the virus. If there is no access to soap or it has a very limited supply then chlorine can be considered as an option. The strength used should be 0.05%. Contact your WASH colleague to get further instructions on the preparation. For more information: https://resources.hygienehub.info/en/ There are two broad approaches to epidemic response. There is not yet evidence or experience for either strategy to fully understand the long-term viability, limitations or future impacts of each. Both strategies are expected to be needed, in various formulations, for a minimum of 12-18 months. First, a suppression strategy aims to eliminate human-to-human transmission. This approach requires intensive and sustained interventions, with a focus on identifying, isolating and treating all cases, and identifying all contacts or people at risk of exposure. These public health measures need to be maintained for as long as the virus is circulating, or until a vaccine is available. While maintained, they can have significant social and economic impacts, and require a significant scale-up of health systems capacity. Second, a mitigation approach seeks to slow but not stop the spread of the virus, with the goal of reducing the demand on health systems and protecting the most vulnerable people (e.g. the elderly and those with underlying diseases). The disruption to health, social and economic systems over this time period is likely to be profound. Mitigation strategies are still expected to result in significant death and health system impacts. The ‘mitigated’ epidemic can still be expected to overwhelm the health system with many times more cases than can be properly cared for, resulting in many avoidable deaths. However, this approach may be the only option in countries with limited health systems capacity. Broadly speaking, there are three ways this pandemic could turn out from an epidemiological perspective. First, efforts to suppress viral transmission could succeed and we could see a complete interruption of human-to-human transmission, meaning that the pandemic ends and the virus is eliminated from the human population. As more and more people are infected, this scenario is unlikely. Second, epidemic control efforts may result in recurring epidemic waves, large or small, where communities or entire regions experience periods of relative normalcy interrupted by waves of more cases. Finally, we could see a continuous outbreak with low-level transmission that results in a sustained but lower-level burden on health systems. The progression of this outbreak depends on the success of suppression and control measures, and on a number of factors related to human immunity, which we don’t yet know enough about to make good predictions. For the first two scenarios, it is likely that efforts to control and respond to the pandemic will last a minimum of 18 months, and potentially several years. For scenario planning assessing the possible humanitarian impact of this pandemic, please see the ACAPS COVID-19 scenarios. National Societies’ health response to COVID-19 National Societies’ and volunteers’ roles will change through the progression of the COVID-19 pandemic in their respective countries/communities, based on which public health, clinical and health systems activities are likely to be most impactful. National Societies must work with their public authorities so that both the government and NS understand their respective mandates and the roles the NS is technically and organisationally able to take on. NS must be prepared to change their operations over time as the outbreak progresses and appropriate outbreak response measures change. Below are some evidenceinformed health activities that National Societies can support. These activities may be complemented by other auxiliary roles particular to each National Society’s context. Throughout, it is important that National Societies identify their high-risk activities and plan to adapt them to ensure they maintain lifesaving humanitarian services throughout the epidemic. National Societies can support critical public health, clinical, and health systems activities during each phase, whether suppression or mitigation approaches are chosen, and support vulnerable communities by helping to mitigate the impact the outbreak has on health, social and economic systems. Support countries and at-risk communities to prepare to respond to first cases (detect, isolate, and treat, trace contacts and screen, as appropriate); promote effective behaviour change and hygiene practices, engage communities and address misinformation and rumours; adapt community and clinical programming to be ready to effectively respond and limit risks to personnel; assess NS auxiliary roles and institutional capacities for public health and clinical responses. Institutional readiness and coordination ● Make sure your NS, branch or local volunteers are ready (business continuity planning; identification of programming changes needed for appropriate outbreak response; institutional linkages to Ministry of Health, Emergency Operations Centres, WHO country offices, Humanitarian Country Teams, and other relevant stakeholders and partners) ● Identify the auxiliary role of your NS and prepare to carry out your designated outbreak response activities (e.g. support to quarantine, contact tracing, point of entry screening); Community-based actions to support health ● Identify and begin to access the most vulnerable communities and systems; assess barriers to adapting behaviours and preventing transmission; ● Enhance NS staff and volunteer capacity to prepare and respond to epidemics and enable community-led planning and action (e.g. community-based health and WASH activities, adaptation of existing community-facing programming); ● Begin to carry out risk communication, community engagement, and health promotion, in coordination with key stakeholders; Prepare for clinical actions ● Assess the overall clinical capacity of the health system to respond; clearly identify and define the role of the NS in clinical service provision, including level of care (primary vs prehospital vs tertiary, community vs inpatient) to be provided and the NS’ ability to increase or adapt to increased demand (see section on scaling up clinical activities). ● Preposition appropriate supplies and PPE for healthcare activities, adapt facilities to allow for isolation activities and correct patient flow, identify clinical referral pathways, train clinical staff on latest COVID-19 clinical guidelines and on IPC policies. The goal is to prevent large-scale outbreak by helping to suppress transmission of the virus once cases or clusters have been detected. Support public confidence in the health system and outbreak response measures, and carry out effective community engagement, risk communication, behaviour change and hygiene promotion approaches. Support clinical and prehospital and home based care (s per mandated role and where the NS has the capacity to do so in a safe and ethical manner, always ensuring a do-noharm approach;) for COVID-19 cases and maintain access to existing essential health services for the broader population, if clinical service provision is an area where your National Society works. Support the most vulnerable communities affected by the health, social and economic impacts of the outbreak and measures to suppress it. Community-based actions for outbreak prevention and response. ● Risk communication, community engagement, and health promotion, including promoting acceptance of public health measures, social cohesion, and communicating about available services; ● Targeted community health, PSS, and WASH programming adapted as “surge” response; ● Screening, contact tracing, quarantine, and other services in support of government activities to suppress transmission; ● Activate community-based surveillance where such systems are in place, and include pneumonia as a health risk; ● Improve community-level prevention, detection of serious cases, and referral through existing community-based health and surveillance activities in the immediate area experiencing transmission; ● Psychosocial support to affected populations, including responders; ● Emergency social services for quarantined or movement-restricted communities, or related to systems failures; Clinical actions to support outbreak response: ● Clinical, paramedical or homecare service provision to provide specific COVID-19 treatment as per mandate and in line with pre-existing clinical capacity, always ensuring a do-no-harm approach; ● Ongoing refresher training for healthcare workers on clinical and IPC protocols; Actions to support health systems and mitigate the indirect health impact on the most vulnerable: ● Support health system and social services (e.g. scale up services required, direct service provision as appropriate); ● Provide psychosocial support to reduce the mental health and social wellbeing impacts of the outbreak; ● Support health services for the general population to make sure that people still have access to life-saving health services; ● Maintain or increase existing non-COVID clinical, paramedical or homecare service provision to supplement health system in cases where capacity is stretched; The goal now is to slow large-scale outbreak by helping to suppress transmission of the virus. Support public confidence in the health system and outbreak response measures, and carry out effective community engagement, risk communication, behaviour change and hygiene promotion approaches. Support clinical and prehospital care for COVID-19 cases where appropriate. Support access to and availability of existing essential health services for the broader population. Task shift and support community-level access to essential health and care services to ease the burden on the health system and to reduce indirect morbidity and mortality. Support the most vulnerable communities affected by the health, social and economic impacts of the outbreak and measures to suppress it. Community-based actions for outbreak prevention and response ● Intensify risk communication and community engagement, with focus on strengthening community-led solutions to prevent and control the outbreak (closely linking to health and PSS approaches); ● Motivate acceptance and adherence to community-based protection and home care for COVID19 and other diseases (based on MOH guidance); ● Provide psychosocial support to reduce the mental health and social wellbeing impacts of the outbreak ● Community health programming (detection, contact tracing and referral, health education and hygiene promotion, support for home-based care as context and mandate dictate), scaled and adapted for medium- to long-term COVID-19 response; ● Targeted interventions within the community for the vulnerable and susceptible individuals (if this is the mandate and the national society has resources to do this) ● Inform community and religious leaders and family members about behaviour changes needed to bury people who have died of COVID-19 (note that “Safe and dignified burial” as for Ebola is not required). Clinical actions to support outbreak response ● Clinical, paramedical or homecare service provision to provide specific COVID-19 treatment as per mandated role and where the NS has the capacity to do so in a safe and ethical manner, always ensuring a do-no-harm approach; ● Support caregiving to those ill with COVID-19 (clinical, paramedical, as context and mandate dictate), and reduce risk of healthcare worker infection and nosocomial transmission in health facilities. Actions to support health systems and mitigate social and economic impact on the most vulnerable ● Task shift to increase the reach of or access to non-COVID health services; ● Further scale-up of non-COVID related clinical, paramedical, or homecare service provision to supplement health system in cases where capacity is exceeded and where NS has technical capacity to safely support; ● Infection prevention and control and other health-system interventions to improve care or access to care ● Cash and/or livelihoods support to address immediate needs/restore income of vulnerable households in communities affected by the pandemic and/or measures taken to contain it ● Promote social cohesion and a culture of non-violence, particularly in communities facing the heaviest burdens; Public health measures to prevent and suppress transmission Community-based surveillance (CBS) Community-based surveillance (CBS) is the systematic detection and reporting of events of public health significance within a community by community members (WHO, 2018). CBS is used to alert about health risks/events related to epidemic- prone diseases. With a large RCRC volunteer network in communities around the world, the RCRC movement is well placed for CBS, and providing volunteers with the tools for CBS in their communities allows for early detection of health risks and can trigger an early response in communities to stop further transmission. CBS can also be used in ongoing outbreaks to monitor transmission as the outbreak evolves to guide response activities. CBS can be useful in contexts where the existing surveillance system is not able to capture health risks/events from the community level that can lead to outbreaks. This is often due to lack of access to health care facilities, which is where traditional surveillance systems are often based. These gaps can also be due to conflicts, emergency situations such as natural disasters, large outbreaks, lack of resources or other challenges. CBS can help fill the surveillance gap by getting information from the community level to the level necessary to trigger an early response. Implementing CBS should be done in collaboration with the national authorities, and as an addition to complement existing surveillance systems. CBS provides information about signs and symptoms of health risks/events which can indicate disease, but it does not report on confirmed cases and can thus not replace a regular surveillance system for diseases. The decision to implement CBS, and which health risks/events to do CBS for, is decided together with the community. The CBS volunteers, who are most often existing National Society volunteers, should also be identified by the community. CBS can be done in different ways using different tools, but the core purpose is to give community members the opportunity to give information about health risks or events directly from their community to trigger responses when needed. This is often done by having CBS volunteers as focal points for the community members. They both report to their NS on health risks/events, while providing a communitylevel response to the health risks. For CBS to be implemented it is necessary to have a mechanism in place to ensure there is a response and investigation to the reports coming from the community. The party responsible for the response can vary between contexts; e.g in most contexts the MoH is responsible for public health, surveillance activities and medical activities, but in some contexts RCRC movement can take responsibility for some of the response. In other contexts different parties may be responsible for different areas of a response. The community-level response carried out by volunteers are based on CBHFA and ECV. CBS reporting can be done in different ways including the use of paper forms, SMS and or mobile apps. Mobile technology has been used widely in recent years to ensure data is shared in real-time. A digital platform called Nyss, has been developed by and for the RCRC Movement. Nyss works to directly aggregate SMS reports from volunteers, as well as automatically analyse and visualise data and trigger alerts to supervisors and share appropriate data with health authorities to facilitate a response. The RCRC CBS technical working group has identified a global list of health risks/events where CBS can add value. These are based on signs, symptoms and events which can indicate that there is a possible transmittable disease in the community. The volunteers are not medically trained and thus do not report on disease, but on the signs,symptoms and events they are trained on. More information about how CBS works and the Nyss platform can be found on the CBS website: https://www.cbsrc.org - CBS is systematic detection and reporting of an observed health risk/event in the community to ensure a rapid response. It is often used to be able to do immediate reporting for early warning. - Contact tracing is identification and follow up of contacts when a confirmed case is identified - Surveys are structured information gathering from a sample of people, often done once, or on a regular basis to obtain specific information More about different types of surveillance/ information gathering during COVID-19, can be found on the Health Help Desk: The RCRC CBS approach is designed in such a way that personal information is not sent out from the community. The volunteers will know who they are sending the report about in order to ensure that response and help can reach the right person if necessary. However, the information they send by SMS or on paper is only which health risk/event they have seen, the sex and age group of the person. This is all the information needed to be able to determine if, and what, response is needed. All volunteers have RCRC supervisors who are the link between the community and the authorities, who support the response. It is essential to have trust within the community and agreement among community members about how CBS should be conducted in their community due to the fact that a CBS alert may require a response/ follow-up from local health authorities. For the CBS platform Nyss, the volunteers are registered with their personal information, but they are informed about what this means and must give consent. Personal identifying information is only visible for the supervisors and the National Society staff involved in CBS. Nyss has defined password protected user access. A description about the data protection measures in place for the CBS platform Nyss can be found in the resource page of the CBS website: https://www.cbsrc.org/resources CBS is already being implemented as part of National Societies’ response to COVID-19 in multiple locations, including using the Nyss platform in Somaliland, Somalia and Senegal. In both places the health risk/event “cough and difficulty breathing” has been added, leading to reports of people with signs and symptoms matching the local community definition of this health risk. In Somaliland, one of the first cases of confirmed COVID-19 was identified through SRCS volunteers and CBS. With the strong network of volunteers in communities around the world, RCRC has a great opportunity to inform and advise community members about necessary measures to avoid transmission, and is well placed to transfer information quickly from the community level at an early stage of an outbreak in areas where getting this information is a challenge. As with many other health risks and events in CBS, this can lead to early detection of COVID-19 and limitation of transmission. Additional guidance on using CBS for COVID-19 can be found here. Several considerations need to be taken before implementing CBS for COVID-19. These are captured in questions below. If you have an ongoing CBS project, it is possible to add reporting on symptoms of COVID-19. In most CBS implementations, the option to report “unusual events” or “cluster of illness or deaths” is added to allow for reporting of health risks/events that are not included in your CBS project. This means that one might be able to pick up on potential COVID-19, without even adding an extra health risk. The National Societies who have added COVID-19 to their CBS as of today have added the health risk/event: “cough and difficulty breathing” with the suggested community definition: “fever with dry cough or difficulty breathing” from the CBS health risk/event list, as these are symptoms that align with symptoms in the case definition for COVID-19 provided by WHO. When a health risk or event is added to your CBS, it is important that the volunteers understand the signs and symptoms they are looking for, what information to give the community members, what community response actions they should do and what protection measures they need to follow. This is facilitated by adding the extra health risk/event for COVID-19 and host trainings specifically for this. If COVID-19 is added to existing CBS projects, the volunteers need to be trained in a safe way which is in coherence with national guidelines for COVID-19. Additional suggestions can be found here. The RCRC movement has developed several tools to be able to implement CBS, including a CBS assessment tool, protocol template and training materials which are available for the Movement wide through the resource page of the CBS website: https://www.cbsrc.org/resources. The following tools are also available directly through the IFRC website in English, French, Spanish and Arabic: ● Community-based surveillance (CBS) Assessment Tool ● Community-based surveillance (CBS) Protocol Template ● Introduction to CBS and Conducting a CBS Assessment webinar Powerpoints If you are considering using CBS for COVID-19, the following should be considered: ● Will implementing CBS add value in detecting people with signs and symptoms of COVID-19 in your context? ○ Is there a gap in the existing surveillance system? ● Is it feasible to implement? ○ Does the NS have the capacity, necessary structures and mandate? ○ Is there sufficient funding to cover the training and implementation of CBS in your context (suggested considerations are shared in the CBS Assessment tool)? ○ Is the NS engaged with the MoH and does the MoH support the use of CBS to compliment their structures? ○ Will the community members approve? ○ Is it possible for the volunteers to be trained and conduct CBS activities without being put at risk? We advise everyone who considers implementing CBS to conduct a needs and feasibility assessment prior to planning implementation. The CBS assessment tool (also found in the resource page mentioned above) and PPT gives guidance on how to conduct this assessment and what to consider. For additional support you can also reach out to [email protected] or [email protected]. See more info regarding decision making around CBS for COVID-19 here. In the future it will be possible to request the Nyss platform from the CBS website (https://www.cbsrc.org/). If you want to request to use the Nyss platform for the COVID-19 response now, please send an email to [email protected] CBS volunteers are a part of the community they volunteer in. The way in which RCRC CBS is designed, volunteers should act as focal points in their community for community members to give information about health risks/events that need to be reported. They are not tasked to conduct house-to-house visits. CBS volunteers are trained to follow-up on health risks by talking to the person reporting signs or symptoms, or asking follow-up questions to the person who reported the health risk/event. CBS volunteers are trained in first aid, community responses and key messaging (such as coughing etiquette, handwashing and physical distancing), but are not trained or tasked to examine a person or animal for which the health risk was reported. It is recommended that volunteers keep a 1-2 meter distance from community members during discussions and no physical contact should take place. If possible, discussions about someone showing health risks should take place outside, through a window or in an open space and between a healthy member of the family/ community member and volunteer rather than the ill person themselves If CBS is conducted by CHVs who have been trained to provide additional medical support they should follow any additional PPE measures that pertain to those activities and government requirements. For COVID-19, the CBS volunteers should follow the guidelines of their national authorities and national societies for protection and to avoid transmission. The volunteers should receive information about how to do this for themselves and how to inform the community members. In the times of COVID-19, the volunteers should report the health risk/event in their community and give guidance if this can be done in a safe way and according to national authorities regulations. For more advice regarding how to work in the communities, see the CBHFA chapter. More information about CBS for COVID-19, can also be found here. Contact tracing is the process of identifying, assessing and supporting people who may have been exposed to COVID-19 ( to prevent onward transmission. Please see the resources folder for available guidance, and submit questions to be answered here. Epidemic control for volunteers (ECV) Please see the resources folder for available guidance, and submit questions to be answered here. There are two scenarios in which quarantine may be implemented: - For travellers from areas with community transmission - For contacts of known cases. WHO provides guidance on this. This is available here. Management of the dead The Red Cross Red Crescent has extensive experience supporting safe management of the dead in infectious disease outbreaks. This includes both safe and dignified burials (SDB)—the name given to the procedures and approaches specifically designed to prevent transmission of viral haemorrhagic fevers—and other approaches to safely manage the dead during outbreaks of cholera and other diseases. These are both in addition to, and separate from, general management of the dead (MotD or DBM) procedures for disasters, conflict, or other mass casualty events. The use of Ebola SDB procedures and approaches for COVID-19 response would be inappropriate and unnecessary to prevent the spread of the pandemic. The IFRC technical guidance lays out the necessary precautions and personal protective equipment (PPE) required for autopsy and burial-related activities when COVID-19 is suspected. For Red Cross Red Crescent National Societies typically involved in safe and dignified burials, MotD or other activities supporting burial of the dead at the community level, the section on “Burial by family members or for deaths at home” in the WHO guidance may be useful in determining whether NS staff or volunteers have a role to play in supporting community burials for suspected COVID-19 cases. Following the Ebola SDB procedures for COVID-19 is intrusive and unnecessary, and may undermine trust between communities and RCRC volunteers and local branches and lead to increased risks for volunteers and staff. For a brief explanation of the difference between the two, see this document. In places where mortuary services are not standard or universally available, National Societies may be called upon to support families and community leaders to safely prepare bodies of suspected COVID-19 cases for burial. These activities are much more limited in scope and scale than SDB programming, and further practical guidance is being developed. As per current evidence, COVID-19 transmits via droplets, fomites and close contact. Dead bodies are generally not considered infectious, except for the lungs of the patient, if handled improperly during an autopsy. Though the safety and well-being of everyone who tends to bodies should be the priority. It is important that standard precautions are respected at all times. ● Use appropriate PPE according to the level of interaction with the body, including a gown and gloves. If there is a risk of splashes from the body fluids or secretions, personnel should use facial protection, including the use of face shield or goggles and a medical mask. ● Prepare the body and ensure that body fluid leaking from orifices are contained. ● There is no need for disinfection of the body or usage of body bags unless it is for other reasons (e.g. excess bodily leakage). ● No specific considerations with transport needed. ● In case of an autopsy: staff involved should be minimized and the autopsy performed with proper PPE, as well as in a properly ventilated room. ● Thorough environmental cleaning of the areas (and instruments, if an autopsy is performed) should be performed. Cultural and local customs should be respected as much as possible. Possibility for the loved one to view the body with instructions not to touch or kiss the body and to use standard precautions such as hand washing should be made available. The IFRC recommendations for management of the dead in the context of COVID-19 can be found here. Personal protective equipment for non-clinical activities PPE for community programming Personal protective equipment (PPE) may be useful for managing risks that can not be managed in other ways. However, the focus should be on the following first: - Reducing non-essential activities that could increase the risk of transmission of COVID-19 - Establishing safe systems of work first, such as using remote methods of working, physical distancing (keeping at least 2m/6ft apart from other people), or using physical barriers (such as plastic screening between volunteers and service users). Where the risk needs to be managed at the personal level (which is much more susceptible to human-error that risks self-contamination and contamination of others) then PPE may be appropriate. Your national government may have issued specific advice on PPE and this advice should be adhered to. Otherwise, the following should be considered: - PPE guidance for IFRC and National Societies can be found here, see “Personal Protective Equipment (PPE) supplies for COVID-19 - specifications and users” which is regularly updated and includes WASH and public health activities. - There may be a shortage of supply of masks, aprons and goggles, and the priority for these items should be the availability for healthcare workers who may be undertaking high-risk activities. - In some contexts, PPE such as masks can exacerbate anxiousness and create distance between volunteers/staff and communities Masks may be considered where it is not possible to stay 2m/6ft away from other people. - Reusable gowns may be considered where it is not possible to stay 2m/6ft away or where clothes can not be changed out of and laundered after completing the activity. - Gloves may only be appropriate if it is not possible to undertake regular hand-washing or use hand sanitiser frequently. Gloves should be changed between seeing different vulnerable people to minimise the risk of gloves transmitting COVID-19 between people. Gloves may give a false sense of security and reduce the frequency of handwashing. PPE training must be undertaken with staff and volunteers to ensure PPE is used properly and effective as shown here, and they do not contaminate themselves or the PPE (in terms of gloves if used) when using the PPE and how to care for the PPE and spot failures and degradation of the PPE. It will be important to be conscious of what other humanitarian agencies and government are doing with regards to PPE in the area you are working in and trying to match PPE levels wherever possible and appropriate, such that staff and volunteers are not made unnecessarily anxious if they perceive they are not adequately protected or protecting others. Ideally used and disposable PPE should be disposed according to WHO guideline on infectious waste management found here. However, there are guidelines for decontamination and re-using of PPE as attached here. The national society can utilise the decontamination procedures that is available for them The IFRC follows WHO guidance about the use of medical masks and respirators for healthcare workers and patients, and advocates for evidence-based interventions to end and mitigate public health emergencies. Where authorities or culture mandate the use of face masks, approaches advocating the use of cloth masks must both seek to prevent harm from inadequate masks and help to maintain the stock of medical PPE for the appropriate users. In epidemic or pandemic periods such as Covid-19, wearing a cloth mask that covers the mouth, nose and chin may contribute to reducing the spread of the virus from infected individuals and therefore may protect others from getting infected. Any person who is in contact with an infected person, with or without visible symptoms, may be exposed to respiratory droplets containing viral particles. Use of cloth face masks may reduce the amount of virus-containing droplets produced by an infected person, thereby decreasing risk of transmission to others. There is no evidence that cloth masks reduce the risk for individuals exposed to infected respiratory droplets. The largest impact of a cloth mask policy is expected to come from replacing the generalised use of medical equipment with the use of cloth face masks in a way that does not cause harm. It is important to re-emphasise the adjunct public health measures that have shown to reduce spread of the virus such as hand hygiene and physical distancing (where possible). This approach to cloth masks has three goals: 1. Reduce the negative impact of widespread use of medical masks on the availability of PPE for frontline health workers, by replacing medical equipment with reusable cloth face masks for members of the general public for whom WHO guidance does not recommend the use of medical masks; 2. Facilitate the correct use of reusable cloth masks meeting minimum standards, where opportunities for social distancing, respiratory etiquette, and handwashing are limited; this may limit infected people’s ability to project respiratory droplets infected with the virus onto surfaces or people who have not been exposed; 3. The correct use of cloth masks may help to reduce face touching, which could help to reduce individual risk. Guidance on cloth masks can be found here. Individuals without respiratory symptoms living in areas with active transmission where the use of a cloth mask has been advised should: ● Avoid as much as possible groups of people (e.g. gatherings, shopping, crowds, public transportation); ● Implement social distancing of at least 1-2 metres from others when outside of their own households; ● Stop shaking hands, social kissing and hugging, including at funerals ● Wash hands frequently, using soap and water or an alcohol-based hand rub ● Refrain from touching mouth, nose or eyes ● Follow instructions below to wear, remove, wash and dispose of the cloth face mask Individuals with respiratory symptoms, in addition to the above measures, are advised to wear a medical/surgical mask, according to WHO standards and follow advice from local authorities. How to use a reusable cloth face mask: ● After washing hands, place a clean and dry mask carefully on the face, ensuring it covers the mouth, nose and chin. Tie it securely to minimize any gap between the skin and the mask. ● Avoid touching the mask while wearing it. ● To remove the mask, do not touch the front part of the mask but untie it from behind. ● Before and after removal or whenever touching the mask, wash hands with soap and water or an alcohol-based hand rub. ● Replace the mask with a clean dry mask as soon as it becomes damp, or at least once a day, or more often for prolonged use. ● Use a dedicated storage bag to keep your used mask (see specifications). ● Wash and then dispose of damaged masks immediately. ● As early as possible after the mask is removed, wash the mask and the protective bag with hot water and soap and dry it completely before using it again. ● Do not discard or leave the mask out without washing it or closing it into a protective bag. ● Wash masks in hot soapy water (at least 60 degrees Celsius) ● Dry cloth masks in the sun or in a dryer until they are completely dry. Damp masks may increase the risk of infection. ● Expected lifespan of a reusable cloth mask is two months with daily washing. ● If planning to provide masks, count at least 3 masks per person per day. Not all cloth masks are equal, and poor quality cloth masks could actually be dangerous. The IFRC has minimum standards for face masks, including instructions and a pattern if you want to make them at home. These instructions are available at the end of the cloth mask guidance document found here. Water, sanitation and hygiene (WASH) Clinical and Pre-hospital care Scaling National Society clinical support during COVID-19 Before increasing the scale or scope of the clinical activities provided by your National Society, consider the following questions: ● Is this activity being formally requested by national or local health authorities? ● Does this activity fit within the mandate of my NS? ● Is there potential that engaging in this activity may cause harm to patients, staff, or the NS reputation? ● Do we have adequate HR capacity (or the realistic ability to scale up appropriately)? If dealing specifically with COVID-19 patients, this capacity must include specialist physicians, high-acuity nurses, clinical support staff, facility management experts, IPC experts, HR management, etc. ● Do we have adequate PPE to carry out the activities? If yes, do we have adequate quantities? If no, how will we source? ● Do we need international support, including global surge human resources or Emergency Response Units, to carry out the planned activities? If yes, strongly reconsider as due to the global nature and scale of this emergency, these resources will most likely not be available for international deployment. Support to health systems for all or vulnerable populations: ● Scale up hospital care (excluding COVID patients) to support the overall system and reduce the burden on facilities/systems caring for COVID patients. Prioritisation of services as per this guideline can also be utilised to help reduce burden on the health system ● Scale up primary health care activities (including NCD care, MNCH services including non-complicated deliveries) Support to clinical care for COVID-19 patients: ● Scale up hospital care to include COVID patients IF this capacity exists and the NS is mandated to do so by MoH ● Support home care and isolation activities (for mild to moderate COVID patients) where mandated by MoH Support to health systems for all or vulnerable populations ● Scale up primary health care activities (including NCD care, MNCH services including non-complicated deliveries and other primary care services) Support to clinical care for COVID-19 patients ● Support home care and quarantine activities (for mild to moderate COVID patients) where mandated by MoH Scale up and adapt prehospital services as mandated by MoH National Societies without existing capacity or mandate in clinical care and National Societies without any current health mandate or capacities are not recommended to become involved in direct clinical care provision, and should instead focus on building community health and WASH capacity, carrying out effective RCCE activities, and supporting the most vulnerable communities. These interventions will likewise have a significant impact on both outbreak response and overall humanitarian impact of the pandemic, without the high risk involved with clinical care. National Societies without previous clinical experience who are asked to support hospitals may consider (in collaboration with local authorities) establishing tented or semi-permanent structures adjacent to existing health facilities, to support with registration of patients, food provision, PSS or other non-clinical services that serve to reduce the burden on clinical care providers. These activities will allow existing health facilities and healthcare workers to dedicate their time to providing direct clinical care. Infection prevention and control (IPC) in clinical contexts Note: Some countries and contexts have specific guidance for IPC in clinical settings for COVID-19. It is important to follow the recommendations of local health authorities for the latest guidance and updates. IPC minimum requirements should be put in place at both national and local level, and to progress to the full achievement of all requirements of the IPC core components according to local priorities. IPC strategies to prevent or limit transmission of COVID-19 in healthcare settings include the following: ● Ensuring triage, early recognition, and source control (isolating patients with suspected COVID-19); ● Applying standard precautions for all patients; ● Implementing empiric additional precautions (droplet and contact and, whenever applicable airborne precautions) for suspected cases of COVID-19; ● Implementing administrative controls; ● Using environmental and engineering controls. More information on WHO IPC minimum standards and tools can be found here. WHO has guidance related to Best-Practices related to Environmental Cleaning in Healthcare Facilities in Resource-Limited Settings here. ● Environmental cleaning in healthcare facilities or homes housing patients with suspected or confirmed 2019-nCoV infection should use disinfectants that are active against enveloped viruses, such as 2019-nCoV and other coronaviruses. There are many disinfectants, including commonly used hospital disinfectants, that are active against enveloped viruses. WHO guidance on cleaning and disinfection practices can be found here and currently recommends the use of: ○ 70% Ethyl alcohol to disinfect reusable dedicated equipment (e.g., thermometers) between uses ○ Sodium hypochlorite at 0.5% (equivalent 5000ppm) for disinfection of frequently touched surfaces in homes or healthcare facilities More information on IPC can be found here: The protection of our frontline (health) workers is paramount and of utmost importance. Protecting yourself and your colleagues is much more than just about the PPE, and standard precautions and other instructions need to be adhered to, to ensure minimizing the transmission risk. PPE is also an important part of protection from the infection, when working closely with suspect or confirmed COVID-19 cases, but ONLY if you work according to instructions, and respect the donning and doffing procedures. Based on current evidence, the COVID-19 virus is transmitted between people through close contact and droplets. Airborne transmission may occur during aerosol-generating procedures and support treatments (e.g. tracheal intubation, non-invasive ventilation, tracheotomy, cardiopulmonary resuscitation, manual ventilation before intubation, bronchoscopy); thus, WHO recommends airborne precautions for these procedures. Current WHO recommendations of PPE when caring for COVID-19 patients are: ● The type of PPE used when caring for COVID-19 patients will vary according to the setting and type of personnel and activity. ● Health care workers involved in the direct care of patients should use the following PPE: gowns, gloves, medical mask, and eye protection (goggles or face shield). ● Specifically, for aerosol-generating procedures (e.g. tracheal intubation, non-invasive ventilation, tracheostomy, cardiopulmonary resuscitation, manual ventilation before intubation, bronchoscopy) health care workers should use respirators (i.e. N95 or FFP2 standard or equivalent), eye protection, gloves and gowns; aprons should also be used if gowns are not fluid resistant. Please see the link below for detailed information on recommended PPE in accordance to setting, personnel and type of activity: ● Personal Protective Equipment (PPE) supplies for COVID-19 - specifications and users (25/3/20) ● IFRC guidance on the generalised use of cloth face masks during the COVID-19 pandemic ● Rational use of personal protective equipment for coronavirus disease (COVID-19) and considerations during severe shortages: For more information please visit: Health care workers collecting nasopharyngeal (NP) and oropharyngeal (OP) swab specimens from suspected or confirmed COVID-19 patients should be well-trained on the procedure and should wear a clean, non-sterile, long-sleeve gown, a medical mask, eye protection (i.e., goggles or face shield), and gloves. The procedure should be conducted in a separate isolation room, and during NP specimen collection health care workers should request the patients to cover their mouth with a medical mask or tissue. Although the collection of NP and OP swabs have the potential to induce fits of coughing from the patient undergoing the procedure, there is no currently available evidence that cough generated via NP/OP specimen collection leads to increased risk of COVID-19 transmission via aerosols. Further information on this is available here No. Disposable medical face masks are intended for single-use only. After use, they should be removed using appropriate techniques (i.e. do not touch the front, remove by pulling the elastic ear straps or laces from behind) and disposed of immediately in an infectious waste bin with a lid, followed by hand hygiene. Handwashing with soap and (clean) water is the most effective way of getting rid of germs. Handwashing points should be set up (if not already) in all the rooms and areas in the clinic. There are also local ways to make hand rub, see attached WHO guidance here. All individuals dealing with soiled bedding, towels and clothes from patients with COVID-19 should: 1. Wear appropriate personal protective equipment, which includes heavy-duty gloves, mask, eye protection (face shield/goggles), long-sleeved gown, apron (if the gown is not fluid-resistant), boots or closed shoes before touching any soiled linen. 2. Never carry soiled linen against the body; place soiled linen in a clearly labelled, leak-proof container (e.g. bag, bucket) 3. If there is any solid excrement on the linen, such as faeces or vomit, scrape it off carefully with a flat, firm object and put it in the commode or designated toilet/latrine before putting linen in the designated container. If the latrine is not in the same room as the patient, place soiled excrement in a covered bucket to dispose of in the toilet or latrine; 4. Wash and disinfect linen: washing by machine with warm water (60-90°C) and laundry detergent is recommended for cleaning and disinfection of linens. If machine washing is not possible, linen can be soaked in hot water and soap in a large drum, using a stick to stir, avoiding splashing. If hot water is not available, soak linen in 0.05% chlorine for approximately 30 minutes. Finally, rinse with clean water and let linen dry fully in the sunlight. For more information please visit: WHO online training on IPC can be found here: https://openwho.org/courses/COVID-19-IPC-EN Clinical care for COVID-19 patients There is no specific treatment for COVID-19. However, many of the symptoms caused by this virus can be treated and therefore treatment should be based on the symptoms of the patient. Moreover, supportive care for infected persons can be highly effective. National Societies involved in clinical care should consult with their Ministry of Health regarding which clinical guidelines should be followed locally. WHO has produced several clinical guidance documents that may be helpful in your healthcare facility: - Clinical management of severe acute respiratory infection when COVID-19 is suspected - Training for clinical management is also available on OpenWHO here - Clinical care of severe acute respiratory infections – Tool kit No. Antibiotics do not work against viruses, they only work on bacterial infections. COVID-19 is caused by a virus, so antibiotics do not work. Antibiotics should not be used as a means of prevention or treatment of COVID-19. They should only be used as directed by a physician to treat a bacterial infection. While some Western, traditional or home remedies may provide comfort and alleviate symptoms of COVID-19, there is no evidence that current medicine can prevent or cure the disease. WHO does not recommend self-medication with any medicines, including antibiotics, as a prevention or cure for COVID-19. However, there are several ongoing clinical trials that include both western and traditional medicines. WHO will continue to provide updated information as soon as clinical findings are available. Providing clinical services for COVID-19 patients is a complex task that requires advanced resources, expertise, personnel, and equipment. Every context is different and care must be integrated into existing systems. WHO has published a practical manual (found here) to set up and manage a SARI treatment centre and a SARI screening facility in healthcare facilities that may be helpful. Clinical triage includes a system for assessing all patients at admission, allowing for early recognition of possible COVID-19 and immediate isolation of patients with suspected disease in an area separate from other patients (source control). To minimize transmission risk at the healthcare setting for healthcare personnel, as well as for other patients (and relatives), well-equipped triage, with trained staff and with a clear flow is essential. ● All health staff should be well trained and have a high level of clinical suspicion and understanding of COVID-19. ● Protocols must be adapted to local context and must take into consideration the various options available. ● All staff (including non-medical staff) that will work at the triage, should have proper training on the disease and what protective measures need to be taken and when. ● A clear flow of patients should be identified: - If possible, a pre-triage should be implemented, where a quick screening for possible symptoms and which ‘flow’ the patient should take is done (this can be done for example in advance by e.g. calling, or at the healthcare facility with information for patients with symptoms directing them to the right flow, or by HCW outside the facility who is screening the patients). - Separate flow and areas for the suspect COVID-19 patients from other patients. (If possible, by physical barriers, such as fences, walls, separate waiting areas etc.) - If appropriate, institute the use of screening questionnaires according to the updated case definition. (https://apps.who.int/iris/bitstream/handle/10665/331506/WHO-2019-nCoV-SurveillanceGuidance-2020.6-eng.pdf ) - Standard precautions should be implemented everywhere, and other protective measures (e.g. appropriate level of PPE) depending on the level of interaction, as well as procedures performed. - Information sharing and guidance at all levels (patients, relatives as well as all staff) should be implemented, to ensure compliance of the different measures taken. The community should be informed on the expected changes and delays as a result of such measures. Mental health and psychosocial support (MHPSS) Note: All Mental Health and Psychosocial Support related COVID-19 resources can be found here on the IFRC Reference Centre for Psychosocial Support website. Many guidelines and tools are available in different languages. Psychological First Aid (PFA) can be adapted for remote delivery. Guidance from the IFRC Reference Centre for Psychosocial Support on how to provide remote PFA can be found here. ● In the context of COVID-19, PFA to affected persons is usually provided online or over the phone. Exceptionally, the provision of PFA to frontline workers may take place in person. ● Where face-to-face PFA is being conducted, the IFRC Reference Centre for Psychosocial support has extensive guidance tools: Guidance from the IFRC Reference Center for Psychosocial Support on ‘Mental Health and Psychosocial Support for Staff, Volunteers and Communities in an Outbreak of Novel Coronavirus’ can be found here Migrants may be more vulnerable to the health, social, and economic risks and impacts of COVID-19 than the general population and often face barriers accessing healthcare services. ● IFRC, UNICEF, and WHO guidance on preventing social stigma can be found here. ● Find here the Interim Guidance on Scaling-up COVID-19 Outbreak in Readiness and Response Operations in Camps and Camp-like Settings (jointly developed by IFRC, IOM, UNHCR and WHO) - COVID-19 has multiple direct and indirect impacts on children’s protection, well-being, and development. - Guidance, including suggested messages, on how to talk to children and young people about COVID-19 from the Australian Red Cross can be found here. - Please find here IFRC key messages for governments on ‘Coordinating the Protection of Children during the COVID-19 Response’ - The Inter-Agency Standing Committee Reference Group on Mental Health and Psychosocial Support in Emergency Settings has published a book, My Hero is You, for children affected by the COVID-19 outbreak. It is intended to be read to children aged 6-11 years by a parent, caregiver, or teacher. Audio versions are also available. The book teaches children how to protect themselves and the people they love and how to manage difficult emotions they might face in this new and changing reality. Guidance from the IFRC Reference Center for Psychosocial Support on ‘Mental Health and Psychosocial Support for Staff, Volunteers and Communities in an Outbreak of Novel Coronavirus’ can be found here Guidance from the IFRC Reference Center for Psychosocial Support on Supportive Supervision for volunteers providing MHPSS during COVID-19 can be found here. - Hong Kong Red Cross has developed guidance on ‘Psychological Coping during a Disease Outbreak for families, friends, colleagues of those in quarantine or self-isolation’, outlining what to do and what not to do when providing support to those in quarantine or isolation. - Hong Kong Red Cross has developed a ’14-day psychological wellbeing diary’, offering ideas for daily activities and goals in order to stay physically and mentally active and healthy in quarantine. Maintaining essential community and clinical health services during the pandemic Maintaining essential community and clinical health services during the pandemic CBHFA is based on behaviour-change-focused health promotion through community-based volunteers. CBHFA volunteers work in the communities where they live, work and play. They use a number of different community mobilisation activities to help community members identify the priority issues and come up with solutions that work best for the community. But in the world of COVID-19, volunteers are modifying their community approaches to ensure that they and their community members stay safe in their interactions. Focus Group Discussions: While CBHFA volunteers use Focus Group Discussions normally, in COVID-19, traditional Focus Group Discussions should be stopped because of the risk this method poses to safe physical distancing. Some volunteers are conducting virtual focus group discussions using online tools like Skype or Zoom, while other volunteers are still using the method as designed BUT talking with a much smaller group of people (maximum four) who each sit an adequate distance apart. These discussions should ideally be held outside or in a well-ventilated area. Household Visits: Household visits are the most effective tools that CBHFA volunteers used pre-COVID-19. With some modifications, these visits are still possible and encouraged where safe and allowed by local authorities. We advise CBHFA volunteers to regularly check in with their vulnerable community members either by phone, through the computer or if allowed, by stopping by and checking in on them through an open door or a window. Just seeing the familiar face of their volunteer can go a long way for the mental health of people struggling in self-isolation. CBHFA volunteers already know where these vulnerable people live and so are best equipped for checking in regularly, and if necessary doing basic chores like fetching water or bringing food to the door, or reporting their health symptoms back to the local health authority. CBHFA volunteers are a vital link between the community’s needs, concerns and health status and the local health authority. Volunteers are trained to follow the guidance of their Ministry of Health and use the CBHFA approach to support the community. The Ministry of Health is the lead public health authority for all CBHFA volunteers and all CBHFA content is written in alignment with WHO guidance and protocols, but accessible and general enough to allow for adjustments to ensure that the materials aligns with government guidance. The CBHFA COVID-19 Module is written in accordance with WHO protocols and aligned with IFRC guidance. This guidance is vast and is kept up to date as we learn more about the virus that causes COVID-19. CBHFA content should be updated regularly to keep pace with these changing protocols, procedures, guidance, and recommendations. In the COVID-19 response, volunteers seek out the community members they know to be at risk of COVID-19 transmission. The volunteers check in on them regularly to provide health messaging, provide necessary resources like water, food, psychosocial support and information. If the person doesn’t feel well or reports having one or more symptoms of COVID-19, the volunteer will coach a person in the household in proper home care and share the health information with local health authorities. Sure, CBHFA is a community-based health approach and tool. It can be used at any time. National Societies wanting to incorporate CBHFA programming can talk with the IFRC regional office to see how they can get support to train volunteers and staff virtually. IFRC is working on a suite of tools and webinars to support National Societies in sustaining or starting up CBHFA programming for a more effective COVID-19 response. CBHFA volunteers are asked to check with their Ministry of Health guidance as well as their National Society regulations around when to use masks. While the IFRC provides guidance, CBHFA volunteers should work in alignment with their National Society guidance. In line with national guidance, we advise volunteers to wear cloth masks when they are interacting with the community. Anyone who is immune-compromised or in a high-risk group (based on age or medical condition) should wear a medical mask. Guidance on the use of cloth masks is available here. The only exception is that volunteers interacting with sick people or caring for a sick person should wear a mask, gloves and use the guidance in proper wearing, removal and disposal of the PPE - please see home care here guidance here. Reproductive, maternal and newborn health Experience in past epidemics has shown that lack of access to essential health services resulted in more deaths than those caused by the epidemic itself. It is important to ensure that essential reproductive, maternal and newborn health services be maintained during the pandemic using appropriate infection prevention and control precautions. This includes intrapartum care for all births, emergency obstetric and newborn care, post-abortion care, safe abortion care to the full extent of the law, contraception, clinical care for rape survivors, and prevention and treatment for HIV and other sexually-transmitted infections. For antenatal care (ANC), prioritize routine visits for women in the third trimester and those with high-risk pregnancies (including hypertension and diabetes). Women with obstetric complications need to have access to care 24/7. Postnatal care is critical for reducing preventable mortality and should be maintained – focus on first week postnatal visits for women and newborns, including breastfeeding support. Health workers should be prepared to care for those subjected to intimate partner violence, as violence is likely to increase during epidemics due to stress, increased confinement and exposure to perpetrators and reduced access to basic needs. This document explains why there is an elevated risk of sexual and gender-based violence (SGBV) in the face of the COVID-19 pandemic, who is likely to be most affected and what operational approaches can be adopted to prevent, mitigate or respond to SGBV by Movement actors. The guidance also includes diplomatic messages to support decision-makers in preventing and responding to SGBV and brief guidance on how to ensure staff and volunteers are protected in their SGBV prevention and response efforts. Latest studies indicate that pregnant women are at a higher risk of severe illness than the general population. The normal physiological changes occuring during pregnancy exposes preganat women to more risk in the context of COVID-19. It is important that pregnant women take precautions to protect themselves against COVID-19 and report possible symptoms to their healthcare provider Although some studies indicated the risk of vertical transmission is low, few cases have been reported with comprehensive serial tests from multiple specimens. COVID-19 during pregnancy is associated with increased risk for adverse maternal and neonatal outcomes, an association that is primarily driven by morbidity associated with severe/critical COVID-19. Intrapartum care of women with suspected or confirmed COVID-19 needs to ensure isolation of the patient from other patients. Appropriate personal protective equipment (PPE) must be used by relevant health staff including mask, goggles, gloves, and gown/apron. At present, data are not sufficient to conclude vertical transmission of COVID-19 through breastfeeding. In infants, the risk of COVID-19 infection is low, the infection is typically mild or asymptomatic, while the consequences of not breastfeeding and separation between mother and child can be significant. At this point it appears that COVID-19 in infants and children represents a much lower threat to survival and health than other infections that breastfeeding is protective against. ● Further, newborns and infants are at low risk of COVID-19 infection. Among the few cases of confirmed COVID-19 infection in young children, most have experienced only mild or asymptomatic illness. ● It is recommended to initiate and continue breastfeeding of infants and young children also apply to mothers with suspected or confirmed COVID-19. ● Breastfeeding women with COVID-19 should practice respiratory hygiene during breastfeeding and wear a mask where available. Wash hands before and after touching the baby and routinely clean and disinfect surfaces. ● Yes. Immediate and continued skin-to-skin care, including kangaroo mother care, improves thermoregulation of newborns and several other physiological outcomes, and is associated with reduced neonatal mortality. Placing the newborn close to the mother also enables early initiation of breastfeeding which also reduces neonatal mortality. ● The numerous benefits of skin-to-skin contact and breastfeeding substantially outweigh the potential risks of transmission and illness associated with COVID-19. If a mother is confirmed/suspected to have COVID-19 she should: ● Wash hands frequently with soap and water or use alcohol-based hand rub, especially before touching the baby ● Wear a medical mask while feeding. However, if the mother does not have a facemask, breastfeeding can still be continued. When wearing a facemask it is important to: - Replace masks as soon as they become damp - Dispose of masks immediately - Not re-use a mask - Not touch the front of the mask but untie it from behind ● Sneeze or cough into a tissue, immediately dispose of it and use alcohol-based hand rub or wash hands again with soap and clean water. ● Regularly clean and disinfect surfaces ● Regularly clean and disinfect surfaces Even when COVID-19 is not a consideration, breast milk pumps, milk storage containers and feeding utensils need to be appropriately cleaned after every use. - Wash the pump/containers after every use with liquid soap, e.g. dishwashing liquid and warm water. Rinse after with hot water for 10-15 seconds. - Some breast pumps parts can be put in the top rack of a dishwasher (if available). Check the instruction manual before doing this. Wet-nursing may be an option depending on acceptability to mothers/families, national guidelines, cultural acceptability, availability of wet-nurses and services to support mothers/wet-nurses. In settings where HIV is prevalent, prospective wet-nurses should undergo HIV counselling and rapid testing, according to national guidelines, where available. In the absence of testing, if feasible undertake HIV risk assessment. If HIV risk assessment/counselling is not possible, facilitate and support wet-nursing. Provide counselling on avoiding HIV infection during breastfeeding. Prioritise wet-nurses for the youngest infants. A mother can start to breastfeed when she feels well enough to do so. There is no fixed time interval to wait after confirmed/suspected COVID-19. There is no evidence that breastfeeding changes the clinical course of COVID-19 in a mother. She should be supported in her general health and nutrition to ensure full recovery. She should also be supported to initiate breastfeeding or relactate. No. There are always risks associated with giving infant formula milk to newborns and infants in all settings. The risks associated with giving infant formula milk are increased whenever home and community conditions are compromised e.g. reduced access to health services if a baby becomes unwell / reduced access to clean water / access to supplies of infant formula milk are difficult or not guaranteed, not affordable and not sustainable. The numerous benefits of breastfeeding substantially outweigh the potential risks of transmission and illness associated with the COVID-19 virus. No. If a mother is confirmed/suspected to have COVID-19 and is breastfeeding, there is no need to provide a ‘top-up’ with infant formula milk. Giving a ‘top-up’ will reduce the amount of milk produced by a mother. Mothers who breastfeed should be counselled and supported to optimise positioning and attachment to ensure adequate milk production. Mothers should be counselled about responsive feeding and perceived milk insufficiency and how to respond to their infants’ hunger and feeding cues to increase the frequency of breastfeeding. No. Donations of infant formula milks should not be sought or accepted. If needed, supplies should be purchased based on assessed need. Donated formula milk is commonly of variable quality, of the wrong type, supplied disproportionate to need, labelled in the wrong language, not accompanied by an essential package of care, distributed indiscriminately, not targeted to those who need it, is not sustained, and takes excessive time and resources to reduce risks. For more information on breastfeeding in the context of COVID-19, including a helpful decision tree, please see here for guidance from WHO. Further detailed information is available from WHO, the Inter-Agency Working Group on Reproductive Health in Crises, and the Safe Delivery App. More resources on Sexual, Reproductive, Maternal and Newborn Health can be found here. The IFRC, UNICEF and World Health Organization recommend that routine immunization services should be maintained to prevent vaccine-preventable diseases. However, as always, National Societies should ultimately follow the guidance of each country’s Ministry of Health. Further guideline on how this should be done is available here The National Society can advocate with the government to maintain immunization services and can share the WHO and UNICEF recommendations to maintain routine immunization during COVID-19. Maintaining immunisation services will help prevent outbreaks of epidemic prone vaccine-preventable diseases, such as measles, and will help protect older adults and individuals with high-risk conditions, such as respiratory diseases from pneumococcus and influenza, both of which can cause pneumonia. The approach to immunisation services should be based on local physical distancing policies and infection prevention and control measures. The immunisation visit also provides an opportunity to share information on behaviours to reduce the risk of transmission of COVID-19. National Societies can help inform communities that immunisation of children is taking place and can explain the importance of vaccinating children to protect them from outbreak prone (and life-threatening diseases) such as measles, diphtheria, pertussis, and meningococcus. During the immunisation session, mothers/caregivers should follow local guidance on physical distancing while in the waiting area. Outbreaks of vaccine-preventable diseases, such as measles, may occur, especially in countries that have delayed a vaccination campaign and/or have temporarily suspended routine immunisation services. National Societies can engage local authorities and community leaders among high-risk population groups with low vaccination coverage to discuss the situation and identify barriers to immunization. These discussions should maintain physical distancing and should include only a limited number of individuals, considering local guidance on the size of meetings. National Societies can then work with government authorities to overcome the identified barriers and share information with these communities on actions taken. In the long run, listening to the community and taking actions based on the community’s concern is likely to improve routine immunisation coverage. In supporting these activities, National Societies should carefully consider the safety of staff and volunteers, and may request the use of cloth face masks and may rely on younger staff and volunteers who do not have underlying health conditions. Community-based surveillance for polio and other health risks can be maintained during the COVID-19 pandemic. In some settings, community-based surveillance for neonatal tetanus can be conducted remotely. If this is possible, then this surveillance should be continued. Persons conducting community-based surveillance should not undertake any in-person investigations or community/group sensitizations unless asked to do so by the local health authorities, and should follow the guidance on CBS in COVID-19 (see CBS section above). Vaccination against COVID-19 Vaccination does not cause COVID-19 infection, and there is no evidence that COVID-19 illness can be made worse by receiving a vaccine. However, individuals who have COVID-19 symptoms such as fever and cough should not be taken to a vaccination session at this time. Rather, a healthcare provider should be consulted who can give advice on the next steps related to the illness. To date, scientists are proving that getting vaccinated could be a powerful way to not only protect yourself but also your entire community. Wearing masks and social distancing help reduce your chance of being exposed to the virus or spreading it to others, but these measures are not enough. Vaccines are preparing your body’s natural defenses to recognize and fight off the virus that causes COVID-19. This means that COVID-19 vaccines will work with your immune system so it will be ready to protect you from the virus if you are exposed. Experts believe that getting a COVID-19 vaccine may also help keep you from getting seriously ill even if you do get COVID-19. Like all vaccines, COVID-19 vaccines are going through rigorous, multi-stage trials, including studies that involve tens of thousands of people. These trials, which include people at high risk for COVID-19, are designed to identify common side effects or other safety concerns. When the results of the trials are available, regulatory agencies review the data to make sure the vaccine is safe and safe. The vaccine is only rolled out when its regulators are confident that the vaccine is safe and effective. After a COVID-19 vaccine is introduced, it will be monitored to identify any unexpected side effects. While COVID-19 vaccines have been developed faster than any other vaccine in history, safety was just as much a focus as in any other vaccine development. Scientists prioritised COVID-19 vaccine development because of the global emergency. The vaccines that are now being reviewed and approved by regulatory bodies have been through the same amount of testing and safety processes as other vaccines. Any approved vaccine will have been rigorously tested on tens of thousands of people. Some of the processes usually involved in scientific research were also sped up so that the vaccine could be made available more quickly. For example, trial participants were recruited while the study was still being set up, so they were ready to start the moment the research was approved. Vaccine confidence is the belief that vaccination, and the providers, private sector, and political actors behind it, serve the public’s best health interests. Like ‘hesitancy,’ it is highly variable and rooted in political-economic context. This will affect the populations’ trust in vaccines. Scientists are developing many potential COVID-19 vaccines. They are all designed to teach the body’s immune system to safely recognize and block the virus that causes COVID-19. Different types of vaccines include: - Inactivated or weakened virus vaccines use a form of the virus that doesn’t cause disease, but still prompts an immune response. Vaccines for hepatitis A and chickenpox work this way. None of the COVID-19 vaccines approved by late 2020 were made this way. - Subunit vaccines, such as protein-based vaccines, inject a protein, protein fragment or other small structure found on the virus to prompt an immune response. Influenza B and whooping cough vaccines are examples of this type of vaccine. None of the COVID-19 vaccines approved by late 2020 were made this way. - Viral vector vaccines use a different virus that has been engineered so it can’t cause disease in people. The engineered virus produces COVID-19 proteins and so prompts an immune response to the virus that causes COVID-19. The Ebola vaccine is an example. The Oxford/AstraZeneca vaccine is this kind of vaccine. - RNA and DNA (nucleic acid) vaccines are a new approach that provide “instructions” for cells in the human body to build a protein that then safely prompts an immune response. Both Pfizer/BioNTech and Moderna vaccines work this way. Because of the urgent global need for the COVID-19 vaccine, different groups of scientists have been working to get a solution as quickly as possible. With any vaccine development, there tend to be many options that are tried and that don’t reach the final stages. The scale of the COVID-19 problem and the worldwide need for a vaccine mean it is better if more than one vaccine exists. This will increase availability and offer more than one way to tackle the problem. So it’s a good thing that there are several promising vaccines. You should have whichever approved and licensed vaccine you are offered by health workers in your area. If more than one vaccine is available in your community, health workers and health authorities will decide. They will base the decision on your own health status and what is most feasible and effective to provide to different populations. Further studies will look at how best to use the different vaccines, which will include looking at which vaccine is most effective in which people. Like all medicines, vaccines can cause side effects. Most of these are mild and short-term, such as pain at the injection site, tiredness or a headache. Many people don't get any side effects. It can happen with many vaccines that some people might feel slightly unwell because their immune system is responding to the protein, but this is not a COVID-19 illness and the vaccine can’t give you COVID-19. Given that these are new vaccines, it is possible that someone could be allergic to a component of them. However, this would show up shortly after administration of the vaccine. Vaccine providers are ready to respond to allergic reactions and other adverse events. Anyone who is known to be allergic to an ingredient of that vaccine shouldn’t receive it. The majority of vaccine side effects occur shortly after receiving a vaccine, not months or years later. Once a vaccine is licensed (approved), scientists and government agencies continue to monitor its safety to identify any rare side effects, such as those that might affect one person in a million. These monitoring efforts also focus on particularly vulnerable people, including people with specific medical conditions, or pregnant women, who may not have been included in the clinical trials. Health workers are trained to identify and report adverse events. People with underlying health conditions may be particularly closely monitored as vaccines are rolled out. If there’s any reason to suspect that a certain group of people might be adversely affected by a vaccine, regulators can require further research before it is allowed to be given to people with the same condition. These vaccines cannot change your genes. They contain a small piece of genetic code that gives instructions to your body’s cellular machinery to make a protein, which then triggers your body’s immune response. The RNA or DNA that is injected falls apart very quickly and doesn’t enter the part of your cells that house your own chromosomes, so it can’t change your genes. Other DNA vaccines in development (including for influenza, HPV, and HIV) have been extensively tested in animals and people and shown to be safe. No. You can’t get COVID-19 from the vaccine. A vaccine would not be approved if it could give you the disease it is meant to protect you from. As of December 2020, vaccines being administered or reviewed for approval did not contain any live virus of the type that causes COVID-19. Your immune system needs to generate a response, so generally the protection from the virus starts after about seven days. In the case of vaccines which need to be given in two doses, that means that you benefit from the full protection of the vaccine about a week after the second dose. We don't yet know for sure how long protection will last. It is likely to be at least several months, but it may be that repeat vaccinations are needed. Researchers are studying this closely. The lingering effects or “long” COVID-19 are concerning and can be debilitating for the people who experience the disease this way. We still have much to learn about this. A vaccine will help from the point of view that if it decreases infections, fewer people will experience illness and, therefore, fewer people will experience long-term effects. But it is not likely that a vaccine will address these effects in someone who was already infected. Yes. For the time being, we recommend that everyone – including those who have been vaccinated – continue using all available tools to help stop the spread of COVID-19, such as physical distancing and the use of masks. Using these tools in combination will provide the best possible protection against getting and spreading COVID-19. The available vaccines are highly effective at preventing you from getting seriously ill with COVID-19. However, we don’t know yet how effective they are at preventing asymptomatic infection, where the vaccinated person would not feel ill but could still spread the virus to others. This recommendation could change once many people have been vaccinated, and we learn more about the protection provided by COVID-19 vaccines. Ongoing research is needed to show if COVID-19 vaccines provide long-term protection. Additional doses of vaccine may be needed to provide continued protection. It will take ongoing evaluation over several years to understand how our immune systems respond to this virus and how vaccines assist in that response. Given that this disease has only existed for a year, and the vaccine trials have been going on for less time, it’s impossible to know how long immunity will last. Researchers will continue to monitor people who have been vaccinated so that, over time, we will have a better picture of how long protection lasts. With this information we will know whether we might need booster shots to maintain our protection. Vaccines for other diseases do not protect against COVID-19. However, it is important to get the flu vaccine and ensure you are up to date on all your vaccines because they prevent other serious infections. A flu vaccine will not protect you from getting COVID-19, but it can prevent you from getting influenza (flu) at the same time as COVID-19. This can prevent you from having a more severe illness. Even with a vaccine, society will not return to normal overnight. We don’t know how quickly COVID-19 vaccines could control the pandemic. That will depend on many factors, such as the level of vaccine effectiveness; how quickly they are approved and manufactured; how many people get vaccinated; and the continuation of measures such as physical distancing and mask use. 60-80% of the population would have to be immune to the virus to achieve herd immunity (or community protection). Even if enough doses of the vaccine were available today for everyone in a country, it would still take time to vaccinate everyone who wants to be vaccinated. Countries will have specific vaccination roll out plans as the vaccine becomes available, but overall initial doses will likely be provided to health workers, social care workers and people at high risk of severe illness such as the elderly or those with underlying conditions, and are unlikely to be given to children. However, it remains important to make sure that children continue to receive routine childhood vaccinations. Anyone can get sick with COVID-19 and become seriously ill or die at any age. Furthermore, asymptomatic people infected with the virus can be contagious and the virus can spread from them to other people. For these reasons, it is important to consider that getting vaccinated may also protect people around you, particularly people at increased risk for severe illness from COVID-19. Furthermore, based on available information about vaccines for other diseases and early data from clinical trials, experts believe that getting a vaccine can also prevent severe illness if you do get COVID-19. According to the WHO, convincing people on the merits of a COVID-19 vaccine would be more effective than making the vaccination mandatory. That said, it is down to individual countries how they conduct their vaccination campaigns. Importantly, the local Red Cross/Red Crescent in each country will play an essential role in ensuring that populations have access to essential information about the vaccine. It is likely that the supply of COVID-19 vaccines will be limited at first, which means not everyone will be able to get vaccinated right away. As doses of COVID-19 vaccines become available, they will be provided first to those at highest risk. The specific groups that are eligible for the first doses of COVID-19 vaccines may vary depending on the vaccine and the country. Initial groups will likely include frontline health and care workers, elders above 65 years of age and people with underlying conditions like heart disease and diabetes. Once adequate doses become available, the rest of the population will be encouraged to get vaccinated. Some countries are already starting to roll out some vaccine candidates. However, we aren’t yet certain when COVID-19 vaccines will be ready for distribution in all countries. Many potential COVID-19 vaccines are being studied to determine if they are safe and effective. Trials of several of these vaccines have had encouraging preliminary results. It is likely that results from other trials will be announced soon. Once a vaccine is shown to be safe and effective, it must be approved by national regulators and manufactured before it can be distributed. We are particularly concerned about the potential exclusion of migrants, displaced and stateless persons, as described in the IFRC’s September 2020 report “Least Protected, Most Affected: Migrants and refugees facing extraordinary risks during the COVID-19 pandemic.” As pointed out there, both formal barriers (migrants denied vaccination) and informal barriers (related to language, fear of arrest, lack of knowledge about available services and how to access them) may stand in the way. The IFRC calls on all governments to ensure that marginalized communities are not left behind. The IFRC is playing an active role as part of the COVAX Facility which aims to accelerate the development and manufacture of COVID-19 vaccines, and to guarantee fair and equitable access for every country in the world. The Global Vaccine Alliance (GAVI) is the lead agency for COVAX. The GAVI financial mechanism aimed to ensure that the 92 middle- and lower-income countries that cannot fully afford to pay for COVID-19 vaccines themselves get the same access to COVID-19 vaccines as higher-income countries. The IFRC is working in close partnership with countries, regional colleagues, and other stakeholders to develop policies and training courses, strengthen regulatory capacity, and guide countries in all the needed preparations for COVID-19 vaccine delivery. Detailed technical guidance and adaptable planning tools and templates to help countries plan for COVID-19 vaccines are being developed by the IFRC. These cover planning and implementation, data and monitoring, supply and logistics, and acceptance and demand, always ensuring community buy-in and trust are at the forefront of all NSs activities. National Red Cross and Red Crescent societies around the world support their local authorities and governments to fill gaps based on their areas of expertise and where needs lie. Many are already in discussion with local authorities to determine how to best support public health and prevention efforts of their communities, including by supporting vaccination. More information will be available in the coming weeks about each National Society’s role in the effort to protect people against COVID-19. The IFRC follows the recommendation of the WHO, which is still in the process of reviewing viable COVID-19 vaccines for public use. At this time, we encourage the public to follow advice of their local health authorities to help ensure the health and safety of our communities to improve the broader health of their communities. We recommend that individuals reach out to their healthcare provider to find out if they are eligible to receive a COVID-19 vaccine and where they can get it. There are no specific international rules for countries to decide whom to prioritise for first vaccine doses. However, the WHO’s Strategic Advisory Group of Experts on Immunization (SAGE) has provided detailed recommendations. Both the SAGE Values Framework and its Roadmap call for frontline health workers to receive priority access to COVID-19 vaccines. As described in the Values Framework, frontline workers respond to the value of “reciprocity,” including gratitude for the risk to which they are exposing themselves in order to help others. This includes not only doctors and nurses, but persons – like Red Cross and Red Crescent National Society volunteers – who support testing, contact tracing and people in quarantine or isolation, organise crowd control at vaccination sites, undertake post-vaccine observation among other activities that requires interaction with high-risk populations. The IFRC recommends support of COVID-19 vaccine introduction if both these conditions are met: - Phase 3 clinical trials have been completed and peer reviewed (published in a reputable academic journal), and the vaccine has shown to be safe and effective in preventing severe disease. - Vaccine is prequalified or has been approved for Expanded Use Listing (EUL) by the WHO vaccine is approved for licensure or EUL by a National Regulatory Authority (NRA) that is fully functioning according to WHO standards. Providing consistent and scientifically accurate information can reduce vaccine hesitancy. However, vaccine confidence may not improve unless efforts are made to increase public trust in vaccine effectiveness and safety, in public health response, and in health systems and government more broadly. Confidence in a vaccine and immunizations will not improve without sustained community engagement to increase public trust in vaccine effectiveness and safety. It is standard practice to ask people who have just been vaccinated to wait at the doctor’s office or vaccination site for a little while after vaccination, to make sure there are no concerning side effects, such as an allergic reaction. The vast majority of severe reactions – which are rare but do occur – happen immediately after vaccination, and this observation period ensures that the rare people who experience these reactions have immediate access to medical help if they need it. Viruses constantly change through mutation, and new variants of a virus are expected to occur over time. Sometimes new variants emerge and disappear. Other times, new variants emerge and persist. Multiple variants of the virus that causes COVID-19 have been documented globally during the pandemic. Public health officials are studying these variants quickly to understand whether the variants change the effectiveness of COVID-19 vaccines. There is no evidence that this is occurring, and most experts believe this is unlikely to occur because of the nature of the immune response to the virus. We encourage everyone to rely on trusted sources of information, such as health care providers and public health officials, to help them make informed choices and stay up to date. The latest information about COVID-19 vaccines from the WHO is available here. Alliance for Malaria Prevention guidance on insecticide-treated net (ITN) distribution during the COVID-19 pandemic can be found on the AMP website under the following link: AMP Guidelines and Statements Although COVID-19 represents a serious threat to health, other diseases, including malaria, may resurge during the pandemic if they are not adapted to deliver prevention and case management interventions safely. When planning ITN distribution during the COVID-19 pandemic, it is critical to work with the COVID-19 national task force to review strategies, discuss risks and mitigation measures and identify appropriate infection prevention and control, as well as define protocols for sick workers and contact tracing in case of sick personnel during the distribution. The AMP guidance on safe ITN distribution during COVID-19 transmission sets out some of the main considerations for the planning and implementation of campaigns in a safe way to minimize exposure to and transmission of COVID-19. In light of the COVID-19 pandemic, many National Malaria Control/Elimination Programmes are reviewing their strategies and operating procedures for their Insecticide-Treated Nets (ITNs) mass distribution. One of the issues to be considered by countries is whether to integrate COVID-19 messages in mass campaign messaging. The AMP guidance on Social and Behavior Change (SBC) activities in the COVID-19 context sets out the advantages and disadvantages of integrating COVID-19 with ITN campaign measures (co-messaging) for several groups of SBC activities. ITNs should be made accessible to people affected or suspected to be a carrier of COVID-19 and they should be encouraged to use them, sleeping separately from others, to prevent co-infection with malaria while ill. Information that should be disseminated needs to include the fact that anyone with symptoms of COVID-19 should be able to sleep under an ITN. Another consideration for countries ordering and/or distributing nets should be to increase the buffer stock of ITNs during the pandemic, to ensure that people infected with the virus that causes COVID-19 can sleep under a net by themselves ITNs, once washed, are safe to be used again after having been used by someone affected by COVID-19 or who died from the virus. Based on current evidence, soap (or equivalent) and water are sufficient for washing ITNs that have been exposed to COVID-19. ITNs that have been used by people with COVID-19, should be washed with soap in cool water. Never use hot water or harsh detergents to wash ITNs. Always dry ITNs in the shade after they have been washed. Discard the water away from clean water sources. Do not repurpose new ITNs that have been used by people with COVID-19 for any other uses other than malaria prevention. As distribution strategies will have to be adapted at country level, with preference for door-to-door, single-household contact distribution modalities instead of fixed distribution points, community channels for distribution should be explored, in particular through integration with existing health programmes. The last mile logistics for ITN distribution is likely to be particularly challenging, and could benefit from existing community distribution channels. The main points of exposure for COVID-19 transmission are related to the handling of the bales. Therefore, it will be important to plan and budget for the required handwashing stations, soap or hand sanitizer for warehouse managers, loaders and off loaders, conveyors (where used, and to reduce risks, conveyors should be reconsidered for ITN distribution in COVID-19 affected countries as physical distancing will be difficult to maintain) and logistics personnel at all levels. On and offloading of bales typically involve bringing workers together in groups to move bales from trucks/containers to warehouses and vice versa, so the approach may need to be revised to fewer people for loading and offloading (in line with restrictions on groups of people and being able to maintain physical distance of two metres between people), which may increase the amount of time required for the operation and should be accounted for in the timeline of activities. The AMP guidance on ITN distribution in COVID-19 context strongly advocates for NMCP and partners involved in ITN distribution to prioritize areas with the highest malaria burden. ITN distribution should also target vulnerable groups, such hard-to-reach and marginalised people, as well as IDPs and refugees. When necessary, urban areas can be deprioritised in order to free up ITNs for rural areas, where distribution is more important for malaria control due to higher disease burden. First Aid guidance for National Societies comes from the IFRC Global First Aid Reference Centre (GFARC). For additional questions related to first aid and COVID-19, contact GFARC at [email protected]. Remember, always follow your local health agency/ government rules. Medical advice changes constantly. This list of FAQs should not be considered current, complete, or exhaustive. National Societies providing clinical services should continue to monitor the WHO and respective Ministries of Health for the latest clinical and infection prevention and control guidance. Yes, however, preventive steps must be taken to reduce the potential risk of transmission. Hand hygiene measures should be followed- where possible soap and water should be used. If unavailable, an alcohol-based hand sanitiser or disinfectant solution can be used instead. Hands should be washed hands BEFORE and AFTER providing first aid. Washing hands with ash is an alternative when soap is not available. Rub your hands with cold ash from a clean wood fire to remove germs. Remove the ash by shaking your hands or using a clean tissue or cloth to wipe them away. Follow your local health authorities’ guidance about what to do if you suspect you may have COVID-19. Some communities test anyone with symptoms in order to isolate all cases, while others recommend that anyone with symptoms of respiratory illness stays home until after their symptoms have resolved. If you are providing home care for someone who has symptoms of COVID-19, use the IFRC home care for coronavirus patients guidance. If the person has a mild fever or cough, they should stay at home and rest in isolation from those they live with, unless the testing policy in their country or community urges them to get tested and treated even for mild COVID-19. IFRC guidance on home care for coronavirus patients can be found here. If the person is experiencing difficulty breathing or has a persistent temperature above 38°C (103-104 Fahrenheit), call a doctor, emergency medical services, or healthcare provider immediately. There are different CPR protocols to follow depending on if the casualty is an adult or child. ● Evidence suggests that CPR has the potential to generate aerosols ● First aid providers caring for persons with a suspected case of COVID-19 should follow the general preventive measures for reducing infection risk when caring for a person with COVID-19 . It is advisable to place a facemask over the casualties nose and mouth. It is also advisable to wear gloves and a disposable gown if possible. ● When providing CPR to adults, it should be limited to continuous chest compressions at a rate of 100 to 120 compressions per minute until the arrival of an artificial ventilation device. ● When providing CPR to children, in addition to chest compressions rescuers who are willing and trained to do so can provide rescue breaths to infants and children. ● If possible, do not shake dirty laundry. This will minimize the possibility of dispersing virus in the air. ● If possible, wash items using the warmest appropriate water setting (40°C minimum). Dry items completely. If clothing cannot be immediately laundered, store in a sealed disposable bag for transport to a cleaning facility. ● The use of the infrared thermometers remains valid for all first aid activities. ● Standard hygiene measures must be applied to protect personnel during the activity and to disinfect the equipment. Infrared thermometers could be used without contact. ● Gatherings are not recommended at this time. Many governments have put restrictions on non-essential gatherings. Follow your local authority/ government rules. ● If a first aid training must take place and is authorized to do so: - Trainers who are ill or vulnerable to illness should not provide trainings. - As a part of pre-class communication, potential participants who are ill or who have underlying conditions should be told to stay home. ● Participants and instructors should wash their hands between each use of equipment with soap and water for at least 20 seconds. Where hand washing is not possible, use an alcohol based hand sanitizer that contains at least 60% alcohol. ● Mannequins and other training devices touched by participants should be cleaned between each use, with the cleaning product routinely used for this purpose, according to the manufacturer's directions. This may include cleaning solutions, sprays and wipes, which should be labeled as effective against viruses. ● Where possible, one mannequin per participant should be used. ● When teaching how to respond to an unresponsive casualty, use a mannequin and not a volunteer participant for demonstration and practice purposes ● Participants should still practice and master the chin lift technique however, do not let participants perform rescue breaths. Emphasize the importance of rescue breaths outside of the COVID-19 pandemic situation. ● Chest compressions and public access defibrillation can be practiced ● Exceptionally, participants who have not practiced giving rescue breaths can receive their first aid certification given that they have mastered the chin lift technique and all other required skills ● When teaching how to respond to bleeding, students should practice the direct pressure method on themselves ● When teaching how to respond to choking, do not practice back blows. Students can practice the correct hand position for abdominal thrusts on themselves. You can find further guidance materials produced by GFARC here. Support for older people ● All age groups are at risk of contracting COVID-19 however, older people (over age 60) are at greater risk of developing severe illness and complications as a result of infection (WHO, 2020). This may be due to the physiological changes associated with ageing and/ or underlying conditions. ● Find here the Interim guidance for Red Cross and Red Crescent staff and volunteers working with older people during COVID-19 response ● Find here the Red Cross Red Crescent key messages for older people during the COVID-19 pandemic, for adaptation by National Societies. ● In the context of physical distancing, Psychological First Aid (PFA) can be adapted for remote delivery to older adults who may be in isolation or quarantine. Guidance from the IFRC Reference Centre for Psychosocial Support on how to provide remote PFA can be found here. ● Hong Kong Red Cross guidance on psychological coping during disease outbreak for elderly and people with chronic conditions can be found here Non communicable diseases (NCDs) All groups are at risk of contracting COVID-19. However, those with underlying conditions and non-communicable diseases (of any age group) are more at risk of severe illness and complications associated with infection (WHO, 2020). Such underlying conditions may include cardiovascular disease, diabetes, chronic respiratory disease, and cancer. Hong Kong Red Cross guidance on psychological coping during disease outbreak for older people and people with chronic conditions can be found here.	2	12	1	0	0	1	0.540509	2	21,472

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