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https://bit.ly/3OvFRS0
A 23-year-old woman presents to the emergency department for evaluation of fever and chills over the last several days along with a progressive rash and diarrhea. She had nasal septoplasty performed 1 week prior and has had nasal packing in place since that time. Her rash has progressed and the skin on her hands is "peeling." She has no significant medical history and takes no medications. Her temperature is 102°F (38.9°C), blood pressure is 84/54 mmHg, pulse is 160/min, respirations are 22/min, and oxygen saturation is 99% on room air. Exam reveals an ill-appearing woman. A diffuse, macular, erythematous rash is noted with desquamation over the patient's hands as shown in Figure A. Bilateral conjunctivitis is noted. The patient's nasal packing is removed revealing copious, foul-smelling, purulent discharge. Which of the following pathogens is likely responsible for this patient's condition?
Borrelia burgdorferi
Neisseria meningitides
Rhinovirus
Rickettsia rickettsii
E
Staphylococcus aureus
This patient with fever, diarrhea, and a diffuse, erythematous rash with prolonged nasal packing in place likely has developed toxic shock syndrome. Toxic shock syndrome occurs most commonly due to infection with Staphylococcus aureus. Toxic shock syndrome (TSS) occurs due to infection with Staphylococcal or Streptococcal species that produce "super-antigen" toxins, such as TSST-1 produced by S. aureus. These toxins bind directly to the MHC-II receptor on T cells with high affinity causing a massive, sustained cytokine release. The result is a "cytokine storm" causing an intense inflammatory reaction and distributive shock. These infections arise in the setting of a retained tampon or retained nasal packing following nasal surgery; however, this is not always the case. The clinical presentation of TSS is characterized by fever, nausea, vomiting, diarrhea, conjunctivitis, and a diffuse "sunburn-like" rash. Desquamation of the palms and soles typically occurs during recovery, 1-2 weeks after the acute phase of the illness. The initial management of TSS begins with broad spectrum antibiotic therapy. Typical regimens include vancomycin, cefepime, and clindamycin. The initial antibiotic regimen should cover methicillin-resistant Staph aureus. Clindamycin is always included due to its ability to suppress bacterial protein production, therefore limiting the production of bacterial toxins. Once a causative organism is identified, antibiotic therapy may be narrowed, though clindamycin is usually administered for the duration of treatment. Figure A is the classic generalized and erythematous macular/desquamating rash of toxic shock syndrome. Incorrect Answers Answer A: Borrelia burgdorferi is a spirochetal bacteria that causes Lyme disease. A erythematous, bulls-eye shaped rash would be expected. Answer B: Neisseria meningitidis causes meningococcemia and meningitis. Fever is common, although a purpuric rash and signs of meningeal irritation would be expected. Patients are often profoundly unstable. Answer C: Rhinovirus is the viral pathogen most commonly responsible for the common cold that causes minor symptoms including a cough, runny nose, fever, and aches. Answer D: Rickettsia rickettsii causes rocky mountain spotted fever which presents with a high fever, headache, malaise, and a centripetal maculopapular rash that starts on the extremities and migrates to the trunk. It is a tick-borne illness. Bullet Summary: Toxic shock syndrome classically develops in patients with prolonged use of nasal packing, and presents with fever, diarrhea, a diffuse erythematous rash, and shock.
Staphylococcus aureus
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A 55-year-old man presents to the emergency department with chest pressure and diaphoresis. His symptoms started at work when he was lifting boxes. He states that he currently feels crushing pressure over his chest. He is given aspirin and an ECG is performed in triage, as seen in Figure A. His past medical history is notable for diabetes and obesity. He has smoked 1 pack of cigarettes per day for the past 30 years. His temperature is 97.9°F (36.6°C), blood pressure is 155/99 mmHg, pulse is 110/min, respirations are 22/min, and oxygen saturation is 98% on room air. Physical exam reveals an uncomfortable, diaphoretic man. His heart and breath sounds are unremarkable. A repeat ECG in the emergency department is performed, also seen in Figure A. The patient’s chest pain is worse when the ECG in the emergency department is taken, compared to the one taken in triage. He is given nitroglycerin, with minimal improvement in his symptoms. A troponin level is drawn. Which of the following is the most appropriate next step in management?
Cardiac catheterization
Chest radiograph
CK-MB level
Heparin
E
Repeat ECG in 10 minutes
This patient is presenting with a pattern of chest pain (pressure and diaphoresis) that is highly concerning for an acute myocardial infarction. This patient initially has ST depressions (in triage) followed by ST segment normalization and possibly hyperacute T waves which raises concern for an acute ST elevation myocardial infarction (STEMI) requiring frequent ECGs to determine if a STEMI evolves. A STEMI presents with crushing substernal chest pain, diaphoresis, chest pressure, nausea, vomiting, and abdominal pain among many other possible presentations. When a patient presents with symptoms concerning for a STEMI, aspirin should be administered and an ECG should be performed. Early in a myocardial infarction, ST depressions with hyperacute T waves may be present. As the infarction evolves, the T waves may become more acute and the ST segment will begin to elevate. There may be a brief period of pseudonormalization where the ST segment appears to be normal. If the patient appears better, this can be reassuring. However, ongoing chest pain in the setting of these findings raises concern for pseudonormalization of the ST segment (an ST segment that is going to continue to rise). Nitroglycerin can be administered to see if the patient can become chest pain-free, and serial ECGs should be performed to determine if a STEMI is present. If symptoms resolve and the ECG is stable, this is reassuring and medical optimization and an elective catheterization may be performed. If the ECG evolves into a STEMI, then cardiac catheterization is indicated emergently. Daga et al. discuss STEMI and its workup. They note the ECG manifestations, and the pathophysiology in addition to the appropriate workup of acute coronary syndrome. They recommend aspirin and catheterization in an acute STEMI. Figure/Illustration A demonstrates an initial ECG with ST depression (blue arrows) followed by pseudonormalization of the ST segment (green arrows) and possibly hyperacute T waves (black arrows), which are concerning for ischemia. Incorrect Answers: Answer A: Cardiac catheterization may be required in this patient; however, if the patient’s chest pain improves or if the troponin is negative, it is unlikely he is having an acute myocardial infarction requiring immediate catheterization. Repeating the ECG will allow for triaging of the patient and will aid in determining if emergency cardiac catheterization is needed. Answer B: Chest radiography is appropriate in the workup of chest pain as it can rule out diagnoses such as pneumothorax or may suggest diagnoses such as an aortic dissection (type A) if there is a widened mediastinum. In this patient, the ST segment changes and characteristic history make acute coronary syndrome a more likely diagnosis, and while a chest radiograph should be performed at some point, repeating the ECG is a more dire initial step in management. Answer C: CK-MB level could be helpful in determining if a myocardial infarction has occurred as it elevates rapidly and also decreases fairly rapidly. This makes it useful when diagnosing a new myocardial infarction after a recent infarction as the troponin may still be elevated from the previous infarction. This patient has no history of recent myocardial infarction, and a repeat ECG is more dire to determine if this patient needs emergent intervention. Of note, the decision for emergent catheterization should not wait on cardiac biomarkers. Answer D: Heparin should not be given empirically to patients in chest pain. It does not necessarily decrease mortality but does increase the risk of bleeding. It is often given to patients with an NSTEMI. Bullet Summary: Pseudonormalization occurs when an ST depression begins to elevate and appears normal in the setting of a myocardial infarction, requiring close monitoring and serial ECGs to ensure that a ST elevation myocardial infarction does not occur.
Repeat ECG in 10 minutes
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A 55-year-old woman is brought to the emergency department by her husband with a 1 hour history of an unremitting headache. The headache started suddenly while she was eating dinner and she says it feels like the “worst headache of my life.” An emergent CT scan of the head without contrast confirms the diagnosis, and a CT angiogram identifies the source of bleeding. The patient undergoes surgical management of her condition. On hospital day 3, she is found to be disoriented to person, place, and time. She also develops nausea and vomiting. Her medical problems consist of heart failure for which she takes furosemide, spironolactone, and metoprolol, which were continued at admission. Her temperature is 99.6°F (37.6°C), blood pressure is 100/60 mmHg, pulse is 112/min, and respirations are 16/min. Physical examination shows poor skin turgor. Capillary refill time is 4 seconds. Serum laboratory results are shown below: Na+: 120 mEq/L Cl-: 92 mEq/L K+: 3.9 mEq/L HCO3-: 26 mEq/L BUN: 32 mg/dL Creatinine: 1.0 mg/dL Serum osmolality is 265 mEq/L and urine osmolality is 340 mEq/L. Urine sodium is 44 mEq/L. Which of the following is the most likely diagnosis?
Cerebral salt wasting
Diuretic overuse
Primary polydipsia
Syndrome of inappropriate anti-diuretic hormone
A
Cerebral salt wasting
This patient presents with a subarachnoid hemorrhage (“worst headache of my life”) which is treated and then subsequently develops nausea, vomiting, and altered mental status with reduced serum sodium concentration, indicative of symptomatic hyponatremia. In the setting of serum hypo-osmolality, urine hyper-osmolality, urine sodium concentration > 20 mEq/L, and hypovolemia (low blood pressure, decreased skin turgor, and prolonged capillary refill), this is indicative of cerebral salt wasting. The mechanism of cerebral salt wasting is unclear, although some hypotheses point to increased release of brain natriuretic peptide after injury leading to suppression of renal sodium reabsorption, or sympathetic nervous system injury resulting in an impaired renin response. Patients classically present several days after cerebral injury or neurosurgery with hypovolemic hyponatremia and hypochloremia. Water retention results in serum hypo-osmolality, hyponatremia, and hypochloremia with urine hyper-osmolality and increased urine sodium excretion. In patients with prolonged cerebral salt wasting, hypokalemia can also occur (potassium may be normal in acute states). Treatment in mild or moderate cases consists of normal saline infusion to correct both volume status and hyponatremia. Treatment in severe cases consists of hypertonic saline. During correction, serum sodium must be monitored closely to prevent central pontine myelinolysis. Cui et al. describe the differences between syndrome of inappropriate anti-diuretic hormone (SIADH) and cerebral salt wasting. They discuss how volume status serves as the key differentiator between these conditions. They recommend paying closer attention to total body electrolyte balance in understanding these conditions. Incorrect Answers: Answer A: Addison disease, also known as primary adrenal insufficiency, results in salt wasting due to decreased mineralocorticoid activity. Because of reduced aldosterone release, patients also have hyperkalemia and metabolic acidosis. In contrast, this patient’s potassium and bicarbonate levels are normal. Treatment is with steroids. Answer C: Diuretic overuse is occasionally observed in patients using loop diuretics. Overuse of loop diuretics can cause hyponatremia, hypokalemia, and hypochloremia. Metabolic alkalosis is commonly seen due to volume contraction. Cerebral salt wasting is more likely in this patient presenting after a recent cerebral injury. Treatment is the cessation of the causative medication. Answer D: Primary polydipsia can cause symptomatic hyponatremia. The urine would be maximally diluted (urine osmolality < 100 mEq/L) due to an appropriate physiological response to hyponatremia and excess free water intake. Treatment is cessation of excessive water consumption. Answer E: Syndrome of inappropriate anti-diuretic hormone (SIADH) presents with identical findings as cerebral salt wasting with the exception of euvolemia as opposed to hypovolemia. An assessment of volume status is essential to differentiate. This patient has clear signs of hypovolemia. Treatment of SIADH is fluid restriction followed by ADH inhibitors, if needed. Bullet Summary: Cerebral salt wasting presents with hyponatremia, hypochloremia, and hypovolemia in the setting of recent cerebral injury or neurosurgery.
nan
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A 67-year-old man presents to the emergency room reporting sudden-onset abdominal and back pain that began 3 hours ago. He was eating dinner when he started feeling severe pain in his abdomen that made him lose his appetite. He denies any diarrhea or hematochezia and says the pain is not affected by movement. He has a history of hypertension, hyperlipidemia, and depression. He currently takes amlodipine and atorvastatin and is compliant with his medications. He has a 45-pack-year smoking history, but he does not drink alcohol or use any illicit drugs. His temperature is 98.6°F (37°C), his blood pressure is 110/64 mmHg, pulse is 97/min, and respirations are 15/min. Physical exam is notable for diffuse abdominal tenderness without rigidity or guarding. During evaluation, the patient becomes diaphoretic and pale and reports that he feels fatigued. Repeat blood pressure is 87/50 and pulse is 127. Intravenous fluid boluses are administered. Which of the following is the most likely diagnosis?
Acute pancreatitis
Aortic dissection
Ischemic colitis
Perforated viscus
E
Ruptured abdominal aortic aneurysm
This patient who presents with sudden-onset abdominal pain, hemodynamic instability (symptomatic hypotension with tachycardia), and significant history of hypertension and smoking most likely has a ruptured abdominal aortic aneurysm (AAA). Abdominal aortic aneurysms are dilations in the vessel wall that can predispose to rupture. Risk factors for AAA include advanced age (> 60 years), smoking history, male sex, and history of atherosclerosis. AAA can be asymptomatic or mildly symptomatic until it markedly expands or ruptures, leading to abdominal pain, back pain, or flank pain. The evaluation of suspected AAA depends on the hemodynamic stability of the patient and prior knowledge (or lack thereof) of the existence of an AAA. A hemodynamically unstable patient with a known history of AAA should be assumed to have a ruptured AAA and undergo surgical intervention without further workup (though bedside ultrasound can support the diagnosis). In an unstable patient without a known history, a bedside ultrasound to confirm the diagnosis should precede surgical intervention. In a hemodynamically stable patient with or without a known AAA history, an abdominal CTA is appropriate. Sakalihasan et al. present a review of the presentation and treatment of abdominal aortic aneurysms. They discuss how patients are often asymptomatic until catastrophic rupture occurs. They recommend immediate surgical repair of the aneurysm during ruptures to prevent death. Incorrect Answers Answer A: Acute pancreatitis also presents with abdominal pain and can present with hemodynamic instability in severe cases of hemorrhagic conversion of pancreatitis. This is a rare entity and is less likely in this patient. Treatment is immediate transcatheter arterial embolization for unstable patients with hemorrhagic pancreatitis. Answer B: Aortic dissection has similar risk factors to AAA (history of hypertension, advanced age) and also presents with acute onset back pain. Dissection normally occurs in the setting of hypertension. This diagnosis is possible but less likely in this man with a history of smoking. Answer C: Ischemic colitis can also present with abdominal pain in a patient with risk factors for atherosclerotic disease. It is associated with hematochezia and diarrhea and is unlikely to cause the level of hypotension in this patient. Treatment is with restoration of blood flow to the ischemic bowel segment or resection if this is not possible. Management is usually centered on medical optimization. Answer D: Perforated viscus is a surgical emergency that can also present acutely with hemodynamic instability and severe abdominal pain. However, signs of peritoneal irritation are usually present (guarding, rigidity). Treatment is with immediate surgical exploration and repair or resection. Bullet Summary: Patients with new-onset back and abdominal pain and a positive smoking history that progresses to hemodynamic instability should be suspected of having a ruptured abdominal aortic aneurysm.
Ruptured abdominal aortic aneurysm
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A 57-year-old man presents to the emergency department with several days of malaise, weakness, and night sweats. Today, he experienced a headache with blurry vision, thus prompting his presentation. The patient has a history of diabetes and is followed closely by an endocrinologist. Otherwise, he has lost 10 pounds over the past month. His temperature is 100°F (37.8°C), blood pressure is 122/90 mmHg, pulse is 84/min, respirations are 16/min, and oxygen saturation is 99% on room air. Physical exam is notable for a thin man but is otherwise unremarkable. His visual acuity is 20/100 in both eyes, though he says that he normally has 20/20 vision. His gait is mildly ataxic as well, but his neurological exam is otherwise nonfocal. Laboratory studies are ordered as seen below. Hemoglobin: 10 g/dL Hematocrit: 31% Leukocyte count: 57,500/mm^3 with 35% blasts Platelet count: 109,000/mm^3 Serum: Na+: 139 mEq/L Cl-: 100 mEq/L K+: 4.1 Eq/L HCO3-: 22 mEq/L BUN: 20 mg/dL Glucose: 99 mg/dL Creatinine: 1.1 mg/dL Calcium: 10.0 mEq/L Which of the following is the most likely diagnosis?
Acute lymphoblastic leukemia
Acute myelogenous leukemia
Chronic lymphocytic leukemia
Chronic myelogenous leukemia
D
Chronic myelogenous leukemia
This patient is presenting with symptoms of malignancy (malaise, weakness, and night sweats) and has a white blood cell count > 50,000/mm^3 in the setting of > 20% blasts, which are concerning for chronic myelogenous leukemia (CML). Given the blurry vision and ataxia in the setting of increased blasts, this patient may be suffering from a hyperviscosity syndrome secondary to a blast crisis, which can be seen in CML. CML is a neoplastic proliferation of myeloid stem cells. Patients are often asymptomatic and may present with a white blood cell count > 50,000/mm^3. There are many different presentations. The chronic phase presents with < 10% blasts and is often asymptomatic. The accelerated phase has increased blasts, and patients present with fatigue, weight loss, malaise, night sweats, abdominal pain, and hepatosplenomegaly. Finally, the blast phase presents similar to the accelerated phase but with more severe symptoms. The blast phase may evolve into a blast crisis (> 20% blasts) which could cause a hyperviscosity syndrome. Hypervicosity syndrome may require IV fluids and leukapheresis. The most common translocation in CML is the 9;22 translocation which forms the BCR:ABL gene product. Jain et al. review CML. They note the outcomes in patients in the blast phase in CML and how there are more poor outcomes associated with this phase. They recommend paying close attention and obtaining a CBC with a differential to determine the percent blasts. Incorrect Answers: Answer A: Acute lymphoblastic leukemia is more common in pediatric patients and presents with pancytopenia, fatigue, recurrent infections (from leukopenia), and bleeding (from thrombocytopenia). Other findings include systemic symptoms, malaise, fever, bone pain, lymphadenopathy, splenomegaly, and hepatomegaly. Answer B: Acute myelogenous leukemia is a malignancy of myeloblasts and presents in the elderly. Patients will present with fatigue, malaise, weight loss, anorexia, fever, leukocytosis, anemia (causing fatigue), thrombocytopenia (causing bleeding), and neutropenia (leading to life-threatening infections). Auer rods can be seen on peripheral smear. Answer C: Chronic lymphocytic leukemia is a monoclonal proliferation of incompetent mature B cells that occurs in elderly men and is the most common form of leukemia in the west. Patients are often asymptomatic and may have a white blood cell count > 50,000/mm^3. When symptomatic, patients may experience chills, weight loss, and weakness (note these symptoms are nonspecific). Peripheral smear will show smudge cells. Answer E: Multiple myeloma is common in the elderly and presents with CRAB: hyperCalcemia, Renal insufficiency, Anemia, and lytic Bone lesions/Back pain. The diagnosis is supported with protein electrophoresis and confirmed when biopsy demonstrates increased plasma cells. Bullet Summary: Chronic myelogenous leukemia presents with increased white blood cells which may be >50,000/mm^3 (possibly with elevated blasts).
Multiple myeloma
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A 6-week-old boy is referred to a pediatric hematologist-oncologist for further evaluation following an abnormal newborn hemoglobinopathy screening. The father’s brother died of sickle cell anemia at an early age. Parental studies reveal that both mother and father are carriers of the sickle cell gene. The patient's temperature is 98.6°F (37.0°C), blood pressure is 80/45 mmHg, pulse is 130/min, and respirations are 25/min. The physician decides to repeat testing for the patient and his 3-year-old sister. The results are shown in Figure A. The physician begins counseling the family. Which of the following is the most likely complication that the patient may have in the future?
Stroke
Hematuria
Splenectomy
Infection
B
Hematuria
Based on the presence of both hemoglobin (Hb) A and S on gel electrophoresis, this patient has sickle cell trait. Episodes of painless hematuria are commonly seen in these patients. Sickle cell trait is a condition that refers to carrying 1 normal and 1 abnormal beta-globin allele. On electrophoresis, this will present as 3 different bands consisting of about 50-60% HbA, 35-45% HbS, and < 2% HbF (fetal hemoglobin). HbF decreases significantly after the first 6 weeks of life. Individuals with sickle cell trait tend to be asymptomatic and do not have a decreased lifespan compared to the general population. However, they are at higher risk of some conditions. Patients are at increased risk of rhabdomyolysis during strenuous physical activity and may have episodic painless hematuria due to renal papillary necrosis. The hyperosmolar, acidotic, and hypoxic environment of the renal medulla is thought to trigger red cell sickling which results in ischemia and subsequent hematuria. Patients with sickle cell trait are managed similarly to the general population. These patients should receive reproductive counseling and use appropriate preventive measures to prevent dehydration during intensive exercise. Hulsizer et al. studied the association between sickle cell trait and common medical conditions using insurance claims data. The authors found that sickle cell trait is associated with increased risks of diabetes, hypertension, heart disease, chronic kidney disease, and retinopathy. The authors recommend further evaluation of these associations using prospective studies derived from clinical data. Figure/Illustration A shows gel electrophoresis with an example of a normal (95-98% HbA and < 2% HbF), sickle cell trait (50-60% HbA, 35-45% HbS, and < 2% HbF), and sickle cell anemia (85-95% HbS and 5-15% HbF) patient from left to right. Incorrect Answers: Answer A: Diabetes has not been associated with sickle cell trait in the majority of studies. Answer C: Infection is a complication of sickle cell anemia. Mechanisms include functional asplenism, reduced tissue perfusion during a sickle crisis, and chronic transfusions with an indwelling catheter. Common infections include bacteremia, meningitis, and pneumonia due to Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitis. Sickle cell trait is not clearly associated with an increased risk of infection and actually confers some protection against malaria. Answer D: Splenectomy from auto-infarction is often seen in sickle cell anemia, but it is rare with sickle cell trait. In sickle cell anemia, splenic sequestration of sickled red blood cells causes infarction, leading to progressive atrophy and functional hyposplenism. Most individuals with sickle cell disease are functionally asplenic by age 2 to 4. The spleen subsequently cannot phagocytose encapsulated organisms properly, which predisposes sickle cell patients to infection. Answer E: Stroke is a complication of sickle cell anemia but not sickle cell trait. This is due to the increased viscosity of the blood and reduced deformability of red cells in patients with sickle cell anemia. Bullet Summary: Sickle cell trait is associated with episodes of painless hematuria.
nan
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A 24-year-old woman presents to clinic for a routine annual exam. She has generally been feeling well, but notes feeling intermittent palpitations over the past few months. Her past medical history is unremarkable and she is not currently taking any medications. She denies drinking alcohol, smoking cigarettes, or using recreational drugs. She attributes her palpitations to recently drinking more caffeine, but would like to obtain an electrocardiogram since her symptoms are worrisome and affecting her ability to concentrate on her career as a fitness instructor. Her temperature is 36.9°C (98.4°F), blood pressure is 116/76 mmHg, pulse is 55/min, respirations are 12/min, and oxygen saturation is 98% on room air. Her electrocardiogram is shown in Figure A. Which of the following is the most likely diagnosis?
First degree atrioventricular block
Sinus bradycardia
Second degree atrioventricular block, Mobitz type II
Second degree atrioventricular block, Mobitz type I
D
Second degree atrioventricular block, Mobitz type I
This patient's progressively increasing PR intervals until a P wave is not followed by a QRS complex on ECG is consistent with second degree atrioventricular (AV) block, Mobitz type I, also referred to as Wenckebach phenomenon. Second degree AV block, Mobitz type I can occur in a variety of settings, such as in the context of digoxin, beta blockers, calcium channel blockers, or increased vagal tone. On ECG, there is progressive lengthening of the PR intervals until a P wave is not followed by a QRS complex (a dropped beat); then, the PR interval resets. Patients are typically asymptomatic, but should undergo evaluation for an underlying etiology of the dysrhythmia, such as electrolyte imbalances, medication side effect, or structural heart disease, as well as a follow-up ECG to evaluate for any progression. If the dysrhythmia is caused by medication, the offending agent should be discontinued, but otherwise there are no specific treatments necessary for asymptomatic patients. Patients who are symptomatic and hemodynamically stable can undergo telemetry monitoring and receive atropine with limited efficacy. Patients who are hemodynamically unstable and not responsive to atropine may need cardiac pacing. Generally, there is a low risk of progression to third degree AV block, and a pacemaker is rarely required (in contrast to second degree heart block type II). Barold and Hayes review second-degree heart block. They note the changes in AV node conduction time on ECG in this condition. They recommend using the correct definition of the different heart blocks for the correct underlying treatment. Figure/Illustration A is an ECG showing PR intervals that gradually prolong until a P wave is seen not followed by a QRS complex (note the blue bars that show the progressive lengthening of the PR intervals until the dropped beat). Incorrect Answers: Answer A: First degree AV block is seen on ECG with prolonged PR intervals greater than 0.2 sec, but a consistent 1:1 ratio of P waves to QRS complexes. This finding is typically found in younger patients with increased vagal tone or in athletes, and does not require treatment. Answer C: Second degree AV block, Mobitz type II is seen with fixed PR intervals and occasional dropped QRS complexes. Patients with Mobitz II sometimes present with syncope and frequently progress to third degree AV block. This finding may be associated with fibrotic changes to the conduction system or from acute, subacute, or prior myocardial infarction, and should be treated with placement of a pacemaker in most cases. Answer D: Sinus bradycardia findings on ECG include normal sinus rhythm with a ventricular rate of less than 60 beats per minute. Patients with sinus bradycardia may be asymptomatic, but may also present with syncope, lightheadedness, chest pain, or hypotension. No treatment is required if the patient is asymptomatic, but atropine may be given to increase the heart rate in symptomatic cases. Pacemaker placement is required for patients with chronic symptoms. Answer E: Third-degree AV block occurs when the atria and ventricles depolarize independently on ECG. There is no relationship between P waves and QRS complexes. Patients may present with syncope, dizziness, acute heart failure, or hypotension. The treatment is pacemaker placement. Bullet Summary: Second degree atrioventricular block, Mobitz type I, is characterized by progressively lengthening PR intervals until a P wave is not followed by a QRS complex.
nan
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A 6-week-old girl presents with her parents to the pediatrician for blood-streaked diapers. The patient is breastfed every 2-3 hours and voids 10-12 times daily. The patient previously had several soft stools per day that ranged in color from mustard yellow to dark green. Over the past 2 days, the patient’s stools have become looser and streaked with blood. The patient has also regurgitated several of her feedings in the last few days. Neither the vomit nor bloody stools have seemed to bother the patient. The patient’s weight continues to trend along the 50th percentile. The patient’s temperature is 98.4°F (36.9°C), blood pressure is 66/51 mmHg, pulse is 127/min, and respirations are 26/min. On physical exam, the patient appears well nourished and is cooing. Her abdomen is soft and non-tender. Physical exam reveals the finding seen in Figure A. Which of the following is the most likely etiology of this patient’s presentation?
Congenital enzyme deficiency
Passage of gastric contents into esophagus
Non-IgE-mediated immunologic reaction
Hypertrophy of sphincter muscle
C
Non-IgE-mediated immunologic reaction
This patient presents with new regurgitation, blood-streaked stools, and eczema, which is consistent with proctocolitis secondary to milk-protein allergy. This type of delayed onset milk-protein allergy is a non-IgE mediated immunologic reaction. Allergy to cow’s milk is the most common food allergy in children and encompasses both IgE-mediated and non-IgE-mediated reactions. IgE-mediated reactions occur minutes to 2 hours after ingestion of the allergen and range from mild symptoms to anaphylaxis. Milk protein-induced enterocolitis and proctocolitis are non-IgE-mediated reactions that present by 6 months of life with regurgitation or vomiting and bloody stools. Management of milk protein-induced enterocolitis and proctocolitis involves the elimination of cow’s milk and soy from the mother’s diet if the infant is breastfed or switching to a hydrolyzed formula if the infant is formula fed. Giannetti et al. review the evidence regarding the diagnosis and treatment of milk protein allergy in infants. They discuss how the selective elimination of certain proteins from the diet is usually effective. They recommend considering oral immunotherapy as an adjuvant in the treatment of this disease. Figure/Illustration A is a clinical photograph demonstrating the physical exam finding of eczema (red circle), which is commonly found in infants with an allergy to milk protein. Incorrect Answers: Answer A: Congenital enzyme deficiency describes lactose intolerance, in which there is an absence of lactase activity in the small intestine. Lactose intolerance classically presents with abdominal pain, bloating, flatulence, and diarrhea. Treatment is with diet modification. Answer C: Hypertrophy of the sphincter muscle describes the pathogenesis of pyloric stenosis, which presents in patients between 3- and 6-weeks of age with non-bilious, projectile vomiting. Treatment is with surgical pyloromyotomy. Answer D: Passage of gastric contents into the esophagus describes gastroesophageal reflux, which may present with regurgitation or vomiting. Treatment is with positioning but can include proton pump inhibitors in refractory cases. Answer E: Telescoping of the intestine describes intussusception, which presents with sudden-onset severe, crampy, abdominal pain with non-bilious vomiting and “currant jelly” stools. Intussusception is rare before 3 months of age and presents with intermittent episodes of pain. Initial treatment is with air or contrast enema. Bullet Summary: Milk-protein allergy presents in infants between 2- to 8-weeks of age with regurgitation or vomiting, blood-streaked stools, and eczema, and management involves the elimination of cow’s milk and soy from the infant’s diet.
nan
https://step2.medbullets.com/testview?qid=210075
A 55-year-old man presents to the emergency department with chest pain and shortness of breath. He has a medical history of hypertension, diabetes, and obesity. His temperature is 98.6°F (37.0°C), blood pressure is 177/118 mmHg, pulse is 127/min, respirations are 11/min, and oxygen saturation is 98% on room air. An ECG is performed and notable for ST elevation in leads II, III, and aVF. The patient is treated appropriately and transferred to the medical floor. On the 2nd day of his hospitalization, the patient has abdominal pain. His serum lipase is 272 U/L and his creatinine is 1.6 mg/dL. A physical exam is notable for the finding in Figure A. He is requesting pain medication for his abdominal pain. Which of the following is the most likely underlying diagnosis?
Cholesterol embolism
Heart failure and reinfarction
Medication side effect
Pancreatitis
A
Cholesterol embolism
This patient is presenting with abdominal pain and livedo reticularis after a catheterization procedure. These symptoms are suggestive of a cholesterol embolism. A cholesterol embolism typically presents after any vascular procedure due to a dislodged cholesterol emboli. Symptoms include livedo reticularis, gastrointestinal problems such as mesenteric ischemia or pancreatitis, and acute kidney injury. Patients who have undergone cardiac catheterization should be monitored for the development of this complication. Treatment is centered around treating the current complications, often using supportive therapy as they self-resolve. In addition, patients should be treated carefully to prevent recurrent embolism. Maningding and Kermani review the evidence regarding the presentation of vasculitis mimics. They discuss how cholesterol embolism can cause diverse systemic symptoms and livedo reticularis. They recommend carefully considering the history of each patient. Figure/Illustration A is a clinical photograph demonstrating a netlike reticular bluish discoloration of the skin (red circle). These findings are consistent with livedo reticularis. Incorrect Answers: Answer B: Heart failure and reinfarction could present with renal failure but would likely present with other symptoms of heart failure as well (pulmonary crackles, edema, and jugular venous distension). Treatment of a repeat infarct involves catheterization and fluid overload involves volume optimization. Answer C: Medication side effects could explain this patient's livedo reticularis, but the drugs that may cause this (certain antivirals and amantadine) would not have been given to this patient. These medications also typically do not cause abdominal pain. Answer D: Pancreatitis presents with abdominal pain and an elevated lipase; however, it is not the underlying pathology for this patient's presentation but rather is a complication of the cholesterol embolism. Treatment of pancreatitis is with gastrointestinal rest and fluid support. Answer E: Renal failure is another complication in this patient secondary to the cholesterol embolism as revealed by the elevated creatinine, but is not the underlying pathology. Treatment is with renal replacement therapy if renal function does not return promptly. Bullet Summary: Cholesterol embolism presents after a vascular procedure with livedo reticularis, gastrointestinal complications, and acute kidney injury.
Renal failure
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A 45-year-old man presents to a physician due to difficulty swallowing and slurred speech. For the past 3 months, the patient has been coughing when he drinks liquids. His speech also sounds different according to his wife. The patient has no significant medical history. His temperature is 98.6°F (37.0°C), blood pressure is 110/80 mmHg, pulse is 60/min, and respirations are 12/min. On physical exam, he is unable to fully extend his tongue, and there are fasciculations on his tongue. When tapping the mandible at the chin while the mouth is held open, there is pronounced upward movement of the lower jaw. Sensory exam reveals no deficits. Which of the following therapies is most likely to improve survival for this patient's disease process?
Baclofen
Dextromethorphan and quinidine
Edaravone
Riluzole
D
Riluzole
This patient with dysphagia (difficulty swallowing water), dysarthria (altered and slurred speech), lower motor neuron deficits (tongue fasciculations and weakness), upper motor neuron deficits (exaggerated jaw jerk reflex), and preserved sensory function most likely has amyotrophic lateral sclerosis (ALS). Riluzole, a glutamate inhibitor, is currently the only Food and Drug Administration (FDA) approved medication that improves survival in patients with ALS. ALS is a neurodegenerative disease that affects both upper and lower motor neurons. Upper motor neuron signs include spasticity and hyperreflexia, while lower motor neuron signs include muscle atrophy and fasciculations (spontaneous, involuntary muscle contraction and relaxation). In patients with bulbar-onset ALS, the initial clinical presentation is dysarthria, dysphagia, tongue atrophy, and abnormal jaw jerk reflex. Riluzole decreases the rate of glutamate release from presynaptic neurons, which is thought to decrease excitatory neurotoxicity. Riluzole improves survival by 2-6 months in patients with ALS and may have a greater survival benefit in patients with bulbar-onset ALS. Masori and Van Damme review the genetics, pathophysiology, classic clinical presentation, extra-motor manifestations, diagnostic workup, and treatment for ALS. They discuss how riluzole provides a survival benefit in patients with this disease. They recommend treating patients with ALS using riluzole as tolerated. Incorrect Answers: Answer A: Baclofen is a gamma-aminobutyric acid (GABA) agonist that reduces the release of excitatory neurotransmitters to decrease spasticity in ALS. Along with tizanidine, baclofen is a first-line symptomatic treatment for spasticity in ALS. Baclofen does not improve survival in ALS. Answer B: Dextromethorphan and quinidine is a combination of a sigma opioid receptor agonist and a sodium channel blocker used to control pseudobulbar affect in patients with ALS. Pseudobulbar affect is a sudden uncontrollable outburst of laughter or tearfulness that affects almost half of patients with ALS. Dextromethorphan and quinidine has not been shown to improve survival in ALS. Answer C: Edaravone is an intravenous antioxidant that may decrease the progression of early ALS. Although it is now FDA approved, studies have not yet shown that edaravone improves survival in patients with ALS. Further studies will be needed in order to determine the risks and benefits of using this drug. Answer E: Tizanidine is a central alpha-2-adrenergic receptor agonist that decreases spasticity in ALS by increasing presynaptic inhibition of motor neurons. Excess spasticity in ALS can negatively impact coordination, ambulation, and other activities of daily living. Tizanidine and other antispastic medications do not improve survival in patients with ALS. Bullet Summary: Riluzole is a glutamate inhibitor that improves survival in patients with amyotrophic lateral sclerosis.
Tizanidine
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A 7-year-old boy is brought to the emergency department by his parents for worsening symptoms of previously diagnosed acute sinusitis. Initially, the pediatrician prescribed decongestants and sent the patient home. Since then, the patient has developed a nasal discharge with worsening pain. The patient has a medical history of asthma which is well-controlled with albuterol. His temperature is 99.5°F (37.5°C), blood pressure is 90/48 mmHg, pulse is 124/min, respirations are 17/min, and oxygen saturation is 98% on room air. The cardiopulmonary exam is within normal limits. Inspection of the patient's nose reveals a unilateral purulent discharge mixed with blood. The rest of the patient's exam is within normal limits. Which of the following is the most likely diagnosis?
Bleeding and infected vessel of Kiesselbach plexus
Foreign body obstruction
Nasopharyngeal carcinoma
Septal perforation
B
Foreign body obstruction
This patient presents with unilateral bloody/purulent discharge from the nose suggesting a diagnosis of a nasal foreign body. The most common cause of unilateral nasal symptoms in a pediatric patient is a nasal foreign body. Nasal foreign bodies tend to be unilateral (though bilateral symptoms are plausible) and present with a purulent and bloody discharge. The treatment of choice is to remove the object which may be done in the emergency department or operating room depending on how deep the object is. Visualization of the object should be attempted when possible in order to allow for safe retrieval. Krulewitz and Fix review the evidence regarding the diagnosis and treatment of patients with epistaxis. They discuss how foreign body insertion is a major risk factor for the development of epistaxis. They recommend assessment of airway and breathing in the setting of severe epistaxis. Incorrect Answers: Answer A: A bleeding and infected vessel of the Kiesselbach plexus could suggest a complicated case of epistaxis. The persistent, unilateral symptoms in this patient point toward a different diagnosis. A high index of suspicion for foreign bodies should be suspected in children. Answer C: Nasopharyngeal carcinoma tends to occur in young male patients and can be associated with infectious mononucleosis. Epistaxis can be a symptom and the mass can be visualized. This disease tends to present in older patients and is far less common than foreign bodies. Treatment is with surgical resection. Answer D: Septal perforation can occur secondary to repeat cocaine use but would not present with unilateral nasal discharge in a pediatric patient. It would be highly unlikely in a pediatric patient with no history of drug use. Treatment is with cessation of the offending agent. Answer E: Sinusitis with bacterial superinfection would typically present with systemic symptoms as well as bilateral symptoms. Though this diagnosis is possible, it is less likely than a foreign body. Treatment is with systemic antibiotic therapy. Bullet Summary: A nasal foreign body presents with unilateral bloody/purulent nasal discharge.
Sinusitis with bacterial superinfection
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A 64-year-old woman presents to the emergency room with whole-body itching. She noticed her symptoms while in the bathtub at home. She has never had symptoms like this before. Over the previous several months she has had episodes of joint swelling and pain in her hands as well as redness, burning pain, and swelling of her hands and feet. Her medical history is significant for type 2 diabetes mellitus, hypertension, and osteoporosis for which she takes metformin, enalapril, and alendronate. She was found to have a deep vein thrombosis of her left leg 3 months prior to presentation. Her temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 135/85 mmHg, and respirations are 13/min. Physical exam is notable for a woman in discomfort with excoriations over the skin on her forearms. Laboratory tests are shown below. Serum: Na+: 135 mEq/L Cl-: 100 mEq/L K+: 5.0 mEq/L HCO3-: 22 mEq/L BUN: 19 mg/dL Glucose: 130 mg/dL Creatinine: 1.0 mg/dL Hematocrit: 64% Leukocyte count: 19,000 cells/mm^3 with normal differential Platelet count: 900,000/mm^3 Which of the following is the most appropriate long-term treatment?
Cyclophosphamide
Diphenhydramine
Febuxostat
Hydroxyurea
D
Hydroxyurea
This patient presents with polycythemia, leukocytosis, and thrombocytosis in the setting of pruritus after bathing, as well as episodes suggestive of acute gout flares. These findings are consistent with polycythemia vera, which can be treated with hydroxyurea. Polycythemia vera is a malignancy of the bone marrow that results in the overproduction of red blood cells, platelets, and white blood cells. Classic symptoms include pruritus after hot baths as well as swelling, burning pain, and rubor of the hands and feet (erythromelalgia). Patients may also have gout due to increased cell turnover leading to hyperuricemia. Older patients (> 60 years old) and those with prior thrombosis should be treated with a myelosuppressive agent, most commonly hydroxyurea with or without aspirin. Therapeutic phlebotomy is often indicated on these patients. Stuart and Viera review the evidence regarding the diagnosis and treatment of polycythemia vera. They discuss how treatment includes phlebotomy with the possible addition of myelosuppressive agents. They recommend consultation with a hematologist for any patients with this syndrome. Incorrect Answers: Answer A: Cyclophosphamide is a chemotherapeutic agent that is used in the treatment of certain leukemias and lymphomas, as well as severe symptoms of autoimmune disease. A malignancy would present with vague systemic symptoms, weight loss, malaise, and abnormalities detected on CBC. Cyclophosphamide is not used in the treatment of polycythemia vera. Answer B: Diphenhydramine is a 1st-generation antihistamine that can be used to treat pruritus, but would not be indicated for the treatment of polycythemia vera which is the underlying condition. This treatment represents a symptomatic treatment versus an agent that addresses the underlying cause. Answer C: Febuxostat is a xanthine oxidase inhibitor that is used to reduce uric acid levels in the management of chronic gout. Gout would present with acute severe joint pain (typically the great toe) with risk factors of alcohol use, obesity, and thiazide use. It is not used in the acute management of gout or in polycythemia vera. Answer E: Prednisone is a glucocorticoid that is used in many different clinical situations but is not used in the management of polycythemia vera. It could be used in acute inflammatory conditions (like Crohn disease or granulomatosis with polyangiitis) or to reduce inflammation during a flare of asthma or COPD. Bullet Summary: Polycythemia vera presents with pruritus after bathing, erythromelalgia, and gout and should be treated with hydroxyurea with or without aspirin.
Prednisone
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A 66-year-old man presents to the emergency department with shortness of breath. He typically has poor exercise tolerance and is only able to walk from his house to his mailbox before becoming dyspneic. He now has shortness of breath even at rest. He had a cold recently but has no new medical problems. His past medical history is notable for a myocardial infarction 7 years ago, COPD, heart failure with a left ventricular ejection fraction of 22%, obesity, diabetes, and peripheral vascular disease. His temperature is 97.7°F (36.5°C), blood pressure is 124/82 mmHg, pulse is 140/min, respirations are 32/min, and oxygen saturation is 78% on room air. Physical exam reveals an uncomfortable man with increased work of breathing. He demonstrates bilateral crackles and wheezing on pulmonary exam. The patient is started on BiPAP, and his oxygen saturation improves to 94%. An ECG is performed, as seen in Figure A, and a chest radiograph is performed, as seen in Figure B. Which of the following is the most appropriate next step in management?
Digoxin
Albuterol and prednisone
Intubation
Metoprolol
A
Digoxin
This patient with a history of heart failure with reduced ejection fraction is presenting with respiratory distress (increased work of breathing, hypoxia, wheezes, and crackles) in the setting of atrial fibrillation (ECG with irregularly irregular rhythm without P waves) with rapid ventricular response and pulmonary edema on chest radiograph. Digoxin is an appropriate medication that can both control the heart rate in atrial fibrillation and increase cardiac contractility and potentially improve respiratory symptoms in this decompensated patient with heart failure. Atrial fibrillation presents on ECG with an irregularly irregular rhythm without discernible P waves. The initial management of atrial fibrillation with rapid ventricular response is rate control. If the patient is stable, possible first-line agents include beta-blockers (e.g., metoprolol) or calcium channel blockers (e.g., diltiazem). These agents may reduce cardiac contractility and worsen complications such as pulmonary edema in the setting of heart failure with reduced ejection fraction when the patient is in a decompensated state. If the patient has a reduced ejection fraction and requires rate control, then digoxin is a highly effective medication that blocks the AV node and increases cardiac contractility. Any hemodynamically unstable patient with atrial fibrillation with rapid ventricular response should immediately be cardioverted. Moskowitz et. al review the management of atrial fibrillation. They note that in the management of atrial fibrillation with rapid ventricular response, metoprolol is commonly used in the intensive care setting, is more successful than amiodarone, and had better rate control when compared to diltiazem. Ehle et. al note that digoxin is approved to control ventricular response in patients with chronic atrial fibrillation and is the only oral agent with positive inotropic effects approved for the management of mild to moderate heart failure. Figure/Illustration A is an ECG demonstrating an irregularly irregular rate/rhythm (note the spacing of the blue arrows indicating the QRS complexes) without P waves, which is suggestive of atrial fibrillation. Figure B is a chest radiograph demonstrating bilateral, hazy interstitial infiltrates, which are concerning for pulmonary edema. Incorrect Answers: Answer A: Albuterol and prednisone would be appropriate management of a COPD flare, which presents in a patient with a history of smoking with dyspnea, wheezing, increased work of breathing, and a barrel chest. While this patient’s wheezing may be secondary to his COPD, it is much more likely to be a cardiac wheeze given his chest radiograph findings including pulmonary edema. Treating his atrial fibrillation is more likely to alleviate his pulmonary symptoms. Other treatments in COPD include ipratropium and BiPAP. Answer C: Diltiazem would be an appropriate rate control agent for atrial fibrillation with rapid ventricular response, particularly in the setting of a COPD flare as it will not impair the mechanism of albuterol (which should be given as a nebulizer in COPD flares). In this patient, further impairing cardiac contractility with a calcium channel blocker may worsen his underlying pulmonary edema (given his reduced ejection fraction). Answer D: Intubation would be indicated if this patient’s respiratory status were worsening, including failure to oxygenate, failure to ventilate, or impending respiratory failure. This patient’s respiratory status has improved on BiPAP; thus intubation is not needed. Answer E: Metoprolol, like diltiazem, would reduce cardiac contractility and may worsen pulmonary edema, in particular, in a patient with a reduced ejection fraction. It also may cause COPD symptoms to emerge, as beta-2 blockade may cause bronchoconstriction (even though metoprolol is beta-1 selective). It would be indicated as outpatient rate control for a patient with atrial fibrillation who can tolerate a beta-blocker, and is preferred in patients with a history of acute coronary syndrome, as it reduces mortality. Bullet Summary: Patients with atrial fibrillation with rapid ventricular response with a reduced ejection fraction may be optimally managed with digoxin, which blocks the AV node and increases cardiac contractility.
nan
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A 31-year-old woman presents to her obstetrician for her first prenatal visit after having a positive home pregnancy test 1 week ago. Her last menstrual period was 8 weeks ago. The patient has a medical history of type 1 diabetes mellitus since childhood and her home medications include insulin. Her hemoglobin A1c 2 weeks ago was 13.7%. At that time, she was also found to have microalbuminuria on routine urinalysis. Her primary care provider prescribed lisinopril but the patient has not yet started taking it. The patient’s brother is autistic but her family history is otherwise unremarkable. Her temperature is 98.6°F (37.0°C), blood pressure is 124/81 mmHg, pulse is 75/min, and respirations are 14/min. A physical exam is unremarkable. This fetus is most likely at increased risk for which of the following complications?
Neonatal hyperglycemia
Neural tube defect
Oligohydramnios
Post-term delivery
B
Neural tube defect
This patient has type 1 diabetes mellitus and presents in early pregnancy with a markedly elevated hemoglobin A1c, suggesting poor glycemic control. Poor glycemic control increases the risk of a neural tube defect in the fetus. Infants of diabetic mothers (IDM) are at increased risk of many congenital malformations as well as postnatal complications. These risks are more likely if there is poor maternal glycemic control, especially in the periconceptional period. Poorly controlled hemoglobin A1c and microalbuminuria on urinalysis indicate long-standing poor glycemic control and indicate a poor prognosis. The most common anomalies in IDM are cardiovascular defects such as septal defects and transposition of the great arteries as well as neural tube defects such as anencephaly and spina bifida. Sacral agenesis, or a poorly developed caudal spine and spinal cord, as well as small left colon syndrome due to a meconium plug, are other common malformations seen in IDM. General complications in IDM include prematurity, intrauterine growth restriction or macrosomia, and miscarriage. Risk is minimized by tight glycemic control throughout pregnancy. Dean et al. summarize the evidence surrounding micronutrients and obesity in pregnancy. They found that patients with diabetes had a higher risk of having infants with a neural tube or a congenital heart defect. They recommend using folic acid supplementation in all patients to reduce the risk of neural tube defects. Incorrect Answers: Answer A: Aneuoploidy risk is not increased in IDM. This complication is associated with advanced maternal age. This patient is at baseline risk since she is below the “advanced maternal age” cutoff of 35 years. Some of the values used in aneuploidy screening (maternal serum alpha­fetoprotein, estriol, and inhibin A) are generally lower in women with diabetes. Guidelines specific to this patient population should be used for the aneuploidy screen so as to avoid false positive results. Answer B: Neonatal hyperglycemia is not seen in IDM. Patients may experience hypoglycemia. This is due to the crossing of glucose from the mother to the fetus via the placenta and subsequent hyperinsulinemia and ß-cell hyperplasia from the fetus. After delivery, the high insulin levels cause hypoglycemia because there is no longer an overabundant source of glucose. Hyperglycemia in infants is much less common and can be due to glucose infusion or sepsis. Answer D: Oligohydramnios is not specifically associated with maternal hyperglycemia. Instead, patients may experience polyhydramnios. The mechanism for increased amniotic fluid is unclear, though some speculate that fetal hyperglycemia results in polyuria and thus polyhydramnios. Severe oligohydramnios can cause pulmonary hypoplasia and clubfoot as well as cord compression, while polyhydramnios is associated with cord prolapse, placental abruption, and prematurity. Answer E: Post-term delivery occurs after 42 weeks gestation. Maternal diabetes typically causes prematurity. Post-term delivery is associated with fetal malnutrition, hypoxemia, and oligohydramnios due to suboptimal functioning of the placenta. The risk of meconium aspiration increases as gestational age increases. Induction of labor is generally performed at 42 weeks to avoid these complications. Bullet Summary: Maternal hyperglycemia from diabetes mellitus is associated with congenital heart defects, neural tube defects, and small left colon syndrome.
nan
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An 8-week-old boy presents with his mother to the pediatrician for a well visit. The patient has been breastfed since birth, and usually feeds for 30 minutes every 2-3 hours. The patient’s mother is concerned that her milk production is not keeping up with the patient’s nutritional requirements. She reports that about 2 weeks ago the patient began regurgitating breastmilk through his nose and mouth after some feeds. He seems mildly upset during the episodes of regurgitation but usually settles down quickly and is hungry again soon afterwards. His mother has already tried limiting the volume of each feed, which seems to have reduced the frequency of regurgitation. She denies any diarrhea, hematochezia, or family history of food allergies. Her older son had a similar problem with vomiting that resolved around 12 months of age. Four weeks ago, the patient’s height and weight were in the 40th and 34th percentiles, respectively. His height and weight are now respectively in the 37th and 36th percentiles. His temperature is 98.6°F (37°C), blood pressure is 72/49 mmHg, pulse is 121/min, and respirations are 32/min. On physical exam, the patient is cooing in his mother’s lap and smiles reciprocally with her. He lifts his head and shoulders off the examination table when placed in the supine position. His abdomen is soft, non-tender and non-distended. Bowel sounds are normoactive. Which of the following is the most appropriate next step in management?
Initiate proton pump inhibitor
Obtain abdominal ultrasound
Reassurance and counseling on positioning
Recommend modification of mother’s diet
C
Reassurance and counseling on positioning
This patient presents with regurgitation after feeding, appropriate appetite after episodes of regurgitation, and good weight gain, which suggests a diagnosis of uncomplicated gastroesophageal reflux. The most appropriate next step in management is reassurance and counseling on proper positioning. Physiologic or uncomplicated gastroesophageal reflux usually presents in patients in the first few months of life with frequent regurgitation or vomiting, sustained appetite after regurgitation, and minimal irritability. The most appropriate first step in management is reassurance and counseling parents to position patients upright for 20-30 minutes after feeding. For patients with persistent symptoms, parents should be encouraged to provide lower volume feeds more frequently throughout the day, and consider a milk-free diet to address possible underlying cow’s milk allergy. Treatment with a proton pump inhibitor is reserved for intractable cases. For most patients, gastroesophageal reflux self-resolves by 1 year of age. Chabra and Peeples review the evidence regarding the treatment of neonatal GERD. They discuss how alterations in the infant's environment and feeding pattern are effective in most cases. They recommend proton pump inhibitors if conservative treatments are not effective. Incorrect Answers: Answer A: Initiating a proton pump inhibitor is appropriate for infants with gastroesophageal reflux disease who have failed management with lifestyle changes. This patient has uncomplicated gastroesophageal reflux without any signs of feeding refusal, worsening irritability, or nutritional compromise. Answer B: Obtaining an abdominal ultrasound would evaluate for pyloric stenosis, which presents with non-bilious, projectile vomiting rather than the regurgitation that this patient’s mother is describing. An abdominal ultrasound would be the appropriate next step in management for patients who present with a description of more forceful vomiting or have a classic “olive-shaped” mass in the epigastrium on physical exam. Answer D: Recommending modification of the mother’s diet would be appropriate if this patient had more severe symptoms that were refractory to lifestyle changes. It would also be appropriate if the patient had signs of an underlying allergy to cow’s milk or soy protein, such as blood-streaked stools. Answer E: Switching to a hydrolyzed formula would be appropriate for formula-fed patients with refractory symptoms of gastroesophageal reflux disease or signs of an allergy to cow’s milk or soy protein. Unless the patient has evidence of severe nutritional compromise, continued breastfeeding should be encouraged for breastfed patients with possible modification of the mother’s diet to eliminate cow’s milk or soy if an allergy is suspected. Bullet Summary: Uncomplicated gastroesophageal reflux in infants should be treated with reassurance and counseling to position the infant upright for 20-30 minutes after feeding.
Switch to hydrolyzed formula
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A 22-year-old man presents to the emergency department with difficulty breathing. He has experienced similar symptoms before. Today, he was hit in the face with a football, followed by significant swelling of his lips and tongue. The patient speaks with a muffled voice. His temperature is 98.1°F (36.7°C), blood pressure is 120/83 mmHg, pulse is 80/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam reveals swollen lips and a protruding, enlarged tongue. His breath sounds are clear. He demonstrates no rash and his abdomen is soft and nontender. Which of the following is the most effective therapy for this patient?
Dexamethasone
Diphenhydramine
Epinephrine
Fresh frozen plasma
D
Fresh frozen plasma
This patient is presenting with recurrent episodes of significant swelling of his lips and tongue with minor trauma, which are concerning for hereditary angioedema. Fresh frozen plasma is the most effective therapy for hereditary angioedema of the options listed. Angioedema is a potentially life-threatening condition that presents with severe edema of the gastrointestinal and respiratory tracts. It can be caused by antigens, medications, and trauma or may occur spontaneously. It often presents with notable edema of the lips and tongue and may threaten the airway. There are 3 subtypes. Hereditary and acquired angioedema occur secondary to a deficiency of C1-esterase inhibitor. As a result, the most effective therapy is to administer fresh frozen plasma which has C1-esterase inhibitor. On the other hand, drug-induced angioedema (often from angiotensin-converting enzyme inhibitors and angiotensin receptor blockers) does not respond well to C1-esterase inhibitor replacement and supportive therapy; discontinuing the offending agent is the mainstay of therapy. Other effective but costly treatments that can be used in hereditary angioedema include: icatibant, ecallantide, and C1 inhibitor. Paige and Anderson note the management of hereditary angioedema. They review the different treatments and discuss C1-esterase inhibitor replacement. They recommend this treatment for acute episodes. Incorrect Answers: Answer A: Dexamethasone and other steroids can be given in acute allergic reactions and may reduce the recurrence of symptoms but are unlikely to be effective in angioedema, nor are they the most effective therapy. Steroids can take up to 4 hours to begin having any effect. Answer B: Diphenhydramine is an antihistamine that could be given for anaphylaxis or urticaria and works immediately; however, it is not the mainstay of therapy for angioedema nor is it the most effective therapy. It is the mainstay of therapy for urticaria. It can also be given in anaphylaxis. Answer C: Epinephrine is the treatment of choice for anaphylaxis which presents with urticaria, upper airway swelling, hypotension, nausea/vomiting, and wheezing. It is often given in angioedema but has limited efficacy when compared to fresh frozen plasma. Answer E: Tranexamic acid is effective in treating angioedema and current evidence supports its use; however, the mainstay of therapy is still fresh frozen plasma. Tranexamic acid is also used in hemorrhagic shock to stop fibrinolysis and reduce bleeding. Bullet Summary: A possible treatment in hereditary or acquired angioedema is the administration of fresh frozen plasma to replace C1-esterase inhibitor.
Tranexamic acid
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A 23-year-old man presents to the emergency department. He was brought in by police for shouting on a subway. The patient claims that little people were trying to kill him and he was acting within his rights to defend himself. The patient has a medical history of marijuana and IV drug use as well as multiple suicide attempts. He is currently homeless. While in the ED, the patient is combative and refuses a physical exam. He is given IM haloperidol and diphenhydramine. The patient is transferred to the inpatient psychiatric unit and is continued on haloperidol throughout the next week. Though he is no longer aggressive, he is seen making "armor" out of paper plates and plastic silverware to defend himself. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. The patient's treatment history is significant for failure to obtain symptom control with risperidone, haloperidol, and ziprasidone. Which of the following is the most appropriate next step in management?
Clozapine
Thioridazine
Chlorpromazine
Fluphenazine
A
Clozapine
This patient is presenting with symptoms of schizophrenia that are refractory to treatment with both typical and atypical antipsychotics. The most appropriate next step in management is treatment with clozapine. Schizophrenia presents with auditory and/or visual hallucinations among many other findings. The most appropriate initial treatments are with a typical antipsychotic (haloperidol) or an atypical antipsychotic (risperidone). Once a patient has failed treatment with multiple medications (at least 2) from different classes, the next best step in management is clozapine. This medication is reserved for patients who have sustained treatment failure (because of the side effects of clozapine). These include weight gain, life-threatening agranulocytosis, and aplastic anemia. Regular complete blood count laboratory testing should be drawn while on clozapine to check for this side effect. Patients who develop this complication should immediately stop the medication but the side effect can be irreversible. Khokhar et al. review the evidence regarding the use of clozapine to treat schizophrenia. They discuss how side effects of this medication include agranulocytosis, myocarditis, and seizures. They recommend using this medication with care and monitoring for the development of these complications. Incorrect Answers: Answer A: Chlorpromazine is a typical antipsychotic that is likely to fail given that haloperidol did not work for him. Important side effects of this medication include hypotension and sedation due to anticholinergic properties. This medication also has an idiosyncratic complication of corneal deposits. Answer C: Fluphenazine is another typical anti-psychotic that is a strong D2 antagonist like haloperidol. If haloperidol failed, it is unlikely that another typical antipsychotic would work with such a similar mechanism of action. This medication has a high association with extrapyramidal side effects such as dystonia, akathisia, and tardive dyskinesia. Answer D: Olanzapine is another atypical antipsychotic. Switching to another atypical antipsychotic is a viable option. Given that this patient has already failed another atypical antipsychotic and that his symptoms remain, the best therapy at this point would be clozapine. Side effects include weight gain and dyslipidemia. Answer E: Thioridazine is a low-potency anti-psychotic that would likely not work if more potent drugs such as haloperidol or risperidone have already failed. Important side effects to be aware of include hypotension and sedation due to anticholinergic properties. This medication also has an idiosyncratic complication of retinal deposits. Bullet Summary: Clozapine is the treatment of choice in patients who have failed therapy with multiple different classes of antipsychotics and who have severe symptoms.
nan
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A 31-year-old woman presents to her obstetrician for her first prenatal visit after having a positive home pregnancy test 1 week ago. Her last menstrual period was 8 weeks ago. The patient has a medical history of type 1 diabetes mellitus since childhood and her home medications include insulin. Her hemoglobin A1c 2 weeks ago was 13.7%. At that time, she was also found to have microalbuminuria on routine urinalysis. Her primary care provider prescribed lisinopril but the patient has not yet started taking it. The patient’s brother is autistic but her family history is otherwise unremarkable. Her temperature is 98.6°F (37.0°C), blood pressure is 124/81 mmHg, pulse is 75/min, and respirations are 14/min. A physical exam is unremarkable. This fetus is most likely at increased risk for which of the following complications?
Aneuploidy
Neonatal hyperglycemia
Neural tube defect
Oligohydramnios
C
Neural tube defect
This patient has type 1 diabetes mellitus and presents in early pregnancy with a markedly elevated hemoglobin A1c, suggesting poor glycemic control. Poor glycemic control increases the risk of a neural tube defect in the fetus. Infants of diabetic mothers (IDM) are at increased risk of many congenital malformations as well as postnatal complications. These risks are more likely if there is poor maternal glycemic control, especially in the periconceptional period. Poorly controlled hemoglobin A1c and microalbuminuria on urinalysis indicate long-standing poor glycemic control and indicate a poor prognosis. The most common anomalies in IDM are cardiovascular defects such as septal defects and transposition of the great arteries as well as neural tube defects such as anencephaly and spina bifida. Sacral agenesis, or a poorly developed caudal spine and spinal cord, as well as small left colon syndrome due to a meconium plug, are other common malformations seen in IDM. General complications in IDM include prematurity, intrauterine growth restriction or macrosomia, and miscarriage. Risk is minimized by tight glycemic control throughout pregnancy. Dean et al. summarize the evidence surrounding micronutrients and obesity in pregnancy. They found that patients with diabetes had a higher risk of having infants with a neural tube or a congenital heart defect. They recommend using folic acid supplementation in all patients to reduce the risk of neural tube defects. Incorrect Answers: Answer A: Aneuoploidy risk is not increased in IDM. This complication is associated with advanced maternal age. This patient is at baseline risk since she is below the “advanced maternal age” cutoff of 35 years. Some of the values used in aneuploidy screening (maternal serum alpha­fetoprotein, estriol, and inhibin A) are generally lower in women with diabetes. Guidelines specific to this patient population should be used for the aneuploidy screen so as to avoid false positive results. Answer B: Neonatal hyperglycemia is not seen in IDM. Patients may experience hypoglycemia. This is due to the crossing of glucose from the mother to the fetus via the placenta and subsequent hyperinsulinemia and ß-cell hyperplasia from the fetus. After delivery, the high insulin levels cause hypoglycemia because there is no longer an overabundant source of glucose. Hyperglycemia in infants is much less common and can be due to glucose infusion or sepsis. Answer D: Oligohydramnios is not specifically associated with maternal hyperglycemia. Instead, patients may experience polyhydramnios. The mechanism for increased amniotic fluid is unclear, though some speculate that fetal hyperglycemia results in polyuria and thus polyhydramnios. Severe oligohydramnios can cause pulmonary hypoplasia and clubfoot as well as cord compression, while polyhydramnios is associated with cord prolapse, placental abruption, and prematurity. Answer E: Post-term delivery occurs after 42 weeks gestation. Maternal diabetes typically causes prematurity. Post-term delivery is associated with fetal malnutrition, hypoxemia, and oligohydramnios due to suboptimal functioning of the placenta. The risk of meconium aspiration increases as gestational age increases. Induction of labor is generally performed at 42 weeks to avoid these complications. Bullet Summary: Maternal hyperglycemia from diabetes mellitus is associated with congenital heart defects, neural tube defects, and small left colon syndrome.
Post-term delivery
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A study is performed that looks at individuals who suffer from opioid use disorder and the effect the disorder has on their daily life and functionality. Individuals are surveyed using a validated method and followed up in 6-month intervals for 5 years. At the end of the study period, 40% of individuals have replied to the survey throughout the entire period. Analyzing only those subjects who responded throughout the entire period, it is determined that opioid use disorder only minimally impairs individuals in their daily functioning. Which of the following most likely occurred in this study?
Confounding variable
Recall bias
Volunteer bias
Attrition bias
D
Attrition bias
This study following individuals who abuse opioids with a 40% follow-up response rate suggests that opioid use disorder (known for impairing daily functioning) has only a minor effect on patient functionality. This is most likely secondary to attrition bias, or a loss to follow-up of participants in a non-random fashion whereby more severe cases stopped responding and were thus left out. Bias in studies distorts outcomes and can make interpreting results challenging. For this reason, it is important to be meticulous when designing a study with every effort to minimize bias. The attrition bias can occur when individuals are lost to follow-up in a non-random fashion. For example, consider the assessment of daily functionality via a validated method in individuals with coronary artery disease. Those who are severely impaired from disease progression may not follow up in this study, thus altering results and removing more severe cases from the analysis. Having a higher response rate can help mitigate this bias, but is more costly as it may be difficult to ensure that every participant follows up. Nunan et al. discuss the attrition bias. They note how it can affect studies and the data collected. They recommend mitigating the attrition bias for more accurate results and clinical application. Incorrect Answers: Answer B: Confounding variables are variables that are correlated with the studied variable that better explain the difference between 2 groups. For example, it may be found that alcohol is associated with lung cancer when in reality, the confounding variable of smoking (more common in individuals who drink alcohol) explains this difference. Answer C: Late-look bias occurs when patients with severe disease are not studied because they are dead, critically ill, or unavailable to be studied, making the disease overall appear less severe. For example, patients with HIV may not have increased morbidity and mortality in a study. However, this may be a result of all the critically ill cases being too sick or dead and thus unable to participate thus distorting the outcome. Answer D: Recall bias occurs when individuals with disease or an adverse outcome are more likely to recall an exposure given the occurred outcome, skewing their memory and thus the results. For example, pregnant mothers who have a newborn with a defect or condition may be more likely to recall certain exposures (such as exposure to pollution). Answer E: Volunteer bias occurs when people who volunteer for a study are fundamentally different from the general population or those who do not volunteer. An example may be individuals volunteering in a study involving exercise as those who are interested in participating in an exercise study may be more motivated or healthier at baseline. Bullet Summary: Attrition bias occurs when individuals are lost to follow-up in a non-random fashion leading to skewed results.
nan
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A 59-year-old man presents accompanied by his wife with nausea and dizziness. He is unsure when his symptoms started, but they have been affecting him for “a while.” It began as episodes of “unsteadiness” that progressed to a feeling of “spinning.” He cannot tell if his symptoms change with position but reports that if he does not lie down he will become nauseous. He also has worsening hearing loss worse on his right side. The patient’s medical history is significant for hypertension, alcohol use disorder, and chronic obstructive pulmonary disease. His medications include aspirin, amlodipine, and fluticasone-salmeterol. He drinks a glass of red wine every night with dinner and smokes a cigar on the weekends. His temperature is 98.6°F (37°C), blood pressure is 135/91 mmHg, pulse is 72/min, and respirations are 12/min. Examination shows delayed horizontal nystagmus. Which of the following is the most appropriate treatment for this patient's condition?
CN VIII ablation
Epley maneuver
Low-salt diet
Meclizine
C
Low-salt diet
This patient is presenting with chronic vertigo, hearing loss, and horizontal nystagmus, which is suggestive of Meniere disease. The most appropriate initial step in the management of this disease is a low-salt diet. Meniere disease is caused by an abnormal accumulation of endolymph within the inner ear. Symptoms will include chronic relapsing and remitting episodes of vertigo, sensorineural hearing loss, tinnitus, and nausea. Physical exam, audiometry, and vestibular testing can be performed to confirm the diagnosis. First-line treatment is a thiazide diuretic as well as lifestyle modifications including a low-salt diet as well as avoidance of alcohol, nicotine, and caffeine. If symptoms persist, additional therapy may involve vestibular nerve ablation or a labyrinthectomy. Tassinari et al. review the evidence regarding the treatment of Meniere disease. They discuss how vertigo, low-pitched tinnitus, and hearing loss are characteristic of this disease. They recommend creating a database to allow for better research into this disease. Incorrect Answers: Answer A: CN VIII ablation can be used to treat Meniere disease that is uncontrolled by diet modifications or thiazide diuretics. Patients usually respond to conservative care so a complete course of these treatments should be trialed first. Answer B: The Epley maneuver can be used to treat benign paroxysmal positional vertigo (BPPV). Patients will present with vertigo that changes with the patient's position without hearing loss or tinnitus. This maneuver functions by dislodging otoliths from the semicircular canals. Answer D: Meclizine can be used to treat other types of vertigo such as BPPV or viral labyrinthitis. Labyrinthitis can present with similar symptoms to the symptoms of Meniere disease, but they will be acute and self-limited. There will also be inflammation of the cochlear portion of the inner ear. Answer E: Thiamine can be used for Wernicke-Korsakoff syndrome. A history of chronic heavy alcohol use, confusion with confabulation, ataxia, memory loss, and nystagmus are all concerning signs that may indicate thiamine deficiency. Bullet Summary: Meniere disease should be treated initially with a low-salt diet, avoidance of triggers, and thiazide diuretics.
Thiamine
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At a local community hospital staffed only by attending physicians, it is noted that the time to tissue plasminogen activator administration in suspected stroke patients is roughly 10 minutes from presentation. At a teaching hospital with residents, the time to tissue plasminogen activator administration is roughly 2 hours. This has led to many adverse outcomes and increased morbidity at the teaching hospital. Which of the following is the most appropriate next step in further elucidating the problem?
Automatically consult neurology for patients with concerning neurologic symptoms
Empirically scan patients with concerning neurologic symptoms
Improve resident education
Perform a root cause analysis of the process
D
Perform a root cause analysis of the process
This case outlines a delay in treating strokes at a teaching hospital. To determine the underlying cause, a root cause analysis to determine the underlying issue is the most appropriate way to begin to address this issue. When a problem is found in healthcare, it is important to elucidate the underlying cause. A root cause analysis is the current preferred method of determining the underlying cause of a problem. A root cause analysis outlines all the people, materials, equipment, and processes in place and outlines them. It then locates the problem and attempts to find the breakdown or issue in all these inputs that lead to the problem. Once the problem is identified, then a strategy or intervention can be implemented to fix the underlying problem. Percarpio et al. review root cause analysis. They note the ubiquity of root cause analysis. They call into question its cost and efficacy despite it being such a widespread practice and recommend a careful assessment of the costs and benefits. Incorrect Answers: Answer A: Automatically consulting neurology for patients with concerning neurologic symptoms may be appropriate if the issue was lack of access to timely neurologist assessment in the workup of stroke. While may hospitals have this as a default for patients with stroke-like symptoms, it would be premature to implement this as a solution without a root cause analysis to first determine what the problem is. Answer B: Empirically scanning patients with concerning neurologic symptoms would be wasteful and not plausible in a healthcare system. There are many concerning neurologic chief complaints including those that originate in the brain, the spine, or the peripheral nerves; thus, merely CT scanning everyone with a neurological chief complaint is an inefficient use of resources. Answer C: Improving resident education is assuming that the problem is a lack of knowledge based on the residents. Before proposing a solution, it is important to first find the underlying cause and address said cause as the delayed intervention may not be the fault of the residents. Answer E: A systems-based approach is the best solution when fixing a medical error. However, a root cause analysis is first needed to determine which solution is needed. If an error in the process is noted, a systems-based approach would be the best method to address it. Bullet Summary: When an error is noted in patient care, the most appropriate initial step in fixing the issue is conducting a root cause analysis.
Perform a systems-based approach implementation
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A 36-year-old G3P2002 woman presents to her obstetrician’s office for her 1st prenatal visit at 10 weeks and 2 days gestation. She has felt nauseous the last several mornings and has been tired for a few weeks. The patient has had 2 uncomplicated spontaneous vaginal deliveries at full term with her last child born 6 years ago. She is concerned about the risk of Down syndrome in this fetus, as her sister gave birth to an affected child at age 43. She has a history of generalized anxiety disorder, atopic dermatitis, and she is currently on escitalopram. Her temperature is 98.6°F (37.0°C), pulse is 70/min, blood pressure is 121/67 mmHg, and respirations are 13/min. The patient appears anxious, but overall comfortable, and cardiopulmonary and abdominal exams are unremarkable. Pelvic exam reveals normal external genitalia, a closed and soft cervix, a 10-week-sized uterus, and no adnexal masses. Which of the following is the most appropriate next step for definitively determining whether this patient’s fetus has Down syndrome?
Amniocentesis
Anatomy ultrasound
Chorionic villus sampling
Genetic testing of patient’s sister
C
Chorionic villus sampling
This patient is over 35 years of age and concerned about the risk of Down syndrome (trisomy 21) in her fetus at just over 10 weeks gestation. At this gestational age, the most definitive method for determining whether such an aneuploidy is present is chorionic villus sampling (CVS). CVS, or sampling of the placental tissue, can be performed as early as 10 weeks gestation. This is in contrast to amniocentesis, which should be avoided prior to 14 weeks. There are other screening tests for a patient concerned about aneuploidy; however, only CVS and amniocentesis offer definitive diagnoses. Complications of this procedure include pregnancy loss (at a rate of 1-2%), transverse limb abnormality, rupture of membranes, and chorioamnionitis. Patients should also have counseling if genetic abnormalities are detected using this procedure. Farina reviews the evidence regarding the complications associated with CVS. He discusses how this procedure is becoming more common as the definitive method of providing an early diagnosis. He recommends being aware of complications such as possible pregnancy loss. Incorrect Answers: Answer A: Amniocentesis is a useful tool for the definitive diagnosis of aneuploidy and other genetic conditions. Due to the higher rate of fetal loss at younger gestational ages, it is reserved for those above 14 weeks. Answer B: The anatomy ultrasound can be helpful in identifying morphologic features that are concerning for Down syndrome, such as a flat nasal bridge or an echogenic cardiac focus. This screening tool is most accurate after 18 weeks gestation. Answer D: Genetic testing of the patient’s sister may be able to help identify if there is a parental translocation or aneuploidy that led to the child’s Down syndrome. This is much less likely than meiotic nondisjunction due to advanced maternal age, and this would not help diagnose Down syndrome in the patient’s fetus. Answer E: The nuchal translucency test examines the thickness of the nuchal fold. When the thickness exceeds normal measurements, there is an increased risk of aneuploidy such as Down syndrome, but this is a screening result that would not provide a definitive diagnosis. Bullet Summary: Chorionic villus sampling is a definitive method of identifying aneuploidy in fetuses starting at 10 weeks of gestational age.
Nuchal translucency test
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A 75-year-old man presents to the emergency department with fatigue. He has had severe nausea, vomiting, and diarrhea for the past 3 days. Today, his wife noticed that he was confused. The patient has a history of coronary artery disease with a drug-eluting stent placed 1 year ago, obesity, hypertension, and dyslipidemia. He denies chest pain or dyspnea, but complains of diffuse abdominal pain. His temperature is 99.0°F (37.2°C), blood pressure is 100/60 mmHg, pulse is 105/min, and respirations are 22/min. Physical exam reveals a confused man with dry mucous membranes. His abdomen is diffusely tender to palpation. Laboratory studies are ordered and notable for the following: Serum: Na+: 147 mEq/L Cl-: 112 mEq/L K+: 3.5 mEq/L HCO3-: 14 mEq/L BUN: 80 mg/dL Glucose: 100 mg/dL Creatinine: 2.1 mg/dL Troponin: 0.10 mcg/L (normal < 0.01 mcg/L) An ECG is ordered and is pending. Which of the following is the most appropriate management of this patient's elevated troponin?
Aspirin, heparin, clopidogrel, and cardiac catheterization
Ringer lactate bolus
Metoprolol and aspirin
Obtain a urinalysis and creatine phosphokinase level
B
Ringer lactate bolus
This patient with likely gastroenteritis (given his nausea, vomiting, and diarrhea) is dehydrated, as indicated by his low blood pressure, tachycardia, dry mucous membranes, and prerenal kidney injury (elevated BUN and creatinine with BUN:creatinine ratio > 20:1) in the setting of an anion gap (anion gap = [Na+] - [Cl-] - [HCO3-] = 147 - 112 - 14 = 21). His elevated troponin is likely secondary to poor renal clearance and dehydration, which is most appropriately addressed with IV fluids such as Ringer lactate. Troponin is a lab marker classically thought to be elevated when there is an injury to the heart causing leakage of this enzyme from the cardiac myocyte. However, there are many possible causes of an elevated troponin that may not be directly cardiac in nature or related to myocardial ischemia. Acute kidney injury is a common cause of an elevated troponin. For this reason, patients with an elevated troponin with kidney dysfunction without any cardiac symptoms or ECG changes should have their renal etiology addressed, and this will often clear the troponin. In the setting of a prerenal injury (dehydration causing poor kidney perfusion and a BUN:creatinine ratio > 20:1), IV fluids will restore perfusion and treat the underlying cause. Patients with chronic kidney disease or kidney failure should have an ECG obtained and their current troponin should be compared to previous troponin levels to ensure it is not higher than their baseline. If there is uncertainty regarding a troponin level, it can be trended to see if it is rising, falling, or staying the same. Correlation with clinical symptoms is important when evaluating an elevated troponin level. Masri et al. review the causes of an elevated troponin. They note that a troponin elevation can be seen in myopericarditis, renal failure, heart failure, pulmonary embolism, septic shock, rhabdomyolysis, stroke, and many other possible causes that are not related to myocardial infarction. They recommend keeping a broad differential and correlating the troponin with the patient's clinical presentation. Incorrect Answers: Answer A: Aspirin would be appropriate management of an elevated troponin secondary to acute coronary syndrome as its antiplatelet activity reduces mortality. It should be administered prior to performing an ECG in patients with chest pain thought to be from a cardiac etiology. Answer B: Aspirin, heparin, clopidogrel, and cardiac catheterization is appropriate management of a ST elevation myocardial infarction, which presents with chest pain, diaphoresis, dyspnea, and ST elevation within a vascular distribution with reciprocal changes on ECG. This patient has no cardiac symptoms and has a better alternative explanation for their troponin elevation. Answer C: Metoprolol and aspirin may be indicated in acute coronary syndrome, and metoprolol would reduce this patient's heart rate. However, note that this patient's tachycardia is secondary to volume depletion; thus, beta-blockade for sinus tachycardia is inappropriate and may make the patient hemodynamically unstable. Answer D: Obtaining a urinalysis and creatine phosphokinase level may be performed in rhabdomyolysis which presents in the setting of seizures, electrical shock, heatstroke, or with vigorous exertion. Patients will complain of muscle pain, oliguria, and dark urine. Management is centered on the administration of large volumes of fluid to protect the kidney and clear the toxic myoglobin. Note that rhabdomyolysis may elevate the troponin; however, there is nothing in this patient's history that suggests rhabdomyolysis. Bullet Summary: Prerenal injury causing an acute kidney injury may elevate the troponin due to decreased renal clearance and should be addressed with the administration of fluids.
nan
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A 30-year-old man is evaluated in the post-anesthesia care unit for neck pain. He underwent a laparoscopic appendectomy and was treated for post-operative nausea and vomiting with metoclopramide. Approximately 20 minutes after receiving the medication, he developed pain and stiffness in his neck and eventually was unable to move his neck. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 130/80 mmHg, respirations are 16/min, and oxygen saturation is 98% on room air. Examination reveals an uncomfortable appearing man. His neck is rotated to the right and is unable to return to midline. Examination of the patient is shown in Figure A. Which of the following is the most appropriate next step in management?
Botulinum toxin injection
Diphenhydramine
Haloperidol
Observation
B
Diphenhydramine
This patient is suffering from a metoclopramide-induced acute dystonic reaction. Treatment of this complication includes IV diphenhydramine or benztropine. Dystonic reactions are reversible extrapyramidal symptoms that may include involuntary spasms or sustained contractions of the face, neck, torso, pelvis, extremities, and larynx. Medications that block dopamine D2 receptors carry the greatest risk for development of a dystonic reaction, most notably including neuroleptics, antidepressants, and antiemetics. A family history of dystonia increases the risk of developing a reaction (there is likely an underlying genetic predisposition). Diagnosis is typically based on history and clinical presentation. Intravenous anticholinergic agents are the first-line treatment, and symptoms typically resolve rapidly (within 1 hour) after initiation of these agents. Dressler et al. discuss the diagnosis and management of medication-induced acute movement disorders, notably dystonic reactions. These reactions are most commonly caused by dopamine-receptor blocking agents, including antipsychotics and antiemetics. Cranial, pharyngeal, and cervical muscles are typically affected within the first 4 days of initiating treatment. Figure A shows a patient with dystonia and torticollis; note the posturing of the extremities as well as how the head is tilted to the patient's right side while the chin is directed up and towards the left. Incorrect Answers: Answer A: Intramuscular botulinum toxin injection may be appropriate for chronic torticollis or other forms of chronic dystonia. This patient has acute dystonia that is generally able to be easily reversed with intravenous diphenhydramine. Answer C: Intravenous haloperidol, a D2 receptor antagonist would not be appropriate. This patient's condition is due to the dopamine antagonist action of metoclopramide and would therefore be worsened with haloperidol. Answer D: Observation would not be appropriate. This patient is experiencing an acute dystonic reaction that is uncomfortable and potentially dangerous. Acute dystonia is not likely to self resolve in a reasonable amount of time. Answer E: Intravenous ondansetron may be a more appropriate option to treat this patient's nausea given his reaction to metoclopramide. However, it will not reverse the acute dystonic reaction and is not the most appropriate first step. Bullet Summary: Acute dystonic reaction may occur with administration of medications that block dopamine receptors (such as metoclopramide), and can be treated with intravenous diphenhydramine.
Ondansetron
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A 72-year-old man presents to his primary care physician for a general checkup. The patient works as a farmer and has no concerns about his health. He has a medical history of hypertension and obesity. His current medications include lisinopril and metoprolol. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for a murmur after S2 over the left sternal border. The patient demonstrates a stable gait and 5/5 strength in his upper and lower extremities. Which of the following is another possible finding in this patient?
Audible click heard at the cardiac apex
Femoral artery murmur
Murmur that radiates to the carotids during systole
Rumbling heard at the cardiac apex
B
Femoral artery murmur
This patient is presenting with aortic regurgitation which could be associated with Duroziez sign (femoral artery murmur). Aortic regurgitation occurs when the aortic valve closes improperly resulting in retrograde flow during diastole. This is auscultated as a diastolic murmur heard best over the left upper sternal border and the left lower sternal border. This murmur typically occurs in elderly patients and the retrograde flow through the aorta cause a number of physical exam findings. These include a wide pulse pressure, fingernail pulsations, head bobbing, and femoral artery murmur. Treatment is with surgical valve replacement. Sapira reviews the evidence regarding the physical exam findings in aortic regurgitation. He discusses how Duroziez sign can often be seen in patients with this disease. He recommends using these signs to properly identify patients. Incorrect Answers: Answer A: An audible click heard at the cardiac apex describes mitral valve prolapse which typically is asymptomatic and presents in younger women. Treatment is with surgical valve replacement in symptomatic patients. Answer C: A murmur that radiates to the carotids during systole describes aortic stenosis which is possible in this patient except for the fact that his murmur is diastolic. Treatment is with valvuloplasty or with surgical valve replacement. Answer D: A rumbling heard at the cardiac apex describes mitral valve stenosis which presents with a diastolic murmur best heard at the cardiac apex. Treatment is with valvuloplasty or with surgical valve replacement. Answer E: A wedge pressure lower than expected would be seen in tricuspid regurgitation. In tricuspid stenosis, less blood flows to the lungs and thus the left heart, resulting in a low wedge pressure. Treatment is with identification of the underlying cause and valve replacement. Bullet Summary: Aortic regurgitation is associated with a wide pulse pressure, fingernail pulsations, head bobbing, and a femoral artery murmur.
Wedge pressure lower than expected
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A 36-year-old G3P2002 woman presents to her obstetrician’s office for her 1st prenatal visit at 10 weeks and 2 days gestation. She has felt nauseous the last several mornings and has been tired for a few weeks. The patient has had 2 uncomplicated spontaneous vaginal deliveries at full term with her last child born 6 years ago. She is concerned about the risk of Down syndrome in this fetus, as her sister gave birth to an affected child at age 43. She has a history of generalized anxiety disorder, atopic dermatitis, and she is currently on escitalopram. Her temperature is 98.6°F (37.0°C), pulse is 70/min, blood pressure is 121/67 mmHg, and respirations are 13/min. The patient appears anxious, but overall comfortable, and cardiopulmonary and abdominal exams are unremarkable. Pelvic exam reveals normal external genitalia, a closed and soft cervix, a 10-week-sized uterus, and no adnexal masses. Which of the following is the most appropriate next step for definitively determining whether this patient’s fetus has Down syndrome?
Anatomy ultrasound
Genetic testing of patient’s sister
Nuchal translucency test
Chorionic villus sampling
D
Chorionic villus sampling
This patient is over 35 years of age and concerned about the risk of Down syndrome (trisomy 21) in her fetus at just over 10 weeks gestation. At this gestational age, the most definitive method for determining whether such an aneuploidy is present is chorionic villus sampling (CVS). CVS, or sampling of the placental tissue, can be performed as early as 10 weeks gestation. This is in contrast to amniocentesis, which should be avoided prior to 14 weeks. There are other screening tests for a patient concerned about aneuploidy; however, only CVS and amniocentesis offer definitive diagnoses. Complications of this procedure include pregnancy loss (at a rate of 1-2%), transverse limb abnormality, rupture of membranes, and chorioamnionitis. Patients should also have counseling if genetic abnormalities are detected using this procedure. Farina reviews the evidence regarding the complications associated with CVS. He discusses how this procedure is becoming more common as the definitive method of providing an early diagnosis. He recommends being aware of complications such as possible pregnancy loss. Incorrect Answers: Answer A: Amniocentesis is a useful tool for the definitive diagnosis of aneuploidy and other genetic conditions. Due to the higher rate of fetal loss at younger gestational ages, it is reserved for those above 14 weeks. Answer B: The anatomy ultrasound can be helpful in identifying morphologic features that are concerning for Down syndrome, such as a flat nasal bridge or an echogenic cardiac focus. This screening tool is most accurate after 18 weeks gestation. Answer D: Genetic testing of the patient’s sister may be able to help identify if there is a parental translocation or aneuploidy that led to the child’s Down syndrome. This is much less likely than meiotic nondisjunction due to advanced maternal age, and this would not help diagnose Down syndrome in the patient’s fetus. Answer E: The nuchal translucency test examines the thickness of the nuchal fold. When the thickness exceeds normal measurements, there is an increased risk of aneuploidy such as Down syndrome, but this is a screening result that would not provide a definitive diagnosis. Bullet Summary: Chorionic villus sampling is a definitive method of identifying aneuploidy in fetuses starting at 10 weeks of gestational age.
nan
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A 62-year-old man presents to the emergency department with increased fatigue and changes in his vision. For the past month, he has felt abnormally tired and today noticed his vision is blurry. The patient also endorses increased sweating at night and new-onset headaches. He currently feels dizzy. The patient has a medical history of diabetes and hypertension. His current medications include insulin, metformin, and lisinopril. His temperature is 99.5°F (37.5°C), blood pressure is 157/98 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. A cardiopulmonary exam is within normal limits. HEENT exam reveals non-tender posterior and anterior chain lymphadenopathy. Abdominal exam reveals splenomegaly and hepatomegaly. There are large, non-tender palpable lymph nodes in the patient's inguinal region. A neurological exam is notable for decreased sensation in the patient's hands and feet. He also complains of numbness and tingling pain in his extremities that has been persistent during this time. The dermatologic exam is notable for multiple bruises on his upper and lower extremities. Which of the following is most likely to be abnormal in this patient?
Calcium
IgA and IgG
IgM
Natural killer cells
C
IgM
This patient is presenting with symptoms of hyperviscosity, fatigue, and organomegaly suggestive of a diagnosis of Waldenstrom macroglobulinemia (WM). WM is characterized by increased IgM production. WM occurs secondary to a clonal B-cell that pathologically overproduces IgM. High levels of IgM can lead to symptoms of hyperviscosity including blurred vision, dizziness, headaches, and peripheral neuropathy. Organomegaly is a unique finding in WM and can present with hepatomegaly and splenomegaly. Other non-specific findings include fatigue, anemia, and lymphadenopathy. This disease can be diagnosed using protein electrophoresis, which would demonstrate a spike in the levels of IgM protein. Treatment can include plasmapheresis and rituximab. Gertz et al. review the evidence regarding the diagnosis and treatment of patients with WM. They discuss how patients often present with organomegaly as well as symptoms of hyperviscosity. They recommend rituximab treatment in symptomatic patients. Incorrect Answers: Answers 1 & 2: Calcium and IgA/IgG can be elevated in multiple myeloma (MM). MM presents with fatigue, weight loss, bone pain (back pain in particular), and other non-specific findings. MM less commonly presents with symptoms of hyperviscosity and organomegaly and this phenomenon has been described primarily with the IgA subtype. This is a late phenomenon and would be unlikely without preceding symptoms of bone pain and weight loss as a substantial protein concentrations (>5 g/dL) are required for this phenomenon. Treatment is with systemic chemotherapeutic agents. Answers 4 & 5: Natural killer cells and T-cells could be elevated in non-Hodgkin lymphoma which would present with fatigue, lymphadenopathy, and "B-symptoms" (e.g., night sweats and chills). It would not present with symptoms of hyperviscosity. Treatment is with multi-agent chemotherapy as well as possible local radiation therapy. Bullet Summary: Waldenstrom macroglobulinemia (hyper IgM syndrome) presents with symptoms of hyperviscosity, fatigue, and organomegaly.
T-cells
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A 59-year-old woman comes to the clinic due to a 3-month history of “always feeling the urge to urinate” as well as vaginal pruritis. She states that she has had involuntary loss of urine about 4 times each day over the past 3 months. It is not associated with coughing or sneezing. Menopause occurred at age 52. She has a history of chlamydial infection in her 20's and has had 3 urinary tract infections over the past year. The patient has not been sexually active with her husband due to pain with intercourse. She has no other significant medical history and does not take any medications. Her temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. On physical examination, the vulvar skin is pale and atrophic with reduced elasticity and multiple areas of petechiae. The vaginal mucosa also has a loss of rugae and several areas of friable irritation. There is minimal clear vaginal discharge. Urinalysis is normal and fecal occult blood test is negative. Pelvic ultrasound reveals a small, retroverted uterus. Which of the following is the most appropriate next step in management?
Metronidazole
Nitrofurantoin
Topical clobetasol
Topical estrogen
E
Vaginal moisturizers
This postmenopausal patient is presenting with vulvovaginal dryness and irritation, dyspareunia, urinary incontinence, and recurrent urinary tract infections, likely due to genitourinary syndrome of menopause (GSM). The most appropriate initial treatment is symptomatic relief with vaginal moisturizers and lubricants. Reduced estrogen levels after menopause lead to vulvovaginal atrophy by causing reduced blood flow and collagen content in vulvovaginal tissues. Patients typically have thin vulvar skin and loss of vaginal tissue pliability. The bladder and urethral epithelium may also atrophy leading to dysuria and urinary frequency. Atrophic tissue becomes thin, dry, and susceptible to injury. The first line of treatment is vaginal moisturizers and lubricants. If this does not improve symptoms, the next step in treatment is vaginal estrogen to help restore blood flow in vulvovaginal tissues. Finally, hormone replacement therapy is indicated if symptoms are refractory to all other modalities. Bhupathiraju et al. studied the association between vaginal estrogen and chronic disease risk. The authors found no association between low-dose vaginal estrogen and cardiovascular disease, cancer, and hip fractures over an 18-year period. The authors recommended that low-dose vaginal estrogen can be used indefinitely without concomitant progestin therapy. Incorrect Answers: Answer A: Metronidazole is the treatment for trichomoniasis or bacterial vaginosis. While patients with trichomoniasis can present with cervical petechiae and punctate hemorrhages, they typically also have frothy green discharge (absent in this patient). While patients with bacterial vaginosis can also have vaginal discharge, the discharge is typically malodorous and symptoms of pruritis are generally absent. Answer B: Nitrofurantoin is the treatment of choice for urinary tract infections. While this patient has increased urinary frequency and a history of recurrent urinary tract infections, her normal urinalysis makes the diagnosis of urinary tract infection unlikely. Answer C: Topical clobetasol cream is the treatment for lichen sclerosis. While lichen sclerosis can present with pain with intercourse and vulvar pruritis, patients often have plaques that disrupt the normal vulvar architecture. Lichen sclerosis does not affect the vaginal tissues. Answer D: Topical estrogen is the treatment of choice for cases of vulvovaginal atrophy resistant to treatment with vaginal moisturizers and lubricants. This patient should first be trialed on vaginal moisturizers and lubricants. Bullet Summary: Vulvovaginal atrophy is initially treated with vaginal moisturizers and lubricants.
Vaginal moisturizers
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A 27-year-old man presents to his primary care physician for a checkup. He has no health concerns and has not seen a physician in years. The patient has a medical history of depression treated with fluoxetine and lithium. His temperature is 99.5°F (37.5°C), blood pressure is 122/78 mmHg, pulse is 90/min, respirations are 13/min, and oxygen saturation is 98% on room air. Physical exam is notable for the finding in Figure A. Which of the following represents the most likely risk factor for this patient's presenting condition?
Alcohol consumption
Antibiotic use
Intravenous drug use
Sexual intercourse
A
Alcohol consumption
This patient is presenting with adherent white plaques on the inside of the cheek consistent with oral leukoplakia. Alcohol consumption is a risk factor for this disease. Oral leukoplakia presents with a white patch/plaque that occurs over the oral mucosa that is adherent and non-scrapable. Risk factors for this condition include the risk factors for squamous cell carcinoma such as consumption of alcohol and smoking. Though the cause of this lesion is largely idiopathic, any irritating substance can induce it. Other potential risk factors include candidiasis, vitamin deficiency, endocrine disturbances, and viral infections. A diagnosis can be made using a biopsy showing the degree of dysplasia. Treatment is with smoking and alcohol cessation as well as surgical removal for suspicious lesions. Mohammed and Fairozekhan review the evidence regarding the diagnosis and risk factors for leukoplakia. They discuss how this disease is associated with smoking and alcohol use. They recommend screening patients for these modifiable risk factors. Figure/Illustration A is a clinical photograph demonstrating adherent white plaques on the inside of the cheek (red circle). These findings are consistent with oral leukoplakia. Incorrect Answers: Answer B: Antibiotic use is a risk factor for candidiasis; however, this patient is not presenting with symptoms of candidiasis which is itself a risk factor for oral leukoplakia. Candidiasis presents with opaque white lesions that can be scraped off. Answer C: Intravenous drug use does make this patient riskier overall for many conditions; however, alcohol is a more direct risk factor for oral leukoplakia. Immunodeficiency conditions can cause widespread fungal and viral infections on the mucosal surfaces. Answer D: Poor dental hygiene may be found more often in patients with oral leukoplakia; however, it is not itself a direct cause. Answer E: Sexual intercourse would predispose this patient to infections and viruses (such as Epstein-Barr virus); however, it is a less likely risk factor than is alcohol consumption or smoking. These infections tend to present with ulcers more commonly than leukoplakia. Bullet Summary: Risk factors for oral leukoplakia and squamous cell carcinoma include alcohol consumption and smoking.
nan
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A 64-year-old man presents with nausea, vomiting, and weakness. He states that his wife made him come in when he fainted today while attempting to stand up. He otherwise states he has noticed some visual changes, including noting a yellow tint to objects. He has a history of heart failure with reduced ejection fraction, obesity, diabetes, hypertension, acute coronary syndrome, atrial fibrillation, and peripheral vascular disease. His temperature is 98.5°F (36.9°C), blood pressure is 153/91 mmHg, pulse is 40/min, respirations are 15/min, and oxygen saturation is 97% on room air. Physical exam reveals a frail man who is unable to ambulate secondary to lightheadedness. His neurological exam is otherwise non-focal. An ECG is performed as seen in Figure A. Which of the following is the most likely etiology of this patient’s symptoms?
Amiodarone
Digoxin
Diltiazem
Procainamide
B
Digoxin
This patient with a history of heart failure with reduced ejection fraction and atrial fibrillation is presenting with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes, which are concerning for digoxin toxicity. Note that digoxin can be given in patients with poor cardiac function and atrial fibrillation; thus it is a plausible home medication for this patient. Digoxin is a cardiac glycoside that is used in conditions such as heart failure with reduced ejection fraction and atrial fibrillation. While it does not lower mortality, it may reduce symptoms of heart failure. Digoxin toxicity classically presents with gastrointestinal symptoms (nausea/vomiting), blurred vision or yellow halos, and cardiac dysrhythmias. While there is a myriad of dysrhythmias, digoxin can cause bradycardia, atrioventricular block, and premature ventricular contractions. ST-segment scooping can be seen with digoxin use both in toxicity but also with regular therapeutic use. Toxicity is treated with anti-digoxin antibodies. Patocka et al review digoxin toxicity. They note that this medication has a narrow therapeutic range and recommend close monitoring. Toxicity depends on severity and can be treated with anti-digoxin antibodies. Figure/Illustration A is an ECG demonstrating bradycardia and premature ventricular complexes (red circles). This is consistent with digoxin toxicity. Incorrect Answers: Answer A: Amiodarone is an antidyshythmic agent with toxicities including bradycardia, heart block, a prolonged QT interval, hepatitis, interstitial lung disease, hypothyroidism, corneal deposits and optic neuropathy. This patient could use amiodarone for his atrial fibrillation, but the visual disturbances are more typical of digoxin toxicity. Answer C: Diltiazem is a calcium channel blocker that would cause hypotension, bradycardia, and hyperglycemia. Treatment would involve immediate administration of calcium and vasopressors such as epinephrine. Other treatments in severe toxicity may include glucagon, dextrose, and insulin (to increase cardiac contractility). Answer D: Procainamide is the preferred antidysrhythmic in tachydysrhythmias associated with Wolff Parkinson White syndrome. It may cause drug-induced lupus and other dysrhythmias in overdose but would not cause this patient’s constellation of symptoms. Procainiamide is used rarely for atrial fibrillation and is only available for intravenous infusion in the US. Answer E: Propranolol toxicity would present with bradycardia, hypotension, confusion, seizures, and hypoglycemia. Treatment is centered on glucagon, calcium, and vasopressors such as epinephrine. In severe toxicity, lipid emulsion therapy and insulin/dextrose should be administered. Bullet Summary: Digoxin toxicity may present with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes.
Propranolol
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A 55-year-old man presents to the emergency department with worsening dyspnea over the past 48 hours. He recently had a cold that kept him home from work for the past week. He has a past medical history of diabetes, obesity, and hypertension. He had his Achilles tendon repaired 4 weeks ago and he has been less mobile. His temperature is 99.2°F (37.3°C), blood pressure is 150/85 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 100% on room air. Physical exam is notable for dyspnea provoked by walking short distances. Jugular venous distension is noted on exam. The patient’s blood pressure is 130/70 mmHg during inspiration. A bedside echocardiogram demonstrates impaired diastolic filling with normal ventricular contractility. An ECG is performed as seen in Figure A. Which of the following is the most likely diagnosis?
Restrictive cardiomyopathy
Myocardial infarction
Constrictive pericarditis
Pulmonary embolism
C
Constrictive pericarditis
This patient is presenting with a preceding viral illness, dyspnea, jugular venous distension, pulsus paradoxus (blood pressure drop from 150/85 to 130/70 mmHg during inspiration), an echocardiogram with impaired diastolic filling, and diffuse ST elevation with PR depression on ECG. This constellation of symptoms is concerning for constrictive pericarditis. Constrictive pericarditis occurs when the pericardium becomes thickened and fibrotic. In contrast to other types of pericarditis, in constrictive pericarditis, there is impaired diastolic filling and thus impaired cardiac function. There are many possible etiologies of constrictive pericarditis including infection, inflammatory conditions, or idiopathic. Patients will present with dyspnea, hypotension, tachycardia, a pericardial friction rub, and positional chest pain (usually worsened by laying back and relieved by leaning forward). Patients may also demonstrate jugular venous distension, lower extremity edema, worsening dyspnea, and congestive hepatopathy. ECG may demonstrate diffuse ST elevation with PR depression, and an echocardiogram will demonstrate impaired diastolic filling and may demonstrate a thickened pericardium. The diagnosis can be supported with pulsus paradoxus (a drop of blood pressure by > 10 mmHg during inspiration). Welch reviews constrictive pericarditis. He discusses constrictive pericarditis and how the inelastic pericardium inhibits cardiac filling possibly causing unexplained heart failure. Welch notes making the diagnosis is very challenging and requires meticulous echocardiograph assessment. Figure/Illustration A demonstrates diffuse ST elevation (blue arrows) with PR depression (black arrows). This is consistent with pericarditis. Incorrect Answers: Answer A: Cardiac tamponade can occur after trauma, in the setting of infection, or in the setting of autoimmune disease and is more likely to occur with a rapid accumulation of pericardial fluid. While pulsus paradoxus would be present in this condition, muffled heart sounds and electrical alternans would be more likely to be seen on ECG. Echocardiography would demonstrate an echogenic rim of fluid around the heart. Answer C: Myocardial infarction presents with chest pain, dyspnea, hypotension, tachycardia, and ST elevation in a vascular distribution. Note that this patient’s ST elevation is diffuse and there is no reciprocal ST depression. Moreover, the pattern of chest pain in myocardial infarction is typically exertional and not positional. Answer D: Pulmonary embolism may present with pleuritic chest pain, shortness of breath, tachycardia, hypoxia, hemoptysis, a history of hypercoagulability, and recent stasis or surgery (such as this patient’s recent Achilles tendon surgery). It can cause obstructive shock leading to hypotension and tachycardia. Note that right heart strain and sinus tachycardia would be seen on ECG. Answer E: Restrictive cardiomyopathy presents very similarly to restrictive pericarditis. Note that in restrictive cardiomyopathy, patients will also have impaired diastolic filling; however, it is due to a myocardium that will not expand rather than the pericardium impairing filling. It can occur secondary to conditions such as amyloidosis or hemochromatosis as well as other chronic inflammatory states. The ECG findings are nonspecific but may include low voltage QRS complexes, ST segment changes, T wave changes, bundle branch blocks, and pathologic Q waves (abnormally deep and wide Q waves). The diagnosis can be supported with an ultrasound demonstrating impaired diastolic filling and possible myocardial speckling if a protein (amyloid) is present and deposited in the myocardial wall. The diagnosis can only definitively be confirmed with a biopsy which is highly invasive. Bullet Summary: Restrictive pericarditis presents with chest pain that is positional, pulsus paradoxus, a friction rub, and diffuse ST elevation with PR depression on ECG.
nan
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A 59-year-old man presents to his primary care physician for fatigue. In general, he has been in good health; however, he recently has experienced weight loss, abdominal pain, and general fatigue. He has a medical history of anxiety, diabetes, a fracture of his foot sustained when he tripped, and a recent cold that caused him to miss work for 1 week. His current medications include metformin, insulin, buspirone, vitamin D, calcium, and sodium docusate. His temperature is 99.5°F (37.5°C), blood pressure is 150/100 mmHg, pulse is 90/min, respirations are 18/min, and oxygen saturation is 98% on room air. Physical exam reveals a calm gentleman. A mild systolic murmur is heard in the left upper sternal region. The rest of the physical exam is within normal limits. Laboratory values are ordered as seen below. Hemoglobin: 12 g/dL Hematocrit: 36% Leukocyte count: 66,500/mm^3 with normal differential Platelet count: 177,000/mm^3 Leukocyte alkaline phosphatase: elevated Serum: Na+: 139 mEq/L Cl-: 100 mEq/L K+: 4.3 mEq/L BUN: 20 mg/dL Glucose: 120 mg/dL Creatinine: 1.1 mg/dL Ca2+: 10.9 mEq/L AST: 12 U/L ALT: 10 U/L Which of the following is the most likely diagnosis?
Acute lymphoblastic lymphoma
Chronic lymphocytic leukemia
Chronic myeloid leukemia
Leukemoid reaction
D
Leukemoid reaction
This patient is presenting with leukocytosis and an elevated leukocyte alkaline phosphatase suggestive of a leukemoid reaction. A leukemoid reaction typically presents with a drastically elevated leukocyte count (typically > 50,000/mm^3) in the setting of an elevated leukocyte alkaline phosphatase score (LAP). This reaction typically occurs after a major infection and is an immunologic response to the pathogen that caused the illness. In contrast, chronic myeloid leukemia presents with a similar elevation in leukocytes but a low LAP score in addition to symptoms of general malaise, fatigue, and weight loss. The LAP score is the critical differentiating factor when comparing these 2 pathologies. Treatment for a leukemoid reaction is by addressing the underlying pathology. Portich and Faulhaber review the evidence regarding the diagnosis of leukemoid reaction. They discuss how the most common cause is infection. They recommend considering paraneoplastic syndromes in patients without evidence of infection. Incorrect Answers: Answer A: Acute lymphoblastic lymphoma presents in a pediatric patient with pallor, fatigue, easy bruising, fever, bone pain, hepatomegaly, and splenomegaly. Treatment is with combination chemotherapy. Answer B: Chronic lymphocytic leukemia can present similarly to a leukemoid reaction with non-specific systemic symptoms and lymphadenopathy but is a less likely diagnosis in this patient given his age and LAP score. Treatment is with ibrutinib and rituximab as well as other agents. Answer C: Chronic myeloid leukemia presents with systemic symptoms, fatigue, malaise, weight loss, and abdominal pain with an elevated leukocyte count (typically > 50,000/mm^3) but a depressed LAP score. Treatment is with tyrosine kinase inhibitors. Answer E: Multiple myeloma presents with systemic symptoms, weight loss, pathologic fractures, and hypercalcemia. It is not associated with an elevated leukocyte score and an elevated LAP score. Treatment is with steroids and chemotherapy. Bullet Summary: Both a leukemoid reaction and chronic myeloid leukemia present with an elevated leukocyte count (> 50,000/mm^3), but an elevated leukocyte alkaline phosphatase score is suggestive of a leukemoid reaction.
Multiple myeloma
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A 25-year-old man presents to his primary care physician with anxiety and fatigue. During this past week, he has been unable to properly study and prepare for final exams because he is exhausted. He has been going to bed early but is unable to get a good night’s sleep. He admits to occasional cocaine and marijuana use. Otherwise, the patient has no significant medical history and is not taking any medications. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. On physical exam, the patient is a tired and anxious appearing young man. His neurological exam is within normal limits. The patient states that he fears he will fail his courses if he does not come up with a solution. Which of the following is the most appropriate initial step in management?
Alprazolam
Melatonin
Polysomnography
Sleep hygiene education
D
Sleep hygiene education
This patient is presenting with trouble sleeping without a known organic cause, for which the most appropriate initial step in management is to discuss sleep hygiene with the patient. In any patient that presents with a chief complaint of fatigue, it is always best to assess how they are sleeping. After a physical exam is performed in order to rule out signs or symptoms of a severe organic cause (neurologic deficits), the most appropriate initial step in management is to discuss sleep hygiene with the patient. Sleep hygiene includes discussing the patient’s exposure to light (eliminate exposure to blue light at night), altering diet (no large meals or caffeine before bed), and behavioral modification (only using the bed for sleep). Once a trial of proper sleep hygiene has been tried, then it would be appropriate to potentially move on to other interventions including pharmacological interventions. Horne presents evidence regarding the best sleep hygiene practices. He discusses how exercise and avoidance of caffeine near bedtime is beneficial to helping with insomnia. He recommends using these interventions prior to pharmacologic treatment. Incorrect Answers: Answer A: Alprazolam is a benzodiazepine that could be used to help with this patient’s anxiety and could help them sleep. However, benzodiazepines are potent drugs with addictive properties and would not be used as first-line treatment for insomnia. Lifestyle modifications should be tried first to determine if these will be effective in helping the patient sleep. Answer B: Melatonin is a supplement that is often used to improve sleep quality. Starting the patient on a supplement is plausible as an initial step in management, but it would not take precedence over educating the patient regarding sleep hygiene. These interventions often are very effective and have no side effects. Answer C: Polysomnography could be the most appropriate initial step in management if an organic cause for this patient’s sleep problems (such as obstructive sleep apnea) is suspected. There is nothing suggesting an organic cause for this patient’s sleep issues and it is highly likely that a college-age student near finals period would benefit from a discussion on sleep hygiene prior to an expensive diagnostic test such as polysomnography. Answer E: Zolpidem is a muscle relaxant and hypnotic with a similar mechanism of action to a benzodiazepine. Zolpidem has the potential to be addicting and would not be tried prior to sleep hygiene interventions. Some patients who are refractory to other interventions may benefit from the use of Zolpidem. Bullet Summary: Any patient that presents with fatigue and trouble sleeping without a clear organic cause should first be counseled in sleep hygiene habits prior to any other interventions or diagnostic tests.
Zolpidem
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A 28-year-old man is brought to the emergency department by helicopter after a high speed motorcycle crash. He was intubated and 2 large bore IVs were placed in the field with administration of 2 liters of lactated ringer's solution while en route. On arrival to the emergency department, his temperature is 98.6°F (37.0°C), pulse is 130/min, blood pressure is 80/50 mmHg, respirations are 22/min, and oxygen saturation is 97%. Examination reveals an ill-appearing intubated patient. Endotracheal tube placement is confirmed with end tidal CO2, and bilateral breath sounds are present. No external bleeding or wounds are noted on exam. Pulses are present in all extremities but are weak and rapid. Significant bruising is noted over the abdomen. The pelvis is stable. Infusion of packed red blood cells is initiated. Which of the following is the most appropriate next step in management?
CT scan of the abdomen and pelvis with contrast
FAST exam
Norepinephrine infusion
Diagnostic peritoneal lavage
B
FAST exam
This patient with hemodynamic instability and abdominal bruising after a high speed motorcycle crash likely has shock secondary to blood loss. The first step in management of patients with blunt trauma and hemodynamic instability is a focused assessment with sonography for trauma (FAST exam) For any patient with blunt or penetrating trauma, the first step in management is an evaluation of airway, breathing, and circulation. This is referred to as the primary survey and includes the verification of endotracheal tube placement, auscultation of bilateral breath sounds, and assessment of external wounds or bleeding. This is followed by the secondary survey which involves a detailed head to toe inspection for injuries. Blunt abdominal trauma with bruising as well as hemodynamic instability warrants a FAST exam. The FAST exam visualizes the heart chambers and pericardium, left upper quadrant (spleen, splenorenal recess), right upper quadrant (Morrison pouch) and the rectovesical (males) or rectouterine (females) pouch. The extended FAST exam (eFAST) includes an examination of the lungs as well to assess for pneumothorax or hemothorax. Hemodynamically unstable patients with free fluid identified in the abdomen on initial FAST exam should proceed directly to the operating room for emergent surgical intervention with ongoing volume resuscitation. Patients with hemodynamic instability and a negative FAST exam should be evaluated for sources of bleeding elsewhere such as the pelvis. Barloon et al. review diagnostic imaging in the evaluation of blunt abdominal trauma. Blunt abdominal trauma is a common cause of death and injury, leading to hemodynamic instability. Intra-abdominal imaging is especially important in comatose or sedated (intubated) patients who are unable to provide a history or provide a reliable physical examination. In stable patients, CT of the abdomen often has a role in deciding whether to pursue observation versus surgical intervention. Stengel et al. evaluate emergency ultrasound-based algorithms for diagnosing blunt abdominal trauma. The systematic review showed that ultrasound-based pathways reduced the number of CT scans obtained. Use of ultrasound can enhance the speed of the primary assessment and cut costs. However, the authors conclude that there is insufficient evidence to promote justification of ultrasound-based pathways for the evaluation of blunt abdominal trauma in terms of patient outcomes. Incorrect Answers: Answer A: AP and lateral radiographs of the abdomen and pelvis may be useful for identifying pelvic fracture or intraperitoneal free air. However, this patient's presentation is suggestive of abdominal trauma warranting FAST exam. Answer B: CT scan of the abdomen and pelvis with contrast would be appropriate for evaluation of blunt abdominal trauma in a patient that is hemodynamically stable. However, this patient's vital signs indicate hemodynamic instability. Answer C: Diagnostic peritoneal lavage is no longer commonly used in the evaluation of blunt abdominal trauma. Answer E: Norepinephrine infusion would not be appropriate for this patient with shock secondary to blood loss. Resuscitation for this patient should be achieved with transfusion of blood products. Bullet Summary: The most appropriate initial step in management for hemodynamically unstable patience with blunt abdominal trauma is a FAST exam.
nan
https://step2.medbullets.com/testview?qid=216385
A 49-year-old man presents with 3 months of generalized weakness. He has had a chronic cough and a 20 pound unintentional weight loss over the past 6 months. He has a medical history significant for high blood pressure, hyperlipidemia, and seasonal allergies with no prior surgical history. He has no history of tobacco use. He moved from India 7 years prior to presentation. He currently lives in Oregon and has not left the west coast since moving. His temperature is 99°F (37.2°C), blood pressure is 100/70 mmHg, pulse is 76/min, and respirations are 16/min. His physical exam is significant for hyperpigmentation of the oral mucosa and temporal muscle wasting. Radiography of the abdomen demonstrates bilateral adrenal calcifications. Laboratory values are as follows: Serum: Na+: 130 mEq/L Cl-: 96 mEq/L K+: 5.2 mEq/L HCO3-: 24 mEq/L Morning cortisol: 2 ug/dL 30-minute cortisol during cosyntropin test: 8 ug/dL (normal > 18 ug/dL) Morning adrenocorticotropic hormone (ACTH) concentration (serum): 80 pg/mL (normal 10-60 pg/mL) Which of the following is the most appropriate next step in management?
Bilateral adrenalectomy, prednisone, and fludrocortisone
Dexamethasone
Hydrocortisone and immunoglobulin therapy
Prednisone taper
E
Rifamycin, isoniazid, pyrazinamide, ethambutol, and hydrocortisone therapy
This man from India presents with generalized weakness, chronic cough, unintentional weight loss, skin hyperpigmentation, hyponatremia, hyperkalemia, hypocortisolism, cosyntropin stimulation test failure, elevated ACTH, and bilateral adrenal calcifications on radiography. These symptoms and signs in this patient are most consistent with adrenal tuberculosis requiring RIPE therapy (rifamycin, isoniazid, pyrazinamide, ethambutol) as well as glucocorticoid and mineralocorticoid therapy. Adrenal insufficiency occurs when the adrenal glands do not produce enough cortisol and aldosterone. Primary adrenal insufficiency is due to dysfunction at the level of the adrenal glands themselves, as opposed to upstream factors such as the pituitary gland. Primary adrenal insufficiency in developed nations is most often caused by autoimmune disease. In developing nations, adrenal tuberculosis is a significant cause of primary adrenal insufficiency. Adrenal tuberculosis often presents with a history of tuberculosis or tuberculosis symptoms, as well as symptoms of adrenal insufficiency such as fatigue, unintentional weight loss, and hyperpigmentation. Patients often have hyponatremia, hyperkalemia, low morning cortisol, a failed cosyntropin test, and high ACTH concentrations. Radiography may demonstrate bilateral adrenal calcifications, and/or adrenal enlargement. Tuberculosis can infect all cortices of the adrenal glands bilaterally, and therefore treatment requires eradicating the infection with multi-drug therapy (RIPE therapy) as well as replacement of glucocorticoids and mineralocorticoids, which may be achieved by high-dose hydrocortisone or other combinations of steroids that together provide glucocorticoid and mineralocorticoid replacement. Vinnard and Blumberg outline the signs and symptoms, histopathology, lab results, imaging, differential diagnosis, and treatments associated with adrenal tuberculosis. They recommend early recognition and treatment. Incorrect Answers: Answer A: Bilateral adrenalectomy, prednisone therapy, and fludrocortisone therapy are incorrect because medical therapy should first be attempted to eradicate the infection before undergoing surgery. Bilateral adrenalectomy may be considered if there is suspicion of lung cancer with metastasis to the bilateral adrenal glands. Patients will require lifelong replacement therapy. Answer B: Dexamethasone alone is incorrect because this patient likely has tuberculosis and should be treated for it. Additionally, this patient has signs of mineralocorticoid deficiency (hyponatremia, hyperkalemia), and patients often require mineralocorticoid therapy or a high dose of a glucocorticoid with mineralocorticoid activity (e.g., hydrocortisone). Dexamethasone has minimal to absent mineralocorticoid activity. Answer C: Hydrocortisone therapy and immunoglobulin therapy are incorrect because this patient has adrenal tuberculosis. Although autoimmune adrenalitis can cause primary adrenal insufficiency, immunoglobulins are not used to treat this condition. Answer D: Prednisone taper would be appropriate for acute conditions such as an asthma or COPD exacerbation. While prednisone will treat the symptoms of primary adrenal insufficiency, it will not treat active tuberculosis infection. Primary adrenal insufficiency in developed nations is most often caused by autoimmune adrenalitis (Addison disease) and presents with fatigue, unintentional weight loss, abdominal pain, hyperpigmentation, and muscle weakness. In this condition, long-term steroid therapy is indicated. Bullet Summary: Patients with adrenal tuberculosis should be treated with rifamycin, isoniazid, pyrazinamide, ethambutol, as well as glucocorticoid and mineralocorticoid replacement.
Rifamycin, isoniazid, pyrazinamide, ethambutol, and hydrocortisone therapy
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A 66-year-old man presents to the clinic for evaluation of a skin lesion on his hand. The lesion has grown rapidly over the previous 2 weeks. He endorses trauma to the area when he accidentally scraped his hand against a window and notes that there was some bleeding. His medical history is significant for chronic renal failure for which he received a renal transplant 5 years prior, as well as multiple surgeries for skin cancer. His current medications include aspirin, atorvastatin, prednisone, tacrolimus, and mycophenolate mofetil. His temperature is 98.6°F (37°C), blood pressure is 136/91 mmHg, pulse is 82/min, and respirations are 11/min. Physical exam is notable for a 2 x 2.5 cm nodular lesion of the dorsal hand as seen in Figure A. Which of the following is the most likely diagnosis?
Basal cell carcinoma
Keratoacanthoma
Molluscum contagiosum
Pyogenic granuloma
B
Keratoacanthoma
This patient presents with a rapidly enlarging, well-circumscribed, volcano-like papule with a central keratotic core consistent with the diagnosis of keratoacanthoma. Keratoacanthomas represent a well-differentiated variant of cutaneous squamous cell carcinoma. They present as a rapidly enlarging skin lesion with a characteristic hyperkeratotic core. Most lesions occur on the sun-exposed areas of the body, especially the head, neck, and extremities. Risk factors for developing a keratoacanthoma include immunosuppression, arsenic exposure, old scars or burns, xeroderma pigmentosum, or exposure to high levels of ionizing radiation. Most keratoacanthomas tend to involute within several months after initial onset; however, surgical excision can be performed because of the histologic similarity to squamous cell carcinoma. Biopsy of lesions with malignant transformation will show atypical keratinocytes and malignant cells, invasion into dermis, and keratin “pearls” on histology. Kwiek and Schwartz present a review of keratoacanthoma diagnosis and treatment. They discuss how these lesions characteristically exhibit spontaneous regression after rapid growth. They recommend surgical excision as first-line therapy for the lesion. Figure/Illustration A is a clinical photograph showing a well-circumscribed, dome-shaped papule with a hyperkeratotic core (red circle) consistent with keratoacanthoma. Incorrect Answers: Answer A: Basal cell carcinoma is the most common skin cancer in humans. It most commonly presents as a pearly pink papule or ulcer with telangiectasias and a rolled border. Compared to keratoacanthoma, basal cell carcinomas are slow-growing and are only locally invasive. Surgical excision is the treatment of choice for most basal cell carcinomas. Answer C: Molluscum contagiosum is a common viral infection caused by a poxvirus that is most frequently seen in children. It presents with solitary or multiple umbilicated papules and is spread by person-to-person contact. Immunosuppressed individuals, such as those with HIV/AIDS, may have disseminated molluscum contagiosum, which is highly recalcitrant to treatment. Answer D: Pyogenic granulomas are vascular proliferations that present as rapidly growing, pink to red, friable papules that are prone to bleeding with minor trauma. They are most commonly seen in children and pregnant women. The oral mucosa is a common area of presentation, although pyogenic granulomas may present in any skin location. Treatment is through mechanical removal via surgical excision, laser ablation, or electrical curettage. Answer E: Verruca vulgaris is a common viral infection of the skin caused by several subtypes of human papilloma virus (HPV). They present as rough-textured or filiform papules. Close examination may reveal pinpoint areas of red/black, which represent thrombosed blood vessels within the wart. Treatment options for common warts include salicylic acid or cryotherapy; however, recurrence after treatment is common. Bullet Summary: Keratoacanthomas presents as rapidly enlarging, well-circumscribed, volcano-like papules with a central hyperkeratotic core.
Verruca vulgaris
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A 67-year-old man presents with a recurrent episode of syncope. He states that this has happened to him multiple times, particularly when he changes body position from sitting to standing, exerts himself, or turns his head quickly. He says that he notes numbness, vertigo, and sometimes even trouble with speaking immediately prior to fainting. The episodes usually last 1-5 minutes. When he wakes up, he is mildly confused but typically returns to baseline within a minute. The patient has a past medical history of diabetes, dyslipidemia, and hypertension. His temperature is 98.5°F (36.9°C), blood pressure is 153/89 mmHg, pulse is 90/min, respirations are 11/min, and oxygen saturation is 97% on room air. The patient's neurological exam is unremarkable. However, when he is asked to stand up and start walking, he experiences the same numbness and tingling and feels like he may faint. Which of the following is the most likely diagnosis?
Blood vessel narrowing
Cardiac dysrhythmia
Seizure
Stroke
A
Blood vessel narrowing
This elderly patient with risk factors for vascular disease (diabetes, dyslipidemia, hypertension) is presenting with episodes of dizziness, vertigo, numbness/tingling, dysarthria, and syncope, which seem to be provoked by standing rapidly and changing head position. This is concerning for vertebrobasilar syndrome, caused by blood vessel narrowing. Vertebrobasilar syndrome is caused by narrowing (via causes such as atherosclerosis, dissection, or congenital narrowing among others) of the vertebrobasilar vascular system. It is a common cause of syncope that may be worsened by changes in head position or standing rapidly. Other unique features include numbness/tingling, a sensation of vertigo, nausea, vomiting, dysphagia, and dysarthria. Note the episodes of syncope in this condition are intermittent and specifically provoked and do not have lasting/permanent neurological deficits in contrast to a stroke. Management is first centered on vascular imaging which may include a CTA head/neck or an MRA head/neck. Further care depends on the symptoms present but typically involves smoking cessation, glycemic control, blood pressure control, aspirin, and a statin. Xuan-Ting et al. review posterior circulation strokes which occur more frequently in patients with atherosclerosis of the vertebrobasilar system. They note that MRI is one of the most accurate tests for assessing posterior circulation strokes which may present with dizziness and syncope. It is far superior to CT imaging. For this reason, they recommend that CT imaging alone cannot definitively rule out a posterior circulation stroke. Incorrect Answers: Answer B: Cardiac dysrhythmias such as ventricular tachycardia can cause sudden onset and offset syncope without prodromal symptoms. The differentiating feature of this form of syncope is the rapid onset and offset in a high-risk patient. The diagnosis can be confirmed with an ECG; however, many patients will require prolonged observation on telemetry. Answer C: Seizure presents with impaired consciousness, tonic-clonic activity, and a post-ictal state which gradually improves over hours. The lactate may be elevated after a seizure, and the prolactin level is a more specific indicator of a seizure. However, the diagnosis is most appropriately made based on history, exam, and EEG. Answer D: Stroke involving the vertebrobasilar system may cause syncope in addition to ongoing dizziness and vertigo with ataxia. However, this patient's symptoms seem to be positional and have entirely resolved; thus, a stroke is unlikely. He is at higher risk of stroke given his vertebrobasilar insufficiency and an MRI should still be performed in this patient. Answer E: Vagal event presents with a warm prodrome with palpitations, flushing, and syncope. Muscle twitching (but no tonic-clonic activity) may be seen. Patients may wake up slightly confused but return to baseline within seconds. Bullet Summary: Vertebrobasilar insufficiency may present with positional episodes of syncope caused by insufficient blood flow through the vertebrobasilar blood vessels.
Vagal event
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A 27-year-old woman presents to the emergency department with altered mental status. Her boyfriend found her at home next to a suicide note and brought her into the emergency department. According to her boyfriend, the patient has a medical history of bipolar disorder for which she takes lithium, as well as chronic lower back pain for which she sees a pain specialist. Her temperature is 100.5°F (38.1°C), blood pressure is 102/78 mmHg, pulse is 127/min, respirations are 19/min, and oxygen saturation is 99% on room air. An ECG is performed as seen in Figure A. Which of the following findings is also likely to be present at some point in this patient’s course?
Miosis
Diaphoresis
Urinary retention
Wheezing
C
Urinary retention
This patient is presenting after a suicide attempt with an unknown overdose and is confused, febrile, tachycardic, and has QRS widening on ECG. This combination of symptoms is highly concerning for a tricyclic antidepressant (TCA) overdose which can cause an anticholinergic toxidrome and urinary retention. Tricyclic antidepressants (TCAs) were once a mainstay in the treatment of depression before the advent of selective serotonin reuptake inhibitors (SSRIs) and serotonin–norepinephrine reuptake inhibitors (SNRIs). They are sometimes still used for depression and neuropathic pain. In overdose, TCAs block sodium channels in the heart and cause a pathognomonic QRS widening. Sodium bicarbonate should be administered until the QRS duration normalizes. TCAs also have anticholinergic properties and can cause an anticholinergic toxidrome including delirium, confusion, constipation, decreased bowel sounds, urinary retention, dry, warm, and flushed skin, and fever. Although physostigmine is typically the antidote of choice for an anticholinergic toxidrome, it is avoided in TCA toxicity due to the theoretical concern for inducing a cardiac arrest. Urquhart et al. studied the utility of tricyclic antidepressants in the treatment of chronic lower back pain. They found no significant difference in pain reduction compared to placebo. They recommend further study of the potential benefits of TCAs, independent of sleep improvement, for the treatment of lower back pain. Figure/Illustration A is an ECG demonstrating QRS widening (note how long the red lines are which demarcate the QRS duration) which is highly concerning for TCA overdose. Incorrect Answers: Answer A: Diaphoresis, tachycardia, increased bowel sounds, and agitation would be seen in a sympathomimetic toxidrome from drugs like cocaine or amphetamines. Rather, dry, flushed skin would be seen in an anticholinergic toxidrome. Answer B: Jaundice, nausea, vomiting, and abdominal pain would be seen in acetaminophen overdose which can cause fulminant liver failure. It is also possible that patients may initially be asymptomatic in acetaminophen overdose, and a high level of suspicion must be maintained for this diagnosis. A serum acetaminophen level should be drawn in any patient suspected of overdose. Answer C: Miosis, bradypnea, decreased bowel sounds, and somnolence would be seen in an opioid toxidrome. Rather, dilated and minimally responsive pupils would be seen in an anticholinergic toxidrome. Answer D: Wheezing, bronchorrhea, urinary incontinence, fecal incontinence, confusion, and drooling would be seen in a cholinergic toxidrome from agents such as organophosphates or nerve gases like sarin. None of these symptoms are seen in this patient. Bullet Summary: A tricyclic antidepressant overdose can cause QRS widening on ECG as well as an anticholinergic toxidrome (dry, flushed skin, constipation, decreased bowel sounds, urinary retention, and altered mental status).
nan
https://bit.ly/45Vk2SF
A 3-year-old boy presents with his parents to a pediatrician for a new patient visit. The child was recently adopted and little is known about his medical or family history. The child seems to be doing well, but the patient is much larger than any of the other 3-year-olds in his preschool class. He eats a varied diet at home and with limited juice and snack foods. His temperature is 98.6°F (37°C), blood pressure is 101/67 mmHg, pulse is 110/min, and respirations are 24/min. On physical exam, the patient is in the 73rd percentile for weight, 99th percentile for height, and 86th percentile for head circumference. He appears mildly developmentally delayed. He has a fair complexion and tall stature with a prominent sternum. The patient also has joint hypermobility and hyperelastic skin. He appears to have poor visual acuity and is referred to an ophthalmologist, who finds downward lens subluxation of the right eye. This child is most likely to develop which of the following complications?
Aortic dissection
Medullary thyroid cancer
Osteoarthritis
Thromboembolic stroke
D
Thromboembolic stroke
This patient presents with Marfanoid habitus, joint hypermobility, hyperelastic skin, developmental delay, and downward lens subluxation, which suggests a diagnosis of homocystinuria. Homocystinuria predisposes to thromboembolic events such as stroke. Although homocystinuria shares many features with Marfan syndrome, patients with homocystinuria also present with a fair complexion, intellectual disability, and downward subluxation of the lens (instead of the upward subluxation seen in Marfan syndrome). Patients with homocystinuria are also at an increased risk of thromboembolic events that disproportionally affect the cerebral vessels. Similar to Marfan syndrome, patients have phenotypic features of a Marfanoid habitus, including tall stature, long limbs, arachnodactyly, joint hypermobility, skin hyperelasticity, pectus deformity, and scoliosis. Treatment includes vitamin B6 supplementation. Hasan et al. review the evidence regarding diseases associated with homocystinuria. They discuss how this disease is associated with cancer and Alzheimer disease. They recommend screening for this disease. Incorrect Answers: Answer A: Aortic dissection is a complication of Marfan syndrome, an autosomal dominant mutation of the fibrillin-1 gene. It is also associated with elevated homocysteine levels. Although Marfan syndrome shares many features with homocystinuria, the fair complexion, and intellectual disability seen in this patient are specific to homocystinuria. Treatment involves avoidance of contact sports and beta blockers. Answer B: Medullary thyroid cancer is part of the syndrome of multiple endocrine neoplasia type 2B (MEN2B), which is caused by a mutation in the RET proto-oncogene. Patients with MEN2B present with Marfanoid habitus, but they do not present with the other features described in this vignette, including fair complexion, intellectual disability, and downward lens subluxation. Treatment is with surgical excision of tumors. Answer C: Osteoarthritis is a complication of Ehlers-Danlos syndrome (EDS). Although patients with EDS may present with many of the phenotypic features of both Marfan syndrome and homocystinuria, patients are much less likely to have lens subluxation. Osteoarthritis may be seen with higher incidence in patients with homocystinuria. Treatment is with brace support of unstable joints. Answer E: Wilms tumor is associated with Beckwith-Wiedemann syndrome (BWS), a pediatric overgrowth disorder usually caused by a mutation at chromosome 11p15. BWS presents with macrosomia (“gigantism”), which is defined as height and weight > 97th percentile. BWS additionally presents with omphalocele, hemihyperplasia, visceromegaly, neonatal hypoglycemia, and other embryonal tumors, including hepatoblastoma and neuroblastoma. Treatment is with surgical correction of defects. Bullet Summary: Homocystinuria presents with Marfanoid habitus, fair complexion, intellectual disability, downward lens subluxation, and an increased risk of thromboembolic events.
Wilms tumor
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A 2-day-old boy has an episode of vomiting in the hospital nursery. The vomitus was described as “bright green” without any traces of blood. The patient has urinated several times since he was born but has not passed any stool. He was born at 37 weeks of gestation to a 38-year-old G3P3 woman. The pregnancy was uncomplicated and the patient’s mother refused all prenatal testing. The patient’s 2 older siblings are both healthy. His temperature is 98.6°F (37°C), blood pressure is 67/43 mmHg, pulse is 135/min, and respirations are 34/min. On physical exam, the patient has upslanting palpebral fissures, epicanthal folds, and a single transverse palmar crease. His abdomen is non-tender, firm, and distended. Bowel sounds are hypoactive. Digital rectal exam evacuates a small amount of stool and flatulence. A nasogastric tube is placed to decompress the stomach. The patient’s abdominal radiograph can be seen in Figure A. Which of the following is the most likely diagnosis?
Pyloric stenosis
Intestinal malrotation
Hirschsprung disease
Meconium ileus
C
Hirschsprung disease
This pediatric patient presents with features of Down syndrome (trisomy 21), bilious vomiting, and colonic distention on an abdominal radiograph, which suggests a diagnosis of Hirschsprung disease. Hirschsprung disease is associated with Down syndrome and presents with bilious vomiting, failure to pass meconium in the first 48 hours of life, and abdominal distension. A digital rectal exam may temporarily relieve the obstruction and lead to the expulsion of gas and stool (the “squirt sign”). Abdominal radiography typically demonstrates signs of distal intestinal obstruction such as proximal dilation and an absence of air in the rectum. Contrast enema may reveal a transition zone between the dilated proximal megacolon and the narrow rectosigmoid colon. A biopsy can confirm the diagnosis. Treatment is with surgical removal of the defective segment of the bowel. Kessman reviews the evidence regarding the diagnosis and treatment of Hirschsprung disease. She discusses how the diagnosis can be made using a rectal suction biopsy. She recommends monitoring patients closely for enterocolitis for years after surgical treatment. Figure/Illustration A is an abdominal radiograph that demonstrates colonic distension (red circle). This finding is consistent with a diagnosis of Hirschsprung disease. Incorrect Answers: Answer A: Duodenal atresia is associated with Down syndrome (trisomy 21) and may also present in the first 48 hours of life with bilious vomiting. The abdominal radiograph will demonstrate the “double bubble” sign, which reflects air trapping in the stomach and first portion of the duodenum. Treatment is with the surgical opening of the atretic segment. Answer C: Intestinal malrotation may preset with bilious vomiting in a neonate due to the development of a midgut volvulus. An abdominal radiograph may show the extension of the nasogastric tube into an abnormally positioned duodenum and would not show diffuse colonic distension. Treatment is with surgical derotation of the volvulus. Answer D: Meconium ileus is associated with cystic fibrosis and also presents with delayed (> 48 hours) passing of meconium. The distal obstruction in these patients will not be temporarily relieved by a digital rectal exam. Contrast enema will demonstrate microcolon involving the entire large bowel. Treatment is with irrigation and removal of the meconium. Answer E: Pyloric stenosis usually presents in the first 3-6 weeks of life with post-prandial, nonbilious, projectile vomiting and a palpable olive-shaped mass in the epigastrium. This patient is presenting at just 2 days of life and has bilious vomiting. Treatment is with surgical release of the stenosis. Bullet Summary: Hirschsprung disease is associated with Down syndrome (trisomy 21) and presents with retention of stool that can be relieved by digital disimpaction, bilious vomiting, and colonic distention on abdominal radiography.
nan
https://bit.ly/3P8eDBc
A 37-year-old woman presents to her physician for mood changes and recent falls over the course of 3 months. She feels more irritable with her husband and children and does not find pleasure in creating art anymore. Over the last 2 weeks, she has been sleeping approximately 5 hours a night and had a fall while walking in her home with the lights off. She denies any head trauma or loss of consciousness associated with the fall. She has a medical history of generalized anxiety disorder and hypothyroidism for which she takes escitalopram and levothyroxine. She had a Roux-en-Y gastric bypass 5 years ago. She has been in a monogamous relationship with her husband for the last 15 years and uses a copper intrauterine device. Physical examination is notable for a depressed affect. Pupils are equal, round, and reactive to light and accommodation. She has 4/5 strength in the bilateral lower extremities and brisk patellar reflexes. Which of the following is most likely the cause of this patient's symptoms?
Subtherapeutic levothyroxine dose
Reduced functional parietal cell count
Reduced central nervous system dopamine and serotonin
Poor iron absorption
B
Reduced functional parietal cell count
This patient's mood changes and findings concerning for subacute combined degeneration (fall in the dark, impaired proprioception, weakness, and brisk patellar reflexes), and history of Roux-en-Y gastric bypass surgery are concerning for vitamin B12 deficiency. Roux-en-y gastric bypass reduces parietal cell count, leading to reduced intrinsic factor secretion, which is needed for vitamin B12 absorption. Roux-en-Y gastric bypass is a laparoscopic bariatric procedure that creates a small gastric pouch from the stomach with a gastrojejunal and jejunojejunal anastomosis. This causes small volumes of food to be consumed and decreases nutrient absorption. The small gastric pouch contains very few parietal cells, which decreases hydrochloric acid and intrinsic factor secretion. The reduced intrinsic factor secretion impairs the protection of vitamin B12 against HCl and reduces absorption in the ileum. Vitamin B12 deficiency can lead to neuropsychiatric changes (depressed mood, irritability, and insomnia) and subacute combined degeneration (weakness, sensory ataxia, peripheral neuropathy, and upper motor neuron signs). Patients who undergo Roux-en-Y gastric bypass must be given high doses of vitamin B12 (often intramuscularly) in order to prevent deficiency. Alexandrou et al. review long-term micronutrient deficiencies after sleeve gastrectomy and Roux-en-Y gastric bypass (RYGB). The authors find that vitamin B12 deficiency was significantly higher in patients with RYGB compared with sleeve gastrectomy. The authors recommend adherence to supplemental iron and vitamin intake in all patients who undergo bariatric surgery. Incorrect Answers: Answer A: Poor iron absorption is a well-known complication of Roux-en-Y gastric bypass surgery due to bypass of the duodenum and proximal jejunum. Poor iron absorption leads to microcytic anemia from iron deficiency and presents with generalized fatigue and conjunctival pallor. Iron deficiency anemia would not present with brisk reflexes and impaired proprioception. Answer B: Prior Treponema pallidum exposure can result in tabes dorsalis, a neurological complication of syphilis, and is seen in patients with a history of multiple sexual partners who do not use barrier protection. The spirochete leads to disease in the posterior columns (vibration and proprioception sensation). Patients present with sensory ataxia (similar to subacute combined degeneration), lancinating pain (in the limbs, back, or face), and an Argyll-Robertson pupil (impaired pupillary constriction to light but intact pupillary constriction to accommodation). Answer C: Reduced central nervous system dopamine and serotonin are seen in patients with major depressive disorder which does not cause sensory ataxia with brisk reflexes. Answer E: Subtherapeutic levothyroxine dose would result in hypothyroidism, which can present with depressed mood, cold intolerance, bradycardia, and delayed relaxation of deep tendon reflexes. Roux-en-Y is an effective means of losing weight, and as the body mass decreases with weight loss, the required dose of levothyroxine is decreased. It would not present with the abnormal neurologic findings seen in this patient. Bullet Summary: Roux-en-Y bariatric surgery increases the risk of vitamin B12 deficiency via reduction of food intake, decreased functional parietal cell count, and reduced absorption of vitamin B12 (which may cause subacute combined degeneration).
nan
https://step2.medbullets.com/testview?qid=216396
A 67-year-old woman presents from a nursing home with altered mental status. She was last known to be normal the night before. The patient has dementia at baseline and is minimally interactive. This morning, she was found to be obtunded. No further history was provided from the nursing home, and the patient is unable to provide any history. Her temperature is 95.0°F (35.0°C), blood pressure is 84/54 mmHg, pulse is 50/min, respirations are 9/min, and oxygen saturation is 98% on room air. Physical exam reveals an obtunded woman who only withdraws her extremities to pain. Her skin is cool and pale, and her hair is thin. There are no signs of trauma noted on exam. Laboratory values are notable for a whole blood sodium of 120 mEq/L. The patient is given several intravenous boluses of fluids and her blood pressure improves to 100/60 mmHg. Which of the following is most likely to confirm the underlying cause of this patient's symptoms?
Perform an echocardiogram
Administer glucagon
Check serum TSH and free T4 levels
Perform a CT scan of the head
C
Check serum TSH and free T4 levels
This patient is presenting with obtundation, bradycardia, hypotension, hypothermia, thinning of her hair, and hyponatremia, which are concerning for myxedema coma. A serum TSH and free T4 would be most informative in confirming this diagnosis. Myxedema coma (decompensated hypothyroidism) is the most life-threatening manifestation of hypothyroidism. Key clinical features include hypothermia (often the temperature is <95.9°F (35.5°C)), bradycardia, hypotension, bradypnea (causing a respiratory acidosis), hypoxia, and obtundation. Immediate management is centered on warming the patient, warmed IV fluids, and protecting the patient's airway, if needed. If there is high clinical suspicion for myxedema coma, then IV levothyroxine can be given empirically. Otherwise, the TSH and free T4 should be checked, which will reveal an elevated TSH and a low free T4. Patients are often critically ill and require active rewarming and close monitoring in an ICU setting. Wall reviews myxedema coma. Wall notes that myxedema coma is a life-threatening manifestation of hypothyroidism. She recommends that generally IV T4 (levothyroxine) is preferred over IV T3 in initial management. Incorrect Answers: Answer A: Administering glucagon would be appropriate in a beta-blocker overdose, which presents with bradycardia, hypotension, and altered mental status. There is no indication that this patient intentionally overdosed, and her hyponatremia and thinning hair supports a diagnosis of hypothyroidism. Other treatments that can be given in a beta-blocker overdose including epinephrine, calcium, insulin, dextrose, and lipid emulsion therapy. Answer C: Obtaining blood cultures and a serum lactate is the appropriate management of sepsis/septic shock. Generally, patients with septic shock present with hypotension (refractory to fluid resuscitation), tachycardia, and fever or hypothermia. Though myocardial depression may happen in critically ill patients who are about to undergo cardiac arrest, hypothyroidism better explains this patient's constellation of symptoms. It would be appropriate to obtain cultures and a serum lactate in this patient while the differential is being narrowed. Answer D: Performing a CT scan of the head would be indicated at some point in the workup of patients with altered mental status if there was a suspected central nervous system etiology such as a subdural hematoma. However, hemodynamically unstable patients should never be sent to the CT scanner prior to stabilization and resuscitation. A subdural hematoma is more common in older patients and patients with alcohol use disorder who experience head trauma as the atrophy of their brain makes the bridging veins more vulnerable. Answer E: Performing an echocardiogram may show reduced cardiac function; however, it is unlikely to reveal the underlying cause of this patient's hypotension and bradycardia other than showing poor cardiac function. Bullet Summary: Myxedema coma presents as a critically ill, obtunded patient who is hypothermic, bradycardic, and hypotensive, and the diagnosis can be supported with a high TSH and a low free T4.
nan
https://bit.ly/3MCzFqw
A 23-year-old woman presents to the emergency department complaining of a worsening headache. The headache started 1 month ago. It is constant and “all over” but gets worse when she is lying down or in the setting of bright lights. A review of systems is significant for a low-grade fever, night sweats, cough, malaise, poor appetite, and unintentional weight loss of 12 pounds in the last 2 months. The patient is sexually active with multiple male partners and reports inconsistent condom use. She has a history of intravenous drug use and has not been to a doctor in the last 2 years. Her temperature is 100.4°F (38.0°C), blood pressure is 110/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 98% O2 on room air. On physical exam, pain is elicited upon passive flexion of the patient’s neck. A CT scan shows ventricular enlargement. A CD4+ count is 57 cells/µL blood. A lumbar puncture is performed with the following findings: Cerebrospinal fluid: Opening pressure: 210 mmH2O Glucose: 32 mg/dL Protein: 204 mg/dL India ink stain: Positive Leukocyte count and differential: Leukocyte count: 200/mm^3 Lymphocytes: 100% Red blood cell count: 2/mm^3 Which of the following treatments most directly addresses the underlying cause of this patient's symptoms?
Fluconazole
Dexamethasone
Acyclovir
Amphotericin B and 5-flucytosine
D
Amphotericin B and 5-flucytosine
The patient with a history of unprotected sex and intravenous drug use who presents with sub-acute fever, meningismus, photophobia, a low CD4+ count, and a positive India ink-stained lumbar puncture most likely has cryptococcal meningitis. Acute treatment for cryptococcal meningitis is intrathecal amphotericin B and 5-flucytosine. Cryptococcal meningitis is the most common fungal meningeal infection in patients with HIV, especially those with a CD4+ count < 100/mm^3. Symptoms are normally gradual in onset and can include headache, fever, neck stiffness, and photophobia. The most appropriate initial test is a lumbar puncture with an India ink stain. Cerebrospinal fluid in fungal meningitis would have low glucose, elevated protein, and an elevated lymphocyte-dominant leukocyte count. The most accurate diagnostic test is latex particle agglutination. Intrathecal amphotericin B and 5-flucytosine are the first-line treatments for acute management. Once the patient is treated, fluconazole is given for life or until the patient’s CD4+ count is > 100/mm^3. Badali et al. present a case of a patient with cryptococcal meningitis. They discuss how despite immediate antifungal therapy, the patient died due to a rapidly progressive infection. They recommend aggressive intrathecal therapy to treat this disease. Incorrect Answers: Answer A: Acyclovir is used to treat Herpes simplex virus encephalitis. Cerebrospinal fluid would show normal protein and glucose, an elevated lymphocyte-dominant leukocyte count, and an elevated red blood cell count. The Herpes virus can lead to a hemorrhage of the temporal lobes. Answer C: Dexamethasone is given in addition to IV antibiotics if Streptococcus pneumoniae meningitis is suspected. This is the most common cause of meningitis in adults, elderly, and asplenic patients, and can spread to cause sinusitis, otitis media, or bacteremia. Answer D: Fluconazole is used for prophylaxis against cryptococcal meningitis after a person is treated acutely. It is either given for life, or until the patient’s CD4+ count is > 100/mm^3. This treatment is not sufficient for patients with acute meningitis with neurological changes. Answer E: Vancomycin and ceftriaxone are given to a patient suspected of having bacterial meningitis until culture results confirm the bacterial organism. Bacterial meningitis would have decreased glucose, elevated protein, and an elevated neutrophil-dominant leukocyte count. Bullet Summary: Cryptococcal meningitis classically presents in a patient with a CD4+ count < 100/mm^3 and should be treated acutely with intrathecal amphotericin B and 5-flucytosine.
nan
https://bit.ly/460v5JM
A 23-year-old woman presents with a 7-day history of abdominal pain, and now bloody diarrhea that brings her to her primary care physician. Review of systems is notable for a 12-pound unintentional weight loss and intermittent loose stools. She has a family history notable for a father with coronary artery disease and a mother with primary sclerosing cholangitis. Her temperature is 37.2°C (98.9°F), blood pressure is 100/72 mmHg, pulse is 72/min, respirations are 12/min, and oxygen saturation is 100% on room air. Her abdomen is soft, mildly diffusely tender to deep palpation, and non-distended. She is found to have the finding on colonoscopy in Figure A. Serum perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) are positive. Which of the following is most likely to be present in this patient?
Continuous lesion beginning in the rectum
Fistulae and stricture formation
Worse disease severity near the ileocecal valve
Perianal disease
A
Continuous lesion beginning in the rectum
This patient presents with features suggestive of inflammatory bowel disease (IBD), and her colonoscopic and serologic studies suggest ulcerative colitis (UC). UC typically begins in the rectum and progresses proximally towards the ileocecal valve in a continuous fashion. The diagnosis of IBD should always prompt further investigation to better delineate between UC, Crohn disease, or indeterminate colitis. Further delineation guides treatment and informs prognosis. UC has a peak incidence in the second and third decades of life. It is characterized by disease extending from the rectum proximally towards the ileocecal valve in a continuous fashion. UC presents as mucosal and submucosal inflammation, with characteristic crypt distortion and presence of crypt abscesses. P-ANCA is positive in most individuals with UC. Repeated mucosal injury can induce pseudopolyp formation, and pancolitis or disease in the descending colon warrants screening colonoscopy due to increased risk of colon cancer. On imaging, the colon can be dilated by edema, losing its haustral markings and giving it a "lead pipe" appearance. Gajendran et al. review the pathophysiology, diagnosis, and treatment of UC. They found that colonoscopy with biopsy is the best way to make a definitive diagnosis of UC. They recommend the Mayo scoring system as a commonly used method of assessing disease severity, guiding treatment options, and monitoring patients during therapy. Figure A is a colonoscopy image showing continuous colonic mucosal inflammation typical of ulcerative colitis. Incorrect Answer: Answer A: Cobblestoning and skip lesions refer to the patchy areas of inflammation noted in Crohn disease. In contrast, the inflammation seen in UC involves continuous stretches of colon mucosa. Answer C: Fistulae and stricture formation are features of Crohn disease, not UC. Fistulizing and stricture formation can affect the entire gastrointestinal tract in Crohn disease. Answer D: Perianal disease is a feature of Crohn disease. Although UC commonly affects the rectum, it tends to spare the anus. Answer E: Worse disease severity near the ileocecal valve is a feature of Crohn disease and not UC. UC tends to be characterized by more uniform disease severity spanning in continuous fashion from rectum towards the cecum. Bullet Summary: Ulcerative colitis is characterized by disease extending from the rectum proximally towards the ileocecal valve in a continuous fashion.
nan
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A 55-year-old man presents to the emergency department with nausea and vomiting for the past week. He decided to come in when his symptoms worsened. He feels that his symptoms are exacerbated with large fatty meals and when he drinks alcohol. His wife recently returned from a cruise with symptoms of vomiting and diarrhea. The patient has a medical history of poorly managed diabetes, constipation, anxiety, dyslipidemia, and hypertension. His temperature is 99.5°F (37.5°C), blood pressure is 197/128 mmHg, pulse is 100/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals a systolic murmur heard loudest along the left upper sternal border. Abdominal exam reveals an obese, tympanitic and distended abdomen with a 3 cm scar in the right lower quadrant. Vascular exam reveals weak pulses in the lower extremities. Which of the following is the most likely underlying etiology of this patient's symptoms?
Adhesions
Enteric nervous system damage
Impacted stool
Twisting of the bowel
A
Adhesions
This patient is presenting with abdominal pain and a tympanitic abdomen, which are symptoms suggestive of a small bowel obstruction. In developed countries, these typically occur secondary to adhesions. Small bowel obstructions (SBO) typically present with an absence of bowel sounds, bowel movements, and flatus, as well as abdominal distension, nausea, and vomiting. They typically occur secondary to adhesions which entrap the bowel and disrupt its normal peristalsis. Adhesions can occur after abdominal surgery or trauma as part of the normal healing process. The diagnosis of SBO can be confirmed using abdominal imaging such as a CT scan. Treatment often involves observation; however, surgical decompression of the SBO may be needed if there is a closed-loop obstruction or an obstruction that does not resolve on its own. Rami Reddy and Cappell review the evidence regarding the diagnosis and treatment of SBO. They discuss how this disease has a 30% mortality rate if the diagnosis is missed and bowel perforation occurs. They recommend early evaluation and treatment of these patients. Incorrect Answers: Answer B: Enteric nervous system damage describes diabetic gastroparesis which can present similarly to SBO; however, this patient's age and history of surgery points toward SBO as the most likely diagnosis. Treatment is with pro-motility agents. Answer C: Impacted stool describes constipation which could present similarly to SBO; however, this diagnosis is less likely to cause nausea and vomiting unless it is severe. The vomit typically smells like stool. Treatment is with dis-impaction of the colon. Answer D: Norovirus typically causes nausea, vomiting, and diarrhea but does not cause distension and a tympanitic abdomen. Treatment is supportive as the virus self-resolves. Answer E: Twisting of bowel describes a volvulus which presents similarly to SBO; however, it is a more common diagnosis in the elderly and is a less common diagnosis than is SBO in a patient with a history of abdominal surgery. Treatment is with surgical untwisting of the bowel. Bullet Summary: Small bowel obstruction (commonly caused by adhesions) presents with nausea, vomiting, absence of flatus/bowel movements, and a distended/tympanic abdomen.
nan
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A 51-year-old man presents for his annual wellness visit with his primary care physician. His wife has noticed a tremor while he is working on his car and knows that his father died of Parkinson disease. He says that the tremor is nothing to worry about and only occurs during a few activities such as pouring drinks, pointing the remote at the television, or fixing his car. The patient states his wife is just overreacting. His medical problems include hypertension, diabetes, and hyperlipidemia for which he takes lisinopril, metformin, and atorvastatin. He has 3-5 drinks of scotch per night before working on his car. On physical exam, the tremor is replicated during finger to nose testing. Romberg sign is negative. Cranial nerves II-XII are intact. The rest of the exam is benign. Which of the following is the most appropriate treatment for this patient's tremor?
Alcoholics anonymous
Aripiprazole
Carbidopa
Phenobarbital
E
Propranolol
This patient who presents with a tremor that only occurs during terminal activity and is likely improved by alcohol (hence why he drinks prior to working on the car) has benign essential tremor. This is best treated with propranolol. Benign essential tremor is a type of rhythmic oscillatory movement that occurs with posturing and action. The pathophysiology of this disorder is unclear; however, it classically gets worse with actions that require terminal positioning of the extremities such as pouring a drink or pointing a television remote. The tremor improves with alcohol consumption so patients will often self-medicate with alcoholic drinks. There is a strong genetic component, with an autosomal dominant pattern of inheritance with incomplete penetrance. The best method of replicating the tremor is with the finger-nose-finger test because this requires the patient to perform a precise activity at a terminal distance. The diagnosis is made clinically. Treatment is with propranolol or primidone, which are both considered first-line agents although the latter has less evidence for its efficacy. Paparella et al. studied the effects of propranolol on head and upper extremity movements in patients with benign essential tremors. They found that this medication has no effect on head movements but reduces the number of upper extremity tremors in these patients. They recommended that agents other than propranolol should be used for the treatment of head tremors in these patients. Incorrect Answers: Answer A: Alcoholics anonymous is the first-line treatment for patients with alcohol use disorder; however, the tremors that occur during alcohol withdrawal are not isolated to movements during terminal activities. Alcohol withdrawal also presents with other signs including restlessness, diaphoresis, and insomnia. More severe cases can present with hallucinations and seizures. The response of this patient's tremor to alcohol consumption is typical of patients with benign essential tremor. Answer B: Aripiprazole and other atypical antipsychotic medications can be used in patients with Tourette syndrome; however, these patients will present with involuntary tics rather than a tremor that only occurs during a voluntary activity at the terminal range of motion. Moreover, for the diagnosis of Tourette syndrome, both motor and phonic tics must be present. Answer C: Carbidopa can be used in the treatment of Parkinson disease; however, the tremor associated with this disease classically occurs at rest and is described in character as a "pill-rolling" tremor. The other features of Parkinson disease include bradykinesia or akinesia, rigidity, and postural instability. Answer D: Phenobarbital can be used in the treatment of Huntington disease; however, the involuntary movements seen in this disease are classically choreiform rather than intention tremor. Huntington disease presents with chorea, psychiatric illness, and depression. There is commonly a strong genetic predisposition, as it is a trinucleotide repeat disorder that exhibits anticipation. Bullet Summary: Propranolol can be used in the treatment of patients with benign essential tremor, which presents with an action tremor that is alleviated by alcohol use.
Propranolol
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A 25-year-old man presents to his primary care physician for trouble with focus and concentration. He states that he lives at home with his parents his entire life and recently was able to get a job at a local factory. Ever since the patient started working, he has had trouble focusing on his job. He is unable to stay focused on any task. His boss often observes him "daydreaming" with a blank stare off into space. His boss will have to yell at him to startle him back to work. The patient states that he feels fatigued all the time and sometimes will suddenly fall asleep while operating equipment. He has tried going to bed early for the past month but is unable to fall asleep until 2 hours prior to his alarm. His temperature is 98.6°F (37°C), blood pressure is 112/68 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is within normal limits. The patient fears that if this continues he will lose his job. Which of the following is the most appropriate initial step in management?
Bright light therapy and decreased light at night
Ethosuximide
Modafinil
Polysomnography
A
Bright light therapy and decreased light at night
This patient is presenting with fatigue and an inability to fall asleep until late in the morning suggesting a diagnosis of delayed sleep phase syndrome. The most appropriate initial therapy is light therapy, behavioral therapy, and sleep hygiene practices in addition to decreasing exposure to light at night. Delayed sleep phase syndrome typically presents with individuals who naturally fall asleep very late and wake up very late. This becomes problematic when they attend school or have a job that requires them to wake up early. The most appropriate initial therapy for delayed sleep phase syndrome is behavioral therapy, sleep hygiene practices, and bright light therapy. Bright light therapy involves consistent timed exposure to a light box to simulate natural outdoor light, with the hope of resetting the circadian rhythm and moving the sleep phase earlier. If these measures fail, or if a different diagnosis is suspected, a referral for polysomnography may be indicated. Sletten et al. studied whether melatonin was effective in the treatment of delayed sleep phase syndrome. They found that using this medication as an adjunct to sleep hygiene was effective in improving symptoms. They recommend using multimodal therapy for this disease. Incorrect Answers: Answer A: Ethosuximide is the treatment of choice for absence seizures. Though this patient does have episodes of blank staring, this likely does not represent a seizure as he is arousable to return to work by his boss. Patients with absence seizures typically present in childhood and are unable to be aroused during seizure episodes. Answer B: Polysomnography or a sleep study would be indicated after sleep hygiene practices have failed. Polysomnography could be the most appropriate initial step in management if there was a suspected diagnosis of obstructive sleep apnea or an unknown cause of this patient's fatigue. Patients with sleep apnea typically snore and this disease is associated with obesity and increasing age. Answer D: Modafinil is a treatment for narcolepsy. Though this patient does have sudden episodes of falling asleep while at work, this is more likely to be related to his sleep schedule. Patients with narcolepsy generally do not feel tired immediately before their episodes of sleep. Answer E: Zolpidem is a muscle relaxant and hypnotic with the mechanism of action of a benzodiazepine. Though a drug like zolpidem could be used to help this patient fall asleep, the most appropriate first step is to address the patient's sleep hygiene prior to prescribing a potentially addictive drug. Bullet Summary: The most appropriate initial step in the management of patients with delayed sleep phase syndrome is behavioral therapy, bright light therapy, and sleep hygiene.
Zolpidem
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A 72-year-old man presents to the clinic for his annual exam. For the past few months, he has pain in his chest with physical activity. The pain goes away after he takes a break. He reports no chest pain while he is resting, and the pain is not worsening. His past medical history is notable for hypertension, type 2 diabetes mellitus, and hyperlipidemia. His medications include amlodipine, atorvastatin, and metformin. His temperature is 36.5°C (97.7°F), blood pressure is 132/80 mmHg, pulse is 74/min, respirations are 14/min, and oxygen saturation is 98% on room air. He has a regular rate and rhythm, normal S1 and S2, and no murmurs, rubs, or gallops. His anterior, lateral, and posterior chest are non-tender to palpation. His electrocardiogram is shown in Figure A. Troponin I level is < 0.017 ng/mL. Which of the following is the most likely diagnosis?
Costochondritis
Non-ST elevation myocardial infarction
ST elevation myocardial infarction
Stable angina
D
Stable angina
This patient who presents with exertional chest pain that is relieved with rest without increasing frequency or intensity of chest pain, a normal electrocardiogram (ECG), and normal troponin level most likely has stable angina. Angina pectoris is chest pain secondary to myocardial ischemia and is most commonly caused by atherosclerosis, though it can be caused by any etiology leading to narrowing of the coronary arteries. The pain tends to be substernal, dull, squeezing, or pressure-like and can also radiate to the neck or arm. It may also be associated with shortness of breath, dizziness, lightheadedness, nausea/vomiting, or diaphoresis. In stable angina, the chest pain is precipitated by exertion or stress and is consistently relieved with rest or nitroglycerin. ECG tends to be normal, and troponin I levels are not elevated. Treatment of stable angina includes beta blockers and nitrates (for episodes of pain), as well as therapy to prevent disease progression disease progression which may include antiplatelet therapy (aspirin), lipid-lowering medications, and glycemic control. Lifestyle measures are also the mainstay of therapy and may include smoking cessation, exercise, and dietary modification. Joshi and de Lemos review stable angina. They note the optimal management of this condition. They recommend lifestyle interventions, lipid-lowering therapy, and antiplatelets to reduce morbidity and mortality. Figure A shows an electrocardiogram with regular rate and rhythm. Every P wave is followed by a narrow QRS complex, which is consistent with normal sinus rhythm. Incorrect Answers: Answer A: Costochondritis presents with pain of the chest wall that can be sharp, aching, or pressure-like and typically worsens with cough or deep breathing. It is typically caused by injury to the chest, physical strain, or can be associated with inflammatory conditions such as arthritis. On exam, the pain can be reproduced with palpation of the chest wall, in particular, the costochondral junction. This patient's chest pain is associated with exertion and relieved with rest, and his pain is not reproduced upon palpation of the chest wall. Thus, he is unlikely to have costochondritis. Answer B: Non-ST elevation myocardial infarction presents similarly to unstable angina with substernal chest pain that is new-onset, accelerating, or occurring at rest, without ST elevations on ECG. However, ST depressions or T wave inversions may be present on ECG, and troponin I and creatine kinase-MB isoenzyme (CK-MB) would be elevated, indicating myocardial injury. Answer C: ST-elevation myocardial infarction presents with acute onset substernal chest pain that can radiate to the neck, arm, shoulders, or jaw and is sometimes associated with diaphoresis, nausea/vomiting, shortness of breath, lightheadedness, or anxiety. ECG shows ST elevations in a vascular distribution, and troponin I and CK-MB levels are elevated. This patient's ECG does not show ST elevations, and his symptoms are more consistent with stable angina. Answer E: Unstable angina is characterized by substernal chest pain occurring at rest or occurring with more severity, longer duration, or increasing frequency. ECG may be normal or show ST depression or T wave inversion, and the troponin level is not elevated. It is caused by incomplete coronary artery occlusion by a thrombus or atherosclerosis, commonly due to a ruptured plaque with subsequent clot formation. This patient's chest pain is exertional, does not occur at rest, and is consistently relieved with rest, and thus would be consistent with stable angina. Bullet Summary: Stable angina is characterized by exertional chest pain that is not changing in quality or quantity and is relieved with rest.
Unstable angina
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A 15-year-old girl is brought to the clinic because her mother is worried the patient has not yet had her period. The patient’s older sister had her first period at age 14. The mother had her first period at age 13. The patient reports she is doing well in school and is on the varsity basketball team. Her medical history is significant for asthma and atopic dermatitis. Her medications include albuterol and topical triamcinolone. Her temperature is 98°F (36.7°C), blood pressure is 111/72 mmHg, pulse is 65/min, and respirations are 14/min with an oxygen saturation of 99% on room air. Her body mass index (BMI) is 19 kg/m^2. Physical exam shows absent breast development and external genitalia at Tanner stage 1. Serum follicle-stimulating hormone (FSH) level is measured to be 38 mIU/mL. Which of the following is the most appropriate next diagnostic step?
CYP17 gene work-up
Gonadotrophin-releasing hormone stimulation test
Karotype
Luteinizing hormone levels
C
Karotype
The patient is presenting with primary amenorrhea and an increased follicle-stimulating hormone (FSH) level, suggesting a gonadal abnormality and making karyotyping the most appropriate next step in management. Primary amenorrhea can be diagnosed if a patient has normal secondary sex characteristics but no menarche by age 16, or if she has no secondary sex characteristics and no menarche as early as age 14. Primary amenorrhea is caused by either hypothalamic/pituitary (central) or gonadal (peripheral) abnormalities. Increased FSH indicates a peripheral cause or hypergonadotropic hypogonadism. Karyotyping would be the most appropriate next diagnostic step because these disorders can include Turner syndrome and androgen insensitivity syndrome. Gravholt et al. review the evidence regarding the diagnosis and treatment of Turner syndrome. They discuss how this disease presents with hypergonadotropic hypogonadism. They recommend growth hormone therapy and hormone replacement therapy in these patients. Incorrect Answers: Answer A: CYP17 gene work-up should be done in patients with primary amenorrhea, an absence of secondary sex characteristics, elevated FSH, and hypertension. Alterations in the CYP17 gene can cause congenital adrenal hyperplasia due to 17-hydroxylase deficiency. The resulting excess of deoxycorticosterone is associated with hypertension and hypokalemia. Treatment is with hormone repletion therapy. Answer B: Estrogen levels would be low in this patient as evidenced by her absence of breast development. Therefore, measuring estrogen levels would provide no additional diagnostic information. Estrogen levels may be useful in cases of peripheral gonadotropin resistance but these are rare. Answer C: Gonadotropin-releasing hormone (GnRH) stimulation test would be the next diagnostic step if FSH were decreased. A decrease in FSH indicates a central cause or hypogonadotropic hypogonadism. A GnRH stimulation test is used to assess whether the central abnormality is due to the hypothalamus or the pituitary. Answer E: Luteinizing hormone (LH) levels with a repeat FSH level may be helpful if FSH is low or normal. Low or normal FSH levels indicate hypogonadotropic hypogonadism as caused by hypothalamic-pituitary axis abnormalities. Cases of high FSH represent a different diagnostic entity as adequate gonadotropins are produced. Bullet Summary: In a patient with primary amenorrhea and an increased FSH, the next step in management is karyotyping.
nan
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A 51-year-old man presents for his annual wellness visit with his primary care physician. His wife has noticed a tremor while he is working on his car and knows that his father died of Parkinson disease. He says that the tremor is nothing to worry about and only occurs during a few activities such as pouring drinks, pointing the remote at the television, or fixing his car. The patient states his wife is just overreacting. His medical problems include hypertension, diabetes, and hyperlipidemia for which he takes lisinopril, metformin, and atorvastatin. He has 3-5 drinks of scotch per night before working on his car. On physical exam, the tremor is replicated during finger to nose testing. Romberg sign is negative. Cranial nerves II-XII are intact. The rest of the exam is benign. Which of the following is the most appropriate treatment for this patient's tremor?
Alcoholics anonymous
Aripiprazole
Phenobarbital
Propranolol
D
Propranolol
This patient who presents with a tremor that only occurs during terminal activity and is likely improved by alcohol (hence why he drinks prior to working on the car) has benign essential tremor. This is best treated with propranolol. Benign essential tremor is a type of rhythmic oscillatory movement that occurs with posturing and action. The pathophysiology of this disorder is unclear; however, it classically gets worse with actions that require terminal positioning of the extremities such as pouring a drink or pointing a television remote. The tremor improves with alcohol consumption so patients will often self-medicate with alcoholic drinks. There is a strong genetic component, with an autosomal dominant pattern of inheritance with incomplete penetrance. The best method of replicating the tremor is with the finger-nose-finger test because this requires the patient to perform a precise activity at a terminal distance. The diagnosis is made clinically. Treatment is with propranolol or primidone, which are both considered first-line agents although the latter has less evidence for its efficacy. Paparella et al. studied the effects of propranolol on head and upper extremity movements in patients with benign essential tremors. They found that this medication has no effect on head movements but reduces the number of upper extremity tremors in these patients. They recommended that agents other than propranolol should be used for the treatment of head tremors in these patients. Incorrect Answers: Answer A: Alcoholics anonymous is the first-line treatment for patients with alcohol use disorder; however, the tremors that occur during alcohol withdrawal are not isolated to movements during terminal activities. Alcohol withdrawal also presents with other signs including restlessness, diaphoresis, and insomnia. More severe cases can present with hallucinations and seizures. The response of this patient's tremor to alcohol consumption is typical of patients with benign essential tremor. Answer B: Aripiprazole and other atypical antipsychotic medications can be used in patients with Tourette syndrome; however, these patients will present with involuntary tics rather than a tremor that only occurs during a voluntary activity at the terminal range of motion. Moreover, for the diagnosis of Tourette syndrome, both motor and phonic tics must be present. Answer C: Carbidopa can be used in the treatment of Parkinson disease; however, the tremor associated with this disease classically occurs at rest and is described in character as a "pill-rolling" tremor. The other features of Parkinson disease include bradykinesia or akinesia, rigidity, and postural instability. Answer D: Phenobarbital can be used in the treatment of Huntington disease; however, the involuntary movements seen in this disease are classically choreiform rather than intention tremor. Huntington disease presents with chorea, psychiatric illness, and depression. There is commonly a strong genetic predisposition, as it is a trinucleotide repeat disorder that exhibits anticipation. Bullet Summary: Propranolol can be used in the treatment of patients with benign essential tremor, which presents with an action tremor that is alleviated by alcohol use.
nan
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A 54-year-old man is brought to the emergency department following a skiing accident. The patient lost control of his skis and collided with a tree. He lost consciousness for about 30 seconds. When he woke up, he was mildly confused but able to ski down the rest of the mountain. The patient initially denied medical treatment but his wife convinced him to be taken by ambulance to the emergency department for evaluation. The paramedics report that at the scene the patient had a Glasgow Coma Score (GCS) of 15. During the ride, he became gradually more somnolent. His medical history is significant for hypertension, hyperlipidemia, coronary artery disease, and a prior transient ischemic attack. His medications include atorvastatin and lisinopril. In the emergency department, his temperature is 97.8°F (36.6°C), blood pressure is 141/84 mmHg, pulse is 71/min, and respirations are 16/min. He is difficult to arouse with a GCS of 7. A head CT is performed and can be seen in Figure A. Which of the following is the most likely cause of this patient’s presentation?
Damage to the cerebral bridging veins
Damage to the sphenoid bone
Occlusion of cerebral small penetrating arteries
Ruptured cerebral aneurysm
B
Damage to the sphenoid bone
This patient presents with a lucid interval followed by worsening somnolence and a biconvex lesion on head CT, which suggests a diagnosis of acute epidural hematoma. Acute epidural hematomas are most commonly caused by damage to the middle meningeal artery from trauma to the sphenoid bone. Patients with an epidural hematoma typically have an initial loss of consciousness followed by a lucid interval, during which their mental status returns to baseline or near baseline. As the hematoma expands, patients display increasing somnolence from the increase in intracranial pressure. For that reason, epidural hematomas are sometimes referred to as the “talk and die” syndrome. If not relieved, this increase in intracranial pressure can lead to uncal herniation or death. Patients with neurologic findings or those with expanding bleeds require urgent surgical decompression. Dadashi et al. present cases of patients with epidural hematoma from acute trauma. They discuss how patients often sustain these injuries from extreme sports. They recommend being aware of this syndrome so that acute interventions can be taken. Figure/Illustration A demonstrates a biconvex hyperdensity that is limited by suture lines (red circle). This is the typical appearance of an epidural hematoma on head CT. Incorrect Answers: Answer A: Damage to the cerebral bridging veins results in a subdural hematoma (SDH). An acute SDH more commonly presents with a gradual increase in headache and confusion rather than the “talk and die” syndrome presented in this vignette. However, a minority of patients with acute SDH may have a lucid interval followed by progressive neurological decline. SDH would have a crescent-shaped appearance on head CT rather than the biconvex appearance of an epidural hematoma. Answer C: Occlusion of cerebral small penetrating arteries describes a lacunar stroke. Although this patient has 2 risk factors for lacunar stroke (hypertension and smoking), this patient’s head CT demonstrates an epidural hematoma. Answer D: A ruptured cerebral aneurysm would most likely lead to a subarachnoid hemorrhage. A subarachnoid hemorrhage presents as a sudden, severe headache (“thunderclap headache”), confusion or somnolence, nausea and vomiting, and meningismus. Surgical clipping or embolization of the bleeding vessel may be required. Answer E: Secondary hemorrhage into a prior area of infarction usually occurs in the setting of a recent embolic stroke. Although this patient has a history of TIA and other risk factors for stroke, his head CT demonstrates an epidural hematoma. Treatment is supportive with the control of underlying risk factors such as hypertension. Bullet Summary: Epidural hematomas are caused by damage to the middle meningeal artery as it passes through the foramen spinosum of the sphenoid bone.
Secondary hemorrhage into prior area of infarction
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A 25-year-old woman is brought to the emergency department by her family. They found her somnolent next to her computer with an empty pill bottle around 3 hours ago. The family cannot recall the types of pill bottles that they found and did not bring them to the ED. The patient's medical history is notable for anxiety, obesity, hypertension, and polycystic ovarian syndrome. She is currently not on any medications. She was given an exercise program but she has not been compliant with these exercises. Her temperature is 99.5°F (37.5°C), pulse is 82/min, blood pressure is 125/85 mmHg, respirations are 11/min, and oxygen saturation is 97% on room air. Physical exam is within normal limits. The patient begins communicating with the physician and states that she did take acetaminophen but it was only a few pills. Her parents are certain the bottle was new. Which of the following is the most appropriate next step in management?
Blood acetaminophen levels
Charcoal
Lactated ringer bolus
N-acetylcysteine
D
N-acetylcysteine
This patient is presenting with acute acetaminophen intoxication. The most appropriate next step in management is to administer the antidote N-acetyl cysteine (NAC). In a toxicology case where the ingestion is unknown, the most appropriate initial step in management is to begin treatment empirically with IV fluids, charcoal, NAC, naloxone, and glucose if needed. These agents are effective against the most common causes of intoxication. In cases where there is known acetaminophen ingestion, the most important treatment is prompt administration of NAC. This medication works by increasing the hepatic production of glutathione, which detoxifies the toxic metabolite of acetaminophen N-acetyl-p-benzoquinone imine (NAPQI). NAC is a safe, cheap, and effective treatment that could be life-saving if administered in a timely fashion. In massive overdoses, it is appropriate to start treatment empirically as it can be discontinued later. Otherwise, a 4 hour acetaminophen level should be collected and the level should be plotted on the Rumack Matthew nomogram to determine treatment. In chronic acetaminophen toxicity, the acute serum level play no role in guiding treatment. Chiew et al. performed a meta-analysis regarding the treatment of acetaminophen overdose. They found that the administration of charcoal immediately after ingestion can decrease the rate of absorption of acetaminophen. They recommend early administration of N-acetyl cysteine in order to decrease the hepatotoxicity of this substance. Incorrect Answers: Answer A: Blood acetaminophen levels should be ordered, but it is much less dire to confirm the diagnosis than it is to begin treatment given the high fatality rate with untreated acetaminophen overdose. Testing for all common intoxication should proceed while treatment is being administered. In this case, the concern for consumption of an entire bottle of acetaminophen would make it reasonable to treat first. Answer B: Charcoal is an appropriate intervention if it is given in a timely manner; however, this patient consumed acetaminophen over 2 hours ago. In general, if the patient's airway is secure, it does no harm and could offer some benefit by decreasing the absorption of the substance of interest. Answer C: IV fluid administration is the most appropriate initial step in the management of trauma when there is suspected bleeding and vitals are unstable (hypotension and tachycardia). Fluids are not urgently needed in this toxicity case with a patient who has stable vitals. Answer E: Syrup of ipecac induces emesis in patients and was previously used in an attempt to have the patient vomit the toxic substance. This is no longer a preferred method of managing toxicities as patients can aspirate the vomitus or the toxic substance can induce more tissue damage during vomiting. In general, syrup of ipecac is the wrong answer choice for all toxicity cases. Bullet Summary: Acetaminophen toxicity should be treated immediately with N-acetyl cysteine followed by further diagnostic workup including serum acetaminophen levels.
Syrup of ipecac
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A 46-year-old man presents with his wife to his primary care provider for depression and strange movements. His wife reports that her husband has not been himself for the last 2 months. Whereas he was previously outgoing and “the life of the party,” the patient is now irritable and withdrawn. He is a partner at an accounting firm, but his colleagues are threatening to fire him if he continues to perform poorly at work. The patient cannot explain the recent changes to his mood and tearfully admits he fears there is something seriously wrong with him. His wife says that he is getting worse. The patient’s medical history is significant for hypertension, for which he takes lisinopril. His family history is unknown as he was adopted. The patient met his mother once and never knew his father but was told he died in his 50s. He drinks a few glasses of wine per week and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 141/87 mmHg, pulse is 81/min, and respirations are 12/min. On physical exam, the patient has a flat affect with a facial grimace and sudden jerky movements of his upper extremities. Which of the following is most likely to be seen on further workup?
Alpha-synuclein aggregates on brain biopsy
Dorsal striatum atrophy on head CT
Frontotemporal atrophy on head CT
Neurofibrillary tangles on brain biopsy
B
Dorsal striatum atrophy on head CT
This patient presents with recent mood changes, jerky movements of the upper extremities, and a history of early death in his father, which suggests a diagnosis of Huntington disease. Huntington disease is characterized by atrophy of the caudate nucleus, which is a structure of the dorsal striatum. Huntington disease (HD) is an autosomal dominant trinucleotide (CAG) repeat disorder located on chromosome 4. HD typically presents with mood changes and abnormal movements (particularly chorea or myoclonus) over weeks to months. Although executive function can be affected early on, memory is usually preserved until relatively late in the disease. Frontotemporal dementia, in contrast, usually presents with behavioral changes, and Alzheimer disease has memory impairment as an early sign. Caudate atrophy is typically seen on head imaging in Huntington disease. This disease cannot be cured but supportive treatment includes benzodiazepines and tetrabenazine for symptomatic relief of muscle spasms. Walker presents a review of the evidence regarding the etiology of Huntington disease. He discusses that typically the onset of symptoms is in middle age after affected individuals have had children. He recommends a better understanding of the function of the mutant protein in this disease. Incorrect Answers: Answer A: Alpha-synuclein aggregates would be found on brain biopsy in Parkinson disease and Lewy body dementia. Lewy body dementia is characterized by parkinsonism and visual hallucinations. Patients would present with cogwheel rigidity, bradykinesia, and a resting tremor. Treatment is with dopamine agonists such as carbidopa/levodopa. Answer C: Frontotemporal atrophy is consistent with a diagnosis of frontotemporal dementia. Frontotemporal dementia presents primarily with early behavioral changes, and it would not present with myoclonus. Treatment is supportive as there is no cure for this disease. Patients and their spouses may require psychiatric support for changes in behavior. Answer D: Neurofibrillary tangles on brain biopsy would suggest a diagnosis of Alzheimer disease. This disease presents with a slowly progressive cognitive decline in elderly patients. Patients have early loss of memory but have maintained motor function and behavior until later in the course of the disease. Answer E: A positive 14-3-3 CSF assay would confirm the diagnosis of Creutzfeldt-Jakob disease (CJD). Although CJD may also present with mood changes and myoclonus, CJD most prominently causes rapidly progressive dementia early in life. Treatment is supportive and death occurs quickly. Bullet Summary: Huntington disease is an autosomal dominant trinucleotide repeat disorder located on chromosome 4 that causes caudate atrophy and presents with mood changes, abnormal movements, and subcortical dementia.
Positive 14-3-3 CSF assay
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A 43-year-old man presents to the emergency department with bright red blood in his stool this morning. He noticed this yesterday as well, but it was much scanter. The patient is otherwise healthy. He was celebrating his birthday last night and admits having "too much to drink" and vomited profusely overnight. He currently feels well and is not nauseous. He has no other significant medical history and does not take any medications. His temperature is 99.2°F (37.3°C), blood pressure is 110/75 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 100% on room air. Physical exam reveals a nontender abdomen with normal bowel sounds. Rectal exam reveals grossly bloody stool. Laboratory studies are ordered as shown below. Hemoglobin: 12 g/dL Hematocrit: 36% Leukocyte count: 5,500/mm^3 with normal differential Platelet count: 179,000/mm^3 Which of the following is the most likely cause of this patient's symptoms?
Erosion into a gastric vessel
Submucosal esophageal tear
Vascular malformation
Malignancy
C
Vascular malformation
This patient is presenting with bright red blood per rectum with a non-tender abdomen. Of the answer choices given, angiodysplasia is the most likely to cause such profuse and sudden bleeding. Angiodysplasia is a vascular malformation found in the gastrointestinal (GI) tract, most commonly in the colon. The exposed vessels can bleed, leading to bright red blood per rectum. The diagnosis can be confirmed with colonoscopy. Ablation can be performed to stop the bleeding. Any patient who is unstable or anemic with a hemoglobin < 7.0 g/dL requires a transfusion with blood products. Angiodysplasia is a common cause of bright red blood per rectum but is not more common than diverticulosis. Garcia-Compeon et al. review the presentation and treatment of gastrointestinal angiodysplasia. The authors note that angiodysplasias are the cause of many "occult" GI bleeds not visualized on upper endoscopy or colonoscopy. The authors recommend the use of capsule endoscopy in occult GI bleeds to identify angiodysplasias as a potential source. Incorrect Answers: Answer A: Erosion into a gastric vessel is the pathophysiology of a bleeding gastric vessel secondary to peptic ulcer disease. This usually presents with melena (black tarry stools) rather than bright red blood per rectum. Only in cases of extremely brisk upper GI bleeding would patients present with bright red blood per rectum. Treatment involves treating Helicobacter pylori infection if present, proton pump inhibitors, and sucralfate. Endoscopy is needed to confirm and treat the bleeding vessel. Answer B: Inflammation and infection of colonic outpouching is the pathophysiology of diverticulitis which presents with a history of constipation with a fever and left lower quadrant abdominal pain. Management involves a computed tomography (CT) scan of the abdomen/pelvis and administration of antibiotics (such as ciprofloxacin and metronidazole or ceftriaxone and metronidazole). Diverticulosis presents with bright red blood and is possible in this patient, but it does not involve inflammation or infection, which defines diverticulitis. Answer C: Malignancy or colon cancer can present with bright red blood per rectum, malaise, weight loss, and microcytic anemia. While it is possible in this patient, his age, lack of risk factors for colon cancer, and the sudden onset of his symptoms make this diagnosis less likely. Answer D: Submucosal esophageal (Mallory-Weiss) tear presents after profuse vomiting (as in this patient) with bloody vomitus. Treatment is supportive and involves antiemetics. Bullet Summary: Angiodysplasia is a vascular malformation that presents with bright red blood per rectum.
nan
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A 28-year-old G1P0 woman presents to initiate prenatal care after a positive home pregnancy test. The first day of her last menstrual period was 7 weeks prior to presentation. The patient has no medical conditions and takes no medications. Her temperature is 98.6°F (37.0°C), blood pressure is 110/70 mmHg, pulse is 64/min, and respirations are 17/min. Physical exam is notable for a nontender abdomen. Pelvic exam reveals a closed cervix with no adnexal or uterine tenderness. A urine pregnancy test is positive, and an intrauterine singleton pregnancy measuring approximately 9 weeks gestational age is visualized on transvaginal ultrasound. The patient asks if she should modify her current exercise regimen due to her pregnancy. She typically swims or uses an exercise bike for 45 minutes, 5 days a week, and plays in an office soccer league for 1 hour on 1 day per week. Which of the following is the most appropriate recommendation regarding exercise for this patient?
Avoid soccer during pregnancy, continue other activities
Avoid exercise during pregnancy
Continue the current exercise regimen
Reduce to walking only, beginning in the third trimester
A
Avoid soccer during pregnancy, continue other activities
This healthy primigravida woman presents to the clinic inquiring about her current exercise regimen involving 285 minutes of moderate to exercise weekly. With the exception of soccer, a contact sport, her exercise regimen is appropriate throughout her pregnancy. The American College of Obstetricians and Gynecologists (ACOG), recommends that healthy pregnant women engage in moderate-intensity exercise for at least 30 minutes or more for 5-7 days weekly provided there are no contraindications to doing so (certain cardiac or pulmonary conditions, history of cerclage placement, multiple pregnancies, preeclampsia, or severe anemia). Highly active patients may continue their more vigorous pre-pregnancy regimens after consulting with a physician. Exercise in pregnancy can ease gastrointestinal symptoms and may decrease the risk of gestational diabetes, preeclampsia, and cesarean delivery. However, activities that may cause fetal or maternal injury, such as contact sports or activities with a significant fall risk should be avoided. Examples of contact sports include football, basketball, hockey, lacrosse, and wrestling. Nascimento et al. review exercise in pregnancy. They note that exercise is beneficial in pregnancy and in the postpartum period and recommend engaging in appropriate exercise in this patient population. Incorrect Answers: Answer A: Avoiding exercise during pregnancy is not recommended, as at least 150 minutes of moderate exercise weekly is suggested during pregnancy for the health of the mother and the fetus. Answer C: Continuing the current exercise regimen is not recommended, as soccer is a high-contact sport that could cause injury to the fetus or placenta. Answer D: Limiting moderate exercise to 30 minutes per day maximum during pregnancy is not recommended, as 30 minutes daily is the minimum recommended time for moderate-exercise. There is no reason for healthy pregnant patients without high-risk pregnancies to significantly limit their exercise. Answer E: Reducing to walking-only beginning in the third trimester is not necessary, as moderate activities are healthy throughout pregnancy. Bullet Summary: Healthy pregnant patients should perform at least 30 minutes of exercise 5-7 days per week and avoid high-contact sports or sports with fall risk.
nan
https://step2.medbullets.com/testview?qid=216610
A 44-year-old nurse presents to the emergency department with confusion. This has happened several times this past month with increasing frequency. Each time her symptoms improved with eating. She is otherwise healthy. She lives with her mother who has diabetes whose medications include metformin, insulin, lisinopril, amlodipine, and glyburide. Her temperature is 98.0°F (36.7°C), blood pressure is 132/81 mmHg, pulse is 85/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam reveals a confused woman. She is moving all her extremities but follows commands poorly. Laboratory studies are ordered as seen below. Hemoglobin: 14 g/dL Hematocrit: 41% Leukocyte count: 7,500/mm^3 with normal differential Platelet count: 199,000/mm^3 Serum: Na+: 140 mEq/L Cl-: 102 mEq/L K+: 4.0 mEq/L HCO3-: 23 mEq/L BUN: 30 mg/dL Glucose: 29 mg/dL Creatinine: 1.4 mg/dL Ca2+: 10.2 mg/dL Sulfonylurea level: undetectable C-peptide level: 55 ng/mL (normal < 5 ng/mL) Which of the following is the most likely etiology of this patient’s symptoms?
Alpha cell tumor
Beta cell destruction
Beta cell tumor
Insulin overdose
C
Beta cell tumor
This patient is presenting with confusion, profound hypoglycemia, an elevated C-peptide level, and a negative sulfonylurea level, which are concerning for an insulinoma. An insulinoma is a beta cell tumor that secretes insulin. An insulinoma is a beta cell tumor of the pancreas. The beta cells normally secrete insulin in response to increased circulating fuel substrate (such as glucose or fat). In the setting of an insulinoma, insulin is pathologically and continuously secreted. This leads to profound, recurrent episodes of hypoglycemia. When a patient presents with hypoglycemia of unclear etiology; a blood glucose level, C-peptide level, and sulfonylurea level should be ordered. An infectious workup may be indicated as sepsis can also cause hypoglycemia. In an insulinoma, patients will be hypoglycemic, have an elevated C-peptide level (as this is a marker of endogenous insulin release), and a negative sulfonylurea level (important to rule out as a sulfonylurea overdose can also cause hypoglycemia in the setting of an elevated C-peptide level). Further workup will involve imaging of the abdomen such as a CT scan or MRI (more accurate) to delineate this insulin-secreting mass. Mathur et al. discuss insulinoma. They note that it is a rare endocrine tumor, and note its association with multiple endocrine neoplasia type 1. They recommend workup for an insulinoma should occur in patients with recurrent episodes of hypoglycemia without a clear cause. Incorrect Answers: Answer A: Alpha cell tumor describes a glucagonoma, which pathologically secretes glucagon leading to profound hyperglycemia, a classic rash termed necrolytic migratory erythema (presents with painful, erythematous papules and plaques), and gastrointestinal symptoms including anorexia, abdominal pain, and diarrhea. Answer B: Beta cell destruction describes the pathophysiology of type I diabetes mellitus, which presents in pediatric patients (most commonly) with hyperglycemia, weight loss, polydipsia, and polyuria. Treatment involves the administration of insulin. An initial presentation of type I diabetes may be diabetic ketoacidosis which presents with dehydration, abdominal pain, nausea, vomiting, Kussmaul respirations, and an anion gap acidosis. Answer D: Insulin overdose would present with hypoglycemia, somnolence, confusion, and tremulousness. Note that exogenous insulin administration would not elevate the C-peptide level, which is a marker of endogenous insulin production. Answer E: Sulfonylurea overdose would cause hypoglycemia that is often persistent given the long half-life of sulfonylureas. It would also elevate the C-peptide level as these medications increase the endogenous release of insulin. However, the sulfonylurea level was negative in this patient. While iatrogenic use of medications is plausible in this patient who has knowledge of medications (as she is a nurse), her repeat episodes and her workup suggesting against an iatrogenic etiology makes an insulinoma a more likely diagnosis. Bullet Summary: Insulinomas are beta cell tumors of the pancreas and lead to severe hypoglycemia and an elevated insulin and C-peptide level.
Sulfonylurea overdose
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A 2-week-old boy is evaluated by his pediatrician for abnormal feet. The patient was born at 39 weeks via vaginal delivery to a G1P1 29-year-old woman. The patient has been breastfeeding and producing 5 stools/day. He is otherwise healthy. His temperature is 99.5°F (37.5°C), blood pressure is 60/38 mmHg, pulse is 150/min, respirations are 24/min, and oxygen saturation is 98% on room air. A cardiopulmonary exam is notable for a benign flow murmur. A musculoskeletal exam reveals the findings shown in Figure A. Which of the following is the most appropriate next step in management?
Botulinum toxin injections
Reassurance and reassessment in 1 month
Serial casting
Surgical pinning
C
Serial casting
This patient is presenting with talipes equinovarus (congenital clubfoot) for which the most appropriate initial step in management is serial casting using the Ponseti method. Talipes equinovarus is described clinically as a foot that is cavus, adductus, varus, and equinus. Cavus occurs when the forefoot is pronated relative to the hindfoot. Adductus occurs when the forefoot is medially deviated compared to the hindfoot. Varus occurs when the heel is medially deviated compared to the ankle. Finally, equinus occurs when the ankle rests in a plantarflexed position. The most appropriate initial step in management for this condition is serial casting using the Ponseti method. Cases that are refractory to this intervention are treated with surgical interventions such as split tibialis anterior transfer. Cady et al. review the evidence regarding the treatment of clubfeet. They discuss how the Ponseti method is now the standard of care in the treatment of this disorder. They recommend early diagnosis and treatment of these patients. Figure/Illustration A is a clinical photograph that demonstrates cavus, adductus, equinus, and varus positioning of the foot (red circles). This appearance is classically seen in congenital talipes equinovarus. Incorrect Answers: Answer A: Botulinum toxin injections are often used to treat spasticity and tension headaches; however, they are not indicated for the treatment of talipes equinovarus. Answer B: Reassurance and reassessment in 1 month would be inappropriate management and could lead to permanent sequelae. Patients require immediate treatment with serial casting in order to correct the deformity. Answers 4 & 5: Surgical interventions are reserved for cases of clubfoot that are refractory to stretching and serial casting. A percutaneous Achilles tenotomy is part of the Ponseti method but tendon transfers and releases are reserved for recurrent cases. Bullet Summary: The most appropriate initial step in management for congenital clubfoot (talipes equinovarus) is serial casting using the Ponseti method.
Surgical soft tissue release
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A 32-year-old man presents to the emergency department for evaluation of headache. His symptoms started last night and have persisted. He occasionally has migraine headaches, but he has not had a headache like this in the past. He reports associated nausea, vomiting, fevers, and neck pain. He has a medical history of asthma. His temperature is 100.4°F (38.0°C), blood pressure is 110/60 mmHg, pulse is 95/min, respirations are 17/min, and oxygen saturation is 98% on room air. On physical exam, he appears uncomfortable. Neurologic exam reveals no focal deficits. There is pain with passive neck flexion. No edema or rashes are noted. Lungs are clear to auscultation bilaterally. Lumbar puncture is performed and CSF results are obtained as shown below. Cerebrospinal fluid (CSF): Cell count: 175/mm^3 RBCs: 0/mm^3 Cl-: 119 mEq/L Glucose: 49 mg/dL Pressure: 150 mmH2O Total protein: 55 mg/dL Based on these results, which of the following is the most appropriate treatment regimen for this patient?
Acyclovir
Amphotericin and 5-flucytosine
Ceftriaxone and vancomycin
Ceftriaxone, vancomycin and steroids
E
Supportive care and monitoring
This patient with headache, fever, and neck stiffness has a presentation and CSF studies consistent with a diagnosis of viral or aseptic meningitis. Supportive care is the most appropriate management strategy for patients with viral meningitis. Aseptic (viral) meningitis presents similarly to bacterial meningitis with neck stiffness, photophobia, fever, malaise, and flu-like symptoms. Patients should be worked up and treated as if they have bacterial meningitis until proven otherwise. CSF findings in aseptic meningitis show an elevated cell count (though typically < 300/mm^3 in contrast to bacterial meningitis), a normal or elevated protein, and a normal glucose. CSF should be sent for Gram stain and culture to confirm the diagnosis and rule out bacterial meningitis. Once the diagnosis has been confirmed, management of viral meningitis consists of supportive care and monitoring for complications such as seizures, cerebral edema, or SIADH. Kohil et. al review viral meningitis. They discuss the pathophysiology, clinical manifestation and diagnosis. They recommend that most patients with viral meningitis be treated with supportive care. Incorrect Answers: Answer A: Acyclovir would be appropriate for suspected HSV or varicella encephalitis or meningitis. This patient with no RBCs in the CSF and no changes in mental status is not likely to have infection with HSV or varicella. Answer B: Amphotericin and 5-flucytosine followed by long-term fluconazole is the appropriate management of cryptococcal meningitis. Cryptococcal meningitis presents similarly to bacterial and viral meningitis, almost exclusively in immunocompromised patients. Answer C: Ceftriaxone and vancomycin would be appropriate if bacterial meningitis was suspected. Steroids are generally added if a bacterial source is likely. However, this patient has a presentation that is less concerning for bacterial meningitis, and CSF studies that are more consistent with aseptic meningitis. Answer D: Ceftriaxone, vancomycin and steroids are an appropriate initial regimen for bacterial meningitis. Dexamethasone reduces inflammatory injury while patients are being treated with antibiotics. Bullet Summary: Viral or aseptic meningitis is managed with supportive care and monitoring.
Supportive care and monitoring
https://step2.medbullets.com/testview?qid=216611
A 72-year-old man presents to his primary care physician for a general checkup. He states that he has been a bit more fatigued lately but believes it is secondary to poor sleep at his new house. The patient is otherwise healthy and takes no medications. His temperature is 98.0°F (36.7°C), blood pressure is 141/90 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam is notable only for minor pallor but is otherwise unremarkable. Basic laboratory studies are ordered as seen below. Hemoglobin: 10 g/dL Hematocrit: 30% Leukocyte count: 67,500/mm^3 Platelet count: 119,000/mm^3 Serum: Na: 141 mEq/L Cl: 103 mEq/L K: 4.0 Eq/L HCO3-: 24 mEq/L BUN: 22 mg/dL Glucose: 109 mg/dL Creatinine: 1.2 mg/dL Ca: 10.0 mg/dL Which of the following is the most likely diagnosis?
Chronic lymphocytic leukemia
Acute lymphoblastic leukemia
Hairy cell leukemia
Chronic myelogenous leukemia
A
Chronic lymphocytic leukemia
This elderly patient is presenting with minor fatigue, anemia, thrombocytopenia, and a leukocyte count >50,000/mm^3, which is most likely caused by chronic lymphocytic leukemia (CLL). CLL is a monoclonal proliferation of incompetent mature B-cells. It is common in elderly men and is the most common form of leukemia in the United States. Patients are often asymptomatic when the diagnosis is made or may present with vague constitutional symptoms including fatigue, malaise, and weight loss. It is important to note that symptoms may be subtle or absent. Other findings include non-tender lymphadenopathy and splenomegaly. The diagnosis is supported when the white blood cell count is >50,000/mm^3 and can be supported with a peripheral smear which will show smudge cells. The cells in CLL may be CD5, CD20, and CD23 positive. The most common cause of death in this population is infection given the lack of competent B-cells. Hallek reviews CLL. They discuss updated guidelines on the diagnosis and management of CLL. They recommend the use of targeted agents in initial therapy and acknowledge the need for further clinical trials to guide therapy. Incorrect Answers: Answer A: Acute lymphoblastic leukemia (ALL) is a common malignancy seen in children and presents with pancytopenia, anemia, fatigue, leukopenia (leading to recurrent infections), and thrombocytopenia (leading to bleeding). Other findings may include fever, bone pain, lymphadenopathy, splenomegaly, and hepatomegaly. Translocations in ALL include: t(12:21) which is the most common and denotes a favorable prognosis and t(9:22) (the Philadelphia chromosome) which is less common and denotes a poor prognosis Answer B: Acute myelogenous leukemia occurs secondary to the proliferation of myeloblasts and presents in older patients. Patients will present with fatigue, malaise, weight loss, anorexia, fever, leukocytosis, anemia, thrombocytopenia, and neutropenia. Auer rods are frequently present on a blood smear. Answer D: Chronic myelogenous leukemia is a neoplastic proliferation of myeloid stem cells and presents with a leukocyte count often >50,000/mm3. The increased myeloid lines include red blood cells, granulocytes, monocytes, and platelets. Many patients present in the chronic phase with minimal symptoms; however, the blast phase can lead to a hematologic emergency termed a blast crisis which may cause a hyperviscosity syndrome. This condition is more symptomatic and less common than CLL. Answer E: Hairy cell leukemia occurs when there is a neoplastic proliferation of mature B cells with abundant cytoplasm with “hairy” cytoplasmic processes. The cells are tartrate-resistant acid phosphatase positive. Patients will present with splenomegaly, pancytopenia, weakness, and fatigue. This is a more rare diagnosis when compared to CLL. Bullet Summary: Chronic lymphocytic leukemia (CLL) presents in elderly patients with malaise, fatigue, and other nonspecific signs (but can also be asymptomatic), with anemia, thrombocytopenia, and a white blood cell count often >50,000/mm^3.
nan
https://step2.medbullets.com/testview?qid=108997
A 16-year-old girl who recently immigrated to the United States from Bolivia presents to her primary care physician with a chief complaint of inattentiveness in school. The patient's teacher describes her as occasionally "daydreaming" for periods of time during where the patient does not respond or participate in school activities. Nothing has helped the patient change her behavior, including parent-teacher conferences or punishment. The patient has no other concerns. The only other concern that the patient's mother has is that, upon awakening, she notices that sometimes the patient's arm will jerk back and forth. The patient states she is not doing this intentionally. The patient has an unknown medical history and is currently not on any medications. She is not sexually active and does not intend to become pregnant in the near future. Her temperature is 98.6°F (37°C), blood pressure is 112/74 mmHg, pulse is 63/min, and respirations are 11/min. On physical exam, you note a young, healthy girl whose neurological exam is within normal limits. Which of the following is the most appropriate initial treatment?
Carbamazepine
Cognitive behavioral therapy
Ethosuximide
Lamotrigine
E
Valproic acid
This patient is presenting with both absence seizures (periods of inattentiveness in school) and juvenile myoclonic epilepsy (abnormal, involuntary spasms of her upper extremity). The most appropriate treatment for these conditions is valproic acid. Absence seizures classically present with cessation of motor activity and speech with a blank facial expression and occasionally automatisms such as flickering of the eyelids. They can be mistaken for inattentiveness in school-aged children. Juvenile myoclonic epilepsy presents with a "jerky" movement of the patient's extremity that typically occurs in the morning and begins around adolescence. The only medication that can be used to treat both absence seizures and juvenile myoclonic epilepsy is valproic acid. Valproic acid inhibits GABA transaminase, blocks thalamic T-type calcium channels, and blocks voltage-gated sodium channels. In post-pubertal female patients, it is important to counsel them on the potential teratogenic effects of this medication and also to consider alternative treatments in patients who are considering becoming pregnant or who cannot guarantee reliable birth control practices. Kessler and McGinnis discuss the treatment of childhood absence seizures. They discuss how the treatment of choice is ethosuximide due to its favorable side effect profile. They recommend using valproic acid if there are concurrent seizure conditions. Incorrect Answers: Answer A: Carbamazepine would be the appropriate therapy for a partial seizure (tonic or clonic movements in addition to an absence of a post-ictal period) or for trigeminal neuralgia (sudden pain in the distribution of the trigeminal nerve). It would not address both absence seizures and juvenile myoclonic epilepsy. Answer B: Cognitive behavioral therapy is a useful treatment that helps a patient develop coping strategies and change unhelpful patterns of thought. It could be useful if this girl was having trouble focusing in school for another reason (such as obsessive-compulsive disorder); however, it is not useful in managing seizures. Answer C: Ethosuximide is the most appropriate initial therapy for absence seizures. In this patient, she is suffering from both absence seizures and juvenile myoclonic epilepsy which could not be treated by ethosuximide alone, in contrast to valproic acid which could treat both conditions. Answer D: Lamotrigine is an anti-epileptic that can also be used as a mood stabilizer. It carries the potentially severe side effect of Stevens-Johnson syndrome. It is not a first-line treatment for absence seizures and juvenile myoclonic epilepsy. Stevens-Johnson syndrome will present with painful desquamation. Bullet Summary: Absence seizures present with periods of inattentiveness and subtle automatisms and are most appropriately treated with ethosuximide or with valproic acid if there is concurrent juvenile myoclonic epilepsy.
Valproic acid
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A 60-year-old man presents to his primary care physician with weight gain. He states that ever since his wife died 4 months ago, he has been eating and sleeping more and no longer engages in any activities he once enjoyed such as hiking or fishing. He feels guilty for not spending more time with his wife before she died. He was recently fired for making several major bookkeeping mistakes at work as an accountant as he had trouble focusing. The patient is requesting oxycodone at this appointment as he states he has burning pain in his legs that feels worse now than it has in years past. The patient has a past medical history of obesity, poorly controlled diabetes, hypertension, and peripheral vascular disease. His temperature is 98.7°F (37.1°C), blood pressure is 129/82 mmHg, pulse is 83/min, respirations are 15/min, and oxygen saturation is 99% on room air. Physical exam reveals a stable gait and reduced sensation symmetrically over the lower extremities. He complains of electric pain when touching his lower extremities. His affect seems depressed. Which of the following is the most appropriate treatment for this patient?
Amitriptyline
Bupropion
Escitalopram
Phenelzine
E
Venlafaxine
This patient is presenting with sleep changes, interest loss, guilt, decrease in energy, and trouble concentrating for greater than 2 weeks concerning for major depressive disorder. In the setting of his diabetic neuropathy (lower extremity burning pain in the setting of poorly managed diabetes), a serotonin-norepinephrine reuptake inhibitor (SNRI) such as venlafaxine is the most appropriate initial treatment for this patient. Major depressive disorder should always initially be managed with cognitive behavioral therapy and often medication. Typically, the first-line medication is a selective serotonin reuptake inhibitor (SSRI). However, SNRIs such as venlafaxine and duloxetine are viable first-line options. They are particularly preferred in patients with diabetic neuropathy as they treat both diabetic neuropathy and depression. SNRIs are also generally considered to be less sedating than SSRIs. For this reason, SNRIs are often a better initial drug in depression than SSRIs in diabetic patients with peripheral neuropathy. Sansone and Sansone discuss SNRIs and their uses. They discuss how there are different efficacies and propensities of the different SNRIs. They recommend choosing an appropriate SNRI based on the patient's needs and comorbidities. Incorrect Answers: Answer A: Amitriptyline is a tricyclic antidepressant that can be used to treat depression in patients who also suffer from other neuropathic pain conditions or conditions such as fibromyalgia or irritable bowel syndrome. While it may also be used in diabetic neuropathy and depression, there is higher toxicity with this medication than SNRIs. For this reason, SNRIs are preferred. A TCA might be appropriate if the patient had already failed other first-line agents. Answer B: Bupropion is a weight-neutral antidepressant that can be used in smoking cessation and patients who are trying to avoid the sexual side effects of SSRIs (anorgasmia). It would not address this patient's diabetic neuropathy, though it could be beneficial for his obesity. Answer C: Escitalopram is an SSRI that could be used first-line for major depressive disorder. However, if a patient has a concomitant condition, it is better to choose a single agent that addresses both problems; thus, SNRIs are better first-line medications in this patient with diabetic neuropathy. Answer D: Phenelzine is a monoamine oxidase inhibitor that can be used in atypical depression or as a third-line or later agent in the management of depression. This agent can cause a hypertensive crisis in the setting of tyramine consumption, has many medication interactions, and a long washout period. Thus, it is not usually a first or second-line agent. However, it is highly effective in patients who have failed other first-line agents and should still be used in appropriate candidates. Bullet Summary: SNRIs are appropriate first-line agents for major depressive disorder in patients with diabetic neuropathy.
Venlafaxine
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A 46-year-old man presents with his wife to his primary care provider for depression and strange movements. His wife reports that her husband has not been himself for the last 2 months. Whereas he was previously outgoing and “the life of the party,” the patient is now irritable and withdrawn. He is a partner at an accounting firm, but his colleagues are threatening to fire him if he continues to perform poorly at work. The patient cannot explain the recent changes to his mood and tearfully admits he fears there is something seriously wrong with him. His wife says that he is getting worse. The patient’s medical history is significant for hypertension, for which he takes lisinopril. His family history is unknown as he was adopted. The patient met his mother once and never knew his father but was told he died in his 50s. He drinks a few glasses of wine per week and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 141/87 mmHg, pulse is 81/min, and respirations are 12/min. On physical exam, the patient has a flat affect with a facial grimace and sudden jerky movements of his upper extremities. Which of the following is most likely to be seen on further workup?
Dorsal striatum atrophy on head CT
Neurofibrillary tangles on brain biopsy
Alpha-synuclein aggregates on brain biopsy
Frontotemporal atrophy on head CT
A
Dorsal striatum atrophy on head CT
This patient presents with recent mood changes, jerky movements of the upper extremities, and a history of early death in his father, which suggests a diagnosis of Huntington disease. Huntington disease is characterized by atrophy of the caudate nucleus, which is a structure of the dorsal striatum. Huntington disease (HD) is an autosomal dominant trinucleotide (CAG) repeat disorder located on chromosome 4. HD typically presents with mood changes and abnormal movements (particularly chorea or myoclonus) over weeks to months. Although executive function can be affected early on, memory is usually preserved until relatively late in the disease. Frontotemporal dementia, in contrast, usually presents with behavioral changes, and Alzheimer disease has memory impairment as an early sign. Caudate atrophy is typically seen on head imaging in Huntington disease. This disease cannot be cured but supportive treatment includes benzodiazepines and tetrabenazine for symptomatic relief of muscle spasms. Walker presents a review of the evidence regarding the etiology of Huntington disease. He discusses that typically the onset of symptoms is in middle age after affected individuals have had children. He recommends a better understanding of the function of the mutant protein in this disease. Incorrect Answers: Answer A: Alpha-synuclein aggregates would be found on brain biopsy in Parkinson disease and Lewy body dementia. Lewy body dementia is characterized by parkinsonism and visual hallucinations. Patients would present with cogwheel rigidity, bradykinesia, and a resting tremor. Treatment is with dopamine agonists such as carbidopa/levodopa. Answer C: Frontotemporal atrophy is consistent with a diagnosis of frontotemporal dementia. Frontotemporal dementia presents primarily with early behavioral changes, and it would not present with myoclonus. Treatment is supportive as there is no cure for this disease. Patients and their spouses may require psychiatric support for changes in behavior. Answer D: Neurofibrillary tangles on brain biopsy would suggest a diagnosis of Alzheimer disease. This disease presents with a slowly progressive cognitive decline in elderly patients. Patients have early loss of memory but have maintained motor function and behavior until later in the course of the disease. Answer E: A positive 14-3-3 CSF assay would confirm the diagnosis of Creutzfeldt-Jakob disease (CJD). Although CJD may also present with mood changes and myoclonus, CJD most prominently causes rapidly progressive dementia early in life. Treatment is supportive and death occurs quickly. Bullet Summary: Huntington disease is an autosomal dominant trinucleotide repeat disorder located on chromosome 4 that causes caudate atrophy and presents with mood changes, abnormal movements, and subcortical dementia.
nan
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A 6-hour-old newborn boy is noted to have a “lump on his head” by his mother. She denies that the lump was present at birth and is concerned about an infection. The child was born at 39 weeks gestation to a 34-year-old G2P2 mother by vacuum-assisted vaginal delivery after prolonged labor. His birth weight was 3.8 kg (8.4 lb) and his length and head circumference are at the 40th and 60th percentiles, respectively. The mother was diagnosed during this pregnancy with gestational diabetes mellitus and received prenatal care throughout. All prenatal screening was normal and the 20-week anatomy ultrasound was unremarkable. His temperature is 98.6°F (37°C), blood pressure is 65/42 mmHg, pulse is 131/min, and respirations are 36/min. On physical exam, the child is in no acute distress. He has a 3x3 cm fluctuant swelling over the right parietal bone that does not cross the midline. There is no discoloration of the overlying scalp. Laboratory testing is performed and reveals the following: Total bilirubin: 5.5 mg/dL Direct bilirubin: 0.7 mg/dL Which of the following is the most appropriate next step in management?
Intensive phototherapy
Incision and drainage
Red blood cell transfusion
Observation only
D
Observation only
This patient presents with a fluctuant swelling of the scalp that is limited by suture lines, which is the classic description of a cephalohematoma. The majority of cases of cephalohematoma self-resolve, thus the most appropriate next step in management is observation. A cephalohematoma is a relatively common traumatic birth injury that occurs after prolonged labor or instrumentation due to rupture of the periosteal bridging veins. Because bleeding through these veins is slow, they typically present several hours after birth with a fluctuant swelling of the scalp that is limited by suture lines. The majority of cases self-resolve between 2 weeks to 3 months. In rare cases, the sequestration of blood in the cephalohematoma may cause an unconjugated hyperbilirubinemia that requires phototherapy but this is a rare complication. Treatment is with reassurance and outpatient monitoring. Raines et al. review the evidence regarding the diagnosis and treatment of cephalohematoma. They discuss how this injury presents gradually and does not cross suture lines. They recommend conservative treatment. Incorrect Answers: Answer A: An incision and drainage procedure is contraindicated in cephalohematoma without signs of infection, such as erythema of the skin overlying the swelling. In cases where the cephalohematoma is thought to be secondarily infected, patients should undergo incision and drainage to prevent osteomyelitis. Patients would present with increasing pain, erythema, and systemic findings such as fever. Answer B: Intensive phototherapy may be used to manage the unconjugated hyperbilirubinemia that may occur due to cephalohematoma, but the majority of cases resolve spontaneously. The unconjugated hyperbilirubinemia found in this patient does not necessitate phototherapy. Unconjugated bilirubin is produced due to the metabolism of hemoglobin. Answer C: Neurosurgical decompression may be necessary to manage other neonatal birth injuries, such as intracranial hemorrhages. Because a cephalohematoma involves bleeding between the periosteum and skull (i.e., outside of the skull), it does not require neurosurgical intervention. Intracranial hemorrhages would present with asymmetric reflexes or obtundation. Answer E: Red blood cell transfusions are sometimes necessary to manage intracranial hemorrhages in neonates with significant blood loss. They are not usually required to manage cephalohematoma as the bleeding in this condition is limited by the suture lines. Patients who are tachycardic or hypotensive may require transfusion. Bullet Summary: Most cases of cephalohematoma resolve spontaneously but can sometimes present with an unconjugated hyperbilirubinemia that requires treatment with phototherapy.
nan
https://step2.medbullets.com/testview?qid=216589
A 64-year-old man presents with nausea, vomiting, and weakness. He states that his wife made him come in when he fainted today while attempting to stand up. He otherwise states he has noticed some visual changes, including noting a yellow tint to objects. He has a history of heart failure with reduced ejection fraction, obesity, diabetes, hypertension, acute coronary syndrome, atrial fibrillation, and peripheral vascular disease. His temperature is 98.5°F (36.9°C), blood pressure is 153/91 mmHg, pulse is 40/min, respirations are 15/min, and oxygen saturation is 97% on room air. Physical exam reveals a frail man who is unable to ambulate secondary to lightheadedness. His neurological exam is otherwise non-focal. An ECG is performed as seen in Figure A. Which of the following is the most likely etiology of this patient’s symptoms?
Amiodarone
Digoxin
Diltiazem
Procainamide
B
Digoxin
This patient with a history of heart failure with reduced ejection fraction and atrial fibrillation is presenting with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes, which are concerning for digoxin toxicity. Note that digoxin can be given in patients with poor cardiac function and atrial fibrillation; thus it is a plausible home medication for this patient. Digoxin is a cardiac glycoside that is used in conditions such as heart failure with reduced ejection fraction and atrial fibrillation. While it does not lower mortality, it may reduce symptoms of heart failure. Digoxin toxicity classically presents with gastrointestinal symptoms (nausea/vomiting), blurred vision or yellow halos, and cardiac dysrhythmias. While there is a myriad of dysrhythmias, digoxin can cause bradycardia, atrioventricular block, and premature ventricular contractions. ST-segment scooping can be seen with digoxin use both in toxicity but also with regular therapeutic use. Toxicity is treated with anti-digoxin antibodies. Patocka et al review digoxin toxicity. They note that this medication has a narrow therapeutic range and recommend close monitoring. Toxicity depends on severity and can be treated with anti-digoxin antibodies. Figure/Illustration A is an ECG demonstrating bradycardia and premature ventricular complexes (red circles). This is consistent with digoxin toxicity. Incorrect Answers: Answer A: Amiodarone is an antidyshythmic agent with toxicities including bradycardia, heart block, a prolonged QT interval, hepatitis, interstitial lung disease, hypothyroidism, corneal deposits and optic neuropathy. This patient could use amiodarone for his atrial fibrillation, but the visual disturbances are more typical of digoxin toxicity. Answer C: Diltiazem is a calcium channel blocker that would cause hypotension, bradycardia, and hyperglycemia. Treatment would involve immediate administration of calcium and vasopressors such as epinephrine. Other treatments in severe toxicity may include glucagon, dextrose, and insulin (to increase cardiac contractility). Answer D: Procainamide is the preferred antidysrhythmic in tachydysrhythmias associated with Wolff Parkinson White syndrome. It may cause drug-induced lupus and other dysrhythmias in overdose but would not cause this patient’s constellation of symptoms. Procainiamide is used rarely for atrial fibrillation and is only available for intravenous infusion in the US. Answer E: Propranolol toxicity would present with bradycardia, hypotension, confusion, seizures, and hypoglycemia. Treatment is centered on glucagon, calcium, and vasopressors such as epinephrine. In severe toxicity, lipid emulsion therapy and insulin/dextrose should be administered. Bullet Summary: Digoxin toxicity may present with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes.
nan
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A 69-year-old patient presents to the emergency department with shortness of breath and substernal pain. The patient has a medical history of obesity, anxiety, and constipation. He is currently not taking any medications and has not filled his prescriptions for over 1 year. An ECG is obtained and is seen in Figure A. The patient is managed appropriately and is discharged on atorvastatin, metoprolol, aspirin, clopidogrel, and sodium docusate. The patient visits his primary care physician 4 weeks later and claims to be doing better. He has been taking his medications and resting. At this visit, his temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 80/min, respirations are 11/min, and oxygen saturation is 96% on room air. The patient states he feels better since the incident. Which of the following is the most appropriate next step in management?
Add nifedipine
Add lisinopril
Add hydrochlorothiazide
Continue current medications and schedule follow up
B
Add lisinopril
This patient is presenting after a myocardial infarction for a follow-up appointment. The most appropriate next step in management is to start an angiotensin-converting enzyme (ACE) inhibitor such as lisinopril. When a patient experiences a myocardial infarction, there are certain medications that the patient should be started on that lower mortality. These medications include beta-blockers, aspirin, statins, other antiplatelet agents (clopidogrel), and ACE inhibitors. ACE inhibitors can reduce mortality after myocardial infarction by reducing afterload, allowing the damaged myocardium to maintain its ejection fraction. These medications also inhibit ventricular remodeling in this disease. ACE inhibitors most prominently reduce mortality if a patient's ejection fraction is reduced. Of note, ACE inhibitors also reduce mortality in diabetes by decreasing hyperfiltration damage to the glomeruli. Jering et al. hypothesized that using an angiotensin receptor neprilysin inhibitor would improve mortality in patients who have sustained a myocardial infarction. They discuss how an ACE inhibitor is the standard of care in patients currently. They recommend performing a randomized controlled trial comparing these treatment modalities. Figure/Illustration A demonstrates an ECG with ST elevation (red circles) in leads II, III, and AVF. These ST elevations are indicative of a STEMI. Incorrect Answers: Answer A: Hydrochlorothiazide can be used to manage this patient's blood pressure; however, this drug does not lower mortality like an ACE inhibitor. In the setting of patients with myocardial infarctions, inhibition of detrimental ventricular remodeling is key for lowering mortality. Answer C: Nifedipine is a calcium channel blocker that can be used to reduce blood pressure; however, it does not reduce mortality in patients who have experienced an MI. Calcium channel blockers tend to have few side effects and can be safely started in most patients. Answer D: Continuing current medications and scheduling a follow-up appointment misses the opportunity for the patient to be started on a mortality-lowering ACE inhibitor. In patients who are amenable to starting a new medication, an ACE inhibitor should be administered to all patients who have sustained an MI. Answer E: Switching to carvedilol would be unnecessary and not likely to benefit this patient as much as adding an ACE inhibitor. Metoprolol is the preferred beta blocker in patients who have sustained an MI. In the absence of idiosyncratic side effects, there is no indication to use another beta blocker. Bullet Summary: Patients who have experienced a myocardial infarction should be discharged on mortality-lowering drugs such as aspirin, beta-blockers, anti-platelet agents, and ACE inhibitors.
nan
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A 55-year-old man presents to the emergency department with right knee pain. He woke last night with sudden-onset, sharp, 10/10 non-radiating pain in his right knee. He denies recent trauma and he has no known medical problems. He denies tobacco use and will often drink 6-8 beers or spirits on weekend nights. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 140/90 mmHg, respirations are 16/min, and oxygen saturation is 97% on room air. Physical exam reveals an erythematous, edematous right knee. Passive extension of the knee is limited by pain. Synovial fluid aspiration is performed and sent for analysis, which reveals 20,000 leukocytes/mm^3, and no organisms visualized. Microscopic examination of synovial fluid is shown in Figure A. Which of the following is the most likely diagnosis?
Gout
Lyme arthritis
Pseudogout
Rheumatoid arthritis
A
Gout
The patient’s sudden-onset knee pain with effusion and synovial fluid findings are characteristic of gout. Gout is caused by monosodium urate crystal deposition in joint spaces, most commonly in the big toe, ankle, or knee. Crystal deposition in the joint space leads to an intense inflammatory reaction that causes severe pain and joint effusion. Gout is most commonly due to lifestyle factors including a diet high in red meat and alcohol. Medications that cause elevated uric acid levels (such as thiazide diuretics) may also play a role. Conditions resulting in high cell turnover and therefore excessive purine release such as hematologic malignancies may also lead to the development of gout. Patients typically present with sudden-onset, severe pain, erythema, and joint effusion. Synovial fluid analysis will reveal an elevated leukocyte count and negatively birefringent, needle-shaped crystals. Treatment of acute gout is with NSAID medications, colchicine, or steroids depending on presentation and patient-specific factors. Prevention of further attacks is centered around lifestyle changes such as avoidance of red meat and alcohol. Xanthine oxidase inhibitors such as allopurinol are also often used for prevention of further episodes. Neogi discusses the management of acute gout. She comments that, while synovial fluid analysis is the gold standard of diagnosis, many physicians do not routinely perform it, instead relying on clinical judgment to diagnose gout. The differential diagnosis for a gout-like presentation includes pseudogout, septic arthritis, Lyme arthritis, reactive arthritis, and rheumatoid arthritis. Figure A shows monosodium urate crystals, which appear as negatively birefringent, needle-shaped crystals under polarized light. Incorrect Answers: Answer B: Lyme disease may also presents as a monoarticular arthritis in later stages. However, a recent history of tick exposure and preceding flu-like illness would also be expected. Answer C: Pseudogout presents similar to gout, but is caused by calcium pyrophosphate deposition. Crystals appear rhomboid shaped and are positively birefringent under polarized light. Answer D: Septic arthritis typically has higher leukocyte counts (> 50,000/mm^3) in the synovial fluid with organisms visualized on gram stain. Fever would also be expected. Answer E: Rheumatoid arthritis may present as a monoarticular arthritis with acute flairs. However, crystals visualized in synovial fluid are more suggestive of acute gout. Bullet Summary: Acute gout presents with sudden-onset, severe joint pain and is characterized by needle-shaped negatively birefringent crystals visualized under polarized light.
Septic arthritis
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A 55-year-old woman is brought to the emergency department by her husband with a 1 hour history of an unremitting headache. The headache started suddenly while she was eating dinner and she says it feels like the “worst headache of my life.” An emergent CT scan of the head without contrast confirms the diagnosis, and a CT angiogram identifies the source of bleeding. The patient undergoes surgical management of her condition. On hospital day 3, she is found to be disoriented to person, place, and time. She also develops nausea and vomiting. Her medical problems consist of heart failure for which she takes furosemide, spironolactone, and metoprolol, which were continued at admission. Her temperature is 99.6°F (37.6°C), blood pressure is 100/60 mmHg, pulse is 112/min, and respirations are 16/min. Physical examination shows poor skin turgor. Capillary refill time is 4 seconds. Serum laboratory results are shown below: Na+: 120 mEq/L Cl-: 92 mEq/L K+: 3.9 mEq/L HCO3-: 26 mEq/L BUN: 32 mg/dL Creatinine: 1.0 mg/dL Serum osmolality is 265 mEq/L and urine osmolality is 340 mEq/L. Urine sodium is 44 mEq/L. Which of the following is the most likely diagnosis?
Addison disease
Cerebral salt wasting
Diuretic overuse
Primary polydipsia
B
Cerebral salt wasting
This patient presents with a subarachnoid hemorrhage (“worst headache of my life”) which is treated and then subsequently develops nausea, vomiting, and altered mental status with reduced serum sodium concentration, indicative of symptomatic hyponatremia. In the setting of serum hypo-osmolality, urine hyper-osmolality, urine sodium concentration > 20 mEq/L, and hypovolemia (low blood pressure, decreased skin turgor, and prolonged capillary refill), this is indicative of cerebral salt wasting. The mechanism of cerebral salt wasting is unclear, although some hypotheses point to increased release of brain natriuretic peptide after injury leading to suppression of renal sodium reabsorption, or sympathetic nervous system injury resulting in an impaired renin response. Patients classically present several days after cerebral injury or neurosurgery with hypovolemic hyponatremia and hypochloremia. Water retention results in serum hypo-osmolality, hyponatremia, and hypochloremia with urine hyper-osmolality and increased urine sodium excretion. In patients with prolonged cerebral salt wasting, hypokalemia can also occur (potassium may be normal in acute states). Treatment in mild or moderate cases consists of normal saline infusion to correct both volume status and hyponatremia. Treatment in severe cases consists of hypertonic saline. During correction, serum sodium must be monitored closely to prevent central pontine myelinolysis. Cui et al. describe the differences between syndrome of inappropriate anti-diuretic hormone (SIADH) and cerebral salt wasting. They discuss how volume status serves as the key differentiator between these conditions. They recommend paying closer attention to total body electrolyte balance in understanding these conditions. Incorrect Answers: Answer A: Addison disease, also known as primary adrenal insufficiency, results in salt wasting due to decreased mineralocorticoid activity. Because of reduced aldosterone release, patients also have hyperkalemia and metabolic acidosis. In contrast, this patient’s potassium and bicarbonate levels are normal. Treatment is with steroids. Answer C: Diuretic overuse is occasionally observed in patients using loop diuretics. Overuse of loop diuretics can cause hyponatremia, hypokalemia, and hypochloremia. Metabolic alkalosis is commonly seen due to volume contraction. Cerebral salt wasting is more likely in this patient presenting after a recent cerebral injury. Treatment is the cessation of the causative medication. Answer D: Primary polydipsia can cause symptomatic hyponatremia. The urine would be maximally diluted (urine osmolality < 100 mEq/L) due to an appropriate physiological response to hyponatremia and excess free water intake. Treatment is cessation of excessive water consumption. Answer E: Syndrome of inappropriate anti-diuretic hormone (SIADH) presents with identical findings as cerebral salt wasting with the exception of euvolemia as opposed to hypovolemia. An assessment of volume status is essential to differentiate. This patient has clear signs of hypovolemia. Treatment of SIADH is fluid restriction followed by ADH inhibitors, if needed. Bullet Summary: Cerebral salt wasting presents with hyponatremia, hypochloremia, and hypovolemia in the setting of recent cerebral injury or neurosurgery.
Syndrome of inappropriate anti-diuretic hormone
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A 29-year-old woman comes to the emergency department complaining of painful genital ulcers that have been present for 4 days as well as low-grade fever and malaise. She denies any recent travel, new sexual partners, or antibiotic use. Physical examination reveals multiple clustered vesicles and shallow ulcers in the vulvar region. A Tzanck smear shows multinucleated giant cells. Which of the following medications is most appropriate for treating her condition?
Acyclovir
Amoxicillin
Fluconazole
Metronidazole
A
Acyclovir
The patient's painful genital ulcers coupled with the presence of multinucleated giant cells on a Tzanck smear are indicative of a herpes simplex virus type 2 (HSV-2) infection. Acyclovir is the treatment of choice. Herpes infections can present with painful genital ulcers and low-grade fever. The Tzanck smear showing multinucleated giant cells further confirms the diagnosis though the diagnosis is typically made on clinical grounds and treated empirically. Acyclovir is the drug of choice for treating HSV-2 as it inhibits viral DNA polymerase, preventing the virus from replicating. The drug can reduce the duration and severity of symptoms but does not eradicate the virus entirely. Groves (2016) emphasizes that genital herpes, caused by the herpes simplex virus (HSV), affects over 400 million people worldwide. The infection is lifelong, with vesicular outbreaks in genital areas that can ulcerate. While HSV-1 and HSV-2 are both culprits, the latter increases the risk of HIV acquisition. The polymerase chain reaction assay is the recommended diagnostic method. Nucleoside analogues are effective treatments, and pregnant women with the condition are advised antiviral prophylaxis from the 36th week until delivery to prevent neonatal complications. Incorrect Answers: Answer B: Amoxicillin is an antibiotic ineffective against viral infections like HSV-2. It is commonly used for bacterial infections such as streptococcal pharyngitis. Answer C: Fluconazole is an antifungal agent is used for treating fungal infections like Candida which may cause esophagitis or vaginitis. Answer D: Metronidazole is used primarily for anaerobic bacterial and protozoal infections. Answer E: Trimethoprim-sulfamethoxazole is used for bacterial infections like urinary tract infections and also offers MRSA coverage. Bullet Summary: Acyclovir is the drug of choice for treating herpes simplex virus infections.
Trimethoprim-sulfamethoxazole
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A 77-year-old man presents to the emergency department acutely obtunded. The patient lives alone and was found unresponsive by his son. Generally, the patient manages his own finances, medications, and works part-time. He has not been responding to phone calls for the past 3 days. The patient is unable to offer a history. He has a past medical history of hypothyroidism, depression, and diabetes. His temperature is 88.0°F (31.1°C), blood pressure is 92/62 mmHg, pulse is 35/min, respirations are 9/min, and oxygen saturation is 92% on room air. The patient is cold to the touch and moves all extremities to painful stimuli. His pupils are reactive and sluggish, and he does not follow commands. There are no signs of trauma or skin infections. The patient is started on IV fluids and hydrocortisone, is externally warmed, and is started on a norepinephrine drip. An ECG is performed as seen in Figure A. Which of the following is the most appropriate next step in management?
Levothyroxine administration
Free T4 level
Thyroid stimulating hormone and free T4 level
Triiodothyronine administration
A
Levothyroxine administration
This elderly patient with a past medical history of hypothyroidism is presenting with altered mental status, hypotension, hypothermia, and bradycardia, which are concerning for myxedema coma. Empiric and immediate treatment with levothyroxine is the appropriate management of this condition prior to confirming the diagnosis with lab studies. Myxedema coma is a life-threatening complication of hypothyroidism. It presents with stupor/obtundation, bradycardia, hemodynamic instability, and hypothermia. With a high clinical suspicion, appropriate history, physical exam, and vitals, it is appropriate to first treat the patient with IV hydrocortisone (or another highly potent steroid) and levothyroxine prior to confirming the diagnosis given the high morbidity and mortality associated with this condition. Triiodothyronine is given in some circumstances as well (in more ill patients) but is less dire than first giving levothyroxine. Subsequently, the diagnosis can be confirmed with a serum thyroid stimulating hormone (TSH) and free T4 level. Patients are critically ill and typically require further care in an ICU. Wall discusses the management of myxedema coma. He notes this is a potentially lethal condition. He recommends immediate administration of IV levothyroxine. Figure A is an ECG with sinus bradycardia. Incorrect Answers: Answers 1, 3, & 4: Thyroid stimulating hormone and free T4 levels should certainly be obtained; however, the mortality for myxedema coma is so high that empiric treatment with levothyroxine should be started with clinical suspicion alone prior to confirming the diagnosis with laboratory studies, as a delay in therapy could increase morbidity and mortality. After administration of levothyroxine and initial supportive therapy, the diagnosis can be supported with laboratory studies. Answer E: Triiodothyronine or T3 could be appropriate in patients with myxedema coma in severe scenarios where patients do not respond to levothyroxine. In some cases, it may also be given empirically but does not take priority over T4 administration. Bullet Summary: In myxedema coma, empiric treatment with levothyroxine is appropriate with a high clinical suspicion for the diagnosis prior to laboratory confirmation.
nan
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A 65-year-old man presents to the emergency department with sudden-onset altered mental status. He was last seen normal 1 hour ago. His wife noticed that he was confused, slurring his speech, and had notable weakness that caused him to drop his cup of coffee. The patient is confused and not able to answer any questions. Facial drooping of the left lower face is noted, and the patient cannot follow any commands. He has a past medical history of diabetes and atrial fibrillation and is currently taking warfarin. His other medications are not known. His temperature is 98.0°F (36.7°C), blood pressure is 150/90 mmHg, pulse is 115/min, and respirations are 17/min. The patient is not able to follow any commands for further neurologic evaluation but does not appear to be moving his right upper extremity. In addition to further physical exam, which of the following is the most appropriate next step in management?
CT head
Fingerstick blood glucose
Fresh frozen plasma and vitamin K
Alteplase
B
Fingerstick blood glucose
This patient is presenting with sudden onset confusion, slurred speech, asymmetric smile, and weakness in the setting of risk factors for stroke (atrial fibrillation, diabetes, and hypertension). The most important initial step in management in a patient with altered mental status and neurological deficits is to obtain a fingerstick blood glucose. The workup of stroke is first centered on a thorough history and physical and immediately calculating an NIH stroke scale (11-domain assessment of stroke severity involving patient consciousness level, orientation, ability to follow commands, degree of gaze palsy, degree of vision loss, ataxia, motor drift, sensation, inattention, language/speech) as well as ascertaining when the patient was last known to be neurologically normal. While performing the exam and NIH stroke scale, a fingerstick blood glucose should be performed in all patients as hypoglycemia may present identically to a stroke. Severe hypoglycemia may present with lethargy, confusion, focal or global weakness, nausea, vomiting, agitation, and even seizures. If the patient is hypoglycemic, dextrose can be given which will rapidly correct the patient's underlying deficits. Ohshita et al. review other possible causes of stroke-like symptoms. They note that hypoglycemia may mimic stroke and cause focal neurological signs with common findings being unilateral motor weakness with mild or moderate alteration of consciousness. They noted that all patients improved within 1 hour of glucose injection in this study, thus the recommendation to check a fingerstick blood glucose in all stroke patients. Incorrect Answers: Answer A: Alteplase would be indicated if a patient presented with disabling neurologic deficits from an ischemic stroke within 4.5 hours of symptom onset only after a CT scan of the head has ruled out an intracranial bleed. The patient must have no other contraindications to thrombolytics including but not limited to recent trauma, known intracranial hemorrhage, rapidly improving neurologic deficits (spontaneously), and no other clear reversible cause (among many other contraindications). Answer B: CT head is the most appropriate initial step in the management of an acute stroke; however, a rapid fingerstick blood glucose takes precedence as hypoglycemia can mimic a stroke and be immediately addressed thus removing the need for further workup if there is a complete resolution of symptoms with normoglycemia. While an exam is ongoing, a fingerstick blood glucose is being obtained, and neuroimaging is also often ordered to be performed immediately after. Answer D: Fresh frozen plasma and vitamin K would be indicated in this patient if he had a hemorrhagic stroke (a CT of the head would need to confirm this) to reverse his anticoagulation with warfarin. Factor concentrate can also be used to rapidly reverse anticoagulation with warfarin. Answer E: MRI brain would be indicated in the workup of a stroke or transient ischemic attack after all emergency interventions and labs including CT head, serum chemistries, CBC, and ECG. It delineates the extent of the stroke and gives useful prognostic and diagnostic information. It also may elucidate strokes missed on other forms of neuroimaging such as CT perfusion. Bullet Summary: A fingerstick blood glucose should be obtained immediately in all patients with stroke-like symptoms or altered mental status.
nan
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A 48-year-old woman, gravida 3 para 3, presents to the clinic with a 5-month history of intermittent loss of urine. Her urinary leaking occurs throughout the day and night, and she often has to get up to use the bathroom while asleep. Exercising does not affect the loss of urine. She states that she often cannot make it to the bathroom in time once she senses a need to void. Her last menstrual period was 2 weeks ago. Her urinalysis is unremarkable, and her postvoid residual volume is 40 mL. On pelvic examination, there is no dribbling of urine when the patient coughs. Physical exam is otherwise unremarkable. Which of the following is the most likely diagnosis?
Overflow incontinence
Urge incontinence
Stress incontinence
Mixed urinary incontinence
B
Urge incontinence
This patient with a sudden need to void followed by loss of urine with urgency (unable to make it to the bathroom in time) most likely has urinary urge incontinence. Urge incontinence is a form of urinary incontinence characterized by a sudden urge to urinate, resulting in the involuntary leakage of urine. Normally, bladder contraction occurs due to the stimulation of muscarinic receptors in the detrusor muscle. Urinary urge incontinence is due to detrusor overactivity, leading to a sudden and frequently overwhelming need to void that occurs throughout the day and night. The most common cause is idiopathic, though it may be secondary to neurologic disorders (e.g., spinal cord injury or multiple sclerosis), bladder abnormalities, or chronic bladder inflammation. Workup is first centered on ruling out a urinary tract infection with a urinalysis. The diagnosis is made with urodynamic studies, and first-line treatment involves bladder training and pelvic floor exercises. Should first-line treatment of urge incontinence fail, antimuscarinic medications (such as oxybutynin) which reduce acetylcholine activity on muscarinic receptors can decrease detrusor contraction and thus the sense of urgency. Nandy and Ranganathan review urge incontinence. They note that it presents with leakage of urine associated with a sudden urge to urinate. They recommend an appropriate history and workup to first rule out other causes. Incorrect Answers Answer A: Genitourinary syndrome of menopause represents urinary symptoms (stress and/or urge incontinence) due to vaginal dryness and atrophy from menopause. This patient still has menstrual cycles and no symptoms of ovarian follicle depletion (narrowed introitus, vulvovaginal dryness and irritation, irregular vaginal bleeding), making this an unlikely diagnosis. Answer B: Mixed urinary incontinence presents with features of both stress and urge incontinence. This patient lacks symptoms of stress incontinence given a lack of incontinence with increases in intrabdominal pressure (such as exercise, coughing, laughing, or sneezing). Answer C: Overflow incontinence is caused by impaired detrusor muscle activity or bladder outlet obstruction. It presents with constant involuntary dribbling or urine and incomplete bladder emptying. Post-void residual volume is increased in patients with overflow incontinence. Answer D: Stress incontinence is caused by urethral hypermobility and otherwise insufficient urethral support. It presents with urinary leakage with activities that increase intraabdominal pressure (such as coughing, sneezing, laughing, and exercising). This patient’s symptoms are unrelated to exercise. Bullet Summary: Urge incontinence presents with sudden urinary urgency with a normal post-void residual volume and absence of findings consistent with urinary tract infection.
nan
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A 4-week-old boy is brought to the pediatrician by his parents for an initial evaluation. His parents are concerned that he is not feeding well and has lost weight over the last 2 weeks. He was born at home via spontaneous vaginal delivery at 37 weeks of gestation to a G1P1 mother. His mother did not receive prenatal care. His blood pressure is 70/44 mmHg, pulse is 151/min, and respirations are 41/min. His weight is 3 kg (4th percentile) compared with 3.5 kg at birth (45th percentile). On physical exam, he is jaundiced with an enlarged liver and spleen. He does not appear to respond to visual stimuli, and further examination reveals bilateral clouding of the lens. Which of the following interventions could have avoided this patient's symptoms?
Avoiding fruit juice and sweetened foods
Changing to a soy based formula
Providing imiglucerase enzyme replacement
Removing phenylalanine from maternal diet during pregnancy
B
Changing to a soy based formula
This patient who presents with failure to thrive, hepatosplenomegaly, and bilateral cataracts most likely has classic galactosemia. Patients with this disorder should avoid lactose-containing products by changing to a soy-based formula. Classic galactosemia is an autosomal recessive defect in galactose-1-phosphate uridyltransferase. This enzyme is involved in the conversion of galactose to glucose, and a deficiency of this enzyme results in the accumulation of galactose 1-phosphate in the liver, kidney, and brain. This metabolite acts as a phosphate sink, meaning that it traps all free phosphate in the cytosol and inhibits the formation of other phosphate-dependent metabolites such as adenosine triphosphate. This results in failure to thrive, vomiting, lethargy, and hepatosplenomegaly after ingestion of galactose-containing products such as milk. Patients will also develop bilateral cataracts if the deficit is not detected in the first few weeks after birth. Avoiding galactose by changing to a soy-based formula will avoid the complications of this disorder. Turck discusses using soy protein for infant feeding. The author notes that there are safety concerns with regards to the high phyto-estrogenic isoflavone content of these formulas. The author recommends that despite these safety concerns, these formulas should still be used in patients with classic galactosemia. Incorrect Answers: Answer A: Avoiding fruit juice and sweetened foods would be effective in the treatment of hereditary fructose intolerance. This disease presents similarly to classic galactosemia, but cataracts would not be seen in patients with this disease. Answer C: Providing imiglucerase enzyme replacement would be effective in the treatment of patients with Gaucher disease, which can also present with failure to thrive. However, associated findings in this disease would be anemia and avascular necrosis of the bones. Answer D: Removing phenylalanine from the maternal diet during pregnancy would be effective in the treatment of patients with phenylketonuria, which can also present with failure to thrive. However, these patients classically present with a "musty smell" and eczema. Answer E: Vitamin B6 supplementation may be effective in mitigating the negative symptoms of homocystinuria. This disease may also present with cataracts or lens dislocations. Associated findings in this disorder would be marfanoid appearance and arachnodactyly. Bullet Summary: Patients with classic galactosemia should not be breastfed and should receive a galactose-free and lactose-free diet such as soy-based formulas.
Vitamin B6 supplementation
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A 46-year-old farmer presents to the office for a follow-up visit. He was seen 4 days ago for a new lesion on his arm that had been present for 1 week prior to that visit. A photograph of the lesion is shown in Figure A. At that time, he was prescribed treatment for the lesion and instructed to follow up as necessary. Today, he reports that he became sunburned this morning within an hour of beginning his workday, despite wearing appropriate sunscreen. He has a medical history significant for type 2 diabetes mellitus that is controlled with metformin. He has no known medical allergies. He smokes 1 pack of cigarettes per day and denies alcohol or illicit drug use. His temperature is 98.6°F (37.0°C), blood pressure is 125/70 mmHg, pulse is 65/min, and respirations are 12/min. On exam, there is a blistering erythematous rash on the arms and shoulders. The previous skin lesion appears unchanged. Which of the following is the most likely explanation for the patient's new rash?
Allergic reaction to prescribed therapy
Progression of disease and alternative therapy is indicated
Progression of disease and surgical debridement is necessary
Adverse effect of doxycycline
D
Adverse effect of doxycycline
This farmer has a cutaneous black eschar most consistent with cutaneous anthrax for which doxycycline is an appropriate treatment. A new blistering, erythematous rash after spending an hour outside that appears 4 days after starting treatment is consistent with a photosensitivity reaction, a known adverse effect of doxycycline. Cutaneous anthrax is the most common form of infection caused by Bacillus anthracis. The infection is contracted during contact with infected livestock. The first-line treatment is doxycycline, ciprofloxacin, levofloxacin, or moxifloxacin. Progression to systemic anthrax would result in pulmonary (flu-like symptoms and hemoptysis) or gastrointestinal (nausea, vomiting, and dysentery) symptoms and would require treatment with clindamycin plus ciprofloxacin. Patients with meningitis due to anthrax require antibiotic therapy with ciprofloxacin plus meropenem plus linezolid. Additionally, antitoxins such as raxibacumab and adjunctive therapies such as glucocorticoids should be considered. In addition to photosensitivity, adverse effects of doxycycline include gastrointestinal upset (most common), teeth discoloration, and inhibition of bone growth in children. Doxycycline is teratogenic and should not be prescribed during pregnancy. Migone et al. discuss the use of raxibacumab, a monoclonal antibody against a component of the anthrax toxin, in treating inhalational anthrax. The authors found that in monkeys, raxibacumab significantly increased survival after exposure to anthrax. The authors recommend the use of antitoxins such as raxibacumab in any patient suspected of having systemic anthrax. Figure/Illustration A demonstrates the characteristic black eschar (blue circle) of cutaneous anthrax. Incorrect Answers: Answer A: Adverse effects of ciprofloxacin do not include photosensitivity, though it is an appropriate antibiotic choice for cutaneous anthrax treatment. Adverse effects of ciprofloxacin include gastrointestinal upset, risk of tendon rupture, and QT prolongation. Answer C: Allergic reactions to prescribed therapy would be characterized by urticaria and wheals. In severe cases, anaphylaxis may be present. Answer D: Progression of disease and alternative therapy is indicated does not fit this case. The patient's symptoms (blistering, erythematous rash) are not concerning for the progression of cutaneous anthrax to systemic disease, which would result in pulmonary or gastrointestinal symptoms. Alternative therapy for systemic anthrax is not indicated in this case. Answer E: Progression of disease and surgical debridement is not indicated. The patient's symptoms (blistering, erythematous rash) are not concerning for the progression of cutaneous anthrax to systemic disease, which would result in pulmonary or gastrointestinal symptoms. Additionally, surgical debridement is not indicated for cutaneous anthrax. Bullet Summary: Cutaneous anthrax presents with a black eschar and is treated with antitoxin and either doxycycline or ciprofloxacin, the former of which can cause a photosensitivity reaction.
nan
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A 54-year-old man is brought to the emergency department following a skiing accident. The patient lost control of his skis and collided with a tree. He lost consciousness for about 30 seconds. When he woke up, he was mildly confused but able to ski down the rest of the mountain. The patient initially denied medical treatment but his wife convinced him to be taken by ambulance to the emergency department for evaluation. The paramedics report that at the scene the patient had a Glasgow Coma Score (GCS) of 15. During the ride, he became gradually more somnolent. His medical history is significant for hypertension, hyperlipidemia, coronary artery disease, and a prior transient ischemic attack. His medications include atorvastatin and lisinopril. In the emergency department, his temperature is 97.8°F (36.6°C), blood pressure is 141/84 mmHg, pulse is 71/min, and respirations are 16/min. He is difficult to arouse with a GCS of 7. A head CT is performed and can be seen in Figure A. Which of the following is the most likely cause of this patient’s presentation?
Damage to the sphenoid bone
Damage to the cerebral bridging veins
Secondary hemorrhage into prior area of infarction
Occlusion of cerebral small penetrating arteries
A
Damage to the sphenoid bone
This patient presents with a lucid interval followed by worsening somnolence and a biconvex lesion on head CT, which suggests a diagnosis of acute epidural hematoma. Acute epidural hematomas are most commonly caused by damage to the middle meningeal artery from trauma to the sphenoid bone. Patients with an epidural hematoma typically have an initial loss of consciousness followed by a lucid interval, during which their mental status returns to baseline or near baseline. As the hematoma expands, patients display increasing somnolence from the increase in intracranial pressure. For that reason, epidural hematomas are sometimes referred to as the “talk and die” syndrome. If not relieved, this increase in intracranial pressure can lead to uncal herniation or death. Patients with neurologic findings or those with expanding bleeds require urgent surgical decompression. Dadashi et al. present cases of patients with epidural hematoma from acute trauma. They discuss how patients often sustain these injuries from extreme sports. They recommend being aware of this syndrome so that acute interventions can be taken. Figure/Illustration A demonstrates a biconvex hyperdensity that is limited by suture lines (red circle). This is the typical appearance of an epidural hematoma on head CT. Incorrect Answers: Answer A: Damage to the cerebral bridging veins results in a subdural hematoma (SDH). An acute SDH more commonly presents with a gradual increase in headache and confusion rather than the “talk and die” syndrome presented in this vignette. However, a minority of patients with acute SDH may have a lucid interval followed by progressive neurological decline. SDH would have a crescent-shaped appearance on head CT rather than the biconvex appearance of an epidural hematoma. Answer C: Occlusion of cerebral small penetrating arteries describes a lacunar stroke. Although this patient has 2 risk factors for lacunar stroke (hypertension and smoking), this patient’s head CT demonstrates an epidural hematoma. Answer D: A ruptured cerebral aneurysm would most likely lead to a subarachnoid hemorrhage. A subarachnoid hemorrhage presents as a sudden, severe headache (“thunderclap headache”), confusion or somnolence, nausea and vomiting, and meningismus. Surgical clipping or embolization of the bleeding vessel may be required. Answer E: Secondary hemorrhage into a prior area of infarction usually occurs in the setting of a recent embolic stroke. Although this patient has a history of TIA and other risk factors for stroke, his head CT demonstrates an epidural hematoma. Treatment is supportive with the control of underlying risk factors such as hypertension. Bullet Summary: Epidural hematomas are caused by damage to the middle meningeal artery as it passes through the foramen spinosum of the sphenoid bone.
nan
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A 4-week-old boy is brought to the pediatrician by his parents for an initial evaluation. His parents are concerned that he is not feeding well and has lost weight over the last 2 weeks. He was born at home via spontaneous vaginal delivery at 37 weeks of gestation to a G1P1 mother. His mother did not receive prenatal care. His blood pressure is 70/44 mmHg, pulse is 151/min, and respirations are 41/min. His weight is 3 kg (4th percentile) compared with 3.5 kg at birth (45th percentile). On physical exam, he is jaundiced with an enlarged liver and spleen. He does not appear to respond to visual stimuli, and further examination reveals bilateral clouding of the lens. Which of the following interventions could have avoided this patient's symptoms?
Vitamin B6 supplementation
Changing to a soy based formula
Providing imiglucerase enzyme replacement
Removing phenylalanine from maternal diet during pregnancy
B
Changing to a soy based formula
This patient who presents with failure to thrive, hepatosplenomegaly, and bilateral cataracts most likely has classic galactosemia. Patients with this disorder should avoid lactose-containing products by changing to a soy-based formula. Classic galactosemia is an autosomal recessive defect in galactose-1-phosphate uridyltransferase. This enzyme is involved in the conversion of galactose to glucose, and a deficiency of this enzyme results in the accumulation of galactose 1-phosphate in the liver, kidney, and brain. This metabolite acts as a phosphate sink, meaning that it traps all free phosphate in the cytosol and inhibits the formation of other phosphate-dependent metabolites such as adenosine triphosphate. This results in failure to thrive, vomiting, lethargy, and hepatosplenomegaly after ingestion of galactose-containing products such as milk. Patients will also develop bilateral cataracts if the deficit is not detected in the first few weeks after birth. Avoiding galactose by changing to a soy-based formula will avoid the complications of this disorder. Turck discusses using soy protein for infant feeding. The author notes that there are safety concerns with regards to the high phyto-estrogenic isoflavone content of these formulas. The author recommends that despite these safety concerns, these formulas should still be used in patients with classic galactosemia. Incorrect Answers: Answer A: Avoiding fruit juice and sweetened foods would be effective in the treatment of hereditary fructose intolerance. This disease presents similarly to classic galactosemia, but cataracts would not be seen in patients with this disease. Answer C: Providing imiglucerase enzyme replacement would be effective in the treatment of patients with Gaucher disease, which can also present with failure to thrive. However, associated findings in this disease would be anemia and avascular necrosis of the bones. Answer D: Removing phenylalanine from the maternal diet during pregnancy would be effective in the treatment of patients with phenylketonuria, which can also present with failure to thrive. However, these patients classically present with a "musty smell" and eczema. Answer E: Vitamin B6 supplementation may be effective in mitigating the negative symptoms of homocystinuria. This disease may also present with cataracts or lens dislocations. Associated findings in this disorder would be marfanoid appearance and arachnodactyly. Bullet Summary: Patients with classic galactosemia should not be breastfed and should receive a galactose-free and lactose-free diet such as soy-based formulas.
nan
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A 32-year-old G1P0 woman delivers a boy at 40 weeks and 3 days of gestation. The delivery is uncomplicated and the neonate has Apgar scores of 9 and 9 at 1 and 5 minutes respectively. After 15 minutes, he attempts to nurse for the 1st time. His mother notices that his face, chest, and extremities turn dusky blue during nursing. She also notes that he seems to be snoring loudly and breathing very quickly. When she removes him from her breast, he begins to cry and his color improves. The neonate’s prenatal course was unremarkable, and his mother has a medical history of asthma with occasional albuterol inhaler use. As the infant is resting, vital signs are taken. His temperature is 98.2°F (36.7°C), pulse is 130/min, respirations are 45/min, and oxygen saturation is 97% on room air. A physical exam demonstrates well-perfused skin but is notable for the facial features shown in Figure A. Which of the following is most likely to be observed in this neonate?
Areflexia in all extremities
Digital clubbing
Inability to pass a nasogastric tube
Markedly decreased lung volume
C
Inability to pass a nasogastric tube
This neonate presents with central cyanosis upon feeding, resolution with crying, and midface hypoplasia on exam, most consistent with choanal atresia. A nasogastric (NG) tube would not pass through the nasal canal. Choanal atresia is caused by failed recanalization of the nasal fossae during fetal development. This disease typically presents with noisy breathing, cyanosis when feeding, and improvement with crying and the use of the mouth for breathing. Cyanosis in this condition affects the entire body as opposed to just the hands and feet. Bilateral atresia often presents early in life. Clinical suspicion of choanal atresia should prompt an attempt to pass an NG tube, which will meet resistance at the posterior part of the nasal canal. Treatment is with the surgical creation of the nasal passages. Lesciotto et al. review the diagnosis and pathophysiology of patients with choanal atresia. They discuss how this condition is often comorbid with craniosynostosis. They recommend a better understanding of how these patients develop decreased nasal airway volume. Figure/Illustration A is a clinical photograph showing midface hypoplasia with a poorly developed nasal bone (red circle). These findings are consistent with a diagnosis of choanal atresia. Incorrect Answers: Answer A: Areflexia in all extremities may be seen in spinal muscular atrophy, a genetic condition that causes loss of motor neurons. This condition may cause difficulty feeding due to weakness of the oropharyngeal and glossopharyngeal muscles, but it typically does not cause cyanosis. Treatment is with steroid use and nusinersen. Answer B: Digital clubbing can result from chronic hypoxemia, as seen in conditions such as cystic fibrosis. It would be unexpected for a neonate to have clubbing as the characteristic finger appearance depends on the long-term deposition of platelet-derived growth factor and would not develop within minutes. This patient also does not have cyanosis at baseline. Treatment of cystic fibrosis is with enzyme repletion and respiratory therapy. Answer D: Markedly decreased lung volume can occur in congenital diaphragmatic hernia, in which the abdominal contents herniate into the chest cavity and cause pulmonary hypoplasia. Infants may present with cyanosis due to respiratory failure, but there would be no association with feeding or improvement with crying. Vital signs would also be abnormal. Treatment is with surgical repair of the diaphragm. Answer E: A patent foramen ovale occurs when the connection between the left and right atria does not close soon after birth. It does not result in cyanosis and is typically asymptomatic, though it may cause paradoxical embolism and stroke later in life. Treatment of a symptomatic patent foramen ovale is surgical closure of the defect. Bullet Summary: Choanal atresia presents with cyanosis and respiratory distress that worsens with feeding and improves with crying associated with failure to pass a nasogastric tube.
Patent foramen ovale
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A 16-year-old boy presents to an ophthalmologist with blurry vision. Over the past several months, he has had increasing difficulty seeing the board from the back of the classroom at school. The patient is otherwise doing well in school and enjoys playing basketball. His medical history is otherwise significant for scoliosis which is managed by an orthopedic surgeon. His family history is significant for a mother with type 2 diabetes mellitus and a father who underwent aortic valve replacement last year. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. On physical exam, the patient is tall for his age and has long arms. He has 20 degrees of thoracic scoliosis, which is stable from previous exams. On slit-lamp examination, the patient is found to have bilateral upward lens subluxation and is prescribed corrective lenses. Which of the following is the most likely cause of this patient’s symptoms?
Defective metabolism of methionine
Extra copy of sex chromosome
Mutation of chromosome 15
Mutation of COL5A1 or COL5A2
C
Mutation of chromosome 15
This patient presents with tall stature, upward lens subluxation, and a family history of aortic valve disease, which suggests a diagnosis of Marfan syndrome. Marfan syndrome is caused by an autosomal dominant mutation in the fibrillin-1 gene on chromosome 15. The fibrillin-1 gene codes for a glycoprotein in the extracellular matrix of connective tissue. Patients with Marfan syndrome classically present with a triad of musculoskeletal, ocular, and cardiovascular symptoms. The musculoskeletal findings include tall stature, long limbs, arachnodactyly, pectus excavatum, joint hypermobility, skin hyperelasticity, and scoliosis. Patients with Marfan syndrome also often present with upward lens subluxation, and they are at increased risk of cardiac disease, including aortic regurgitation and aortic dissection. Treatment is symptomatic with avoidance of contact sports, beta-blockers, and valve repair to prevent cardiac complications. Bitterman and Sponseller review the evidence regarding the diagnosis and treatment of Marfan syndrome. They discuss how skeletal manifestations are often the initial presentation of this condition leading to diagnosis. They recommend screening for patients who are suspected to have this disease. Incorrect Answers: Answer A: Defective metabolism of methionine describes homocystinuria, an autosomal recessive deficiency of cystathionine synthase. Although patients with homocystinuria may also have tall stature, arachnodactyly, and pectus deformity, the lens dislocation in homocystinuria is downward rather than upwards. Patients with homocystinuria also tend to have a fair complexion, intellectual disability, and thrombophilia. Treatment includes vitamin B6 supplementation. Answer B: Extra copy of a sex chromosome describes Klinefelter syndrome (47,XXY). Although patients with Klinefelter syndrome classically present with tall stature, they do not have the other clinical features found in this patient, including joint hypermobility and lens subluxation. Patients with Klinefelter also present with hypogonadism and azoospermia. Treatment is supportive Answer D: Mutation of COL5A1 or COL5A2 leads to Ehlers-Danlos syndrome, a collagen disorder that commonly presents with joint hypermobility, skin hyperextensibility, and skin fragility with easy bruising. Ehlers-Danlos would not explain this patient’s lens subluxation. Treatment may include bracing of unstable joints. Answer E: Mutation of RET proto-oncogene describes multiple endocrine neoplasia type 2B (MEN2B). Patients with MEN2B may present with Marfanoid habitus and are predisposed to medullary thyroid cancer, pheochromocytoma, and mucosal neuromas. MEN2B would not explain lens subluxation. Treatment is with surgical resection of tumors. Bullet Summary: Marfan syndrome is caused by an autosomal dominant mutation in the fibrillin-1 gene on chromosome 15 and presents with a triad of musculoskeletal, ocular, and cardiovascular findings.
Mutation of RET proto-oncogene
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A newborn boy is assessed in the newborn nursery due to central cyanosis that does not respond to oxygen therapy. The patient was born to a G1P1 mother at 39 weeks gestation. The mother had diabetes that was managed with exercise and insulin during the pregnancy. The mother has never been vaccinated and did not follow routine prenatal care. The newborn's temperature is 99.5°F (37.5°C), blood pressure is 70/30 mmHg, pulse is 160/min, respirations are 27/min, and oxygen saturation is 80% on room air. The initial workup of the patient includes a chest radiograph seen in Figure A as well as lab values as seen below. Hemoglobin: 14 g/dL Hematocrit: 42% Leukocyte count: 6,500/mm^3 with normal differential Platelet count: 197,000/mm^3 Serum: Na+: 139 mEq/L Cl-: 101 mEq/L K+: 4.3 mEq/L HCO3-: 24 mEq/L BUN: 12 mg/dL Glucose: 99 mg/dL Creatinine: 0.5 mg/dL Ca2+: 10.0 mg/dL AST: 12 U/L ALT: 10 U/L An electrocardiogram is obtained as seen in Figure B. Which of the following is the most likely diagnosis?
Tetralogy of Fallot
Total anomalous pulmonary venous return
Transposition of the great vessels
Tricuspid valve atresia
C
Transposition of the great vessels
This patient is presenting with right axis deviation on ECG and an "egg on a string" heart on chest radiograph suggesting a diagnosis of transposition of the great vessels. Transposition of the great vessels occurs when the spiral septum that divides the truncus arteriosus into the aorta and pulmonary artery fails to spiral and connect the right ventricle to the pulmonary artery and the left ventricle to the aorta. Patients will present with central cyanosis and must have another cardiac defect/shunt in order to remain viable (such as an atrial septal defect, ventricular septal defect, or patent ductus arteriosus). Chest radiograph will reveal an "egg on a string" heart and ECG will reveal right axis deviation. The diagnosis is confirmed with echocardiography and treated surgically. Cohen et al. review the treatment of patients with transposition of the great vessels. They discuss how multiple surgical procedures have been performed in an attempt to address this congenital abnormality. They recommend using MRI to characterize the success of these procedures. Figure A is a chest radiograph demonstrating an elongated cardiac shadow (red circle). This classic "egg on a string" appearance of the heart is seen in transposition of the great vessels. Figure B is an ECG demonstrating right axis deviation as revealed by being down in lead I (red circle) and up in aVF. Right axis deviation is classically seen in transposition of the great vessels. Incorrect Answers: Answer A: Tetralogy of Fallot presents with a boot-shaped heart on a chest radiograph and classic "Tet spells" where patients will squat thus improving shunting and cardiac performance. Treatment is with surgical correction of the abnormal spiral septum. Answer B: Total anomalous pulmonary venous return occurs when pulmonary veins drain into the right heart circulation. It presents with cyanosis that does not correct with oxygenation but does not present with an "egg on a string" heart on a chest radiograph. Treatment is with surgical reconfiguration of pulmonary venous return. Answer D: Tricuspid valve atresia occurs when the tricuspid valve never forms and thus there is no connection between the right atrium and right ventricle. Patients will present with cyanosis and poor feeding/poor growth. Treatment is with surgical creation of a connection between these chambers. Answer E: Truncus arteriosus occurs secondary to failure of the spiral septum to divide the aorta and pulmonary artery. It presents with poor feeding, diaphoresis, tachypnea, and cyanosis. Treatment is with surgical separation of these arterial chambers. Bullet Summary: Transposition of the great vessels presents with central cyanosis, an "egg on a string" appearance on a chest radiograph, and right axis deviation on ECG.
Truncus arteriosus
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A 24-year-old woman with no significant medical history presents to the emergency department for progressive shortness of breath. She endorses decreased exercise tolerance as well as intermittent palpitations. She returned from a recent missionary trip to South America 12 months prior where she endorsed eating a local delicacy as well as a period of fevers and eyelid swelling during her stay. Her temperature is 99.1°F (37.3°C), pulse is 95/min, blood pressure is 130/85 mmHg, respirations are 15/min, and oxygen saturation is 97% on room air. Physical exam is notable for a laterally displaced point of maximal impulse as well as a 2/6 diastolic murmur radiating to the axilla. A blood smear is shown in Figure A. Her laboratory results are: Leukocyte count and differential: Leukocyte count: 10,000/mm^3 Neutrophils: 57% Lymphocytes: 30% Monocytes: 3% Eosinophils: 9% Basophils: 1% Hemoglobin: 13.1 g/dL Platelet count: 420,000/mm^3 Which of the following is the most appropriate treatment?
Amphotericin B
Benznidazole
Erythromycin
Ganciclovir
B
Benznidazole
This patient presents with symptoms of heart failure, eosinophilia, trypanosomes on peripheral smear, and a history of recent travel to South America consistent with the diagnosis of Chagas disease. Treatment options for Chagas disease include benznidazole and nifurtimox. Chagas disease is caused by the parasite Trypanosoma cruzi and is most commonly transmitted by the reduvvid (kissing) bug. The disease is endemic to South America and presents in an acute and chronic phase. The acute phase is characterized by fever, headaches, myalgias, and unilateral eyelid swelling (Romaña’ sign) where the infectious agent has been rubbed into the eye. The chronic phase of the disease is characterized by dilated apical cardiomyopathy leading to conduction abnormalities and valvular dysfunction, megacolon, and megaesophagus. Laboratory testing reveals a peripheral eosinophilia and a peripheral blood smear may reveal the infectious trypanosomes. Treatment is with benznidazole and/or nifurtimox. Perez-Molina and Molina present the data surrounding the diagnosis and treatment of Chagas disease. They discuss how many cases progress to a chronic state characterized by cardiomyopathy, arrhythmias, and organomegaly. They recommend prompt treatment of the disease to avoid end organ damage. Figure/Illustration A shows a peripheral blood smear with the trypomastigote form of Trypanosoma cruzi (red circle). Note the anterior location of the dense basophilic nucleus. Incorrect Answers: Answer A: Amphotericin B is a potent antifungal that has application in the treatment of multiple fungal infections. It is used as a first-line agent for mucormycosis and cryptococcal meningitis (in addition to flucytosine). Amphotericin B with sodium stibogluconate is used to treat visceral leishmaniasis, a protozoal infection most commonly caused by Leishmania donovani. Side effects can include nausea, vomiting, rigors, fever, hypertension or hypotension, renal dysfunction, and hypoxia. Answer C: Erythromycin is a macrolide antibiotic that is used in the treatment of bacterial infections. It has a pro-motility effect and can also be used as a gastric pro-motility agent. Potential side effects include gastrointestinal upset, prolonged QT interval, inhibition of the P450 system, and pyloric stenosis in infants. Answer D: Ganciclovir is a synthetic nucleoside analogue that is mainly used in the treatment of cytomegalovirus (CMV) infections. The most common side effects are bone marrow suppression (granulocytopenia, thrombocytopenia, and anemia) and gastrointestinal upset. Answer E: Praziquantel is an anti-helminthic used in the treatment of tapeworms and flukes including Echinococcus, Taenia solium (cysticerosis), Schistosoma sp., Clonorchis sinensis (liver fluke), and Paragonimus westermani (lung fluke). Side effects of praziquantel develop due to release of contents from dead parasites and can include gastrointestinal upset, elevated liver enzymes, and dizziness. Bullet Summary: Treatment of Chagas disease is with benznidazole or nifurtimox.
Praziquantel
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A 40-year-old south asian man presents to a primary care provider with a chronic cough that is worse at night. He has had the cough for several years but it has been getting worse over the last few months. He recently moved to the United States to work in construction. He has lost 10 pounds in the last 3 months along with darker stools which he believes is caused by a Western diet. He denies any difficulty swallowing or feeling of food getting stuck in his throat. He drinks alcohol 1-2 times per week and has never smoked. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 114/72 mmHg, pulse is 82/min, and respirations are 12/min. On physical exam, his lungs are clear to auscultation bilaterally without wheezing. His abdomen is soft and non-distended. He has no tenderness to palpation and bowel sounds are present. Which of the following is the most appropriate next step in management?
Barium swallow
Upper endoscopy
Helicobacter pylori stool antigen test
Trial of lansoprazole
B
Upper endoscopy
This patient presents with a chronic cough that is worse at night, which is consistent with gastroesophageal reflux disease (GERD). In light of this patient’s new weight loss and melena, the most appropriate next step in management would be upper endoscopy to rule out cancer. GERD may present as a chronic or nighttime cough, substernal or epigastric pain, regurgitation, nausea, or bitter taste in the mouth. Symptoms are typically worse after eating or lying down. Patients with typical GERD should undergo upper endoscopy if they have concerning symptoms, including dysphagia, odynophagia, vomiting, weight loss, gastrointestinal bleeding, or melena. They should also undergo upper endoscopy if they are men over the age of 50 with chronic symptoms (>5 years) and cancer risk factors, such as a history of tobacco use or a family history of cancer. Young et al. review the evidence regarding the treatment of GERD. They discuss how patients with concerning symptoms or those who are failing symptomatic treatment should undergo upper endoscopy. They recommend treatment with a proton pump inhibitor in patients without alarm symptoms. Incorrect Answers: Answer A: A barium swallow would be indicated in a patient who presented with symptoms of esophageal dysphagia. Possible etiologies would include Zenker diverticulum or achalasia. This patient denied any difficulty swallowing or feeling of food being stuck in the throat. Answer B: Colonoscopy could be considered in this patient if his upper endoscopy is unrevealing, as weight loss and melena could be explained by colorectal cancer. In light of this patient’s GERD, upper endoscopy should be performed first. Answer C: Helicobacter pylori testing can be useful in the workup of gastric and duodenal ulcers but is not usually performed in the evaluation of GERD. Weight loss and melena raise concerns for carcinoma. Treatment of patients with H. pylori includes a proton pump inhibitor, clarithromycin, and metronidazole. Answer D: A trial of lansoprazole would not be appropriate for this patient presenting with several worrisome symptoms including melena and weight loss. If the patient had presented with typical GERD without these symptoms, a trial of a PPI would be indicated. Cancer should be ruled out first prior to symptomatic treatment. Bullet Summary: Patients with gastroesophageal reflux disease who present with alarm symptoms of weight loss, dysphagia, odynophagia, vomiting, gastrointestinal bleeding, or melena should be evaluated with upper endoscopy.
nan
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A 33-year-old woman presents to the emergency department with foul-smelling vaginal discharge. She states that it started 1 day ago and has not improved with hygiene products. She is sexually active and uses condoms. She does not complain of any abdominal or pelvic pain but states she has some mild burning when urinating. Her temperature is 97.6°F (36.4°C), blood pressure is 111/74 mmHg, pulse is 81/min, respirations are 12/min, and oxygen saturation is 98% on room air. Physical exam is notable for thick, white discharge from the cervix. There is no cervical motion or adnexal tenderness or masses. Some whitish discharge is noted from the urethra. A urine pregnancy test is negative. Which of the following is the most appropriate treatment for this patient?
Azithromycin
Ceftriaxone
Ceftriaxone and azithromycin
Ceftriaxone and doxycycline
D
Ceftriaxone and doxycycline
This nonpregnant woman is presenting with a thick, white cervical discharge, dysuria, and urethral discharge without cervical motion tenderness or adnexal tenderness which is concerning for urethritis without pelvic inflammatory disease. She should be treated with ceftriaxone and doxycycline to cover for the most common organisms. The most common bacterial sexually transmitted infections include Neisseria gonorrhoeae and Chlamydia trachomatis. They can cause urethritis, cervicitis, and pelvic inflammatory disease. The diagnosis is made clinically with the appropriate history (vaginal discharge, high-risk sexual behavior) and physical exam (purulent cervical discharge +/- cervical motion tenderness, adnexal tenderness). The diagnosis should be confirmed with PCR; however, this should not delay treatment. Treatment should be given empirically, and the preferred regimen is ceftriaxone and doxycycline in men and nonpregnant women. In pregnant women, ceftriaxone and azithromycin may be used. Metronidazole is added empirically to cover for Trichomonas vaginalis. Jennings and Krywko review pelvic inflammatory disease. They note that pelvic inflammatory disease is an inflammation of the upper genital tract due to an infection that can affect the uterus, fallopian tubes, and ovaries. They discuss the need for treatment and complications that can occur without treatment. They recommend timely diagnosis and treatment. Incorrect Answers: Answer A: Azithromycin is only indicated to cover chlamydia in pregnancy since doxycycline cannot be given to pregnant patients. Coverage for gonorrhea is also mandatory given this patient’s symptoms. Answer B: Ceftriaxone is insufficient coverage for chlamydia or gonorrhea as chlamydia requires treatment with doxycycline, and gonorrhea requires double coverage from ceftriaxone in addition to doxycycline. Answer C: Ceftriaxone and azithromycin is the preferred regimen to treat chlamydia and gonorrhea in pregnant patients since pregnant patients cannot receive doxycycline. Answer E: Metronidazole is an appropriate treatment for bacterial vaginosis which presents with a fishy odor, white/gray vaginal discharge, and positive clue cells on wet mount. Bullet Summary: The treatment of choice for urethritis is ceftriaxone and doxycycline in nonpregnant patients.
Metronidazole
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A 26-year-old medical student presents to occupational health after sustaining a needlestick injury. She was drawing blood from an HIV-positive patient when she stuck herself percutaneously while capping the needle. She immediately washed the puncture wound with saline. The medical student has had a negative HIV serology from the beginning of medical school 2 years ago. She is monogamous with one male partner and denies any intravenous drug use. The source patient was recently diagnosed with HIV and has a CD4 count of 550 cells/µL. His most recent viral load is 1,800,000 copies/mL, and he was started on HAART 3 days ago. Which of the following is the most appropriate next step in management?
Repeat HIV serology and initiate triple antiretroviral therapy if negative
Immediately initiate triple antiretroviral therapy
Repeat HIV serology and initiate triple antiretroviral therapy if positive
Repeat HIV serology and initiate triple antiretroviral therapy immediately
D
Repeat HIV serology and initiate triple antiretroviral therapy immediately
This medical student has sustained a hollow-bore needlestick injury. The most appropriate next step is to repeat HIV serology and immediately initiate triple antiretroviral therapy with tenofovir, emtricitabine, and raltegravir. Healthcare exposures to HIV are divided into 2 categories based on the level of contact with the source patient. If the healthcare worker’s mucous membrane or nonintact skin comes into contact with an HIV-positive patient’s blood, semen, or vaginal secretions, immediate prophylaxis is recommended. This category also includes all needlestick exposures. If the healthcare worker’s mucous membrane or nonintact skin comes into contact with an HIV-positive patient’s urine, feces, or bodily secretions, prophylaxis is not recommended. If the source patient’s HIV status is unknown, it should be assumed that the patient is HIV positive until testing proves otherwise. The triple antiretroviral drug combination of tenofovir, emtricitabine, and raltegravir is favored due to the low incidence of side effects. Prophylactic treatment should be initiated immediately (ideally within 1-2 hours) and continued for 4 weeks at which time the healthcare worker’s serology should be repeated. Parikh et al. studied technologies for monitoring HIV infection. They discuss how high throughput methods are required in order to surveil for infection on a population level. They recommend using next-generation sequencing methods to improve surveillance methods. Incorrect Answers: Answer A: Immediately initiating triple antiretroviral therapy is appropriate after drawing the medical student’s HIV serology. The patient’s serology at the time of exposure must be established to compare to future testing. Despite her previous negative serology and low-risk lifestyle, it would still be best to repeat the testing at the same time prophylactic treatment is initiated. Answer B: Perform genotype testing on source patient and initiate antiretroviral therapy tailored to results is incorrect because this option would delay the initiation of prophylaxis. Empiric treatment with a triple antiretroviral drug combination is appropriate for all patients with high-risk exposure. Answer C: Waiting to hear that the medical student’s HIV serology is negative would delay the initiation of prophylaxis. Triple antiretroviral drug treatment should be initiated without waiting for the results. Empiric treatment of all patients with high-risk exposure is recommended in all cases. Answer D: Waiting for the results of the medical student’s serology would delay treatment for her exposure. If her serology is already positive, she will need to be treated past the 4-week period of prophylaxis. Even though she does not engage in high-risk behaviors, a positive serology would demonstrate that she was already infected with HIV. Bullet Summary: After a needlestick injury from a HIV-positive source patient, a healthcare worker’s HIV serology should be drawn and they should be immediately initiated on triple antiretroviral drug therapy.
nan
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A 6-month-old boy is brought to the physician by his mother for abnormal movements. Several times a day, he experiences paroxysmal episodes of crying, bending at the waist, and jerking of the arms and legs. These episodes last 1-2 seconds, but occur in clusters every 20-30 seconds for several minutes. His mother's pregnancy, labor, delivery, and his neonatal course have been unremarkable. He is rolling over less frequently than before, has worse head control, and no longer smiles socially. His temperature is 98.6°F (37°C), blood pressure is 92/46 mmHg, pulse is 115/min, and respirations are 34/min. On examination, no dysmorphic features are noted. A 24-hour electroencephalography (EEG) is performed, during which the patient has one of these spells. A chaotic pattern of slowing and multifocal epileptiform charges is seen. Which of the following is the pathophysiology of this patient's condition?
Abnormalities in T-type calcium channels
Anti-N-methyl-D-aspartate (NMDA) receptor antibodies
Decreased gamma aminobutyric acid (GABA) levels
Excess of corticotropin releasing hormone
D
Excess of corticotropin releasing hormone
This patient with infantile spasms (bending at the waist and jerking out the arms and legs), developmental regression (rolling over less frequently, worsening head control, loss of social smile), and hypsarrhythmia on video-EEG (slowing and multifocal epileptiform discharges) are characteristic of West syndrome. West syndrome is caused in part by an excess of corticotropin-releasing hormone which leads to increased neuronal excitability. The pathogenesis of West syndrome is incompletely understood. Infantile spasms are often idiopathic, but may also be secondary to tuberous sclerosis, hypoxic-ischemic encephalopathy, congenital brain malformations, aminoacidopathies, and organic acidopathies. Brain insults may activate a neuroendocrine stress response, which in turn leads to abnormalities of the hypothalamic-pituitary-adrenal axis. Disturbed immune activation may also play a role. Patients will present with infantile spasms, developmental regression, and slowing and multifocal epileptiform discharges known as hypsarrhythmia. Many affected infants are responsive to ACTH therapy, with cessation of spasms and resolution of hypsarrhythmia in many cases. D’Alonzo et al. review the epidemiology, clinical presentation, pathophysiology, and treatment of West syndrome. They discuss how patients will present with the classic symptoms of spasms and regression. They recommend early diagnosis of this syndrome so that patients can be treated appropriately. Incorrect Answers: Answer B: Abnormalities in T-type calcium channels may contribute to absence seizures. Absence seizures are generalized seizures that involve brief, sudden lapses of consciousness and are more common in children. This child’s developmental regression, infantile spasms, and EEG findings are more consistent with West syndrome. Answer C: Anti-N-methyl-D-aspartate (NMDA) receptor antibodies may cause anti-NMDA encephalitis. Anti-NMDA encephalitis presents with seizures, psychiatric manifestations, and decreased level of consciousness. Hypsarrhythmia is characteristic of West syndrome and is not consistent with anti-NMDA encephalitis. Answer D: Decreased gamma-aminobutyric acid (GABA) levels were once theorized to be the basis of epilepsy syndromes. This theory is less accepted today. Moreover, West syndrome is likely not mediated by disturbances in GABA. Nonetheless, vigabatrin, which in part works by inhibiting GABA metabolism, is an effective medication for patients with West syndrome. Answer E: Vitamin B6 deficiency has not been documented in West syndrome. Some studies have suggested vitamin B6 may be of some benefit in the treatment of infantile spasms; however, there is no evidence that response rates to B6 are different from the spontaneous remission rates predicted from natural history data. Therefore, the pathophysiology of West syndrome is more likely CRH-excess mediated rather than due to a deficiency of vitamin B6. Bullet Summary: West syndrome, which may be caused by CRH excess, presents with hypsarrhythmia, developmental regression, and infantile spasms.
Vitamin B6 deficiency
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An 8-year-old boy is brought to the emergency department by his parents for evaluation. He has had headache and fever for the last 2 days, and this morning he was confused and had difficulty answering questions. He also developed a rash this morning. He recently returned from summer camp. He has no significant medical history and takes no medications. His temperature is 104°F (40°C), pulse is 120/min, blood pressure is 105/60 mmHg, and respirations are 22/min. On exam, flexion of the neck causes flexion of the hips and knees. Fundoscopic exam reveals no papilledema. Examination of the patient's rash is shown in Figure A. Which of the following is the most appropriate next step in management?
Lumbar puncture, ceftriaxone, vancomycin, and methylprednisolone
MRI brain
Vancomycin and ceftriaxone
Vancomycin, ceftriaxone, and dexamethasone
A
Lumbar puncture, ceftriaxone, vancomycin, and methylprednisolone
This patient with fever, headache, signs of meningeal irritation, and a purpuric rash likely has bacterial meningitis. The most appropriate initial step in management is a lumbar puncture as well as ceftriaxone, vancomycin, and methylprednisolone. Meningitis classically presents with fever, neck stiffness or headache, and altered mental status. Specific physical exam findings that can suggest the diagnosis include meningeal signs such as a positive Kernig sign (inability to extend the knee while the hip is flexed) and Brudzinski sign (flexion of the neck results in flexion of lower extremities). Common causative agents include Streptococcus pneumoniae and Neisseria meningitidis. The latter of which is associated with the purpuric rash seen in this patient. The best initial step in management is a lumbar puncture followed promptly by IV broad spectrum antibiotics. Once the diagnosis/infectious agent is confirmed, the antibiotic agents can be refined. Other early steps in management include early blood cultures and fluid resuscitation. Steroids should be given in the setting of suspected Waterhouse-Friderichsen syndrome and concern for adrenal failure. Linder et. al review meningococcal meningitis. They discuss the pathophysiology, clinical manifestations, and diagnosis. They describe the association of purpuric rash with meningococcal infection, and note that this is indicative of disseminated intravascular coagulation. Figure A. demonstrates a purpuric rash over the lower extremities, which is classically associated with meningococcal meningitis. However, this can occur with meningitis or bacteremia from other infectious agents, as it is indicative of disseminated intravascular coagulation. Incorrect Answers: Answer A: Blood and urine cultures would be appropriate initial management for a fever of unknown origin and in this patient. Early antibiotics must be given as well to reduce mortality and improve outcomes. Answer C: MRI brain would not be appropriate in this setting. While CNS imaging is sometimes indicated prior to lumbar puncture if signs of increased intracranial pressure such as papilledema are present, CT imaging is typically preferred. Time required to obtain MR imaging is prohibitive and would delay definitive care for this patient. Answer D: Vancomycin and ceftriaxone are an appropriate initial antibiotic regimen for meningitis in children and adults. However, steroids are often added empirically. Lumbar puncture should be performed prior to administration of antibiotics (if possible) to avoid sterilization of the CSF. Answer E: Vancomycin, ceftriaxone, and prednisone are an appropriate initial regimen for meningitis in adults. Steroids have been shown to reduce inflammation and prevent neurologic sequelae. However, lumbar puncture should be performed prior to administration of antibiotics if possible to avoid sterilization of the CSF. Also, IV steroids are preferred rather than oral in ill patients. Bullet Summary: The best initial step in management for meningitis is a lumbar puncture followed by broad-spectrum antibiotics and steroids.
nan
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A 57-year-old man presents to the emergency department for shortness of breath. He was riding the train home from a business conference when his symptoms began. The patient has a medical history of obesity, diabetes, diabetic nephropathy, hypertension, and a 40-pack-year smoking history. His current medications include atorvastatin, lisinopril, insulin, metformin, and nicotine gum. His temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 120/min, respirations are 15/min, and oxygen saturation is 93% on room air. An ECG is ordered as seen in Figure A. A chest radiograph is ordered as seen in Figure B. Laboratory values are ordered as seen below: Serum: Na+: 137 mEq/L Cl-: 105 mEq/L K+: 4.1 mEq/L HCO3-: 24 mEq/L BUN: 22 mg/dL Glucose: 129 mg/dL Creatinine: 1.2 mg/dL Ca2+: 10.1 mg/dL AST: 11 U/L ALT: 12 U/L On physical exam, the patient appears to be in distress. His cardiac exam is notable for tachycardia. Pulmonary exam reveals bilateral air movement without any wheezes or crackles. The patient's right lower extremity appears mildly inflamed, and palpation of the calf elicits pain. Which of the following is the most appropriate next step in management?
Aspirin
Cardiac troponins
Heparin
Ventilation perfusion scan
C
Heparin
This patient is presenting with a score of 7.5 on Wells criteria suggesting a diagnosis of pulmonary embolism. The most appropriate initial step in management is heparin administration. Patients with suspected pulmonary embolism (PE) can be evaluated with Wells criteria which consists of: clinical signs and symptoms of DVT (3 points), PE is the most likely diagnosis (3 points), pulse > 100 (1.5 points), immobilization for 3 days, or surgery within 4 weeks (1.5 points), history of DVT or PE (1.5 points), hemoptysis (1 point), and malignancy (1 point). Scores higher than 3 suggest a high probability of PE. Patients will often present with shortness of breath, with ECG findings of sinus tachycardia, and a chest radiograph that is typically normal. With a high pre-test probability, the most important initial step in management is anticoagulation with heparin versus further diagnostic workup. Doherty reviews the evidence regarding the diagnosis and treatment of pulmonary embolism. He discusses how clinical criteria such as Wells criteria can be helpful in assessing the likelihood of a PE. He recommends immediate treatment in patients who have a PE. Figure/Illustration A is an ECG demonstrating sinus tachycardia with a P wave, a QRS complex, and a T wave during every beat (red circle). This is the most common ECG presentation in PE, though right heart strain can also be seen. Figure B is a chest radiograph that is within normal limits without evidence of fluid in the dependent portions of the lung (red circles). This is the most common radiological presentation in PE. Incorrect Answers: Answer A: Aspirin would be the most appropriate initial step in the management of MI and can reduce mortality by up to 25% if administered promptly. PE is a more likely diagnosis in this patient given the ECG that only demonstrates sinus tachycardia. Answer B: Cardiac troponins could be used to confirm the diagnosis of myocardial infarction (MI). In the presence of this patient's ECG (which does not demonstrate ST elevation), MI is a less likely diagnosis. Treatment would be with aspirin in patients who had an MI followed by emergent revascularization. Answer C: A CT scan of the chest without contrast would not confirm the diagnosis in this patient; however, a CT angiogram would be the most accurate test. It is more important to begin treatment with heparin when there is a very high clinical suspicion of PE. Answer E: A ventilation perfusion scan is the most accurate test for PE in a patient who cannot tolerate contrast. This test has a low specificity. Bullet Summary: The most appropriate initial step in the management of a pulmonary embolism in patients with a high pre-test probability is anticoagulation with heparin.
nan
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A 69-year-old man presents to his primary care physician for a general checkup. Overall he is doing well. Since he retired, he has been working on projects at home and taking time to exercise every day. He eats a balanced diet and has been spending time with his wife every evening. Despite this, the patient claims that he feels less well-rested when he wakes up in the morning. The patient states that he used to sleep 9 hours a night in his youth and felt excellent. Now he sleeps 7 hours a night and doesn’t feel as well rested as he used to. The patient’s wife states that he seems to sleep peacefully. His medical history is significant for hypertension and diabetes for which he takes lisinopril and metformin. His temperature is 98.6°F (37°C), blood pressure is 131/85 mmHg, pulse is 71/min, and respirations are 12/min. His neurological exam is within normal limits. The patient is muscular and has a healthy weight with a pleasant demeanor. He denies feeling fatigued or tired currently. Which of the following physiological changes would most likely be seen in this patient?
Increased dopamine
Decreased orexin
Decreased melatonin
Increased norepinephrine
C
Decreased melatonin
This elderly patient is presenting with sleep that is sufficient but less refreshing than sleep was for him in his past suggesting a diagnosis of normal aging. Melatonin levels naturally decrease as patients age. Normal aging shortens most phases of sleep. It is normal for melatonin levels to decline during normal aging; however, this change is not responsible for all the symptoms associated with declining quality of sleep. Other changes that occur include decreased REM sleep, decreased stage N3 sleep, and a compensatory increase in stage N2 sleep. Patients will often complain of sleep that is less refreshing than it was in their youth, yet they are still functional and not excessively tired. Treatment is with sleep hygiene though some patients will also benefit from treatment with melatonin supplementation. Cardinali reviews the role of melatonin in aging. He discusses how natural levels of melatonin decrease in aging animals, which may have implications on the circadian clock. He recommends studies on whether melatonin supplementation may be beneficial to mitigate the effects of aging. Incorrect Answers: Answer B: Decreased orexin levels would be seen in patients with narcolepsy, which presents with sudden episodes of falling asleep. This tends to occur in younger patients and is also associated with cataplexy or sudden loss in muscle tone. Treatment of this disorder is with stimulant medications such as modafinil. Answer C: Increased acetylcholine levels would not be seen in patients as they age and would not explain the changes this patient is experiencing. Rather, acetylcholine levels tend to decline as we age. Drinking caffeinated drinks is associated with increased acetylcholine levels in the brain. Answer D: Increased dopamine would not be a principle change seen in age-related sleep changes. Higher dopamine levels would increase feelings of being awake as well as alertness. In contrast, decreased dopamine levels can be seen in patients with Parkinson disease, which would present with bradykinesia and a resting tremor. Answer E: Increased norepinephrine would result in increased wakefulness and potentially insomnia; however, it would not explain the changes seen in normal aging. This chemical is naturally produced during the fight or flight response and can be artificially increased by stimulant drugs such as cocaine. Bullet Summary: Normal aging results in a shortening of most phases of sleep as well as decreased melatonin levels and presents with sleep that is subjectively less restful.
nan
https://step2.medbullets.com/testview?qid=109241
A 3-day-old boy is evaluated in the hospital nursery for the delayed passage of meconium. The patient is breastfed, has been feeding well every 2 hours, and is urinating over 8 times per day. He was born at 35 weeks gestation to a 27-year-old G4P4 mother. The patient is of Ashkenazi Jewish descent and the patient’s parents refused all prenatal genetic testing. The pregnancy was uncomplicated until the patient’s mother had a spontaneous rupture of membranes at 35 weeks of gestation. The patient’s 3 older siblings are all healthy. The patient’s temperature is 98.2°F (36.8°C), blood pressure is 48/32 mmHg, pulse is 164/min, and respirations are 48/min. On physical exam, he appears to be in moderate distress. He has no dysmorphic features, and his abdomen is distended and non-tender. Bowel sounds are absent. Which of the following is the most appropriate next step in management?
Abdominal CT
Abdominal radiograph
Contrast enema
Sweat testing
B
Abdominal radiograph
Delayed passage of meconium should be evaluated with an abdominal radiograph to rule out bowel perforation. Patients with a perforation require emergency surgery. All neonates should pass meconium in the first 48 hours of life. Patients that fail do to so should be evaluated with an abdominal radiograph to exclude the possibility of bowel perforation. If free air is seen on the abdominal radiograph, patients should be taken for emergent surgery to repair the perforation. If bowel perforation is ruled out, a workup should be done to determine the etiology of the delayed passage, which would most likely include a contrast enema to differentiate between meconium ileus and Hirschsprung disease. Treatment is with meconium removal or excision of the defective segment respectively. Adams and Stanton review the evidence regarding the diagnosis and treatment of neonatal bowel obstruction. They discuss how malrotation, jejunoileal atresia, duodenal atresia, and colonic atresia are common causes of this finding. They recommend surgical management of these conditions. Incorrect Answers: Answer A: An abdominal radiograph would be faster than an abdominal CT to rule out an indication for emergency surgery. The use of CT should also be restricted in the pediatric population due to the radiation exposure involved. Adult patients with unclear radiographic findings can be further assessed using a contrast CT scan. Answer C: Contrast enema is the test of choice to differentiate meconium ileus from Hirschsprung disease, but the most important first step is to rule out bowel perforation. Patients who do not have perforation can then undergo this test to determine the etiology of failure to pass meconium. Answer D: Sweat testing would aid in the diagnosis of cystic fibrosis, particularly in this patient who has had no prenatal screening. The first step in management should be an abdominal radiograph to rule out bowel perforation. Cystic fibrosis can be treated with pancreatic enzyme replacement and pulmonary therapy. Answer E: Upper gastrointestinal series is used to make the diagnosis of intestinal malrotation, which more commonly presents with bilious vomiting secondary to midgut volvulus rather than the delayed passage of meconium. Treatment is with surgical derotation. Bullet Summary: Neonates who fail to pass meconium in the first 48 hours of life should be urgently evaluated with an abdominal radiograph to rule out bowel perforation.
Upper gastrointestinal series
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A 67-year-old man presents to the emergency room with a 2 day history of progressively worsening fatigue and shortness of breath. His past medical history is significant for previous myocardial infarction, a 30-pack-year smoking history, gastroesophageal reflux disease, and poorly controlled hypertension. The patient’s only home medication is omeprazole. His temperature is 98.6°F (37°C), blood pressure is 140/90 mmHg, pulse is 90/min, respirations are 30/min, and oxygen saturation is 88% on room air. Physical exam is significant for a jugular venous pressure of 15 cm, an S3 heart sound, bibasilar crackles, and 2+ pitting edema to the knees bilaterally. His abdomen is soft and non-tender. His neurological exam is nonfocal and he walks with a steady gait. An arterial blood gas shows the following: pH: 7.56 (normal 7.35-7.45) pCO2: 25 mmHg (normal 35-45 mmHg) HCO3: 29 mEq/L (normal 22-26 mEq/L) pO2: 62 mmHg (normal 80-100 mmHg) SaO2: 87% (normal 95-100%) Which of the following is the most appropriate treatment?
Furosemide
Ivabradine
Metoprolol
Sacubitril and valsartan
A
Furosemide
This patient with multiple risk factors for heart failure (previous myocardial infarction, smoking history, hypertension), worsening fatigue, shortness of breath, respiratory alkalosis, and signs of fluid overload (S3 heart sound, elevated jugular venous pressure (JVP), bibasilar crackles, peripheral edema) most likely has acute decompensated heart failure (ADHF). The most appropriate initial management for patients in ADHF is a loop diuretic such as furosemide. Heart failure (HF) is a condition in which the heart does not pump effectively and can be due to either difficulty with relaxation in diastole or contraction in systole or a combination of both. Risk factors for this condition include myocardial infarction, diabetes mellitus, valvular disease, cigarette smoking, hypertension, and obesity. Left-sided HF leads to decreased forward flow into the systemic circulation and increased congestion in the pulmonary circulation which clinically manifests as shortness of breath, pulmonary edema, and crackles on lung exam. Early pulmonary edema leads to a ventilation/perfusion (V/Q) mismatch which causes hypoxia. Hypoxia leads to a compensatory increase in the respiratory rate, which leads to hypocapnia and respiratory alkalosis. The most appropriate initial treatment for patients in ADHF is a loop diuretic such as furosemide, which helps alleviate fluid overload. Other treatments for acute exacerbations may include nitroglycerin (in hypertensive patients) and BIPAP. Yancy et al. discuss the most recent ACC/AHA guidelines for the management of heart failure. They review current testing and treatment regimens. Acute episodes may be managed with loop diuretics to reduce volume overload and improve symptoms. Incorrect Answers: Answer B: Ivabradine inhibits the funny current, an electrical current that leads to spontaneous depolarization in pacemaker cells, leading to decreased heart rate. It reduces the risk of hospitalization in patients with chronic heart failure with reduced ejection fraction (HFrEF). However, it should not be started in ADHF due to lack of mortality benefit and should be held in ADHF patients with severe decompensation due to decreased heart rate leading to decreased cardiac output. Answer C: Metoprolol is a beta-blocker that reduces mortality when used in patients with chronic HFrEF. However, beta-blockers can worsen decompensated HFrEF due to negative inotropy and should not be started in ADHF. Answer D: Sacubitril and valsartan is a combination angiotensin receptor-neprilysin inhibitor (ARNI) that improves mortality in patients with chronic HFrEF. ARNIs, angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs) should not be started in ADHF due to concern for hypotension and worsening renal function. However, either an ARNI, ACE inhibitor or ARB should be started prior to discharge. Answer E: Spironolactone is a mineralocorticoid receptor antagonist that reduces mortality in chronic HFrEF. However, high-dose spironolactone does not improve outcomes as first-line therapy in ADHF (Anstrom et al. JAMA Cardiology 2017). Bullet Summary: Patients with acute decompensated heart failure present with fatigue, dyspnea, bibasilar crackles, respiratory alkalosis on arterial blood gas, edema, and other signs of fluid overload.
Spironolactone
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A 68-year-old woman presents to her primary care provider for dizziness. She often feels lightheaded and like she may “pass out” but never actually loses consciousness. She denies dyspnea on exertion or orthopnea. The patient had a myocardial infarction 6 months ago, and she now has multiple drug-eluting stents. Her ECG upon discharge from that hospitalization was normal. The patient has been taking all of her medications as instructed. Her medical history is otherwise significant for rheumatoid arthritis and migraines. Her home medications include methotrexate, atorvastatin, clopidogrel, and aspirin. Her temperature is 98.6°F (37°C), blood pressure is 118/75 mmHg, pulse is 92/min, and respirations are 12/min. On physical exam, she has an irregular heartbeat with normal cardiac sounds. Her abdomen is non-distended and she has mild lower extremity edema. Her current ECG can be seen in Figure A. Which component of the cardiac conduction system is most likely affected in this patient?
Atrioventricular node
Bundle branches
Bundle of His
Purkinje fibers
A
Atrioventricular node
This patient who presents with lightheadedness and is found to have progressive prolongation of the PR interval on ECG most likely has Mobitz type 1 (Wenckebach) 2nd-degree heart block. Mobitz type 1 heart block is usually caused by atrioventricular node dysfunction. Second degree heart block can be divided into Mobitz type 1 and Mobitz type 2 patterns. The Mobitz type 1 pattern is usually a result of dysfunction at the atrioventricular (AV) node, whereas the Mobitz type 2 pattern is usually caused by dysfunction below the AV node in the His-Purkinje system. In Mobitz type 1 2nd-degree heart block, the PR interval becomes progressively longer with each beat before the dropped QRS complex. In Mobitz type 2 2nd-degree heart block, the PR intervals are consistent in length and the dropped QRS complexes occur at regular intervals. The easiest way to determine whether the PR interval is getting longer is to compare the PR interval just before the dropped beat to the PR interval immediately following the dropped beat. Patients with Mobitz type 1 blocks generally do not require treatment unless symptomatic whereas those with Mobitz type 2 blocks generally require pacemaker placement. Clark and Prystowsky review the pathophysiology of heart block. They discuss how a type 1 2nd-degree heart block is caused by dysfunction of the atrioventricular node. They recommend symptomatic treatment of this condition. Figure/Illustration A is an ECG that demonstrates the progressive prolongation of the PR interval (blue lines) until a beat is dropped. This progressive prolongation is characteristically seen in Mobitz type 1 (Wenckebach) heart block. Incorrect Answers: Answer B: A bundle branch block would produce a classic right or left bundle branch block pattern on ECG (RSR') described colloquially as "bunny ears." Bundle branch blocks are defined in part by a QRS duration > 120 ms and can be seen in lead V1. Treatment is with control of underlying risk factors such as hypertension. Answer C: The bundle of His conducts the electrical impulse from the atrioventricular node to the ventricles. Dysfunction at the level of the bundle of His would more likely cause Mobitz type 2 2nd-degree heart block or 3rd-degree heart block rather than the Mobitz type 1 heart block seen in this patient. Treatment is with pacemaker placement. Answer D: Purkinje fibers rapidly conduct along the ventricular walls to create synchronized contraction of the ventricles. Aberrant conduction of the Purkinje fibers leads to premature ventricular complexes. Treatment is with beta-blockers if patients are symptomatic. Answer E: The sinoatrial node is the heart’s primary pacemaker. Dysfunction at this level can lead to sick sinus syndrome. Sick sinus syndrome can manifest as many types of arrhythmias, including bradycardia-tachycardia syndrome, in which there is alternating bradycardia with paroxysmal tachycardia. Sinoatrial node dysfunction can be treated with pacemaker placement. Bullet Summary: Mobitz type 1 (Wenckebach) 2nd-degree heart block is characterized by progressive prolongation of the PR interval before a dropped QRS complex and is usually caused by dysfunction of the atrioventricular node.
Sinoatrial node
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A 7-year-old boy is brought to the emergency department by his parents for worsening symptoms of previously diagnosed acute sinusitis. Initially, the pediatrician prescribed decongestants and sent the patient home. Since then, the patient has developed a nasal discharge with worsening pain. The patient has a medical history of asthma which is well-controlled with albuterol. His temperature is 99.5°F (37.5°C), blood pressure is 90/48 mmHg, pulse is 124/min, respirations are 17/min, and oxygen saturation is 98% on room air. The cardiopulmonary exam is within normal limits. Inspection of the patient's nose reveals a unilateral purulent discharge mixed with blood. The rest of the patient's exam is within normal limits. Which of the following is the most likely diagnosis?
Foreign body obstruction
Nasopharyngeal carcinoma
Bleeding and infected vessel of Kiesselbach plexus
Septal perforation
A
Foreign body obstruction
This patient presents with unilateral bloody/purulent discharge from the nose suggesting a diagnosis of a nasal foreign body. The most common cause of unilateral nasal symptoms in a pediatric patient is a nasal foreign body. Nasal foreign bodies tend to be unilateral (though bilateral symptoms are plausible) and present with a purulent and bloody discharge. The treatment of choice is to remove the object which may be done in the emergency department or operating room depending on how deep the object is. Visualization of the object should be attempted when possible in order to allow for safe retrieval. Krulewitz and Fix review the evidence regarding the diagnosis and treatment of patients with epistaxis. They discuss how foreign body insertion is a major risk factor for the development of epistaxis. They recommend assessment of airway and breathing in the setting of severe epistaxis. Incorrect Answers: Answer A: A bleeding and infected vessel of the Kiesselbach plexus could suggest a complicated case of epistaxis. The persistent, unilateral symptoms in this patient point toward a different diagnosis. A high index of suspicion for foreign bodies should be suspected in children. Answer C: Nasopharyngeal carcinoma tends to occur in young male patients and can be associated with infectious mononucleosis. Epistaxis can be a symptom and the mass can be visualized. This disease tends to present in older patients and is far less common than foreign bodies. Treatment is with surgical resection. Answer D: Septal perforation can occur secondary to repeat cocaine use but would not present with unilateral nasal discharge in a pediatric patient. It would be highly unlikely in a pediatric patient with no history of drug use. Treatment is with cessation of the offending agent. Answer E: Sinusitis with bacterial superinfection would typically present with systemic symptoms as well as bilateral symptoms. Though this diagnosis is possible, it is less likely than a foreign body. Treatment is with systemic antibiotic therapy. Bullet Summary: A nasal foreign body presents with unilateral bloody/purulent nasal discharge.
nan
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A 23-year-old woman presents to the emergency department with severe abdominal pain. The pain has been dull and progressive, but became suddenly worse while she was exercising. Her medical history is notable for depression, anxiety, and gonococcal urethritis that was appropriately treated. She is sexually active and does not use condoms. She admits to drinking at least 5 standard alcoholic drinks a day. The patient also recently lost a large amount of weight for a fitness show she planned on entering. The patient's current medications include oral contraceptive pills, fluoxetine, alprazolam, ibuprofen, acetaminophen, and folate. Her temperature is 99.5°F (37.5°C), blood pressure is 80/40 mmHg, pulse is 110/minute, and respirations are 15/minute with an oxygen saturation of 96% on room air. On physical exam, you note an athletic young woman with burly shoulders, a thick neck, and acne on her forehead and back. On abdominal exam you note diffuse tenderness with 10/10 pain upon palpation of the right upper quadrant. Intravenous fluids are started and labs are sent. A urinary ß-hCG has been ordered. Which of the following is the most likely the diagnosis?
Ectopic implantation of a blastocyst
Inflammation of the pancreas
Obstruction of blood flow through the hepatic vein
Obstruction of the common bile duct by radio-opaque stones
E
Vascular ectasia within the liver
This patient is presenting with constant right upper quadrant pain in the setting of oral contraceptives (OCP) and likely anabolic steroid use, suggesting a diagnosis of a ruptured hepatic adenoma (HA). The pathophysiology of HA is vascular ectasia from exposure to OCPs and androgens. A hepatic adenoma typically occurs secondary to exposure to OCPs and androgens (anabolic steroids) causing a generalized vascular ectasia. Both of these drugs should be discontinued in patients with hepatic adenoma. Symptoms include right upper quadrant abdominal pain. Sudden worsening of pain with unstable vitals suggests a ruptured hepatic adenoma. Patients should be managed with fluids and an ultrasound should be performed to support the diagnosis. Additional imaging tests include an abdominal contrast-enhanced CT to better characterize the pathologic anatomy in this disease. The definitive treatment in this disease is surgery in order to remove the hepatic adenoma. In cases of a ruptured hepatic adenomas, patients require transfusion and operative management. Bioulac-Sage et al. present the current data that are available for classifying hepatic adenomas. They discuss how many of these lesions involve alterations of the HNF1A gene, the JAK/STAT3 pathway, and the CTNNB1 gene. They recommend performing molecular analysis in order to better understand which lesions are likely to undergo malignant transformation. Incorrect Answers: Answer A: Ectopic implantation of a blastocyst (ectopic pregnancy) that has ruptured could present similarly to what this patient is experiencing. Symptoms would include lower abdominal pain and unstable vitals. However, this patient's pain is more pronounced in the right upper quadrant and is occurring in the setting of OCP and possible anabolic steroid use. Answer B: Inflammation of the pancreas represents a diagnosis of pancreatitis. This would present with epigastric pain after a fatty meal or alcohol consumption. Though this patient does drink more than recommended and should be counseled to reduce her consumption, the pain began after exercising at the gym and there are no other symptoms of pancreatitis. Answer C: Obstruction of blood flow through the hepatic vein causes Budd-Chiari syndrome, which presents with a non-specific triad of abdominal pain, ascites, and hepatomegaly in the setting of a hypercoagulable state (factor V Leiden, polycythemia vera, OCP use, and paroxysmal nocturnal hemoglobinuria). Though this patient has risk factors for this pathology, they do not have any classic symptoms. Answer D: Obstruction of the common bile duct with radio-opaque stones would present with colicky right upper quadrant pain in a patient that is "fat, female, fertile, and forty." Though the pain presented in this case is localized to the right upper quadrant, it is not colicky and there is not a positive Murphy sign described. OCP's are a risk factor for cholesterol stones as are rapid weight loss, both of which this patient has. This is a strong possibility on the differential; however, the symptoms presented suggest a diagnosis of ruptured hepatic adenoma given the demographics of the patient. Bullet Summary: A hepatic adenoma presents with right upper quadrant pain in the setting of OCP and/or androgen use and can rupture leading to unstable vitals.
Vascular ectasia within the liver
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A 65-year-old man presents to the emergency department with sudden-onset altered mental status. He was last seen normal 1 hour ago. His wife noticed that he was confused, slurring his speech, and had notable weakness that caused him to drop his cup of coffee. The patient is confused and not able to answer any questions. Facial drooping of the left lower face is noted, and the patient cannot follow any commands. He has a past medical history of diabetes and atrial fibrillation and is currently taking warfarin. His other medications are not known. His temperature is 98.0°F (36.7°C), blood pressure is 150/90 mmHg, pulse is 115/min, and respirations are 17/min. The patient is not able to follow any commands for further neurologic evaluation but does not appear to be moving his right upper extremity. In addition to further physical exam, which of the following is the most appropriate next step in management?
Alteplase
CT head
Fingerstick blood glucose
Fresh frozen plasma and vitamin K
C
Fingerstick blood glucose
This patient is presenting with sudden onset confusion, slurred speech, asymmetric smile, and weakness in the setting of risk factors for stroke (atrial fibrillation, diabetes, and hypertension). The most important initial step in management in a patient with altered mental status and neurological deficits is to obtain a fingerstick blood glucose. The workup of stroke is first centered on a thorough history and physical and immediately calculating an NIH stroke scale (11-domain assessment of stroke severity involving patient consciousness level, orientation, ability to follow commands, degree of gaze palsy, degree of vision loss, ataxia, motor drift, sensation, inattention, language/speech) as well as ascertaining when the patient was last known to be neurologically normal. While performing the exam and NIH stroke scale, a fingerstick blood glucose should be performed in all patients as hypoglycemia may present identically to a stroke. Severe hypoglycemia may present with lethargy, confusion, focal or global weakness, nausea, vomiting, agitation, and even seizures. If the patient is hypoglycemic, dextrose can be given which will rapidly correct the patient's underlying deficits. Ohshita et al. review other possible causes of stroke-like symptoms. They note that hypoglycemia may mimic stroke and cause focal neurological signs with common findings being unilateral motor weakness with mild or moderate alteration of consciousness. They noted that all patients improved within 1 hour of glucose injection in this study, thus the recommendation to check a fingerstick blood glucose in all stroke patients. Incorrect Answers: Answer A: Alteplase would be indicated if a patient presented with disabling neurologic deficits from an ischemic stroke within 4.5 hours of symptom onset only after a CT scan of the head has ruled out an intracranial bleed. The patient must have no other contraindications to thrombolytics including but not limited to recent trauma, known intracranial hemorrhage, rapidly improving neurologic deficits (spontaneously), and no other clear reversible cause (among many other contraindications). Answer B: CT head is the most appropriate initial step in the management of an acute stroke; however, a rapid fingerstick blood glucose takes precedence as hypoglycemia can mimic a stroke and be immediately addressed thus removing the need for further workup if there is a complete resolution of symptoms with normoglycemia. While an exam is ongoing, a fingerstick blood glucose is being obtained, and neuroimaging is also often ordered to be performed immediately after. Answer D: Fresh frozen plasma and vitamin K would be indicated in this patient if he had a hemorrhagic stroke (a CT of the head would need to confirm this) to reverse his anticoagulation with warfarin. Factor concentrate can also be used to rapidly reverse anticoagulation with warfarin. Answer E: MRI brain would be indicated in the workup of a stroke or transient ischemic attack after all emergency interventions and labs including CT head, serum chemistries, CBC, and ECG. It delineates the extent of the stroke and gives useful prognostic and diagnostic information. It also may elucidate strokes missed on other forms of neuroimaging such as CT perfusion. Bullet Summary: A fingerstick blood glucose should be obtained immediately in all patients with stroke-like symptoms or altered mental status.
MRI brain
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A 25-year-old man presents to his primary care physician for trouble with focus and concentration. He states that he lives at home with his parents his entire life and recently was able to get a job at a local factory. Ever since the patient started working, he has had trouble focusing on his job. He is unable to stay focused on any task. His boss often observes him "daydreaming" with a blank stare off into space. His boss will have to yell at him to startle him back to work. The patient states that he feels fatigued all the time and sometimes will suddenly fall asleep while operating equipment. He has tried going to bed early for the past month but is unable to fall asleep until 2 hours prior to his alarm. His temperature is 98.6°F (37°C), blood pressure is 112/68 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is within normal limits. The patient fears that if this continues he will lose his job. Which of the following is the most appropriate initial step in management?
Ethosuximide
Zolpidem
Bright light therapy and decreased light at night
Modafinil
C
Bright light therapy and decreased light at night
This patient is presenting with fatigue and an inability to fall asleep until late in the morning suggesting a diagnosis of delayed sleep phase syndrome. The most appropriate initial therapy is light therapy, behavioral therapy, and sleep hygiene practices in addition to decreasing exposure to light at night. Delayed sleep phase syndrome typically presents with individuals who naturally fall asleep very late and wake up very late. This becomes problematic when they attend school or have a job that requires them to wake up early. The most appropriate initial therapy for delayed sleep phase syndrome is behavioral therapy, sleep hygiene practices, and bright light therapy. Bright light therapy involves consistent timed exposure to a light box to simulate natural outdoor light, with the hope of resetting the circadian rhythm and moving the sleep phase earlier. If these measures fail, or if a different diagnosis is suspected, a referral for polysomnography may be indicated. Sletten et al. studied whether melatonin was effective in the treatment of delayed sleep phase syndrome. They found that using this medication as an adjunct to sleep hygiene was effective in improving symptoms. They recommend using multimodal therapy for this disease. Incorrect Answers: Answer A: Ethosuximide is the treatment of choice for absence seizures. Though this patient does have episodes of blank staring, this likely does not represent a seizure as he is arousable to return to work by his boss. Patients with absence seizures typically present in childhood and are unable to be aroused during seizure episodes. Answer B: Polysomnography or a sleep study would be indicated after sleep hygiene practices have failed. Polysomnography could be the most appropriate initial step in management if there was a suspected diagnosis of obstructive sleep apnea or an unknown cause of this patient's fatigue. Patients with sleep apnea typically snore and this disease is associated with obesity and increasing age. Answer D: Modafinil is a treatment for narcolepsy. Though this patient does have sudden episodes of falling asleep while at work, this is more likely to be related to his sleep schedule. Patients with narcolepsy generally do not feel tired immediately before their episodes of sleep. Answer E: Zolpidem is a muscle relaxant and hypnotic with the mechanism of action of a benzodiazepine. Though a drug like zolpidem could be used to help this patient fall asleep, the most appropriate first step is to address the patient's sleep hygiene prior to prescribing a potentially addictive drug. Bullet Summary: The most appropriate initial step in the management of patients with delayed sleep phase syndrome is behavioral therapy, bright light therapy, and sleep hygiene.
nan
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A 3-week-old boy presents to the emergency department with vomiting. His parents report that he suddenly started vomiting this morning and has shown no interest in feeding since then. They describe the vomitus as green and without any traces of blood. Prior to today, the patient was feeding, voiding, and stooling well. He was noted to have surpassed his birth weight at his last office visit 1 week ago. His temperature is 97.6°F (36.4°C), blood pressure is 78/56 mmHg, pulse is 148/min, and respirations are 44/min. On physical exam, the patient is in mild distress. He has no dysmorphic features, and his mucous membranes are dry. His abdomen is soft and distended. Bowel sounds are hypoactive. An abdominal radiograph is performed as in Figure A. Which of the following is the most appropriate next step in the diagnosis of this condition?
Upper gastrointestinal contrast series
Contrast enema
Abdominal CT
Rectal suction biopsy
A
Upper gastrointestinal contrast series
This neonatal patient presents with sudden-onset bilious vomiting and an abdominal radiograph revealing a gasless abdomen, which is consistent with intestinal malrotation complicated by a midgut volvulus. The most appropriate next step in the diagnosis of intestinal malrotation is an upper gastrointestinal series. Malrotation classically presents with bilious vomiting due to the formation of midgut volvulus. These patients will have normal development until the development of the obstruction followed by inability to feed and abdominal distention. Findings of malrotation on an upper gastrointestinal series include an abnormally placed ligament of Treitz on the right side of the abdomen and a "corkscrew" appearance of the distal duodenum. If an upper gastrointestinal contrast series confirms the diagnosis, patients should undergo surgery to reduce the volvulus in order to avoid intestinal perforation or other complications. Adams and Stanton review the evidence regarding the diagnosis and treatment of neonatal bowel obstruction. They discuss how malrotation, jejunoileal atresia, duodenal atresia, and colonic atresia are common causes of this finding. They recommend surgical management of these conditions. Figure/Illustration A is an abdominal radiograph demonstrating the nonspecific finding of a paucity of air in the abdomen (red circle). This is consistent with intestinal malrotation complicated by midgut volvulus. Incorrect Answers: Answer A: Although abdominal CT would detect intestinal malrotation, CT should be avoided in the pediatric population in light of the radiation exposure. A CT scan with oral contrast can be useful in the adult setting in order to determine if there is extravasation of fluid from the gastrointestinal tract. Answer B: Abdominal ultrasound is the test of choice for diagnosing pyloric stenosis, which presents with non-bilious vomiting and a palpable olive mass in the abdomen. Treatment of this condition is with surgical release of the stenotic pyloric muscle. Answer C: A contrast enema is used to differentiate between Hirschsprung disease and meconium ileus but would not be helpful in diagnosing malrotation as the midgut volvulus occurs in the small intestine. Both Hirschsprung disease and meconium ileus present in neonatal patients with bilious vomiting and failure to pass meconium in the first 48 hours of life. Treatment is with excision of the defective segment or removal of the meconium respectively. Answer D: Rectal suction biopsy is the most accurate test to diagnose Hirschsprung disease. Although Hirschsprung disease commonly presents in neonatal patients with bilious vomiting, an abdominal radiograph in Hirschsprung disease would likely show dilation of the proximal colon. Bullet Summary: The most appropriate diagnostic test for malrotation in an infant is an upper gastrointestinal series, which will show the abnormal placement of the duodenum and ligament of Treitz on the right side of the abdomen.
nan
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A 44-year-old man presents to the emergency department with palpitations. He has a history of anxiety managed with fluoxetine and lorazepam. He also has hypertension but does not take any medications. Today, his symptoms have been persistent despite taking his medications. He says that he feels lightheaded and can’t walk. His temperature is 98.7°F (37.1°C), blood pressure is 120/72 mmHg, pulse is 183/min, respirations are 25/min, and oxygen saturation is 99% on room air. Physical exam reveals a somnolent and uncomfortable man. His pulse is rapid and regular. An ECG is performed as seen in Figure A. During the exam, the patient states that he feels lightheaded and appears occasionally somnolent. Which of the following is the most appropriate treatment for this patient?
Adenosine
Amiodarone
Procainamide
Cardioversion
D
Cardioversion
This patient is presenting with palpitations and an ECG demonstrating a wide complex tachycardia with a delta wave which are concerning for a tachydysrhythmia secondary to Wolff Parkinson White syndrome. Given his poor CNS perfusion (somnolence during his tachycardia despite a “normal” blood pressure), the most appropriate next step in management is cardioversion. Wolff Parkinson White syndrome (WPW) is a pre-excitation syndrome where an accessory electrical pathway exists between the atria and ventricles that bypasses the AV node. WPW can demonstrate antidromic conduction where the accessory pathway leads to anterograde conduction causing a wide-complex tachycardia that is often mistaken for ventricular tachycardia. In this circumstance, the QRS is wide and a delta wave is often present but may be difficult to discern. If ever uncertain as to whether the diagnosis is ventricular tachycardia or WPW, cardioversion is a safe and effective intervention for both rhythms. Unstable patients with either WPW or ventricular tachycardia should undergo cardioversion immediately. Other treatments for WPW in stable patients may include procainamide. Ablation may present future episodes. Bartlett and Friedman review the pathophysiology of WPW. They discuss the congenital presence of impulse-conducting fascicles leading to this condition. They also delve into the management and recommend rhythm agents and ablation as a modality of treatment. Figure/Illustration A is an ECG demonstrating a wide complex (red arrow) tachycardia with a delta wave (blue arrow). These ECG findings are characteristically seen in WPW syndrome. Incorrect Answers: Answer A: Adenosine would be indicated after vagal maneuvers in the management of supraventricular tachycardia (SVT). SVT presents as a narrow complex tachycardia without P waves. It is regular (in contrast to atrial fibrillation). Failure to respond to vagal maneuvers or adenosine warrants cardioversion. If this patient was perfusing his CNS, a trial of adenosine could be appropriate given it has a short half-life and may differentiate ventricular tachycardia from SVT with aberrancy or a bundle branch block. Answer B: Amiodarone may be used in stable ventricular tachycardia to control the rhythm and convert it to sinus. It would not be appropriate in a hemodynamically unstable patient. It can be used in other dysrhythmias as well including atrial fibrillation and atrial flutter. Answer D: Defibrillation is only indicated in pulseless, shockable rhythms including ventricular fibrillation (disorganized electrical activity), pulseless ventricular tachycardia (wide complex tachycardia), and pulseless torsades des pointes (twisting of the QRS complexes around an isoelectric baseline). It could cause cardiac arrest if performed on a patient with a pulse. Answer E: Procainamide is an antidysrhythmic that can be used in tachydysrhythmias that occur in WPW. Given that this patient is unstable, it is more appropriate to perform cardioversion. Bullet Summary: Unstable tachyarrhythmias in the setting of Wolff Parkinson White syndrome require cardioversion.
nan
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A 25-year-old man presents to his gastroenterologist for trouble swallowing. Whenever he eats solids, he regurgitates them back up. Given this patient's suspected diagnosis, the gastroenterologist performs a diagnostic test. Several hours later, the patient presents to the emergency department with chest pain and shortness of breath. His temperature is 99.5°F (37.5°C), blood pressure is 130/85 mmHg, pulse is 60/min, respirations are 12/min, and oxygen saturation is 99% on room air. On physical exam, the patient demonstrates a normal cardiopulmonary exam. His physical exam demonstrates no tenderness of the neck, a normal oropharynx, palpable crepitus above the clavicles, and minor lymphadenopathy. Which of the following is the most appropriate next step in management?
Barium swallow
Urgent surgery
Magnetic resonance imaging
Gastrografin swallow
D
Gastrografin swallow
This patient is presenting with chest pain and palpable crepitus after endoscopy suggesting a diagnosis of esophageal perforation. The most appropriate next step in management is a gastrografin swallow. Esophageal perforation presents with severe chest pain and palpable crepitus typically occurring after endoscopy or repeated forceful vomiting. The most appropriate initial step in management is a water-soluble contrast image (gastrografin) in order to confirm the diagnosis. Alternatively, a CT scan or radiograph may also be appropriate initially. Once the diagnosis is confirmed, surgical correction is necessary in order to prevent gastric contents from continuing to enter the mediastinum. Broad-spectrum antibiotics and proton pump inhibitors should also be administered. Kim reviews the factors associated with esophageal perforation and rupture. They discuss how causes include foreign body, iatrogenic, trauma, and forceful vomiting. They recommend urgent surgical treatment. Incorrect Answers: Answer A: Barium swallow would be inappropriate as it is an irritating substance and could travel out of the perforated esophagus. This substance is only used in rare cases when gastrografin is not revealing, per the discretion of the surgeon. Answer C: MRI would appropriately diagnose the condition but acquiring such imaging would take too long for such an urgent presentation. A gastrografin swallow is a more appropriate initial test. Answer D: Ultrasound is inappropriate as it does not demonstrate the location of the perforation and the information obtained is dependent on the habitus of the patient and the operator. Answer E: Urgent surgery would be necessary once the diagnosis is confirmed. Performing a rapid diagnostic step is necessary in order to establish the diagnosis first. Bullet Summary: The most appropriate initial step in the management of esophageal perforation is a gastrografin swallow followed by surgery.
nan
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A 55-year-old woman presents to a primary care physician with persistent nausea and abdominal discomfort. She has experienced these symptoms daily for the past 3 months. She feels bloated and has episodic loose and watery stools. She has a history of hypertension and hyperlipidemia for which she takes amlodipine and atorvastatin. She immigrated from Vietnam 6 months ago. Her temperature is 98.6°F (37.0°C), blood pressure is 110/60 mmHg, pulse is 70/min, and respirations are 18/min. Physical exam reveals a thin-appearing woman in no acute distress. Her mucous membranes are moist. Cardiac and lung examinations are unremarkable. Laboratory results are as follows: Leukocyte count: 13,000/mm^3 Segmented neutrophils: 54% Bands: 2% Eosinophils: 6% Basophils: 0.4% Lymphocytes: 30% Monocytes: 5% A vitamin D level is 26 ng/mL (reference: >= 30 ng/mL). Which of the following is the most appropriate test in making the diagnosis?
Lower endoscopy
Bowel wall biopsy
CT scan of the abdomen
Stool ova and parasite assay
D
Stool ova and parasite assay
This patient who presents with persistent abdominal discomfort, nausea, bloating, diarrhea, eosinophilia, and signs of malabsorption (thin-appearing, vitamin D deficiency) in the setting of immigration from a resource-limited country most likely has a gastrointestinal parasite infection (Ascaris lumbricoides). The most appropriate next step in management is to obtain a stool ova and parasite assay. Ascaris lumbricoides is a roundworm that is transmitted via the fecal-oral route (egg ingestion). While most patients are asymptomatic, there can be pulmonary and intestinal manifestations in the early and late phases, respectively. Pulmonary signs include dry cough, dyspnea, fever, and wheezing. Intestinal signs include abdominal discomfort, diarrhea, nausea, vomiting, malabsorption, and/or anorexia. The diagnosis should be suspected in patients with vague abdominal symptoms with travel history to an endemic area. The diagnosis is secured with stool microscopy for eggs or worms. Complications of this condition include intestinal obstruction or hepatobiliary/pancreatic involvement, presenting as biliary colic, obstructive jaundice, and/or ascending cholangitis. Treatment in patients with uncomplicated infection is with anti-helminth therapy (albendazole or mebendazole). Parija et al. review the epidemiology of helminth infections globally. The authors note that many cases are caused by Ascaris species. They recommend prompt identification of these infections as they can cause lack of school attendance, anemia, and cognitive deficits if left untreated. Incorrect Answers: Answer A: Bowel wall biopsy is used in the diagnostic work-up of suspected Whipple disease, which presents with chronic diarrhea, malabsorption, weight loss, and joint symptoms (arthralgias). This disease is progressive over years and would be less likely in this patient with disease onset of 3 months. Answer B: CT scan of the abdomen is used in the diagnostic work-up of small bowel obstruction, which presents as crampy abdominal pain, constipation, obstipation, and/or vomiting. Bowel obstruction is an acute process that would be unlikely in this patient with chronic symptoms. Although bowel obstruction is a complication of Ascaris infection, this patient has no signs of obstruction. Diarrhea may occur secondary to infection of the colon as well including colitis or diverticulitis; however, these conditions typically cause pain and low grade fever. Answer C: Lower endoscopy (colonoscopy) is used in the diagnosis of inflammatory bowel disease, consisting of Crohn disease and ulcerative colitis. Ulcerative colitis presents with bloody diarrhea, abdominal pain, weight loss, and fatigue. Eosinophilia would be unexpected, and this patient does not have bloody diarrhea. Answer E: Stool toxin assay is used to diagnose Clostridioides difficile infection. This presents after recent antibiotic use with watery diarrhea, dehydration, fever, and nausea. It presents acutely and generally without eosinophilia. Bullet Summary: Parasitic gastrointestinal infections present with chronic diarrhea, abdominal discomfort, malabsorption, and eosinophilia, and are diagnosed using stool ova and parasite assays.
nan
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A 51-year-old man presents to his primary care provider for intermittent shaking of his hands. For the last several years he has noticed this "shaking" when he brushes his teeth and prepares a cup of coffee in the morning. The shaking then gradually improves over the course of the day. His medical history is otherwise notable for hypertension and hyperlipidemia. His only home medication is pravastatin. The patient smokes half a pack per day of cigarettes and drinks 2-3 beers throughout the day. His family history is significant for Parkinson disease in his father. On physical exam, his temperature is 98.6°F (37.0°C), blood pressure is 159/84, pulse is 74/min, and respirations are 12/min. He has a high-frequency bilateral hand tremor elicited on finger-to-nose testing. His neurological exam is otherwise unremarkable. Which of the following medications should this patient be started on?
Alprazolam
Primidone
Propranolol
Topiramate
C
Propranolol
This patient presents with a high frequency bilateral action tremor, which suggests a diagnosis of benign essential tremor. In light of his concomitant hypertension, the patient should be started on propranolol. Benign essential tremor usually presents as a bilateral tremor of the hands that is observed during fine motor movements. Patients present with worsening tremors with posturing and with voluntary movement and improve with alcohol and rest. First-line treatment includes propranolol and primidone, with propranolol being preferred for patients with hypertension or vascular risk factors. Refractory cases can be treated with deep brain stimulation which is often a last-line intervention. Shanker reviewed the diagnosis and treatment of patients with essential tremors. She discusses how this disease presents with an action tremor that lasts for at least 3 years. She recommends treatment with propranolol or primidone. Incorrect Answers: Answer A: Alprazolam is sometimes used as second-line therapy for essential tremor refractory to propranolol and primidone but would not be used as initial therapy in this patient. Benzodiazepines can be used in patients with panic disorders. Answer B: Primidone is a barbiturate that is another first-line treatment for essential tremor. For this patient, propranolol would be preferred to also treat this patient’s underlying hypertension. Primidone can be used in patients without other underlying comorbidities. Answer D: Topiramate is an antiepileptic that may be used as a second-line treatment option for essential tremor. It would not be used as initial drug therapy. Topiramate can be used in the treatment of patients with partial seizures that present with motor symptoms. Answer E: Trihexyphenidyl is an anticholinergic medication that is used to treat early Parkinson disease in which tremor is the predominant symptom. Despite this patient’s family history of Parkinson disease, the tremor in Parkinson disease usually presents as an asymmetric, “pill-rolling” resting tremor rather than an action tremor. Bullet Summary: Propranolol is the first-line treatment for benign essential tremor in patients with concomitant hypertension.
Trihexyphenidyl
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A 28-year-old G1P0 woman presents to initiate prenatal care after a positive home pregnancy test. The first day of her last menstrual period was 7 weeks prior to presentation. The patient has no medical conditions and takes no medications. Her temperature is 98.6°F (37.0°C), blood pressure is 110/70 mmHg, pulse is 64/min, and respirations are 17/min. Physical exam is notable for a nontender abdomen. Pelvic exam reveals a closed cervix with no adnexal or uterine tenderness. A urine pregnancy test is positive, and an intrauterine singleton pregnancy measuring approximately 9 weeks gestational age is visualized on transvaginal ultrasound. The patient asks if she should modify her current exercise regimen due to her pregnancy. She typically swims or uses an exercise bike for 45 minutes, 5 days a week, and plays in an office soccer league for 1 hour on 1 day per week. Which of the following is the most appropriate recommendation regarding exercise for this patient?
Avoid exercise during pregnancy
Avoid soccer during pregnancy, continue other activities
Continue the current exercise regimen
Limit moderate exercise to a maximum of 30 minutes per day during pregnancy
B
Avoid soccer during pregnancy, continue other activities
This healthy primigravida woman presents to the clinic inquiring about her current exercise regimen involving 285 minutes of moderate to exercise weekly. With the exception of soccer, a contact sport, her exercise regimen is appropriate throughout her pregnancy. The American College of Obstetricians and Gynecologists (ACOG), recommends that healthy pregnant women engage in moderate-intensity exercise for at least 30 minutes or more for 5-7 days weekly provided there are no contraindications to doing so (certain cardiac or pulmonary conditions, history of cerclage placement, multiple pregnancies, preeclampsia, or severe anemia). Highly active patients may continue their more vigorous pre-pregnancy regimens after consulting with a physician. Exercise in pregnancy can ease gastrointestinal symptoms and may decrease the risk of gestational diabetes, preeclampsia, and cesarean delivery. However, activities that may cause fetal or maternal injury, such as contact sports or activities with a significant fall risk should be avoided. Examples of contact sports include football, basketball, hockey, lacrosse, and wrestling. Nascimento et al. review exercise in pregnancy. They note that exercise is beneficial in pregnancy and in the postpartum period and recommend engaging in appropriate exercise in this patient population. Incorrect Answers: Answer A: Avoiding exercise during pregnancy is not recommended, as at least 150 minutes of moderate exercise weekly is suggested during pregnancy for the health of the mother and the fetus. Answer C: Continuing the current exercise regimen is not recommended, as soccer is a high-contact sport that could cause injury to the fetus or placenta. Answer D: Limiting moderate exercise to 30 minutes per day maximum during pregnancy is not recommended, as 30 minutes daily is the minimum recommended time for moderate-exercise. There is no reason for healthy pregnant patients without high-risk pregnancies to significantly limit their exercise. Answer E: Reducing to walking-only beginning in the third trimester is not necessary, as moderate activities are healthy throughout pregnancy. Bullet Summary: Healthy pregnant patients should perform at least 30 minutes of exercise 5-7 days per week and avoid high-contact sports or sports with fall risk.
Reduce to walking only, beginning in the third trimester
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A 72-year-old man presents to his primary care physician with weakness. He has felt very weak every morning with his symptoms persisting throughout the day. He notes minor improvement when he rides his bike but otherwise has not noticed any change in his symptoms with rest or ibuprofen use. The patient has lost 12 pounds recently and has had a chronic cough. The patient lives alone and drinks 7 alcoholic beverages per day and smokes 1-2 packs of cigarettes per day for the past 40 years. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 93/min, respirations are 17/min, and oxygen saturation is 92% on room air. A chest radiograph is ordered as seen in Figure A. Which of the following diagnostic tests would most likely elucidate the cause of this patient's weakness?
CT scan of the chest
Electromyography
Lung biopsy
Muscle biopsy
B
Electromyography
This patient who presents with weakness that improves with activity in the setting of a lung mass most likely has Lambert-Eaton syndrome. The most appropriate diagnostic test to confirm this syndrome is electromyography. Small cell lung cancer presents with fatigue, weight loss, cough, and a coin lesion on chest radiograph typically in a smoker. A common paraneoplastic syndrome in small cell lung cancer is Lambert-Eaton syndrome which presents with muscle weakness that improves with activity and worsens with rest. Activity improves symptoms due to accumulation of calcium with exercise which allows for acetylcholine release. This occurs secondary to antibodies against presynaptic calcium channels which decrease acetylcholine release. The diagnosis of Lambert Eaton syndrome can be confirmed with electromyography. Treatment includes addressing the underlying lung tumor. Kesner et al. review the diagnosis and etiology of Lambert-Eaton syndrome. They discuss how this disease is associated with lung cancer. They recommend making the diagnosis using electrophysiologic studies. Figure/Illustration A is a chest radiograph with a coin lesion in the left lung field (red circle). This finding is suggestive of small cell lung cancer. Incorrect Answers: Answer A: Blood laboratory tests could assess for multidrug resistance 1 protein (MDR-1 aka P-glycoprotein or ABCB1) which predicts resistance to chemotherapy for small cell lung cancer; however, it would not further elucidate this patient's muscle weakness. Answers 2 & 4: CT scan of the chest and lung biopsy would further elucidate this patient's small cell lung cancer; however, it would not explain his weakness that improves with activity. In the setting of an elderly smoker with fatigue and weight loss and a coin lesion, the diagnosis of lung cancer is essentially established. Answer E: Muscle biopsy would be the most accurate diagnostic test for dermatomyositis, polymyositis, and inclusion body myositis; however, it would not assess for conditions such as myasthenia gravis and Lambert-Eaton syndrome. Bullet Summary: Electromyography is the diagnostic test of choice for Lambert-Eaton syndrome.
nan