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https://step2.medbullets.com/testview?qid=216371
A 68-year-old woman presents to the emergency room with several days of frequent, nonbloody, watery stools. She recently completed a course of clindamycin 2 weeks ago for a tooth infection. Her medical history is notable for hypertension and hyperlipidemia for which she takes amlodipine and atorvastatin. She traveled recently and has no sick contacts. Her temperature is 37.0°C (98.6°F), blood pressure is 118/82 mmHg, pulse is 98/min, respirations are 14/min, and oxygen saturation is 98% on room air. On exam, her abdomen is soft, non-distended, and non-tender to palpation; however she has diffuse discomfort upon palpation of the abdomen with no rebound, rigidity, or guarding. Which of the following is most likely to confirm the diagnosis?
Abdominal radiograph
CT imaging of abdomen and pelvis
Flexible sigmoidoscopy
Rectal swab for selective anaerobic culture
Stool toxin assay
E
Stool toxin assay
This patient presenting with cramping abdominal pain and frequent watery diarrhea a few weeks after finishing a course of antibiotics most likely has Clostridioides difficile infection, for which the most appropriate confirmatory test is a stool toxin assay. C. difficile infection should be suspected in patients with acute and clinically significant diarrhea with no obvious alternative explanation. It is associated with risk factors such as recent antibiotic use, hospitalization, and advanced age. Diagnosis is established via a positive enzyme immunoassay for C. difficile toxins and C. difficile glutamate dehydrogenase (GDH), or a positive nucleic acid amplification test (NAAT) for C. difficile toxins. The GDH enzyme immunoassay is highly sensitive for detecting C. difficile, but not specific for toxin-producing C. difficile, and thus the toxin enzyme immunoassay is submitted in conjunction. If the results of both are discordant, then NAAT is used to make the diagnosis. Treatment for C. difficile infection differs by disease severity and recurrence. Commonly used medications include fidaxomicin and oral vancomycin. McDonald et al. review the guidelines for diagnosis and treatment of C. difficile and provide the algorithm for C. difficile testing as mentioned above. The authors also note that only liquid stool from patients with clinically significant and acute diarrhea should be submitted for testing. The authors recommend that formed stool from asymptomatic patients should not be tested because the presence of the C. difficile toxin gene does not distinguish between infection and asymptomatic carriage. Incorrect Answers: Answer A: Abdominal radiograph is an appropriate adjunctive diagnostic tool in patients with severe disease (e.g., severe abdominal pain and distension, fever, hypovolemia, lactic acidosis, marked leukocytosis) or fulminant colitis (marked by hypotension and ileus) to evaluate for toxic megacolon, bowel perforation, or other findings that warrant surgical intervention. In patients who are stable with low concern for severe disease or fulminant colitis, it would not be the preferred initial test for diagnosis nor would it be confirmatory. Answer B: CT imaging of the abdomen and pelvis with oral and intravenous contrast is the preferred modality for detecting the presence of toxic megacolon, bowel perforation, or other findings that warrant surgical intervention in the setting of C. difficile colitis. Colonic dilatation of > 7 cm in diameter in the clinical setting of severe C. difficile infection is diagnostic. Other findings may include small bowel dilatation or scalloping of the bowel wall due to submucosal edema. While it would be reasonable to perform a CT scan in this patient, it would not confirm the diagnosis. Answer C: Flexible sigmoidoscopy would be appropriate for cases in which an alternative diagnosis is suspected that requires direct visualization and/or biopsy of the bowel mucosa. It may also be helpful for patients with ileus or colitis in the absence of diarrhea as it allows for visualization of pseudomembranes on the inflamed mucosal surface, which are highly suggestive of C. difficile infection. In general, colonoscopy is a preferred and more accurate test. Answer D: Rectal swab for selective anaerobic culture involves culture on a selective medium with toxin testing of isolated C. difficile. It is a highly sensitive test, but culture cannot distinguish toxin-producing strains from non-toxin-producing strains. A second test is required to detect toxin production by the cultured C. difficile strain (either enzyme immunoassay or nucleic acid amplification testing). Culture takes several days to finalize and as such, it is seldom used to establish a clinical diagnosis. It is most useful in patients with ileus and suspected C. difficile infection. Bullet Summary: The most appropriate confirmatory test for diagnosis of Clostridioides difficile infection is a stool toxin assay.
https://step2.medbullets.com/testview?qid=216746
A 21-year-old woman presents to her primary care physician due to a history of abdominal pain. She states that she has had recurrent bouts of mood swings, bloating, and abdominal pain that occur together approximately every 4 weeks. She states that when these symptoms occur, she gets into frequent arguments at work, is unable to concentrate, and sleeps longer than usual. Furthermore, she has episodes of extreme anxiety during these periods, leading her to take off time from work. She notes that these symptoms are causing distress in her interpersonal relationships as well. She has no past medical history and takes no medications. She experienced menarche at age 12 and has regular periods. Her temperature is 98.2°F (36.8°C), blood pressure is 100/70 mmHg, pulse is 75/min, and respirations are 12/min. Physical exam reveals a nontender abdomen. Pelvic exam reveals a closed cervix with no cervical motion or adnexal tenderness. Her cardiopulmonary and neurological exams are unremarkable. Which of the following is the most likely diagnosis?
Generalized anxiety disorder
Major depressive disorder
Panic disorder
Premenstrual dysophoric disorder
Premenstrual syndrome
D
Premenstrual dysophoric disorder
This patient with recurrent abdominal pain, bloating, and mood changes causing interpersonal difficulties related to the menstrual cycle likely has premenstrual dysphoric disorder. Premenstrual dysphoric disorder is a variant of premenstrual syndrome (PMS) characterized by prominent irritability and disruption of everyday life. Premenstrual dysphoric disorder is a variant of PMS characterized by recurrent physical and behavioral symptoms that manifest during the luteal phase of the menstrual cycle and resolve with menstruation. The diagnosis is made by criteria outlined by the Diagnostic and Statistical Manual of Mental Disorders (DSM-5). To make a diagnosis of premenstrual dysphoric disorder, symptoms such as marked affective lability or marked irritability or anger must be present along with signs such as decreased concentration, hypersomnia or insomnia, and decreased interest in usual activities. These symptoms must cause significant distress or interfere with work, school, or relationships and must present 1 week before the onset of menses and resolve the week following menses. The initial management of premenstrual dysphoric disorder involves a pregnancy test to rule out pregnancy and a menstrual diary to prospectively confirm symptoms and their relationship to the menstrual cycle for 2 consecutive cycles. Following confirmation of a diagnosis of premenstrual dysphoric disorder, treatment involves lifestyle changes, antidepressant/anxiolytic medications, or hormonal therapies such as oral contraceptive pills (OCPs). Bhatia et al. discuss premenstrual dysphoric disorder. They note that premenstrual dysphoric disorder affects 2-10% of women of reproductive age. They note that serotonin dysregulation may be relevant to the etiology and treatment of premenstrual dysphoric disorder. Incorrect Answers: Answer A: Generalized anxiety disorder (GAD) is anxiety characterized by symptoms such as restlessness, fatigue, and irritability lasting > 6 months unrelated to any specific triggers. The topics of anxiety should be unrelated to one another. This patient's anxiety and symptoms are temporally related to her menstrual cycle, making premenstrual dysphoric disorder a more likely diagnosis. Answer B: Major depressive disorder (MDD) is characterized by depressed mood and at least 5 of 9 "SIG E CAPS" symptoms for 2 weeks or longer. These symptoms include sleep disturbance, anhedonia, guilt, loss of energy, loss of concentration, appetite/weight changes, psychomotor retardation or agitation, and suicidal ideation. Though this patient exhibits some of these symptoms, premenstrual dysphoric disorder is a more likely diagnosis as she exhibits these symptoms temporally related to her menstrual cycle. Answer C: Panic disorder is characterized by 4 or more panic attacks (sweating, palpitations, shaking, paresthesias) in a 4-week period or ≥ 1 panic attack followed by at least 1 month of fear of another panic attack. Though this patient experiences anxiety that leads her to avoid her work, she does not have any of the characteristic signs of a panic attack and therefore does not meet the criteria for diagnosis of panic disorder. Answer E: PMS and premenstrual dysphoric disorder have overlap in their symptoms; however, premenstrual dysphoric disorder is characterized by symptoms that significantly affect daily functioning. This patient's symptoms have affected her work and relationships, making premenstrual dysphoric disorder a more likely diagnosis. Note that the workup and treatment is similar between these 2 conditions. Bullet Summary: Premenstrual dysphoric disorder (PMDD) is a variant of premenstrual syndrome (PMS) characterized by more severe physical and mood symptoms that affect daily functioning.
https://step2.medbullets.com/testview?qid=216240
A 15-year-old girl presents to the clinic due to concern that she may be pregnant. She had unprotected sexual intercourse earlier that morning and requests emergency contraception. She has had regular menses since menarche, and her last menstrual period ended 8 days ago. She has no significant medical history. Her temperature is 98.6°F (37°C), blood pressure is 114/71 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is unremarkable. A urine pregnancy test is negative. The patient asks for an emergency contraceptive pill and would prefer that her parents not be notified about her visit. In most states, which of the following is the most appropriate next step in the management for this patient?
Do not prescribe emergency contraception given that the patient is under 18 years old
Obtain parental consent before providing emergency contraception
Prescribe high-dose oral contraceptives
Prescribe levonorgestrel pill
Prescribe misoprostol pill
D
Prescribe levonorgestrel pill
This patient with recent unprotected intercourse and a negative pregnancy test is a candidate for postcoital emergency contraception. She should receive the levonorgestrel or ulipristal pill, which in most states does not require parental consent. Patients with a positive pregnancy test are not candidates for emergency contraception because implantation has already occurred, but patients with recent unprotected intercourse but a negative pregnancy test should receive the levonorgestrel pill (plan B) or ulipristal pill. These pills should be administered as soon as possible after intercourse. This is because the efficacy of the pills decrease after 72-120 hours post-intercourse. The high doses of progestin in these pills delay ovulation by blocking the LH surge, thereby preventing pregnancy. In most states, adolescents can receive emergency contraception without parental consent or notification. Batur et al. present the current options available for emergency contraception. They discuss how ulipristal acetate tends to be more efficacious compared to levonorgestrel when used more than 72 hours post-coitally. They recommend that all providers who treat young women be familiar with the options and indications for emergency contraception. Incorrect Answers: Answer A: It is inappropriate to not administer emergency contraception despite the patient being under 18 years old. Adolescent patients at risk of pregnancy after unprotected intercourse who request emergency contraception should be presented with viable options. Answer B: Obtaining parental consent before providing emergency contraception is an incorrect choice in most states. Most states allow adolescents to receive confidential care for contraception and pregnancy without parental involvement, consent, or notification. Answer C: Prescribing high-dose oral contraceptives can be done in special circumstances to delay ovulation by patients taking multiple pills at once. However, combination oral contraceptives are less commonly used for post-coital contraception as they have significant side effects at high doses and are less effective than levonorgestrel pills. Answer E: In patients with a positive pregnancy test (after implantation has occurred), misoprostol can be used for pregnancy termination by stimulating myometrial contractions. It does not prevent pregnancy. The use of drugs to terminate pregnancy varies significantly from state to state. Bullet Summary: Adolescents seeking emergency contraception may do so without parental consent or involvement in most states.
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A 65-year-old woman presents to her family doctor to reestablish care since losing her insurance. She has not had time for regular check-ups. She exercises 3-4 times a week and consumes red meat sparingly. She drank and smoked cigarettes socially with coworkers but never at home or on vacation. She wakes up with achy wrists and elbows that she suspects is from years of using a computer keyboard. She completed menopause at age 52. Her family history is notable for coronary artery disease on her father's side and colon cancer on her mother's side. She last had a colonoscopy 5 years ago that revealed no abnormal findings. Her temperature is 99°F (37.2°C), blood pressure is 125/83 mmHg, pulse is 82/min, respirations are 12/min, and oxygen saturation is 99% on room air. Her physical exam is grossly unremarkable. What diagnostic test should this patient receive?
Chest radiograph
Colonoscopy
Mammography
Pelvic ultrasound
Vitamin D level
C
Mammography
This vignette describes an otherwise healthy elderly woman seeking to establish primary care. Among the answer choices, mammography is the most appropriate diagnostic test to recommend. Mammography is a screening tool for breast cancer that becomes most effective in postmenopausal patients because of less glandular breast tissue compared to younger patients. According to the United States Preventive Services Task Force, all women aged 50-74 should have mammograms every 1-2 years. It is controversial whether screening mammography should begin as early as age 40. Women with 1st-degree relatives with breast cancer should begin screening 10 years before the age of the family member's diagnosis. Seely and Alhassan review the evidence regarding the screening methods used for breast cancer. They discuss how screening every 1-2 years decreases breast cancer mortality. They recommend using judicious mixed modal methods of screening. Incorrect Answers: Answer A: Routine chest radiography for lung cancer screening in low-risk patients is not recommended by the USPSTF. Chest imaging can be used to screen for lung cancer in patients with a significant smoking history. Answer B: Colonoscopy for colon cancer screening is recommended every 10 years starting at age 50. This patient already had a colonoscopy 5 years ago so she does not need another exam at this time. Answer D: Pelvic ultrasound for ovarian cancer screening is not currently recommended by the USPSTF. Ovarian cancer detection should also be performed in patients who are symptomatic. Answer E: Vitamin D level for osteoporosis screening is not recommended. DEXA scans would be more appropriate for testing bone density. Bullet Summary According to the United States Preventive Services Task Force, all women aged 50-74 should have mammograms every 1-2 years.
https://step2.medbullets.com/testview?qid=214967
A 27-year-old man presents to the emergency department with altered mental status. He was running a marathon but became confused halfway through the race. He arrives unable to coherently answer questions and is not sure where he is. He has a history of a seizure disorder. His temperature is 105°F (40.6°C), blood pressure is 116/68 mmHg, pulse is 167/min, respirations are 29/min, and oxygen saturation is 99% on room air. Physical exam reveals a confused man who is moving all 4 extremities and is protecting his airway. During the exam, he develops a tonic-clonic seizure, which lasts 1 minute and then terminates on its own. The patient is given intravenous fluids, and basic laboratory studies are drawn and pending. A urine dipstick is positive for red blood cells. Which of the following is most likely to be seen on laboratory testing?
Hyperkalemia
Hyperphosphatemia
Hyperuricemia
Hypocalcemia
Hyponatremia
D
Hypocalcemia
This patient is presenting after running a marathon with hyperthermia and altered mental status suggestive of heatstroke. In the setting of his recent exertion, tonic-clonic seizure, and urine dipstick positive for red blood cells, he is likely suffering from rhabdomyolysis, which most commonly causes hypocalcemia. Rhabdomyolysis occurs when there is a breakdown of the muscle cells. It can occur with exertion (such as marathon running or weight lifting), seizures (from prolonged and intense contraction of the muscles), cocaine use, lightning strikes, electrical injuries, and crush injuries. Damage to muscle cells most commonly causes hypocalcemia from the deposition of calcium in necrotic muscle cells as well as calcium sequestration by the muscle sarcoplasmic reticulum. Symptoms of hypocalcemia include perioral numbness and tetany, altered mental status, and seizure. ECG manifestations of hypocalcemia include QT prolongation. There is no treatment needed for hypocalcemia in rhabdomyolysis unless it is severe or is causing symptoms such as seizure or ECG changes. The management of rhabdomyolysis involves aggressive IV fluid administration titrated to urine output in order to prevent myoglobin nephrotoxicity. Myoglobin causes the classic false-positive finding of red blood cells on urine dipstick given the cross-reactivity of myoglobin and hemoglobin on this test. Giannoglou et al. review the pathophysiology and diagnosis of rhabdomyolysis. The authors find that hypocalcemia is the most common electrolyte derangement seen in rhabdomyolysis. The authors recommend carefully monitoring ionized calcium in patients with rhabdomyolysis. Incorrect Answers: Answer A: Hyperkalemia is a common electrolyte derangement that occurs in rhabdomyolysis and can cause life-threatening dysrhythmias. Initial ECG findings suggestive of hyperkalemia include peaked T waves and QRS widening. The management of hyperkalemia involves the administration of calcium chloride to stabilize cardiac membranes followed by insulin, albuterol, and bicarbonate. Severe/refractory hyperkalemia can be treated with dialysis, but this is usually not needed in rhabdomyolysis. Hyperkalemia is less common when compared to hypocalcemia in rhabdomyolysis and does not cause seizures. Answer B: Hyperphosphatemia is common in rhabdomyolysis and occurs secondary to the release of phosphorus from damaged muscle cells. Hyperphosphatemia does not usually require treatment in rhabdomyolysis, though phosphate restriction and phosphate binders can be administered. Hyperphosphatemia can rarely lead to seizures, though this is uncommon. Answer C: Hyperuricemia is possible in rhabdomyolysis, occurs secondary to the release of purines from injured muscle cells, and is worsened by acute kidney injury which is common in rhabdomyolysis. The treatment of hyperuricemia in rhabdomyolysis involves IV fluids, rasburicase, and allopurinol (which works slowly). Answer E: Hyponatremia may occur after running a marathon where the individual sweats out sodium, chloride, and water but rehydrates with only water. While this is possible in this patient and his seizure could reflect a hyponatremic seizure (which usually would not spontaneously resolve and requires hypertonic saline), there is concrete evidence of rhabdomyolysis with the positive urine dipstick for red blood cells. Bullet Summary: Rhabdomyolysis can cause hyperkalemia, hyperphosphatemia, and hypocalcemia (the most common electrolyte disturbance).
https://bit.ly/44Zo5vi
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?
Abdominal CT
Abdominal ultrasound
Contrast enema
Rectal suction biopsy
Upper gastrointestinal contrast series
E
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.
https://bit.ly/3ZAMN3W
A 6-year-old boy is brought to the emergency department by his father for elbow pain. He was riding his bicycle when he fell onto his outstretched right hand. He immediately clutched his right elbow in pain and began crying. He refuses to move his elbow. He has no medical problems and takes no medications. His temperature is 98.4°F (36.9°C), blood pressure is 108/64 mmHg, pulse is 100/min, and respirations are 20/min. On exam, the boy is holding his right wrist with his left hand and cries upon palpation of a region that is 2 cm proximal to his elbow. The radial head is palpated just distal to the lateral epicondyle. He is unable to make a pincer with his right thumb and index finger. When asked to hold a pen between his right thumb and index fingers, he drops it. The radial pulse is diminished on the right. Which of the following is the most likely diagnosis?
Both bone forearm fracture
Distal radius fracture
Monteggia fracture-dislocation
Olecranon fracture
Supracondylar humerus fracture
E
Supracondylar humerus fracture
This young patient presenting with elbow pain and associated signs of anterior interosseous nerve neurapraxia (inability to make pincer, weak pincer grasp) and vascular injury (diminished radial pulse) after a fall on an outstretched hand most likely has a supracondylar humerus fracture. Supracondylar humerus fractures are one of the most common fractures seen in the pediatric population. This site is particularly prone to fracture due to the thin bone separating the coronoid and olecranon fossae in the supracondylar region. Due to the proximity of the brachial artery and median nerve, which course anteriorly, these structures are at risk for injury. Neurapraxia of the anterior interosseous nerve, which is a pure motor branch of the median nerve, presents as motor deficits in interphalangeal (IP) joint flexion of the thumb and distal IP joint flexion of the index finger. The radial and ulnar arteries are terminal branches of the brachial artery, which if injured results in diminished distal pulses. The diagnosis is made with anteroposterior and lateral view elbow radiographs. Radiographs will demonstrate the "fat pad sign" seen as a posterior fat pad that is indicative of fracture. Management depends on fracture type but can consist of cast immobilization (if minimally displaced with no neurovascular injury) or closed reduction and percutaneous pinning (for more displaced fractures). Micheloni et al. reviews supracondylar fractures in children. They note it is the most common elbow fracture in the pediatric population. They recommend early and correct diagnosis and management to avoid early and late complications including neurovascular impairment and malunion. Incorrect Answers: Answer A: Both bone forearm fracture is another common pediatric fracture sustained from a fall, involving both the radius and ulna. It can be associated with neurovascular injury depending on the plane of deformity, but the location of the pain would be in the forearm, not proximal to the elbow. Answer B: Distal radius fracture is a common fracture after a fall on an outstretched hand mechanism of injury. Neurovascular injury is possible, though uncommon. This patient’s pain is localized around his elbow, rather than near the distal radius. Often, a deformity can be visualized on exam. Answer C: Monteggia fracture-dislocation is characterized by a proximal ulna fracture with radial head dislocation. It is commonly caused by direct trauma to the ulna. In contrast, this patient’s radial head is located inferior to the lateral epicondyle. Answer D: Olecranon fracture is an uncommon pediatric fracture caused primarily by a fall onto the elbow in flexion. Median nerve and brachial artery injury are unlikely, given that these structures pass anteriorly in the cubital fossa. Bullet Summary: Supracondylar humerus fractures are common in the pediatric population and may present with elbow pain and concomitant median nerve and/or brachial artery injury.
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A 65-year-old man presents to the emergency department for evaluation of left arm and leg weakness. He was walking in the park about 45 minutes prior to arrival when the patient stumbled and fell. He had sudden weakness in his left leg and was unable to grasp a handrail with his left hand. He currently takes no medications. His temperature is 98.0°F (36.6°C), blood pressure is 128/89 mmHg, pulse is 130/min, and respirations are 18/min. On exam, he has 3/5 strength in the left upper and lower extremities and a left-sided facial droop. A non-contrast CT scan of the head is performed, which suggests parenchymal ischemia in the distribution of the right middle cerebral artery. An ECG is performed, as shown in Figure A. He is given intravenous alteplase with rapid improvement in his strength deficits and facial asymmetry. Which of the following is the most appropriate long-term therapy for secondary stroke prevention for this patient?
Aspirin
Atorvastatin
Cardiac pacemaker
Rivaroxaban
Warfarin
D
Rivaroxaban
This patient presents with acute onset unilateral left-sided weakness secondary to an ischemic stroke in the right MCA in the setting of an irregularly irregular rhythm without P waves (suggesting that he had an embolic stroke secondary to atrial fibrillation). First-line primary and secondary stroke prevention in patients with atrial fibrillation involves long term anticoagulation with a direct oral anticoagulant (DOAC) such as rivaroxaban. Atrial fibrillation is caused by uncoordinated atrial contractions, typically due to atrial enlargement, inflammation, or infiltrative disease. Atrial fibrillation can lead to the formation of a thrombus, typically in the left atrial appendage, which can embolize to the brain and lead to an ischemic stroke. For this reason, there is a low threshold to start patients on long term anticoagulation for both primary and secondary stroke prevention (risk stratification is done with the CHA2DS2-VASc score, which considers several cardiovascular risk factors, age, sex, and diabetes). First-line long term anticoagulation is a DOAC medication such as apixaban, dabigatran, rivaroxaban, or edoxaban. Delayed initiation of anticoagulation is recommended at 4-14 days after a stroke, due to the interim risk of hemorrhagic conversion. Further management of atrial fibrillation includes a rate control agent such as a beta blocker or a calcium channel blocker. JS Alpert reviews the latest American Heart Association/American College of Cardiology guidelines regarding atrial fibrillation. The article reports that for stroke prevention in atrial fibrillation, DOACs are first-line compared to warfarin due to a decreased risk for bleeding with DOACs. In patients with moderate to severe mitral stenosis or a mechanical heart valve, warfarin is the preferred agent. Figure/Illustration A shows an ECG with an irregularly irregular rhythm (highlighted by the spacing of blue arrows) and no discernable P waves before QRS complexes, which is characteristic of atrial fibrillation. Incorrect Answers: Answer A: Aspirin exerts antiplatelet adhesion effects through the inhibition of cyclooxygenase. Patients with atrial fibrillation who are at low risk for stroke (CHA2DS2-VASc score of < 1) can use aspirin monotherapy for stroke prophylaxis. This patient presented after a stroke and will require long term anticoagulation, not anti-platelet monotherapy for stroke prevention. Answer B: Atorvastatin is an HMG-CoA reductase inhibitor, which works to lower LDL cholesterol and triglycerides, and increase HDL cholesterol. It is most useful in secondary stroke prevention in patients who have suffered a thrombotic or thromboembolic stroke secondary to atherosclerosis. This patient may have atherosclerosis and could benefit from statin therapy; however, anticoagulation for his underlying atrial fibrillation is the highest priority. Answer C: A cardiac pacemaker is indicated in a large variety of cardiac conditions that primarily involve sinus node dysfunction or high-grade atrioventricular block. Atrial fibrillation does not typically require a pacemaker, as it is typically managed with rate control agents such as beta-blockers, calcium channel blockers, digoxin, or with electrical cardioversion. Rate control will not lower the stroke risk, and thus long-term anticoagulation is required. Answer E: Warfarin has historically been a mainstay anticoagulant for stroke prevention in atrial fibrillation. DOACs are non-inferior to warfarin, and have a lower risk of bleeding. Note, only the initiation of warfarin requires a heparin bridge to mitigate the transient protein C deficiency that results in a temporary hypercoagulable state (often presenting as warfarin skin necrosis). Bullet Summary: For patients who present with an ischemic stroke secondary to a cardiac embolus from atrial fibrillation, first-line long term anticoagulation is a direct oral anticoagulant (apixaban, dabigatran, rivaroxaban, or edoxaban).
https://step2.medbullets.com/testview?qid=216625
A previously healthy 15-year-old boy presents to the emergency department in cardiac arrest. He was in class when he suddenly fainted and became unresponsive. His teacher began chest compressions and he has received epinephrine and defibrillation from paramedics while in transport. On arrival, the patient remains pulseless and is pronounced dead. An autopsy is performed and is notable for no clear abnormalities. The patient’s laboratory studies were unremarkable from a visit to the pediatrician 3 weeks ago. A previous ECG is seen in Figure A. Which of the following is the most likely underlying etiology of this patient’s death?
Complete heart block
Short QT syndrome
Torsades des pointes
Ventricular fibrillation
Ventricular tachycardia
B
Short QT syndrome
This healthy pediatric patient is presenting with cardiac arrest and an ECG demonstrating a short QT segment. Short QT syndrome is lethal and can lead to cardiac arrest in young, otherwise healthy patients. Short QT syndrome is an inherited condition that is caused by ion channel mutations (the most common identified mutation is a mutation in the cardiac myocyte potassium channel). There is no strict criteria for short QT syndrome, but the QT segment is often < 320 ms. Patients are usually young and healthy, and it is very common for the first presentation of this disease to be syncope or sudden cardiac death. ECG features suggestive of this condition include a short QT interval (without a clearly defined value but often < 360 ms) that is fixed (does not change with exercise or rest), peaked T waves, short or even absent ST segments, and episodes of atrial fibrillation or ventricular fibrillation in a patient in whom this would not be expected (such as a young patient). The only effective intervention is the placement of an implantable cardioverter-defibrillator. Bjerregaard and Gussak discuss short QT syndrome. They note it is a heritable condition of the conduction system of the heart that can lead to lethal dysrhythmias. They discuss the management of this condition and recommend an implantable cardioverter-defibrillator. Figure/Illustration A is an ECG demonstrating a short QT segment (red arrow). Note the T waves appear peaked which can be seen in this syndrome. This may suggest hyperkalemia, but this patient’s labs were normal at his appointment when the ECG was taken. Incorrect Answers: Answer A: Complete heart block is more common in elderly patients with acute coronary syndrome and presents with regular P-P and R-R intervals with complete P-R dissociation. It may cause syncope but is not associated with short QT syndrome. Management involves the placement of a pacemaker. Answer C: Torsades des pointes is associated with a prolonged QT segment that progresses to the classic twisting of the QRS complexes around an isoelectric baseline. It can lead to cardiac arrest and requires immediate cessation of QT-prolonging medications and administration of magnesium. Note that this patient’s QT segment is short rather than long. Answer D: Ventricular fibrillation presents with disorganized electrical activity with no discernible rhythm. While this patient may have arrested from ventricular fibrillation, the most likely underlying etiology for his cardiac arrest given his age, ECG, and risk factors is an underlying condition such as short QT syndrome. For this reason, short QT syndrome is a better answer. Answer E: Ventricular tachycardia could also occur in short QT syndrome and may have caused this patient’s cardiac arrest. It presents with a monomorphic wide complex tachycardia that may cause arrest. Unstable patients require cardioversion while pulseless patients require defibrillation. Note that if this patient arrested from ventricular tachycardia, it was likely secondary to short QT syndrome. Bullet Summary: Short QT syndrome is a rare but possible diagnosis in young, healthy patients who present with recurrent syncope or cardiac arrest.
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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?
Blood laboratory test
CT scan of the chest
Electromyography
Lung biopsy
Muscle biopsy
C
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.
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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?
Anterior-posterior and lateral radiographs of the abdomen and pelvis
CT scan of the abdomen and pelvis with contrast
Diagnostic peritoneal lavage
FAST exam
Norepinephrine infusion
D
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.
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A 55-year-old man presents to his primary care physician with fatigue, malaise, and a painful rash. The patient states that his symptoms have been worsening over the past week. He also has experienced abdominal pain and diarrhea. He has a medical history of obesity and smokes regularly. His temperature is 97.5°F (36.4°C), blood pressure is 142/82 mmHg, pulse is 85/min, respirations are 15/min, and oxygen saturation is 98% on room air. Physical exam is notable for an erythematous rash with papules and plaques on the patient's face, torso, and extremities. He states that the rash is painful. A fingerstick blood glucose is unable to accurately read his blood glucose and gives a reading of > 500 mg/dL. Which of the following is the most likely underlying pathophysiology of this patient’s condition?
Alpha cell tumor
Beta cell destruction
Beta cell tumor
Hypercortisolism
Insulin resistance
A
Alpha cell tumor
This patient is presenting with necrolytic migratory erythema (a painful, erythematous rash with papules and plaques) with gastrointestinal symptoms (abdominal pain and diarrhea) and hyperglycemia, which are concerning for a glucagonoma. A glucagonoma is a tumor of the alpha cells. A glucagonoma is a tumor of the alpha cells of the pancreas. This tumor pathologically secretes glucagon leading to profound hyperglycemia that is refractory to the normal treatments for diabetes. Patients also present with a classic rash termed necrolytic migratory erythema which presents with painful, erythematous papules and plaques. Other findings include gastrointestinal symptoms including anorexia, abdominal pain, and diarrhea. Hematologic findings include a normocytic, normochromic anemia. The diagnosis can be supported with a glucagon level, which will be elevated. Imaging of the pancreas by CT or MRI (more accurate) can further support the diagnosis. Zandee et al. review glucagonoma. They note the presentation and symptoms associated with a glucagonoma, including the classic rash and elevated blood glucose. They recommend considering this diagnosis in patients who present with rash and hyperglycemia refractory to typical treatments. Incorrect Answers: Answer B: Beta-cell destruction is the pathophysiology of type I diabetes mellitus which presents with polydipsia, polyuria, weight loss, and hyperglycemia (typically in a pediatric patient). It can progress to diabetic ketoacidosis which presents with hyperglycemia, an anion gap acidosis, altered mental status, and Kussmaul respirations. Answer C: Beta-cell tumor describes an insulinoma, which would present with hypoglycemia refractory to glucose administration. This condition is caused by increased release of insulin. The diagnosis should be suspected in the setting of hypoglycemia, an elevated insulin level, and an elevated C-peptide level. Imaging of the abdomen/pancreas with an MRI will reveal the insulin-secreting mass. Answer D: Hypercortisolism can cause Cushing syndrome which presents with hyperglycemia, obesity, striae, limb/muscle atrophy, mood changes, and hypertension. A buffalo hump and moon faces may also be seen. This condition can either be from endogenous ACTH production or cortisol production or can be from exogenous steroid use. Answer E: Insulin resistance is the pathophysiology of type 2 diabetes mellitus and presents with obesity, hyperglycemia, and an elevated hemoglobin A1c. The mainstay of management is weight loss, though medications such as metformin, sulfonylureas, or insulin are often needed. Bullet Summary: A glucagonoma is a tumor of the alpha cells of the pancreas that hypersecretes glucagon.
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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
Vagal event
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.
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A 30-year-old man presents to the emergency department with diarrhea. He states he has had profuse, watery diarrhea for the past 24 hours. He notes that eating and drinking make him defecate more, thus he has been refraining from drinking. He is otherwise healthy and takes no medications. His temperature is 97.7°F (36.5°C), blood pressure is 84/64 mmHg, pulse is 130/min, respirations are 17/min, and oxygen saturation is 99% on room air. Physical exam reveals dry mucous membranes with an otherwise benign exam. The patient is given 1L of normal saline and drinks 1L of oral fluids. His blood pressure is subsequently 120/70 mmHg and pulse 95/min. Which of the following is the most appropriate next step in management?
Ciprofloxacin
Rest and oral rehydration
Stool culture
Stool ova and parasite study
Stool PCR
B
Rest and oral rehydration
This patient is presenting with a likely diarrheal illness given he has profuse, watery diarrhea and his vitals normalized with oral and IV hydration. Since the patient is young and healthy, oral hydration and rest are all that is needed for management. Diarrheal illness is a common chief complaint with variable workups pending on the patient's clinical status. Young, healthy, non-immunosuppressed patients who can tolerate an oral diet require only reassurance and oral hydration, as most cases of viral and bacterial diarrhea will resolve on their own. Moreover, most cases of diarrhea are caused by viral etiologies which simply require supportive therapy. This is even true in bacterial diarrhea as long as the patient's vitals normalize and they can tolerate an oral diet. It is important to consider inflammatory causes of diarrhea (such as ulcerative colitis or Crohn disease) as this would change the workup and management. Schiller et al. discuss different types of diarrhea. They review the management and diagnosis of diarrhea and its different forms, including chronic forms. They recommend noting the difference between different causes and working up and treating them appropriately. Incorrect Answers: Answer A: Ciprofloxacin is an antibiotic that can be used for bacterial/infectious diarrhea. It should not be given in young, healthy patients and would only be indicated in the setting of bacterial diarrhea in ill patients, patients with persistent symptoms, or patients who are immunosuppressed. Bacterial diarrhea would present with fever, abdominal pain, and bloody/purulent diarrhea. Answer C: Stool culture should not be empirically performed in young, well-appearing patients. It may be performed in hospitalized patients who are more ill or in patients who are going to start antibiotic therapy. It may also be performed in patients with persistent diarrhea. Answer D: A stool ova and parasite study would not be performed unless there was a suggestive history or exposure (such as drinking unfiltered water) in the setting of fatty, foul-smelling diarrhea. This would raise suspicion for Giardia lamblia which would be treated with metronidazole. Answer E: Stool PCR for viral etiologies of diarrhea is not useful as the management of viral diarrhea is supportive therapy in most cases. On occasion, it may be performed in critically ill patients to determine the etiology of their symptoms. It should not be performed routinely. Bullet Summary: Healthy patients with limited diarrheal illness require supportive therapy only (in particular, oral hydration) and do not need more invasive diagnostic tests.
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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
Staphylococcus aureus
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.
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Figure A is the lateral radiograph of a 44-year-old male who sustained a fall on his outstretched hand while rollerblading. The patient reports pain localized to the posterior elbow and refuses to attempt elbow extension secondary to pain and swelling. Which of the following is the most likely diagnosis in this patient?
Exacerbation of elbow arthritis
Triceps rupture
Olecranon bursitis
Lateral collateral ligament tear
Anconeus avulsion fracture
B
Triceps rupture
The flake sign, as seen in Figure A is pathognomonic of a triceps tendon rupture. Tricep tendon ruptures are rare injuries to the elbow extensor mechanism that most commonly occur as a result of forceful elbow contraction. Diagnosis can be made clinically, as patients with complete ruptures are unable to extend the elbow against gravity. An MRI may be useful in the diagnosis in patients whom are too painful to examine or who may have partial tears of the triceps tendon. Surgical repair is indicated in patients with complete rupture or in those with >50% tears and significant weakness. Yeh et al. review distal triceps ruptures. They report that these injuries are commonly caused by a fall on an outstretched hand or a direct blow. If identifiable on plain radiograph a flake sign or avulsion of the olecranon tip will be appreciated. They conclude that diagnosis may be difficult initially in a painful elbow, however, a combination of plain radiographs and MRI can assist in making the correct diagnosis. Beazley et al. review distal biceps and triceps injuries. They report that these injuries most commonly occur in middle-aged males as a result of eccentric loading of the tendon. They conclude that patients who undergo early diagnosis and surgical treatment can expect to have excellent outcomes. Keener et al. review distal tricep tendon injuries. They report that most complete tears are treated surgically in medically fit patients. Partial-thickness tears are managed according to the tear severity, functional demands, and response to conservative treatment. They conclude that anatomic footprint repair of the triceps with optimal tendon to bone healing results in an acceptable functional outcome. Figure A is the lateral radiograph of an elbow demonstrating a flake sign from the olecranon tip indicative of a triceps rupture. Incorrect Answers: Answer A: The patient does not show significant elbow degenerative disease on plain radiographs. Answer C: Olecranon bursitis is not likely to worsen acutely after a fall, nor are there any other indications of this diagnosis in the present patient. Answer D: While lateral collateral ligament tears may occur following a fall on an outstretched arm, there is no indication that an elbow dislocation occurred here and based on current radiographs a triceps rupture is more likely. Answer E: Anconeus avulsions fractures are identified as lateral epicondyle fractures.
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A 46-year-old woman presents to her primary care doctor to request a referral to ophthalmology. Her vision has been steadily declining over the past 2 years and she thinks she needs a prescription for glasses. She further complains that her lips and feet feel numb. Her medical history is notable for medullary thyroid cancer status post total thyroidectomy. Her periods are regular. She enjoys a diverse diet and takes levothyroxine. Her blood pressure is 110/70 mmHg, pulse is 80/min, and respirations are 12/min. She is alert and oriented. Her pupils are equal, round, and reactive to light, but appear opacified. Extraocular movements are intact, albeit slow. Her visual acuity is decreased bilaterally. The remainder of her physical exam is unremarkable. Her basic metabolic panel is shown below: Na+: 139 mEq/L Cl-: 100 mEq/L K+: 4.9 mEq/L HCO3-: 25 mEq/L BUN: 10 mg/dL Glucose: 110 mg/dL Creatinine: 0.8 mg/dL Thyroid-stimulating hormone: 1.5 µU/mL Ca2+: 7.0 mEq/L Phosphorus: 6.5 mEq/L Albumin: 3.6 mg/dL The patient's ECG is shown in Figure A. What is the most likely cause of this presentation?
Hyperkalemia
Iatrogenic hypoparathyroidism
Iatrogenic hypothyroidism
Multiple endocrine neoplasia
Somatization
B
Iatrogenic hypoparathyroidism
This patient who presents with cataracts and a prolonged QTc interval on ECG most likely has symptomatic hypocalcemia secondary to the surgical removal of all of her parathyroid glands (iatrogenic hypoparathyroidism). Thyroid surgery may mandate the removal of parathyroid glands if there is evidence of malignant invasion. One parathyroid gland is enough to maintain eucalcemia, but accidental/intentional removal of all the glands is possible. Less common causes are autoimmune parathyroid destruction or parathyroid infiltration by disorders like sarcoidosis or Wilson disease. Many patients are asymptomatic, but chronic hypocalcemia is associated with increased phosphate levels, prolonged QT interval, Parkinsonian signs, cataracts, and cognitive impairment. Severe, acute hypocalcemia can present with tetany, papilledema, and seizures. Treatment is with calcium and parathyroid hormone replacement. Clarke et al. review the evidence regarding the diagnosis and etiologies of hypoparathyroidism. They discuss how the most common cause is post-surgical. They recommend obtaining genetic testing in cases where the etiology is unclear. Figure/Illustration A shows an ECG with a prolonged QTc interval at approximately 500 msec (blue circle). Incorrect Answers: Answer A: This patient is mildly hyperkalemic, but her symptoms and signs are not associated with hyperkalemia. Patients with hyperkalemia present with heart palpitations, shortness of breath, chest pain, nausea, or vomiting. ECG will demonstrate peaked T waves. Answer C: The patient is on levothyroxine with appropriate TSH levels. Hypothyroidism is not associated with QT prolongation. Patients with hypothyroidism will present with cold intolerance, delayed responsiveness, and myxedema coma. Treatment is with increasing the levothyroxine dose. Answer D: Multiple endocrine neoplasia is associated with medullary thyroid cancer, but this patient has no evidence of additional organ disease. Other signs of multiple endocrine neoplasia include pancreatic tumors, pheochromocytoma, or a Marfanoid habitus. Treatment is with surgical excision of tumors. Answer E: This patient most likely has a biological reason for her symptoms, so somatization is less likely. Patients with somatizing disorders have psychological distress that manifests as physical symptoms. These patients will not have organic correlates of disease such as abnormal ECG results. Bullet Summary: Iatrogenic hyperparathyroidism after the removal of the thyroid gland is the most common cause of hypocalcemia.
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A 35-year-old G0P0000 woman presents to her gynecologist with complaints of irregular menstruation. She has had only 2 periods in the last year. She feels flushed without provocation and is experiencing occasional dyspareunia with post-coital spotting. She has also had more frequent headaches than usual. The patient has a medical history of Hashimoto thyroiditis and takes levothyroxine daily. Her mother has type 1 diabetes mellitus. Her temperature is 98.5°F (36.9°C), pulse is 70/min, blood pressure is 118/76 mmHg, and respirations are 13/min. Cardiopulmonary and abdominal exams are unremarkable. The patient has Tanner 5 breasts and pubic hair. A pelvic exam reveals a normal cervix, an anteverted uterus without tenderness, and no adnexal masses. The following laboratory studies are performed: Serum: Thyroid stimulating hormone (TSH): 28 µIU/mL (9-30 µIU/mL) Cycle day 3 follicle stimulating hormone (FSH): 49 mIU/mL (4.7-21.5 mIU/mL) Cycle day 3 estradiol: 8 pg/mL (27-123 pg/mL) Prolactin: 14 ng/mL (4-23 ng/mL) Testosterone: 42 ng/dL (15-70 ng/dL) Which of the following is the most appropriate next step in management?
Brain MRI
Combined oral contraceptive
Estradiol patch with oral medroxyprogesterone
Increase levothyroxine dose
Vaginal estradiol gel
C
Estradiol patch with oral medroxyprogesterone
This patient presents with oligomenorrhea, hot flashes, and dyspareunia, and her labs are notable for a high FSH and low estradiol, most consistent with premature ovarian insufficiency/failure (POI). The most appropriate next step in management is an estradiol patch with oral medroxyprogesterone. POI is defined by the depletion or dysfunction of ovarian follicles, resulting in oligomenorrhea or amenorrhea with symptoms of menopause (hot flashes, vaginal atrophy and dryness, mood swings, etc.) in women under age 40. The cause is unclear and thought to be autoimmune. The main diagnostic markers are a high FSH and low estradiol at the start of the follicular phase. Patients require estrogen supplementation until around age 50, as low estrogen increases the risk of coronary artery disease, osteoporosis, and sexual dysfunction. Vaginal gel or a transdermal patch is the first-line option, but both must be used in conjunction with medroxyprogesterone to avoid unopposed estrogen and the risk of endometrial hyperplasia. Tsiligiannis et al. review the evidence regarding the outcomes of patients with POI. They discuss how this diagnosis is made in patients younger than the age of 40. They recommend closely managing the cardiovascular risk factors associated with this disease. Incorrect Answers: Answer A: Brain MRI would be useful for evaluating for the presence of a prolactinoma, which could cause the headaches and oligomenorrhea seen in this patient. However, her prolactin level is normal, and she has no visual disturbances (e.g., bitemporal hemianopsia), making this study less relevant. Answer B: The combined oral contraceptive has both estrogen and progesterone, but the doses are higher than those needed for hormone replacement. For this reason, it is generally not considered first-line for the treatment of POI. Answer D: Increasing this patient’s levothyroxine dose is unnecessary, as her TSH level is within the normal range. Furthermore, while hypothyroidism may cause menstrual irregularities and temperature dysregulation, it is unlikely to cause vaginal dryness and subsequent dyspareunia. Answer E: Vaginal estradiol gel is an effective method of estrogen replacement in women with POI. However, used alone it may increase the risk of endometrial hyperplasia and cancer, so it should only be used in combination with a systemic progesterone. Bullet Summary: Premature ovarian insufficiency should be treated with estrogen and progesterone replacement.
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A 27-year-old man presents to his primary care physician for exposure to toxic materials. The patient states that when he left for work this morning he was certain that he had closed the door to his pantry. Upon returning home, he saw that the door to his pantry was wide open. The patient is certain that his neighbors have been tampering with his food and potentially poisoned him. He further states that he knows they have been trying to break into his house and steal his things. He has tried multiple times to get them evicted from the building to no avail. It is for this reason that he is certain that they are trying to get their revenge upon him. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. The physician performs a physical exam and tells the patient that he thinks there is nothing to be concerned about, but that he should call him or come into the office if he experiences any symptoms. The patient is outraged at this news and requests a competent doctor who is not colluding with his enemies. He storms out of the office angrily, stating that he deserves the best in medical care. Which of the following is the most likely disorder that this patient is suffering from?
Borderline personality disorder
Intermittent explosive disorder
Narcissistic personality disorder
Paranoid personality disorder
Schizoid personality disorder
D
Paranoid personality disorder
This patient is presenting with a history of mistrust and suspicion of others without frankly psychotic features suggesting a diagnosis of paranoid personality disorder. Paranoid personality disorder is a cluster A personality disorder that is genetically associated with schizophrenia. These patients present with a pervasive mistrust of others based on little or no evidence. These patients are inclined to believe others have bad intentions that are directed toward them. They are often odd, very emotionally cold, and typically are involved in frequent litigations. Patients can be hard to manage though they often benefit from cognitive behavioral therapy. Anxiolytics may also be helpful in calming these patients. Triebwasser et al. review the evidence regarding the diagnosis of paranoid personality disorder. They discuss how there is relatively little research on these patients because it is difficult to recruit them for studies. They recommend considering the removal of this diagnosis and replacing it with a domain of paranoia. Incorrect Answers: Answer A: Borderline personality disorder presents with emotional instability, unstable relationships, and recurrent self-harm behaviors. These patients will typically demonstrate the defense mechanism of splitting (seeing things as all good or all bad). Dialectical behavioral therapy can be used in order to help these patients mitigate self-injurious behaviors. Answer B: Intermittent explosive disorder presents with sudden episodes of aggression out of proportion to the stressor. This patient’s response is a result of his paranoia rather than an excessive response to reasonable stimuli. Cognitive behavioral therapy can be used in order to help these patients control their emotional outbursts. Answer C: Narcissistic personality disorder presents with a grand sense of self-importance and preoccupation with success and admiration. These individuals are typically selfish and lack empathy. Though this patient’s demand for the best medical care may be reflective of narcissistic personality disorder, the majority of his behaviors point more toward a diagnosis of paranoid personality disorder. Answer E: Schizoid personality disorder presents in patients with isolated and emotionally restricted behavior. These patients are typically alone but enjoy being alone and often seem disinterested in others. These patients typically do not seek relationships and do not seek therapy as they are not bothered by their behaviors. Bullet Summary: Paranoid personality disorder presents with mistrustful behavior, suspicion of others, oddness, emotional coldness, and they are typically involved in many litigations.
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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?
Diaphoresis
Jaundice
Miosis
Urinary retention
Wheezing
D
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).
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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
Colonoscopy
Helicobacter pylori stool antigen test
Trial of lansoprazole
Upper endoscopy
E
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.
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A 10-year-old girl is admitted to the medical floor for a respiratory infection. The patient lives in a foster home and has been admitted many times. Since birth, the patient has had repeated episodes of pain/pressure over her frontal sinuses and a chronic cough that produces mucus. She was recently treated with amoxicillin for an infection. The patient is in the 25th percentile for height and weight which has been constant since birth. Her guardians state that the patient has normal bowel movements and has been gaining weight appropriately. The patient has a history of tricuspid stenosis. She also recently had magnetic resonance imaging (MRI) of her chest which demonstrated dilation of her airways. Her temperature is 99.5°F (37.5°C), blood pressure is 90/58 mmHg, pulse is 120/min, respirations are 18/min, and oxygen saturation is 94% on room air. Physical exam is notable for bruises along the patient's shins which the guardians state are from playing soccer. The rest of the exam is deferred because the patient starts crying. Which of the following findings is associated with this patient's most likely underlying diagnosis?
Diastolic murmur best heard along the right lower sternal border
Hypocalcemia
Increased chloride in the patient's sweat
Repeat sinus infections secondary to seasonal allergies
Social withdrawal and avoidance of eye contact
A
Diastolic murmur best heard along the right lower sternal border
This patient is presenting with recurrent sinus infections suggestive of Kartagener syndrome. Dextrocardia is a common feature in this pathology, which results in right-sided heart sounds or a diastolic murmur best heard along the right lower sternal border for this patient's tricuspid stenosis. Kartagener syndrome (primary ciliary dyskinesia) occurs secondary to a genetic defect in a patient's cilia. This results in a clinical picture of recurrent sinusitis, recurrent upper respiratory infections, bronchiectasis, and situs inversus (all major organs are a mirror image of where they should be). A physical exam will reveal right-sided heart sounds and a chest radiograph will demonstrate dextrocardia. Treatment is with respiratory support, assistance with mucociliary clearance, and treatment of infections. Leigh et al. review the evidence regarding the manifestations of Kartagener syndrome. They discuss how about 50% of patients have situs inversus. They recommend respiratory support for these patients given the overlap with other mucociliary clearance disorders such as cystic fibrosis. Incorrect Answers: Answer B: Hypocalcemia would be seen in DiGeorge syndrome which presents with CATCH-22 (Cleft palate/lip, Abnormal facies, Thymic aplasia, Cardiac defects, Hypocalcemia, and chromosome 22 deletion). Treatment is with calcium repletion, surgical treatment of cardiac defects, and treatment of infections. Answer C: Increased chloride in the patient's sweat describes cystic fibrosis which would present with weight loss, failure to thrive, and fatty/foul-smelling stools. Treatment is with respiratory support, assistance with mucociliary clearance, and treatment of infections. Answer D: Repeat sinus infections secondary to seasonal allergies describe sinusitis, which may be present in this patient but does not explain the entire clinical picture. Treatment is with antihistamines. Answer E: Social withdrawal and avoidance of eye contact describe child abuse which seems unlikely in this case given the constellation of symptoms that suggest Kartagener syndrome. Bullet Summary: Kartagener syndrome presents with recurrent sinusitis, bronchiectasis, and situs inversus.
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A 4-day-old boy presents to the pediatrician with his mother for his 1st well visit. The patient was born at 36 weeks gestation to a 26-year-old primigravid mother via cesarean section for cervical incompetence. The patient required no resuscitation at birth and both mother and child were discharged from the hospital at 2 days of life. The patient has been exclusively breastfed since birth, and his mother reports that he feeds for 20-30 minutes every 2 hours. The patient urinates 7 times per day and has begun passing 2-3 stools per day that his mother describes as “grainy” and the color of “mustard.” His birth weight was 3670 g (8 lb 1 oz), and his current weight is 3487 (7 lb 11 oz). His temperature is 97.1°F (36.2°C), blood pressure is 57/42 mmHg, pulse is 140/min, and respirations are 38/min. On physical exam, the patient’s anterior fontanelle is soft and flat, and his eyes are moderately icteric. His abdomen is soft and non-distended. The patient has the physical exam finding seen in Figure A. His diaper can be seen in Figure B. Laboratory studies reveal the following: Total bilirubin: 13 mg/dL Conjugated bilirubin: 0.6 mg/dL Which of the following is the most appropriate next step in management?
Administer intravenous hydration
Continue current breastfeeding regimen
Order serum uric acid concentration
Order spot uric acid to creatinine ratio
Recommend increasing frequency of breastfeeding
B
Continue current breastfeeding regimen
This patient presents at day 4 of life with weight loss of 5% of his birth weight, uric acid crystals in his diaper, desquamation of the palms and soles, and unconjugated hyperbilirubinemia, which are normal findings in a newborn. The most appropriate next step in management is continuing his current breastfeeding regimen. Healthy newborns may lose up to 7% of their birth weight in the first 5 days of life and exhibit dry, peeling skin on the palms and soles as the skin adapts to a drier environment outside the womb. Uric acid crystals that look like “brick dust” are also a normal finding in the diaper during the 1st week of life, as uric acid excretion is highest at birth. All newborns should be evaluated for signs of dehydration, including sunken fontanelles, dry mucous membranes, low urine output, excessive weight loss, and tachycardia. If these findings are not present, continued breastfeeding should be encouraged. Westerfield et al. review the evidence regarding the benefits of breastfeeding. They discuss how breastfeeding is associated with decreased risk of atopic dermatitis and gastroenteritis. They recommend ensuring proper breastfeeding techniques. Figure A demonstrates flaking and peeling of the soles (red circles), which is a normal finding in the 1st week of life. Figure B demonstrates uric acid crystals (“brick dust”) in the diaper (red circle), which is another normal finding that is caused by high uric acid excretion in the 1st few days of life. Incorrect Answers: Answer A: Administering intravenous hydration would be appropriate for a severely dehydrated neonate who cannot tolerate hydration orally. This patient is not exhibiting signs of dehydration. Answer C: Ordering a serum uric acid concentration may be part of an evaluation for Lesch-Nyhan syndrome, an X-linked recessive disorder of purine metabolism caused by deficiency of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HGPRT) that causes hyperuricemia and hyperuricosuria. The uric acid crystals found in this patient’s diapers are a normal finding in the 1st week of life. Treatment is supportive including allopurinol for gout. Answer D: Ordering a spot uric acid to creatinine ratio would be an appropriate first step in evaluation for Lesch-Nyhan syndrome, but the uric acid crystals in this patient’s diaper are not concerning for Lesch-Nyhan syndrome. Treatment is supportive as well as with allopurinol for gout. Answer E: Recommending increasing the frequency of breastfeeding would be appropriate for a dehydrated infant with excessive weight loss, but this patient’s weight loss is within the expected limits for the 4th day of life. Bullet Summary: Healthy newborns may present with desquamation of the skin and uric acid crystals in the diaper and continued breastfeeding should be encouraged if no signs of dehydration are present.
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A 32-year-old G1P0 presents to the antenatal testing unit at 32 weeks of gestation for a non-stress test (NST). Yesterday she had several episodes of diarrhea and vomited once after attending a company picnic 2 days ago. She took loperamide with symptomatic relief. She is worried that she is dehydrated but otherwise feels well and has recovered from her gastrointestinal illness. The patient was diagnosed with gestational diabetes after routine screening in the 2nd trimester and has been started on insulin after unsuccessful attempts at lifestyle management. She also has migraines and gastroesophageal reflux disorder. The patient has a 15-pack-year smoking history but quit smoking in her 1st month of pregnancy. She denies alcohol or drug use. Her temperature is 98.5°F (36.9°C), blood pressure is 122/82 mmHg, pulse is 84/min, and respirations are 14/min. A representative portion of the NST is shown in Figure A. Which of the following is the most likely explanation for this patient’s non-stress test (NST) results?
Fetal sleep cycle
Maternal dehydration
Maternal medication use
Maternal smoking
Normal results
A
Fetal sleep cycle
This patient presents with a nonreactive NST on routine testing. The most common explanation for a nonreactive NST is the fetal sleep cycle. Patients with gestational diabetes are at increased risk of fetal demise and other perinatal complications. They require closer monitoring during the 3rd trimester of pregnancy. The NST, which lasts 20 minutes, is the most common method of doing so. After 32 weeks, a reactive (i.e. “normal”) NST requires at least 2 episodes of 15-beat-per-minute accelerations lasting for at least 15 seconds. An NST with no accelerations is considered nonreactive. A common cause of a nonreactive NST is the fetal sleep cycle and it is reasonable to continue the test for an additional 20 minutes to increase the chance of monitoring the fetus while awake. Otherwise, a nonreactive NST should prompt vibroacoustic stimulation or further testing such as a biophysical profile (BPP). Umana and Siccardi review the evidence regarding the use of the NST during pregnancy. They discuss how the test is a measurement of fetal cardiovascular function at the time of the test. They recommend monitoring the baseline fetal heart rate and variability as well as the presence of accelerations and decelerations when interpreting test results. Figure/Illustration A shows a representative portion of a NST where the fetal heart rate remains within a narrow band without accelerations (red circle). This pattern is characteristically seen in a nonreactive NST that can be due to the fetal sleep cycle. Incorrect Answers: Answer B: Maternal dehydration generally should not affect the reactivity of an NST. Although severe dehydration may reduce amniotic fluid volume and therefore increase the baseline fetal pulse, it is unlikely to change the accelerations or variability in an NST. Though this patient had diarrhea the day before, her pulse is not elevated and any dehydration is probably not clinically significant. Answer C: Maternal medication use can cause nonreactivity on an NST if drugs such as morphine or other sedatives are taken. Although this patient did take loperamide (an opioid), it is unlikely that this has a sedative effect on the fetus as it acts selectively on the mu-opioid receptors in the large intestine and does not cross the blood-brain barrier. Answer D: Maternal smoking in close proximity to an NST has been linked to nonreactivity, but this patient has not smoked in several months. Mothers who smoke are counseled to avoid smoking on the day of their NST to avoid the effects of nicotine on their test results. Answer E: A normal result is known as a reactive NST and would present with accelerations and no concerning or late decelerations. This is not a reactive NST due to the lack of appropriate accelerations. Bullet Summary: The most common cause of a non-reactive non-stress test is the fetal sleep cycle.
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A 25-year-old man presents to his primary care physician with a chief complaint of "failing health." He states that he typically can converse with animals via telepathy but is having trouble right now due to the weather. He has begun taking an assortment of Peruvian herbs to little avail. Otherwise, he is not currently taking any medications. The patient lives alone and works in a health food store. He states that his symptoms have persisted for the past 8 months. When obtaining the patient's medical history, there are several times he attempts to telepathically connect with the animals in the vicinity. 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, you note a healthy young man who is dressed in an all-burlap ensemble. Which of the following is the most likely diagnosis?
Brief psychotic disorder
Schizoid personality disorder
Schizophrenia
Schizophreniform disorder
Schizotypal personality disorder
E
Schizotypal personality disorder
This patient has several odd and eccentric beliefs without frankly psychotic features and he is able to function in society. This suggests a diagnosis of schizotypal personality disorder. Schizotypal personality disorder presents with eccentric behavior, magical thinking, odd beliefs, and perceptual disturbances. These patients may seem to have symptoms of schizophrenia; however, they do not respond to internal stimuli and do not experience visual or auditory hallucinations. They are able to function in society and care for themselves. Patients with schizotypal personality disorder typically struggle in maintaining close relationships or making friends. Patients are typically not distressed about their thought patterns but can undergo treatment with psychotherapy if they seek to change their behaviors. Sher reviews the evidence regarding the treatment of patients with schizotypal personality disorder. He discusses how this disorder often goes undiagnosed and is difficult to treat. He recommends providing suicide prevention measures as this disease is associated with suicidal ideation. Incorrect Answers: Answer A: Brief psychotic disorder presents with either visual or auditory hallucinations with symptoms that have lasted for less than 1 month. Patients have frankly psychotic features and should be closely monitored for the development of schizophrenia. Answer B: Schizoid personality disorder presents with social isolation. These patients are alone but do not desire social contact and are content in their isolation. A typical example is an individual who works in a solitary job on the graveyard shift. Patients do not have strange or magical beliefs. Answer C: Schizophrenia presents with either visual or auditory hallucinations with symptoms that have lasted for 6 months or longer. These patients typically have trouble caring for themselves and struggle to function in society. Though this patient is attempting to connect/communicate with animals, this likely represents an odd belief rather than responding to internal stimuli. Schizophrenia should be treated with antipsychotic medications. Answer D: Schizophreniform disorder presents with either visual or auditory hallucinations with symptoms that have lasted for 1-6 months. Patients have symptoms with frankly psychotic features and should be treated with antipsychotic medications. Bullet Summary: Schizotypal personality disorder presents with eccentric behavior, magical thoughts, and odd beliefs but patients are able to function within society.
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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
Renal failure
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.
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A 25-year-old man is brought to the emergency department by paramedics for altered mental status. He was found lying down at a bus stop unresponsive. On arrival, the patient has slurred speech and is somnolent. Further is unable to be elicited. His temperature is 98.0°F (36.7°C), blood pressure is 130/80 mmHg, pulse is 80/min, and respirations are 12/min with a saturation of 97% on room air. Physical exam reveals a sleeping man with miotic pupils. The patient is easily arousable to voice and sternal rub but falls asleep rapidly. He has decreased bowel sounds. He is able to state his name and knows that he is at the hospital and has an otherwise normal neurologic exam. Which of the following is the most appropriate management?
Atropine
Disulfiram
Flumazenil
Naloxone
Observation
E
Observation
This patient's presentation with altered mental status, pinpoint pupils, and decreased bowel sounds is most consistent with an opioid overdose. Given his stable respiratory status and oxygen saturation without notable bradypnea, observation alone until the patient is clinically sober is the most appropriate management. Opioids are commonly used to treat painful conditions but have the potential to become drugs of abuse. Opioid overdose presents with bradypnea, somnolence, decreased bowel sounds, and pinpoint pupils. Any patient who is not protecting their airway, failing to oxygenate, or failing to ventilate should immediately be given naloxone which rapidly reverses the effect of opioids. However, this precipitates withdrawal which is unpleasant for patients and leads to nausea, vomiting, agitation, and diaphoresis among other findings. For this reason, patients who have overdosed on opioids and are stable can be observed until clinically sober. Dietze et al. studied the effect of intranasal versus intramuscular naloxone for opioid overdose in a randomized control trial. The authors found that patients who received intranasal naloxone needed a second dose of naloxone more frequently compared to patients who received intramuscular naloxone. The authors recommend further study of optimal dosing and concentration of intranasal naloxone to respond to opioid overdoses outside of the hospital. Incorrect Answers: Answer A: Atropine is an appropriate treatment for a cholinergic toxidrome. This would manifest with bronchorrhea, bronchospasm, bradycardia, urinary incontinence, diarrhea, and drooling. Atropine should be administered until respiratory symptoms improve; large doses may be required. Answer B: Disulfiram inhibits the enzyme acetaldehyde dehydrogenase causing severe nausea, vomiting, and a very ill feeling when a patient drinks alcohol from the accumulation of byproducts. This drug is a second-line agent used to reduce drinking in chronic alcohol use disorder but has no indication in managing acute alcohol intoxication as it is a preventive medication. Answer C: Flumazenil is the reversal agent for benzodiazepines. Its use has fallen out of favor as it can precipitate seizures in chronic benzodiazepine users. Any patient with a suspected benzodiazepine overdose who is protecting their airway should be observed until clinically sober as there is no need to rapidly reverse them and possibly cause seizures. Answer D: Naloxone is the antidote of choice in opioid overdose in patients who are not adequately oxygenating, ventilating, or protecting their airway. It is not indicated in opioid overdose in a clinically stable patient as the patient can merely be observed without the unpleasant side effects of immediate withdrawal from naloxone (including vomiting which in some cases could lead to aspiration). This patient is oxygenating well, protecting his airway, and does not have profound bradypnea. Bullet Summary: Opioid overdose presents with somnolence, pinpoint pupils, decreased bowel sounds, and bradypnea; stable patients who are adequately breathing and protecting their airway require only observation until clinically sober.
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A 32-year-old man presents to the emergency department with pain upon defecation. His symptoms started a few weeks ago but have been worsening. Sometimes he notes blood on the toilet paper. He has to strain to have bowel movements and has trouble defecating secondary to pain. He is generally healthy and does not smoke. The patient is a software engineer and enjoys hiking, going to nightclubs, and dancing. His temperature is 98.1°F (36.7°C), blood pressure is 112/83 mmHg, pulse is 83/min, respirations are 13/min, and oxygen saturation is 98% on room air. Physical exam reveals a small, superficial fissure that is lateral to the anus and lateral to the midline. Which of the following is the most appropriate management for this patient?
CT scan of the abdomen and pelvis
Incision, drainage, ciprofloxacin, and metronidazole
Surgical excision
Testing for HIV, fecal occult blood, and sexually transmitted diseases
Topical lidocaine, sitz baths, and fiber supplements
D
Testing for HIV, fecal occult blood, and sexually transmitted diseases
This patient is presenting with pain upon defecation, constipation, and a fissure lateral to the midline which is suggestive of an anal fissure. Anal fissures lateral to the midline are associated with inflammatory and infectious etiologies; thus, further workup (testing for HIV, fecal occult blood testing, and sexually transmitted disease testing) is needed rather than merely symptomatic treatment. Anal fissures present with exquisite pain upon defecation which may lead to fecal retention and constipation. Commonly, anal fissures are caused by constipation, local trauma (such as anal intercourse), or a tight anal sphincter. Anal fissures that occur secondary to trauma are usually midline. On the other hand, lateral anal fissures are a harbinger of more serious alternative diagnoses and require further workup. Lateral anal fissures can be caused by infections (HIV being one of the most concerning as well as syphilis or tuberculosis), Crohn disease, granulomatous disease, malignancy, as well as foreign bodies. For this reason, lateral anal fissures should not only be treated symptomatically but require testing for sexually transmitted diseases, inflammatory conditions, and malignancy to determine the underlying etiology based on the patient’s risk factors and associated symptoms. Steele and Madoff review anal fissures. They note that while both operative and non-operative interventions may be indicated in the management of an anal fissure after alternative diagnoses have been ruled out, advances in medical management may spare patients sphincter-dividing surgery. They recommend considering all modalities. Incorrect Answers: Answer A: CT scan of the abdomen and pelvis followed by an MRI may be the most appropriate testing if a perirectal abscess is suspected. These abscesses can track very deep. For this reason, further imaging and drainage in the operating room followed by antibiotics are indicated rather than a simple bedside incision and drainage. Imaging may be indicated in this patient after medical workup fails to elucidate an etiology. Answer B: Incision, drainage, ciprofloxacin, and metronidazole may be indicated for a perianal abscess (note, this is not a perirectal abscess), which presents with a tender, fluctuant mass right next to the anus. The diagnosis can be made clinically or supported by an ultrasound. Answer C: Surgical excision may be performed in the management of a perianal fistula, which presents with a draining tract next to the anus that releases purulence or fecal contents causing staining of the patient’s underwear and irritation of the skin. The diagnosis is made with an exam and a CT, MRI, or fistulogram followed by a fistulotomy. Answer E: Topical lidocaine, sitz baths, and fiber supplements are the appropriate management of an anal fissure that is midline and secondary to a clear cause such as trauma or constipation. It would be inappropriate to only treat this patient symptomatically with a lateral anal fissure as this is a sign of a more serious diagnosis. Bullet Summary: Anal fissures that are lateral to the midline require workup for sexually transmitted infections, cancer, and autoimmune disease.
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A 31-year-old man presents to the emergency department for fever, malaise, and cough. For the last week, his cough has been progressively worsening, and he has been noticing blood in his sputum. He denies any recent travel or sick contacts and infrequently smokes cigarettes. Approximately 5 weeks ago, he presented with similar symptoms and was found to have a right upper lobe lung infiltrate, a CD4 count of 40/mm^3, and an HIV viral load of 115,000 copies/mL. He was appropriately treated and discharged home. Four weeks after initiation of treatment, his CD4 count was over 400/mm^3 and HIV viral load was negligible. His temperature today is 102°F (38.9°C), blood pressure is 130/90 mmHg, pulse is 100/min, and respirations are 20/min. A radiograph of the chest demonstrates new nodules in the left upper lobe and hilar adenopathy. Which of the following is the most appropriate next step in management?
Continue current anti-retroviral treatment, antimicrobial, and anti-tuberculoid therapies
Determine drug sensitivities against the patient’s pulmonary infection
Discontinue antimicrobial treatment
Obtain a D-dimer level
Start high-dose glucocorticoid treatment and discontinue antiretroviral therapy
A
Continue current anti-retroviral treatment, antimicrobial, and anti-tuberculoid therapies
This patient with recently diagnosed HIV and tuberculosis infection (upper lung lobe infiltrate) is presenting with worsening symptoms after being appropriately treated. Worsening of symptoms after initiation of these therapies is concerning for immune reconstitution inflammatory syndrome (IRIS), which is managed by continuing current combined antiretroviral medications, antibiotics for opportunistic infections and symptom management. IRIS describes a paradoxical worsening of a patient's underlying infection after initiating antiretroviral therapy for patients with HIV. IRIS can also occur in HIV-uninfected patients with tuberculosis or leprosy who are started on antimycobacterial treatment. The pathogenesis of IRIS is unclear, but in patients with HIV, antiretroviral therapy increases the amount of CD4+ T-helper cells, which can lead to an exaggerated inflammatory response (e.g., increased cytokine release) towards the infection. Thus, there is a paradoxical worsening of the infection symptoms. When patients have a paradoxical worsening of their symptoms in the setting of starting their antiretroviral therapy, the therapy should be continued with symptomatic management (acetaminophen for fever). Nelson et al review IRIS. They note it is seen in patients with HIV. They recommend antiretroviral therapy and antibiotics for bacterial infections. Incorrect Answers: Answer B: Determining drug sensitivities against the patient’s pulmonary infection would be of low diagnostic utility since IRIS best explains his current clinical presentation. Answer C: Discontinuing antimicrobial treatment would not be appropriate since the patient has findings concerning for tuberculosis (upper lobe lung infiltrate), and lack of treatment can lead to progression of his infection. Answer D: Obtaining a D-dimer level is only indicated in low-risk patients for pulmonary embolism by Wells criteria. Answer E: Starting high-dose glucocorticoid treatment and discontinuing antiretroviral therapy would not be appropriate since discontinuing his antiretroviral therapy would not control his HIV infection, which would place him at greater risk of acquiring opportunistic infections. Corticosteroid use is part of symptom management as it decreases the inflammatory response. Bullet Summary: Immune reconstitution inflammatory syndrome (IRIS) can result in worsening of infectious symptoms and is managed by continuing antiviral therapy with symptomatic treatment.
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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
Renal failure
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.
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A 32-year-old man presents to his primary care provider for a headache. He reports that he has headaches at night several times a week. He first developed these headaches over 1 year ago, but they started up again 3 weeks ago. The episodes start suddenly and feel like a stabbing, electrical pain over his left eye. He also reports tearing of the left eye during these episodes. The headaches self-resolve over the course of 2-3 hours, but the patient complains that he is avoiding going to sleep for fear of waking up in pain. His medical history includes type 1 diabetes mellitus and an episode of herpes zoster on his right flank 1 year ago. His only home medication is insulin. His temperature is 98.6°F (37°C), blood pressure is 112/69 mmHg, pulse is 61/min, and respirations are 14/min. On physical exam, his extraocular muscles are intact and his eyes are not injected. A CT of the head and sinuses shows no acute abnormalities. Which of the following is most likely to prevent future episodes of headache in this patient?
Carbamazepine
High-flow oxygen
Sumatriptan
Topiramate
Verapamil
E
Verapamil
This patient presents with an episodic, stabbing pain in the periorbital region that occurs at night, which is consistent with a diagnosis of cluster headaches. Verapamil is used as prophylaxis against cluster headaches. Cluster headaches usually occur at night and may wake patients out of sleep. Cluster headaches may occur several times a week to multiple times daily and the attacks may be followed by a period of remission. Cluster headaches also typically involve the periorbital region and are unilateral. Patients also classically describe their headaches as “stabbing”. Tension-type headaches are more commonly described as dull and migraines are usually described as pulsating or throbbing. Treatment is with high-flow oxygen during acute episodes and prophylaxis against long-term headaches includes calcium channel blockers such as verapamil. Hoffman and May review the evidence regarding the treatment of cluster headaches. They discuss how high-flow oxygen and triptans are the most effective abortive treatment for acute attacks. They recommend using chronic prophylactic medications such as verapamil. Incorrect Answers: Answer A: Carbamazepine is the treatment of choice for trigeminal neuralgia, which presents as shooting pain in the face. They have exquisite tenderness to palpation over the affected neurologic distribution. Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in men with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye. This treatment is not effective for long-term prophylaxis. Answer C: Sumatriptan is used as an abortive treatment for cluster headaches and migraines, but it is not used as prophylaxis for either. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Answer D: Topiramate is used as prophylaxis for migraines rather than cluster headaches. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Bullet Summary: Cluster headaches classically present as sharp or stabbing periorbital headaches that occur at night, with verapamil being the prophylactic treatment of choice and high-flow oxygen being the most effective abortive treatment.
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A 62-year-old man presents to the emergency department with skin lesions. He felt itchy recently, then noticed skin lesions that broke out prompting him to come to the emergency department. He recently went camping and hiking in the woods. The patient drinks 1-2 beers per day and smokes 1 pack of cigarettes per day. He is currently sexually active with multiple partners and does not use condoms. The patient was discharged 1 week ago for pneumonia which was treated with azithromycin; however, he feels that his symptoms may be returning. His temperature is 99.5°F (37.5°C), blood pressure is 157/78 mmHg, pulse is 90/min, respirations are 15/min, and oxygen saturation is 98% on room air. Physical exam reveals the findings in Figure A on the patient's arm. The skin lesions are thick and do not break when pressure is applied. Which of the following most appropriately describes the most likely diagnosis?
IgG in a fish-net pattern
Linear IgG along the basement membrane
Multi-nucleated giant cells
Panniculitis
Type IV hypersensitivity reaction
B
Linear IgG along the basement membrane
This patient is presenting with pruritic bullous skin lesions with a thick/tough covering suggesting a diagnosis of bullous pemphigoid. Linear IgG binding of anti-hemidesmosome antibodies along the basement membrane is the underlying cause of this disease. Bullous pemphigoid presents with a urticarial prodrome in elderly patients followed by the formation of tense bullae that do not break when pressure is applied to them. IgG antibodies bind the hemidesmosome along the basement membrane and appear linear on immunofluorescence. Risk factors that are associated with this condition include the initiation of new medications such as loop diuretics, metformin, and neuroleptics. Steroids are the treatment of choice. Miyamoto et al. review the evidence regarding the diagnosis and treatment of bullous pemphigoid. They discuss how high-potency corticosteroids are the mainstay of treatment for this disease. They recommend considering adjuvant therapy with doxycycline, dapsone, and immunosuppressants. Figure/Illustration A is a clinical photograph showing bullous skin lesions (red circles). These lesions are tense and do not separate/break when pressure is applied to them and are classically seen in bullous pemphigoid. Incorrect Answers: Answer A: IgG in a fish-net pattern suggests a diagnosis of pemphigus vulgaris, which presents with skin lesions that are flaccid and break easily when pressure is applied. Treatment is with corticosteroids. Answer C: Multi-nucleated giant cells suggests a diagnosis of HSV or herpes zoster virus, which presents with potentially painful vesicles distributed in a dermatomal pattern (for zoster). Treatment is with antivirals such as valacyclovir. Answer D: Panniculitis suggests a diagnosis of erythema nodosum, which presents with painful, raised nodules that are associated with inflammatory disorders and infection. Treatment is centered on addressing the underlying pathology. Answer E: Type IV hypersensitivity reaction suggests a diagnosis of contact dermatitis, which presents with pruritic lesions after exposure to a particular antigen such as poison ivy, poison oak, or nickel. Treatment is avoidance of the precipitating agent and steroids as well as symptomatic control. Bullet Summary: Bullous pemphigoid occurs secondary to anti-hemidesmosome antibodies which appear linear along the basement membrane on immunofluorescence.
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A 45-year-old man presents to his primary care physician with a rash. He first noticed the rash 2 weeks prior and he endorses intermittent itchiness from the rash. His medical history is significant for chronic hepatitis C infection, hypertension, type 2 diabetes mellitus, and heart failure. The patient works as a butcher at a local meat processing shop and is sexually active with several partners. He does not use barrier protection. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 155/95 mmHg, and respirations are 12/min. On physical exam, his skin has the finding shown in Figure A. Fine, lace-like white lines are also noted on the patient’s buccal mucosa. Which of the following is the most likely diagnosis?
Atopic dermatitis
Lichen planus
Prurigo nodularis
Psoriasis
Scabies
B
Lichen planus
This patient presents with several purple, polygonal, pruritic papules and plaques. These findings are most consistent with a diagnosis of lichen planus. Lichen planus is an inflammatory skin disorder most commonly characterized by an eruption of Purple, Polygonal, Planar, Pruritic, Papules and Plaques (the 6 P’s), often on extensor surfaces such as the wrist. Development of fine white lines on the surfaces of skin lesions, as well as on the buccal mucosa (Wickham striae), are also characteristically seen in this disease. Concurrent hepatitis C infection is a risk factor for lichen planus. A skin biopsy will show a sawtooth infiltrate of lymphocytes at the dermal-epidermal junction. Treatment is with antihistamines for pruritus and topical steroids for localized disease as well as addressing the underlying cause. Tziotzios et al. review the evidence regarding the diagnosis and treatment of lichen planus. They discuss how the molecular basis of this disorder is relatively unknown. They recommend making a clear diagnosis to allow for treatment. Figure/Illustration A shows multiple purple, polygonal, papules and plaques on the wrist (red circles). These findings are characteristic of lichen planus. Incorrect Answers: Answer A: Atopic dermatitis most commonly presents with pruritic, lichenified plaques on the flexural surfaces. The mucous membranes are not involved. Treatment is with topical moisturizers and antihistamines for pruritus. This disease is also associated with other atopic conditions such as asthma and seasonal allergies. Answer C: Prurigo nodularis is characterized by hypertrophic, dome-shaped papules that develop secondary to chronic scratching. The mucous membranes are not involved. Treatment is with avoidance of the triggering behavior such as scratching and antihistamines for pruritus. Answer D: Psoriasis is characterized by silver, scaly plaques that bleed easily when the scale is physically removed (Auspitz sign). Patients with cutaneous psoriasis are at increased risk for the development of psoriatic arthritis. Psoriasis can be treated with topical corticosteroids as well as calcipotriene. Answer E: Scabies is a cutaneous infection with the mite Sarcoptes scabei that presents with severe pruritus, especially in the intertriginous areas. Patients may have serpiginous scaling lesions that represent the burrowing of the mite through the skin, but mucous membrane lesions are absent. Treatment can include topical permethrin first line. Bullet Summary: Lichen planus presents with the 6 P’s – Purple, Pruritic, Polygonal, Planar, Papules and Plaques.
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A 27-year-old woman presents to her primary care physician with new hair growth on her face and lower abdomen over the last month. She has started to develop pimples on her face and back over the last several months. Her last menstrual period was over 3 months ago and her periods have been irregular over the last year. She has been gaining weight recently. The patient has a medical history of obesity and prediabetes with a hemoglobin A1c of 6.0% last year. Her temperature is 98.5°F (36.9°C), pulse is 80/min, blood pressure is 139/88 mmHg, and respirations are 13/min. Cardiopulmonary exam is unremarkable, and the patient’s abdomen appears slightly distended but exhibits no tenderness to palpation. The patient’s face has coarse stubble along the jawline and on the upper lip, and there is similar hair along the midline of her lower abdomen. A pelvic exam reveals mild clitoromegaly, a normal anteverted uterus, and a large left adnexal mass that is mildly tender. Her laboratory test results are shown below: Hemoglobin: 13.9 g/dL Leukocyte count: 8,000 cells/mm^3 Platelet count: 142,000/mm^3 DHEAS: 73 ug/dL (Normal: 145-395 ug/dL) Testosterone: 256 ng/dL 17-hydroxyprogesterone: 214 ng/dL (Normal: < 200 ng/dL) Ultrasound findings are shown in Figure A. Which of the following is the most likely diagnosis in this patient?
Adrenal tumor
Congenital adrenal hyperplasia
Ovarian hyperthecosis
Ovarian tumor
Polycystic ovary syndrome
D
Ovarian tumor
This young woman presents with rapid virilization, elevated testosterone, and a large left adnexal mass that is primarily solid on ultrasound. These findings are most consistent with an ovarian androgen-secreting tumor. Sertoli-Leydig cell tumors are the most common androgen-secreting tumor of the ovaries and classically result in rapid virilization of affected women. Testosterone levels are often over 200 ng/dL, and 17-hydroxyprogesterone may also be slightly elevated as it is an androgen precursor. The androgen excess may result in symptoms such as facial and abdominal hirsutism, oligomenorrhea, deepening voice, and acne. Treatment involves staging the tumor and surgical resection of the tumor if possible. Durmus et al. review the evidence regarding the diagnosis and treatment of Sertoli-Leydig cell tumors. They discuss how this tumor generally has a favorable prognosis compared to malignant epithelial tumors. They recommend using fertility-preserving surgeries in younger patients. Figure/Illustration A shows an ultrasound of the left ovary, which has a predominantly solid mass with vascularity and some cystic spaces (red circle). These findings are consistent with an ovarian tumor. Incorrect Answers: Answer A: Adrenal tumors may secrete androgens and cause similar symptoms of virilization, but the presence of a malignant-appearing ovarian mass in this patient is more consistent with ovarian pathology. Furthermore, adrenal tumors typically secrete DHEAS, which can be a helpful test for localizing the source of androgen excess. Treatment is with surgical excision. Answer B: Congenital adrenal hyperplasia (CAH) results from one of several possible enzyme deficiencies in the steroid pathway and may appear later in life as virilization of a woman. While 17-hydroxyprogesterone elevation is a strong indicator of the most common subtype of CAH, this patient’s ultrasound findings suggest ovarian pathology instead, which could also elevate this androgen precursor. Treatment is with hormone replacement therapy. Answer C: Ovarian hyperthecosis is hyperplasia of the theca interna of the ovary, which produces androgens. Testosterone levels would be elevated and virilization may occur, but ultrasound findings would show a bilateral increase in stromal tissue as opposed to a unilateral solid mass. Treatment is with hormonal therapy. Answer E: Polycystic ovary syndrome (PCOS) can also cause elevated testosterone, though levels are typically lower than 100 ng/dL. Frank virilization with clitoromegaly and voice deepening would not be expected with PCOS, nor would an ovarian mass. Treatment is with metformin and lifestyle modifications. Bullet Summary: Sertoli-Leydig cell tumors of the ovary can produce androgens and result in rapid onset of virilization with elevated testosterone levels and an adnexal mass on ultrasound.
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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?
Attrition bias
Confounding variable
Late-look bias
Recall bias
Volunteer bias
A
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.
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A 62-year-old woman is brought to a physician by her husband for fluctuating changes in behavior and hallucinations over the past 2 years. On occasion, she becomes difficult to understand and her speech becomes disorganized. She also frequently sees "small people" and animals on the dining room table, which is disturbing to her. Her medical problems include hypertension and chronic obstructive pulmonary disease for which she takes amlodipine, albuterol, ipratropium, and a fluticasone inhaler. She was formerly an executive at a software company but had to retire due to frequent memory lapses. Her temperature is 98.6°F (37°C), blood pressure is 120/80 mmHg, pulse is 80/min, and respirations are 12/min. Physical exam is notable for slowed movements and difficulty with balance at rest, which were not present at her wellness visit 1 year ago. Which of the following pathological findings (Figures A-E) would most likely be seen if a brain biopsy were performed?
Figure A
Figure B
Figure C
Figure D
Figure E
C
Figure C
This patient with fluctuating changes in behavior and cognition, visual hallucinations, memory loss, and extrapyramidal symptoms (bradykinesia, postural instability) most likely has Lewy body dementia. Lewy body dementia is characterized histologically by Lewy bodies, which are intracytoplasmic eosinophilic inclusions of alpha-synuclein (shown in Figure C). Alpha-synuclein is normally abundantly present in its unfolded form in neurons. Abnormal folding of alpha-synuclein leads to aggregation into insoluble fibrils. These intracytoplasmic aggregates (Lewy bodies) are seen in Parkinson disease, Lewy body dementia, and multiple system atrophy, which are collectively referred to as alpha-synucleinopathies. Lewy body dementia is the second most common degenerative dementia and presents with fluctuating cognition, visual hallucinations, parkinsonism (bradykinesia, postural instability, rigidity, and a resting tremor), rapid eye movement (REM) sleep disorder, autonomic dysfunction, and sensitivity to antipsychotics. Lewy body dementia can be distinguished from Parkinson disease with dementia by the timing of symptoms. In Lewy body dementia, cognitive symptoms begin at the same time as or 1 year prior to the onset of movement symptoms whereas in Parkinson disease the cognitive symptoms classically occur over 1 year after motor symptoms. There is no cure for Lewy body dementia, and treatment is aimed at alleviating symptoms. Arnaoutoglou et al. reviewed the epidemiology, pathology, treatment, and future directions for research of Lewy body dementia. The authors found potential genetic bases for Lewy body dementia, including mutations in the GBA gene. They recommended that further research be conducted on the therapeutic implications of these genetic findings. Figure/Illustration A shows a hematoxylin and eosin (H&E) stained brain biopsy of a patient with Creutzfeldt-Jakob disease. Characteristic spongiform changes in neurons (yellow boxes) and agglomerated prions (orange arrows) are seen in this slide. Figure/Illustration B shows an H&E stained brain biopsy of a patient with a small lacunar infarct on low magnification. A central area of tissue loss (yellow outline) and surrounding residual gliosis (orange arrows) are seen in this slide. Figure/Illustration C shows a brain biopsy of a patient with Lewy body dementia stained with mouse anti-alpha-synuclein antibody and counterstained with hematoxylin. Lewy bodies (intracytoplasmic eosinophilic inclusions of alpha-synuclein) are indicated by orange arrows. Note that Lewy bodies are always found intracellularly (neurons highlighted by blue boxes). Figure/Illustration D shows an H&E stained brain biopsy of a patient with Alzheimer disease. Extracellular neurofibrillary tangles found in Alzheimer disease are indicated by black arrows. Figure/Illustration E shows an H&E stained brain biopsy of a patient with frontotemporal dementia. A characteristic Pick cell (red box), or ballooned neuron, with dissolved chromatin (yellow arrow), is seen in this slide. Incorrect Answers: Answer A: Figure A shows an H&E stained brain biopsy of a patient with Creutzfeldt-Jakob disease. Creutzfeldt-Jakob disease is a rapidly progressive dementia that manifests with poor concentration, declining executive function, startle myoclonus, 14-3-3 protein on lumbar puncture, and synchronous sharp wave complexes on electroencephalogram (EEG). Clinical decline occurs over the course of weeks and months, culminating in death within a year. Creutzfeldt-Jakob disease shows spongiform vacuolation due to the accumulation of abnormal prion protein (PrPsc). Answer B: Figure B shows an H&E stained brain biopsy of a patient with a small lacunar infarct. Lacunar infarcts may be asymptomatic or cause focal neurologic deficits such as motor hemiparesis, pure sensory stroke, ataxic hemiparesis, or dysarthria-clumsy hand syndrome depending on the territory involved. Lacunar strokes will show diffuse eosinophilia of the affected area, followed by liquefactive necrosis with neutrophilic infiltration, followed by macrophages with surrounding reactive gliosis, finally leading to an area of tissue loss with residual gliosis. Answer D: Figure D shows an H&E stained brain biopsy of a patient with Alzheimer disease. Alzheimer disease presents with a gradual cognitive decline with difficulty completing activities of daily living. Memory is the most prominently affected domain. Visual hallucinations, fluctuating levels of cognition, and extrapyramidal symptoms are not common in Alzheimer disease. Amyloid plaques and neurofibrillary tangles may be seen on brain biopsy. Answer E: Figure E shows an H&E stained brain biopsy of a patient with frontotemporal dementia (Pick disease). The behavioral variant of frontotemporal dementia is associated with early-onset personality and behavioral changes, and it may be associated with extrapyramidal symptoms. Unlike Lewy body dementia, memory is usually not affected in frontotemporal dementia until late in the disease course. frontotemporal dementia is characterized by Pick cells (ballooned neurons with dissolved chromatin) and Pick bodies, round intracellular aggregates of hyperphosphorylated tau protein. Bullet Summary: Lewy body dementia presents with alterations in consciousness, disorganized speech, visual hallucinations, extrapyramidal symptoms, early compromise of executive functions, and eosinophilic intracytoplasmic inclusions of alpha-synuclein (Lewy bodies) on biopsy.
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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
Vitamin B6 deficiency
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.
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A 23-year-old woman is admitted to the inpatient psychiatry unit after her boyfriend reported she was “acting funny and refusing to talk.” The patient’s boyfriend states that he came home from work and found the patient sitting up in bed staring at the wall. When he said her name or waved his hand in front of her, she did not respond. When he tried to move her, she would remain in whatever position she was placed. Her temperature is 99°F (37.2°C), blood pressure is 122/79 mmHg, pulse is 68/min, and respirations are 12/min with an oxygen saturation of 98% on room air. The patient is lying on the bed with her left arm raised and pointing at the ceiling. She resists any attempt to change her position. The patient remains mute and ignores any external stimuli. The patient’s medical history is significant for depression. She was recently switched from phenelzine to fluoxetine. Which of the following is the most appropriate initial therapy?
Benztropine
Cyproheptadine
Electroconvulsive therapy
Haloperidol
Lorazepam
E
Lorazepam
The patient has a history of depression and is presenting with decreased motor activity, waxy flexibility, lack of response to external stimuli, and mutism, suggesting the diagnosis of catatonia. Catatonia should initially be treated with benzodiazepines like lorazepam. Catatonia is a state of psychomotor immobility and apathy that is associated with a mood disorder or psychosis. The diagnosis is made clinically by having at least 3 of the following symptoms: stupor (no psychomotor activity), negativism (no response to external stimuli), catalepsy (muscle rigidity in a position against gravity), waxy flexibility (maintaining positioning placed by the examiner), mutism, echolalia (mimicking speech), echopraxia (mimicking movements), stereotypy, grimacing, and non-purposeful agitation or mannerisms. Initial treatment involves benzodiazepines for symptomatic relief. If the patient is unresponsive to benzodiazepines, electroconvulsive therapy (ECT) can be used. Francis reviews the evidence regarding the treatment of patients with catatonia. He discusses how benzodiazepines can be used with rapid improvement in symptoms. He recommends electroconvulsive therapy in refractory cases. Incorrect Answers: Answer A: Benztropine and IV diphenhydramine can be used to treat an acute dystonic reaction. Dystonic reactions present as involuntary spasms or sustained contractions of the face, neck, torso, pelvis, extremities, and larynx. Medications that block dopamine D2 receptors, such as neuroleptics and antiemetics, carry the greatest risk. Answer B: Cyproheptadine is a serotonin antagonist that may be part of the treatment for serotonin syndrome. Serotonin syndrome may occur as a result of the simultaneous use of monoamine oxidase inhibitors (MAOIs), selective serotonin reuptake inhibitors (SSRIs), or serotonin-norepinephrine reuptake inhibitors (SNRIs). It presents with myoclonus, hyperthermia, flushing, tachycardia, and delirium. Answer C: Electroconvulsive therapy is an effective treatment for catatonia but would be considered 2nd line to benzodiazepines for refractory cases. Answer D: Haloperidol is an antipsychotic, which may worsen catatonia. It can cause neuroleptic malignant syndrome, which can mimic catatonia. Bullet Summary: Catatonia can present in patients with a history of mood disorders and psychosis with symptoms of decreased motor activity, rigid posture, and mutism, and should be treated initially with a benzodiazepine.
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A 30-year-old man presents to the emergency room with a laceration over the 4th metacarpophalangeal (MCP) joint of his right hand. He reports that he is a mailman, and his closed fist was bitten by a dog while he was delivering mail yesterday. He reports pain but denies fevers, chills, drainage, or any other symptoms. The dog is up to date on vaccinations. His last tetanus vaccine was 3 years ago. He has no medical history and takes no medications. His temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 125/75 mmHg, and respirations are 16/min. Examination of the patient's right hand is shown in Figure A. Which of the following is the most appropriate next step in management?
Amoxicillin-clavulanate
Cephalexin
Clindamycin
Clindamycin plus doxycycline
Wound closure and amoxicillin-clavulanate
A
Amoxicillin-clavulanate
This patient has suffered a clenched fist injury which are generally managed with irrigation and amoxicillin-clavulanate. Amoxicillin-clavulanate is the usual first-line antibiotic for patients with a human or dog bite. Lacerations sustained from animal or human bites should not be sutured due to the risk of infection. Amoxicillin-clavulanate is first-line therapy for prophylaxis and treatment of human bites and dog bites, in general. Closed fist injuries put the patient at a high risk for osteomyelitis, septic arthritis, and tenosynovitis; therefore, prophylactic antibiotics are appropriate. Amoxicillin-clavulanate provides essential coverage against Pasturella multocida, commonly found in the mouths of dogs, cats, and other mammals. In patients with a penicillin allergy, agents such as TMP-SMX, doxycycline, or cefdinir paired with anaerobic coverage (clindamycin or metronidazole) is appropriate. Presutti discusses the prevention and treatment of dog bites. Appropriate treatment consists of immediate, copious irrigation, assessment for risk of tetanus and rabies, and administration of prophylactic antibiotics as discussed above. Figure A shows a dog bite wound over the 4th MCP of the right hand. Incorrect Answers: Answer B: Cephalexin does not cover species that are typically present in the mouths of dogs and other mammals and is therefore not an appropriate antibiotic choice for this patient as monotherapy. Answer C: Clindamycin provides coverage of anaerobic species, but is not sufficient alone for treatment of this patient's injury. Answer D: Clindamycin plus doxycycline may be appropriate coverage for a dog bite in a patient with an allergy to amoxicillin-clavulanate. However, this patient has no stated allergies and should receive amoxicillin-clavulanate. Answer E: Wound closure and amoxicillin-clavulanate would not be appropriate. Lacerations sustained from dog or cat bites are not sutured closed due to the risk of infection. Bullet Summary: Amoxicillin-clavulanate is the first line antibiotic choice for patients with dog or cat bites.
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A 59-year-old woman presents to her primary care physician for trouble sleeping. When she goes to bed at night, she has an urge to get up out of bed and walk around. The patient often wakes her husband when she does this which irritates him. There is a perpetual uneasiness and feeling of a need to move at night which is relieved by getting up and walking around. The patient denies symptoms during the day. She works as a mail carrier and is nearing retirement. She has a medical history of anxiety, depression, irritable bowel syndrome, and dysmenorrhea. She is not currently taking any medications. Her temperature is 99.5°F (37.5°C), blood pressure is 157/98 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals 5/5 strength in the upper and lower extremities, 2+ reflexes in the upper and lower extremities, a stable gait pattern, and normal sensation. Cardiopulmonary and abdominal exams are within normal limits. Which of the following is the most appropriate initial step in management?
Alprazolam
Ferrous sulfate
Iron studies
Pramipexole
Supportive therapy and an exercise routine
C
Iron studies
This patient is presenting with the need to get up and walk around at night, which is suggestive of restless leg syndrome. The most appropriate initial step in management is collecting iron studies to assess for iron deficiency. Restless leg syndrome presents with a sustained sense of restlessness and a feeling of a need to move one's legs. Symptoms typically occur at night and are relieved by activities such as walking. Iron deficiency can be a precipitating factor in restless leg syndrome as iron is a cofactor in the production of dopamine. Prior to administering iron, it is important to first confirm or rule out the diagnosis of iron deficiency with laboratory values which should include a CBC and iron studies. If iron deficiency exists, iron should be administered. If iron deficiency is not present, then dopamine agonists such as pramipexole or ropinirole can be administered. Gossard et al. review the evidence regarding the diagnosis and treatment of restless leg syndrome. They discuss how iron deficiency is associated with the development of this disorder. They recommend iron repletion in deficient patients. Incorrect Answers: Answer A: Alprazolam could be an appropriate step in management if the patient suffers from episodic panic attacks/anxiety. It is not an optimal long-term therapy for anxiety or depression. Benzodiazepines are not first-line in treating restless leg syndrome. Answer B: Ferrous sulfate would not be indicated until iron deficiency is confirmed. Iron deficiency is associated with restless leg syndrome as iron is a cofactor in the production of dopamine. Answer D: Pramipexole is a dopamine agonist that is an effective therapy for restless leg syndrome. However, it is more important to treat any underlying causes for restless leg syndrome such as iron deficiency prior to starting a dopamine agonist. Answer E: Supportive therapy and an exercise routine ignore the potential underlying pathology and do not offer any specific treatment for this patient's restless leg syndrome. Exercise can improve symptoms of restless leg syndrome but iron deficiency must still be ruled out. Bullet Summary: Patients with restless leg syndrome should be assessed for iron deficiency and treated if they are iron deficient.
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A 35-year-old man presents to the emergency department with altered mental status. He was found by his roommate to be disoriented this morning. He had complained of a right-sided headache for the past 5 days. He has a history of human immunodeficiency virus (HIV) infection being treated with bictegravir, emtricitabine, and tenofovir. His last CD4 cell count was 500/mm^3. He does not smoke tobacco and drinks alcohol socially. His temperature is 104.2°F (40.1°C), blood pressure is 110/70 mmHg, pulse is 110/min, and respirations are 22/min. Examination reveals 3/5 strength to right shoulder abduction and elbow flexion/extension and 4/5 strength to right hip flexion/extension. The remainder of the strength exam on the right and left sides is normal. Neck flexion does not elicit pain, and there are no tongue lacerations. During the exam, the patient’s eyes are seen to roll backward with repeated flexion/extension of his extremities and loss of urine. This stops after 1 minute. Which of the following is the most likely diagnosis?
Bacterial meningitis
Brain abscess
Cytomegalovirus encephalitis
Epilepsy
Fungal meningitis
B
Brain abscess
This HIV-positive patient presents with fever, unilateral headache, altered mental status, focal neurological deficits (3/5 strength to right shoulder abduction, elbow flexion/extension, hip flexion/extension), and a generalized tonic-clonic seizure, suggestive of a brain abscess. A brain abscess typically presents in patients with underlying risk factors for infection, such as positive HIV status or bacterial endocarditis with hematogenous bacterial seeding. Patients present with fever, unilateral headache, altered mental status, focal neurological deficits, and fevers. Work-up consists of blood cultures, CT scan, and confirmatory magnetic resonance imaging with gadolinium contrast, showing a focal, hyperintense lesion with restricted diffusion. Treatment is with empiric broad-spectrum antibiotic therapy (e.g., vancomycin, metronidazole, and cefepime for gram-positive, gram-negative, and Pseudomonas coverage in immunocompromised patients) and surgical drainage. Brouwer et al. review the causes, diagnosis, and treatment of brain abscesses. They discuss the importance of rapid initiation of antimicrobial therapy in these patients. Incorrect Answers: Answers 1 & 5: Bacterial or fungal meningitis can present with fever, headache, focal neurological deficits, and seizures but patients typically have meningeal signs such as pain with neck flexion. Cryptococcal meningitis is commonly seen in HIV-positive patients with CD4 counts < 100/mm^3. Answer C: Cytomegalovirus encephalitis can present with fever, altered mental status, headache, and focal neurological deficits in patients with HIV infection. It typically presents in patients with severe immunodeficiency, often with CD4 cell count < 50/mm^3. This patient’s CD4 cell count is 500/mm^3, making this less likely than an abscess. Answer D: Epilepsy is a primary neurological disorder that can cause generalized tonic-clonic seizures but would not explain this patient’s fever or unilateral headache. While Todd paralysis (focal weakness after a seizure) can occur after seizures, this patient’s focal weakness predates his seizure. A primary seizure disorder is not diagnosed when seizures occur secondary to another process. Bullet Summary: Brain abscess presents with fever, altered mental status, unilateral headache, seizures, and focal neurological deficits in patients with risk factors for infection (for example HIV, bacterial endocarditis).
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A 24-year-old man with epilepsy refractory to valproic acid, phenytoin, and levetiracetam undergoes magnetic resonance imaging of his brain while monitored by anesthetic care. He wakes up screaming in pain due to an electrocardiogram lead having caused a significant thermal burn circumferentially around his left leg. He is admitted to the medical intensive care unit for continuous electroencephalogram monitoring while on a midazolam infusion for seizure suppression and supportive care for his burn. Overnight, the nurse continued to increase the patient's midazolam infusion rate, but she also noticed that his left toes were cold to the touch with significant edema. His temperature is now 100°F (37.8°C), blood pressure is 110/75 mmHg, pulse is 80/min, respirations are 10/min and oxygen saturation is 95% on 2 liters nasal cannula. No dorsalis pedis or posterior tibial pulses are detected on the left lower extremity. A delta pressure of 25 mmHg is obtained in the left leg. What is the most appropriate next step in management?
Amputation
Escharotomy
Fasciotomy
Intravenous fluid infusion based on Parkland formula
Transfer to burn center
B
Escharotomy
This patient with a circumferential burn due to an ECG cable wrapped around his leg in an MRI machine now has clear evidence of compartment syndrome due to the eschar. The most appropriate next step in management is emergent escharotomy to restore blood flow. Burns can be caused by chemical, thermal, electrical, and radiation sources. The initial inflammation causes vasodilatation and hyperemia, which can progress to edema and local microvascular thrombosis. If not promptly addressed, tissue necrosis and irreversible damage can occur. Circumferential burns can evolve into eschars that constrict blood flow and cause distal compartment syndrome. A delta pressure (diastolic pressure minus local compartment pressure) lower than 30 mmHg is diagnostic. In the case of eschar-induced compartment syndrome, the most appropriate next step is escharotomy to alleviate pressure. Ormiston and Marappa-Ganeshan review the evidence regarding the diagnosis and treatment of patients with compartment syndrome. They discuss how this condition can be caused by many different pathologies including burns. They recommend the emergent release of constrictive structures. Incorrect Answers: Answer A: Amputation may be necessary if the distal limb is completely necrotic and not salvageable. Timely release of the eschar can allow for reperfusion of the limb and prevent this outcome. Answer C: Fasciotomy is the treatment of choice for compartment syndrome not due to a circumferential eschar, but in this case the least invasive surgical intervention should be performed first without releasing all the compartments of the lower leg. If escharotomy does not relieve the compartment syndrome then fasciotomy may be required. Answer D: Intravenous fluid infusion based on the Parkland formula is part of comprehensive burn management, but would not address the underlying compartment syndrome, which is more urgent at this time. Answer E: Transfer to a specialized burn center can be considered since circumferential burns are one of the admission criteria, but early escharotomy should be performed first. Bullet Summary: Circumferential burns that cause eschars and compartment syndrome require an escharotomy.
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A 27-year-old woman presents for her first prenatal visit at an estimated gestational age of 16 weeks and 4 days by last menstrual period. She is presenting late to prenatal care due to significant anxiety about her pregnancy, as she and her husband had struggled with infertility for several years prior to conceiving. She has had nausea and vomiting for about 2 months, tender and swollen breasts, and cravings for foods she typically does not eat. She took a pregnancy test about 10 weeks ago and was too nervous to read the result immediately. After 24 hours, she saw that it was positive. The patient has a past medical history of bulimia nervosa but has not been symptomatic in 2 years. She also had an appendectomy at age 15 for appendicitis. She is a Ph.D. student and her husband is a physician. Her temperature is 98.5°F (36.9°C), pulse is 75/min, blood pressure is 122/76 mmHg, and respirations are 13/min. The patient’s affect is normal and she is pleasant and excited about her pregnancy. A physical exam is notable for abdominal distension but is otherwise unremarkable. Ultrasound is shown in Figure A, and urine human chorionic gonadotropin is negative in the office. Which of the following is the most likely diagnosis?
Couvade syndrome
Delusion of pregnancy
Feigned pregnancy
Pseudocyesis
Recent miscarriage
D
Pseudocyesis
This patient presents with signs and symptoms of pregnancy but a nongravid uterus on ultrasound and a negative pregnancy test. The most likely diagnosis is pseudocyesis. Pseudocyesis, or false pregnancy, most commonly presents with abdominal distension, breast tenderness, nausea, and other typical symptoms of pregnancy. Patients truly believe they are pregnant and may report positive pregnancy tests at home (perhaps due to false positives from waiting too long before reading the result) but office testing is negative. Ultrasound will be normal and show the absence of a developing fetus. The mainstay of treatment is explaining the diagnosis in a therapeutic manner and offering counseling to help patients cope. Small reviews the evidence regarding the diagnosis and treatment of pseudocyesis. He discusses how this disease is a heterogenous entity involving neuroendocrine changes. He recommends clearly defining the cause of this disease in patients. Figure/Illustration A shows a transabdominal ultrasound with an empty cavity without signs of pregnancy (red circle). These findings are consistent with pseudocyesis. Incorrect Answers: Answer A: Couvade syndrome refers to a “sympathetic pregnancy,” in which a patient experiences pregnancy symptoms when someone close to her is pregnant. This does not apply to this patient because she believes that she is pregnant herself. Answer B: Delusion of pregnancy is a false and fixed belief about being pregnant, despite factual evidence to suggest otherwise. Patients typically do not experience actual pregnancy symptoms and may have a history of psychotic disorders, both of which are inconsistent with this patient’s presentation. Treatment is with cognitive behavioral therapy. Answer C: Feigned pregnancy is the intentional falsifying of symptoms for a clear external motive and is a subtype of factitious disorder or malingering. This patient does not express any desire for secondary gain and seems to truly believe that she is pregnant, which is more consistent with pseudocyesis. Answer E: A recent miscarriage is unlikely, as urine hCG testing often takes up to a month to become negative after pregnancy loss. Furthermore, the patient reports that her symptoms have been consistently present, whereas a miscarriage would typically cause a cessation of pregnancy symptoms. Bullet Summary: Pseudocyesis is a somatic symptom disorder in which women believe they are pregnant and experience pregnancy symptoms without an actual pregnancy.
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A 42-year-old man presents to the urgent care clinic with low back pain. He was working on a home improvement project the day prior to presentation when the pain started. He describes the pain as "achy and sore." It is not positional and does not radiate. He denies fevers, chills, paresthesias, and bowel or bladder incontinence. He has a history of a distal radius fracture 2 years ago from falling off a ladder. He drinks 3 alcoholic beverages weekly and denies illicit drug use. The patient’s temperature is 98.4°F (36.9°C), blood pressure is 124/80 mmHg, pulse is 90/min, and respirations are 16/min. His body mass index (BMI) is 22.4 kg/m^2. There is tenderness to palpation of his paravertebral lumbar region bilaterally. Perineal and dermatomal sensation is symmetric and intact. Strength is 5/5 to knee flexion/extension and ankle dorsiflexion/plantarflexion. Patellar and Achilles reflexes are 2+ bilaterally. Raising either leg while the patient is in the supine position does not elicit any pain. Which of the following is the most likely diagnosis?
Disc herniation
Lumbar stenosis
Lumbar strain
Osteoarthritis
Vertebral compression fracture
C
Lumbar strain
This patient presents with low back pain after activity with paravertebral tenderness and without red flag signs/symptoms, such as history of trauma or malignancy, intravenous drug use, saddle anesthesia (reduced perineal sensation), or neurological deficits. The most likely diagnosis is a lumbar strain. The differential diagnosis of low back pain is broad and includes mechanical causes such as lumbar strain, osteoarthritis, spondylolisthesis, disc herniation, spinal stenosis, and fractures as well as non-mechanical etiologies such as osteomyelitis and malignancy. History and physical should focus on eliciting red flag symptoms, including traumatic etiology, constitutional symptoms (e.g., fever, weight loss), history of malignancy, intravenous drug use, steroid use, and neurological deficits (e.g., saddle anesthesia, incontinence). The presence of any red flags should prompt further evaluation with imaging. Lumbar strain typically presents with acute low back pain after a precipitating event (e.g., lifting weight) with no red flag signs/symptoms and responds well to conservative management including activity modification, ice, and non-steroidal anti-inflammatory drugs. Prevention measures include strengthening of core muscles and education on proper lifting techniques. Physical therapy is a core component of treating patients with muscle strains. Knezevic et al. review lower back pain. They note the many potential causes and symptoms that may present. They recommend a multimodal interdisciplinary approach to back pain given the many mechanisms, especially in complex cases. Incorrect Answers: Answer A: Disc herniation presents with radicular symptoms due to compression of spinal nerve roots as they exit the spinal canal, manifesting as a burning or shooting pain that radiates down either leg. Dermatomal sensory changes or hyporeflexia may be present depending on the spinal level affected (e.g., reduced Achilles reflex with S1 radiculopathy). The straight leg raise reproduces pain radiating down the leg on the affected side. Answer B: Lumbar stenosis characteristically causes low back pain or lower extremity paresthesias with walking or standing that resolves when leaning forward (“shopping cart sign”). This is referred to as neurogenic claudication. Involvement of spinal nerve roots can lead to sensory loss and weakness in the lower extremities. Answer D: Osteoarthritis can cause low back pain and stiffness, but is an age-related degenerative disease that is less likely in this patient who presents acutely after activity. The patient also does not have the typical risk factors for osteoarthritis, which include advanced age, female sex, or obesity. Answer E: Vertebral compression fracture occurs mainly in osteoporotic patients, whose reduced bone mineral density increases their risk for vertebral body collapse under stress. Vertebral compression fractures present with point tenderness at the midline over the site of fracture. Additionally, this patient has no risk factors for osteoporosis (e.g., advanced age, post-menopausal women, low BMI). Bullet Summary: Lumbar strain presents as low back pain without red flag signs or symptoms (e.g., constitutional symptoms, neurologic deficits) and is treated conservatively with activity modification, ice, and core strengthening.
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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
Multiple myeloma
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).
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A 35-year-old man presents to the emergency department for evaluation of chest pain. The pain starts in his chest and moves into his back, arms, and abdomen. He describes the pain as tearing. He has no known medical history and takes no medications. His temperature is 37.0°C (98.6°F), pulse is 130/min, blood pressure is 210/145 mmHg, respirations are 22/min, and pulse oximetry is 98% on room air. Exam reveals a diaphoretic, anxious-appearing man. Pulses are diminished over the left wrist when compared to the right. A chest radiograph is obtained as shown in Figure A. Which of the following is the most appropriate next step in management?
Aspirin
CT angiography of the chest, abdomen, and pelvis
Echocardiography
Lorazepam
Serum troponin testing
B
CT angiography of the chest, abdomen, and pelvis
This patient with "tearing" chest pain, hypertension, and a chest radiograph demonstrating mediastinal widening likely has an aortic dissection. The most appropriate next step in management would be to obtain CT angiography of the chest, abdomen, and pelvis to confirm the diagnosis. Aortic dissections develop due to a tear in the intimal layer of the aorta, causing a second blood-filled lumen to form. Risk factors include hypertension, trauma, Marfan syndrome, Turner syndrome, and pregnancy. Prognosis depends on the location and severity of the intimal tear. Aortic dissections are often described by using the Stanford classification, where Type A involves the ascending aorta and Type B involves tears distal to the left subclavian artery. Patients classically present with chest pain described as "tearing" or "ripping" with radiation into the back, arms, or abdomen. Hypertension is common. Chest radiography characteristically demonstrates widening of the mediastinum due to accumulation of blood between layers of the wall of the aorta. Type A dissections are managed with heart rate control, blood pressure control, and surgery. Type B dissections are managed with blood pressure and heart rate control only. Cooper et al. report on aortic dissections in adolescence. Although rare, aortic dissections have been seen in children with congenital heart disease, connective tissue disorders, or severe traumatic accidents. Severe, migrating abdominal pain should heighten suspicion for this diagnosis. The patient will often look worse clinically than can be explained by physical findings. Figure A depicts a chest radiograph demonstrating a widened superior mediastinum. Incorrect Answers: Answer A: Aspirin is administered for chest pain that is thought to be due to acute coronary syndrome. This patient's presentation, exam, and imaging findings suggest aortic dissection. Aspirin is contraindicated in aortic dissection due to the increased risk for bleeding. Answer C: Echocardiography offers information on cardiac contractility, valve function, and the presence of effusions. It would not be an appropriate initial diagnostic test in aortic dissection. Transesophageal echocardiography may be used to make the diagnosis in patients who cannot undergo a CTA. Answer D: Lorazepam would be appropriate for patients presenting with chest pain that is secondary to use of cocaine or other sympathomimetic drugs. Tachycardia, tachypnea, diaphoresis, hyperactive bowel sounds, and dilated pupils suggest a sympathomimetic toxidrome. Answer E: Serum troponin testing is indicated for risk stratification of patients with suspected acute coronary syndrome. Bullet Summary: The most appropriate next step in management for patients with suspected aortic dissection and mediastinal widening on chest radiograph is CT angiography of the chest, abdomen, and pelvis.
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A 36-year-old man presents to the office for follow up after initiating antidepressant therapy. Two months ago, he was started on citalopram after several weeks of depressed mood, loss of interest in activities, depressed appetite, and inability to sleep. Today, he reports minimal improvement in his symptoms. He had some nausea after starting the medication, but this has resolved. He denies thoughts of suicide or self-harm. His temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 120/80 mmHg, respirations are 22/min, and oxygen saturation is 97% on room air. Exam reveals a depressed, tired appearing man. Which of the following is the most appropriate next step in management?
Add mirtazapine
Electroconvulsive therapy
Switch to amitriptyline
Switch to paroxetine
Switch to phenelzine
A
Add mirtazapine
This patient is being treated for depression without improvement in his symptoms after 6 weeks of medication use. In the setting of his reduced appetite and trouble sleeping, mirtazapine is an appropriate medication to add. SSRIs (including paroxetine, citalopram, fluvoxamine, sertraline, and fluoxetine) are the first line treatment for major depression. For patients in whom an adequate trial of SSRI therapy has been ineffective, the first step in management is a trial of augmentation with a separate agent prior to switching to a different primary antidepressant medication. Mirtazapine, a noradrenergic and specific serotonergic antidepressant, acts by inhibiting pre-synaptic alpha-2 receptors as well as 5HT2 and 5HT3 serotonin receptors. Notably, its side effect profile includes increased appetite and mild sleepiness, making it a good option for depressed patients with insomnia or decreased appetite. Mirtazapine is commonly used to augment primary antidepressant therapy. Augmentation with an additional agent should generally be attempted prior to switching to a different SSRI or other first line medication. Davies et. al review the pharmacologic management of depression. They note that while evidence is limited, there is some data to suggest benefit in augmentation with a secondary agent prior to switching to another first line therapy. Incorrect Answers: Answer B: Electroconvulsive therapy, while often extremely effective, is typically reserved for patients that are actively suicidal, unable to eat, or displaying psychotic symptoms. It is more commonly used after medications have failed. Answer C: Switching to amitriptyline, a tricyclic antidepressant, would not be appropriate. Tricyclic antidepressants have a negative side effect profile and potential for severe toxicity. Answer D: Switching to paroxetine, another SSRI medication, may be appropriate. However, this patient's particular constellation of symptoms (decreased appetite and insomnia) suggest that he would likely respond well to mirtazapine. Answer E: Switching to phenelzine, a monoamine oxidase inhibitor, would not be appropriate. Monoamine oxidase inhibitors have a negative side effect profile and potential for severe drug-drug interaction. Bullet Summary: Mirtazapine (a noradrenergic and serotonergic antidepressant) is an appropriate next step for depressed patients with significant insomnia and decreased appetite who fail to respond to a trial of SSRI monotherapy.
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A 65-year-old man presents to his primary care physician for stiffness in his arm. He has been having trouble combing his hair and reaching objects that are high on the shelf. The patient has a medical history of type 2 diabetes mellitus, obesity, and hypertension. His current medications include metformin, insulin, lisinopril, and hydrochlorothiazide. The patient leads a sedentary life in which he tends to stay home and watch television. He does not engage in any physical or strenuous activity. His temperature is 98.6°F (37°C), blood pressure is 138/85 mmHg, pulse is 75/min, and respirations are 12/min. On physical exam, the patient has decreased passive and active range of motion of his shoulder. The strength of the patient's upper extremity is 4/5 limited by pain. Which of the following is the most likely diagnosis?
Adhesive capsulitis
Biceps tendinopathy
Glenohumeral osteoarthritis
Rotator cuff impingement
Subacromial bursitis
A
Adhesive capsulitis
This patient is presenting with stiffness and a decreased range of motion in his shoulders suggestive of a diagnosis of adhesive capsulitis. Adhesive capsulitis classically presents in elderly, sedentary patients with a chief complaint of stiffness. On physical exam, the key findings are a loss of active, but more importantly, passive range of motion. It is the loss of passive range of motion that suggests a diagnosis of adhesive capsulitis over rotator cuff injuries, biceps tendinopathy, glenohumeral osteoarthritis, and subacromial bursitis. The diagnosis of adhesive capsulitis is made clinically; however, radiography, MRI, and ultrasound can be used to rule out other diagnoses. Treatment includes NSAIDs and physical therapy and can include steroids. Ramirez reviews the evidence regarding the diagnosis and treatment of adhesive capsulitis. He discusses how non-operative management consists of nonsteroidal anti-inflammatory drugs, short-term oral corticosteroids, intra-articular corticosteroid injections, and physiotherapy. He recommends manipulation under anesthesia and arthroscopic capsule release in patients who fail conservative management. Incorrect Answers: Answer B: Biceps tendinopathy presents with pain and weakness overlying the long head of the biceps tendon. This tendon runs in the intertubercular groove on the anterior aspect of the proximal humerus. Patients will often present with pain with resisted flexion of the elbow. Treatment is surgical debridement in patients who fail conservative measures. Answer C: Glenohumeral osteoarthritis presents with vague and diffuse pain that is exacerbated by activity in addition to a loss of range of motion of the shoulder. Though this patient is experiencing a decreased range of motion, the decreased passive range of motion is more specific for adhesive capsulitis. Treatment is with a total shoulder replacement in patients who fail conservative measures. Answer D: Rotator cuff impingement presents with pain and weakness of the upper extremity, but also can present with a decreased range of motion as this patient experienced. Of note, the passive range of motion will not be significantly affected by this disease. Treatment is with surgical reconstruction or debridement of the rotator cuff if initial measures fail. Answer E: Subacromial bursitis presents with localized tenderness, decreased range of motion, pain with motion, and sometimes erythema/edema. Though this patient is experiencing a decreased range of motion and pain with motion, the decreased passive range of motion suggests a diagnosis of adhesive capsulitis. Treatment is with rest and oral anti-inflammatory medications. Bullet Summary: Adhesive capsulitis presents with pain and a decreased passive and active range of motion.
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A 27-year-old man presents to the emergency department after a bar fight. He was punched in the eye and is having pain and blurry vision. The patient is otherwise healthy and does not take any medications. His temperature is 98.1°F (36.7°C), blood pressure is 132/84 mmHg, pulse is 103/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for conjunctival injection of the patient’s left eye as shown in Figure A. Visual acuity reveals 20/20 vision in his right eye and 20/40 vision in his left. His left pupil is sluggish to constrict when compared to the right. A fluorescein stain is unremarkable and tonometry reveals a pressure of 45 mmHg in the left eye. Which of the following is the most appropriate next step in management?
CT scan of the head and facial bones
Cyclopentolate
Lateral canthotomy
Orbital ultrasound
Topical timolol and pilocarpine
C
Lateral canthotomy
This patient is presenting after blunt trauma to the eye with eye pain, blurry vision, conjunctival injection, a sluggish pupil, and elevated intraocular pressure (> 20 mmHg) suggesting a diagnosis of a retrobulbar hematoma/hemorrhage causing orbital compartment syndrome. The most important initial step in management is a lateral canthotomy to alleviate retrobulbar pressure. A retrobulbar hematoma (or hemorrhage) occurs after blunt trauma to the eye. Presenting symptoms may include eye pain, photophobia, proptosis (an eye that appears to be anteriorly displaced), decreased visual acuity, and conjunctival injection. It is important to perform a thorough exam including a slit lamp exam and a fluorescein stain to rule out other traumatic injuries such as a globe rupture. Tonometry can be used to assess for increased intraocular pressure. A pressure > 20 mmHg suggests a diagnosis of orbital compartment syndrome, a vision-threatening condition. Immediate management with a lateral canthotomy is indicated in order to relieve pressure on the optic nerve and retina and save the patient’s vision. Further management may require drainage of the hematoma in the operating room. Burkat et al. review the technique for performing a lateral canthotomy to relieve pressure in retrobulbar hematoma. The authors first excise the skin and orbicularis muscle at the lateral canthal angle in a horizontal direction for approximately 10 mm. The authors then recommend performing an inferior cantholysis by incising the inferior crus (a thick fibrous band connecting the lateral lower eyelid to the orbital rim) near the orbital rim. Figure A demonstrates proptosis on physical exam which raises concern for a retrobulbar hematoma that is likely increasing orbital compartment pressure. Incorrect Answers: Answer A: CT scan of the head and facial bones would be appropriate if there was a concern for facial fracture. It would delay necessary and vision-saving interventions to perform a CT scan in the setting of orbital compartment syndrome. However, cross-sectional imaging may be performed after the pressure is relieved. Answer B: Cyclopentolate is a cycloplegic that can be used to reduce pain in traumatic iritis. This typically presents after blunt trauma to the eye with erythema, photophobia, blurry vision, and cell/flare in the anterior chamber on slit lamp exam. A sluggish pupil may also be seen; however, increased intraocular pressure would not be seen in this condition. Answer D: Orbital ultrasound could be used for diagnoses such as a retinal detachment which presents with flashes of light, floaters, and visual field loss like a “curtain coming down over the eye.” An ultrasound would demonstrate the detached retina in the back of the eye. Management involves elevating the head of the bed and surgical reattachment. Answer E: Topical timolol and pilocarpine in addition to brimonidine, acetazolamide, and possible iridotomy would be the appropriate management of acute closed-angle glaucoma which presents with a rock-hard eye, blurry vision, a mid-dilated and fixed pupil, as well as a headache. This diagnosis can be supported by elevated intraocular pressure on tonometry. This is unlikely for this patient in the context of trauma. Bullet Summary: A retroorbital hematoma can cause orbital compartment syndrome which requires immediate treatment with a lateral canthotomy.
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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
Vascular ectasia within the liver
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.
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A 56-year-old man presents from prison with 1 month of subjective fevers, chills, cough, and night sweats. He noticed a gradual weight loss over the past year despite no changes to his diet or physical activity. He has never smoked cigarettes but does have a history of opiate use prior to incarceration. He does not take any medications. His temperature is 100.8°F (38.2°C), blood pressure is 142/88 mmHg, pulse is 78/min, and respirations are 12/min. Physical exam reveals a thin, ill-appearing man. Pulmonary auscultation reveals faint, inspiratory crackles in the right upper lobe. His chest radiograph is shown in Figure A. Which of the following is the most appropriate next step in management?
Ampicillin-sulbactam
Biopsy
Itraconazole
Rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy
Vancomycin
D
Rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy
This patient likely has active tuberculosis (TB), as evidenced by his symptoms (fever, night sweats, cough, and weight loss), inmate status, and apical cavitary lesion on chest radiograph. He should be treated with RIPE therapy. An active TB infection should be suspected in patients with a febrile illness, cough, night sweats, and weight loss along with epidemiological risk factors for TB infection. Risk factors include residence in or previous visits to endemic regions, work in hospitals or nursing homes, imprisonment, and immunosuppression. Treatment typically requires 2 phases: an intensive phase involving 2 months of rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy and a continuation phase involving 4 additional months of just isoniazid and rifampin. Vitamin B6 (pyridoxine) should be taken with isoniazid to prevent peripheral neuropathy. Rifampin, isoniazid, and pyrazinamide use should be monitored for hepatotoxicity. Ethambutol can cause optic neuropathy. Conradie et al. discuss the use of a bedaquiline-pretomanid-linezolid (BPal) regimen in the treatment of extensively drug-resistant tuberculosis. The authors find that most patients treated with BPal have a favorable outcome (no treatment failure or disease relapse) after 6 months. The authors recommend the use of this regimen in highly drug resistant TB. Figure/Illustration A demonstrates a right upper lobe cavitating lesion (arrow) that resulted in extensive necrosis and fibrosis. These lesions are usually localized to the upper regions of the lung due to favorable oxygen concentrations in the apex. Incorrect Answers: Answer A: Ampicillin-sulbactam can be used as empiric treatment for a lung abscess to cover anaerobic pathogens and facultative anaerobic streptococci. A lung abscess is associated with aspiration risk factors (such as alcohol use) or poor dentition. Answer B: Biopsy may be indicated for a solitary cavitary nodule that is suggestive of malignancy. In this case, the patient's inmate status, along with his fever, night sweats, and cough are more suggestive of an infectious rather than malignant etiology. Answer C: Itraconazole can be used to treat chronic pulmonary aspergillosis. These patients typically have prior or current lung disease such as chronic obstructive pulmonary disease or tuberculosis. The classic air crescent sign may be seen on imaging in a patient with an aspergilloma, a form of pulmonary aspergillosis in which a fungal ball develops within an existing cavitary lesion. Answer D: Vancomycin can be used to treat a lung abscess due to methicillin-resistant Staphylococcus aureus. S. aureus pneumonia classically presents as a secondary infection that follows an influenza infection. Bullet Summary: Active tuberculosis infection should be treated with rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy.
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A resident physician places a patient’s home medications in for their admission orders during an overnight admission. They order the patient’s home medications including clobazam; however, they accidentally order clonazepam. Subsequently, the patient is over-sedated and has to be transferred to the ICU for airway monitoring. Which of the following is the most appropriate method for preventing future similar occurrences?
Add an alert in the electronic medical system for medications that sound similar
Enter medications only on morning rounds with multiple providers
Have nursing perform the medication reconciliation
Reprimand and educate the resident to check medications more carefully
Verify the patient’s home medications with the patient
A
Add an alert in the electronic medical system for medications that sound similar
A medical error occurred where 2 similar-sounding medications were confused, leading to an adverse event. The most appropriate way to prevent this error from occurring is a system-based approach, such as adding an alert in the electronic medical system for medications that sound similar. Medical errors commonly occur in the medical system given the complexity of delivering patient care. The best way to prevent future errors from occurring is a systems-based approach where the system is built to catch errors rather than relying on the diligence of staff. In a systems-based approach, there is an algorithmic method set in place for situations where errors are common. Examples of systems-based approaches to avoiding medical errors include time-outs for procedures, use of the electronic medical record and ordering system to generate stops and checks, and built-in procedures such as a pharmacist verifying all medical orders. The best way to prevent future errors, when possible, is a systems-based approach as it does not rely solely on individuals given that individuals are fallible and may not always avoid mistakes. Condren et al. review a systems based approach in the medical system. They note that a systems-based approach reduces medication errors. For this reason, systems-based approaches are recommended whenever possible. Incorrect Answers: Answer B: Entering medications only on morning rounds with multiple providers is not a plausible strategy as patients will not receive medications they may need at other times, such as overnight. It is always a good idea to verify medications with the team on morning rounds to catch any possible errors that may be missed, but this would not necessarily fix errors that occur at other times of the day. Answer C: Having nursing perform the medication reconciliation is not the best solution in this case, as the core problem was that the wrong medication was entered due to similar-sounding medication names. However, it is always a good idea to have multiple providers (e.g., nurses, pharmacists) verify information, check this with the physician team, and then verify the dose and route of administration when giving the medication. Answer D: Reprimanding and educating the resident to check medications more carefully is not a solution to this problem. It does not seem that the resident does not understand the pharmacology or medication prescribed but made an understandable error. Explaining the need for care is important; reprimanding the resident is not. Answer E: Verifying the patient’s home medications with the patient should always be performed, but it would not fix this issue with similar-sounding medications. It would catch errors such as new medications that are not on the patient’s medication list or out-of-date medications remaining on the list. Bullet Summary: A systems-based approach is the most appropriate way to prevent future errors from occurring.
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A 33-year-old man presents to the emergency department after slamming his finger in the car door 5 hours ago. He initially experienced pain which is currently well controlled with acetaminophen. The patient is otherwise healthy and does not take any medications. His temperature is 98.5°F (36.9°C), blood pressure is 123/79 mmHg, pulse is 90/min, respirations are 11/min, and oxygen saturation is 97% on room air. Physical exam is notable for the finding in Figure A. A radiograph of the affected digits shows a smooth cortex without disruption of the bone. Which of the following is the most appropriate management for this patient?
Excision and histological examination
Fingernail removal
Observation
Operative repair
Trephination
E
Trephination
This patient is presenting after trauma to his finger with a subungual hematoma. Given that he has no other associated injuries, trephination is the only management indicated. A subungual hematoma occurs after trauma to the finger leading to a collection of blood under the fingernail. Depending on the mechanism, this can be associated with a distal phalanx fracture. Evaluation for a fracture should include a thorough history, exam, and radiography. If there are no other signs of injury including nailbed dislocation or an open fracture, then management requires only trephination (putting a hole in the nail to allow blood to drain) as this alleviates pressure on the nailbed matrix thus preventing possible irreversible damage. Rsoer et al. compare nail bed repair to nail trephination for subungual hematomas in children. The authors find that there was no difference in complications or outcomes between nail bed repair and trephination; however, the cost in the nail trephination group was lower. The authors recommend nail trephination for children with a subungual hematoma and an intact nail and nail margin. Figure/Illustration A shows the physical exam finding of a subungual hematoma with a collection of blood underneath the fingernail bed (green circle). Incorrect Answers: Answer A: Excision and histological examination could be appropriate management of melanoma, which may present under the fingernails with a hyperpigmented, irregular skin lesion, or a “barcode” appearance if it is at the base of the nail. A spontaneous subungual hematoma could be suggestive of melanoma; however, in the setting of trauma, it is a much less likely diagnosis. Answer B: Fingernail removal should only be performed if there is also nail avulsion or nail fold disruption to allow for inspection of the nail bed and repair of any laceration. Failure to repair a nailbed laceration could lead to permanent deformity of the fingernail when it regrows. Answer C: Observation is inappropriate as the accumulation of blood under the nail in a subungual hematoma could lead to ischemia and permanent injury to the nailbed. When a subungual hematoma is present, trephination is indicated. Answer D: Operative repair may be necessary for fractures; however, this patient has no fracture and only has a subungual hematoma which can be drained easily. Bullet Summary: A subungual hematoma is common after trauma, presents with a collection of blood under the fingernail, and should be treated with trephination.
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A 27-year-old woman presents to her primary care physician for an abnormal vaginal discharge. The patient has engaged in unprotected intercourse with 12 different partners. She has a medical history of asthma, IV drug abuse, and depression. She has 1 to 2 alcoholic drinks per day. She feels ashamed of her behavior and is requesting treatment for her condition and advice for safe sex. The patient is given antibiotics and is advised on safe sex practices. The patient presents to the emergency department 3 days later with a complaint of a rash. She states that every time she has sex a rash has emerged on her skin. Her temperature is 99.5°F (37.5°C), blood pressure is 127/68 mmHg, pulse is 91/min, respirations are 14/min, and oxygen saturation is 98% on room air. A physical exam is notable for the finding in Figure A. Which of the following is the most likely cause of this patient's symptoms?
Antigen exposure
Drug reaction
Infection
IV drug use
Photosensitivity
A
Antigen exposure
This patient is presenting with urticaria whenever she engages in sexual intercourse suggesting a latex allergy (found in condoms). These allergies are due to antigen exposure. Anaphylaxis and urticaria are immunologically mediated responses to environmental antigens that are typically IgE-mediated. This results in crosslinking of antigen receptors and the subsequent release of histamine and other inflammatory signals. Patients can present with a spectrum of symptoms from urticaria (hives alone) all the way to full-blown anaphylaxis (hypotension, tachycardia, and bronchoconstriction). Minor cases can be treated with avoidance of antigen exposure. Anaphylaxis is treated with intramuscular epinephrine (1:1,000). Gawchik reviewed the evidence regarding the diagnosis of latex allergy. She discusses how the diagnosis is primarily made by observing the relationship between exposure and symptoms. She recommends avoidance of latex in these patients. Figure/Illustration A is a clinical photograph that demonstrates dermal edema and urticaria (red circles). This can occur secondary to allergen exposure such as from a new drug or latex. Incorrect Answers: Answer B: Drug reaction could be possible if this patient has an antibiotic allergy (ceftriaxone is a treatment for gonorrhea); however, a rash that only occurs during intercourse is more likely related to latex exposure. Answer C: Infection is possible given this patient’s IV drug abuse and risky behavior; however, the hives present in this patient are likely not related to an infection. Infectious rashes tend to be painful, ulcerative, or vesicular. Answer D: IV drug use could expose this patient to any number of foreign substances that could induce an allergic reaction; however, the symptoms that are associated with sexual intercourse point toward latex exposure. Answer E: Photosensitivity would not present as hives but could occur in this patient given that doxycycline is an appropriate drug to treat her initial sexually transmitted infection and offers coverage for Chlamydia trachomatis. Patients would present with a photosensitive rash. Bullet Summary: Anaphylaxis and urticaria can occur secondary to latex exposure (such as from condoms or gloves).
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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?
Adverse effect of ciprofloxicin
Adverse effect of doxycycline
Allergic reaction to prescribed therapy
Progression of disease and alternative therapy is indicated
Progression of disease and surgical debridement is necessary
B
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.
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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
Mutation of RET proto-oncogene
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.
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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
T-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.
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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?
Immediately initiate triple antiretroviral therapy
Perform genotype testing on source patient and initiate antiretroviral therapy tailored to results
Repeat HIV serology and initiate triple antiretroviral therapy if negative
Repeat HIV serology and initiate triple antiretroviral therapy if positive
Repeat HIV serology and initiate triple antiretroviral therapy immediately
E
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.
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A 51-year-old man presents to the emergency department complaining of fever and a cough with yellow-green sputum for the past 3 days. He denies any hemoptysis, facial pain, rhinorrhea, or night sweats. The patient reported similar symptoms 2 months ago. Chest radiograph performed at the time was notable for a right middle lobe consolidation. He was diagnosed with lobar pneumonia and completed a 7-day course of antibiotics with complete resolution of his symptoms. He has no other significant medical history and does not take any medications. He has smoked 1 pack of cigarettes a day for 35 years and drinks 3 beers per week. He is sexually active with his wife. His temperature is 101.7°F (38.7°C), blood pressure is 127/85, pulse is 102/min, and respirations are 22/min. Physical exam is notable for decreased breath sounds on the right side without wheezing and increased dullness to percussion on the right side. A chest radiograph is ordered and reveals a consolidation in the right middle lobe. Which of the following is the most appropriate next step in management?
Barium swallow
CT of the chest without contrast
Human immunodeficiency virus (HIV) antibody assay
Interferon-gamma release assay
Serum immunoglobulin levels
B
CT of the chest without contrast
This patient's presentation with recurrent pneumonia in the same anatomical location is concerning for an anatomic abnormality. Given his history of heavy smoking, lung cancer should be ruled out with a chest CT with possible subsequent bronchoscopy for tissue biopsy. Recurrent pneumonia can be classified either as those confined to 1 anatomical location of the lung or those involving different portions of the lung. If recurrent pneumonia is limited to a single region, the patient should be evaluated for an anatomic obstruction, such as a mass or mucous plug. Obstruction will hinder mucociliary clearance and allow for the proliferation of bacteria, leading to recurrent infections. The most concerning etiology of such an obstruction is a tumor causing external bronchial compression. Thus, patients should be screened with a chest CT to rule out lung cancer in these cases. If lesions are identified on CT, subsequent bronchoscopy or CT-guided biopsy may be warranted. Treatment may involve radiotherapy, chemoimmunotherapy, or surgery depending on the underlying malignancy. Other causes of recurrent pneumonia limited to an anatomical region include recurrent aspiration secondary to tracheoesophageal disorders or seizures. Purysko et al. discuss the role of contrast enhancement in chest CTs. The authors find common indications for contrast-enhanced chest CTs include investigation for pulmonary embolism, pleural pathology, or hilar pathology. The authors recommend against the regular use of contrast enhancement in chest CTs outside of selected indications as they rarely provide additional diagnostic benefits while subjecting the patient to the risk of adverse effects with contrast agents. Incorrect Answers: Answer A: Barium swallow is often done to investigate anatomical abnormalities of the upper gastrointestinal tract such as tracheoesophageal fistula or Zenker diverticulum. These can increase the risk of aspiration and lead to recurrent pneumonia. In a patient with positive risk factors for cancer, chest CT to rule out malignancy would be the most appropriate next step. Answer C: Human immunodeficiency virus (HIV) can cause secondary immunodeficiency, which can predispose patients to recurrent infections. These recurrent pneumonias would be expected to be in different regions of the lung and not anatomically confined. Answer D: Interferon-gamma release assay can be used to diagnose tuberculosis (TB). Reactivation of TB will present clinically with fever, cough, hemoptysis, and night sweats. Chest radiography will commonly reveal an upper lobe infiltrate. Answer E: Serum immunoglobulin levels would be an appropriate next step to diagnose primary immunodeficiencies such as common variable immunodeficiency or other antibody defects. This would present with multiple lung infections in different regions of the lung. Moreover, these patients would be expected to have other infections such as sinusitis, otitis, and gastrointestinal infections. Bullet Summary: Recurrent pneumonia in the same anatomical region should be worked up with a CT with subsequent bronchoscopy and biopsy to rule out malignancy.
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A 15-year-old boy is brought to his pediatrician for a follow-up visit. His parents report that he is doing well in school and has many friends. Their only concern is that he has a “difficult stomach.” Every few months, he complains of intermittent abdominal pain that takes several days to pass. He occasionally misses school due to the pain. There is blood in his stool during these episodes. These symptoms have persisted for many years. The parents have so far managed the condition using herbal teas and essential oils but have not sought formal medical care. His temperature is 98.2°F (36.8°C), blood pressure is 106/61 mmHg, pulse is 88/min, and respirations are 12/min. He has lost 10 pounds since his annual visit the year before and is the same height. On physical exam, the patient is thin with conjunctival pallor. His sclerae are anicteric and his abdomen is soft and non-tender without hepatosplenomegaly. He also has the findings seen in Figure A. He undergoes an abdominal CT that shows disease affecting the terminal ileum. Which of the following is most likely to be present in this patient?
Decreased mean corpuscular volume
Decreased serum homocysteine level
Increased serum methylmalonic acid level
Microcytes
Prolonged partial thromboplastin time
C
Increased serum methylmalonic acid level
This patient with episodic abdominal pain, bloody stool, growth failure, and erythema nodosum most likely has Crohn disease. Disease involving the terminal ileum predisposes patients to vitamin B12 deficiency, which would cause anemia with an elevated serum methylmalonic acid level. Crohn disease is an inflammatory bowel disease that presents with abdominal pain, bloody stools, and growth failure in children. Extra-intestinal manifestations of Crohn disease include fistula formation and erythema nodosum, which appear as multiple tender erythematous nodules that fade after 1-2 weeks into bluish plaques or macules that can be confused with bruises. Complications of Crohn disease when the disease involves the terminal ileum is vitamin deficiency and malabsorption. These deficiencies can include vitamin A, vitamin D, vitamin E, vitamin K, zinc, and vitamin B12. Vitamin B12 deficiency presents with a macrocytic, megaloblastic anemia with hypersegmented neutrophils on peripheral blood smear. Lab findings include elevated serum homocysteine and methylmalonic acid levels. Vitamin B12 deficiency is also associated with peripheral neuropathy and should be treated with intramuscular vitamin repletion. Akbulut presents the current state of evidence regarding vitamin deficiency in patients with Crohn disease. They found that vitamin B12 and folate levels are often low in patients with this disease. They recommend monitoring vitamin levels and providing repletion when necessary. Figure/Illustration A is a clinical photograph showing erythematous nodules on the extensor surfaces of the legs (red circles). This finding is consistent with the latter stage of erythema nodosum. Incorrect Answers: Answer A: Decreased mean corpuscular volume may be seen in iron deficiency anemia, which can be co-morbid with Crohn disease. While this patient does have recurrent gastrointestinal blood loss, the very low frequency of his symptoms is unlikely to result in iron deficiency, making a decreased MCV less likely compared to an increased MCV given the underlying terminal ileum pathology. Answer B: Decreased serum homocysteine level is not seen in any common human disease. An elevated (not decreased) serum homocysteine level is seen in vitamin B12 deficiency in addition to folate deficiency. This increase is due to the impaired processing of homocysteine in the absence of these cofactors. Answer D: Microcytes would not be seen in vitamin B12 deficiency or folate deficiency, which instead present with macro-ovalocytes. Microcytic anemia would be seen in patients with iron deficiency anemia, which can present with fatigue and pallor in patients with occult bleeding. Iron supplementation and treatment of the underlying source of bleeding is appropriate care. Answer E: A prolonged PTT could be seen in this patient as vitamin K absorption is likely affected. This patient is not presenting with a chief complaint of bruising/bleeding (the physical exam finding is erythema nodosum rather than ecchymosis), and symptoms point toward a diagnosis of anemia which would be associated with poor B12 absorption. Bullet Summary: Crohn disease may affect the terminal ileum, resulting in vitamin B12 deficiency (increasing the serum methylmalonic acid level) that would present with macrocytic, megaloblastic anemia with hypersegmented neutrophils on a peripheral blood smear.
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A 55-year-old woman presents to the emergency department for evaluation of headache. She had a headache that was present for a few hours before subsiding 2 days ago, and then today she developed a headache that was sudden in onset and at maximal intensity within minutes. She has never had a headache like this in the past. Her temperature is 99.0°F (37.2°C), blood pressure is 157/99 mmHg, pulse is 90/min, respirations are 16/min, and oxygen saturation is 99% on room air. On exam, she has pain with passive and active movement of the neck. No focal neurologic deficits are appreciated. A non-contrast head CT is obtained as shown in Figure A. Which of the following is the most likely underlying etiology of this patient's headache?
CNS vascular endothelial dysfunction
Dehydration and stress
Hypersensitivity of the trigeminal nerve
Muscular pain and strain
Rupture of a berry aneurysm
E
Rupture of a berry aneurysm
This patient with sudden-onset headache, neck pain, and a CT scan showing blood in the basal cisterns likely has a subarachnoid hemorrhage. The most common cause of non-traumatic subarachnoid hemorrhage (SAH) is rupture of a saccular (berry) aneurysm. SAH typically presents with a sudden onset "thunderclap" headache that is often described as the "worst headache of my life." Hypertension is a common risk factor. The diagnosis can be confirmed with a non-contrast head CT. Non-contrast CT scan of the head is sufficient to rule out subarachnoid hemorrhage if performed within 6 hours of symptom onset. If the head CT is negative but SAH is still suspected, a lumbar puncture can help confirm the diagnosis (demonstrating xanthochromia) or CT angiography can be performed. Patients should be admitted for neurosurgery and neuro-IR consultation and may require calcium channel blockers and possible intervention. Macdonald et. al review spontaneous subarachnoid hemorrhage. They discuss the pathophysiology, clinical manifestations, and diagnosis. They note the typical finding of accumulation of blood in the basal cisterns and sylvian fissures on CT imaging. Figure A shows a non-contrast head CT demonstrating blood in the subarachnoid space supporting the diagnosis of SAH. Note the hyperdensity in the sylvian fissures and basal cisterns. Incorrect Answers: Answer A: CNS vascular endothelial dysfunction results in posterior reversible encephalopathy syndrome, which typically presents with hypertension and encephalopathy. Answer B: Dehydration and stress could explain a migraine headache or a tension headache. Migraine headaches are often pulsating and may be associated with aura. Answer C: Hypersensitivity of the trigeminal nerve is thought to be involved in the pathophysiology of migraine headache, and also could describe trigeminal neuralgia which presents with sudden and severe electrical pain in the distribution of the trigeminal nerve with minor stimulation. Answer D: Muscular pain and strain describes a tension headache which presents with diffuse head pain that is mild and not localized. CT imaging is normal in tension headache. Bullet Summary: Spontaneous subarachnoid hemorrhages are most commonly caused by rupture of a berry aneurysm.
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A 55-year-old man presents with a 2-day history of mono-articular joint pain. He is otherwise healthy and denies fever, chills, or recent trauma. His medical history is notable for a kidney stone 6 months ago. He underwent a dental procedure to extract an infected wisdom tooth 3 weeks ago. The patient does not take any medications. He is a non-smoker and does not drink alcohol or use recreational drugs. His family history is significant for osteoarthritis in his father who is 78. His temperature is 98.6°F (37°C), blood pressure is 133/84 mmHg, pulse is 84/min, and respirations are 15/min. His body mass index is 27 kg/m^2. Physical examination shows a swollen and tender joint with overlying erythema. Diagnostic arthrocentesis is performed and shows a leukocyte count of 30,000/mm^3 with 85% neutrophils. Polarized microscopy of the synovial fluid is shown in Figure A. Which of the following would most likely be found on this patient’s radiograph?
Boutonniere deformity
Chondrocalcinosis
Heberden nodes
Juxta-articular osteoporosis
Tophi
B
Chondrocalcinosis
This patient is presenting with a joint aspiration positive for calcium pyrophosphate dehydrate crystals, suggesting a diagnosis of pseudogout, which may show chondrocalcinosis on radiography. Pseudogout presents classically with mono- or oligo-articular arthritis that most often affects the knee and proximal joints of elderly patients (> 60 years of age). The disease can be associated with hyperparathyroidism, Gitelman syndrome, familial hypocalciuric hypercalcemia, and hemochromatosis. The patient will have a leukocyte count < 50,000/mm^3 on joint aspiration and positively birefringent, rhomboid-shaped cells on microscopy, which represent calcium pyrophosphate dehydrate (CPPD) crystals. On radiography, chondrocalcinosis can be found, which is the calcification of adjacent cartilaginous structures. Treatment is with indomethacin. Sidari and Hill review the evidence regarding the diagnosis and treatment of gout and pseudogout. They discuss how diagnosis can be made with fluid microscopy. They recommend treatment with non-steroidal anti-inflammatory drugs. Figure/Illustration A is polarized microscopy of joint fluid showing rhomboid-shaped crystals that are positively birefringent (red circles). These represent calcium pyrophosphate dehydrate crystals seen in pseudogout. Incorrect Answers: Answer A: Boutonniere deformity is characterized by proximal interphalangeal (PIP) joint flexion and distal interphalangeal (DIP) joint extension and is a common hand deformity of rheumatoid arthritis. Rheumatoid arthritis is characterized by insidious onset of morning stiffness and pain that usually affects symmetric PIP and metacarpophalangeal (MCP) joints first. On joint aspiration, leukocyte count ranges from 5,000-50,000/mm^3, but rheumatoid arthritis would not explain the CPPD crystals. Treatment may include methotrexate. Answer C: Heberden nodes are bony protuberances in the DIP joints, which are characteristic of osteoarthritis. Osteoarthritis presents with insidious onset of joint pain that worsens with activity and weight-bearing. On joint aspiration, the leukocyte count is < 2,000/mm^3. Answer D: Juxta-articular osteoporosis is decreased bone density of the bone surrounding the joint space. It is a nonspecific sign that can be seen with many inflammatory joint diseases such as rheumatoid arthritis or septic arthritis. Septic arthritis presents with mono-articular joint pain, erythema, and swelling. Joint aspiration will show a leukocyte count > 50,000/mm^3 and Gram stain may be positive. Answer E: Tophi are deposits of urate crystals in the soft tissue, which are nearly pathognomonic for gout. Gout presents as acute, mono-articular joint pain with physical examination showing a red, inflamed joint (usually the first metatarsophalangeal, knee, or ankle joint). Needle-shaped, negatively birefringent urate crystals are seen in the joint fluid aspirate. Prevention may include allopurinol. Bullet Summary: Pseudogout is associated with positively birefringent, rhomboid-shaped crystals and chondrocalcinosis.
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A 1-year-old girl is brought to a neurologist due to increasing seizure frequency over the past 2 months. She recently underwent a neurology evaluation which revealed hypsarrhythmia on electroencephalography (EEG) with a mix of slow waves, multifocal spikes, and asynchrony. Her parents have noticed the patient occasionally stiffens and spreads her arms at home. She was born at 38-weeks gestational age without complications. She has no other medical problems. Her medications consist of lamotrigine and valproic acid. Her temperature is 98.3°F (36.8°C), blood pressure is 90/75 mmHg, pulse is 94/min, and respirations are 22/min. Physical exam reveals innumerable hypopigmented macules on the skin and an irregularly shaped, thickened, and elevated plaque on the lower back. Which of the following is most strongly associated with this patient's condition?
Cardiac rhabdomyoma
Glaucoma
Optic glioma
Polyostotic fibrous dysplasia
Renal cell carcinoma
A
Cardiac rhabdomyoma
This patient with a seizure disorder, ash-leaf spots (innumerable hypopigmented macules), Shagreen patch (elevated irregular plaque on the lower back), and West syndrome (hypsarrhythmia on EEG and movements consistent with infantile spasms) likely has tuberous sclerosis. Tuberous sclerosis is associated with cardiac rhabdomyomas. Tuberous sclerosis is a neurocutaneous disorder that is inherited in an autosomal dominant fashion. Mutations in TSC1 and TSC2 lead to unregulated cell growth, leading to the formation of hamartomas in various locations. Manifestations of tuberous sclerosis include seizures (secondary to subependymal nodules and cortical dysplasia), mental retardation, renal angiomyolipomas, facial angiofibromas, mitral regurgitation, and hypomelanotic macules (ash-leaf spots). Cardiac rhabdomyoma is strongly associated with tuberous sclerosis and can lead to arrhythmias. West syndrome, which is characterized by infantile spasms, intellectual disability, and hypsarrhythmia (a chaotic mixture of high-amplitude slow waves, multifocal spikes, and intrahemispheric-interhemispheric asynchrony) on EEG, is also associated with tuberous sclerosis. Diagnosis of tuberous sclerosis is made with genetic testing. Advanced imaging (CT or MRI) may reveal cortical tubers and lesions in the third and fourth ventricles. Abdominal imaging may reveal renal angiomyolipomas. The management of seizures in tuberous sclerosis includes anti-seizure medications, though surgery may also be pursued in select cases. Hinton et al. studied the cardiac manifestations of tuberous sclerosis. They found that cardiac rhabdomyomas can lead to arrhythmia later in life. They recommended that infantile spasms in tuberous sclerosis be treated with vigabatrin but other seizure types should be treated similar to other epileptic disorders. Incorrect Answers: Answer B: Glaucoma may occur in patients with Sturge-Weber syndrome, which is characterized by "tram-track" calcifications in the brain, port-wine stains of the face, mental retardation, and epilepsy. Diagnosis is with MRI of the brain with contrast to demonstrate leptomeningeal vascular malformations that are characteristic of the disease. Answer C: Optic glioma is associated with neurofibromatosis type 1 (NF1), which presents with hyperpigmented macules (cafe-au-lait spots), axillary freckling, and cutaneous neurofibromas. Neurofibromas in NF1 may also grow on peripheral nerves, leading to malignant peripheral nerve sheath tumors. Lisch nodules, or benign growths of the iris, may also be seen in NF1. Diagnosis of NF1 is clinical, but can be aided by genetic testing. Answer D: Polyostotic fibrous dysplasia is associated with McCune-Albright syndrome. Polyostotic fibrous dysplasia causes fibrous tissue in bones, leading to growth abnormalities and pathologic fractures. McCune-Albright syndrome is also associated with cafe-au-lait macules, which have irregular borders, and endocrine abnormalities such as precocious puberty and hyperthyroidism. Diagnosis is supported with endocrine tests as well as genetic testing. Answer E: Renal cell carcinoma is associated with Von-Hippel-Lindau syndrome (VHL), which presents with tumors arising in multiple organs. Patients with VHL present with hemangioblastomas in the brain, spinal cord, cerebellum, and retina, and pheochromocytoma. Pheochromocytomas release catecholamines, leading to episodic hypertension, diaphoresis, and palpitations. Diagnosis of VHL is with genetic testing. Bullet Summary: Tuberous sclerosis has numerous manifestations including seizures, angiofibromas, mitral regurgitation, renal angiomyolipoma, ash-leaf spots, mental retardation, and cardiac rhabdomyoma.
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A 55-year-old man presents to urgent care for weakness and weight loss. For the past several months, he has felt progressively weaker and has lost 25 pounds with intermittent abdominal pain. The patient has not seen a physician in 30 years and recalls being current on most of his vaccinations. A few years ago, he went to the emergency department due to abdominal pain and was found to have increased liver enzymes due to excessive alcohol use and incidental gallstones. The patient has a 50 pack-year smoking history. His temperature is 99.5°F (37.5°C), blood pressure is 161/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals an emaciated man. The patient has a negative Murphy sign and his abdomen is non-tender. Cardiopulmonary exam is within normal limits. Which of the following is the most appropriate next step in management?
CT scan of the abdomen and pelvis
CT scan of the chest
HIDA scan
Right upper quadrant ultrasound
Smoking cessation advice and primary care follow up
A
CT scan of the abdomen and pelvis
This patient with weight loss and painless liver dysfunction has symptoms that are suggestive of pancreatic cancer. The most appropriate next diagnostic test is a CT scan of the abdomen and pelvis. Pancreatic cancer presents with weight loss, jaundice, epigastric pain, and fatigue/malaise typically in an older male smoker who uses alcohol. Laboratory tests will demonstrate an elevated alkaline phosphatase and bilirubin. If a patient is presenting with the classic "painless jaundice" then the most appropriate next step in management could be an ultrasound to evaluate for obstruction. Otherwise, the best diagnostic test for this condition is a CT scan of the abdomen to characterize the pancreas and any metastasis to local organs. Treatment is with excision of the tumor through a Whipple procedure in appropriate candidates. Lee and Lee review the evidence regarding the diagnosis of pancreatic cancer. They discuss how abdominal CT or MRI are important in making this diagnosis. They recommend having a high clinical index of suspicion given this diagnosis carries a high mortality rate. Incorrect Answers: Answer B: CT scan of the chest would be appropriate to screen a smoker for lung cancer. If this patient had concern for malignancy with a smoking history, then a CT scan of the chest would be appropriate. It would be appropriate at a later time to assess for pulmonary nodules and lung cancer. Answer C: HIDA (hepatobiliary iminodiacetic acid) scan is an appropriate diagnostic test to characterize a gallstone blockage of the common bile duct if a right upper quadrant ultrasound is not revealing in the diagnosis of acute cholecystitis. Answer D: A right upper quadrant ultrasound can assess the gallbladder for stones and the liver for an abscess, but it will not assess the pancreas for pancreatic cancer (at least in a reliable manner that assesses the entire pancreas). Answer E: Smoking cessation and primary care follow up would be appropriate management for this patient if he only presented requesting general advice to improve his health or stop smoking. His current symptoms are too alarming for malignancy. He should stop smoking regardless. Bullet Summary: The most appropriate initial test for pancreatic cancer is an abdominal CT.
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A 26-year-old woman presents to the emergency department with 1 day of vaginal bleeding and lower abdominal pain. The pain is most pronounced in the lower abdomen and has gotten worse over the past day. Her last menstrual period was 7 weeks ago. She denies any recent abdominal or vaginal trauma. She has no other past medical history and takes no medications. She uses a copper intrauterine device and is in a monogamous relationship. Her temperature is 99°F (37.2°C), blood pressure is 79/50 mmHg, pulse is 135/min, and respirations are 20/min. Physical examination is notable for diffuse abdominal tenderness upon palpation. Pelvic examination demonstrates uterine and adnexal tenderness and uterine bleeding. Laboratory studies are shown below: Hemoglobin: 9.5 g/dL Leukocyte count: 6,000/mm^3 Serum beta-human chorionic gonadotropin (hCG): 2,700 IU/L The patient is started on intravenous fluids. Which of the following is the most appropriate next step in management?
CT of the abdomen and pelvis
Laparoscopy
Methotrexate therapy
Repeat beta-hCG in 48 hours
Transvaginal ultrasound
B
Laparoscopy
This patient’s acute onset abdominal pain with uterine bleeding and tenderness, positive pregnancy test, anemia, and hemodynamic instability are concerning for a ruptured ectopic pregnancy. Emergency laparoscopy is the most appropriate next step in management. An ectopic pregnancy, due to implantation of a fertilized ovum outside the uterine cavity, most commonly in the fallopian tube, can be life-threatening if it ruptures leading to intra-abdominal hemorrhage and shock. Initial laboratory studies should include a complete blood count and a pregnancy test. A patient’s hemodynamic stability should be ascertained immediately as it determines the most appropriate next step in management. In patients who are hemodynamically unstable (hypotensive and tachycardic), a Focused Assessment with Sonography for Trauma (FAST) exam should be immediately performed to evaluate for intraperitoneal bleeding, and ob/gyn should be immediately consulted for emergent surgical intervention (e.g., salpingectomy or salpingotomy). In patients who are hemodynamically stable, transvaginal ultrasound should be performed to determine the gestational sac location, and medical therapy can be initiated (e.g., methotrexate therapy, depending on the size and location of the pregnancy). Cheng et al. compare fertility outcomes of salpingotomy and salpingectomy in women with tubal pregnancies. The authors found that there was no difference in intrauterine pregnancy rates or repeat ectopic pregnancy rates between patients that underwent salpingotomy or salpingectomy. The authors recommend that both salpingectomy and salpingotomy are reasonable options for the surgical management of tubal pregnancies. Incorrect Answers: Answer A: CT of the abdomen and pelvis is useful in determining if there is an intra-abdominal hemorrhage in patients who are hemodynamically stable. Unstable patients should generally not undergo CT. Answer C: Methotrexate therapy is an effective treatment option in patients who are hemodynamically stable. with smaller ectopic pregnancies and lower hCG levels. Answer D: Repeat beta-hCG in 48 hours is reserved for hemodynamically stable patients with non-diagnostic findings on transvaginal ultrasound. If the beta-hCG level is below the discriminatory zone (beta-hCG level 1,500-2,500 IU/L), then it should be repeated in 48 hours. Answer E: Transvaginal ultrasound is reserved for hemodynamically stable patients who are presenting with abnormal uterine bleeding with abdominal/pelvic pain. Bullet Summary: Patients who present with a ruptured ectopic pregnancy with hemodynamic instability are managed with emergent surgical intervention.
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A 24-year-old man presents to his primary care physician with left groin pain. The pain started a year ago and has progressed. He now has 8/10 pain when climbing stairs or rising from a seated position. He localizes the pain to the front of his groin. He denies any erythema or swelling. He has a history of hypertension, sickle cell anemia, and a splenectomy. He drinks a glass of wine with dinner every night and took up scuba diving 2 months ago but otherwise does not exercise. His temperature is 97.5°F (36.4°C), blood pressure is 124/68 mmHg, pulse is 82/min, and respirations are 16/min. His body mass index (BMI) is 18.1 kg/m^2. Physical exam reveals left groin pain at the terminal ranges of hip abduction and internal rotation. There is no point tenderness. A radiograph is obtained as in Figure A. Which of the following components of this patient’s history most strongly predisposed him to this condition?
Alcohol use
History of sickle cell disease
Lack of physical activity
Low BMI
Scuba diving
B
History of sickle cell disease
This patient with sickle cell disease (SCD) presents with anterior groin pain, especially with weight-bearing, and radiographic findings of amorphous lytic and sclerotic lesions of the femoral head, consistent with avascular necrosis. SCD is a predisposing factor for avascular necrosis. Avascular necrosis has a multitude of direct causes, including irradiation, trauma (history of femoral neck fracture), hematologic diseases such as leukemia, SCD, and idiopathic etiology. In traumatic causes, injury to the medial femoral circumflex artery supplying the femoral head causes vascular disruption with resulting osteonecrosis. In SCD, the propensity for sickle-shaped erythrocytes to stick to one another and occlude blood vessels results in loss of circulation to the femoral head with consequent osteonecrosis. Avascular necrosis of the femoral head presents with insidious onset of anterior groin pain. The diagnosis can be secured with plain radiographs, although magnetic resonance imaging has the highest sensitivity and specificity for uncertain cases. Treatment consists of bisphosphonates with crutches (non-weight-bearing) and/or surgery depending on the degree of femoral head collapse and etiology. Akinyoola et al. studied the risk factors associated with avascular necrosis of the femoral head in patients with sickle cell disease. The authors found that the rate of pain crises and annual hospitalizations are associated with avascular necrosis. The authors recommend further study to explain differences in the prevalence of osteonecrosis in different populations with sickle cell disease. Figure/Illustration A shows a plain radiograph of the left hip in a skeletally mature individual, demonstrating amorphous cortical lesions (red arrows) in the femoral head and cortical irregularity (blue arrow) typical of avascular necrosis. Incorrect Answers: Answer A: Excess alcohol use has been associated with the risk of avascular necrosis. This patient uses alcohol in moderation (2 drinks or fewer per day for men) and has no history of alcohol use disorder. Answer C: Lack of physical activity is a predisposing factor for osteoporosis but is not associated with avascular necrosis. Osteoporosis may increase the risk of a hip fracture, which would present with a painful, shortened, and externally rotated lower extremity. Answer D: Low BMI is not a predisposing factor for avascular necrosis. In contrast, obesity has been associated with an increased risk of avascular necrosis. Answer E: Scuba diving can indirectly predispose patients to avascular necrosis through decompression sickness (Caisson Disease). In decompression, dissolved gases in the arterial supply form gas emboli, leading to microvascular occlusion and dysbaric osteonecrosis. However, this patient’s symptoms have been present for 1 year whereas he only recently began scuba diving. Bullet Summary: Sickle cell disease is a predisposing factor for avascular necrosis of the femoral head, which presents as insidious anterior groin pain.
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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
Ondansetron
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.
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A 48-year-old man presents to the emergency room with a 2-hour history of severe abdominal pain, nausea, and vomiting. He states that he has not passed gas or had a bowel movement in 4 days and his pain has worsened and become constant over the past 2 hours. His only medical history includes an appendectomy that he underwent as a child, and he takes no daily medications. His temperature is 38.5°C (101.3°F), blood pressure is 92/60 mmHg, pulse is 138/min, and respirations are 25/min. His pulse oximetry is 99% on room air.There are no cardiopulmonary abnormalities on auscultation. His abdomen is distended and tender in all quadrants, with guarding and rebound present. He also has increased bowel sounds throughout. Laboratory results are as follows: Hemoglobin: 11 g/dL Leukocyte count: 16,500/mm^3 with normal differential Platelets: 250,000/mm^3 Serum: Creatinine: 1.0 mg/dL Glucose: 95 mg/dL Lipase: 45 U/L Total bilirubin: 0.8 mg/dL Alkaline phosphatase: 74 U/L Aspartate aminotransferase (AST, GOT): 32 U/L Alanine aminotransferase (ALT, GPT): 45 U/L Lactate: 7.0 mmol/L Which of the following is the most appropriate next step in management?
Broad-spectrum antibiotics and serial abdominal radiographs
CT angiography of the abdomen and pelvis
Nasogastric tube placement
Supportive care, NPO, and intravenous fluids
Urgent surgical intervention
E
Urgent surgical intervention
This patient with abdominal pain, vomiting, and obstipation is now hemodynamically unstable with fever, leukocytosis, rising lactate, and worsening pain. He most likely has a complicated small-bowel obstruction (SBO) and should undergo immediate surgical intervention. SBO occurs when the normal progression of intraluminal contents in the intestines is interrupted, with most cases stemming from mechanical blockages. A history of abdominal surgery is a risk factor for SBO due to the formation of adhesions. Patients with SBO can typically be managed conservatively including nasogastric tube suction (if discomfort and active vomiting), bowel rest, and intravenous fluid resuscitation. However, patients with findings of a complicated SBO (changes in abdominal pain, fever, leukocytosis, guarding, and hemodynamic instability) require emergent surgical intervention. Delay in abdominal exploration may lead to necrosis, perforation, infection and a significant risk of mortality. Bower et al. review small bowel obstruction. They note the need for operative intervention only in those who fail conservative management. They recommend nonoperative management for most cases of small bowel obstruction. Incorrect Answers: Answer A: Broad-spectrum antibiotics and serial abdominal radiographs alone would not be appropriate for this patient with a deteriorating clinical picture (hemodynamically unstable, worsening abdominal pain, fever, leukocytosis, high lactate). Antibiotics are not indicated in patients with uncomplicated SBO but may be useful in reducing infection risk in patients with complicated SBO. This patient should undergo emergent abdominal exploration in addition to receiving broad-spectrum antibiotics as it is possible he has already experienced bowel perforation. Answer B: Computed tomography angiography (CTA) is useful in the diagnosis of acute mesenteric ischemia, which classically presents with abdominal pain out of proportion to exam (severe pain without much tenderness), vomiting, abdominal distention, and decreased bowel sounds. However, severe peritonitis and obstipation are more characteristic of SBO, and this patient is too hemodynamically unstable to undergo further imaging before proceeding to surgery. Answers 3 & 4: Nasogastric tube placement with supportive care can be used to conservatively manage patients with uncomplicated SBO. This hemodynamically unstable patient has a fever, guarding, peritoneal signs, and newly worsened abdominal pain, indicative of a complicated SBO. He should be taken emergently to the operating room as the next step in management. Bullet Summary: Patients with intestinal obstruction who have a surgical abdomen or hemodynamic instability should undergo immediate surgical intervention.
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A 60-year-old woman presents to the emergency department with back pain after gardening. Her pain is 7/10 in severity, non-radiating, and not relieved by rest. She has never experienced this pain in the past and denies fever, night sweats, unintentional weight loss, and bowel or bladder incontinence. She has hypertension for which she takes hydrochlorothiazide and had a recent asthma flare requiring a prednisone taper. She does not drink alcohol or smoke. Her temperature is 98.6°F (37.0°C), blood pressure is 120/80 mmHg, pulse is 90/min, and respirations are 18/min. Physical exam reveals an uncomfortable middle-aged woman in no acute distress. There is no tenderness to palpation of the spinous processes. Flexion of the hip with the knee extended while the patient is supine does not elicit any pain, nor does forced dorsiflexion of the foot at terminal hip extension. She has 5/5 strength to hip flexion, extension, abduction, and adduction; knee flexion and extension; and ankle dorsiflexion and plantarflexion bilaterally. Bilateral patellar and Achilles reflexes are 2+. Serum laboratory results are as follows: Hemoglobin: 12.0 g/dL Creatinine: 1.1 mg/dL Ca2+: 10.6 mg/dL Which of the following is the most likely diagnosis?
Herniated disc
Lumbosacral strain
Multiple myeloma
Spondylolisthesis
Vertebral compression fracture
B
Lumbosacral strain
This patient presents with acute low back pain after physical exertion without radicular signs (e.g., motor or sensory changes in a nerve root distribution) and a negative straight leg raise, which are most consistent with lumbosacral strain. The evaluation of acute low back pain is focused on eliciting clinical history and signs that would indicate further work-up. In patients without significant trauma, the presence of signs of spinal cord compression (e.g., urinary retention, bowel incontinence, saddle anesthesia, focal neurologic deficits), history of or strong risk factors for cancer, signs of infection, or risk of vertebral compression fracture should prompt additional work-up with imaging such as radiographs or magnetic resonance imaging. If none of these findings are present (most patients with acute low back pain in the primary care setting), then conservative therapy for 4-6 weeks consisting of non-steroidal anti-inflammatory drugs with or without physical therapy is indicated. Patel and Ogle review the management of acute low back pain in the primary care setting. They outline the Waddell signs, which indicate the presence of a functional (signs of excessive pain behavior) aspect of pain. The authors recommend surgical evaluation only in patients with worsening neurologic deficits or intractable pain resistant to conservative treatment. Incorrect Answers: Answer A: Herniated discs occurs when the nucleus pulposus herniates through a weakened part of the annulus fibrosus of an intervertebral disc, resulting in spinal nerve root compression. This causes radicular findings at the level of compression, including weakness in hip abduction and ankle dorsiflexion with L5 involvement and weakness in ankle plantarflexion and reduced Achilles reflex with S1 involvement, and radiation of pain into the ipsilateral lower extremity. The straight leg raise test would be positive, as it exacerbates the nerve root compression. Answer C: Multiple myeloma presents with hypercalcemia, anemia, renal failure, and lytic bone lesions causing bone pain. This patient has no constitutional symptoms to suggest a malignant process and has a mild elevation in serum calcium, which is likely due to her thiazide diuretic. The clear association of her back pain with exertion makes lumbosacral strain more likely. Answer D: Spondylolisthesis occurs when a vertebral body is forwardly subluxated relative to the adjacent vertebral body. This presents as back pain alleviated by rest. Since this can narrow the spinal canal, it may also present with neurogenic claudication, manifesting as buttock or leg pain with walking that can be alleviated by bending forward. This patient’s back pain is not alleviated by rest and is associated with exertion. Answer E: Vertebral compression fractures can present as back pain in a patient with minor or no trauma. These patients have osteoporosis and other risk factors like advanced age or chronic corticosteroid use and would have point tenderness at the site of fracture. This patient does not have point tenderness or risk factors. Bullet Summary: Lumbosacral strain is a common cause of acute low back pain that presents after physical exertion with no radicular signs and a negative straight leg raise test.
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A 4-week-old boy presents with his parents to the pediatrician for a well-child visit. His mother reports he was eating well until 1 week ago when he began vomiting after breastfeeding. His mother has tried increasing the frequency of feeds and decreasing the amount of each feed, but the vomiting seems to be getting worse. The patient now vomits after every feed. The vomitus looks like breast milk. The patient’s mother is exclusively breastfeeding and would prefer not to switch to formula but worries that the patient is not getting the nutrition he needs. Two weeks ago, the patient was in the 75th percentile for weight and 70th for height. He is now in the 60th percentile for weight and 68th percentile for height. His temperature is 98.2°F (36.8°C), blood pressure is 58/37 mmHg, pulse is 144/min, and respirations are 34/min. On physical exam, the patient has dry mucous membranes. His abdomen is soft and non-distended. Which of the following is the most appropriate next step in management?
Abdominal radiograph
Abdominal ultrasound
Supplement breastfeeding with formula
Trial of dairy-free diet
Trial of empiric proton pump inhibitor
B
Abdominal ultrasound
This patient presents with a history of nonbilious, forceful vomiting, poor weight gain, and signs of dehydration, which is consistent with pyloric stenosis. The most appropriate next step in management is abdominal ultrasound to confirm the diagnosis. Pyloric stenosis presents in 4- to 6-week-old infants with nonbilious, post-prandial projectile vomiting. Although pyloric stenosis classically presents with an “olive-shaped” mass in the epigastrium, the mass cannot be palpated in some patients. Any infant who develops new, persistent vomiting in the first 2 months of life and has associated dehydration or failure to thrive should undergo an abdominal ultrasound to evaluate for pyloric stenosis. Patients should undergo resuscitation prior to the definitive treatment, which is a pyloromyotomy. 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: Abdominal radiograph is not helpful in evaluating for pyloric stenosis. It is more useful in the workup of bilious vomiting, as several etiologies of bilious vomiting (such as duodenal atresia and Hirschsprung disease) can be seen on an abdominal radiograph. Treatment of these conditions is surgical excision of the defective segment of colon. Answer C: Supplementing breastfeeding with formula would not be appropriate for this patient as his failure to thrive is driven by gastric outlet obstruction. In the case of pyloric stenosis, formula would cause vomiting just as breastmilk does. Answer D: A trial of a dairy-free diet is used to improve gastroesophageal reflux, but in the setting of this patient’s age and the description of vomiting, he should undergo an abdominal ultrasound to evaluate for pyloric stenosis. Treatment of reflux also involves positional changes during feeding. Answer E: A trial of empiric proton pump inhibitor is indicated for patients with gastroesophageal reflux disease, but it would not address this patient’s pyloric stenosis. Patients can also undergo conservative treatments such as changing positioning during feeding. Bullet Summary: The diagnostic test of choice for pyloric stenosis in infants is an abdominal ultrasound.
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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
Aspirin, heparin, clopidogrel, and cardiac catheterization
Metoprolol and aspirin
Obtain a urinalysis and creatine phosphokinase level
Ringer lactate bolus
E
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.
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A girl presents to her pediatrician for a well-child visit. Her mother reports that she is eating well at home and sleeping well throughout the night. She can jump and walk up and down stairs with both feet on each step. In the doctor’s office, the patient builds a 6-cube tower and imitates a circle. She seems to have a vocabulary of over 50 words that she uses in 2-word sentences. The patient enjoys playing near other children and sometimes argues over toys with her older brother. Her temperature is 98.6°F (37°C), blood pressure is 92/56 mmHg, pulse is 106/min, and respirations are 23/min. On physical exam, she appears well developed and well nourished, and she is following along her growth curves. The child is assessed as developmentally normal. Which of the following is an additional milestone associated with this child’s age?
Balances on one foot
Cuts with scissors
Follows two-step commands
Points to one body part
Turns pages in book
C
Follows two-step commands
This patient is able to walk up and down stairs, can build a 6 cube tower, has a 50+ word vocabulary, and uses 2-word phrases, which suggests she is 2 years of age. Another milestone at 2 years of age is following 2-step commands. In the gross motor category, a child at 2 years of age should be able to jump and walk up and down stairs with both feet on each step. In the fine motor category, the child should be able to copy a line or circle and build a tower of 6 cubes. In the language category, the child should be using over 50 words and putting them together in 2-word phrases. In the social category, a child should be participating in parallel play. In the receptive language category, a child should be able to follow a 2-step command. Persistent developmental delays should prompt investigation for correctable causes such as hearing loss. Scharf et al. review the evidence regarding the use of developmental milestones in assessing children. They discuss how early identification of delays can allow for referral to required services. They recommend paying special attention to sensory function to avoid missing a correctable hearing deficit. Incorrect Answers: Answer A: Balancing on 1 foot is a milestone at 3 years of age. A 2-year-old child would not be expected to maintain balance in that position. Answer B: Cutting with scissors is a milestone at 3 years and 6 months of age. A 2-year-old child would not be expected to be able to hold or use scissors correctly. Answer D: Pointing to one body part is a milestone at 15 months of age. A 2-year-old child should be able to point to 5-6 body parts. Answer E: Turning pages in a book is a milestone at 16 months of age. It would not be considered a milestone for a 2-year-old child. Bullet Summary: At 2 years of age, a child should be able to walk up and down stairs, build a tower of 6 cubes, use a vocabulary of over 50 words in 2-word phrases, and follow a 2-step command.
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A 41-year-old man presents to his primary care provider with a 2-month history of abdominal pain. He says that the pain “comes and goes” throughout the day and usually lasts 20-30 minutes per episode. The pain is above his umbilicus. He denies any feeling of regurgitation or nighttime cough but endorses nausea. He used to eat 3 large meals per day but has found that eating smaller meals more frequently improves his pain. He tried ibuprofen with food and thinks it helped. He has gained 4 pounds since his last appointment 3 months ago. He denies any diarrhea or change in his stools. He has no known medical history. He drinks 5-6 beers on the weekend and has a 20 pack-year smoking history. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 117/72 mmHg, pulse is 72/min, and respirations are 13/min. On physical exam, he is tender to palpation above the umbilicus. Bowel sounds are present. A stool guaiac test is positive. The patient undergoes an endoscopy with a biopsy to diagnose his condition. Which of the following is most likely to be found on histology?
Crypt abscesses in the large intestine
Mucosal defect in the stomach
Noncaseating granulomas in the small intestine
PAS-positive material in the small intestine
Urease-producing organism in the small intestine
E
Urease-producing organism in the small intestine
This patient presents with episodic epigastric pain and nausea that improves with the consumption of food, which suggests a diagnosis of a duodenal ulcer. Duodenal ulcers are often caused by infection with Helicobacter pylori, a urease-producing organism. Duodenal ulcers are erosions and defects in the mucosal lining of the small intestines that allow for damage to the underlying tissues. Patients will classically present with abdominal pain that is localized to the epigastric region. Duodenal ulcers improve upon eating due to the secretion of bicarbonate in the small intestines, whereas gastric ulcers are exacerbated by food due to the release of gastric acid in the stomach. Duodenal ulcers are most commonly caused by infection with H. pylori. If the patient tests positive for H. pylori infection, treatment involves triple therapy with amoxicillin, clarithromycin, and a proton pump inhibitor. Patients who fail to improve should undergo esophagogastroduodenoscopy, which remains the gold standard for diagnosis. Ciociola et al. studied the prevalence of H. pylori infection in patients with gastric ulcers. They found that about many had confirmed infections. They recommend against empiric treatment without prior confirmation of infection. Incorrect Answers: Answer A: Crypt abscesses are found on histology in ulcerative colitis. Ulcerative colitis presents with bloody diarrhea and abdominal pain. Patients can also experience musculoskeletal pain, uveitis, and dermatologic findings such as erythema nodosum or pyoderma gangrenosum. Treatment may include mesalazine. Answer B: A mucosal defect in the stomach is the pathologic description of a gastric ulcer. This patient’s presentation of epigastric pain that improves with the consumption of food is more consistent with an ulcer in the duodenum. Treatment of a gastric ulcer may include proton pump inhibitors. Answer C: Noncaseating granulomas are found on histology in Crohn disease. This patient denies any diarrhea (often associated with pain), which is the primary feature of Crohn disease. Other findings include abscesses, fistulas, and strictures. Treatment may include mesalamine and infliximab. Answer D: PAS-positive material is found in the lamina propria on small intestinal biopsy in Whipple disease. In addition to abdominal pain, Whipple disease is characterized by diarrhea with flatulence and steatorrhea, fever, arthralgias, and weight loss. Treatment is with ceftriaxone or penicillin. Bullet Summary: Duodenal ulcers typically present with episodic epigastric abdominal that is improved after eating and are associated with H. pylori infection.
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A 62-year-old man arrives at the emergency room complaining of chest pain and difficulty breathing. He reports that the dyspnea started 2 months ago after he had the flu. At first, the difficulty breathing occurred whenever he went up and down 1 flight of stairs, but the dyspnea progressively worsened since then. This morning, he developed chest pain and difficulty breathing while sitting at the kitchen table. The patient’s medical history is significant for hypertension, type 2 diabetes mellitus, and rheumatoid arthritis. His medications include aspirin, lisinopril, metformin, and sulfasalazine. His temperature is 97°F (36.1°C), blood pressure is 130/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 97% O2 on room air. On physical exam, jugular venous distension during both inspiration and expiration, mild abdominal distention, and 2+ bilateral lower extremity edema is noted. A chest radiograph is obtained and the result is shown in Figure A. Which of the following is the most likely cause of this patient's symptoms?
Cardiac tamponade
Cirrhosis
Constrictive pericarditis
Pulmonary arterial hypertension
Restrictive cardiomyopathy
C
Constrictive pericarditis
This patient is presenting with peripheral edema, ascites, elevated jugular venous distension, and pericardial calcifications on chest radiograph, which are all consistent with a diagnosis of constrictive pericarditis. Constrictive pericarditis is the result of scarring and loss of elasticity of the pericardial sac. Common etiologies include idiopathic, post-viral, post-cardiac surgery, post-radiation, tuberculosis-related, or connective tissue disorder-related. It presents as right heart failure including symptoms such as dyspnea, chest pain relieved by leaning forward, progressive peripheral edema, and ascites. A physical exam will identify an early diastolic pericardial knock, pericardial calcifications on chest radiograph, and jugular venous distension (JVD). Kussmaul sign can also occur where the JVD paradoxically rises during inspiration instead of falling. The diagnosis is supported with echocardiography. Management may require pericardiectomy for severe cases. Welch and Oh review the evidence regarding the diagnosis and treatment of constrictive pericarditis. They discuss how this disease can be evaluated using a cardiac MRI. They recommend surgical pericardiectomy in many cases. Figure/Illustration A is a frontal chest radiograph with pericardial calcifications (red circle). These findings are consistent with constrictive pericarditis. Incorrect Answers: Answer A: Cardiac tamponade can present similarly to constrictive pericarditis. Hypotension and pulsus paradoxus (a greater than 10 mmHg decrease in the systolic blood pressure during inspiration), will commonly present in patients with cardiac tamponade. A chest radiograph will show an enlarged cardiac silhouette rather than calcifications in some cases. Treatment is with emergent pericardial decompression. Answer B: Cirrhosis can present with ascites and peripheral edema, but will not have a marked elevation in jugular venous pressure. Cirrhosis is the end-stage result of many hepatic pathologies. Treatment is with the control of sequelae such as hepatic encephalopathy and venous congestion. Answer D: Pulmonary arterial hypertension, like constrictive pericarditis, can present with exertional dyspnea, chest pain, and peripheral edema if it progresses to right ventricular failure. A chest radiograph may show enlarged central pulmonary arteries and peripheral pulmonary vessels. Treatment is with reduction of pulmonary pressure such as with endothelin inhibitors. Answer E: Restrictive cardiomyopathy can also present as right heart failure with similar symptoms to constrictive pericarditis. A history of an infiltrative disease (e.g., amyloidosis and sarcoidosis) often favors restrictive cardiomyopathy, as does an audible S3 on a physical exam. Calcification of the pericardium on the chest radiograph is more consistent with constrictive pericarditis. Bullet Summary: Constrictive pericarditis presents with symptoms of right heart failure, progressive peripheral edema, ascites, jugular venous distension, pericardial knock, and pericardial calcifications on a chest radiograph.
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A 25-year-old woman presents to her primary care physician with joint and muscle pain. She has a general aching/pain in her joints that lasts all day. The pain is constant and is not relieved by rest or activity. She also has pain in her hands at times and occasionally notices trouble swallowing when she is eating. She has a medical history of anxiety and is not currently taking any medications. Her temperature is 98.6°F (37°C), blood pressure is 115/72 mmHg, pulse is 74/min, and respirations are 11/min. On physical exam, the patient is a healthy young woman with a sunburn. Her hands are mildly edematous with the findings in Figure A. Laboratory values are below: Serum: Na+: 145 mEq/L K+: 4.4 mEq/L Cl-: 100 mEq/L HCO3-: 24 mEq/L BUN: 9 mg/dL Glucose: 70 mg/dL Creatinine: .7 mg/dL Ca2+: 10 mg/dL Mg2+: 1.8 mEq/L Which of the following antibodies is most likely to aid in making the diagnosis in this patient?
Anti-dsDNA
Anti-IgG Fc region
Anti-nuclear antibody
Anti-ribonuleoprotein
Anti-Ro
D
Anti-ribonuleoprotein
This patient is presenting with diffuse muscle/joint pain, Raynaud phenomenon, and dysphagia suggesting a diagnosis of mixed connective tissue disease. Anti-ribonucleoprotein (U1) antibodies are found in this disease. Mixed connective tissue disease can present with a vast array of symptoms. The most common symptoms are Raynaud phenomenon (vascular hyper-reactivity that presents with burning/pain in the hands where the hands change color from white to blue to red, seen in Figure A), arthralgias, myalgias, dysphagia, and edema of the hands. Anti-U1 ribonucleoprotein antibodies are commonly found in mixed connective tissue disease and are a specific marker. In this disease, there is typically an absence of renal involvement. Treatment involves steroids as well as calcium channel blockers for symptomatic control of Raynaud phenomenon. Batu et al. review the evidence regarding patients with mixed connective tissue disease. They found that many patients have overlapping symptoms with systemic lupus erythematosus and systemic sclerosis. They recommend studying patients with these mixed characteristics in order to better understand which treatment modalities are effective. Figure/Illustration A is a clinical photograph showing the blanching of the distal fingers (red box). This finding demonstrates the Raynaud phenomenon during the early phase where the vessels have spasmed. Incorrect Answers: Answer A: Anti-dsDNA is a specific marker in systemic lupus erythematosus (SLE). SLE presents with SOAP BRAIN MD - Serositis, Oral ulcers, Arthritis, Photosensitivity, Blood disorders (hemolytic anemia, etc.), Renal involvement, ANA, Immune phenomena (anti-dsDNA, Smith), Neurological symptoms, Malar rash, and Discoid rash. Four of these criteria must be met to suspect a diagnosis of SLE. Treatment is with hydroxychloroquine. Answer B: Anti-IgG Fc region is representative of rheumatoid factor which is found in rheumatoid arthritis. Rheumatoid arthritis can present with fever, weight loss, and joint pain that worsens in the morning and improves with activity. It is common for the joints of the hands to become deformed due to the disease process. Treatment is with methotrexate. Answer C: Anti-nuclear antibodies (ANA) are likely positive in this patient; however, they are not more likely to aid in making the diagnosis than anti-ribonucleoprotein antibodies are. ANA are non-specific and can be found in many rheumatological conditions such as MCTD, lupus, and rheumatoid arthritis. Answer E: Anti-Ro antibodies are found in Sjogren syndrome which presents with dry eyes, dry mouth, dental caries, and dyspareunia. A salivary gland biopsy can be used to confirm the diagnosis. Treatment includes artificial tears, vitamin D supplementation, and pilocarpine or cevimeline. Bullet Summary: Mixed connective tissue disease is associated with anti-ribonuleoprotein antibodies and presents with Raynaud phenomenon, arthralgia/arthritis, swollen hands, sclerodactyly, and myositis.
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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
Supportive care and monitoring
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.
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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
Cardioversion
Defibrillation
Procainamide
C
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.
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A 53-year-old African-American woman presents to her primary care doctor with increasing difficulty climbing the stairs. She noticed the weakness approximately 3 months ago. It has gotten progressively worse since that time. She has also noticed increasing difficulty combing her hair, standing from a seated position, and experiences muscle aches. Her medical history is significant for hypertension treated with lifestyle modification and gastroesophageal reflux disease (GERD). Her temperature is 98.6°F (37.0°C), blood pressure is 130/65 mmHg, pulse is 80/min, and respirations are 16/min. She has 3/5 strength to shoulder abduction and hip flexion bilaterally. Her strength is 5/5 to wrist extension and ankle plantar flexion. No skin rashes are noted. Which of the following is the most accurate test to confirm this patient’s diagnosis?
Electromyography
MRI
Muscle biopsy
Serum aldolase
Serum creatine kinase
C
Muscle biopsy
This patient with insidious, subacute, progressive, symmetric proximal muscle weakness (difficulty climbing the stairs, combing her hair, standing from a seated position) without associated skin lesions most likely has polymyositis. The most accurate diagnostic test for polymyositis is muscle biopsy. Polymyositis is caused by an autoimmune reaction that leads to cell-mediated cytotoxicity against unidentified skeletal muscle antigens, primarily affecting the endomysium. It is more often found in women, patients between 30-60 years of age, and African-Americans. Clinical features of polymyositis include symmetric proximal muscle weakness that may be accompanied by myalgia, dysphagia (due to pharyngeal muscle weakness), restrictive lung disease (due to respiratory muscle weakness), cardiac involvement (myocarditis, heart failure), interstitial lung disease, and increased risk of malignancy. There are no accepted diagnostic criteria for polymyositis; however, diagnosis is usually secured with a combination of clinical features and other tests. Routine initial studies can include a complete blood count, complete metabolic panel, C-reactive protein, erythrocyte sedimentation rate, lactate dehydrogenase, aldolase, creatine kinase, autoantibodies (anti-nuclear antibody, anti-Jo-1), and electromyography (EMG). Muscle biopsy of an affected muscle is the gold standard for diagnosis and will typically show muscle fiber damage, CD8+ T cells in the endomysium, and overexpression of MHC-I on the sarcolemma. First-line treatment of polymyositis includes glucocorticoids and a steroid-sparing agent (methotrexate, azathioprine). Subsequent treatments can include intravenous immunoglobulins, rituximab, or plasmapheresis. Amato et al. review the evaluation and treatment of inflammatory myopathies such as polymyositis, dermatomyositis, and inclusion body myositis. They found that polymyositis typically presents in adult life with symmetric proximal leg greater than arm weakness. They recommend that a muscle biopsy be done on any patient considered to have polymyositis, preferably prior to starting immunosuppressive treatment. Incorrect Answers: Answer A: Electromyography (EMG) can help distinguish myopathic weakness (e.g., polymyositis) from neuropathic causes of weakness (e.g., amyotrophic lateral sclerosis, myasthenia gravis). Characteristic EMG findings include fibrillations, early recruitment of motor unit potentials, and abnormal low-amplitude motor unit potentials. EMG is not a confirmatory test and is less sensitive than muscle biopsy for diagnosing myositis. Answer B: MRI can be used to demonstrate areas of edema, atrophy, fatty replacement, and calcification in patients with suspected polymyositis. MRI can assess large regions of muscle, thus avoiding sampling error with muscle biopsy. The findings in MRI are nonspecific and require correlation with muscle biopsy for definitive diagnosis. MRI can also be used to help select the site of muscle biopsy. Answer D: Serum aldolase can occasionally be used in the diagnostic investigation of suspected polymyositis if creatine kinase is normal. Aldolase elevation is nonspecific and may be seen in fasciitis, drug-induced myositis, and connective tissue disease-associated interstitial lung disease. Answer E: Serum creatine kinase (CK) is the most sensitive muscle enzyme exam and should be part of the initial diagnostic investigation of any patient with suspected polymyositis. CK elevation is nonspecific and may be seen in myriad conditions such as inflammatory insult, metabolic injury, drug-induced injury, and infection. Bullet Summary: Muscle biopsy is the gold standard for the diagnosis of polymyositis.
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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
Valproic acid
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.
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A 31-year-old man presents to the emergency department for fever, malaise, and cough. For the last week, his cough has been progressively worsening, and he has been noticing blood in his sputum. He denies any recent travel or sick contacts and infrequently smokes cigarettes. Approximately 5 weeks ago, he presented with similar symptoms and was found to have a right upper lobe lung infiltrate, a CD4 count of 40/mm^3, and an HIV viral load of 115,000 copies/mL. He was appropriately treated and discharged home. Four weeks after initiation of treatment, his CD4 count was over 400/mm^3 and HIV viral load was negligible. His temperature today is 102°F (38.9°C), blood pressure is 130/90 mmHg, pulse is 100/min, and respirations are 20/min. A radiograph of the chest demonstrates new nodules in the left upper lobe and hilar adenopathy. Which of the following is the most appropriate next step in management?
Continue current anti-retroviral treatment, antimicrobial, and anti-tuberculoid therapies
Determine drug sensitivities against the patient’s pulmonary infection
Discontinue antimicrobial treatment
Obtain a D-dimer level
Start high-dose glucocorticoid treatment and discontinue antiretroviral therapy
A
Continue current anti-retroviral treatment, antimicrobial, and anti-tuberculoid therapies
This patient with recently diagnosed HIV and tuberculosis infection (upper lung lobe infiltrate) is presenting with worsening symptoms after being appropriately treated. Worsening of symptoms after initiation of these therapies is concerning for immune reconstitution inflammatory syndrome (IRIS), which is managed by continuing current combined antiretroviral medications, antibiotics for opportunistic infections and symptom management. IRIS describes a paradoxical worsening of a patient's underlying infection after initiating antiretroviral therapy for patients with HIV. IRIS can also occur in HIV-uninfected patients with tuberculosis or leprosy who are started on antimycobacterial treatment. The pathogenesis of IRIS is unclear, but in patients with HIV, antiretroviral therapy increases the amount of CD4+ T-helper cells, which can lead to an exaggerated inflammatory response (e.g., increased cytokine release) towards the infection. Thus, there is a paradoxical worsening of the infection symptoms. When patients have a paradoxical worsening of their symptoms in the setting of starting their antiretroviral therapy, the therapy should be continued with symptomatic management (acetaminophen for fever). Nelson et al review IRIS. They note it is seen in patients with HIV. They recommend antiretroviral therapy and antibiotics for bacterial infections. Incorrect Answers: Answer B: Determining drug sensitivities against the patient’s pulmonary infection would be of low diagnostic utility since IRIS best explains his current clinical presentation. Answer C: Discontinuing antimicrobial treatment would not be appropriate since the patient has findings concerning for tuberculosis (upper lobe lung infiltrate), and lack of treatment can lead to progression of his infection. Answer D: Obtaining a D-dimer level is only indicated in low-risk patients for pulmonary embolism by Wells criteria. Answer E: Starting high-dose glucocorticoid treatment and discontinuing antiretroviral therapy would not be appropriate since discontinuing his antiretroviral therapy would not control his HIV infection, which would place him at greater risk of acquiring opportunistic infections. Corticosteroid use is part of symptom management as it decreases the inflammatory response. Bullet Summary: Immune reconstitution inflammatory syndrome (IRIS) can result in worsening of infectious symptoms and is managed by continuing antiviral therapy with symptomatic treatment.
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A 57-year-old woman presents to her primary care physician with weakness for the past 6 months with symptoms that have worsened recently. She feels fatigued, depressed, and has gained 10 pounds which she attributes to feeling too weak to go to the gym. She was recently treated for an ear infection with an antibiotic which she completed 3 weeks ago, and experienced a rash which she believes was an allergic reaction to her treatment. The patient has a medical history of diabetes mellitus that is well-controlled with insulin and metformin. She has been admitted multiple times for hypoglycemia secondary to missing meals. Her temperature is 99.5°F (37.5°C), blood pressure is 127/68 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for 2/5 strength in the patient's upper and lower extremities, sparse fine hair on her body, and a rash on her face and arms. Laboratory values are ordered as seen below. Serum: Na+: 139 mEq/L K+: 3.3 mEq/L Cl-: 100 mEq/L HCO3-: 25 mEq/L BUN: 20 mg/dL Glucose: 70 mg/dL Creatinine: 1.1 mg/dL Ca2+: 10.2 mg/dL AST: 12 U/L ALT: 10 U/L A muscle biopsy is obtained as seen in Figure A. Which of the following is the most likely explanation for this patient's presentation?
Dermatomyositis
Hypokalemia
Hypothyroidism
Inclusion body myositis
Polymyositis
D
Inclusion body myositis
This patient who presents with weakness and a rash has a biopsy suggestive of inclusion body myositis. Inclusion body myositis presents with an insidious onset of weakness, a mildly elevated CRP and CK, and a rash. A muscle biopsy in this disease will demonstrate endomysial inflammation with basophilic-rimmed vacuoles within the muscle fiber sarcoplasm. Electromyography is typically abnormal, and the most accurate/confirmatory test is a muscle biopsy. There is no cure for this disease and no standard course of treatment. Corticosteroids are not usually effective but intravenous immunoglobulins can be effective. Naddaf et al. review the evidence regarding the diagnosis and treatment of patients with inclusion body myositis. They discuss how this disease is still refractory to treatment. They recommend a muscle biopsy to establish a definitive diagnosis. Figure/Illustration A is a muscle biopsy demonstrating endomysial inflammation with basophilic-rimmed vacuoles (red circle) within the muscle fiber sarcoplasm. These findings are classically seen in patients with inclusion body myositis. Incorrect Answers: Answers 1 & 5: Dermatomyositis and polymyositis present similarly with muscle weakness and an elevated CK, CRP, and aldolase. However, dermatomyositis presents with dermatologic findings. A biopsy would demonstrate inflammation surrounding the muscle fascicle (dermatomyositis) or within the muscle fascicle (polymyositis). Treatment may include azathioprine and methotrexate. Answer B: Hypokalemia could present with weakness and cardiac abnormalities. Though this patient is hypokalemic, her biopsy demonstrates inclusion body myositis. Treatment of hypokalemia is with potassium repletion. Answer C: Hypothyroidism presents with depression, fatigue, scarce lanugo, myopathy, and weight gain (as this patient has). Though myopathy from hypothyroidism is plausible, her symptoms of weakness have a confirmatory biopsy. Treatment of hypothyroidism is with thyroid hormone replacement. Bullet Summary: Inclusion body myositis presents with weakness and an elevated CRP/CK with a muscle biopsy demonstrating endomysial inflammation with basophilic-rimmed vacuoles within the muscle fiber sarcoplasm.
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A 6-month-old girl is brought to the pediatrician by her father for a scheduled check-up. She was in her usual state of health until 6 weeks ago when she was hospitalized for bacterial pneumonia. She received amoxicillin and had a resolution of her symptoms. Her father reports that she seems “back to normal.” She can roll over by herself and can sit up without support. She often babbles but sometimes does make identifiable sounds like “dada” and “baba.” They recently started feeding her mushy cereal, which she seems to like but occasionally spits up. She has started teething and sometimes becomes “fussy.” Vital signs are within normal limits. A physical examination is unremarkable. The patient’s father brought a 4-week follow-up chest radiograph taken at the hospital, as shown in Figure A. Which of the structures labeled in Figure A would be most likely to change in response to another infectious illness?
A
B
C
D
E
A
A
The thymus gland (labeled in A) is visible on a chest radiograph in infants and will change in response to illness due to its immunologic role. The thymus gland is the site of T-cell maturation. The thymus is visible on a chest radiograph in infants and continues to grow throughout childhood. It is located in the anterior part of the superior mediastinum. On a chest radiograph, the thymus may appear as a “widened mediastinum,” continuous with the superior border of the heart (Figure A) or as a triangular-shaped opacity known as the thymic “sail sign," located towards the right of the mediastinum. At puberty, the gland will decrease in size and eventually be replaced by fatty tissue. Askin and Young review the evidence regarding the history and function of the thymus. They discuss how this structure is involved in immune function. They recommend monitoring for the absence of this structure in immunodeficient patients. Figure A shows a normal chest radiograph of an infant with a normal cardiothymic contour. Incorrect Answers: Answer B: B points to the trachea. Deviation of this structure can be seen in patients with tension pneumothorax. Treatment is with needle thoracostomy followed by chest tube placement. Answer C: C points to the apex of the heart. This structure can be enlarged in congenital heart conditions. Treatment is with surgical correction of these conditions. Answer D: D points to the stomach. This structure can be abnormal in patients with pyloric stenosis. Treatment is with pyloromyotomy. Answer E: E points to the diaphragm. This structure can be abnormal in patients with diaphragmatic hernias. Treatment is with surgical closure of the defect. Bullet Summary: The thymus is normally visible on a pediatric chest radiograph, but it is replaced by fatty tissue after puberty.
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A 57-year-old man presents to the emergency department for weight loss and abdominal pain. The patient has felt steadily more fatigued over the past month and has lost 22 pounds without effort. Today, he fainted prompting his presentation. The patient has no significant medical history. He does have a 33-pack-year smoking history and drinks 4 to 5 alcoholic drinks per day. His temperature is 99.5°F (37.5°C), blood pressure is 100/58 mmHg, pulse is 100/min, respirations are 17/min, and oxygen saturation is 98% on room air. The patient is very thin and appears pale. Stool fecal occult blood testing is positive. A CT scan of the abdomen is performed demonstrating a mass in the colon with multiple metastatic lesions scattered throughout the abdomen. The patient is informed of his diagnosis of metastatic colon cancer. When the patient conveys the information to his family, he focuses his efforts on discussing the current literature in the field and the novel therapies that have been invented. He demonstrates his likely mortality outcome which he calculated using the results of a large multi-center study. Which of the following is this patient most likely demonstrating?
Dissociation
Intellectualization
Optimism
Pessimism
Rationalization
B
Intellectualization
This patient is using facts and logic to emotionally distance himself from his stressful diagnosis. This ego defense method is suggestive of intellectualization. Intellectualization typically occurs in patients who have received a very stressful diagnosis or bad news for a close loved one. They will often focus on the science and research of their disease rather than focus on emotionally processing this stressful event. This is a way of distancing themselves from the diagnosis and their circumstances. These patients may have difficulty connecting with others because they become extremely focused on data. Arnold reviews the evidence regarding the diagnosis of intellectualization. He discusses how this term can either refer to the isolation of affect or the general use of reason. He recommends a better understanding of how this ego defense works. Incorrect Answers: Answer A: Dissociation is the detachment of the mind from the emotional state/body. A more specific diagnosis for this patient would be intellectualization as he is detaching himself from the emotional state of the diagnosis and instead focusing on the science behind his condition. Answer C: Optimism is trying to find the best in a bad situation. This patient's focus on treatment outcomes and absence of emotion suggests intellectualization as the employed defense mechanism. Patients who are optimistic will still be able to emotionally process information. Answer D: Pessimism is finding the worst in most situations. Pessimism would be a possible answer if the patient had a very treatable disease but thought that poor outcomes would occur regardless. Answer E: Rationalization is the use of logic or reasoning (that can be inappropriate) in order to substantiate one's behavior even when it is inappropriate. An example is justifying theft because a person was in need. Bullet Summary: Intellectualization is the use of facts and logic to distance oneself from a stressful situation.
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A 55-year-old man presents to his primary care physician with persistent symptoms of depression. He started taking fluoxetine 3 weeks ago when he was diagnosed with major depressive disorder after a divorce. Since then, he notes that his symptoms are roughly unchanged. His other medications include albuterol. His temperature is 99.3°F (37.4°C), blood pressure is 132/88 mmHg, pulse is 77/min, respirations are 14/min, and oxygen saturation is 99% on room air. Physical exam reveals a depressed affect. His neurologic exam is non-focal. A serum thyroid stimulating hormone (TSH) resulted from his last visit and is noted to be 4.8 µU/mL (normal: 0.5-5 µU/mL). Which of the following is the most appropriate next step in management?
Administer levothyroxine
Change medication to bupropion
Change medication to escitalopram
Increase dose of current medication
Maintain dose of current medication
E
Maintain dose of current medication
This patient is presenting with major depressive disorder but has not given his current selective serotonin reuptake inhibitor (SSRI) at least 4 weeks to begin working. Thus, he should be reassured and maintained on the current dose of his SSRI, with follow-up after the 4 week mark to see if his symptoms have improved. Major depressive disorder is managed initially with cognitive behavioral therapy and pharmacotherapy. The typical first-line medication is an SSRI. SSRIs may take at least 4 weeks or more to begin working, so patients that return before this time period should be reassured and encouraged to let the medication take effect as long as they are not experiencing suicidal thoughts or intent. If the medication is not working after at least 4 weeks, the next steps in management could include increasing the dose of the current medication, changing SSRIs, adding another antidepressant agent, or adding a low dose of levothyroxine. All patients with suicidal ideation and either intent or a plan to hurt themselves should be hospitalized involuntarily. Gautam et al. discuss the current management algorithms for major depressive disorder. They note different options including CBT and pharmacotherapy. They recommend appropriate treatment with a first-line agent based on the patient and their circumstances. Incorrect Answers: Answer A: Administering levothyroxine is appropriate as an augmenting agent in patients who do not have an improvement in symptoms on SSRIs or other antidepressants even if their TSH is within the normal range. However, it would not be attempted at this point given this patient has not given his current medication sufficient time to begin working. There is limited evidence to support its use overall. Note that this patient's TSH is within normal limits. In some cases, T3 administration is used in refractory depression. Answer B: Changing the medication to bupropion could be appropriate in patients who want to avoid weight gain, those who want to quit smoking, or those who are trying to avoid sexual side effects. It also is an appropriate second-line agent or could be added to a first-line agent; however, this patient’s current medication should be tried for at least 4 weeks before changing medications. Answers 3 & 4: Changing medication to escitalopram or increasing the dose of the current medication are both viable strategies if a patient has failed to improve on their first medication after at least 4 weeks on the medication. Increasing the dose early may lead to symptoms of serotonin excess when the drug takes effect. Bullet Summary: SSRIs are the first-line agents in the management of major depressive disorder and should be tried for at least 4 weeks before changing the treatment regimen.
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A 24-year-old man presents to the emergency department with abdominal pain, nausea, and vomiting. The symptoms began towards the end of running a marathon and persisted after finishing the race. The patient is otherwise healthy and has no other medical problems. His temperature is 98.0°F (36.7°C), blood pressure is 105/71 mmHg, pulse is 113/min, respirations are 19/min, and oxygen saturation is 98% on room air. Physical exam reveals a distended and tender abdomen. A radiograph is performed as seen in Figure A. Which of the following is the most likely diagnosis?
Cecal volvulus
Ileus
Ogilvie syndrome
Sigmoid volvulus
Small bowel obstruction
A
Cecal volvulus
This young, healthy patient presents with abdominal pain, nausea, vomiting, and a distended, tender abdomen with a radiograph demonstrating the "coffee bean sign," likely has a cecal volvulus. Cecal volvulus is the second most common cause of volvulus after sigmoid volvulus and presents with abdominal pain, constipation, vomiting, abdominal distention, and a tympanitic abdomen. It is more common in young patients who are active (e.g. marathon runners). The classic conventional radiographic finding in cecal volvulus is the coffee bean sign, in which closed loops of intestine distend with gas, making visible the medial and lateral walls of dilated bowel. Diagnosis of cecal volvulus can be confirmed with a CT scan. Management involves analgesia, intravenous fluids, and surgical detorsion. Hasbahceci et al. review the presentation, diagnosis, and management of cecal volvulus. The authors find that abdominal radiography has a low sensitivity for cecal volvulus. The authors recommend surgical management of cecal volvulus with resection with right hemicolectomy and primary ileocolic anastomosis. Figure/Illustration A is a radiograph demonstrating the "coffee bean" sign which is suggestive of a cecal volvulus. Incorrect Answers: Answer B: Ileus presents after surgery or manipulation of the intestines with decreased peristalsis of the intestine leading to a failure to pass gas or have bowel movements. Treatment involves treating any electrolyte abnormalities, encouraging ambulation, and avoiding medications that decrease peristalsis (such as opioids). Answer C: Ogilvie syndrome occurs when the colon wall is not appropriately undergoing peristalsis leading to a dilated colon with abdominal distension. It is more common in the elderly and critically ill patients. Treatment involves the placement of a rectal tube to decompress the intestines and treatment of any underlying medical conditions. Answer D: Sigmoid volvulus is more common in elderly and critically ill patients and presents with constipation, an absence of bowel movements, and a distended, tympanitic abdomen. Although the coffee bean sign may also be seen in patients with sigmoid volvulus, patients tend to be older. In addition, plain radiography typically demonstrates multiple air-fluid levels in a sigmoid volvulus versus usually only a single air-fluid level in a cecal volvulus. Treatment involves flexible sigmoidoscopy and surgery. Answer E: Small bowel obstruction presents with a distended and tympanitic abdomen with nausea, vomiting, and an absence of flatus or bowel movements. Radiography may demonstrate distended bowel and air-fluid levels, and the most accurate test is a CT scan of the abdomen. It occurs secondary to adhesions entrapping the bowel. Management involves the placement of a nasogastric tube, intravenous fluids, and surgery. Bullet Summary: Cecal volvulus presents in younger patients with abdominal pain, distention, constipation, nausea, vomiting, and the "coffee bean" sign on radiography.
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A 26-year-old woman presents to the emergency department because she feels as though her heart is "beating out of her chest." She states that she feels well apart from the discomfort of that sensation. Her temperature is 97.9°F (36.6°C), blood pressure is 124/84 mmHg, pulse is 180/min, respirations are 22/min, and oxygen saturation is 98% on room air. An ECG is obtained as seen in Figure A. After unsuccessful attempts at vagal maneuvers, the emergency physician administers an IV medication. The effect of the medication wears off in seconds. Which of the following is a potential side effect of this medication?
Disulfiram-like reaction
Flushing
Photosensitivity
Seizure
Tachycardia
B
Flushing
This patient presents with a narrow complex tachycardia that is regular which is suggestive of supraventricular tachycardia (SVT) and was pharmacologically cardioverted with adenosine (as it has a very short half-life). Side effects of adenosine include flushing, hypotension, bronchospasm, and heart block. SVT presents with a narrow-complex, regular tachycardia. The diagnosis is made with these findings on ECG. Management in an unstable patient is centered on cardioversion. Otherwise, the first step in management is centered on vagal maneuvers. If these fail, adenosine is the first-line pharmacologic treatment. While a relatively safe drug, side effects of adenosine include hypotension, heart block, flushing, and bronchospasm. Patients may also report a "sense of impending doom" following administration, thought to be the result of the transient asystole adenosine produces. Persistent SVT refractory to adenosine may be further managed with calcium channel blockers, beta blockers, or cardioversion. Colucci et al. review the diagnosis and management of common types of supraventricular tachycardias. The most common types of supraventricular tachycardia are caused by a reentry phenomenon producing accelerated heart rates. Symptoms may include palpitations, chest pain, lightheadedness or dizziness, and dyspnea. If Wolff-Parkinson-White syndrome is present, expedient referral to a cardiologist is warranted because ablation is a potentially curative option. Figure A is an ECG demonstrating SVT. Note the regular narrow complex tachycardia that is a defining feature of this condition. Incorrect Answers: Answer A: A disulfiram-like reaction was once thought to be associated with medications such as metronidazole. Metronidazole is an antibiotic that covers anaerobes. When alcohol is consumed in a patient on disulfiram, headache, malaise, nausea, and vomiting may be experienced. Answer C: Photosensitivity is associated with medications such as sulfonamides, amiodarone, and tetracycline. Patients should be advised to stay out of the sun on these medications. Answer D: Seizures are not associated with adenosine. Isoniazid, bupropion, imipenem/cilastatin, tramadol, enflurane, and metoclopramide are all known to increase the risk of seizures. Seizures often present with tonic-clonic activity, tongue biting, urinary incontinence, and a postictal phase. Answer E: Tachycardia can be associated with medications such as atropine which reduce vagal tone on the heart increasing the heart rate. Medications that block the AV node would be expected to cause bradycardia. Bullet Summary: Adenosine is a first-line pharmacological agent in supraventricular tachycardia and can cause hypotension, heart block, flushing, and bronchospasm.
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A 69-year-old man presents to his primary care physician for a general checkup. The patient works as a farmer and gardens in his spare time. He has a medical history of poorly managed type 2 diabetes mellitus and irritable bowel syndrome. His current medications include lisinopril, metformin, insulin, fiber supplements, and a multivitamin. On physical exam, there is a calm, obese gentleman. Inspection of the patient's scalp reveals the finding shown in Figure A. The patient is sent home and instructed to keep a blood glucose journal. Three months later, the patient returns with his glucose journal. In addition, he complains of a cut on his scalp that is not seeming to heal. The patient's glucose journal reveals an average blood glucose of 175 mg/dL. His temperature is 98.8°F (37.1°C), blood pressure is 134/86 mmHg, pulse is 80/min, and respirations are 13/min. On inspection of the patient's scalp, the finding in Figure B is noted. Which of the following is the most likely diagnosis?
Actinic keratosis
Basal cell carcinoma
Melanoma
Poor wound healing secondary to diabetes
Squamous cell carcinoma
E
Squamous cell carcinoma
This patient with a non-healing ulcer on the site of a previous actinic keratosis most likely has sustained progression of the lesion to squamous cell carcinoma. Squamous cell carcinoma (SCC) is a common dermatological malignancy. Patients can present with actinic keratosis, which is a pre-malignant lesion in sun-exposed areas. These lesions can progress to squamous cell carcinoma if risk factors such as sun exposure continue. The presentation of SCC is typically an ulcerative lesion in a sun-exposed area. A biopsy will demonstrate atypical keratinocytes and malignant cells, invasion into the dermis, and keratin “pearls” on histology. Treatment is with wide local surgical excision with histologic confirmation of negative margins. Waldman and Schmults present a review of the evidence regarding the epidemiology and treatment of squamous cell skin cancer. They discuss how the incidence of this disease continues to increase due to an aging population. They recommend local excision and treatment due to the risk of metastasis. Figure/Illustration A is a clinical photograph demonstrating a rough scaly patch on the skin (red circle). This finding is consistent with actinic keratosis, which is a pre-malignant lesion typically found on sun-exposed areas. Figure/Illustration B demonstrates an ulcerative lesion with wound formation on the skin (red circle). This finding is consistent with squamous cell carcinoma, a malignant lesion that can progress from actinic keratosis. Incorrect Answers: Answer A: Actinic keratosis represents a pre-malignant condition that this patient initially presented with (as demonstrated in Figure A). His current presentation reflects SCC, a progression from actinic keratosis given the new ulceration and non-healing wound. Answer B: Basal cell carcinoma presents with a shiny or pearly appearance, rather than the ulcerative appearance that is seen in SCC. This disease should be treated with surgical excision. Answer C: Melanoma presents with a pigmented lesion that has an irregular border, an uneven shape, and areas that differ in pigmentation. Patients with melanoma should undergo surgical staging as this disease can become metastatic and should be treated with surgical excision. Answer D: Poor wound healing secondary to diabetes would be possible if there was a history of trauma to the scalp. The history of actinic keratosis and the current ulcerative lesion, point more toward a diagnosis of SCC. Non-healing wound in patients with diabetes are usually located on the foot rather than on the scalp. Bullet Summary: Actinic keratosis is a pre-malignant lesion that occurs in sun-exposed areas that can progress to squamous cell carcinoma.
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A 25-year-old woman presents for a new patient physical exam. Aside from occasional shin splints, she has a relatively unremarkable medical history. She takes oral contraceptive pills and a multivitamin daily. All of her age appropriate immunizations are up to date. Her periods have been regular, occurring once every 28 to 30 days with normal flow. She is sexually active with 2 partners, who use condoms routinely. Her prior pap smear was performed last year and was normal. Her mother has diabetes and coronary artery disease, and her father passed away at age 45 after being diagnosed with colon cancer at age 40. Her grand-aunt underwent bilateral mastectomies after being diagnosed with breast cancer at age 60. Her temperature is 36.7°C (98°F), blood pressure is 115/80 mmHg, pulse is 65/min, respirations are 12/min, and oxygen saturation is 100% on room air. Her physical exam is within normal limits. Which of the following is the most appropriate recommendation for this patient?
Colonoscopy in 5 years
Colonoscopy in 10 years
Mammogram now
Pap smear in 5 years
Pap smear with human papillomavirus (HPV) DNA testing now
A
Colonoscopy in 5 years
This patient is presenting for a health evaluation with a family history significant for her father's death due to colon cancer at age 45. She should thus get a colonoscopy when she reaches the age 10 years prior to her father's age of diagnosis or in 5 years (at age 30). The age threshold for colon cancer screening varies based on the patient's risk factors, including family history of colon cancer, inflammatory bowel disease, and/or genetic disorders. In the absence of significant risk factors, colonoscopies can be used for screening starting at age 45 and repeated every 10 years if results are normal. If there is family history of colon cancer (one or more first degree relatives, including parent, sibling, or child), colonoscopies should be recommended starting at age 40 or 10 years prior to the age of the family member's diagnosis, whichever is earlier. Polyps or grossly abnormal colon tissue may be biopsied for grading and guide further diagnostic imaging workup. Aasma et al. review the clinical guidelines for colorectal cancer screening. They found that recent epidemiological data shows an increase in the incidence of colorectal cancer in individuals younger than 50 years. They recommend starting colonoscopy screening at 45 years of age in the average-risk adult population. Incorrect Answers: Answer B: Colonoscopy in 10 years is reasonable, but it is not the earlier of the 2 age thresholds mentioned above. Answer C: Pap smear with HPV DNA screening for cervical cancer is indicated every 5 years for women aged 30 to 65 who wish to extend the screening interval from the 3 year interval for pap smear alone. Answer D: Pap smear alone is indicated every 3 years for women of ages 21 to 65. Answer E: Mammogram to screen for breast cancer is recommended every 2 years for women aged 50 to 74. Bullet Summary: For patients with a family history of colon cancer, screening should start at age 40 or 10 years prior to the age of the family member's diagnosis, whichever is earlier.
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A 67-year-old man is seen on the surgical floor after a transplant procedure. The previous day, the patient had a renal transplant from a matched donor. He is currently recovering and doing well. The patient has a medical history of IV drug use, diabetes mellitus, oral cold sores, hypertension, renal failure, and dyslipidemia. The patient's current medications include lisinopril, atorvastain, insulin, and aspirin. Prior to the procedure, he was also on dialysis. The patient is started on cyclosporine. The patient successfully recovers over the next few days. His temperature is 98.5°F (36.9°C), blood pressure is 121/76 mmHg, pulse is 70/min, and respirations are 13/min. Which of the following medications should be started in this patient?
Azithromycin
Low dose acyclovir
Penicillin
TMP-SMX
Valacyclovir
D
TMP-SMX
This patient is presenting status post renal transplant and is currently on immunosuppressive therapy. This patient should be started on TMP-SMX as prophylaxis against Pneumocystis jiroveci pneumonia (PJP). After a transplant procedure, patients are started on immunosuppressive therapy in order to avoid rejection of the transplanted organ. The most important interventions for a patient after a transplant procedure are to receive all of their vaccinations and to receive TMP-SMX as prophylaxis against PJP. Other appropriate but less dire measures include giving ganciclovir as prophylaxis against cytomegalovirus (CMV). Patients should be closely monitored to avoid transplant rejection as well as infection. Weyant et al. review the evidence regarding the treatment of patients with PJP. They discuss how TMP-SMX has been the classic prophylaxis and treatment for this disease. They recommend considering echinocandins for patients who experience significant side effects. Incorrect Answers: Answer A: Azithromycin is appropriate prophylaxis for patients with HIV with CD4 counts < 50. Patients on immunosuppressive drugs should receive some of the same prophylactic medications but they do not require azithromycin upon initiation of immunosuppressive medications. Answer B: Low-dose acyclovir would be an inappropriate intervention in this patient after a transplant procedure. Ganciclovir and high-dose acyclovir are the appropriate options for prophylaxis of CMV. Answer C: Penicillin can be used as prophylaxis for patients with rheumatic heart disease or asplenia; however, it would not be the most appropriate initial measure in a transplant patient. Answer E: Valacyclovir is the treatment for herpes simplex virus (HSV). It is not routinely indicated as prophylaxis in transplant patients. HSV will present with vesicular rashes developing on the affected areas. Bullet Summary: Patients on immunosuppressive therapy after a transplant should be vaccinated (pneumovax, hepatitis A and B, influenza, and tetanus-diphtheria) and started on TMP-SMX.
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An 83-year-old man is brought to the emergency department by his daughter due to confusion over the last 2 weeks. The patient initially became less talkative than usual before starting to confuse her with her sister. The patient also could not remember what day of the week it was and he was out of groceries and likely had been for several days when visited. While she was talking to him, he fell asleep and was difficult to rouse. The patient is unable to provide a clear history. He has a medical history of hypertension, glaucoma, and osteoarthritis in his left knee that requires him to walk with a cane. He takes hydrochlorothiazide, timolol eyedrops, and occasional acetaminophen. His temperature is 98.5° F (36.9° C), blood pressure is 122/74 mmHg, pulse is 75/min, and respirations are 14/min. The patient is lethargic but arousable, oriented only to person, and disheveled. Neurologic exam reveals intact cranial nerves, 1+ deep tendon reflexes throughout, and 4/5 strength in all extremities. There is a 3 x 5 cm green-blue area of ecchymosis on the lateral aspect of his left knee. A CT of the head without contrast is performed and shown in Figure A. Which of the following factors plays the earliest role in the progression towards this patient’s presentation?
Aneurysmal rupture
Cerebral atrophy
Elderly abuse
Hypertension
Increased cerebrospinal fluid
B
Cerebral atrophy
This patient is an elderly man with progressive confusion over weeks, a nonfocal neurologic exam, and a CT of the head showing a hypodense crescent, most consistent with chronic subdural hematoma (SDH). Cerebral atrophy plays the earliest role in this presentation, resulting in exposure of the bridging veins and subsequent rupture with even minor trauma. Chronic subdural hematomas present gradually, most often with headaches, somnolence, light-headedness, cognitive impairment, and rarely, seizures. Typically, the neurologic exam is largely normal but rarely focal deficits such as contralateral hemiparesis from compression of the cortex underlying the hematoma or ipsilateral hemiparesis from the lateral displacement of the opposite cortex may occur. Chronic SDH most often occurs in the setting of cerebral atrophy related to increased age, chronic alcohol use, and prior traumatic brain injury. Exposure of the bridging veins allows even minor trauma to precipitate subdural bleeding. A non-contrast CT of the head is indicated in any elderly patient with decreased mentation and concern for chronic SDH. Since old blood appears hypodense, imaging will allow for the determination of whether the bleed is acute or chronic. Supportive care is usually sufficient for treatment but surgical evacuation of the hematoma can be considered in patients with focal deficits or an expanding hematoma. Mehta et al. review the evidence regarding the diagnosis and management of chronic subdural hematomas in elderly patients. They discuss how crescentic layering of fluid in the subdural space on a non-contrast computed tomography scan is diagnostic of this condition. They recommend close follow-up as many patients will develop a recurrence of this condition. Figure/Illustration A shows a non-contrast CT scan of the head with a hypodense crescent left side (red circle). This finding is consistent with a chronic subdural hematoma. Incorrect Answers: Answer A: Aneurysmal rupture is an extremely uncommon cause of SDH and typically causes subarachnoid hemorrhage instead. It often presents with “the worst headache of my life” or “thunderclap headache,” and would show hyperdensity in the subarachnoid space on head CT. Surgical clipping or endovascular treatment can prevent rebleeding. Answer C: Elder abuse can be subtle and should always be considered when an elderly patient presents with confusion, bruising, or dishevelment. In children, abuse is an important cause of SDH, which may occur in the setting of “shaken baby syndrome.” However, abuse is not especially associated with chronic SDH in the elderly population, and there is another plausible reason for this patient’s presentation (trauma from a fall as evidenced by the resolving ecchymosis on his knee). Answer D: Hypertension is a major risk factor for stroke, which typically presents with focal neurologic deficits such as hemiparesis, aphasia, or sensory loss. Although this patient does have a history of hypertension, he is normotensive in the emergency room and has non-focal neurologic decline that has been slowly progressive, which is less consistent with stroke. Furthermore, in contrast to this patient’s CT scan, acute ischemic stroke would likely present with a normal head CT and prior infarct would cause a hypodensity in the parenchyma itself. Treatment is with risk factor optimization when a patient presents outside the window for thrombolytic agents. Answer E: Increased CSF due to poor reabsorption by arachnoid granules is the cause of normal pressure hydrocephalus (NPH). Patients typically present with the “wet, wacky, and wobbly” triad – urinary incontinence, mental status changes, and magnetic gait. Head CT would show dilated ventricles and management includes CSF shunting. Bullet Summary: Chronic subdural hematomas may occur in elderly patients due to cerebral atrophy and subsequent exposure of bridging veins that may tear with minor trauma.
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A 45-year-old African American woman presents to her primary care physician for not feeling well. She states she has had a cough for the past week. She also has abdominal pain and trouble focusing that has been worsening and has lost 5 pounds recently. Her gastroesophageal reflux disease (GERD) has been poorly controlled recently. The patient is a non-smoker and has a history of GERD for which she takes antacids. Her temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. A physical exam is within normal limits. Laboratory studies are ordered and are below: Serum: Na+: 139 mEq/L K+: 4.1 mEq/L Cl-: 101 mEq/L HCO3-: 24 mEq/L Urea nitrogen: 12 mg/dL Glucose: 70 mg/dL Creatinine: 0.9 mg/dL Ca2+: 12.5 mg/dL Alkaline phosphatase: 150 U/L Phosphorus: 2.0 mg/dL Urine: Color: amber Nitrites: negative Sodium: 5 mmol/24 hours Red blood cells: 0/hpf Which of the following is the most likely explanation of this patient's current presentation?
Antacid overuse
Increased 1,25-dihydroxyvitamin D
Increased parathyroid hormone
Malignancy
Viral illness
C
Increased parathyroid hormone
This patient is presenting with hypercalcemia and low phosphate levels suggesting a diagnosis of hyperparathyroidism. Hyperparathyroidism can cause hypercalcemia which typically presents with "bones, stones, moans, and groans," which corresponds to symptoms of bone pain (increased bone resorption), kidney stones, GI pain, and change in mental status/trouble focusing. The increased PTH results in renal phosphate wasting, increased production of 1,25-dihydroxy vitamin D, and increased renal calcium reabsorption. It also results in increased osteoclast activity resulting in increased bone breakdown. This will lead to laboratory changes of hypercalcemia and hypophosphatemia. Taniegra reviews the evidence regarding hyperparathyroidism. She discusses how this disease is usually discovered asymptomatically with routine blood work. She recommends surgical treatment with intractable cases as success rates are high. Figure A is a chest radiograph that is within normal limits. No masses are seen which suggests that malignancy is a less likely diagnosis. Incorrect Answers: Answer A: Antacid overuse for this patient's GERD can lead to a milk-alkali syndrome which presents with hypercalcemia and metabolic alkalosis. This patient does not have metabolic alkalosis. Treatment is with the discontinuation of the offending agent. Answer B: Increased 1,25-dihydroxy vitamin D occurs in sarcoidosis which presents with cough, fatigue, and weight loss in an African American woman. Increased vitamin D by itself will result in hypercalcemia, and hyperphosphatemia rather than hypophosphatemia. Treatment is supportive and/or with corticosteroids. Answer D: Malignancy (lung or renal cancer) is possible given this patient's presentation. Hypercalcemia of malignancy is a common feature with PTH-related protein effects; however, the patient's normal urinalysis and normal chest radiograph as well as non-smoking history point more toward a diagnosis of increased PTH. Answer E: Viral illness is possible given this patient's systemic symptoms; however, it does not explain this patient's hypercalcemia and hypophosphatemia. Treatment of seasonal viruses is generally supportive though specific viruses such as influenza have agents such as oseltamivir that may be helpful. Bullet Summary: Hyperparathyroidism presents with "bones, stones, moans, and groans," as well as laboratory findings of hypercalcemia and hypophosphatemia.
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A 57-year-old man is brought to a neurologist by his wife for abnormal behavior. He was normal until around 3 months ago when he started forgetting important meetings and misplacing items around the house. One month ago, the patient was fired from his job as a software engineer after attempting to grope a coworker. The patient expresses frustration that he is no longer able to concentrate enough to enjoy his hobby of solving puzzles. His medical history is significant for gastroesophageal reflux disease for which he takes omeprazole. He has no family history of neurologic disease. His temperature is 98.6°F (37.0°C), blood pressure is 120/75 mmHg, pulse is 70/min, and respirations are 16/min. During the examination, the patient’s cell phone rings unexpectedly causing the patient to suddenly jerk his arms and legs. Further diagnostic investigation is performed which reveals an elevated 14-3-3 protein on cerebrospinal fluid analysis. Which of the following is the most likely diagnosis?
Alzheimer disease
Creutzfeldt-Jacob disease
Dementia with Lewy bodies
Frontotemporal dementia
Subcortical leukoencephalopathy
B
Creutzfeldt-Jacob disease
This patient with rapidly progressive dementia over 3 months, declining executive function, impaired concentration, decreased memory, startle myoclonus (sudden jerking of extremities in response to unexpected stimulus), and elevated 14-3-3 protein levels on cerebrospinal fluid (CSF) analysis most likely has Creutzfeldt-Jakob disease (CJD). CJD is a human prion disease in which the normal prion protein (PrPc) converts to a beta-pleated sheet disease-associated form (PrPsc) which is resistant to proteases. CJD is usually spontaneous. Rarely, CJD may be familial or acquired (from iatrogenic transmission or dietary exposure). Aggregates of PrPsc lead to spongiform vacuolation. Clinically, this manifests with rapidly progressive mental deterioration with prominent executive dysfunction (declining concentration, judgment, and memory) and startle myoclonus. Further diagnostic investigation may reveal 14-3-3 protein on CSF analysis and synchronous sharp wave complexes on electroencephalogram (EEG). CJD usually leads to death within 1 year. There is no known effective treatment for this disease. Uttley et al. review the diagnosis, clinical presentation, and epidemiology of Creutzfeldt-Jakob disease (CJD). They discuss how CJD in a global context is increasing in prevalence and how incubation periods as long as 40 years have been observed for iatrogenic cases. They recommend heightened awareness of this disease in order to mitigate iatrogenic transmission. Incorrect Answers: Answer A: Alzheimer disease is characterized by gradual cognitive decline with difficulty completing activities of daily living. Impaired executive function early in the disease course, rapidly progressive disease, elevated 14-3-3 protein on CSF analysis, and startle myoclonus are not found in Alzheimer disease. Answer C: Lewy body dementia (LBD) presents with alterations in consciousness, disorganized speech, visual hallucinations, extrapyramidal symptoms, and early compromise of executive function. Although this patient’s dementia is characterized by early compromise of executive function, the absence of fluctuating levels of consciousness, absence of extrapyramidal symptoms, and presence of startle myoclonus make CJD more likely than LBD. Answer D: The behavioral variant of frontotemporal dementia (FTD) is associated with early-onset personality and behavioral changes, and it may be associated with extrapyramidal symptoms. Memory is not usually affected until late in the disease course. Answer E: Subcortical leukoencephalopathy (Binswanger disease) is a type of small vessel vascular disease that presents with prominent subcortical dementia. This manifests clinically as slowed mental processing, impaired cognition, apathy, and depression. This disease almost always presents in the context of chronic hypertension and is usually seen in older patients. Bullet Summary: Creutzfeldt-Jacob disease is a fatal prion disease that presents with sudden-onset and rapidly progressive dementia and startle myoclonus or seizures.
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A 24-year-old man presents to the emergency room after a motor vehicle collision. He was the driver in a head-on collision and was not wearing a seatbelt. The patient is acutely intoxicated and is attempting to attack the nurses. Soon after presentation, he is sedated, paralyzed, and intubated. His medical history is unknown. His temperature is 97.6°F (36.4°C), blood pressure is 74/34 mmHg, pulse is 180/min, respirations are 32/min, and oxygen saturation is 98% on room air. Physical exam is notable for jugular venous distension. An electrocardiogram (ECG) is performed as seen in Figure A. Which of the following is the most appropriate next step in management?
Needle thoracostomy
Packed red blood cells
Pericardial window
Pericardiocentesis
Tube thoracostomy
D
Pericardiocentesis
This patient is presenting after trauma with hypotension, tachycardia, jugular venous distension, and electrical alternans on ECG, which is concerning for cardiac tamponade. The most important initial step in management is pericardiocentesis. Cardiac tamponade can occur after trauma, infections, or malignancy. It can occur if there is an accumulation of fluid in the pericardial sac with inadequate time for the pericardial sac to stretch and for the heart to adapt to the compression that causes diastolic dysfunction. Patients will present with hypotension, tachycardia, jugular venous distension, muffled heart sounds, and pulsus paradoxus (blood pressure drop > 10 mmHg with inspiration). The diagnosis can be supported with ultrasonography (in the clinical setting a point of care ultrasound will often be performed first); however, the initial step in management is a pericardiocentesis which will stabilize the patient. Subsequent management requires the placement of a pericardial drain or window, as well as treatment of the underlying cause. Prabhakar et al. discuss pericardial decompression syndrome, an infrequent complication of pericardiocentesis. The authors find that pericardial decompression syndrome presents with paradoxical hemodynamic instability and/or pulmonary edema following pericardiocentesis, especially in patients with underlying myocardial dysfunction. The authors recommend careful monitoring of patients after pericardiocentesis for the development of this complication. Figure/Illustration A is an ECG demonstrating electrical alternans. Note the alternating large QRS complexes (blue arrows) and small QRS complexes (green arrows) which are suggestive of the heart swinging in a fluid-filled pericardium. Incorrect Answers: Answers 1 & 5: Needle thoracostomy followed by tube thoracostomy is the appropriate management of a tension pneumothorax. A tension pneumothorax would also present with hypotension, tachycardia, and jugular venous distension; however, it would not present with muffled heart sounds and electrical alternans on ECG. On exam, there would be an absence of unilateral breath sounds and the presence of tracheal deviation. Answer B: Packed red blood cells are appropriate management of a hypotensive and tachycardic trauma patient when it is suspected that their unstable vitals are secondary to hemorrhage. In this patient, there is conclusive evidence (jugular venous distension and electrical alternans) that this patient has cardiac tamponade. Answer C: Pericardial window is the appropriate long-term treatment of recurrent pericardial effusions as it allows for a permanent conduit for the drainage of fluid. An acutely unstable patient should first be stabilized with a pericardiocentesis before going to the operating room for this procedure. Bullet Summary: The most important initial step in the management of cardiac tamponade in a hemodynamically unstable patient is pericardiocentesis.
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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
Sinoatrial node
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.
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A 69-year-old man presents to his primary care provider for a general checkup. He has a medical history of type 2 diabetes mellitus, hypertension, depression, obesity, and a myocardial infarction 7 years ago. The patient's prescribed medications are metoprolol, aspirin, lisinopril, hydrochlorothiazide, fluoxetine, metformin, and insulin. The patient has not been filling his prescriptions regularly and can not remember what medications he has been taking. His temperature is 99.5°F (37.5°C), pulse is 96/min, blood pressure is 180/120 mmHg, respirations are 18/min, and oxygen saturation is 97% on room air. Laboratory values are obtained and the results are shown below: Serum: Na+: 139 mEq/L K+: 4.3 mEq/L Cl-: 100 mEq/L HCO3-: 24 mEq/L BUN: 7 mg/dL Glucose: 170 mg/dL Creatinine: 1.2 mg/dL Which of the following cardiac findings would be expected on physical exam?
Fixed splitting of S1 and S2
Heart sound after S2
Heart sound before S1
Holosystolic murmur at the apex
Normal S1 and S2
C
Heart sound before S1
This patient who presents with poorly managed hypertension over a long period of time most likely has compensatory hypertrophy of the heart. The most likely heart sound in this patient is an S4 gallop that occurs immediately before S1. Chronic hypertension that is poorly controlled can lead to concentric hypertrophy of the heart. This adaptive mechanism creates a stiff and non-compliant left ventricle. When blood enters this stiff ventricle, the typical sound heard is an S4 heart sound which is heard right before S1. This sound represents the atrial kick that pushes blood against a non-compliant left ventricle in the last phase of diastole. Treatment is by meticulous control of the underlying cardiac pathophysiology such as hypertension. Kino et al studied the relationship between the S1 and S4 heart sounds in aging patients. They found that an S4 heart sound can be missed in cases of prominent S1 splitting. They recommend studying heart sounds in order to not accidentally miss an S4 finding. Incorrect Answers: Answer A: Fixed splitting of S1 and S2 describes an atrial septal defect. The atrial septic defect allows blood to communicate between both sides of the heart and equalizes atrial pressures. Asymptomatic patients may not need treatment but surgical repair of the defect should be performed in symptomatic cases. Answer B: A heart sound after S2 suggests a S3 heart sound which would be found in fluid-overloaded states such as heart failure. This post-systolic gallop is the result of incomplete emptying of the ventricle prior to the start of diastole so that the blood from the atria falls against an already full chamber. Treatment is with diuresis and treatment of the underlying heart failure. Answer D: A holosystolic murmur heard at the apex describes mitral valve regurgitation. This would be possible if a papillary muscle had ruptured in an acute myocardial infarction. Treatment is with mitral valve replacement in symptomatic patients. Answer E: A normal S1 and S2 is possible but is less likely given this patient's long-standing history of poorly managed hypertension. Hypertension usually causes concentric hypertrophy of the ventricle and thereby leads to increased resistance to diastolic filling. Bullet Summary: An S4 heart sound is created by blood entering a stiff and non-compliant left ventricle and is often heard in patients with long-standing hypertension.
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A 66-year-old man presents to the clinic with a 6-week history of bloody stools. He describes the blood as bright red wisps that float on the surface of the stool. He has no pain with bowel movements but notes on occasion he may strain. He does not endorse fatigue, fever, constipation, diarrhea, nausea, vomiting, abdominal pain, weight loss, or dyspnea. His last colonoscopy was 3 years prior to presentation and was benign. He has a medical history significant for hypertension that is well-controlled with lisinopril. He had an uncomplicated appendectomy when he was 23 years old. He has no family history of gastrointestinal malignancy. His temperature is 98.6°F (37.0°C), blood pressure is 140/90 mmHg, pulse is 76/min, and respirations are 16/min. On physical exam, the patient's abdomen is soft, non-distended, and non-tender with normal bowel sounds and without organomegaly or masses. The anus is patent without fissures, hemorrhoids, or skin tags. Which of the following is the most appropriate next step in management?
Colonoscopy
Computerized tomography scan of abdomen and pelvis
Increase fiber in diet
Observation
Sigmoidoscopy
A
Colonoscopy
This patient is presenting with painless bright red blood per rectum. Because he is over 50 years old and having rectal bleeding, he should be evaluated for malignancy with a colonoscopy. Bright red blood per rectum has a broad differential diagnosis including anal fissures, hemorrhoids, angiodysplasia, inflammatory bowel disease, diverticular bleed, infection, polyps, and colon cancer. However, as patients age, their risk for malignancy increases significantly. Therefore, stable patients older than 50 years of age with rectal bleeding, regardless of their symptoms or family history, should receive a colonoscopy to rule out malignancy. Patients younger than 50 with warning signs of colon cancer (abnormal bowel habits, weight loss, family history of colon cancer, iron deficiency anemia) should also be evaluated with a colonoscopy. Often, colonoscopy is performed routinely at the age of 50 or 10 years prior to the age of presentation for malignancy in family members (whichever is sooner). Of note, even if there is an apparent cause for a lower GI bleed in patients 50 or older (such as hemorrhoids) colonoscopy is still indicated to rule out malignancy. Barry et al. note the guidelines for colon cancer screening. They note the different age cutoffs. The USPSTF recommends screening for colorectal cancer in all adults aged 50-75 years. Incorrect Answers: Answer B: Computerized tomography scan of the abdomen and pelvis is incorrect because colonoscopy is the gold standard for diagnosis of suspected colon cancer. A CT scan may detect larger masses and a CTA may be performed if there is active bleeding and concern for an arterial source of bleeding that can be intervened upon. Answer C: Increasing fiber in the diet may help patients with constipation or hemorrhoidal bleeding. Constipation presents with straining to have bowel movements and hard stools. However, because of this patient's age, he needs a colonoscopy to rule out malignancy. Answer D: Observation is not appropriate at this patient's age. Patients greater than 50 years old with bright red blood per rectum should receive a colonoscopy to rule out malignancy despite any recent negative colonoscopy results. Answer E: Sigmoidoscopy is less preferred to colonoscopy, as a smaller portion of the colon would be visualized, and colonic polyps or malignancies may be missed. For this reason, it is not the preferred study in this patient. Bullet Summary: Patients with bright red bleeding per rectum over 50 years old or with warning signs of colonic malignancy (weight loss, abnormal bowel habits, positive family history, or anemia) should receive a colonoscopy.
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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
Hypertrophy of sphincter muscle
Non-IgE-mediated immunologic reaction
Passage of gastric contents into esophagus
Telescoping of the intestine
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.
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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
Tranexamic acid
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.
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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
Zolpidem
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.
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A 68-year-old man presents for evaluation by a neurologist for an involuntary hand tremor that started approximately 3 months ago. The shaking improves when he reaches for objects but returns when he is not using his hand. His temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. His left hand exhibits a 4-6 Hz tremor when resting on his lap. The tremor extinguishes with voluntary movement. In addition, his left upper extremity has increased tone with passive range of motion. Which of the following is the most likely diagnosis?
Cerebellar stroke
Essential tremor
Huntington disease
Lacunar stroke
Parkinson disease
E
Parkinson disease
This patient who presents with a low amplitude, 4-6 Hz resting tremor that resolves with voluntary movement and associated rigidity most likely has Parkinson disease. Parkinson disease is caused by degeneration of dopaminergic neurons in the substantia nigra pars compacta of the basal ganglia. This neurodegenerative process leads to movement abnormalities such as bradykinesia, “cogwheel” muscle rigidity (intermittently increased muscle tone with passive range of motion), shuffling gait, postural instability, and a resting tremor sometimes referred to as “pill-rolling” in quality. Parkinson disease usually presents with unilateral symptoms and subsequently progresses to bilateral involvement. First-line treatment involves dopaminergic drugs such as carbidopa-levodopa. Antimuscarinic agents can also be used for symptom control. Zhong et al. review the role of the cerebellum in tremor in Parkinson disease. The authors find that the cerebellum may modulate tremor amplitude via cerebello-thalamo-cortical circuits. The authors recommend further study of the cerebellum as an alternative therapeutic target in Parkinson patients with dopamine-resistant tremors. Incorrect Answers: Answer A: Cerebellar stroke can manifest as an intention tremor, which presents as an oscillatory tremor that increases in amplitude at the endpoint of visually goal-directed movement. Intention tremors are best exhibited in the finger-nose-finger test in which the patient repeatedly touches their nose and then extends their arm to touch the examiner's finger. Patients would also have more severe symptoms including ataxia, nausea, vomiting, and nystagmus. Answer B: Essential tremor is a condition that is often familial and presents as a high frequency (4-9 Hz) tremor that occurs with sustained posture and is worsened with movement. Treatment includes propranolol and primidone. Answer C: Huntington disease is an autosomal dominant trinucleotide repeat disorder that leads to neurodegeneration of the caudate and putamen. Chorea is a hallmark movement abnormality associated with Huntington disease and presents with spontaneous, jerking, purposeless involuntary movements. Answer D: Lacunar stroke can result in ischemia to the subthalamic nucleus and produce hemiballismus. This presents as spontaneous unilateral explosive movements of an upper extremity and occasionally the ipsilateral lower extremity. Other motor deficits are also expected when there is a lacunar infarct. Bullet Summary: Resting tremor is a hallmark of Parkinson disease and presents with low amplitude, 4-6 Hz oscillatory rhythmic movements that temporarily extinguish with voluntary movement.