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https://step2.medbullets.com/testview?qid=108992
A 64-year-old man presents to the emergency room with a headache and nausea. He reports that he was rocking his grandson to sleep when the symptoms began. He states the pain is constant and is primarily located on his right side. When asked to indicate the area of pain, he says that it surrounds his eye and upper forehead. He had one episode of vomiting. The patient also reports difficulty seeing out of his right eye, which he attributes to excessive tearing. The patient’s past medical history is significant for hypertension. His medications include hydrochlorothiazide. His temperature is 98.6°F (37°C), blood pressure is 135/91 mmHg, pulse is 72/min, and respirations are 12/min. The patient’s right eye is shown in Figure A. Upon physical examination, the right pupil is minimally responsive to light and the globe feels firm. A right-sided carotid bruit is appreciated. Which of the following is the most appropriate prophylaxis for this patient’s condition?
Acetazolamide
Amitriptyline
Clopidogrel
Epinephrine
Verapamil
A
Acetazolamide
This patient is presenting with sudden-onset unilateral vision loss and an orbitofrontal headache with a dilated pupil and a hard ocular globe suggesting a diagnosis of acute angle-closure glaucoma. Long-term management of angle-closure glaucoma can include acetazolamide. Examination of the eye in a patient with acute-closure glaucoma will reveal a red eye that is rock-hard and a mid-dilated pupil which is minimally reactive to light. The fundoscopic exam will show an increased optic cup-to-disk ratio (>0.4) and tonometry will show increased intraocular pressure. Gonioscopy is the diagnostic gold standard. Acute treatment and long-term management involve the administration of beta-blockers, alpha-2-agonists, and carbonic anhydrase inhibitors such as acetazolamide to decrease intraocular pressure. The definitive treatment is iridotomy. Airaksinen et al. review the treatment of closed-angle glaucoma. They discuss how a combination of acetazolamide and beta-blockers can terminate an attack. They recommend using 1 drop of pilocarpine 3 hours after intravenous acetazolamide administration. Figure/Illustration A is a clinical photograph showing an eye with injected conjunctiva (red circles) and a mid-dilated pupil. These findings are consistent with angle-closure glaucoma. Incorrect Answers: Answer B: Amitriptyline can be used as prophylaxis for migraines. Migraines present as unilateral, pulsating headaches that may be associated with nausea or photophobia. Patients with migraines can sometimes experience an aura with visual field changes; however, they would not have exam findings of a rock-hard eye or injection. Answer C: Clopidogrel may be used as a conservative treatment for carotid atherosclerosis, which can be an embolic source for central retinal artery occlusion (CRAO). CRAO presents as acute, painless, monocular vision loss. A fundoscopic exam will demonstrate ischemia of the retina. Answer D: Epinephrine is contraindicated in the treatment of angle-closure glaucoma as it increases pupillary dilation. It is used in the management of open-angle glaucoma. This would present with gradually patchy loss of vision and is generally painless. Answer E: Verapamil is used as prophylaxis for cluster headaches. Cluster headaches present as unilateral, repetitive, brief headaches associated with severe peri-orbital pain, lacrimation, rhinorrhea, and Horner syndrome (miosis, ptosis, and anhidrosis). Bullet Summary: Pharmacologic management of acute angle-closure glaucoma involves beta-blockers, alpha-2 agonists, and carbonic anhydrase inhibitors.
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A 42-year-old woman is enrolled in a randomized controlled trial to study cardiac function in the setting of several different drugs. She is started on verapamil and instructed to exercise at 50% of her VO2 max while several cardiac parameters are being measured. During this experiment, which of the following represents the relative conduction speed through the heart from fastest to slowest?
Atria > Purkinje fibers > ventricles > AV node
AV node > ventricles > atria > Purkinje fibers
Purkinje fibers > ventricles > atria > AV node
Purkinje fibers > atria > ventricles > AV node
Purkinje fibers > AV node > ventricles > atria
D
Purkinje fibers > atria > ventricles > AV node
The conduction velocity of the structures of the heart are in the following order: Purkinje fibers > atria > ventricles > AV node. A calcium channel blocker such as verapamil would only slow conduction in the AV node. The conduction velocity of cardiac tissue is determined by a wide variety of factors, including the number and identity of various ion channels, the number and type of gap junctions, and the size and composition of muscle fibers. Conduction through the Purkinje system is the fastest within the heart, allowing for coordinated contraction of the ventricles, and atrial muscle conducts faster than ventricular muscle. Conduction through the AV node is the slowest, to allow the ventricles enough time to fill with blood. Bonke et al. review the evidence regarding the conduction velocity of parts of the heart. They discuss how the AV node is slower than the SA node. They recommend understanding how different parts of the heart conducts electricity. Incorrect Answers: Answers A-C & E: These choices do not correspond to the correct conduction velocities in each part of the cardiac conduction system. A calcium channel blocker would not change the relative velocities of conduction in Purkinje fibers, atria, and ventricles. Bullet Summary: The conduction velocity through the heart in order of speed is Purkinje fibers > atria > ventricles > AV node.
https://step2.medbullets.com/testview?qid=216239
A 3-year-old girl is brought to the office for a routine well-child appointment. She was delivered preterm at 35 weeks, and her medical history is significant for several ear infections over the last year. Her older brother has been diagnosed with attention-deficit hyperactivity disorder (ADHD), but the rest of her family history is otherwise unremarkable. The patient has become more withdrawn lately and has sometimes been ignoring her parents when asked to perform tasks at home. This seems to have worsened over the last 8 weeks. Otherwise, the patient is energetic and started preschool 3 months ago. Her temperature is 98.6°F (37°C), blood pressure is 98/62 mmHg, pulse is 97/min, and respirations are 26/min. The patient successfully draws a square and can stand on 1 foot. Her language skills are unchanged from her appointment 6 months ago. Her neurological examination is normal, and she is at the 50th percentile for height and weight. She seems distracted during the interview and responds only intermittently to the physician’s directions. Which of the following is the most appropriate next step in management?
Audiometry testing
Autism spectrum screening questionnaire
Parent and teacher ADHD rating scales
Refer to genetic testing
Speech and language assessment
A
Audiometry testing
This patient who presents with trouble following directions, inconsistent response to the physician's questions, and lack of progression in her language skills may have an acquired hearing impairment. The patient should undergo audiometry testing. Hearing impairment in children can be genetic or acquired. In a patient with a history of recurrent ear infections, conductive hearing loss is the most common underlying cause of hearing impairment. Hearing impairment can often present similarly to behavioral or persistent developmental disorders. In a child with poor language skills, social skills, and self-isolation, hearing loss should be ruled out first before considering other diagnoses. Undetected hearing loss can progress with poor academic performance, personal-social maladjustment, and emotional difficulties as children progress through critical development stages. The treatment for hearing loss is the restoration of hearing through hearing aids, cochlear implants, and other supportive measures. Dammeyer et al. studied the impact of childhood hearing loss on family life. They found that children with additional disabilities were more likely to be ignored by parents. They recommend understanding family dynamics in order to better support patients and families. Incorrect Answers Answer B: Autism spectrum screening questionnaire is useful in the diagnosis of autism spectrum disorder in children presenting with social isolation and poor communication skills. Autism spectrum disorder often presents with repetitive behaviors and fixed interests, which are absent in this child. Hearing loss should be ruled out first before pursuing this diagnosis. Treatment of autism spectrum disorders is early intervention and counseling. Answer C: Parent and teacher ADHD rating scales should be obtained to aid in the diagnosis of attention-deficit hyperactivity disorder (ADHD). While children with ADHD often appear inattentive and impulsive in multiple domains, poor language development, and social isolation are not features of ADHD. This patient should first undergo an audiometry test. ADHD can be treated with stimulants such as methylphenidate. Answer D: Referral for genetic testing is an incorrect first step in the management of this patient. While delayed language can be seen with several genetic conditions, there are usually signs of other global motor, cognitive, and social delays. Additional genetic testing can be pursued if the patient’s audiometry testing results are normal. Answer E: Referral for speech and language assessment is an incorrect first step. A formal assessment should be obtained in the diagnosis of communication and language disorders. However, this should only be done after ruling out hearing loss. Therefore audiometric testing should be performed first. Bullet Summary: Children with a history of recurrent ear infections presenting with signs of social or language development delays should be evaluated for hearing impairment with an audiometry test.
https://step2.medbullets.com/testview?qid=216589
A 64-year-old man presents with nausea, vomiting, and weakness. He states that his wife made him come in when he fainted today while attempting to stand up. He otherwise states he has noticed some visual changes, including noting a yellow tint to objects. He has a history of heart failure with reduced ejection fraction, obesity, diabetes, hypertension, acute coronary syndrome, atrial fibrillation, and peripheral vascular disease. His temperature is 98.5°F (36.9°C), blood pressure is 153/91 mmHg, pulse is 40/min, respirations are 15/min, and oxygen saturation is 97% on room air. Physical exam reveals a frail man who is unable to ambulate secondary to lightheadedness. His neurological exam is otherwise non-focal. An ECG is performed as seen in Figure A. Which of the following is the most likely etiology of this patient’s symptoms?
Amiodarone
Digoxin
Diltiazem
Procainamide
Propranolol
B
Digoxin
This patient with a history of heart failure with reduced ejection fraction and atrial fibrillation is presenting with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes, which are concerning for digoxin toxicity. Note that digoxin can be given in patients with poor cardiac function and atrial fibrillation; thus it is a plausible home medication for this patient. Digoxin is a cardiac glycoside that is used in conditions such as heart failure with reduced ejection fraction and atrial fibrillation. While it does not lower mortality, it may reduce symptoms of heart failure. Digoxin toxicity classically presents with gastrointestinal symptoms (nausea/vomiting), blurred vision or yellow halos, and cardiac dysrhythmias. While there is a myriad of dysrhythmias, digoxin can cause bradycardia, atrioventricular block, and premature ventricular contractions. ST-segment scooping can be seen with digoxin use both in toxicity but also with regular therapeutic use. Toxicity is treated with anti-digoxin antibodies. Patocka et al review digoxin toxicity. They note that this medication has a narrow therapeutic range and recommend close monitoring. Toxicity depends on severity and can be treated with anti-digoxin antibodies. Figure/Illustration A is an ECG demonstrating bradycardia and premature ventricular complexes (red circles). This is consistent with digoxin toxicity. Incorrect Answers: Answer A: Amiodarone is an antidyshythmic agent with toxicities including bradycardia, heart block, a prolonged QT interval, hepatitis, interstitial lung disease, hypothyroidism, corneal deposits and optic neuropathy. This patient could use amiodarone for his atrial fibrillation, but the visual disturbances are more typical of digoxin toxicity. Answer C: Diltiazem is a calcium channel blocker that would cause hypotension, bradycardia, and hyperglycemia. Treatment would involve immediate administration of calcium and vasopressors such as epinephrine. Other treatments in severe toxicity may include glucagon, dextrose, and insulin (to increase cardiac contractility). Answer D: Procainamide is the preferred antidysrhythmic in tachydysrhythmias associated with Wolff Parkinson White syndrome. It may cause drug-induced lupus and other dysrhythmias in overdose but would not cause this patient’s constellation of symptoms. Procainiamide is used rarely for atrial fibrillation and is only available for intravenous infusion in the US. Answer E: Propranolol toxicity would present with bradycardia, hypotension, confusion, seizures, and hypoglycemia. Treatment is centered on glucagon, calcium, and vasopressors such as epinephrine. In severe toxicity, lipid emulsion therapy and insulin/dextrose should be administered. Bullet Summary: Digoxin toxicity may present with nausea, vomiting, visual changes, bradycardia, and premature ventricular complexes.
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A 23-year-old woman presents to the emergency department complaining of a worsening headache. The headache started 1 month ago. It is constant and “all over” but gets worse when she is lying down or in the setting of bright lights. A review of systems is significant for a low-grade fever, night sweats, cough, malaise, poor appetite, and unintentional weight loss of 12 pounds in the last 2 months. The patient is sexually active with multiple male partners and reports inconsistent condom use. She has a history of intravenous drug use and has not been to a doctor in the last 2 years. Her temperature is 100.4°F (38.0°C), blood pressure is 110/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 98% O2 on room air. On physical exam, pain is elicited upon passive flexion of the patient’s neck. A CT scan shows ventricular enlargement. A CD4+ count is 57 cells/µL blood. A lumbar puncture is performed with the following findings: Cerebrospinal fluid: Opening pressure: 210 mmH2O Glucose: 32 mg/dL Protein: 204 mg/dL India ink stain: Positive Leukocyte count and differential: Leukocyte count: 200/mm^3 Lymphocytes: 100% Red blood cell count: 2/mm^3 Which of the following treatments most directly addresses the underlying cause of this patient's symptoms?
Acyclovir
Amphotericin B and 5-flucytosine
Dexamethasone
Fluconazole
Vancomycin and ceftriaxone
B
Amphotericin B and 5-flucytosine
The patient with a history of unprotected sex and intravenous drug use who presents with sub-acute fever, meningismus, photophobia, a low CD4+ count, and a positive India ink-stained lumbar puncture most likely has cryptococcal meningitis. Acute treatment for cryptococcal meningitis is intrathecal amphotericin B and 5-flucytosine. Cryptococcal meningitis is the most common fungal meningeal infection in patients with HIV, especially those with a CD4+ count < 100/mm^3. Symptoms are normally gradual in onset and can include headache, fever, neck stiffness, and photophobia. The most appropriate initial test is a lumbar puncture with an India ink stain. Cerebrospinal fluid in fungal meningitis would have low glucose, elevated protein, and an elevated lymphocyte-dominant leukocyte count. The most accurate diagnostic test is latex particle agglutination. Intrathecal amphotericin B and 5-flucytosine are the first-line treatments for acute management. Once the patient is treated, fluconazole is given for life or until the patient’s CD4+ count is > 100/mm^3. Badali et al. present a case of a patient with cryptococcal meningitis. They discuss how despite immediate antifungal therapy, the patient died due to a rapidly progressive infection. They recommend aggressive intrathecal therapy to treat this disease. Incorrect Answers: Answer A: Acyclovir is used to treat Herpes simplex virus encephalitis. Cerebrospinal fluid would show normal protein and glucose, an elevated lymphocyte-dominant leukocyte count, and an elevated red blood cell count. The Herpes virus can lead to a hemorrhage of the temporal lobes. Answer C: Dexamethasone is given in addition to IV antibiotics if Streptococcus pneumoniae meningitis is suspected. This is the most common cause of meningitis in adults, elderly, and asplenic patients, and can spread to cause sinusitis, otitis media, or bacteremia. Answer D: Fluconazole is used for prophylaxis against cryptococcal meningitis after a person is treated acutely. It is either given for life, or until the patient’s CD4+ count is > 100/mm^3. This treatment is not sufficient for patients with acute meningitis with neurological changes. Answer E: Vancomycin and ceftriaxone are given to a patient suspected of having bacterial meningitis until culture results confirm the bacterial organism. Bacterial meningitis would have decreased glucose, elevated protein, and an elevated neutrophil-dominant leukocyte count. Bullet Summary: Cryptococcal meningitis classically presents in a patient with a CD4+ count < 100/mm^3 and should be treated acutely with intrathecal amphotericin B and 5-flucytosine.
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A 9-year-old girl presents to the emergency department with a fever and a change in her behavior. She presented with similar symptoms 6 weeks ago and was treated for an Escherchia coli infection. She also was treated for a urinary tract infection 10 weeks ago. Her mother says that last night her daughter felt ill, and her condition has been worsening. Her daughter experienced a severe headache and had a stiff neck. This morning she was minimally responsive, vomited several times, and produced a small amount of dark cloudy urine. The patient was born at 39 weeks and met all her developmental milestones. She is currently up to date on her vaccinations and did not have infections during early childhood. Her parents are divorced and her father has noted she does not seem to get sick when he takes care of her. Her temperature is 99.5°F (37.5°C), blood pressure is 60/35 mmHg, pulse is 190/min, respirations are 33/min, and oxygen saturation is 98% on room air. The patient is started on intravenous fluids, vasopressors, and broad-spectrum antibiotics. Which of the following is the most appropriate underlying explanation for this patient's presentation?
Gastroenteritis
Immunodeficiency
Intentional contamination
Meningitis
Urinary tract infection
C
Intentional contamination
This patient is presenting with repeat sepsis, which is uncommon in a pediatric patient with no significant medical history. This abnormal concentration of infections when the patient is with her mother suggests a diagnosis of intentional contamination. Factitious disorder by proxy (formerly Munchausen syndrome) occurs when 1 individual creates symptoms in another for attention. Typically, this is a parent feigning or creating symptoms in a child in order to receive the psychiatric gain of attention from healthcare personnel. The patient should be treated and removed from the parents as this is considered child abuse. Factitious disorder by proxy should be suspected in any pediatric patient who has repeat infections that are atypical or not expected epidemiologically. Patients should also be evaluated for organic causes of symptoms such as immunodeficiency syndromes. Abeln and Love review the evidence regarding the diagnosis of factitious disorder by proxy. They discuss how these disorders have high morbidity and mortality when not accurately recognized. They recommend paying attention to red flags such as abnormal constellations of symptoms. Incorrect Answers: Answer A: Gastroenteritis would present with nausea, vomiting, and diarrhea; however, it would not present with sepsis routinely. Gastroenteritis does not best explain the most likely underlying cause of this patient's presentation. Answer B: Immunodeficiency such as severe combined immunodeficiency (SCID) would present with lifelong infections from birth that are bacterial, viral, and fungal. Immunodeficiency would not present with repeat infections in a pediatric patient only when they are with one patient. Answer D: Meningitis would present with headache, altered mental status, and meningeal signs. In the setting of repeat sepsis in a healthy pediatric patient, intentional contamination is more likely. Similarly, an absence of photophobia and no further history/physical exam supporting this diagnosis suggests an alternative diagnosis. Answer E: Urinary tract infection would present with dysuria rather than repeat episodes of sepsis. Though a urinary tract infection could lead to sepsis, this would be rare and more likely to occur in an immunosuppressed patient, the elderly, or a neonate. UTIs are a common cause of infection and fever in pediatric patients. Bullet Summary: Factitious disorder by proxy occurs when 1 individual feigns or creates symptoms in another.
https://bit.ly/47U1Ppp
A 55-year-old man presents to the emergency department with right knee pain. He woke last night with sudden-onset, sharp, 10/10 non-radiating pain in his right knee. He denies recent trauma and he has no known medical problems. He denies tobacco use and will often drink 6-8 beers or spirits on weekend nights. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 140/90 mmHg, respirations are 16/min, and oxygen saturation is 97% on room air. Physical exam reveals an erythematous, edematous right knee. Passive extension of the knee is limited by pain. Synovial fluid aspiration is performed and sent for analysis, which reveals 20,000 leukocytes/mm^3, and no organisms visualized. Microscopic examination of synovial fluid is shown in Figure A. Which of the following is the most likely diagnosis?
Gout
Lyme arthritis
Pseudogout
Rheumatoid arthritis
Septic arthritis
A
Gout
The patient’s sudden-onset knee pain with effusion and synovial fluid findings are characteristic of gout. Gout is caused by monosodium urate crystal deposition in joint spaces, most commonly in the big toe, ankle, or knee. Crystal deposition in the joint space leads to an intense inflammatory reaction that causes severe pain and joint effusion. Gout is most commonly due to lifestyle factors including a diet high in red meat and alcohol. Medications that cause elevated uric acid levels (such as thiazide diuretics) may also play a role. Conditions resulting in high cell turnover and therefore excessive purine release such as hematologic malignancies may also lead to the development of gout. Patients typically present with sudden-onset, severe pain, erythema, and joint effusion. Synovial fluid analysis will reveal an elevated leukocyte count and negatively birefringent, needle-shaped crystals. Treatment of acute gout is with NSAID medications, colchicine, or steroids depending on presentation and patient-specific factors. Prevention of further attacks is centered around lifestyle changes such as avoidance of red meat and alcohol. Xanthine oxidase inhibitors such as allopurinol are also often used for prevention of further episodes. Neogi discusses the management of acute gout. She comments that, while synovial fluid analysis is the gold standard of diagnosis, many physicians do not routinely perform it, instead relying on clinical judgment to diagnose gout. The differential diagnosis for a gout-like presentation includes pseudogout, septic arthritis, Lyme arthritis, reactive arthritis, and rheumatoid arthritis. Figure A shows monosodium urate crystals, which appear as negatively birefringent, needle-shaped crystals under polarized light. Incorrect Answers: Answer B: Lyme disease may also presents as a monoarticular arthritis in later stages. However, a recent history of tick exposure and preceding flu-like illness would also be expected. Answer C: Pseudogout presents similar to gout, but is caused by calcium pyrophosphate deposition. Crystals appear rhomboid shaped and are positively birefringent under polarized light. Answer D: Septic arthritis typically has higher leukocyte counts (> 50,000/mm^3) in the synovial fluid with organisms visualized on gram stain. Fever would also be expected. Answer E: Rheumatoid arthritis may present as a monoarticular arthritis with acute flairs. However, crystals visualized in synovial fluid are more suggestive of acute gout. Bullet Summary: Acute gout presents with sudden-onset, severe joint pain and is characterized by needle-shaped negatively birefringent crystals visualized under polarized light.
https://step2.medbullets.com/testview?qid=216610
A 44-year-old nurse presents to the emergency department with confusion. This has happened several times this past month with increasing frequency. Each time her symptoms improved with eating. She is otherwise healthy. She lives with her mother who has diabetes whose medications include metformin, insulin, lisinopril, amlodipine, and glyburide. Her temperature is 98.0°F (36.7°C), blood pressure is 132/81 mmHg, pulse is 85/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam reveals a confused woman. She is moving all her extremities but follows commands poorly. Laboratory studies are ordered as seen below. Hemoglobin: 14 g/dL Hematocrit: 41% Leukocyte count: 7,500/mm^3 with normal differential Platelet count: 199,000/mm^3 Serum: Na+: 140 mEq/L Cl-: 102 mEq/L K+: 4.0 mEq/L HCO3-: 23 mEq/L BUN: 30 mg/dL Glucose: 29 mg/dL Creatinine: 1.4 mg/dL Ca2+: 10.2 mg/dL Sulfonylurea level: undetectable C-peptide level: 55 ng/mL (normal < 5 ng/mL) Which of the following is the most likely etiology of this patient’s symptoms?
Alpha cell tumor
Beta cell destruction
Beta cell tumor
Insulin overdose
Sulfonylurea overdose
C
Beta cell tumor
This patient is presenting with confusion, profound hypoglycemia, an elevated C-peptide level, and a negative sulfonylurea level, which are concerning for an insulinoma. An insulinoma is a beta cell tumor that secretes insulin. An insulinoma is a beta cell tumor of the pancreas. The beta cells normally secrete insulin in response to increased circulating fuel substrate (such as glucose or fat). In the setting of an insulinoma, insulin is pathologically and continuously secreted. This leads to profound, recurrent episodes of hypoglycemia. When a patient presents with hypoglycemia of unclear etiology; a blood glucose level, C-peptide level, and sulfonylurea level should be ordered. An infectious workup may be indicated as sepsis can also cause hypoglycemia. In an insulinoma, patients will be hypoglycemic, have an elevated C-peptide level (as this is a marker of endogenous insulin release), and a negative sulfonylurea level (important to rule out as a sulfonylurea overdose can also cause hypoglycemia in the setting of an elevated C-peptide level). Further workup will involve imaging of the abdomen such as a CT scan or MRI (more accurate) to delineate this insulin-secreting mass. Mathur et al. discuss insulinoma. They note that it is a rare endocrine tumor, and note its association with multiple endocrine neoplasia type 1. They recommend workup for an insulinoma should occur in patients with recurrent episodes of hypoglycemia without a clear cause. Incorrect Answers: Answer A: Alpha cell tumor describes a glucagonoma, which pathologically secretes glucagon leading to profound hyperglycemia, a classic rash termed necrolytic migratory erythema (presents with painful, erythematous papules and plaques), and gastrointestinal symptoms including anorexia, abdominal pain, and diarrhea. Answer B: Beta cell destruction describes the pathophysiology of type I diabetes mellitus, which presents in pediatric patients (most commonly) with hyperglycemia, weight loss, polydipsia, and polyuria. Treatment involves the administration of insulin. An initial presentation of type I diabetes may be diabetic ketoacidosis which presents with dehydration, abdominal pain, nausea, vomiting, Kussmaul respirations, and an anion gap acidosis. Answer D: Insulin overdose would present with hypoglycemia, somnolence, confusion, and tremulousness. Note that exogenous insulin administration would not elevate the C-peptide level, which is a marker of endogenous insulin production. Answer E: Sulfonylurea overdose would cause hypoglycemia that is often persistent given the long half-life of sulfonylureas. It would also elevate the C-peptide level as these medications increase the endogenous release of insulin. However, the sulfonylurea level was negative in this patient. While iatrogenic use of medications is plausible in this patient who has knowledge of medications (as she is a nurse), her repeat episodes and her workup suggesting against an iatrogenic etiology makes an insulinoma a more likely diagnosis. Bullet Summary: Insulinomas are beta cell tumors of the pancreas and lead to severe hypoglycemia and an elevated insulin and C-peptide level.
https://step2.medbullets.com/testview?qid=216616
A 67-year-old man presents to the emergency department after fainting. He was outside tending to his lawn for several hours in the heat, when he stood up suddenly from pulling weeds and fainted. He denies any preceding symptoms and returned to baseline within 1 minute. The patient is not aware of any medical problems and just started seeing a primary care physician last week. He recently used a friend's prescription for ondansetron for nausea. His temperature is 99.3°F (37.4°C), blood pressure is 142/88 mmHg, pulse is 107/min, respirations are 14/min, and oxygen saturation is 99% on room air. Physical exam reveals intact cranial nerves, normal strength and sensation, and a stable gait. His abdomen is soft and nontender. An ECG is performed as seen in Figure A. Which of the following is the most likely diagnosis based on this patient’s ECG?
Acute myocardial infarction
Hypokalemia
Intermittent torsades des pointes
Previous myocardial ischemia
Pulmonary embolism
D
Previous myocardial ischemia
This patient is presenting after syncope, likely secondary to dehydration and orthostatic hypotension given his outdoor gardening in the heat and symptoms when standing up rapidly. The patient’s ECG reveals deep, pathologic Q waves, which in this case are an incidental finding indicative of a previous myocardial infarction or a previous ischemic event. The Q wave is a negative deflection just prior to the R wave. Q waves are a normal finding when they are small and are seen on most ECGs. However, pathologic Q waves are indicative of myocardial ischemia. Pathologic Q waves are generally diagnosed when they are >2 mm deep, >1 mm wide, >25% of the QRS complex height, or are seen in leads V1-V3. The interpretation of Q waves depends on other findings on ECG and the patient's symptoms. Deep Q waves in the setting of ST elevation indicate an acute myocardial infarction. On the other hand, pathologic Q waves found incidentally indicate a previous myocardial infarction or previous ischemic event. Rovai et al. review Q waves. They discuss how Q waves can be used to predict the location and size of a myocardial infarction as correlated with MRI. They recommend testing such as cardiac MRI to assess for previous infarction in the appropriate patient population. Figure/Illustration A is an ECG demonstrating pathologic Q waves (red arrows). Note the deep and wide Q waves; this raises concern for a past ischemic event. Incorrect Answers: Answer A: Acute myocardial infarctions would present with ST elevation in a vascular distribution on ECG with possible reciprocal depressions. While T wave inversions may be indicative of new or acute ischemia, deep pathologic Q waves without ST elevation are indicative of previous infarction. Answer B: Hypokalemia would cause U waves on ECG, which appear as a small, "second T wave" after the initial T wave. Note they are not U-shaped despite their name. Management is centered on repletion of potassium and often magnesium as well. Answer C: Intermittent torsades de pointes can cause syncope and should be suspected in a patient with syncope in the setting of a prolonged QT interval. This patient’s QT interval is not particularly prolonged, and his syncope seems to be secondary to orthostatic hypotension. Management is centered on cessation of QT-prolonging medications and magnesium administration. Note that while ondansetron does prolong the QT interval, it is unlikely to cause torsades given the patient took it a week ago. Answer E: Pulmonary emboli would present on ECG with sinus tachycardia in addition to pleuritic chest pain and hypoxia. Note that the ECG is a nonspecific test for pulmonary emboli, and CT angiography is the preferred confirmatory test for this condition. Syncope is a possible presentation of larger pulmonary emboli (though the patient would also be hemodynamically unstable). Bullet Summary: Pathologic Q waves are indicative of a previous myocardial infarction.
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A 34-year-old man is brought to a rural emergency department by ambulance after being involved in a motor vehicle accident. Paramedics report that the patient was driving the car and crashed into a tree at roughly 25 miles per hour. There were no passengers and he was awake but disoriented at the scene. His temperature is 97.9°F (36.6°C), blood pressure is 131/88 mmHg, pulse is 89/min, and respirations are 14/min. He is speaking but is confused, opens his eyes to voice commands, and follows simple commands. He has multiple lacerations on his face and arms and smells of alcohol and marijuana. His cardiac exam is normal and his lungs are clear to auscultation bilaterally. He has bruising over his abdomen without any tenderness to palpation, distension, or rigidity. Which of the following is the most appropriate next step in management?
Abdominal and chest CT
Chest radiograph anterior-posterior and lateral
Diagnostic laparoscopy
Diagnostic peritoneal lavage
Focused abdominal sonography for trauma exam
A
Abdominal and chest CT
This patient presents following a motor vehicle accident with a tender abdomen and stable vital signs. Given his stability, the most appropriate next step is an abdominal and chest CT. In blunt abdominal trauma, the next step in management depends upon whether the patient is hemodynamically stable. With a normal blood pressure and pulse within the normal range, patients can undergo imaging to evaluate for injury to the abdominal organs with the most appropriate study being an abdominal CT with contrast. This study can accurately localize bleeding and aid in operative planning. A bedside ultrasound known as the FAST exam is often performed in unstable patients. Unstable patients, patients with abdominal tenderness, and patients with a severe mechanism of trauma may have a bedside FAST exam performed. If the free fluid is localized, the patient can be transferred directly to the operating room. Note that in many hospitals, the FAST exam is done simultaneously with the primary and secondary survey; however, if asked to choose which exam to perform on a trauma patient who is stable with no signs of a surgical abdomen, a CT scan should be performed to more accurately assess the patient's injuries and assess for injuries that may be missed by a FAST exam. Feliciano reviewed the evidence surrounding the current diagnosis and management of abdominal trauma. He discusses how contrast-enhanced CT of the abdomen and pelvis is an important method for evaluating the etiology of intra-abdominal bleeding. He recommends considering damage control methods in the polytrauma patient. Incorrect Answers: Answer B: Chest radiography has limited utility in the setting of blunt abdominal trauma; however, a portable anterior-posterior film is often taken in trauma, in particular, when the patient is intoxicated. However, an anterior-posterior and lateral film would involve transporting the patient to radiology and positioning the patient. Thus it would be both difficult to obtain and less useful when compared to a CT scan given his traumatic injuries. Findings on chest radiograph could suggest abdominal injury, such as lower rib fractures or free air under the diaphragm, which would suggest intestinal perforation. Answer C: Diagnostic laparoscopy may play a role in evaluating for injury in penetrating traumas such as gunshot wounds or stabbing wounds but would not be indicated as the most appropriate initial step in management in a stable patient. Unstable patients, surgical abdomens, and positive FAST exams or CT scans warrant transfer to the operating room. Answer D: Diagnostic peritoneal lavage is a historical test to assess for intra-abdominal injury that was typically used with ambiguous FAST exams or patients where it was unclear whether there was abdominal bleeding; however, it has almost entirely been supplanted by the FAST exam. Answer E: FAST exams are often performed with the primary or secondary survey in large institutions where resources are available. However, in small hospitals with limited resources, the most appropriate next step in management when deciding between a FAST exam or a CT scan should be made based on the mechanism and concern for bleeding. This stable patient with a benign exam can undergo a CT scan, which will offer more data than a FAST exam and would be performed regardless of whether a FAST exam was positive or negative given this patient's intoxication and abdominal bruising. Bullet Summary: In a patient who has experienced blunt abdominal trauma the most appropriate initial step in a stable patient is an abdominal CT scan.
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A 26-year-old man presents to the emergency department with fatigue and dark urine over the past day. He was recently diagnosed with cellulitis of his left leg, for which he was prescribed trimethoprim-sulfamethoxazole. He denies having nausea, vomiting, or diarrhea. He has no significant medical history and does not currently take any medications. His temperature is 98.6°F (37°C), blood pressure is 115/72 mmHg, pulse is 98/min, and respirations are 14/min. Physical exam reveals conjunctival icterus, bilateral flank tenderness, and erythema of the left leg. Laboratory workup is performed, and the results are shown below: Serum: Hemoglobin: 7.2 g/dL Total bilirubin: 2.1 mg/dL Direct bilirubin: 0.8 mg/dL Lactate dehydrogenase: 470 U/L Glucose-6-phosphate dehydrogenase: 7 U/gHb (normal: 6-20 U/gHb) Which of the following would most likely be seen on a peripheral blood smear in this patient?
Acanthocytes
Codocytes
Degmacytes
Schistocytes
Spherocytes
C
Degmacytes
This patient presents with hemolytic anemia (low hemoglobin, elevated indirect bilirubin, and elevated lactate dehydrogenase) shortly after starting a sulfa medication (trimethoprim-sulfamethoxazole) in the setting of likely glucose-6-phosphate dehydrogenase deficiency. Degmacytes ("bite cells") will be seen on peripheral blood smears in these patients. Patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency will experience hemolytic anemia when exposed to an oxidizing substance such as sulfa drugs or fava beans. Laboratory tests will show a decrease in hemoglobin and hematocrit levels. Since hemolysis releases intracellular enzymes into the bloodstream, serum levels of lactate dehydrogenase will be increased. Hemoglobin is then metabolized in the spleen into unconjugated bilirubin, which will cause an elevated bilirubin level that is primarily indirect. In the acute setting, G6PD levels will be normal because the most compromised cells have all been hemolyzed. Obtaining a G6PD level in between episodes of hemolysis is therefore required to observe the chronically decreased G6PD in these patients. A peripheral blood smear will reveal degmacytes ("bite cells") and Heinz bodies. Avoidance of oxidative stress to red blood cells is the most important component of management in these patients. Belfield and Tichy discuss the classic presentation of G6PD deficiency in patients. The authors review oxidizing medications such as rasburicase, primaquine, dapsone, pegloticase, and methylene blue. The authors recommend avoiding initiation of these medications whenever possible until a G6PD diagnostic test has been performed. Incorrect Answers: Answer A: Acanthocytes can be seen in patients with liver disease due to defective production of lipoproteins; however, these cells do not undergo acute hemolytic destruction. Patients with abetalipoproteinemia usually present with symptoms consistent with fat-soluble vitamin deficiency such as bleeding (vitamin K) or osteoporosis (vitamin D). Answer B: Codocytes are most commonly seen in patients with thalassemia but can also generally be seen in all microcytic anemias. Patients with thalassemia will present with chronic anemia without acute episodes of hemolysis. The increased lactate dehydrogenase and bilirubin levels in this patient indicate an acute hemolytic event. Answer D: Schistocytes can be seen in thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation (DIC). Patients with TTP present with thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic symptoms, and acute kidney injury. DIC is a common downstream pathway for many processes such as sepsis, malignancy, and eclampsia; however, it would present with diffuse clotting and bleeding from all sites. Answer E: Spherocytes can be seen in hereditary spherocytosis, which may result in hemolysis during times of acute stress; however, this patient denies symptoms of nausea, vomiting, or diarrhea. Instead, the hemolysis in response to new foods is most likely due to exposure to oxidizing substances such as trimethoprim-sulfamethoxazole. Bullet Summary: Patients with glucose-6-phosphate dehydrogenase deficiency will experience hemolytic anemia in response to oxidizing substances and will have degmacytes (bite cells) on peripheral blood smear.
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A 45-year-old man with history of IV drug use presents to the outpatient clinic with a history of fever for 5 days. He also endorses sweats, headache behind the eyes, sore throat, muscle and joint pain, and a new rash seen in Figure A. He reports that he traveled to Africa last year and returned from a vacation to Thailand 10 days ago. His immunizations are up to date, although he missed the pre-departure travel medicine consult before his trip to Thailand. His temperature is 101.5°F (38.6°C), blood pressure is 125/80 mmHg, pulse is 105/min, and respirations are 14/min. Laboratory testing reveals the following: Leukocyte count: 2,400/mm^3 Hemoglobin: 13.4 g/dL Hematocrit: 40% Mean corpuscular volume: 81 µm^3 Platelets: 92,000/mm^3 Serum: AST: 112 IU/L ALT: 69 IU/L ALP: 78 IU/L Bilirubin: 0.9 mg/dL Which of the following is the diagnostic test of choice for this disease?
Blood culture
Blood smear
Serologic testing
Sputum with acid fast stain
Stool culture
C
Serologic testing
This patient is presenting with fever, headache with retro-orbital pain, muscle aches, joint pain, rash, leukopenia and thrombocytopenia, which are classic findings for dengue fever. The diagnostic test of choice for dengue is serology. Dengue is a viral infection endemic to South Asia, Central and South America, and the Caribbean. Although most infections are asymptomatic, notable characteristics include headache with retro-orbital pain, bone pain ("break-bone fever"), leukopenia, and thrombocytopenia (< 100,000 /µL). Symptoms typically develop several days to 2 weeks following the bite of an infected mosquito. The diagnostic test of choice is a serologic antibody test, although PCR can also be used in the acute (< 3 days) phase of illness. Treatment is with supportive care including rehydration. Jasamai et al. present the current treatment options for dengue fever. They discuss how supportive therapy is the mainstay of treatment as no antibody or antiviral treatments have been effective at this time. They recommend using effective preventive measures such as mosquito control in endemic areas. Figure A is a clinical photograph demonstrating the maculopapular rash seen in dengue fever. Incorrect Answers: Answer A: Blood culture is used for the detection of bacterial infections of the blood. This patient's IV drug use puts him at risk for bacterial endocarditis, but he does not have other expected findings, such as new murmur, Janeway lesions, Roth spots, or Osler nodes. Endocarditis should be treated with intravenous antibiotics. Answer B: Blood smear is the diagnostic test of choice for other mosquito-borne diseases such as malaria. It is not used in the detection of dengue. Malaria presents with cyclical fevers and organisms on blood smear. It can be treated with chloroquine and artesunate. Answer D: Sputum with acid fast stain is the test of choice for tuberculosis. This patient has a risk factor of TB as he recently traveled to Africa, but he does not have any pulmonary symptoms. Treatment for tuberculosis is rifampin, isoniazid, pyrazinamide, and ethambutol therapy (among other possible regimens). Answer E: Stool culture may be used to diagnose typhoid fever. Typhoid fever presents with many of the same non-specific symptoms as dengue but would also present with prominent abdominal pain and gastrointestinal symptoms. Typhoid can be treated with antibiotics such as ciprofloxacin. Bullet Summary: Dengue fever is characterized by fever, headache with retro-orbital pain, muscle and joint pain, rash, leukopenia and thrombocytopenia that can be diagnosed through serologic testing.
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A 30-year-old woman presents to the emergency department for palpitations. For 2 months, she has experienced intermittent palpitations along with menstrual irregularity and hair loss. At home, the air conditioner is at its coldest setting, but the patient still feels overheated. Her temperature is 99.5°F (37.5°C), blood pressure is 135/90 mmHg, pulse is 120/min, and respirations are 22/min. The patient is diaphoretic with tremulous hands, demonstrates thyromegaly, and protrusion of her eyes is noted. She is given atenolol in the emergency department with improvement of her palpitations and is instructed to follow up with her primary doctor for further management. Which of the following is most likely to be found in this patient after definitive treatment of her condition?
Exophthalmos
Hair loss
Heat intolerance
Menstrual irregularity
Thyromegaly
A
Exophthalmos
This patient presents with palpitations, tachycardia, tremor, heat intolerance, hair loss, menstrual irregularity, goiter, and exophthalmos concerning for hyperthyroidism due to Graves disease. Radioiodine ablation is the preferred definitive management for Graves disease, which can worsen exophthalmos. Graves disease is an autoimmune hyperthyroid disorder caused by antibodies to the thyroid stimulating hormone receptor (TSHR), which induce production of T4 and triiodothyronine (T3). Overstimulation of the thyroid gland by TSHR antibodies results in diffuse enlargement of the thyroid gland, termed “goiter.” TSHR is also highly expressed in retro-ocular adipocytes and fibroblasts, where overstimulation by TSHR antibodies (and activated T-cells) leads to the expansion of retro-ocular connective tissue and extra-ocular muscle volume. This causes exophthalmos (bulging eyes) and is referred to as Graves ophthalmopathy or orbitopathy. Beta blockers are used to achieve acute sympathetic control in hyperthyroid Graves patients, and antithyroid medications (propylthiouracil or methimazole) are initiated to achieve a euthyroid state. Radioactive iodine ablation is first-line definitive management, particularly, for individuals with unsuccessful anti-thyroid medication management. Radioiodine ablation has been known to increase TSHR-antibodies, likely secondary to the leakage of thyroid antigens, and thus exophthalmos is often worsened after treatment. For this reason, corticosteroids are often co-administrated as a prophylactic measure. Davies et al. review Graves disease including the pathophysiology and management. They note that Graves orbitopathy may be worsened by radioiodine ablation. They recommend further studies and advancements in care given this lasting complication. Incorrect Answers: Answer B: Hair loss can occur in hyperthyroid and hypothyroid states due to altered hair follicle stem cell function. However, this process is typically reversed, not worsened, after achieving a euthyroid state. Answer C: Heat intolerance occurs in Graves disease due to hyperthyroid-induced sympathetic overactivity, which resolves after successful treatment. Answer D: Menstrual irregularity occurs in hyperthyroidism through altered levels of sex hormone binding globulin, gonadotropin-releasing hormone, and prolactin. However, treatment of hyperthyroidism typically restores menstrual regularity. After ablation, patients will be hypothyroid; however, the standard of care is to start the patient on levothyroxine which should prevent this complication. Answer E: Thyromegaly in Graves disease is caused by diffuse overactivation of the thyroid gland by the TSHR antibodies. Once treated with radioactive iodine (I-131), the thyroid gland gradually shrinks in size. Patients who undergo radioactive iodine ablation will later require life-long thyroxine supplementation due to resulting hypothyroidism. Bullet Summary: Definitive therapy for Graves disease is radioiodine ablation, which leads to increased TSHR antibodies and worsened exophthalmos from overactivation of TSHR-rich retro-ocular adipocytes and fibroblasts.
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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 17-year-old boy presents to his primary care physician with a chief concern of "bad" skin that has not improved despite home remedies. The patient has had lesions on his face that have persisted since he was 13 years of age. He has a diet high in refined carbohydrates and has gained 20 pounds since starting high school. Physical exam is notable for the findings in Figure A. The patient is started on benzoyl peroxide and topical retinoids. He returns 1 month later stating that his symptoms are roughly the same. Which of the following is the most appropriate next step in management?
Continue current therapy for 1 more month
Dietary intervention
Isoretinoin
Oral antibiotics
Topical antibiotics
E
Topical antibiotics
This patient is presenting with persistent acne vulgaris that is refractory to topical benzoyl peroxide and retinoids. The next step in management is topical antibiotics. Acne vulgaris is caused by blockage or outlet obstruction of the pilosebaceous unit. It presents with erythema, pustules, and comedones typically in young men going through puberty. Risk factors include stress, excessive sweating, greasy skin care products, and steroid use. The management of acne vulgaris, in order, is to start with topical benzoyl peroxide and topical retinoids, topical antibiotics, oral antibiotics, and isoretinoin. It is important to try a therapy for a proper duration before switching to a more invasive therapy. Eichenfield et al. review the evidence regarding the treatment of patients with acne vulgaris. They discuss how this disease can be treated with benzoyl peroxide. They recommend using antibiotics or isotretinoin for refractory cases. Figure/Illustration A is a clinical photograph demonstrating diffuse comedones over the forehead (red circle). These findings are classically seen in patients with acne vulgaris. Incorrect Answers: Answer A: Continuing current therapy for 1 more month is unnecessary as this patient's symptoms have not improved at all with 1 month of therapy. Continuing current therapy would be appropriate if the patient had only tried therapy for a few days. Answer B: Dietary interventions have not been shown to improve outcomes in acne vulgaris. Stopping illicit substances such as performance enhancing steroids can be effective in reducing acne symptoms but there is no evidence this patient has been using steroids. Answer C: Isoretinoin is the last-line therapy for acne vulgaris. It should not be given to pregnant women as it is teratogenic. Women taking isoretinoin should be on reliable birth control. Topical and oral antibiotics should be used first. Answer D: Oral antibiotics would be indicated after topical antibiotics fail. Topical antibiotics should be tried first as these do not have systemic side effects. Bullet Summary: The management of acne vulgaris is to start with topical benzoyl peroxide and topical retinoids, followed by topical antibiotics, oral antibiotics, and isoretinoin in increasing order of severity.
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A 55-year-old woman is brought to the emergency department by her husband with a 1 hour history of an unremitting headache. The headache started suddenly while she was eating dinner and she says it feels like the “worst headache of my life.” An emergent CT scan of the head without contrast confirms the diagnosis, and a CT angiogram identifies the source of bleeding. The patient undergoes surgical management of her condition. On hospital day 3, she is found to be disoriented to person, place, and time. She also develops nausea and vomiting. Her medical problems consist of heart failure for which she takes furosemide, spironolactone, and metoprolol, which were continued at admission. Her temperature is 99.6°F (37.6°C), blood pressure is 100/60 mmHg, pulse is 112/min, and respirations are 16/min. Physical examination shows poor skin turgor. Capillary refill time is 4 seconds. Serum laboratory results are shown below: Na+: 120 mEq/L Cl-: 92 mEq/L K+: 3.9 mEq/L HCO3-: 26 mEq/L BUN: 32 mg/dL Creatinine: 1.0 mg/dL Serum osmolality is 265 mEq/L and urine osmolality is 340 mEq/L. Urine sodium is 44 mEq/L. Which of the following is the most likely diagnosis?
Addison disease
Cerebral salt wasting
Diuretic overuse
Primary polydipsia
Syndrome of inappropriate anti-diuretic hormone
B
Cerebral salt wasting
This patient presents with a subarachnoid hemorrhage (“worst headache of my life”) which is treated and then subsequently develops nausea, vomiting, and altered mental status with reduced serum sodium concentration, indicative of symptomatic hyponatremia. In the setting of serum hypo-osmolality, urine hyper-osmolality, urine sodium concentration > 20 mEq/L, and hypovolemia (low blood pressure, decreased skin turgor, and prolonged capillary refill), this is indicative of cerebral salt wasting. The mechanism of cerebral salt wasting is unclear, although some hypotheses point to increased release of brain natriuretic peptide after injury leading to suppression of renal sodium reabsorption, or sympathetic nervous system injury resulting in an impaired renin response. Patients classically present several days after cerebral injury or neurosurgery with hypovolemic hyponatremia and hypochloremia. Water retention results in serum hypo-osmolality, hyponatremia, and hypochloremia with urine hyper-osmolality and increased urine sodium excretion. In patients with prolonged cerebral salt wasting, hypokalemia can also occur (potassium may be normal in acute states). Treatment in mild or moderate cases consists of normal saline infusion to correct both volume status and hyponatremia. Treatment in severe cases consists of hypertonic saline. During correction, serum sodium must be monitored closely to prevent central pontine myelinolysis. Cui et al. describe the differences between syndrome of inappropriate anti-diuretic hormone (SIADH) and cerebral salt wasting. They discuss how volume status serves as the key differentiator between these conditions. They recommend paying closer attention to total body electrolyte balance in understanding these conditions. Incorrect Answers: Answer A: Addison disease, also known as primary adrenal insufficiency, results in salt wasting due to decreased mineralocorticoid activity. Because of reduced aldosterone release, patients also have hyperkalemia and metabolic acidosis. In contrast, this patient’s potassium and bicarbonate levels are normal. Treatment is with steroids. Answer C: Diuretic overuse is occasionally observed in patients using loop diuretics. Overuse of loop diuretics can cause hyponatremia, hypokalemia, and hypochloremia. Metabolic alkalosis is commonly seen due to volume contraction. Cerebral salt wasting is more likely in this patient presenting after a recent cerebral injury. Treatment is the cessation of the causative medication. Answer D: Primary polydipsia can cause symptomatic hyponatremia. The urine would be maximally diluted (urine osmolality < 100 mEq/L) due to an appropriate physiological response to hyponatremia and excess free water intake. Treatment is cessation of excessive water consumption. Answer E: Syndrome of inappropriate anti-diuretic hormone (SIADH) presents with identical findings as cerebral salt wasting with the exception of euvolemia as opposed to hypovolemia. An assessment of volume status is essential to differentiate. This patient has clear signs of hypovolemia. Treatment of SIADH is fluid restriction followed by ADH inhibitors, if needed. Bullet Summary: Cerebral salt wasting presents with hyponatremia, hypochloremia, and hypovolemia in the setting of recent cerebral injury or neurosurgery.
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A 69-year-old man passes away after a 3-week hospitalization for liver failure, complicated by a myocardial infarction. A medical student rotating in the intensive care unit asked if she can practice performing intubations during her rotation. The resident suggests performing one on the deceased patient, as the student cannot harm him. Which of the following is the most appropriate course of action for the medical student?
Ask the resident if the patient’s family or the patient has been consented for this procedure
Consult the hospital ethics committee
Contact the patient's family to ask for permission to perform the procedure
Intubate the patient with the resident's supervision
Report the resident to hospital administration
A
Ask the resident if the patient’s family or the patient has been consented for this procedure
This medical student wants to perform a procedure on a deceased patient for educational purposes. Before proceeding, the student should ensure that permission has been obtained from the patient's family or the patient when he was alive. Performing procedures on deceased patients is ethically permissible with appropriate consent and consideration of risks and benefits for patients, families, learners, staff, and the field of medicine. Supervising physicians must obtain informed consent from the patient while alive and with capacity or from a newly deceased patient’s healthcare proxy or family. During the consent process, the supervisor should outline the patient's and family's rights, wider educational and societal benefits, potential risks, and ensure that performing the procedure would be consistent with the patient's values and wishes. Additionally, the supervising physician has a responsibility to ensure that a medical examiner has cleared the patient and that the patient is not an organ donor before proceeding. Finally, the procedure should be documented in the patient's chart. Berger and Cassell review the ethics of practicing procedures on deceased patients. They note that some ethical norms may suggest this practice is not appropriate. They recommend performing procedures when consent is obtained and it is ethically acceptable to do so. Incorrect Answers: Answer B: Consulting the hospital ethics committee is not necessary, for with proper permission, trainees may perform procedures on deceased patients for educational purposes. The ethics committee should only be consulted when there is no clear course of action (an example may be no advanced directive with family members disagreeing on what the patient's wishes would have been for a critically ill patient). Answer C: Contacting the patient's family to ask for permission to perform the procedure is incorrect, as the supervising physician needs to verify and document consent. Medical students cannot consent patients and family members. Moreover, the family should not be contacted if the patient has already approved or refused this when he had capacity. Answer D: Intubating the patient with the resident's supervision is incorrect because proper permission must be ensured prior to performing educational procedures on deceased patients. Answer E: Reporting the resident to hospital administration is incorrect because it is ethically permissible to perform an intubation on a deceased patient provided there is proper permission. The resident is not asking the medical student to do anything illegal or against hospital policy. Reporting the resident may be indicated if the resident was performing procedures against the patient's will. Bullet Summary: Trainees may perform procedures for educational purposes on deceased patients if permission is obtained from the patient prior to death, the patient's family, or a healthcare proxy.
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A 26-year-old man presents to his primary care physician with a 6-month history of increasing lower back pain. He first felt the pain while lifting boxes at work but thought that he had just strained a muscle. The pain appears to be worse in the mornings and after rest. Exercise and physical activity appear to temporarily make the pain better. He has taken acetaminophen and ibuprofen for the pain. He has no significant medical history and takes no medications. His temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. On physical exam, he is found to have limited flexion and extension of his spine as well as tenderness to palpation over the insertion of his patellar tendons bilaterally. Results from laboratory tests are shown below: Serum: Erythrocyte sedimentation rate (ESR): 61 mm/hr C-reactive protein (CRP): 36 mg/L (normal <10 mg/L) Which of the following is the most accurate test for this patient's condition?
Bone scan
Computed tomography
Magnetic resonance imaging
Radiograph
Ultrasound
C
Magnetic resonance imaging
This young man presents with low back pain that is relieved by physical activity, decreased spinal mobility, enthesitis of his patellar tendons, and elevated ESR/CRP values most likely has ankylosing spondylitis (AS). The most accurate diagnostic test for early ankylosing spondylitis is MRI. Multiple imaging tests can diagnose ankylosing spondylitis. Radiographs of the spine will show squaring of vertebrae with vertical or marginal syndesmophytes resulting in a "bamboo spine" appearance. The earliest radiographic sign is the erosion of the iliac side of the sacroiliac joint, but this finding is not sensitive in the early stages of the disease. MRI is sensitive in detecting sacroiliac inflammation, which makes this the best modality for early detection of ankylosing spondylitis in young patients. Initial therapy for ankylosing spondylitis includes lifestyle modifications (exercise, smoking cessation, physical therapy) and non-steroidal anti-inflammatory drugs (NSAIDs). Therapy for patients with inadequate response to NSAIDs includes tumor necrosis factor inhibitors (etanercept, infliximab, adalimumab) and interleukin 17 inhibitors (secukinumab, ixekizumab). Jung et al. studied whether signs of inflammation on MRI are correlated with bone quality in patients with ankylosing spondylitis. The authors find that bone marrow edema is negatively correlated with the trabecular bone score. The authors recommend control of active bone inflammation to prevent osteoporosis in patients with ankylosing spondylitis. Incorrect Answers: Answer A: Bone scan will show inflammation in the sacroiliac joints, but this study lacks specificity for this disease compared with other inflammatory forms of arthritis. Answer B: CT will show bony changes but not active inflammation, so it is most commonly used to diagnose cervical fractures in patients with ankylosing spondylitis. While it can support the diagnosis, it is less accurate than an MRI. Answer D: Radiographs will show erosion of the iliac side of the sacroiliac joint, but this finding is not sensitive in the early stages of the disease when bony ankylosis has not yet occurred. Radiographs may be an appropriate initial test. Answer E: Ultrasound is useful in evaluating muscle strains or tendon tears; however, it is not able to detect bony abnormalities and is therefore of limited use in ankylosing spondylitis. Bullet Summary: MRI is the most sensitive and accurate test for the early detection of ankylosing spondylitis.
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A 62-year-old man presents to the emergency department with chest pain and shortness of breath that he experienced while barbecuing. The pain is over his chest and in his left arm. The patient has a medical history of obesity, type 2 diabetes mellitus, and hypertension. His current medications include insulin, metformin, lisinopril, and hydrochlorothiazide. An ECG is performed and is seen in Figure A. His temperature is 99.5°F (37.5°C), pulse is 112/min, blood pressure is 100/70 mmHg, respirations are 18/min, and oxygen saturation is 95% on room air. The patient is appropriately managed and is admitted to the step-down unit. 2 days later, he complains of chest pain. A repeat ECG is performed and is seen in Figure B. Which of the following is the most appropriate next step in management for this patient's underlying condition?
Angiography
Aspirin
Ibuprofen
Prednisone
Stenting
B
Aspirin
This patient is presenting with chest pain several days after sustaining a myocardial infarction and has an ECG suggestive of pericarditis. This presentation is consistent with Dressler syndrome and the most appropriate initial step in management is aspirin. Dressler syndrome is pericarditis that can occur after a myocardial infarction (MI). It most commonly occurs weeks to months after the MI, but can also occur within 1 week from an MI in rare cases. It is believed to result from an autoimmune reaction that occurs after damage to the heart (surgery and MI). It presents with chest pain, fever, pleuritic pain, and a pericardial friction rub. ECG demonstrates diffuse ST elevation with PR depression consistent with pericarditis. The most appropriate initial step in the management of this condition is symptomatic management with aspirin. Leib et al. review the evidence regarding the diagnosis and treatment of Dressler syndrome. They discuss how this condition is secondary pericarditis with or without pericardial effusion resulting from injury to the heart or pericardium. They recommend considering this syndrome in all patients with chest pain after a myocardial infarction. Figure/Illustration A demonstrates an ECG with ST elevation in leads II, III, and aVF (blue circles) suggesting a diagnosis of right-sided MI. Figure/Illustration B demonstrates an ECG with diffuse ST elevation and PR depression (blue circles) suggesting a diagnosis of pericarditis. Incorrect Answers: Answers 1 & 5: Angiography and stenting would be appropriate management if this patient were experiencing a repeat MI. The diffuse ST elevation and PR depression seen several days after his myocardial infarction are more indicative of pericarditis rather than a repeat MI. In addition, the chest pain the patient is experiencing can also occur in pericarditis and obfuscates the diagnosis of repeat MI. Answers 3 & 4: Ibuprofen, other NSAIDs, and corticosteroids such as prednisone are not first-line therapy in post-MI pericarditis as they can hinder the healing and remodeling process of the myocardium. Current guidelines dictate that they may be used in cases that are refractory to initial therapy. Bullet Summary: Dressler syndrome (post-MI pericarditis) presents with chest pain, pericardial friction rub, fever, and diffuse ST elevation with PR depression on ECG, and should be treated initially with aspirin.
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A 6-year-old boy presents to the emergency department after falling from his scooter. He has dull, aching pain along his left side where he hit the ground. He fell sideways off the scooter as he rounded a curve in the road. He has never had any serious injuries but that he always seems to bruise easily, especially after he started playing soccer this fall. His parents deny that he has an abnormal number of nosebleeds or bleeding from the gums. They have never seen blood in his stool or urine. His mother notes that her brother has had similar problems. His temperature is 98.6°F (37°C), blood pressure is 112/74 mmHg, pulse is 82/min, and respirations are 11/min. On physical exam, the patient has extensive bruising of the lateral left thigh and tenderness to palpation. Laboratory tests are performed and reveal the following: Hemoglobin: 14 g/dL Hematocrit: 41% Mean corpuscular volume: 89 µm3 Reticulocyte count: 0.8% Leukocyte count: 4,700/mm3 Prothrombin time (PT): 13 seconds Partial thromboplastin time (PTT): 56 seconds Bleeding time (BT): 4 minutes Which of the following is the most likely underlying pathophysiology?
Anti-platelet antibodies
Factor 8 deficiency
Factor 9 deficiency
GP1b deficiency
Von Willebrand factor deficiency
B
Factor 8 deficiency
This young patient presents with extensive bruising following an injury, a family history of blood disorder, and a prolonged partial thromboplastin time (PTT) with a normal prothrombin time (PT) and bleeding time. These findings suggest a diagnosis of hemophilia A caused by factor 8 deficiency. Hemophilia is a deficiency of factor 8 (hemophilia A) or factor 9 (hemophilia B). Common symptoms of hemophilia include hemarthrosis, intramuscular hematoma, and gastrointestinal or genitourinary bleeding. Laboratory testing is helpful in confirming the diagnosis, as hemophilia is characterized by an isolated prolongation of PTT without a change in PT. The factors involved in hemophilia participate only in the indirect cascade of coagulation factor activation. Similarly, since bleeding time depends only upon platelet function and not coagulation factors, hemophilia presents with a normal bleeding time. Treatment of hemophilia includes desmopressin in mild cases and factor replacement in moderate to severe disease. Berntorp et al. present a review of the evidence regarding the diagnosis and treatment of hemophilia. They discuss how these patients now have normal life expectancies with the advent of factor replacement therapy. They recommend being vigilant for the development of anti-factor antibodies. Incorrect Answers: Answer A: Anti-platelet antibodies are responsible for idiopathic thrombocytopenic purpura (ITP). This disease is caused by autoimmune antibody formation that damages platelets and causes them to be removed by splenic macrophages in the reticuloendothelial system. It usually occurs in children after an acute viral infection and it presents with bleeding from mucosal surfaces with laboratory findings showing an increased bleeding time, decreased platelets, and a normal PT/PTT. Answer C: Factor 9 deficiency (hemophilia B) presents nearly identically to hemophilia A with a normal PT, prolonged PTT, and normal bleeding time with a history of bleeding in male relatives. However, because hemophilia B is epidemiologically less common than hemophilia A, hemophilia A is a more likely diagnosis. Answer D: GP1b deficiency causes Bernard-Soulier syndrome, which is a bleeding disorder characterized by unusually large platelets and a low platelet count. Like von Willebrand disease (vWD), patients often present with cutaneous and mucosal bleeding, abnormal bleeding from minor trauma, and a prolonged bleeding time, which this patient does not have. It can be distinguished from vWD by a ristocetin assay. Treatment can include tranexamic acid. Answer E: Von Willebrand factor deficiency results in vWD, which is a bleeding disorder characterized by cutaneous and mucosal bleeding, menorrhagia, and gastrointestinal bleeding. Unlike this patient, patients with vWD also present with a prolonged bleeding time. Patients with vWD will also have an increased PT because this factor is responsible for stabilizing coagulation factor 8. Bullet Summary: Hemophilia A is caused by factor 8 deficiency and presents with hemarthrosis, frequent bruising, intramuscular hematoma, and gastrointestinal bleeding with a prolonged PTT with a normal PT and bleeding time.
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A 4-week-old boy is brought to the pediatrician by his parents for an initial evaluation. His parents are concerned that he is not feeding well and has lost weight over the last 2 weeks. He was born at home via spontaneous vaginal delivery at 37 weeks of gestation to a G1P1 mother. His mother did not receive prenatal care. His blood pressure is 70/44 mmHg, pulse is 151/min, and respirations are 41/min. His weight is 3 kg (4th percentile) compared with 3.5 kg at birth (45th percentile). On physical exam, he is jaundiced with an enlarged liver and spleen. He does not appear to respond to visual stimuli, and further examination reveals bilateral clouding of the lens. Which of the following interventions could have avoided this patient's symptoms?
Avoiding fruit juice and sweetened foods
Changing to a soy based formula
Providing imiglucerase enzyme replacement
Removing phenylalanine from maternal diet during pregnancy
Vitamin B6 supplementation
B
Changing to a soy based formula
This patient who presents with failure to thrive, hepatosplenomegaly, and bilateral cataracts most likely has classic galactosemia. Patients with this disorder should avoid lactose-containing products by changing to a soy-based formula. Classic galactosemia is an autosomal recessive defect in galactose-1-phosphate uridyltransferase. This enzyme is involved in the conversion of galactose to glucose, and a deficiency of this enzyme results in the accumulation of galactose 1-phosphate in the liver, kidney, and brain. This metabolite acts as a phosphate sink, meaning that it traps all free phosphate in the cytosol and inhibits the formation of other phosphate-dependent metabolites such as adenosine triphosphate. This results in failure to thrive, vomiting, lethargy, and hepatosplenomegaly after ingestion of galactose-containing products such as milk. Patients will also develop bilateral cataracts if the deficit is not detected in the first few weeks after birth. Avoiding galactose by changing to a soy-based formula will avoid the complications of this disorder. Turck discusses using soy protein for infant feeding. The author notes that there are safety concerns with regards to the high phyto-estrogenic isoflavone content of these formulas. The author recommends that despite these safety concerns, these formulas should still be used in patients with classic galactosemia. Incorrect Answers: Answer A: Avoiding fruit juice and sweetened foods would be effective in the treatment of hereditary fructose intolerance. This disease presents similarly to classic galactosemia, but cataracts would not be seen in patients with this disease. Answer C: Providing imiglucerase enzyme replacement would be effective in the treatment of patients with Gaucher disease, which can also present with failure to thrive. However, associated findings in this disease would be anemia and avascular necrosis of the bones. Answer D: Removing phenylalanine from the maternal diet during pregnancy would be effective in the treatment of patients with phenylketonuria, which can also present with failure to thrive. However, these patients classically present with a "musty smell" and eczema. Answer E: Vitamin B6 supplementation may be effective in mitigating the negative symptoms of homocystinuria. This disease may also present with cataracts or lens dislocations. Associated findings in this disorder would be marfanoid appearance and arachnodactyly. Bullet Summary: Patients with classic galactosemia should not be breastfed and should receive a galactose-free and lactose-free diet such as soy-based formulas.
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A 25-year-old man presents to his gastroenterologist for trouble swallowing. Whenever he eats solids, he regurgitates them back up. Given this patient's suspected diagnosis, the gastroenterologist performs a diagnostic test. Several hours later, the patient presents to the emergency department with chest pain and shortness of breath. His temperature is 99.5°F (37.5°C), blood pressure is 130/85 mmHg, pulse is 60/min, respirations are 12/min, and oxygen saturation is 99% on room air. On physical exam, the patient demonstrates a normal cardiopulmonary exam. His physical exam demonstrates no tenderness of the neck, a normal oropharynx, palpable crepitus above the clavicles, and minor lymphadenopathy. Which of the following is the most appropriate next step in management?
Barium swallow
Gastrografin swallow
Magnetic resonance imaging
Ultrasound
Urgent surgery
B
Gastrografin swallow
This patient is presenting with chest pain and palpable crepitus after endoscopy suggesting a diagnosis of esophageal perforation. The most appropriate next step in management is a gastrografin swallow. Esophageal perforation presents with severe chest pain and palpable crepitus typically occurring after endoscopy or repeated forceful vomiting. The most appropriate initial step in management is a water-soluble contrast image (gastrografin) in order to confirm the diagnosis. Alternatively, a CT scan or radiograph may also be appropriate initially. Once the diagnosis is confirmed, surgical correction is necessary in order to prevent gastric contents from continuing to enter the mediastinum. Broad-spectrum antibiotics and proton pump inhibitors should also be administered. Kim reviews the factors associated with esophageal perforation and rupture. They discuss how causes include foreign body, iatrogenic, trauma, and forceful vomiting. They recommend urgent surgical treatment. Incorrect Answers: Answer A: Barium swallow would be inappropriate as it is an irritating substance and could travel out of the perforated esophagus. This substance is only used in rare cases when gastrografin is not revealing, per the discretion of the surgeon. Answer C: MRI would appropriately diagnose the condition but acquiring such imaging would take too long for such an urgent presentation. A gastrografin swallow is a more appropriate initial test. Answer D: Ultrasound is inappropriate as it does not demonstrate the location of the perforation and the information obtained is dependent on the habitus of the patient and the operator. Answer E: Urgent surgery would be necessary once the diagnosis is confirmed. Performing a rapid diagnostic step is necessary in order to establish the diagnosis first. Bullet Summary: The most appropriate initial step in the management of esophageal perforation is a gastrografin swallow followed by surgery.
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A 25-year-old woman is brought to the emergency department by her family. They found her somnolent next to her computer with an empty pill bottle around 3 hours ago. The family cannot recall the types of pill bottles that they found and did not bring them to the ED. The patient's medical history is notable for anxiety, obesity, hypertension, and polycystic ovarian syndrome. She is currently not on any medications. She was given an exercise program but she has not been compliant with these exercises. Her temperature is 99.5°F (37.5°C), pulse is 82/min, blood pressure is 125/85 mmHg, respirations are 11/min, and oxygen saturation is 97% on room air. Physical exam is within normal limits. The patient begins communicating with the physician and states that she did take acetaminophen but it was only a few pills. Her parents are certain the bottle was new. Which of the following is the most appropriate next step in management?
Blood acetaminophen levels
Charcoal
Lactated ringer bolus
N-acetylcysteine
Syrup of ipecac
D
N-acetylcysteine
This patient is presenting with acute acetaminophen intoxication. The most appropriate next step in management is to administer the antidote N-acetyl cysteine (NAC). In a toxicology case where the ingestion is unknown, the most appropriate initial step in management is to begin treatment empirically with IV fluids, charcoal, NAC, naloxone, and glucose if needed. These agents are effective against the most common causes of intoxication. In cases where there is known acetaminophen ingestion, the most important treatment is prompt administration of NAC. This medication works by increasing the hepatic production of glutathione, which detoxifies the toxic metabolite of acetaminophen N-acetyl-p-benzoquinone imine (NAPQI). NAC is a safe, cheap, and effective treatment that could be life-saving if administered in a timely fashion. In massive overdoses, it is appropriate to start treatment empirically as it can be discontinued later. Otherwise, a 4 hour acetaminophen level should be collected and the level should be plotted on the Rumack Matthew nomogram to determine treatment. In chronic acetaminophen toxicity, the acute serum level play no role in guiding treatment. Chiew et al. performed a meta-analysis regarding the treatment of acetaminophen overdose. They found that the administration of charcoal immediately after ingestion can decrease the rate of absorption of acetaminophen. They recommend early administration of N-acetyl cysteine in order to decrease the hepatotoxicity of this substance. Incorrect Answers: Answer A: Blood acetaminophen levels should be ordered, but it is much less dire to confirm the diagnosis than it is to begin treatment given the high fatality rate with untreated acetaminophen overdose. Testing for all common intoxication should proceed while treatment is being administered. In this case, the concern for consumption of an entire bottle of acetaminophen would make it reasonable to treat first. Answer B: Charcoal is an appropriate intervention if it is given in a timely manner; however, this patient consumed acetaminophen over 2 hours ago. In general, if the patient's airway is secure, it does no harm and could offer some benefit by decreasing the absorption of the substance of interest. Answer C: IV fluid administration is the most appropriate initial step in the management of trauma when there is suspected bleeding and vitals are unstable (hypotension and tachycardia). Fluids are not urgently needed in this toxicity case with a patient who has stable vitals. Answer E: Syrup of ipecac induces emesis in patients and was previously used in an attempt to have the patient vomit the toxic substance. This is no longer a preferred method of managing toxicities as patients can aspirate the vomitus or the toxic substance can induce more tissue damage during vomiting. In general, syrup of ipecac is the wrong answer choice for all toxicity cases. Bullet Summary: Acetaminophen toxicity should be treated immediately with N-acetyl cysteine followed by further diagnostic workup including serum acetaminophen levels.
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A 22-year-old woman presents to the emergency department with shortness of breath. She was hiking when she suddenly felt unable to breathe and had to take slow deep breaths to improve her symptoms. The patient is a Swedish foreign exchange student and does not speak any English. Her medical history and current medications are unknown. Her temperature is 99.5°F (37.5°C), blood pressure is 127/68 mmHg, pulse is 120/min, respirations are 22/min, and oxygen saturation is 90% on room air. Physical exam is notable for poor air movement bilaterally and tachycardia. The patient is started on treatment. Which of the following parameters including forced expiratory volume in 1 second (FEV1), forced vital capacity (FVC), and diffusing capacity of carbon monoxide (DLCO) most appropriately describes this patient's underlying pathology?
Decreased airway tone
Increased FEV1
Increased FEV1/FVC
Increased FVC
Normal DLCO
E
Normal DLCO
This patient who presents with dyspnea and poor air movement bilaterally most likely has an acute asthma exacerbation. DLCO is the one parameter that is normal in these episodes. Asthma is an obstructive lung disease that results in intermittent episodes of respiratory compromise. Asthma presents with shortness of breath typically secondary to an allergen/antigen or with exposure to exercise or cold air that causes bronchospasm. Pulmonary function testing will typically reveal a decreased FEV1, a decreased FVC, and a decreased FEV1/FVC in both asthma and emphysema. The DLCO will be normal in asthma in contrast to emphysema where the DLCO is decreased. Patients should be treated with inhaled beta-adrenergic agonists and corticosteroids for acute exacerbations. Peng et al. review the evidence regarding patients who have COPD versus asthma. They discuss how patients with COPD have reduced DLCO. They recommend measuring parameters to distinguish between these entities. Incorrect Answers: Answer A: Decreased airway tone does not describe asthma. Rather, hyperreactive airways with increased tone describes an asthma attack. Decreased airway tone may be seen in patients with connective tissue diseases such as Ehlers-Danlos syndrome. Answers 2-4: Increased FEV1, FEV1/FVC, and FVC do not describe asthma. All of these laboratory values are decreased in asthma and emphysema. Increased respiratory performance can be seen in patients who are athletes or training for marathons. Bullet Summary: Both asthma and emphysema present with a decreased FEV1, FVC, and FEV1/FVC; however, asthma has a normal DLCO while COPD has a reduced DLCO.
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A 7-year-old boy presents to the emergency department after an episode of jerking his extremities followed by confusion. This has happened in the past, but his mother thought he was fabricating his symptoms. She has taken him to a free health clinic to have him seen by a doctor who prescribed medication; however, she has been unable to fill the prescription. He has had an MRI of the brain with no acute findings in the past. The mother states this episode lasted about 3 minutes and involved full-body jerking. After the episode, the child was non-responsive but was breathing on his own. The child has a history of migraine headaches with aura that resolve with ibuprofen. He is otherwise healthy and has no medical problems or recent illness and is up to date on his vaccinations. His temperature is 97.6°F (36.4°C), blood pressure is 100/64 mmHg, pulse is 90/min, respirations are 12/min, and oxygen saturation is 99% on room air. The patient is protecting his airway but is unable to answer questions. He is able to move the right upper and lower extremity. He initially is unable to move his left upper and lower extremity and has a left facial droop; however, after a few minutes, he has 4/5 strength in the affected side with resolution of his facial weakness. The mother notes that this is similar to his past episodes. Which of the following is the most appropriate management?
Alteplase
CT head
Diazepam
Observation
Sumatriptan
D
Observation
This patient is presenting after a seizure (tonic-clonic jerking of his extremities) and is currently postictal given that he is obtunded. Given that the patient is not moving the left side of his body, he is likely suffering from Todd paralysis, which can occur after seizures and resolves on its own with only observation. A generalized tonic-clonic seizure may present with full-body tonic-clonic activity with altered mental status. After a generalized tonic-clonic seizure, patients can be postictal and appear to be obtunded. Other than ensuring adequate oxygenation/ventilation and evaluating for the cause of the seizure, there is no further intervention needed as this postictal state will improve with time. After seizures, some patients present with Todd paralysis, which is a focal paralysis after a seizure. This may mimic a stroke, and thus the patient’s risk factors and known seizure history should be factored into the decision. Todd paralysis does not require any intervention and will improve with time and observation only. Xu et al. review Todd paralysis. They note that Todd paralysis is characterized by temporary limb weakness or hemiplegia and usually occurs following a seizure. They recommend that it can be difficult to differentiate Todd paralysis from a stroke in certain patient populations. Bullet Summary: Answer A: Alteplase is a thrombolytic that could be used in an acute ischemic stroke if given within 4.5 hours of symptom onset without any contraindications to its use. It would only be given after the CT scan of the head has been performed and other reversible causes have been addressed in the setting of acute ischemic stroke. Alteplase is not given when neurologic symptoms are thought to be secondary to Todd paralysis. In fact, seizure and Todd paralysis would be a contraindication to thrombolytic use in stroke. Answer B: CT head, in addition to a fingerstick blood glucose, should immediately be performed in all patients with sudden onset focal neurologic deficits thought to be secondary to a stroke. A stroke would present with sudden onset focal neurologic deficits. However, it is common for patients to be obtunded and have focal neurologic deficits after a seizure and this would not warrant a stroke workup unless clinical suspicion exists. This patient has no risk factors for stroke and his improving strength suggests against a stroke. Moreover, he has a history of a normal MRI of the brain. Answer C: Diazepam would only be given for a patient who is actively seizing as it is a benzodiazepine with a long half-life. Note that it is not indicated in a postictal patient as there is no ongoing seizure. Benzodiazepines are not given as preventive measures for seizures. Answer E: Sumatriptan may be indicated to treat a migraine, which presents with a pulsatile, unilateral headache and is more common in women. Hemiplegic migraine can cause focal neurologic deficits, and it would be reasonable to treat a migraine headache if a patient had focal neurologic deficits and a history of known hemiplegic migraines. Patients will either have a frequent history of hemiplegic migraines themselves or in their family. Bullet Summary: Focal neurologic deficits (Todd paralysis) can be seen after a seizure and will resolve with observation.
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A 44-year-old man presents to the emergency department acutely confused. The patient’s wife states she found him lethargic at home. He has been sick the past week with diarrhea and has been staying home from work. He is otherwise healthy and does not take any medications. His temperature is 97.5°F (36.4°C), blood pressure is 62/32 mmHg, pulse is 185/min, respirations are 25/min, and oxygen saturation is 98% on room air. The patient has profuse, bloody diarrhea while in the emergency department. The patient is given 3L of ringer lactate and subsequently appears less confused, with a blood pressure of 100/70 mmHg. He is able to drink oral fluids and protect his airway. An ECG is performed as seen in Figure A. He continues to have diarrhea while in the emergency department. Which of the following is contraindicated in the management of this patient?
Ceftriaxone
Loperamide
Magnesium
Norepinephrine
Normal saline
B
Loperamide
This patient is presenting with infectious diarrhea (given his bloody diarrhea and hypotension) with unstable vitals that improve with fluids. In bloody diarrhea, loperamide is contraindicated and could worsen illness. Bacterial diarrhea is commonly caused by organisms such as Campylobacter, Shigella, and Escherichia coli. It may present with bloody or purulent diarrhea and fever and may progress to sepsis or septic shock. Immediate management is centered on hydration. Unstable patients or those who cannot tolerate PO require IV fluids immediately. Otherwise, patients can rehydrate orally. Loperamide is an antimotility agent that decreases diarrheal output. It is contraindicated in bloody diarrhea as it can decrease the rate of fecal shedding and lead to bacteremia and a worsening clinical picture. Loperamide may be appropriate in controlling diarrhea in milder infectious forms of diarrhea, or in non-infectious conditions such as irritable bowel syndrome. Baker reviews loperamide. He discusses its indications, contraindications, and mechanism of action. They recommend that loperamide should not be given in invasive diarrhea. Figure/Illustration A demonstrates U waves on ECG (red arrows) that are significant for hypokalemia, which would be expected in a dehydrated patient with profuse diarrhea. Incorrect Answers: Answer A: Ceftriaxone would be appropriate in this patient who has bacterial diarrhea and appears septic as his critically ill state warrants antibiotics. Note that this patient may receive broader antibiotics than ceftriaxone given how ill he appears. Antibiotics should not be routinely used for bacterial diarrhea in young, healthy patients with stable vitals. Answer C: Magnesium could be appropriate in this patient even though his magnesium level is not stated as he is hypokalemic given his ECG is demonstrating U waves. The serum magnesium level does not adequately reflect intracellular magnesium levels. It requires replacement in many cases of hypokalemia. Answer D: Norepinephrine could be appropriate in this patient if he remained persistently hypotensive despite fluid administration. Note that this patient could receive more fluids and is not frankly unstable. While norepinephrine may not be appropriate at this time, it is not contraindicated. Answer E: Normal saline is generally avoided in diarrheal illness in patients who can tolerate PO as oral hydration is preferred to IV. However, IV fluids are not contraindicated and would be appropriate in this persistently hypotensive patient. Normal saline has a very high sodium and chloride load and can cause a hyperchloremic metabolic acidosis. For this reason, a lower-chloride solution may be preferred (e.g, Lactated Ringer). Bullet Summary: Loperamide is contraindicated in bloody diarrhea.
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A 14-year-old boy is brought to the pediatrician for behavioral issues. At school, he interrupts class by making barking noises. His mother notes that he has been making throat-clearing noises daily for the past 2 years. He has no medical problems and takes no medications. He gets along with his 2 brothers, plays turn-taking games with his friends, and completes his chores. His temperature is 98.4°F (36.9°C), blood pressure is 118/72 mmHg, pulse is 72/min, and respirations are 16/min. On exam, he grimaces several times and blinks his eyes rapidly. He does not appear to be reacting to internal stimuli. His mood and affect are normal. Which of the following is the most appropriate treatment for this patient?
Clozapine
Dextroamphetamine
Fluoxetine
Haloperidol
Methylphenidate
D
Haloperidol
This patient presents with multiple motor tics (grimacing, eye blinking) and phonic tics (barking, throat-clearing) on a daily basis for more than 1 year, which are consistent with Tourette syndrome. Tourette syndrome can be medically treated with antipsychotic agents such as haloperidol. Tourette syndrome presents with multiple motor tics (e.g., grimacing, sniffing) and 1 or more phonic tics (e.g., throat-clearing, barking) that occur for at least 1 year. Tics must begin before the age of 18 years, and their location, frequency, type, complexity, or severity must change over time. Attention-deficit hyperactivity disorder (ADHD) and obsessive compulsive disorder (OCD) are often co-morbid conditions. Common first-line treatments for Tourette syndrome-related tics include habit reversal training and alpha-2 agonists such as guanfacine or clonidine. However, the only pharmacologic treatments approved for Tourette syndrome in the United States are the antipsychotics haloperidol, pimozide, and aripiprazole. Risperidone is also used due to its favorable side effect profile. Seideman and Seideman review the current treatments for Tourette syndrome. They note it is a hyperkinetic movement disorder that often occurs in children and presents with motor and phonic tics. They recommend treating with both non-pharmacological and pharmacological treatments for optimal care. Incorrect Answers: Answer A: Clozapine is an atypical antipsychotic that blocks dopamine D2 receptors but is not approved for treatment of Tourette syndrome. It is typically avoided due to the risk for agranulocytosis and is mainly used for treatment-resistant schizophrenia as a last-line agent. Weight gain is another significant side-effect. Answer B: Dextroamphetamine is a stimulant commonly used for the treatment of ADHD. ADHD can be comorbid with Tourette syndrome but presents with hyperactivity (e.g., fidgeting, difficulty taking turns, blurting out answers) and/or inattentive symptoms (e.g., daydreaming, difficulty completing tasks) in at least 2 settings. While this patient’s phonic tics are disruptive, he has no difficulty waiting his turn and completing his chores, making Tourette syndrome more likely. Answer C: Fluoxetine is a selective serotonin reuptake inhibitor (SSRI) that may be used for the treatment of OCD. OCD is often co-morbid with Tourette syndrome but compulsions are present, which manifest as repetitive counting, hoarding, tapping, or error checking. This patient’s behavior is better attributed to his tics rather than compulsions. Answer E: Methylphenidate is a stimulant used for the treatment of ADHD. ADHD would present with hyperactivity and/or inattentive symptoms. The patient's vocal interruptions are better attributed to his phonic tics. Bullet Summary: Tourette syndrome is treated with habit reversal training, alpha-2 agonists (e.g., guanfacine, clonidine), and/or antipsychotics (e.g., haloperidol, risperidone).
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A 38-year-old man presents to the emergency department with nasal congestion, blurry vision, and diplopia. His nasal congestion started about 1 week ago and he noticed the blurry vision this morning when he struggled to read the words on his television screen. His medical history is significant for IV drug use, HIV (CD4: 47/mm^3), hypertension, hyperlipidemia, diabetes mellitus, and seasonal allergies. His home medications include hydrochlorothiazide, atorvastatin, metformin, cetirizine, darunavir, tenofovir, and emtricitabine. He denies recent IV drug use. His temperature is 100.8°F (38.2°C), blood pressure is 127/85 mmHg, pulse is 78/min, and respirations are 12/min. He has injected conjunctiva and rhinorrhea. His cranial nerves are intact, and his motor and sensory neurologic exam is normal. A MRI of the brain is performed and can be seen in Figure A. Which of the following is the most appropriate next step in management?
Brain biopsy
Empiric treatment with pyrimethamine-sulfadiazine
Empiric treatment with dexamethasone
Lumbar puncture
Serology for anti-John Cunningham (JC) virus antibodies
B
Empiric treatment with pyrimethamine-sulfadiazine
This patient with a past medical history of immunosuppression due to HIV (CD4 < 100/mm^3), new focal neurologic findings, and ring-enhancing lesions on head MRI should be treated empirically with pyrimethamine-sulfadiazine for 10-14 days. The differential diagnosis for ring-enhancing lesions in patients with HIV is determined by the degree of immunosuppression. For patients with CD4 counts > 200/mm^3, the most likely diagnoses include primary brain tumors and metastases. In patients with CD4 counts < 200/mm^3, the differential includes toxoplasma encephalitis, primary CNS lymphoma, progressive multifocal leukoencephalopathy (PML), and other infections. Patients with CD4 counts < 100/mm^3 should have empiric treatment for toxoplasma encephalitis with pyrimethamine-sulfadiazine with follow-up head imaging after 10-14 days. If the patient fails to improve clinically or the size of the lesion does not change, the next step would be a biopsy of the lesion. Robert-Gangneux and Darde present a review of toxoplasmosis diagnosis and treatment. They discuss how the incidence of these infections has increased dramatically since the advent of organ transplantation and HIV. They recommend increasing vigilance and prophylaxis for this disease. Figure/Illustration A is an MRI of the brain that demonstrates a ring-enhancing lesion (red circle) in the right occipital lobe. This lesion is likely causing the patient's visual complaints and is consistent with toxoplasmosis. Incorrect Answers: Answer A: Brain biopsy should be performed if the patient has no response clinically or on follow-up head imaging to empiric treatment with pyrimethamine-sulfadiazine for 10-14 days. Biopsy in these cases may reveal a CNS lymphoma or other neoplastic process. Answer C: Empiric treatment with dexamethasone is indicated for patients with substantial mass effect on imaging and severely depressed mental status, neither of which is present in this patient. Answer D: Lumbar puncture is contraindicated in patients with focal neurologic findings due to the risk of transtentorial herniation. Lumbar puncture would be indicated in the diagnosis of meningitis which presents with fever, headache, photophobies, and meningeal signs. Answer E: There is no role for serology for anti-John Cunningham (JC) virus antibodies in the workup of ring-enhancing lesions. If it were safe to perform a lumbar puncture (if the patient had no focal findings and no evidence of mass effect on head imaging), it would be reasonable to perform PCR of the CSF for JC virus. Bullet Summary: For patients with a past medical history of HIV with CD4 <100 cells/µL, focal neurologic findings, and ring-enhancing lesions on head imaging, the next step in management is empiric treatment for toxoplasma encephalitis with pyrimethamine-sulfadiazine for 10-14 days.
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A 2-week-old boy is evaluated by his pediatrician for abnormal feet. The patient was born at 39 weeks via vaginal delivery to a G1P1 29-year-old woman. The patient has been breastfeeding and producing 5 stools/day. He is otherwise healthy. His temperature is 99.5°F (37.5°C), blood pressure is 60/38 mmHg, pulse is 150/min, respirations are 24/min, and oxygen saturation is 98% on room air. A cardiopulmonary exam is notable for a benign flow murmur. A musculoskeletal exam reveals the findings shown in Figure A. Which of the following is the most appropriate next step in management?
Botulinum toxin injections
Reassurance and reassessment in 1 month
Serial casting
Surgical pinning
Surgical soft tissue release
C
Serial casting
This patient is presenting with talipes equinovarus (congenital clubfoot) for which the most appropriate initial step in management is serial casting using the Ponseti method. Talipes equinovarus is described clinically as a foot that is cavus, adductus, varus, and equinus. Cavus occurs when the forefoot is pronated relative to the hindfoot. Adductus occurs when the forefoot is medially deviated compared to the hindfoot. Varus occurs when the heel is medially deviated compared to the ankle. Finally, equinus occurs when the ankle rests in a plantarflexed position. The most appropriate initial step in management for this condition is serial casting using the Ponseti method. Cases that are refractory to this intervention are treated with surgical interventions such as split tibialis anterior transfer. Cady et al. review the evidence regarding the treatment of clubfeet. They discuss how the Ponseti method is now the standard of care in the treatment of this disorder. They recommend early diagnosis and treatment of these patients. Figure/Illustration A is a clinical photograph that demonstrates cavus, adductus, equinus, and varus positioning of the foot (red circles). This appearance is classically seen in congenital talipes equinovarus. Incorrect Answers: Answer A: Botulinum toxin injections are often used to treat spasticity and tension headaches; however, they are not indicated for the treatment of talipes equinovarus. Answer B: Reassurance and reassessment in 1 month would be inappropriate management and could lead to permanent sequelae. Patients require immediate treatment with serial casting in order to correct the deformity. Answers 4 & 5: Surgical interventions are reserved for cases of clubfoot that are refractory to stretching and serial casting. A percutaneous Achilles tenotomy is part of the Ponseti method but tendon transfers and releases are reserved for recurrent cases. Bullet Summary: The most appropriate initial step in management for congenital clubfoot (talipes equinovarus) is serial casting using the Ponseti method.
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A 56-year-old woman presents to the emergency department with muscle weakness. Her symptoms have progressively worsened over the course of 2 weeks and are most significant in her lower extremities. She also notices increased urinary frequency. Approximately 1 month ago, she was diagnosed with calcium phosphate nephrolithiasis. Medical history is significant for rheumatoid arthritis diagnosed approximately 10 years ago and treated with methotrexate as well as type 2 diabetes mellitus treated with metformin. Her temperature is 98.6°F (37°C), blood pressure is 138/92 mmHg, pulse is 92/min, and respirations are 17/min. On physical exam, there is mild tenderness to palpation of the metacarpophalangeal and proximal interphalangeal joints. There is 4/5 power throughout the lower extremity. Laboratory testing is shown. Serum: Na+: 137 mEq/L Cl-: 106 mEq/L K+: 2.9 mEq/L HCO3-: 18 mEq/L Glucose: 115 mg/dL Creatinine: 1.0 mg/dL Urine pH: 5.6 Which of the following is the most appropriate next step in management?
Administer hydrochlorothiazide
Administer intravenous insulin
Administer intravenous sodium bicarbonate
Begin potassium replacement therapy with dextrose
Increase methotrexate dose
C
Administer intravenous sodium bicarbonate
This patient's non-anion gap metabolic acidosis, hypokalemia, alkaline urine, and history of a calcium phosphate kidney stone and rheumatoid arthritis are suggestive of type 1 (distal) renal tubular acidosis. The treatment of choice is alkali replacement (sodium bicarbonate). Type 1 (distal) renal tubular acidosis results from impaired acid (H+) secretion in the distal nephron. Impaired acid secretion decreases the kidney's ability to acidify the urine, thus increasing the urine pH. Impaired acid secretion also results in potassium wasting resulting in hypokalemia. Acidemia causes the bone to release calcium phosphate which buffers the acid. The alkaline urine along with decreased citrate excretion (caused by acidemia) leads to a favorable environment to form calcium phosphate stones. Therefore, correction of metabolic acidosis is essential in managing this condition. Palmer et al. review the evidence regarding the treatment of renal tubular acidosis. They discuss how this disease can be divided into types based on the location of the deficit. They recommend using alkali therapy in patients with distal renal tubular acidosis. Incorrect Answers: Answer A: Administering hydrochlorothiazide would be a treatment option for type 2 (proximal) renal tubular acidosis. Patients with this condition can have associated hypokalemia but would be expected to have an acidic urine pH (less than 5.5) after significant bicarbonate excretion. Answer B: Administering intravenous insulin would further decrease the serum potassium and place the patient at risk of developing cardiac arrhythmia. This patient's most important concern is her renal tubular acidosis that is resulting in muscle weakness from hypokalemia. Her polyuria is caused by hypokalemia, as it decreases the kidney's responsiveness to antidiuretic hormone. Her serum glucose is also not significantly elevated to cause osmotic diuresis. Answer D: The dextrose (in the replacement therapy with potassium) would trigger the pancreas to secrete insulin and would lead to an intracellular shift of potassium. This would not particularly improve extracellular potassium levels. Treatment should be aimed at addressing the root of the patient's symptoms. Potassium citrate can be administered in cases of severe hypokalemia and hypokalemia that persists after correcting the serum bicarbonate level. Answer E: Increasing the methotrexate dose is not appropriate in this case. The patient's muscle weakness is more suggestive of hypokalemia secondary to type 1 renal tubular acidosis. She does not report symptoms of inflammatory arthritis (joint pain/stiffness that is worse in the morning and improves with use). Bullet Summary: Type 1 (distal) renal tubular acidosis is treated with alkali replacement (sodium bicarbonate or sodium citrate).
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A 3-month-old boy presents to the pediatrician with his mother for a well-child visit. The patient drinks 4 ounces of cow’s milk formula every 3 hours. He usually stools 1 time per day and urinates up to 6 times per day. He regurgitates a moderate amount of formula through his nose and mouth after most feeds. He does not seem interested in additional feeding after these episodes of regurgitation and he has become progressively more irritable around meal times. The patient is starting to refuse some feeds. His mother denies seeing blood or streaks of red in his stool and she denies any family history of food allergies or dermatological problems. The patient’s weight was in the 62nd percentile 4 weeks ago and he is now in the 48th percentile. His height and head circumference have followed similar trends. His temperature is 98.2°F (36.8°C), blood pressure is 72/47 mmHg, pulse is 125/min, and respirations are 27/min. On physical exam, the patient smiles reciprocally and can lift his head and chest when in the prone position. His abdomen is soft, non-tender, and non-distended. Which of the following is the most appropriate next step in management?
Counsel on positioning and thickening feeds
Initiate proton pump inhibitor
Obtain abdominal ultrasound
Provide reassurance
Switch to hydrolyzed formula
A
Counsel on positioning and thickening feeds
This patient presents with frequent regurgitation after feeding, worsening irritability, feeding aversion, and poor weight gain, which is concerning for gastroesophageal reflux disease (GERD). The most appropriate next step in management is counseling on proper positioning and thickening feeds. Infants with GERD may present with regurgitation or vomiting after feeding, significant irritability, feeding aversion, and poor weight gain or failure to thrive. Symptoms can usually be managed with lifestyle changes including more frequent, thicker, and lower volume feeds and positioning the patient upright during and after feeding. If symptoms continue, patients should undergo a trial of a dairy-free diet to address possible underlying food protein intolerance. Pharmacotherapy with proton pump inhibitors (PPI) is appropriate for patients with persistent symptoms despite these changes, although PPIs can increase the risk of pneumonia and infection with C. difficile. Chabra and Peeples review the evidence regarding the treatment of neonatal GERD. They discuss how alterations in the infant's environment and feeding pattern are effective in most cases. They recommend proton pump inhibitors if conservative treatments are not effective. Incorrect Answers: Answer B: Initiating medical therapy with a proton pump inhibitor is appropriate for patients who have already failed lifestyle changes, including positioning therapy, adjusting feeding patterns, and eliminating cow’s milk and soy from the diet. Answer C: Obtaining an abdominal ultrasound would help diagnose pyloric stenosis. Patients with pyloric stenosis typically present at a younger age (between 3 and 6 weeks of life) with non-bilious, projectile vomiting and a palpable “olive-shaped” mass in the epigastrium. Treatment is with a pyloromyotomy. Answer D: Providing reassurance would not be appropriate due to this patient’s poor weight gain. Although most cases of gastroesophageal reflux are uncomplicated and self-resolve by 1 year of age, this patient’s symptoms should be addressed to ensure he is meeting his nutritional requirements. Answer E: Switching to a hydrolyzed formula to eliminate cow’s milk and soy protein from the patient’s diet would be appropriate if the patient’s symptoms did not improve with changing position and thickening feeds. Bullet Summary: The most appropriate initial step in managing gastroesophageal reflux disease in infants is counseling on proper positioning and thickening feeds.
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A 1-month-old girl presents to her pediatrician with her mother. The patient was born at 38 weeks gestation via Caesarean section for cervical incompetence. The patient’s mother has no complaints. The child had a runny nose and cough for a few days last week. The patient’s mother endorses decreased appetite during the aforementioned illness which has now returned to baseline. The patient’s family history is significant for an older brother with glucose-6-phosphate dehydrogenase (G6PD) deficiency and a maternal uncle with cirrhosis secondary to chronic hepatitis B. On physical exam, the patient has scleral icterus and dark urine staining her diaper. Laboratory testing reveals the following: Serum: Na+: 137 mEq/L Cl-: 102 mEq/L K+: 4.2 mEq/L HCO3-: 24 mEq/L Urea nitrogen: 12 mg/dL Glucose: 96 mg/dL Creatinine: 0.36 mg/dL Alkaline phosphatase: 146 U/L Aspartate aminotransferase (AST): 86 U/L Alanine aminotransferase (ALT): 76 U/L Total bilirubin: 4.6 mg/dL Direct bilirubin: 3.8 mg/dL Which of the following is the most likely diagnosis?
Decreased metabolism of bilirubin
Increased enterohepatic circulation of bilirubin
Increased production of bilirubin
Obstruction of the extrahepatic biliary tree
Obstruction of the intrahepatic biliary tree
D
Obstruction of the extrahepatic biliary tree
This 2-month-old patient presents with jaundice, dark urine, mild transaminitis, and conjugated hyperbilirubinemia, which suggests the diagnosis of biliary atresia. Biliary atresia is characterized by obstruction of the extrahepatic biliary tree. Biliary atresia is a progressive, obliterative disease of the extrahepatic biliary tree. It presents in the first 8 weeks of life with jaundice, acholic stools, dark urine, hepatomegaly, mild transaminitis, and conjugated hyperbilirubinemia. The definitive diagnosis is made by a cholangiogram that will demonstrate the obliteration of these ducts. Patients who are untreated experience fatal neurological defects due to kernicterus and accumulation of bilirubin in the brain. Treatment usually involves hepatoportoenterostomy (Kasai procedure), in which the duodenum is directly anastomosed to the liver. Most patients also eventually require a liver transplant. Hartley et al. review the evidence regarding the diagnosis and treatment of patients with biliary atresia. They discuss how researchers are trying to reverse the liver fibrosis associated with this disease. They recommend early referral for a Kasai procedure or liver transplant as needed. Incorrect Answers: Answer A: Decreased metabolism of bilirubin occurs with enzymatic deficiencies such as those seen in Crigler-Najjar or Gilbert syndrome. Both Crigler-Najjar and Gilbert syndromes lead to unconjugated hyperbilirubinemia (much more severe in Crigler-Najjar syndrome) rather than the conjugated hyperbilirubinemia seen in this patient. Answer B: Increased enterohepatic circulation describes the pathophysiology underlying breast milk jaundice. Breast milk jaundice classically peaks in the first 2 weeks of life and leads to unconjugated rather than conjugated hyperbilirubinemia. Treatment is usually supportive and patients can typically continue to breastfeed. Answer C: Increased production of bilirubin can occur in hemolytic diseases such as G6PD deficiency or hereditary spherocytosis. These conditions would similarly lead to an unconjugated hyperbilirubinemia. Treatment is with addressing the underlying anemia with possible transfusion or splenectomy. Answer E: Obstruction of the intrahepatic biliary tree describes Alagille syndrome, in which patients have a paucity of interlobular bile ducts. Alagille syndrome is less common than biliary atresia and usually presents with other associated features, such as short stature, dysmorphic facies, ocular findings, and cardiac or renal anomalies. Treatment is with ursodiol. Bullet Summary: Biliary atresia presents in the first 8 weeks of life with jaundice and conjugated hyperbilirubinemia.
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A 59-year-old man presents to his primary care physician for abdominal pain and foul-smelling diarrhea that has persisted for the past 2 years. The patient has a medical history of alcoholism and has been admitted to the hospital multiple times for withdrawal, abdominal pain, and traumatic injuries from his drinking. The patient states that his last drink was more than 1 year ago. His temperature is 99.3°F (37.4°C), blood pressure is 115/78 mmHg, pulse is 78/min, respirations are 15/min, and oxygen saturation is 99% on room air. Physical exam reveals a non-tender, non-distended abdomen. Laboratory studies are notable for a serum calcium of 7.0 mg/dL. A computed tomography (CT) scan of the abdomen is performed as seen in Figure A. A Sudan Black stain of the patient’s stool is positive. Which of the following is the most appropriate treatment for the underlying cause of this patient’s diarrhea?
Ciprofloxacin
Loperamide
Oral rehydration
Pantoprazole
Rifaximin
D
Pantoprazole
This patient with a history of alcoholism is presenting with steatorrhea (foul-smelling diarrhea that stains with Sudan Black, a stain that detects fat in the stool), hypocalcemia, and findings suggestive of chronic pancreatitis on CT scan (inflammatory changes and calcification). His steatorrhea can be improved with the administration of proton pump inhibitors such as pantoprazole. Chronic pancreatitis is a sequela that occurs after repeated bouts of acute pancreatitis. It can occur secondary to alcoholism or gallstones, and it may present with chronic abdominal pain, hypocalcemia, and signs of malnutrition and weight loss. Malnutrition and weight loss occur secondary to the pancreas’ inability to produce pancreatic enzymes which leads to poor nutrient absorption. Lack of lipase can lead to fat wasting in the stool, steatorrhea, and fat-soluble vitamin deficiency (A, D, E, and K). The management of chronic pancreatitis is the cessation of the underlying cause, pancreatic enzyme replacement, and the administration of proton pump inhibitors. Proton pump inhibitors help with steatorrhea by increasing the pH as low pH inactivates pancreatic enzymes. Singh et al. review the diagnosis and management of chronic pancreatitis. The authors find that the diagnosis can be secured by typical findings on CT or magnetic resonance imaging (MRI) including pancreatic calcifications, ductal dilatation, and pancreatic atrophy. The authors recommend the use of endoscopic ultrasound in securing the diagnosis in patients with equivocal imaging findings and a high index of suspicion, such as patients with repeated episodes of acute pancreatitis. Figure/Illustration A is a CT scan of the abdomen demonstrating calcifications and chronic inflammatory injury (red arrows) suggesting a diagnosis of chronic pancreatitis. Incorrect Answers: Answer A: Ciprofloxacin or ceftriaxone is the appropriate management of infectious diarrhea from a bacterial organism. This usually presents with fever, as well as bloody or purulent diarrhea. In general, young and healthy patients who are able to hydrate themselves should not be given antibiotics, even for infectious diarrhea. Elderly patients, immunosuppressed patients, or patients with symptoms that do not improve spontaneously should be managed with a stool culture and antibiotics. Answer B: Loperamide is an anti-diarrheal agent that could be used for irritable bowel syndrome to decrease bowel motility and improve symptoms of diarrhea. Loperamide and other antidiarrheals should never be used for infectious causes of diarrhea as they will delay the fecal shedding of the organism or virus and could prolong symptoms. Answer C: Oral rehydration is recommended for any patient with diarrhea and signs of volume depletion and is preferred to IV fluids as the integrity of the gut is protected. This patient’s diarrhea is likely caused by his chronic pancreatitis; thus, oral rehydration may be indicated but does not address the underlying cause of this patient’s diarrhea. Answer E: Rifaximin is an antibiotic that targets gut flora and can decrease enteric flora and treat conditions such as small bowel bacterial overgrowth. Small bowel bacterial overgrowth presents in patients with conditions such as diabetic gastroparesis or scleroderma secondary to decreased bowel motility causing bacterial overgrowth and profuse diarrhea. Treating the underlying cause in addition to rifaximin will eliminate the excess bacteria. Bullet Summary: Proton pump inhibitors should be given in patients with persistent diarrhea in the setting of chronic pancreatitis to prevent the inactivation of pancreatic enzymes.
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A newborn boy is evaluated in the hospital nursery 2 hours after birth. The patient was born at 39 weeks of gestation to a 30-year-old primigravid via vaginal delivery. The patient’s mother received routine prenatal care and the pregnancy was uncomplicated. The patient’s anatomy ultrasound at 20 weeks of gestation was unremarkable. The patient’s mother denies any family history of genetic diseases. The patient’s APGAR scores were notable for poor muscle tone at both 1 and 5 minutes of life. The patient’s birth weight is 2.6 kg (5 lb 11 oz), which is at the 5th percentile. His height and head circumference are in the 15th and 3rd percentile, respectively. 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 has a wide nasal bridge, down slanting palpebral fissures, and widely spaced eyes. He has good respiratory effort with a high-pitched cry. This patient is most likely to have experienced a deletion on which of the following chromosomes?
4p
5p
5q
7q
15q
B
5p
This patient presents with a characteristic mew, microcephaly, hypertelorism, wide nasal bridge, and hypotonia, which suggests a diagnosis of cri-du-chat syndrome. Cri-du-chat syndrome is caused by a deletion on chromosome 5p. Cri-du-chat syndrome is most commonly caused by a de novo partial deletion of chromosome 5p. Other cases may be caused by a parental translocation involving the relevant section of chromosome 5p. Patients present with a mew-like cry, microcephaly, hypertelorism, down slanting palpebral fissures, a broad nasal bridge, low-set ears, low birth weight, hypotonia, and intellectual disability. The cat-like cry usually resolves by 2 years of age. Treatment is supportive though some patients may have cardiac abnormalities that require surgical intervention. Cerruti Mainardi reviews the evidence regarding the etiology and diagnosis of patients with Cri-du-chat syndrome. She discusses how the diagnosis is mostly made clinically at this time. She recommends karyotype and FISH studies for those with ambiguous features. Incorrect Answers: Answer A: A deletion on chromosome 4p causes Wolf-Hirshhorn syndrome, which presents with growth restriction, microcephaly, congenital heart disease, severe intellectual disability and dysmorphic "Greek warrior helmet" facies of a high forehead, prominent glabella, arched eyebrows, hypertelorism, and epicanthal folds. Treatment is supportive though some patients may require surgical intervention for heart defects. Answer C: A deletion on chromosome 5q results in Sotos syndrome, a pediatric overgrowth disorder characterized by an increase in head circumference, hypotonia, delayed acquisition of motor skills, and mild intellectual disability. Answer D: A deletion on chromosome 7q may results in Williams syndrome, which presents with dysmorphic, “elfin” facies, global developmental delay with a hypersocial personality, and supravalvular aortic stenosis. Treatment is supportive though some patients will require surgical intervention for aortic stenosis. Answer E: A deletion on chromosome 15q may cause either Prader-Willi syndrome or Angelman syndrome, depending on whether the deletion affects the maternal or paternal chromosome. Prader-Willi syndrome classically presents with dysmorphic facies, hyperphagia, and hypogonadism. Angelman syndrome classically presents with intellectual disability, frequent smiling or laughing, and gait ataxia. Treatment is supportive with seizure control for those who develop seizures. Bullet Summary: Cri-du-chat syndrome (presents with a characteristic mew, microcephaly, hypertelorism, wide nasal bridge, and hypotonia) is most commonly caused by a deletion on chromosome 5p.
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A 55-year-old woman presents to a primary care physician with persistent nausea and abdominal discomfort. She has experienced these symptoms daily for the past 3 months. She feels bloated and has episodic loose and watery stools. She has a history of hypertension and hyperlipidemia for which she takes amlodipine and atorvastatin. She immigrated from Vietnam 6 months ago. Her temperature is 98.6°F (37.0°C), blood pressure is 110/60 mmHg, pulse is 70/min, and respirations are 18/min. Physical exam reveals a thin-appearing woman in no acute distress. Her mucous membranes are moist. Cardiac and lung examinations are unremarkable. Laboratory results are as follows: Leukocyte count: 13,000/mm^3 Segmented neutrophils: 54% Bands: 2% Eosinophils: 6% Basophils: 0.4% Lymphocytes: 30% Monocytes: 5% A vitamin D level is 26 ng/mL (reference: >= 30 ng/mL). Which of the following is the most appropriate test in making the diagnosis?
Bowel wall biopsy
CT scan of the abdomen
Lower endoscopy
Stool ova and parasite assay
Stool toxin assay
D
Stool ova and parasite assay
This patient who presents with persistent abdominal discomfort, nausea, bloating, diarrhea, eosinophilia, and signs of malabsorption (thin-appearing, vitamin D deficiency) in the setting of immigration from a resource-limited country most likely has a gastrointestinal parasite infection (Ascaris lumbricoides). The most appropriate next step in management is to obtain a stool ova and parasite assay. Ascaris lumbricoides is a roundworm that is transmitted via the fecal-oral route (egg ingestion). While most patients are asymptomatic, there can be pulmonary and intestinal manifestations in the early and late phases, respectively. Pulmonary signs include dry cough, dyspnea, fever, and wheezing. Intestinal signs include abdominal discomfort, diarrhea, nausea, vomiting, malabsorption, and/or anorexia. The diagnosis should be suspected in patients with vague abdominal symptoms with travel history to an endemic area. The diagnosis is secured with stool microscopy for eggs or worms. Complications of this condition include intestinal obstruction or hepatobiliary/pancreatic involvement, presenting as biliary colic, obstructive jaundice, and/or ascending cholangitis. Treatment in patients with uncomplicated infection is with anti-helminth therapy (albendazole or mebendazole). Parija et al. review the epidemiology of helminth infections globally. The authors note that many cases are caused by Ascaris species. They recommend prompt identification of these infections as they can cause lack of school attendance, anemia, and cognitive deficits if left untreated. Incorrect Answers: Answer A: Bowel wall biopsy is used in the diagnostic work-up of suspected Whipple disease, which presents with chronic diarrhea, malabsorption, weight loss, and joint symptoms (arthralgias). This disease is progressive over years and would be less likely in this patient with disease onset of 3 months. Answer B: CT scan of the abdomen is used in the diagnostic work-up of small bowel obstruction, which presents as crampy abdominal pain, constipation, obstipation, and/or vomiting. Bowel obstruction is an acute process that would be unlikely in this patient with chronic symptoms. Although bowel obstruction is a complication of Ascaris infection, this patient has no signs of obstruction. Diarrhea may occur secondary to infection of the colon as well including colitis or diverticulitis; however, these conditions typically cause pain and low grade fever. Answer C: Lower endoscopy (colonoscopy) is used in the diagnosis of inflammatory bowel disease, consisting of Crohn disease and ulcerative colitis. Ulcerative colitis presents with bloody diarrhea, abdominal pain, weight loss, and fatigue. Eosinophilia would be unexpected, and this patient does not have bloody diarrhea. Answer E: Stool toxin assay is used to diagnose Clostridioides difficile infection. This presents after recent antibiotic use with watery diarrhea, dehydration, fever, and nausea. It presents acutely and generally without eosinophilia. Bullet Summary: Parasitic gastrointestinal infections present with chronic diarrhea, abdominal discomfort, malabsorption, and eosinophilia, and are diagnosed using stool ova and parasite assays.
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A 55-year-old male bodybuilder presents to the emergency department with weakness of his right arm. The patient states he has experienced these symptoms for a few weeks; however, today his hand felt so weak he dropped his cup of tea. The patient has a medical history of diabetes. He drinks 2-7 alcoholic drinks per day and has smoked 2 packs of cigarettes per day since he was 25. The patient uses anabolic steroids. He has lost 17 pounds since he last came to the emergency department 1 month ago. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals decreased sensation in the right arm and 2/5 strength in the right arm and 5/5 strength in in the left arm. The patient states that he is experiencing a dull aching and burning pain in his right arm during the exam. Symptoms are not changed with changes in head and neck position. Which of the following is the most likely diagnosis?
Apical lung tumor
Brachial plexopathy
Cerebral infarction
Scalenus anticus syndrome
Subclavian steal syndrome
A
Apical lung tumor
This patient is presenting with unilateral upper extremity paresthesia and weakness suggestive of compression of the neurovascular structures of the upper extremity secondary to an apical lung tumor. An apical lung tumor can compress the neurovascular structures in its proximity and cause pain, numbness, and weakness of the ipsilateral arm. A Horner syndrome can also be observed with compression of the sympathetic chain leading to ipsilateral miosis, ptosis, and anhidrosis in some cases. These malignancies typically occur in older male smokers and can be associated with symptoms such as fatigue, cough, and weight loss. Chest radiography will demonstrate an apical opacity and CT scan can confirm the size and location of the mass. Treatment is with chemotherapy and surgical excision of the tumor. Davis and Knight review the evidence regarding the diagnosis and treatment of patients with apical lung tumors. They discuss how these tumors can be associated with brachial plexus injuries. They recommend surgical excision of these tumors if they are resectable. Incorrect Answers: Answer B: Brachial plexopathy could present with weakness and numbness of the ipsilateral arm but is a less likely diagnosis (and a diagnosis of exclusion). Treatment is with identification and surgical release of any underlying anatomic defects (such as compressive bands) and supportive therapy if none are found. Answer C: Cerebral infarction (stroke) would present with sudden onset of acute neurological deficits. These would typically only affect one side of the body and would not necessarily be associated with a dull pain or numbness that is chronic. Treatment is with thrombolytics if patients meet criteria for acute therapy. Answer D: Scalenus anticus syndrome occurs in bodybuilders or athletes with hypertrophied neck muscles that compress the brachial plexus. Symptoms are typically worsened by certain positions. Treatment is with physical therapy and surgical decompression in refractory cases. Answer E: Subclavian steal syndrome presents with numbness, tingling, weakness in a limb, and CNS symptoms (vertigo, slurred speech, or stroke-like symptoms) that are worsened with activity and relieved with rest. Treatment is with vascular intervention to restore blood flow. Bullet Summary: Apical lung tumors can compress neurovascular structures in the upper extremity and cause pain, numbness, and tingling in the ipsilateral arm.
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A 27-year-old nurse presents to the emergency department with a 30-minute history of palpitations and chest pain. She has been experiencing nausea, vomiting, and diarrhea after eating leftovers 2 days ago. Her husband tells the physician that he is concerned that the patient has been increasingly obsessed about her weight recently. She has a history of major depressive disorder for which she takes escitalopram. Her temperature is 98.6°F (37.0°C), blood pressure is 110/72 mmHg, pulse is 110/min, and respirations are 11/min. Physical examination is notable for dry mucous membranes and tachycardia with regular rhythm. Laboratory testing shows the following results: Serum: Na+: 135 mEq/L Cl-: 96 mEq/L K+: 2.9 mEq/L HCO3-: 36 mEq/L Venous blood gas shows a pH of 7.52. Which of the following tests is most appropriate in differentiating between different etiologies of this patient's lab abnormalities?
Arterial blood gas
Complete blood count
Serum glucose
Urine chloride
Urine toxicology
D
Urine chloride
This patient who presents with hypokalemia and metabolic alkalosis may either be vomiting or engaging in diuretic abuse. A urine chloride test can be used to distinguish between these possibilities because urine chloride will be high in diuretic abuse and will be low/normal in surreptitious vomiting. Metabolic alkalosis is an acid-base disorder that is due to an increased level of serum bicarbonate. This increase can be secondary to increased bicarbonate intake or loss of hydrogen ions. Etiologies consistent with increased intake include antacid use or milk-alkali syndrome. Etiologies consistent with hydrogen ion loss include gastrointestinal losses such as vomiting or renal losses such as hyperaldosteronism, Bartter syndrome, or diuretic abuse. Urine chloride can distinguish between gastrointestinal and renal losses because urine chloride levels will be high in patients with renal tubule dysfunction or diuretic abuse. This is due to increased urinary excretion of ammonium chloride in cases of diarrhea. On the other hand, urine chloride will generally be low in hyperaldosteronism due to loss of chloride with vomiting. Wu et al. studied the causes of chronic hypokalemia as well as laboratory findings that can be used to distinguish between different etiologies. They show how urine sodium and chloride can be helpful for identifying the surreptitious use of diuretics. The authors recommend the use of the sodium-to-chloride excretion ratio to help diagnose patients with chronic normotensive hypokalemia. Incorrect Answers: Answer A: Arterial blood gas is useful for determining the acid-base state of a patient because it simultaneously measures carbon dioxide and bicarbonate levels. This test is not as useful in determining different causes of metabolic alkalosis because all of these etiologies will have similar findings (high bicarbonate and compensatory high carbon dioxide), especially in patient who has already received a venous blood gas. Answer B: Complete blood count is useful for determining whether a patient is anemic resulting in decreased oxygen delivery to end organs due to decreased carrying capacity for oxygen. In cases of severe anemia and hypoperfusion, peripheral tissues produce lactic acid which will result in an anion gap metabolic acidosis rather than metabolic alkalosis. Answer C: Serum glucose elevation can result in increased diuresis as well as pseudohyponatremia (decreased measured sodium due to increased glucose). Alternatively, acidosis may also be caused by insulin deficiency in diabetic ketoacidosis. Neither of these findings is consistent with the lab results seen in this patient. Answer E: Urine toxicology can be used to determine whether the patient is taking any substances of abuse. There is no pathognomonic acid-base finding that can aid in diagnosing overdose and/or substance abuse. However, most standard urine toxicology screens do not detect the use of diuretics. Bullet Summary: Urine chloride can distinguish between gastrointestinal and renal causes of metabolic alkalosis.
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A 2-year-old boy is brought to the emergency room by his mother, who states that her son has been coughing uncontrollably for 3 hours. The child has a medical history of obesity and atopic dermatitis and uses topical steroid cream. His birth history is unremarkable. His family history is notable for asthma in his older sister and irritable bowel syndrome in his father. His temperature is 98.5°F (36.9°C), pulse is 120/min, blood pressure is 90/52 mmHg, and respirations are 32/min. On exam, he appears uncomfortable, is coughing continuously, and is drooling. Cardiopulmonary and abdominal exams are unremarkable. A chest radiograph is performed and the results are shown in Figures A and B. Which of the following is the most appropriate next step in management?
Balloon catheter removal
Endoscopic removal
Ipecac administration
Observation
Surgical extraction
B
Endoscopic removal
This child presents with foreign body ingestion with a button battery seen in the esophagus on radiography. The most appropriate next step in management is urgent endoscopic removal of the battery. Button batteries are commonly used in watches and small electronic devices. If these objects are ingested (usually by children), they can become lodged in the esophagus. Coughing, drooling, and chest discomfort are classic symptoms but a lack of symptoms does not negate the need for evaluation if suspicion is high. The location of the battery can be confirmed by radiograph and orthogonal views can localize the object in the esophagus versus the trachea. Endoscopy is the preferred method of removal as direct visualization of any associated esophageal injury is possible. The battery can cause ulceration or perforation if left in the esophagus due to chemical extravasation. Baharudin et al. review a number of cases of pediatric ingestions resulting in esophageal lodging. They discuss how a stack of coins can mimic the appearance of a battery. They recommend urgent intervention in cases of suspected battery ingestion. Figure/Illustration A is a chest radiograph depicting a round object lodged in the esophagus, with a “double halo” sign (red circle). This sign is an indication that this object is a battery rather than a coin. Figure/Illustration B is a lateral chest radiograph demonstrating that the battery is posterior to the trachea (red circle). This view confirms that the location of the battery is in the esophagus. Incorrect Answers: Answer A: Balloon catheter removal can be attempted for ingested button batteries but is typically used only if endoscopy is not available. There is more potential for tissue damage without direct visualization, so ingestion time greater than 2 hours is generally a contraindication for this method. Answer B: Ipecac administration can induce emesis and is rarely used for toxic ingestions. Vomiting can cause further lodging in the esophagus and even more tissue damage. Ipecac is therefore not recommended in the modern management of toxic ingestions. Answer D: Observation would be appropriate if there is an ingested object that has already traveled further into the gastrointestinal tract (if they are small and smooth), as these objects would likely pass on their own. A classic example would be a coin ingestion that would appear as a single round object rather than a double ring appearance. A button battery in the esophagus is an emergency and should be removed urgently. Answer E: Surgical extraction of a battery with associated repair may be required if there is suspicion of an esophageal-vascular fistula, mediastinitis, or other such complications. It may also be necessary if a magnet was simultaneously ingested. In the absence of these complications, endoscopic removal should be attempted first. Bullet Summary: Button batteries lodged in the esophagus require urgent endoscopic removal.
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A 45-year-old man presents to a physician due to difficulty swallowing and slurred speech. For the past 3 months, the patient has been coughing when he drinks liquids. His speech also sounds different according to his wife. The patient has no significant medical history. His temperature is 98.6°F (37.0°C), blood pressure is 110/80 mmHg, pulse is 60/min, and respirations are 12/min. On physical exam, he is unable to fully extend his tongue, and there are fasciculations on his tongue. When tapping the mandible at the chin while the mouth is held open, there is pronounced upward movement of the lower jaw. Sensory exam reveals no deficits. Which of the following therapies is most likely to improve survival for this patient's disease process?
Baclofen
Dextromethorphan and quinidine
Edaravone
Riluzole
Tizanidine
D
Riluzole
This patient with dysphagia (difficulty swallowing water), dysarthria (altered and slurred speech), lower motor neuron deficits (tongue fasciculations and weakness), upper motor neuron deficits (exaggerated jaw jerk reflex), and preserved sensory function most likely has amyotrophic lateral sclerosis (ALS). Riluzole, a glutamate inhibitor, is currently the only Food and Drug Administration (FDA) approved medication that improves survival in patients with ALS. ALS is a neurodegenerative disease that affects both upper and lower motor neurons. Upper motor neuron signs include spasticity and hyperreflexia, while lower motor neuron signs include muscle atrophy and fasciculations (spontaneous, involuntary muscle contraction and relaxation). In patients with bulbar-onset ALS, the initial clinical presentation is dysarthria, dysphagia, tongue atrophy, and abnormal jaw jerk reflex. Riluzole decreases the rate of glutamate release from presynaptic neurons, which is thought to decrease excitatory neurotoxicity. Riluzole improves survival by 2-6 months in patients with ALS and may have a greater survival benefit in patients with bulbar-onset ALS. Masori and Van Damme review the genetics, pathophysiology, classic clinical presentation, extra-motor manifestations, diagnostic workup, and treatment for ALS. They discuss how riluzole provides a survival benefit in patients with this disease. They recommend treating patients with ALS using riluzole as tolerated. Incorrect Answers: Answer A: Baclofen is a gamma-aminobutyric acid (GABA) agonist that reduces the release of excitatory neurotransmitters to decrease spasticity in ALS. Along with tizanidine, baclofen is a first-line symptomatic treatment for spasticity in ALS. Baclofen does not improve survival in ALS. Answer B: Dextromethorphan and quinidine is a combination of a sigma opioid receptor agonist and a sodium channel blocker used to control pseudobulbar affect in patients with ALS. Pseudobulbar affect is a sudden uncontrollable outburst of laughter or tearfulness that affects almost half of patients with ALS. Dextromethorphan and quinidine has not been shown to improve survival in ALS. Answer C: Edaravone is an intravenous antioxidant that may decrease the progression of early ALS. Although it is now FDA approved, studies have not yet shown that edaravone improves survival in patients with ALS. Further studies will be needed in order to determine the risks and benefits of using this drug. Answer E: Tizanidine is a central alpha-2-adrenergic receptor agonist that decreases spasticity in ALS by increasing presynaptic inhibition of motor neurons. Excess spasticity in ALS can negatively impact coordination, ambulation, and other activities of daily living. Tizanidine and other antispastic medications do not improve survival in patients with ALS. Bullet Summary: Riluzole is a glutamate inhibitor that improves survival in patients with amyotrophic lateral sclerosis.
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A 55-year-old male bodybuilder presents to the emergency department with weakness of his right arm. The patient states he has experienced these symptoms for a few weeks; however, today his hand felt so weak he dropped his cup of tea. The patient has a medical history of diabetes. He drinks 2-7 alcoholic drinks per day and has smoked 2 packs of cigarettes per day since he was 25. The patient uses anabolic steroids. He has lost 17 pounds since he last came to the emergency department 1 month ago. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals decreased sensation in the right arm and 2/5 strength in the right arm and 5/5 strength in in the left arm. The patient states that he is experiencing a dull aching and burning pain in his right arm during the exam. Symptoms are not changed with changes in head and neck position. Which of the following is the most likely diagnosis?
Apical lung tumor
Brachial plexopathy
Cerebral infarction
Scalenus anticus syndrome
Subclavian steal syndrome
A
Apical lung tumor
This patient is presenting with unilateral upper extremity paresthesia and weakness suggestive of compression of the neurovascular structures of the upper extremity secondary to an apical lung tumor. An apical lung tumor can compress the neurovascular structures in its proximity and cause pain, numbness, and weakness of the ipsilateral arm. A Horner syndrome can also be observed with compression of the sympathetic chain leading to ipsilateral miosis, ptosis, and anhidrosis in some cases. These malignancies typically occur in older male smokers and can be associated with symptoms such as fatigue, cough, and weight loss. Chest radiography will demonstrate an apical opacity and CT scan can confirm the size and location of the mass. Treatment is with chemotherapy and surgical excision of the tumor. Davis and Knight review the evidence regarding the diagnosis and treatment of patients with apical lung tumors. They discuss how these tumors can be associated with brachial plexus injuries. They recommend surgical excision of these tumors if they are resectable. Incorrect Answers: Answer B: Brachial plexopathy could present with weakness and numbness of the ipsilateral arm but is a less likely diagnosis (and a diagnosis of exclusion). Treatment is with identification and surgical release of any underlying anatomic defects (such as compressive bands) and supportive therapy if none are found. Answer C: Cerebral infarction (stroke) would present with sudden onset of acute neurological deficits. These would typically only affect one side of the body and would not necessarily be associated with a dull pain or numbness that is chronic. Treatment is with thrombolytics if patients meet criteria for acute therapy. Answer D: Scalenus anticus syndrome occurs in bodybuilders or athletes with hypertrophied neck muscles that compress the brachial plexus. Symptoms are typically worsened by certain positions. Treatment is with physical therapy and surgical decompression in refractory cases. Answer E: Subclavian steal syndrome presents with numbness, tingling, weakness in a limb, and CNS symptoms (vertigo, slurred speech, or stroke-like symptoms) that are worsened with activity and relieved with rest. Treatment is with vascular intervention to restore blood flow. Bullet Summary: Apical lung tumors can compress neurovascular structures in the upper extremity and cause pain, numbness, and tingling in the ipsilateral arm.
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A 41-year-old man presents to his primary care provider after seeing bright red blood in the toilet bowl after his last 2 bowel movements. He reports that he also noticed some blood mixed with his stool. The patient denies abdominal pain or any changes in his stool habits. He notes a weight loss of 8 pounds in the last 2 months with no changes in his diet or exercise habits. His medical history is significant for an episode of pancreatitis 2 years ago for which he was hospitalized for several days. He drinks 2-3 beers on the weekend and he has never smoked. He has no family history of colon cancer. His temperature is 97.6°F (36.4°C), blood pressure is 135/78 mmHg, pulse is 88/min, and respirations are 14/min. On physical exam, his abdomen is soft and non-tender to palpation. Bowel sounds are present, and there is no hepatomegaly. Which of the following is the most appropriate next step in management?
Abdominal CT
Anoscopy
Barium enema
Colonoscopy
Ultrasound of abdomen
D
Colonoscopy
This patient presents with hematochezia and weight loss, which raises concern for colorectal cancer. The most appropriate next step in management is performing a colonoscopy. The differential diagnosis of hematochezia includes colorectal cancer, inflammatory bowel disease, diverticulosis, and hemorrhoids. The evaluation depends in large part on the age of the patient and whether the patient presents with alarm symptoms such as weight loss, abdominal pain, change in bowel habits, or iron-deficiency anemia. If the patient is over the age of 50, has alarm symptoms, or has a family history of colorectal cancer, the evaluation should begin with a colonoscopy to rule out malignancy. If the patient is between the ages of 40 and 50 and without other red flags, the patient can elect to undergo sigmoidoscopy as a less invasive procedure than colonoscopy. If the patient is under the age of 40 and without alarm symptoms, the workup may begin with an anoscopy to first evaluate for hemorrhoids. Once cancer has been ruled out, additional evaluation for chronic inflammatory diseases such as ulcerative colitis can be conducted. Church presents evidence regarding the current use of colonoscopy. He discusses how the detection of neoplasia during colonoscopy is suboptimal despite its use as the current screening tool of choice. He recommends understanding which patients are missed and how to better detect neoplasias in these patients. Incorrect Answers: Answer A: Abdominal CT might be used to evaluate for metastases if the diagnosis of colorectal cancer is confirmed but it would not be used to diagnose the primary malignancy. A CT scan would be the most appropriate initial step in diagnosis if diffuse metastasis were already suspected such as a presentation of severe weight loss and an enlarged, palpable, and nodular liver. Answer B: Anoscopy is used for the evaluation of hemorrhoids in a patient < 40 years of age who presents with bright red blood per rectum in the absence of alarm symptoms. It would not be the most appropriate next step for a patient who presents with unexplained weight loss as it cannot be used to diagnose colorectal cancer. Answer C: Barium enema is sometimes used to screen for colorectal cancer in conjunction with other tests in asymptomatic patients, but it would not be used to diagnose malignancy in a patient with alarm symptoms such as hematochezia and unexpected weight loss. Answer E: Ultrasound of the abdomen would be effective in diagnosing conditions such as intraperitoneal bleeding and cholecystitis. There is no evidence of abdominal trauma that would suggest intraperitoneal bleeding in this patient. A positive ultrasound with free fluid would be treated with diagnostic laparoscopy. Bullet Summary: If a patient is over the age of 50 or has alarm symptoms of weight loss, abdominal pain, change in bowel habits, or iron-deficiency anemia, the patient should undergo a colonoscopy to rule out colorectal cancer.
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A 57-year-old man presents to the emergency department for shortness of breath. He was riding the train home from a business conference when his symptoms began. The patient has a medical history of obesity, diabetes, diabetic nephropathy, hypertension, and a 40-pack-year smoking history. His current medications include atorvastatin, lisinopril, insulin, metformin, and nicotine gum. His temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 120/min, respirations are 15/min, and oxygen saturation is 93% on room air. An ECG is ordered as seen in Figure A. A chest radiograph is ordered as seen in Figure B. Laboratory values are ordered as seen below: Serum: Na+: 137 mEq/L Cl-: 105 mEq/L K+: 4.1 mEq/L HCO3-: 24 mEq/L BUN: 22 mg/dL Glucose: 129 mg/dL Creatinine: 1.2 mg/dL Ca2+: 10.1 mg/dL AST: 11 U/L ALT: 12 U/L On physical exam, the patient appears to be in distress. His cardiac exam is notable for tachycardia. Pulmonary exam reveals bilateral air movement without any wheezes or crackles. The patient's right lower extremity appears mildly inflamed, and palpation of the calf elicits pain. Which of the following is the most appropriate next step in management?
Aspirin
Cardiac troponins
CT scan of the chest without contrast
Heparin
Ventilation perfusion scan
D
Heparin
This patient is presenting with a score of 7.5 on Wells criteria suggesting a diagnosis of pulmonary embolism. The most appropriate initial step in management is heparin administration. Patients with suspected pulmonary embolism (PE) can be evaluated with Wells criteria which consists of: clinical signs and symptoms of DVT (3 points), PE is the most likely diagnosis (3 points), pulse > 100 (1.5 points), immobilization for 3 days, or surgery within 4 weeks (1.5 points), history of DVT or PE (1.5 points), hemoptysis (1 point), and malignancy (1 point). Scores higher than 3 suggest a high probability of PE. Patients will often present with shortness of breath, with ECG findings of sinus tachycardia, and a chest radiograph that is typically normal. With a high pre-test probability, the most important initial step in management is anticoagulation with heparin versus further diagnostic workup. Doherty reviews the evidence regarding the diagnosis and treatment of pulmonary embolism. He discusses how clinical criteria such as Wells criteria can be helpful in assessing the likelihood of a PE. He recommends immediate treatment in patients who have a PE. Figure/Illustration A is an ECG demonstrating sinus tachycardia with a P wave, a QRS complex, and a T wave during every beat (red circle). This is the most common ECG presentation in PE, though right heart strain can also be seen. Figure B is a chest radiograph that is within normal limits without evidence of fluid in the dependent portions of the lung (red circles). This is the most common radiological presentation in PE. Incorrect Answers: Answer A: Aspirin would be the most appropriate initial step in the management of MI and can reduce mortality by up to 25% if administered promptly. PE is a more likely diagnosis in this patient given the ECG that only demonstrates sinus tachycardia. Answer B: Cardiac troponins could be used to confirm the diagnosis of myocardial infarction (MI). In the presence of this patient's ECG (which does not demonstrate ST elevation), MI is a less likely diagnosis. Treatment would be with aspirin in patients who had an MI followed by emergent revascularization. Answer C: A CT scan of the chest without contrast would not confirm the diagnosis in this patient; however, a CT angiogram would be the most accurate test. It is more important to begin treatment with heparin when there is a very high clinical suspicion of PE. Answer E: A ventilation perfusion scan is the most accurate test for PE in a patient who cannot tolerate contrast. This test has a low specificity. Bullet Summary: The most appropriate initial step in the management of a pulmonary embolism in patients with a high pre-test probability is anticoagulation with heparin.
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An 18-year-old man presents to his primary care provider with his parents for a sports physical. He was last seen in the clinic several months ago when he was diagnosed with attention deficit hyperactivity disorder (ADHD). He was started on methylphenidate at that time and the patient now reports improvement in his ability to concentrate in school and at home. He hopes to play baseball in college and has begun lifting weights daily in preparation for baseball season. The patient has a healthy diet to fuel his exercise regimen. His parents have no concerns and are pleased with the recent improvement in his grades. 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 has tall stature with average muscle mass for his age. He has no dysmorphic features. His chest has a normal appearance other than mild gynecomastia. The patient has sparse facial hair and a moderate amount of coarse pubic hair that extends across the pubis and spares the medial thighs. His testes are small and firm. Laboratory testing is performed and reveals the following: Follicle-stimulating hormone (FSH): 42 mIU/mL Luteinizing hormone (LH): 38 mIU/mL Which of the following is the most likely etiology of this patient’s presentation?
Anabolic steroid use
CGG trinucleotide repeat disorder
CTG trinucleotide repeat disorder
Failure of neuronal migration
Meiotic nondisjunction
E
Meiotic nondisjunction
This patient presents with tall stature, gynecomastia, and small testes with elevated FSH and LH, which suggests a diagnosis of Klinefelter syndrome. Klinefelter syndrome is usually caused by meiotic nondisjunction that results in a 47,XXY genotype. Klinefelter syndrome is the most common cause of primary hypogonadism. Patients with Klinefelter syndrome present with tall stature, neurocognitive difficulties (ADHD), and features of hypogonadism including gynecomastia, small testes, small phallus, hypospadias, underdeveloped secondary sex characteristics, and cryptorchidism. Patients without hypospadias or cryptorchidism are often not diagnosed until after puberty when the symptoms of gynecomastia and small testes become more prominent. Because the hypogonadism in Klinefelter syndrome is caused by testicular fibrosis, laboratory results demonstrate low testosterone and elevated FSH and LH levels. Treatment for this condition includes androgen replacement therapy. Lanfranco et al. review the evidence regarding the treatment of Klinefelter syndrome. They discuss how modern fertility augmentation therapy has allowed some of these patients to produce viable offspring. They recommend having a detailed discussion with these patients and their partners regarding possible outcomes. Incorrect Answers: Answer A: Anabolic steroid use causes decreased levels of FSH and LH due to the suppression of GnRH release by the hypothalamus. This results in decreased FSH and LH release by the pituitary gland. Anabolic steroid use would not present with signs of hypogonadism. Answer B: The CGG trinucleotide repeat disorder characterizes fragile X syndrome. Fragile X presents with macroorchidism rather than hypogonadism and patients typically have dysmorphic features of a long, narrow face with large ears, a prominent forehead, and a prominent chin. Fragile X is the most common cause of inherited intellectual disability. Answer C: The CTG trinucleotide repeat disorder characterizes myotonic dystrophy. Although myotonic dystrophy presents with hypogonadism, patients would also present with symptoms of progressive weakness, such as facial weakness, dysphagia, or hand grip weakness. Treatment is with cardiac pacemakers and ventilatory support. Answer D: Failure of neuronal migration characterizes Kallmann syndrome. Kallmann syndrome presents with the classic symptoms of anosmia and hypogonadism, but patients with Kallmann syndrome have a low FSH and LH. Treatment is with hormone replacement therapy. Bullet Summary: Klinefelter syndrome results in primary hypogonadism and presents with tall stature, gynecomastia, small testes, a small phallus, hypospadias, and cryptorchidism.
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A 57-year-old immigrant from Nigeria presents to the emergency department for sudden, severe pain and swelling in her lower extremity. She was at a rehabilitation hospital when her symptoms started. The patient has a medical history of obesity, diabetes, bipolar disorder, and tonic-clonic seizures. Her current medications include metformin, insulin, lisinopril, and valproic acid. The patient has IV drug and alcohol use disorder and has presented to the ED many times for intoxication. Her temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. Physical exam reveals anasarca and asymmetric lower extremity swelling. Based on the results of a doppler ultrasound of her swollen lower extremity, heparin is started. The patient is then transferred to the general medicine floor for continued management. Laboratory studies are shown below. Serum: Na+: 137 mEq/L K+: 5.5 mEq/L Cl-: 100 mEq/L HCO3-: 24 mEq/L Urea nitrogen: 22 mg/dL Ca2+: 5.7 mg/dL Creatinine: 1.7 mg/dL Glucose: 70 mg/dL Which of the following is the most likely diagnosis?
Antithrombin III deficiency
Factor V Leiden
Liver failure
Nephrotic syndrome
Prothrombin gene mutation
D
Nephrotic syndrome
This patient who presents with a deep venous thrombosis (lower extremity swelling, ultrasound followed by treatment with heparin), anasarca, and asymptomatic hypocalcemia most likely has a diagnosis of nephrotic syndrome. Nephrotic syndrome occurs when large amounts of protein are lost in the urine. This can lead to hyperlipidemia (due to loss of lipoproteins), hypercoagulable state (due to loss of antithrombin III and protein C/S), and hypoalbuminemia. Most serum calcium is bound to albumin, so when albumin is lost in the urine, it can lead to profound hypocalcemia. The ionized or free portion of calcium remains the same; therefore, these patients do not exhibit symptoms of hypocalcemia. Furthermore, the loss of albumin will reduce the oncotic pressure in the vessels resulting in massive extravasation of fluid and anasarca. Ambler et al. report a case of a patient who presented with a DVT and was found to have nephrotic syndrome. They discuss how the patient had asymptomatic changes in lab values including calcium. They recommend performing a urine dipstick for protein in cases of suspected nephrotic syndrome. Incorrect Answers: Answer A: Antithrombin III deficiency is a common cause of a hypercoagulable state because antithrombin III normally helps to neutralize coagulation factors. This disease is a possible explanation for this patient's DVT as it predisposes patients to thrombotic events; however, it does not explain her profound hypocalcemia. Asymptomatic antithrombin III deficiency does not need to be treated; however, patients should have anticoagulation if they undergo surgery. Answer B: Factor V Leiden is the most common cause of a hypercoagulable state because this mutation renders factor V unable to be inactivated by proteins C and S. This is a possible explanation for this patient's DVT; however, it does not explain her hypocalcemia. Asymptomatic factor V Leiden mutation does not need to be treated; however, patients should have anticoagulation if they undergo surgery. Answer D: Liver dysfunction is possible given this patient's history of alcohol use and IV drug abuse, placing her at risk for chronic hepatitis C infection. Despite the anasarca, this patient has no other stigmata of liver failure such as jaundice or ascites, making nephrotic syndrome more likely. Answer E: Prothrombin gene mutation could explain this patient's hypercoagulable state because high levels of this gene can result in unprovoked thrombotic events. This disease would not explain why the patient has anasarca or hypocalcemia. Asymptomatic patients with this mutation do not require treatment. Bullet Summary: Nephrotic syndrome can present with a hypercoagulable state due to loss of antithrombin III and protein C/S and hypocalcemia from loss of albumin in the urine.
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A 57-year-old woman presents to the emergency department for a headache. She was at home when a sudden headache began with greater severity than she had experienced in the past. She presented within 30 minutes of symptom onset. The patient has a medical history of sinusitis, constipation, diabetes, and pelvic inflammatory disease. Her current medications include fexofenadine, oral contraceptive pills, and metformin. She is currently taking amoxicillin for a sinus infection. She smokes 1 pack of cigarettes per day and drinks 2-3 alcoholic beverages daily. Her temperature is 99.5°F (37.5°C), blood pressure is 187/118 mmHg, pulse is 120/min, respirations are 17/min, and oxygen saturation is 98% on room air. On physical exam, the patient is noted to be uncomfortable with the appearance shown in Figure A. Neurological exam reveals a confused woman who is now complaining of "seeing double." Cardiopulmonary exam reveals mild bibasilar crackles and tachycardia. Abdominal exam is within normal limits. Which of the following is the most appropriate initial step in management?
Amoxicillin-clavulinic acid
CT of the head
Dexamethasone
MRI of the head
Piperacillin-tazobactam
E
Piperacillin-tazobactam
This patient is presenting with symptoms suggestive of septic cavernous sinus thrombosis (CST). The most appropriate initial step in management is urgent broad-spectrum antibiotics and surgical drainage. Cavernous sinus thrombosis is an emergency that requires immediate intervention. It typically occurs in a patient with a history of sinusitis, oral contraceptive pill usage, smoking, or other risk factors that could cause a hypercoagulable state. These patients will often present with severe pain, a sudden headache, and diplopia. A distinct appearance is typically noted with periorbital edema. These patients should be immediately treated with broad-spectrum antibiotics and undergo surgical drainage of the inciting source of infection. In some cases, thrombolytics can be used as well. Caranfa and Yoon review the evidence regarding the diagnosis and treatment of septic cavernous sinus thrombosis. They discuss how this diagnosis was almost universally fatal in the pre-antibiotic era. They recommend using broad-spectrum antibiotics and debridement to address this disease. Figure/Illustration A is a clinical photograph demonstrating a face with swelling and periorbital edema (red circles). This appearance is classically seen in patients with CST. Incorrect Answers: Answer A: Amoxicillin-clavulanic acid would be appropriate management of acute otitis media or sinusitis; however, it is insufficient coverage for CST. Broad-spectrum antibiotics and surgical intervention are needed to prevent loss of vision. Answer B: A CT scan of the head could be indicated potentially for surgical planning in a more stable patient; however, in this situation, immediate intervention is more dire. After antibiotics and stabilization, CNS imaging will be needed. Answer C: Dexamethasone would be appropriate treatment of a relapse of multiple sclerosis, which can also present with visual changes; however, it would not present with sudden headache and periorbital edema. Answer D: An MRI of the brain would be appropriate for further characterizing neurologic deficits of unclear etiology. In this case, the patient's acute presentation with neurologic changes secondary to CST requires immediate treatment rather than additional imaging. Bullet Summary: Cavernous sinus thrombosis should be immediately managed with broad-spectrum antibiotics and neurosurgery.
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A 43-year-old man presents to the emergency department with bright red blood in his stool this morning. He noticed this yesterday as well, but it was much scanter. The patient is otherwise healthy. He was celebrating his birthday last night and admits having "too much to drink" and vomited profusely overnight. He currently feels well and is not nauseous. He has no other significant medical history and does not take any medications. His temperature is 99.2°F (37.3°C), blood pressure is 110/75 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 100% on room air. Physical exam reveals a nontender abdomen with normal bowel sounds. Rectal exam reveals grossly bloody stool. Laboratory studies are ordered as shown below. Hemoglobin: 12 g/dL Hematocrit: 36% Leukocyte count: 5,500/mm^3 with normal differential Platelet count: 179,000/mm^3 Which of the following is the most likely cause of this patient's symptoms?
Erosion into a gastric vessel
Inflammation and infection of colonic outpouching
Malignancy
Submucosal esophageal tear
Vascular malformation
E
Vascular malformation
This patient is presenting with bright red blood per rectum with a non-tender abdomen. Of the answer choices given, angiodysplasia is the most likely to cause such profuse and sudden bleeding. Angiodysplasia is a vascular malformation found in the gastrointestinal (GI) tract, most commonly in the colon. The exposed vessels can bleed, leading to bright red blood per rectum. The diagnosis can be confirmed with colonoscopy. Ablation can be performed to stop the bleeding. Any patient who is unstable or anemic with a hemoglobin < 7.0 g/dL requires a transfusion with blood products. Angiodysplasia is a common cause of bright red blood per rectum but is not more common than diverticulosis. Garcia-Compeon et al. review the presentation and treatment of gastrointestinal angiodysplasia. The authors note that angiodysplasias are the cause of many "occult" GI bleeds not visualized on upper endoscopy or colonoscopy. The authors recommend the use of capsule endoscopy in occult GI bleeds to identify angiodysplasias as a potential source. Incorrect Answers: Answer A: Erosion into a gastric vessel is the pathophysiology of a bleeding gastric vessel secondary to peptic ulcer disease. This usually presents with melena (black tarry stools) rather than bright red blood per rectum. Only in cases of extremely brisk upper GI bleeding would patients present with bright red blood per rectum. Treatment involves treating Helicobacter pylori infection if present, proton pump inhibitors, and sucralfate. Endoscopy is needed to confirm and treat the bleeding vessel. Answer B: Inflammation and infection of colonic outpouching is the pathophysiology of diverticulitis which presents with a history of constipation with a fever and left lower quadrant abdominal pain. Management involves a computed tomography (CT) scan of the abdomen/pelvis and administration of antibiotics (such as ciprofloxacin and metronidazole or ceftriaxone and metronidazole). Diverticulosis presents with bright red blood and is possible in this patient, but it does not involve inflammation or infection, which defines diverticulitis. Answer C: Malignancy or colon cancer can present with bright red blood per rectum, malaise, weight loss, and microcytic anemia. While it is possible in this patient, his age, lack of risk factors for colon cancer, and the sudden onset of his symptoms make this diagnosis less likely. Answer D: Submucosal esophageal (Mallory-Weiss) tear presents after profuse vomiting (as in this patient) with bloody vomitus. Treatment is supportive and involves antiemetics. Bullet Summary: Angiodysplasia is a vascular malformation that presents with bright red blood per rectum.
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A 5-year-old girl presents to the emergency room due to difficulty walking. She has been complaining of pain in her right leg for the last few days. Her neighbor’s cat bit her last week and her parents attributed the pain to her healing bite. At the time of the bite, they cleaned the wound and irrigated it with sterile saline water from a first aid kit. She has no medical history and has never been hospitalized. Her temperature is 102.2°F (39°C), blood pressure is 118/78 mmHg, pulse is 90/min, respirations are 21/min, and pulse oximetry is 99% on room air. The open wound remains present on the thigh with surrounding erythema and edema. MRI is consistent with osteomyelitis. Which of the following is the most appropriate next step in management?
Amoxicillin and clavulanate
Ampicillin and sulbactam
Doxycycline
Flucanozole
Vancomycin
B
Ampicillin and sulbactam
This young girl with a recent history of a cat bite and difficulty walking most likely has likely Pasteurella multocida osteomyelitis. Empiric IV antibiotic therapy with ampicillin-sulbactam is the most appropriate initial management. Pasteurella multocida is a gram-negative rod that is associated with cat and dog bites. Infection can lead to cellulitis and osteomyelitis. Treatment of minor infection is with amoxicillin-clavulanate; however, patients suspected of having osteomyelitis should be treated empirically with IV antibiotics (e.g., ampicillin and sulbactam) and taken for biopsy/culture to identify the pathogen. Antibiotics can then be narrowed down accordingly. Patients will often require between 4-8 weeks of antibiotic treatment. MRSA coverage may initially be started and tapered later once cultures result. Mollitt reviews the evidence regarding the microbiology of various injuries. He discusses how Pasteurella multocida is a common complication of animal bites. He recommends early effective antibiotic treatment. Incorrect Answer: Answer A: Amoxicillin and clavulanate can be used to treat Pasteurella infection. Patients with osteomyelitis should not be treated with oral antibiotics initially, in particular, when there are systemic manifestations as is present in this patient. IV antibiotics such as ampicillin and sulbactam are the mainstay for empiric osteomyelitis treatment after an animal bite in a patient with systemic manifestations. Answer C: Doxycycline is used to treat uncomplicated chlamydia infections. Gonococcal osteomyelitis may present in sexually active individuals and may be associated with septic arthritis. Sexually active patients may be suspected of having this disease. Answer D: Fluconazole is an antifungal that is used to treat Candida osteomyelitis. Candida osteomyelitis would be highly unusual in patients with no other risk factors. Instead, this disease is more common in immunodeficient patients or those who use IV drugs. Answer E: Vancomycin is used for MRSA infections. Although S. aureus is the most common overall cause of osteomyelitis, this patient with a recent animal bite most likely has Pasteurella infection and this organism should be covered accordingly. Bullet Summary: Patients with suspicion of Pasteurella osteomyelitis infection after an animal bite should be treated with empiric IV antibiotics such as ampicillin and sulbactam.
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A 14-year-old boy presents to his pediatrician with a 2-month history of intermittent arm and leg spasms. He says that the spasms usually occur after he wakes up as he is getting ready for school, and consist of disorganized jerking motions that stop after a few minutes. He has also noticed that they occur when he stays up late to cram last minute for exams. He has not noticed any other symptoms. He has no medical history and takes no medications. He recently started experimenting with alcohol and marijuana that one of his friends has been stealing from their parents. His temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. Physical exam reveals a healthy child. Which of the following is the most likely cause of this patient's symptoms?
Absence seizures
Alcohol intoxication
Cannabinoid use
Juvenile myoclonic epilepsy
West syndrome
D
Juvenile myoclonic epilepsy
This patient who presents with intermittent jerking of his extremities that occur primarily after waking up or when sleep-deprived most likely has juvenile myoclonic epilepsy. Juvenile myoclonic epilepsy (JME) is a poorly understood condition that presents with intermittent myoclonic jerks of the extremities. These myoclonic jerks usually have an onset occurring in early adolescence in otherwise healthy children. Myoclonic jerks are most frequently seen right after waking from sleep, but they may also be triggered by lack of sleep, stress, or alcohol consumption. Patients with this disease rarely have associated symptoms but may occasionally also experience absence seizures or generalized tonic-clonic seizures. The diagnosis can be secured with a history of seizures alongside an interictal electroencephalogram (EEG) that shows 4 to 6 Hz bilateral polyspikes. Valproate is the first-line treatment. Johannessen et al. discuss the treatment of juvenile myoclonic epilepsy. They find that standard antiepileptic treatment with agents such as valproate leads to only 33% of patients remaining seizure-free. They recommend trials of alternative antiepileptic medications such as levetiracetam, lamotrigine, and topiramate in patients that do not respond to valproate. Incorrect Answers: Answer A: Absence seizures commonly present during childhood in otherwise healthy children; however, these seizures are typically characterized by short episodes of loss of awareness known as "staring spells" without physical manifestations. Answer B: Alcohol intoxication is a risk factor for precipitating juvenile myoclonic epilepsy, but alcohol use does not cause this condition. Seizure activity may also occur during alcohol withdrawal, but the timing of this patient's symptoms is more suggestive of seizure symptoms. Alcohol withdrawal requires chronic drinking of large amounts leading to an increase in central nervous system N-methyl-D-aspartate receptors and a decrease in CNS gamma-aminobutyric acid type A receptors. When alcohol cessation occurs, seizures ensue. Answer C: Cannabinoid use is not associated with seizure activity either during intoxication or withdrawal. Patients may have altered mental status, but this does not manifest as myoclonic jerks. Answer E: West syndrome is an infantile seizure disorder that usually presents between 3 months and 12 months of age. These patients will present with recurrent flexion spasms of the extremities. Bullet Summary: Juvenile myoclonic epilepsy presents in adolescents with recurrent spasms after waking from sleep or when sleep-deprived.
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A 4-week-old boy presents to the pediatrician for vomiting. The patient is breastfed and typically feeds for 20-30 minutes every 2 hours. One week ago, he began vomiting and regurgitating breastmilk through his nose after most feedings. His stools over the past week have also become blood-streaked. The patient does not seem to be in any distress when he passes these bloody stools. The patient surpassed his birth weight by 2 weeks of age but has fallen 1 standard deviation on the growth curve since then. His temperature is 98.6°F (37°C), blood pressure is 78/47 mmHg, pulse is 115/min, and respirations are 28/min. On physical exam, the patient is well-appearing. His face and back have the physical exam finding seen in Figure A. The patient’s abdomen is soft, non-tender, and non-distended. A digital rectal exam reveals a small amount of blood in the rectal vault. Which of the following is the most appropriate next step in management?
Initiate a proton pump inhibitor
Modify mother's diet
Obtain abdominal ultrasound
Provide reassurance
Switch to hydrolyzed formula
B
Modify mother's diet
This infant presents with vomiting, bloody stools, and poor weight gain, which suggests a diagnosis of milk-protein allergy. The most appropriate next step in management is the modification of the mother’s diet to eliminate cow’s milk and soy. Infant food protein-induced proctocolitis is caused by distal colon inflammation secondary to an immune reaction to certain food proteins. Milk-protein allergy is the most common food allergy in children and presents with vomiting, bloody stools, and poor weight gain or failure to thrive. In an infant that is breastfed, the most appropriate next step in management is the elimination of cow’s milk and soy from the mother’s diet. In an infant who is formula fed, the most appropriate next step would be switching to either an extensively hydrolyzed or amino acid-based infant formula. 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 that demonstrates the finding of eczema (red circle). This finding is commonly found in patients with milk-protein allergy. Incorrect Answers: Answer A: Initiating a proton pump inhibitor would be appropriate in patients with refractory gastroesophageal reflux disease, which presents with regurgitation or vomiting and symptoms of feeding aversion or failure to thrive. Because this patient presents with bloody stools, he is more likely to have a milk protein allergy. Answer B: Obtaining an abdominal ultrasound would help evaluate for pyloric stenosis, which presents in infants between 3-6 months of age with non-bilious, projectile vomiting. Pyloric stenosis does not cause bloody stools. Treatment is with surgical pyloromyotomy. Answer C: Providing reassurance would not be appropriate for an infant presenting with bloody stools and evidence of poor weight gain. The cause of his blood-streaked stools should be investigated to ensure this patient is getting the nutrition he needs. Answer E: Switching to a hydrolyzed formula would be appropriate if this patient were formula fed, but it is not necessary to discontinue breastfeeding if the mother is willing to modify her diet to exclude cow’s milk and soy. Bullet Summary: A milk-protein allergy in a breastfed infant should be treated with modification of the mother’s diet to eliminate cow’s milk and soy.
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A 4-year-old boy comes to the pediatrician with his mother with a 3-day history of cough and runny nose with decreased oral intake over the past 24 hours. His activity level has been normal and he has been attending school until this morning. He has no medical history or known allergies, but he has not received any of the recommended vaccines due to parental refusal. His temperature is 100.3°F (37.9°C), blood pressure is 98/62 mmHg, pulse is 85/min, respirations are 22/min, and oxygen saturation is 98% on room air. On examination, the child is fussy. Bilateral conjunctivae are injected, and there is redness on and around the tonsils. There is no lymphadenopathy. Faint rhonchi are heard at the lung bases bilaterally. Which of the following is the most appropriate next step in management?
Administer antitoxin and toxoid vaccine
Amoxicillin
Influenza testing and oseltamivir
Obtain chest radiography and respiratory virus panel
Supportive care with return precautions
E
Supportive care with return precautions
This boy presenting with 3 days of progressive fever, rhinorrhea, cough, conjunctivitis, and tonsilar erythema most likely has viral pharyngitis. The appropriate treatment for this is supportive care with return precautions (instructing the mother to bring the child back to a physician if he becomes short of breath). Viral pharyngitis in children is most often caused by adenovirus, though other viruses can cause the condition as well. The diagnosis is clinical, with a presentation of fever, rhinorrhea, conjunctivitis, sore throat, and cough. Features that can help distinguish viral pharyngitis from pneumonia include a lack of tachypnea, increased work of breathing, hypoxemia, or adventitial lung sounds. No additional testing or intervention is necessary. The illness is typically self-limited and resolves with supportive care. Shapiro et al. study features predictive of group A streptococcal (GAS) pharyngitis in children. The authors found that among patients with features of viral illness, patients older than 11 years of age who did not have a fever, cervical lymphadenopathy, or tonsillar exudates were at the lowest risk for GAS. The authors recommend against testing low-risk children for GAS to avoid antibiotic treatment in patients merely colonized with GAS. Incorrect Answers: Answer A: Administering antitoxin and toxoid vaccine is the treatment for Corynebacterium diphtheriae infection. Although this child has not received his age-appropriate vaccinations, his presentation is not concerning for infection with diphtheria. C. diphtheriae infection presents with cervical lymphadenopathy and gray pseudomembranes on pharyngeal exam, neither of which this child has. Answer B: Amoxicillin should be given in cases of group A streptococcal (GAS) pharyngitis. Before amoxicillin is prescribed, testing with Streptococcal rapid antigen detection testing should be performed. Additionally, rapid antigen detection testing is not indicated in this patient as she has a cough and does not have cervical lymphadenopathy, tonsillar exudates, or palatal petechiae. All of these findings are not typical of GAS pharyngitis. Answer C: Influenza testing and oseltamivir are indicated if the child had myalgias or high fevers that would make influenza more likely and if he presented within 2 days of symptom onset. Oseltamivir is only approved for treating influenza in uncomplicated cases if started within this 2-day window from symptom onset. Exceptions to this 2-day guideline include adults greater than 65 years of age, pregnant women, residents of long-term care facilities, American Indians, persons with BMI greater than 40 kg/m^2, and those receiving immunosuppressive medications. Answer D: Obtaining a chest radiograph and respiratory virus panel can be done when a lower respiratory infection such as bacterial pneumonia is expected. However, viral infections are much more common than bacterial pneumonia in this age group. Additionally, this patient is not tachypneic, does not have evidence of increased work of breathing, and is not hypoxemic, all of which make bacterial pneumonia less likely. Bullet Summary: Viral pharyngitis in children is a clinical diagnosis consisting of fever, rhinorrhea, conjunctivitis, cough, sore throat, and pharyngeal erythema, and is treated with supportive care.
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A 37-year-old woman presents to the emergency department in cardiac arrest. She was found to have altered mental status at home, and during transport to the hospital, she became pulseless. Cardiopulmonary resuscitation is ongoing and 2 intravenous lines are placed. The patient is obese, and her only medication is an oral contraceptive pill. She recently underwent Achilles tendon repair. She otherwise is known to smoke cigarettes on occasion. Physical exam is notable for a critically ill patient with chest compressions ongoing. The monitor shows pulseless electrical activity. Her right lower extremity is in a post-operative splint. The patient regains her pulse after several rounds of CPR and epinephrine. A bedside echocardiograph is notable for a dilated and hypokinetic right ventricle with septal bowing into a hyperkinetic left ventricle. Which of the following is the most likely diagnosis?
Cardiogenic shock
Hemorrhagic shock
Neurogenic shock
Obstructive shock
Septic shock
D
Obstructive shock
This obese patient on oral contraceptive pills with recent orthopedic surgery (Achilles tendon repair) is presenting in cardiac arrest. Given the bedside echocardiograph that is notable for a dilated and hypokinetic right ventricle with septal bowing into a hyperkinetic left ventricle, the most likely diagnosis is a pulmonary embolism that is causing obstructive shock. Pulmonary embolism is most commonly caused by a deep vein thrombosis in the veins of the lower extremity that travels to the pulmonary circulation. Patients will present with pleuritic chest pain, hypoxia, sinus tachycardia, and hemoptysis. Massive pulmonary emboli can cause hemodynamic instability. Patients may become hypotensive, tachycardic, and ultimately pulseless. The pulmonary embolism can obstruct blood flow from the right ventricle to the pulmonary artery, leading to a dilated and hypokinetic right ventricle with septal bowing and a hyperdynamic left ventricle. This is a type of obstructive shock. Treatment of a massive pulmonary embolism causing hemodynamic instability may involve thrombolytics or thrombectomy. Standl et al. review obstructive shock. They note that obstructive shock is caused by an obstruction of blood flow out of (or in the case of tamponade, into) the heart. They recommend that treating the underlying cause is the only effective management option. Incorrect Answers: Answer A: Cardiogenic shock is caused by cardiac dysfunction in conditions such as myocardial infarction or heart failure. Cardiac dysfunction then leads to impaired systemic perfusion. Initial management includes treating the underlying cause (e.g., cardiac catheterization for an ST-elevation myocardial infarction) and vasopressors (e.g., norepinephrine) to maintain the patient’s blood pressure. Answer B: Hemorrhagic shock is a common cause of shock in trauma and presents with hypotension and tachycardia secondary to blood loss. A narrowed pulse pressure may be the first sign of hemorrhagic shock. The mainstay of management is blood transfusion and an intervention to stop the bleeding. Answer C: Neurogenic shock occurs when there is central nervous system trauma (often to the cervical spine) leading to decreased sympathetic tone systemically. Patients will present with hypotension and bradycardia (contrast with tachycardia in hemorrhagic shock), and management is centered on vasopressors to maintain blood pressure and neurosurgical intervention for the underlying etiology. IV fluids can also be given to maintain blood pressure. Answer E: Septic shock occurs when a systemic infection leads to vasodilation and endothelial dysfunction with hypotension refractory to fluid resuscitation. The mainstay of management includes fluids, blood cultures, vasopressors, and broad-spectrum antibiotics. Early administration of antibiotics improves outcomes. Bullet Summary: Pulmonary embolism is a common cause of obstructive shock.
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A 23-year-old woman presents to the emergency room for a self-inflicted laceration of her distal volar forearm. The patient states she knew her boyfriend was having sexual thoughts about the woman from the grocery store, prompting her decision to cut her own wrist. In the emergency department, the bleeding has stopped and the patient is currently medically stable. When interviewing the patient, she is teary and apologizes for her behavior. She is grateful to you for her care and regrets her actions. Of note, the patient has presented to the emergency department before for a similar reason when she was struggling with online dating. The patient states that she struggles with her romantic relationships though she deeply desires them. Her temperature is 98.6°F (37°C), blood pressure is 112/71 mmHg, pulse is 73/min, and respirations are 14/min. On physical exam, you note a frightened young woman who is wearing a revealing dress that prominently displays her breasts. You tell the patient that she will have to stay in the psychiatric emergency department for the night which makes her furious. Which of the following personality disorders is the most likely diagnosis?
Antisocial
Avoidant
Borderline
Dependent
Histrionic
C
Borderline
This patient is presenting with a suicide attempt, unstable relationships, and emotional lability suggesting a diagnosis of borderline personality disorder. Borderline personality disorder classically presents with emotional lability, and intense, unstable relationships. These patients fear abandonment and will often engage in self-harming behavior like cutting and suicide attempts. The main defense mechanism these patients employ is splitting, in which patients divide the world into dichotomous categories where people are either all good or all bad. This personality disorder is associated with mood disorders and substance use. The treatment of choice for these patients is long-term dialectical behavioral therapy and mood stabilizers. Leichsenring et al. review the evidence regarding the treatment of patients with borderline personality disorder. They discuss no specific form of psychotherapy is superior to others. They recommend starting any form of psychotherapy for these patients to improve outcomes. Incorrect Answers: Answer A: Antisocial personality disorder presents with aggressive, criminal behavior. These individuals do not conform to social norms or rules and violate the rights of others. Patients often are not amenable to behavioral modification and therefore incarceration may be necessary in order to protect the rights of others. Answer B: Avoidant personality disorder presents with excessive shyness and hypersensitivity to rejection. These patients fear social interaction though they strongly desire relationships. Cognitive behavioral therapy can be used in order to encourage these patients to be more open to seeking out relationships. Answer D: Dependent personality disorder presents with submissive and clingy behavior. They often have a need to be cared for and will be very agreeable and dependent on others. This can be harmful as patients may stay in abusive or degrading relationships due to a fear of being alone. Treatment is with cognitive behavioral therapy. Answer E: Histrionic personality disorder presents with colorful and exaggerated attention-seeking behavior. These patients will often employ sexuality to draw attention to themselves. Though this patient is displaying her breasts and wearing seductive clothing, her history of self-harm behavior and unstable relationships suggests a diagnosis of borderline personality disorder. Bullet Summary: Borderline personality disorder presents with emotional instability, unstable relationships, and self-harming behavior.
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An 8-week-old boy presents with his mother to the pediatrician for a well visit. The patient has been breastfed since birth, and usually feeds for 30 minutes every 2-3 hours. The patient’s mother is concerned that her milk production is not keeping up with the patient’s nutritional requirements. She reports that about 2 weeks ago the patient began regurgitating breastmilk through his nose and mouth after some feeds. He seems mildly upset during the episodes of regurgitation but usually settles down quickly and is hungry again soon afterwards. His mother has already tried limiting the volume of each feed, which seems to have reduced the frequency of regurgitation. She denies any diarrhea, hematochezia, or family history of food allergies. Her older son had a similar problem with vomiting that resolved around 12 months of age. Four weeks ago, the patient’s height and weight were in the 40th and 34th percentiles, respectively. His height and weight are now respectively in the 37th and 36th percentiles. His temperature is 98.6°F (37°C), blood pressure is 72/49 mmHg, pulse is 121/min, and respirations are 32/min. On physical exam, the patient is cooing in his mother’s lap and smiles reciprocally with her. He lifts his head and shoulders off the examination table when placed in the supine position. His abdomen is soft, non-tender and non-distended. Bowel sounds are normoactive. Which of the following is the most appropriate next step in management?
Initiate proton pump inhibitor
Obtain abdominal ultrasound
Reassurance and counseling on positioning
Recommend modification of mother’s diet
Switch to hydrolyzed formula
C
Reassurance and counseling on positioning
This patient presents with regurgitation after feeding, appropriate appetite after episodes of regurgitation, and good weight gain, which suggests a diagnosis of uncomplicated gastroesophageal reflux. The most appropriate next step in management is reassurance and counseling on proper positioning. Physiologic or uncomplicated gastroesophageal reflux usually presents in patients in the first few months of life with frequent regurgitation or vomiting, sustained appetite after regurgitation, and minimal irritability. The most appropriate first step in management is reassurance and counseling parents to position patients upright for 20-30 minutes after feeding. For patients with persistent symptoms, parents should be encouraged to provide lower volume feeds more frequently throughout the day, and consider a milk-free diet to address possible underlying cow’s milk allergy. Treatment with a proton pump inhibitor is reserved for intractable cases. For most patients, gastroesophageal reflux self-resolves by 1 year of age. Chabra and Peeples review the evidence regarding the treatment of neonatal GERD. They discuss how alterations in the infant's environment and feeding pattern are effective in most cases. They recommend proton pump inhibitors if conservative treatments are not effective. Incorrect Answers: Answer A: Initiating a proton pump inhibitor is appropriate for infants with gastroesophageal reflux disease who have failed management with lifestyle changes. This patient has uncomplicated gastroesophageal reflux without any signs of feeding refusal, worsening irritability, or nutritional compromise. Answer B: Obtaining an abdominal ultrasound would evaluate for pyloric stenosis, which presents with non-bilious, projectile vomiting rather than the regurgitation that this patient’s mother is describing. An abdominal ultrasound would be the appropriate next step in management for patients who present with a description of more forceful vomiting or have a classic “olive-shaped” mass in the epigastrium on physical exam. Answer D: Recommending modification of the mother’s diet would be appropriate if this patient had more severe symptoms that were refractory to lifestyle changes. It would also be appropriate if the patient had signs of an underlying allergy to cow’s milk or soy protein, such as blood-streaked stools. Answer E: Switching to a hydrolyzed formula would be appropriate for formula-fed patients with refractory symptoms of gastroesophageal reflux disease or signs of an allergy to cow’s milk or soy protein. Unless the patient has evidence of severe nutritional compromise, continued breastfeeding should be encouraged for breastfed patients with possible modification of the mother’s diet to eliminate cow’s milk or soy if an allergy is suspected. Bullet Summary: Uncomplicated gastroesophageal reflux in infants should be treated with reassurance and counseling to position the infant upright for 20-30 minutes after feeding.
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A 28-year-old G1P0 woman presents to initiate prenatal care after a positive home pregnancy test. The first day of her last menstrual period was 7 weeks prior to presentation. The patient has no medical conditions and takes no medications. Her temperature is 98.6°F (37.0°C), blood pressure is 110/70 mmHg, pulse is 64/min, and respirations are 17/min. Physical exam is notable for a nontender abdomen. Pelvic exam reveals a closed cervix with no adnexal or uterine tenderness. A urine pregnancy test is positive, and an intrauterine singleton pregnancy measuring approximately 9 weeks gestational age is visualized on transvaginal ultrasound. The patient asks if she should modify her current exercise regimen due to her pregnancy. She typically swims or uses an exercise bike for 45 minutes, 5 days a week, and plays in an office soccer league for 1 hour on 1 day per week. Which of the following is the most appropriate recommendation regarding exercise for this patient?
Avoid exercise during pregnancy
Avoid soccer during pregnancy, continue other activities
Continue the current exercise regimen
Limit moderate exercise to a maximum of 30 minutes per day during pregnancy
Reduce to walking only, beginning in the third trimester
B
Avoid soccer during pregnancy, continue other activities
This healthy primigravida woman presents to the clinic inquiring about her current exercise regimen involving 285 minutes of moderate to exercise weekly. With the exception of soccer, a contact sport, her exercise regimen is appropriate throughout her pregnancy. The American College of Obstetricians and Gynecologists (ACOG), recommends that healthy pregnant women engage in moderate-intensity exercise for at least 30 minutes or more for 5-7 days weekly provided there are no contraindications to doing so (certain cardiac or pulmonary conditions, history of cerclage placement, multiple pregnancies, preeclampsia, or severe anemia). Highly active patients may continue their more vigorous pre-pregnancy regimens after consulting with a physician. Exercise in pregnancy can ease gastrointestinal symptoms and may decrease the risk of gestational diabetes, preeclampsia, and cesarean delivery. However, activities that may cause fetal or maternal injury, such as contact sports or activities with a significant fall risk should be avoided. Examples of contact sports include football, basketball, hockey, lacrosse, and wrestling. Nascimento et al. review exercise in pregnancy. They note that exercise is beneficial in pregnancy and in the postpartum period and recommend engaging in appropriate exercise in this patient population. Incorrect Answers: Answer A: Avoiding exercise during pregnancy is not recommended, as at least 150 minutes of moderate exercise weekly is suggested during pregnancy for the health of the mother and the fetus. Answer C: Continuing the current exercise regimen is not recommended, as soccer is a high-contact sport that could cause injury to the fetus or placenta. Answer D: Limiting moderate exercise to 30 minutes per day maximum during pregnancy is not recommended, as 30 minutes daily is the minimum recommended time for moderate-exercise. There is no reason for healthy pregnant patients without high-risk pregnancies to significantly limit their exercise. Answer E: Reducing to walking-only beginning in the third trimester is not necessary, as moderate activities are healthy throughout pregnancy. Bullet Summary: Healthy pregnant patients should perform at least 30 minutes of exercise 5-7 days per week and avoid high-contact sports or sports with fall risk.
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A 39-year-old man presents to his doctor for a wellness checkup. He is concerned about a rash that does not seem to be improving. He was recently exposed to his grandfather who has vesicular lesions on his skin and is being treated. He has a family history of skin cancer, colon cancer, and ovarian cancer. The patient has a medical history of asthma and seasonal allergies. His temperature is 98.6°F (37.0°C), blood pressure is 137/98 mmHg, pulse is 90/min, respirations are 14/min, and oxygen saturation is 98% on room air. Physical exam reveals the finding in Figure A. Which of the following describes this patient's most likely diagnosis?
Benign capillary proliferation
Edema of the epidermis
Healthy dermatologic development
Malignant blood vessel proliferation
Viral infection
A
Benign capillary proliferation
This patient who presents with red raised papules is presenting with a cherry angioma. These lesions are composed of benign capillary proliferations. Cherry angiomas present with cherry red macules/papules that occur secondary to benign capillary and venule proliferation. The lesions are entirely benign and are cosmetic. These lesions are common in middle-aged adults. As the patient ages, more lesions will appear. In the absence of symptoms, observation and reassurance can be used for conservative management. They can be removed surgically if the patient desires. Nazer et al. study the risk factors associated with the development of cherry angioma. They found that the use of clopidogrel and tamsulosin is associated with these lesions. They recommend ruling out underlying conditions. Figure/Illustration A demonstrates cherry red macules/papules classically seen in patients with cherry angioma. Incorrect Answers: Answer B: Edema of the epidermis describes eczema or atopic dermatitis. This disease would present with pruritic, erythematous, and vesicular lesions. Topical creams and hygiene can be used for symptomatic management of these lesions. Answer C: Healthy dermatologic development misses the diagnosis of a cherry angioma, which although benign, is a more accurate diagnosis for this case. Answer D: Malignant blood vessel proliferation does not describe a cherry angioma which is benign and purely cosmetic. An angiosarcoma usually occurs in deep tissues and requires surgical excision. Answer E: Viral infection describes herpes simplex virus which would present with vesicular lesions and neurogenic pain. These patients can be treated with valacyclovir as well as gabapentin for neuropathic pain. Bullet Summary: Cherry angiomas are benign capillary proliferations that appear as cherry red macules/papules.
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A 46-year-old man presents with his wife to his primary care provider for depression and strange movements. His wife reports that her husband has not been himself for the last 2 months. Whereas he was previously outgoing and “the life of the party,” the patient is now irritable and withdrawn. He is a partner at an accounting firm, but his colleagues are threatening to fire him if he continues to perform poorly at work. The patient cannot explain the recent changes to his mood and tearfully admits he fears there is something seriously wrong with him. His wife says that he is getting worse. The patient’s medical history is significant for hypertension, for which he takes lisinopril. His family history is unknown as he was adopted. The patient met his mother once and never knew his father but was told he died in his 50s. He drinks a few glasses of wine per week and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 141/87 mmHg, pulse is 81/min, and respirations are 12/min. On physical exam, the patient has a flat affect with a facial grimace and sudden jerky movements of his upper extremities. Which of the following is most likely to be seen on further workup?
Alpha-synuclein aggregates on brain biopsy
Dorsal striatum atrophy on head CT
Frontotemporal atrophy on head CT
Neurofibrillary tangles on brain biopsy
Positive 14-3-3 CSF assay
B
Dorsal striatum atrophy on head CT
This patient presents with recent mood changes, jerky movements of the upper extremities, and a history of early death in his father, which suggests a diagnosis of Huntington disease. Huntington disease is characterized by atrophy of the caudate nucleus, which is a structure of the dorsal striatum. Huntington disease (HD) is an autosomal dominant trinucleotide (CAG) repeat disorder located on chromosome 4. HD typically presents with mood changes and abnormal movements (particularly chorea or myoclonus) over weeks to months. Although executive function can be affected early on, memory is usually preserved until relatively late in the disease. Frontotemporal dementia, in contrast, usually presents with behavioral changes, and Alzheimer disease has memory impairment as an early sign. Caudate atrophy is typically seen on head imaging in Huntington disease. This disease cannot be cured but supportive treatment includes benzodiazepines and tetrabenazine for symptomatic relief of muscle spasms. Walker presents a review of the evidence regarding the etiology of Huntington disease. He discusses that typically the onset of symptoms is in middle age after affected individuals have had children. He recommends a better understanding of the function of the mutant protein in this disease. Incorrect Answers: Answer A: Alpha-synuclein aggregates would be found on brain biopsy in Parkinson disease and Lewy body dementia. Lewy body dementia is characterized by parkinsonism and visual hallucinations. Patients would present with cogwheel rigidity, bradykinesia, and a resting tremor. Treatment is with dopamine agonists such as carbidopa/levodopa. Answer C: Frontotemporal atrophy is consistent with a diagnosis of frontotemporal dementia. Frontotemporal dementia presents primarily with early behavioral changes, and it would not present with myoclonus. Treatment is supportive as there is no cure for this disease. Patients and their spouses may require psychiatric support for changes in behavior. Answer D: Neurofibrillary tangles on brain biopsy would suggest a diagnosis of Alzheimer disease. This disease presents with a slowly progressive cognitive decline in elderly patients. Patients have early loss of memory but have maintained motor function and behavior until later in the course of the disease. Answer E: A positive 14-3-3 CSF assay would confirm the diagnosis of Creutzfeldt-Jakob disease (CJD). Although CJD may also present with mood changes and myoclonus, CJD most prominently causes rapidly progressive dementia early in life. Treatment is supportive and death occurs quickly. Bullet Summary: Huntington disease is an autosomal dominant trinucleotide repeat disorder located on chromosome 4 that causes caudate atrophy and presents with mood changes, abnormal movements, and subcortical dementia.
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A 69-year-old man presents to the emergency department with shortness of breath. The patient has presented 3 times this month with similar complaints. He states his shortness of breath started when he was walking from his car to a local restaurant. He does not see a primary care physician and is not currently taking any medications. He drinks alcohol socially and does not smoke. His temperature is 99.5°F (37.5°C), pulse is 100/min, blood pressure is 130/90 mmHg, respirations are 18/min, and oxygen saturation is 96% on room air. On physical exam, he appears fatigued and the cardiovascular exam reveals an additional heart sound after S2. A pulmonary exam is notable for bilateral crackles and an abdominal exam reveals an obese abdomen without pain in any of the quadrants. Lower extremity pitting edema is noted bilaterally. Which of the sets of lab values shown in Figure A would most likely be seen in this patient?
A
B
C
D
E
C
C
This patient is presenting with symptoms of heart failure (CHF). The most likely laboratory abnormalities are elevated brain natriuretic peptide (BNP), high anti-diuretic hormone (ADH), low sodium, and low potassium. Heart failure is characterized by the decreased performance of the cardiac ventricles resulting in ventricular dilation. As the ventricles dilate, they release BNP as a marker of stretching of myocardial tissue. BNP levels can therefore serve as a marker of CHF. The kidneys will also have decreased perfusion levels in this disease leading to several key physiologic changes. First, is the activation of the renin-angiotensin-aldosterone system, which serves to increase perfusion to the kidneys by increasing blood pressure. This system achieves increased blood pressure by maintaining sodium and fluid retention at the expense of decreased potassium (hypokalemia) and decreased hydronium ions (metabolic alkalosis). In addition, ADH is also increased to further increase perfusion, resulting in water retention, hemodilution, and hyponatremia. Ma et al. studied the correlation between BNP levels and cardiovascular outcomes. They found that patients with CHF and high levels of BNP had increased rates of cardiovascular complications. They recommend assessing BNP levels in patients who are suspected of having CHF. Figure A is a chart with changes in brain natriuretic peptide, anti-diuretic hormone, sodium, and potassium that would be seen in various diseases. Incorrect Answers: Answer A: High BNP, high ADH, high sodium, and high potassium does not reflect the changes that would be seen in CHF. Though aldosterone serves to retain sodium, it also absorbs water. Thus, hypernatremia would not be seen. This pattern could be seen in patients with heart failure who have high free water losses such as after a burn or in desert environments. Answer B: High BNP, low ADH, normal sodium, and low potassium does not reflect the appropriate increase in ADH and subsequent decrease in sodium that would be seen in CHF. Patients who have diabetes insipidus can have low ADH in this setting resulting in loss of free water. Answer D: Low BNP, high ADH, low sodium, and low potassium does not reflect the finding of elevated BNP that is classically found in the dilated ventricles of CHF. This pattern would be seen in patients with the syndrome of inappropriate ADH where excessive resorption of free water results in hemodilution. Answer E: Low BNP, low ADH, normal sodium, and normal potassium reflect the findings in a healthy patient. This patient most likely has CHF, which would result in increased aldosterone and ADH function. Bullet Summary: Common lab findings in heart failure include elevated BNP, elevated ADH, low sodium, and low potassium.
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A 19-year-old woman presents to her primary care physician with headaches, blurry vision, and tinnitus over the past month. She started treatment for a skin condition with a new oral medication 2 months prior. She has not noticed any changes in her complexion as of yet. Her temperature is 98.2°F (36.8°C), blood pressure 100/65 mmHg, pulse 87/min, and respiratory rate 14/min. She is oriented to person, place, and time. Neurological exam reveals papilledema upon examination of both eyes. Skin examination is notable for the findings in Figure A. Which of the following is the most likely diagnosis?
Cerebrovascular accident
Giant cell arteritis
Idiopathic intracranial hypertension
Meniere disease
Migraine
C
Idiopathic intracranial hypertension
This young patient with severe nodulocytic acne who was started on a new oral medication (likely isotretinoin) is likely suffering from idiopathic intracranial hypertension, which can present with papilledema, headache, blurry vision, and diplopia. Idiopathic intracranial hypertension is characterized by an increase in intracranial pressure without an identifiable cause. Patients often present with symptoms that include headache, blurry vision, and tinnitus. Fundoscopic examination typically reveals bilateral papilledema. Vision loss is a complication of this condition with high morbidity. While there is no identifiable cause, common associations include obesity or a recent increase in weight as well as medications such as isotretinoin - a vitamin A derivative that is often used in the management of severe nodulocystic acne vulgaris. Management should first involve imaging of the brain (CT or MRI) to exclude elevated cerebrospinal fluid pressure due to other causes such as brain tumor, dural sinus thrombosis, or hydrocephalus. The diagnosis is made via lumbar puncture revealing increased opening pressure. Lumbar puncture can also result in therapeutic relief. The specimen should be sent for gram stain, culture, and viral PCR to rule out other etiologies. Long term treatment is centered on weight loss and avoiding causative agents. Acetazolamide and serial lumbar punctures are other treatments. Boyter reviews the clinical presentation and management of patients with idiopathic intracranial hypertension. They found the condition is most common among obese women in their 20's, but it also may occur in children, men, and older adults. They recommend referral for patients to see a neurologist for ongoing management of headaches and other symptoms, as well as an ophthalmologist or neuro-ophthalmologist for monitoring papilledema, given the risk of vision loss. Figure/Illustration A: The figure depicts a patient with severe nodules and cysts (red arrows) consistent with severe inflammatory nodulocystic acne that would be suitable for treatment with isotretinoin. Incorrect Answers: Answer A: Cerebrovascular accidents can be embolic or hemorrhagic in nature and often result in persistent neurologic deficits or resolution of an acute neurologic deficit in a patient with cerebrovascular risk factors (diabetes, hypertension, hyperlipidemia). This young, healthy patient with intermittent headache and neurologic symptoms without a family history of hypercoagulable state is unlikely to have suffered from a cerebrovascular accident. Answer B: Giant cell arteritis (GCA) is a vasculitis that is typically seen in elderly patients (>60 years of age) and presents with tenderness of the ipsilateral temple and elevated inflammatory markers. Many patients with GCA have one or more constitutional symptoms (weight loss, fever, fatigue, anorexia, malaise) in addition to symptoms such as new onset headache or jaw claudication. Answer D: Meniere disease affects the inner ear and is characterized by severe episodes of vertigo, tinnitus, hearing loss, and fullness in the ear. Patients are often women between the ages of 40 and 60. Headaches and blurry vision are not a classic part of the clinical presentation. Answer E: Migraines present with severe headaches that can be incapacitating with associated photophobia and phonophobia. In some cases, patients may present with an aura that precedes or occurs during the migraine. The presence of papilledema suggests elevated intracranial pressure and is not typically seen in migraines. Bullet Summary: Idiopathic intracranial hypertension may be a side effect of isotretinoin.
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A 52-year-old man presents to the emergency department by ambulance for evaluation of hematemesis. His wife states that he began vomiting bright red blood this evening while eating dinner, and has produced about 5 cups total. He has a history of alcohol use disorder and has vomited small streaks of blood in the past, but has never undergone formal medical workup for it. On exam, he has bright red blood in his mouth and pharynx, and also displays a distended abdomen with a fluid wave, jaundice, scattered spider angiomas, and multiple ecchymoses. His temperature is 98.4°F (36.9°C), blood pressure is 98/68 mmHg, pulse is 125/min, and respirations are 22/min. A rapid point-of-care hemoglobin and platelet count is 8.8 g/dL and 80,000/mm^3, respectively. The patient is given 1L of normal saline. Which of the following is the most appropriate initial step in management?
Balloon tamponade
Ceftriaxone
Nadolol
Octreotide
Platelet transfusion
D
Octreotide
This patient with a history of alcohol use disorder and signs of liver failure (jaundice, ascites, spider angiomas, and ecchymoses) presents with acute hematemesis and hypovolemic shock (hypotension, tachycardia, tachypnea) most concerning for a variceal hemorrhage. The first step in the resuscitation of patients with variceal hemorrhage is rapid administration of intravenous (IV) fluids and octreotide. Esophageal and gastric varices, most commonly seen in patients with cirrhosis, are abnormally distended veins that are a product of venous congestion from portal hypertension. As these varices can project into the gastrointestinal lumen, they are subject to rupture either spontaneously or by mechanical disruption (e.g., tearing by food particles). Variceal hemorrhage is a life-threatening complication of cirrhosis as it can quickly progress to hemorrhagic shock or airway loss (if profuse vomiting is present). Patients should immediately receive 2 large-bore peripheral IVs (or central IV access) with which to administer IV fluid boluses to increase circulating volume. Blood should be transfused as needed, and a massive transfusion protocol may be needed. Patients should also be given octreotide, a splanchnic vasoconstrictor, in an effort to divert blood away from the hemorrhaging varices. Other treatments that should be given include ceftriaxone (to reduce bacteremia and mortality), proton pump inhibitors, and possibly interventions such as tranexamic acid. Upper GI endoscopy and banding are the definitive therapy. Stanley et al review the management of acute upper gastrointestinal bleeding with initial IV fluid resuscitation as the first measure to achieve hemodynamic stability. They also report that for patients with cirrhosis, vasoactive drugs such as octreotide should be started as soon as variceal hemorrhage is suspected. Red blood cell transfusion is indicated at a hemoglobin transfusion of 7.0-8.0 g/dL. Definitive therapy is endoscopy with variceal ligation. Incorrect Answers: Answer A: Balloon tamponade is often performed in the emergency department or intensive care unit as a temporizing measure to achieve hemostasis for patients with severe and persistent variceal hemorrhage. The balloon is inserted into the esophagus or stomach and inflated to apply direct pressure to the bleeding varices; this buys valuable time in the transition to definitive endoscopic management. Answer B: Ceftriaxone is administered to patients with cirrhosis who present with an upper gastrointestinal bleed as a prophylactic measure against spontaneous bacterial peritonitis. This patient is hemodynamically unstable, and antibiotics should not delay initial intravenous fluid resuscitation as well as other measures to reduce and/or stop the bleeding. While this patient should receive ceftriaxone, it is more dire to first give fluids, blood, and octreotide. Answer C: Nadolol or other nonselective beta-blocker therapy is indicated as secondary prophylaxis to prevent future bleeding episodes for patients with a history of variceal hemorrhage. Non-selective beta blockers such as nadolol decrease portal pressure, but are contraindicated in the acute setting as they could exacerbate hypovolemic shock by reducing cardiac output. Answer D: Platelet transfusion is required for patients with variceal hemorrhage with an initial platelet count < 50,000/mm^3, and for those who receive a significant amount of blood products during their resuscitation (significant dilutional thrombocytopenia can occur after 10-12 units of transfused red blood cells). This patient's platelet count is above 50,000/mm^3, and he has not yet received blood products. Bullet Summary: The most appropriate initial step in the management of a cirrhosis patient with acute variceal hemorrhage is the rapid administration of intravenous fluids and octreotide, a splanchnic vasoconstrictor.
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A 38-year-old woman presents to the emergency department with difficulty eating. Over the past week, she has had increasing difficulty chewing her food and progressive pain in the left side of her jaw. This morning, she was unable to close her mouth for several minutes after taking a bite of her breakfast. Two months ago, the patient had a root canal on a left molar. Her medical history is significant for hyperlipidemia, mild intermittent asthma, and type 2 diabetes mellitus. She has never smoked and has 10-15 alcoholic drinks per week. She works as an aide at a nursing home. Her temperature is 100.8°F (38.2°C), blood pressure is 133/74 mmHg, pulse is 105/min, and respirations are 14/min. On physical exam, there is a bluish hue to the skin on the lower left side of the patient’s face. A 3x4 cm non-tender mass can be palpated inferior to the angle of the left mandible. Thick exudate is draining from an opening in the skin. The gram stain of the exudate can be seen in Figure A. Which of the following is the most appropriate treatment for this patient?
Amphotericin B
Clindamycin
Metronidazole
Penicillin
Trimethoprim-sulfamethoxazole
D
Penicillin
This patient presents with fever, trismus, and cutaneous drainage of sulfur granules in the setting of a recent dental procedure, which points to a diagnosis of cervicofacial actinomycosis. The most appropriate treatment for this disease is penicillin. Actinomyces is a gram-positive, filamentous rod that causes a cervicofacial infection after direct inoculation during a dental procedure or trauma. It typically presents in an indolent fashion over a time course of weeks to months in which a hard, indurated mass evolves into multiple abscesses that drain through sinus tracts through the skin. The exudate is classically thick with small yellow “sulfur” granules, although it may also be serosanguinous. Since the infection spreads by direct extension, patients may complain of pain when the infection infiltrates or compresses nearby structures. Trismus may occur for the same reasons. The treatment of choice is penicillin. Valour et al. review the evidence regarding the treatment of Actinomyces infection. They discuss how prolonged (6- to 12-month) high-dose penicillin or amoxicillin is required for the treatment of this disease. They recommend preventive measures such as improvement of dental hygiene to prevent this disease. Figure/Illustration A demonstrates the histologic appearance of a basophilic “sulfur granule” (red circle). These granules are found in the draining exudate in patients with actinomycosis. Incorrect Answers: Answer A: Amphotericin B is the treatment of choice for fungal infections such as mucormycosis. This disease generally presents with a rapidly progressive soft tissue infection that can be fatal if left untreated. Patients would present with black fungal lesions generally in immunocompromised or diabetic hosts. Answer B: Clindamycin may be used in the treatment of empyema as it has activity against many anaerobes. Although this patient has a substantial weekly alcohol intake and has a chief complaint of “difficulty eating,” she has no true pulmonary symptoms and her presentation is more likely related to her recent dental procedure. Answer C: Metronidazole may be used in the treatment of empyema. Although this patient has a substantial weekly alcohol intake and has a chief complaint of “difficulty eating,” she has no true pulmonary symptoms and her presentation is more likely related to her recent dental procedure. Answer E: Trimethoprim-sulfamethoxazole is the treatment of choice for Nocardiosis, which may cause cutaneous lesions in immunocompromised hosts. This disease more commonly causes pulmonary or central nervous system disease. Nocardia would not explain the granule seen in histology. Bullet Summary: Actinomyces is a gram-positive anaerobic rod that presents as an indurated mass with draining sinus tracts and should be treated with penicillin.
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A 3-year-old boy presents with his parents to a pediatrician for a new patient visit. The child was recently adopted and little is known about his medical or family history. The child seems to be doing well, but the patient is much larger than any of the other 3-year-olds in his preschool class. He eats a varied diet at home and with limited juice and snack foods. His temperature is 98.6°F (37°C), blood pressure is 101/67 mmHg, pulse is 110/min, and respirations are 24/min. On physical exam, the patient is in the 73rd percentile for weight, 99th percentile for height, and 86th percentile for head circumference. He appears mildly developmentally delayed. He has a fair complexion and tall stature with a prominent sternum. The patient also has joint hypermobility and hyperelastic skin. He appears to have poor visual acuity and is referred to an ophthalmologist, who finds downward lens subluxation of the right eye. This child is most likely to develop which of the following complications?
Aortic dissection
Medullary thyroid cancer
Osteoarthritis
Thromboembolic stroke
Wilms tumor
D
Thromboembolic stroke
This patient presents with Marfanoid habitus, joint hypermobility, hyperelastic skin, developmental delay, and downward lens subluxation, which suggests a diagnosis of homocystinuria. Homocystinuria predisposes to thromboembolic events such as stroke. Although homocystinuria shares many features with Marfan syndrome, patients with homocystinuria also present with a fair complexion, intellectual disability, and downward subluxation of the lens (instead of the upward subluxation seen in Marfan syndrome). Patients with homocystinuria are also at an increased risk of thromboembolic events that disproportionally affect the cerebral vessels. Similar to Marfan syndrome, patients have phenotypic features of a Marfanoid habitus, including tall stature, long limbs, arachnodactyly, joint hypermobility, skin hyperelasticity, pectus deformity, and scoliosis. Treatment includes vitamin B6 supplementation. Hasan et al. review the evidence regarding diseases associated with homocystinuria. They discuss how this disease is associated with cancer and Alzheimer disease. They recommend screening for this disease. Incorrect Answers: Answer A: Aortic dissection is a complication of Marfan syndrome, an autosomal dominant mutation of the fibrillin-1 gene. It is also associated with elevated homocysteine levels. Although Marfan syndrome shares many features with homocystinuria, the fair complexion, and intellectual disability seen in this patient are specific to homocystinuria. Treatment involves avoidance of contact sports and beta blockers. Answer B: Medullary thyroid cancer is part of the syndrome of multiple endocrine neoplasia type 2B (MEN2B), which is caused by a mutation in the RET proto-oncogene. Patients with MEN2B present with Marfanoid habitus, but they do not present with the other features described in this vignette, including fair complexion, intellectual disability, and downward lens subluxation. Treatment is with surgical excision of tumors. Answer C: Osteoarthritis is a complication of Ehlers-Danlos syndrome (EDS). Although patients with EDS may present with many of the phenotypic features of both Marfan syndrome and homocystinuria, patients are much less likely to have lens subluxation. Osteoarthritis may be seen with higher incidence in patients with homocystinuria. Treatment is with brace support of unstable joints. Answer E: Wilms tumor is associated with Beckwith-Wiedemann syndrome (BWS), a pediatric overgrowth disorder usually caused by a mutation at chromosome 11p15. BWS presents with macrosomia (“gigantism”), which is defined as height and weight > 97th percentile. BWS additionally presents with omphalocele, hemihyperplasia, visceromegaly, neonatal hypoglycemia, and other embryonal tumors, including hepatoblastoma and neuroblastoma. Treatment is with surgical correction of defects. Bullet Summary: Homocystinuria presents with Marfanoid habitus, fair complexion, intellectual disability, downward lens subluxation, and an increased risk of thromboembolic events.
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A 67-year-old woman presents from a nursing home with altered mental status. She was last known to be normal the night before. The patient has dementia at baseline and is minimally interactive. This morning, she was found to be obtunded. No further history was provided from the nursing home, and the patient is unable to provide any history. Her temperature is 95.0°F (35.0°C), blood pressure is 84/54 mmHg, pulse is 50/min, respirations are 9/min, and oxygen saturation is 98% on room air. Physical exam reveals an obtunded woman who only withdraws her extremities to pain. Her skin is cool and pale, and her hair is thin. There are no signs of trauma noted on exam. Laboratory values are notable for a whole blood sodium of 120 mEq/L. The patient is given several intravenous boluses of fluids and her blood pressure improves to 100/60 mmHg. Which of the following is most likely to confirm the underlying cause of this patient's symptoms?
Administer glucagon
Check serum TSH and free T4 levels
Obtain blood cultures and a serum lactate
Perform a CT scan of the head
Perform an echocardiogram
B
Check serum TSH and free T4 levels
This patient is presenting with obtundation, bradycardia, hypotension, hypothermia, thinning of her hair, and hyponatremia, which are concerning for myxedema coma. A serum TSH and free T4 would be most informative in confirming this diagnosis. Myxedema coma (decompensated hypothyroidism) is the most life-threatening manifestation of hypothyroidism. Key clinical features include hypothermia (often the temperature is <95.9°F (35.5°C)), bradycardia, hypotension, bradypnea (causing a respiratory acidosis), hypoxia, and obtundation. Immediate management is centered on warming the patient, warmed IV fluids, and protecting the patient's airway, if needed. If there is high clinical suspicion for myxedema coma, then IV levothyroxine can be given empirically. Otherwise, the TSH and free T4 should be checked, which will reveal an elevated TSH and a low free T4. Patients are often critically ill and require active rewarming and close monitoring in an ICU setting. Wall reviews myxedema coma. Wall notes that myxedema coma is a life-threatening manifestation of hypothyroidism. She recommends that generally IV T4 (levothyroxine) is preferred over IV T3 in initial management. Incorrect Answers: Answer A: Administering glucagon would be appropriate in a beta-blocker overdose, which presents with bradycardia, hypotension, and altered mental status. There is no indication that this patient intentionally overdosed, and her hyponatremia and thinning hair supports a diagnosis of hypothyroidism. Other treatments that can be given in a beta-blocker overdose including epinephrine, calcium, insulin, dextrose, and lipid emulsion therapy. Answer C: Obtaining blood cultures and a serum lactate is the appropriate management of sepsis/septic shock. Generally, patients with septic shock present with hypotension (refractory to fluid resuscitation), tachycardia, and fever or hypothermia. Though myocardial depression may happen in critically ill patients who are about to undergo cardiac arrest, hypothyroidism better explains this patient's constellation of symptoms. It would be appropriate to obtain cultures and a serum lactate in this patient while the differential is being narrowed. Answer D: Performing a CT scan of the head would be indicated at some point in the workup of patients with altered mental status if there was a suspected central nervous system etiology such as a subdural hematoma. However, hemodynamically unstable patients should never be sent to the CT scanner prior to stabilization and resuscitation. A subdural hematoma is more common in older patients and patients with alcohol use disorder who experience head trauma as the atrophy of their brain makes the bridging veins more vulnerable. Answer E: Performing an echocardiogram may show reduced cardiac function; however, it is unlikely to reveal the underlying cause of this patient's hypotension and bradycardia other than showing poor cardiac function. Bullet Summary: Myxedema coma presents as a critically ill, obtunded patient who is hypothermic, bradycardic, and hypotensive, and the diagnosis can be supported with a high TSH and a low free T4.
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A 43-year-old man presents to the emergency department acutely with altered mental status. He uses intravenous drugs and is unable to offer a history. He was found unconscious in a park. His temperature is 99.0°F (37.2°C), blood pressure is 80/40 mmHg, pulse is 156/min, and respirations are 26/min. Physical exam reveals an obtunded man covered in vomit, with track marks on his arm and several skin abscesses. The patient is given 4 liters of Ringer lactate and intravenous antibiotics. As the fourth liter of fluids is finishing, the patient is noted to have jugular venous distension and an oxygen saturation of 92% on nonrebreather. He is subsequently intubated. An ECG is obtained as seen in Figure A. The patient is started on norepinephrine, and his blood pressure is subsequently found to be 85/44 mmHg. Which of the following is the most appropriate next step in management?
Cardioversion
Epinephrine
Hydrocortisone
Normal saline
Phenylephrine
B
Epinephrine
This patient who uses intravenous drugs with skin abscesses (the likely source of his infection) is hypotensive and tachycardic, with a blood pressure that is not responding to IV fluids; this is concerning for septic shock. Given his persistent hypotension, he should be started on vasopressors of which norepinephrine is first line and epinephrine is second line. Septic shock is a life-threatening, systemic bacterial infection that leads to vasodilation and endothelial dysfunction. Early management is centered on IV fluids, blood cultures, broad-spectrum antibiotics, and workup to uncover the source. If the patient's blood pressure does not respond to IV fluids (by definition, their blood pressure will not respond to fluids in septic shock), vasopressors should be started with a goal mean arterial pressure > 65 mmHg. The first line vasopressor is norepinephrine, which can be titrated up for maximal effect. If this vasopressor fails to reach blood pressure goals, then the next vasopressor is either vasopressin or epinephrine (either is acceptable). If the second line vasopressor fails to increase blood pressure, then the third line vasopressor is vasopressin or epinephrine (whichever was not used as the second line pressor). Finally, the fourth line vasopressor for septic shock is phenylephrine. Levy et al. review sepsis. They update the Surviving Sepsis campaign which gives the most up to date management guidelines of sepsis including adequate fluid resuscitation, broad-spectrum antibiotics, and vasopressors. They recommend early antibiotics and resuscitation to minimize morbidity and mortality. Figure/Illustration A is an ECG demonstrating sinus tachycardia. Note the presence of P waves (red arrows highlighting several examples) that are present throughout all leads. Incorrect Answers: Answer A: Cardioversion would be appropriate in an unstable patient with unstable vital signs in the setting of atrial fibrillation with rapid ventricular response to convert the patient to normal sinus rhythm. Atrial fibrillation would present with an irregularly irregular rhythm without P waves. Cardioversion would not be indicated in sinus tachycardia. Answer C: Hydrocortisone is a potent glucocorticoid that also has mineralocorticoid properties. While giving an IV steroid may have a permissive effect on blood pressure (via upregulating alpha-1 receptors on the vasculature) there is no reason that this patient would have a low cortisol level (such as in chronic steroid use). Epinephrine is a better answer in this case, though steroids can be considered. Steroids are typically reserved for patients failing at least 2 vasopressors (unless they have a known cortisol-deficient state). Answer D: Normal saline is unlikely to benefit this patient as he has already been sufficiently fluid resuscitated. Moreover, it seems the patient may be developing pulmonary edema (hypoxia, jugular venous distension), a sign of fluid overload. Answer E: Phenylephrine is the fourth line vasopressor and is an alpha-1 agonist. It is much less potent than norepinephrine and epinephrine, and would not be started in the management of septic shock until the patient has received epinephrine, norepinephrine, and vasopressin. Bullet Summary: In septic shock, the vasopressors given (in order) are norepinephrine, epinephrine/vasopressin, then phenylephrine.
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A 53-year-old man presents to the clinic with a non-tender mass in his right anterior neck. The mass has been slowly enlarging over the past 1 year. He reports no dysphagia, difficulty breathing, or changes to his voice. He also denies heat intolerance, palpitations, tremors, or diarrhea. He has hypertension and hyperlipidemia, for which he takes lisinopril and atorvastatin. He has never smoked cigarettes. His temperature is 37.1°C (98.8°F), blood pressure is 130/84 mmHg, pulse is 86/min, respirations are 12/min, and oxygen saturation is 99% on room air. Bedside ultrasound reveals a 1x2 cm hypoechogenic nodule with microcalcifications in the right thyroid lobe, which is biopsied. Pathology results are shown in Figure A. Which of the following is the most appropriate next step in management?
Levothyroxine
Methimazole
Radioactive iodine
Repeat ultrasound in 6 months
Thyroidectomy followed by radioactive iodine
E
Thyroidectomy followed by radioactive iodine
This patient who presents with a slowly enlarging neck mass has ultrasound (hypoechogenic nodule with microcalcifications) and histology findings (concentrically laminated calcifications called psammoma bodies) consistent with papillary thyroid carcinoma. The most appropriate next step in management would be thyroidectomy followed by radioiodine ablation. Papillary thyroid carcinoma represents the majority of malignant thyroid cancers. They are slow-growing and are found in the hormone-producing follicular cells of the thyroid. Typically, these tumors are found incidentally on imaging. Risk factors for thyroid malignancy include a history of radiation exposure, female sex, and positive family history. Nodules with minimal uptake on radioactive iodine scan, firm and immobile solitary nodules, and rapidly growing nodules with associated hoarseness are more suggestive of thyroid malignancy. Findings on histology include empty-appearing nuclei with central clearing, psammoma bodies, and nuclear grooves. The treatment is thyroidectomy followed by radioactive iodine ablation of any remaining thyroid cells. Long-term care should include levothyroxine at a dose sufficient to suppress thyroid-stimulating hormone (TSH) release that may stimulate any remaining malignant cells. Oakley et al. studied if relatives of patients diagnosed as having papillary thyroid carcinoma are at increased risk using a retrospective cohort study. The authors found first-degree relatives were at a 5-fold increased risk, and second-degree relatives were at a 2-fold increased risk. The authors recommend more research into optimal approaches to screening for thyroid cancers. Figure/Illustration A is a histology section showing a psammoma body (blue circle), which are round, concentrically laminated calcifications that are formed after necrosis of papillary structures. Psammoma bodies and nuclei with central clearing (yellow circle) and nuclear grooves are common histology findings for papillary thyroid carcinoma. Incorrect Answers: Answer A: Levothyroxine would be the treatment of choice in hypothyroidism and is important in the long-term care of papillary thyroid carcinoma to suppress the TSH release that may stimulate any remaining malignant cells after treatment (thyroidectomy and radioactive iodine). Answer B: Methimazole is an agent that blocks thyroid peroxidase, thus inhibiting the synthesis of thyroid hormones. It is used in the treatment of hyperthyroidism and does not have a role in the treatment of papillary thyroid carcinoma. Answer C: Radioactive iodine is used as an adjunct after thyroidectomy in the treatment of papillary thyroid carcinoma in order to target any thyroid cells that may remain even after surgical excision. Surgery is the primary therapy for papillary thyroid carcinoma. Radioactive iodine is the primary method of therapy in cases of Grave disease, toxic adenoma, or multinodular goiter. Answer D: Repeating an ultrasound in 6 months to assess the growth of the nodule or the development of suspicious characteristics is appropriate in cases where the ultrasound and biopsy findings are consistent with a benign thyroid nodule. The ultrasound findings of hypoechogenicity, microcalcifications, hypervascularity, and taller-than-wide nodules suggest malignancy, and together with a biopsy consistent with thyroid malignancy would warrant surgical treatment with subsequent radioactive iodine ablation. Bullet Summary: Papillary thyroid carcinoma is managed with thyroidectomy followed by radioactive iodine ablation and long-term levothyroxine to suppress thyroid-stimulating hormone stimulation of any remaining malignant cells.
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An 84-year-old man presents to the physician with numbness and tingling in the right hand for the last 6 months. He reports these symptoms started intermittently in the middle finger after returning from a golfing trip but has progressed to being nearly constant. The thenar eminence, thumb, and other digits are spared. He reports no other symptoms. His medical problems include hypertension, hyperlipidemia, hypothyroidism, and chronic neck pain for which he takes amlodipine, hydrochlorothiazide, atorvastatin, levothyroxine, and duloxetine. Surgical history consists of posterior lumbar fusion and elective left total hip arthroplasty. He has a 90-pack-year smoking history and quit 10 years ago. The patient’s temperature is 99.6°F (37.6°C), blood pressure is 134/80 mmHg, pulse is 90/min, and respirations are 16/min. Physical exam reveals 4/5 strength in elbow extension and wrist flexion on the right. He has 5/5 strength in the remainder of the motor exam on the right upper extremity and on the left upper extremity. The triceps reflex is 1+ on the right compared to 2+ on the left. There is no spasticity. His neck pain, which radiates down his right arm, is reproduced with axial loading during neck extension with simultaneous rightward rotation and lateral bending. Which of the following is the most likely diagnosis in this patient?
Amyotrophic lateral sclerosis
Carpal tunnel syndrome
Cervical spondylosis
Syringomyelia
Transverse myelitis
C
Cervical spondylosis
This patient presents with chronic middle finger paresthesia, weakness in elbow extension and wrist flexion, and a diminished triceps reflex in the setting of a positive Spurling test (pain with axial loading during neck extension, rotation, and lateral bending towards the affected side), indicative of C7 cervical radiculopathy. Cervical spondylosis is a common cause of cervical radiculopathy in elderly patients with chronic neck pain, especially in patients with findings suggestive of degenerative joint diseases (history of lumbar fusion and hip arthroplasty). Cervical spondylosis is caused by degeneration of the intervertebral disc with possible disc herniation and facet joints with osteophyte formation. These osteophytes and cervical disc herniation can cause compression of exiting nerve roots, leading to radiculopathy. Radicular symptoms depend on the nerve root affected. For example, C6 radiculopathy causes weakness in wrist extension and a decreased brachioradialis reflex while C7 radiculopathy causes weakness in elbow extension and wrist flexion with a decreased triceps reflex. Sensory symptoms may be present depending on the dermatome affected. The diagnosis is confirmed with MRI. Treatment consists of non-steroidal anti-inflammatory drugs (NSAIDs), muscle relaxants, and/or corticosteroids for pain. Surgical treatments for severe or refractory cases include anterior cervical discectomy and fusion. Young outlines the diagnosis and management of cervical spondylotic myelopathy. The discusses its pathophysiology, including osteophyte formation and spinal cord dysfunction. They recommend considering decompression and fusion operations in selected patients. Incorrect Answers: Answer A: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease of the brain and spinal cord that leads to upper and lower motor neuron signs. ALS presents with hyperreflexia (as opposed to hyporeflexia), spasticity, weakness, and fasciculations. Treatment includes riluzole (to improve survival) and symptomatic management. Answer B: Carpal tunnel syndrome is caused by compression of the median nerve as it travels through the carpal tunnel at the wrist. While the thenar eminence is typically spared due to take-off of the palmar cutaneous branch proximal to the carpal tunnel, the thumb and index finger are also affected. This patient’s symptoms primarily involve the middle finger, which is more indicative of a radiculopathy. Treatment is activity modification, splinting, and ultimately, surgical decompression of the carpal tunnel if refractory. Answer D: Syringomyelia is caused by a fluid-filled cavity (syrinx) within the spinal cord that leads to central cord syndrome, characterized by weakness that is greater in the upper extremities compared to lower extremities and loss of pain and temperature sensation in a cape-like distribution in the upper back and upper extremities. This patient’s sensory loss is unilateral and in a dermatomal distribution. Treatment of syringomyelia can include surgical decompression. Answer E: Transverse myelitis is characterized by segmental inflammation of the spinal cord that would present with acute, bilateral flaccid paresis based on the level affected and sensory loss within a sensory level. Autonomic symptoms such as incontinence may also be present. This patient’s symptoms are unilateral and localizable to nerve root compression as opposed to the spinal cord. Bullet Summary: Cervical spondylosis is caused by degeneration of cervical discs and facet joints with osteophyte formation and can present with symptoms of cervical radiculopathy or myelopathy in elderly patients with chronic neck pain.
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A 2-year-old boy is brought to the pediatrician’s office for a well-child visit. The parents have no concerns at this time and note he is starting to run around and speak in 3-word sentences. He has no remarkable medical history and his mother had an uncomplicated full-term pregnancy and delivery. He is appropriately meeting the developmental milestones for his age. The patient’s temperature is 98.4°F (36.9°C), blood pressure is 96/52 mmHg, pulse is 102/min, and respirations are 25/min. He is in the 55th percentile for height and 62nd percentile for weight, which is consistent with his documented growth curves. Fundoscopic exam shows the finding in Figure A. His pediatrician continues the ocular examination and further notes that the pupils are equal, round, and reactive, extraocular movements are full, and ocular alignment appears normal. Which of the following is the most appropriate next step in management?
Biopsy
Genetic testing
Magnetic resonance imaging of the brain and orbits
Ophthalmology referral
Reevaluation at the next well-child visit
D
Ophthalmology referral
This otherwise healthy patient presents with leukocoria, or a white pupillary reflex, and requires an urgent referral to ophthalmology for further evaluation given the concern for retinoblastoma. The differential diagnosis for leukocoria includes retinoblastoma, cataract, vitreous hemorrhage, retinal detachment, and other intraocular/systemic disease processes. Newly diagnosed leukocoria should be urgently referred to ophthalmology to exclude retinoblastoma. Retinoblastoma is the most common intraocular cancer of childhood caused by mutations in the RB1 tumor suppressor gene that encodes the retinoblastoma protein. Retinoblastoma typically presents as leukocoria in a child under the age of 3 with other signs including strabismus, nystagmus, or a red, inflamed eye. Treatment options include chemotherapy, laser photocoagulation, radiation, cryotherapy, and enucleation. Prompt diagnosis and treatment are important for optimal visual outcome and survival. Francis et al. study the risk of extraocular extension in eyes with retinoblastoma that received intravitreous chemotherapy injections. The authors find that the risk of extraocular extension is small. The authors recommend the regular use of precautionary injection methods including lowering of intraocular pressure, cryotherapy, ocular surface irrigation, and ultrasonic biomicroscopy surveillance of the injection site. Figure/Illustration A shows a child with leukocoria (blue circle) in the left eye. Incorrect Answers: Answer A: Biopsy of the eye is contraindicated in retinoblastoma due to the risk of tumor seeding. Answer B: Genetic testing can be used to determine if a patient has the hereditary form of retinoblastoma, characterized by germline RB1 mutations, as opposed to the nonheritable form, which is associated with somatic RB1 mutations. An ophthalmological evaluation is required first to confirm the diagnosis. Answer C: While magnetic resonance imaging of the brain and orbits should be performed in all children with suspected retinoblastoma to assess disease extent, an ophthalmological evaluation should be completed first. Answer E: Reassurance or reevaluation at the next well-child visit is not appropriate management of newly diagnosed leukocoria given that undiagnosed retinoblastoma can be vision- and life-threatening. Bullet Summary: Leukocoria, or a white pupillary reflex, in a child requires urgent ophthalmologic referral to rule out retinoblastoma.
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A 17-year-old boy presents to the emergency department after a car accident. He drove his car into a pole while speeding at night. He has no past medical history and takes no medications. The patient is unable to offer a history and repetitively asks the nurses what happened. He knows his name and address and is able to remember other information about himself. He is complaining of a headache. His temperature is 97.6°F (36.4°C), blood pressure is 122/74 mmHg, pulse is 90/min, respirations are 12/min, and oxygen saturation is 98% of room air. Physical exam reveals a confused young man. Cranial nerves 2-12 are intact and the patient has normal strength and sensation. He demonstrates a stable gait. Throughout the exam, he continuously asks what happened to him. A head CT scan is ordered. Which of the following is the most likely diagnosis?
Cerebral contusion
Concussion
Diffuse axonal injury
Epidural hematoma
Subarachnoid hemorrhage
B
Concussion
This patient is presenting after a motor vehicle accident with confusion, repeated questioning, and a normal neurologic exam aside from his inability to remember the events that occurred. This constellation of symptoms is concerning for a concussion. A concussion occurs after blunt trauma to the head and is common in athletics and motor vehicle collisions. Patients may present initially with symptoms including confusion, repeat questioning, repeating themselves, and amnesia surrounding the event. A thorough neurological exam is important as there should be no neurologic deficits in a concussion. Patients with a classic history and reassuring exam require rest from physical and cognitive activity. Post-concussive syndrome may present with chronic headaches, dizziness, trouble sleeping, trouble focusing, nausea, and difficulties with learning and concentrating. Mood changes such as depression and anxiety are common. Any patient with a concerning mechanism or symptoms should have a head CT to rule out other more severe diagnoses. Pertab et al. review concussion and its presentation. They note that concussion can cause many symptoms including trouble focusing, mood changes, headaches, and also autonomic nervous system anomalies. They recommend reviewing the constellation of symptoms to make the diagnosis. Incorrect Answers: Answer A: Cerebral contusions are common after head trauma and may present with confusion, obtundation, neurologic deficits, and amnesia. While a severe concussion and a cerebral contusion have overlap, given this patient’s age, mechanism, and symptoms, concussion is more likely. A head CT would demonstrate contusion (hyperdense intraparenchymal lesion) with a possible contrecoup injury. Answer C: Diffuse axonal injury occurs after a severe CNS injury with an obtunded patient who is typically unresponsive. CT imaging will show blurring of the gray-white interface with punctate hemorrhages. These patients have a very poor prognosis. They often are obtunded with minimal neurologic function. Answer D: Epidural hematoma presents with trauma and the classic “talk and die” syndrome including a lucid interval followed by progressive obtundation and fixed, dilated pupils. Symptoms are rapidly progressive as this is an arterial bleed. CT scan would demonstrate a hyperdense lens-shaped lesion (composed of blood). A burr hole may relieve pressure and can be life-saving. Answer E: Subarachnoid hemorrhage may present after trauma with a severe headache and meningeal signs. Patients may demonstrate focal neurologic deficits in severe cases. CT scan will demonstrate blood in the subarachnoid space and lumbar puncture would demonstrate blood in the CSF. A CTA may also localize the site of bleeding and if any aneurysm is present. Bullet Summary: A severe concussion can present with confusion, repeat questioning, and amnesia.
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A 47-year-old man presents to the emergency department with a headache. He suddenly developed a throbbing, bitemporal headache about 5 hours ago "out of nowhere." He has a history of migraine headaches, but he feels that this headache is significantly more painful than his typical migraines. The patient took his prescribed sumatriptan with no relief of his symptoms. He also endorses nausea and he reports that he vomited once before arrival in the emergency department. The patient denies any recent trauma to the head. His medical history is significant for migraines and hypertension. He has a 20-pack-year smoking history and a history of cocaine use. He drinks 5-6 beers per week. His temperature is 98.6°F (37°C), blood pressure is 147/91 mmHg, pulse is 62/min, and respirations are 12/min. On physical exam, he appears to be in moderate distress and has pain with neck flexion. He has no focal neurologic deficits. A head CT is performed and can be seen in Figure A. This patient’s condition affects the space in between which of the following brain layers?
Arachnoid mater and pia mater
Dura mater and arachnoid mater
Periosteum and galea aponeurosis
Periosteum and skull
Skull and dura mater
A
Arachnoid mater and pia mater
This patient presents with a sudden, severe headache and a head CT showing bleeding in the subarachnoid space, which confirms a diagnosis of subarachnoid hemorrhage. The bleeding in a subarachnoid hemorrhage occurs between the arachnoid and pia mater. Subarachnoid hemorrhages present with a sudden, severe headache referred to as a “thunderclap” headache. Patients typically complain that the headache is the "worst headache of their life." Some patients also present with symptoms of meningeal irritation such as nausea and vomiting, nuchal rigidity, or focal neurologic deficits. Although most cases of subarachnoid hemorrhage are secondary to trauma, patients with risk factors such as hypertension and cocaine use can have non-traumatic presentations. Patients with an expanding bleed or acute neurologic changes require surgical clipping or embolization of the bleeding vessels. Macdonald and Schweizer review the evidence regarding the treatment of patients with subarachnoid hemorrhage. They discuss how survival rates have improved with early aneurysm repair, prescription of nimodipine, and advanced intensive care support. They recommend early diagnosis in order to improve outcomes in these patients. Figure/Illustration A is an axial slice of a head CT showing hyperintensity in the basal cisterns (red circle). This finding is consistent with bleeding in the subarachnoid space. Incorrect Answers: Answer B: Bleeding between the dura and arachnoid mater describes a subdural hematoma. A subdural hematoma presents as a headache with progressive confusion. It appears as a crescent-shaped lesion on head CT. Patients with an expanding bleed will require surgical decompression to avoid herniation syndromes. Answer C: Bleeding between the periosteum and galea aponeurosis describes a subgaleal hemorrhage. Subgaleal hemorrhages occur in newborns and are caused by vacuum-assisted deliveries. Treatment is usually supportive as the bleeding will resolve by itself. Some patients with severe bleeds may require transfusion due to excessive blood loss. Answer D: Bleeding between the periosteum and skull describes a cephalohematoma. Cephalohematomas occur in newborns and are caused by rupture of the blood vessels crossing the periosteum. They are usually secondary to a prolonged second stage of labor. Treatment is supportive as the blood will resolve and be resorbed. Answer E: Bleeding between the skull and dura mater describes an epidural hematoma. An epidural hematoma presents as a brief loss of consciousness followed by a lucid interval and then progressive confusion or somnolence. It appears as a biconvex lesion on head CT. Treatment is with urgent surgical decompression as patients will rapidly decompensate after the lucid interval. Bullet Summary: Subarachnoid hemorrhage classically presents as a thunderclap headache with a brief loss of consciousness and meningismus.
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An 82-year-old man with a history of atrial fibrillation presents to the emergency room complaining of sudden-onset, painless vision loss in his right eye starting 2 days ago. When it started, he felt like a “curtain was pulled down” over his right eye but wasn't able to seek care immediately because it was snowing outside and he didn't want to travel in inclement weather. A review of systems is significant for occasional palpitations and presyncope. The patient’s medications include aspirin and carvedilol. He reports that he is a smoker with a 50-pack-year history. The patient’s temperature is 99°F (37.2°C), blood pressure is 112/72 mmHg, pulse is 84/min, and respirations are 14/min with an oxygen saturation of 97% on room air. Physical exam reveals 20/800 vision in the right eye. A fundoscopic exam of the right eye is shown in Figure A. Physical and fundoscopic examinations of the left eye are unremarkable. Which of the following is the most appropriate next step in management?
Administer ranibizumab
Administer tissue plasma activator
Continue aspirin and start statin therapy
Start retinal photocoagulation
Start timolol drops
C
Continue aspirin and start statin therapy
This patient with a history of atrial fibrillation is presenting with acute-onset, painless, monocular vision loss and a fundoscopic exam positive for retinal whitening. This suggests a diagnosis of central retinal artery occlusion and management should focus on control of underlying risk factors given that he is 2 days out from the event. Symptoms of central retinal artery occlusion (CRAO) include sudden, painless, complete, and normally unilateral vision loss. A fundoscopic exam can show pallor of the optic disc, diffuse ischemic whitening of the retina, cherry-red macula, retinal edema, and constricted/bloodless retinal arterioles. The center of the macula appears red because the photoreceptor layer at the fovea is perfused by the posterior choroidal vasculature rather than the central retinal artery. Treatment involves surgical decompression of the retinal anterior chamber, ideally within 1 hour of the occlusion. Tissue plasminogen activator should be administered if the patient presents within an acceptable time frame. If surgical intervention is not readily available, additional steps with minimal evidence to support their use include ocular massage (to dislodge the embolus) and either carbogen therapy or hyperbaric oxygen. Chronic management includes control of underlying risk factors with aspirin and statin therapy. Dattilo et al. review the evidence regarding the treatment of central retinal artery occlusion. They discuss how treatments have not been particularly effective in the treatment of this disorder. They recommend engaging in secondary prevention of vascular events by controlling risk factors. Figure/Illustration A is a fundoscopic photograph showing retinal pallor surrounding the superior temporal arcade (red circle). This finding is characteristically seen in central retinal artery occlusion. Incorrect Answers: Answer A: Administering ranibizumab is not appropriate because this medication is an anti-VEGF antibody, which can be used to treat wet age-related macular degeneration. Symptoms of wet age-related macular degeneration include acute visual distortion and loss of central vision that occurs over months. A fundoscopic exam would show neovascularization. Answer B: Administration of tissue plasma activator has not been shown to be beneficial in central retinal artery occlusion if the event has occurred several days ago. This medication is used in patients who sustain an ischemic stroke within 4.5 hours of developing symptoms. A stroke in the posterior lobe can present with cortical hemianopsia. Answer D: Retinal photocoagulation is used to treat diabetic retinopathy. In diabetic retinopathy, symptoms of painless vision loss present gradually. A retinal exam would show retinal hemorrhage and possibly neovascularization. Answer E: Timolol is a beta-blocker used to treat glaucoma. Assessment of the optic disk would show an enlarged cup-to-disk ratio. Glaucoma can present with pain and pressure in the affected eye. Acute angle closure glaucoma would present with a rock-hard eye and may lead to permanent vision loss if not treated promptly. Bullet Summary: Chronic management of central retinal artery occlusion involves control of underlying risk factors with aspirin and statin therapy.
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A 34-year-old nulliparous woman presents to the clinic with 2 weeks of copper-colored discharge from her right nipple. Her breast has not been tender, and she has not noticed any changes to the skin. She is otherwise healthy and does not take any medications. Her temperature is 36.5°C (97.7°F), blood pressure is 110/82 mmHg, pulse is 68/min, respirations are 12/min, and oxygen saturation is 99% on room air. Exam of the right breast reveals no mass on palpation. Bloody discharge is expressed from the right nipple. There is no axillary lymphadenopathy. Which of the following is the most likely diagnosis?
Ductal carcinoma in situ
Intraductal papilloma
Invasive ductal carcinoma
Mammary duct ectasia
Paget disease of the breast
B
Intraductal papilloma
This young patient who presents with non-painful unilateral bloody nipple discharge and no palpable mass on breast exam most likely has an intraductal papilloma. Intraductal papilloma is a benign epithelial tumor that develops in the lactiferous ducts of the breast and is the most common cause of bloody nipple discharge in premenopausal women. Patients typically present with painless unilateral bloody nipple discharge, and often there is no mass palpable on breast exam. Ultrasound is the initial method for evaluation and may reveal an intraductal mass, but if results are inconclusive, further imaging with mammography is indicated. If the results of both still remain inconclusive, galactography (radiography after injecting radiopaque contrast into the duct system) is the most accurate imaging method for diagnosing intraductal papilloma. A core biopsy of the intraductal mass should be obtained, and if results show papilloma with atypia, treatment would be surgical excision of the involved duct to rule out a concomitant malignant neoplasm. Khan et al. review the treatment of intraductal papilloma and note that in lesions with core biopsy showing papilloma with atypia, surgical excision revealed the diagnosis of a concomitant invasive or ductal in situ cancer is common. The authors also find that in patients whose core biopsies showed papilloma without atypia, the likelihood of cancer was much lower. The authors recommend that since the diagnosis of intraductal papilloma carries an increased risk of breast cancer compared to the general population, these patients should be monitored accordingly. Incorrect Answers: Answer A: Ductal carcinoma in situ (DCIS) is a neoplastic lesion confined to the breast duct that if untreated develops into invasive ductal carcinoma. Typically, patients are asymptomatic, and lesions are detected via screening mammography. Though it can sometimes present with nipple discharge, this is less common. Unilateral painless bloody nipple discharge is more likely to be due to intraductal papilloma, though no mass may be palpable for either diagnosis. Answer C: Invasive ductal carcinoma is the most common type of breast cancer, which typically presents as a firm, immobile, painless lump in the breast, sometimes associated with nipple discharge, overlying skin changes, inverted nipple, and/or axillary lymphadenopathy. As with DCIS, nipple discharge may be present but is usually not bloody as in cases of intraductal papilloma. Answer D: Mammary duct ectasia is a benign condition in which a milk duct in the breast widens and its walls thicken, leading to blockage and build-up of fluid in the duct. This is usually asymptomatic but can present with off-white or greenish nipple discharge. It may also result in infection of the affected duct, leading to periductal mastitis and a tender, erythematous breast. It tends to occur in perimenopausal women and often resolves without treatment, though if symptoms persist it can be treated with antibiotics and duct excision. Answer E: Paget disease of the breast often presents as a scaly, raw, vesicular, or ulcerated lesion that begins on the nipple and then spreads to the areola, sometimes associated with bloody nipple discharge. Pain, burning, and pruritis are also common presenting symptoms. There is often an underlying breast carcinoma; thus, any patient with Paget disease should be evaluated with a mammogram. Although this patient has bloody nipple discharge, she does not present with a painful, scaly, raw, or vesiculated lesion that would be concerning for Paget disease of the breast. Bullet Summary: Intraductal papilloma often manifests in patients as painless unilateral bloody nipple discharge, and typically no mass is palpable on exam.
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A 21-year-old man presents to the emergency room with right lower quadrant pain that started this afternoon at his wrestling match. He also endorses loss of appetite and subjective fever. He has had vague, mild discomfort in the right upper quadrant since the start of this wrestling season. He endorses a history of unprotected sex with multiple partners and intravenous drug use during a recent vacation to Mexico but has no other significant medical history. He drinks socially and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 119/72 mmHg, pulse is 102/min, and respirations are 12/min. Physical exam demonstrates pain on palpation of the right lower quadrant and mild pain in the right upper quadrant. He has significant acne on his face, chest, and back. A CT of the abdomen confirms appendicitis. The scan also shows a large liver mass measuring 6 cm as seen in Figure A. Aside from addressing his appendicitis, which of the following is the most appropriate next step in management?
Administer metronidazole
Administer sofosbuvir
Obtain follow-up CT of the abdomen in 3 months
Perform liver mass biopsy
Perform liver mass resection
E
Perform liver mass resection
This patient with a history of possible anabolic steroid use (wrestler with acne) who presents with right upper quadrant pain and an enhancing lesion in the liver most likely has a hepatic adenoma. The treatment of hepatic adenomas is surgical removal and discontinuation of precipitating agents. Hepatic adenomas are benign epithelial tumors that typically arise in the right hepatic lobe. They are typically solitary masses but multiple lesions can be seen in patients with prolonged contraceptive or anabolic steroid use and those who have glycogen storage disorders. Patients present with abdominal pain that is localized in the epigastrium or in the right upper quadrant. Sudden, severe abdominal pain with hypotension suggests rupture of the hepatic adenoma into the peritoneum. The lesions can be confirmed on imaging such as ultrasound, CT, or MRI. For hepatic adenomas that are < 5 cm and asymptomatic, simply discontinuing the causative drug and observing are acceptable. Large or symptomatic lesions requiring surgical resection in addition to discontinuation of the offending agent. Because these tumors are highly vascular, failure to resect may result in rupture, hemoperitoneum, and shock. Biopsy is contraindicated to avoid rupturing the mass and causing hemorrhage. Klompenhouwer et al. studied the current management strategies in patients with hepatocellular adenoma. They found that various molecular groups have been identified allowing for subtyping of these lesions. They recommend performing a CT scan to better characterize these lesions. Figure/Illustration A is a CT with an enhancing mass with a hypoattenuating fatty component. This is characteristically seen in large hepatic adenomas, which are solitary well-marginated lesions. Incorrect Answers: Answer A: Metronidazole would be an effective treatment if this patient had contracted Entamoeba histolytica from contaminated food or water on his recent trip to Mexico. While this amoeba can lead to a liver abscess presenting as a mass on CT, it often also causes abdominal pain and dysentery due to intestinal involvement. Answer B: Sofosbuvir is an antiviral medication used for hepatitis C. This patient does have risk factors for hepatitis C (risky sexual behavior and intravenous drug use). Although hepatitis C could lead to hepatocellular carcinoma and cause a liver mass, this patient’s presentation is not consistent with that diagnosis. Even if he did have hepatocellular carcinoma, treating hepatitis C alone without surgical resection or adjuvant therapies would be insufficient. Answer C: Follow-up CT in 3 months would be dangerous for this man with a large hepatic adenoma. In the interim, the mass will likely continue to grow if he continues to use anabolic steroids, increasing the risk of rupture. Observation and repeat imaging may be suitable for asymptomatic hepatic lesions that are of uncertain but likely benign etiology. Answer D: Biopsying the liver mass is appropriate if malignancy is suspected and there is no concern for hepatic adenoma. If an adenoma is on the differential, biopsy is contraindicated due to the risk of rupture and hemorrhage intraoperatively. Bullet Summary: Hepatic adenomas are benign, vascular liver masses associated with oral contraceptives and anabolic steroids that should be promptly resected if large and symptomatic.
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A 19-year-old man presents to his primary care physician. He is asymptomatic and has no acute complaints, has no medical problems, and takes no medications. He was born in the United States but moved to El Salvador at age 11 and has only recently moved back to the United States in the past year. He was up to date on his vaccinations prior to his move to El Salvador. He last received a diphtheria, tetanus, and acellular pertussis (DTaP) vaccine 14 years ago at age 5, the second dose of a meningococcal vaccine 3 years ago, and an influenza vaccine 2 months ago. The patient’s temperature is 98.2°F (36.8°C), blood pressure is 120/76 mmHg, pulse is 82/min, and respirations are 16/min. There are no rashes noted on physical exam. Which of the following vaccine regimens is indicated in this patient?
Influenza
Tetanus and diphtheria
Tetanus, diphtheria, acellular pertussis, and influenza
Tetanus, diphtheria, and acellular pertussis
Tetanus, diphtheria, and influenza
D
Tetanus, diphtheria, and acellular pertussis
This patient has recently moved back to the United States after living in another country for several years and is behind on his tetanus, diphtheria, and pertussis vaccine series. The tetanus, diphtheria, and acellular pertussis (Tdap) vaccine is indicated at least once in adults who have not previously received Tdap. The standard vaccination schedule for tetanus, diphtheria, and pertussis includes a 5-dose childhood vaccine series consisting of DTaP at ages 2 months, 4 months, 6 months, 15-18 months, and 4-6 years. The first booster dose then consists of Tdap at age 11-12, with a tetanus and diphtheria (Td) vaccine every 10 years afterwards. In adults who have not previously received Tdap, a single Tdap dose should be administered regardless of time interval since the last dose of Td. Subsequent Td booster doses should then be given at 10-year intervals. Contraindications to Tdap include prior anaphylaxis after a tetanus, diphtheria, or pertussis vaccine or a history of seizures or encephalopathy within 7 days of receiving a previous pertussis-containing vaccine. Kerr et al. studied the risk of major birth defects after administering the Tdap vaccine in pregnant patients. The authors found no increase in the risk of malformations in the offspring of pregnant patients that received the Tdap vaccine. The authors recommend the administration of Tdap during pregnancy. Incorrect Answers: Answer A: Influenza is incorrect because the influenza vaccine is recommended only once per flu season. This patient already received the flu vaccine during this flu season, so the influenza vaccine is not indicated. Answer B: Tetanus and diphtheria only are incorrect because the Td vaccine booster is indicated every 10 years in adults who have already received a prior dose of Tdap. In adults who have never received a previous Tdap dose, Tdap is indicated unless contraindications exist. Answer C: Tetanus, diphtheria, acellular pertussis, and influenza are incorrect because influenza vaccine is not indicated in this patient. This patient has already received a dose of the influenza vaccine during this flu season. Answer E: Tetanus, diphtheria, and influenza are incorrect because neither the Td nor influenza vaccines are indicated in this patient. The Td vaccine is indicated every 10 years in adults who have already received a prior dose of Tdap. The influenza vaccine is only indicated once per flu season. Bullet Summary: The tetanus, diphtheria, and acellular pertussis (Tdap) vaccine is usually administered to adolescents at age 11-12 and is indicated at least once in adults who have not previously received Tdap.
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A 7-week-old boy presents to the pediatrician for vomiting. Three weeks ago, the patient began vomiting after meals. The vomitus appears to be normal stomach contents without streaks of red or green. His parents have already tried repositioning him during mealtimes and switching his formula to eliminate cow’s milk and soy. Despite these adjustments, the vomiting has become more frequent and forceful. The patient is voiding about 4 times per day and his urine appears dark yellow. He has fallen 1 standard deviation off of the growth curve. The patient's mother reports that the pregnancy was uncomplicated other than an episode of sinusitis in the 3rd trimester, for which she was treated with azithromycin. The patient's temperature is 98.7°F (37.1°C), blood pressure is 58/41 mmHg, pulse is 166/min, and respirations are 16/min. On physical exam, the patient looks small for his age. His abdomen is soft, non-tender, and non-distended. Which of the following is the most appropriate next step in management?
Abdominal ultrasound
Intravenous hydration
MRI of the head
Pyloromyotomy
Thickening feeds
B
Intravenous hydration
This patient presents with nonbilious, forceful vomiting, poor weight gain, signs of dehydration, and a history of exposure to azithromycin, which suggests a diagnosis of pyloric stenosis. The most appropriate next step in management is intravenous hydration. Pyloric stenosis classically presents in a male infant between 4 to 6 weeks of age with nonbilious, projectile vomiting after meals. Risk factors for pyloric stenosis include bottle feeding and exposure to macrolide antibiotics in the patient or mother. Protracted vomiting frequently leads to hypochloremic, hypokalemic metabolic alkalosis. Patients should be resuscitated with intravenous hydration and evaluated for electrolyte abnormalities that require repletion. After a patient is adequately resuscitated, patients can undergo an ultrasound to confirm the diagnosis and then be treated surgically for definitive therapy. 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 ultrasound is the test of choice to diagnose pyloric stenosis, but evaluation for electrolyte derangements and subsequent intravenous hydration and electrolyte repletion should be initiated first in this patient with signs of dehydration and malnutrition. Further evaluation is reserved after a patient is adequately resuscitated. Answer B: MRI of the head would be appropriate if this patient had additional signs of a mass in the head, such as neurological changes or worsening of the vomiting in the morning. In the absence of these signs and the presence of a mass in the epigastrium, this patient is more likely to have pyloric stenosis. Answer C: Pyloromyotomy is the definitive management of pyloric stenosis, but this patient should undergo fluid replacement and have any electrolyte derangements detected and managed before undergoing surgery. Surgical intervention can carry significant risks if patients are not adequately resuscitated. Answer E: Thickening feeds are one of the techniques used to manage gastroesophageal reflux. In the setting of this patient’s epigastric mass and clinical picture, pyloric stenosis is the more likely etiology of his vomiting. Reflex presents with non-projectile vomiting that is dependent on position. Bullet Summary: Patients with pyloric stenosis should first undergo intravenous rehydration and electrolyte repletion, as protracted vomiting may lead to severe hypochloremic, hypokalemic metabolic alkalosis.
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A 34-year-old man presents to the emergency room with pain behind his eyes. He had a similar episode about 1 month ago which resolved after a few hours. The pain feels like a stabbing sensation behind his right eye. He denies any symptoms on the left side. The last time he had an episode like this, his eye was tearing. His temperature is 98.2°F (36.8°C), blood pressure is 120/90 mmHg, pulse is 110/min, and respirations are 12/min. Physical exam reveals a 2 mm pupil on the right and a 5 mm pupil on the left. Extraocular movements are intact bilaterally. The remainder of the neurologic exam is unremarkable. Which of the following is the most appropriate prophylactic treatment?
Ibuprofen
Oxygen
Propranolol
Sumatriptan
Verapamil
E
Verapamil
This patient with a history of retro-orbital pain, unilateral miosis (2 mm pupil on the right), and history of lacrimation during a similar episode is likely experiencing cluster headaches. Verapamil may be used as prophylaxis in the treatment of cluster headaches. Cluster headaches occur more commonly in men and present with severe, unilateral, retro-orbital pain. Typical episodes last 15 minutes to 3 hours. Attacks may be associated with the circadian rhythm, as patients may notice that they get headaches at night. Because of the extreme pain, cluster headaches are sometimes colloquially referred to as "suicide" headaches. These headaches may present with autonomic symptoms, such as lacrimation and rhinorrhea. Horner syndrome may also occur, defined by a unilateral triad of ptosis, miosis, and anhidrosis. Acute management of cluster headaches includes administration of 100% oxygen. Other options include triptans. The first-line medication for prophylaxis of cluster headaches is verapamil. Studies have shown that lithium, topiramate, and ergotamine may also be effective as prophylaxis for cluster headaches; however, these therapies are less established than verapamil. Blau and Engel review the use of medications for cluster headaches. They note that most of patients with episodic cluster headaches had complete suppression of attacks with the use of verapamil. It is recommended to give verapamil in this patient population. Incorrect Answers: Answer A: Ibuprofen is a non-steroidal anti-inflammatory drug (NSAID) that is used in the treatment of migraine and tension headaches. Migraine headaches present with unilateral, pulsating pain with photophobia and phonophobia, whereas tension headaches present with constant bilateral pain. Ibuprofen should not be used as prophylaxis for any type of headache as it has side effects including kidney dysfunction, ulcers, and even premature aging. Answer B: Oxygen is first-line treatment for acute cluster headaches; however, it does not have any role in the prophylaxis of cluster headaches. Answer C: Propranolol is a beta-blocker that is a prophylactic medication for migraine headaches. Migraines are more common in women and typically present as a unilateral headache with photophobia or phonophobia. They may also be associated with an aura, which presents as a visual, auditory, olfactory, or motor disturbance. Answer D: Sumatriptan is a triptan that is used as an abortive treatment for migraine headaches and cluster headaches. It should not be used as prophylaxis for headaches. Bullet Summary: Verapamil may be used as prophylaxis for cluster headaches.
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A 65-year-old man presents to his primary care physician for a routine appointment. He has no concerns. His past medical history is significant for human immunodeficiency virus (HIV) infection, diagnosed 15 years ago. The patient is intermittently compliant with his antiretroviral therapy (ART). His temperature is 99.0°F (37.2°C), blood pressure is 130/84 mmHg, pulse is 92/min, and respirations are 11/min. His most recent lab work showed a cluster of differentiation CD4 count of 150 cells/µL. He received a dose of the 13-valent pneumococcal vaccine and a dose of the 23-valent pneumococcal vaccine 15 years ago, as well as a second dose of the 23-valent pneumococcal vaccine 10 years ago. In addition to encouraging greater compliance with his ART, which of the following is indicated in this patient?
Azithromycin and pneumococcal vaccine
Azithromycin and zoster vaccine
Trimethoprim-sulfamethoxazole and pneumococcal vaccine
Trimethoprim-sulfamethoxazole and varicella vaccine
Trimethoprim-sulfamethoxazole and zoster vaccine
C
Trimethoprim-sulfamethoxazole and pneumococcal vaccine
This patient with HIV and a CD4 count of 150 cells/μL requires trimethoprim-sulfamethoxazole (TMP-SMX) prophylaxis and another dose of the 23-valent pneumococcal vaccine. All HIV patients with a CD4 count < 200 cells/μL require TMP-SMX as prophylaxis against Pneumocystis jirovecci pneumonia. Patients with CD4 counts < 100 cells/μL also require TMP-SMX as prophylaxis against toxoplasmosis. HIV patients require a dose of the 23-valent pneumococcal vaccine at age 65. Patients should initially receive the 13-valent pneumococcal vaccine, followed by the 23-valent pneumococcal vaccine 8 weeks later, 5 years later, and at age 65. Compliance with ART is also critical in patients with HIV. Kaplan et al. discuss recommendations for the prevention of opportunistic infections in HIV-infected adults. The authors find that daily prophylaxis with TMP-SMX is an appropriate regimen for the prevention of Pneumocystis pneumonia. The authors recommend dapsone, atovaquone, or pentamidine for patients that cannot receive TMP-SMX. Incorrect Answers: Answer A: Azithromycin is used as prophylaxis against Mycobacterium avium complex (MAC) in patients with a CD4 count < 50 cells/μL that has not been started on ART. Since this patient has a CD4 count of 150 cells/μL, azithromycin is not indicated at this time. Answer B: Azithromycin is used as prophylaxis against Mycobacterium avium complex (MAC) in patients with a CD4 count < 50 cells/μL that has not been started on ART. This patient should not receive the zoster vaccine as it is a live vaccine and is contraindicated in immunocompromised patients. Answer D: Although this patient does require TMP-SMX as prophylaxis against Pneumocystis pneumonia, he should not receive the varicella vaccine as it is a live vaccine and therefore contraindicated in immunocompromised patients. Answer E: Although this patient does require TMP-SMX as prophylaxis against Pneumocystis pneumonia, he should not receive the zoster vaccine as it is a live vaccine and therefore contraindicated in immunocompromised patients. Bullet Summary: Patients with a CD4 count < 200 cells/μL require prophylaxis with TMP-SMX and all patients with HIV should receive a dose of the 23-valent pneumococcal vaccine at age 65.
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A 55-year-old woman is brought to the emergency department by her husband with a 1 hour history of an unremitting headache. The headache started suddenly while she was eating dinner and she says it feels like the “worst headache of my life.” An emergent CT scan of the head without contrast confirms the diagnosis, and a CT angiogram identifies the source of bleeding. The patient undergoes surgical management of her condition. On hospital day 3, she is found to be disoriented to person, place, and time. She also develops nausea and vomiting. Her medical problems consist of heart failure for which she takes furosemide, spironolactone, and metoprolol, which were continued at admission. Her temperature is 99.6°F (37.6°C), blood pressure is 100/60 mmHg, pulse is 112/min, and respirations are 16/min. Physical examination shows poor skin turgor. Capillary refill time is 4 seconds. Serum laboratory results are shown below: Na+: 120 mEq/L Cl-: 92 mEq/L K+: 3.9 mEq/L HCO3-: 26 mEq/L BUN: 32 mg/dL Creatinine: 1.0 mg/dL Serum osmolality is 265 mEq/L and urine osmolality is 340 mEq/L. Urine sodium is 44 mEq/L. Which of the following is the most likely diagnosis?
Addison disease
Cerebral salt wasting
Diuretic overuse
Primary polydipsia
Syndrome of inappropriate anti-diuretic hormone
B
Cerebral salt wasting
This patient presents with a subarachnoid hemorrhage (“worst headache of my life”) which is treated and then subsequently develops nausea, vomiting, and altered mental status with reduced serum sodium concentration, indicative of symptomatic hyponatremia. In the setting of serum hypo-osmolality, urine hyper-osmolality, urine sodium concentration > 20 mEq/L, and hypovolemia (low blood pressure, decreased skin turgor, and prolonged capillary refill), this is indicative of cerebral salt wasting. The mechanism of cerebral salt wasting is unclear, although some hypotheses point to increased release of brain natriuretic peptide after injury leading to suppression of renal sodium reabsorption, or sympathetic nervous system injury resulting in an impaired renin response. Patients classically present several days after cerebral injury or neurosurgery with hypovolemic hyponatremia and hypochloremia. Water retention results in serum hypo-osmolality, hyponatremia, and hypochloremia with urine hyper-osmolality and increased urine sodium excretion. In patients with prolonged cerebral salt wasting, hypokalemia can also occur (potassium may be normal in acute states). Treatment in mild or moderate cases consists of normal saline infusion to correct both volume status and hyponatremia. Treatment in severe cases consists of hypertonic saline. During correction, serum sodium must be monitored closely to prevent central pontine myelinolysis. Cui et al. describe the differences between syndrome of inappropriate anti-diuretic hormone (SIADH) and cerebral salt wasting. They discuss how volume status serves as the key differentiator between these conditions. They recommend paying closer attention to total body electrolyte balance in understanding these conditions. Incorrect Answers: Answer A: Addison disease, also known as primary adrenal insufficiency, results in salt wasting due to decreased mineralocorticoid activity. Because of reduced aldosterone release, patients also have hyperkalemia and metabolic acidosis. In contrast, this patient’s potassium and bicarbonate levels are normal. Treatment is with steroids. Answer C: Diuretic overuse is occasionally observed in patients using loop diuretics. Overuse of loop diuretics can cause hyponatremia, hypokalemia, and hypochloremia. Metabolic alkalosis is commonly seen due to volume contraction. Cerebral salt wasting is more likely in this patient presenting after a recent cerebral injury. Treatment is the cessation of the causative medication. Answer D: Primary polydipsia can cause symptomatic hyponatremia. The urine would be maximally diluted (urine osmolality < 100 mEq/L) due to an appropriate physiological response to hyponatremia and excess free water intake. Treatment is cessation of excessive water consumption. Answer E: Syndrome of inappropriate anti-diuretic hormone (SIADH) presents with identical findings as cerebral salt wasting with the exception of euvolemia as opposed to hypovolemia. An assessment of volume status is essential to differentiate. This patient has clear signs of hypovolemia. Treatment of SIADH is fluid restriction followed by ADH inhibitors, if needed. Bullet Summary: Cerebral salt wasting presents with hyponatremia, hypochloremia, and hypovolemia in the setting of recent cerebral injury or neurosurgery.
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A 23-year-old woman presents to labor and delivery at 40 weeks gestation with abdominal contractions and leakage of fluid from her vagina. She subsequently undergoes an uncomplicated vaginal delivery. The child is healthy and is heated, suctioned, and stimulated. While holding her newborn, the mother endorses a headache, blurry vision, and abdominal pain. Her temperature is 98.0°F (36.7°C), blood pressure is 194/104 mmHg, pulse is 100/min, respirations are 18/min, and oxygen saturation is 98% on room air. Physical exam reveals an uncomfortable woman. There is blood in her vagina and her uterus is contracted. Her cranial nerve exam is unremarkable and she has normal strength and sensation. Laboratory studies and a urinalysis are pending. Which of the following is the most appropriate next step in management?
CT head
Labetalol
Magnesium
MRV head
Ondansetron and morphine
C
Magnesium
This postpartum patient is presenting with hypertension, headache, blurry vision, and abdominal pain, which are concerning for a diagnosis of preeclampsia. The most appropriate initial intervention is magnesium. Preeclampsia classically presents after 20 weeks gestation (or earlier in the setting of a molar pregnancy) with hypertension and proteinuria. Other symptoms that may be indicative of preeclampsia with severe features include headaches, visual changes, and abdominal pain. Other features consistent with preeclampsia include thrombocytopenia, kidney insufficiency, impaired liver function, and pulmonary edema. The diagnosis can be supported when a pregnant patient is both hypertensive and demonstrates an elevated protein in her urine. It is possible for postpartum patients to experience preeclampsia. Though the fetus and placenta have been delivered, which is the definitive treatment of preeclampsia, further medical management is still needed. The most important initial step in management is to administer magnesium which is neuroprotective and also modestly lowers blood pressure. Further care involves blood pressure control and benzodiazepines as needed to abort seizure episodes (though note that if a patient with preeclampsia is having seizures, by definition, they have eclampsia). Al-Safi et al. discuss preeclampsia and eclampsia. They note that postpartum preeclampsia and eclampsia can occur within the first week after delivery. They recommend that mothers should be educated on what symptoms to look for. Incorrect Answers: Answer A: CT head may be indicated, in particular, if an intracranial hemorrhage was thought to be the cause of this patient’s headache and vision changes. A subarachnoid hemorrhage would present with the classic “thunderclap” headache with blood in the subarachnoid space on head CT. A CTA may localize a bleeding aneurysm and a lumbar puncture would show an elevated red blood cell count in the CSF. It would not explain this patient’s hypertension and abdominal pain. Note that a CT head is not a CTV which is specific venous imaging. Answer B: Labetalol is an appropriate blood pressure medication to give in pregnancy; however, it is more appropriate to first give magnesium as this agent is neuroprotective and is the first-line agent of choice in preeclampsia. Answer D: MRV head or a CTV head would be appropriate in the workup of cerebral venous thrombosis which is common in the setting of hypercoagulability (such as in the postpartum period) and presents with headache, blurry vision, and seizures. Note that it would not explain this patient’s profound hypertension. It would be more reasonable to first empirically treat this patient's possible preeclampsia. Answer E: Ondansetron and morphine would be appropriate symptomatic management of this patient but would not treat her underlying preeclampsia. It is more appropriate to first treat the life-threatening diagnosis before symptom management. If this patient’s hypertension was thought to be due to pain, then morphine could be helpful. Bullet Summary: Preeclampsia can occur in the postpartum period and should be managed with magnesium sulfate.
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A 57-year-old man is brought to the emergency department by his wife with confusion. He was seen in the emergency department 2 days ago for profuse epistaxis. His primary care provider was concerned and had advised him to go to the hospital, as the patient is on warfarin for atrial fibrillation. However, his bleeding resolved with pressure and nasal packing. Upon presentation to the ED today, his temperature is 97.6°F (36.4°C), blood pressure is 64/33 mmHg, pulse is 160/min, respirations are 32/min, and oxygen saturation is 100% on room air. Physical exam reveals an obtunded man who cannot answer questions. A dark, purpuric rash is noted on his torso and extremities. The patient’s nasal packing is removed with no further bleeding. The patient is given IV fluids and blood cultures are drawn. Which of the following should be administered first?
Clindamycin
Meropenem
Penicillin
Piperacillin-tazobactam
Vancomycin
A
Clindamycin
This patient is presenting with nasal packing, hypotension, tachycardia, a dark, purpuric rash, and confusion, which are concerning for toxic shock syndrome. The most important initial step in management is to administer clindamycin. Toxic shock syndrome is a life-threatening diagnosis caused by Streptococcus pyogenes or Staphylococcus aureus and occurs commonly secondary to retained tampons or nasal packing. Patients are critically ill and require immediate management with fluids, vasopressors, and blood cultures. Source control is imperative, and the foreign body causing the infection must be removed. Next, broad-spectrum antibiotics should be started immediately. Clindamycin is often preferred as the first agent as this lincosamide antibiotic not only covers Staphylococcus aureus and Streptococcus pyogenes, but also has the theoretical benefit of suppressing toxin production. Further management is centered on fluid administration, vasopressors, and monitoring and care in an ICU setting as these patients are typically critically ill. Russell and Pachorek review the treatments for toxic shock syndrome. They note that clindamycin is particularly important in toxic shock syndrome that is complicated by toxin production. They recommend that administration may reduce toxin production and could improve outcomes. Incorrect Answers: Answer B: Meropenem is a broad-spectrum antibiotic that covers most organisms except for MRSA. It is generally not used as a first-line agent unless the patient has allergies to other antibiotics or the infectious organism is only sensitive to this agent. Even in toxic shock syndrome, it would not be used as a first-line agent. Answer C: Penicillin may be used for infections such as streptococcal pharyngitis, which presents with a sore throat, fever, and tonsillar exudates. It is a relatively weaker antibiotic and would not be used in a critically ill patient with toxic shock syndrome. Answers 4 & 5: Piperacillin-tazobactam and vancomycin represent a powerful broad-spectrum regimen of antibiotics that covers gram-positives, gram-negatives, Pseudomonas aeruginosa, and anaerobes (piperacillin-tazobactam) as well as further gram-positive coverage and MRSA (vancomycin). Though this is a potent antibiotic combination, neither of these takes priority over clindamycin given the possible benefit of toxin suppression from clindamycin. In this patient, clindamycin and piperacillin-tazobactam would be an appropriate antibiotic regimen. Bullet Summary: Clindamycin may suppress toxin production and should be given early in toxic shock syndrome.
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A 69-year-old woman presents with pain in her hip and groin. The pain is present in the morning but is nearly unbearable by the end of the day. Her medical history is notable for a treated episode of acute renal failure, diabetes mellitus, obesity, and hypertension. Her current medications include losartan, metformin, insulin, and ibuprofen. The patient recently started taking high doses of vitamin D as she believes that it could help her symptoms. She also recently fell off the treadmill while exercising at the gym. Her temperature is 98.6°F (37°C), blood pressure is 135/91 mmHg, pulse is 72/min, and respirations are 12/min. On exam, an obese woman in no distress is noted. There is pain, decreased range of motion, and crepitus on exam of her right hip. The patient points to the areas that cause her pain stating that it is mostly over the groin. Which of the following is most likely to be found on plain radiographic imaging of this patient?
Femoral neck fracture
Hyperdense foci in the ureters
Loss of joint space and osteophytes
Normal radiography
Posterior displacement of the femoral head
C
Loss of joint space and osteophytes
This patient is presenting with pain worse on exertion, decreased range of motion, and crepitus in her hip, suggesting a diagnosis of osteoarthritis. A loss of joint space and osteophytes (reactive bone formation) would be seen on radiography. Osteoarthritis occurs due to wear and tear of the joint typically from repeat trauma in the setting of risk factors such as obesity. Pain can be located in the hip, and can be referred to the groin or knee. The classic presentation is pain that worsens with activity. Crepitus and decreased range of motion can be found on physical exam. Common radiological findings include a loss of joint space and osteophytes. Treatment should start with conservative measures such as activity modification, weight loss, and an unloading brace. A corticosteroid injection may be appropriate as a second-line measure. Refractory cases can be treated with a total joint replacement. Glyn-Jones et al. review the evidence regarding the diagnosis and treatment of osteoarthritis. They discuss how total joint arthroplasty operations can be limited by the lifestyle of the prosthesis and other complications. They recommend considering joint preserving options such as lifestyle modification. Incorrect Answers: Answer A: Femoral neck fracture would present acutely with pain in the hip and a shortened and externally rotated leg. This diagnosis is possible given the patient's recent fall; however, her other symptoms and less acute presentation suggest a diagnosis of osteoarthritis. Treatment is with a hemiarthroplasty. Answer B: Hyperdense foci in the ureters suggest a diagnosis of renal calculi. Though this patient's referred pain to the groin, history of renal failure, and possible hypercalcemia (due to increased vitamin D intake) could suggest this diagnosis, her other symptoms are more suggestive of osteoarthritis. Treatment is lithotripsy or stone removal for larger kidney stones. Answer D: Normal radiography would be found with a minor sprain/muscular injury which presents with a self-limited course of pain and tenderness over the affected muscle. Treatment is with rest, ice, compression, and elevation. Answer E: Posterior displacement of the femoral head suggests a diagnosis of posterior hip dislocation. Posterior hip dislocation would present with a shortened and internally rotated leg and a more acute presentation. Treatment is with closed reduction of the hip under sedation. Bullet Summary: Osteoarthritis presents with joint pain that is worse with exertion and radiographical findings of decreased joint space and osteophytes.
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A 7-year-old adopted boy presents to the emergency department after fainting. He just switched from being home-schooled to public school and joined the basketball team. He has fainted several times during basketball practices. He did not sustain any injuries or have any prodromes prior to these episodes. He is usually unconscious for less than a minute. He is otherwise healthy and has met all developmental milestones. He does not currently take any medications. His temperature is 97.6°F (36.4°C), blood pressure is 104/54 mmHg, pulse is 100/min, respirations are 19/min, and oxygen saturation is 98% on room air. Physical exam reveals a well-appearing and playful boy. An ECG is performed as seen in Figure A. An echocardiogram is ordered and pending. Which of the following is the most likely diagnosis?
Arrhythmogenic right ventricular dysplasia
Brugada syndrome
Hypertrophic obstructive cardiomyopathy
QT prolongation
Wolff Parkinson White syndrome
A
Arrhythmogenic right ventricular dysplasia
This adopted pediatric patient is presenting with recurrent syncope and an ECG demonstrating an epsilon wave and T wave inversions, which are concerning for arrhythmogenic right ventricular dysplasia. An epsilon wave is a small positive deflection at the end of the QRS complex. This is an ominous finding and suggests a diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) though it may also be seen in other infiltrative diseases such as sarcoidosis or after ischemic events. ARVD is an autosomal dominant condition where there is fatty infiltration of the right ventricular wall predisposing the patient (classically younger patients) to sudden cardiac death from lethal ventricular dysrhythmias. The epsilon wave is the most specific finding; however, other findings include T wave inversion in V1-V3 or a left bundle branch appearance with frequent premature ventricular complexes among other possibilities. This is a potentially lethal diagnosis and requires management with antiarrhythmics (such as beta-blockers or amiodarone) and the placement of an implantable cardioverter-defibrillator. Kayser et al. review AVRD. They note the presentation and management of ARVD and how it may present initially only with syncope. Cardiac arrest is a possible presentation as well. They recommend early detection and treatment with an implantable cardioverter-defibrillator to prevent death in this population. Figure/Illustration A is an ECG with an epsilon wave (red arrow) and inverted T waves in V1/V2 (blue arrows) concerning for ARVD. Incorrect Answers: Answer B: Brugada syndrome is an autosomal dominant mutation in cardiac ion channels that presents with a possible right bundle branch pattern with ST segment elevation in V1-V3 or ST coving in these same leads. It may present with syncope. Answer C: Hypertrophic obstructive cardiomyopathy presents with syncope, sudden death, or cardiac arrest in a young patient associated with exertion or dehydration. It can cause obstruction of the left ventricular outflow tract. A systolic murmur can be heard on cardiac auscultation and echocardiography can confirm the diagnosis. ECG will demonstrate large voltages in V1-V5. Answer D: QT prolongation can lead to torsades des pointes which presents with twisting of the QRS complexes around an isoelectric baseline. It may present with syncope, hemodynamic instability, or cardiac arrest. Medications, infection, and congenital conditions may prolong the QT interval. Answer E: Wolff Parkinson White syndrome presents with a delta wave on ECG or a slurred upstroke of the QRS complex. It may cause syncope and evolve into other dysrhythmias including supraventricular tachycardia and ventricular tachycardia. Bullet Summary: Arrhythmogenic right ventricular dysplasia (ARVD) may cause syncope or cardiac arrest, and presents on ECG with an epsilon wave.
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A 48-year-old woman, gravida 3 para 3, presents to the clinic with a 5-month history of intermittent loss of urine. Her urinary leaking occurs throughout the day and night, and she often has to get up to use the bathroom while asleep. Exercising does not affect the loss of urine. She states that she often cannot make it to the bathroom in time once she senses a need to void. Her last menstrual period was 2 weeks ago. Her urinalysis is unremarkable, and her postvoid residual volume is 40 mL. On pelvic examination, there is no dribbling of urine when the patient coughs. Physical exam is otherwise unremarkable. Which of the following is the most likely diagnosis?
Genitourinary syndrome of menopause
Mixed urinary incontinence
Overflow incontinence
Stress incontinence
Urge incontinence
E
Urge incontinence
This patient with a sudden need to void followed by loss of urine with urgency (unable to make it to the bathroom in time) most likely has urinary urge incontinence. Urge incontinence is a form of urinary incontinence characterized by a sudden urge to urinate, resulting in the involuntary leakage of urine. Normally, bladder contraction occurs due to the stimulation of muscarinic receptors in the detrusor muscle. Urinary urge incontinence is due to detrusor overactivity, leading to a sudden and frequently overwhelming need to void that occurs throughout the day and night. The most common cause is idiopathic, though it may be secondary to neurologic disorders (e.g., spinal cord injury or multiple sclerosis), bladder abnormalities, or chronic bladder inflammation. Workup is first centered on ruling out a urinary tract infection with a urinalysis. The diagnosis is made with urodynamic studies, and first-line treatment involves bladder training and pelvic floor exercises. Should first-line treatment of urge incontinence fail, antimuscarinic medications (such as oxybutynin) which reduce acetylcholine activity on muscarinic receptors can decrease detrusor contraction and thus the sense of urgency. Nandy and Ranganathan review urge incontinence. They note that it presents with leakage of urine associated with a sudden urge to urinate. They recommend an appropriate history and workup to first rule out other causes. Incorrect Answers Answer A: Genitourinary syndrome of menopause represents urinary symptoms (stress and/or urge incontinence) due to vaginal dryness and atrophy from menopause. This patient still has menstrual cycles and no symptoms of ovarian follicle depletion (narrowed introitus, vulvovaginal dryness and irritation, irregular vaginal bleeding), making this an unlikely diagnosis. Answer B: Mixed urinary incontinence presents with features of both stress and urge incontinence. This patient lacks symptoms of stress incontinence given a lack of incontinence with increases in intrabdominal pressure (such as exercise, coughing, laughing, or sneezing). Answer C: Overflow incontinence is caused by impaired detrusor muscle activity or bladder outlet obstruction. It presents with constant involuntary dribbling or urine and incomplete bladder emptying. Post-void residual volume is increased in patients with overflow incontinence. Answer D: Stress incontinence is caused by urethral hypermobility and otherwise insufficient urethral support. It presents with urinary leakage with activities that increase intraabdominal pressure (such as coughing, sneezing, laughing, and exercising). This patient’s symptoms are unrelated to exercise. Bullet Summary: Urge incontinence presents with sudden urinary urgency with a normal post-void residual volume and absence of findings consistent with urinary tract infection.
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A 51-year-old man presents for his annual wellness visit with his primary care physician. His wife has noticed a tremor while he is working on his car and knows that his father died of Parkinson disease. He says that the tremor is nothing to worry about and only occurs during a few activities such as pouring drinks, pointing the remote at the television, or fixing his car. The patient states his wife is just overreacting. His medical problems include hypertension, diabetes, and hyperlipidemia for which he takes lisinopril, metformin, and atorvastatin. He has 3-5 drinks of scotch per night before working on his car. On physical exam, the tremor is replicated during finger to nose testing. Romberg sign is negative. Cranial nerves II-XII are intact. The rest of the exam is benign. Which of the following is the most appropriate treatment for this patient's tremor?
Alcoholics anonymous
Aripiprazole
Carbidopa
Phenobarbital
Propranolol
E
Propranolol
This patient who presents with a tremor that only occurs during terminal activity and is likely improved by alcohol (hence why he drinks prior to working on the car) has benign essential tremor. This is best treated with propranolol. Benign essential tremor is a type of rhythmic oscillatory movement that occurs with posturing and action. The pathophysiology of this disorder is unclear; however, it classically gets worse with actions that require terminal positioning of the extremities such as pouring a drink or pointing a television remote. The tremor improves with alcohol consumption so patients will often self-medicate with alcoholic drinks. There is a strong genetic component, with an autosomal dominant pattern of inheritance with incomplete penetrance. The best method of replicating the tremor is with the finger-nose-finger test because this requires the patient to perform a precise activity at a terminal distance. The diagnosis is made clinically. Treatment is with propranolol or primidone, which are both considered first-line agents although the latter has less evidence for its efficacy. Paparella et al. studied the effects of propranolol on head and upper extremity movements in patients with benign essential tremors. They found that this medication has no effect on head movements but reduces the number of upper extremity tremors in these patients. They recommended that agents other than propranolol should be used for the treatment of head tremors in these patients. Incorrect Answers: Answer A: Alcoholics anonymous is the first-line treatment for patients with alcohol use disorder; however, the tremors that occur during alcohol withdrawal are not isolated to movements during terminal activities. Alcohol withdrawal also presents with other signs including restlessness, diaphoresis, and insomnia. More severe cases can present with hallucinations and seizures. The response of this patient's tremor to alcohol consumption is typical of patients with benign essential tremor. Answer B: Aripiprazole and other atypical antipsychotic medications can be used in patients with Tourette syndrome; however, these patients will present with involuntary tics rather than a tremor that only occurs during a voluntary activity at the terminal range of motion. Moreover, for the diagnosis of Tourette syndrome, both motor and phonic tics must be present. Answer C: Carbidopa can be used in the treatment of Parkinson disease; however, the tremor associated with this disease classically occurs at rest and is described in character as a "pill-rolling" tremor. The other features of Parkinson disease include bradykinesia or akinesia, rigidity, and postural instability. Answer D: Phenobarbital can be used in the treatment of Huntington disease; however, the involuntary movements seen in this disease are classically choreiform rather than intention tremor. Huntington disease presents with chorea, psychiatric illness, and depression. There is commonly a strong genetic predisposition, as it is a trinucleotide repeat disorder that exhibits anticipation. Bullet Summary: Propranolol can be used in the treatment of patients with benign essential tremor, which presents with an action tremor that is alleviated by alcohol use.
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A 26-year-old woman presents to the clinic with diarrhea, bloating, flatulence, and abdominal cramps. These symptoms have been going on for 2 months. She has lost 6 pounds (2.7 kg) over these 2 months. Her medical history is significant for mild intermittent asthma for which she uses an albuterol inhaler as needed. She is sexually active with 1 male partner. She recently returned from South India following a mission trip for 6 weeks. Her temperature is 98.6°F (37.0°C), blood pressure is 105/70 mmHg, pulse is 95/min, and respirations are 14/min. On examination, the patient’s skin is pale. Labs are obtained and show the following results: Leukocyte count: 4,500/mm^3 Hemoglobin: 10.5 g/dL Platelets: 110,000/mm^3 Mean corpuscular volume (MCV): 116 µm^3 Reticulocyte count: 0.5% A biopsy of the small bowel reveals blunting of villi and a mixed infiltration of lymphocytes, plasma cells, and eosinophils. Which of the following is the most likely diagnosis?
Celiac disease
Crohn disease
Giardia
Tropical sprue
Whipple disease
D
Tropical sprue
The patient is presenting with chronic diarrhea, weight loss, megaloblastic anemia (low hemoglobin and elevated MCV), and a biopsy showing blunted villi and mixed infiltrate following significant travel to South India. This is consistent with tropical sprue. Tropical sprue is characterized by chronic diarrhea that occurs after travel to an endemic area. It is thought to be due to a bacterial overgrowth/infection that then damages the intestinal mucosa. Endemic areas include the Caribbean, Southeast Asia, and South India. Patients present with chronic diarrhea, abdominal discomfort/cramps, progressive weight loss, and clinical features of malabsorption. Patients with tropical sprue can develop megaloblastic anemia (low hemoglobin and elevated MCV) from a deficiency in folate and vitamin B12 absorption. A small bowel biopsy would show blunting of the villi and infiltration of lymphocytes, plasma cells, and eosinophils. Microorganisms may also be seen. Treatment is an oral tetracycline and folic acid for 3-6 months. McCarroll et al. study the relationship between infectious gastroenteritis and tropical sprue. In a case-control study, the authors found that prior infectious gastroenteritis increased the risk of tropical sprue. The authors recommend treatment of suspected tropical sprue with oral folic acid replacement. Incorrect Answers: Answer A: Celiac disease can present similarly to tropical sprue with chronic diarrhea, abdominal pain, and weight loss. A small intestine biopsy can similarly show villous atrophy and a lymphocytic infiltrate, as well as crypt hyperplasia. Celiac disease is an immune response to gluten, while symptoms of tropical sprue are not diet-associated. The development of symptoms following significant travel (lasting at least several weeks) to an endemic country should raise suspicion for tropical sprue. Answer B: Crohn disease presents with chronic diarrhea, abdominal pain, weight loss, skip lesions anywhere from mouth to anus, extraintestinal manifestations, and signs of malabsorption. A biopsy will show transmural inflammation with noncaseating granulomas, giant cells, and aggregates of lymphocytes in the lamina propria. Answer C: Giardia is a parasite that is endemic in the tropics and North American mountain regions. Patients present with foul-smelling diarrhea, excessive gas, and nausea. Diagnosis can be confirmed with a stool analysis or immunoassay. Though a biopsy is normally not indicated, it would show mild lymphocytic infiltration and Giardia trophozoites on the intestinal surface. Answer E: Whipple disease is caused by the bacteria Tropheryma whipplei. Patients present with diarrhea, abdominal pain, and signs of malabsorption. Extraintestinal symptoms such as arthritis are common. A biopsy of the small intestine would show Periodic acid-Schiff (PAS)-positive foamy macrophages in the lamina propria. Bullet Summary: Tropical sprue presents with diarrhea, abdominal cramping, weight loss, megaloblastic anemia due to malabsorption, and a small bowel biopsy showing villi blunting following travel to an endemic area such as South Asia or the Caribbean.
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A 33-year-old woman presents to the emergency department with muscle spasms. She has noticed "twitching" in both her upper and lower extremities over the past 3 days that have interfered with her training for a marathon. She has felt more fatigued over the past month. She has no significant medical history. She takes no medication other than whey protein supplements, a multivitamin, and fish oil. Her temperature is 98.5°F (36.9°C), blood pressure is 103/71 mmHg, pulse is 105/min, respirations are 11/min, and oxygen saturation is 100% on room air. Physical exam reveals a thin woman with thinning hair. She appears pale and her mucous membranes are dry. Her neurological and cardiac exams are unremarkable. Laboratory studies are ordered as seen below. Hemoglobin: 10 g/dL Hematocrit: 30% Leukocyte count: 6,100/mm^3 with normal differential Platelet count: 187,500/mm^3 Serum: Na+: 130 mEq/L Cl-: 100 mEq/L K+: 2.3 mEq/L HCO3-: 28 mEq/L BUN: 40 mg/dL Glucose: 79 mg/dL Creatinine: 0.9 mg/dL Ca2+: 8.2 mg/dL Mg2+: 1.8 mg/dL Thyroid stimulating hormone (TSH): 4.0 mIU/L Free T4: 0.4 ng/dL (normal: 0.7-1.53 ng/dL) The patient is given 4 liters of lactated ringer solution and 40 mEq of potassium. Her repeat whole blood potassium 3 hours later is 2.4 mEq/L, and her repeat BUN is 20 mEq/L. Another 40 mEq of potassium is administered. Another repeat whole blood potassium is 2.5 mEq/L. The patient’s muscle spasms persist. Which of the following is the most likely underlying etiology for this patient’s chief concern?
Hypocalcemia
Hypomagnesemia
Hyponatremia
Hypothyroidism
Hypovolemia
B
Hypomagnesemia
This patient is presenting with what appears to be anorexia nervosa (thin habitus, possible excessive exercise in the setting of marathon running, thinning hair, dry mucous membranes, decreased free T4 level, and multiple electrolyte derangements). The lack of improvement in either her muscle spasms with fluid repletion or her hypokalemia with adequate repletion, even in the setting of normal serum magnesium, suggests depleted magnesium stores are the underlying etiology of her symptoms. Hypomagnesemia typically occurs in alcoholics, malnourished patients, and patients with eating disorders such as anorexia or bulimia nervosa. Patients may present with a wide array of symptoms including weakness, confusion, dysrhythmias, hypotension, and overall malaise. Hypomagnesemia can cause hypocalcemia as magnesium is needed for proper parathyroid hormone secretion. Another pathognomonic finding associated with hypomagnesemia is hypokalemia which is refractory to repletion. The pathophysiology is thought to be due to increased potassium wasting in the kidney due to increased activity of renal outer medullary potassium (ROMK) channels. The serum magnesium level should be checked and repletion should occur accordingly. Serum magnesium levels represent 1% or less of total body magnesium stores and do not reflect if a patient truly has hypomagnesemia, particularly if there is intracellular depletion (such as in chronically malnourished patients). Thus, any patient with findings consistent with hypomagnesemia with normal serum magnesium should have their other electrolytes repleted and be given magnesium. Magnesium repletion is a safe intervention with a wide therapeutic index, and the serum level often does not correlate with total body stores. Razzaque discusses the relationship between serum magnesium levels and total body magnesium status. The author finds that inadequate magnesium balance is associated with myriad disorders including skeletal deformities, cardiovascular diseases, and metabolic syndrome. The author recommends the use of the magnesium loading test to provide more accurate reflections of total body magnesium status. Incorrect Answers: Answer A: Hypocalcemia may present with perioral numbness, paresthesias, and muscle spasms. It can occur secondary to poor nutrition, vitamin D deficiency, and hypoparathyroidism. Low serum calcium may also be seen in patients with low albumin and in states of hyperventilation (which lowers the free calcium but not the total calcium due to a respiratory alkalosis that causes albumin to bind free calcium and release protons). Hypomagnesemia can also cause hypocalcemia as magnesium is needed for proper parathyroid hormone release. Answer C: Hyponatremia may present with malaise, nausea, vomiting, headache, and confusion. Patients may also be asymptomatic in mild cases. Asymptomatic patients with hyponatremia and functioning kidneys require only fluid restriction. On the other hand, severe hyponatremia presents with very low sodium (often < 120 mEq/L) and neurological symptoms such as profound confusion or seizures. Treatment with hypertonic saline would be indicated in this situation. Answer D: Hypothyroidism presents with fatigue, malaise, weight gain, bradycardia, scarce lanugo, and depression. This patient’s TSH is on the high end of normal and her T4 is lower than the cutoff value. However, this is likely a compensatory response to her low BMI and excessive exercise in order to slow down her metabolism to conserve energy. Answer E: Hypovolemia was certainly present in this patient given her dry mucous membranes and prerenal pattern of labs (BUN:Cr ratio > 20:1). After receiving 4L of fluids, it is clear that dehydration is not the cause of her symptoms as her BUN corrects and her symptoms persist. In general, hypovolemia should be treated with oral rehydration, if possible. Bullet Summary: Hypomagnesemia can cause hypocalcemia and hypokalemia refractory to repletion (a normal serum level does not indicate normal intracellular levels of magnesium).
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A 70-year-old woman is brought to the emergency department by ambulance. She was found on the floor of her apartment after her neighbor called 911. She is confused and is unable to provide any history, but complains of generalized pain. Her temperature is 99.2°F (37.3°C), blood pressure is 129/64 mmHg, pulse is 63/min, respirations are 13/min, and oxygen saturation is 99% on room air. Physical exam reveals, a confused, ill-appearing woman. Lungs are clear to auscultation bilaterally. An electrocardiogram is obtained as shown in Figure A. Dipstick urinalysis is notable for 4+ blood and dark colored urine. Which of the following is the most appropriate next step in management?
Albuterol and IV fluid resuscitation
Calcium gluconate
Insulin, glucose, and IV fluid resuscitation
IV fluid resuscitation
Sodium polystyrene sulfonate
B
Calcium gluconate
This patient with confusion, dark urine, and peaked T waves on electrocardiogram in the setting of a prolonged time spent down likely has developed rhabdomyolysis with associated hyperkalemia. For patients with suspected hyperkalemia and electrocardiogram changes, the most appropriate next step in management is treatment with calcium gluconate Rhabdomyolysis occurs when muscle cells lyse and release their intracellular contents. This can occur secondary to intense athletics, seizures, stimulant drug use, or a prolonged period of immobility. Lysis of muscle cells leads to release of potassium, myoglobin, purines, and other intracellular contents. Release of large amounts of myoglobin can result in renal impairment and myoglobinuria. On dipstick urinalysis, myoglobin causes a positive result for blood. Renal impairment further increases the risk for development of hyperkalemia. On ECG, hyperkalemia manifests with a "peaked" appearance of T waves, especially in the precordial leads. As hyperkalemia worsens, electrocardiogram changes progress to loss of P waves, QRS complex widening, and eventual sine wave rhythm. For patients with suspected hyperkalemia and characteristic changes noted on the electrocardiogram, the most appropriate immediate step in management is treatment with intravenous calcium gluconate. Calcium gluconate acts to stabilize cardiac myocyte membranes and prevent development of life-threatening arrhythmias. Gupta et. al review rhabdomyolysis. They discuss the pathophysiology, clinical manifestations and diagnosis. They further detail complications that may arise such as hyperkalemia, and discuss respective management strategies. Figure A demonstrates an electrocardiogram with features characteristic of hyperkalemia. Note the peaked appearance of the T waves in the precordial leads. Incorrect Answers: Answer A: Albuterol and IV fluid resuscitation may eventually be appropriate. Albuterol (a beta agonist) will act to shift potassium to the intracellular compartment. This patient's electrocardiogram changes warrant immediate treatment with calcium gluconate in order to stabilize cardiac myocyte membranes. Answer C: Insulin, glucose, and IV fluid resuscitation are indicated in this patient. Insulin acts to shift potassium to the intracellular compartment, and fluid resuscitation is warranted for the patient's likely renal impairment. Calcium gluconate is the more immediate priority to prevent development of arrhythmia. Answer D: IV fluid resuscitation is indicated for this patient with likely renal impairment. However, calcium gluconate is the more immediate priority to address this patient's hyperkalemia and prevent development of arrhythmia. Answer E: Sodium polystyrene is an oral potassium binder that increases GI excretion of potassium. It may be appropriate to treat mild hyperkalemia in some patients. It takes a prolonged amount of time to have any significant effect. Bullet Summary: For patients with suspected hyperkalemia and characteristic changes noted on ECG, the most appropriate immediate step in management is treatment with intravenous calcium gluconate.
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A 3-week-old girl presents with her parents to her pediatrician for a routine visit. The child is sleeping at regular 2-hour intervals and feeding and stooling well. The parents have noticed a “swelling of the belly button.” Cord separation occurred at 7 days of age. The swelling seems to come and go but is never larger than the size of a blueberry. They deny any drainage from the swelling. Her temperature is 98.6°F (37°C), blood pressure is 68/43 mmHg, pulse is 128/min, and respirations are 32/min. On physical exam, the child is in no acute distress and appears developmentally appropriate for her age. Her abdomen is soft and non-tender with a soft, 1 cm bulge at the umbilicus. The bulge increases in size when the child cries and can be easily reduced inside the umbilical ring without apparent pain. Which of the following is the most appropriate next step in management?
Elective surgical management
Expectant management
Histopathologic evaluation
Immediate surgical management
Thyroid hormone replacement
B
Expectant management
This infant presents with a soft, 1 cm bulge at the umbilicus that is easily reducible inside the umbilical ring, which suggests a diagnosis of an umbilical hernia. Most umbilical hernias in children resolve spontaneously and can be managed expectantly. An umbilical hernia is a protrusion of bowel, omentum, or preperitoneal fat through the umbilical ring that can change in size with increases in intra-abdominal pressure (e.g., crying). The majority of umbilical hernias in patients under 5 years of age resolve spontaneously. The indications for surgery on umbilical hernias in that age group are limited to incarcerated hernias or hernias > 1.5 cm in diameter. Incarcerated hernias present with an irreducible mass that is associated with pain and erythema. Surgical closure may be considered in patients whose defects fail to close by the age of 5. Chirdan et al. review the evidence regarding the diagnosis and treatment of pediatric patients with an incarcerated umbilical hernia. They discuss how most patients with hernias resolve spontaneously before 5 years of age but a small number will have incarcerated hernias that require surgery earlier. They recommend active observation of patients to diagnose incarceration earlier. Incorrect Answers: Answer A: Elective surgical management later on in childhood is usually not required to manage umbilical hernias as most umbilical hernias in patients less than 5 years of age close spontaneously. Hernias that persist past age 5 can be closed surgically in an elective fashion. Answer C: Histopathologic evaluation would be the most appropriate next step in management if the patient had an unknown umbilical mass, such as an umbilical granuloma or polyp. Umbilical granulomas form shortly after birth from excess tissue at the base of the umbilicus and can be treated topically, whereas umbilical polyps are firm masses of omphalomesenteric duct or urachal embryologic remnants and require surgical excision. Answer D: Immediate surgical management is required in patients with incarcerated hernias, but this patient’s hernia is easily reducible and < 1.5 cm. Incarcerated hernias present with an irreducible mass that is associated with pain and erythema. Answer E: Thyroid hormone replacement would be indicated in newborns that suffer from hypothyroidism which could present with an umbilical hernia. This patient has no other symptoms of hypothyroidism such as dry skin, hypoactive reflexes, constipation, poor muscle tone, and coarse facial features. Bullet Summary: Umbilical hernias are common in children and usually resolve spontaneously in children under 5 years of age.
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A 72-year-old man presents to his primary care physician for a general checkup. The patient works as a farmer and has no concerns about his health. He has a medical history of hypertension and obesity. His current medications include lisinopril and metoprolol. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for a murmur after S2 over the left sternal border. The patient demonstrates a stable gait and 5/5 strength in his upper and lower extremities. Which of the following is another possible finding in this patient?
Audible click heard at the cardiac apex
Femoral artery murmur
Murmur that radiates to the carotids during systole
Rumbling heard at the cardiac apex
Wedge pressure lower than expected
B
Femoral artery murmur
This patient is presenting with aortic regurgitation which could be associated with Duroziez sign (femoral artery murmur). Aortic regurgitation occurs when the aortic valve closes improperly resulting in retrograde flow during diastole. This is auscultated as a diastolic murmur heard best over the left upper sternal border and the left lower sternal border. This murmur typically occurs in elderly patients and the retrograde flow through the aorta cause a number of physical exam findings. These include a wide pulse pressure, fingernail pulsations, head bobbing, and femoral artery murmur. Treatment is with surgical valve replacement. Sapira reviews the evidence regarding the physical exam findings in aortic regurgitation. He discusses how Duroziez sign can often be seen in patients with this disease. He recommends using these signs to properly identify patients. Incorrect Answers: Answer A: An audible click heard at the cardiac apex describes mitral valve prolapse which typically is asymptomatic and presents in younger women. Treatment is with surgical valve replacement in symptomatic patients. Answer C: A murmur that radiates to the carotids during systole describes aortic stenosis which is possible in this patient except for the fact that his murmur is diastolic. Treatment is with valvuloplasty or with surgical valve replacement. Answer D: A rumbling heard at the cardiac apex describes mitral valve stenosis which presents with a diastolic murmur best heard at the cardiac apex. Treatment is with valvuloplasty or with surgical valve replacement. Answer E: A wedge pressure lower than expected would be seen in tricuspid regurgitation. In tricuspid stenosis, less blood flows to the lungs and thus the left heart, resulting in a low wedge pressure. Treatment is with identification of the underlying cause and valve replacement. Bullet Summary: Aortic regurgitation is associated with a wide pulse pressure, fingernail pulsations, head bobbing, and a femoral artery murmur.
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A 69-year-old man presents to clinic due to shortness of breath, worsening pain in his right shoulder, and episodes of hemoptysis. His symptoms began 3 months ago. He has also lost 18 pounds. He has a history of coronary artery disease and underwent an uncomplicated coronary angioplasty with stent placement 2 years ago. He routinely travels both domestically and internationally as a senior shipyard worker and has a 30-pack-year smoking history. He used to drink 4 cocktails a week. He has not smoke or drank alcohol in over 7 years. His temperature is 99.5°F (37.7°C), blood pressure is 140/60 mmHg, pulse is 97/min, and respirations are 13/min. Physical exam is notable for right pupillary constriction as well as paresthesias in his right fourth and fifth digits. There is no pain with active shoulder rotation. His chest imaging is shown in Figure A. Which of the following is most likely responsible for the patient’s symptoms?
Lung adenocarcinoma
Mesothelioma
Rotator cuff tendinopathy
Syringomyelia
Tuberculosis
A
Lung adenocarcinoma
This elderly patient with an extensive smoking history is presenting with hemoptysis, weight loss, and shortness of breath. Combined with neurological findings in the ulnar nerve distribution (paresthesias in fourth and fifth digits) and imaging evidence of a superior right lung mass, the most likely diagnosis is a Pancoast tumor, which is most commonly a lung adenocarcinoma. A Pancoast tumor is a peripheral lung cancer often caused by lung adenocarcinoma or squamous cell carcinoma and is often located in the superior sulcus of the lung. The most important risk factor is smoking. This tumor causes compression of adjacent structures. Patients can present with severe, localized axilla pain (due to invasion of the brachial plexus), weakness of intrinsic hand muscles with radicular pain and paresthesias in the fourth and fifth digits (due to invasion of the C8-T1 portion of the brachial plexus), Horner syndrome (miosis, ptosis, and facial anhidrosis due to invasion of the cervical sympathetic nerves), hoarseness (due to invasion of the recurrent laryngeal nerve), and edema of the arm with facial swelling (due to compression of the superior vena cava). Treatment is varied and involves a combination of radiation, chemotherapy, and surgery. Wu et al. studied the role of the tyrosine-kinase inhibitor, osimertinib, in the treatment of completed resected epidermal growth factor receptor (EGFR)-mutated non-small cell lung cancer (NCSLC). The authors find that 89% of patients in the osimertinib group and 52% of the placebo groups were alive and disease free at 24 months. The authors recommend the use of osimertinib in patients with stage IB to IIIA EGFR-mutated NSCLC. Figure/Illustration A demonstrates a right apical lung mass found on computed tomography (CT) scan (green arrow). Incorrect Answers: Answer B: Mesothelioma is a neoplasm of the pleura found in people with occupational exposures to asbestos, such as shipyard workers. While this neoplasm can present with hemoptysis and dyspnea, chest imaging of mesothelioma reveals pleural plaques and thickening, not an isolated apical lung lesion. Answer C: Rotator cuff tendinopathy presents with pain with abduction and external rotation of the arm. This patient has no pain with active shoulder rotation. Answer D: Syringomyelia is a cyst or cavity in the spinal column that can cause Horner syndrome and fluctuating radicular pain. It is unlikely to cause hemoptysis, weight loss, and the chest imaging findings in this patient. Answer E: Tuberculosis is a possible diagnosis in this patient with hemoptysis, weight loss, and extensive travel history. Tuberculosis often presents with a fever and does not usually cause shoulder pain. Bullet Summary: Pancoast tumor presents with shoulder pain, cervical radiculopathy, Horner syndrome, and hoarseness and should be suspected in a patient with a smoking history and an apical lung mass on imaging.
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A 36-year-old woman, G1P1001, presents to her gynecologist for an annual visit. She has a medical history of hypertension for which she takes hydrochlorothiazide. The patient’s mother had breast cancer at age 68, and her sister has endometriosis. She states that she feels well and has no complaints. Her temperature is 98.6°F (37.0°C), blood pressure is 138/74 mmHg, pulse is 80/min, and respirations are 13/min. Her BMI is 32.4 kg/m^2. A pelvic exam reveals a non-tender, 12-week-size uterus with an irregular contour. A transvaginal ultrasound is performed and demonstrates two intramural leiomyomas. Which of the following is a likely complication of this patient’s condition?
Amenorrhea
Endometrial cancer
Iron deficiency anemia
Uterine prolapse
Uterine sarcoma
C
Iron deficiency anemia
This patient presents with an irregularly enlarged uterus on examination consistent with the intramural leiomyomas (fibroids) seen on ultrasound. Leiomyomas increase the risk of iron deficiency anemia due to heavy menstrual bleeding. Leiomyoma are benign overgrowths of myometrial tissue. The most common presentation is heavy or prolonged menstrual bleeding or symptoms related to mass effect such as pelvic pressure or pain.. Affected women often develop these symptoms in their 30s or 40s, though they may occur at any age, and many are asymptomatic. Risk factors include early menarche, obesity, family history, and African American race. Submucosal fibroids that disrupt the endometrium may cause heavy menstrual bleeding. Intramural fibroids also commonly cause heavy bleeding despite the fact that they do not disrupt the endometrium due to alterations in the uterine vasculature. Patients may be managed medically with a wide range of options including oral contraceptive pills, tranexamic acid, and GnRH agonists. Definitive management is with surgical intervention. Myomectomy is preferred for patients that desire child bearing in the future. For those that do not have plans for pregnancy in the future, hysterectomy is recommended. Yang et. al review the diagnosis and management of uterine fibroids. They note the range of clinical presentations to include heavy menstrual bleeding. They discuss management to include surgical intervention with myomectomy or hysterectomy. Incorrect Answers: Answer A: Amenorrhea, or the absence of menstrual bleeding, is often due to anovulation or uterine adhesions. Uterine fibroids are not associated with amenorrhea. Answer B: Endometrial cancer is a known cause of heavy menstrual bleeding. However, this patient’s ultrasound notes the presence of intramural leiomyomas. Her age, history, and ultrasound findings are not suggestive of endometrial cancer. Answer D: Uterine prolapse is associated with multiparity, chronically increased intra-abdominal pressure (from chronic obstructive pulmonary disease, obesity, etc.), and connective tissue diseases. While fibroids rarely may prolapse, they typically do not cause the entire uterus to prolapse. Answer E: Uterine sarcoma may very rarely result from uterine fibroids, as both arise from the uterine smooth muscle. However, this patient’s history and ultrasound findings are more suggestive of uterine leiomyoma. Bullet Summary: Patients with uterine leiomyomata (fibroids) are at risk for iron deficiency anemia due to heavy bleeding.
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A 77-year-old man presents to the emergency department acutely obtunded. The patient lives alone and was found unresponsive by his son. Generally, the patient manages his own finances, medications, and works part-time. He has not been responding to phone calls for the past 3 days. The patient is unable to offer a history. He has a past medical history of hypothyroidism, depression, and diabetes. His temperature is 88.0°F (31.1°C), blood pressure is 92/62 mmHg, pulse is 35/min, respirations are 9/min, and oxygen saturation is 92% on room air. The patient is cold to the touch and moves all extremities to painful stimuli. His pupils are reactive and sluggish, and he does not follow commands. There are no signs of trauma or skin infections. The patient is started on IV fluids and hydrocortisone, is externally warmed, and is started on a norepinephrine drip. An ECG is performed as seen in Figure A. Which of the following is the most appropriate next step in management?
Free T4 level
Levothyroxine administration
Thyroid stimulating hormone and free T4 level
Thyroid stimulating hormone level
Triiodothyronine administration
B
Levothyroxine administration
This elderly patient with a past medical history of hypothyroidism is presenting with altered mental status, hypotension, hypothermia, and bradycardia, which are concerning for myxedema coma. Empiric and immediate treatment with levothyroxine is the appropriate management of this condition prior to confirming the diagnosis with lab studies. Myxedema coma is a life-threatening complication of hypothyroidism. It presents with stupor/obtundation, bradycardia, hemodynamic instability, and hypothermia. With a high clinical suspicion, appropriate history, physical exam, and vitals, it is appropriate to first treat the patient with IV hydrocortisone (or another highly potent steroid) and levothyroxine prior to confirming the diagnosis given the high morbidity and mortality associated with this condition. Triiodothyronine is given in some circumstances as well (in more ill patients) but is less dire than first giving levothyroxine. Subsequently, the diagnosis can be confirmed with a serum thyroid stimulating hormone (TSH) and free T4 level. Patients are critically ill and typically require further care in an ICU. Wall discusses the management of myxedema coma. He notes this is a potentially lethal condition. He recommends immediate administration of IV levothyroxine. Figure A is an ECG with sinus bradycardia. Incorrect Answers: Answers 1, 3, & 4: Thyroid stimulating hormone and free T4 levels should certainly be obtained; however, the mortality for myxedema coma is so high that empiric treatment with levothyroxine should be started with clinical suspicion alone prior to confirming the diagnosis with laboratory studies, as a delay in therapy could increase morbidity and mortality. After administration of levothyroxine and initial supportive therapy, the diagnosis can be supported with laboratory studies. Answer E: Triiodothyronine or T3 could be appropriate in patients with myxedema coma in severe scenarios where patients do not respond to levothyroxine. In some cases, it may also be given empirically but does not take priority over T4 administration. Bullet Summary: In myxedema coma, empiric treatment with levothyroxine is appropriate with a high clinical suspicion for the diagnosis prior to laboratory confirmation.
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A 39-year-old man presents to his doctor for a wellness checkup. He is concerned about a rash that does not seem to be improving. He was recently exposed to his grandfather who has vesicular lesions on his skin and is being treated. He has a family history of skin cancer, colon cancer, and ovarian cancer. The patient has a medical history of asthma and seasonal allergies. His temperature is 98.6°F (37.0°C), blood pressure is 137/98 mmHg, pulse is 90/min, respirations are 14/min, and oxygen saturation is 98% on room air. Physical exam reveals the finding in Figure A. Which of the following describes this patient's most likely diagnosis?
Benign capillary proliferation
Edema of the epidermis
Healthy dermatologic development
Malignant blood vessel proliferation
Viral infection
A
Benign capillary proliferation
This patient who presents with red raised papules is presenting with a cherry angioma. These lesions are composed of benign capillary proliferations. Cherry angiomas present with cherry red macules/papules that occur secondary to benign capillary and venule proliferation. The lesions are entirely benign and are cosmetic. These lesions are common in middle-aged adults. As the patient ages, more lesions will appear. In the absence of symptoms, observation and reassurance can be used for conservative management. They can be removed surgically if the patient desires. Nazer et al. study the risk factors associated with the development of cherry angioma. They found that the use of clopidogrel and tamsulosin is associated with these lesions. They recommend ruling out underlying conditions. Figure/Illustration A demonstrates cherry red macules/papules classically seen in patients with cherry angioma. Incorrect Answers: Answer B: Edema of the epidermis describes eczema or atopic dermatitis. This disease would present with pruritic, erythematous, and vesicular lesions. Topical creams and hygiene can be used for symptomatic management of these lesions. Answer C: Healthy dermatologic development misses the diagnosis of a cherry angioma, which although benign, is a more accurate diagnosis for this case. Answer D: Malignant blood vessel proliferation does not describe a cherry angioma which is benign and purely cosmetic. An angiosarcoma usually occurs in deep tissues and requires surgical excision. Answer E: Viral infection describes herpes simplex virus which would present with vesicular lesions and neurogenic pain. These patients can be treated with valacyclovir as well as gabapentin for neuropathic pain. Bullet Summary: Cherry angiomas are benign capillary proliferations that appear as cherry red macules/papules.
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A newborn boy is evaluated 30 minutes after birth. He was born at 39 weeks gestation to a 27-year-old primigravid mother via cesarean section for cervical insufficiency. The pregnancy was complicated by gestational diabetes and the amniotic fluid was clear. Upon delivery, the patient had strong respiratory effort and a strong cry. His Apgar scores at 1 and 5 minutes were 7 and 8, respectively. The patient now is exhibiting increased work of breathing and is progressively more tachypneic. His birth weight is 3,568 g (7 lb 14 oz). His temperature is 99.0°F (37.2°C), blood pressure is 60/44 mmHg, pulse is 146/min, and respirations are 72/min. On physical exam, the patient is grunting with nasal flaring and subcostal retractions. Breath sounds are decreased at the bases bilaterally. The patient has central cyanosis. His chest radiograph can be seen in Figure A. Which of the following is the most likely etiology of this patient’s presentation?
Meconium aspiration syndrome
Neonatal respiratory distress syndrome
Persistent pulmonary hypertension
Transient tachypnea of the newborn
Viral pneumonia
D
Transient tachypnea of the newborn
This newborn is presenting with increased work of breathing and progressive tachypnea in the 1st hour of life, which is consistent with a diagnosis of transient tachypnea of the newborn. Transient tachypnea of the newborn presents with respiratory distress and marked tachypnea within 2 hours of delivery. Transient tachypnea of the newborn is caused by delayed resorption and clearance of alveolar fluid, which results in mild pulmonary edema. Chest radiography typically demonstrates bilateral perihilar streaking and hyperinflation of the lungs. Transient tachypnea of the newborn is benign and usually resolves by day 2 of life. The management of this condition is supportive. Alhassen et al. review the evidence regarding the diagnosis and treatment of TTN. They discuss how this condition sometimes requires extended hospitalization and is associated with wheezing in childhood. They recommend using ultrasound to help make this diagnosis. Figure/Illustration A demonstrates prominent vascular markings around the hilum (yellow arrows), and increased lung volumes on chest radiograph, which is consistent with transient tachypnea of the newborn. Incorrect Answers: Answer A: Meconium aspiration syndrome can cause respiratory distress in the immediate newborn period, but it would be unlikely in a patient with a history of clear amniotic fluid, and a more mild clinical picture. Meconium aspiration syndrome would be seen on chest radiograph as coarse, irregular infiltrates and hyper expansion. Treatment is with antibiotics and airway clearance. Answer B: Neonatal respiratory distress syndrome is a disease of prematurity and does not often occur in infants born at term to healthy mothers. The chest radiograph in neonatal respiratory distress syndrome is also more likely to demonstrate low lung volumes. Treatment is with surfactant administration. Answer C: Persistent pulmonary hypertension is a cause of respiratory distress in the newborn period, but a chest radiograph typically shows clear lungs. Treatment is with respiratory support as well as inhaled nitric oxide. Answer E: Viral pneumonia is uncommon in the immediate newborn period. Bacterial pneumonia is more common but usually presents with signs of infection in the mother, which are not present in this case. Treatment is with broad-spectrum antibiotics. Bullet Summary: Transient tachypnea of the newborn presents with worsening tachypnea within 2 hours of delivery and is caused by pulmonary edema resulting from inadequate clearance of alveolar fluid.
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A 55-year-old man presents to the emergency department after fainting while moving furniture. He states that he has been working in a hot, humid attic all day and forgot his water bottle. He felt dizzy when standing up and then fainted and was unconscious for roughly 1 minute. He awoke at his neurologic baseline afterwards. The patient has a history of hypertension being managed by his primary care doctor. His temperature is 98.7°F (37.1°C), blood pressure is 149/82 mmHg, pulse is 86/min, respirations are 15/min, and oxygen saturation is 99% on room air. Physical exam reveals a diaphoretic man. He demonstrates an unremarkable cardiopulmonary exam. The patient walks with a steady gait and his neurologic exam is non-focal. An ECG is performed as seen in Figure A. The patient is given 2 liters of oral fluids and feels back to his baseline. Which of the following conditions is most likely to occur in this patient based on his current history and ECG?
Atrial fibrillation
Complete heart block
Myocardial infarction
Stroke
Torsades des pointes
B
Complete heart block
This patient is presenting with dizziness and syncope in the setting of likely dehydration (working in a hot/humid environment without water) and feels better after rehydration, suggesting a diagnosis of orthostatic hypotension. An ECG is performed and incidentally demonstrates a bifascicular block, which is at high risk of progressing to a complete heart block. Bifascular block is a conduction abnormality in 2 of the 3 fascicles that conduct electricity from atria to ventricles. This leaves conduction to the ventricles via the single remaining fascicle. There are different possibilities that may present on ECG. One possibility is a right bundle branch block with a left anterior fascicular block; the other is a right bundle branch block with a left posterior fascicular block. Note that the pathophysiology of complete heart block is an inability to conduct electricity from the atria to the ventricles. If there is only 1 fascicle left to conduct electricity, the patient would be in complete heart block if this fascicle were to no longer work, which is a common outcome in patients with bifascicular block. Treatment of complete heart block involves the placement of a pacemaker. Wiberg et al. discuss bifascicular block. They note the possible outcome of a complete heart block. It is recommended to look for this finding on ECG and appropriately workup patients to prevent progression of disease. Figure/Illustration A is an ECG demonstrating bifascicular block. Note the left axis deviation as suggested by the net upward deflection of the QRS in lead I and net downward deflection in lead II (blue arrows). Then, note the appearance of a right bundle branch block with the RSR pattern in aVR (black arrow) and upward R’ in lead V1 (green arrow). This is a classic pattern for bifascicular block. Contrast this to the other pattern of bifascicular block which would present with a right bundle branch block and right axis deviation (upward QRS in lead II and downward in lead I), which was not seen in this case. Incorrect Answers: Answer A: Atrial fibrillation may occur secondary to aging, hypertension, or ischemia and presents with an irregularly irregular rate and rhythm with an absence of P waves. It may cause complications such as stroke. Management typically involves rate control agents such as beta-blockers or calcium channel blockers. Answer C: Myocardial infarction is a likely outcome in individuals with risk factors such as obesity, hypertension, smoking, and dyslipidemia, in particular, without proper preventive measures and medical therapy including statins, beta blockers, and ACE inhibitors. This patient may be at risk of a myocardial infarction with his hypertension, and his underlying bifascicular block could suggest undiagnosed cardiac ischemia; however, it is more likely this patient progresses to complete heart block in the setting of his bifascicular block. Answer D: Stroke is a possible complication of atrial fibrillation. For this reason, most patients with atrial fibrillation are on blood thinners to prevent this complication. It occurs secondary to stasis of blood in the fibrillating left atrium. Patients are often risk stratified prior to starting blood thinners with tools such as CHADS2VASC and HASBLED. Isolated syncope is a rare manifestation of stroke. Answer E: Torsades des pointes is a progression from QT prolongation which may occur secondary to genetic conditions, medications, and/or infection. It presents on ECG with QRS complexes that twist in amplitude around the electrical baseline. Treatment involves the administration of magnesium sulfate and, if needed, electrical cardioversion. Bullet Summary: A bifascicular block can progress to complete heart block.
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A 68-year-old man presents to the emergency department with right ear pain that he developed 4 days prior, and it has progressively worsened. The pain is worse at night. He has associated fever and chills. This morning, he awoke with purulent, foul-smelling discharge on his pillow. He has a history of type II diabetes mellitus and hypertension. He takes metformin, insulin, and lisinopril. His temperature is 102.2°F (39.0°C), blood pressure is 130/87 mmHg, pulse is 110/min, and respirations are 16/min. The patient is toxic in appearance and in mild distress due to pain. Examination of the external ear reveals findings shown in Figure A. Granulation tissue and purulent drainage are noted in the external auditory canal. Physical exam is otherwise unremarkable. Which of the following is the most appropriate therapy for this patient?
Acyclovir
Amoxicillin
Ceftriaxone
Ofloxacin ear drops
Vancomycin and piperacillin-tazobactam
E
Vancomycin and piperacillin-tazobactam
The patient in this vignette most likely has malignant otitis externa (also referred to as necrotizing otitis externa), which is a life-threatening infection requiring therapy with intravenous anti-pseudomonal and broad-spectrum antibiotics. Piperacillin-tazobactam and vancomycin is an appropriate initial regimen. Malignant otitis externa is an invasive infection of the external auditory canal and skull base, and is seen almost exclusively in immunocompromised patients. Patients with poorly controlled type II diabetes mellitus are at particularly increased risk. The infection spreads from the floor of the ear canal to the nearby tissues including the bones at the base of the skull and the temporomandibular joint. The resulting skull base osteomyelitis distinguishes the "necrotizing" or "malignant" presentation of otitis externa. Patients often present with severe otalgia and purulent drainage from the external auditory canal. Granulation tissue in the external auditory canal is a characteristic exam finding. Pseudomonas aeruginosa is the causative organism in most cases of necrotizing otitis externa. Therefore, intravenous antibiotics with sufficient coverage of Pseudomonas are the therapy of choice. As infection progresses, involvement of cranial nerves with resultant palsy may be observed. Aaraj et. al review the clinical manifestations, diagnosis and management of malignant otitis externa. They note the risk for this disease among patients with diabetes or other etiologies of an immunocompromised state. They recommend management with intravenous ciprofloxacin. Figure A shows the external ear of a patient with malignant otitis externa. Note the classic granulation tissue at the cartilage-bone junction and crusted, purulent discharge. Incorrect Answers: Answer A: Intravenous acyclovir would be appropriate for suspected herpes zoster, which may present with ear pain and a vesicular rash in the external auditory canal. This patient's granulation tissue in the external auditory canal and purulent drainage make malignant otitis externa more likely. Answer B: Oral amoxicillin may be appropriate for management of acute otitis media which presents with ear pain and a distended tympanic membrane that is immobile. Answer C: Ceftriaxone is appropriate coverage for cellulitis which presents with warmth, erythema, and tenderness of the skin. It would be too narrow of spectrum to treat malignant otitis externa. Answer E: Topical ofloxacin may be appropriate for some cases of simple otitis externa ("swimmer's ear") which presents with ear pain and drainage in young, healthy patients who swim regularly. Patients are well-appearing. Bullet Summary: Malignant otitis externa is an invasive infection of the external auditory canal and skull base that requires management with broad-spectrum intravenous antibiotics that cover Pseudomonas aeruginosa.
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A 54-year-old man is brought to the emergency department following a skiing accident. The patient lost control of his skis and collided with a tree. He lost consciousness for about 30 seconds. When he woke up, he was mildly confused but able to ski down the rest of the mountain. The patient initially denied medical treatment but his wife convinced him to be taken by ambulance to the emergency department for evaluation. The paramedics report that at the scene the patient had a Glasgow Coma Score (GCS) of 15. During the ride, he became gradually more somnolent. His medical history is significant for hypertension, hyperlipidemia, coronary artery disease, and a prior transient ischemic attack. His medications include atorvastatin and lisinopril. In the emergency department, his temperature is 97.8°F (36.6°C), blood pressure is 141/84 mmHg, pulse is 71/min, and respirations are 16/min. He is difficult to arouse with a GCS of 7. A head CT is performed and can be seen in Figure A. Which of the following is the most likely cause of this patient’s presentation?
Damage to the cerebral bridging veins
Damage to the sphenoid bone
Occlusion of cerebral small penetrating arteries
Ruptured cerebral aneurysm
Secondary hemorrhage into prior area of infarction
B
Damage to the sphenoid bone
This patient presents with a lucid interval followed by worsening somnolence and a biconvex lesion on head CT, which suggests a diagnosis of acute epidural hematoma. Acute epidural hematomas are most commonly caused by damage to the middle meningeal artery from trauma to the sphenoid bone. Patients with an epidural hematoma typically have an initial loss of consciousness followed by a lucid interval, during which their mental status returns to baseline or near baseline. As the hematoma expands, patients display increasing somnolence from the increase in intracranial pressure. For that reason, epidural hematomas are sometimes referred to as the “talk and die” syndrome. If not relieved, this increase in intracranial pressure can lead to uncal herniation or death. Patients with neurologic findings or those with expanding bleeds require urgent surgical decompression. Dadashi et al. present cases of patients with epidural hematoma from acute trauma. They discuss how patients often sustain these injuries from extreme sports. They recommend being aware of this syndrome so that acute interventions can be taken. Figure/Illustration A demonstrates a biconvex hyperdensity that is limited by suture lines (red circle). This is the typical appearance of an epidural hematoma on head CT. Incorrect Answers: Answer A: Damage to the cerebral bridging veins results in a subdural hematoma (SDH). An acute SDH more commonly presents with a gradual increase in headache and confusion rather than the “talk and die” syndrome presented in this vignette. However, a minority of patients with acute SDH may have a lucid interval followed by progressive neurological decline. SDH would have a crescent-shaped appearance on head CT rather than the biconvex appearance of an epidural hematoma. Answer C: Occlusion of cerebral small penetrating arteries describes a lacunar stroke. Although this patient has 2 risk factors for lacunar stroke (hypertension and smoking), this patient’s head CT demonstrates an epidural hematoma. Answer D: A ruptured cerebral aneurysm would most likely lead to a subarachnoid hemorrhage. A subarachnoid hemorrhage presents as a sudden, severe headache (“thunderclap headache”), confusion or somnolence, nausea and vomiting, and meningismus. Surgical clipping or embolization of the bleeding vessel may be required. Answer E: Secondary hemorrhage into a prior area of infarction usually occurs in the setting of a recent embolic stroke. Although this patient has a history of TIA and other risk factors for stroke, his head CT demonstrates an epidural hematoma. Treatment is supportive with the control of underlying risk factors such as hypertension. Bullet Summary: Epidural hematomas are caused by damage to the middle meningeal artery as it passes through the foramen spinosum of the sphenoid bone.
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A 2-day-old boy has an episode of vomiting in the hospital nursery. The vomitus was described as “bright green” without any traces of blood. The patient has urinated several times since he was born but has not passed any stool. He was born at 37 weeks of gestation to a 38-year-old G3P3 woman. The pregnancy was uncomplicated and the patient’s mother refused all prenatal testing. The patient’s 2 older siblings are both healthy. His temperature is 98.6°F (37°C), blood pressure is 67/43 mmHg, pulse is 135/min, and respirations are 34/min. On physical exam, the patient has upslanting palpebral fissures, epicanthal folds, and a single transverse palmar crease. His abdomen is non-tender, firm, and distended. Bowel sounds are hypoactive. Digital rectal exam evacuates a small amount of stool and flatulence. A nasogastric tube is placed to decompress the stomach. The patient’s abdominal radiograph can be seen in Figure A. Which of the following is the most likely diagnosis?
Duodenal atresia
Hirschsprung disease
Intestinal malrotation
Meconium ileus
Pyloric stenosis
B
Hirschsprung disease
This pediatric patient presents with features of Down syndrome (trisomy 21), bilious vomiting, and colonic distention on an abdominal radiograph, which suggests a diagnosis of Hirschsprung disease. Hirschsprung disease is associated with Down syndrome and presents with bilious vomiting, failure to pass meconium in the first 48 hours of life, and abdominal distension. A digital rectal exam may temporarily relieve the obstruction and lead to the expulsion of gas and stool (the “squirt sign”). Abdominal radiography typically demonstrates signs of distal intestinal obstruction such as proximal dilation and an absence of air in the rectum. Contrast enema may reveal a transition zone between the dilated proximal megacolon and the narrow rectosigmoid colon. A biopsy can confirm the diagnosis. Treatment is with surgical removal of the defective segment of the bowel. Kessman reviews the evidence regarding the diagnosis and treatment of Hirschsprung disease. She discusses how the diagnosis can be made using a rectal suction biopsy. She recommends monitoring patients closely for enterocolitis for years after surgical treatment. Figure/Illustration A is an abdominal radiograph that demonstrates colonic distension (red circle). This finding is consistent with a diagnosis of Hirschsprung disease. Incorrect Answers: Answer A: Duodenal atresia is associated with Down syndrome (trisomy 21) and may also present in the first 48 hours of life with bilious vomiting. The abdominal radiograph will demonstrate the “double bubble” sign, which reflects air trapping in the stomach and first portion of the duodenum. Treatment is with the surgical opening of the atretic segment. Answer C: Intestinal malrotation may preset with bilious vomiting in a neonate due to the development of a midgut volvulus. An abdominal radiograph may show the extension of the nasogastric tube into an abnormally positioned duodenum and would not show diffuse colonic distension. Treatment is with surgical derotation of the volvulus. Answer D: Meconium ileus is associated with cystic fibrosis and also presents with delayed (> 48 hours) passing of meconium. The distal obstruction in these patients will not be temporarily relieved by a digital rectal exam. Contrast enema will demonstrate microcolon involving the entire large bowel. Treatment is with irrigation and removal of the meconium. Answer E: Pyloric stenosis usually presents in the first 3-6 weeks of life with post-prandial, nonbilious, projectile vomiting and a palpable olive-shaped mass in the epigastrium. This patient is presenting at just 2 days of life and has bilious vomiting. Treatment is with surgical release of the stenosis. Bullet Summary: Hirschsprung disease is associated with Down syndrome (trisomy 21) and presents with retention of stool that can be relieved by digital disimpaction, bilious vomiting, and colonic distention on abdominal radiography.
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A 15-year-old girl is brought to the clinic because her mother is worried the patient has not yet had her period. The patient’s older sister had her first period at age 14. The mother had her first period at age 13. The patient reports she is doing well in school and is on the varsity basketball team. Her medical history is significant for asthma and atopic dermatitis. Her medications include albuterol and topical triamcinolone. Her temperature is 98°F (36.7°C), blood pressure is 111/72 mmHg, pulse is 65/min, and respirations are 14/min with an oxygen saturation of 99% on room air. Her body mass index (BMI) is 19 kg/m^2. Physical exam shows absent breast development and external genitalia at Tanner stage 1. Serum follicle-stimulating hormone (FSH) level is measured to be 38 mIU/mL. Which of the following is the most appropriate next diagnostic step?
CYP17 gene work-up
Estrogen levels
Gonadotrophin-releasing hormone stimulation test
Karotype
Luteinizing hormone levels
D
Karotype
The patient is presenting with primary amenorrhea and an increased follicle-stimulating hormone (FSH) level, suggesting a gonadal abnormality and making karyotyping the most appropriate next step in management. Primary amenorrhea can be diagnosed if a patient has normal secondary sex characteristics but no menarche by age 16, or if she has no secondary sex characteristics and no menarche as early as age 14. Primary amenorrhea is caused by either hypothalamic/pituitary (central) or gonadal (peripheral) abnormalities. Increased FSH indicates a peripheral cause or hypergonadotropic hypogonadism. Karyotyping would be the most appropriate next diagnostic step because these disorders can include Turner syndrome and androgen insensitivity syndrome. Gravholt et al. review the evidence regarding the diagnosis and treatment of Turner syndrome. They discuss how this disease presents with hypergonadotropic hypogonadism. They recommend growth hormone therapy and hormone replacement therapy in these patients. Incorrect Answers: Answer A: CYP17 gene work-up should be done in patients with primary amenorrhea, an absence of secondary sex characteristics, elevated FSH, and hypertension. Alterations in the CYP17 gene can cause congenital adrenal hyperplasia due to 17-hydroxylase deficiency. The resulting excess of deoxycorticosterone is associated with hypertension and hypokalemia. Treatment is with hormone repletion therapy. Answer B: Estrogen levels would be low in this patient as evidenced by her absence of breast development. Therefore, measuring estrogen levels would provide no additional diagnostic information. Estrogen levels may be useful in cases of peripheral gonadotropin resistance but these are rare. Answer C: Gonadotropin-releasing hormone (GnRH) stimulation test would be the next diagnostic step if FSH were decreased. A decrease in FSH indicates a central cause or hypogonadotropic hypogonadism. A GnRH stimulation test is used to assess whether the central abnormality is due to the hypothalamus or the pituitary. Answer E: Luteinizing hormone (LH) levels with a repeat FSH level may be helpful if FSH is low or normal. Low or normal FSH levels indicate hypogonadotropic hypogonadism as caused by hypothalamic-pituitary axis abnormalities. Cases of high FSH represent a different diagnostic entity as adequate gonadotropins are produced. Bullet Summary: In a patient with primary amenorrhea and an increased FSH, the next step in management is karyotyping.
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A 36-year-old G3P2002 woman presents to her obstetrician’s office for her 1st prenatal visit at 10 weeks and 2 days gestation. She has felt nauseous the last several mornings and has been tired for a few weeks. The patient has had 2 uncomplicated spontaneous vaginal deliveries at full term with her last child born 6 years ago. She is concerned about the risk of Down syndrome in this fetus, as her sister gave birth to an affected child at age 43. She has a history of generalized anxiety disorder, atopic dermatitis, and she is currently on escitalopram. Her temperature is 98.6°F (37.0°C), pulse is 70/min, blood pressure is 121/67 mmHg, and respirations are 13/min. The patient appears anxious, but overall comfortable, and cardiopulmonary and abdominal exams are unremarkable. Pelvic exam reveals normal external genitalia, a closed and soft cervix, a 10-week-sized uterus, and no adnexal masses. Which of the following is the most appropriate next step for definitively determining whether this patient’s fetus has Down syndrome?
Amniocentesis
Anatomy ultrasound
Chorionic villus sampling
Genetic testing of patient’s sister
Nuchal translucency test
C
Chorionic villus sampling
This patient is over 35 years of age and concerned about the risk of Down syndrome (trisomy 21) in her fetus at just over 10 weeks gestation. At this gestational age, the most definitive method for determining whether such an aneuploidy is present is chorionic villus sampling (CVS). CVS, or sampling of the placental tissue, can be performed as early as 10 weeks gestation. This is in contrast to amniocentesis, which should be avoided prior to 14 weeks. There are other screening tests for a patient concerned about aneuploidy; however, only CVS and amniocentesis offer definitive diagnoses. Complications of this procedure include pregnancy loss (at a rate of 1-2%), transverse limb abnormality, rupture of membranes, and chorioamnionitis. Patients should also have counseling if genetic abnormalities are detected using this procedure. Farina reviews the evidence regarding the complications associated with CVS. He discusses how this procedure is becoming more common as the definitive method of providing an early diagnosis. He recommends being aware of complications such as possible pregnancy loss. Incorrect Answers: Answer A: Amniocentesis is a useful tool for the definitive diagnosis of aneuploidy and other genetic conditions. Due to the higher rate of fetal loss at younger gestational ages, it is reserved for those above 14 weeks. Answer B: The anatomy ultrasound can be helpful in identifying morphologic features that are concerning for Down syndrome, such as a flat nasal bridge or an echogenic cardiac focus. This screening tool is most accurate after 18 weeks gestation. Answer D: Genetic testing of the patient’s sister may be able to help identify if there is a parental translocation or aneuploidy that led to the child’s Down syndrome. This is much less likely than meiotic nondisjunction due to advanced maternal age, and this would not help diagnose Down syndrome in the patient’s fetus. Answer E: The nuchal translucency test examines the thickness of the nuchal fold. When the thickness exceeds normal measurements, there is an increased risk of aneuploidy such as Down syndrome, but this is a screening result that would not provide a definitive diagnosis. Bullet Summary: Chorionic villus sampling is a definitive method of identifying aneuploidy in fetuses starting at 10 weeks of gestational age.
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A 9-hour-old newborn girl is found in the newborn nursery with diffuse swelling of the scalp not present at birth. The child was born at 38 weeks of gestation to a 28-year-old G3P3 mother. The mother went into spontaneous labor but the delivery was complicated by a prolonged 2nd stage of labor. A vacuum-assisted vaginal delivery was eventually performed. The child’s Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. The pregnancy was complicated by preeclampsia in the mother which was well-controlled throughout the pregnancy. Her temperature is 98.6°F (37°C), blood pressure is 67/43 mmHg, pulse is 135/min, and respirations are 34/min. On physical exam, she appears to be in mild distress and has a 4x5 cm ecchymotic area of swelling over the bilateral parietal bones. Serial assessments of the child’s head circumference over the next 12 hours show no change in the size of the swelling. This patient’s condition most likely affects which of the following spaces or potential spaces?
Between dura and arachnoid mater
Between periosteum and galea aponeurosis
Between periosteum and skull
Between scalp and galea aponeurosis
Into the lateral ventricles
D
Between scalp and galea aponeurosis
This patient presents following a vacuum-assisted vaginal delivery with an ecchymotic swelling of the scalp that crosses the midline, which suggests a diagnosis of caput succedaneum. Caput succedaneum is caused by bleeding between the skin and the galea aponeurosis. Caput succedaneum is an uncommon extracranial injury that occurs during a traumatic birth, particularly in deliveries in which a vacuum-assist device is used. It involves bleeding between the skin of the scalp and the galea aponeurosis. Unlike a cephalohematoma, caput succedaneum lesions may cross the suture lines of the cranial bones. Caput succedaneum is typically described as fluctuant and ecchymotic, and the swelling usually self-resolves within a few days. Treatment is supportive though patients may occasionally require phototherapy if they develop high levels of bilirubin. Transfusion is indicated if there is significant anemia present (though this is uncommon). Jacob and Hoerter present evidence regarding the diagnosis and treatment of patients with caput succedaneum. They discuss how this lesion commonly crosses cranial suture lines as well as the midline. They recommend differentiating this disease from more malignant etiologies such as intracranial hemorrhage. Incorrect Answers: Answer A: Bleeding between the dura and arachnoid mater describes a subdural hematoma. Subdural hematomas are the most common type of intracranial hemorrhage found in neonates, but they usually present with more serious signs of respiratory depression, apnea, or seizures. Patients with a subdural hematoma should be evaluated for non-accidental trauma. Treatment of expanding lesions may require surgical decompression. Answer B: Bleeding between the periosteum and galea aponeurosis describes a subgaleal hemorrhage, an uncommon but serious complication of traumatic birth. Like caput succedaneum, subgaleal hemorrhages occur most commonly in newborns delivered via vacuum-assisted delivery, but the swelling in a subgaleal hemorrhage involves the entire scalp. Patients may also present with signs of extensive blood loss and neurological disturbances. Treatment involves resuscitation with fluids or blood and possibly neurosurgical intervention. Answer C: Bleeding between the periosteum and skull describes a cephalohematoma. Cephalohematoma occurs in newborns and is caused by the rupture of the blood vessels crossing the periosteum. They are usually secondary to a prolonged second stage of labor and, unlike caput succedaneum, do not cross suture lines. The treatment for these lesions is supportive as most of these lesions resolve spontaneously. Answer E: Bleeding into the lateral ventricles describes an intraventricular hemorrhage. Intraventricular hemorrhage is associated with premature delivery and usually presents with neurological symptoms. Neurologic findings include seizures, apnea, respiratory depression, or asymmetric reflexes. Treatment may involve urgent surgical decompression to prevent herniation. Bullet Summary: Caput succedaneum is an extracranial injury caused by bleeding between the skin of the scalp and the galea aponeurotica in neonates.
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At a local community hospital staffed only by attending physicians, it is noted that the time to tissue plasminogen activator administration in suspected stroke patients is roughly 10 minutes from presentation. At a teaching hospital with residents, the time to tissue plasminogen activator administration is roughly 2 hours. This has led to many adverse outcomes and increased morbidity at the teaching hospital. Which of the following is the most appropriate next step in further elucidating the problem?
Automatically consult neurology for patients with concerning neurologic symptoms
Empirically scan patients with concerning neurologic symptoms
Improve resident education
Perform a root cause analysis of the process
Perform a systems-based approach implementation
D
Perform a root cause analysis of the process
This case outlines a delay in treating strokes at a teaching hospital. To determine the underlying cause, a root cause analysis to determine the underlying issue is the most appropriate way to begin to address this issue. When a problem is found in healthcare, it is important to elucidate the underlying cause. A root cause analysis is the current preferred method of determining the underlying cause of a problem. A root cause analysis outlines all the people, materials, equipment, and processes in place and outlines them. It then locates the problem and attempts to find the breakdown or issue in all these inputs that lead to the problem. Once the problem is identified, then a strategy or intervention can be implemented to fix the underlying problem. Percarpio et al. review root cause analysis. They note the ubiquity of root cause analysis. They call into question its cost and efficacy despite it being such a widespread practice and recommend a careful assessment of the costs and benefits. Incorrect Answers: Answer A: Automatically consulting neurology for patients with concerning neurologic symptoms may be appropriate if the issue was lack of access to timely neurologist assessment in the workup of stroke. While may hospitals have this as a default for patients with stroke-like symptoms, it would be premature to implement this as a solution without a root cause analysis to first determine what the problem is. Answer B: Empirically scanning patients with concerning neurologic symptoms would be wasteful and not plausible in a healthcare system. There are many concerning neurologic chief complaints including those that originate in the brain, the spine, or the peripheral nerves; thus, merely CT scanning everyone with a neurological chief complaint is an inefficient use of resources. Answer C: Improving resident education is assuming that the problem is a lack of knowledge based on the residents. Before proposing a solution, it is important to first find the underlying cause and address said cause as the delayed intervention may not be the fault of the residents. Answer E: A systems-based approach is the best solution when fixing a medical error. However, a root cause analysis is first needed to determine which solution is needed. If an error in the process is noted, a systems-based approach would be the best method to address it. Bullet Summary: When an error is noted in patient care, the most appropriate initial step in fixing the issue is conducting a root cause analysis.