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001
A 17-year-old girl presents to the emergency department with a headache. The patient has had headaches in the past but this is the worst headache of her life. Her symptoms started yesterday and have been getting progressively worse. The patient states that the pain is mostly on the left side of her head. There has been a recent outbreak of measles at the patient’s school and the patient’s mother has been trying to give her daughter medicine to prevent her from getting sick. Her mother fears that her daughter may have caught measles. Her temperature is 98.6°F (37°C), blood pressure is 123/74 mmHg, pulse is 85/min, and respirations are 13/min. On exam, the patient is an obese girl who is clutching her head with the light in the room turned off. Her neurological exam is within normal limits. Fundoscopic exam reveals mild bilateral papilledema. An MRI of the head is obtained and reveals cerebral edema. A lumbar puncture reveals an increased opening pressure with a normal glucose level. Which of the following is the most likely diagnosis?
{ "A": "Subarachnoid hemorrhage", "B": "Fat-soluble vitamin overuse", "C": "Migraine headache", "D": "Bacterial meningitis", "E": "" }
B
This patient is presenting with headache, bilateral papilledema, obesity, and increased opening pressure on lumbar puncture suggesting a diagnosis of Idiopathic intracranial hypertension (IIH or pseudotumor cerebri). This disease is associated with hypervitaminosis A (a fat-soluble vitamin). Pseudotumor cerebri classically presents in an obese woman with symptoms of headache and double vision. Overuse of supplements containing vitamin A is a risk factor for developing idiopathic intracranial hypertension. Vitamin A is often used to decrease the duration and severity of a measles infection. On exam, patients will have bilateral papilledema, an increased CSF opening pressure, and cerebral edema. A lumbar puncture is the most accurate diagnostic test for this disease. Treatment is with cessation of offending agents and with carbonic anhydrase inhibitors such as acetazolamide to decrease the rate of cerebrospinal fluid production. Optic nerve sheath fenestration can also be performed in patients who fail medical management. Fuerst et al. present evidence regarding patients with hypervitaminosis A. They discuss how these patients can present with pseudotumor cerebri. They recommend being vigilant about vitamin A toxicity. Incorrect Answers: Answer A: Bacterial meningitis presents with a stiff neck, photophobia, fever, and a CSF finding of low glucose in contrast to this patient’s normal glucose. The most common causes of bacterial meningitis in young adults are N. meningitidis or S. pneumonia. Patients should be treated empirically with ceftriaxone and vancomycin because this is a medical emergency. Answer C: Migraine headache can present with a unilateral pulsing/pounding headache and photophobia; however, it would not present with physical exam findings of papilledema or cerebral edema. Acute migraines can be treated with NSAIDs and triptan medications. Chronic prophylaxis is with beta-blockers or calcium channel blockers. Answer D: Subarachnoid hemorrhage presents with the classic, “worst headache of my life,” as this patient has; however, there would be blood visible on MRI which was not described. In addition, there are no symptoms of meningeal irritation from a subarachnoid bleed. Patients may need surgical embolization or clipping of the bleeding vessel. Answer E: Viral meningitis would present with a stiff neck, photophobia, fever, and CSF that would only demonstrate lymphocytic pleocytosis. Though this patient’s CSF glucose is normal, her other symptoms do not point toward a diagnosis of meningitis. Treatment is either supportive or with antiviral agents depending on the causative organism. Bullet Summary: Hypervitaminosis A can cause pseudotumor cerebri, which typically presents in overweight women with headache, double vision, papilledema, and cerebral edema on MRI.
https://bit.ly/3AgFkva
002
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?
{ "A": "CGG trinucleotide repeat disorder", "B": "Anabolic steroid use", "C": "Failure of neuronal migration", "D": "Meiotic nondisjunction", "E": "" }
D
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.
https://bit.ly/3BSwAMD
003
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?
{ "A": "Prednisone", "B": "Aspirin", "C": "Stenting", "D": "Ibuprofen", "E": "" }
B
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.
https://bit.ly/3BV4U9Y
004
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?
{ "A": "Anti-platelet antibodies", "B": "Von Willebrand factor deficiency", "C": "GP1b deficiency", "D": "Factor 8 deficiency", "E": "" }
D
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.
https://bit.ly/3BoKeXI
005
A 69-year-old man presents to his primary care physician for a general checkup. The patient works as a farmer and gardens in his spare time. He has a medical history of poorly managed type 2 diabetes mellitus and irritable bowel syndrome. His current medications include lisinopril, metformin, insulin, fiber supplements, and a multivitamin. On physical exam, there is a calm, obese gentleman. Inspection of the patient's scalp reveals the finding shown in Figure A. The patient is sent home and instructed to keep a blood glucose journal. Three months later, the patient returns with his glucose journal. In addition, he complains of a cut on his scalp that is not seeming to heal. The patient's glucose journal reveals an average blood glucose of 175 mg/dL. His temperature is 98.8°F (37.1°C), blood pressure is 134/86 mmHg, pulse is 80/min, and respirations are 13/min. On inspection of the patient's scalp, the finding in Figure B is noted. Which of the following is the most likely diagnosis?
{ "A": "Basal cell carcinoma", "B": "Squamous cell carcinoma", "C": "Melanoma", "D": "Poor wound healing secondary to diabetes", "E": "" }
B
This patient with a non-healing ulcer on the site of a previous actinic keratosis most likely has sustained progression of the lesion to squamous cell carcinoma. Squamous cell carcinoma (SCC) is a common dermatological malignancy. Patients can present with actinic keratosis, which is a pre-malignant lesion in sun-exposed areas. These lesions can progress to squamous cell carcinoma if risk factors such as sun exposure continue. The presentation of SCC is typically an ulcerative lesion in a sun-exposed area. A biopsy will demonstrate atypical keratinocytes and malignant cells, invasion into the dermis, and keratin “pearls” on histology. Treatment is with wide local surgical excision with histologic confirmation of negative margins. Waldman and Schmults present a review of the evidence regarding the epidemiology and treatment of squamous cell skin cancer. They discuss how the incidence of this disease continues to increase due to an aging population. They recommend local excision and treatment due to the risk of metastasis. Figure/Illustration A is a clinical photograph demonstrating a rough scaly patch on the skin (red circle). This finding is consistent with actinic keratosis, which is a pre-malignant lesion typically found on sun-exposed areas. Figure/Illustration B demonstrates an ulcerative lesion with wound formation on the skin (red circle). This finding is consistent with squamous cell carcinoma, a malignant lesion that can progress from actinic keratosis. Incorrect Answers: Answer A: Actinic keratosis represents a pre-malignant condition that this patient initially presented with (as demonstrated in Figure A). His current presentation reflects SCC, a progression from actinic keratosis given the new ulceration and non-healing wound. Answer B: Basal cell carcinoma presents with a shiny or pearly appearance, rather than the ulcerative appearance that is seen in SCC. This disease should be treated with surgical excision. Answer C: Melanoma presents with a pigmented lesion that has an irregular border, an uneven shape, and areas that differ in pigmentation. Patients with melanoma should undergo surgical staging as this disease can become metastatic and should be treated with surgical excision. Answer D: Poor wound healing secondary to diabetes would be possible if there was a history of trauma to the scalp. The history of actinic keratosis and the current ulcerative lesion, point more toward a diagnosis of SCC. Non-healing wound in patients with diabetes are usually located on the foot rather than on the scalp. Bullet Summary: Actinic keratosis is a pre-malignant lesion that occurs in sun-exposed areas that can progress to squamous cell carcinoma.
https://bit.ly/3CB9Cdn
006
A 76-year-old woman is brought to a primary care physician by her daughter who is concerned about her mother's growing inability to take care of herself. Initially, she attributed her mother's forgetfulness and word-finding difficulties to normal aging, but over the past few years, her mother's memory has worsened, her mood has grown more irritable, and her mother has been found wandering the neighborhood unsure of how to get home. Her medical history includes type 2 diabetes mellitus which is well-controlled with metformin. On exam, her temperature is 98.2°F (36.8°C), blood pressure is 115/82 mmHg, pulse is 73/min, respirations are 12/min, and oxygen saturation is 99% on room air. Cardiopulmonary exam reveals normal S1 and S2, no murmurs, and clear lungs bilaterally. She scores 16/30 on the Montreal Cognitive Assessment (MoCA) test. Which of the following is the most likely diagnosis?
{ "A": "Normal pressure hydrocephalus", "B": "Frontotemporal dementia", "C": "Alzheimer dementia", "D": "Creutzfeldt-Jakob disease", "E": "" }
C
This elderly patient who presents with slowly progressive memory loss, language difficulties, and progression to inability to care for herself most likely has Alzheimer dementia. Alzheimer dementia is a neurodegenerative disorder that is characterized by a gradual and progressive cognitive decline (memory loss, visuospatial and language deficits, impaired judgment), difficulties with executive function or carrying out activities of daily living, and sometimes psychiatric disturbances and behavioral changes (apathy, irritability, social isolation). It is the most common cause of dementia and its risk factors include increasing age, female gender, family history, and trisomy 21 (due to the amyloid precursor protein found on chromosome 21). This is a clinical diagnosis based on cognitive testing (such as the Montreal Cognitive Assessment or the mini-mental status exam) and ruling out other causes of dementia (vitamin B12 deficiency, depression, or hypothyroidism). Weller et al. review the current understanding of Alzheimer dementia pathophysiology. They discuss recent advances in clinical evaluation and treatment. They recommend having a high index of suspicion for the disease in elderly patients who appear to be regressing in their ability to take care of themselves. Incorrect Answers: Answer B: Creutzfeldt-Jakob disease is a rapidly progressive neurodegenerative disorder caused by a prion protein that is fatal, leading to death usually within 1 year of illness onset. Dementia progresses rapidly in this disease and is sometimes associated with startle myoclonus. Answer C: Frontotemporal dementia (FTD) is characterized primarily by disinhibition, apathy, or aphasia as a result of atrophy of the frontal and temporal lobes. It tends to occur at a younger age than does Alzheimer dementia. Behavioral changes are often the first noticeable symptom in FTD while they tend to occur later in Alzheimer disease progression. Problems with visuospatial orientation are more common in Alzheimer dementia than in FTD. Answer D: Normal pressure hydrocephalus (NPH) typically presents with a triad of dementia, gait instability (specifically magnetic gait, marked by an inability to lift feet off the floor), and urinary incontinence. In NPH, excess cerebrospinal fluid accumulates in the brain's ventricles, and treatment involves drainage of that fluid, usually via surgical placement of a shunt. Answer E: Vascular dementia typically presents as stepwise cognitive decline in the setting of stroke or brain injury and usually occurs in patients with the same risk factors as stroke, such as hypertension, hyperlipidemia, peripheral arterial disease, and smoking. This patient has neither hypertension nor stepwise decline. Bullet Summary: Alzheimer dementia presents with slowly progressive dementia, typically manifesting with memory loss, visuospatial and language difficulties, impaired judgment, and inability to care for oneself.
https://bit.ly/3CrrhE1
007
A 69-year-old man presents to his primary care physician for trouble sleeping. He recently retired from working the day shift at a cemetery. When the patient retired, his goal was to finally be able to go out with his wife; however, he finds that he is unable to stay awake past 6 pm in the evening. His wife is disappointed that they cannot do any activities in the evening together. The patient has tried drinking caffeine but finds that it does not help. The patient’s wife claims that the patient seems to sleep peacefully, and the patient states he feels rested when he awakes. The patient has a medical history of irritable bowel syndrome which is managed with fiber supplements. His temperature is 98.6°F (37°C), blood pressure is 125/83 mmHg, pulse is 87/min, and respirations are 11/min. The patient’s neurological exam is within normal limits. Which of the following is the most likely diagnosis?
{ "A": "Normal aging", "B": "Delayed sleep phase disorder", "C": "Chronic insomnia", "D": "Advanced sleep phase disorder", "E": "" }
D
This patient is presenting with difficulty staying awake in the early evening suggesting a diagnosis of advanced sleep phase disorder (also known as advanced sleep phase syndrome). Advanced sleep phase disorder can present in patients of all ages. Patients will experience difficulty staying awake in the early evening (5 to 8 pm); however, they will typically sleep a normal quantity of hours. These patients generally feel well rested in the morning and are able to function normally during the day. This condition can impair social activities that occur in the evening, which can cause distress to the patient. The diagnosis of advanced sleep phase disorder can be made when these symptoms are present in addition to an absence of another organic cause (such as obstructive sleep apnea). Treatment is with bright light visual stimulation therapy and reconditioning of circadian rhythms. Yaremchuk reviews the diagnosis and treatment of patients with sleep phase disorder. She discusses how sleep disorders such as advanced sleep phase disorder should be distinguished from normal aging. She recommends intervening when sleep quality is compromised as it reduces quality of life in the elderly. Incorrect Answers: Answer B: Chronic insomnia will present with trouble falling asleep or staying asleep, often alternating with other sleep pathologies. This patient does not claim to have any trouble falling or staying asleep. He also feels well rested in the morning so his sleep quality is adequate. Treatment of insomnia may include melatonin and sleep hygiene. Answer C: Delayed sleep phase disorder typically presents in younger patients with difficulty falling asleep early. These patients will often fall asleep very late and struggle to wake up early for obligations such as school or work. Treatment is with melatonin and sleep hygiene interventions. Answer D: Normal aging presents with patients claiming that their sleep is less restful in the absence of an organic cause (such as obstructive sleep apnea). As patients age, it is natural for all phases of their sleep to shorten. However, it is not normal for this patient to have such difficulty staying awake in the early evening, so advanced sleep phase disorder is a more likely diagnosis. Answer E: Obstructive sleep apnea presents with fatigue, morning headaches, and non-restful sleep. Patients will typically snore loudly and typically are overweight. This patient does not have any of these risk factors or symptoms in his history. Treatment is with positive airway pressure machines and weight loss. Bullet Summary: Advanced sleep phase disorder presents with difficulty staying awake in the early evening that impairs social functioning.
https://bit.ly/3CxfeFC
008
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?
{ "A": "Vitamin B6 supplementation", "B": "Changing to a soy based formula", "C": "Providing imiglucerase enzyme replacement", "D": "Removing phenylalanine from maternal diet during pregnancy", "E": "" }
B
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.
https://bit.ly/3D10TBk
009
A 4-week-old boy presents with his parents to the pediatrician for a well-child visit. His mother reports he was eating well until 1 week ago when he began vomiting after breastfeeding. His mother has tried increasing the frequency of feeds and decreasing the amount of each feed, but the vomiting seems to be getting worse. The patient now vomits after every feed. The vomitus looks like breast milk. The patient’s mother is exclusively breastfeeding and would prefer not to switch to formula but worries that the patient is not getting the nutrition he needs. Two weeks ago, the patient was in the 75th percentile for weight and 70th for height. He is now in the 60th percentile for weight and 68th percentile for height. His temperature is 98.2°F (36.8°C), blood pressure is 58/37 mmHg, pulse is 144/min, and respirations are 34/min. On physical exam, the patient has dry mucous membranes. His abdomen is soft and non-distended. Which of the following is the most appropriate next step in management?
{ "A": "Abdominal ultrasound", "B": "Trial of empiric proton pump inhibitor", "C": "Abdominal radiograph", "D": "Supplement breastfeeding with formula", "E": "" }
A
This patient presents with a history of nonbilious, forceful vomiting, poor weight gain, and signs of dehydration, which is consistent with pyloric stenosis. The most appropriate next step in management is abdominal ultrasound to confirm the diagnosis. Pyloric stenosis presents in 4- to 6-week-old infants with nonbilious, post-prandial projectile vomiting. Although pyloric stenosis classically presents with an “olive-shaped” mass in the epigastrium, the mass cannot be palpated in some patients. Any infant who develops new, persistent vomiting in the first 2 months of life and has associated dehydration or failure to thrive should undergo an abdominal ultrasound to evaluate for pyloric stenosis. Patients should undergo resuscitation prior to the definitive treatment, which is a pyloromyotomy. Adams and Stanton review the evidence regarding the diagnosis and treatment of neonatal bowel obstruction. They discuss how malrotation, jejunoileal atresia, duodenal atresia, and colonic atresia are common causes of this finding. They recommend surgical management of these conditions. Incorrect Answers: Answer A: Abdominal radiograph is not helpful in evaluating for pyloric stenosis. It is more useful in the workup of bilious vomiting, as several etiologies of bilious vomiting (such as duodenal atresia and Hirschsprung disease) can be seen on an abdominal radiograph. Treatment of these conditions is surgical excision of the defective segment of colon. Answer C: Supplementing breastfeeding with formula would not be appropriate for this patient as his failure to thrive is driven by gastric outlet obstruction. In the case of pyloric stenosis, formula would cause vomiting just as breastmilk does. Answer D: A trial of a dairy-free diet is used to improve gastroesophageal reflux, but in the setting of this patient’s age and the description of vomiting, he should undergo an abdominal ultrasound to evaluate for pyloric stenosis. Treatment of reflux also involves positional changes during feeding. Answer E: A trial of empiric proton pump inhibitor is indicated for patients with gastroesophageal reflux disease, but it would not address this patient’s pyloric stenosis. Patients can also undergo conservative treatments such as changing positioning during feeding. Bullet Summary: The diagnostic test of choice for pyloric stenosis in infants is an abdominal ultrasound.
https://bit.ly/3D2V2LR
010
A 27-year-old woman presents for her first prenatal visit at an estimated gestational age of 16 weeks and 4 days by last menstrual period. She is presenting late to prenatal care due to significant anxiety about her pregnancy, as she and her husband had struggled with infertility for several years prior to conceiving. She has had nausea and vomiting for about 2 months, tender and swollen breasts, and cravings for foods she typically does not eat. She took a pregnancy test about 10 weeks ago and was too nervous to read the result immediately. After 24 hours, she saw that it was positive. The patient has a past medical history of bulimia nervosa but has not been symptomatic in 2 years. She also had an appendectomy at age 15 for appendicitis. She is a Ph.D. student and her husband is a physician. Her temperature is 98.5°F (36.9°C), pulse is 75/min, blood pressure is 122/76 mmHg, and respirations are 13/min. The patient’s affect is normal and she is pleasant and excited about her pregnancy. A physical exam is notable for abdominal distension but is otherwise unremarkable. Ultrasound is shown in Figure A, and urine human chorionic gonadotropin is negative in the office. Which of the following is the most likely diagnosis?
{ "A": "Pseudocyesis", "B": "Couvade syndrome", "C": "Feigned pregnancy", "D": "Recent miscarriage", "E": "" }
A
This patient presents with signs and symptoms of pregnancy but a nongravid uterus on ultrasound and a negative pregnancy test. The most likely diagnosis is pseudocyesis. Pseudocyesis, or false pregnancy, most commonly presents with abdominal distension, breast tenderness, nausea, and other typical symptoms of pregnancy. Patients truly believe they are pregnant and may report positive pregnancy tests at home (perhaps due to false positives from waiting too long before reading the result) but office testing is negative. Ultrasound will be normal and show the absence of a developing fetus. The mainstay of treatment is explaining the diagnosis in a therapeutic manner and offering counseling to help patients cope. Small reviews the evidence regarding the diagnosis and treatment of pseudocyesis. He discusses how this disease is a heterogenous entity involving neuroendocrine changes. He recommends clearly defining the cause of this disease in patients. Figure/Illustration A shows a transabdominal ultrasound with an empty cavity without signs of pregnancy (red circle). These findings are consistent with pseudocyesis. Incorrect Answers: Answer A: Couvade syndrome refers to a “sympathetic pregnancy,” in which a patient experiences pregnancy symptoms when someone close to her is pregnant. This does not apply to this patient because she believes that she is pregnant herself. Answer B: Delusion of pregnancy is a false and fixed belief about being pregnant, despite factual evidence to suggest otherwise. Patients typically do not experience actual pregnancy symptoms and may have a history of psychotic disorders, both of which are inconsistent with this patient’s presentation. Treatment is with cognitive behavioral therapy. Answer C: Feigned pregnancy is the intentional falsifying of symptoms for a clear external motive and is a subtype of factitious disorder or malingering. This patient does not express any desire for secondary gain and seems to truly believe that she is pregnant, which is more consistent with pseudocyesis. Answer E: A recent miscarriage is unlikely, as urine hCG testing often takes up to a month to become negative after pregnancy loss. Furthermore, the patient reports that her symptoms have been consistently present, whereas a miscarriage would typically cause a cessation of pregnancy symptoms. Bullet Summary: Pseudocyesis is a somatic symptom disorder in which women believe they are pregnant and experience pregnancy symptoms without an actual pregnancy.
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011
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?
{ "A": "Observation", "B": "Balloon catheter removal", "C": "Surgical extraction", "D": "Endoscopic removal", "E": "" }
D
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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012
A 54-year-old man presents to the clinic with 4 weeks of persistently worsening back pain. The pain is localized to 1 spot in his lower back and is worse with physical activity. His medical history is pertinent for intravenous drug use. He has no past surgical history. His temperature is 36.8°C (98.2°F), blood pressure is 118/90 mmHg, pulse is 92/min, respirations are 13/min, and oxygen saturation is 99% on room air. On physical exam, he has midline back tenderness to palpation at L2-L3. Laboratory workup reveals an erythrocyte sedimentation rate of 112 mm/h and C-reactive protein of 10 mg/dL. Which of the following is the most likely diagnosis?
{ "A": "Ankylosing spondylitis", "B": "Osteomyelitis", "C": "Degenerative spine disease", "D": "Metastatic tumor", "E": "" }
B
This patient with a history of intravenous drug use who presents with worsening focal back pain and elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) most likely has vertebral osteomyelitis. Vertebral osteomyelitis typically occurs via three routes: hematogenous spread from a distant site (the most common mechanism), direct inoculation from spinal surgery or trauma, or contiguous spread from adjacent soft tissue infection. Patients usually present with back or neck pain, with or without fever, and the most common clinical finding is local tenderness to percussion over the involved posterior spinous process. Risk factors include injection drug use, degenerative spine disease, prior spinal surgery, infective endocarditis, diabetes mellitus, corticosteroid therapy, and other immunocompromised states. Vital signs may be normal, and laboratory evaluation commonly reveals an elevated white blood cell count, ESR, and CRP. An MRI of the spine is the most appropriate diagnostic test. In stable patients, antimicrobial therapy should be withheld until a microbiological diagnosis is confirmed; once the diagnosis is confirmed, pathogen-directed therapy should then be administered. Beronius et al. conducted a retrospective study on vertebral osteomyelitis in pursuit of diagnostic criteria to simplify the diagnosis and classification of vertebral osteomyelitis. The authors found that elevated ESR and CRP are found in most patients with vertebral osteomyelitis. The authors recommend clinicians have a high index of suspicion for vertebral osteomyelitis in order to avoid delayed diagnosis. Incorrect Answers: Answer A: Ankylosing spondylitis is an inflammatory arthritis of the spine and is often associated with one or more articular or periarticular extraspinal features, including synovitis, dactylitis, and enthesitis. Patients typically present with chronic back pain and loss of mobility before the age of 45. Answer B: Degenerative spine disease involves osteoarthritis of the spine and is associated with increasing age, presenting commonly as sharp or chronic pain in the neck or back. It typically does not result in radiculopathy, defined as irritation or injury of a nerve root, causing pain, weakness, numbness, or tingling in specific distributions depending on the location of the affected nerve root. Though it is certainly on the differential for this patient, his elevated ESR and CRP make osteomyelitis a more likely diagnosis. Answer C: Herniated disc can cause a variety of symptoms depending on the position and size of the herniation. If the herniated disk impinges on a nerve, patients may have radiculopathy. Answer D: Metastatic tumor is typically suspected in patients with back pain in the setting of a known malignancy. The most common primary cancers that metastasize to bone include breast, prostate, thyroid, lung, and renal cancer. Bullet Summary: Vertebral osteomyelitis typically presents as focal back pain, with or without fever, with an elevated erythrocyte sedimentation rate and C-reactive protein.
https://bit.ly/3DkrY2C
013
A 27-year-old man presents to his primary care physician for a checkup. He has no health concerns and has not seen a physician in years. The patient has a medical history of depression treated with fluoxetine and lithium. His temperature is 99.5°F (37.5°C), blood pressure is 122/78 mmHg, pulse is 90/min, respirations are 13/min, and oxygen saturation is 98% on room air. Physical exam is notable for the finding in Figure A. Which of the following represents the most likely risk factor for this patient's presenting condition?
{ "A": "Alcohol consumption", "B": "Antibiotic use", "C": "Intravenous drug use", "D": "Sexual intercourse", "E": "" }
A
This patient is presenting with adherent white plaques on the inside of the cheek consistent with oral leukoplakia. Alcohol consumption is a risk factor for this disease. Oral leukoplakia presents with a white patch/plaque that occurs over the oral mucosa that is adherent and non-scrapable. Risk factors for this condition include the risk factors for squamous cell carcinoma such as consumption of alcohol and smoking. Though the cause of this lesion is largely idiopathic, any irritating substance can induce it. Other potential risk factors include candidiasis, vitamin deficiency, endocrine disturbances, and viral infections. A diagnosis can be made using a biopsy showing the degree of dysplasia. Treatment is with smoking and alcohol cessation as well as surgical removal for suspicious lesions. Mohammed and Fairozekhan review the evidence regarding the diagnosis and risk factors for leukoplakia. They discuss how this disease is associated with smoking and alcohol use. They recommend screening patients for these modifiable risk factors. Figure/Illustration A is a clinical photograph demonstrating adherent white plaques on the inside of the cheek (red circle). These findings are consistent with oral leukoplakia. Incorrect Answers: Answer B: Antibiotic use is a risk factor for candidiasis; however, this patient is not presenting with symptoms of candidiasis which is itself a risk factor for oral leukoplakia. Candidiasis presents with opaque white lesions that can be scraped off. Answer C: Intravenous drug use does make this patient riskier overall for many conditions; however, alcohol is a more direct risk factor for oral leukoplakia. Immunodeficiency conditions can cause widespread fungal and viral infections on the mucosal surfaces. Answer D: Poor dental hygiene may be found more often in patients with oral leukoplakia; however, it is not itself a direct cause. Answer E: Sexual intercourse would predispose this patient to infections and viruses (such as Epstein-Barr virus); however, it is a less likely risk factor than is alcohol consumption or smoking. These infections tend to present with ulcers more commonly than leukoplakia. Bullet Summary: Risk factors for oral leukoplakia and squamous cell carcinoma include alcohol consumption and smoking.
https://bit.ly/3FB3X8u
014
A 47-year-old man is brought in after a motor vehicle accident. He was driving under the influence of alcohol when he sustained a crash against a pole. His medical history is significant for hypertension but he does not take any medications. 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 a ventilator. His Glasgow coma score (GCS) on presentation is 6. Initial urine toxicology is negative. The patient is admitted to the intensive care unit and appropriate care is initiated. One week later, the patient’s GCS score is now 3. Laboratory studies do not show significant electrolyte, acid-base, or endocrine disturbances. Sedative and paralytic agents are withdrawn. Which of the following findings on physical exam would be compatible with brain death?
{ "A": "Conjugate eye movement to the left with rotation of the head to the right", "B": "Grimacing after noxious stimulus to the arm", "C": "Flexion of the thigh, leg, and foot upon noxious stimulation of the foot", "D": "Spontaneous respiration 1 minute after disconnection from a ventilator", "E": "" }
C
This patient with a Glasgow coma score (GCS) of 3 and a known mechanism of injury due to motor vehicle accident trauma is normothermic, normotensive, not on sedatives, and without laboratory abnormalities so they are appropriate for examination of brain death. Cortical and brain stem function must be absent in brain death; however, spinal cord reflexes such as triple flexion (flexion of the thigh, leg, and foot upon noxious stimulation of the foot) may remain intact in patients with brain death. The clinical brain death exam may be performed in patients with evidence of irreversible central nervous system catastrophe after a known mechanism of trauma or medical injury. The patient must not have other causes for obtundation, and should be euthermic, not on sedative or paralytic agents, not be intoxicated due to drugs or poisoning, and not have other severe electrolyte, acid-base, or endocrine disturbances. In brain death, there must be no cerebral motor response such as moaning or grimacing to pain in all extremities and the face. Brain stem reflexes such as the pupillary light reflex, corneal reflex, oculocephalic reflex, vestibulo-ocular reflex, gag reflex, and apnea test should also be absent in brain death. Spinal cord and deep tendon reflexes may still be present in brain death. Complex movements mediated by the spinal cord such as triple flexion and the Lazarus sign (raising the arms and dropping them crossed on the chest after noxious stimulus) do not preclude a diagnosis of brain death. Greer et al. review neurologic criteria for the determination of brain death. They discuss how to clinically differentiate spinal motor responses from brain-mediated motor responses. They recommend understanding these distinctions in order to properly examine patients with brain death. Incorrect Answers: Answer A: Blinking after saline is applied to the cornea indicates a positive corneal reflex. In the corneal reflex, the ophthalmic branch of the trigeminal (V1) nerve senses stimulus to the cornea, and bilateral temporal branches of the facial nerve (VII) activate the orbicularis oculi muscles to blink. Brainstem reflexes must be absent in brain death, so the presence of a corneal reflex is not compatible with a diagnosis of brain death. Answer B: Conjugate eye movement to the left with rotation of the head to the right describes the oculocephalic reflex. In this reflex, movement of the head stimulates endolymph in the horizontal semicircular canal to rotate, causing the firing of the ipsilateral vestibulocochlear (VIII) nerve. This leads to activation or inhibition of the contralateral abducens (VI) and oculomotor (III) nerves. Brainstem reflexes must be absent in brain death, so the presence of an oculocephalic reflex is not compatible with a diagnosis of brain death. Answer D: Grimacing after the noxious stimulus of the arm indicates some residual cortical function. Grimacing is not a spinal cord reflex and requires some cortical processing of sensory information. All cortical function must be absent in brain death, so grimacing to pain would not be compatible with brain death. Answer E: Spontaneous respiration 1 minute after disconnection from a ventilator in a patient without diabetes insipidus and with normal blood gas values and systolic blood pressure above 90 mmHg indicates a negative apnea test. The apnea test assesses respiratory control system reflexes in the brainstem. Apnea testing is an important component of the brain death exam, and a negative apnea test is incompatible with brain death. Bullet Summary: Although brain death criteria universally include the absence of cortical and brainstem function, spinal cord and deep tendon reflexes may be preserved in patients with brain death.
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015
A 67-year-old man presents to the emergency department 1 hour after sudden-onset weakness and double vision. His medical history is significant for hyperlipidemia and poorly controlled hypertension. His current medications include atorvastatin and amlodipine. His temperature is 98.6°F (37.0°C), blood pressure is 175/95 mmHg, pulse is 90/min, respirations are 16/min. On cranial nerve exam, his left pupil is 6 mm in diameter compared to 3 mm on the right. His left eye is displaced laterally and downward and he also has left-sided ptosis. Strength is 2/5 in the right upper and right lower extremities and 5/5 in the left upper and left lower extremities. He has marked right-sided dysdiadochokinesia. Sensation is intact throughout. Which of the following regions of his brain is most likely affected?
{ "A": "Lateral medulla", "B": "Lateral pons", "C": "Posterior limb of internal capsule", "D": "Midbrain", "E": "" }
D
This patient with a history of poorly controlled hypertension, acute onset of left-sided oculomotor nerve palsy (mydriasis, ptosis, and “down and out” pupil), right-sided paresis, and right-sided dysdiadochokinesia most likely has a lacunar stroke of the left midbrain. Important structures in the midbrain often affected by stroke include the oculomotor nucleus, substantia nigra, corticospinal tract, red nucleus, and superior cerebellar peduncle. Occlusions to branches of the basilar artery lead to infarctions of various parts of the midbrain. Midbrain lesions almost always manifest with an ipsilateral oculomotor palsy (ptosis from the levator palpebrae muscle; mydriasis from loss of parasympathetic inflow; abduction and excyclotorsion of the globe from the superior rectus, medial rectus, inferior rectus, and inferior obliques). There may also be contralateral ataxia (cerebellar peduncle after decussation and red nucleus), contralateral hemiparesis (corticospinal tract before decussation at the medulla), and parkinsonian tremor (substantia nigra). Sciacca et al. review the anatomy and clinical signs of strokes of the midbrain, pons, and medulla. They discuss how a stroke of the midbrain will present with oculomotor palsy and paresis. They recommend being vigilant for symptoms of strokes to allow for early diagnosis. Incorrect Answers: Answer A: Lateral medullary syndrome (Wallenberg syndrome) is usually due to a stroke of the posterior inferior cerebellar artery. This presents with ipsilateral ataxia, loss of pain and temperature sensation in the face, dysarthria, and Horner syndrome, as well as contralateral loss of pain and temperature sensation in the body. In this patient with intact sensorium and without dysarthria, lateral medullary syndrome is less likely. Answer B: Lateral pontine syndrome can be caused by interruption of blood supply by the anterior inferior cerebellar artery. It presents with ipsilateral limb and gait ataxia, ipsilateral Horner syndrome, ipsilateral facial hemianesthesia, ipsilateral facial paralysis, nystagmus and vertigo, and contralateral loss of pain and temperature in the body. In this patient with intact sensorium and without facial paralysis, vertigo, and oculomotor palsy, midbrain stroke is more likely than a pontine stroke. Answer C: Medial pontine syndrome can be due to occlusion of the paramedian branches of the basilar artery and disrupts the abducens nerve, the dorsal column-medial lemniscus, and the corticospinal tract. This results in contralateral spastic hemiparesis, ipsilateral lateral rectus paralysis (eye turned down and in), and contralateral loss of vibration and stereognosis. In this patient with ptosis, and an eye turned down and out, oculomotor palsy and a midbrain stroke are more likely than a pontine stroke. Answer E: Strokes of the posterior limb of the internal capsule lead to contralateral hemiparesis. Strokes of the internal capsule would not be expected to lead to oculomotor nerve palsy or dysdiadochokinesia. The constellation of ataxia, hemiparesis, and cranial nerve palsy is more suggestive of a brainstem pathology than of a lacunar stroke in the internal capsule. Bullet Summary: Midbrain strokes commonly present with ipsilateral oculomotor nerve palsy and contralateral hemiparesis/hemiplegia, contralateral hemiataxia, and/or contralateral tremor.
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016
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?
{ "A": "Gastroenteritis", "B": "Intentional contamination", "C": "Meningitis", "D": "Urinary tract infection", "E": "" }
B
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/3GZtkBx
017
A 55-year-old man presents to urgent care for weakness and weight loss. For the past several months, he has felt progressively weaker and has lost 25 pounds with intermittent abdominal pain. The patient has not seen a physician in 30 years and recalls being current on most of his vaccinations. A few years ago, he went to the emergency department due to abdominal pain and was found to have increased liver enzymes due to excessive alcohol use and incidental gallstones. The patient has a 50 pack-year smoking history. His temperature is 99.5°F (37.5°C), blood pressure is 161/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals an emaciated man. The patient has a negative Murphy sign and his abdomen is non-tender. Cardiopulmonary exam is within normal limits. Which of the following is the most appropriate next step in management?
{ "A": "CT scan of the abdomen and pelvis", "B": "CT scan of the chest", "C": "HIDA scan", "D": "Smoking cessation advice and primary care follow up", "E": "" }
A
This patient with weight loss and painless liver dysfunction has symptoms that are suggestive of pancreatic cancer. The most appropriate next diagnostic test is a CT scan of the abdomen and pelvis. Pancreatic cancer presents with weight loss, jaundice, epigastric pain, and fatigue/malaise typically in an older male smoker who uses alcohol. Laboratory tests will demonstrate an elevated alkaline phosphatase and bilirubin. If a patient is presenting with the classic "painless jaundice" then the most appropriate next step in management could be an ultrasound to evaluate for obstruction. Otherwise, the best diagnostic test for this condition is a CT scan of the abdomen to characterize the pancreas and any metastasis to local organs. Treatment is with excision of the tumor through a Whipple procedure in appropriate candidates. Lee and Lee review the evidence regarding the diagnosis of pancreatic cancer. They discuss how abdominal CT or MRI are important in making this diagnosis. They recommend having a high clinical index of suspicion given this diagnosis carries a high mortality rate. Incorrect Answers: Answer B: CT scan of the chest would be appropriate to screen a smoker for lung cancer. If this patient had concern for malignancy with a smoking history, then a CT scan of the chest would be appropriate. It would be appropriate at a later time to assess for pulmonary nodules and lung cancer. Answer C: HIDA (hepatobiliary iminodiacetic acid) scan is an appropriate diagnostic test to characterize a gallstone blockage of the common bile duct if a right upper quadrant ultrasound is not revealing in the diagnosis of acute cholecystitis. Answer D: A right upper quadrant ultrasound can assess the gallbladder for stones and the liver for an abscess, but it will not assess the pancreas for pancreatic cancer (at least in a reliable manner that assesses the entire pancreas). Answer E: Smoking cessation and primary care follow up would be appropriate management for this patient if he only presented requesting general advice to improve his health or stop smoking. His current symptoms are too alarming for malignancy. He should stop smoking regardless. Bullet Summary: The most appropriate initial test for pancreatic cancer is an abdominal CT.
https://bit.ly/3GigfDn
018
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?
{ "A": "Borderline", "B": "Dependent", "C": "Avoidant", "D": "Antisocial", "E": "" }
A
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.
https://bit.ly/3HvRZyr
019
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?
{ "A": "Ductal carcinoma in situ", "B": "Mammary duct ectasia", "C": "Invasive ductal carcinoma", "D": "Intraductal papilloma", "E": "" }
D
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.
https://bit.ly/3IgfxrD
020
A 45-year-old man presents to his primary care physician with a rash. He first noticed the rash 2 weeks prior and he endorses intermittent itchiness from the rash. His medical history is significant for chronic hepatitis C infection, hypertension, type 2 diabetes mellitus, and heart failure. The patient works as a butcher at a local meat processing shop and is sexually active with several partners. He does not use barrier protection. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 155/95 mmHg, and respirations are 12/min. On physical exam, his skin has the finding shown in Figure A. Fine, lace-like white lines are also noted on the patient’s buccal mucosa. Which of the following is the most likely diagnosis?
{ "A": "Atopic dermatitis", "B": "Lichen planus", "C": "Scabies", "D": "Psoriasis", "E": "" }
B
This patient presents with several purple, polygonal, pruritic papules and plaques. These findings are most consistent with a diagnosis of lichen planus. Lichen planus is an inflammatory skin disorder most commonly characterized by an eruption of Purple, Polygonal, Planar, Pruritic, Papules and Plaques (the 6 P’s), often on extensor surfaces such as the wrist. Development of fine white lines on the surfaces of skin lesions, as well as on the buccal mucosa (Wickham striae), are also characteristically seen in this disease. Concurrent hepatitis C infection is a risk factor for lichen planus. A skin biopsy will show a sawtooth infiltrate of lymphocytes at the dermal-epidermal junction. Treatment is with antihistamines for pruritus and topical steroids for localized disease as well as addressing the underlying cause. Tziotzios et al. review the evidence regarding the diagnosis and treatment of lichen planus. They discuss how the molecular basis of this disorder is relatively unknown. They recommend making a clear diagnosis to allow for treatment. Figure/Illustration A shows multiple purple, polygonal, papules and plaques on the wrist (red circles). These findings are characteristic of lichen planus. Incorrect Answers: Answer A: Atopic dermatitis most commonly presents with pruritic, lichenified plaques on the flexural surfaces. The mucous membranes are not involved. Treatment is with topical moisturizers and antihistamines for pruritus. This disease is also associated with other atopic conditions such as asthma and seasonal allergies. Answer C: Prurigo nodularis is characterized by hypertrophic, dome-shaped papules that develop secondary to chronic scratching. The mucous membranes are not involved. Treatment is with avoidance of the triggering behavior such as scratching and antihistamines for pruritus. Answer D: Psoriasis is characterized by silver, scaly plaques that bleed easily when the scale is physically removed (Auspitz sign). Patients with cutaneous psoriasis are at increased risk for the development of psoriatic arthritis. Psoriasis can be treated with topical corticosteroids as well as calcipotriene. Answer E: Scabies is a cutaneous infection with the mite Sarcoptes scabei that presents with severe pruritus, especially in the intertriginous areas. Patients may have serpiginous scaling lesions that represent the burrowing of the mite through the skin, but mucous membrane lesions are absent. Treatment can include topical permethrin first line. Bullet Summary: Lichen planus presents with the 6 P’s – Purple, Pruritic, Polygonal, Planar, Papules and Plaques.
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021
A 40-year-old south asian man presents to a primary care provider with a chronic cough that is worse at night. He has had the cough for several years but it has been getting worse over the last few months. He recently moved to the United States to work in construction. He has lost 10 pounds in the last 3 months along with darker stools which he believes is caused by a Western diet. He denies any difficulty swallowing or feeling of food getting stuck in his throat. He drinks alcohol 1-2 times per week and has never smoked. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 114/72 mmHg, pulse is 82/min, and respirations are 12/min. On physical exam, his lungs are clear to auscultation bilaterally without wheezing. His abdomen is soft and non-distended. He has no tenderness to palpation and bowel sounds are present. Which of the following is the most appropriate next step in management?
{ "A": "Barium swallow", "B": "Upper endoscopy", "C": "Helicobacter pylori stool antigen test", "D": "Trial of lansoprazole", "E": "" }
B
This patient presents with a chronic cough that is worse at night, which is consistent with gastroesophageal reflux disease (GERD). In light of this patient’s new weight loss and melena, the most appropriate next step in management would be upper endoscopy to rule out cancer. GERD may present as a chronic or nighttime cough, substernal or epigastric pain, regurgitation, nausea, or bitter taste in the mouth. Symptoms are typically worse after eating or lying down. Patients with typical GERD should undergo upper endoscopy if they have concerning symptoms, including dysphagia, odynophagia, vomiting, weight loss, gastrointestinal bleeding, or melena. They should also undergo upper endoscopy if they are men over the age of 50 with chronic symptoms (>5 years) and cancer risk factors, such as a history of tobacco use or a family history of cancer. Young et al. review the evidence regarding the treatment of GERD. They discuss how patients with concerning symptoms or those who are failing symptomatic treatment should undergo upper endoscopy. They recommend treatment with a proton pump inhibitor in patients without alarm symptoms. Incorrect Answers: Answer A: A barium swallow would be indicated in a patient who presented with symptoms of esophageal dysphagia. Possible etiologies would include Zenker diverticulum or achalasia. This patient denied any difficulty swallowing or feeling of food being stuck in the throat. Answer B: Colonoscopy could be considered in this patient if his upper endoscopy is unrevealing, as weight loss and melena could be explained by colorectal cancer. In light of this patient’s GERD, upper endoscopy should be performed first. Answer C: Helicobacter pylori testing can be useful in the workup of gastric and duodenal ulcers but is not usually performed in the evaluation of GERD. Weight loss and melena raise concerns for carcinoma. Treatment of patients with H. pylori includes a proton pump inhibitor, clarithromycin, and metronidazole. Answer D: A trial of lansoprazole would not be appropriate for this patient presenting with several worrisome symptoms including melena and weight loss. If the patient had presented with typical GERD without these symptoms, a trial of a PPI would be indicated. Cancer should be ruled out first prior to symptomatic treatment. Bullet Summary: Patients with gastroesophageal reflux disease who present with alarm symptoms of weight loss, dysphagia, odynophagia, vomiting, gastrointestinal bleeding, or melena should be evaluated with upper endoscopy.
https://bit.ly/3KuUPFj
022
A 6-week-old boy is referred to a pediatric hematologist-oncologist for further evaluation following an abnormal newborn hemoglobinopathy screening. The father’s brother died of sickle cell anemia at an early age. Parental studies reveal that both mother and father are carriers of the sickle cell gene. The patient's temperature is 98.6°F (37.0°C), blood pressure is 80/45 mmHg, pulse is 130/min, and respirations are 25/min. The physician decides to repeat testing for the patient and his 3-year-old sister. The results are shown in Figure A. The physician begins counseling the family. Which of the following is the most likely complication that the patient may have in the future?
{ "A": "Stroke", "B": "Hematuria", "C": "Splenectomy", "D": "Infection", "E": "" }
B
Based on the presence of both hemoglobin (Hb) A and S on gel electrophoresis, this patient has sickle cell trait. Episodes of painless hematuria are commonly seen in these patients. Sickle cell trait is a condition that refers to carrying 1 normal and 1 abnormal beta-globin allele. On electrophoresis, this will present as 3 different bands consisting of about 50-60% HbA, 35-45% HbS, and < 2% HbF (fetal hemoglobin). HbF decreases significantly after the first 6 weeks of life. Individuals with sickle cell trait tend to be asymptomatic and do not have a decreased lifespan compared to the general population. However, they are at higher risk of some conditions. Patients are at increased risk of rhabdomyolysis during strenuous physical activity and may have episodic painless hematuria due to renal papillary necrosis. The hyperosmolar, acidotic, and hypoxic environment of the renal medulla is thought to trigger red cell sickling which results in ischemia and subsequent hematuria. Patients with sickle cell trait are managed similarly to the general population. These patients should receive reproductive counseling and use appropriate preventive measures to prevent dehydration during intensive exercise. Hulsizer et al. studied the association between sickle cell trait and common medical conditions using insurance claims data. The authors found that sickle cell trait is associated with increased risks of diabetes, hypertension, heart disease, chronic kidney disease, and retinopathy. The authors recommend further evaluation of these associations using prospective studies derived from clinical data. Figure/Illustration A shows gel electrophoresis with an example of a normal (95-98% HbA and < 2% HbF), sickle cell trait (50-60% HbA, 35-45% HbS, and < 2% HbF), and sickle cell anemia (85-95% HbS and 5-15% HbF) patient from left to right. Incorrect Answers: Answer A: Diabetes has not been associated with sickle cell trait in the majority of studies. Answer C: Infection is a complication of sickle cell anemia. Mechanisms include functional asplenism, reduced tissue perfusion during a sickle crisis, and chronic transfusions with an indwelling catheter. Common infections include bacteremia, meningitis, and pneumonia due to Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitis. Sickle cell trait is not clearly associated with an increased risk of infection and actually confers some protection against malaria. Answer D: Splenectomy from auto-infarction is often seen in sickle cell anemia, but it is rare with sickle cell trait. In sickle cell anemia, splenic sequestration of sickled red blood cells causes infarction, leading to progressive atrophy and functional hyposplenism. Most individuals with sickle cell disease are functionally asplenic by age 2 to 4. The spleen subsequently cannot phagocytose encapsulated organisms properly, which predisposes sickle cell patients to infection. Answer E: Stroke is a complication of sickle cell anemia but not sickle cell trait. This is due to the increased viscosity of the blood and reduced deformability of red cells in patients with sickle cell anemia. Bullet Summary: Sickle cell trait is associated with episodes of painless hematuria.
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023
A 4-day-old girl is brought in by her mother for bilateral purulent eye discharge. Pregnancy and delivery were uncomplicated. The newborn had been sleeping regularly and feeding well. Then 1 day ago, the patient developed eyelid edema and eye redness. This morning, the newborn developed a thick yellow-white eye discharge. The mother denies that anyone in the house smokes. The family has a cat at home. The patient’s rectal temperature is 99°F (37.2°C), blood pressure is 80/50 mmHg, pulse is 134/min, and respirations are 34/min with an oxygen saturation of 98% O2 on room air. Figure A shows a photograph of one of the patient's eyes. The patient's physical exam is otherwise unrevealing. Which of the following most appropriately would have prevented this infant's condition?
{ "A": "Topical erythromycin", "B": "Topical bacitracin", "C": "Artificial tears", "D": "Oral erythromycin", "E": "" }
A
This patient is presenting with bilateral purulent eye discharge 4 days post-birth, suggesting the diagnosis of gonococcal conjunctivitis. Standard prophylaxis for this condition is topical erythromycin ointment. Neonatal gonococcal conjunctivitis is caused by Neisseria gonorrhea and presents in newborns that are 0-5 days old. Symptoms include purulent discharge and eye swelling. Standard prophylaxis for gonococcal conjunctivitis is erythromycin ointment. Silver nitrate drops can also be used as prophylaxis, but this treatment has been abandoned in most practices as it can cause chemical conjunctivitis. Treatment for gonococcal conjunctivitis includes intravenous or intramuscular ceftriaxone. All neonates with gonococcal conjunctivitis should also be treated for chlamydia. Franco and Hammerschlag prevent the current methods of providing prophylaxis against gonococcal conjunctivitis. They discuss how the development of macrolide resistance may make erythromycin ointment ineffective. They recommend screening and treatment of pregnant women to prevent the development of this disease. Figure/Illustration A shows a newborn's eye that is exhibiting eyelid swelling, redness, and purulent discharge (red circle). These findings are consistent with gonococcal conjunctivitis. Incorrect Answers: Answer A: Artificial tears can be used in the management of chemical conjunctivitis. The most common cause of neonatal chemical conjunctivitis is post-delivery use of silver nitrate drops. Symptoms usually present within the first 24 hours following birth/exposure as mild conjunctival injection and tearing without purulent discharge. Answer B: Intramuscular ceftriaxone is used as treatment, not prophylaxis, of neonatal gonococcal conjunctivitis. This disease would present with purulent discharge and eye swelling. The goal of prophylaxis is to prevent the development of this condition. Answer C: Oral erythromycin is used to treat neonatal chlamydial conjunctivitis. Symptoms of chlamydial conjunctivitis usually present 5-14 days post-birth with mild hyperemia and scant mucoid or watery discharge. Patients who are diagnosed with gonorrhea should also be treated empirically for chlamydia. Answer D: Topical bacitracin can be used as a treatment for gram-positive neonatal bacterial conjunctivitis. It would not be used as a prophylaxis. These patients would present with rapidly progressive infection associated with surrounding soft tissue cellulitis or abscess formation. Bullet Summary: Neonatal gonococcal conjunctivitis is treated with an intramuscular shot of ceftriaxone and standard prophylaxis to prevent this condition is with erythromycin ointment.
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024
A 30-year-old man is evaluated in the post-anesthesia care unit for neck pain. He underwent a laparoscopic appendectomy and was treated for post-operative nausea and vomiting with metoclopramide. Approximately 20 minutes after receiving the medication, he developed pain and stiffness in his neck and eventually was unable to move his neck. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 130/80 mmHg, respirations are 16/min, and oxygen saturation is 98% on room air. Examination reveals an uncomfortable appearing man. His neck is rotated to the right and is unable to return to midline. Examination of the patient is shown in Figure A. Which of the following is the most appropriate next step in management?
{ "A": "Botulinum toxin injection", "B": "Ondansetron", "C": "Diphenhydramine", "D": "Observation", "E": "" }
C
This patient is suffering from a metoclopramide-induced acute dystonic reaction. Treatment of this complication includes IV diphenhydramine or benztropine. Dystonic reactions are reversible extrapyramidal symptoms that may include involuntary spasms or sustained contractions of the face, neck, torso, pelvis, extremities, and larynx. Medications that block dopamine D2 receptors carry the greatest risk for development of a dystonic reaction, most notably including neuroleptics, antidepressants, and antiemetics. A family history of dystonia increases the risk of developing a reaction (there is likely an underlying genetic predisposition). Diagnosis is typically based on history and clinical presentation. Intravenous anticholinergic agents are the first-line treatment, and symptoms typically resolve rapidly (within 1 hour) after initiation of these agents. Dressler et al. discuss the diagnosis and management of medication-induced acute movement disorders, notably dystonic reactions. These reactions are most commonly caused by dopamine-receptor blocking agents, including antipsychotics and antiemetics. Cranial, pharyngeal, and cervical muscles are typically affected within the first 4 days of initiating treatment. Figure A shows a patient with dystonia and torticollis; note the posturing of the extremities as well as how the head is tilted to the patient's right side while the chin is directed up and towards the left. Incorrect Answers: Answer A: Intramuscular botulinum toxin injection may be appropriate for chronic torticollis or other forms of chronic dystonia. This patient has acute dystonia that is generally able to be easily reversed with intravenous diphenhydramine. Answer C: Intravenous haloperidol, a D2 receptor antagonist would not be appropriate. This patient's condition is due to the dopamine antagonist action of metoclopramide and would therefore be worsened with haloperidol. Answer D: Observation would not be appropriate. This patient is experiencing an acute dystonic reaction that is uncomfortable and potentially dangerous. Acute dystonia is not likely to self resolve in a reasonable amount of time. Answer E: Intravenous ondansetron may be a more appropriate option to treat this patient's nausea given his reaction to metoclopramide. However, it will not reverse the acute dystonic reaction and is not the most appropriate first step. Bullet Summary: Acute dystonic reaction may occur with administration of medications that block dopamine receptors (such as metoclopramide), and can be treated with intravenous diphenhydramine.
https://bit.ly/3LH7lCu
025
A 69-year-old patient presents to the emergency department with shortness of breath and substernal pain. The patient has a medical history of obesity, anxiety, and constipation. He is currently not taking any medications and has not filled his prescriptions for over 1 year. An ECG is obtained and is seen in Figure A. The patient is managed appropriately and is discharged on atorvastatin, metoprolol, aspirin, clopidogrel, and sodium docusate. The patient visits his primary care physician 4 weeks later and claims to be doing better. He has been taking his medications and resting. At this visit, his temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 80/min, respirations are 11/min, and oxygen saturation is 96% on room air. The patient states he feels better since the incident. Which of the following is the most appropriate next step in management?
{ "A": "Add nifedipine", "B": "Add lisinopril", "C": "Add hydrochlorothiazide", "D": "Continue current medications and schedule follow up", "E": "" }
B
This patient is presenting after a myocardial infarction for a follow-up appointment. The most appropriate next step in management is to start an angiotensin-converting enzyme (ACE) inhibitor such as lisinopril. When a patient experiences a myocardial infarction, there are certain medications that the patient should be started on that lower mortality. These medications include beta-blockers, aspirin, statins, other antiplatelet agents (clopidogrel), and ACE inhibitors. ACE inhibitors can reduce mortality after myocardial infarction by reducing afterload, allowing the damaged myocardium to maintain its ejection fraction. These medications also inhibit ventricular remodeling in this disease. ACE inhibitors most prominently reduce mortality if a patient's ejection fraction is reduced. Of note, ACE inhibitors also reduce mortality in diabetes by decreasing hyperfiltration damage to the glomeruli. Jering et al. hypothesized that using an angiotensin receptor neprilysin inhibitor would improve mortality in patients who have sustained a myocardial infarction. They discuss how an ACE inhibitor is the standard of care in patients currently. They recommend performing a randomized controlled trial comparing these treatment modalities. Figure/Illustration A demonstrates an ECG with ST elevation (red circles) in leads II, III, and AVF. These ST elevations are indicative of a STEMI. Incorrect Answers: Answer A: Hydrochlorothiazide can be used to manage this patient's blood pressure; however, this drug does not lower mortality like an ACE inhibitor. In the setting of patients with myocardial infarctions, inhibition of detrimental ventricular remodeling is key for lowering mortality. Answer C: Nifedipine is a calcium channel blocker that can be used to reduce blood pressure; however, it does not reduce mortality in patients who have experienced an MI. Calcium channel blockers tend to have few side effects and can be safely started in most patients. Answer D: Continuing current medications and scheduling a follow-up appointment misses the opportunity for the patient to be started on a mortality-lowering ACE inhibitor. In patients who are amenable to starting a new medication, an ACE inhibitor should be administered to all patients who have sustained an MI. Answer E: Switching to carvedilol would be unnecessary and not likely to benefit this patient as much as adding an ACE inhibitor. Metoprolol is the preferred beta blocker in patients who have sustained an MI. In the absence of idiosyncratic side effects, there is no indication to use another beta blocker. Bullet Summary: Patients who have experienced a myocardial infarction should be discharged on mortality-lowering drugs such as aspirin, beta-blockers, anti-platelet agents, and ACE inhibitors.
https://bit.ly/3LKCkh8
026
A 65-year-old man presents to the emergency department with low back pain. The pain started 6 hours ago when he was gardening and carrying a heavy bag. The pain is rated as 10/10 in severity and radiates down the posterior aspect of the right thigh. He reports no fevers or chills. His only medical problem is hypertension for which he takes amlodipine. The patient is a non-smoker, uses alcohol infrequently, and has never used illicit drugs. His temperature is 99.6°F (37.6°C), blood pressure is 140/70 mmHg, pulse is 90/min, and respirations are 20/min. Physical examination reveals 3/5 strength to hip extension, knee flexion and extension, and plantar flexion bilaterally. Sensation to pinprick is diminished over the posterolateral legs and lateral aspects of both feet. Ankle and knee reflexes are absent bilaterally. The patient’s underwear is wet and a bladder scan reveals 800 mL of urine. Which of the following is the most likely underlying diagnosis?
{ "A": "Acute inflammatory demyelinating polyneuropathy", "B": "L5/S1 disc herniation", "C": "Spinal stenosis", "D": "Cauda equina syndrome", "E": "" }
D
This patient presenting with acute, severe, radiating low back pain with sensorimotor deficits (3/5 strength in hip extension, knee flexion/extension, plantar flexion, reduced sensation to pinprick) in multiple nerve root distributions (L3-S5), lower motor neuron signs, urinary incontinence (wet underwear), and acute urinary retention (bladder scan showing 800 mL of urine) most likely has cauda equina syndrome. Cauda equina syndrome is characterized by deficits in 2 or more nerve roots that comprise the cauda equina of the spinal cord. It has multiple etiologies, including disc herniation, epidural abscess, tumor, or lumbar spine spondylosis. Cauda equina syndrome presents with severe low back pain radiating into the legs, flaccid paralysis or lower extremity weakness which can be symmetric or asymmetric (often asymmetric), bladder and/or rectal sphincter paralysis, impotence/erectile dysfunction, sensory loss in affected dermatomes, and saddle anesthesia (sensory loss in the perineum). Urinary retention occurs due to bladder paralysis. Diagnosis is aided by emergency magnetic resonance imaging. Cauda equina syndrome is a surgical emergency; the goal of surgical treatment is to decompress the cauda equina to prevent permanent neurological injury, such as with a lumbar laminectomy. This can be differentiated from conus medullaris syndrome, which has perianal numbness without numbness through other dermatomes (down the leg in the S1 or S2 patterns), less severe pain (cauda equina presents with more severe pain), and hyperreflexia in the knees (cauda equina presents with lower motor neuron signs in the legs which lead to decreased reflexes - though conus medullaris may present with absent ankle reflexes, the knees are typically hyperreflexic). Gardner et al. review the symptoms, diagnosis, and medico-legal landscape of cauda equina syndrome. They discuss how the most frequent etiology is a large central lumbar disc herniation. They recommend being vigilant for urinary retention in patients who may have compression as many patients have urinary retention on presentation. Incorrect Answers: Answer A: Acute inflammatory demyelinating polyneuropathy, also known as Guillain-Barre syndrome, occurs after respiratory or gastrointestinal infections and presents with ascending paralysis and diminished reflexes. While bladder dysfunction can occur, back pain is not typical. Guillain-Barre syndrome can be treated with intravenous immunoglobulin. Answer C: L5/S1 disc herniation affects the traversing S1 nerve root. S1 radiculopathy presents with posterolateral leg and dorsolateral foot sensory loss and absence of the ankle reflex. Typically, bowel/bladder symptoms are not present. Treatment includes decompression of the affected nerve root. Answer D: Overflow incontinence is caused by either bladder outlet obstruction or poor bladder tone. While this could explain this patient’s urinary retention, it does not account for his back pain or sensorimotor deficits. Isolated overflow incontinence can be treated with catheterization. Answer E: Spinal stenosis presents with lower extremity claudication, characterized by pain with standing or ambulation. The pain of neurogenic claudication is characteristically relieved by spinal flexion (“shopping cart sign”). Sensory loss and motor weakness can be present; however, bladder dysfunction is atypical. Treatment includes surgical decompression of the area of stenosis. Bullet Summary: Cauda equina syndrome is characterized by impingement of 2 or more nerve roots of the cauda equina, resulting in radicular pain, bilateral lower extremity weakness, lower motor neuron signs, sensory loss in affected dermatomes, bowel/bladder dysfunction, and saddle anesthesia.
https://bit.ly/3LSMEDm
027
A 27-year-old man presents to the emergency department with altered mental status. He has become gradually more confused over the past several days. His wife also notes he has had diarrhea, nausea and vomiting, and abdominal pain for the past week. The patient has a history of depression and multiple suicide attempts. He takes fluoxetine as well as over-the-counter pain medications for a recent muscle strain. He works in a large industrial compound that manufactures semiconductors. He exercises regularly and recently has started making his own beer at home. His temperature is 98.3°F (36.8°C), blood pressure is 107/75 mmHg, pulse is 110/min, respirations are 22/min, and oxygen saturation is 99% on room air. Physical exam reveals a confused man with a garlic odor on his breath and the finding in Figure A. Cardiopulmonary exam reveals a rapid heart rate with no murmurs and clear breath sounds. Which of the following is the most likely etiology of this patient's symptoms?
{ "A": "Arsenic", "B": "Cyanide", "C": "Acetaminophen", "D": "Iron", "E": "" }
A
This patient who works in the semiconductor industry and presents with confusion, hypotension, tachycardia, a garlic odor, and Mees lines (Figure A) most likely has arsenic poisoning. Arsenic poisoning can occur due to exposure to contaminated drinking water, industrial jobs, insecticides, and certain preservatives. Arsenic inhibits enzymes requiring lipoic acid as a cofactor; thus, it disrupts ATP production and causes oxidative stress. Signs and symptoms of arsenic poisoning include Mees lines (white horizontal/parallel lines on the nail beds), nausea, vomiting, abdominal pain, diarrhea, confusion, hypotension, tachycardia, and a garlic odor on the breath. Severe complications are more common with acute exposures and can include shock, pulmonary edema, rhabdomyolysis, and death. Findings consistent with chronic poisoning include anemia, neuropathy, ataxia, and somnolence. A urine arsenic level can support the diagnosis. The treatment of arsenic poisoning is chelating agents such as dimercaprol and supportive therapy. Dimercaprol is a nephrotoxic chelating agent with a narrow therapeutic index and should be used with care. Moon et al. study the association between low and moderate arsenic exposure and cardiovascular disease. The authors find that even low-dose exposure to arsenic increased the risk of cardiovascular disease, coronary artery disease, and stroke mortality. The authors recommend population-level interventions to decrease exposure to arsenic. Figure/Illustration A is the physical exam finding of Mees lines with multiple parallel white lines in the nail bed (blue arrows). Incorrect Answers: Answer A: Acetaminophen overdose has a spectrum of presentations, from non-specific symptoms which can include no symptoms at all to a toxic appearance with nausea, vomiting, jaundice, and altered mental status. Patients should have a 4-hour acetaminophen level drawn. If it falls above the treatment line on the Rummack Matthew nomogram, treatment with N-acetylcysteine should be started. Acetaminophen can cause fulminant liver failure and markedly elevated liver enzymes. Answer C: Cyanide inhibits complex IV in the electron transport chain. It can present after exposure to industrial fires. Patients may present with altered mental status and a profound lactic acidosis that does not respond to oxygen administration with rapid progression to death. The treatment involves the administration of hydroxocobalamin which combines with cyanide to form the non-toxic compound cyanocobalamin. Answer D: Iron overdose presents with nausea, vomiting, diarrhea (bloody), hemorrhagic gastritis, and lactic acidosis. A serum iron level should be ordered to aid in diagnosis. Treatment of acute overdose includes chelation therapy with deferoxamine. Answer E: Lead poisoning can present with constipation, irritability, lethargy, lead lines on radiography, and microcytic anemia with basophilic stippling. Treatment depends on the severity and can involve removal from exposure or chelating therapy with agents such as succimer. Bullet Summary: Arsenic poisoning presents with Mees lines, nausea, vomiting, abdominal pain, confusion, unstable vitals, and a classic “garlic odor.”
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028
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?
{ "A": "Alcohol intoxication", "B": "Cannabinoid use", "C": "Juvenile myoclonic epilepsy", "D": "Absence seizures", "E": "" }
C
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.
https://bit.ly/3LZVzD7
029
A 25-year-old man presents to his primary care provider for a routine appointment. He feels well and has no complaints. His medical and surgical history is significant for the reconstruction of a torn anterior cruciate ligament in his right knee. His family history is significant for a mother with “thyroid problems” and a father with coronary artery disease. The patient is an information technology specialist at a local company. He is sexually active with men. His temperature is 98.6°F (37.0°C), blood pressure is 124/82 mmHg, pulse is 71/min, and respirations are 12/min. On physical exam, the patient is tall and slender with a BMI of 21 kg/m2. A single nodule can be palpated on the patient’s thyroid. He has palpable cervical lymphadenopathy. On cardiac exam, he has a normal S1/S2 with no murmurs, rubs, or gallops, and his lungs are clear bilaterally. His abdomen is soft and non-tender without hepatosplenomegaly. A physical exam of the perioral region can be seen in Figure A. Which of the following additional findings is most likely to be found in this patient?
{ "A": "Elevated serum prolactin level", "B": "Elevated serum calcium level", "C": "Elevated plasma metanephrines", "D": "Fasting hypoglycemia", "E": "" }
C
This patient presents with tall stature, mucosal neuromas of the lips and tongue, an isolated thyroid nodule, and a family history of thyroid disease, which suggests a diagnosis of multiple endocrine neoplasia type 2 (MEN2B). MEN2B is also associated with pheochromocytomas, which cause elevated plasma metanephrines. MEN2B is caused by a gain-of-function mutation in the RET proto-oncogene and presents with medullary carcinoma of the thyroid, pheochromocytoma, Marfanoid habitus, and mucosal neuromas. Medullary thyroid carcinoma (MTC) occurs in almost all patients with MEN2B and is often metastatic at the time of diagnosis. Patients should be screened for pheochromocytoma using plasma fractionated metanephrines at the time of diagnosis of MTC. Intestinal neuromas may also lead to intestinal manifestations such as chronic constipation and megacolon. Treatment is with surgical excision of malignant lesions and active screening for future development of tumors. Castinetti et al. review the evidence regarding the diagnosis and treatment of MEN2B. They discuss how often the first presentation of this disease involves the diagnosis of medullary thyroid carcinoma. They recommend developing techniques for earlier diagnosis of this disease in order to allow for better survival. Figure/Illustration A is a clinical photograph demonstrating mucosal neuromas, which are painless nodules on the lips or tongue (red circles). These findings are characteristically seen in multiple endocrine neoplasia type 2B. Incorrect Answers: Answer B: An elevated serum calcium level would suggest parathyroid hyperplasia, which is a feature of both type 1 and type 2A multiple endocrine neoplasia. Rather than hypercalcemia, this patient is likely to have an elevated serum calcitonin level due to his medullary thyroid carcinoma. Answer C: An elevated serum prolactin level would suggest the presence of a prolactinoma, which is a pituitary tumor that may be seen in type 1 multiple endocrine neoplasia (MEN1). Other findings in this disorder include parathyroid and pancreatic tumors. Treatment is with surgical excision of malignant lesions. Answer D: Fasting hypoglycemia would suggest the presence of an insulinoma, which is a less common pancreatic tumor that may be seen in MEN1. Other findings in this disorder include parathyroid and pancreatic tumors. Patients with an insulinoma require surgical excision in order to allow for proper glucose homeostasis. Answer E: A positive fecal occult blood test would raise suspicion for colorectal cancer, which is a feature of Peutz-Jeghers syndrome (PJS). The extraintestinal manifestations of PJS include mucocutaneous pigmented macules on the lips and perioral region. Treatment is with screening for colorectal cancer and surgical excision of malignant lesions. Bullet Summary: Multiple endocrine neoplasia type 2B presents with medullary thyroid carcinoma, pheochromocytoma, Marfanoid habitus, and mucosal neuromas.
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030
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?
{ "A": "Damage to the sphenoid bone", "B": "Damage to the cerebral bridging veins", "C": "Secondary hemorrhage into prior area of infarction", "D": "Occlusion of cerebral small penetrating arteries", "E": "" }
A
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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031
A 36-year-old man presents to the office for follow up after initiating antidepressant therapy. Two months ago, he was started on citalopram after several weeks of depressed mood, loss of interest in activities, depressed appetite, and inability to sleep. Today, he reports minimal improvement in his symptoms. He had some nausea after starting the medication, but this has resolved. He denies thoughts of suicide or self-harm. His temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 120/80 mmHg, respirations are 22/min, and oxygen saturation is 97% on room air. Exam reveals a depressed, tired appearing man. Which of the following is the most appropriate next step in management?
{ "A": "Switch to paroxetine", "B": "Switch to phenelzine", "C": "Electroconvulsive therapy", "D": "Add mirtazapine", "E": "" }
D
This patient is being treated for depression without improvement in his symptoms after 6 weeks of medication use. In the setting of his reduced appetite and trouble sleeping, mirtazapine is an appropriate medication to add. SSRIs (including paroxetine, citalopram, fluvoxamine, sertraline, and fluoxetine) are the first line treatment for major depression. For patients in whom an adequate trial of SSRI therapy has been ineffective, the first step in management is a trial of augmentation with a separate agent prior to switching to a different primary antidepressant medication. Mirtazapine, a noradrenergic and specific serotonergic antidepressant, acts by inhibiting pre-synaptic alpha-2 receptors as well as 5HT2 and 5HT3 serotonin receptors. Notably, its side effect profile includes increased appetite and mild sleepiness, making it a good option for depressed patients with insomnia or decreased appetite. Mirtazapine is commonly used to augment primary antidepressant therapy. Augmentation with an additional agent should generally be attempted prior to switching to a different SSRI or other first line medication. Davies et. al review the pharmacologic management of depression. They note that while evidence is limited, there is some data to suggest benefit in augmentation with a secondary agent prior to switching to another first line therapy. Incorrect Answers: Answer B: Electroconvulsive therapy, while often extremely effective, is typically reserved for patients that are actively suicidal, unable to eat, or displaying psychotic symptoms. It is more commonly used after medications have failed. Answer C: Switching to amitriptyline, a tricyclic antidepressant, would not be appropriate. Tricyclic antidepressants have a negative side effect profile and potential for severe toxicity. Answer D: Switching to paroxetine, another SSRI medication, may be appropriate. However, this patient's particular constellation of symptoms (decreased appetite and insomnia) suggest that he would likely respond well to mirtazapine. Answer E: Switching to phenelzine, a monoamine oxidase inhibitor, would not be appropriate. Monoamine oxidase inhibitors have a negative side effect profile and potential for severe drug-drug interaction. Bullet Summary: Mirtazapine (a noradrenergic and serotonergic antidepressant) is an appropriate next step for depressed patients with significant insomnia and decreased appetite who fail to respond to a trial of SSRI monotherapy.
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032
A 28-year-old woman presents to her primary care provider with a headache. Every few weeks she has an episode of a right-sided, throbbing headache. The episodes began several years ago and are accompanied by nausea and bright spots in her vision. The headache usually subsides if she lies still in a dark, quiet room for several hours. The patient denies any weakness, numbness, or tingling during these episodes. Her medical history is significant for acne, hypothyroidism, obesity, and endometriosis. Her home medications include levothyroxine, oral contraceptive pills, and topical trans-retinoin. Her temperature is 98.6°F (37°C), blood pressure is 125/81 mmHg, pulse is 64/min, and respirations are 11/min. She has 2 glasses of wine with dinner several nights a week and has never smoked. She works as a receptionist at a marketing company. On physical exam, the patient has no focal neurologic deficits. A CT of the head is performed and shows no acute abnormalities. Which of the following is the most appropriate treatment for this patient during these episodes?
{ "A": "Topiramate", "B": "Sumatriptan", "C": "Acetazolamide", "D": "High-flow oxygen", "E": "" }
B
This patient presents with episodic, unilateral throbbing headaches with photophobia, which suggests a diagnosis of migraine. The most appropriate treatment during these episodes is an abortive medication such as sumatriptan. Migraines present with unilateral, "throbbing" pain and are associated with nausea and vomiting, photophobia, and phonophobia. Migraines can occur after exposure to specific triggers or they can occur idiopathically. Patients may also endorse visual auras such as bright lines or shapes in the visual field and sensory auras such as paresthesias. Medications like NSAIDs or sumatriptan are usually used as abortive treatment during acute episodes of migraine. Patients with recurrent migraines can be managed with prophylactic medications like topiramate, beta-blockers, or amitriptyline. Mayans and Walling present evidence regarding the treatment of migraines. They discuss how acetaminophen, nonsteroidal anti-inflammatory drugs, triptans, antiemetics, ergot alkaloids, and combination analgesics are effective against this disease. They recommend providing these treatments in order to mitigate symptoms. Incorrect Answers: Answer A: Acetazolamide is used to treat idiopathic intracranial hypertension (IIH). IIH is most commonly seen in obese women of childbearing age and can be caused by medications such as oral isotretinoin (not topical tretinoin, as in this case) and tetracyclines. The initial symptom is usually a pulsatile headache that worsens with lying down and improves upon standing. Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in male patients with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye. Answer D: Topiramate can be used as prophylaxis for migraines but is not commonly used as an abortive medication. Chronic prophylactic medications generally function by decreasing spasms of the cranial blood supply in order to prevent a migraine from developing. They have minimal effectiveness after a migraine has already developed. Answer E: Verapamil is a prophylactic medication used for cluster headaches. In addition to presenting in the peri-orbital region, cluster headaches are typically accompanied by lacrimation, rhinorrhea, and focal findings such as ptosis. It would not be effective in terminating a migraine. Bullet Summary: Triptans are used as an abortive treatment for migraines, which present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura.
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033
A 26-year-old medical student presents to occupational health after sustaining a needlestick injury. She was drawing blood from an HIV-positive patient when she stuck herself percutaneously while capping the needle. She immediately washed the puncture wound with saline. The medical student has had a negative HIV serology from the beginning of medical school 2 years ago. She is monogamous with one male partner and denies any intravenous drug use. The source patient was recently diagnosed with HIV and has a CD4 count of 550 cells/µL. His most recent viral load is 1,800,000 copies/mL, and he was started on HAART 3 days ago. Which of the following is the most appropriate next step in management?
{ "A": "Repeat HIV serology and initiate triple antiretroviral therapy if negative", "B": "Immediately initiate triple antiretroviral therapy", "C": "Repeat HIV serology and initiate triple antiretroviral therapy if positive", "D": "Repeat HIV serology and initiate triple antiretroviral therapy immediately", "E": "" }
D
This medical student has sustained a hollow-bore needlestick injury. The most appropriate next step is to repeat HIV serology and immediately initiate triple antiretroviral therapy with tenofovir, emtricitabine, and raltegravir. Healthcare exposures to HIV are divided into 2 categories based on the level of contact with the source patient. If the healthcare worker’s mucous membrane or nonintact skin comes into contact with an HIV-positive patient’s blood, semen, or vaginal secretions, immediate prophylaxis is recommended. This category also includes all needlestick exposures. If the healthcare worker’s mucous membrane or nonintact skin comes into contact with an HIV-positive patient’s urine, feces, or bodily secretions, prophylaxis is not recommended. If the source patient’s HIV status is unknown, it should be assumed that the patient is HIV positive until testing proves otherwise. The triple antiretroviral drug combination of tenofovir, emtricitabine, and raltegravir is favored due to the low incidence of side effects. Prophylactic treatment should be initiated immediately (ideally within 1-2 hours) and continued for 4 weeks at which time the healthcare worker’s serology should be repeated. Parikh et al. studied technologies for monitoring HIV infection. They discuss how high throughput methods are required in order to surveil for infection on a population level. They recommend using next-generation sequencing methods to improve surveillance methods. Incorrect Answers: Answer A: Immediately initiating triple antiretroviral therapy is appropriate after drawing the medical student’s HIV serology. The patient’s serology at the time of exposure must be established to compare to future testing. Despite her previous negative serology and low-risk lifestyle, it would still be best to repeat the testing at the same time prophylactic treatment is initiated. Answer B: Perform genotype testing on source patient and initiate antiretroviral therapy tailored to results is incorrect because this option would delay the initiation of prophylaxis. Empiric treatment with a triple antiretroviral drug combination is appropriate for all patients with high-risk exposure. Answer C: Waiting to hear that the medical student’s HIV serology is negative would delay the initiation of prophylaxis. Triple antiretroviral drug treatment should be initiated without waiting for the results. Empiric treatment of all patients with high-risk exposure is recommended in all cases. Answer D: Waiting for the results of the medical student’s serology would delay treatment for her exposure. If her serology is already positive, she will need to be treated past the 4-week period of prophylaxis. Even though she does not engage in high-risk behaviors, a positive serology would demonstrate that she was already infected with HIV. Bullet Summary: After a needlestick injury from a HIV-positive source patient, a healthcare worker’s HIV serology should be drawn and they should be immediately initiated on triple antiretroviral drug therapy.
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034
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?
{ "A": "Fluconazole", "B": "Dexamethasone", "C": "Acyclovir", "D": "Amphotericin B and 5-flucytosine", "E": "" }
D
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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035
A 16-year-old boy presents to his primary care physician for a routine follow-up visit. He feels disappointed because his voice has not changed. He is concerned that he is not as tall as his classmates. He denies any headache, vision changes, nausea, or vomiting. He recently started playing for his high school basketball team and generally eats healthy food. 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 examination, the patient does not have facial hair or acne. His height is 60 inches and his arm span is 50 inches. His visual fields are full and he has no cranial nerve abnormalities. His strength is 5/5 in bilateral upper and lower extremities. Chest palpation and abdominal examination are unremarkable. He has no pubic hair and his testicles are symmetric with a volume of 3 mL. Which of the following is the most appropriate next step in management?
{ "A": "Testicular ultrasound", "B": "Radiograph of the hand and wrist", "C": "Karyotyping", "D": "Reassurance with close follow-up", "E": "" }
B
This patient is greater than 14 years of age without the development of secondary sex characteristics (e.g., testicular volume < 4 mL), which is concerning for delayed puberty. The patient should have a radiograph of the hand and wrist to evaluate for discrepancies between bone age and chronological age. Delayed puberty in boys is defined as the absence or incomplete development of secondary sexual characteristics (increased testicular volume and pubic hair) at 14 years or older. It has a variety of etiologies including chromosomal abnormalities, gonadotropin-releasing hormone (GnRH) deficiencies, and constitutional delay of puberty. In addition to obtaining a history and physical examination, initial diagnostic studies used to work up delayed puberty includes a radiograph of the hand and wrist to determine bone age, and serum studies (complete blood count, complete metabolic panel, follicular-stimulating hormone [FSH], luteinizing hormone [LH], and testosterone). Radiography can compare the patient's bone age to the chronological age (as constitutional delay presents with delayed bone age), and serum endocrine studies can help distinguish between primary causes of hypogonadism (high FSH and LH) and secondary causes of hypogonadism (low to normal FSH and LH). Treatment in cases with a specific cause of delayed puberty is aimed at the underlying cause. For male patients with presumed constitutional delay of puberty, therapy can include hormone replacement with testosterone. Saengkaew et al. discuss the genetics of pubertal delay. The authors find that pathologic mechanisms that may underlie constitutional delay of puberty are largely related to GnRH neuronal development and biology. The authors recommend further study of GnRH neuronal biology and network functionality to provide the opportunity for improved therapies for patients with disorders of puberty. Incorrect Answers: Answer A: Karyotyping is a useful diagnostic study in patients with Klinefelter syndrome, which would demonstrate 47, XXY. Patients would present with a eunuchoid habitus (arm span at least 5 cm longer than height), gynecomastia, and testicular atrophy. Answer B: Magnetic resonance imaging (MRI) of the brain with gadolinium is a useful diagnostic study in patients with neurological symptoms in addition to hypogonadism. These symptoms would suggest that the patient has a prolactinoma causing impingement of the optic chiasm leading to bitemporal hemianopsia and sometimes a headache. Laboratory studies consistent with a prolactinoma include elevated serum prolactin with low to normal FSH and TSH. Answer C: Reassurance with close follow-up would be appropriate in some patients with constitutional delay of puberty, which is the most common cause of delayed puberty. It is believed to be caused by a transient defect in the production of GnRH. Patients typically have a family history of "late bloomers," characterized as family members having a late growth spurt or late puberty. Answer E: Testicular ultrasound would be appropriate in patients with asymmetric testicles since gonadal tumors can occur in a number of intersex disorders (e.g., androgen insensitivity syndrome) and present with impaired sexual maturation. Buller Summary: Delayed puberty in boys is defined as a lack of testicular enlargement by age 14 and initial diagnostic evaluation should include radiography of the hand and wrist as well as serum testing (FSH, LH, and testosterone).
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036
A 55-year-old man presents to the emergency department with nausea and vomiting for the past week. He decided to come in when his symptoms worsened. He feels that his symptoms are exacerbated with large fatty meals and when he drinks alcohol. His wife recently returned from a cruise with symptoms of vomiting and diarrhea. The patient has a medical history of poorly managed diabetes, constipation, anxiety, dyslipidemia, and hypertension. His temperature is 99.5°F (37.5°C), blood pressure is 197/128 mmHg, pulse is 100/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals a systolic murmur heard loudest along the left upper sternal border. Abdominal exam reveals an obese, tympanitic and distended abdomen with a 3 cm scar in the right lower quadrant. Vascular exam reveals weak pulses in the lower extremities. Which of the following is the most likely underlying etiology of this patient's symptoms?
{ "A": "Adhesions", "B": "Enteric nervous system damage", "C": "Impacted stool", "D": "Twisting of the bowel", "E": "" }
A
This patient is presenting with abdominal pain and a tympanitic abdomen, which are symptoms suggestive of a small bowel obstruction. In developed countries, these typically occur secondary to adhesions. Small bowel obstructions (SBO) typically present with an absence of bowel sounds, bowel movements, and flatus, as well as abdominal distension, nausea, and vomiting. They typically occur secondary to adhesions which entrap the bowel and disrupt its normal peristalsis. Adhesions can occur after abdominal surgery or trauma as part of the normal healing process. The diagnosis of SBO can be confirmed using abdominal imaging such as a CT scan. Treatment often involves observation; however, surgical decompression of the SBO may be needed if there is a closed-loop obstruction or an obstruction that does not resolve on its own. Rami Reddy and Cappell review the evidence regarding the diagnosis and treatment of SBO. They discuss how this disease has a 30% mortality rate if the diagnosis is missed and bowel perforation occurs. They recommend early evaluation and treatment of these patients. Incorrect Answers: Answer B: Enteric nervous system damage describes diabetic gastroparesis which can present similarly to SBO; however, this patient's age and history of surgery points toward SBO as the most likely diagnosis. Treatment is with pro-motility agents. Answer C: Impacted stool describes constipation which could present similarly to SBO; however, this diagnosis is less likely to cause nausea and vomiting unless it is severe. The vomit typically smells like stool. Treatment is with dis-impaction of the colon. Answer D: Norovirus typically causes nausea, vomiting, and diarrhea but does not cause distension and a tympanitic abdomen. Treatment is supportive as the virus self-resolves. Answer E: Twisting of bowel describes a volvulus which presents similarly to SBO; however, it is a more common diagnosis in the elderly and is a less common diagnosis than is SBO in a patient with a history of abdominal surgery. Treatment is with surgical untwisting of the bowel. Bullet Summary: Small bowel obstruction (commonly caused by adhesions) presents with nausea, vomiting, absence of flatus/bowel movements, and a distended/tympanic abdomen.
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037
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": "D", "B": "E", "C": "A", "D": "C", "E": "" }
D
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.
https://bit.ly/3Mqa3f2
038
A 72-year-old man presents to his primary care physician with weakness. He has felt very weak every morning with his symptoms persisting throughout the day. He notes minor improvement when he rides his bike but otherwise has not noticed any change in his symptoms with rest or ibuprofen use. The patient has lost 12 pounds recently and has had a chronic cough. The patient lives alone and drinks 7 alcoholic beverages per day and smokes 1-2 packs of cigarettes per day for the past 40 years. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 93/min, respirations are 17/min, and oxygen saturation is 92% on room air. A chest radiograph is ordered as seen in Figure A. Which of the following diagnostic tests would most likely elucidate the cause of this patient's weakness?
{ "A": "CT scan of the chest", "B": "Electromyography", "C": "Lung biopsy", "D": "Muscle biopsy", "E": "" }
B
This patient who presents with weakness that improves with activity in the setting of a lung mass most likely has Lambert-Eaton syndrome. The most appropriate diagnostic test to confirm this syndrome is electromyography. Small cell lung cancer presents with fatigue, weight loss, cough, and a coin lesion on chest radiograph typically in a smoker. A common paraneoplastic syndrome in small cell lung cancer is Lambert-Eaton syndrome which presents with muscle weakness that improves with activity and worsens with rest. Activity improves symptoms due to accumulation of calcium with exercise which allows for acetylcholine release. This occurs secondary to antibodies against presynaptic calcium channels which decrease acetylcholine release. The diagnosis of Lambert Eaton syndrome can be confirmed with electromyography. Treatment includes addressing the underlying lung tumor. Kesner et al. review the diagnosis and etiology of Lambert-Eaton syndrome. They discuss how this disease is associated with lung cancer. They recommend making the diagnosis using electrophysiologic studies. Figure/Illustration A is a chest radiograph with a coin lesion in the left lung field (red circle). This finding is suggestive of small cell lung cancer. Incorrect Answers: Answer A: Blood laboratory tests could assess for multidrug resistance 1 protein (MDR-1 aka P-glycoprotein or ABCB1) which predicts resistance to chemotherapy for small cell lung cancer; however, it would not further elucidate this patient's muscle weakness. Answers 2 & 4: CT scan of the chest and lung biopsy would further elucidate this patient's small cell lung cancer; however, it would not explain his weakness that improves with activity. In the setting of an elderly smoker with fatigue and weight loss and a coin lesion, the diagnosis of lung cancer is essentially established. Answer E: Muscle biopsy would be the most accurate diagnostic test for dermatomyositis, polymyositis, and inclusion body myositis; however, it would not assess for conditions such as myasthenia gravis and Lambert-Eaton syndrome. Bullet Summary: Electromyography is the diagnostic test of choice for Lambert-Eaton syndrome.
https://bit.ly/3Mv3U1Q
039
A 59-year-old woman presents to her primary care physician for trouble sleeping. When she goes to bed at night, she has an urge to get up out of bed and walk around. The patient often wakes her husband when she does this which irritates him. There is a perpetual uneasiness and feeling of a need to move at night which is relieved by getting up and walking around. The patient denies symptoms during the day. She works as a mail carrier and is nearing retirement. She has a medical history of anxiety, depression, irritable bowel syndrome, and dysmenorrhea. She is not currently taking any medications. Her temperature is 99.5°F (37.5°C), blood pressure is 157/98 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals 5/5 strength in the upper and lower extremities, 2+ reflexes in the upper and lower extremities, a stable gait pattern, and normal sensation. Cardiopulmonary and abdominal exams are within normal limits. Which of the following is the most appropriate initial step in management?
{ "A": "Alprazolam", "B": "Iron studies", "C": "Pramipexole", "D": "Supportive therapy and an exercise routine", "E": "" }
B
This patient is presenting with the need to get up and walk around at night, which is suggestive of restless leg syndrome. The most appropriate initial step in management is collecting iron studies to assess for iron deficiency. Restless leg syndrome presents with a sustained sense of restlessness and a feeling of a need to move one's legs. Symptoms typically occur at night and are relieved by activities such as walking. Iron deficiency can be a precipitating factor in restless leg syndrome as iron is a cofactor in the production of dopamine. Prior to administering iron, it is important to first confirm or rule out the diagnosis of iron deficiency with laboratory values which should include a CBC and iron studies. If iron deficiency exists, iron should be administered. If iron deficiency is not present, then dopamine agonists such as pramipexole or ropinirole can be administered. Gossard et al. review the evidence regarding the diagnosis and treatment of restless leg syndrome. They discuss how iron deficiency is associated with the development of this disorder. They recommend iron repletion in deficient patients. Incorrect Answers: Answer A: Alprazolam could be an appropriate step in management if the patient suffers from episodic panic attacks/anxiety. It is not an optimal long-term therapy for anxiety or depression. Benzodiazepines are not first-line in treating restless leg syndrome. Answer B: Ferrous sulfate would not be indicated until iron deficiency is confirmed. Iron deficiency is associated with restless leg syndrome as iron is a cofactor in the production of dopamine. Answer D: Pramipexole is a dopamine agonist that is an effective therapy for restless leg syndrome. However, it is more important to treat any underlying causes for restless leg syndrome such as iron deficiency prior to starting a dopamine agonist. Answer E: Supportive therapy and an exercise routine ignore the potential underlying pathology and do not offer any specific treatment for this patient's restless leg syndrome. Exercise can improve symptoms of restless leg syndrome but iron deficiency must still be ruled out. Bullet Summary: Patients with restless leg syndrome should be assessed for iron deficiency and treated if they are iron deficient.
https://bit.ly/3Mv8f5p
040
A 57-year-old man is brought to a neurologist by his wife for abnormal behavior. He was normal until around 3 months ago when he started forgetting important meetings and misplacing items around the house. One month ago, the patient was fired from his job as a software engineer after attempting to grope a coworker. The patient expresses frustration that he is no longer able to concentrate enough to enjoy his hobby of solving puzzles. His medical history is significant for gastroesophageal reflux disease for which he takes omeprazole. He has no family history of neurologic disease. His temperature is 98.6°F (37.0°C), blood pressure is 120/75 mmHg, pulse is 70/min, and respirations are 16/min. During the examination, the patient’s cell phone rings unexpectedly causing the patient to suddenly jerk his arms and legs. Further diagnostic investigation is performed which reveals an elevated 14-3-3 protein on cerebrospinal fluid analysis. Which of the following is the most likely diagnosis?
{ "A": "Alzheimer disease", "B": "Creutzfeldt-Jacob disease", "C": "Frontotemporal dementia", "D": "Subcortical leukoencephalopathy", "E": "" }
B
This patient with rapidly progressive dementia over 3 months, declining executive function, impaired concentration, decreased memory, startle myoclonus (sudden jerking of extremities in response to unexpected stimulus), and elevated 14-3-3 protein levels on cerebrospinal fluid (CSF) analysis most likely has Creutzfeldt-Jakob disease (CJD). CJD is a human prion disease in which the normal prion protein (PrPc) converts to a beta-pleated sheet disease-associated form (PrPsc) which is resistant to proteases. CJD is usually spontaneous. Rarely, CJD may be familial or acquired (from iatrogenic transmission or dietary exposure). Aggregates of PrPsc lead to spongiform vacuolation. Clinically, this manifests with rapidly progressive mental deterioration with prominent executive dysfunction (declining concentration, judgment, and memory) and startle myoclonus. Further diagnostic investigation may reveal 14-3-3 protein on CSF analysis and synchronous sharp wave complexes on electroencephalogram (EEG). CJD usually leads to death within 1 year. There is no known effective treatment for this disease. Uttley et al. review the diagnosis, clinical presentation, and epidemiology of Creutzfeldt-Jakob disease (CJD). They discuss how CJD in a global context is increasing in prevalence and how incubation periods as long as 40 years have been observed for iatrogenic cases. They recommend heightened awareness of this disease in order to mitigate iatrogenic transmission. Incorrect Answers: Answer A: Alzheimer disease is characterized by gradual cognitive decline with difficulty completing activities of daily living. Impaired executive function early in the disease course, rapidly progressive disease, elevated 14-3-3 protein on CSF analysis, and startle myoclonus are not found in Alzheimer disease. Answer C: Lewy body dementia (LBD) presents with alterations in consciousness, disorganized speech, visual hallucinations, extrapyramidal symptoms, and early compromise of executive function. Although this patient’s dementia is characterized by early compromise of executive function, the absence of fluctuating levels of consciousness, absence of extrapyramidal symptoms, and presence of startle myoclonus make CJD more likely than LBD. Answer D: The behavioral variant of frontotemporal dementia (FTD) is associated with early-onset personality and behavioral changes, and it may be associated with extrapyramidal symptoms. Memory is not usually affected until late in the disease course. Answer E: Subcortical leukoencephalopathy (Binswanger disease) is a type of small vessel vascular disease that presents with prominent subcortical dementia. This manifests clinically as slowed mental processing, impaired cognition, apathy, and depression. This disease almost always presents in the context of chronic hypertension and is usually seen in older patients. Bullet Summary: Creutzfeldt-Jacob disease is a fatal prion disease that presents with sudden-onset and rapidly progressive dementia and startle myoclonus or seizures.
https://bit.ly/3My1xLG
041
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?
{ "A": "Benign capillary proliferation", "B": "Edema of the epidermis", "C": "Healthy dermatologic development", "D": "Viral infection", "E": "" }
A
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.
https://bit.ly/3N80FxZ
042
A 71-year-old man arrives to the emergency room appearing cyanotic and having weak, shallow respirations. He is brought in by his home care nurse, who reports that the patient has a history of myasthenia gravis and frequent urinary tract infections. The patient was in his normal state of health until 5 days ago when he developed a urinary tract infection. He was receiving gentamicin infusions for his infections. This morning, he experienced poor grip strength and progressive difficulty breathing. The patient’s medications include pyridostigmine and aspirin, both of which he takes as prescribed. His temperature is 99.0°F (37.2°C), blood pressure is 128/78 mmHg, pulse is 92/min, and respirations are 28/min with an oxygen saturation of 86% O2 on room air. The patient has gray-blue skin, hypophonia, weak upper extremities, and normal leg strength. An arterial blood gas is drawn with results as shown below: PO2: 55 mmHg PCO2: 60 mmHg pH: 7.30 The patient is intubated. Which of the following is the most appropriate next step in management?
{ "A": "Edrophonium", "B": "Plasmapheresis", "C": "Thymectomy", "D": "Atropine", "E": "" }
B
This patient is presenting with a history of myasthenia gravis, recent exposure to an aminoglycoside, and respiratory failure, suggesting a diagnosis of myasthenic crisis. The most appropriate initial step in management is intubation and plasmapheresis. Myasthenic crisis is an exacerbation of myasthenia gravis caused by under-dosing myasthenia gravis treatments or precipitated by infection, surgery, and certain medications such as aminoglycosides and beta-blockers. Symptoms include bulbar muscle weakness and type 2 respiratory failure (inadequate alveolar ventilation). The most appropriate initial step in management is to maintain adequate respiratory function by intubation. Plasmapheresis or IVIG is used to remove acetylcholine receptor antibodies from circulation, and steroids are started for long-term therapy. Avoiding risk factors and pre-medication before procedures with acetylcholinesterase inhibitors is important for preventing repeat occurrences. Rodrigues et al. review the evidence regarding the occurrence of myasthenic crisis in patients with COVID. They discuss how treatment with IVIG and plasma exchange were important in the care of these patients. They recommend also considering whether rituximab and tocilizumab may be effective in these cases. Incorrect Answers: Answer A: Atropine can be used in a cholinergic crisis. A cholinergic crisis is an acute exacerbation of muscle weakness due to over-medication with cholinergic anti-cholinesterases. This disease would present with diarrhea, sweating, miosis, bronchoconstriction, and bradycardia. It rarely occurs with proper dosing of pyridostigmine. Answer B: Edrophonium can be used in the diagnosis of myasthenia gravis. It is a cholinesterase inhibitor. In a positive test, a patient will exhibit temporary resolution of muscle weakness. In this patient’s case, the diagnosis of myasthenia gravis has already been made and he also had a recent exposure to a pharmaceutical known to precipitate myasthenic crisis. Answer C: Neostigmine is an acetylcholinesterase inhibitor used in the management of myasthenia gravis, but it is contraindicated in the initial treatment of myasthenic crisis. Acetylcholinesterase inhibitors may increase respiratory secretions, which can worsen respiratory failure. Answer E: Thymectomy is indicated in refractory cases of myasthenia gravis (and for increased risk of thymoma). In an unstable condition such as myasthenic crisis, thymectomy should be deferred. For patients over the age of 60, thymectomy is controversial. Bullet Summary: Myasthenic crisis can be precipitated by infection, surgery, and medications (aminoglycosides and beta-blockers) and should be managed with intubation followed by plasmapheresis or IVIG.
https://bit.ly/3N81HdG
043
A 17-year-old high school student was in shop class when he accidentally sawed off a portion of his right index finger. The teacher applied dressings and pressure to the patient's injured digit and immediately transported the patient to the emergency department. He arrived within 20 minutes of the accident. The patient has a medical history of asthma and his only medication is albuterol. His temperature is 98.0°F (36.6°C), blood pressure is 120/70 mmHg, pulse is 105/min, and respirations are 17/min. Exam was significant for pulsatile bleeding from a clean-cut wound on his right second finger. Radiography of the hand revealed a complete amputation of the right finger from the distal interphalangeal joint. The wound was cleaned, compression applied, analgesics administered, and the hand surgeons were notified. The teacher states that he left the amputated finger in the classroom, but the principal would be transporting it to the hospital. Which of the following is the correct method for transporting the amputated finger?
{ "A": "Rinse the finger in normal saline, wrap the finger in moist gauze, put in a plastic bag, and place the bag in ice water", "B": "Wrap the finger in moist gauze and place in a plastic bag", "C": "Submerge the finger in ice water", "D": "Rinse the finger with hydrogen peroxide, wrap the finger in moist gauze, and place on ice", "E": "" }
A
This patient is presenting with a fingertip amputation with loss of pulp, nail, and bone (with a possibility for surgical reattachment and repair, efforts should be made to recover the severed tissue). The correct way to transport an amputated digit is to rinse the finger in normal saline to remove gross contaminants, wrap the appendage in gauze moistened by normal saline, place it in a plastic bag, and then place the bag in ice water. Amputation injuries are common with finger and thumb amputations being the most common. With rapid and proper transport of the patient and appendage, successful reattachment could be possible pending a surgical evaluation. It is important to know how to appropriately package the severed digit so as to optimize tissue viability. Cooling with ice water can slow the metabolic rate of the tissue and result in less tissue damage; however, there is a delicate balance. There must be proper barriers between the digit and ice water to avoid ice burns which could compromise the tissue. Patients should be given IV antibiotics to prevent infection as well. Zhang et al. note that once a finger amputation has occurred, ischemic tolerance times are 12 hours if warm and up to 24 hours if cold. For more proximal amputations, these times are halved due to the presence of muscle tissue, which can undergo irreversible changes after 6 hours of ischemia. Incorrect Answers: Answer B: Rinsing the amputated finger with hydrogen peroxide could damage the tissue decreasing the chances of salvageability. Avoiding the use of caustic solutions with amputated appendages preserves tissue. Answer C: Submerging the finger in ice water could lead to irreversible tissue damage and swelling making reattachment impossible. Answer D: Wrapping the finger in moist gauze and then placing in a plastic bag is not enough to optimize a severed finger for transport. Cooling further with ice water is necessary in order to slow down tissue metabolism and prevent further ischemic damage. Answer E: Wrapping the finger in moist gauze and placing it directly on ice could damage the finger by direct contact with ice rendering the amputated part non-viable. Bullet Summary: The correct handling of an amputated appendage is to first rinse it off with normal saline to remove any gross debris, wrap it in normal saline soaked gauze, put it in a plastic bag, and then place that bag in ice water.
https://bit.ly/3NOcdHz
044
A 4-year-old boy is brought in by his grandmother for worsening seizure activity. She reports that occasionally he “suddenly drops” when running around the house. She says that his first seizure occurred 1 year ago. Since then, he has been on valproic acid but the seizure activity remains unchanged. He was recently enrolled in daycare because she could no longer care for him full-time. Since then, the seizure activity has increased and the daycare facility also noted concerns over his lack of response to voice commands. Family history is significant for a cousin who died suddenly at a young age. His temperature is 98.6°F (37°C), blood pressure is 105/52 mmHg, pulse is 110/min, and respirations are 25/min. A physical exam is significant for lack of response to auditory stimulation. An EEG is performed, which is unrevealing. An ECG is shown in Figure A. The patient is then startled when a door is closed quickly and he suddenly loses consciousness. An ECG is quickly performed and is shown in Figure B. An electrolyte panel is drawn with the following findings: Serum: Na+: 142 mEq/L Cl-: 88 mEq/dL K+: 3.4 mEq/L HCO3-: 24 mEq/L BUN: 11 mg/dL Glucose: 67 mg/dL Creatinine: 0.6 mg/dL Mg2+: 1.7 mg/dL Which of the following is the most appropriate next step in management?
{ "A": "Implant a pacemaker", "B": "Replete potassium and magnesium and start a beta-blocker", "C": "Start ethosuximide", "D": "Administer intravenous lorazepam", "E": "" }
B
The patient who presents with deafness and long QT syndrome that progressed to torsades de pointes most likely has Jervell and Lange-Nielsen syndrome. The most appropriate next step in management is to replete potassium and magnesium and then start a beta-blocker. Jervell and Lange-Nielsen syndrome is an autosomal recessive disorder caused by defective potassium ion channels. This causes abnormal function of the inner ear resulting in congenital sensorineural deafness as well as abnormal myocardial conduction resulting in long QT syndrome. A prolonged QT interval increases the risk of ventricular dysrhythmias, especially torsades de pointes, which may manifest as syncope, or sudden death. These episodes are usually triggered by exertion or by being startled. Management of symptomatic congenital long QT syndromes or torsades de pointes should involve the repletion of potassium and magnesium as well as the initiation of a beta-blocker medication. If symptoms are refractory to beta-blockers, a pacemaker can be implanted to avoid cardiac arrest. Adam et al. review the evidence regarding the diagnosis and treatment of Jervell and Lange-Nielsen syndrome. They discuss how a beta blocker medication should be used for medical control of this syndrome. They recommend pacemaker placement in refractory cases. Figure/Illustration A is an ECG showing a prolonged QT interval (blue lines). This interval can be prolonged due to certain medications such as ondansetron or it can be due to long QT syndrome, which may be acquired or congenital. Figure/Illustration B is an EKG showing a polymorphic ventricular tachycardia with varying QRS complex amplitudes over time (blue curve). This feature where the QRS complexes appear to “twist” around the isoelectric line is seen in torsades de pointes. Incorrect Answers: Answer A: IV lorazepam is the medical treatment for pediatric status epilepticus. Status epilepticus is a seizure that lasts for greater than 5 minutes or recurrent seizures with no return to baseline. This patient has an ECG showing torsades de pointes, which explains his sudden loss of consciousness as well as a normal EEG. Answer B: A pacemaker can be used to treat refractory dysrhythmias caused by Jervell and Lange-Nielsen syndrome, but would not be the initial step in management for this patient. Beta-blocker medications should be used prior to pacemaker placement. Answer D: While potassium and magnesium should be repleted, procainamide is a class 1A anti-arrhythmic medication that prolongs the QT interval. This class of medication is contraindicated in patients with long QT syndrome or torsades de pointes. Answer E: Ethosuximide is used to treat absence seizures. The loss of consciousness associated with Jervell and Lange-Nielsen syndrome can be mistaken for absence seizures, but the patient's prolonged QT interval on ECG makes Jervell and Lange-Nielsen syndrome the most likely diagnosis. Of note, valproic acid is also a medication for absence seizures. Bullet Summary: Jervell and Lange-Nielsen syndrome should be treated with repletion of potassium and magnesium as well as initiation of beta-blocker medication with pacemaker placement reserved for refractory cases.
https://bit.ly/3O2B9LC
045
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?
{ "A": "Lung adenocarcinoma", "B": "Mesothelioma", "C": "Rotator cuff tendinopathy", "D": "Tuberculosis", "E": "" }
A
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.
https://bit.ly/3OFDpHw
046
A 57-year-old man presents to the emergency department for weight loss and abdominal pain. The patient has felt steadily more fatigued over the past month and has lost 22 pounds without effort. Today, he fainted prompting his presentation. The patient has no significant medical history. He does have a 33-pack-year smoking history and drinks 4 to 5 alcoholic drinks per day. His temperature is 99.5°F (37.5°C), blood pressure is 100/58 mmHg, pulse is 100/min, respirations are 17/min, and oxygen saturation is 98% on room air. The patient is very thin and appears pale. Stool fecal occult blood testing is positive. A CT scan of the abdomen is performed demonstrating a mass in the colon with multiple metastatic lesions scattered throughout the abdomen. The patient is informed of his diagnosis of metastatic colon cancer. When the patient conveys the information to his family, he focuses his efforts on discussing the current literature in the field and the novel therapies that have been invented. He demonstrates his likely mortality outcome which he calculated using the results of a large multi-center study. Which of the following is this patient most likely demonstrating?
{ "A": "Intellectualization", "B": "Pessimism", "C": "Rationalization", "D": "Optimism", "E": "" }
A
This patient is using facts and logic to emotionally distance himself from his stressful diagnosis. This ego defense method is suggestive of intellectualization. Intellectualization typically occurs in patients who have received a very stressful diagnosis or bad news for a close loved one. They will often focus on the science and research of their disease rather than focus on emotionally processing this stressful event. This is a way of distancing themselves from the diagnosis and their circumstances. These patients may have difficulty connecting with others because they become extremely focused on data. Arnold reviews the evidence regarding the diagnosis of intellectualization. He discusses how this term can either refer to the isolation of affect or the general use of reason. He recommends a better understanding of how this ego defense works. Incorrect Answers: Answer A: Dissociation is the detachment of the mind from the emotional state/body. A more specific diagnosis for this patient would be intellectualization as he is detaching himself from the emotional state of the diagnosis and instead focusing on the science behind his condition. Answer C: Optimism is trying to find the best in a bad situation. This patient's focus on treatment outcomes and absence of emotion suggests intellectualization as the employed defense mechanism. Patients who are optimistic will still be able to emotionally process information. Answer D: Pessimism is finding the worst in most situations. Pessimism would be a possible answer if the patient had a very treatable disease but thought that poor outcomes would occur regardless. Answer E: Rationalization is the use of logic or reasoning (that can be inappropriate) in order to substantiate one's behavior even when it is inappropriate. An example is justifying theft because a person was in need. Bullet Summary: Intellectualization is the use of facts and logic to distance oneself from a stressful situation.
https://bit.ly/3OGVR2b
047
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?
{ "A": "Administer intravenous sodium bicarbonate", "B": "Begin potassium replacement therapy with dextrose", "C": "Increase methotrexate dose", "D": "Administer intravenous insulin", "E": "" }
A
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).
https://bit.ly/3OXaArd
048
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?
{ "A": "Schistocytes", "B": "Codocytes", "C": "Acanthocytes", "D": "Degmacytes", "E": "" }
D
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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049
A 41-year-old man presents to his primary care provider with a 2-month history of abdominal pain. He says that the pain “comes and goes” throughout the day and usually lasts 20-30 minutes per episode. The pain is above his umbilicus. He denies any feeling of regurgitation or nighttime cough but endorses nausea. He used to eat 3 large meals per day but has found that eating smaller meals more frequently improves his pain. He tried ibuprofen with food and thinks it helped. He has gained 4 pounds since his last appointment 3 months ago. He denies any diarrhea or change in his stools. He has no known medical history. He drinks 5-6 beers on the weekend and has a 20 pack-year smoking history. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 117/72 mmHg, pulse is 72/min, and respirations are 13/min. On physical exam, he is tender to palpation above the umbilicus. Bowel sounds are present. A stool guaiac test is positive. The patient undergoes an endoscopy with a biopsy to diagnose his condition. Which of the following is most likely to be found on histology?
{ "A": "Crypt abscesses in the large intestine", "B": "Mucosal defect in the stomach", "C": "Noncaseating granulomas in the small intestine", "D": "Urease-producing organism in the small intestine", "E": "" }
D
This patient presents with episodic epigastric pain and nausea that improves with the consumption of food, which suggests a diagnosis of a duodenal ulcer. Duodenal ulcers are often caused by infection with Helicobacter pylori, a urease-producing organism. Duodenal ulcers are erosions and defects in the mucosal lining of the small intestines that allow for damage to the underlying tissues. Patients will classically present with abdominal pain that is localized to the epigastric region. Duodenal ulcers improve upon eating due to the secretion of bicarbonate in the small intestines, whereas gastric ulcers are exacerbated by food due to the release of gastric acid in the stomach. Duodenal ulcers are most commonly caused by infection with H. pylori. If the patient tests positive for H. pylori infection, treatment involves triple therapy with amoxicillin, clarithromycin, and a proton pump inhibitor. Patients who fail to improve should undergo esophagogastroduodenoscopy, which remains the gold standard for diagnosis. Ciociola et al. studied the prevalence of H. pylori infection in patients with gastric ulcers. They found that about many had confirmed infections. They recommend against empiric treatment without prior confirmation of infection. Incorrect Answers: Answer A: Crypt abscesses are found on histology in ulcerative colitis. Ulcerative colitis presents with bloody diarrhea and abdominal pain. Patients can also experience musculoskeletal pain, uveitis, and dermatologic findings such as erythema nodosum or pyoderma gangrenosum. Treatment may include mesalazine. Answer B: A mucosal defect in the stomach is the pathologic description of a gastric ulcer. This patient’s presentation of epigastric pain that improves with the consumption of food is more consistent with an ulcer in the duodenum. Treatment of a gastric ulcer may include proton pump inhibitors. Answer C: Noncaseating granulomas are found on histology in Crohn disease. This patient denies any diarrhea (often associated with pain), which is the primary feature of Crohn disease. Other findings include abscesses, fistulas, and strictures. Treatment may include mesalamine and infliximab. Answer D: PAS-positive material is found in the lamina propria on small intestinal biopsy in Whipple disease. In addition to abdominal pain, Whipple disease is characterized by diarrhea with flatulence and steatorrhea, fever, arthralgias, and weight loss. Treatment is with ceftriaxone or penicillin. Bullet Summary: Duodenal ulcers typically present with episodic epigastric abdominal that is improved after eating and are associated with H. pylori infection.
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050
A 23-year-old woman presents to the emergency department for evaluation of fever and chills over the last several days along with a progressive rash and diarrhea. She had nasal septoplasty performed 1 week prior and has had nasal packing in place since that time. Her rash has progressed and the skin on her hands is "peeling." She has no significant medical history and takes no medications. Her temperature is 102°F (38.9°C), blood pressure is 84/54 mmHg, pulse is 160/min, respirations are 22/min, and oxygen saturation is 99% on room air. Exam reveals an ill-appearing woman. A diffuse, macular, erythematous rash is noted with desquamation over the patient's hands as shown in Figure A. Bilateral conjunctivitis is noted. The patient's nasal packing is removed revealing copious, foul-smelling, purulent discharge. Which of the following pathogens is likely responsible for this patient's condition?
{ "A": "Rhinovirus", "B": "Borrelia burgdorferi", "C": "Staphylococcus aureus", "D": "Rickettsia rickettsii", "E": "" }
C
This patient with fever, diarrhea, and a diffuse, erythematous rash with prolonged nasal packing in place likely has developed toxic shock syndrome. Toxic shock syndrome occurs most commonly due to infection with Staphylococcus aureus. Toxic shock syndrome (TSS) occurs due to infection with Staphylococcal or Streptococcal species that produce "super-antigen" toxins, such as TSST-1 produced by S. aureus. These toxins bind directly to the MHC-II receptor on T cells with high affinity causing a massive, sustained cytokine release. The result is a "cytokine storm" causing an intense inflammatory reaction and distributive shock. These infections arise in the setting of a retained tampon or retained nasal packing following nasal surgery; however, this is not always the case. The clinical presentation of TSS is characterized by fever, nausea, vomiting, diarrhea, conjunctivitis, and a diffuse "sunburn-like" rash. Desquamation of the palms and soles typically occurs during recovery, 1-2 weeks after the acute phase of the illness. The initial management of TSS begins with broad spectrum antibiotic therapy. Typical regimens include vancomycin, cefepime, and clindamycin. The initial antibiotic regimen should cover methicillin-resistant Staph aureus. Clindamycin is always included due to its ability to suppress bacterial protein production, therefore limiting the production of bacterial toxins. Once a causative organism is identified, antibiotic therapy may be narrowed, though clindamycin is usually administered for the duration of treatment. Figure A is the classic generalized and erythematous macular/desquamating rash of toxic shock syndrome. Incorrect Answers Answer A: Borrelia burgdorferi is a spirochetal bacteria that causes Lyme disease. A erythematous, bulls-eye shaped rash would be expected. Answer B: Neisseria meningitidis causes meningococcemia and meningitis. Fever is common, although a purpuric rash and signs of meningeal irritation would be expected. Patients are often profoundly unstable. Answer C: Rhinovirus is the viral pathogen most commonly responsible for the common cold that causes minor symptoms including a cough, runny nose, fever, and aches. Answer D: Rickettsia rickettsii causes rocky mountain spotted fever which presents with a high fever, headache, malaise, and a centripetal maculopapular rash that starts on the extremities and migrates to the trunk. It is a tick-borne illness. Bullet Summary: Toxic shock syndrome classically develops in patients with prolonged use of nasal packing, and presents with fever, diarrhea, a diffuse erythematous rash, and shock.
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051
A 68-year-old woman presents to her primary care provider for dizziness. She often feels lightheaded and like she may “pass out” but never actually loses consciousness. She denies dyspnea on exertion or orthopnea. The patient had a myocardial infarction 6 months ago, and she now has multiple drug-eluting stents. Her ECG upon discharge from that hospitalization was normal. The patient has been taking all of her medications as instructed. Her medical history is otherwise significant for rheumatoid arthritis and migraines. Her home medications include methotrexate, atorvastatin, clopidogrel, and aspirin. Her temperature is 98.6°F (37°C), blood pressure is 118/75 mmHg, pulse is 92/min, and respirations are 12/min. On physical exam, she has an irregular heartbeat with normal cardiac sounds. Her abdomen is non-distended and she has mild lower extremity edema. Her current ECG can be seen in Figure A. Which component of the cardiac conduction system is most likely affected in this patient?
{ "A": "Bundle branches", "B": "Bundle of His", "C": "Atrioventricular node", "D": "Sinoatrial node", "E": "" }
C
This patient who presents with lightheadedness and is found to have progressive prolongation of the PR interval on ECG most likely has Mobitz type 1 (Wenckebach) 2nd-degree heart block. Mobitz type 1 heart block is usually caused by atrioventricular node dysfunction. Second degree heart block can be divided into Mobitz type 1 and Mobitz type 2 patterns. The Mobitz type 1 pattern is usually a result of dysfunction at the atrioventricular (AV) node, whereas the Mobitz type 2 pattern is usually caused by dysfunction below the AV node in the His-Purkinje system. In Mobitz type 1 2nd-degree heart block, the PR interval becomes progressively longer with each beat before the dropped QRS complex. In Mobitz type 2 2nd-degree heart block, the PR intervals are consistent in length and the dropped QRS complexes occur at regular intervals. The easiest way to determine whether the PR interval is getting longer is to compare the PR interval just before the dropped beat to the PR interval immediately following the dropped beat. Patients with Mobitz type 1 blocks generally do not require treatment unless symptomatic whereas those with Mobitz type 2 blocks generally require pacemaker placement. Clark and Prystowsky review the pathophysiology of heart block. They discuss how a type 1 2nd-degree heart block is caused by dysfunction of the atrioventricular node. They recommend symptomatic treatment of this condition. Figure/Illustration A is an ECG that demonstrates the progressive prolongation of the PR interval (blue lines) until a beat is dropped. This progressive prolongation is characteristically seen in Mobitz type 1 (Wenckebach) heart block. Incorrect Answers: Answer B: A bundle branch block would produce a classic right or left bundle branch block pattern on ECG (RSR') described colloquially as "bunny ears." Bundle branch blocks are defined in part by a QRS duration > 120 ms and can be seen in lead V1. Treatment is with control of underlying risk factors such as hypertension. Answer C: The bundle of His conducts the electrical impulse from the atrioventricular node to the ventricles. Dysfunction at the level of the bundle of His would more likely cause Mobitz type 2 2nd-degree heart block or 3rd-degree heart block rather than the Mobitz type 1 heart block seen in this patient. Treatment is with pacemaker placement. Answer D: Purkinje fibers rapidly conduct along the ventricular walls to create synchronized contraction of the ventricles. Aberrant conduction of the Purkinje fibers leads to premature ventricular complexes. Treatment is with beta-blockers if patients are symptomatic. Answer E: The sinoatrial node is the heart’s primary pacemaker. Dysfunction at this level can lead to sick sinus syndrome. Sick sinus syndrome can manifest as many types of arrhythmias, including bradycardia-tachycardia syndrome, in which there is alternating bradycardia with paroxysmal tachycardia. Sinoatrial node dysfunction can be treated with pacemaker placement. Bullet Summary: Mobitz type 1 (Wenckebach) 2nd-degree heart block is characterized by progressive prolongation of the PR interval before a dropped QRS complex and is usually caused by dysfunction of the atrioventricular node.
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052
A 37-year-old woman presents to her physician for mood changes and recent falls over the course of 3 months. She feels more irritable with her husband and children and does not find pleasure in creating art anymore. Over the last 2 weeks, she has been sleeping approximately 5 hours a night and had a fall while walking in her home with the lights off. She denies any head trauma or loss of consciousness associated with the fall. She has a medical history of generalized anxiety disorder and hypothyroidism for which she takes escitalopram and levothyroxine. She had a Roux-en-Y gastric bypass 5 years ago. She has been in a monogamous relationship with her husband for the last 15 years and uses a copper intrauterine device. Physical examination is notable for a depressed affect. Pupils are equal, round, and reactive to light and accommodation. She has 4/5 strength in the bilateral lower extremities and brisk patellar reflexes. Which of the following is most likely the cause of this patient's symptoms?
{ "A": "Subtherapeutic levothyroxine dose", "B": "Reduced functional parietal cell count", "C": "Reduced central nervous system dopamine and serotonin", "D": "Poor iron absorption", "E": "" }
B
This patient's mood changes and findings concerning for subacute combined degeneration (fall in the dark, impaired proprioception, weakness, and brisk patellar reflexes), and history of Roux-en-Y gastric bypass surgery are concerning for vitamin B12 deficiency. Roux-en-y gastric bypass reduces parietal cell count, leading to reduced intrinsic factor secretion, which is needed for vitamin B12 absorption. Roux-en-Y gastric bypass is a laparoscopic bariatric procedure that creates a small gastric pouch from the stomach with a gastrojejunal and jejunojejunal anastomosis. This causes small volumes of food to be consumed and decreases nutrient absorption. The small gastric pouch contains very few parietal cells, which decreases hydrochloric acid and intrinsic factor secretion. The reduced intrinsic factor secretion impairs the protection of vitamin B12 against HCl and reduces absorption in the ileum. Vitamin B12 deficiency can lead to neuropsychiatric changes (depressed mood, irritability, and insomnia) and subacute combined degeneration (weakness, sensory ataxia, peripheral neuropathy, and upper motor neuron signs). Patients who undergo Roux-en-Y gastric bypass must be given high doses of vitamin B12 (often intramuscularly) in order to prevent deficiency. Alexandrou et al. review long-term micronutrient deficiencies after sleeve gastrectomy and Roux-en-Y gastric bypass (RYGB). The authors find that vitamin B12 deficiency was significantly higher in patients with RYGB compared with sleeve gastrectomy. The authors recommend adherence to supplemental iron and vitamin intake in all patients who undergo bariatric surgery. Incorrect Answers: Answer A: Poor iron absorption is a well-known complication of Roux-en-Y gastric bypass surgery due to bypass of the duodenum and proximal jejunum. Poor iron absorption leads to microcytic anemia from iron deficiency and presents with generalized fatigue and conjunctival pallor. Iron deficiency anemia would not present with brisk reflexes and impaired proprioception. Answer B: Prior Treponema pallidum exposure can result in tabes dorsalis, a neurological complication of syphilis, and is seen in patients with a history of multiple sexual partners who do not use barrier protection. The spirochete leads to disease in the posterior columns (vibration and proprioception sensation). Patients present with sensory ataxia (similar to subacute combined degeneration), lancinating pain (in the limbs, back, or face), and an Argyll-Robertson pupil (impaired pupillary constriction to light but intact pupillary constriction to accommodation). Answer C: Reduced central nervous system dopamine and serotonin are seen in patients with major depressive disorder which does not cause sensory ataxia with brisk reflexes. Answer E: Subtherapeutic levothyroxine dose would result in hypothyroidism, which can present with depressed mood, cold intolerance, bradycardia, and delayed relaxation of deep tendon reflexes. Roux-en-Y is an effective means of losing weight, and as the body mass decreases with weight loss, the required dose of levothyroxine is decreased. It would not present with the abnormal neurologic findings seen in this patient. Bullet Summary: Roux-en-Y bariatric surgery increases the risk of vitamin B12 deficiency via reduction of food intake, decreased functional parietal cell count, and reduced absorption of vitamin B12 (which may cause subacute combined degeneration).
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053
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?
{ "A": "Provide reassurance", "B": "Counsel on positioning and thickening feeds", "C": "Obtain abdominal ultrasound", "D": "Initiate proton pump inhibitor", "E": "" }
B
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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054
A 35-year-old man presents to the emergency department with altered mental status. He was found by his roommate to be disoriented this morning. He had complained of a right-sided headache for the past 5 days. He has a history of human immunodeficiency virus (HIV) infection being treated with bictegravir, emtricitabine, and tenofovir. His last CD4 cell count was 500/mm^3. He does not smoke tobacco and drinks alcohol socially. His temperature is 104.2°F (40.1°C), blood pressure is 110/70 mmHg, pulse is 110/min, and respirations are 22/min. Examination reveals 3/5 strength to right shoulder abduction and elbow flexion/extension and 4/5 strength to right hip flexion/extension. The remainder of the strength exam on the right and left sides is normal. Neck flexion does not elicit pain, and there are no tongue lacerations. During the exam, the patient’s eyes are seen to roll backward with repeated flexion/extension of his extremities and loss of urine. This stops after 1 minute. Which of the following is the most likely diagnosis?
{ "A": "Bacterial meningitis", "B": "Fungal meningitis", "C": "Brain abscess", "D": "Epilepsy", "E": "" }
C
This HIV-positive patient presents with fever, unilateral headache, altered mental status, focal neurological deficits (3/5 strength to right shoulder abduction, elbow flexion/extension, hip flexion/extension), and a generalized tonic-clonic seizure, suggestive of a brain abscess. A brain abscess typically presents in patients with underlying risk factors for infection, such as positive HIV status or bacterial endocarditis with hematogenous bacterial seeding. Patients present with fever, unilateral headache, altered mental status, focal neurological deficits, and fevers. Work-up consists of blood cultures, CT scan, and confirmatory magnetic resonance imaging with gadolinium contrast, showing a focal, hyperintense lesion with restricted diffusion. Treatment is with empiric broad-spectrum antibiotic therapy (e.g., vancomycin, metronidazole, and cefepime for gram-positive, gram-negative, and Pseudomonas coverage in immunocompromised patients) and surgical drainage. Brouwer et al. review the causes, diagnosis, and treatment of brain abscesses. They discuss the importance of rapid initiation of antimicrobial therapy in these patients. Incorrect Answers: Answers 1 & 5: Bacterial or fungal meningitis can present with fever, headache, focal neurological deficits, and seizures but patients typically have meningeal signs such as pain with neck flexion. Cryptococcal meningitis is commonly seen in HIV-positive patients with CD4 counts < 100/mm^3. Answer C: Cytomegalovirus encephalitis can present with fever, altered mental status, headache, and focal neurological deficits in patients with HIV infection. It typically presents in patients with severe immunodeficiency, often with CD4 cell count < 50/mm^3. This patient’s CD4 cell count is 500/mm^3, making this less likely than an abscess. Answer D: Epilepsy is a primary neurological disorder that can cause generalized tonic-clonic seizures but would not explain this patient’s fever or unilateral headache. While Todd paralysis (focal weakness after a seizure) can occur after seizures, this patient’s focal weakness predates his seizure. A primary seizure disorder is not diagnosed when seizures occur secondary to another process. Bullet Summary: Brain abscess presents with fever, altered mental status, unilateral headache, seizures, and focal neurological deficits in patients with risk factors for infection (for example HIV, bacterial endocarditis).
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055
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?
{ "A": "Obtain abdominal ultrasound", "B": "Initiate a proton pump inhibitor", "C": "Switch to hydrolyzed formula", "D": "Modify mother's diet", "E": "" }
D
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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056
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?
{ "A": "Radiograph", "B": "Bone scan", "C": "Magnetic resonance imaging", "D": "Ultrasound", "E": "" }
C
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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057
A 2-year-old girl presents to the pediatrician with her mother for a routine well-child visit. Her mother is concerned that the patient is a picky eater and refuses to eat vegetables. She drinks milk with meals and has juice sparingly. She goes to sleep easily at night and usually sleeps for 11-12 hours. The patient has trouble falling asleep for naps but does nap for 1-2 hours a few times per week. She is doing well in daycare and enjoys parallel play with the other children. Her mother reports that she can walk downstairs with both feet on each step. She has a vocabulary of 10-25 words that she uses in the form of 1 word commands. She is in the 42nd percentile for height and 48th percentile for weight, which is consistent with her growth curves. Her temperature is 98.6°F (37°C), blood pressure is 92/56 mmHg, pulse is 106/min, and respirations are 23/min. On physical exam, she appears well nourished. She can copy a line and throw a ball. She can follow the command to “give me the ball and then close the door.” Which of the following is abnormal in this patient?
{ "A": "Fine motor skills", "B": "Social and receptive language skills", "C": "This child is developmentally normal", "D": "Expressive language skills", "E": "" }
D
This child is meeting her milestones in every category but expressive language. By 2 years of age, a child should have a vocabulary of over 50 words and be using 2-word phrases. Assessing whether a child is meeting developmental milestones is important for identifying delays and enabling early intervention. In the gross motor category, a 2-year-old child should be able to jump and walk both up and down stairs with both feet on each step. In the fine motor category, a child should be able to copy a line and build a tower of 6 cubes. In the expressive language category, a child should have a vocabulary of over 50 words and be using 2-word phrases. In the social category, a child should participate in parallel play and be able to follow 2-step commands. Detecting a persistent developmental delay can be important as the first step in identifying a correctable condition such as hearing loss. Scharf et al. review the evidence regarding the use of developmental milestones in assessing children. They discuss how early identification of delays can allow for referral to required services. They recommend paying special attention to sensory function to avoid missing a correctable hearing deficit. Incorrect Answers: Answer B: This child's fine motor skills are appropriate, as she can copy a line. She may also be able to copy a circle and make a tower of 6 cubes at this age. Answer C: This child's gross motor skills are appropriate because she can walk downstairs with both feet on each step and throw a ball. She should also be able to jump and run at this age. Answer D: This child's social and receptive language skills are developing appropriately since she can follow 2-step commands and engages in parallel play. She should also be able to point to pictures, body parts, and pieces of clothing when named. Answer E: This child is not developmentally normal due to her deficits in expressive language. She should be using 2-word sentences and have a vocabulary of over 50 words. Her language should also be intelligible to strangers 50% of the time. Bullet Summary: By 2 years of age, a child should be able to walk up and down stairs, copy a line, follow a 2-step command, have a vocabulary of at least 50 words, and use 2-word phrases.
https://bit.ly/3Pmj3mJ
058
A 59-year-old man presents accompanied by his wife with nausea and dizziness. He is unsure when his symptoms started, but they have been affecting him for “a while.” It began as episodes of “unsteadiness” that progressed to a feeling of “spinning.” He cannot tell if his symptoms change with position but reports that if he does not lie down he will become nauseous. He also has worsening hearing loss worse on his right side. The patient’s medical history is significant for hypertension, alcohol use disorder, and chronic obstructive pulmonary disease. His medications include aspirin, amlodipine, and fluticasone-salmeterol. He drinks a glass of red wine every night with dinner and smokes a cigar on the weekends. His temperature is 98.6°F (37°C), blood pressure is 135/91 mmHg, pulse is 72/min, and respirations are 12/min. Examination shows delayed horizontal nystagmus. Which of the following is the most appropriate treatment for this patient's condition?
{ "A": "Low-salt diet", "B": "Thiamine", "C": "Epley maneuver", "D": "Meclizine", "E": "" }
A
This patient is presenting with chronic vertigo, hearing loss, and horizontal nystagmus, which is suggestive of Meniere disease. The most appropriate initial step in the management of this disease is a low-salt diet. Meniere disease is caused by an abnormal accumulation of endolymph within the inner ear. Symptoms will include chronic relapsing and remitting episodes of vertigo, sensorineural hearing loss, tinnitus, and nausea. Physical exam, audiometry, and vestibular testing can be performed to confirm the diagnosis. First-line treatment is a thiazide diuretic as well as lifestyle modifications including a low-salt diet as well as avoidance of alcohol, nicotine, and caffeine. If symptoms persist, additional therapy may involve vestibular nerve ablation or a labyrinthectomy. Tassinari et al. review the evidence regarding the treatment of Meniere disease. They discuss how vertigo, low-pitched tinnitus, and hearing loss are characteristic of this disease. They recommend creating a database to allow for better research into this disease. Incorrect Answers: Answer A: CN VIII ablation can be used to treat Meniere disease that is uncontrolled by diet modifications or thiazide diuretics. Patients usually respond to conservative care so a complete course of these treatments should be trialed first. Answer B: The Epley maneuver can be used to treat benign paroxysmal positional vertigo (BPPV). Patients will present with vertigo that changes with the patient's position without hearing loss or tinnitus. This maneuver functions by dislodging otoliths from the semicircular canals. Answer D: Meclizine can be used to treat other types of vertigo such as BPPV or viral labyrinthitis. Labyrinthitis can present with similar symptoms to the symptoms of Meniere disease, but they will be acute and self-limited. There will also be inflammation of the cochlear portion of the inner ear. Answer E: Thiamine can be used for Wernicke-Korsakoff syndrome. A history of chronic heavy alcohol use, confusion with confabulation, ataxia, memory loss, and nystagmus are all concerning signs that may indicate thiamine deficiency. Bullet Summary: Meniere disease should be treated initially with a low-salt diet, avoidance of triggers, and thiazide diuretics.
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059
A 66-year-old woman presents to the emergency room after experiencing a tonic-clonic seizure. She has been having worsening headaches and intermittent nausea over the past 5 months. The headaches are constant and dull, and they typically worsen when she sneezes or laughs. Recently, she has become increasingly nauseous and has vomited twice in the past 10 days. Her medical history is significant for type 2 diabetes mellitus and hypertension for which she takes metformin and losartan. Her temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. She is noted to have papilledema on fundoscopy, but physical exam is otherwise normal. Her CT scan findings are presented in Figure A. Which of the following is the most likely diagnosis?
{ "A": "Glioblastoma multiforme", "B": "Cerebral toxoplasmosis", "C": "Neurosarcoidosis", "D": "Hemorrhagic stroke", "E": "" }
A
This patient is presenting with symptoms of increased intracranial pressure (i.e., positional headaches, nausea, vomiting, and papilledema). Combined with her CT finding of a space-occupying butterfly-shaped lesion, the most likely diagnosis is glioblastoma multiforme. Glioblastoma multiforme is the most common malignant primary nervous system cancer, most commonly occurring in adults above the age of 65. The tumor arises from the cerebral hemispheres and classically crosses the corpus callosum, producing a butterfly appearance on CT/MRI. Pseudopalasading necrosis is seen on biopsy, and tissue biopsy is usually glial fibrillary acidic protein (GFAP) positive. It has an unfavorable prognosis with a life expectancy of 6 months to 1 year. Management of glioblastoma includes surgical resection and palliative radiotherapy/chemotherapy. Stupp et al. studied the role of electrical field therapy (TTFields) in conjunction with temozolomide in the treatment of glioblastoma multiforme. The authors found that combination therapy improved median progression-free survival (6.7 vs. 4.0 months, p < 0.001). The authors recommend that clinicians consider the use of TTFields to treat selected patients with glioblastoma multiforme. Figure/Illustration A demonstrates a non-contrast axial head CT showing the classic butterfly lesion characteristic of glioblastoma multiforme (red arrow). Incorrect Answers: Answer A: Cerebral toxoplasmosis presents on imaging with multiple ring-enhancing lesions and is most common in patients with acquired immunodeficiency syndrome (AIDS), especially with CD4 T-cell counts of less than 100 cells/mm^3. This patient has no signs of infection or ring-enhancing lesions. Answer C: Hemorrhagic stroke presents acutely with hemiparesis, altered mental status, slurred speech, or sensorimotor symptoms. CT imaging would reveal a hemorrhage. Answer D: Metastatic brain tumor often presents with imaging findings of multiple well-circumscribed lesions with surrounding edema. These lesions are generally found at the highly vascularized gray-white junction. In a patient with no smoking history, a single brain lesion, and no other extra-cranial symptoms, this is a less likely diagnosis. Answer E: Neurosarcoidosis can be caused by infiltrative sarcoidosis and often presents with bitemporal hemianopsia due to infiltration of the pituitary gland. Patients with sarcoidosis typically also present with pulmonary symptoms (e.g., wheezing and coughing) which are absent in this patient. Bullet Summary: Glioblastoma multiforme should be suspected in patients presenting with symptoms of increasing intracranial pressure and classic CT/MRI findings of a butterfly shaped lesion with central necrosis.
https://bit.ly/3Pv88aE
060
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?
{ "A": "Alcoholics anonymous", "B": "Aripiprazole", "C": "Phenobarbital", "D": "Propranolol", "E": "" }
D
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.
https://bit.ly/3Q3WlAi
061
A girl presents to her pediatrician for a well-child visit. Her mother reports that she is eating well at home and sleeping well throughout the night. She can jump and walk up and down stairs with both feet on each step. In the doctor’s office, the patient builds a 6-cube tower and imitates a circle. She seems to have a vocabulary of over 50 words that she uses in 2-word sentences. The patient enjoys playing near other children and sometimes argues over toys with her older brother. Her temperature is 98.6°F (37°C), blood pressure is 92/56 mmHg, pulse is 106/min, and respirations are 23/min. On physical exam, she appears well developed and well nourished, and she is following along her growth curves. The child is assessed as developmentally normal. Which of the following is an additional milestone associated with this child’s age?
{ "A": "Cuts with scissors", "B": "Turns pages in book", "C": "Follows two-step commands", "D": "Points to one body part", "E": "" }
C
This patient is able to walk up and down stairs, can build a 6 cube tower, has a 50+ word vocabulary, and uses 2-word phrases, which suggests she is 2 years of age. Another milestone at 2 years of age is following 2-step commands. In the gross motor category, a child at 2 years of age should be able to jump and walk up and down stairs with both feet on each step. In the fine motor category, the child should be able to copy a line or circle and build a tower of 6 cubes. In the language category, the child should be using over 50 words and putting them together in 2-word phrases. In the social category, a child should be participating in parallel play. In the receptive language category, a child should be able to follow a 2-step command. Persistent developmental delays should prompt investigation for correctable causes such as hearing loss. Scharf et al. review the evidence regarding the use of developmental milestones in assessing children. They discuss how early identification of delays can allow for referral to required services. They recommend paying special attention to sensory function to avoid missing a correctable hearing deficit. Incorrect Answers: Answer A: Balancing on 1 foot is a milestone at 3 years of age. A 2-year-old child would not be expected to maintain balance in that position. Answer B: Cutting with scissors is a milestone at 3 years and 6 months of age. A 2-year-old child would not be expected to be able to hold or use scissors correctly. Answer D: Pointing to one body part is a milestone at 15 months of age. A 2-year-old child should be able to point to 5-6 body parts. Answer E: Turning pages in a book is a milestone at 16 months of age. It would not be considered a milestone for a 2-year-old child. Bullet Summary: At 2 years of age, a child should be able to walk up and down stairs, build a tower of 6 cubes, use a vocabulary of over 50 words in 2-word phrases, and follow a 2-step command.
https://bit.ly/3Q5BZZe
062
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?
{ "A": "Cerebral salt wasting", "B": "Diuretic overuse", "C": "Primary polydipsia", "D": "Syndrome of inappropriate anti-diuretic hormone", "E": "" }
A
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.
https://bit.ly/3QOJp1p
063
A 35-year-old man is brought into the emergency department by ambulance for a stab wound to the left leg near the inguinal ligament. The patient has no significant medical history, and takes no other medication. He was not stabbed or otherwise injured elsewhere. His temperature is 98.6°F (37.0°C), pulse is 130, blood pressure is 85/50, and respirations are 22. Primary and secondary survey and FAST exam are unremarkable for other associated injuries. Pressure is removed from the wound, and pulsatile bleeding from the wound is noted. Which of the following is the most appropriate next step in management?
{ "A": "Emergent surgical intervention", "B": "Wound closure", "C": "Observation", "D": "CT scan of the left lower extremity", "E": "" }
A
This patient with pulsatile bleeding from a wound to the left thigh and hemodynamic instability has hard signs of vascular injury. Patients with penetrating wounds and hard signs of vascular injury should be managed with emergent surgical intervention. Vascular injury commonly occurs as a result of penetrating trauma, such as a stab wound as in this patient. Evaluation begins with a primary and secondary trauma survey to assess for other injuries. Hard signs of vascular injury include pulsatile bleeding, expanding hematoma, and absent pulses distal to the wound. For patients with suspected vascular injury that display either hard signs or hemodynamic instability, emergent surgical intervention is the most appropriate next step in management. For those with soft signs of vascular injury such as non-pulsatile bleeding or associated bony injury, the most appropriate next step in management is CT angiography of the affected limb. Ball et al. discuss penetrating trauma to the extremities. They state that extremity trauma is the most common source of vascular injury (51%), with femoral artery as the most frequently injured vessel (35%). They also state that though obtaining pulses is important, distal pulses can continue to be present in many limbs with arterial injuries and therefore may require confirmatory evaluation via doppler, CT scan, or even angiography. Incorrect Answers: Answer A: Angiography of the left lower extremity may be utilized if this patient did not present with hard signs of vascular injury. However, this patient's presentation warrants emergent surgical intervention. Answer B: CT scan of the left lower extremity may be utilized if this patient did not present with hard signs of vascular injury. This patient's presentation warrants emergent surgical intervention. Answer D: Observation may be appropriate if this patient was hemodynamically stable, and showed no hard or soft signs of vascular injury. Answer E: Wound closure may eventually be appropriate if this patient was hemodynamically stable, and showed no hard or soft signs of vascular injury. Bullet Summary: For patients with suspected vascular injury with hard signs of vascular injury the first step in management is emergent surgical intervention.
https://bit.ly/3QOZdTR
064
A 55-year-old woman presents to the emergency department for evaluation of headache. She had a headache that was present for a few hours before subsiding 2 days ago, and then today she developed a headache that was sudden in onset and at maximal intensity within minutes. She has never had a headache like this in the past. Her temperature is 99.0°F (37.2°C), blood pressure is 157/99 mmHg, pulse is 90/min, respirations are 16/min, and oxygen saturation is 99% on room air. On exam, she has pain with passive and active movement of the neck. No focal neurologic deficits are appreciated. A non-contrast head CT is obtained as shown in Figure A. Which of the following is the most likely underlying etiology of this patient's headache?
{ "A": "Dehydration and stress", "B": "Hypersensitivity of the trigeminal nerve", "C": "Muscular pain and strain", "D": "Rupture of a berry aneurysm", "E": "" }
D
This patient with sudden-onset headache, neck pain, and a CT scan showing blood in the basal cisterns likely has a subarachnoid hemorrhage. The most common cause of non-traumatic subarachnoid hemorrhage (SAH) is rupture of a saccular (berry) aneurysm. SAH typically presents with a sudden onset "thunderclap" headache that is often described as the "worst headache of my life." Hypertension is a common risk factor. The diagnosis can be confirmed with a non-contrast head CT. Non-contrast CT scan of the head is sufficient to rule out subarachnoid hemorrhage if performed within 6 hours of symptom onset. If the head CT is negative but SAH is still suspected, a lumbar puncture can help confirm the diagnosis (demonstrating xanthochromia) or CT angiography can be performed. Patients should be admitted for neurosurgery and neuro-IR consultation and may require calcium channel blockers and possible intervention. Macdonald et. al review spontaneous subarachnoid hemorrhage. They discuss the pathophysiology, clinical manifestations, and diagnosis. They note the typical finding of accumulation of blood in the basal cisterns and sylvian fissures on CT imaging. Figure A shows a non-contrast head CT demonstrating blood in the subarachnoid space supporting the diagnosis of SAH. Note the hyperdensity in the sylvian fissures and basal cisterns. Incorrect Answers: Answer A: CNS vascular endothelial dysfunction results in posterior reversible encephalopathy syndrome, which typically presents with hypertension and encephalopathy. Answer B: Dehydration and stress could explain a migraine headache or a tension headache. Migraine headaches are often pulsating and may be associated with aura. Answer C: Hypersensitivity of the trigeminal nerve is thought to be involved in the pathophysiology of migraine headache, and also could describe trigeminal neuralgia which presents with sudden and severe electrical pain in the distribution of the trigeminal nerve with minor stimulation. Answer D: Muscular pain and strain describes a tension headache which presents with diffuse head pain that is mild and not localized. CT imaging is normal in tension headache. Bullet Summary: Spontaneous subarachnoid hemorrhages are most commonly caused by rupture of a berry aneurysm.
https://bit.ly/3QRYhxm
065
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?
{ "A": "Dextromethorphan and quinidine", "B": "Tizanidine", "C": "Riluzole", "D": "Edaravone", "E": "" }
C
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.
https://bit.ly/3QdXJSe
066
A 27-year-old woman presents to her primary care physician with pain in her hands, shoulders, and knees. The pain has lasted for several months but seems to have worsened recently. Any activity such as opening jars, walking, or brushing her teeth is painful. The patient has a medical history of a suicide attempt in college, constipation, anxiety, depression, and a sunburn associated with surfing which was treated with aloe vera gel. Her temperature is 99.5°F (37.5°C), blood pressure is 137/78 mmHg, pulse is 92/min, respirations are 14/min, and oxygen saturation is 98% on room air. Laboratory values are obtained and shown below. Hemoglobin: 9 g/dL Hematocrit: 33% Leukocyte count: 2,500/mm^3 with normal differential Platelet count: 107,000/mm^3 Serum: Na+: 139 mEq/L Cl-: 102 mEq/L K+: 4.4 mEq/L HCO3-: 24 mEq/L BUN: 21 mg/dL Glucose: 90 mg/dL Creatinine: 1.0 mg/dL Ca2+: 10.2 mg/dL AST: 12 U/L ALT: 10 U/L Which of the following findings is the most likely to be positive in this patient?
{ "A": "Anti-cyclic citrullinated peptide antibodies", "B": "Degenerated cartilage in weight bearing joints", "C": "Anti-dsDNA antibodies", "D": "Anti-histone antibodies", "E": "" }
C
This patient is presenting with arthralgias, pancytopenia, possible malar rash, and a history of depression/abnormal behavior. Epidemiologically, the most likely diagnosis is systemic lupus erythematosus (SLE) which is associated with anti-dsDNA antibodies. SLE is an autoimmune disorder that most commonly occurs in young adult women. It presents with at least 4 criteria from SOAP BRAIN MD - Serositis, Oropharyngeal ulcers, Anti-nuclear antibodies, Photosensitivity, Blood disorders (pancytopenia and hemolytic anemia), Renal abnormalities (proteinuria), Arthralgias, Immune disease (hemolytic anemia and anti-dsDNA), Neurologic (psychosis, stroke, and seizure), Malar rash, and Discoid rash. The most common presenting symptoms for SLE can often be just arthralgias/arthritis with vague other symptoms suggestive of a rheumatologic disorder. Anti-nuclear antibodies are very sensitive for this pathology and anti-dsDNA antibodies are very specific. Treatment may include hydroxychloroquine as well as other immunomodulators and management of end-organ dysfunction. Wang and Xia review the evidence regarding double stranded DNA in patients with SLE. They discuss how these antibodies are a very specific finding in this disease. They recommend measuring these levels. Incorrect Answers: Answer A: Anti-cyclic citrullinated peptide is associated with rheumatoid arthritis which is a possible diagnosis in this patient, but it is less likely epidemiologically given her age and other symptoms of pancytopenia and a history of sunburn (which is likely a malar rash or photosensitivity). Treatment is with methotrexate. Answer C: Anti-histone antibodies would be found in drug-induced lupus. This patient's medication history is not known; however, this is a less likely diagnosis compared to SLE. Treatment is with discontinuation of the offending medication. Answer D: Degenerated cartilage in weight bearing joints describes osteoarthritis which tends to affect the knees and hips in older patients. Treatment is with conservative management or with total joint replacement. Answer E: IgM against parvovirus B19 describes a parvovirus B19 infection which commonly presents with arthralgias; however, her other symptoms point towards a diagnosis of SLE. Treatment is supportive. Bullet Summary: Arthritis/arthralgias are often the most common presenting symptom for SLE.
https://bit.ly/3Qj2QR9
067
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?
{ "A": "Ceftriaxone", "B": "Ofloxacin ear drops", "C": "Amoxicillin", "D": "Vancomycin and piperacillin-tazobactam", "E": "" }
D
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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068
A 23-year-old man presents to the emergency department for altered mental status after a finishing a marathon. He has a medical history of obesity and anxiety and is not currently taking any medications. His temperature is 104°F (40°C), blood pressure is 147/88 mmHg, pulse is 200/min, respirations are 33/min, and oxygen saturation is 99% on room air. Physical exam reveals dry mucous membranes, hot flushed skin, and inappropriate responses to the physician's questions. Laboratory values are ordered as seen below. Hemoglobin: 15 g/dL Hematocrit: 44% Leukocyte count: 8,500/mm^3 with normal differential Platelet count: 199,000/mm^3 Serum: Na+: 165 mEq/L Cl-: 110 mEq/L K+: 4.0 mEq/L HCO3-: 20 mEq/L BUN: 30 mg/dL Glucose: 133 mg/dL Creatinine: 1.5 mg/dL Ca2+: 10.2 mg/dL AST: 12 U/L ALT: 10 U/L Which of the following is the most appropriate next step in management?
{ "A": "Dextrose solution", "B": "Hypotonic saline", "C": "Lactated ringer", "D": "Normal saline", "E": "" }
C
This patient is presenting with hypernatremia and heat stroke for which the most appropriate next step in management is the administration of lactated ringer solution. Heat stroke presents with hot/flushed skin, fever, altered mental status, and hypernatremia secondary to loss of free water. Patients should immediately be cooled with external measures and started on fluids. Minor cases where the patient is cognitively intact can be treated with oral replacement with an electrolyte balanced solution (though this would be heat exhaustion and not heat stroke). Patients with altered mental status should be started on normal saline or lactated ringer, and sodium levels should be corrected slowly. Rapid correction of serum sodium or administration of hypotonic fluids can cause cerebral edema and seizures; however, this is more common in hypernatremia that has persisted for days as it has led to cerebral adaptations. Tinawi reviewed the evidence regarding the use of intravenous fluids in the hospital setting. He discusses how a large volume of normal saline can result in hypernatremia and hyperchloremia. He recommends considering the risks and benefits of colloid versus crystalloid usage. Incorrect Answers: Answers 1-3: 50% normal saline 50% dextrose, dextrose solution, and hypotonic saline are hypotonic solutions and would treat the hypernatremia too rapidly predisposing the patient to seizures. Answer E: Normal saline would be a reasonable option for this patient; however, it is less desirable given that this patient is suffering from hypernatremia, hyperchloremia, and a metabolic acidosis which could be worsened by normal saline given the large sodium and chloride load of this solution. Bullet Summary: Hypernatremia and dehydration should be treated with lactated ringer and sodium levels should be corrected no more rapidly than 1 mEq/L/hour.
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069
A newborn boy is assessed in the newborn nursery due to central cyanosis that does not respond to oxygen therapy. The patient was born to a G1P1 mother at 39 weeks gestation. The mother had diabetes that was managed with exercise and insulin during the pregnancy. The mother has never been vaccinated and did not follow routine prenatal care. The newborn's temperature is 99.5°F (37.5°C), blood pressure is 70/30 mmHg, pulse is 160/min, respirations are 27/min, and oxygen saturation is 80% on room air. The initial workup of the patient includes a chest radiograph seen in Figure A as well as lab values as seen below. Hemoglobin: 14 g/dL Hematocrit: 42% Leukocyte count: 6,500/mm^3 with normal differential Platelet count: 197,000/mm^3 Serum: Na+: 139 mEq/L Cl-: 101 mEq/L K+: 4.3 mEq/L HCO3-: 24 mEq/L BUN: 12 mg/dL Glucose: 99 mg/dL Creatinine: 0.5 mg/dL Ca2+: 10.0 mg/dL AST: 12 U/L ALT: 10 U/L An electrocardiogram is obtained as seen in Figure B. Which of the following is the most likely diagnosis?
{ "A": "Truncus arteriosus", "B": "Transposition of the great vessels", "C": "Tricuspid valve atresia", "D": "Total anomalous pulmonary venous return", "E": "" }
B
This patient is presenting with right axis deviation on ECG and an "egg on a string" heart on chest radiograph suggesting a diagnosis of transposition of the great vessels. Transposition of the great vessels occurs when the spiral septum that divides the truncus arteriosus into the aorta and pulmonary artery fails to spiral and connect the right ventricle to the pulmonary artery and the left ventricle to the aorta. Patients will present with central cyanosis and must have another cardiac defect/shunt in order to remain viable (such as an atrial septal defect, ventricular septal defect, or patent ductus arteriosus). Chest radiograph will reveal an "egg on a string" heart and ECG will reveal right axis deviation. The diagnosis is confirmed with echocardiography and treated surgically. Cohen et al. review the treatment of patients with transposition of the great vessels. They discuss how multiple surgical procedures have been performed in an attempt to address this congenital abnormality. They recommend using MRI to characterize the success of these procedures. Figure A is a chest radiograph demonstrating an elongated cardiac shadow (red circle). This classic "egg on a string" appearance of the heart is seen in transposition of the great vessels. Figure B is an ECG demonstrating right axis deviation as revealed by being down in lead I (red circle) and up in aVF. Right axis deviation is classically seen in transposition of the great vessels. Incorrect Answers: Answer A: Tetralogy of Fallot presents with a boot-shaped heart on a chest radiograph and classic "Tet spells" where patients will squat thus improving shunting and cardiac performance. Treatment is with surgical correction of the abnormal spiral septum. Answer B: Total anomalous pulmonary venous return occurs when pulmonary veins drain into the right heart circulation. It presents with cyanosis that does not correct with oxygenation but does not present with an "egg on a string" heart on a chest radiograph. Treatment is with surgical reconfiguration of pulmonary venous return. Answer D: Tricuspid valve atresia occurs when the tricuspid valve never forms and thus there is no connection between the right atrium and right ventricle. Patients will present with cyanosis and poor feeding/poor growth. Treatment is with surgical creation of a connection between these chambers. Answer E: Truncus arteriosus occurs secondary to failure of the spiral septum to divide the aorta and pulmonary artery. It presents with poor feeding, diaphoresis, tachypnea, and cyanosis. Treatment is with surgical separation of these arterial chambers. Bullet Summary: Transposition of the great vessels presents with central cyanosis, an "egg on a string" appearance on a chest radiograph, and right axis deviation on ECG.
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070
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?
{ "A": "Botulinum toxin injections", "B": "Reassurance and reassessment in 1 month", "C": "Serial casting", "D": "Surgical pinning", "E": "" }
C
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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071
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?
{ "A": "Albuterol and IV fluid resuscitation", "B": "Calcium gluconate", "C": "IV fluid resuscitation", "D": "Sodium polystyrene sulfonate", "E": "" }
B
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.
https://bit.ly/3R0iH7u
072
A 51-year-old man presents to his primary care provider for intermittent shaking of his hands. For the last several years he has noticed this "shaking" when he brushes his teeth and prepares a cup of coffee in the morning. The shaking then gradually improves over the course of the day. His medical history is otherwise notable for hypertension and hyperlipidemia. His only home medication is pravastatin. The patient smokes half a pack per day of cigarettes and drinks 2-3 beers throughout the day. His family history is significant for Parkinson disease in his father. On physical exam, his temperature is 98.6°F (37.0°C), blood pressure is 159/84, pulse is 74/min, and respirations are 12/min. He has a high-frequency bilateral hand tremor elicited on finger-to-nose testing. His neurological exam is otherwise unremarkable. Which of the following medications should this patient be started on?
{ "A": "Alprazolam", "B": "Primidone", "C": "Propranolol", "D": "Topiramate", "E": "" }
C
This patient presents with a high frequency bilateral action tremor, which suggests a diagnosis of benign essential tremor. In light of his concomitant hypertension, the patient should be started on propranolol. Benign essential tremor usually presents as a bilateral tremor of the hands that is observed during fine motor movements. Patients present with worsening tremors with posturing and with voluntary movement and improve with alcohol and rest. First-line treatment includes propranolol and primidone, with propranolol being preferred for patients with hypertension or vascular risk factors. Refractory cases can be treated with deep brain stimulation which is often a last-line intervention. Shanker reviewed the diagnosis and treatment of patients with essential tremors. She discusses how this disease presents with an action tremor that lasts for at least 3 years. She recommends treatment with propranolol or primidone. Incorrect Answers: Answer A: Alprazolam is sometimes used as second-line therapy for essential tremor refractory to propranolol and primidone but would not be used as initial therapy in this patient. Benzodiazepines can be used in patients with panic disorders. Answer B: Primidone is a barbiturate that is another first-line treatment for essential tremor. For this patient, propranolol would be preferred to also treat this patient’s underlying hypertension. Primidone can be used in patients without other underlying comorbidities. Answer D: Topiramate is an antiepileptic that may be used as a second-line treatment option for essential tremor. It would not be used as initial drug therapy. Topiramate can be used in the treatment of patients with partial seizures that present with motor symptoms. Answer E: Trihexyphenidyl is an anticholinergic medication that is used to treat early Parkinson disease in which tremor is the predominant symptom. Despite this patient’s family history of Parkinson disease, the tremor in Parkinson disease usually presents as an asymmetric, “pill-rolling” resting tremor rather than an action tremor. Bullet Summary: Propranolol is the first-line treatment for benign essential tremor in patients with concomitant hypertension.
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073
A 24-year-old man with epilepsy refractory to valproic acid, phenytoin, and levetiracetam undergoes magnetic resonance imaging of his brain while monitored by anesthetic care. He wakes up screaming in pain due to an electrocardiogram lead having caused a significant thermal burn circumferentially around his left leg. He is admitted to the medical intensive care unit for continuous electroencephalogram monitoring while on a midazolam infusion for seizure suppression and supportive care for his burn. Overnight, the nurse continued to increase the patient's midazolam infusion rate, but she also noticed that his left toes were cold to the touch with significant edema. His temperature is now 100°F (37.8°C), blood pressure is 110/75 mmHg, pulse is 80/min, respirations are 10/min and oxygen saturation is 95% on 2 liters nasal cannula. No dorsalis pedis or posterior tibial pulses are detected on the left lower extremity. A delta pressure of 25 mmHg is obtained in the left leg. What is the most appropriate next step in management?
{ "A": "Amputation", "B": "Escharotomy", "C": "Fasciotomy", "D": "Intravenous fluid infusion based on Parkland formula", "E": "" }
B
This patient with a circumferential burn due to an ECG cable wrapped around his leg in an MRI machine now has clear evidence of compartment syndrome due to the eschar. The most appropriate next step in management is emergent escharotomy to restore blood flow. Burns can be caused by chemical, thermal, electrical, and radiation sources. The initial inflammation causes vasodilatation and hyperemia, which can progress to edema and local microvascular thrombosis. If not promptly addressed, tissue necrosis and irreversible damage can occur. Circumferential burns can evolve into eschars that constrict blood flow and cause distal compartment syndrome. A delta pressure (diastolic pressure minus local compartment pressure) lower than 30 mmHg is diagnostic. In the case of eschar-induced compartment syndrome, the most appropriate next step is escharotomy to alleviate pressure. Ormiston and Marappa-Ganeshan review the evidence regarding the diagnosis and treatment of patients with compartment syndrome. They discuss how this condition can be caused by many different pathologies including burns. They recommend the emergent release of constrictive structures. Incorrect Answers: Answer A: Amputation may be necessary if the distal limb is completely necrotic and not salvageable. Timely release of the eschar can allow for reperfusion of the limb and prevent this outcome. Answer C: Fasciotomy is the treatment of choice for compartment syndrome not due to a circumferential eschar, but in this case the least invasive surgical intervention should be performed first without releasing all the compartments of the lower leg. If escharotomy does not relieve the compartment syndrome then fasciotomy may be required. Answer D: Intravenous fluid infusion based on the Parkland formula is part of comprehensive burn management, but would not address the underlying compartment syndrome, which is more urgent at this time. Answer E: Transfer to a specialized burn center can be considered since circumferential burns are one of the admission criteria, but early escharotomy should be performed first. Bullet Summary: Circumferential burns that cause eschars and compartment syndrome require an escharotomy.
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074
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?
{ "A": "Reevaluation at the next well-child visit", "B": "Biopsy", "C": "Genetic testing", "D": "Ophthalmology referral", "E": "" }
D
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.
https://bit.ly/3RhK2Tp
075
A 57-year-old woman presents to her primary care physician with weakness for the past 6 months with symptoms that have worsened recently. She feels fatigued, depressed, and has gained 10 pounds which she attributes to feeling too weak to go to the gym. She was recently treated for an ear infection with an antibiotic which she completed 3 weeks ago, and experienced a rash which she believes was an allergic reaction to her treatment. The patient has a medical history of diabetes mellitus that is well-controlled with insulin and metformin. She has been admitted multiple times for hypoglycemia secondary to missing meals. Her temperature is 99.5°F (37.5°C), blood pressure is 127/68 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for 2/5 strength in the patient's upper and lower extremities, sparse fine hair on her body, and a rash on her face and arms. Laboratory values are ordered as seen below. Serum: Na+: 139 mEq/L K+: 3.3 mEq/L Cl-: 100 mEq/L HCO3-: 25 mEq/L BUN: 20 mg/dL Glucose: 70 mg/dL Creatinine: 1.1 mg/dL Ca2+: 10.2 mg/dL AST: 12 U/L ALT: 10 U/L A muscle biopsy is obtained as seen in Figure A. Which of the following is the most likely explanation for this patient's presentation?
{ "A": "Dermatomyositis", "B": "Hypothyroidism", "C": "Inclusion body myositis", "D": "Polymyositis", "E": "" }
C
This patient who presents with weakness and a rash has a biopsy suggestive of inclusion body myositis. Inclusion body myositis presents with an insidious onset of weakness, a mildly elevated CRP and CK, and a rash. A muscle biopsy in this disease will demonstrate endomysial inflammation with basophilic-rimmed vacuoles within the muscle fiber sarcoplasm. Electromyography is typically abnormal, and the most accurate/confirmatory test is a muscle biopsy. There is no cure for this disease and no standard course of treatment. Corticosteroids are not usually effective but intravenous immunoglobulins can be effective. Naddaf et al. review the evidence regarding the diagnosis and treatment of patients with inclusion body myositis. They discuss how this disease is still refractory to treatment. They recommend a muscle biopsy to establish a definitive diagnosis. Figure/Illustration A is a muscle biopsy demonstrating endomysial inflammation with basophilic-rimmed vacuoles (red circle) within the muscle fiber sarcoplasm. These findings are classically seen in patients with inclusion body myositis. Incorrect Answers: Answers 1 & 5: Dermatomyositis and polymyositis present similarly with muscle weakness and an elevated CK, CRP, and aldolase. However, dermatomyositis presents with dermatologic findings. A biopsy would demonstrate inflammation surrounding the muscle fascicle (dermatomyositis) or within the muscle fascicle (polymyositis). Treatment may include azathioprine and methotrexate. Answer B: Hypokalemia could present with weakness and cardiac abnormalities. Though this patient is hypokalemic, her biopsy demonstrates inclusion body myositis. Treatment of hypokalemia is with potassium repletion. Answer C: Hypothyroidism presents with depression, fatigue, scarce lanugo, myopathy, and weight gain (as this patient has). Though myopathy from hypothyroidism is plausible, her symptoms of weakness have a confirmatory biopsy. Treatment of hypothyroidism is with thyroid hormone replacement. Bullet Summary: Inclusion body myositis presents with weakness and an elevated CRP/CK with a muscle biopsy demonstrating endomysial inflammation with basophilic-rimmed vacuoles within the muscle fiber sarcoplasm.
https://bit.ly/3SyN6Lf
076
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?
{ "A": "Blood smear", "B": "Sputum with acid fast stain", "C": "Serologic testing", "D": "Stool culture", "E": "" }
C
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.
https://bit.ly/3UV1eNW
077
A 25-year-old woman presents to her primary care physician with joint and muscle pain. She has a general aching/pain in her joints that lasts all day. The pain is constant and is not relieved by rest or activity. She also has pain in her hands at times and occasionally notices trouble swallowing when she is eating. She has a medical history of anxiety and is not currently taking any medications. Her temperature is 98.6°F (37°C), blood pressure is 115/72 mmHg, pulse is 74/min, and respirations are 11/min. On physical exam, the patient is a healthy young woman with a sunburn. Her hands are mildly edematous with the findings in Figure A. Laboratory values are below: Serum: Na+: 145 mEq/L K+: 4.4 mEq/L Cl-: 100 mEq/L HCO3-: 24 mEq/L BUN: 9 mg/dL Glucose: 70 mg/dL Creatinine: .7 mg/dL Ca2+: 10 mg/dL Mg2+: 1.8 mEq/L Which of the following antibodies is most likely to aid in making the diagnosis in this patient?
{ "A": "Anti-nuclear antibody", "B": "Anti-IgG Fc region", "C": "Anti-ribonuleoprotein", "D": "Anti-dsDNA", "E": "" }
C
This patient is presenting with diffuse muscle/joint pain, Raynaud phenomenon, and dysphagia suggesting a diagnosis of mixed connective tissue disease. Anti-ribonucleoprotein (U1) antibodies are found in this disease. Mixed connective tissue disease can present with a vast array of symptoms. The most common symptoms are Raynaud phenomenon (vascular hyper-reactivity that presents with burning/pain in the hands where the hands change color from white to blue to red, seen in Figure A), arthralgias, myalgias, dysphagia, and edema of the hands. Anti-U1 ribonucleoprotein antibodies are commonly found in mixed connective tissue disease and are a specific marker. In this disease, there is typically an absence of renal involvement. Treatment involves steroids as well as calcium channel blockers for symptomatic control of Raynaud phenomenon. Batu et al. review the evidence regarding patients with mixed connective tissue disease. They found that many patients have overlapping symptoms with systemic lupus erythematosus and systemic sclerosis. They recommend studying patients with these mixed characteristics in order to better understand which treatment modalities are effective. Figure/Illustration A is a clinical photograph showing the blanching of the distal fingers (red box). This finding demonstrates the Raynaud phenomenon during the early phase where the vessels have spasmed. Incorrect Answers: Answer A: Anti-dsDNA is a specific marker in systemic lupus erythematosus (SLE). SLE presents with SOAP BRAIN MD - Serositis, Oral ulcers, Arthritis, Photosensitivity, Blood disorders (hemolytic anemia, etc.), Renal involvement, ANA, Immune phenomena (anti-dsDNA, Smith), Neurological symptoms, Malar rash, and Discoid rash. Four of these criteria must be met to suspect a diagnosis of SLE. Treatment is with hydroxychloroquine. Answer B: Anti-IgG Fc region is representative of rheumatoid factor which is found in rheumatoid arthritis. Rheumatoid arthritis can present with fever, weight loss, and joint pain that worsens in the morning and improves with activity. It is common for the joints of the hands to become deformed due to the disease process. Treatment is with methotrexate. Answer C: Anti-nuclear antibodies (ANA) are likely positive in this patient; however, they are not more likely to aid in making the diagnosis than anti-ribonucleoprotein antibodies are. ANA are non-specific and can be found in many rheumatological conditions such as MCTD, lupus, and rheumatoid arthritis. Answer E: Anti-Ro antibodies are found in Sjogren syndrome which presents with dry eyes, dry mouth, dental caries, and dyspareunia. A salivary gland biopsy can be used to confirm the diagnosis. Treatment includes artificial tears, vitamin D supplementation, and pilocarpine or cevimeline. Bullet Summary: Mixed connective tissue disease is associated with anti-ribonuleoprotein antibodies and presents with Raynaud phenomenon, arthralgia/arthritis, swollen hands, sclerodactyly, and myositis.
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078
A 32-year-old G1P0 presents to the antenatal testing unit at 32 weeks of gestation for a non-stress test (NST). Yesterday she had several episodes of diarrhea and vomited once after attending a company picnic 2 days ago. She took loperamide with symptomatic relief. She is worried that she is dehydrated but otherwise feels well and has recovered from her gastrointestinal illness. The patient was diagnosed with gestational diabetes after routine screening in the 2nd trimester and has been started on insulin after unsuccessful attempts at lifestyle management. She also has migraines and gastroesophageal reflux disorder. The patient has a 15-pack-year smoking history but quit smoking in her 1st month of pregnancy. She denies alcohol or drug use. Her temperature is 98.5°F (36.9°C), blood pressure is 122/82 mmHg, pulse is 84/min, and respirations are 14/min. A representative portion of the NST is shown in Figure A. Which of the following is the most likely explanation for this patient’s non-stress test (NST) results?
{ "A": "Maternal medication use", "B": "Normal results", "C": "Fetal sleep cycle", "D": "Maternal smoking", "E": "" }
C
This patient presents with a nonreactive NST on routine testing. The most common explanation for a nonreactive NST is the fetal sleep cycle. Patients with gestational diabetes are at increased risk of fetal demise and other perinatal complications. They require closer monitoring during the 3rd trimester of pregnancy. The NST, which lasts 20 minutes, is the most common method of doing so. After 32 weeks, a reactive (i.e. “normal”) NST requires at least 2 episodes of 15-beat-per-minute accelerations lasting for at least 15 seconds. An NST with no accelerations is considered nonreactive. A common cause of a nonreactive NST is the fetal sleep cycle and it is reasonable to continue the test for an additional 20 minutes to increase the chance of monitoring the fetus while awake. Otherwise, a nonreactive NST should prompt vibroacoustic stimulation or further testing such as a biophysical profile (BPP). Umana and Siccardi review the evidence regarding the use of the NST during pregnancy. They discuss how the test is a measurement of fetal cardiovascular function at the time of the test. They recommend monitoring the baseline fetal heart rate and variability as well as the presence of accelerations and decelerations when interpreting test results. Figure/Illustration A shows a representative portion of a NST where the fetal heart rate remains within a narrow band without accelerations (red circle). This pattern is characteristically seen in a nonreactive NST that can be due to the fetal sleep cycle. Incorrect Answers: Answer B: Maternal dehydration generally should not affect the reactivity of an NST. Although severe dehydration may reduce amniotic fluid volume and therefore increase the baseline fetal pulse, it is unlikely to change the accelerations or variability in an NST. Though this patient had diarrhea the day before, her pulse is not elevated and any dehydration is probably not clinically significant. Answer C: Maternal medication use can cause nonreactivity on an NST if drugs such as morphine or other sedatives are taken. Although this patient did take loperamide (an opioid), it is unlikely that this has a sedative effect on the fetus as it acts selectively on the mu-opioid receptors in the large intestine and does not cross the blood-brain barrier. Answer D: Maternal smoking in close proximity to an NST has been linked to nonreactivity, but this patient has not smoked in several months. Mothers who smoke are counseled to avoid smoking on the day of their NST to avoid the effects of nicotine on their test results. Answer E: A normal result is known as a reactive NST and would present with accelerations and no concerning or late decelerations. This is not a reactive NST due to the lack of appropriate accelerations. Bullet Summary: The most common cause of a non-reactive non-stress test is the fetal sleep cycle.
https://bit.ly/3Waen6G
079
A 27-year-old G1P0000 woman presents to her obstetrician’s office at 24 weeks gestation complaining of new hair growth on her upper lip. She now needs to shave her face every day or else thick hair appears. She also has worsening acne that started several weeks ago. Her pregnancy thus far has been complicated by gestational diabetes, which is managed by diet and exercise. She has a male fetus based on her 20 week anatomy ultrasound. The patient has a medical history of polycystic ovary syndrome. The patient’s temperature is 98.5°F (36.9°C), blood pressure is 121/76 mmHg, pulse is 70/min, and respirations are 13/min. The cardiopulmonary exam is normal, and the patient’s abdomen has a fundal height of 31 cm. A pelvic exam reveals an unremarkable closed cervix and a right-sided adnexal mass without tenderness. Transvaginal ultrasound is performed and shown in Figure A. Which of the following is the most appropriate next step in management?
{ "A": "Spironolactone to treat hirsutism and acne", "B": "Laparoscopy to remove a malignancy", "C": "Reassurance", "D": "Percutaneous umbilical blood sampling to determine prognosis", "E": "" }
C
This patient presents with hirsutism and acne, symptoms of hyperandrogenism, as well as a solid pelvic mass on ultrasound, most consistent with a luteoma. The most appropriate next step in management is reassurance as the mass tends to self-resolve after pregnancy. Luteomas are ovarian tumors that arise during pregnancy and produce androgens, causing maternal and sometimes fetal virilization. They appear as solid masses on ultrasound and are not malignant. The likelihood of virilizing the fetus increases if the luteoma arises in the 1st trimester and affected female fetuses present with clitoromegaly or labioscrotal fusion. Male fetuses are not affected. Treatment is supportive as the masses tend to resolve after pregnancy. Wang et al. review the evidence regarding the diagnosis and treatment of luteomas. They discuss how this is a rare tumor that primarily affects women who have had multiple pregnancies. They recommend early diagnosis of this condition in order to inform treatment options. Figure/Illustration A shows a transvaginal ultrasound with a solid mass on the right ovary (red circle). This appearance is consistent with a luteoma. Incorrect Answers: Answer A: Laparoscopy to prevent detrimental effects on the fetus is not considered a treatment option for luteomas. In this patient, the exposure to high androgen levels is late in pregnancy and the fetus is a male, making it especially unnecessary to perform surgery. Answer B: Laparoscopy to remove a malignancy is not indicated for luteomas, which are benign tumors and self-regress after delivery. Malignancies that can cause virilization in or outside of pregnancy include Sertoli-Leydig cell tumors and Krukenberg tumors. Answer C: Percutaneous umbilical blood sampling to determine prognosis may be performed in women who have early virilization and female fetuses. High androgen levels in the umbilical cord are required for the virilization of the fetus, whereas high androgen levels in the mother’s peripheral blood may not predict this outcome. Answer E: Spironolactone to treat hirsutism and acne is used outside of pregnancy in women with polycystic ovary syndrome. However, because it is an anti-androgen, spironolactone should not be used in pregnancy as it can cause feminization of male fetuses. Bullet Summary: Luteomas generally do not require treatment as they regress after pregnancy.
https://bit.ly/3YmWmmI
080
A 6-year-old boy is brought to the emergency department by his father for elbow pain. He was riding his bicycle when he fell onto his outstretched right hand. He immediately clutched his right elbow in pain and began crying. He refuses to move his elbow. He has no medical problems and takes no medications. His temperature is 98.4°F (36.9°C), blood pressure is 108/64 mmHg, pulse is 100/min, and respirations are 20/min. On exam, the boy is holding his right wrist with his left hand and cries upon palpation of a region that is 2 cm proximal to his elbow. The radial head is palpated just distal to the lateral epicondyle. He is unable to make a pincer with his right thumb and index finger. When asked to hold a pen between his right thumb and index fingers, he drops it. The radial pulse is diminished on the right. Which of the following is the most likely diagnosis?
{ "A": "Distal radius fracture", "B": "Both bone forearm fracture", "C": "Supracondylar humerus fracture", "D": "Olecranon fracture", "E": "" }
C
This young patient presenting with elbow pain and associated signs of anterior interosseous nerve neurapraxia (inability to make pincer, weak pincer grasp) and vascular injury (diminished radial pulse) after a fall on an outstretched hand most likely has a supracondylar humerus fracture. Supracondylar humerus fractures are one of the most common fractures seen in the pediatric population. This site is particularly prone to fracture due to the thin bone separating the coronoid and olecranon fossae in the supracondylar region. Due to the proximity of the brachial artery and median nerve, which course anteriorly, these structures are at risk for injury. Neurapraxia of the anterior interosseous nerve, which is a pure motor branch of the median nerve, presents as motor deficits in interphalangeal (IP) joint flexion of the thumb and distal IP joint flexion of the index finger. The radial and ulnar arteries are terminal branches of the brachial artery, which if injured results in diminished distal pulses. The diagnosis is made with anteroposterior and lateral view elbow radiographs. Radiographs will demonstrate the "fat pad sign" seen as a posterior fat pad that is indicative of fracture. Management depends on fracture type but can consist of cast immobilization (if minimally displaced with no neurovascular injury) or closed reduction and percutaneous pinning (for more displaced fractures). Micheloni et al. reviews supracondylar fractures in children. They note it is the most common elbow fracture in the pediatric population. They recommend early and correct diagnosis and management to avoid early and late complications including neurovascular impairment and malunion. Incorrect Answers: Answer A: Both bone forearm fracture is another common pediatric fracture sustained from a fall, involving both the radius and ulna. It can be associated with neurovascular injury depending on the plane of deformity, but the location of the pain would be in the forearm, not proximal to the elbow. Answer B: Distal radius fracture is a common fracture after a fall on an outstretched hand mechanism of injury. Neurovascular injury is possible, though uncommon. This patient’s pain is localized around his elbow, rather than near the distal radius. Often, a deformity can be visualized on exam. Answer C: Monteggia fracture-dislocation is characterized by a proximal ulna fracture with radial head dislocation. It is commonly caused by direct trauma to the ulna. In contrast, this patient’s radial head is located inferior to the lateral epicondyle. Answer D: Olecranon fracture is an uncommon pediatric fracture caused primarily by a fall onto the elbow in flexion. Median nerve and brachial artery injury are unlikely, given that these structures pass anteriorly in the cubital fossa. Bullet Summary: Supracondylar humerus fractures are common in the pediatric population and may present with elbow pain and concomitant median nerve and/or brachial artery injury.
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081
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?
{ "A": "Barium swallow", "B": "Urgent surgery", "C": "Magnetic resonance imaging", "D": "Gastrografin swallow", "E": "" }
D
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.
https://bit.ly/3ZLKJGM
082
A 4-day-old girl presents with her parents to the pediatrician for a routine visit. She was feeding well until this morning when she had several episodes of vomiting. The parents describe the vomitus as “bright green” and the patient has shown little interest in feeding since then. She was born at 36 weeks gestation to a 37-year-old G2P2 mother. The pregnancy was complicated by gestational diabetes, and all prenatal testing was unremarkable. The patient has not yet regained her birth weight and is in the 46th percentile for height and the 36th percentile for weight. The patient’s temperature is 99.2°F (37.3°C), blood pressure is 68/46 mmHg, pulse is 132/min, and respirations are 32/min. On physical exam, the patient is in mild distress. She has no dysmorphic features. Her abdomen is distended and firm without guarding. Bowel sounds are hypoactive. The patient’s abdominal radiograph can be seen in Figure A and the patient’s upper gastrointestinal series can be seen in Figure B. Which of the following is the most likely etiology of this patient’s condition?
{ "A": "Hirschsprung disease", "B": "Duodenal atresia", "C": "Meconium ileus", "D": "Intestinal malrotation", "E": "" }
D
This patient presents with bilious vomiting and an upper gastrointestinal series demonstrating the “corkscrew” appearance of the distal duodenum and proximal jejunum, which is consistent with a diagnosis of intestinal malrotation. Intestinal malrotation most commonly presents in newborns with bilious emesis. This condition increases the risk of midgut volvulus where the intestines create a closed loop. Abdominal radiographs are rarely diagnostic but are useful in excluding bowel perforation. An upper gastrointestinal series is the diagnostic test of choice and demonstrates an abnormally placed duodenum with a ligament of Treitz on the right side of the abdomen and a “corkscrew” appearance of the distal duodenum and proximal jejunum. Treatment is with surgical derotation of the bowel or exploratory laparotomy if perforation has occurred. Adams and Stanton review the evidence regarding the diagnosis and treatment of neonatal bowel obstruction. They discuss how malrotation, jejunoileal atresia, duodenal atresia, and colonic atresia are common causes of this finding. They recommend surgical management of these conditions. Figure/Illustration A is an abdominal radiograph showing a paucity of air in the abdomen (red circle). These findings are consistent with nonspecific findings of intestinal malrotation. Figure/Illustration B is an upper gastrointestinal series showing a right-sided ligament of Treitz and a “corkscrew” appearance of the distal duodenum (red circle). These are classic findings of intestinal malrotation. Incorrect Answers: Answer A: Although duodenal atresia presents with bilious vomiting, the “double bubble” sign would be seen on an abdominal radiograph, which reflects air trapping in both the stomach and the 1st portion of the duodenum. Treatment is with surgical repair of the obstructed segment. Answer B: Hirschsprung disease may also cause bilious vomiting, but an abdominal radiograph would demonstrate proximal colonic distension with little air in the rectosigmoid colon. Hirschsprung disease is also well visualized on contrast enema, on which a transition point can be seen between the proximal dilated colon and the narrow sigmoid. Treatment is with excision of the defective segment. Answer D: Jejunal atresia can also lead to bilious vomiting, although a “triple bubble” sign would be seen on an abdominal radiograph with air additionally filling the proximal jejunum. Treatment is with excision of the atretic segment and anastomosis of the bowel lumen. Answer E: Meconium ileus may present with bilious emesis, but multiple dilated loops of bowel would be seen on an abdominal radiograph. Meconium ileus is better characterized by contrast enema, on which a microcolon can be visualized. Treatment is with the removal of the meconium and treatment of the underlying cystic fibrosis, if present. Bullet Summary: Intestinal malrotation presents in newborns with bilious emesis, an upper gastrointestinal series demonstrating a misplaced duodenum, and a “corkscrew” appearance of the distal duodenum and proximal jejunum.
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083
A 27-year-old woman presents to the emergency department with altered mental status. Her boyfriend found her at home next to a suicide note and brought her into the emergency department. According to her boyfriend, the patient has a medical history of bipolar disorder for which she takes lithium, as well as chronic lower back pain for which she sees a pain specialist. Her temperature is 100.5°F (38.1°C), blood pressure is 102/78 mmHg, pulse is 127/min, respirations are 19/min, and oxygen saturation is 99% on room air. An ECG is performed as seen in Figure A. Which of the following findings is also likely to be present at some point in this patient’s course?
{ "A": "Miosis", "B": "Diaphoresis", "C": "Urinary retention", "D": "Wheezing", "E": "" }
C
This patient is presenting after a suicide attempt with an unknown overdose and is confused, febrile, tachycardic, and has QRS widening on ECG. This combination of symptoms is highly concerning for a tricyclic antidepressant (TCA) overdose which can cause an anticholinergic toxidrome and urinary retention. Tricyclic antidepressants (TCAs) were once a mainstay in the treatment of depression before the advent of selective serotonin reuptake inhibitors (SSRIs) and serotonin–norepinephrine reuptake inhibitors (SNRIs). They are sometimes still used for depression and neuropathic pain. In overdose, TCAs block sodium channels in the heart and cause a pathognomonic QRS widening. Sodium bicarbonate should be administered until the QRS duration normalizes. TCAs also have anticholinergic properties and can cause an anticholinergic toxidrome including delirium, confusion, constipation, decreased bowel sounds, urinary retention, dry, warm, and flushed skin, and fever. Although physostigmine is typically the antidote of choice for an anticholinergic toxidrome, it is avoided in TCA toxicity due to the theoretical concern for inducing a cardiac arrest. Urquhart et al. studied the utility of tricyclic antidepressants in the treatment of chronic lower back pain. They found no significant difference in pain reduction compared to placebo. They recommend further study of the potential benefits of TCAs, independent of sleep improvement, for the treatment of lower back pain. Figure/Illustration A is an ECG demonstrating QRS widening (note how long the red lines are which demarcate the QRS duration) which is highly concerning for TCA overdose. Incorrect Answers: Answer A: Diaphoresis, tachycardia, increased bowel sounds, and agitation would be seen in a sympathomimetic toxidrome from drugs like cocaine or amphetamines. Rather, dry, flushed skin would be seen in an anticholinergic toxidrome. Answer B: Jaundice, nausea, vomiting, and abdominal pain would be seen in acetaminophen overdose which can cause fulminant liver failure. It is also possible that patients may initially be asymptomatic in acetaminophen overdose, and a high level of suspicion must be maintained for this diagnosis. A serum acetaminophen level should be drawn in any patient suspected of overdose. Answer C: Miosis, bradypnea, decreased bowel sounds, and somnolence would be seen in an opioid toxidrome. Rather, dilated and minimally responsive pupils would be seen in an anticholinergic toxidrome. Answer D: Wheezing, bronchorrhea, urinary incontinence, fecal incontinence, confusion, and drooling would be seen in a cholinergic toxidrome from agents such as organophosphates or nerve gases like sarin. None of these symptoms are seen in this patient. Bullet Summary: A tricyclic antidepressant overdose can cause QRS widening on ECG as well as an anticholinergic toxidrome (dry, flushed skin, constipation, decreased bowel sounds, urinary retention, and altered mental status).
https://bit.ly/3ZzPHpE
084
A 32-year-old man presents to his primary care provider for a headache. He reports that he has headaches at night several times a week. He first developed these headaches over 1 year ago, but they started up again 3 weeks ago. The episodes start suddenly and feel like a stabbing, electrical pain over his left eye. He also reports tearing of the left eye during these episodes. The headaches self-resolve over the course of 2-3 hours, but the patient complains that he is avoiding going to sleep for fear of waking up in pain. His medical history includes type 1 diabetes mellitus and an episode of herpes zoster on his right flank 1 year ago. His only home medication is insulin. His temperature is 98.6°F (37°C), blood pressure is 112/69 mmHg, pulse is 61/min, and respirations are 14/min. On physical exam, his extraocular muscles are intact and his eyes are not injected. A CT of the head and sinuses shows no acute abnormalities. Which of the following is most likely to prevent future episodes of headache in this patient?
{ "A": "Verapamil", "B": "Sumatriptan", "C": "Carbamazepine", "D": "High-flow oxygen", "E": "" }
A
This patient presents with an episodic, stabbing pain in the periorbital region that occurs at night, which is consistent with a diagnosis of cluster headaches. Verapamil is used as prophylaxis against cluster headaches. Cluster headaches usually occur at night and may wake patients out of sleep. Cluster headaches may occur several times a week to multiple times daily and the attacks may be followed by a period of remission. Cluster headaches also typically involve the periorbital region and are unilateral. Patients also classically describe their headaches as “stabbing”. Tension-type headaches are more commonly described as dull and migraines are usually described as pulsating or throbbing. Treatment is with high-flow oxygen during acute episodes and prophylaxis against long-term headaches includes calcium channel blockers such as verapamil. Hoffman and May review the evidence regarding the treatment of cluster headaches. They discuss how high-flow oxygen and triptans are the most effective abortive treatment for acute attacks. They recommend using chronic prophylactic medications such as verapamil. Incorrect Answers: Answer A: Carbamazepine is the treatment of choice for trigeminal neuralgia, which presents as shooting pain in the face. They have exquisite tenderness to palpation over the affected neurologic distribution. Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in men with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye. This treatment is not effective for long-term prophylaxis. Answer C: Sumatriptan is used as an abortive treatment for cluster headaches and migraines, but it is not used as prophylaxis for either. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Answer D: Topiramate is used as prophylaxis for migraines rather than cluster headaches. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Bullet Summary: Cluster headaches classically present as sharp or stabbing periorbital headaches that occur at night, with verapamil being the prophylactic treatment of choice and high-flow oxygen being the most effective abortive treatment.
https://bit.ly/3n1kBse
085
A 67-year-old man presents to the emergency department with shortness of breath. His dyspnea began 1 hour ago while he was sitting in a chair watching television. He also endorses lightheadedness and fatigue but denies chest pain. Two weeks ago, he had a mitral valve repair for mitral insufficiency secondary to myxomatous degeneration. His medical history is otherwise significant for hypertension and diabetes mellitus, for which he takes metformin and lisinopril. His temperature is 98.8°F (37.1°C), blood pressure is 85/54 mmHg, pulse is 48/min, and respirations are 22/min. On physical exam, he appears to be in moderate distress. He has normal cardiac sounds with an irregular rhythm. His lungs are clear to auscultation bilaterally, and his electrocardiogram can be seen in Figure A. The patient is given an initial dose of atropine but this has no effect. Which of the following is the most appropriate next step in management?
{ "A": "Cardioversion", "B": "Atropine", "C": "Procainamide", "D": "Transvenous pacing", "E": "" }
D
This patient who presents with lightheadedness has an ECG consistent with a Mobitz type 2 2nd-degree heart block. Because the patient is symptomatic from his dysrhythmia and it did not respond to atropine, the most appropriate next step is transvenous pacing. Mobitz type 2 2nd-degree heart block can be distinguished from Mobitz type 1 (Wenckebach) by the length of the PR interval. Whereas the PR interval in Mobitz type 1 becomes progressively longer with each beat, the PR intervals in Mobitz type 2 are consistent in length and the QRS complexes drop suddenly. While Mobitz type 1 heart block is usually caused by atrioventricular (AV) node dysfunction, Mobitz type 2 is caused by dysfunction below the AV node in the His-Purkinje system. Patients with either type of heart block can present with lightheadedness or syncope. Since this pattern has a risk of progressing into 3rd-degree heart block, hemodynamic compromise, and asystole, Mobitz type 2 heart block is an indication for placement of a permanent pacemaker. Unstable patients who do not respond to atropine and/or other medical management should undergo transcutaneous or transvenous pacing. Clark and Prystowsky present the pathophysiology of heart block. They discuss how dysfunction at the infranodal level leads to type 2 2nd-degree heart block. They recommend pacing in symptomatic patients. Figure/Illustration A is an ECG that is characterized by consistent PR intervals (red lines) and sudden drops in the QRS complex. This pattern is classically seen with a Mobitz type 2 2nd-degree heart block. Incorrect Answers: Answer A: Adenosine can be used to terminate supraventricular tachycardia. These patients will present with tachycardia rather than bradycardia and will experience palpitations in addition to lightheadedness. The rhythm is rapid and regular with a narrow QRS complex. Answer C: Cardioversion can be used to convert atrial flutter, atrial fibrillation, stable monomorphic ventricular tachycardia, or recalcitrant supraventricular tachycardia (non-responsive to adenosine or vagal maneuvers) to sinus rhythm, but it is not used for 2nd-degree heart block. Answer B: Atropine can improve Mobitz type 1 2nd-degree heart block but tends not to be effective in Mobitz type 2 heart block in a stable patient. Atropine might be an appropriate treatment in symptomatic 3rd-degree heart block or Mobitz type 2 heart block; however, if it fails to improve a patient's symptoms, pacing is the most appropriate next step. Answer D: Procainamide is a class 1A antiarrhythmic medication used to treat Wolff-Parkinson-White syndrome. This would present with an upgoing delta wave that occurs prior to the QRS complex. This medication does not play a role in the management of 2nd-degree heart block. Bullet Summary: Hemodynamically unstable patients with Mobitz type 2 2nd-degree heart block should be managed with transvenous pacing followed by a permanent pacemaker.
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086
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?
{ "A": "Dorsal striatum atrophy on head CT", "B": "Neurofibrillary tangles on brain biopsy", "C": "Alpha-synuclein aggregates on brain biopsy", "D": "Frontotemporal atrophy on head CT", "E": "" }
A
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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087
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?
{ "A": "Between periosteum and galea aponeurosis", "B": "Between periosteum and skull", "C": "Between scalp and galea aponeurosis", "D": "Into the lateral ventricles", "E": "" }
C
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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088
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?
{ "A": "Lactated ringer bolus", "B": "N-acetylcysteine", "C": "Charcoal", "D": "Syrup of ipecac", "E": "" }
B
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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089
A 65-year-old man presents to the emergency department for evaluation of left arm and leg weakness. He was walking in the park about 45 minutes prior to arrival when the patient stumbled and fell. He had sudden weakness in his left leg and was unable to grasp a handrail with his left hand. He currently takes no medications. His temperature is 98.0°F (36.6°C), blood pressure is 128/89 mmHg, pulse is 130/min, and respirations are 18/min. On exam, he has 3/5 strength in the left upper and lower extremities and a left-sided facial droop. A non-contrast CT scan of the head is performed, which suggests parenchymal ischemia in the distribution of the right middle cerebral artery. An ECG is performed, as shown in Figure A. He is given intravenous alteplase with rapid improvement in his strength deficits and facial asymmetry. Which of the following is the most appropriate long-term therapy for secondary stroke prevention for this patient?
{ "A": "Aspirin", "B": "Rivaroxaban", "C": "Cardiac pacemaker", "D": "Atorvastatin", "E": "" }
B
This patient presents with acute onset unilateral left-sided weakness secondary to an ischemic stroke in the right MCA in the setting of an irregularly irregular rhythm without P waves (suggesting that he had an embolic stroke secondary to atrial fibrillation). First-line primary and secondary stroke prevention in patients with atrial fibrillation involves long term anticoagulation with a direct oral anticoagulant (DOAC) such as rivaroxaban. Atrial fibrillation is caused by uncoordinated atrial contractions, typically due to atrial enlargement, inflammation, or infiltrative disease. Atrial fibrillation can lead to the formation of a thrombus, typically in the left atrial appendage, which can embolize to the brain and lead to an ischemic stroke. For this reason, there is a low threshold to start patients on long term anticoagulation for both primary and secondary stroke prevention (risk stratification is done with the CHA2DS2-VASc score, which considers several cardiovascular risk factors, age, sex, and diabetes). First-line long term anticoagulation is a DOAC medication such as apixaban, dabigatran, rivaroxaban, or edoxaban. Delayed initiation of anticoagulation is recommended at 4-14 days after a stroke, due to the interim risk of hemorrhagic conversion. Further management of atrial fibrillation includes a rate control agent such as a beta blocker or a calcium channel blocker. JS Alpert reviews the latest American Heart Association/American College of Cardiology guidelines regarding atrial fibrillation. The article reports that for stroke prevention in atrial fibrillation, DOACs are first-line compared to warfarin due to a decreased risk for bleeding with DOACs. In patients with moderate to severe mitral stenosis or a mechanical heart valve, warfarin is the preferred agent. Figure/Illustration A shows an ECG with an irregularly irregular rhythm (highlighted by the spacing of blue arrows) and no discernable P waves before QRS complexes, which is characteristic of atrial fibrillation. Incorrect Answers: Answer A: Aspirin exerts antiplatelet adhesion effects through the inhibition of cyclooxygenase. Patients with atrial fibrillation who are at low risk for stroke (CHA2DS2-VASc score of < 1) can use aspirin monotherapy for stroke prophylaxis. This patient presented after a stroke and will require long term anticoagulation, not anti-platelet monotherapy for stroke prevention. Answer B: Atorvastatin is an HMG-CoA reductase inhibitor, which works to lower LDL cholesterol and triglycerides, and increase HDL cholesterol. It is most useful in secondary stroke prevention in patients who have suffered a thrombotic or thromboembolic stroke secondary to atherosclerosis. This patient may have atherosclerosis and could benefit from statin therapy; however, anticoagulation for his underlying atrial fibrillation is the highest priority. Answer C: A cardiac pacemaker is indicated in a large variety of cardiac conditions that primarily involve sinus node dysfunction or high-grade atrioventricular block. Atrial fibrillation does not typically require a pacemaker, as it is typically managed with rate control agents such as beta-blockers, calcium channel blockers, digoxin, or with electrical cardioversion. Rate control will not lower the stroke risk, and thus long-term anticoagulation is required. Answer E: Warfarin has historically been a mainstay anticoagulant for stroke prevention in atrial fibrillation. DOACs are non-inferior to warfarin, and have a lower risk of bleeding. Note, only the initiation of warfarin requires a heparin bridge to mitigate the transient protein C deficiency that results in a temporary hypercoagulable state (often presenting as warfarin skin necrosis). Bullet Summary: For patients who present with an ischemic stroke secondary to a cardiac embolus from atrial fibrillation, first-line long term anticoagulation is a direct oral anticoagulant (apixaban, dabigatran, rivaroxaban, or edoxaban).
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090
A 62-year-old man presents to the emergency department with skin lesions. He felt itchy recently, then noticed skin lesions that broke out prompting him to come to the emergency department. He recently went camping and hiking in the woods. The patient drinks 1-2 beers per day and smokes 1 pack of cigarettes per day. He is currently sexually active with multiple partners and does not use condoms. The patient was discharged 1 week ago for pneumonia which was treated with azithromycin; however, he feels that his symptoms may be returning. His temperature is 99.5°F (37.5°C), blood pressure is 157/78 mmHg, pulse is 90/min, respirations are 15/min, and oxygen saturation is 98% on room air. Physical exam reveals the findings in Figure A on the patient's arm. The skin lesions are thick and do not break when pressure is applied. Which of the following most appropriately describes the most likely diagnosis?
{ "A": "IgG in a fish-net pattern", "B": "Type IV hypersensitivity reaction", "C": "Linear IgG along the basement membrane", "D": "Multi-nucleated giant cells", "E": "" }
C
This patient is presenting with pruritic bullous skin lesions with a thick/tough covering suggesting a diagnosis of bullous pemphigoid. Linear IgG binding of anti-hemidesmosome antibodies along the basement membrane is the underlying cause of this disease. Bullous pemphigoid presents with a urticarial prodrome in elderly patients followed by the formation of tense bullae that do not break when pressure is applied to them. IgG antibodies bind the hemidesmosome along the basement membrane and appear linear on immunofluorescence. Risk factors that are associated with this condition include the initiation of new medications such as loop diuretics, metformin, and neuroleptics. Steroids are the treatment of choice. Miyamoto et al. review the evidence regarding the diagnosis and treatment of bullous pemphigoid. They discuss how high-potency corticosteroids are the mainstay of treatment for this disease. They recommend considering adjuvant therapy with doxycycline, dapsone, and immunosuppressants. Figure/Illustration A is a clinical photograph showing bullous skin lesions (red circles). These lesions are tense and do not separate/break when pressure is applied to them and are classically seen in bullous pemphigoid. Incorrect Answers: Answer A: IgG in a fish-net pattern suggests a diagnosis of pemphigus vulgaris, which presents with skin lesions that are flaccid and break easily when pressure is applied. Treatment is with corticosteroids. Answer C: Multi-nucleated giant cells suggests a diagnosis of HSV or herpes zoster virus, which presents with potentially painful vesicles distributed in a dermatomal pattern (for zoster). Treatment is with antivirals such as valacyclovir. Answer D: Panniculitis suggests a diagnosis of erythema nodosum, which presents with painful, raised nodules that are associated with inflammatory disorders and infection. Treatment is centered on addressing the underlying pathology. Answer E: Type IV hypersensitivity reaction suggests a diagnosis of contact dermatitis, which presents with pruritic lesions after exposure to a particular antigen such as poison ivy, poison oak, or nickel. Treatment is avoidance of the precipitating agent and steroids as well as symptomatic control. Bullet Summary: Bullous pemphigoid occurs secondary to anti-hemidesmosome antibodies which appear linear along the basement membrane on immunofluorescence.
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091
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?
{ "A": "MRI of the head", "B": "Piperacillin-tazobactam", "C": "Dexamethasone", "D": "Amoxicillin-clavulinic acid", "E": "" }
B
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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092
A 64-year-old woman presents to the emergency room with whole-body itching. She noticed her symptoms while in the bathtub at home. She has never had symptoms like this before. Over the previous several months she has had episodes of joint swelling and pain in her hands as well as redness, burning pain, and swelling of her hands and feet. Her medical history is significant for type 2 diabetes mellitus, hypertension, and osteoporosis for which she takes metformin, enalapril, and alendronate. She was found to have a deep vein thrombosis of her left leg 3 months prior to presentation. Her temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 135/85 mmHg, and respirations are 13/min. Physical exam is notable for a woman in discomfort with excoriations over the skin on her forearms. Laboratory tests are shown below. Serum: Na+: 135 mEq/L Cl-: 100 mEq/L K+: 5.0 mEq/L HCO3-: 22 mEq/L BUN: 19 mg/dL Glucose: 130 mg/dL Creatinine: 1.0 mg/dL Hematocrit: 64% Leukocyte count: 19,000 cells/mm^3 with normal differential Platelet count: 900,000/mm^3 Which of the following is the most appropriate long-term treatment?
{ "A": "Hydroxyurea", "B": "Cyclophosphamide", "C": "Prednisone", "D": "Diphenhydramine", "E": "" }
A
This patient presents with polycythemia, leukocytosis, and thrombocytosis in the setting of pruritus after bathing, as well as episodes suggestive of acute gout flares. These findings are consistent with polycythemia vera, which can be treated with hydroxyurea. Polycythemia vera is a malignancy of the bone marrow that results in the overproduction of red blood cells, platelets, and white blood cells. Classic symptoms include pruritus after hot baths as well as swelling, burning pain, and rubor of the hands and feet (erythromelalgia). Patients may also have gout due to increased cell turnover leading to hyperuricemia. Older patients (> 60 years old) and those with prior thrombosis should be treated with a myelosuppressive agent, most commonly hydroxyurea with or without aspirin. Therapeutic phlebotomy is often indicated on these patients. Stuart and Viera review the evidence regarding the diagnosis and treatment of polycythemia vera. They discuss how treatment includes phlebotomy with the possible addition of myelosuppressive agents. They recommend consultation with a hematologist for any patients with this syndrome. Incorrect Answers: Answer A: Cyclophosphamide is a chemotherapeutic agent that is used in the treatment of certain leukemias and lymphomas, as well as severe symptoms of autoimmune disease. A malignancy would present with vague systemic symptoms, weight loss, malaise, and abnormalities detected on CBC. Cyclophosphamide is not used in the treatment of polycythemia vera. Answer B: Diphenhydramine is a 1st-generation antihistamine that can be used to treat pruritus, but would not be indicated for the treatment of polycythemia vera which is the underlying condition. This treatment represents a symptomatic treatment versus an agent that addresses the underlying cause. Answer C: Febuxostat is a xanthine oxidase inhibitor that is used to reduce uric acid levels in the management of chronic gout. Gout would present with acute severe joint pain (typically the great toe) with risk factors of alcohol use, obesity, and thiazide use. It is not used in the acute management of gout or in polycythemia vera. Answer E: Prednisone is a glucocorticoid that is used in many different clinical situations but is not used in the management of polycythemia vera. It could be used in acute inflammatory conditions (like Crohn disease or granulomatosis with polyangiitis) or to reduce inflammation during a flare of asthma or COPD. Bullet Summary: Polycythemia vera presents with pruritus after bathing, erythromelalgia, and gout and should be treated with hydroxyurea with or without aspirin.
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093
A 27-year-old woman presents to her primary care physician with new hair growth on her face and lower abdomen over the last month. She has started to develop pimples on her face and back over the last several months. Her last menstrual period was over 3 months ago and her periods have been irregular over the last year. She has been gaining weight recently. The patient has a medical history of obesity and prediabetes with a hemoglobin A1c of 6.0% last year. Her temperature is 98.5°F (36.9°C), pulse is 80/min, blood pressure is 139/88 mmHg, and respirations are 13/min. Cardiopulmonary exam is unremarkable, and the patient’s abdomen appears slightly distended but exhibits no tenderness to palpation. The patient’s face has coarse stubble along the jawline and on the upper lip, and there is similar hair along the midline of her lower abdomen. A pelvic exam reveals mild clitoromegaly, a normal anteverted uterus, and a large left adnexal mass that is mildly tender. Her laboratory test results are shown below: Hemoglobin: 13.9 g/dL Leukocyte count: 8,000 cells/mm^3 Platelet count: 142,000/mm^3 DHEAS: 73 ug/dL (Normal: 145-395 ug/dL) Testosterone: 256 ng/dL 17-hydroxyprogesterone: 214 ng/dL (Normal: < 200 ng/dL) Ultrasound findings are shown in Figure A. Which of the following is the most likely diagnosis in this patient?
{ "A": "Polycystic ovary syndrome", "B": "Adrenal tumor", "C": "Ovarian tumor", "D": "Ovarian hyperthecosis", "E": "" }
C
This young woman presents with rapid virilization, elevated testosterone, and a large left adnexal mass that is primarily solid on ultrasound. These findings are most consistent with an ovarian androgen-secreting tumor. Sertoli-Leydig cell tumors are the most common androgen-secreting tumor of the ovaries and classically result in rapid virilization of affected women. Testosterone levels are often over 200 ng/dL, and 17-hydroxyprogesterone may also be slightly elevated as it is an androgen precursor. The androgen excess may result in symptoms such as facial and abdominal hirsutism, oligomenorrhea, deepening voice, and acne. Treatment involves staging the tumor and surgical resection of the tumor if possible. Durmus et al. review the evidence regarding the diagnosis and treatment of Sertoli-Leydig cell tumors. They discuss how this tumor generally has a favorable prognosis compared to malignant epithelial tumors. They recommend using fertility-preserving surgeries in younger patients. Figure/Illustration A shows an ultrasound of the left ovary, which has a predominantly solid mass with vascularity and some cystic spaces (red circle). These findings are consistent with an ovarian tumor. Incorrect Answers: Answer A: Adrenal tumors may secrete androgens and cause similar symptoms of virilization, but the presence of a malignant-appearing ovarian mass in this patient is more consistent with ovarian pathology. Furthermore, adrenal tumors typically secrete DHEAS, which can be a helpful test for localizing the source of androgen excess. Treatment is with surgical excision. Answer B: Congenital adrenal hyperplasia (CAH) results from one of several possible enzyme deficiencies in the steroid pathway and may appear later in life as virilization of a woman. While 17-hydroxyprogesterone elevation is a strong indicator of the most common subtype of CAH, this patient’s ultrasound findings suggest ovarian pathology instead, which could also elevate this androgen precursor. Treatment is with hormone replacement therapy. Answer C: Ovarian hyperthecosis is hyperplasia of the theca interna of the ovary, which produces androgens. Testosterone levels would be elevated and virilization may occur, but ultrasound findings would show a bilateral increase in stromal tissue as opposed to a unilateral solid mass. Treatment is with hormonal therapy. Answer E: Polycystic ovary syndrome (PCOS) can also cause elevated testosterone, though levels are typically lower than 100 ng/dL. Frank virilization with clitoromegaly and voice deepening would not be expected with PCOS, nor would an ovarian mass. Treatment is with metformin and lifestyle modifications. Bullet Summary: Sertoli-Leydig cell tumors of the ovary can produce androgens and result in rapid onset of virilization with elevated testosterone levels and an adnexal mass on ultrasound.
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094
A 65-year-old woman presents to her family doctor to reestablish care since losing her insurance. She has not had time for regular check-ups. She exercises 3-4 times a week and consumes red meat sparingly. She drank and smoked cigarettes socially with coworkers but never at home or on vacation. She wakes up with achy wrists and elbows that she suspects is from years of using a computer keyboard. She completed menopause at age 52. Her family history is notable for coronary artery disease on her father's side and colon cancer on her mother's side. She last had a colonoscopy 5 years ago that revealed no abnormal findings. Her temperature is 99°F (37.2°C), blood pressure is 125/83 mmHg, pulse is 82/min, respirations are 12/min, and oxygen saturation is 99% on room air. Her physical exam is grossly unremarkable. What diagnostic test should this patient receive?
{ "A": "Chest radiograph", "B": "Mammography", "C": "Colonoscopy", "D": "Vitamin D level", "E": "" }
B
This vignette describes an otherwise healthy elderly woman seeking to establish primary care. Among the answer choices, mammography is the most appropriate diagnostic test to recommend. Mammography is a screening tool for breast cancer that becomes most effective in postmenopausal patients because of less glandular breast tissue compared to younger patients. According to the United States Preventive Services Task Force, all women aged 50-74 should have mammograms every 1-2 years. It is controversial whether screening mammography should begin as early as age 40. Women with 1st-degree relatives with breast cancer should begin screening 10 years before the age of the family member's diagnosis. Seely and Alhassan review the evidence regarding the screening methods used for breast cancer. They discuss how screening every 1-2 years decreases breast cancer mortality. They recommend using judicious mixed modal methods of screening. Incorrect Answers: Answer A: Routine chest radiography for lung cancer screening in low-risk patients is not recommended by the USPSTF. Chest imaging can be used to screen for lung cancer in patients with a significant smoking history. Answer B: Colonoscopy for colon cancer screening is recommended every 10 years starting at age 50. This patient already had a colonoscopy 5 years ago so she does not need another exam at this time. Answer D: Pelvic ultrasound for ovarian cancer screening is not currently recommended by the USPSTF. Ovarian cancer detection should also be performed in patients who are symptomatic. Answer E: Vitamin D level for osteoporosis screening is not recommended. DEXA scans would be more appropriate for testing bone density. Bullet Summary According to the United States Preventive Services Task Force, all women aged 50-74 should have mammograms every 1-2 years.
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095
A 6-month-old boy is brought to the physician by his mother for abnormal movements. Several times a day, he experiences paroxysmal episodes of crying, bending at the waist, and jerking of the arms and legs. These episodes last 1-2 seconds, but occur in clusters every 20-30 seconds for several minutes. His mother's pregnancy, labor, delivery, and his neonatal course have been unremarkable. He is rolling over less frequently than before, has worse head control, and no longer smiles socially. His temperature is 98.6°F (37°C), blood pressure is 92/46 mmHg, pulse is 115/min, and respirations are 34/min. On examination, no dysmorphic features are noted. A 24-hour electroencephalography (EEG) is performed, during which the patient has one of these spells. A chaotic pattern of slowing and multifocal epileptiform charges is seen. Which of the following is the pathophysiology of this patient's condition?
{ "A": "Decreased gamma aminobutyric acid (GABA) levels", "B": "Vitamin B6 deficiency", "C": "Excess of corticotropin releasing hormone", "D": "Anti-N-methyl-D-aspartate (NMDA) receptor antibodies", "E": "" }
C
This patient with infantile spasms (bending at the waist and jerking out the arms and legs), developmental regression (rolling over less frequently, worsening head control, loss of social smile), and hypsarrhythmia on video-EEG (slowing and multifocal epileptiform discharges) are characteristic of West syndrome. West syndrome is caused in part by an excess of corticotropin-releasing hormone which leads to increased neuronal excitability. The pathogenesis of West syndrome is incompletely understood. Infantile spasms are often idiopathic, but may also be secondary to tuberous sclerosis, hypoxic-ischemic encephalopathy, congenital brain malformations, aminoacidopathies, and organic acidopathies. Brain insults may activate a neuroendocrine stress response, which in turn leads to abnormalities of the hypothalamic-pituitary-adrenal axis. Disturbed immune activation may also play a role. Patients will present with infantile spasms, developmental regression, and slowing and multifocal epileptiform discharges known as hypsarrhythmia. Many affected infants are responsive to ACTH therapy, with cessation of spasms and resolution of hypsarrhythmia in many cases. D’Alonzo et al. review the epidemiology, clinical presentation, pathophysiology, and treatment of West syndrome. They discuss how patients will present with the classic symptoms of spasms and regression. They recommend early diagnosis of this syndrome so that patients can be treated appropriately. Incorrect Answers: Answer B: Abnormalities in T-type calcium channels may contribute to absence seizures. Absence seizures are generalized seizures that involve brief, sudden lapses of consciousness and are more common in children. This child’s developmental regression, infantile spasms, and EEG findings are more consistent with West syndrome. Answer C: Anti-N-methyl-D-aspartate (NMDA) receptor antibodies may cause anti-NMDA encephalitis. Anti-NMDA encephalitis presents with seizures, psychiatric manifestations, and decreased level of consciousness. Hypsarrhythmia is characteristic of West syndrome and is not consistent with anti-NMDA encephalitis. Answer D: Decreased gamma-aminobutyric acid (GABA) levels were once theorized to be the basis of epilepsy syndromes. This theory is less accepted today. Moreover, West syndrome is likely not mediated by disturbances in GABA. Nonetheless, vigabatrin, which in part works by inhibiting GABA metabolism, is an effective medication for patients with West syndrome. Answer E: Vitamin B6 deficiency has not been documented in West syndrome. Some studies have suggested vitamin B6 may be of some benefit in the treatment of infantile spasms; however, there is no evidence that response rates to B6 are different from the spontaneous remission rates predicted from natural history data. Therefore, the pathophysiology of West syndrome is more likely CRH-excess mediated rather than due to a deficiency of vitamin B6. Bullet Summary: West syndrome, which may be caused by CRH excess, presents with hypsarrhythmia, developmental regression, and infantile spasms.
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096
A 67-year-old man is seen on the surgical floor after a transplant procedure. The previous day, the patient had a renal transplant from a matched donor. He is currently recovering and doing well. The patient has a medical history of IV drug use, diabetes mellitus, oral cold sores, hypertension, renal failure, and dyslipidemia. The patient's current medications include lisinopril, atorvastain, insulin, and aspirin. Prior to the procedure, he was also on dialysis. The patient is started on cyclosporine. The patient successfully recovers over the next few days. His temperature is 98.5°F (36.9°C), blood pressure is 121/76 mmHg, pulse is 70/min, and respirations are 13/min. Which of the following medications should be started in this patient?
{ "A": "Valacyclovir", "B": "Low dose acyclovir", "C": "TMP-SMX", "D": "Azithromycin", "E": "" }
C
This patient is presenting status post renal transplant and is currently on immunosuppressive therapy. This patient should be started on TMP-SMX as prophylaxis against Pneumocystis jiroveci pneumonia (PJP). After a transplant procedure, patients are started on immunosuppressive therapy in order to avoid rejection of the transplanted organ. The most important interventions for a patient after a transplant procedure are to receive all of their vaccinations and to receive TMP-SMX as prophylaxis against PJP. Other appropriate but less dire measures include giving ganciclovir as prophylaxis against cytomegalovirus (CMV). Patients should be closely monitored to avoid transplant rejection as well as infection. Weyant et al. review the evidence regarding the treatment of patients with PJP. They discuss how TMP-SMX has been the classic prophylaxis and treatment for this disease. They recommend considering echinocandins for patients who experience significant side effects. Incorrect Answers: Answer A: Azithromycin is appropriate prophylaxis for patients with HIV with CD4 counts < 50. Patients on immunosuppressive drugs should receive some of the same prophylactic medications but they do not require azithromycin upon initiation of immunosuppressive medications. Answer B: Low-dose acyclovir would be an inappropriate intervention in this patient after a transplant procedure. Ganciclovir and high-dose acyclovir are the appropriate options for prophylaxis of CMV. Answer C: Penicillin can be used as prophylaxis for patients with rheumatic heart disease or asplenia; however, it would not be the most appropriate initial measure in a transplant patient. Answer E: Valacyclovir is the treatment for herpes simplex virus (HSV). It is not routinely indicated as prophylaxis in transplant patients. HSV will present with vesicular rashes developing on the affected areas. Bullet Summary: Patients on immunosuppressive therapy after a transplant should be vaccinated (pneumovax, hepatitis A and B, influenza, and tetanus-diphtheria) and started on TMP-SMX.
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097
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?
{ "A": "Apical lung tumor", "B": "Cerebral infarction", "C": "Scalenus anticus syndrome", "D": "Subclavian steal syndrome", "E": "" }
A
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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098
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?
{ "A": "Decreased metabolism of bilirubin", "B": "Increased enterohepatic circulation of bilirubin", "C": "Obstruction of the extrahepatic biliary tree", "D": "Obstruction of the intrahepatic biliary tree", "E": "" }
C
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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099
A 7-year-old boy is brought to the emergency department by his parents for worsening symptoms of previously diagnosed acute sinusitis. Initially, the pediatrician prescribed decongestants and sent the patient home. Since then, the patient has developed a nasal discharge with worsening pain. The patient has a medical history of asthma which is well-controlled with albuterol. His temperature is 99.5°F (37.5°C), blood pressure is 90/48 mmHg, pulse is 124/min, respirations are 17/min, and oxygen saturation is 98% on room air. The cardiopulmonary exam is within normal limits. Inspection of the patient's nose reveals a unilateral purulent discharge mixed with blood. The rest of the patient's exam is within normal limits. Which of the following is the most likely diagnosis?
{ "A": "Foreign body obstruction", "B": "Nasopharyngeal carcinoma", "C": "Bleeding and infected vessel of Kiesselbach plexus", "D": "Septal perforation", "E": "" }
A
This patient presents with unilateral bloody/purulent discharge from the nose suggesting a diagnosis of a nasal foreign body. The most common cause of unilateral nasal symptoms in a pediatric patient is a nasal foreign body. Nasal foreign bodies tend to be unilateral (though bilateral symptoms are plausible) and present with a purulent and bloody discharge. The treatment of choice is to remove the object which may be done in the emergency department or operating room depending on how deep the object is. Visualization of the object should be attempted when possible in order to allow for safe retrieval. Krulewitz and Fix review the evidence regarding the diagnosis and treatment of patients with epistaxis. They discuss how foreign body insertion is a major risk factor for the development of epistaxis. They recommend assessment of airway and breathing in the setting of severe epistaxis. Incorrect Answers: Answer A: A bleeding and infected vessel of the Kiesselbach plexus could suggest a complicated case of epistaxis. The persistent, unilateral symptoms in this patient point toward a different diagnosis. A high index of suspicion for foreign bodies should be suspected in children. Answer C: Nasopharyngeal carcinoma tends to occur in young male patients and can be associated with infectious mononucleosis. Epistaxis can be a symptom and the mass can be visualized. This disease tends to present in older patients and is far less common than foreign bodies. Treatment is with surgical resection. Answer D: Septal perforation can occur secondary to repeat cocaine use but would not present with unilateral nasal discharge in a pediatric patient. It would be highly unlikely in a pediatric patient with no history of drug use. Treatment is with cessation of the offending agent. Answer E: Sinusitis with bacterial superinfection would typically present with systemic symptoms as well as bilateral symptoms. Though this diagnosis is possible, it is less likely than a foreign body. Treatment is with systemic antibiotic therapy. Bullet Summary: A nasal foreign body presents with unilateral bloody/purulent nasal discharge.
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100
A 33-year-old man presents to the emergency department after slamming his finger in the car door 5 hours ago. He initially experienced pain which is currently well controlled with acetaminophen. The patient is otherwise healthy and does not take any medications. His temperature is 98.5°F (36.9°C), blood pressure is 123/79 mmHg, pulse is 90/min, respirations are 11/min, and oxygen saturation is 97% on room air. Physical exam is notable for the finding in Figure A. A radiograph of the affected digits shows a smooth cortex without disruption of the bone. Which of the following is the most appropriate management for this patient?
{ "A": "Fingernail removal", "B": "Trephination", "C": "Excision and histological examination", "D": "Operative repair", "E": "" }
B
This patient is presenting after trauma to his finger with a subungual hematoma. Given that he has no other associated injuries, trephination is the only management indicated. A subungual hematoma occurs after trauma to the finger leading to a collection of blood under the fingernail. Depending on the mechanism, this can be associated with a distal phalanx fracture. Evaluation for a fracture should include a thorough history, exam, and radiography. If there are no other signs of injury including nailbed dislocation or an open fracture, then management requires only trephination (putting a hole in the nail to allow blood to drain) as this alleviates pressure on the nailbed matrix thus preventing possible irreversible damage. Rsoer et al. compare nail bed repair to nail trephination for subungual hematomas in children. The authors find that there was no difference in complications or outcomes between nail bed repair and trephination; however, the cost in the nail trephination group was lower. The authors recommend nail trephination for children with a subungual hematoma and an intact nail and nail margin. Figure/Illustration A shows the physical exam finding of a subungual hematoma with a collection of blood underneath the fingernail bed (green circle). Incorrect Answers: Answer A: Excision and histological examination could be appropriate management of melanoma, which may present under the fingernails with a hyperpigmented, irregular skin lesion, or a “barcode” appearance if it is at the base of the nail. A spontaneous subungual hematoma could be suggestive of melanoma; however, in the setting of trauma, it is a much less likely diagnosis. Answer B: Fingernail removal should only be performed if there is also nail avulsion or nail fold disruption to allow for inspection of the nail bed and repair of any laceration. Failure to repair a nailbed laceration could lead to permanent deformity of the fingernail when it regrows. Answer C: Observation is inappropriate as the accumulation of blood under the nail in a subungual hematoma could lead to ischemia and permanent injury to the nailbed. When a subungual hematoma is present, trephination is indicated. Answer D: Operative repair may be necessary for fractures; however, this patient has no fracture and only has a subungual hematoma which can be drained easily. Bullet Summary: A subungual hematoma is common after trauma, presents with a collection of blood under the fingernail, and should be treated with trephination.
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