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001
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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.
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002
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An 18-year-old man presents to his primary care provider with his parents for a sports physical. He was last seen in the clinic several months ago when he was diagnosed with attention deficit hyperactivity disorder (ADHD). He was started on methylphenidate at that time and the patient now reports improvement in his ability to concentrate in school and at home. He hopes to play baseball in college and has begun lifting weights daily in preparation for baseball season. The patient has a healthy diet to fuel his exercise regimen. His parents have no concerns and are pleased with the recent improvement in his grades. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. On physical exam, the patient has tall stature with average muscle mass for his age. He has no dysmorphic features. His chest has a normal appearance other than mild gynecomastia. The patient has sparse facial hair and a moderate amount of coarse pubic hair that extends across the pubis and spares the medial thighs. His testes are small and firm. Laboratory testing is performed and reveals the following:
Follicle-stimulating hormone (FSH): 42 mIU/mL
Luteinizing hormone (LH): 38 mIU/mL
Which of the following is the most likely etiology of this patient’s presentation?
|
{
"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.
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003
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A 62-year-old man presents to the emergency department with chest pain and shortness of breath that he experienced while barbecuing. The pain is over his chest and in his left arm. The patient has a medical history of obesity, type 2 diabetes mellitus, and hypertension. His current medications include insulin, metformin, lisinopril, and hydrochlorothiazide. An ECG is performed and is seen in Figure A. His temperature is 99.5°F (37.5°C), pulse is 112/min, blood pressure is 100/70 mmHg, respirations are 18/min, and oxygen saturation is 95% on room air. The patient is appropriately managed and is admitted to the step-down unit. 2 days later, he complains of chest pain. A repeat ECG is performed and is seen in Figure B. Which of the following is the most appropriate next step in management for this patient's underlying condition?
|
{
"A": "Prednisone",
"B": "Aspirin",
"C": "Stenting",
"D": "Ibuprofen",
"E": ""
}
|
B
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This patient is presenting with chest pain several days after sustaining a myocardial infarction and has an ECG suggestive of pericarditis. This presentation is consistent with Dressler syndrome and the most appropriate initial step in management is aspirin.
Dressler syndrome is pericarditis that can occur after a myocardial infarction (MI). It most commonly occurs weeks to months after the MI, but can also occur within 1 week from an MI in rare cases. It is believed to result from an autoimmune reaction that occurs after damage to the heart (surgery and MI). It presents with chest pain, fever, pleuritic pain, and a pericardial friction rub. ECG demonstrates diffuse ST elevation with PR depression consistent with pericarditis. The most appropriate initial step in the management of this condition is symptomatic management with aspirin.
Leib et al. review the evidence regarding the diagnosis and treatment of Dressler syndrome. They discuss how this condition is secondary pericarditis with or without pericardial effusion resulting from injury to the heart or pericardium. They recommend considering this syndrome in all patients with chest pain after a myocardial infarction.
Figure/Illustration A demonstrates an ECG with ST elevation in leads II, III, and aVF (blue circles) suggesting a diagnosis of right-sided MI.
Figure/Illustration B demonstrates an ECG with diffuse ST elevation and PR depression (blue circles) suggesting a diagnosis of pericarditis.
Incorrect Answers:
Answers 1 & 5: Angiography and stenting would be appropriate management if this patient were experiencing a repeat MI. The diffuse ST elevation and PR depression seen several days after his myocardial infarction are more indicative of pericarditis rather than a repeat MI. In addition, the chest pain the patient is experiencing can also occur in pericarditis and obfuscates the diagnosis of repeat MI.
Answers 3 & 4: Ibuprofen, other NSAIDs, and corticosteroids such as prednisone are not first-line therapy in post-MI pericarditis as they can hinder the healing and remodeling process of the myocardium. Current guidelines dictate that they may be used in cases that are refractory to initial therapy.
Bullet Summary:
Dressler syndrome (post-MI pericarditis) presents with chest pain, pericardial friction rub, fever, and diffuse ST elevation with PR depression on ECG, and should be treated initially with aspirin.
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https://bit.ly/3BV4U9Y
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004
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A 6-year-old boy presents to the emergency department after falling from his scooter. He has dull, aching pain along his left side where he hit the ground. He fell sideways off the scooter as he rounded a curve in the road. He has never had any serious injuries but that he always seems to bruise easily, especially after he started playing soccer this fall. His parents deny that he has an abnormal number of nosebleeds or bleeding from the gums. They have never seen blood in his stool or urine. His mother notes that her brother has had similar problems. His temperature is 98.6°F (37°C), blood pressure is 112/74 mmHg, pulse is 82/min, and respirations are 11/min. On physical exam, the patient has extensive bruising of the lateral left thigh and tenderness to palpation. Laboratory tests are performed and reveal the following:
Hemoglobin: 14 g/dL
Hematocrit: 41%
Mean corpuscular volume: 89 µm3
Reticulocyte count: 0.8%
Leukocyte count: 4,700/mm3
Prothrombin time (PT): 13 seconds
Partial thromboplastin time (PTT): 56 seconds
Bleeding time (BT): 4 minutes
Which of the following is the most likely underlying pathophysiology?
|
{
"A": "Anti-platelet antibodies",
"B": "Von Willebrand factor deficiency",
"C": "GP1b deficiency",
"D": "Factor 8 deficiency",
"E": ""
}
|
D
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This young patient presents with extensive bruising following an injury, a family history of blood disorder, and a prolonged partial thromboplastin time (PTT) with a normal prothrombin time (PT) and bleeding time. These findings suggest a diagnosis of hemophilia A caused by factor 8 deficiency.
Hemophilia is a deficiency of factor 8 (hemophilia A) or factor 9 (hemophilia B). Common symptoms of hemophilia include hemarthrosis, intramuscular hematoma, and gastrointestinal or genitourinary bleeding. Laboratory testing is helpful in confirming the diagnosis, as hemophilia is characterized by an isolated prolongation of PTT without a change in PT. The factors involved in hemophilia participate only in the indirect cascade of coagulation factor activation. Similarly, since bleeding time depends only upon platelet function and not coagulation factors, hemophilia presents with a normal bleeding time. Treatment of hemophilia includes desmopressin in mild cases and factor replacement in moderate to severe disease.
Berntorp et al. present a review of the evidence regarding the diagnosis and treatment of hemophilia. They discuss how these patients now have normal life expectancies with the advent of factor replacement therapy. They recommend being vigilant for the development of anti-factor antibodies.
Incorrect Answers:
Answer A: Anti-platelet antibodies are responsible for idiopathic thrombocytopenic purpura (ITP). This disease is caused by autoimmune antibody formation that damages platelets and causes them to be removed by splenic macrophages in the reticuloendothelial system. It usually occurs in children after an acute viral infection and it presents with bleeding from mucosal surfaces with laboratory findings showing an increased bleeding time, decreased platelets, and a normal PT/PTT.
Answer C: Factor 9 deficiency (hemophilia B) presents nearly identically to hemophilia A with a normal PT, prolonged PTT, and normal bleeding time with a history of bleeding in male relatives. However, because hemophilia B is epidemiologically less common than hemophilia A, hemophilia A is a more likely diagnosis.
Answer D: GP1b deficiency causes Bernard-Soulier syndrome, which is a bleeding disorder characterized by unusually large platelets and a low platelet count. Like von Willebrand disease (vWD), patients often present with cutaneous and mucosal bleeding, abnormal bleeding from minor trauma, and a prolonged bleeding time, which this patient does not have. It can be distinguished from vWD by a ristocetin assay. Treatment can include tranexamic acid.
Answer E: Von Willebrand factor deficiency results in vWD, which is a bleeding disorder characterized by cutaneous and mucosal bleeding, menorrhagia, and gastrointestinal bleeding. Unlike this patient, patients with vWD also present with a prolonged bleeding time. Patients with vWD will also have an increased PT because this factor is responsible for stabilizing coagulation factor 8.
Bullet Summary:
Hemophilia A is caused by factor 8 deficiency and presents with hemarthrosis, frequent bruising, intramuscular hematoma, and gastrointestinal bleeding with a prolonged PTT with a normal PT and bleeding time.
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https://bit.ly/3BoKeXI
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005
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A 69-year-old man presents to his primary care physician for a general checkup. The patient works as a farmer and gardens in his spare time. He has a medical history of poorly managed type 2 diabetes mellitus and irritable bowel syndrome. His current medications include lisinopril, metformin, insulin, fiber supplements, and a multivitamin. On physical exam, there is a calm, obese gentleman. Inspection of the patient's scalp reveals the finding shown in Figure A. The patient is sent home and instructed to keep a blood glucose journal. Three months later, the patient returns with his glucose journal. In addition, he complains of a cut on his scalp that is not seeming to heal. The patient's glucose journal reveals an average blood glucose of 175 mg/dL. His temperature is 98.8°F (37.1°C), blood pressure is 134/86 mmHg, pulse is 80/min, and respirations are 13/min. On inspection of the patient's scalp, the finding in Figure B is noted. Which of the following is the most likely diagnosis?
|
{
"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.
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https://bit.ly/3CB9Cdn
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006
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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.
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https://bit.ly/3CrrhE1
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007
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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
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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.
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https://bit.ly/3CxfeFC
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008
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A 4-week-old boy is brought to the pediatrician by his parents for an initial evaluation. His parents are concerned that he is not feeding well and has lost weight over the last 2 weeks. He was born at home via spontaneous vaginal delivery at 37 weeks of gestation to a G1P1 mother. His mother did not receive prenatal care. His blood pressure is 70/44 mmHg, pulse is 151/min, and respirations are 41/min. His weight is 3 kg (4th percentile) compared with 3.5 kg at birth (45th percentile). On physical exam, he is jaundiced with an enlarged liver and spleen. He does not appear to respond to visual stimuli, and further examination reveals bilateral clouding of the lens. Which of the following interventions could have avoided this patient's symptoms?
|
{
"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.
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https://bit.ly/3D10TBk
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009
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A 4-week-old boy presents with his parents to the pediatrician for a well-child visit. His mother reports he was eating well until 1 week ago when he began vomiting after breastfeeding. His mother has tried increasing the frequency of feeds and decreasing the amount of each feed, but the vomiting seems to be getting worse. The patient now vomits after every feed. The vomitus looks like breast milk. The patient’s mother is exclusively breastfeeding and would prefer not to switch to formula but worries that the patient is not getting the nutrition he needs. Two weeks ago, the patient was in the 75th percentile for weight and 70th for height. He is now in the 60th percentile for weight and 68th percentile for height. His temperature is 98.2°F (36.8°C), blood pressure is 58/37 mmHg, pulse is 144/min, and respirations are 34/min. On physical exam, the patient has dry mucous membranes. His abdomen is soft and non-distended. Which of the following is the most appropriate next step in management?
|
{
"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.
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010
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A 27-year-old woman presents for her first prenatal visit at an estimated gestational age of 16 weeks and 4 days by last menstrual period. She is presenting late to prenatal care due to significant anxiety about her pregnancy, as she and her husband had struggled with infertility for several years prior to conceiving. She has had nausea and vomiting for about 2 months, tender and swollen breasts, and cravings for foods she typically does not eat. She took a pregnancy test about 10 weeks ago and was too nervous to read the result immediately. After 24 hours, she saw that it was positive. The patient has a past medical history of bulimia nervosa but has not been symptomatic in 2 years. She also had an appendectomy at age 15 for appendicitis. She is a Ph.D. student and her husband is a physician. Her temperature is 98.5°F (36.9°C), pulse is 75/min, blood pressure is 122/76 mmHg, and respirations are 13/min. The patient’s affect is normal and she is pleasant and excited about her pregnancy. A physical exam is notable for abdominal distension but is otherwise unremarkable. Ultrasound is shown in Figure A, and urine human chorionic gonadotropin is negative in the office. Which of the following is the most likely diagnosis?
|
{
"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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https://bit.ly/3DDvy7X
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011
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A 2-year-old boy is brought to the emergency room by his mother, who states that her son has been coughing uncontrollably for 3 hours. The child has a medical history of obesity and atopic dermatitis and uses topical steroid cream. His birth history is unremarkable. His family history is notable for asthma in his older sister and irritable bowel syndrome in his father. His temperature is 98.5°F (36.9°C), pulse is 120/min, blood pressure is 90/52 mmHg, and respirations are 32/min. On exam, he appears uncomfortable, is coughing continuously, and is drooling. Cardiopulmonary and abdominal exams are unremarkable. A chest radiograph is performed and the results are shown in Figures A and B. Which of the following is the most appropriate next step in management?
|
{
"A": "Observation",
"B": "Balloon catheter removal",
"C": "Surgical extraction",
"D": "Endoscopic removal",
"E": ""
}
|
D
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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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https://bit.ly/3DIAhW0
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012
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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?
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{
"A": "Ankylosing spondylitis",
"B": "Osteomyelitis",
"C": "Degenerative spine disease",
"D": "Metastatic tumor",
"E": ""
}
|
B
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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.
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https://bit.ly/3DkrY2C
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013
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A 27-year-old man presents to his primary care physician for a checkup. He has no health concerns and has not seen a physician in years. The patient has a medical history of depression treated with fluoxetine and lithium. His temperature is 99.5°F (37.5°C), blood pressure is 122/78 mmHg, pulse is 90/min, respirations are 13/min, and oxygen saturation is 98% on room air. Physical exam is notable for the finding in Figure A. Which of the following represents the most likely risk factor for this patient's presenting condition?
|
{
"A": "Alcohol consumption",
"B": "Antibiotic use",
"C": "Intravenous drug use",
"D": "Sexual intercourse",
"E": ""
}
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A
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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.
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https://bit.ly/3FB3X8u
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014
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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?
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{
"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
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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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https://bit.ly/3FgV041
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015
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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": ""
}
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D
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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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https://bit.ly/3FwRzGJ
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016
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A 9-year-old girl presents to the emergency department with a fever and a change in her behavior. She presented with similar symptoms 6 weeks ago and was treated for an Escherchia coli infection. She also was treated for a urinary tract infection 10 weeks ago. Her mother says that last night her daughter felt ill, and her condition has been worsening. Her daughter experienced a severe headache and had a stiff neck. This morning she was minimally responsive, vomited several times, and produced a small amount of dark cloudy urine. The patient was born at 39 weeks and met all her developmental milestones. She is currently up to date on her vaccinations and did not have infections during early childhood. Her parents are divorced and her father has noted she does not seem to get sick when he takes care of her. Her temperature is 99.5°F (37.5°C), blood pressure is 60/35 mmHg, pulse is 190/min, respirations are 33/min, and oxygen saturation is 98% on room air. The patient is started on intravenous fluids, vasopressors, and broad-spectrum antibiotics. Which of the following is the most appropriate underlying explanation for this patient's presentation?
|
{
"A": "Gastroenteritis",
"B": "Intentional contamination",
"C": "Meningitis",
"D": "Urinary tract infection",
"E": ""
}
|
B
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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.
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https://bit.ly/3GZtkBx
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017
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A 55-year-old man presents to urgent care for weakness and weight loss. For the past several months, he has felt progressively weaker and has lost 25 pounds with intermittent abdominal pain. The patient has not seen a physician in 30 years and recalls being current on most of his vaccinations. A few years ago, he went to the emergency department due to abdominal pain and was found to have increased liver enzymes due to excessive alcohol use and incidental gallstones. The patient has a 50 pack-year smoking history. His temperature is 99.5°F (37.5°C), blood pressure is 161/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals an emaciated man. The patient has a negative Murphy sign and his abdomen is non-tender. Cardiopulmonary exam is within normal limits. Which of the following is the most appropriate next step in management?
|
{
"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.
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https://bit.ly/3GigfDn
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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.
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https://bit.ly/3HvRZyr
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019
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A 34-year-old nulliparous woman presents to the clinic with 2 weeks of copper-colored discharge from her right nipple. Her breast has not been tender, and she has not noticed any changes to the skin. She is otherwise healthy and does not take any medications. Her temperature is 36.5°C (97.7°F), blood pressure is 110/82 mmHg, pulse is 68/min, respirations are 12/min, and oxygen saturation is 99% on room air. Exam of the right breast reveals no mass on palpation. Bloody discharge is expressed from the right nipple. There is no axillary lymphadenopathy. Which of the following is the most likely diagnosis?
|
{
"A": "Ductal carcinoma in situ",
"B": "Mammary duct ectasia",
"C": "Invasive ductal carcinoma",
"D": "Intraductal papilloma",
"E": ""
}
|
D
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This young patient who presents with non-painful unilateral bloody nipple discharge and no palpable mass on breast exam most likely has an intraductal papilloma.
Intraductal papilloma is a benign epithelial tumor that develops in the lactiferous ducts of the breast and is the most common cause of bloody nipple discharge in premenopausal women. Patients typically present with painless unilateral bloody nipple discharge, and often there is no mass palpable on breast exam. Ultrasound is the initial method for evaluation and may reveal an intraductal mass, but if results are inconclusive, further imaging with mammography is indicated. If the results of both still remain inconclusive, galactography (radiography after injecting radiopaque contrast into the duct system) is the most accurate imaging method for diagnosing intraductal papilloma. A core biopsy of the intraductal mass should be obtained, and if results show papilloma with atypia, treatment would be surgical excision of the involved duct to rule out a concomitant malignant neoplasm.
Khan et al. review the treatment of intraductal papilloma and note that in lesions with core biopsy showing papilloma with atypia, surgical excision revealed the diagnosis of a concomitant invasive or ductal in situ cancer is common. The authors also find that in patients whose core biopsies showed papilloma without atypia, the likelihood of cancer was much lower. The authors recommend that since the diagnosis of intraductal papilloma carries an increased risk of breast cancer compared to the general population, these patients should be monitored accordingly.
Incorrect Answers:
Answer A: Ductal carcinoma in situ (DCIS) is a neoplastic lesion confined to the breast duct that if untreated develops into invasive ductal carcinoma. Typically, patients are asymptomatic, and lesions are detected via screening mammography. Though it can sometimes present with nipple discharge, this is less common. Unilateral painless bloody nipple discharge is more likely to be due to intraductal papilloma, though no mass may be palpable for either diagnosis.
Answer C: Invasive ductal carcinoma is the most common type of breast cancer, which typically presents as a firm, immobile, painless lump in the breast, sometimes associated with nipple discharge, overlying skin changes, inverted nipple, and/or axillary lymphadenopathy. As with DCIS, nipple discharge may be present but is usually not bloody as in cases of intraductal papilloma.
Answer D: Mammary duct ectasia is a benign condition in which a milk duct in the breast widens and its walls thicken, leading to blockage and build-up of fluid in the duct. This is usually asymptomatic but can present with off-white or greenish nipple discharge. It may also result in infection of the affected duct, leading to periductal mastitis and a tender, erythematous breast. It tends to occur in perimenopausal women and often resolves without treatment, though if symptoms persist it can be treated with antibiotics and duct excision.
Answer E: Paget disease of the breast often presents as a scaly, raw, vesicular, or ulcerated lesion that begins on the nipple and then spreads to the areola, sometimes associated with bloody nipple discharge. Pain, burning, and pruritis are also common presenting symptoms. There is often an underlying breast carcinoma; thus, any patient with Paget disease should be evaluated with a mammogram. Although this patient has bloody nipple discharge, she does not present with a painful, scaly, raw, or vesiculated lesion that would be concerning for Paget disease of the breast.
Bullet Summary:
Intraductal papilloma often manifests in patients as painless unilateral bloody nipple discharge, and typically no mass is palpable on exam.
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020
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A 45-year-old man presents to his primary care physician with a rash. He first noticed the rash 2 weeks prior and he endorses intermittent itchiness from the rash. His medical history is significant for chronic hepatitis C infection, hypertension, type 2 diabetes mellitus, and heart failure. The patient works as a butcher at a local meat processing shop and is sexually active with several partners. He does not use barrier protection. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 155/95 mmHg, and respirations are 12/min. On physical exam, his skin has the finding shown in Figure A. Fine, lace-like white lines are also noted on the patient’s buccal mucosa. Which of the following is the most likely diagnosis?
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{
"A": "Atopic dermatitis",
"B": "Lichen planus",
"C": "Scabies",
"D": "Psoriasis",
"E": ""
}
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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
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A 40-year-old south asian man presents to a primary care provider with a chronic cough that is worse at night. He has had the cough for several years but it has been getting worse over the last few months. He recently moved to the United States to work in construction. He has lost 10 pounds in the last 3 months along with darker stools which he believes is caused by a Western diet. He denies any difficulty swallowing or feeling of food getting stuck in his throat. He drinks alcohol 1-2 times per week and has never smoked. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 114/72 mmHg, pulse is 82/min, and respirations are 12/min. On physical exam, his lungs are clear to auscultation bilaterally without wheezing. His abdomen is soft and non-distended. He has no tenderness to palpation and bowel sounds are present. Which of the following is the most appropriate next step in management?
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{
"A": "Barium swallow",
"B": "Upper endoscopy",
"C": "Helicobacter pylori stool antigen test",
"D": "Trial of lansoprazole",
"E": ""
}
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B
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This patient presents with a chronic cough that is worse at night, which is consistent with gastroesophageal reflux disease (GERD). In light of this patient’s new weight loss and melena, the most appropriate next step in management would be upper endoscopy to rule out cancer.
GERD may present as a chronic or nighttime cough, substernal or epigastric pain, regurgitation, nausea, or bitter taste in the mouth. Symptoms are typically worse after eating or lying down. Patients with typical GERD should undergo upper endoscopy if they have concerning symptoms, including dysphagia, odynophagia, vomiting, weight loss, gastrointestinal bleeding, or melena. They should also undergo upper endoscopy if they are men over the age of 50 with chronic symptoms (>5 years) and cancer risk factors, such as a history of tobacco use or a family history of cancer.
Young et al. review the evidence regarding the treatment of GERD. They discuss how patients with concerning symptoms or those who are failing symptomatic treatment should undergo upper endoscopy. They recommend treatment with a proton pump inhibitor in patients without alarm symptoms.
Incorrect Answers:
Answer A: A barium swallow would be indicated in a patient who presented with symptoms of esophageal dysphagia. Possible etiologies would include Zenker diverticulum or achalasia. This patient denied any difficulty swallowing or feeling of food being stuck in the throat.
Answer B: Colonoscopy could be considered in this patient if his upper endoscopy is unrevealing, as weight loss and melena could be explained by colorectal cancer. In light of this patient’s GERD, upper endoscopy should be performed first.
Answer C: Helicobacter pylori testing can be useful in the workup of gastric and duodenal ulcers but is not usually performed in the evaluation of GERD. Weight loss and melena raise concerns for carcinoma. Treatment of patients with H. pylori includes a proton pump inhibitor, clarithromycin, and metronidazole.
Answer D: A trial of lansoprazole would not be appropriate for this patient presenting with several worrisome symptoms including melena and weight loss. If the patient had presented with typical GERD without these symptoms, a trial of a PPI would be indicated. Cancer should be ruled out first prior to symptomatic treatment.
Bullet Summary:
Patients with gastroesophageal reflux disease who present with alarm symptoms of weight loss, dysphagia, odynophagia, vomiting, gastrointestinal bleeding, or melena should be evaluated with upper endoscopy.
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022
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A 6-week-old boy is referred to a pediatric hematologist-oncologist for further evaluation following an abnormal newborn hemoglobinopathy screening. The father’s brother died of sickle cell anemia at an early age. Parental studies reveal that both mother and father are carriers of the sickle cell gene. The patient's temperature is 98.6°F (37.0°C), blood pressure is 80/45 mmHg, pulse is 130/min, and respirations are 25/min. The physician decides to repeat testing for the patient and his 3-year-old sister. The results are shown in Figure A. The physician begins counseling the family. Which of the following is the most likely complication that the patient may have in the future?
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{
"A": "Stroke",
"B": "Hematuria",
"C": "Splenectomy",
"D": "Infection",
"E": ""
}
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B
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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
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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?
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{
"A": "Topical erythromycin",
"B": "Topical bacitracin",
"C": "Artificial tears",
"D": "Oral erythromycin",
"E": ""
}
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A
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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
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A 30-year-old man is evaluated in the post-anesthesia care unit for neck pain. He underwent a laparoscopic appendectomy and was treated for post-operative nausea and vomiting with metoclopramide. Approximately 20 minutes after receiving the medication, he developed pain and stiffness in his neck and eventually was unable to move his neck. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 130/80 mmHg, respirations are 16/min, and oxygen saturation is 98% on room air. Examination reveals an uncomfortable appearing man. His neck is rotated to the right and is unable to return to midline. Examination of the patient is shown in Figure A. Which of the following is the most appropriate next step in management?
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{
"A": "Botulinum toxin injection",
"B": "Ondansetron",
"C": "Diphenhydramine",
"D": "Observation",
"E": ""
}
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C
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This patient is suffering from a metoclopramide-induced acute dystonic reaction. Treatment of this complication includes IV diphenhydramine or benztropine.
Dystonic reactions are reversible extrapyramidal symptoms that may include involuntary spasms or sustained contractions of the face, neck, torso, pelvis, extremities, and larynx. Medications that block dopamine D2 receptors carry the greatest risk for development of a dystonic reaction, most notably including neuroleptics, antidepressants, and antiemetics. A family history of dystonia increases the risk of developing a reaction (there is likely an underlying genetic predisposition). Diagnosis is typically based on history and clinical presentation. Intravenous anticholinergic agents are the first-line treatment, and symptoms typically resolve rapidly (within 1 hour) after initiation of these agents.
Dressler et al. discuss the diagnosis and management of medication-induced acute movement disorders, notably dystonic reactions. These reactions are most commonly caused by dopamine-receptor blocking agents, including antipsychotics and antiemetics. Cranial, pharyngeal, and cervical muscles are typically affected within the first 4 days of initiating treatment.
Figure A shows a patient with dystonia and torticollis; note the posturing of the extremities as well as how the head is tilted to the patient's right side while the chin is directed up and towards the left.
Incorrect Answers:
Answer A: Intramuscular botulinum toxin injection may be appropriate for chronic torticollis or other forms of chronic dystonia. This patient has acute dystonia that is generally able to be easily reversed with intravenous diphenhydramine.
Answer C: Intravenous haloperidol, a D2 receptor antagonist would not be appropriate. This patient's condition is due to the dopamine antagonist action of metoclopramide and would therefore be worsened with haloperidol.
Answer D: Observation would not be appropriate. This patient is experiencing an acute dystonic reaction that is uncomfortable and potentially dangerous. Acute dystonia is not likely to self resolve in a reasonable amount of time.
Answer E: Intravenous ondansetron may be a more appropriate option to treat this patient's nausea given his reaction to metoclopramide. However, it will not reverse the acute dystonic reaction and is not the most appropriate first step.
Bullet Summary:
Acute dystonic reaction may occur with administration of medications that block dopamine receptors (such as metoclopramide), and can be treated with intravenous diphenhydramine.
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025
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A 69-year-old patient presents to the emergency department with shortness of breath and substernal pain. The patient has a medical history of obesity, anxiety, and constipation. He is currently not taking any medications and has not filled his prescriptions for over 1 year. An ECG is obtained and is seen in Figure A. The patient is managed appropriately and is discharged on atorvastatin, metoprolol, aspirin, clopidogrel, and sodium docusate. The patient visits his primary care physician 4 weeks later and claims to be doing better. He has been taking his medications and resting. At this visit, his temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 80/min, respirations are 11/min, and oxygen saturation is 96% on room air. The patient states he feels better since the incident. Which of the following is the most appropriate next step in management?
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{
"A": "Add nifedipine",
"B": "Add lisinopril",
"C": "Add hydrochlorothiazide",
"D": "Continue current medications and schedule follow up",
"E": ""
}
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B
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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.
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026
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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?
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{
"A": "Acute inflammatory demyelinating polyneuropathy",
"B": "L5/S1 disc herniation",
"C": "Spinal stenosis",
"D": "Cauda equina syndrome",
"E": ""
}
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D
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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.
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027
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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
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A 14-year-old boy presents to his pediatrician with a 2-month history of intermittent arm and leg spasms. He says that the spasms usually occur after he wakes up as he is getting ready for school, and consist of disorganized jerking motions that stop after a few minutes. He has also noticed that they occur when he stays up late to cram last minute for exams. He has not noticed any other symptoms. He has no medical history and takes no medications. He recently started experimenting with alcohol and marijuana that one of his friends has been stealing from their parents. His temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. Physical exam reveals a healthy child. Which of the following is the most likely cause of this patient's symptoms?
|
{
"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.
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https://bit.ly/3LZVzD7
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029
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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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https://bit.ly/3LbQCXB
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030
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A 54-year-old man is brought to the emergency department following a skiing accident. The patient lost control of his skis and collided with a tree. He lost consciousness for about 30 seconds. When he woke up, he was mildly confused but able to ski down the rest of the mountain. The patient initially denied medical treatment but his wife convinced him to be taken by ambulance to the emergency department for evaluation. The paramedics report that at the scene the patient had a Glasgow Coma Score (GCS) of 15. During the ride, he became gradually more somnolent. His medical history is significant for hypertension, hyperlipidemia, coronary artery disease, and a prior transient ischemic attack. His medications include atorvastatin and lisinopril. In the emergency department, his temperature is 97.8°F (36.6°C), blood pressure is 141/84 mmHg, pulse is 71/min, and respirations are 16/min. He is difficult to arouse with a GCS of 7. A head CT is performed and can be seen in Figure A. Which of the following is the most likely cause of this patient’s presentation?
|
{
"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
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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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https://bit.ly/3LeIdlx
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031
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A 36-year-old man presents to the office for follow up after initiating antidepressant therapy. Two months ago, he was started on citalopram after several weeks of depressed mood, loss of interest in activities, depressed appetite, and inability to sleep. Today, he reports minimal improvement in his symptoms. He had some nausea after starting the medication, but this has resolved. He denies thoughts of suicide or self-harm. His temperature is 98.6°F (37.0°C), pulse is 80/min, blood pressure is 120/80 mmHg, respirations are 22/min, and oxygen saturation is 97% on room air. Exam reveals a depressed, tired appearing man. Which of the following is the most appropriate next step in management?
|
{
"A": "Switch to paroxetine",
"B": "Switch to phenelzine",
"C": "Electroconvulsive therapy",
"D": "Add mirtazapine",
"E": ""
}
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D
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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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https://bit.ly/3Lm4zC8
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032
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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
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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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https://bit.ly/3LoaHJF
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033
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A 26-year-old medical student presents to occupational health after sustaining a needlestick injury. She was drawing blood from an HIV-positive patient when she stuck herself percutaneously while capping the needle. She immediately washed the puncture wound with saline. The medical student has had a negative HIV serology from the beginning of medical school 2 years ago. She is monogamous with one male partner and denies any intravenous drug use. The source patient was recently diagnosed with HIV and has a CD4 count of 550 cells/µL. His most recent viral load is 1,800,000 copies/mL, and he was started on HAART 3 days ago. Which of the following is the most appropriate next step in management?
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{
"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
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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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https://bit.ly/3M2S7YO
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034
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A 23-year-old woman presents to the emergency department complaining of a worsening headache. The headache started 1 month ago. It is constant and “all over” but gets worse when she is lying down or in the setting of bright lights. A review of systems is significant for a low-grade fever, night sweats, cough, malaise, poor appetite, and unintentional weight loss of 12 pounds in the last 2 months. The patient is sexually active with multiple male partners and reports inconsistent condom use. She has a history of intravenous drug use and has not been to a doctor in the last 2 years. Her temperature is 100.4°F (38.0°C), blood pressure is 110/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 98% O2 on room air. On physical exam, pain is elicited upon passive flexion of the patient’s neck. A CT scan shows ventricular enlargement. A CD4+ count is 57 cells/µL blood. A lumbar puncture is performed with the following findings:
Cerebrospinal fluid:
Opening pressure: 210 mmH2O
Glucose: 32 mg/dL
Protein: 204 mg/dL
India ink stain: Positive
Leukocyte count and differential:
Leukocyte count: 200/mm^3
Lymphocytes: 100%
Red blood cell count: 2/mm^3
Which of the following treatments most directly addresses the underlying cause of this patient's symptoms?
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{
"A": "Fluconazole",
"B": "Dexamethasone",
"C": "Acyclovir",
"D": "Amphotericin B and 5-flucytosine",
"E": ""
}
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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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https://bit.ly/3MCzFqw
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035
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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
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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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https://bit.ly/3MdLLon
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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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https://bit.ly/3MoRM2B
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037
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A 69-year-old man presents to the emergency department with shortness of breath. The patient has presented 3 times this month with similar complaints. He states his shortness of breath started when he was walking from his car to a local restaurant. He does not see a primary care physician and is not currently taking any medications. He drinks alcohol socially and does not smoke. His temperature is 99.5°F (37.5°C), pulse is 100/min, blood pressure is 130/90 mmHg, respirations are 18/min, and oxygen saturation is 96% on room air. On physical exam, he appears fatigued and the cardiovascular exam reveals an additional heart sound after S2. A pulmonary exam is notable for bilateral crackles and an abdominal exam reveals an obese abdomen without pain in any of the quadrants. Lower extremity pitting edema is noted bilaterally. Which of the sets of lab values shown in Figure A would most likely be seen in this patient?
|
{
"A": "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.
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https://bit.ly/3Mqa3f2
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038
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A 72-year-old man presents to his primary care physician with weakness. He has felt very weak every morning with his symptoms persisting throughout the day. He notes minor improvement when he rides his bike but otherwise has not noticed any change in his symptoms with rest or ibuprofen use. The patient has lost 12 pounds recently and has had a chronic cough. The patient lives alone and drinks 7 alcoholic beverages per day and smokes 1-2 packs of cigarettes per day for the past 40 years. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 93/min, respirations are 17/min, and oxygen saturation is 92% on room air. A chest radiograph is ordered as seen in Figure A. Which of the following diagnostic tests would most likely elucidate the cause of this patient's weakness?
|
{
"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.
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https://bit.ly/3Mv3U1Q
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039
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A 59-year-old woman presents to her primary care physician for trouble sleeping. When she goes to bed at night, she has an urge to get up out of bed and walk around. The patient often wakes her husband when she does this which irritates him. There is a perpetual uneasiness and feeling of a need to move at night which is relieved by getting up and walking around. The patient denies symptoms during the day. She works as a mail carrier and is nearing retirement. She has a medical history of anxiety, depression, irritable bowel syndrome, and dysmenorrhea. She is not currently taking any medications. Her temperature is 99.5°F (37.5°C), blood pressure is 157/98 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals 5/5 strength in the upper and lower extremities, 2+ reflexes in the upper and lower extremities, a stable gait pattern, and normal sensation. Cardiopulmonary and abdominal exams are within normal limits. Which of the following is the most appropriate initial step in management?
|
{
"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.
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https://bit.ly/3Mv8f5p
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040
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A 57-year-old man is brought to a neurologist by his wife for abnormal behavior. He was normal until around 3 months ago when he started forgetting important meetings and misplacing items around the house. One month ago, the patient was fired from his job as a software engineer after attempting to grope a coworker. The patient expresses frustration that he is no longer able to concentrate enough to enjoy his hobby of solving puzzles. His medical history is significant for gastroesophageal reflux disease for which he takes omeprazole. He has no family history of neurologic disease. His temperature is 98.6°F (37.0°C), blood pressure is 120/75 mmHg, pulse is 70/min, and respirations are 16/min. During the examination, the patient’s cell phone rings unexpectedly causing the patient to suddenly jerk his arms and legs. Further diagnostic investigation is performed which reveals an elevated 14-3-3 protein on cerebrospinal fluid analysis. Which of the following is the most likely diagnosis?
|
{
"A": "Alzheimer disease",
"B": "Creutzfeldt-Jacob disease",
"C": "Frontotemporal dementia",
"D": "Subcortical leukoencephalopathy",
"E": ""
}
|
B
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This patient with rapidly progressive dementia over 3 months, declining executive function, impaired concentration, decreased memory, startle myoclonus (sudden jerking of extremities in response to unexpected stimulus), and elevated 14-3-3 protein levels on cerebrospinal fluid (CSF) analysis most likely has Creutzfeldt-Jakob disease (CJD).
CJD is a human prion disease in which the normal prion protein (PrPc) converts to a beta-pleated sheet disease-associated form (PrPsc) which is resistant to proteases. CJD is usually spontaneous. Rarely, CJD may be familial or acquired (from iatrogenic transmission or dietary exposure). Aggregates of PrPsc lead to spongiform vacuolation. Clinically, this manifests with rapidly progressive mental deterioration with prominent executive dysfunction (declining concentration, judgment, and memory) and startle myoclonus. Further diagnostic investigation may reveal 14-3-3 protein on CSF analysis and synchronous sharp wave complexes on electroencephalogram (EEG). CJD usually leads to death within 1 year. There is no known effective treatment for this disease.
Uttley et al. review the diagnosis, clinical presentation, and epidemiology of Creutzfeldt-Jakob disease (CJD). They discuss how CJD in a global context is increasing in prevalence and how incubation periods as long as 40 years have been observed for iatrogenic cases. They recommend heightened awareness of this disease in order to mitigate iatrogenic transmission.
Incorrect Answers:
Answer A: Alzheimer disease is characterized by gradual cognitive decline with difficulty completing activities of daily living. Impaired executive function early in the disease course, rapidly progressive disease, elevated 14-3-3 protein on CSF analysis, and startle myoclonus are not found in Alzheimer disease.
Answer C: Lewy body dementia (LBD) presents with alterations in consciousness, disorganized speech, visual hallucinations, extrapyramidal symptoms, and early compromise of executive function. Although this patient’s dementia is characterized by early compromise of executive function, the absence of fluctuating levels of consciousness, absence of extrapyramidal symptoms, and presence of startle myoclonus make CJD more likely than LBD.
Answer D: The behavioral variant of frontotemporal dementia (FTD) is associated with early-onset personality and behavioral changes, and it may be associated with extrapyramidal symptoms. Memory is not usually affected until late in the disease course.
Answer E: Subcortical leukoencephalopathy (Binswanger disease) is a type of small vessel vascular disease that presents with prominent subcortical dementia. This manifests clinically as slowed mental processing, impaired cognition, apathy, and depression. This disease almost always presents in the context of chronic hypertension and is usually seen in older patients.
Bullet Summary:
Creutzfeldt-Jacob disease is a fatal prion disease that presents with sudden-onset and rapidly progressive dementia and startle myoclonus or seizures.
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https://bit.ly/3My1xLG
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041
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A 39-year-old man presents to his doctor for a wellness checkup. He is concerned about a rash that does not seem to be improving. He was recently exposed to his grandfather who has vesicular lesions on his skin and is being treated. He has a family history of skin cancer, colon cancer, and ovarian cancer. The patient has a medical history of asthma and seasonal allergies. His temperature is 98.6°F (37.0°C), blood pressure is 137/98 mmHg, pulse is 90/min, respirations are 14/min, and oxygen saturation is 98% on room air. Physical exam reveals the finding in Figure A. Which of the following describes this patient's most likely diagnosis?
|
{
"A": "Benign capillary proliferation",
"B": "Edema of the epidermis",
"C": "Healthy dermatologic development",
"D": "Viral infection",
"E": ""
}
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A
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This patient who presents with red raised papules is presenting with a cherry angioma. These lesions are composed of benign capillary proliferations.
Cherry angiomas present with cherry red macules/papules that occur secondary to benign capillary and venule proliferation. The lesions are entirely benign and are cosmetic. These lesions are common in middle-aged adults. As the patient ages, more lesions will appear. In the absence of symptoms, observation and reassurance can be used for conservative management. They can be removed surgically if the patient desires.
Nazer et al. study the risk factors associated with the development of cherry angioma. They found that the use of clopidogrel and tamsulosin is associated with these lesions. They recommend ruling out underlying conditions.
Figure/Illustration A demonstrates cherry red macules/papules classically seen in patients with cherry angioma.
Incorrect Answers:
Answer B: Edema of the epidermis describes eczema or atopic dermatitis. This disease would present with pruritic, erythematous, and vesicular lesions. Topical creams and hygiene can be used for symptomatic management of these lesions.
Answer C: Healthy dermatologic development misses the diagnosis of a cherry angioma, which although benign, is a more accurate diagnosis for this case.
Answer D: Malignant blood vessel proliferation does not describe a cherry angioma which is benign and purely cosmetic. An angiosarcoma usually occurs in deep tissues and requires surgical excision.
Answer E: Viral infection describes herpes simplex virus which would present with vesicular lesions and neurogenic pain. These patients can be treated with valacyclovir as well as gabapentin for neuropathic pain.
Bullet Summary:
Cherry angiomas are benign capillary proliferations that appear as cherry red macules/papules.
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https://bit.ly/3N80FxZ
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042
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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
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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.
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https://bit.ly/3N81HdG
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043
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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": ""
}
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A
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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.
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https://bit.ly/3NOcdHz
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044
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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.
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https://bit.ly/3O2B9LC
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045
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A 69-year-old man presents to clinic due to shortness of breath, worsening pain in his right shoulder, and episodes of hemoptysis. His symptoms began 3 months ago. He has also lost 18 pounds. He has a history of coronary artery disease and underwent an uncomplicated coronary angioplasty with stent placement 2 years ago. He routinely travels both domestically and internationally as a senior shipyard worker and has a 30-pack-year smoking history. He used to drink 4 cocktails a week. He has not smoke or drank alcohol in over 7 years. His temperature is 99.5°F (37.7°C), blood pressure is 140/60 mmHg, pulse is 97/min, and respirations are 13/min. Physical exam is notable for right pupillary constriction as well as paresthesias in his right fourth and fifth digits. There is no pain with active shoulder rotation. His chest imaging is shown in Figure A. Which of the following is most likely responsible for the patient’s symptoms?
|
{
"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.
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https://bit.ly/3OFDpHw
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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.
|
https://bit.ly/3OhgJgC
|
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.
|
https://bit.ly/3OrGevt
|
End of preview. Expand
in Data Studio
Medbullets
HuggingFace upload of a multiple-choice QA dataset of USMLE Step 2 and Step 3 style questions sourced from Medbullets. If used, please cite the original authors using the citation below.
Dataset Details
Dataset Description
The dataset contains four splits:
- op4_test: four-option multiple-choice QA (choices A-D)
- op5_test: five-option multiple-choice QA (choices A-E)
op5_test contains the same content as op4_test, but with one additional answer choice to increase difficulty. Note that while the content is the same, the letter choice corresponding to the correct answer is sometimes different between these splits.
Dataset Sources
- Repository: https://github.com/HanjieChen/ChallengeClinicalQA
- Paper: https://arxiv.org/pdf/2402.18060v3
Direct Use
import json
from datasets import load_dataset, Dataset
def _strip_E(split):
for ex in split:
ex = dict(ex)
ex["options"] = {k: v for k, v in ex["options"].items() if k != "E"}
yield ex
if __name__ == "__main__":
op4_test, op5_test = load_dataset("mkieffer/Medbullets", split=["op4_test", "op5_test"])
# remove the "E" option from op4 split
op4_test = Dataset.from_generator(lambda: _strip_E(op4_test))
print("\nop4_test:\n", json.dumps(op4_test[0], indent=2))
print("\nop5_test:\n", json.dumps(op5_test[0], indent=2))
Citation
@inproceedings{chen-etal-2025-benchmarking,
title = "Benchmarking Large Language Models on Answering and Explaining Challenging Medical Questions",
author = "Chen, Hanjie and
Fang, Zhouxiang and
Singla, Yash and
Dredze, Mark",
editor = "Chiruzzo, Luis and
Ritter, Alan and
Wang, Lu",
booktitle = "Proceedings of the 2025 Conference of the Nations of the Americas Chapter of the Association for Computational Linguistics: Human Language Technologies (Volume 1: Long Papers)",
month = apr,
year = "2025",
address = "Albuquerque, New Mexico",
publisher = "Association for Computational Linguistics",
url = "https://aclanthology.org/2025.naacl-long.182/",
doi = "10.18653/v1/2025.naacl-long.182",
pages = "3563--3599",
ISBN = "979-8-89176-189-6",
abstract = "LLMs have demonstrated impressive performance in answering medical questions, such as achieving passing scores on medical licensing examinations. However, medical board exams or general clinical questions do not capture the complexity of realistic clinical cases. Moreover, the lack of reference explanations means we cannot easily evaluate the reasoning of model decisions, a crucial component of supporting doctors in making complex medical decisions. To address these challenges, we construct two new datasets: JAMA Clinical Challenge and Medbullets. JAMA Clinical Challenge consists of questions based on challenging clinical cases, while Medbullets comprises simulated clinical questions. Both datasets are structured as multiple-choice question-answering tasks, accompanied by expert-written explanations. We evaluate seven LLMs on the two datasets using various prompts. Experiments demonstrate that our datasets are harder than previous benchmarks. In-depth automatic and human evaluations of model-generated explanations provide insights into the promise and deficiency of LLMs for explainable medical QA."
}
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