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A 66-year-old woman presents to the emergency room after experiencing a tonic-clonic seizure. She has been having worsening headaches and intermittent nausea over the past 5 months. The headaches are constant and dull, and they typically worsen when she sneezes or laughs. Recently, she has become increasingly nauseous and has vomited twice in the past 10 days. Her medical history is significant for type 2 diabetes mellitus and hypertension for which she takes metformin and losartan. Her temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. She is noted to have papilledema on fundoscopy, but physical exam is otherwise normal. Her CT scan findings are presented in Figure A. Which of the following is the most likely diagnosis?
Cerebral toxoplasmosis
Glioblastoma multiforme
Hemorrhagic stroke
Metastatic brain tumor
B
Glioblastoma multiforme
This patient is presenting with symptoms of increased intracranial pressure (i.e., positional headaches, nausea, vomiting, and papilledema). Combined with her CT finding of a space-occupying butterfly-shaped lesion, the most likely diagnosis is glioblastoma multiforme. Glioblastoma multiforme is the most common malignant primary nervous system cancer, most commonly occurring in adults above the age of 65. The tumor arises from the cerebral hemispheres and classically crosses the corpus callosum, producing a butterfly appearance on CT/MRI. Pseudopalasading necrosis is seen on biopsy, and tissue biopsy is usually glial fibrillary acidic protein (GFAP) positive. It has an unfavorable prognosis with a life expectancy of 6 months to 1 year. Management of glioblastoma includes surgical resection and palliative radiotherapy/chemotherapy. Stupp et al. studied the role of electrical field therapy (TTFields) in conjunction with temozolomide in the treatment of glioblastoma multiforme. The authors found that combination therapy improved median progression-free survival (6.7 vs. 4.0 months, p < 0.001). The authors recommend that clinicians consider the use of TTFields to treat selected patients with glioblastoma multiforme. Figure/Illustration A demonstrates a non-contrast axial head CT showing the classic butterfly lesion characteristic of glioblastoma multiforme (red arrow). Incorrect Answers: Answer A: Cerebral toxoplasmosis presents on imaging with multiple ring-enhancing lesions and is most common in patients with acquired immunodeficiency syndrome (AIDS), especially with CD4 T-cell counts of less than 100 cells/mm^3. This patient has no signs of infection or ring-enhancing lesions. Answer C: Hemorrhagic stroke presents acutely with hemiparesis, altered mental status, slurred speech, or sensorimotor symptoms. CT imaging would reveal a hemorrhage. Answer D: Metastatic brain tumor often presents with imaging findings of multiple well-circumscribed lesions with surrounding edema. These lesions are generally found at the highly vascularized gray-white junction. In a patient with no smoking history, a single brain lesion, and no other extra-cranial symptoms, this is a less likely diagnosis. Answer E: Neurosarcoidosis can be caused by infiltrative sarcoidosis and often presents with bitemporal hemianopsia due to infiltration of the pituitary gland. Patients with sarcoidosis typically also present with pulmonary symptoms (e.g., wheezing and coughing) which are absent in this patient. Bullet Summary: Glioblastoma multiforme should be suspected in patients presenting with symptoms of increasing intracranial pressure and classic CT/MRI findings of a butterfly shaped lesion with central necrosis.
Neurosarcoidosis
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Figure A is the lateral radiograph of a 44-year-old male who sustained a fall on his outstretched hand while rollerblading. The patient reports pain localized to the posterior elbow and refuses to attempt elbow extension secondary to pain and swelling. Which of the following is the most likely diagnosis in this patient?
Exacerbation of elbow arthritis
Triceps rupture
Olecranon bursitis
Lateral collateral ligament tear
B
Triceps rupture
The flake sign, as seen in Figure A is pathognomonic of a triceps tendon rupture. Tricep tendon ruptures are rare injuries to the elbow extensor mechanism that most commonly occur as a result of forceful elbow contraction. Diagnosis can be made clinically, as patients with complete ruptures are unable to extend the elbow against gravity. An MRI may be useful in the diagnosis in patients whom are too painful to examine or who may have partial tears of the triceps tendon. Surgical repair is indicated in patients with complete rupture or in those with >50% tears and significant weakness. Yeh et al. review distal triceps ruptures. They report that these injuries are commonly caused by a fall on an outstretched hand or a direct blow. If identifiable on plain radiograph a flake sign or avulsion of the olecranon tip will be appreciated. They conclude that diagnosis may be difficult initially in a painful elbow, however, a combination of plain radiographs and MRI can assist in making the correct diagnosis. Beazley et al. review distal biceps and triceps injuries. They report that these injuries most commonly occur in middle-aged males as a result of eccentric loading of the tendon. They conclude that patients who undergo early diagnosis and surgical treatment can expect to have excellent outcomes. Keener et al. review distal tricep tendon injuries. They report that most complete tears are treated surgically in medically fit patients. Partial-thickness tears are managed according to the tear severity, functional demands, and response to conservative treatment. They conclude that anatomic footprint repair of the triceps with optimal tendon to bone healing results in an acceptable functional outcome. Figure A is the lateral radiograph of an elbow demonstrating a flake sign from the olecranon tip indicative of a triceps rupture. Incorrect Answers: Answer A: The patient does not show significant elbow degenerative disease on plain radiographs. Answer C: Olecranon bursitis is not likely to worsen acutely after a fall, nor are there any other indications of this diagnosis in the present patient. Answer D: While lateral collateral ligament tears may occur following a fall on an outstretched arm, there is no indication that an elbow dislocation occurred here and based on current radiographs a triceps rupture is more likely. Answer E: Anconeus avulsions fractures are identified as lateral epicondyle fractures.
Anconeus avulsion fracture
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A 55-year-old man presents to the emergency department with nausea and vomiting for the past week. He decided to come in when his symptoms worsened. He feels that his symptoms are exacerbated with large fatty meals and when he drinks alcohol. His wife recently returned from a cruise with symptoms of vomiting and diarrhea. The patient has a medical history of poorly managed diabetes, constipation, anxiety, dyslipidemia, and hypertension. His temperature is 99.5°F (37.5°C), blood pressure is 197/128 mmHg, pulse is 100/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals a systolic murmur heard loudest along the left upper sternal border. Abdominal exam reveals an obese, tympanitic and distended abdomen with a 3 cm scar in the right lower quadrant. Vascular exam reveals weak pulses in the lower extremities. Which of the following is the most likely underlying etiology of this patient's symptoms?
Adhesions
Enteric nervous system damage
Impacted stool
Norovirus
A
Adhesions
This patient is presenting with abdominal pain and a tympanitic abdomen, which are symptoms suggestive of a small bowel obstruction. In developed countries, these typically occur secondary to adhesions. Small bowel obstructions (SBO) typically present with an absence of bowel sounds, bowel movements, and flatus, as well as abdominal distension, nausea, and vomiting. They typically occur secondary to adhesions which entrap the bowel and disrupt its normal peristalsis. Adhesions can occur after abdominal surgery or trauma as part of the normal healing process. The diagnosis of SBO can be confirmed using abdominal imaging such as a CT scan. Treatment often involves observation; however, surgical decompression of the SBO may be needed if there is a closed-loop obstruction or an obstruction that does not resolve on its own. Rami Reddy and Cappell review the evidence regarding the diagnosis and treatment of SBO. They discuss how this disease has a 30% mortality rate if the diagnosis is missed and bowel perforation occurs. They recommend early evaluation and treatment of these patients. Incorrect Answers: Answer B: Enteric nervous system damage describes diabetic gastroparesis which can present similarly to SBO; however, this patient's age and history of surgery points toward SBO as the most likely diagnosis. Treatment is with pro-motility agents. Answer C: Impacted stool describes constipation which could present similarly to SBO; however, this diagnosis is less likely to cause nausea and vomiting unless it is severe. The vomit typically smells like stool. Treatment is with dis-impaction of the colon. Answer D: Norovirus typically causes nausea, vomiting, and diarrhea but does not cause distension and a tympanitic abdomen. Treatment is supportive as the virus self-resolves. Answer E: Twisting of bowel describes a volvulus which presents similarly to SBO; however, it is a more common diagnosis in the elderly and is a less common diagnosis than is SBO in a patient with a history of abdominal surgery. Treatment is with surgical untwisting of the bowel. Bullet Summary: Small bowel obstruction (commonly caused by adhesions) presents with nausea, vomiting, absence of flatus/bowel movements, and a distended/tympanic abdomen.
Twisting of the bowel
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A 24-year-old man presents to his primary care physician with left groin pain. The pain started a year ago and has progressed. He now has 8/10 pain when climbing stairs or rising from a seated position. He localizes the pain to the front of his groin. He denies any erythema or swelling. He has a history of hypertension, sickle cell anemia, and a splenectomy. He drinks a glass of wine with dinner every night and took up scuba diving 2 months ago but otherwise does not exercise. His temperature is 97.5°F (36.4°C), blood pressure is 124/68 mmHg, pulse is 82/min, and respirations are 16/min. His body mass index (BMI) is 18.1 kg/m^2. Physical exam reveals left groin pain at the terminal ranges of hip abduction and internal rotation. There is no point tenderness. A radiograph is obtained as in Figure A. Which of the following components of this patient’s history most strongly predisposed him to this condition?
Low BMI
Scuba diving
Alcohol use
History of sickle cell disease
D
History of sickle cell disease
This patient with sickle cell disease (SCD) presents with anterior groin pain, especially with weight-bearing, and radiographic findings of amorphous lytic and sclerotic lesions of the femoral head, consistent with avascular necrosis. SCD is a predisposing factor for avascular necrosis. Avascular necrosis has a multitude of direct causes, including irradiation, trauma (history of femoral neck fracture), hematologic diseases such as leukemia, SCD, and idiopathic etiology. In traumatic causes, injury to the medial femoral circumflex artery supplying the femoral head causes vascular disruption with resulting osteonecrosis. In SCD, the propensity for sickle-shaped erythrocytes to stick to one another and occlude blood vessels results in loss of circulation to the femoral head with consequent osteonecrosis. Avascular necrosis of the femoral head presents with insidious onset of anterior groin pain. The diagnosis can be secured with plain radiographs, although magnetic resonance imaging has the highest sensitivity and specificity for uncertain cases. Treatment consists of bisphosphonates with crutches (non-weight-bearing) and/or surgery depending on the degree of femoral head collapse and etiology. Akinyoola et al. studied the risk factors associated with avascular necrosis of the femoral head in patients with sickle cell disease. The authors found that the rate of pain crises and annual hospitalizations are associated with avascular necrosis. The authors recommend further study to explain differences in the prevalence of osteonecrosis in different populations with sickle cell disease. Figure/Illustration A shows a plain radiograph of the left hip in a skeletally mature individual, demonstrating amorphous cortical lesions (red arrows) in the femoral head and cortical irregularity (blue arrow) typical of avascular necrosis. Incorrect Answers: Answer A: Excess alcohol use has been associated with the risk of avascular necrosis. This patient uses alcohol in moderation (2 drinks or fewer per day for men) and has no history of alcohol use disorder. Answer C: Lack of physical activity is a predisposing factor for osteoporosis but is not associated with avascular necrosis. Osteoporosis may increase the risk of a hip fracture, which would present with a painful, shortened, and externally rotated lower extremity. Answer D: Low BMI is not a predisposing factor for avascular necrosis. In contrast, obesity has been associated with an increased risk of avascular necrosis. Answer E: Scuba diving can indirectly predispose patients to avascular necrosis through decompression sickness (Caisson Disease). In decompression, dissolved gases in the arterial supply form gas emboli, leading to microvascular occlusion and dysbaric osteonecrosis. However, this patient’s symptoms have been present for 1 year whereas he only recently began scuba diving. Bullet Summary: Sickle cell disease is a predisposing factor for avascular necrosis of the femoral head, which presents as insidious anterior groin pain.
nan
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A 26-year-old woman presents to the emergency department with 1 day of vaginal bleeding and lower abdominal pain. The pain is most pronounced in the lower abdomen and has gotten worse over the past day. Her last menstrual period was 7 weeks ago. She denies any recent abdominal or vaginal trauma. She has no other past medical history and takes no medications. She uses a copper intrauterine device and is in a monogamous relationship. Her temperature is 99°F (37.2°C), blood pressure is 79/50 mmHg, pulse is 135/min, and respirations are 20/min. Physical examination is notable for diffuse abdominal tenderness upon palpation. Pelvic examination demonstrates uterine and adnexal tenderness and uterine bleeding. Laboratory studies are shown below: Hemoglobin: 9.5 g/dL Leukocyte count: 6,000/mm^3 Serum beta-human chorionic gonadotropin (hCG): 2,700 IU/L The patient is started on intravenous fluids. Which of the following is the most appropriate next step in management?
CT of the abdomen and pelvis
Laparoscopy
Methotrexate therapy
Repeat beta-hCG in 48 hours
B
Laparoscopy
This patient’s acute onset abdominal pain with uterine bleeding and tenderness, positive pregnancy test, anemia, and hemodynamic instability are concerning for a ruptured ectopic pregnancy. Emergency laparoscopy is the most appropriate next step in management. An ectopic pregnancy, due to implantation of a fertilized ovum outside the uterine cavity, most commonly in the fallopian tube, can be life-threatening if it ruptures leading to intra-abdominal hemorrhage and shock. Initial laboratory studies should include a complete blood count and a pregnancy test. A patient’s hemodynamic stability should be ascertained immediately as it determines the most appropriate next step in management. In patients who are hemodynamically unstable (hypotensive and tachycardic), a Focused Assessment with Sonography for Trauma (FAST) exam should be immediately performed to evaluate for intraperitoneal bleeding, and ob/gyn should be immediately consulted for emergent surgical intervention (e.g., salpingectomy or salpingotomy). In patients who are hemodynamically stable, transvaginal ultrasound should be performed to determine the gestational sac location, and medical therapy can be initiated (e.g., methotrexate therapy, depending on the size and location of the pregnancy). Cheng et al. compare fertility outcomes of salpingotomy and salpingectomy in women with tubal pregnancies. The authors found that there was no difference in intrauterine pregnancy rates or repeat ectopic pregnancy rates between patients that underwent salpingotomy or salpingectomy. The authors recommend that both salpingectomy and salpingotomy are reasonable options for the surgical management of tubal pregnancies. Incorrect Answers: Answer A: CT of the abdomen and pelvis is useful in determining if there is an intra-abdominal hemorrhage in patients who are hemodynamically stable. Unstable patients should generally not undergo CT. Answer C: Methotrexate therapy is an effective treatment option in patients who are hemodynamically stable. with smaller ectopic pregnancies and lower hCG levels. Answer D: Repeat beta-hCG in 48 hours is reserved for hemodynamically stable patients with non-diagnostic findings on transvaginal ultrasound. If the beta-hCG level is below the discriminatory zone (beta-hCG level 1,500-2,500 IU/L), then it should be repeated in 48 hours. Answer E: Transvaginal ultrasound is reserved for hemodynamically stable patients who are presenting with abnormal uterine bleeding with abdominal/pelvic pain. Bullet Summary: Patients who present with a ruptured ectopic pregnancy with hemodynamic instability are managed with emergent surgical intervention.
Transvaginal ultrasound
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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?
Acetaminophen
Arsenic
Cyanide
Iron
B
Arsenic
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.”
Lead
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A 4-week-old boy presents to the pediatrician for vomiting. The patient is breastfed and typically feeds for 20-30 minutes every 2 hours. One week ago, he began vomiting and regurgitating breastmilk through his nose after most feedings. His stools over the past week have also become blood-streaked. The patient does not seem to be in any distress when he passes these bloody stools. The patient surpassed his birth weight by 2 weeks of age but has fallen 1 standard deviation on the growth curve since then. His temperature is 98.6°F (37°C), blood pressure is 78/47 mmHg, pulse is 115/min, and respirations are 28/min. On physical exam, the patient is well-appearing. His face and back have the physical exam finding seen in Figure A. The patient’s abdomen is soft, non-tender, and non-distended. A digital rectal exam reveals a small amount of blood in the rectal vault. Which of the following is the most appropriate next step in management?
Initiate a proton pump inhibitor
Modify mother's diet
Obtain abdominal ultrasound
Provide reassurance
B
Modify mother's diet
This infant presents with vomiting, bloody stools, and poor weight gain, which suggests a diagnosis of milk-protein allergy. The most appropriate next step in management is the modification of the mother’s diet to eliminate cow’s milk and soy. Infant food protein-induced proctocolitis is caused by distal colon inflammation secondary to an immune reaction to certain food proteins. Milk-protein allergy is the most common food allergy in children and presents with vomiting, bloody stools, and poor weight gain or failure to thrive. In an infant that is breastfed, the most appropriate next step in management is the elimination of cow’s milk and soy from the mother’s diet. In an infant who is formula fed, the most appropriate next step would be switching to either an extensively hydrolyzed or amino acid-based infant formula. Giannetti et al. review the evidence regarding the diagnosis and treatment of milk protein allergy in infants. They discuss how the selective elimination of certain proteins from the diet is usually effective. They recommend considering oral immunotherapy as an adjuvant in the treatment of this disease. Figure/Illustration A is a clinical photograph that demonstrates the finding of eczema (red circle). This finding is commonly found in patients with milk-protein allergy. Incorrect Answers: Answer A: Initiating a proton pump inhibitor would be appropriate in patients with refractory gastroesophageal reflux disease, which presents with regurgitation or vomiting and symptoms of feeding aversion or failure to thrive. Because this patient presents with bloody stools, he is more likely to have a milk protein allergy. Answer B: Obtaining an abdominal ultrasound would help evaluate for pyloric stenosis, which presents in infants between 3-6 months of age with non-bilious, projectile vomiting. Pyloric stenosis does not cause bloody stools. Treatment is with surgical pyloromyotomy. Answer C: Providing reassurance would not be appropriate for an infant presenting with bloody stools and evidence of poor weight gain. The cause of his blood-streaked stools should be investigated to ensure this patient is getting the nutrition he needs. Answer E: Switching to a hydrolyzed formula would be appropriate if this patient were formula fed, but it is not necessary to discontinue breastfeeding if the mother is willing to modify her diet to exclude cow’s milk and soy. Bullet Summary: A milk-protein allergy in a breastfed infant should be treated with modification of the mother’s diet to eliminate cow’s milk and soy.
Switch to hydrolyzed formula
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A 30-year-old woman presents to the emergency department for palpitations. For 2 months, she has experienced intermittent palpitations along with menstrual irregularity and hair loss. At home, the air conditioner is at its coldest setting, but the patient still feels overheated. Her temperature is 99.5°F (37.5°C), blood pressure is 135/90 mmHg, pulse is 120/min, and respirations are 22/min. The patient is diaphoretic with tremulous hands, demonstrates thyromegaly, and protrusion of her eyes is noted. She is given atenolol in the emergency department with improvement of her palpitations and is instructed to follow up with her primary doctor for further management. Which of the following is most likely to be found in this patient after definitive treatment of her condition?
Exophthalmos
Hair loss
Heat intolerance
Thyromegaly
A
Exophthalmos
This patient presents with palpitations, tachycardia, tremor, heat intolerance, hair loss, menstrual irregularity, goiter, and exophthalmos concerning for hyperthyroidism due to Graves disease. Radioiodine ablation is the preferred definitive management for Graves disease, which can worsen exophthalmos. Graves disease is an autoimmune hyperthyroid disorder caused by antibodies to the thyroid stimulating hormone receptor (TSHR), which induce production of T4 and triiodothyronine (T3). Overstimulation of the thyroid gland by TSHR antibodies results in diffuse enlargement of the thyroid gland, termed “goiter.” TSHR is also highly expressed in retro-ocular adipocytes and fibroblasts, where overstimulation by TSHR antibodies (and activated T-cells) leads to the expansion of retro-ocular connective tissue and extra-ocular muscle volume. This causes exophthalmos (bulging eyes) and is referred to as Graves ophthalmopathy or orbitopathy. Beta blockers are used to achieve acute sympathetic control in hyperthyroid Graves patients, and antithyroid medications (propylthiouracil or methimazole) are initiated to achieve a euthyroid state. Radioactive iodine ablation is first-line definitive management, particularly, for individuals with unsuccessful anti-thyroid medication management. Radioiodine ablation has been known to increase TSHR-antibodies, likely secondary to the leakage of thyroid antigens, and thus exophthalmos is often worsened after treatment. For this reason, corticosteroids are often co-administrated as a prophylactic measure. Davies et al. review Graves disease including the pathophysiology and management. They note that Graves orbitopathy may be worsened by radioiodine ablation. They recommend further studies and advancements in care given this lasting complication. Incorrect Answers: Answer B: Hair loss can occur in hyperthyroid and hypothyroid states due to altered hair follicle stem cell function. However, this process is typically reversed, not worsened, after achieving a euthyroid state. Answer C: Heat intolerance occurs in Graves disease due to hyperthyroid-induced sympathetic overactivity, which resolves after successful treatment. Answer D: Menstrual irregularity occurs in hyperthyroidism through altered levels of sex hormone binding globulin, gonadotropin-releasing hormone, and prolactin. However, treatment of hyperthyroidism typically restores menstrual regularity. After ablation, patients will be hypothyroid; however, the standard of care is to start the patient on levothyroxine which should prevent this complication. Answer E: Thyromegaly in Graves disease is caused by diffuse overactivation of the thyroid gland by the TSHR antibodies. Once treated with radioactive iodine (I-131), the thyroid gland gradually shrinks in size. Patients who undergo radioactive iodine ablation will later require life-long thyroxine supplementation due to resulting hypothyroidism. Bullet Summary: Definitive therapy for Graves disease is radioiodine ablation, which leads to increased TSHR antibodies and worsened exophthalmos from overactivation of TSHR-rich retro-ocular adipocytes and fibroblasts.
nan
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A 59-year-old man presents to the emergency department after a sudden onset of slurred speech and right upper extremity weakness. His symptoms lasted 20 minutes but by the time he arrived at the emergency department he was symptom-free. The patient denies any preceding symptoms. He has a history of diabetes, obesity, and hypertension but does not see a physician for his underlying conditions. His temperature is 99.2°F (37.3°C), blood pressure is 140/85 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 100% on room air. Physical exam reveals normal vision, strength, and sensation with a stable gait. Finger-nose testing and tandem gait are unremarkable. A CT scan of the head is performed and is unremarkable. Which of the following is the most appropriate next step in management?
Aspirin
Atorvastatin
CTA head/neck
Labetalol
A
Aspirin
This patient is presenting with a transient episode of neurologic dysfunction (slurred speech and right upper extremity weakness) that has completely resolved, which is concerning for a transient ischemic attack. In the setting of his normal head CT, aspirin should be administered next. A transient ischemic attack (TIA) is a brief episode of focal neurologic dysfunction that completely resolves without any residual deficits. Patients with a TIA are at high risk for future TIAs and stroke. For this reason, management is immediately centered on a thorough neurological exam followed by a CT scan of the head to rule out a hemorrhage. Subsequently, aspirin should immediately be administered which reduces the incidence of future stroke. Patients will subsequently be discharged on aspirin in addition to other morbidity-lowering agents including a statin and tight blood pressure and glycemic control as well as a second antiplatelet agent in appropriate patients. Further workup for a TIA may include a CTA head/neck, an MRI brain, and an echocardiogram. Modification of risk factors is also critical in these patients including weight loss, smoking cessation, exercise, glycemic control, and blood pressure control. Clissold et al. review transient ischemic attacks. They note that transient ischemic attacks (TIA), if untreated, carry a high risk of early subsequent stroke. They recommend, after appropriate diagnostic workup, early administration of an antiplatelet agent. Incorrect Answers: Answer B: Atorvastatin is an important initial intervention in the management of patients who have TIA/stroke. However, this intervention takes weeks of consistent therapy in order to have a significant effect on morbidity, thus it would not be the most important next step in management when compared to aspirin, which is more important in reducing acute stroke risk. Further, a lipid panel is generally performed first to determine if a statin is needed. Answer C: CTA head/neck is a part of the workup of a TIA to rule out carotid stenosis/dissection/atherosclerosis as the cause of the patient's symptoms. It is more important to give aspirin early in TIA/stroke prior to obtaining further imaging (though this study would still be performed). Answer D: Labetalol and other blood pressure medications are important in risk factor modification to prevent future strokes/TIAs. Patients with diabetic nephropathy should be on an ACE inhibitor or angiotensin-receptor blocker which are nephroprotective. Of note, his blood pressure currently is not very elevated nor is it causing end-organ dysfunction. Answer E: MRI brain should be performed to further characterize whether there was any ischemic injury to the brain; however, this usually will occur after a CTA head/neck and certainly after the patient receives an aspirin. It can more accurately characterize if a stroke occurred, and the extent of the injury. Bullet Summary: The workup of a transient ischemic attack should be centered on a thorough neurological exam, a non-contrast CT scan of the head, and administration of aspirin assuming there is no intracranial bleed.
MRI brain
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A 31-year-old man presents to clinic for evaluation of a 3-year history of infertility. He has no significant medical history and takes no medications. He exercises twice a day, often with heavy weightlifting, and eats a high-protein diet. He reports occasional alcohol use and remote marijuana use. His temperature is 99.5°F (37.5°C), blood pressure is 148/90 mmHg, pulse is 84/min, and respirations are 15/min. He is sexually active with his wife and has no history of sexually transmitted infections. Physical exam reveals small testes and a receding hairline. He also has palpable tissue underneath his nipples bilaterally. His muscle tone is normal in his upper and lower extremities bilaterally. His laboratory values are below: Serum: Creatinine: 1.3 mg/dL Glucose: 110 mg/dL Total bilirubin: 0.8 mg/dL Aspartate aminotransferase (AST, GOT): 410 U/L Alanine aminotransferase (ALT, GPT): 275 U/L Hemoglobin: 12 g/dL Leukocyte count: 5,300/mm^3 Platelet count: 250,000/mm^3 Which of the following is the most likely cause of this patient’s infertility?
Klinefelter syndrome
Cryptorchidism
Anabolic steroid use
Excess growth hormone use
C
Anabolic steroid use
This patient with infertility is presenting with decreased testicular size, receding hairline, gynecomastia (palpable breast tissue), and transaminitis. This is most likely due to exogenous testosterone from anabolic (androgenic) steroid use. Young people (especially athletes and heavy weightlifters) looking to improve their physical performance may rely on the use of anabolic steroids (testosterone, boldenone, and stanozolol). These steroids adversely impair endogenous testicular function, causing infertility secondary to decreased testicular size and sperm count. Furthermore, hepatic dysfunction and cardiac dysfunction can occur at high doses. Excess testosterone is converted to estradiol, leading to breast enlargement and signs of gynecomastia. Aggression and mood disturbances may also occur in patients with excessive anabolic steroid use. Finally, skin findings of anabolic steroid use include accelerated male pattern baldness and acne. Patients should be counseled on avoiding these agents. Van Wagoner et al. study the chemical composition of products marketed as selective androgen receptor modulators (SARMs). The authors find that less than half of the analyzed products contained compounds in the amount listed on the label. The authors recommend that clinicians counsel athletes and bodybuilders that these products are often mislabeled, are not regulated, and have not been shown to be safe or effective. Incorrect Answers: Answer B: Cryptorchidism is the failure of testicular descent into the scrotum and typically manifests in infancy. While uncorrected cryptorchidism can be a cause of infertility, this is an unlikely diagnosis in a man with palpable testes. Furthermore, cryptorchidism would not explain this patient’s receding hairline or gynecomastia. Answer C: Excess growth hormone use should be suspected because growth hormone can be taken exogenously to improve athletic performance and can present with hypertension. This typically also presents with hyperglycemia and enlarged hands and feet. Answer D: Klinefelter syndrome is an inherited disorder that also presents with infertility, gynecomastia, and decreased testicular size. This disorder also causes mental impairment and would not explain the patient’s elevated aminotransferases. Answer E: Myotonic dystrophy is a multi-system disorder that can also present with testicular atrophy, reduced fertility, and hair loss. Patients with myotonic dystrophy also have widespread muscular atrophy and weakness, as well as clinical features of myotonia (e.g., difficulty releasing a handshake). Bullet Summary: Anabolic steroid use in men can cause reduced fertility by suppressing endogenous testosterone activity, and presents with gynecomastia, testicular atrophy, accelerated male balding patterns, and hepatotoxicity.
nan
https://step2.medbullets.com/testview?qid=108516
A 44-year-old woman presents to the emergency department with fluctuating right upper quadrant abdominal pain. The pain was initially a 4/10 in severity but has increased recently to a 6/10 prompting her to come in. The patient has a medical history of type 2 diabetes mellitus, depression, anxiety, and irritable bowel syndrome. Her current medications include metformin, glyburide, escitalopram, and psyllium husks. Her temperature is 99.2°F (37.3°C), pulse of 95/min, blood pressure of 135/90 mmHg, respirations of 15/min with 98% oxygen saturation on room air. On exam, the patient is an obese woman with pain upon palpation of the right upper quadrant. Initial labs are are below: Na+: 140 mEq/L K+: 4.0 mEq/L Cl-: 100 mEq/L HCO3-: 24 mEq/L AST: 100 U/L ALT: 110 U/L Amylase: 30 U/L Alkaline phosphatase: 125 U/L Bilirubin Total: 2.5 mg/dL Direct: 1.8 mg/dL The patient is sent for a right upper quadrant ultrasound demonstrating an absence of stones, no pericholecystic fluid, a normal gallbladder contour and no abnormalities noted in the common bile duct. MRCP with secretin infusion is performed demonstrating patent biliary and pancreatic ductal systems. Her lab values and clinical presentation remain unchanged 24 hours later. Which of the following is the most appropriate next step in management?
Laparoscopy
ERCP with manometry
Analgesics and await resolution of symptoms
MRI of the abdomen
B
ERCP with manometry
This patient is presenting with classic symptoms of acute cholecystitis or choledocholithiasis such as right upper quadrant pain and direct hyperbilirubinemia with ultrasound findings demonstrating the absence of gallstones. In this setting, sphincter of Oddi dysfunction should be suspected, and ERCP with manometry should be performed to confirm the diagnosis. Cholecystitis is inflammation of the gallbladder caused by irritation due to an intraluminal stone and possible bacterial translocation. The classic risk factors for this disease can be remembered as "fat, female, fertile and forty". Symptoms of acute cholecystitis are right upper quadrant pain that relapses and remits over time. Lab values will show elevated AST, ALT, alkaline phosphatase, and bilirubin which are also seen in patients where a stone is impacted in the common bile duct. A right upper quadrant ultrasound and MRCP can be used to confirm the diagnosis. Dysfunction of the Sphincter of Oddi should be suspected in patients with classic findings but negative imaging studies. In these cases, the most appropriate test is ERCP with manometry of the sphincter of Oddi. Ultimately, a sphincterotomy will be needed once the diagnosis is confirmed. Coucke et al. present the current evidence for the diagnosis and treatment of biliary obstruction. They discuss how the most common etiology of biliary obstruction is choledocholithiasis or gallstones. They recommend having a high index of suspicion for cholangitis as this condition can be rapidly fatal if untreated. Incorrect Answers: Answer A: Analgesics and await resolution of symptoms (in addition to NPO and IV fluids) would be appropriate management for pancreatitis. The diagnosis in this patient has not yet been confirmed (given the absence of findings on ultrasound) thus prompting another diagnostic test. Answer B: An elective cholecystectomy would be the most appropriate next step in management if the patient had cholecystitis. Answer D: Laparoscopy is an inappropriate and invasive method of diagnosing and treating disease of the biliary tree as there are better alternatives such as MRCP or ERCP. Laparoscopy would be effective in the treatment of intestinal tract disorders such as appendicitis. Answer E: An MRI of the abdomen would not confirm the diagnosis of sphincter of Oddi dysfunction. Though a CT scan could be performed as a more cost-effective alternative, given the absence of stones on ultrasound it is likely that a CT scan will not give more information. Bullet Summary: Sphincter of Oddi dysfunction can be diagnosed with ERCP and manometry.
nan
https://step2.medbullets.com/testview?qid=109452
A newborn boy is evaluated in the hospital nursery 2 hours after birth. The patient was born at 39 weeks of gestation to a 30-year-old primigravid via vaginal delivery. The patient’s mother received routine prenatal care and the pregnancy was uncomplicated. The patient’s anatomy ultrasound at 20 weeks of gestation was unremarkable. The patient’s mother denies any family history of genetic diseases. The patient’s APGAR scores were notable for poor muscle tone at both 1 and 5 minutes of life. The patient’s birth weight is 2.6 kg (5 lb 11 oz), which is at the 5th percentile. His height and head circumference are in the 15th and 3rd percentile, respectively. His temperature is 97.1°F (36.2°C), blood pressure is 57/42 mmHg, pulse is 140/min, and respirations are 38/min. On physical exam, the patient has a wide nasal bridge, down slanting palpebral fissures, and widely spaced eyes. He has good respiratory effort with a high-pitched cry. This patient is most likely to have experienced a deletion on which of the following chromosomes?
4p
5p
5q
7q
B
5p
This patient presents with a characteristic mew, microcephaly, hypertelorism, wide nasal bridge, and hypotonia, which suggests a diagnosis of cri-du-chat syndrome. Cri-du-chat syndrome is caused by a deletion on chromosome 5p. Cri-du-chat syndrome is most commonly caused by a de novo partial deletion of chromosome 5p. Other cases may be caused by a parental translocation involving the relevant section of chromosome 5p. Patients present with a mew-like cry, microcephaly, hypertelorism, down slanting palpebral fissures, a broad nasal bridge, low-set ears, low birth weight, hypotonia, and intellectual disability. The cat-like cry usually resolves by 2 years of age. Treatment is supportive though some patients may have cardiac abnormalities that require surgical intervention. Cerruti Mainardi reviews the evidence regarding the etiology and diagnosis of patients with Cri-du-chat syndrome. She discusses how the diagnosis is mostly made clinically at this time. She recommends karyotype and FISH studies for those with ambiguous features. Incorrect Answers: Answer A: A deletion on chromosome 4p causes Wolf-Hirshhorn syndrome, which presents with growth restriction, microcephaly, congenital heart disease, severe intellectual disability and dysmorphic "Greek warrior helmet" facies of a high forehead, prominent glabella, arched eyebrows, hypertelorism, and epicanthal folds. Treatment is supportive though some patients may require surgical intervention for heart defects. Answer C: A deletion on chromosome 5q results in Sotos syndrome, a pediatric overgrowth disorder characterized by an increase in head circumference, hypotonia, delayed acquisition of motor skills, and mild intellectual disability. Answer D: A deletion on chromosome 7q may results in Williams syndrome, which presents with dysmorphic, “elfin” facies, global developmental delay with a hypersocial personality, and supravalvular aortic stenosis. Treatment is supportive though some patients will require surgical intervention for aortic stenosis. Answer E: A deletion on chromosome 15q may cause either Prader-Willi syndrome or Angelman syndrome, depending on whether the deletion affects the maternal or paternal chromosome. Prader-Willi syndrome classically presents with dysmorphic facies, hyperphagia, and hypogonadism. Angelman syndrome classically presents with intellectual disability, frequent smiling or laughing, and gait ataxia. Treatment is supportive with seizure control for those who develop seizures. Bullet Summary: Cri-du-chat syndrome (presents with a characteristic mew, microcephaly, hypertelorism, wide nasal bridge, and hypotonia) is most commonly caused by a deletion on chromosome 5p.
15q
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A 41-year-old man presents to his primary care provider with a 2-month history of abdominal pain. He says that the pain “comes and goes” throughout the day and usually lasts 20-30 minutes per episode. The pain is above his umbilicus. He denies any feeling of regurgitation or nighttime cough but endorses nausea. He used to eat 3 large meals per day but has found that eating smaller meals more frequently improves his pain. He tried ibuprofen with food and thinks it helped. He has gained 4 pounds since his last appointment 3 months ago. He denies any diarrhea or change in his stools. He has no known medical history. He drinks 5-6 beers on the weekend and has a 20 pack-year smoking history. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 117/72 mmHg, pulse is 72/min, and respirations are 13/min. On physical exam, he is tender to palpation above the umbilicus. Bowel sounds are present. A stool guaiac test is positive. The patient undergoes an endoscopy with a biopsy to diagnose his condition. Which of the following is most likely to be found on histology?
Crypt abscesses in the large intestine
Mucosal defect in the stomach
Noncaseating granulomas in the small intestine
PAS-positive material in the small intestine
E
Urease-producing organism in the small intestine
This patient presents with episodic epigastric pain and nausea that improves with the consumption of food, which suggests a diagnosis of a duodenal ulcer. Duodenal ulcers are often caused by infection with Helicobacter pylori, a urease-producing organism. Duodenal ulcers are erosions and defects in the mucosal lining of the small intestines that allow for damage to the underlying tissues. Patients will classically present with abdominal pain that is localized to the epigastric region. Duodenal ulcers improve upon eating due to the secretion of bicarbonate in the small intestines, whereas gastric ulcers are exacerbated by food due to the release of gastric acid in the stomach. Duodenal ulcers are most commonly caused by infection with H. pylori. If the patient tests positive for H. pylori infection, treatment involves triple therapy with amoxicillin, clarithromycin, and a proton pump inhibitor. Patients who fail to improve should undergo esophagogastroduodenoscopy, which remains the gold standard for diagnosis. Ciociola et al. studied the prevalence of H. pylori infection in patients with gastric ulcers. They found that about many had confirmed infections. They recommend against empiric treatment without prior confirmation of infection. Incorrect Answers: Answer A: Crypt abscesses are found on histology in ulcerative colitis. Ulcerative colitis presents with bloody diarrhea and abdominal pain. Patients can also experience musculoskeletal pain, uveitis, and dermatologic findings such as erythema nodosum or pyoderma gangrenosum. Treatment may include mesalazine. Answer B: A mucosal defect in the stomach is the pathologic description of a gastric ulcer. This patient’s presentation of epigastric pain that improves with the consumption of food is more consistent with an ulcer in the duodenum. Treatment of a gastric ulcer may include proton pump inhibitors. Answer C: Noncaseating granulomas are found on histology in Crohn disease. This patient denies any diarrhea (often associated with pain), which is the primary feature of Crohn disease. Other findings include abscesses, fistulas, and strictures. Treatment may include mesalamine and infliximab. Answer D: PAS-positive material is found in the lamina propria on small intestinal biopsy in Whipple disease. In addition to abdominal pain, Whipple disease is characterized by diarrhea with flatulence and steatorrhea, fever, arthralgias, and weight loss. Treatment is with ceftriaxone or penicillin. Bullet Summary: Duodenal ulcers typically present with episodic epigastric abdominal that is improved after eating and are associated with H. pylori infection.
Urease-producing organism in the small intestine
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An 18-year-old man presents to his primary care provider with his parents for a sports physical. He was last seen in the clinic several months ago when he was diagnosed with attention deficit hyperactivity disorder (ADHD). He was started on methylphenidate at that time and the patient now reports improvement in his ability to concentrate in school and at home. He hopes to play baseball in college and has begun lifting weights daily in preparation for baseball season. The patient has a healthy diet to fuel his exercise regimen. His parents have no concerns and are pleased with the recent improvement in his grades. His temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. On physical exam, the patient has tall stature with average muscle mass for his age. He has no dysmorphic features. His chest has a normal appearance other than mild gynecomastia. The patient has sparse facial hair and a moderate amount of coarse pubic hair that extends across the pubis and spares the medial thighs. His testes are small and firm. Laboratory testing is performed and reveals the following: Follicle-stimulating hormone (FSH): 42 mIU/mL Luteinizing hormone (LH): 38 mIU/mL Which of the following is the most likely etiology of this patient’s presentation?
Anabolic steroid use
CGG trinucleotide repeat disorder
CTG trinucleotide repeat disorder
Failure of neuronal migration
E
Meiotic nondisjunction
This patient presents with tall stature, gynecomastia, and small testes with elevated FSH and LH, which suggests a diagnosis of Klinefelter syndrome. Klinefelter syndrome is usually caused by meiotic nondisjunction that results in a 47,XXY genotype. Klinefelter syndrome is the most common cause of primary hypogonadism. Patients with Klinefelter syndrome present with tall stature, neurocognitive difficulties (ADHD), and features of hypogonadism including gynecomastia, small testes, small phallus, hypospadias, underdeveloped secondary sex characteristics, and cryptorchidism. Patients without hypospadias or cryptorchidism are often not diagnosed until after puberty when the symptoms of gynecomastia and small testes become more prominent. Because the hypogonadism in Klinefelter syndrome is caused by testicular fibrosis, laboratory results demonstrate low testosterone and elevated FSH and LH levels. Treatment for this condition includes androgen replacement therapy. Lanfranco et al. review the evidence regarding the treatment of Klinefelter syndrome. They discuss how modern fertility augmentation therapy has allowed some of these patients to produce viable offspring. They recommend having a detailed discussion with these patients and their partners regarding possible outcomes. Incorrect Answers: Answer A: Anabolic steroid use causes decreased levels of FSH and LH due to the suppression of GnRH release by the hypothalamus. This results in decreased FSH and LH release by the pituitary gland. Anabolic steroid use would not present with signs of hypogonadism. Answer B: The CGG trinucleotide repeat disorder characterizes fragile X syndrome. Fragile X presents with macroorchidism rather than hypogonadism and patients typically have dysmorphic features of a long, narrow face with large ears, a prominent forehead, and a prominent chin. Fragile X is the most common cause of inherited intellectual disability. Answer C: The CTG trinucleotide repeat disorder characterizes myotonic dystrophy. Although myotonic dystrophy presents with hypogonadism, patients would also present with symptoms of progressive weakness, such as facial weakness, dysphagia, or hand grip weakness. Treatment is with cardiac pacemakers and ventilatory support. Answer D: Failure of neuronal migration characterizes Kallmann syndrome. Kallmann syndrome presents with the classic symptoms of anosmia and hypogonadism, but patients with Kallmann syndrome have a low FSH and LH. Treatment is with hormone replacement therapy. Bullet Summary: Klinefelter syndrome results in primary hypogonadism and presents with tall stature, gynecomastia, small testes, a small phallus, hypospadias, and cryptorchidism.
Meiotic nondisjunction
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A 3-month-old boy presents to the pediatrician with his mother for a well-child visit. The patient drinks 4 ounces of cow’s milk formula every 3 hours. He usually stools 1 time per day and urinates up to 6 times per day. He regurgitates a moderate amount of formula through his nose and mouth after most feeds. He does not seem interested in additional feeding after these episodes of regurgitation and he has become progressively more irritable around meal times. The patient is starting to refuse some feeds. His mother denies seeing blood or streaks of red in his stool and she denies any family history of food allergies or dermatological problems. The patient’s weight was in the 62nd percentile 4 weeks ago and he is now in the 48th percentile. His height and head circumference have followed similar trends. His temperature is 98.2°F (36.8°C), blood pressure is 72/47 mmHg, pulse is 125/min, and respirations are 27/min. On physical exam, the patient smiles reciprocally and can lift his head and chest when in the prone position. His abdomen is soft, non-tender, and non-distended. Which of the following is the most appropriate next step in management?
Provide reassurance
Counsel on positioning and thickening feeds
Obtain abdominal ultrasound
Initiate proton pump inhibitor
B
Counsel on positioning and thickening feeds
This patient presents with frequent regurgitation after feeding, worsening irritability, feeding aversion, and poor weight gain, which is concerning for gastroesophageal reflux disease (GERD). The most appropriate next step in management is counseling on proper positioning and thickening feeds. Infants with GERD may present with regurgitation or vomiting after feeding, significant irritability, feeding aversion, and poor weight gain or failure to thrive. Symptoms can usually be managed with lifestyle changes including more frequent, thicker, and lower volume feeds and positioning the patient upright during and after feeding. If symptoms continue, patients should undergo a trial of a dairy-free diet to address possible underlying food protein intolerance. Pharmacotherapy with proton pump inhibitors (PPI) is appropriate for patients with persistent symptoms despite these changes, although PPIs can increase the risk of pneumonia and infection with C. difficile. Chabra and Peeples review the evidence regarding the treatment of neonatal GERD. They discuss how alterations in the infant's environment and feeding pattern are effective in most cases. They recommend proton pump inhibitors if conservative treatments are not effective. Incorrect Answers: Answer B: Initiating medical therapy with a proton pump inhibitor is appropriate for patients who have already failed lifestyle changes, including positioning therapy, adjusting feeding patterns, and eliminating cow’s milk and soy from the diet. Answer C: Obtaining an abdominal ultrasound would help diagnose pyloric stenosis. Patients with pyloric stenosis typically present at a younger age (between 3 and 6 weeks of life) with non-bilious, projectile vomiting and a palpable “olive-shaped” mass in the epigastrium. Treatment is with a pyloromyotomy. Answer D: Providing reassurance would not be appropriate due to this patient’s poor weight gain. Although most cases of gastroesophageal reflux are uncomplicated and self-resolve by 1 year of age, this patient’s symptoms should be addressed to ensure he is meeting his nutritional requirements. Answer E: Switching to a hydrolyzed formula to eliminate cow’s milk and soy protein from the patient’s diet would be appropriate if the patient’s symptoms did not improve with changing position and thickening feeds. Bullet Summary: The most appropriate initial step in managing gastroesophageal reflux disease in infants is counseling on proper positioning and thickening feeds.
nan
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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?
Advanced sleep phase disorder
Chronic insomnia
Delayed sleep phase disorder
Normal aging
A
Advanced sleep phase disorder
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.
Obstructive sleep apnea
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A 72-year-old man presents to his primary care physician with weakness. He has felt very weak every morning with his symptoms persisting throughout the day. He notes minor improvement when he rides his bike but otherwise has not noticed any change in his symptoms with rest or ibuprofen use. The patient has lost 12 pounds recently and has had a chronic cough. The patient lives alone and drinks 7 alcoholic beverages per day and smokes 1-2 packs of cigarettes per day for the past 40 years. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 93/min, respirations are 17/min, and oxygen saturation is 92% on room air. A chest radiograph is ordered as seen in Figure A. Which of the following diagnostic tests would most likely elucidate the cause of this patient's weakness?
Blood laboratory test
CT scan of the chest
Electromyography
Lung biopsy
C
Electromyography
This patient who presents with weakness that improves with activity in the setting of a lung mass most likely has Lambert-Eaton syndrome. The most appropriate diagnostic test to confirm this syndrome is electromyography. Small cell lung cancer presents with fatigue, weight loss, cough, and a coin lesion on chest radiograph typically in a smoker. A common paraneoplastic syndrome in small cell lung cancer is Lambert-Eaton syndrome which presents with muscle weakness that improves with activity and worsens with rest. Activity improves symptoms due to accumulation of calcium with exercise which allows for acetylcholine release. This occurs secondary to antibodies against presynaptic calcium channels which decrease acetylcholine release. The diagnosis of Lambert Eaton syndrome can be confirmed with electromyography. Treatment includes addressing the underlying lung tumor. Kesner et al. review the diagnosis and etiology of Lambert-Eaton syndrome. They discuss how this disease is associated with lung cancer. They recommend making the diagnosis using electrophysiologic studies. Figure/Illustration A is a chest radiograph with a coin lesion in the left lung field (red circle). This finding is suggestive of small cell lung cancer. Incorrect Answers: Answer A: Blood laboratory tests could assess for multidrug resistance 1 protein (MDR-1 aka P-glycoprotein or ABCB1) which predicts resistance to chemotherapy for small cell lung cancer; however, it would not further elucidate this patient's muscle weakness. Answers 2 & 4: CT scan of the chest and lung biopsy would further elucidate this patient's small cell lung cancer; however, it would not explain his weakness that improves with activity. In the setting of an elderly smoker with fatigue and weight loss and a coin lesion, the diagnosis of lung cancer is essentially established. Answer E: Muscle biopsy would be the most accurate diagnostic test for dermatomyositis, polymyositis, and inclusion body myositis; however, it would not assess for conditions such as myasthenia gravis and Lambert-Eaton syndrome. Bullet Summary: Electromyography is the diagnostic test of choice for Lambert-Eaton syndrome.
Muscle biopsy
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A 69-year-old man passes away after a 3-week hospitalization for liver failure, complicated by a myocardial infarction. A medical student rotating in the intensive care unit asked if she can practice performing intubations during her rotation. The resident suggests performing one on the deceased patient, as the student cannot harm him. Which of the following is the most appropriate course of action for the medical student?
Ask the resident if the patient’s family or the patient has been consented for this procedure
Contact the patient's family to ask for permission to perform the procedure
Intubate the patient with the resident's supervision
Report the resident to hospital administration
A
Ask the resident if the patient’s family or the patient has been consented for this procedure
This medical student wants to perform a procedure on a deceased patient for educational purposes. Before proceeding, the student should ensure that permission has been obtained from the patient's family or the patient when he was alive. Performing procedures on deceased patients is ethically permissible with appropriate consent and consideration of risks and benefits for patients, families, learners, staff, and the field of medicine. Supervising physicians must obtain informed consent from the patient while alive and with capacity or from a newly deceased patient’s healthcare proxy or family. During the consent process, the supervisor should outline the patient's and family's rights, wider educational and societal benefits, potential risks, and ensure that performing the procedure would be consistent with the patient's values and wishes. Additionally, the supervising physician has a responsibility to ensure that a medical examiner has cleared the patient and that the patient is not an organ donor before proceeding. Finally, the procedure should be documented in the patient's chart. Berger and Cassell review the ethics of practicing procedures on deceased patients. They note that some ethical norms may suggest this practice is not appropriate. They recommend performing procedures when consent is obtained and it is ethically acceptable to do so. Incorrect Answers: Answer B: Consulting the hospital ethics committee is not necessary, for with proper permission, trainees may perform procedures on deceased patients for educational purposes. The ethics committee should only be consulted when there is no clear course of action (an example may be no advanced directive with family members disagreeing on what the patient's wishes would have been for a critically ill patient). Answer C: Contacting the patient's family to ask for permission to perform the procedure is incorrect, as the supervising physician needs to verify and document consent. Medical students cannot consent patients and family members. Moreover, the family should not be contacted if the patient has already approved or refused this when he had capacity. Answer D: Intubating the patient with the resident's supervision is incorrect because proper permission must be ensured prior to performing educational procedures on deceased patients. Answer E: Reporting the resident to hospital administration is incorrect because it is ethically permissible to perform an intubation on a deceased patient provided there is proper permission. The resident is not asking the medical student to do anything illegal or against hospital policy. Reporting the resident may be indicated if the resident was performing procedures against the patient's will. Bullet Summary: Trainees may perform procedures for educational purposes on deceased patients if permission is obtained from the patient prior to death, the patient's family, or a healthcare proxy.
nan
https://step2.medbullets.com/testview?qid=109967
A 55-year-old male bodybuilder presents to the emergency department with weakness of his right arm. The patient states he has experienced these symptoms for a few weeks; however, today his hand felt so weak he dropped his cup of tea. The patient has a medical history of diabetes. He drinks 2-7 alcoholic drinks per day and has smoked 2 packs of cigarettes per day since he was 25. The patient uses anabolic steroids. He has lost 17 pounds since he last came to the emergency department 1 month ago. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals decreased sensation in the right arm and 2/5 strength in the right arm and 5/5 strength in in the left arm. The patient states that he is experiencing a dull aching and burning pain in his right arm during the exam. Symptoms are not changed with changes in head and neck position. Which of the following is the most likely diagnosis?
Apical lung tumor
Brachial plexopathy
Cerebral infarction
Scalenus anticus syndrome
A
Apical lung tumor
This patient is presenting with unilateral upper extremity paresthesia and weakness suggestive of compression of the neurovascular structures of the upper extremity secondary to an apical lung tumor. An apical lung tumor can compress the neurovascular structures in its proximity and cause pain, numbness, and weakness of the ipsilateral arm. A Horner syndrome can also be observed with compression of the sympathetic chain leading to ipsilateral miosis, ptosis, and anhidrosis in some cases. These malignancies typically occur in older male smokers and can be associated with symptoms such as fatigue, cough, and weight loss. Chest radiography will demonstrate an apical opacity and CT scan can confirm the size and location of the mass. Treatment is with chemotherapy and surgical excision of the tumor. Davis and Knight review the evidence regarding the diagnosis and treatment of patients with apical lung tumors. They discuss how these tumors can be associated with brachial plexus injuries. They recommend surgical excision of these tumors if they are resectable. Incorrect Answers: Answer B: Brachial plexopathy could present with weakness and numbness of the ipsilateral arm but is a less likely diagnosis (and a diagnosis of exclusion). Treatment is with identification and surgical release of any underlying anatomic defects (such as compressive bands) and supportive therapy if none are found. Answer C: Cerebral infarction (stroke) would present with sudden onset of acute neurological deficits. These would typically only affect one side of the body and would not necessarily be associated with a dull pain or numbness that is chronic. Treatment is with thrombolytics if patients meet criteria for acute therapy. Answer D: Scalenus anticus syndrome occurs in bodybuilders or athletes with hypertrophied neck muscles that compress the brachial plexus. Symptoms are typically worsened by certain positions. Treatment is with physical therapy and surgical decompression in refractory cases. Answer E: Subclavian steal syndrome presents with numbness, tingling, weakness in a limb, and CNS symptoms (vertigo, slurred speech, or stroke-like symptoms) that are worsened with activity and relieved with rest. Treatment is with vascular intervention to restore blood flow. Bullet Summary: Apical lung tumors can compress neurovascular structures in the upper extremity and cause pain, numbness, and tingling in the ipsilateral arm.
nan
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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?
Diabetes
Hematuria
Infection
Splenectomy
B
Hematuria
Based on the presence of both hemoglobin (Hb) A and S on gel electrophoresis, this patient has sickle cell trait. Episodes of painless hematuria are commonly seen in these patients. Sickle cell trait is a condition that refers to carrying 1 normal and 1 abnormal beta-globin allele. On electrophoresis, this will present as 3 different bands consisting of about 50-60% HbA, 35-45% HbS, and < 2% HbF (fetal hemoglobin). HbF decreases significantly after the first 6 weeks of life. Individuals with sickle cell trait tend to be asymptomatic and do not have a decreased lifespan compared to the general population. However, they are at higher risk of some conditions. Patients are at increased risk of rhabdomyolysis during strenuous physical activity and may have episodic painless hematuria due to renal papillary necrosis. The hyperosmolar, acidotic, and hypoxic environment of the renal medulla is thought to trigger red cell sickling which results in ischemia and subsequent hematuria. Patients with sickle cell trait are managed similarly to the general population. These patients should receive reproductive counseling and use appropriate preventive measures to prevent dehydration during intensive exercise. Hulsizer et al. studied the association between sickle cell trait and common medical conditions using insurance claims data. The authors found that sickle cell trait is associated with increased risks of diabetes, hypertension, heart disease, chronic kidney disease, and retinopathy. The authors recommend further evaluation of these associations using prospective studies derived from clinical data. Figure/Illustration A shows gel electrophoresis with an example of a normal (95-98% HbA and < 2% HbF), sickle cell trait (50-60% HbA, 35-45% HbS, and < 2% HbF), and sickle cell anemia (85-95% HbS and 5-15% HbF) patient from left to right. Incorrect Answers: Answer A: Diabetes has not been associated with sickle cell trait in the majority of studies. Answer C: Infection is a complication of sickle cell anemia. Mechanisms include functional asplenism, reduced tissue perfusion during a sickle crisis, and chronic transfusions with an indwelling catheter. Common infections include bacteremia, meningitis, and pneumonia due to Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitis. Sickle cell trait is not clearly associated with an increased risk of infection and actually confers some protection against malaria. Answer D: Splenectomy from auto-infarction is often seen in sickle cell anemia, but it is rare with sickle cell trait. In sickle cell anemia, splenic sequestration of sickled red blood cells causes infarction, leading to progressive atrophy and functional hyposplenism. Most individuals with sickle cell disease are functionally asplenic by age 2 to 4. The spleen subsequently cannot phagocytose encapsulated organisms properly, which predisposes sickle cell patients to infection. Answer E: Stroke is a complication of sickle cell anemia but not sickle cell trait. This is due to the increased viscosity of the blood and reduced deformability of red cells in patients with sickle cell anemia. Bullet Summary: Sickle cell trait is associated with episodes of painless hematuria.
Stroke
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A 15-month-old boy presents with his mother for his well-child exam. His mother is concerned about his vision, as she noticed him squinting while watching television. The patient was born at 39 weeks of gestation via spontaneous vaginal delivery. He is up to date with routine vaccinations. He is able to walk alone, speak 3 words, and scribble with a crayon. His temperature is 98.6°F (37.0°C), blood pressure is 80/55 mmHg, pulse is 105/min, and respirations are 25/min. On exam, his pupils are round and reactive to light. A photograph of his eyes is shown in Figure A. When focusing on the pediatrician's penlight, the patient's left eye wanders. However, when the right eye is covered, the left eye refocuses on the light. The rest of his neurological exam, including extraocular movements, is within normal limits. Which of the following is the most appropriate next step in management for this patient?
Perform a Bielschowsky head tilt test
Brain MRI
Patch the left eye
Patch the right eye
D
Patch the right eye
This patient, with a history that suggests decreased visual acuity, a positive cover test (refixation of the misaligned eye upon covering the appropriately aligned eye), and an asymmetric corneal light reflex in Figure A, is presenting with strabismus. Of the answer choices, only patching the sound eye (right eye) is an appropriate treatment. Strabismus occurs when a patient's eyes are not aligned properly. It often presents in children with one or both eyes turning inward, outward, upward, or downward. Patients will often tilt their heads or squint their eyes to correct this misalignment. Without proper treatment, there is a significant risk of developing amblyopia. Amblyopia is a developmental disorder in the visual cortex caused by abnormal visual stimuli. Untreated strabismus is the most common cause of amblyopia; it is secondary to visual loss from the deviated eye. The treatment of choice is wearing an eye patch over the appropriately tracking eye (sound eye), thereby encouraging the use of the deviated eye and preventing vision loss in this eye. Other treatments include surgical correction of strabismus, pharmacological penalization of the sound eye with atropine, and dichoptic movies and video games. Gopal et al. discuss the pathophysiology, prevention, and therapy for amblyopia as it relates to strabismus. The authors find that the earlier clinically refractive error and strabismus are detected and treated, the greater the likelihood of preventing amblyopia. The authors recommend treating amblyopia with the correction of refractive errors, patching, and pharmacologic treatments. Figure/Illustration A shows displacement of the light reflex from the center of the pupil in this patient's right eye, as indicated by the black arrow. This is known as an asymmetric corneal light reflex and is seen in strabismus. Incorrect Answers: Answer A: Brain MRI is part of the initial workup for retinoblastoma. Retinoblastoma typically presents in early childhood with visual loss, leukocoria, and an abnormal red reflex, which this patient does not have. Answer B: Head CT in a pediatric patient could be used to rule out hemorrhage following blunt head trauma. This patient has not experienced head trauma and has no symptoms of traumatic brain injury (abnormal neurologic exam, loss of consciousness seizures). CT scans are not preferred in pediatric patients to avoid radiation exposure. Answer C: Patching the left eye (the inappropriately tracking eye) would not treat this patient's strabismus and would likely increase his risk for amblyopia due to further decreasing his visual stimuli in this eye. Answer E: The Bielschowsky head tilt test is used to help diagnose trochlear nerve (CN IV) palsy. Patients with a unilateral trochlear nerve and subsequent superior oblique palsy experience double vision when tilting their head to one side, which is improved by tilting the head towards the shoulder on the unaffected side. A trochlear nerve palsy typically presents with difficulty looking down and a head tilt away from the side of the lesion, unlike this patient who appears to have a visual acuity deficit. Bullet Summary: Patching the appropriately aligned eye is the treatment of choice for strabismus to prevent progression to amblyopia.
nan
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A 37-year-old woman presents to her physician for mood changes and recent falls over the course of 3 months. She feels more irritable with her husband and children and does not find pleasure in creating art anymore. Over the last 2 weeks, she has been sleeping approximately 5 hours a night and had a fall while walking in her home with the lights off. She denies any head trauma or loss of consciousness associated with the fall. She has a medical history of generalized anxiety disorder and hypothyroidism for which she takes escitalopram and levothyroxine. She had a Roux-en-Y gastric bypass 5 years ago. She has been in a monogamous relationship with her husband for the last 15 years and uses a copper intrauterine device. Physical examination is notable for a depressed affect. Pupils are equal, round, and reactive to light and accommodation. She has 4/5 strength in the bilateral lower extremities and brisk patellar reflexes. Which of the following is most likely the cause of this patient's symptoms?
Poor iron absorption
Prior Treponema pallidum exposure
Reduced central nervous system dopamine and serotonin
Reduced functional parietal cell count
D
Reduced functional parietal cell count
This patient's mood changes and findings concerning for subacute combined degeneration (fall in the dark, impaired proprioception, weakness, and brisk patellar reflexes), and history of Roux-en-Y gastric bypass surgery are concerning for vitamin B12 deficiency. Roux-en-y gastric bypass reduces parietal cell count, leading to reduced intrinsic factor secretion, which is needed for vitamin B12 absorption. Roux-en-Y gastric bypass is a laparoscopic bariatric procedure that creates a small gastric pouch from the stomach with a gastrojejunal and jejunojejunal anastomosis. This causes small volumes of food to be consumed and decreases nutrient absorption. The small gastric pouch contains very few parietal cells, which decreases hydrochloric acid and intrinsic factor secretion. The reduced intrinsic factor secretion impairs the protection of vitamin B12 against HCl and reduces absorption in the ileum. Vitamin B12 deficiency can lead to neuropsychiatric changes (depressed mood, irritability, and insomnia) and subacute combined degeneration (weakness, sensory ataxia, peripheral neuropathy, and upper motor neuron signs). Patients who undergo Roux-en-Y gastric bypass must be given high doses of vitamin B12 (often intramuscularly) in order to prevent deficiency. Alexandrou et al. review long-term micronutrient deficiencies after sleeve gastrectomy and Roux-en-Y gastric bypass (RYGB). The authors find that vitamin B12 deficiency was significantly higher in patients with RYGB compared with sleeve gastrectomy. The authors recommend adherence to supplemental iron and vitamin intake in all patients who undergo bariatric surgery. Incorrect Answers: Answer A: Poor iron absorption is a well-known complication of Roux-en-Y gastric bypass surgery due to bypass of the duodenum and proximal jejunum. Poor iron absorption leads to microcytic anemia from iron deficiency and presents with generalized fatigue and conjunctival pallor. Iron deficiency anemia would not present with brisk reflexes and impaired proprioception. Answer B: Prior Treponema pallidum exposure can result in tabes dorsalis, a neurological complication of syphilis, and is seen in patients with a history of multiple sexual partners who do not use barrier protection. The spirochete leads to disease in the posterior columns (vibration and proprioception sensation). Patients present with sensory ataxia (similar to subacute combined degeneration), lancinating pain (in the limbs, back, or face), and an Argyll-Robertson pupil (impaired pupillary constriction to light but intact pupillary constriction to accommodation). Answer C: Reduced central nervous system dopamine and serotonin are seen in patients with major depressive disorder which does not cause sensory ataxia with brisk reflexes. Answer E: Subtherapeutic levothyroxine dose would result in hypothyroidism, which can present with depressed mood, cold intolerance, bradycardia, and delayed relaxation of deep tendon reflexes. Roux-en-Y is an effective means of losing weight, and as the body mass decreases with weight loss, the required dose of levothyroxine is decreased. It would not present with the abnormal neurologic findings seen in this patient. Bullet Summary: Roux-en-Y bariatric surgery increases the risk of vitamin B12 deficiency via reduction of food intake, decreased functional parietal cell count, and reduced absorption of vitamin B12 (which may cause subacute combined degeneration).
Subtherapeutic levothyroxine dose
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A 55-year-old male bodybuilder presents to the emergency department with weakness of his right arm. The patient states he has experienced these symptoms for a few weeks; however, today his hand felt so weak he dropped his cup of tea. The patient has a medical history of diabetes. He drinks 2-7 alcoholic drinks per day and has smoked 2 packs of cigarettes per day since he was 25. The patient uses anabolic steroids. He has lost 17 pounds since he last came to the emergency department 1 month ago. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals decreased sensation in the right arm and 2/5 strength in the right arm and 5/5 strength in in the left arm. The patient states that he is experiencing a dull aching and burning pain in his right arm during the exam. Symptoms are not changed with changes in head and neck position. Which of the following is the most likely diagnosis?
Apical lung tumor
Brachial plexopathy
Cerebral infarction
Scalenus anticus syndrome
A
Apical lung tumor
This patient is presenting with unilateral upper extremity paresthesia and weakness suggestive of compression of the neurovascular structures of the upper extremity secondary to an apical lung tumor. An apical lung tumor can compress the neurovascular structures in its proximity and cause pain, numbness, and weakness of the ipsilateral arm. A Horner syndrome can also be observed with compression of the sympathetic chain leading to ipsilateral miosis, ptosis, and anhidrosis in some cases. These malignancies typically occur in older male smokers and can be associated with symptoms such as fatigue, cough, and weight loss. Chest radiography will demonstrate an apical opacity and CT scan can confirm the size and location of the mass. Treatment is with chemotherapy and surgical excision of the tumor. Davis and Knight review the evidence regarding the diagnosis and treatment of patients with apical lung tumors. They discuss how these tumors can be associated with brachial plexus injuries. They recommend surgical excision of these tumors if they are resectable. Incorrect Answers: Answer B: Brachial plexopathy could present with weakness and numbness of the ipsilateral arm but is a less likely diagnosis (and a diagnosis of exclusion). Treatment is with identification and surgical release of any underlying anatomic defects (such as compressive bands) and supportive therapy if none are found. Answer C: Cerebral infarction (stroke) would present with sudden onset of acute neurological deficits. These would typically only affect one side of the body and would not necessarily be associated with a dull pain or numbness that is chronic. Treatment is with thrombolytics if patients meet criteria for acute therapy. Answer D: Scalenus anticus syndrome occurs in bodybuilders or athletes with hypertrophied neck muscles that compress the brachial plexus. Symptoms are typically worsened by certain positions. Treatment is with physical therapy and surgical decompression in refractory cases. Answer E: Subclavian steal syndrome presents with numbness, tingling, weakness in a limb, and CNS symptoms (vertigo, slurred speech, or stroke-like symptoms) that are worsened with activity and relieved with rest. Treatment is with vascular intervention to restore blood flow. Bullet Summary: Apical lung tumors can compress neurovascular structures in the upper extremity and cause pain, numbness, and tingling in the ipsilateral arm.
Subclavian steal syndrome
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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?
Switch to paroxetine
Switch to phenelzine
Electroconvulsive therapy
Add mirtazapine
D
Add mirtazapine
This patient is being treated for depression without improvement in his symptoms after 6 weeks of medication use. In the setting of his reduced appetite and trouble sleeping, mirtazapine is an appropriate medication to add. SSRIs (including paroxetine, citalopram, fluvoxamine, sertraline, and fluoxetine) are the first line treatment for major depression. For patients in whom an adequate trial of SSRI therapy has been ineffective, the first step in management is a trial of augmentation with a separate agent prior to switching to a different primary antidepressant medication. Mirtazapine, a noradrenergic and specific serotonergic antidepressant, acts by inhibiting pre-synaptic alpha-2 receptors as well as 5HT2 and 5HT3 serotonin receptors. Notably, its side effect profile includes increased appetite and mild sleepiness, making it a good option for depressed patients with insomnia or decreased appetite. Mirtazapine is commonly used to augment primary antidepressant therapy. Augmentation with an additional agent should generally be attempted prior to switching to a different SSRI or other first line medication. Davies et. al review the pharmacologic management of depression. They note that while evidence is limited, there is some data to suggest benefit in augmentation with a secondary agent prior to switching to another first line therapy. Incorrect Answers: Answer B: Electroconvulsive therapy, while often extremely effective, is typically reserved for patients that are actively suicidal, unable to eat, or displaying psychotic symptoms. It is more commonly used after medications have failed. Answer C: Switching to amitriptyline, a tricyclic antidepressant, would not be appropriate. Tricyclic antidepressants have a negative side effect profile and potential for severe toxicity. Answer D: Switching to paroxetine, another SSRI medication, may be appropriate. However, this patient's particular constellation of symptoms (decreased appetite and insomnia) suggest that he would likely respond well to mirtazapine. Answer E: Switching to phenelzine, a monoamine oxidase inhibitor, would not be appropriate. Monoamine oxidase inhibitors have a negative side effect profile and potential for severe drug-drug interaction. Bullet Summary: Mirtazapine (a noradrenergic and serotonergic antidepressant) is an appropriate next step for depressed patients with significant insomnia and decreased appetite who fail to respond to a trial of SSRI monotherapy.
nan
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A 35-year-old man presents to his primary care physician with pain along the bottom of his foot. The patient is a long-time runner but states that the pain has been getting worse recently. When running and at rest he has a burning and aching pain along the bottom of his foot that sometimes turns to numbness. Taking time off from training does not improve his symptoms. The patient has a medical history of surgical repair of his Achilles tendon, ACL, and medial meniscus. He is currently not taking any medications. The patient lives with his wife and they both practice a vegan lifestyle. His temperature is 98.6°F (37°C), blood pressure is 114/72 mmHg, pulse is 81/min, and respirations are 12/min. On physical exam, the patient states that he is currently not experiencing any pain in his foot but rather is experiencing numbness/tingling along the plantar surface of his foot. Strength is 5/5 and reflexes are 2+ in the lower extremities. Which of the following is the most likely diagnosis?
Plantar fasciitis
Vitamin B12 deficiency
Tarsal tunnel syndrome
Herniated disc
C
Tarsal tunnel syndrome
This patient is presenting with pain, tingling, and numbness on the plantar surface of his foot that is not associated with exertion or rest suggesting a diagnosis of tarsal tunnel syndrome. Tarsal tunnel syndrome occurs due to compression of the tibial nerve as it travels through the tarsal tunnel. Compression of the nerve leads to symptoms along the plantar surface of the foot which can include pain, tingling, or numbness. These symptoms can be exacerbated/triggered by tapping on the tunnel, which lies posterior to the medial malleolus. In addition, the symptoms can occur at rest or during activity and are not improved with rest. Treatment is rest, NSAIDs, properly fitted shoes/orthotics, and in refractory cases, surgical release of the tarsal tunnel. Gould reviews the evidence regarding the diagnosis and treatment of tarsal tunnel syndrome. He discusses how this syndrome represents a collection of different entities including space-filling lesions and local inflammation. He recommends definitive diagnosis prior to surgical treatment given that the syndrome is often misdiagnosed. Incorrect Answers: Answer A: Common fibular nerve compression typically occurs when there is trauma/compression just inferior to the head of the fibula. This can occur when an individual wears work boots and after fractures of the fibular neck. Symptoms of common fibular nerve compression include foot drop and numbness on the dorsum of the foot. Treatment is with activity modification and a foot orthosis to prevent an equinus contracture. Answer B: Herniated disc presents with symptoms of radiculopathy including pain that travels down the leg through the toes. Symptoms are worsened with flexion of the leg such as during the straight leg raise test. Treatment is with NSAIDs and corticosteroid injections with surgical decompression of the disc in refractory cases. Answer C: Plantar fasciitis can present very similarly to tarsal tunnel syndrome with pain along the plantar surface of the foot. The pain is typically exacerbated by activity and improves with rest in contrast to this patient. In addition, there is an absence of burning, pain, and numbness that occur in tarsal tunnel syndrome. Tenderness will be at the medial insertion of the plantar fascia into the calcaneal tuberosity. Treatment is conservative with NSAIDs and activity modification. Answer E: Vitamin B12 deficiency could present with symptoms of peripheral neuropathy. This is possible given this patient's vegan diet. This patient has pain only in one of his feet and he does not have symptoms of anemia such as fatigue or pallor. Treatment is with the repletion of vitamin B12. Bullet Summary: Tarsal tunnel syndrome presents with pain, numbness, tingling, and aching along the plantar surface of the foot that is not associated with activity.
nan
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A 33-year-old man presents to the emergency department after slamming his finger in the car door 5 hours ago. He initially experienced pain which is currently well controlled with acetaminophen. The patient is otherwise healthy and does not take any medications. His temperature is 98.5°F (36.9°C), blood pressure is 123/79 mmHg, pulse is 90/min, respirations are 11/min, and oxygen saturation is 97% on room air. Physical exam is notable for the finding in Figure A. A radiograph of the affected digits shows a smooth cortex without disruption of the bone. Which of the following is the most appropriate management for this patient?
Excision and histological examination
Fingernail removal
Observation
Operative repair
E
Trephination
This patient is presenting after trauma to his finger with a subungual hematoma. Given that he has no other associated injuries, trephination is the only management indicated. A subungual hematoma occurs after trauma to the finger leading to a collection of blood under the fingernail. Depending on the mechanism, this can be associated with a distal phalanx fracture. Evaluation for a fracture should include a thorough history, exam, and radiography. If there are no other signs of injury including nailbed dislocation or an open fracture, then management requires only trephination (putting a hole in the nail to allow blood to drain) as this alleviates pressure on the nailbed matrix thus preventing possible irreversible damage. Rsoer et al. compare nail bed repair to nail trephination for subungual hematomas in children. The authors find that there was no difference in complications or outcomes between nail bed repair and trephination; however, the cost in the nail trephination group was lower. The authors recommend nail trephination for children with a subungual hematoma and an intact nail and nail margin. Figure/Illustration A shows the physical exam finding of a subungual hematoma with a collection of blood underneath the fingernail bed (green circle). Incorrect Answers: Answer A: Excision and histological examination could be appropriate management of melanoma, which may present under the fingernails with a hyperpigmented, irregular skin lesion, or a “barcode” appearance if it is at the base of the nail. A spontaneous subungual hematoma could be suggestive of melanoma; however, in the setting of trauma, it is a much less likely diagnosis. Answer B: Fingernail removal should only be performed if there is also nail avulsion or nail fold disruption to allow for inspection of the nail bed and repair of any laceration. Failure to repair a nailbed laceration could lead to permanent deformity of the fingernail when it regrows. Answer C: Observation is inappropriate as the accumulation of blood under the nail in a subungual hematoma could lead to ischemia and permanent injury to the nailbed. When a subungual hematoma is present, trephination is indicated. Answer D: Operative repair may be necessary for fractures; however, this patient has no fracture and only has a subungual hematoma which can be drained easily. Bullet Summary: A subungual hematoma is common after trauma, presents with a collection of blood under the fingernail, and should be treated with trephination.
Trephination
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A 56-year-old man presents from prison with 1 month of subjective fevers, chills, cough, and night sweats. He noticed a gradual weight loss over the past year despite no changes to his diet or physical activity. He has never smoked cigarettes but does have a history of opiate use prior to incarceration. He does not take any medications. His temperature is 100.8°F (38.2°C), blood pressure is 142/88 mmHg, pulse is 78/min, and respirations are 12/min. Physical exam reveals a thin, ill-appearing man. Pulmonary auscultation reveals faint, inspiratory crackles in the right upper lobe. His chest radiograph is shown in Figure A. Which of the following is the most appropriate next step in management?
Ampicillin-sulbactam
Biopsy
Itraconazole
Rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy
D
Rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy
This patient likely has active tuberculosis (TB), as evidenced by his symptoms (fever, night sweats, cough, and weight loss), inmate status, and apical cavitary lesion on chest radiograph. He should be treated with RIPE therapy. An active TB infection should be suspected in patients with a febrile illness, cough, night sweats, and weight loss along with epidemiological risk factors for TB infection. Risk factors include residence in or previous visits to endemic regions, work in hospitals or nursing homes, imprisonment, and immunosuppression. Treatment typically requires 2 phases: an intensive phase involving 2 months of rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy and a continuation phase involving 4 additional months of just isoniazid and rifampin. Vitamin B6 (pyridoxine) should be taken with isoniazid to prevent peripheral neuropathy. Rifampin, isoniazid, and pyrazinamide use should be monitored for hepatotoxicity. Ethambutol can cause optic neuropathy. Conradie et al. discuss the use of a bedaquiline-pretomanid-linezolid (BPal) regimen in the treatment of extensively drug-resistant tuberculosis. The authors find that most patients treated with BPal have a favorable outcome (no treatment failure or disease relapse) after 6 months. The authors recommend the use of this regimen in highly drug resistant TB. Figure/Illustration A demonstrates a right upper lobe cavitating lesion (arrow) that resulted in extensive necrosis and fibrosis. These lesions are usually localized to the upper regions of the lung due to favorable oxygen concentrations in the apex. Incorrect Answers: Answer A: Ampicillin-sulbactam can be used as empiric treatment for a lung abscess to cover anaerobic pathogens and facultative anaerobic streptococci. A lung abscess is associated with aspiration risk factors (such as alcohol use) or poor dentition. Answer B: Biopsy may be indicated for a solitary cavitary nodule that is suggestive of malignancy. In this case, the patient's inmate status, along with his fever, night sweats, and cough are more suggestive of an infectious rather than malignant etiology. Answer C: Itraconazole can be used to treat chronic pulmonary aspergillosis. These patients typically have prior or current lung disease such as chronic obstructive pulmonary disease or tuberculosis. The classic air crescent sign may be seen on imaging in a patient with an aspergilloma, a form of pulmonary aspergillosis in which a fungal ball develops within an existing cavitary lesion. Answer D: Vancomycin can be used to treat a lung abscess due to methicillin-resistant Staphylococcus aureus. S. aureus pneumonia classically presents as a secondary infection that follows an influenza infection. Bullet Summary: Active tuberculosis infection should be treated with rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy.
Vancomycin
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A 35-year-old G0P0000 woman presents to her gynecologist with complaints of irregular menstruation. She has had only 2 periods in the last year. She feels flushed without provocation and is experiencing occasional dyspareunia with post-coital spotting. She has also had more frequent headaches than usual. The patient has a medical history of Hashimoto thyroiditis and takes levothyroxine daily. Her mother has type 1 diabetes mellitus. Her temperature is 98.5°F (36.9°C), pulse is 70/min, blood pressure is 118/76 mmHg, and respirations are 13/min. Cardiopulmonary and abdominal exams are unremarkable. The patient has Tanner 5 breasts and pubic hair. A pelvic exam reveals a normal cervix, an anteverted uterus without tenderness, and no adnexal masses. The following laboratory studies are performed: Serum: Thyroid stimulating hormone (TSH): 28 µIU/mL (9-30 µIU/mL) Cycle day 3 follicle stimulating hormone (FSH): 49 mIU/mL (4.7-21.5 mIU/mL) Cycle day 3 estradiol: 8 pg/mL (27-123 pg/mL) Prolactin: 14 ng/mL (4-23 ng/mL) Testosterone: 42 ng/dL (15-70 ng/dL) Which of the following is the most appropriate next step in management?
Combined oral contraceptive
Increase levothyroxine dose
Brain MRI
Estradiol patch with oral medroxyprogesterone
D
Estradiol patch with oral medroxyprogesterone
This patient presents with oligomenorrhea, hot flashes, and dyspareunia, and her labs are notable for a high FSH and low estradiol, most consistent with premature ovarian insufficiency/failure (POI). The most appropriate next step in management is an estradiol patch with oral medroxyprogesterone. POI is defined by the depletion or dysfunction of ovarian follicles, resulting in oligomenorrhea or amenorrhea with symptoms of menopause (hot flashes, vaginal atrophy and dryness, mood swings, etc.) in women under age 40. The cause is unclear and thought to be autoimmune. The main diagnostic markers are a high FSH and low estradiol at the start of the follicular phase. Patients require estrogen supplementation until around age 50, as low estrogen increases the risk of coronary artery disease, osteoporosis, and sexual dysfunction. Vaginal gel or a transdermal patch is the first-line option, but both must be used in conjunction with medroxyprogesterone to avoid unopposed estrogen and the risk of endometrial hyperplasia. Tsiligiannis et al. review the evidence regarding the outcomes of patients with POI. They discuss how this diagnosis is made in patients younger than the age of 40. They recommend closely managing the cardiovascular risk factors associated with this disease. Incorrect Answers: Answer A: Brain MRI would be useful for evaluating for the presence of a prolactinoma, which could cause the headaches and oligomenorrhea seen in this patient. However, her prolactin level is normal, and she has no visual disturbances (e.g., bitemporal hemianopsia), making this study less relevant. Answer B: The combined oral contraceptive has both estrogen and progesterone, but the doses are higher than those needed for hormone replacement. For this reason, it is generally not considered first-line for the treatment of POI. Answer D: Increasing this patient’s levothyroxine dose is unnecessary, as her TSH level is within the normal range. Furthermore, while hypothyroidism may cause menstrual irregularities and temperature dysregulation, it is unlikely to cause vaginal dryness and subsequent dyspareunia. Answer E: Vaginal estradiol gel is an effective method of estrogen replacement in women with POI. However, used alone it may increase the risk of endometrial hyperplasia and cancer, so it should only be used in combination with a systemic progesterone. Bullet Summary: Premature ovarian insufficiency should be treated with estrogen and progesterone replacement.
nan
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A 23-year-old woman presents to the emergency department complaining of a worsening headache. The headache started 1 month ago. It is constant and “all over” but gets worse when she is lying down or in the setting of bright lights. A review of systems is significant for a low-grade fever, night sweats, cough, malaise, poor appetite, and unintentional weight loss of 12 pounds in the last 2 months. The patient is sexually active with multiple male partners and reports inconsistent condom use. She has a history of intravenous drug use and has not been to a doctor in the last 2 years. Her temperature is 100.4°F (38.0°C), blood pressure is 110/78 mmHg, pulse is 88/min, and respirations are 14/min with an oxygen saturation of 98% O2 on room air. On physical exam, pain is elicited upon passive flexion of the patient’s neck. A CT scan shows ventricular enlargement. A CD4+ count is 57 cells/µL blood. A lumbar puncture is performed with the following findings: Cerebrospinal fluid: Opening pressure: 210 mmH2O Glucose: 32 mg/dL Protein: 204 mg/dL India ink stain: Positive Leukocyte count and differential: Leukocyte count: 200/mm^3 Lymphocytes: 100% Red blood cell count: 2/mm^3 Which of the following treatments most directly addresses the underlying cause of this patient's symptoms?
Acyclovir
Amphotericin B and 5-flucytosine
Dexamethasone
Fluconazole
B
Amphotericin B and 5-flucytosine
The patient with a history of unprotected sex and intravenous drug use who presents with sub-acute fever, meningismus, photophobia, a low CD4+ count, and a positive India ink-stained lumbar puncture most likely has cryptococcal meningitis. Acute treatment for cryptococcal meningitis is intrathecal amphotericin B and 5-flucytosine. Cryptococcal meningitis is the most common fungal meningeal infection in patients with HIV, especially those with a CD4+ count < 100/mm^3. Symptoms are normally gradual in onset and can include headache, fever, neck stiffness, and photophobia. The most appropriate initial test is a lumbar puncture with an India ink stain. Cerebrospinal fluid in fungal meningitis would have low glucose, elevated protein, and an elevated lymphocyte-dominant leukocyte count. The most accurate diagnostic test is latex particle agglutination. Intrathecal amphotericin B and 5-flucytosine are the first-line treatments for acute management. Once the patient is treated, fluconazole is given for life or until the patient’s CD4+ count is > 100/mm^3. Badali et al. present a case of a patient with cryptococcal meningitis. They discuss how despite immediate antifungal therapy, the patient died due to a rapidly progressive infection. They recommend aggressive intrathecal therapy to treat this disease. Incorrect Answers: Answer A: Acyclovir is used to treat Herpes simplex virus encephalitis. Cerebrospinal fluid would show normal protein and glucose, an elevated lymphocyte-dominant leukocyte count, and an elevated red blood cell count. The Herpes virus can lead to a hemorrhage of the temporal lobes. Answer C: Dexamethasone is given in addition to IV antibiotics if Streptococcus pneumoniae meningitis is suspected. This is the most common cause of meningitis in adults, elderly, and asplenic patients, and can spread to cause sinusitis, otitis media, or bacteremia. Answer D: Fluconazole is used for prophylaxis against cryptococcal meningitis after a person is treated acutely. It is either given for life, or until the patient’s CD4+ count is > 100/mm^3. This treatment is not sufficient for patients with acute meningitis with neurological changes. Answer E: Vancomycin and ceftriaxone are given to a patient suspected of having bacterial meningitis until culture results confirm the bacterial organism. Bacterial meningitis would have decreased glucose, elevated protein, and an elevated neutrophil-dominant leukocyte count. Bullet Summary: Cryptococcal meningitis classically presents in a patient with a CD4+ count < 100/mm^3 and should be treated acutely with intrathecal amphotericin B and 5-flucytosine.
Vancomycin and ceftriaxone
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A 55-year-old man presents to the emergency department with worsening dyspnea over the past 48 hours. He recently had a cold that kept him home from work for the past week. He has a past medical history of diabetes, obesity, and hypertension. He had his Achilles tendon repaired 4 weeks ago and he has been less mobile. His temperature is 99.2°F (37.3°C), blood pressure is 150/85 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 100% on room air. Physical exam is notable for dyspnea provoked by walking short distances. Jugular venous distension is noted on exam. The patient’s blood pressure is 130/70 mmHg during inspiration. A bedside echocardiogram demonstrates impaired diastolic filling with normal ventricular contractility. An ECG is performed as seen in Figure A. Which of the following is the most likely diagnosis?
Cardiac tamponade
Constrictive pericarditis
Myocardial infarction
Pulmonary embolism
B
Constrictive pericarditis
This patient is presenting with a preceding viral illness, dyspnea, jugular venous distension, pulsus paradoxus (blood pressure drop from 150/85 to 130/70 mmHg during inspiration), an echocardiogram with impaired diastolic filling, and diffuse ST elevation with PR depression on ECG. This constellation of symptoms is concerning for constrictive pericarditis. Constrictive pericarditis occurs when the pericardium becomes thickened and fibrotic. In contrast to other types of pericarditis, in constrictive pericarditis, there is impaired diastolic filling and thus impaired cardiac function. There are many possible etiologies of constrictive pericarditis including infection, inflammatory conditions, or idiopathic. Patients will present with dyspnea, hypotension, tachycardia, a pericardial friction rub, and positional chest pain (usually worsened by laying back and relieved by leaning forward). Patients may also demonstrate jugular venous distension, lower extremity edema, worsening dyspnea, and congestive hepatopathy. ECG may demonstrate diffuse ST elevation with PR depression, and an echocardiogram will demonstrate impaired diastolic filling and may demonstrate a thickened pericardium. The diagnosis can be supported with pulsus paradoxus (a drop of blood pressure by > 10 mmHg during inspiration). Welch reviews constrictive pericarditis. He discusses constrictive pericarditis and how the inelastic pericardium inhibits cardiac filling possibly causing unexplained heart failure. Welch notes making the diagnosis is very challenging and requires meticulous echocardiograph assessment. Figure/Illustration A demonstrates diffuse ST elevation (blue arrows) with PR depression (black arrows). This is consistent with pericarditis. Incorrect Answers: Answer A: Cardiac tamponade can occur after trauma, in the setting of infection, or in the setting of autoimmune disease and is more likely to occur with a rapid accumulation of pericardial fluid. While pulsus paradoxus would be present in this condition, muffled heart sounds and electrical alternans would be more likely to be seen on ECG. Echocardiography would demonstrate an echogenic rim of fluid around the heart. Answer C: Myocardial infarction presents with chest pain, dyspnea, hypotension, tachycardia, and ST elevation in a vascular distribution. Note that this patient’s ST elevation is diffuse and there is no reciprocal ST depression. Moreover, the pattern of chest pain in myocardial infarction is typically exertional and not positional. Answer D: Pulmonary embolism may present with pleuritic chest pain, shortness of breath, tachycardia, hypoxia, hemoptysis, a history of hypercoagulability, and recent stasis or surgery (such as this patient’s recent Achilles tendon surgery). It can cause obstructive shock leading to hypotension and tachycardia. Note that right heart strain and sinus tachycardia would be seen on ECG. Answer E: Restrictive cardiomyopathy presents very similarly to restrictive pericarditis. Note that in restrictive cardiomyopathy, patients will also have impaired diastolic filling; however, it is due to a myocardium that will not expand rather than the pericardium impairing filling. It can occur secondary to conditions such as amyloidosis or hemochromatosis as well as other chronic inflammatory states. The ECG findings are nonspecific but may include low voltage QRS complexes, ST segment changes, T wave changes, bundle branch blocks, and pathologic Q waves (abnormally deep and wide Q waves). The diagnosis can be supported with an ultrasound demonstrating impaired diastolic filling and possible myocardial speckling if a protein (amyloid) is present and deposited in the myocardial wall. The diagnosis can only definitively be confirmed with a biopsy which is highly invasive. Bullet Summary: Restrictive pericarditis presents with chest pain that is positional, pulsus paradoxus, a friction rub, and diffuse ST elevation with PR depression on ECG.
Restrictive cardiomyopathy
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A 26-year-old woman presents to the clinic with diarrhea, bloating, flatulence, and abdominal cramps. These symptoms have been going on for 2 months. She has lost 6 pounds (2.7 kg) over these 2 months. Her medical history is significant for mild intermittent asthma for which she uses an albuterol inhaler as needed. She is sexually active with 1 male partner. She recently returned from South India following a mission trip for 6 weeks. Her temperature is 98.6°F (37.0°C), blood pressure is 105/70 mmHg, pulse is 95/min, and respirations are 14/min. On examination, the patient’s skin is pale. Labs are obtained and show the following results: Leukocyte count: 4,500/mm^3 Hemoglobin: 10.5 g/dL Platelets: 110,000/mm^3 Mean corpuscular volume (MCV): 116 µm^3 Reticulocyte count: 0.5% A biopsy of the small bowel reveals blunting of villi and a mixed infiltration of lymphocytes, plasma cells, and eosinophils. Which of the following is the most likely diagnosis?
Celiac disease
Crohn disease
Giardia
Tropical sprue
D
Tropical sprue
The patient is presenting with chronic diarrhea, weight loss, megaloblastic anemia (low hemoglobin and elevated MCV), and a biopsy showing blunted villi and mixed infiltrate following significant travel to South India. This is consistent with tropical sprue. Tropical sprue is characterized by chronic diarrhea that occurs after travel to an endemic area. It is thought to be due to a bacterial overgrowth/infection that then damages the intestinal mucosa. Endemic areas include the Caribbean, Southeast Asia, and South India. Patients present with chronic diarrhea, abdominal discomfort/cramps, progressive weight loss, and clinical features of malabsorption. Patients with tropical sprue can develop megaloblastic anemia (low hemoglobin and elevated MCV) from a deficiency in folate and vitamin B12 absorption. A small bowel biopsy would show blunting of the villi and infiltration of lymphocytes, plasma cells, and eosinophils. Microorganisms may also be seen. Treatment is an oral tetracycline and folic acid for 3-6 months. McCarroll et al. study the relationship between infectious gastroenteritis and tropical sprue. In a case-control study, the authors found that prior infectious gastroenteritis increased the risk of tropical sprue. The authors recommend treatment of suspected tropical sprue with oral folic acid replacement. Incorrect Answers: Answer A: Celiac disease can present similarly to tropical sprue with chronic diarrhea, abdominal pain, and weight loss. A small intestine biopsy can similarly show villous atrophy and a lymphocytic infiltrate, as well as crypt hyperplasia. Celiac disease is an immune response to gluten, while symptoms of tropical sprue are not diet-associated. The development of symptoms following significant travel (lasting at least several weeks) to an endemic country should raise suspicion for tropical sprue. Answer B: Crohn disease presents with chronic diarrhea, abdominal pain, weight loss, skip lesions anywhere from mouth to anus, extraintestinal manifestations, and signs of malabsorption. A biopsy will show transmural inflammation with noncaseating granulomas, giant cells, and aggregates of lymphocytes in the lamina propria. Answer C: Giardia is a parasite that is endemic in the tropics and North American mountain regions. Patients present with foul-smelling diarrhea, excessive gas, and nausea. Diagnosis can be confirmed with a stool analysis or immunoassay. Though a biopsy is normally not indicated, it would show mild lymphocytic infiltration and Giardia trophozoites on the intestinal surface. Answer E: Whipple disease is caused by the bacteria Tropheryma whipplei. Patients present with diarrhea, abdominal pain, and signs of malabsorption. Extraintestinal symptoms such as arthritis are common. A biopsy of the small intestine would show Periodic acid-Schiff (PAS)-positive foamy macrophages in the lamina propria. Bullet Summary: Tropical sprue presents with diarrhea, abdominal cramping, weight loss, megaloblastic anemia due to malabsorption, and a small bowel biopsy showing villi blunting following travel to an endemic area such as South Asia or the Caribbean.
Whipple disease
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A 55-year-old man presents to the emergency department with chest pain and shortness of breath. He has a medical history of hypertension, diabetes, and obesity. His temperature is 98.6°F (37.0°C), blood pressure is 177/118 mmHg, pulse is 127/min, respirations are 11/min, and oxygen saturation is 98% on room air. An ECG is performed and notable for ST elevation in leads II, III, and aVF. The patient is treated appropriately and transferred to the medical floor. On the 2nd day of his hospitalization, the patient has abdominal pain. His serum lipase is 272 U/L and his creatinine is 1.6 mg/dL. A physical exam is notable for the finding in Figure A. He is requesting pain medication for his abdominal pain. Which of the following is the most likely underlying diagnosis?
Cholesterol embolism
Heart failure and reinfarction
Medication side effect
Pancreatitis
A
Cholesterol embolism
This patient is presenting with abdominal pain and livedo reticularis after a catheterization procedure. These symptoms are suggestive of a cholesterol embolism. A cholesterol embolism typically presents after any vascular procedure due to a dislodged cholesterol emboli. Symptoms include livedo reticularis, gastrointestinal problems such as mesenteric ischemia or pancreatitis, and acute kidney injury. Patients who have undergone cardiac catheterization should be monitored for the development of this complication. Treatment is centered around treating the current complications, often using supportive therapy as they self-resolve. In addition, patients should be treated carefully to prevent recurrent embolism. Maningding and Kermani review the evidence regarding the presentation of vasculitis mimics. They discuss how cholesterol embolism can cause diverse systemic symptoms and livedo reticularis. They recommend carefully considering the history of each patient. Figure/Illustration A is a clinical photograph demonstrating a netlike reticular bluish discoloration of the skin (red circle). These findings are consistent with livedo reticularis. Incorrect Answers: Answer B: Heart failure and reinfarction could present with renal failure but would likely present with other symptoms of heart failure as well (pulmonary crackles, edema, and jugular venous distension). Treatment of a repeat infarct involves catheterization and fluid overload involves volume optimization. Answer C: Medication side effects could explain this patient's livedo reticularis, but the drugs that may cause this (certain antivirals and amantadine) would not have been given to this patient. These medications also typically do not cause abdominal pain. Answer D: Pancreatitis presents with abdominal pain and an elevated lipase; however, it is not the underlying pathology for this patient's presentation but rather is a complication of the cholesterol embolism. Treatment of pancreatitis is with gastrointestinal rest and fluid support. Answer E: Renal failure is another complication in this patient secondary to the cholesterol embolism as revealed by the elevated creatinine, but is not the underlying pathology. Treatment is with renal replacement therapy if renal function does not return promptly. Bullet Summary: Cholesterol embolism presents after a vascular procedure with livedo reticularis, gastrointestinal complications, and acute kidney injury.
Renal failure
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A 27-year-old G1P0000 woman presents to her obstetrician’s office at 24 weeks gestation complaining of new hair growth on her upper lip. She now needs to shave her face every day or else thick hair appears. She also has worsening acne that started several weeks ago. Her pregnancy thus far has been complicated by gestational diabetes, which is managed by diet and exercise. She has a male fetus based on her 20 week anatomy ultrasound. The patient has a medical history of polycystic ovary syndrome. The patient’s temperature is 98.5°F (36.9°C), blood pressure is 121/76 mmHg, pulse is 70/min, and respirations are 13/min. The cardiopulmonary exam is normal, and the patient’s abdomen has a fundal height of 31 cm. A pelvic exam reveals an unremarkable closed cervix and a right-sided adnexal mass without tenderness. Transvaginal ultrasound is performed and shown in Figure A. Which of the following is the most appropriate next step in management?
Laparoscopy to prevent detrimental effects on the fetus
Laparoscopy to remove a malignancy
Percutaneous umbilical blood sampling to determine prognosis
Reassurance
D
Reassurance
This patient presents with hirsutism and acne, symptoms of hyperandrogenism, as well as a solid pelvic mass on ultrasound, most consistent with a luteoma. The most appropriate next step in management is reassurance as the mass tends to self-resolve after pregnancy. Luteomas are ovarian tumors that arise during pregnancy and produce androgens, causing maternal and sometimes fetal virilization. They appear as solid masses on ultrasound and are not malignant. The likelihood of virilizing the fetus increases if the luteoma arises in the 1st trimester and affected female fetuses present with clitoromegaly or labioscrotal fusion. Male fetuses are not affected. Treatment is supportive as the masses tend to resolve after pregnancy. Wang et al. review the evidence regarding the diagnosis and treatment of luteomas. They discuss how this is a rare tumor that primarily affects women who have had multiple pregnancies. They recommend early diagnosis of this condition in order to inform treatment options. Figure/Illustration A shows a transvaginal ultrasound with a solid mass on the right ovary (red circle). This appearance is consistent with a luteoma. Incorrect Answers: Answer A: Laparoscopy to prevent detrimental effects on the fetus is not considered a treatment option for luteomas. In this patient, the exposure to high androgen levels is late in pregnancy and the fetus is a male, making it especially unnecessary to perform surgery. Answer B: Laparoscopy to remove a malignancy is not indicated for luteomas, which are benign tumors and self-regress after delivery. Malignancies that can cause virilization in or outside of pregnancy include Sertoli-Leydig cell tumors and Krukenberg tumors. Answer C: Percutaneous umbilical blood sampling to determine prognosis may be performed in women who have early virilization and female fetuses. High androgen levels in the umbilical cord are required for the virilization of the fetus, whereas high androgen levels in the mother’s peripheral blood may not predict this outcome. Answer E: Spironolactone to treat hirsutism and acne is used outside of pregnancy in women with polycystic ovary syndrome. However, because it is an anti-androgen, spironolactone should not be used in pregnancy as it can cause feminization of male fetuses. Bullet Summary: Luteomas generally do not require treatment as they regress after pregnancy.
Spironolactone to treat hirsutism and acne
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A 15-year-old girl presents to the clinic due to concern that she may be pregnant. She had unprotected sexual intercourse earlier that morning and requests emergency contraception. She has had regular menses since menarche, and her last menstrual period ended 8 days ago. She has no significant medical history. Her temperature is 98.6°F (37°C), blood pressure is 114/71 mmHg, pulse is 81/min, and respirations are 12/min. A physical exam is unremarkable. A urine pregnancy test is negative. The patient asks for an emergency contraceptive pill and would prefer that her parents not be notified about her visit. In most states, which of the following is the most appropriate next step in the management for this patient?
Prescribe misoprostol pill
Prescribe levonorgestrel pill
Obtain parental consent before providing emergency contraception
Prescribe high-dose oral contraceptives
B
Prescribe levonorgestrel pill
This patient with recent unprotected intercourse and a negative pregnancy test is a candidate for postcoital emergency contraception. She should receive the levonorgestrel or ulipristal pill, which in most states does not require parental consent. Patients with a positive pregnancy test are not candidates for emergency contraception because implantation has already occurred, but patients with recent unprotected intercourse but a negative pregnancy test should receive the levonorgestrel pill (plan B) or ulipristal pill. These pills should be administered as soon as possible after intercourse. This is because the efficacy of the pills decrease after 72-120 hours post-intercourse. The high doses of progestin in these pills delay ovulation by blocking the LH surge, thereby preventing pregnancy. In most states, adolescents can receive emergency contraception without parental consent or notification. Batur et al. present the current options available for emergency contraception. They discuss how ulipristal acetate tends to be more efficacious compared to levonorgestrel when used more than 72 hours post-coitally. They recommend that all providers who treat young women be familiar with the options and indications for emergency contraception. Incorrect Answers: Answer A: It is inappropriate to not administer emergency contraception despite the patient being under 18 years old. Adolescent patients at risk of pregnancy after unprotected intercourse who request emergency contraception should be presented with viable options. Answer B: Obtaining parental consent before providing emergency contraception is an incorrect choice in most states. Most states allow adolescents to receive confidential care for contraception and pregnancy without parental involvement, consent, or notification. Answer C: Prescribing high-dose oral contraceptives can be done in special circumstances to delay ovulation by patients taking multiple pills at once. However, combination oral contraceptives are less commonly used for post-coital contraception as they have significant side effects at high doses and are less effective than levonorgestrel pills. Answer E: In patients with a positive pregnancy test (after implantation has occurred), misoprostol can be used for pregnancy termination by stimulating myometrial contractions. It does not prevent pregnancy. The use of drugs to terminate pregnancy varies significantly from state to state. Bullet Summary: Adolescents seeking emergency contraception may do so without parental consent or involvement in most states.
nan
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A 27-year-old nurse presents to the emergency department with a 30-minute history of palpitations and chest pain. She has been experiencing nausea, vomiting, and diarrhea after eating leftovers 2 days ago. Her husband tells the physician that he is concerned that the patient has been increasingly obsessed about her weight recently. She has a history of major depressive disorder for which she takes escitalopram. Her temperature is 98.6°F (37.0°C), blood pressure is 110/72 mmHg, pulse is 110/min, and respirations are 11/min. Physical examination is notable for dry mucous membranes and tachycardia with regular rhythm. Laboratory testing shows the following results: Serum: Na+: 135 mEq/L Cl-: 96 mEq/L K+: 2.9 mEq/L HCO3-: 36 mEq/L Venous blood gas shows a pH of 7.52. Which of the following tests is most appropriate in differentiating between different etiologies of this patient's lab abnormalities?
Serum glucose
Complete blood count
Urine chloride
Arterial blood gas
C
Urine chloride
This patient who presents with hypokalemia and metabolic alkalosis may either be vomiting or engaging in diuretic abuse. A urine chloride test can be used to distinguish between these possibilities because urine chloride will be high in diuretic abuse and will be low/normal in surreptitious vomiting. Metabolic alkalosis is an acid-base disorder that is due to an increased level of serum bicarbonate. This increase can be secondary to increased bicarbonate intake or loss of hydrogen ions. Etiologies consistent with increased intake include antacid use or milk-alkali syndrome. Etiologies consistent with hydrogen ion loss include gastrointestinal losses such as vomiting or renal losses such as hyperaldosteronism, Bartter syndrome, or diuretic abuse. Urine chloride can distinguish between gastrointestinal and renal losses because urine chloride levels will be high in patients with renal tubule dysfunction or diuretic abuse. This is due to increased urinary excretion of ammonium chloride in cases of diarrhea. On the other hand, urine chloride will generally be low in hyperaldosteronism due to loss of chloride with vomiting. Wu et al. studied the causes of chronic hypokalemia as well as laboratory findings that can be used to distinguish between different etiologies. They show how urine sodium and chloride can be helpful for identifying the surreptitious use of diuretics. The authors recommend the use of the sodium-to-chloride excretion ratio to help diagnose patients with chronic normotensive hypokalemia. Incorrect Answers: Answer A: Arterial blood gas is useful for determining the acid-base state of a patient because it simultaneously measures carbon dioxide and bicarbonate levels. This test is not as useful in determining different causes of metabolic alkalosis because all of these etiologies will have similar findings (high bicarbonate and compensatory high carbon dioxide), especially in patient who has already received a venous blood gas. Answer B: Complete blood count is useful for determining whether a patient is anemic resulting in decreased oxygen delivery to end organs due to decreased carrying capacity for oxygen. In cases of severe anemia and hypoperfusion, peripheral tissues produce lactic acid which will result in an anion gap metabolic acidosis rather than metabolic alkalosis. Answer C: Serum glucose elevation can result in increased diuresis as well as pseudohyponatremia (decreased measured sodium due to increased glucose). Alternatively, acidosis may also be caused by insulin deficiency in diabetic ketoacidosis. Neither of these findings is consistent with the lab results seen in this patient. Answer E: Urine toxicology can be used to determine whether the patient is taking any substances of abuse. There is no pathognomonic acid-base finding that can aid in diagnosing overdose and/or substance abuse. However, most standard urine toxicology screens do not detect the use of diuretics. Bullet Summary: Urine chloride can distinguish between gastrointestinal and renal causes of metabolic alkalosis.
nan
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A 1-year-old girl is brought to a neurologist due to increasing seizure frequency over the past 2 months. She recently underwent a neurology evaluation which revealed hypsarrhythmia on electroencephalography (EEG) with a mix of slow waves, multifocal spikes, and asynchrony. Her parents have noticed the patient occasionally stiffens and spreads her arms at home. She was born at 38-weeks gestational age without complications. She has no other medical problems. Her medications consist of lamotrigine and valproic acid. Her temperature is 98.3°F (36.8°C), blood pressure is 90/75 mmHg, pulse is 94/min, and respirations are 22/min. Physical exam reveals innumerable hypopigmented macules on the skin and an irregularly shaped, thickened, and elevated plaque on the lower back. Which of the following is most strongly associated with this patient's condition?
Cardiac rhabdomyoma
Glaucoma
Optic glioma
Polyostotic fibrous dysplasia
A
Cardiac rhabdomyoma
This patient with a seizure disorder, ash-leaf spots (innumerable hypopigmented macules), Shagreen patch (elevated irregular plaque on the lower back), and West syndrome (hypsarrhythmia on EEG and movements consistent with infantile spasms) likely has tuberous sclerosis. Tuberous sclerosis is associated with cardiac rhabdomyomas. Tuberous sclerosis is a neurocutaneous disorder that is inherited in an autosomal dominant fashion. Mutations in TSC1 and TSC2 lead to unregulated cell growth, leading to the formation of hamartomas in various locations. Manifestations of tuberous sclerosis include seizures (secondary to subependymal nodules and cortical dysplasia), mental retardation, renal angiomyolipomas, facial angiofibromas, mitral regurgitation, and hypomelanotic macules (ash-leaf spots). Cardiac rhabdomyoma is strongly associated with tuberous sclerosis and can lead to arrhythmias. West syndrome, which is characterized by infantile spasms, intellectual disability, and hypsarrhythmia (a chaotic mixture of high-amplitude slow waves, multifocal spikes, and intrahemispheric-interhemispheric asynchrony) on EEG, is also associated with tuberous sclerosis. Diagnosis of tuberous sclerosis is made with genetic testing. Advanced imaging (CT or MRI) may reveal cortical tubers and lesions in the third and fourth ventricles. Abdominal imaging may reveal renal angiomyolipomas. The management of seizures in tuberous sclerosis includes anti-seizure medications, though surgery may also be pursued in select cases. Hinton et al. studied the cardiac manifestations of tuberous sclerosis. They found that cardiac rhabdomyomas can lead to arrhythmia later in life. They recommended that infantile spasms in tuberous sclerosis be treated with vigabatrin but other seizure types should be treated similar to other epileptic disorders. Incorrect Answers: Answer B: Glaucoma may occur in patients with Sturge-Weber syndrome, which is characterized by "tram-track" calcifications in the brain, port-wine stains of the face, mental retardation, and epilepsy. Diagnosis is with MRI of the brain with contrast to demonstrate leptomeningeal vascular malformations that are characteristic of the disease. Answer C: Optic glioma is associated with neurofibromatosis type 1 (NF1), which presents with hyperpigmented macules (cafe-au-lait spots), axillary freckling, and cutaneous neurofibromas. Neurofibromas in NF1 may also grow on peripheral nerves, leading to malignant peripheral nerve sheath tumors. Lisch nodules, or benign growths of the iris, may also be seen in NF1. Diagnosis of NF1 is clinical, but can be aided by genetic testing. Answer D: Polyostotic fibrous dysplasia is associated with McCune-Albright syndrome. Polyostotic fibrous dysplasia causes fibrous tissue in bones, leading to growth abnormalities and pathologic fractures. McCune-Albright syndrome is also associated with cafe-au-lait macules, which have irregular borders, and endocrine abnormalities such as precocious puberty and hyperthyroidism. Diagnosis is supported with endocrine tests as well as genetic testing. Answer E: Renal cell carcinoma is associated with Von-Hippel-Lindau syndrome (VHL), which presents with tumors arising in multiple organs. Patients with VHL present with hemangioblastomas in the brain, spinal cord, cerebellum, and retina, and pheochromocytoma. Pheochromocytomas release catecholamines, leading to episodic hypertension, diaphoresis, and palpitations. Diagnosis of VHL is with genetic testing. Bullet Summary: Tuberous sclerosis has numerous manifestations including seizures, angiofibromas, mitral regurgitation, renal angiomyolipoma, ash-leaf spots, mental retardation, and cardiac rhabdomyoma.
Renal cell carcinoma
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A 26-year-old woman presents to the emergency department because she feels as though her heart is "beating out of her chest." She states that she feels well apart from the discomfort of that sensation. Her temperature is 97.9°F (36.6°C), blood pressure is 124/84 mmHg, pulse is 180/min, respirations are 22/min, and oxygen saturation is 98% on room air. An ECG is obtained as seen in Figure A. After unsuccessful attempts at vagal maneuvers, the emergency physician administers an IV medication. The effect of the medication wears off in seconds. Which of the following is a potential side effect of this medication?
Disulfiram-like reaction
Flushing
Photosensitivity
Seizure
B
Flushing
This patient presents with a narrow complex tachycardia that is regular which is suggestive of supraventricular tachycardia (SVT) and was pharmacologically cardioverted with adenosine (as it has a very short half-life). Side effects of adenosine include flushing, hypotension, bronchospasm, and heart block. SVT presents with a narrow-complex, regular tachycardia. The diagnosis is made with these findings on ECG. Management in an unstable patient is centered on cardioversion. Otherwise, the first step in management is centered on vagal maneuvers. If these fail, adenosine is the first-line pharmacologic treatment. While a relatively safe drug, side effects of adenosine include hypotension, heart block, flushing, and bronchospasm. Patients may also report a "sense of impending doom" following administration, thought to be the result of the transient asystole adenosine produces. Persistent SVT refractory to adenosine may be further managed with calcium channel blockers, beta blockers, or cardioversion. Colucci et al. review the diagnosis and management of common types of supraventricular tachycardias. The most common types of supraventricular tachycardia are caused by a reentry phenomenon producing accelerated heart rates. Symptoms may include palpitations, chest pain, lightheadedness or dizziness, and dyspnea. If Wolff-Parkinson-White syndrome is present, expedient referral to a cardiologist is warranted because ablation is a potentially curative option. Figure A is an ECG demonstrating SVT. Note the regular narrow complex tachycardia that is a defining feature of this condition. Incorrect Answers: Answer A: A disulfiram-like reaction was once thought to be associated with medications such as metronidazole. Metronidazole is an antibiotic that covers anaerobes. When alcohol is consumed in a patient on disulfiram, headache, malaise, nausea, and vomiting may be experienced. Answer C: Photosensitivity is associated with medications such as sulfonamides, amiodarone, and tetracycline. Patients should be advised to stay out of the sun on these medications. Answer D: Seizures are not associated with adenosine. Isoniazid, bupropion, imipenem/cilastatin, tramadol, enflurane, and metoclopramide are all known to increase the risk of seizures. Seizures often present with tonic-clonic activity, tongue biting, urinary incontinence, and a postictal phase. Answer E: Tachycardia can be associated with medications such as atropine which reduce vagal tone on the heart increasing the heart rate. Medications that block the AV node would be expected to cause bradycardia. Bullet Summary: Adenosine is a first-line pharmacological agent in supraventricular tachycardia and can cause hypotension, heart block, flushing, and bronchospasm.
Tachycardia
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A 62-year-old man presents to the emergency department with skin lesions. He felt itchy recently, then noticed skin lesions that broke out prompting him to come to the emergency department. He recently went camping and hiking in the woods. The patient drinks 1-2 beers per day and smokes 1 pack of cigarettes per day. He is currently sexually active with multiple partners and does not use condoms. The patient was discharged 1 week ago for pneumonia which was treated with azithromycin; however, he feels that his symptoms may be returning. His temperature is 99.5°F (37.5°C), blood pressure is 157/78 mmHg, pulse is 90/min, respirations are 15/min, and oxygen saturation is 98% on room air. Physical exam reveals the findings in Figure A on the patient's arm. The skin lesions are thick and do not break when pressure is applied. Which of the following most appropriately describes the most likely diagnosis?
IgG in a fish-net pattern
Linear IgG along the basement membrane
Multi-nucleated giant cells
Panniculitis
B
Linear IgG along the basement membrane
This patient is presenting with pruritic bullous skin lesions with a thick/tough covering suggesting a diagnosis of bullous pemphigoid. Linear IgG binding of anti-hemidesmosome antibodies along the basement membrane is the underlying cause of this disease. Bullous pemphigoid presents with a urticarial prodrome in elderly patients followed by the formation of tense bullae that do not break when pressure is applied to them. IgG antibodies bind the hemidesmosome along the basement membrane and appear linear on immunofluorescence. Risk factors that are associated with this condition include the initiation of new medications such as loop diuretics, metformin, and neuroleptics. Steroids are the treatment of choice. Miyamoto et al. review the evidence regarding the diagnosis and treatment of bullous pemphigoid. They discuss how high-potency corticosteroids are the mainstay of treatment for this disease. They recommend considering adjuvant therapy with doxycycline, dapsone, and immunosuppressants. Figure/Illustration A is a clinical photograph showing bullous skin lesions (red circles). These lesions are tense and do not separate/break when pressure is applied to them and are classically seen in bullous pemphigoid. Incorrect Answers: Answer A: IgG in a fish-net pattern suggests a diagnosis of pemphigus vulgaris, which presents with skin lesions that are flaccid and break easily when pressure is applied. Treatment is with corticosteroids. Answer C: Multi-nucleated giant cells suggests a diagnosis of HSV or herpes zoster virus, which presents with potentially painful vesicles distributed in a dermatomal pattern (for zoster). Treatment is with antivirals such as valacyclovir. Answer D: Panniculitis suggests a diagnosis of erythema nodosum, which presents with painful, raised nodules that are associated with inflammatory disorders and infection. Treatment is centered on addressing the underlying pathology. Answer E: Type IV hypersensitivity reaction suggests a diagnosis of contact dermatitis, which presents with pruritic lesions after exposure to a particular antigen such as poison ivy, poison oak, or nickel. Treatment is avoidance of the precipitating agent and steroids as well as symptomatic control. Bullet Summary: Bullous pemphigoid occurs secondary to anti-hemidesmosome antibodies which appear linear along the basement membrane on immunofluorescence.
Type IV hypersensitivity reaction
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A 37-year-old man presents to the emergency department for a persistent fever. The patient has felt unwell for the past week and has felt subjectively febrile. The patient has a medical history of a suicide attempt and alcohol abuse. He is not currently taking any medications. The patient admits to using heroin and cocaine and drinking 5-8 alcoholic drinks per day. His temperature is 103°F (39.4°C), blood pressure is 92/59 mmHg, pulse is 110/min, respirations are 20/min, and oxygen saturation is 96% on room air. Cardiopulmonary exam is notable for a systolic murmur heard best along the left sternal border. Dermatologic exam reveals scarring in the antecubital fossa. IV fluids are administered and the blood pressure is subsequently 120/70 mmHg. Which of the following is the most appropriate next step in management for the most likely underlying diagnosis?
Blood cultures
Chest radiograph
CT scan
Ultrasound
A
Blood cultures
This patient is presenting with fever and a systolic murmur suggestive of bacterial endocarditis. The most appropriate initial step in management is to draw blood cultures. Bacterial endocarditis presents with fever, systemic symptoms (aches, myalgias, and fatigue), and a new murmur typically in a patient with intravenous drug use. These patients introduce bacteria intravenously which then flows to the right side of the heart and can cause right-sided endocarditis. The most appropriate initial step in management is to draw blood cultures. Once blood cultures have been drawn, broad-spectrum antibiotics can be started (vancomycin and gentamicin), and an ultrasound can be performed to further support the diagnosis (or rule out vegetations) and plan operative interventions if necessary. Wang et al. review the evidence regarding the diagnosis and treatment of infectious endocarditis. They discuss how echocardiogram and blood cultures are the mainstay of diagnosis. They recommend broad spectrum antibiotics. Incorrect Answers: Answer B: A chest radiograph may be performed in the workup for bacterial endocarditis; however, it is not the most appropriate initial step in management and is neither sensitive nor specific. Answer C: A CT scan would not be a very helpful confirmatory test in the diagnosis of bacterial endocarditis. A better confirmatory test would be an ultrasound. It may workup alternative causes of fever such as pneumonia. Answer D: Ultrasound (echocardiography) would be the imaging test of choice for bacterial endocarditis. It would be performed after blood cultures have been drawn and antibiotics had been started. Answer E: Vancomycin and gentamicin are the broad-spectrum antibiotics of choice for bacterial endocarditis. They would be started after blood cultures have been drawn and reduce mortality when given early in sepsis. Bullet Summary: The most appropriate initial step in the management of bacterial endocarditis after initial stabilization is to draw blood cultures.
Vancomycin and gentamicin
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An 84-year-old man presents to the physician with numbness and tingling in the right hand for the last 6 months. He reports these symptoms started intermittently in the middle finger after returning from a golfing trip but has progressed to being nearly constant. The thenar eminence, thumb, and other digits are spared. He reports no other symptoms. His medical problems include hypertension, hyperlipidemia, hypothyroidism, and chronic neck pain for which he takes amlodipine, hydrochlorothiazide, atorvastatin, levothyroxine, and duloxetine. Surgical history consists of posterior lumbar fusion and elective left total hip arthroplasty. He has a 90-pack-year smoking history and quit 10 years ago. The patient’s temperature is 99.6°F (37.6°C), blood pressure is 134/80 mmHg, pulse is 90/min, and respirations are 16/min. Physical exam reveals 4/5 strength in elbow extension and wrist flexion on the right. He has 5/5 strength in the remainder of the motor exam on the right upper extremity and on the left upper extremity. The triceps reflex is 1+ on the right compared to 2+ on the left. There is no spasticity. His neck pain, which radiates down his right arm, is reproduced with axial loading during neck extension with simultaneous rightward rotation and lateral bending. Which of the following is the most likely diagnosis in this patient?
Amyotrophic lateral sclerosis
Carpal tunnel syndrome
Cervical spondylosis
Syringomyelia
C
Cervical spondylosis
This patient presents with chronic middle finger paresthesia, weakness in elbow extension and wrist flexion, and a diminished triceps reflex in the setting of a positive Spurling test (pain with axial loading during neck extension, rotation, and lateral bending towards the affected side), indicative of C7 cervical radiculopathy. Cervical spondylosis is a common cause of cervical radiculopathy in elderly patients with chronic neck pain, especially in patients with findings suggestive of degenerative joint diseases (history of lumbar fusion and hip arthroplasty). Cervical spondylosis is caused by degeneration of the intervertebral disc with possible disc herniation and facet joints with osteophyte formation. These osteophytes and cervical disc herniation can cause compression of exiting nerve roots, leading to radiculopathy. Radicular symptoms depend on the nerve root affected. For example, C6 radiculopathy causes weakness in wrist extension and a decreased brachioradialis reflex while C7 radiculopathy causes weakness in elbow extension and wrist flexion with a decreased triceps reflex. Sensory symptoms may be present depending on the dermatome affected. The diagnosis is confirmed with MRI. Treatment consists of non-steroidal anti-inflammatory drugs (NSAIDs), muscle relaxants, and/or corticosteroids for pain. Surgical treatments for severe or refractory cases include anterior cervical discectomy and fusion. Young outlines the diagnosis and management of cervical spondylotic myelopathy. The discusses its pathophysiology, including osteophyte formation and spinal cord dysfunction. They recommend considering decompression and fusion operations in selected patients. Incorrect Answers: Answer A: Amyotrophic lateral sclerosis (ALS) is a motor neuron disease of the brain and spinal cord that leads to upper and lower motor neuron signs. ALS presents with hyperreflexia (as opposed to hyporeflexia), spasticity, weakness, and fasciculations. Treatment includes riluzole (to improve survival) and symptomatic management. Answer B: Carpal tunnel syndrome is caused by compression of the median nerve as it travels through the carpal tunnel at the wrist. While the thenar eminence is typically spared due to take-off of the palmar cutaneous branch proximal to the carpal tunnel, the thumb and index finger are also affected. This patient’s symptoms primarily involve the middle finger, which is more indicative of a radiculopathy. Treatment is activity modification, splinting, and ultimately, surgical decompression of the carpal tunnel if refractory. Answer D: Syringomyelia is caused by a fluid-filled cavity (syrinx) within the spinal cord that leads to central cord syndrome, characterized by weakness that is greater in the upper extremities compared to lower extremities and loss of pain and temperature sensation in a cape-like distribution in the upper back and upper extremities. This patient’s sensory loss is unilateral and in a dermatomal distribution. Treatment of syringomyelia can include surgical decompression. Answer E: Transverse myelitis is characterized by segmental inflammation of the spinal cord that would present with acute, bilateral flaccid paresis based on the level affected and sensory loss within a sensory level. Autonomic symptoms such as incontinence may also be present. This patient’s symptoms are unilateral and localizable to nerve root compression as opposed to the spinal cord. Bullet Summary: Cervical spondylosis is caused by degeneration of cervical discs and facet joints with osteophyte formation and can present with symptoms of cervical radiculopathy or myelopathy in elderly patients with chronic neck pain.
nan
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A 50-year-old man presents to the emergency department with chest pain. He states that the pain is dull in quality, started 30 minutes ago, has been gradually worsening, and is worse with exertion. He also endorses some shortness of breath. He also believes the pain is worse when leaning back and improved when leaning forward. His wife noticed he fainted after the pain started but regained consciousness shortly after. He has a medical history of diabetes and hypertension for which he is on metformin and losartan. His temperature is 99.2°F (37.3°C), blood pressure is 130/87 mmHg, pulse is 99/min, respirations are 22/min, and oxygen saturation is 100% on room air. Physical exam reveals an overweight man. A normal S1 and S2 are auscultated with clear breath sounds. An ECG is performed as seen in Figure A. Which of the following is the most likely diagnosis?
Hyperkalemia
Hypertrophic obstructive cardiomyopathy
Hypokalemia
Myocardial infarction
D
Myocardial infarction
This patient is presenting with chest pain and an ECG demonstrating hyperacute T waves (leads V2-V6) which is an early finding concerning for a myocardial infarction. A myocardial infarction occurs when an atherosclerotic plaque ruptures causing occlusion of a coronary artery. Patients classically present with chest pain, dyspnea, and diaphoresis. Atypical manifestations of an acute myocardial infarction may include abdominal pain, nausea, reflux symptoms, and dizziness. The most important initial step in management is to administer aspirin as it lowers mortality and carries minimal risk. An ECG should then be performed. Classically, an ST-elevation myocardial infarction (STEMI) will demonstrate ST elevations in a vascular distribution. However, the progression to a STEMI is usually stepwise and may present with ST depressions, hyperacute T waves, pseudonormalization of the ST segment (a normal ST segment after a depression as it elevates), ST elevations, and finally tombstoning (a concave down wave that looks like a tombstone). If a STEMI is suspected after an ECG is performed, the patient should be started on a second antiplatelet agent (e.g., ticagrelor), heparin, and sent immediately to the cardiac catheterization laboratory. Nable et al. review the typical electrophysiologic changes found in STEMI. The authors note that T-wave inversions and ST-segment depression can before, during, or after the STEMI event. The authors recommend obtaining serial ECGs in patients with suspected STEMI. Figure/Illustration A demonstrates hyperacute T waves (blue arrows) that are only occurring in a vascular distribution (V2-V6). Incorrect Answers: Answer A: Hyperkalemia can present with peaked T waves; however, peaked T waves would be demonstrated in all leads. Other findings of hyperkalemia can include QRS widening. Immediate management of hyperkalemia with ECG changes should include calcium administration followed by drugs that shift potassium intracellularly such as insulin, albuterol, or sodium bicarbonate. Further management may include diuresis and/or dialysis. Answer B: Hypertrophic obstructive cardiomyopathy may demonstrate increased voltages throughout the ECG, in particular, in leads V2-V6 with deep Q waves in the septal leads. It may present with syncope upon exertion in a young patient (and in rare cases, sudden cardiac death). Ultrasound would show hypertrophy and left ventricular outflow tract obstruction. Management involves staying hydrated, avoiding strenuous activities, and beta blockers. Answer C: Hypokalemia can present initially on ECG with U waves and may progress to cardiac dysrhythmias. Repletion with potassium is the preferred treatment, and the magnesium level should be checked as low magnesium can lead to potassium wasting in the kidneys. Answer E: Pericarditis presents with chest pain worse by laying back and relieved by leaning forward. ECG may demonstrate diffuse ST elevations with PR depressions, and echocardiography may demonstrate a pericardial effusion. Management involves the administration of ibuprofen or colchicine and steroids in refractory cases. However, these therapies should not be used after myocardial infarction. Bullet Summary: Hyperacute T waves are an early finding that is concerning for myocardial infarction.
Pericarditis
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A 30-year-old man presents to the emergency department with diarrhea. He states he has had profuse, watery diarrhea for the past 24 hours. He notes that eating and drinking make him defecate more, thus he has been refraining from drinking. He is otherwise healthy and takes no medications. His temperature is 97.7°F (36.5°C), blood pressure is 84/64 mmHg, pulse is 130/min, respirations are 17/min, and oxygen saturation is 99% on room air. Physical exam reveals dry mucous membranes with an otherwise benign exam. The patient is given 1L of normal saline and drinks 1L of oral fluids. His blood pressure is subsequently 120/70 mmHg and pulse 95/min. Which of the following is the most appropriate next step in management?
Stool PCR
Ciprofloxacin
Stool ova and parasite study
Rest and oral rehydration
D
Rest and oral rehydration
This patient is presenting with a likely diarrheal illness given he has profuse, watery diarrhea and his vitals normalized with oral and IV hydration. Since the patient is young and healthy, oral hydration and rest are all that is needed for management. Diarrheal illness is a common chief complaint with variable workups pending on the patient's clinical status. Young, healthy, non-immunosuppressed patients who can tolerate an oral diet require only reassurance and oral hydration, as most cases of viral and bacterial diarrhea will resolve on their own. Moreover, most cases of diarrhea are caused by viral etiologies which simply require supportive therapy. This is even true in bacterial diarrhea as long as the patient's vitals normalize and they can tolerate an oral diet. It is important to consider inflammatory causes of diarrhea (such as ulcerative colitis or Crohn disease) as this would change the workup and management. Schiller et al. discuss different types of diarrhea. They review the management and diagnosis of diarrhea and its different forms, including chronic forms. They recommend noting the difference between different causes and working up and treating them appropriately. Incorrect Answers: Answer A: Ciprofloxacin is an antibiotic that can be used for bacterial/infectious diarrhea. It should not be given in young, healthy patients and would only be indicated in the setting of bacterial diarrhea in ill patients, patients with persistent symptoms, or patients who are immunosuppressed. Bacterial diarrhea would present with fever, abdominal pain, and bloody/purulent diarrhea. Answer C: Stool culture should not be empirically performed in young, well-appearing patients. It may be performed in hospitalized patients who are more ill or in patients who are going to start antibiotic therapy. It may also be performed in patients with persistent diarrhea. Answer D: A stool ova and parasite study would not be performed unless there was a suggestive history or exposure (such as drinking unfiltered water) in the setting of fatty, foul-smelling diarrhea. This would raise suspicion for Giardia lamblia which would be treated with metronidazole. Answer E: Stool PCR for viral etiologies of diarrhea is not useful as the management of viral diarrhea is supportive therapy in most cases. On occasion, it may be performed in critically ill patients to determine the etiology of their symptoms. It should not be performed routinely. Bullet Summary: Healthy patients with limited diarrheal illness require supportive therapy only (in particular, oral hydration) and do not need more invasive diagnostic tests.
nan
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A 1-hour-old newborn boy is evaluated in the delivery room. He was born at 37 weeks gestation to a 39-year-old G3P3 mother. The mother initially labored at home with a midwife but was transferred to the hospital for failure to progress. The infant was eventually delivered via Caesarean section. The mother declined all prenatal screening tests during this pregnancy. His temperature is 98.6°F (37°C), blood pressure is 63/41 mmHg, pulse is 133/min, and respirations are 39/min. His Apgar scores were 7 and 8 at 1 and 5 minutes, respectively. His weight is 3.0 kg (6.6 lb), and his height and his head circumference are in the 30th and 40th percentiles, respectively. On physical exam, he is found to have a 3 cm full-thickness defect in the abdominal wall to the right of the umbilicus with evisceration of a loop of the bowel. The abdominal defect is immediately covered in sterile saline dressings and an orogastric tube and 2 peripheral intravenous lines are placed. This condition is most likely associated with which of the following additional findings?
Ventricular septal defect
Bicuspid aortic valve
A normal cardiac exam
Tetralogy of Fallot
C
A normal cardiac exam
This patient presents with a full-thickness defect in the abdominal wall lateral to the umbilicus with evisceration of the bowel, which suggests a diagnosis of gastroschisis. Gastroschisis is not associated with any cardiac defects. Gastroschisis and omphalocele are the most common abdominal wall defects found in infants and there are important differences between them. Whereas the eviscerated bowel in gastroschisis is not covered by a membrane, the exposed bowel in the omphalocele is covered by a membranous sac. The abdominal wall defect in gastroschisis is located lateral to the umbilicus, whereas in omphalocele the defect is midline. The distinction is also important for management, as omphalocele is commonly associated with other extraintestinal defects whereas fewer than 10% of cases of gastroschisis are associated with an extraintestinal abnormality. Treatment is with surgical correction of the abdominal wall defect. Skarsgard reviews the evidence regarding the diagnosis and treatment of gastroschisis. He discusses how this is one of the most common birth defects managed in the ICU setting. He recommends urgent surgical correction. Incorrect Answers: Answer B: A bicuspid aortic valve occurs in many cases of Turner syndrome. This disease presents with a webbed neck, short stature, low hairline, broad (“shield”) chest, bicuspid aortic valve, horseshoe kidney, and streak ovaries. Treatment is supportive with surgical fixation of cardiac defects and hormone supplementation with growth hormone. Answer C: Endocardial cushion defects are most commonly found in trisomy 21 (Down syndrome). Over a third of individuals with trisomy 21 have a complete atrioventricular septal defect, and another third have isolated ventricular septal defects. Infants with Down syndrome commonly present with the upslanting palpebral fissures, flat nasal bridge, transverse palmar crease, and hypotonia. Treatment is with supportive care of these deformities such as surgical correction of cardiac defects. Answer D: Tetralogy of Fallot is most often associated with trisomy 21 (Down syndrome) and 22q11 deletion syndromes. Infants with Down syndrome commonly present with upslanting palpebral fissures, flat nasal bridge, transverse palmar crease, and hypotonia. Treatment is with supportive care of these deformities such as surgical correction of cardiac defects. Answer E: Ventricular septal defects occur in several genetic syndromes, including trisomy 21 (Down syndrome) and trisomy 13 (Patau syndrome). Infants with trisomy 13 commonly present with cleft lip or palate, polydactyly, and severe intellectual disability. This disease is not compatible with survival past 1 year of age so treatment can involve termination of pregnancy or supportive care. Bullet Summary: Gastroschisis presents with an abdominal wall defect with evisceration of the bowel that is lateral to the umbilicus and is not usually associated with other birth defects.
nan
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A 32-year-old G1P0 woman delivers a boy at 40 weeks and 3 days of gestation. The delivery is uncomplicated and the neonate has Apgar scores of 9 and 9 at 1 and 5 minutes respectively. After 15 minutes, he attempts to nurse for the 1st time. His mother notices that his face, chest, and extremities turn dusky blue during nursing. She also notes that he seems to be snoring loudly and breathing very quickly. When she removes him from her breast, he begins to cry and his color improves. The neonate’s prenatal course was unremarkable, and his mother has a medical history of asthma with occasional albuterol inhaler use. As the infant is resting, vital signs are taken. His temperature is 98.2°F (36.7°C), pulse is 130/min, respirations are 45/min, and oxygen saturation is 97% on room air. A physical exam demonstrates well-perfused skin but is notable for the facial features shown in Figure A. Which of the following is most likely to be observed in this neonate?
Digital clubbing
Patent foramen ovale
Inability to pass a nasogastric tube
Areflexia in all extremities
C
Inability to pass a nasogastric tube
This neonate presents with central cyanosis upon feeding, resolution with crying, and midface hypoplasia on exam, most consistent with choanal atresia. A nasogastric (NG) tube would not pass through the nasal canal. Choanal atresia is caused by failed recanalization of the nasal fossae during fetal development. This disease typically presents with noisy breathing, cyanosis when feeding, and improvement with crying and the use of the mouth for breathing. Cyanosis in this condition affects the entire body as opposed to just the hands and feet. Bilateral atresia often presents early in life. Clinical suspicion of choanal atresia should prompt an attempt to pass an NG tube, which will meet resistance at the posterior part of the nasal canal. Treatment is with the surgical creation of the nasal passages. Lesciotto et al. review the diagnosis and pathophysiology of patients with choanal atresia. They discuss how this condition is often comorbid with craniosynostosis. They recommend a better understanding of how these patients develop decreased nasal airway volume. Figure/Illustration A is a clinical photograph showing midface hypoplasia with a poorly developed nasal bone (red circle). These findings are consistent with a diagnosis of choanal atresia. Incorrect Answers: Answer A: Areflexia in all extremities may be seen in spinal muscular atrophy, a genetic condition that causes loss of motor neurons. This condition may cause difficulty feeding due to weakness of the oropharyngeal and glossopharyngeal muscles, but it typically does not cause cyanosis. Treatment is with steroid use and nusinersen. Answer B: Digital clubbing can result from chronic hypoxemia, as seen in conditions such as cystic fibrosis. It would be unexpected for a neonate to have clubbing as the characteristic finger appearance depends on the long-term deposition of platelet-derived growth factor and would not develop within minutes. This patient also does not have cyanosis at baseline. Treatment of cystic fibrosis is with enzyme repletion and respiratory therapy. Answer D: Markedly decreased lung volume can occur in congenital diaphragmatic hernia, in which the abdominal contents herniate into the chest cavity and cause pulmonary hypoplasia. Infants may present with cyanosis due to respiratory failure, but there would be no association with feeding or improvement with crying. Vital signs would also be abnormal. Treatment is with surgical repair of the diaphragm. Answer E: A patent foramen ovale occurs when the connection between the left and right atria does not close soon after birth. It does not result in cyanosis and is typically asymptomatic, though it may cause paradoxical embolism and stroke later in life. Treatment of a symptomatic patent foramen ovale is surgical closure of the defect. Bullet Summary: Choanal atresia presents with cyanosis and respiratory distress that worsens with feeding and improves with crying associated with failure to pass a nasogastric tube.
nan
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A 67-year-old man presents with a recurrent episode of syncope. He states that this has happened to him multiple times, particularly when he changes body position from sitting to standing, exerts himself, or turns his head quickly. He says that he notes numbness, vertigo, and sometimes even trouble with speaking immediately prior to fainting. The episodes usually last 1-5 minutes. When he wakes up, he is mildly confused but typically returns to baseline within a minute. The patient has a past medical history of diabetes, dyslipidemia, and hypertension. His temperature is 98.5°F (36.9°C), blood pressure is 153/89 mmHg, pulse is 90/min, respirations are 11/min, and oxygen saturation is 97% on room air. The patient's neurological exam is unremarkable. However, when he is asked to stand up and start walking, he experiences the same numbness and tingling and feels like he may faint. Which of the following is the most likely diagnosis?
Vagal event
Seizure
Blood vessel narrowing
Stroke
C
Blood vessel narrowing
This elderly patient with risk factors for vascular disease (diabetes, dyslipidemia, hypertension) is presenting with episodes of dizziness, vertigo, numbness/tingling, dysarthria, and syncope, which seem to be provoked by standing rapidly and changing head position. This is concerning for vertebrobasilar syndrome, caused by blood vessel narrowing. Vertebrobasilar syndrome is caused by narrowing (via causes such as atherosclerosis, dissection, or congenital narrowing among others) of the vertebrobasilar vascular system. It is a common cause of syncope that may be worsened by changes in head position or standing rapidly. Other unique features include numbness/tingling, a sensation of vertigo, nausea, vomiting, dysphagia, and dysarthria. Note the episodes of syncope in this condition are intermittent and specifically provoked and do not have lasting/permanent neurological deficits in contrast to a stroke. Management is first centered on vascular imaging which may include a CTA head/neck or an MRA head/neck. Further care depends on the symptoms present but typically involves smoking cessation, glycemic control, blood pressure control, aspirin, and a statin. Xuan-Ting et al. review posterior circulation strokes which occur more frequently in patients with atherosclerosis of the vertebrobasilar system. They note that MRI is one of the most accurate tests for assessing posterior circulation strokes which may present with dizziness and syncope. It is far superior to CT imaging. For this reason, they recommend that CT imaging alone cannot definitively rule out a posterior circulation stroke. Incorrect Answers: Answer B: Cardiac dysrhythmias such as ventricular tachycardia can cause sudden onset and offset syncope without prodromal symptoms. The differentiating feature of this form of syncope is the rapid onset and offset in a high-risk patient. The diagnosis can be confirmed with an ECG; however, many patients will require prolonged observation on telemetry. Answer C: Seizure presents with impaired consciousness, tonic-clonic activity, and a post-ictal state which gradually improves over hours. The lactate may be elevated after a seizure, and the prolactin level is a more specific indicator of a seizure. However, the diagnosis is most appropriately made based on history, exam, and EEG. Answer D: Stroke involving the vertebrobasilar system may cause syncope in addition to ongoing dizziness and vertigo with ataxia. However, this patient's symptoms seem to be positional and have entirely resolved; thus, a stroke is unlikely. He is at higher risk of stroke given his vertebrobasilar insufficiency and an MRI should still be performed in this patient. Answer E: Vagal event presents with a warm prodrome with palpitations, flushing, and syncope. Muscle twitching (but no tonic-clonic activity) may be seen. Patients may wake up slightly confused but return to baseline within seconds. Bullet Summary: Vertebrobasilar insufficiency may present with positional episodes of syncope caused by insufficient blood flow through the vertebrobasilar blood vessels.
nan
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A 3-week-old boy presents to the emergency department with vomiting. His parents report that he suddenly started vomiting this morning and has shown no interest in feeding since then. They describe the vomitus as green and without any traces of blood. Prior to today, the patient was feeding, voiding, and stooling well. He was noted to have surpassed his birth weight at his last office visit 1 week ago. His temperature is 97.6°F (36.4°C), blood pressure is 78/56 mmHg, pulse is 148/min, and respirations are 44/min. On physical exam, the patient is in mild distress. He has no dysmorphic features, and his mucous membranes are dry. His abdomen is soft and distended. Bowel sounds are hypoactive. An abdominal radiograph is performed as in Figure A. Which of the following is the most appropriate next step in the diagnosis of this condition?
Abdominal CT
Abdominal ultrasound
Contrast enema
Rectal suction biopsy
E
Upper gastrointestinal contrast series
This neonatal patient presents with sudden-onset bilious vomiting and an abdominal radiograph revealing a gasless abdomen, which is consistent with intestinal malrotation complicated by a midgut volvulus. The most appropriate next step in the diagnosis of intestinal malrotation is an upper gastrointestinal series. Malrotation classically presents with bilious vomiting due to the formation of midgut volvulus. These patients will have normal development until the development of the obstruction followed by inability to feed and abdominal distention. Findings of malrotation on an upper gastrointestinal series include an abnormally placed ligament of Treitz on the right side of the abdomen and a "corkscrew" appearance of the distal duodenum. If an upper gastrointestinal contrast series confirms the diagnosis, patients should undergo surgery to reduce the volvulus in order to avoid intestinal perforation or other complications. Adams and Stanton review the evidence regarding the diagnosis and treatment of neonatal bowel obstruction. They discuss how malrotation, jejunoileal atresia, duodenal atresia, and colonic atresia are common causes of this finding. They recommend surgical management of these conditions. Figure/Illustration A is an abdominal radiograph demonstrating the nonspecific finding of a paucity of air in the abdomen (red circle). This is consistent with intestinal malrotation complicated by midgut volvulus. Incorrect Answers: Answer A: Although abdominal CT would detect intestinal malrotation, CT should be avoided in the pediatric population in light of the radiation exposure. A CT scan with oral contrast can be useful in the adult setting in order to determine if there is extravasation of fluid from the gastrointestinal tract. Answer B: Abdominal ultrasound is the test of choice for diagnosing pyloric stenosis, which presents with non-bilious vomiting and a palpable olive mass in the abdomen. Treatment of this condition is with surgical release of the stenotic pyloric muscle. Answer C: A contrast enema is used to differentiate between Hirschsprung disease and meconium ileus but would not be helpful in diagnosing malrotation as the midgut volvulus occurs in the small intestine. Both Hirschsprung disease and meconium ileus present in neonatal patients with bilious vomiting and failure to pass meconium in the first 48 hours of life. Treatment is with excision of the defective segment or removal of the meconium respectively. Answer D: Rectal suction biopsy is the most accurate test to diagnose Hirschsprung disease. Although Hirschsprung disease commonly presents in neonatal patients with bilious vomiting, an abdominal radiograph in Hirschsprung disease would likely show dilation of the proximal colon. Bullet Summary: The most appropriate diagnostic test for malrotation in an infant is an upper gastrointestinal series, which will show the abnormal placement of the duodenum and ligament of Treitz on the right side of the abdomen.
Upper gastrointestinal contrast series
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A 13-day-old boy is brought by his mother for eye redness and ocular discharge. The mother reports that the patient has developed a cough and nasal discharge. Pregnancy and delivery were uncomplicated but the mother had limited prenatal care during the third trimester. Immediately after delivery, the baby was given silver nitrate drops and vitamin K. His temperature is 99°F (37.2°C), blood pressure is 81/52 mmHg, pulse is 135/min, and respirations are 36/min with an oxygen saturation of 98% O2 on room air. Upon visual examination of the eyes, mucoid ocular discharge and eyelid swelling are noted. A fluorescein test is negative. On lung exam, scattered crackles are appreciated. A chest radiograph is performed that shows hyperinflation with bilateral infiltrates. Which of the following is the most appropriate pharmacotherapy?
Artificial tears
Intravenous acyclovir
Intravenous ceftriaxone
Oral erythromycin
D
Oral erythromycin
This patient is presenting with mucoid ocular discharge around 2 weeks post-birth, suggesting the diagnosis of neonatal chlamydial conjunctivitis. The most appropriate treatment for this disease includes oral erythromycin. Neonatal chlamydial conjunctivitis is caused by Chlamydia trachomatis. It presents in newborns that are 5-14 days old. Symptoms include watery or mucoid discharge and eye swelling. The conjunctiva may be especially irritated. Diagnosis can be established by direct antibody testing or PCR. Treatment for chlamydial conjunctivitis includes both topical and oral erythromycin as there is likely dissemination of the infection. The most common complication of chlamydial conjunctivitis is pneumonia. Of note, all neonates with gonococcal conjunctivitis should also be treated for chlamydia. Smith-Norowitz et al. studied whether antibiotic prophylaxis was beneficial compared with silver nitrate for chlamydia conjunctivitis. They found that there was no benefit to topical antibiotic treatment compared with silver nitrate alone. They recommend testing infants for the disease if it is suspected so that oral antibiotics can be administered effectively. Incorrect Answers: Answer A: Artificial tears can be used in chemical conjunctivitis. The most common cause of neonatal chemical conjunctivitis is post-delivery silver nitrate drops. It usually presents within the first 24 hours following birth/exposure with mild conjunctival injection and tearing, and will spontaneously resolve within 2-4 days. Answer B: Intravenous acyclovir is used to treat conjunctivitis caused by herpes simplex virus. The newborn would present between 1-2 weeks post-birth with lid edema, conjunctival injection, and non-purulent serosanguineous discharge. Corneal involvement with microdendrites or ulcers may be seen with fluorescein stain. Answer C: Intravenous ceftriaxone is used to treat gonococcal conjunctivitis. Gonococcal conjunctivitis usually presents in newborns 0-5 days old with irritation or discharge. Patients with this disease will have very thick discharge with a purulent appearance. Answer E: Topical erythromycin would be used to treat chlamydial conjunctivitis in conjunction with oral erythromycin; however, it is insufficient on its own. Oral erythromycin is the best answer choice for this patient as the disease has likely disseminated. Topical erythromycin can also be used as prophylaxis for gonococcal conjunctivitis. Bullet Summary: Neonatal chlamydial conjunctivitis presents with watery or mucoid discharge as well as eye swelling and is treated with topical and oral erythromycin.
Topical erythromycin
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A 44-year-old homeless man presents to the emergency department after being stabbed multiple times in the abdomen. The patient is intoxicated and is unable to offer further history. His temperature is 97.5°F (36.4°C), blood pressure is 92/52 mmHg, pulse is 145/min, respirations are 33/min, and oxygen saturation is 90% on room air. He is intubated to protect his airway, given 2 units of packed red blood cells, and sent to the operating room for an exploratory laparotomy. The patient is subsequently admitted to the intensive care unit. He is extubated the next day and appears well. His vitals are within normal limits. Physical exam reveals sparse hair that is falling out throughout the patient’s body. He has a rash by the corners of his mouth. The patient has multiple loose bowel movements and on day 6 the patient’s surgical wound does not appear to be healing. It is held together only by the sutures with minimal underlying healing. Which of the following is most likely deficient in this patient?
Riboflavin
Thiamine
Vitamin C
Vitamin D
E
Zinc
This homeless patient who likely has poor nutritional status presents with angular cheilitis, sparse hair that is falling out, diarrhea, and poor wound healing, all of which are suggestive of zinc deficiency. Zinc is involved in many physiologic processes and is an important mineral to include in the diet. Zinc deficiency is common in patients with poor nutritional status such as the homeless, individuals living in developing nations without access to food, and patients with eating disorders. Many malabsorptive conditions can cause zinc deficiency including steatorrhea and chronic pancreatitis. Zinc deficiency presents with impaired wound healing and impaired immune function. Other symptoms suggestive of zinc deficiency include xerosis, alopecia, stomatitis, oral ulceration, angular cheilitis, impaired vision (night blindness), impaired smell/taste, diarrhea, and anorexia. The treatment of zinc deficiency involves zinc supplementation and workup for any other nutritional deficiencies. Nagata etc al. study the incidence of zinc deficiency among younger patients with anorexia nervosa. The authors find that men and women are both equally likely to have zinc deficiency when admitted. The authors recommend screening all patients with eating disorders for zinc deficiency. Incorrect Answers: Answer A: Riboflavin deficiency may present with angular cheilitis, stomatitis, and a non-specific rash. The treatment involves supplementing or consuming foods with riboflavin; however, it is rare in the developed world to be riboflavin deficient (due to fortified foods) and would not cause such significant wound healing issues as in this patient. Answer B: Thiamine deficiency is common in alcoholics and can cause Wernicke-Korsakoff syndrome, which presents with ataxia, nystagmus, and ophthalmoplegia in addition to amnesia. The treatment involves the administration of high-dose thiamine. Answer C: Vitamin C deficiency causes scurvy, which presents with easy fractures, bleeding gums or loose teeth, delayed wound healing, and perifollicular hemorrhages. It is common in the homeless and alcoholics. Treatment involves vitamin C replacement or a diet rich in fruits and vegetables. Answer D: Vitamin D deficiency is common in the homeless, alcoholics, or those who suffer from steatorrhea (such as chronic pancreatitis or Crohn disease). It presents with weak, soft bones (osteomalacia/osteoporosis), and hypocalcemia which may present with paresthesias and tetany. Treatment involves replacement of vitamin D. Bullet Summary: Zinc deficiency presents with poor wound healing, hair loss, infertility, diarrhea, anorexia, and impaired immune function.
Zinc
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A 55-year-old male bodybuilder presents to the emergency department with weakness of his right arm. The patient states he has experienced these symptoms for a few weeks; however, today his hand felt so weak he dropped his cup of tea. The patient has a medical history of diabetes. He drinks 2-7 alcoholic drinks per day and has smoked 2 packs of cigarettes per day since he was 25. The patient uses anabolic steroids. He has lost 17 pounds since he last came to the emergency department 1 month ago. His temperature is 99.5°F (37.5°C), blood pressure is 177/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals decreased sensation in the right arm and 2/5 strength in the right arm and 5/5 strength in in the left arm. The patient states that he is experiencing a dull aching and burning pain in his right arm during the exam. Symptoms are not changed with changes in head and neck position. Which of the following is the most likely diagnosis?
Apical lung tumor
Cerebral infarction
Scalenus anticus syndrome
Subclavian steal syndrome
A
Apical lung tumor
This patient is presenting with unilateral upper extremity paresthesia and weakness suggestive of compression of the neurovascular structures of the upper extremity secondary to an apical lung tumor. An apical lung tumor can compress the neurovascular structures in its proximity and cause pain, numbness, and weakness of the ipsilateral arm. A Horner syndrome can also be observed with compression of the sympathetic chain leading to ipsilateral miosis, ptosis, and anhidrosis in some cases. These malignancies typically occur in older male smokers and can be associated with symptoms such as fatigue, cough, and weight loss. Chest radiography will demonstrate an apical opacity and CT scan can confirm the size and location of the mass. Treatment is with chemotherapy and surgical excision of the tumor. Davis and Knight review the evidence regarding the diagnosis and treatment of patients with apical lung tumors. They discuss how these tumors can be associated with brachial plexus injuries. They recommend surgical excision of these tumors if they are resectable. Incorrect Answers: Answer B: Brachial plexopathy could present with weakness and numbness of the ipsilateral arm but is a less likely diagnosis (and a diagnosis of exclusion). Treatment is with identification and surgical release of any underlying anatomic defects (such as compressive bands) and supportive therapy if none are found. Answer C: Cerebral infarction (stroke) would present with sudden onset of acute neurological deficits. These would typically only affect one side of the body and would not necessarily be associated with a dull pain or numbness that is chronic. Treatment is with thrombolytics if patients meet criteria for acute therapy. Answer D: Scalenus anticus syndrome occurs in bodybuilders or athletes with hypertrophied neck muscles that compress the brachial plexus. Symptoms are typically worsened by certain positions. Treatment is with physical therapy and surgical decompression in refractory cases. Answer E: Subclavian steal syndrome presents with numbness, tingling, weakness in a limb, and CNS symptoms (vertigo, slurred speech, or stroke-like symptoms) that are worsened with activity and relieved with rest. Treatment is with vascular intervention to restore blood flow. Bullet Summary: Apical lung tumors can compress neurovascular structures in the upper extremity and cause pain, numbness, and tingling in the ipsilateral arm.
nan
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A 45-year-old man presents to his primary care physician with a rash. He first noticed the rash 2 weeks prior and he endorses intermittent itchiness from the rash. His medical history is significant for chronic hepatitis C infection, hypertension, type 2 diabetes mellitus, and heart failure. The patient works as a butcher at a local meat processing shop and is sexually active with several partners. He does not use barrier protection. His temperature is 98.6°F (37.0°C), pulse is 90/min, blood pressure is 155/95 mmHg, and respirations are 12/min. On physical exam, his skin has the finding shown in Figure A. Fine, lace-like white lines are also noted on the patient’s buccal mucosa. Which of the following is the most likely diagnosis?
Atopic dermatitis
Lichen planus
Scabies
Psoriasis
B
Lichen planus
This patient presents with several purple, polygonal, pruritic papules and plaques. These findings are most consistent with a diagnosis of lichen planus. Lichen planus is an inflammatory skin disorder most commonly characterized by an eruption of Purple, Polygonal, Planar, Pruritic, Papules and Plaques (the 6 P’s), often on extensor surfaces such as the wrist. Development of fine white lines on the surfaces of skin lesions, as well as on the buccal mucosa (Wickham striae), are also characteristically seen in this disease. Concurrent hepatitis C infection is a risk factor for lichen planus. A skin biopsy will show a sawtooth infiltrate of lymphocytes at the dermal-epidermal junction. Treatment is with antihistamines for pruritus and topical steroids for localized disease as well as addressing the underlying cause. Tziotzios et al. review the evidence regarding the diagnosis and treatment of lichen planus. They discuss how the molecular basis of this disorder is relatively unknown. They recommend making a clear diagnosis to allow for treatment. Figure/Illustration A shows multiple purple, polygonal, papules and plaques on the wrist (red circles). These findings are characteristic of lichen planus. Incorrect Answers: Answer A: Atopic dermatitis most commonly presents with pruritic, lichenified plaques on the flexural surfaces. The mucous membranes are not involved. Treatment is with topical moisturizers and antihistamines for pruritus. This disease is also associated with other atopic conditions such as asthma and seasonal allergies. Answer C: Prurigo nodularis is characterized by hypertrophic, dome-shaped papules that develop secondary to chronic scratching. The mucous membranes are not involved. Treatment is with avoidance of the triggering behavior such as scratching and antihistamines for pruritus. Answer D: Psoriasis is characterized by silver, scaly plaques that bleed easily when the scale is physically removed (Auspitz sign). Patients with cutaneous psoriasis are at increased risk for the development of psoriatic arthritis. Psoriasis can be treated with topical corticosteroids as well as calcipotriene. Answer E: Scabies is a cutaneous infection with the mite Sarcoptes scabei that presents with severe pruritus, especially in the intertriginous areas. Patients may have serpiginous scaling lesions that represent the burrowing of the mite through the skin, but mucous membrane lesions are absent. Treatment can include topical permethrin first line. Bullet Summary: Lichen planus presents with the 6 P’s – Purple, Pruritic, Polygonal, Planar, Papules and Plaques.
nan
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A 2-month-old girl is brought to the pediatrician by her mother for a follow-up visit. The patient was born at 29 weeks gestation to a G1P1 mother. Her postnatal course was complicated by a prolonged neonatal intensive care unit stay for neonatal respiratory distress syndrome (NRDS). Since discharge from the hospital, her mother reports that the patient has been doing well without health problems. She has been gaining weight appropriately. Her temperature is 98.6°F (37.0°C), blood pressure is 80/50 mmHg, pulse is 120/min, and respirations are 25/min. The patient's mother asks the pediatrician about potentially delaying the 2-month vaccine doses due to the baby's medical history. Which of the following is the correct vaccination plan for this infant in terms of the vaccinations below? - Diphtheria, Tetanus, and acellular Pertussis - DTaP - Hepatitis A - HAV - Hepatitis B - HBV - Haemophilus influenzae type B conjugate - HiB - Pneumococcal conjugate - PCV 13
Administer DTaP, HAV, HBV, HiB, PCV 13, poliovirus, and rotavirus vaccines
Administer DTaP, HBV, HiB, PCV 13, and poliovirus vaccines
Administer DTaP, HBV, HiB, PCV 13, poliovirus, and rotavirus vaccines
Delay all vaccines for 2 months due to the infant's history of NRDS
C
Administer DTaP, HBV, HiB, PCV 13, poliovirus, and rotavirus vaccines
This 2-month-old girl has a history of prematurity (born at 29 weeks gestation) complicated by a NICU stay for NRDS but is healthy at this time. It is recommended that she receive all vaccines (DTaP, HBV, HiB, PCV 13, poliovirus, and rotavirus vaccines) as scheduled during this visit. Premature infants can safely be vaccinated on the regular schedule against serious infections. A history of both prematurity and respiratory disease makes a patient more at risk for these serious infections, increasing the importance of vaccinations. The regular vaccine schedule recommends the following vaccines at 2 months of age: DTaP (inactivated), HBV (subunit), HiB (inactivated), PCV 13 (inactivated), poliovirus (inactivated), and rotavirus (live-attenuated). The first dose of the HBV (subunit) vaccine is still given during the first 24 hours of life unless the child is born with a birth weight of < 2000 grams (4 pounds 6 ounces). Saari et al. discuss the recommendations of the American Academy of Pediatrics Committee on Infectious Diseases for the immunization of preterm infants. The authors find that preterm infants are less likely to receive recommended vaccines on time. The authors recommend also administering the influenza vaccine beginning at 6 months of age during the influenza season. Incorrect Answers: Answer A: Administering DTaP, HAV, HBV, HiB, PCV 13, poliovirus, and rotavirus vaccines is incorrect as this answer choice includes the vaccine for the hepatitis A virus. The first dose of this vaccine is given at 12-24 months of age. Answer B: Administering DTaP, HBV, HiB, PCV 13, and poliovirus vaccines is incorrect as this answer choice is missing the rotavirus vaccine. Despite being premature and having a history of NRDS, live vaccines are safe to administer on schedule to this infant. Answer D: Delaying all vaccines for 2 months due to the infant's history of NRDS is incorrect as while this child has a history of NRDS, she is currently healthy. It is safe to administer all vaccines on the regular schedule to this patient. Answer E: Delaying all vaccines for 2 months because the infant was born 2 months premature is incorrect as it is not recommended that previously premature infants, with or without a perinatal complication such as NRDS, delay vaccines to account for the discrepancy between gestational and chronologic age. Bullet Summary: Children born with a history of prematurity, with or without health complications in their early life secondary to their prematurity, should receive all recommended vaccines as scheduled.
Delay all vaccines for 2 months because the infant was born 2 months premature
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A 1-year-old girl is brought to a neurologist due to increasing seizure frequency over the past 2 months. She recently underwent a neurology evaluation which revealed hypsarrhythmia on electroencephalography (EEG) with a mix of slow waves, multifocal spikes, and asynchrony. Her parents have noticed the patient occasionally stiffens and spreads her arms at home. She was born at 38-weeks gestational age without complications. She has no other medical problems. Her medications consist of lamotrigine and valproic acid. Her temperature is 98.3°F (36.8°C), blood pressure is 90/75 mmHg, pulse is 94/min, and respirations are 22/min. Physical exam reveals innumerable hypopigmented macules on the skin and an irregularly shaped, thickened, and elevated plaque on the lower back. Which of the following is most strongly associated with this patient's condition?
Cardiac rhabdomyoma
Glaucoma
Optic glioma
Polyostotic fibrous dysplasia
A
Cardiac rhabdomyoma
This patient with a seizure disorder, ash-leaf spots (innumerable hypopigmented macules), Shagreen patch (elevated irregular plaque on the lower back), and West syndrome (hypsarrhythmia on EEG and movements consistent with infantile spasms) likely has tuberous sclerosis. Tuberous sclerosis is associated with cardiac rhabdomyomas. Tuberous sclerosis is a neurocutaneous disorder that is inherited in an autosomal dominant fashion. Mutations in TSC1 and TSC2 lead to unregulated cell growth, leading to the formation of hamartomas in various locations. Manifestations of tuberous sclerosis include seizures (secondary to subependymal nodules and cortical dysplasia), mental retardation, renal angiomyolipomas, facial angiofibromas, mitral regurgitation, and hypomelanotic macules (ash-leaf spots). Cardiac rhabdomyoma is strongly associated with tuberous sclerosis and can lead to arrhythmias. West syndrome, which is characterized by infantile spasms, intellectual disability, and hypsarrhythmia (a chaotic mixture of high-amplitude slow waves, multifocal spikes, and intrahemispheric-interhemispheric asynchrony) on EEG, is also associated with tuberous sclerosis. Diagnosis of tuberous sclerosis is made with genetic testing. Advanced imaging (CT or MRI) may reveal cortical tubers and lesions in the third and fourth ventricles. Abdominal imaging may reveal renal angiomyolipomas. The management of seizures in tuberous sclerosis includes anti-seizure medications, though surgery may also be pursued in select cases. Hinton et al. studied the cardiac manifestations of tuberous sclerosis. They found that cardiac rhabdomyomas can lead to arrhythmia later in life. They recommended that infantile spasms in tuberous sclerosis be treated with vigabatrin but other seizure types should be treated similar to other epileptic disorders. Incorrect Answers: Answer B: Glaucoma may occur in patients with Sturge-Weber syndrome, which is characterized by "tram-track" calcifications in the brain, port-wine stains of the face, mental retardation, and epilepsy. Diagnosis is with MRI of the brain with contrast to demonstrate leptomeningeal vascular malformations that are characteristic of the disease. Answer C: Optic glioma is associated with neurofibromatosis type 1 (NF1), which presents with hyperpigmented macules (cafe-au-lait spots), axillary freckling, and cutaneous neurofibromas. Neurofibromas in NF1 may also grow on peripheral nerves, leading to malignant peripheral nerve sheath tumors. Lisch nodules, or benign growths of the iris, may also be seen in NF1. Diagnosis of NF1 is clinical, but can be aided by genetic testing. Answer D: Polyostotic fibrous dysplasia is associated with McCune-Albright syndrome. Polyostotic fibrous dysplasia causes fibrous tissue in bones, leading to growth abnormalities and pathologic fractures. McCune-Albright syndrome is also associated with cafe-au-lait macules, which have irregular borders, and endocrine abnormalities such as precocious puberty and hyperthyroidism. Diagnosis is supported with endocrine tests as well as genetic testing. Answer E: Renal cell carcinoma is associated with Von-Hippel-Lindau syndrome (VHL), which presents with tumors arising in multiple organs. Patients with VHL present with hemangioblastomas in the brain, spinal cord, cerebellum, and retina, and pheochromocytoma. Pheochromocytomas release catecholamines, leading to episodic hypertension, diaphoresis, and palpitations. Diagnosis of VHL is with genetic testing. Bullet Summary: Tuberous sclerosis has numerous manifestations including seizures, angiofibromas, mitral regurgitation, renal angiomyolipoma, ash-leaf spots, mental retardation, and cardiac rhabdomyoma.
Renal cell carcinoma
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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?
Rinse the finger in normal saline, wrap the finger in moist gauze, put in a plastic bag, and place the bag in ice water
Wrap the finger in moist gauze and place in a plastic bag
Submerge the finger in ice water
Rinse the finger with hydrogen peroxide, wrap the finger in moist gauze, and place on ice
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
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.
nan
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A 3-week-old girl presents with her parents to her pediatrician for a routine visit. The child is sleeping at regular 2-hour intervals and feeding and stooling well. The parents have noticed a “swelling of the belly button.” Cord separation occurred at 7 days of age. The swelling seems to come and go but is never larger than the size of a blueberry. They deny any drainage from the swelling. Her temperature is 98.6°F (37°C), blood pressure is 68/43 mmHg, pulse is 128/min, and respirations are 32/min. On physical exam, the child is in no acute distress and appears developmentally appropriate for her age. Her abdomen is soft and non-tender with a soft, 1 cm bulge at the umbilicus. The bulge increases in size when the child cries and can be easily reduced inside the umbilical ring without apparent pain. Which of the following is the most appropriate next step in management?
Elective surgical management
Expectant management
Histopathologic evaluation
Immediate surgical management
B
Expectant management
This infant presents with a soft, 1 cm bulge at the umbilicus that is easily reducible inside the umbilical ring, which suggests a diagnosis of an umbilical hernia. Most umbilical hernias in children resolve spontaneously and can be managed expectantly. An umbilical hernia is a protrusion of bowel, omentum, or preperitoneal fat through the umbilical ring that can change in size with increases in intra-abdominal pressure (e.g., crying). The majority of umbilical hernias in patients under 5 years of age resolve spontaneously. The indications for surgery on umbilical hernias in that age group are limited to incarcerated hernias or hernias > 1.5 cm in diameter. Incarcerated hernias present with an irreducible mass that is associated with pain and erythema. Surgical closure may be considered in patients whose defects fail to close by the age of 5. Chirdan et al. review the evidence regarding the diagnosis and treatment of pediatric patients with an incarcerated umbilical hernia. They discuss how most patients with hernias resolve spontaneously before 5 years of age but a small number will have incarcerated hernias that require surgery earlier. They recommend active observation of patients to diagnose incarceration earlier. Incorrect Answers: Answer A: Elective surgical management later on in childhood is usually not required to manage umbilical hernias as most umbilical hernias in patients less than 5 years of age close spontaneously. Hernias that persist past age 5 can be closed surgically in an elective fashion. Answer C: Histopathologic evaluation would be the most appropriate next step in management if the patient had an unknown umbilical mass, such as an umbilical granuloma or polyp. Umbilical granulomas form shortly after birth from excess tissue at the base of the umbilicus and can be treated topically, whereas umbilical polyps are firm masses of omphalomesenteric duct or urachal embryologic remnants and require surgical excision. Answer D: Immediate surgical management is required in patients with incarcerated hernias, but this patient’s hernia is easily reducible and < 1.5 cm. Incarcerated hernias present with an irreducible mass that is associated with pain and erythema. Answer E: Thyroid hormone replacement would be indicated in newborns that suffer from hypothyroidism which could present with an umbilical hernia. This patient has no other symptoms of hypothyroidism such as dry skin, hypoactive reflexes, constipation, poor muscle tone, and coarse facial features. Bullet Summary: Umbilical hernias are common in children and usually resolve spontaneously in children under 5 years of age.
Thyroid hormone replacement
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A 36-year-old woman, G1P1001, presents to her gynecologist for an annual visit. She has a medical history of hypertension for which she takes hydrochlorothiazide. The patient’s mother had breast cancer at age 68, and her sister has endometriosis. She states that she feels well and has no complaints. Her temperature is 98.6°F (37.0°C), blood pressure is 138/74 mmHg, pulse is 80/min, and respirations are 13/min. Her BMI is 32.4 kg/m^2. A pelvic exam reveals a non-tender, 12-week-size uterus with an irregular contour. A transvaginal ultrasound is performed and demonstrates two intramural leiomyomas. Which of the following is a likely complication of this patient’s condition?
Amenorrhea
Endometrial cancer
Iron deficiency anemia
Uterine prolapse
C
Iron deficiency anemia
This patient presents with an irregularly enlarged uterus on examination consistent with the intramural leiomyomas (fibroids) seen on ultrasound. Leiomyomas increase the risk of iron deficiency anemia due to heavy menstrual bleeding. Leiomyoma are benign overgrowths of myometrial tissue. The most common presentation is heavy or prolonged menstrual bleeding or symptoms related to mass effect such as pelvic pressure or pain.. Affected women often develop these symptoms in their 30s or 40s, though they may occur at any age, and many are asymptomatic. Risk factors include early menarche, obesity, family history, and African American race. Submucosal fibroids that disrupt the endometrium may cause heavy menstrual bleeding. Intramural fibroids also commonly cause heavy bleeding despite the fact that they do not disrupt the endometrium due to alterations in the uterine vasculature. Patients may be managed medically with a wide range of options including oral contraceptive pills, tranexamic acid, and GnRH agonists. Definitive management is with surgical intervention. Myomectomy is preferred for patients that desire child bearing in the future. For those that do not have plans for pregnancy in the future, hysterectomy is recommended. Yang et. al review the diagnosis and management of uterine fibroids. They note the range of clinical presentations to include heavy menstrual bleeding. They discuss management to include surgical intervention with myomectomy or hysterectomy. Incorrect Answers: Answer A: Amenorrhea, or the absence of menstrual bleeding, is often due to anovulation or uterine adhesions. Uterine fibroids are not associated with amenorrhea. Answer B: Endometrial cancer is a known cause of heavy menstrual bleeding. However, this patient’s ultrasound notes the presence of intramural leiomyomas. Her age, history, and ultrasound findings are not suggestive of endometrial cancer. Answer D: Uterine prolapse is associated with multiparity, chronically increased intra-abdominal pressure (from chronic obstructive pulmonary disease, obesity, etc.), and connective tissue diseases. While fibroids rarely may prolapse, they typically do not cause the entire uterus to prolapse. Answer E: Uterine sarcoma may very rarely result from uterine fibroids, as both arise from the uterine smooth muscle. However, this patient’s history and ultrasound findings are more suggestive of uterine leiomyoma. Bullet Summary: Patients with uterine leiomyomata (fibroids) are at risk for iron deficiency anemia due to heavy bleeding.
Uterine sarcoma
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A 32-year-old man presents to his primary care provider for a headache. He reports that he has headaches at night several times a week. He first developed these headaches over 1 year ago, but they started up again 3 weeks ago. The episodes start suddenly and feel like a stabbing, electrical pain over his left eye. He also reports tearing of the left eye during these episodes. The headaches self-resolve over the course of 2-3 hours, but the patient complains that he is avoiding going to sleep for fear of waking up in pain. His medical history includes type 1 diabetes mellitus and an episode of herpes zoster on his right flank 1 year ago. His only home medication is insulin. His temperature is 98.6°F (37°C), blood pressure is 112/69 mmHg, pulse is 61/min, and respirations are 14/min. On physical exam, his extraocular muscles are intact and his eyes are not injected. A CT of the head and sinuses shows no acute abnormalities. Which of the following is most likely to prevent future episodes of headache in this patient?
Carbamazepine
High-flow oxygen
Sumatriptan
Topiramate
E
Verapamil
This patient presents with an episodic, stabbing pain in the periorbital region that occurs at night, which is consistent with a diagnosis of cluster headaches. Verapamil is used as prophylaxis against cluster headaches. Cluster headaches usually occur at night and may wake patients out of sleep. Cluster headaches may occur several times a week to multiple times daily and the attacks may be followed by a period of remission. Cluster headaches also typically involve the periorbital region and are unilateral. Patients also classically describe their headaches as “stabbing”. Tension-type headaches are more commonly described as dull and migraines are usually described as pulsating or throbbing. Treatment is with high-flow oxygen during acute episodes and prophylaxis against long-term headaches includes calcium channel blockers such as verapamil. Hoffman and May review the evidence regarding the treatment of cluster headaches. They discuss how high-flow oxygen and triptans are the most effective abortive treatment for acute attacks. They recommend using chronic prophylactic medications such as verapamil. Incorrect Answers: Answer A: Carbamazepine is the treatment of choice for trigeminal neuralgia, which presents as shooting pain in the face. They have exquisite tenderness to palpation over the affected neurologic distribution. Answer B: High-flow oxygen is an abortive treatment for cluster headaches. Cluster headaches typically present in men with unilateral pain around the eye that is worse at night. Associated symptoms include spasms of the unilateral face as well as tearing and visual abnormalities in the eye. This treatment is not effective for long-term prophylaxis. Answer C: Sumatriptan is used as an abortive treatment for cluster headaches and migraines, but it is not used as prophylaxis for either. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Answer D: Topiramate is used as prophylaxis for migraines rather than cluster headaches. Migraines present as unilateral, throbbing headaches that may be associated with nausea and/or vomiting, photophobia, phonophobia, and aura. Bullet Summary: Cluster headaches classically present as sharp or stabbing periorbital headaches that occur at night, with verapamil being the prophylactic treatment of choice and high-flow oxygen being the most effective abortive treatment.
Verapamil
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A 14-year-old boy presents to his pediatrician with a 2-month history of intermittent arm and leg spasms. He says that the spasms usually occur after he wakes up as he is getting ready for school, and consist of disorganized jerking motions that stop after a few minutes. He has also noticed that they occur when he stays up late to cram last minute for exams. He has not noticed any other symptoms. He has no medical history and takes no medications. He recently started experimenting with alcohol and marijuana that one of his friends has been stealing from their parents. His temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. Physical exam reveals a healthy child. Which of the following is the most likely cause of this patient's symptoms?
Absence seizures
Alcohol intoxication
Cannabinoid use
Juvenile myoclonic epilepsy
D
Juvenile myoclonic epilepsy
This patient who presents with intermittent jerking of his extremities that occur primarily after waking up or when sleep-deprived most likely has juvenile myoclonic epilepsy. Juvenile myoclonic epilepsy (JME) is a poorly understood condition that presents with intermittent myoclonic jerks of the extremities. These myoclonic jerks usually have an onset occurring in early adolescence in otherwise healthy children. Myoclonic jerks are most frequently seen right after waking from sleep, but they may also be triggered by lack of sleep, stress, or alcohol consumption. Patients with this disease rarely have associated symptoms but may occasionally also experience absence seizures or generalized tonic-clonic seizures. The diagnosis can be secured with a history of seizures alongside an interictal electroencephalogram (EEG) that shows 4 to 6 Hz bilateral polyspikes. Valproate is the first-line treatment. Johannessen et al. discuss the treatment of juvenile myoclonic epilepsy. They find that standard antiepileptic treatment with agents such as valproate leads to only 33% of patients remaining seizure-free. They recommend trials of alternative antiepileptic medications such as levetiracetam, lamotrigine, and topiramate in patients that do not respond to valproate. Incorrect Answers: Answer A: Absence seizures commonly present during childhood in otherwise healthy children; however, these seizures are typically characterized by short episodes of loss of awareness known as "staring spells" without physical manifestations. Answer B: Alcohol intoxication is a risk factor for precipitating juvenile myoclonic epilepsy, but alcohol use does not cause this condition. Seizure activity may also occur during alcohol withdrawal, but the timing of this patient's symptoms is more suggestive of seizure symptoms. Alcohol withdrawal requires chronic drinking of large amounts leading to an increase in central nervous system N-methyl-D-aspartate receptors and a decrease in CNS gamma-aminobutyric acid type A receptors. When alcohol cessation occurs, seizures ensue. Answer C: Cannabinoid use is not associated with seizure activity either during intoxication or withdrawal. Patients may have altered mental status, but this does not manifest as myoclonic jerks. Answer E: West syndrome is an infantile seizure disorder that usually presents between 3 months and 12 months of age. These patients will present with recurrent flexion spasms of the extremities. Bullet Summary: Juvenile myoclonic epilepsy presents in adolescents with recurrent spasms after waking from sleep or when sleep-deprived.
West syndrome
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A 23-year-old woman is admitted to the inpatient psychiatry unit after her boyfriend reported she was “acting funny and refusing to talk.” The patient’s boyfriend states that he came home from work and found the patient sitting up in bed staring at the wall. When he said her name or waved his hand in front of her, she did not respond. When he tried to move her, she would remain in whatever position she was placed. Her temperature is 99°F (37.2°C), blood pressure is 122/79 mmHg, pulse is 68/min, and respirations are 12/min with an oxygen saturation of 98% on room air. The patient is lying on the bed with her left arm raised and pointing at the ceiling. She resists any attempt to change her position. The patient remains mute and ignores any external stimuli. The patient’s medical history is significant for depression. She was recently switched from phenelzine to fluoxetine. Which of the following is the most appropriate initial therapy?
Benztropine
Cyproheptadine
Electroconvulsive therapy
Haloperidol
E
Lorazepam
The patient has a history of depression and is presenting with decreased motor activity, waxy flexibility, lack of response to external stimuli, and mutism, suggesting the diagnosis of catatonia. Catatonia should initially be treated with benzodiazepines like lorazepam. Catatonia is a state of psychomotor immobility and apathy that is associated with a mood disorder or psychosis. The diagnosis is made clinically by having at least 3 of the following symptoms: stupor (no psychomotor activity), negativism (no response to external stimuli), catalepsy (muscle rigidity in a position against gravity), waxy flexibility (maintaining positioning placed by the examiner), mutism, echolalia (mimicking speech), echopraxia (mimicking movements), stereotypy, grimacing, and non-purposeful agitation or mannerisms. Initial treatment involves benzodiazepines for symptomatic relief. If the patient is unresponsive to benzodiazepines, electroconvulsive therapy (ECT) can be used. Francis reviews the evidence regarding the treatment of patients with catatonia. He discusses how benzodiazepines can be used with rapid improvement in symptoms. He recommends electroconvulsive therapy in refractory cases. Incorrect Answers: Answer A: Benztropine and IV diphenhydramine can be used to treat an acute dystonic reaction. Dystonic reactions present as involuntary spasms or sustained contractions of the face, neck, torso, pelvis, extremities, and larynx. Medications that block dopamine D2 receptors, such as neuroleptics and antiemetics, carry the greatest risk. Answer B: Cyproheptadine is a serotonin antagonist that may be part of the treatment for serotonin syndrome. Serotonin syndrome may occur as a result of the simultaneous use of monoamine oxidase inhibitors (MAOIs), selective serotonin reuptake inhibitors (SSRIs), or serotonin-norepinephrine reuptake inhibitors (SNRIs). It presents with myoclonus, hyperthermia, flushing, tachycardia, and delirium. Answer C: Electroconvulsive therapy is an effective treatment for catatonia but would be considered 2nd line to benzodiazepines for refractory cases. Answer D: Haloperidol is an antipsychotic, which may worsen catatonia. It can cause neuroleptic malignant syndrome, which can mimic catatonia. Bullet Summary: Catatonia can present in patients with a history of mood disorders and psychosis with symptoms of decreased motor activity, rigid posture, and mutism, and should be treated initially with a benzodiazepine.
Lorazepam
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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?
Lateral medulla
Lateral pons
Posterior limb of internal capsule
Midbrain
D
Midbrain
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.
nan
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A 55-year-old man presents to urgent care for weakness and weight loss. For the past several months, he has felt progressively weaker and has lost 25 pounds with intermittent abdominal pain. The patient has not seen a physician in 30 years and recalls being current on most of his vaccinations. A few years ago, he went to the emergency department due to abdominal pain and was found to have increased liver enzymes due to excessive alcohol use and incidental gallstones. The patient has a 50 pack-year smoking history. His temperature is 99.5°F (37.5°C), blood pressure is 161/108 mmHg, pulse is 90/min, respirations are 17/min, and oxygen saturation is 95% on room air. Physical exam reveals an emaciated man. The patient has a negative Murphy sign and his abdomen is non-tender. Cardiopulmonary exam is within normal limits. Which of the following is the most appropriate next step in management?
CT scan of the abdomen and pelvis
CT scan of the chest
HIDA scan
Smoking cessation advice and primary care follow up
A
CT scan of the abdomen and pelvis
This patient with weight loss and painless liver dysfunction has symptoms that are suggestive of pancreatic cancer. The most appropriate next diagnostic test is a CT scan of the abdomen and pelvis. Pancreatic cancer presents with weight loss, jaundice, epigastric pain, and fatigue/malaise typically in an older male smoker who uses alcohol. Laboratory tests will demonstrate an elevated alkaline phosphatase and bilirubin. If a patient is presenting with the classic "painless jaundice" then the most appropriate next step in management could be an ultrasound to evaluate for obstruction. Otherwise, the best diagnostic test for this condition is a CT scan of the abdomen to characterize the pancreas and any metastasis to local organs. Treatment is with excision of the tumor through a Whipple procedure in appropriate candidates. Lee and Lee review the evidence regarding the diagnosis of pancreatic cancer. They discuss how abdominal CT or MRI are important in making this diagnosis. They recommend having a high clinical index of suspicion given this diagnosis carries a high mortality rate. Incorrect Answers: Answer B: CT scan of the chest would be appropriate to screen a smoker for lung cancer. If this patient had concern for malignancy with a smoking history, then a CT scan of the chest would be appropriate. It would be appropriate at a later time to assess for pulmonary nodules and lung cancer. Answer C: HIDA (hepatobiliary iminodiacetic acid) scan is an appropriate diagnostic test to characterize a gallstone blockage of the common bile duct if a right upper quadrant ultrasound is not revealing in the diagnosis of acute cholecystitis. Answer D: A right upper quadrant ultrasound can assess the gallbladder for stones and the liver for an abscess, but it will not assess the pancreas for pancreatic cancer (at least in a reliable manner that assesses the entire pancreas). Answer E: Smoking cessation and primary care follow up would be appropriate management for this patient if he only presented requesting general advice to improve his health or stop smoking. His current symptoms are too alarming for malignancy. He should stop smoking regardless. Bullet Summary: The most appropriate initial test for pancreatic cancer is an abdominal CT.
nan
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A 23-year-old woman presents to the emergency room for a self-inflicted laceration of her distal volar forearm. The patient states she knew her boyfriend was having sexual thoughts about the woman from the grocery store, prompting her decision to cut her own wrist. In the emergency department, the bleeding has stopped and the patient is currently medically stable. When interviewing the patient, she is teary and apologizes for her behavior. She is grateful to you for her care and regrets her actions. Of note, the patient has presented to the emergency department before for a similar reason when she was struggling with online dating. The patient states that she struggles with her romantic relationships though she deeply desires them. Her temperature is 98.6°F (37°C), blood pressure is 112/71 mmHg, pulse is 73/min, and respirations are 14/min. On physical exam, you note a frightened young woman who is wearing a revealing dress that prominently displays her breasts. You tell the patient that she will have to stay in the psychiatric emergency department for the night which makes her furious. Which of the following personality disorders is the most likely diagnosis?
Antisocial
Avoidant
Borderline
Dependent
C
Borderline
This patient is presenting with a suicide attempt, unstable relationships, and emotional lability suggesting a diagnosis of borderline personality disorder. Borderline personality disorder classically presents with emotional lability, and intense, unstable relationships. These patients fear abandonment and will often engage in self-harming behavior like cutting and suicide attempts. The main defense mechanism these patients employ is splitting, in which patients divide the world into dichotomous categories where people are either all good or all bad. This personality disorder is associated with mood disorders and substance use. The treatment of choice for these patients is long-term dialectical behavioral therapy and mood stabilizers. Leichsenring et al. review the evidence regarding the treatment of patients with borderline personality disorder. They discuss no specific form of psychotherapy is superior to others. They recommend starting any form of psychotherapy for these patients to improve outcomes. Incorrect Answers: Answer A: Antisocial personality disorder presents with aggressive, criminal behavior. These individuals do not conform to social norms or rules and violate the rights of others. Patients often are not amenable to behavioral modification and therefore incarceration may be necessary in order to protect the rights of others. Answer B: Avoidant personality disorder presents with excessive shyness and hypersensitivity to rejection. These patients fear social interaction though they strongly desire relationships. Cognitive behavioral therapy can be used in order to encourage these patients to be more open to seeking out relationships. Answer D: Dependent personality disorder presents with submissive and clingy behavior. They often have a need to be cared for and will be very agreeable and dependent on others. This can be harmful as patients may stay in abusive or degrading relationships due to a fear of being alone. Treatment is with cognitive behavioral therapy. Answer E: Histrionic personality disorder presents with colorful and exaggerated attention-seeking behavior. These patients will often employ sexuality to draw attention to themselves. Though this patient is displaying her breasts and wearing seductive clothing, her history of self-harm behavior and unstable relationships suggests a diagnosis of borderline personality disorder. Bullet Summary: Borderline personality disorder presents with emotional instability, unstable relationships, and self-harming behavior.
Histrionic
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A 69-year-old man presents to clinic due to shortness of breath, worsening pain in his right shoulder, and episodes of hemoptysis. His symptoms began 3 months ago. He has also lost 18 pounds. He has a history of coronary artery disease and underwent an uncomplicated coronary angioplasty with stent placement 2 years ago. He routinely travels both domestically and internationally as a senior shipyard worker and has a 30-pack-year smoking history. He used to drink 4 cocktails a week. He has not smoke or drank alcohol in over 7 years. His temperature is 99.5°F (37.7°C), blood pressure is 140/60 mmHg, pulse is 97/min, and respirations are 13/min. Physical exam is notable for right pupillary constriction as well as paresthesias in his right fourth and fifth digits. There is no pain with active shoulder rotation. His chest imaging is shown in Figure A. Which of the following is most likely responsible for the patient’s symptoms?
Lung adenocarcinoma
Mesothelioma
Rotator cuff tendinopathy
Tuberculosis
A
Lung adenocarcinoma
This elderly patient with an extensive smoking history is presenting with hemoptysis, weight loss, and shortness of breath. Combined with neurological findings in the ulnar nerve distribution (paresthesias in fourth and fifth digits) and imaging evidence of a superior right lung mass, the most likely diagnosis is a Pancoast tumor, which is most commonly a lung adenocarcinoma. A Pancoast tumor is a peripheral lung cancer often caused by lung adenocarcinoma or squamous cell carcinoma and is often located in the superior sulcus of the lung. The most important risk factor is smoking. This tumor causes compression of adjacent structures. Patients can present with severe, localized axilla pain (due to invasion of the brachial plexus), weakness of intrinsic hand muscles with radicular pain and paresthesias in the fourth and fifth digits (due to invasion of the C8-T1 portion of the brachial plexus), Horner syndrome (miosis, ptosis, and facial anhidrosis due to invasion of the cervical sympathetic nerves), hoarseness (due to invasion of the recurrent laryngeal nerve), and edema of the arm with facial swelling (due to compression of the superior vena cava). Treatment is varied and involves a combination of radiation, chemotherapy, and surgery. Wu et al. studied the role of the tyrosine-kinase inhibitor, osimertinib, in the treatment of completed resected epidermal growth factor receptor (EGFR)-mutated non-small cell lung cancer (NCSLC). The authors find that 89% of patients in the osimertinib group and 52% of the placebo groups were alive and disease free at 24 months. The authors recommend the use of osimertinib in patients with stage IB to IIIA EGFR-mutated NSCLC. Figure/Illustration A demonstrates a right apical lung mass found on computed tomography (CT) scan (green arrow). Incorrect Answers: Answer B: Mesothelioma is a neoplasm of the pleura found in people with occupational exposures to asbestos, such as shipyard workers. While this neoplasm can present with hemoptysis and dyspnea, chest imaging of mesothelioma reveals pleural plaques and thickening, not an isolated apical lung lesion. Answer C: Rotator cuff tendinopathy presents with pain with abduction and external rotation of the arm. This patient has no pain with active shoulder rotation. Answer D: Syringomyelia is a cyst or cavity in the spinal column that can cause Horner syndrome and fluctuating radicular pain. It is unlikely to cause hemoptysis, weight loss, and the chest imaging findings in this patient. Answer E: Tuberculosis is a possible diagnosis in this patient with hemoptysis, weight loss, and extensive travel history. Tuberculosis often presents with a fever and does not usually cause shoulder pain. Bullet Summary: Pancoast tumor presents with shoulder pain, cervical radiculopathy, Horner syndrome, and hoarseness and should be suspected in a patient with a smoking history and an apical lung mass on imaging.
nan
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A 25-year-old G1P0 woman presents to her physician for a prenatal visit. She is at 12 weeks gestational age and has not received prior prenatal care. She has mild morning sickness but no other symptoms. She has no known medical problems and takes only a prenatal multivitamin. She has unprotected sexual intercourse with 3 male partners. Her temperature is 98.6°F (37.0°C), blood pressure is 118/70 mmHg, pulse is 80/min, and respirations are 18/min. Physical exam reveals a well-appearing woman in no acute distress. There are no lesions appreciated on skin exam. Cardiopulmonary exam is unremarkable. An antibody test for human immunodeficiency virus (HIV)-1/2 is positive. The patient is started on an appropriate treatment regimen. Assuming that her HIV viral load is undetectable by the time of delivery, which of the following maternal regimens is she most likely receiving and what neonatal prophylaxis is indicated (Figure A)?
B
A
C
E
C
C
This pregnant patient presents with a positive HIV antibody test in the setting of unprotected sexual intercourse. An appropriate maternal HIV treatment regimen is abacavir, lamivudine, and raltegravir; prophylaxis in the infant consists of zidovudine alone if maternal HIV viral load is suppressed to < 50 copies/mL. All pregnant women should be screened for HIV as early as possible during pregnancy. Maternal treatment for HIV should be initiated as early as possible (before pregnancy if possible) with highly-active anti-retroviral therapy (HAART) consisting of 2 nucleoside reverse transcriptase inhibitors (NRTIs) such as abacavir/lamivudine, plus an integrase inhibitor (e.g., raltegravir) or a protease inhibitor (e.g., ritonavir/atazanavir). Regimens for prophylaxis in infants of HIV-positive mothers depends on the HIV viral load at the time of delivery. If the viral load is < 50 copies/mL, infant prophylaxis with zidovudine alone for 4 weeks is sufficient. If the viral load is ≥ 50 copies/mL or the mother did not receive HAART during pregnancy, infant prophylaxis should consist of the 3-drug regimen of: zidovudine, lamivudine, and either nevirapine or raltegravir for 6 weeks. Harris et al. reviewed the treatment of HIV infection in pregnancy. They found that although anti-retroviral therapy during pregnancy significantly reduces mother-to-child transmission of HIV, it is still associated with certain perinatal complications such as low birthweight and preterm delivery. Despite these risks, they recommend anti-retroviral therapy during pregnancy and during breastfeeding to prevent transmission. Figure A shows the answer choices. Incorrect Answers: Answer A: Maternal treatment with abacavir/lamivudine/raltegravir and no neonatal treatment is inappropriate because all infants of HIV-positive mothers should receive zidovudine (if maternal viral load < 50 copies/mL at delivery). Lamivudine and raltegravir would be added if maternal viral load is ≥ 50 copies/mL at delivery. Answer B: Maternal treatment with abacavir/lamivudine/raltegravir and neonatal prophylaxis with zidovudine/lamivudine/raltegravir would be appropriate if the maternal viral load were ≥ 50 copies/mL at delivery. The HIV viral load in this patient was assumed to be undetectable at delivery. Answer D: Maternal treatment with raltegravir/ritonavir/atazanavir and neonatal prophylaxis with zidovudine/lamivudine/raltegravir is inappropriate because raltegravir is an integrase inhibitor and ritonavir/atazanavir are protease inhibitors. HAART must include 2 NRTIs. Atazanavir is often co-administered with ritonavir to increase the former’s bioavailability. Additionally, zidovudine/lamivudine/raltegravir is the neonatal regimen for infants whose mothers have a viral load ≥ 50 copies/mL at delivery. Answer E: Maternal treatment with raltegravir/ritonavir/atazanavir and neonatal prophylaxis with zidovudine only is inappropriate because HAART for the mother must include 2 NRTIs. Bullet Summary: Maternal HIV infection should consist of highly-active anti-retroviral therapy consisting of 2 nucleoside reverse transcriptase inhibitors plus either an integrase inhibitor or protease inhibito, while neonatal prophylaxis consists of zidovudine only for infants of mothers with viral load < 50 copies/mL at delivery.
nan
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A 30-year-old man presents to the emergency department with diarrhea. He states he has had profuse, watery diarrhea for the past 24 hours. He notes that eating and drinking make him defecate more, thus he has been refraining from drinking. He is otherwise healthy and takes no medications. His temperature is 97.7°F (36.5°C), blood pressure is 84/64 mmHg, pulse is 130/min, respirations are 17/min, and oxygen saturation is 99% on room air. Physical exam reveals dry mucous membranes with an otherwise benign exam. The patient is given 1L of normal saline and drinks 1L of oral fluids. His blood pressure is subsequently 120/70 mmHg and pulse 95/min. Which of the following is the most appropriate next step in management?
Ciprofloxacin
Rest and oral rehydration
Stool culture
Stool ova and parasite study
B
Rest and oral rehydration
This patient is presenting with a likely diarrheal illness given he has profuse, watery diarrhea and his vitals normalized with oral and IV hydration. Since the patient is young and healthy, oral hydration and rest are all that is needed for management. Diarrheal illness is a common chief complaint with variable workups pending on the patient's clinical status. Young, healthy, non-immunosuppressed patients who can tolerate an oral diet require only reassurance and oral hydration, as most cases of viral and bacterial diarrhea will resolve on their own. Moreover, most cases of diarrhea are caused by viral etiologies which simply require supportive therapy. This is even true in bacterial diarrhea as long as the patient's vitals normalize and they can tolerate an oral diet. It is important to consider inflammatory causes of diarrhea (such as ulcerative colitis or Crohn disease) as this would change the workup and management. Schiller et al. discuss different types of diarrhea. They review the management and diagnosis of diarrhea and its different forms, including chronic forms. They recommend noting the difference between different causes and working up and treating them appropriately. Incorrect Answers: Answer A: Ciprofloxacin is an antibiotic that can be used for bacterial/infectious diarrhea. It should not be given in young, healthy patients and would only be indicated in the setting of bacterial diarrhea in ill patients, patients with persistent symptoms, or patients who are immunosuppressed. Bacterial diarrhea would present with fever, abdominal pain, and bloody/purulent diarrhea. Answer C: Stool culture should not be empirically performed in young, well-appearing patients. It may be performed in hospitalized patients who are more ill or in patients who are going to start antibiotic therapy. It may also be performed in patients with persistent diarrhea. Answer D: A stool ova and parasite study would not be performed unless there was a suggestive history or exposure (such as drinking unfiltered water) in the setting of fatty, foul-smelling diarrhea. This would raise suspicion for Giardia lamblia which would be treated with metronidazole. Answer E: Stool PCR for viral etiologies of diarrhea is not useful as the management of viral diarrhea is supportive therapy in most cases. On occasion, it may be performed in critically ill patients to determine the etiology of their symptoms. It should not be performed routinely. Bullet Summary: Healthy patients with limited diarrheal illness require supportive therapy only (in particular, oral hydration) and do not need more invasive diagnostic tests.
Stool PCR
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A 21-year-old man presents to the emergency room with right lower quadrant pain that started this afternoon at his wrestling match. He also endorses loss of appetite and subjective fever. He has had vague, mild discomfort in the right upper quadrant since the start of this wrestling season. He endorses a history of unprotected sex with multiple partners and intravenous drug use during a recent vacation to Mexico but has no other significant medical history. He drinks socially and has never smoked. His temperature is 98.6°F (37°C), blood pressure is 119/72 mmHg, pulse is 102/min, and respirations are 12/min. Physical exam demonstrates pain on palpation of the right lower quadrant and mild pain in the right upper quadrant. He has significant acne on his face, chest, and back. A CT of the abdomen confirms appendicitis. The scan also shows a large liver mass measuring 6 cm as seen in Figure A. Aside from addressing his appendicitis, which of the following is the most appropriate next step in management?
Administer metronidazole
Administer sofosbuvir
Obtain follow-up CT of the abdomen in 3 months
Perform liver mass resection
D
Perform liver mass resection
This patient with a history of possible anabolic steroid use (wrestler with acne) who presents with right upper quadrant pain and an enhancing lesion in the liver most likely has a hepatic adenoma. The treatment of hepatic adenomas is surgical removal and discontinuation of precipitating agents. Hepatic adenomas are benign epithelial tumors that typically arise in the right hepatic lobe. They are typically solitary masses but multiple lesions can be seen in patients with prolonged contraceptive or anabolic steroid use and those who have glycogen storage disorders. Patients present with abdominal pain that is localized in the epigastrium or in the right upper quadrant. Sudden, severe abdominal pain with hypotension suggests rupture of the hepatic adenoma into the peritoneum. The lesions can be confirmed on imaging such as ultrasound, CT, or MRI. For hepatic adenomas that are < 5 cm and asymptomatic, simply discontinuing the causative drug and observing are acceptable. Large or symptomatic lesions requiring surgical resection in addition to discontinuation of the offending agent. Because these tumors are highly vascular, failure to resect may result in rupture, hemoperitoneum, and shock. Biopsy is contraindicated to avoid rupturing the mass and causing hemorrhage. Klompenhouwer et al. studied the current management strategies in patients with hepatocellular adenoma. They found that various molecular groups have been identified allowing for subtyping of these lesions. They recommend performing a CT scan to better characterize these lesions. Figure/Illustration A is a CT with an enhancing mass with a hypoattenuating fatty component. This is characteristically seen in large hepatic adenomas, which are solitary well-marginated lesions. Incorrect Answers: Answer A: Metronidazole would be an effective treatment if this patient had contracted Entamoeba histolytica from contaminated food or water on his recent trip to Mexico. While this amoeba can lead to a liver abscess presenting as a mass on CT, it often also causes abdominal pain and dysentery due to intestinal involvement. Answer B: Sofosbuvir is an antiviral medication used for hepatitis C. This patient does have risk factors for hepatitis C (risky sexual behavior and intravenous drug use). Although hepatitis C could lead to hepatocellular carcinoma and cause a liver mass, this patient’s presentation is not consistent with that diagnosis. Even if he did have hepatocellular carcinoma, treating hepatitis C alone without surgical resection or adjuvant therapies would be insufficient. Answer C: Follow-up CT in 3 months would be dangerous for this man with a large hepatic adenoma. In the interim, the mass will likely continue to grow if he continues to use anabolic steroids, increasing the risk of rupture. Observation and repeat imaging may be suitable for asymptomatic hepatic lesions that are of uncertain but likely benign etiology. Answer D: Biopsying the liver mass is appropriate if malignancy is suspected and there is no concern for hepatic adenoma. If an adenoma is on the differential, biopsy is contraindicated due to the risk of rupture and hemorrhage intraoperatively. Bullet Summary: Hepatic adenomas are benign, vascular liver masses associated with oral contraceptives and anabolic steroids that should be promptly resected if large and symptomatic.
nan
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A 41-year-old man presents to his primary care provider with a 2-month history of abdominal pain. He says that the pain “comes and goes” throughout the day and usually lasts 20-30 minutes per episode. The pain is above his umbilicus. He denies any feeling of regurgitation or nighttime cough but endorses nausea. He used to eat 3 large meals per day but has found that eating smaller meals more frequently improves his pain. He tried ibuprofen with food and thinks it helped. He has gained 4 pounds since his last appointment 3 months ago. He denies any diarrhea or change in his stools. He has no known medical history. He drinks 5-6 beers on the weekend and has a 20 pack-year smoking history. He denies any family history of cancer. His temperature is 98.6°F (37°C), blood pressure is 117/72 mmHg, pulse is 72/min, and respirations are 13/min. On physical exam, he is tender to palpation above the umbilicus. Bowel sounds are present. A stool guaiac test is positive. The patient undergoes an endoscopy with a biopsy to diagnose his condition. Which of the following is most likely to be found on histology?
Crypt abscesses in the large intestine
Mucosal defect in the stomach
Noncaseating granulomas in the small intestine
Urease-producing organism in the small intestine
D
Urease-producing organism in the small intestine
This patient presents with episodic epigastric pain and nausea that improves with the consumption of food, which suggests a diagnosis of a duodenal ulcer. Duodenal ulcers are often caused by infection with Helicobacter pylori, a urease-producing organism. Duodenal ulcers are erosions and defects in the mucosal lining of the small intestines that allow for damage to the underlying tissues. Patients will classically present with abdominal pain that is localized to the epigastric region. Duodenal ulcers improve upon eating due to the secretion of bicarbonate in the small intestines, whereas gastric ulcers are exacerbated by food due to the release of gastric acid in the stomach. Duodenal ulcers are most commonly caused by infection with H. pylori. If the patient tests positive for H. pylori infection, treatment involves triple therapy with amoxicillin, clarithromycin, and a proton pump inhibitor. Patients who fail to improve should undergo esophagogastroduodenoscopy, which remains the gold standard for diagnosis. Ciociola et al. studied the prevalence of H. pylori infection in patients with gastric ulcers. They found that about many had confirmed infections. They recommend against empiric treatment without prior confirmation of infection. Incorrect Answers: Answer A: Crypt abscesses are found on histology in ulcerative colitis. Ulcerative colitis presents with bloody diarrhea and abdominal pain. Patients can also experience musculoskeletal pain, uveitis, and dermatologic findings such as erythema nodosum or pyoderma gangrenosum. Treatment may include mesalazine. Answer B: A mucosal defect in the stomach is the pathologic description of a gastric ulcer. This patient’s presentation of epigastric pain that improves with the consumption of food is more consistent with an ulcer in the duodenum. Treatment of a gastric ulcer may include proton pump inhibitors. Answer C: Noncaseating granulomas are found on histology in Crohn disease. This patient denies any diarrhea (often associated with pain), which is the primary feature of Crohn disease. Other findings include abscesses, fistulas, and strictures. Treatment may include mesalamine and infliximab. Answer D: PAS-positive material is found in the lamina propria on small intestinal biopsy in Whipple disease. In addition to abdominal pain, Whipple disease is characterized by diarrhea with flatulence and steatorrhea, fever, arthralgias, and weight loss. Treatment is with ceftriaxone or penicillin. Bullet Summary: Duodenal ulcers typically present with episodic epigastric abdominal that is improved after eating and are associated with H. pylori infection.
nan
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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?
Acute inflammatory demyelinating polyneuropathy
L5/S1 disc herniation
Spinal stenosis
Cauda equina syndrome
D
Cauda equina syndrome
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.
nan
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A 66-year-old woman presents to the emergency room after experiencing a tonic-clonic seizure. She has been having worsening headaches and intermittent nausea over the past 5 months. The headaches are constant and dull, and they typically worsen when she sneezes or laughs. Recently, she has become increasingly nauseous and has vomited twice in the past 10 days. Her medical history is significant for type 2 diabetes mellitus and hypertension for which she takes metformin and losartan. Her temperature is 98.6°F (37.0°C), blood pressure is 115/70 mmHg, pulse is 70/min, and respirations are 12/min. She is noted to have papilledema on fundoscopy, but physical exam is otherwise normal. Her CT scan findings are presented in Figure A. Which of the following is the most likely diagnosis?
Glioblastoma multiforme
Cerebral toxoplasmosis
Neurosarcoidosis
Hemorrhagic stroke
A
Glioblastoma multiforme
This patient is presenting with symptoms of increased intracranial pressure (i.e., positional headaches, nausea, vomiting, and papilledema). Combined with her CT finding of a space-occupying butterfly-shaped lesion, the most likely diagnosis is glioblastoma multiforme. Glioblastoma multiforme is the most common malignant primary nervous system cancer, most commonly occurring in adults above the age of 65. The tumor arises from the cerebral hemispheres and classically crosses the corpus callosum, producing a butterfly appearance on CT/MRI. Pseudopalasading necrosis is seen on biopsy, and tissue biopsy is usually glial fibrillary acidic protein (GFAP) positive. It has an unfavorable prognosis with a life expectancy of 6 months to 1 year. Management of glioblastoma includes surgical resection and palliative radiotherapy/chemotherapy. Stupp et al. studied the role of electrical field therapy (TTFields) in conjunction with temozolomide in the treatment of glioblastoma multiforme. The authors found that combination therapy improved median progression-free survival (6.7 vs. 4.0 months, p < 0.001). The authors recommend that clinicians consider the use of TTFields to treat selected patients with glioblastoma multiforme. Figure/Illustration A demonstrates a non-contrast axial head CT showing the classic butterfly lesion characteristic of glioblastoma multiforme (red arrow). Incorrect Answers: Answer A: Cerebral toxoplasmosis presents on imaging with multiple ring-enhancing lesions and is most common in patients with acquired immunodeficiency syndrome (AIDS), especially with CD4 T-cell counts of less than 100 cells/mm^3. This patient has no signs of infection or ring-enhancing lesions. Answer C: Hemorrhagic stroke presents acutely with hemiparesis, altered mental status, slurred speech, or sensorimotor symptoms. CT imaging would reveal a hemorrhage. Answer D: Metastatic brain tumor often presents with imaging findings of multiple well-circumscribed lesions with surrounding edema. These lesions are generally found at the highly vascularized gray-white junction. In a patient with no smoking history, a single brain lesion, and no other extra-cranial symptoms, this is a less likely diagnosis. Answer E: Neurosarcoidosis can be caused by infiltrative sarcoidosis and often presents with bitemporal hemianopsia due to infiltration of the pituitary gland. Patients with sarcoidosis typically also present with pulmonary symptoms (e.g., wheezing and coughing) which are absent in this patient. Bullet Summary: Glioblastoma multiforme should be suspected in patients presenting with symptoms of increasing intracranial pressure and classic CT/MRI findings of a butterfly shaped lesion with central necrosis.
nan
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A 45-year-old African American woman presents to her primary care physician for not feeling well. She states she has had a cough for the past week. She also has abdominal pain and trouble focusing that has been worsening and has lost 5 pounds recently. Her gastroesophageal reflux disease (GERD) has been poorly controlled recently. The patient is a non-smoker and has a history of GERD for which she takes antacids. Her temperature is 98.6°F (37°C), blood pressure is 115/71 mmHg, pulse is 72/min, and respirations are 12/min. A physical exam is within normal limits. Laboratory studies are ordered and are below: Serum: Na+: 139 mEq/L K+: 4.1 mEq/L Cl-: 101 mEq/L HCO3-: 24 mEq/L Urea nitrogen: 12 mg/dL Glucose: 70 mg/dL Creatinine: 0.9 mg/dL Ca2+: 12.5 mg/dL Alkaline phosphatase: 150 U/L Phosphorus: 2.0 mg/dL Urine: Color: amber Nitrites: negative Sodium: 5 mmol/24 hours Red blood cells: 0/hpf Which of the following is the most likely explanation of this patient's current presentation?
Increased parathyroid hormone
Malignancy
Viral illness
Antacid overuse
A
Increased parathyroid hormone
This patient is presenting with hypercalcemia and low phosphate levels suggesting a diagnosis of hyperparathyroidism. Hyperparathyroidism can cause hypercalcemia which typically presents with "bones, stones, moans, and groans," which corresponds to symptoms of bone pain (increased bone resorption), kidney stones, GI pain, and change in mental status/trouble focusing. The increased PTH results in renal phosphate wasting, increased production of 1,25-dihydroxy vitamin D, and increased renal calcium reabsorption. It also results in increased osteoclast activity resulting in increased bone breakdown. This will lead to laboratory changes of hypercalcemia and hypophosphatemia. Taniegra reviews the evidence regarding hyperparathyroidism. She discusses how this disease is usually discovered asymptomatically with routine blood work. She recommends surgical treatment with intractable cases as success rates are high. Figure A is a chest radiograph that is within normal limits. No masses are seen which suggests that malignancy is a less likely diagnosis. Incorrect Answers: Answer A: Antacid overuse for this patient's GERD can lead to a milk-alkali syndrome which presents with hypercalcemia and metabolic alkalosis. This patient does not have metabolic alkalosis. Treatment is with the discontinuation of the offending agent. Answer B: Increased 1,25-dihydroxy vitamin D occurs in sarcoidosis which presents with cough, fatigue, and weight loss in an African American woman. Increased vitamin D by itself will result in hypercalcemia, and hyperphosphatemia rather than hypophosphatemia. Treatment is supportive and/or with corticosteroids. Answer D: Malignancy (lung or renal cancer) is possible given this patient's presentation. Hypercalcemia of malignancy is a common feature with PTH-related protein effects; however, the patient's normal urinalysis and normal chest radiograph as well as non-smoking history point more toward a diagnosis of increased PTH. Answer E: Viral illness is possible given this patient's systemic symptoms; however, it does not explain this patient's hypercalcemia and hypophosphatemia. Treatment of seasonal viruses is generally supportive though specific viruses such as influenza have agents such as oseltamivir that may be helpful. Bullet Summary: Hyperparathyroidism presents with "bones, stones, moans, and groans," as well as laboratory findings of hypercalcemia and hypophosphatemia.
nan
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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?
Testicular ultrasound
Radiograph of the hand and wrist
Karyotyping
Reassurance with close follow-up
B
Radiograph of the hand and wrist
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).
nan
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A 27-year-old woman presents to her primary care physician with new hair growth on her face and lower abdomen over the last month. She has started to develop pimples on her face and back over the last several months. Her last menstrual period was over 3 months ago and her periods have been irregular over the last year. She has been gaining weight recently. The patient has a medical history of obesity and prediabetes with a hemoglobin A1c of 6.0% last year. Her temperature is 98.5°F (36.9°C), pulse is 80/min, blood pressure is 139/88 mmHg, and respirations are 13/min. Cardiopulmonary exam is unremarkable, and the patient’s abdomen appears slightly distended but exhibits no tenderness to palpation. The patient’s face has coarse stubble along the jawline and on the upper lip, and there is similar hair along the midline of her lower abdomen. A pelvic exam reveals mild clitoromegaly, a normal anteverted uterus, and a large left adnexal mass that is mildly tender. Her laboratory test results are shown below: Hemoglobin: 13.9 g/dL Leukocyte count: 8,000 cells/mm^3 Platelet count: 142,000/mm^3 DHEAS: 73 ug/dL (Normal: 145-395 ug/dL) Testosterone: 256 ng/dL 17-hydroxyprogesterone: 214 ng/dL (Normal: < 200 ng/dL) Ultrasound findings are shown in Figure A. Which of the following is the most likely diagnosis in this patient?
Adrenal tumor
Congenital adrenal hyperplasia
Ovarian hyperthecosis
Ovarian tumor
D
Ovarian tumor
This young woman presents with rapid virilization, elevated testosterone, and a large left adnexal mass that is primarily solid on ultrasound. These findings are most consistent with an ovarian androgen-secreting tumor. Sertoli-Leydig cell tumors are the most common androgen-secreting tumor of the ovaries and classically result in rapid virilization of affected women. Testosterone levels are often over 200 ng/dL, and 17-hydroxyprogesterone may also be slightly elevated as it is an androgen precursor. The androgen excess may result in symptoms such as facial and abdominal hirsutism, oligomenorrhea, deepening voice, and acne. Treatment involves staging the tumor and surgical resection of the tumor if possible. Durmus et al. review the evidence regarding the diagnosis and treatment of Sertoli-Leydig cell tumors. They discuss how this tumor generally has a favorable prognosis compared to malignant epithelial tumors. They recommend using fertility-preserving surgeries in younger patients. Figure/Illustration A shows an ultrasound of the left ovary, which has a predominantly solid mass with vascularity and some cystic spaces (red circle). These findings are consistent with an ovarian tumor. Incorrect Answers: Answer A: Adrenal tumors may secrete androgens and cause similar symptoms of virilization, but the presence of a malignant-appearing ovarian mass in this patient is more consistent with ovarian pathology. Furthermore, adrenal tumors typically secrete DHEAS, which can be a helpful test for localizing the source of androgen excess. Treatment is with surgical excision. Answer B: Congenital adrenal hyperplasia (CAH) results from one of several possible enzyme deficiencies in the steroid pathway and may appear later in life as virilization of a woman. While 17-hydroxyprogesterone elevation is a strong indicator of the most common subtype of CAH, this patient’s ultrasound findings suggest ovarian pathology instead, which could also elevate this androgen precursor. Treatment is with hormone replacement therapy. Answer C: Ovarian hyperthecosis is hyperplasia of the theca interna of the ovary, which produces androgens. Testosterone levels would be elevated and virilization may occur, but ultrasound findings would show a bilateral increase in stromal tissue as opposed to a unilateral solid mass. Treatment is with hormonal therapy. Answer E: Polycystic ovary syndrome (PCOS) can also cause elevated testosterone, though levels are typically lower than 100 ng/dL. Frank virilization with clitoromegaly and voice deepening would not be expected with PCOS, nor would an ovarian mass. Treatment is with metformin and lifestyle modifications. Bullet Summary: Sertoli-Leydig cell tumors of the ovary can produce androgens and result in rapid onset of virilization with elevated testosterone levels and an adnexal mass on ultrasound.
Polycystic ovary syndrome
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A 68-year-old woman presents to the emergency room with several days of frequent, nonbloody, watery stools. She recently completed a course of clindamycin 2 weeks ago for a tooth infection. Her medical history is notable for hypertension and hyperlipidemia for which she takes amlodipine and atorvastatin. She traveled recently and has no sick contacts. Her temperature is 37.0°C (98.6°F), blood pressure is 118/82 mmHg, pulse is 98/min, respirations are 14/min, and oxygen saturation is 98% on room air. On exam, her abdomen is soft, non-distended, and non-tender to palpation; however she has diffuse discomfort upon palpation of the abdomen with no rebound, rigidity, or guarding. Which of the following is most likely to confirm the diagnosis?
CT imaging of abdomen and pelvis
Rectal swab for selective anaerobic culture
Abdominal radiograph
Stool toxin assay
D
Stool toxin assay
This patient presenting with cramping abdominal pain and frequent watery diarrhea a few weeks after finishing a course of antibiotics most likely has Clostridioides difficile infection, for which the most appropriate confirmatory test is a stool toxin assay. C. difficile infection should be suspected in patients with acute and clinically significant diarrhea with no obvious alternative explanation. It is associated with risk factors such as recent antibiotic use, hospitalization, and advanced age. Diagnosis is established via a positive enzyme immunoassay for C. difficile toxins and C. difficile glutamate dehydrogenase (GDH), or a positive nucleic acid amplification test (NAAT) for C. difficile toxins. The GDH enzyme immunoassay is highly sensitive for detecting C. difficile, but not specific for toxin-producing C. difficile, and thus the toxin enzyme immunoassay is submitted in conjunction. If the results of both are discordant, then NAAT is used to make the diagnosis. Treatment for C. difficile infection differs by disease severity and recurrence. Commonly used medications include fidaxomicin and oral vancomycin. McDonald et al. review the guidelines for diagnosis and treatment of C. difficile and provide the algorithm for C. difficile testing as mentioned above. The authors also note that only liquid stool from patients with clinically significant and acute diarrhea should be submitted for testing. The authors recommend that formed stool from asymptomatic patients should not be tested because the presence of the C. difficile toxin gene does not distinguish between infection and asymptomatic carriage. Incorrect Answers: Answer A: Abdominal radiograph is an appropriate adjunctive diagnostic tool in patients with severe disease (e.g., severe abdominal pain and distension, fever, hypovolemia, lactic acidosis, marked leukocytosis) or fulminant colitis (marked by hypotension and ileus) to evaluate for toxic megacolon, bowel perforation, or other findings that warrant surgical intervention. In patients who are stable with low concern for severe disease or fulminant colitis, it would not be the preferred initial test for diagnosis nor would it be confirmatory. Answer B: CT imaging of the abdomen and pelvis with oral and intravenous contrast is the preferred modality for detecting the presence of toxic megacolon, bowel perforation, or other findings that warrant surgical intervention in the setting of C. difficile colitis. Colonic dilatation of > 7 cm in diameter in the clinical setting of severe C. difficile infection is diagnostic. Other findings may include small bowel dilatation or scalloping of the bowel wall due to submucosal edema. While it would be reasonable to perform a CT scan in this patient, it would not confirm the diagnosis. Answer C: Flexible sigmoidoscopy would be appropriate for cases in which an alternative diagnosis is suspected that requires direct visualization and/or biopsy of the bowel mucosa. It may also be helpful for patients with ileus or colitis in the absence of diarrhea as it allows for visualization of pseudomembranes on the inflamed mucosal surface, which are highly suggestive of C. difficile infection. In general, colonoscopy is a preferred and more accurate test. Answer D: Rectal swab for selective anaerobic culture involves culture on a selective medium with toxin testing of isolated C. difficile. It is a highly sensitive test, but culture cannot distinguish toxin-producing strains from non-toxin-producing strains. A second test is required to detect toxin production by the cultured C. difficile strain (either enzyme immunoassay or nucleic acid amplification testing). Culture takes several days to finalize and as such, it is seldom used to establish a clinical diagnosis. It is most useful in patients with ileus and suspected C. difficile infection. Bullet Summary: The most appropriate confirmatory test for diagnosis of Clostridioides difficile infection is a stool toxin assay.
nan
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A 26-year-old man presents to the emergency department with fatigue and dark urine over the past day. He was recently diagnosed with cellulitis of his left leg, for which he was prescribed trimethoprim-sulfamethoxazole. He denies having nausea, vomiting, or diarrhea. He has no significant medical history and does not currently take any medications. His temperature is 98.6°F (37°C), blood pressure is 115/72 mmHg, pulse is 98/min, and respirations are 14/min. Physical exam reveals conjunctival icterus, bilateral flank tenderness, and erythema of the left leg. Laboratory workup is performed, and the results are shown below: Serum: Hemoglobin: 7.2 g/dL Total bilirubin: 2.1 mg/dL Direct bilirubin: 0.8 mg/dL Lactate dehydrogenase: 470 U/L Glucose-6-phosphate dehydrogenase: 7 U/gHb (normal: 6-20 U/gHb) Which of the following would most likely be seen on a peripheral blood smear in this patient?
Schistocytes
Codocytes
Acanthocytes
Degmacytes
D
Degmacytes
This patient presents with hemolytic anemia (low hemoglobin, elevated indirect bilirubin, and elevated lactate dehydrogenase) shortly after starting a sulfa medication (trimethoprim-sulfamethoxazole) in the setting of likely glucose-6-phosphate dehydrogenase deficiency. Degmacytes ("bite cells") will be seen on peripheral blood smears in these patients. Patients with glucose-6-phosphate dehydrogenase (G6PD) deficiency will experience hemolytic anemia when exposed to an oxidizing substance such as sulfa drugs or fava beans. Laboratory tests will show a decrease in hemoglobin and hematocrit levels. Since hemolysis releases intracellular enzymes into the bloodstream, serum levels of lactate dehydrogenase will be increased. Hemoglobin is then metabolized in the spleen into unconjugated bilirubin, which will cause an elevated bilirubin level that is primarily indirect. In the acute setting, G6PD levels will be normal because the most compromised cells have all been hemolyzed. Obtaining a G6PD level in between episodes of hemolysis is therefore required to observe the chronically decreased G6PD in these patients. A peripheral blood smear will reveal degmacytes ("bite cells") and Heinz bodies. Avoidance of oxidative stress to red blood cells is the most important component of management in these patients. Belfield and Tichy discuss the classic presentation of G6PD deficiency in patients. The authors review oxidizing medications such as rasburicase, primaquine, dapsone, pegloticase, and methylene blue. The authors recommend avoiding initiation of these medications whenever possible until a G6PD diagnostic test has been performed. Incorrect Answers: Answer A: Acanthocytes can be seen in patients with liver disease due to defective production of lipoproteins; however, these cells do not undergo acute hemolytic destruction. Patients with abetalipoproteinemia usually present with symptoms consistent with fat-soluble vitamin deficiency such as bleeding (vitamin K) or osteoporosis (vitamin D). Answer B: Codocytes are most commonly seen in patients with thalassemia but can also generally be seen in all microcytic anemias. Patients with thalassemia will present with chronic anemia without acute episodes of hemolysis. The increased lactate dehydrogenase and bilirubin levels in this patient indicate an acute hemolytic event. Answer D: Schistocytes can be seen in thrombotic thrombocytopenic purpura (TTP) and disseminated intravascular coagulation (DIC). Patients with TTP present with thrombocytopenia, microangiopathic hemolytic anemia, fever, neurologic symptoms, and acute kidney injury. DIC is a common downstream pathway for many processes such as sepsis, malignancy, and eclampsia; however, it would present with diffuse clotting and bleeding from all sites. Answer E: Spherocytes can be seen in hereditary spherocytosis, which may result in hemolysis during times of acute stress; however, this patient denies symptoms of nausea, vomiting, or diarrhea. Instead, the hemolysis in response to new foods is most likely due to exposure to oxidizing substances such as trimethoprim-sulfamethoxazole. Bullet Summary: Patients with glucose-6-phosphate dehydrogenase deficiency will experience hemolytic anemia in response to oxidizing substances and will have degmacytes (bite cells) on peripheral blood smear.
nan
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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?
Blinking after saline is applied to the cornea
Conjugate eye movement to the left with rotation of the head to the right
Flexion of the thigh, leg, and foot upon noxious stimulation of the foot
Grimacing after noxious stimulus to the arm
C
Flexion of the thigh, leg, and foot upon noxious stimulation of the foot
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.
Spontaneous respiration 1 minute after disconnection from a ventilator
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A 44-year-old man presents to the emergency department acutely confused. The patient’s wife states she found him lethargic at home. He has been sick the past week with diarrhea and has been staying home from work. He is otherwise healthy and does not take any medications. His temperature is 97.5°F (36.4°C), blood pressure is 62/32 mmHg, pulse is 185/min, respirations are 25/min, and oxygen saturation is 98% on room air. The patient has profuse, bloody diarrhea while in the emergency department. The patient is given 3L of ringer lactate and subsequently appears less confused, with a blood pressure of 100/70 mmHg. He is able to drink oral fluids and protect his airway. An ECG is performed as seen in Figure A. He continues to have diarrhea while in the emergency department. Which of the following is contraindicated in the management of this patient?
Loperamide
Norepinephrine
Ceftriaxone
Normal saline
A
Loperamide
This patient is presenting with infectious diarrhea (given his bloody diarrhea and hypotension) with unstable vitals that improve with fluids. In bloody diarrhea, loperamide is contraindicated and could worsen illness. Bacterial diarrhea is commonly caused by organisms such as Campylobacter, Shigella, and Escherichia coli. It may present with bloody or purulent diarrhea and fever and may progress to sepsis or septic shock. Immediate management is centered on hydration. Unstable patients or those who cannot tolerate PO require IV fluids immediately. Otherwise, patients can rehydrate orally. Loperamide is an antimotility agent that decreases diarrheal output. It is contraindicated in bloody diarrhea as it can decrease the rate of fecal shedding and lead to bacteremia and a worsening clinical picture. Loperamide may be appropriate in controlling diarrhea in milder infectious forms of diarrhea, or in non-infectious conditions such as irritable bowel syndrome. Baker reviews loperamide. He discusses its indications, contraindications, and mechanism of action. They recommend that loperamide should not be given in invasive diarrhea. Figure/Illustration A demonstrates U waves on ECG (red arrows) that are significant for hypokalemia, which would be expected in a dehydrated patient with profuse diarrhea. Incorrect Answers: Answer A: Ceftriaxone would be appropriate in this patient who has bacterial diarrhea and appears septic as his critically ill state warrants antibiotics. Note that this patient may receive broader antibiotics than ceftriaxone given how ill he appears. Antibiotics should not be routinely used for bacterial diarrhea in young, healthy patients with stable vitals. Answer C: Magnesium could be appropriate in this patient even though his magnesium level is not stated as he is hypokalemic given his ECG is demonstrating U waves. The serum magnesium level does not adequately reflect intracellular magnesium levels. It requires replacement in many cases of hypokalemia. Answer D: Norepinephrine could be appropriate in this patient if he remained persistently hypotensive despite fluid administration. Note that this patient could receive more fluids and is not frankly unstable. While norepinephrine may not be appropriate at this time, it is not contraindicated. Answer E: Normal saline is generally avoided in diarrheal illness in patients who can tolerate PO as oral hydration is preferred to IV. However, IV fluids are not contraindicated and would be appropriate in this persistently hypotensive patient. Normal saline has a very high sodium and chloride load and can cause a hyperchloremic metabolic acidosis. For this reason, a lower-chloride solution may be preferred (e.g, Lactated Ringer). Bullet Summary: Loperamide is contraindicated in bloody diarrhea.
nan
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A 15-year-old boy presents to his pediatrician after an appendectomy 1 week ago. The patient denies any abdominal pain, fevers, chills, nausea, vomiting, diarrhea, or constipation. He eats solids and drinks liquids without difficulty. He is back to playing basketball for his school team without any difficulty. His urine appears more amber than usual but he suspects dehydration. His temperature is 98.6°F (37°C), blood pressure is 110/70 mmHg, pulse is 76/min, and respirations are 15/min. His physical exam is unremarkable. The laparoscopic incision sites are all clean without erythema. The pediatrician orders a urinalysis, which is notable for the following. Urine: Epithelial cells: Scant Glucose: Negative Protein: 3+ WBC: 3/hpf Bacteria: None Leukocyte esterase: Negative Nitrites: Negative The patient is told to return in 3 days for a follow-up appointment; however, his urinalysis at that time is similar. Which of the following is the most appropriate next step in management?
CT scan abdomen and pelvis with contrast
Renal biopsy
Repeat urinalysis
Urine dipstick in the morning and in the afternoon
D
Urine dipstick in the morning and in the afternoon
This patient is found to have proteinuria on urinalysis in the absence of clinical symptoms or signs of nephrotic syndrome. Given the likely diagnosis of transient proteinuria, the most appropriate next step is to repeat the urine dipstick in the morning and in the afternoon. Transient proteinuria can be due to febrile illnesses, seizures, strenuous exercise, emotional stress, serious illnesses like heart failure, and abdominal surgery. A detailed workup is not needed if the clinical story is clear, but an orthostatic test can be completed. One way to do this is to assess the urine dipstick in the morning and in the afternoon. Another way requires the patient to collect urine at multiple times throughout the day. A diagnosis is confirmed if the morning sample is protein-free but the evening sample contains protein. In general, pediatric patients with orthostatic proteinuria should have under 1 gram of protein in a full 24 hours. Treatment is with reassurance because this condition usually resolves by itself. Ingold and Bhatt review the evidence regarding the diagnosis and treatment of orthostatic proteinuria in patients. They discuss how this condition is benign and typically occurs in adolescents. They recommend reassurance and supportive treatment. Incorrect Answers: Answer A: A CT scan of the abdomen and pelvis with contrast could assess for any anatomic obstruction and a possible post-renal cause of kidney dysfunction. It would be more prudent to obtain basic labs and a timed urinalysis before exposing a pediatric patient to radiation. Answer B: Renal biopsy is indicated if the patient's clinical presentation is concerning for a glomerular or tubulointerstitial etiology for proteinuria. Patients will have edema, hematuria, or dysuria. Answer C: Repeat urinalysis will not change the presence of proteinuria. Generally, a patient who has proteinuria should have a repeat urinalysis performed to rule out transient proteinuria. If the repeat urinalysis demonstrates proteinuria (as in this patient), then the diagnosis is unlikely to be transient proteinuria, and further workup is needed. Answer E: Urine electrolytes and creatinine will assess the kidney's ability to concentrate urine and assess the etiology of acute kidney injury. Patients will present with oliguria or anuria. Bullet Summary: Consistent proteinuria in a child should by evaluated by performing a urinalysis in the morning and in the afternoon to see if proteinuria comes on toward the end of the day.
Urine electrolytes and creatinine
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A 42-year-old woman is enrolled in a randomized controlled trial to study cardiac function in the setting of several different drugs. She is started on verapamil and instructed to exercise at 50% of her VO2 max while several cardiac parameters are being measured. During this experiment, which of the following represents the relative conduction speed through the heart from fastest to slowest?
Atria > Purkinje fibers > ventricles > AV node
AV node > ventricles > atria > Purkinje fibers
Purkinje fibers > ventricles > atria > AV node
Purkinje fibers > atria > ventricles > AV node
D
Purkinje fibers > atria > ventricles > AV node
The conduction velocity of the structures of the heart are in the following order: Purkinje fibers > atria > ventricles > AV node. A calcium channel blocker such as verapamil would only slow conduction in the AV node. The conduction velocity of cardiac tissue is determined by a wide variety of factors, including the number and identity of various ion channels, the number and type of gap junctions, and the size and composition of muscle fibers. Conduction through the Purkinje system is the fastest within the heart, allowing for coordinated contraction of the ventricles, and atrial muscle conducts faster than ventricular muscle. Conduction through the AV node is the slowest, to allow the ventricles enough time to fill with blood. Bonke et al. review the evidence regarding the conduction velocity of parts of the heart. They discuss how the AV node is slower than the SA node. They recommend understanding how different parts of the heart conducts electricity. Incorrect Answers: Answers A-C & E: These choices do not correspond to the correct conduction velocities in each part of the cardiac conduction system. A calcium channel blocker would not change the relative velocities of conduction in Purkinje fibers, atria, and ventricles. Bullet Summary: The conduction velocity through the heart in order of speed is Purkinje fibers > atria > ventricles > AV node.
Purkinje fibers > AV node > ventricles > atria
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A 6-month-old boy is brought to the pediatrician by his parents for a routine visit. His parents report that he is feeding and stooling well and they have just started experimenting with solid foods. His parents are concerned because a few weeks ago he started rolling from back to front in addition to front to back, but he now struggles when placed on his back. The patient was noted to be developmentally appropriate for his age at his last visit 2 months ago. On physical exam, the patient seems well-nourished and has no dysmorphic features. His temperature is 98.6°F (37°C), blood pressure is 77/49 mmHg, pulse is 112/min, and respirations are 24/min. In the examination room, he is able to roll from front to back but cannot roll from back to front. He is not able to sit without support and makes no attempt at bouncing when supported in a standing position. The patient’s abdomen is non-tender and soft without hepatosplenomegaly. His patellar reflexes are 4+ bilaterally. He is found to have the ocular finding seen in Figure A. Accumulation of which of the following would most likely be found in this patient’s cells?
Cerebroside sulfate
Galactocerebroside
Glucocerebroside
GM2-ganglioside
D
GM2-ganglioside
This 6-month-old boy who presents with developmental regression, a “cherry-red” macula, and hyperreflexia most likely has a diagnosis of Tay-Sachs disease. Tay-Sachs disease is caused by a deficiency of beta-hexosaminidase A that results in the accumulation of GM2-ganglioside. Tay-Sachs disease is a genetic disorder caused by an autosomal recessive deficiency of the beta-hexosaminidase A enzyme. This disease presents in infants aged 2-6 months with progressive neurodegeneration, hypotonia, feeding difficulties, and a “cherry red” spot on the macula. Tay-Sachs disease shares many features with Niemann-Pick disease; however, Niemann-Pick disease presents with the additional findings of hepatosplenomegaly and hyporeflexia whereas Tay-Sachs presents with hyperreflexia without hepatosplenomegaly. Both diseases are more common in the Ashkenazi Jewish population. There is no curative treatment for this disease so treatment is generally supportive. Solovyeva et al. review the evidence regarding the diagnosis and treatment of Tay-Sachs disease. They discuss how this disease normally presents with muscle weakness, ataxia, speech, and mental disorders. They recommend considering novel treatments such as substrate reduction therapy. Figure/Illustration A is a fundoscopic exam demonstrating a “cherry-red” spot on the macula (blue circle). This finding is seen in both Tay-Sachs disease and Niemann-Pick disease. Incorrect Answers: Answer A: Cerebroside sulfate accumulates in metachromic leukodystrophy, which is caused by an autosomal recessive deficiency in arylsufatase A. This disease presents with optic atrophy and seizures in childhood. Patients will later develop dementia, psychiatric disease, and neuropathy. There is no cure so treatment is supportive. Answer B: Galactocerebroside accumulates in Krabbe disease, which is caused by an autosomal recessive deficiency in galactocerebrosidase. Krabbe disease presents in early infancy with developmental regression, hypotonia, and areflexia. Treatment is supportive with musculoskeletal bracing as needed. Answer C: Glucocerebroside accumulates in Gaucher disease, which is caused by an autosomal recessive deficiency in glucocerebrosidase. Gaucher disease presents with pancytopenia and hepatosplenomegaly. “Gaucher cells,” or lipid-laden macrophages resembling crumpled tissue paper, can be seen on histology. Treatment is with enzyme replacement therapy. Answer E: Sphingomyelin accumulates in Niemann-Pick disease, an autosomal recessive sphingolipidosis caused by a deficiency in sphingomyelinase. Niemann-Pick disease presents with many of the same features as Tay-Sachs disease but has additional findings of hyporeflexia and hepatosplenomegaly. Bullet Summary: Tay-Sachs disease is caused by a deficiency of beta-hexosaminidase A resulting in the accumulation of GM2-ganglioside.
Sphingomyelin
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A 27-year-old man presents to the emergency department after a bar fight. He was punched in the eye and is having pain and blurry vision. The patient is otherwise healthy and does not take any medications. His temperature is 98.1°F (36.7°C), blood pressure is 132/84 mmHg, pulse is 103/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam is notable for conjunctival injection of the patient’s left eye as shown in Figure A. Visual acuity reveals 20/20 vision in his right eye and 20/40 vision in his left. His left pupil is sluggish to constrict when compared to the right. A fluorescein stain is unremarkable and tonometry reveals a pressure of 45 mmHg in the left eye. Which of the following is the most appropriate next step in management?
CT scan of the head and facial bones
Cyclopentolate
Lateral canthotomy
Orbital ultrasound
C
Lateral canthotomy
This patient is presenting after blunt trauma to the eye with eye pain, blurry vision, conjunctival injection, a sluggish pupil, and elevated intraocular pressure (> 20 mmHg) suggesting a diagnosis of a retrobulbar hematoma/hemorrhage causing orbital compartment syndrome. The most important initial step in management is a lateral canthotomy to alleviate retrobulbar pressure. A retrobulbar hematoma (or hemorrhage) occurs after blunt trauma to the eye. Presenting symptoms may include eye pain, photophobia, proptosis (an eye that appears to be anteriorly displaced), decreased visual acuity, and conjunctival injection. It is important to perform a thorough exam including a slit lamp exam and a fluorescein stain to rule out other traumatic injuries such as a globe rupture. Tonometry can be used to assess for increased intraocular pressure. A pressure > 20 mmHg suggests a diagnosis of orbital compartment syndrome, a vision-threatening condition. Immediate management with a lateral canthotomy is indicated in order to relieve pressure on the optic nerve and retina and save the patient’s vision. Further management may require drainage of the hematoma in the operating room. Burkat et al. review the technique for performing a lateral canthotomy to relieve pressure in retrobulbar hematoma. The authors first excise the skin and orbicularis muscle at the lateral canthal angle in a horizontal direction for approximately 10 mm. The authors then recommend performing an inferior cantholysis by incising the inferior crus (a thick fibrous band connecting the lateral lower eyelid to the orbital rim) near the orbital rim. Figure A demonstrates proptosis on physical exam which raises concern for a retrobulbar hematoma that is likely increasing orbital compartment pressure. Incorrect Answers: Answer A: CT scan of the head and facial bones would be appropriate if there was a concern for facial fracture. It would delay necessary and vision-saving interventions to perform a CT scan in the setting of orbital compartment syndrome. However, cross-sectional imaging may be performed after the pressure is relieved. Answer B: Cyclopentolate is a cycloplegic that can be used to reduce pain in traumatic iritis. This typically presents after blunt trauma to the eye with erythema, photophobia, blurry vision, and cell/flare in the anterior chamber on slit lamp exam. A sluggish pupil may also be seen; however, increased intraocular pressure would not be seen in this condition. Answer D: Orbital ultrasound could be used for diagnoses such as a retinal detachment which presents with flashes of light, floaters, and visual field loss like a “curtain coming down over the eye.” An ultrasound would demonstrate the detached retina in the back of the eye. Management involves elevating the head of the bed and surgical reattachment. Answer E: Topical timolol and pilocarpine in addition to brimonidine, acetazolamide, and possible iridotomy would be the appropriate management of acute closed-angle glaucoma which presents with a rock-hard eye, blurry vision, a mid-dilated and fixed pupil, as well as a headache. This diagnosis can be supported by elevated intraocular pressure on tonometry. This is unlikely for this patient in the context of trauma. Bullet Summary: A retroorbital hematoma can cause orbital compartment syndrome which requires immediate treatment with a lateral canthotomy.
Topical timolol and pilocarpine
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A 68-year-old woman presents to the emergency room with several days of frequent, nonbloody, watery stools. She recently completed a course of clindamycin 2 weeks ago for a tooth infection. Her medical history is notable for hypertension and hyperlipidemia for which she takes amlodipine and atorvastatin. She traveled recently and has no sick contacts. Her temperature is 37.0°C (98.6°F), blood pressure is 118/82 mmHg, pulse is 98/min, respirations are 14/min, and oxygen saturation is 98% on room air. On exam, her abdomen is soft, non-distended, and non-tender to palpation; however she has diffuse discomfort upon palpation of the abdomen with no rebound, rigidity, or guarding. Which of the following is most likely to confirm the diagnosis?
Abdominal radiograph
CT imaging of abdomen and pelvis
Flexible sigmoidoscopy
Rectal swab for selective anaerobic culture
E
Stool toxin assay
This patient presenting with cramping abdominal pain and frequent watery diarrhea a few weeks after finishing a course of antibiotics most likely has Clostridioides difficile infection, for which the most appropriate confirmatory test is a stool toxin assay. C. difficile infection should be suspected in patients with acute and clinically significant diarrhea with no obvious alternative explanation. It is associated with risk factors such as recent antibiotic use, hospitalization, and advanced age. Diagnosis is established via a positive enzyme immunoassay for C. difficile toxins and C. difficile glutamate dehydrogenase (GDH), or a positive nucleic acid amplification test (NAAT) for C. difficile toxins. The GDH enzyme immunoassay is highly sensitive for detecting C. difficile, but not specific for toxin-producing C. difficile, and thus the toxin enzyme immunoassay is submitted in conjunction. If the results of both are discordant, then NAAT is used to make the diagnosis. Treatment for C. difficile infection differs by disease severity and recurrence. Commonly used medications include fidaxomicin and oral vancomycin. McDonald et al. review the guidelines for diagnosis and treatment of C. difficile and provide the algorithm for C. difficile testing as mentioned above. The authors also note that only liquid stool from patients with clinically significant and acute diarrhea should be submitted for testing. The authors recommend that formed stool from asymptomatic patients should not be tested because the presence of the C. difficile toxin gene does not distinguish between infection and asymptomatic carriage. Incorrect Answers: Answer A: Abdominal radiograph is an appropriate adjunctive diagnostic tool in patients with severe disease (e.g., severe abdominal pain and distension, fever, hypovolemia, lactic acidosis, marked leukocytosis) or fulminant colitis (marked by hypotension and ileus) to evaluate for toxic megacolon, bowel perforation, or other findings that warrant surgical intervention. In patients who are stable with low concern for severe disease or fulminant colitis, it would not be the preferred initial test for diagnosis nor would it be confirmatory. Answer B: CT imaging of the abdomen and pelvis with oral and intravenous contrast is the preferred modality for detecting the presence of toxic megacolon, bowel perforation, or other findings that warrant surgical intervention in the setting of C. difficile colitis. Colonic dilatation of > 7 cm in diameter in the clinical setting of severe C. difficile infection is diagnostic. Other findings may include small bowel dilatation or scalloping of the bowel wall due to submucosal edema. While it would be reasonable to perform a CT scan in this patient, it would not confirm the diagnosis. Answer C: Flexible sigmoidoscopy would be appropriate for cases in which an alternative diagnosis is suspected that requires direct visualization and/or biopsy of the bowel mucosa. It may also be helpful for patients with ileus or colitis in the absence of diarrhea as it allows for visualization of pseudomembranes on the inflamed mucosal surface, which are highly suggestive of C. difficile infection. In general, colonoscopy is a preferred and more accurate test. Answer D: Rectal swab for selective anaerobic culture involves culture on a selective medium with toxin testing of isolated C. difficile. It is a highly sensitive test, but culture cannot distinguish toxin-producing strains from non-toxin-producing strains. A second test is required to detect toxin production by the cultured C. difficile strain (either enzyme immunoassay or nucleic acid amplification testing). Culture takes several days to finalize and as such, it is seldom used to establish a clinical diagnosis. It is most useful in patients with ileus and suspected C. difficile infection. Bullet Summary: The most appropriate confirmatory test for diagnosis of Clostridioides difficile infection is a stool toxin assay.
Stool toxin assay
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A 53-year-old man presents to the clinic with a non-tender mass in his right anterior neck. The mass has been slowly enlarging over the past 1 year. He reports no dysphagia, difficulty breathing, or changes to his voice. He also denies heat intolerance, palpitations, tremors, or diarrhea. He has hypertension and hyperlipidemia, for which he takes lisinopril and atorvastatin. He has never smoked cigarettes. His temperature is 37.1°C (98.8°F), blood pressure is 130/84 mmHg, pulse is 86/min, respirations are 12/min, and oxygen saturation is 99% on room air. Bedside ultrasound reveals a 1x2 cm hypoechogenic nodule with microcalcifications in the right thyroid lobe, which is biopsied. Pathology results are shown in Figure A. Which of the following is the most appropriate next step in management?
Levothyroxine
Methimazole
Radioactive iodine
Repeat ultrasound in 6 months
E
Thyroidectomy followed by radioactive iodine
This patient who presents with a slowly enlarging neck mass has ultrasound (hypoechogenic nodule with microcalcifications) and histology findings (concentrically laminated calcifications called psammoma bodies) consistent with papillary thyroid carcinoma. The most appropriate next step in management would be thyroidectomy followed by radioiodine ablation. Papillary thyroid carcinoma represents the majority of malignant thyroid cancers. They are slow-growing and are found in the hormone-producing follicular cells of the thyroid. Typically, these tumors are found incidentally on imaging. Risk factors for thyroid malignancy include a history of radiation exposure, female sex, and positive family history. Nodules with minimal uptake on radioactive iodine scan, firm and immobile solitary nodules, and rapidly growing nodules with associated hoarseness are more suggestive of thyroid malignancy. Findings on histology include empty-appearing nuclei with central clearing, psammoma bodies, and nuclear grooves. The treatment is thyroidectomy followed by radioactive iodine ablation of any remaining thyroid cells. Long-term care should include levothyroxine at a dose sufficient to suppress thyroid-stimulating hormone (TSH) release that may stimulate any remaining malignant cells. Oakley et al. studied if relatives of patients diagnosed as having papillary thyroid carcinoma are at increased risk using a retrospective cohort study. The authors found first-degree relatives were at a 5-fold increased risk, and second-degree relatives were at a 2-fold increased risk. The authors recommend more research into optimal approaches to screening for thyroid cancers. Figure/Illustration A is a histology section showing a psammoma body (blue circle), which are round, concentrically laminated calcifications that are formed after necrosis of papillary structures. Psammoma bodies and nuclei with central clearing (yellow circle) and nuclear grooves are common histology findings for papillary thyroid carcinoma. Incorrect Answers: Answer A: Levothyroxine would be the treatment of choice in hypothyroidism and is important in the long-term care of papillary thyroid carcinoma to suppress the TSH release that may stimulate any remaining malignant cells after treatment (thyroidectomy and radioactive iodine). Answer B: Methimazole is an agent that blocks thyroid peroxidase, thus inhibiting the synthesis of thyroid hormones. It is used in the treatment of hyperthyroidism and does not have a role in the treatment of papillary thyroid carcinoma. Answer C: Radioactive iodine is used as an adjunct after thyroidectomy in the treatment of papillary thyroid carcinoma in order to target any thyroid cells that may remain even after surgical excision. Surgery is the primary therapy for papillary thyroid carcinoma. Radioactive iodine is the primary method of therapy in cases of Grave disease, toxic adenoma, or multinodular goiter. Answer D: Repeating an ultrasound in 6 months to assess the growth of the nodule or the development of suspicious characteristics is appropriate in cases where the ultrasound and biopsy findings are consistent with a benign thyroid nodule. The ultrasound findings of hypoechogenicity, microcalcifications, hypervascularity, and taller-than-wide nodules suggest malignancy, and together with a biopsy consistent with thyroid malignancy would warrant surgical treatment with subsequent radioactive iodine ablation. Bullet Summary: Papillary thyroid carcinoma is managed with thyroidectomy followed by radioactive iodine ablation and long-term levothyroxine to suppress thyroid-stimulating hormone stimulation of any remaining malignant cells.
Thyroidectomy followed by radioactive iodine
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A 32-year-old G1P0 presents to the antenatal testing unit at 32 weeks of gestation for a non-stress test (NST). Yesterday she had several episodes of diarrhea and vomited once after attending a company picnic 2 days ago. She took loperamide with symptomatic relief. She is worried that she is dehydrated but otherwise feels well and has recovered from her gastrointestinal illness. The patient was diagnosed with gestational diabetes after routine screening in the 2nd trimester and has been started on insulin after unsuccessful attempts at lifestyle management. She also has migraines and gastroesophageal reflux disorder. The patient has a 15-pack-year smoking history but quit smoking in her 1st month of pregnancy. She denies alcohol or drug use. Her temperature is 98.5°F (36.9°C), blood pressure is 122/82 mmHg, pulse is 84/min, and respirations are 14/min. A representative portion of the NST is shown in Figure A. Which of the following is the most likely explanation for this patient’s non-stress test (NST) results?
Fetal sleep cycle
Maternal dehydration
Maternal medication use
Maternal smoking
A
Fetal sleep cycle
This patient presents with a nonreactive NST on routine testing. The most common explanation for a nonreactive NST is the fetal sleep cycle. Patients with gestational diabetes are at increased risk of fetal demise and other perinatal complications. They require closer monitoring during the 3rd trimester of pregnancy. The NST, which lasts 20 minutes, is the most common method of doing so. After 32 weeks, a reactive (i.e. “normal”) NST requires at least 2 episodes of 15-beat-per-minute accelerations lasting for at least 15 seconds. An NST with no accelerations is considered nonreactive. A common cause of a nonreactive NST is the fetal sleep cycle and it is reasonable to continue the test for an additional 20 minutes to increase the chance of monitoring the fetus while awake. Otherwise, a nonreactive NST should prompt vibroacoustic stimulation or further testing such as a biophysical profile (BPP). Umana and Siccardi review the evidence regarding the use of the NST during pregnancy. They discuss how the test is a measurement of fetal cardiovascular function at the time of the test. They recommend monitoring the baseline fetal heart rate and variability as well as the presence of accelerations and decelerations when interpreting test results. Figure/Illustration A shows a representative portion of a NST where the fetal heart rate remains within a narrow band without accelerations (red circle). This pattern is characteristically seen in a nonreactive NST that can be due to the fetal sleep cycle. Incorrect Answers: Answer B: Maternal dehydration generally should not affect the reactivity of an NST. Although severe dehydration may reduce amniotic fluid volume and therefore increase the baseline fetal pulse, it is unlikely to change the accelerations or variability in an NST. Though this patient had diarrhea the day before, her pulse is not elevated and any dehydration is probably not clinically significant. Answer C: Maternal medication use can cause nonreactivity on an NST if drugs such as morphine or other sedatives are taken. Although this patient did take loperamide (an opioid), it is unlikely that this has a sedative effect on the fetus as it acts selectively on the mu-opioid receptors in the large intestine and does not cross the blood-brain barrier. Answer D: Maternal smoking in close proximity to an NST has been linked to nonreactivity, but this patient has not smoked in several months. Mothers who smoke are counseled to avoid smoking on the day of their NST to avoid the effects of nicotine on their test results. Answer E: A normal result is known as a reactive NST and would present with accelerations and no concerning or late decelerations. This is not a reactive NST due to the lack of appropriate accelerations. Bullet Summary: The most common cause of a non-reactive non-stress test is the fetal sleep cycle.
Normal results
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A 7-year-old adopted boy presents to the emergency department after fainting. He just switched from being home-schooled to public school and joined the basketball team. He has fainted several times during basketball practices. He did not sustain any injuries or have any prodromes prior to these episodes. He is usually unconscious for less than a minute. He is otherwise healthy and has met all developmental milestones. He does not currently take any medications. His temperature is 97.6°F (36.4°C), blood pressure is 104/54 mmHg, pulse is 100/min, respirations are 19/min, and oxygen saturation is 98% on room air. Physical exam reveals a well-appearing and playful boy. An ECG is performed as seen in Figure A. An echocardiogram is ordered and pending. Which of the following is the most likely diagnosis?
Brugada syndrome
Wolff Parkinson White syndrome
Hypertrophic obstructive cardiomyopathy
Arrhythmogenic right ventricular dysplasia
D
Arrhythmogenic right ventricular dysplasia
This adopted pediatric patient is presenting with recurrent syncope and an ECG demonstrating an epsilon wave and T wave inversions, which are concerning for arrhythmogenic right ventricular dysplasia. An epsilon wave is a small positive deflection at the end of the QRS complex. This is an ominous finding and suggests a diagnosis of arrhythmogenic right ventricular dysplasia (ARVD) though it may also be seen in other infiltrative diseases such as sarcoidosis or after ischemic events. ARVD is an autosomal dominant condition where there is fatty infiltration of the right ventricular wall predisposing the patient (classically younger patients) to sudden cardiac death from lethal ventricular dysrhythmias. The epsilon wave is the most specific finding; however, other findings include T wave inversion in V1-V3 or a left bundle branch appearance with frequent premature ventricular complexes among other possibilities. This is a potentially lethal diagnosis and requires management with antiarrhythmics (such as beta-blockers or amiodarone) and the placement of an implantable cardioverter-defibrillator. Kayser et al. review AVRD. They note the presentation and management of ARVD and how it may present initially only with syncope. Cardiac arrest is a possible presentation as well. They recommend early detection and treatment with an implantable cardioverter-defibrillator to prevent death in this population. Figure/Illustration A is an ECG with an epsilon wave (red arrow) and inverted T waves in V1/V2 (blue arrows) concerning for ARVD. Incorrect Answers: Answer B: Brugada syndrome is an autosomal dominant mutation in cardiac ion channels that presents with a possible right bundle branch pattern with ST segment elevation in V1-V3 or ST coving in these same leads. It may present with syncope. Answer C: Hypertrophic obstructive cardiomyopathy presents with syncope, sudden death, or cardiac arrest in a young patient associated with exertion or dehydration. It can cause obstruction of the left ventricular outflow tract. A systolic murmur can be heard on cardiac auscultation and echocardiography can confirm the diagnosis. ECG will demonstrate large voltages in V1-V5. Answer D: QT prolongation can lead to torsades des pointes which presents with twisting of the QRS complexes around an isoelectric baseline. It may present with syncope, hemodynamic instability, or cardiac arrest. Medications, infection, and congenital conditions may prolong the QT interval. Answer E: Wolff Parkinson White syndrome presents with a delta wave on ECG or a slurred upstroke of the QRS complex. It may cause syncope and evolve into other dysrhythmias including supraventricular tachycardia and ventricular tachycardia. Bullet Summary: Arrhythmogenic right ventricular dysplasia (ARVD) may cause syncope or cardiac arrest, and presents on ECG with an epsilon wave.
nan
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A 72-year-old man presents to his primary care physician for a general checkup. He states that he has been a bit more fatigued lately but believes it is secondary to poor sleep at his new house. The patient is otherwise healthy and takes no medications. His temperature is 98.0°F (36.7°C), blood pressure is 141/90 mmHg, pulse is 82/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam is notable only for minor pallor but is otherwise unremarkable. Basic laboratory studies are ordered as seen below. Hemoglobin: 10 g/dL Hematocrit: 30% Leukocyte count: 67,500/mm^3 Platelet count: 119,000/mm^3 Serum: Na: 141 mEq/L Cl: 103 mEq/L K: 4.0 Eq/L HCO3-: 24 mEq/L BUN: 22 mg/dL Glucose: 109 mg/dL Creatinine: 1.2 mg/dL Ca: 10.0 mg/dL Which of the following is the most likely diagnosis?
Acute lymphoblastic leukemia
Acute myelogenous leukemia
Chronic lymphocytic leukemia
Chronic myelogenous leukemia
C
Chronic lymphocytic leukemia
This elderly patient is presenting with minor fatigue, anemia, thrombocytopenia, and a leukocyte count >50,000/mm^3, which is most likely caused by chronic lymphocytic leukemia (CLL). CLL is a monoclonal proliferation of incompetent mature B-cells. It is common in elderly men and is the most common form of leukemia in the United States. Patients are often asymptomatic when the diagnosis is made or may present with vague constitutional symptoms including fatigue, malaise, and weight loss. It is important to note that symptoms may be subtle or absent. Other findings include non-tender lymphadenopathy and splenomegaly. The diagnosis is supported when the white blood cell count is >50,000/mm^3 and can be supported with a peripheral smear which will show smudge cells. The cells in CLL may be CD5, CD20, and CD23 positive. The most common cause of death in this population is infection given the lack of competent B-cells. Hallek reviews CLL. They discuss updated guidelines on the diagnosis and management of CLL. They recommend the use of targeted agents in initial therapy and acknowledge the need for further clinical trials to guide therapy. Incorrect Answers: Answer A: Acute lymphoblastic leukemia (ALL) is a common malignancy seen in children and presents with pancytopenia, anemia, fatigue, leukopenia (leading to recurrent infections), and thrombocytopenia (leading to bleeding). Other findings may include fever, bone pain, lymphadenopathy, splenomegaly, and hepatomegaly. Translocations in ALL include: t(12:21) which is the most common and denotes a favorable prognosis and t(9:22) (the Philadelphia chromosome) which is less common and denotes a poor prognosis Answer B: Acute myelogenous leukemia occurs secondary to the proliferation of myeloblasts and presents in older patients. Patients will present with fatigue, malaise, weight loss, anorexia, fever, leukocytosis, anemia, thrombocytopenia, and neutropenia. Auer rods are frequently present on a blood smear. Answer D: Chronic myelogenous leukemia is a neoplastic proliferation of myeloid stem cells and presents with a leukocyte count often >50,000/mm3. The increased myeloid lines include red blood cells, granulocytes, monocytes, and platelets. Many patients present in the chronic phase with minimal symptoms; however, the blast phase can lead to a hematologic emergency termed a blast crisis which may cause a hyperviscosity syndrome. This condition is more symptomatic and less common than CLL. Answer E: Hairy cell leukemia occurs when there is a neoplastic proliferation of mature B cells with abundant cytoplasm with “hairy” cytoplasmic processes. The cells are tartrate-resistant acid phosphatase positive. Patients will present with splenomegaly, pancytopenia, weakness, and fatigue. This is a more rare diagnosis when compared to CLL. Bullet Summary: Chronic lymphocytic leukemia (CLL) presents in elderly patients with malaise, fatigue, and other nonspecific signs (but can also be asymptomatic), with anemia, thrombocytopenia, and a white blood cell count often >50,000/mm^3.
Hairy cell leukemia
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A 57-year-old man presents to the emergency department for shortness of breath. He was riding the train home from a business conference when his symptoms began. The patient has a medical history of obesity, diabetes, diabetic nephropathy, hypertension, and a 40-pack-year smoking history. His current medications include atorvastatin, lisinopril, insulin, metformin, and nicotine gum. His temperature is 99.5°F (37.5°C), blood pressure is 130/87 mmHg, pulse is 120/min, respirations are 15/min, and oxygen saturation is 93% on room air. An ECG is ordered as seen in Figure A. A chest radiograph is ordered as seen in Figure B. Laboratory values are ordered as seen below: Serum: Na+: 137 mEq/L Cl-: 105 mEq/L K+: 4.1 mEq/L HCO3-: 24 mEq/L BUN: 22 mg/dL Glucose: 129 mg/dL Creatinine: 1.2 mg/dL Ca2+: 10.1 mg/dL AST: 11 U/L ALT: 12 U/L On physical exam, the patient appears to be in distress. His cardiac exam is notable for tachycardia. Pulmonary exam reveals bilateral air movement without any wheezes or crackles. The patient's right lower extremity appears mildly inflamed, and palpation of the calf elicits pain. Which of the following is the most appropriate next step in management?
Aspirin
Cardiac troponins
CT scan of the chest without contrast
Heparin
D
Heparin
This patient is presenting with a score of 7.5 on Wells criteria suggesting a diagnosis of pulmonary embolism. The most appropriate initial step in management is heparin administration. Patients with suspected pulmonary embolism (PE) can be evaluated with Wells criteria which consists of: clinical signs and symptoms of DVT (3 points), PE is the most likely diagnosis (3 points), pulse > 100 (1.5 points), immobilization for 3 days, or surgery within 4 weeks (1.5 points), history of DVT or PE (1.5 points), hemoptysis (1 point), and malignancy (1 point). Scores higher than 3 suggest a high probability of PE. Patients will often present with shortness of breath, with ECG findings of sinus tachycardia, and a chest radiograph that is typically normal. With a high pre-test probability, the most important initial step in management is anticoagulation with heparin versus further diagnostic workup. Doherty reviews the evidence regarding the diagnosis and treatment of pulmonary embolism. He discusses how clinical criteria such as Wells criteria can be helpful in assessing the likelihood of a PE. He recommends immediate treatment in patients who have a PE. Figure/Illustration A is an ECG demonstrating sinus tachycardia with a P wave, a QRS complex, and a T wave during every beat (red circle). This is the most common ECG presentation in PE, though right heart strain can also be seen. Figure B is a chest radiograph that is within normal limits without evidence of fluid in the dependent portions of the lung (red circles). This is the most common radiological presentation in PE. Incorrect Answers: Answer A: Aspirin would be the most appropriate initial step in the management of MI and can reduce mortality by up to 25% if administered promptly. PE is a more likely diagnosis in this patient given the ECG that only demonstrates sinus tachycardia. Answer B: Cardiac troponins could be used to confirm the diagnosis of myocardial infarction (MI). In the presence of this patient's ECG (which does not demonstrate ST elevation), MI is a less likely diagnosis. Treatment would be with aspirin in patients who had an MI followed by emergent revascularization. Answer C: A CT scan of the chest without contrast would not confirm the diagnosis in this patient; however, a CT angiogram would be the most accurate test. It is more important to begin treatment with heparin when there is a very high clinical suspicion of PE. Answer E: A ventilation perfusion scan is the most accurate test for PE in a patient who cannot tolerate contrast. This test has a low specificity. Bullet Summary: The most appropriate initial step in the management of a pulmonary embolism in patients with a high pre-test probability is anticoagulation with heparin.
Ventilation perfusion scan
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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?
Artificial tears
Intramuscular ceftriaxone
Oral erythromycin
Topical bacitracin
E
Topical erythromycin
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.
Topical erythromycin
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A 25-year-old G1P0 woman presents to her physician for a prenatal visit. She is at 12 weeks gestational age and has not received prior prenatal care. She has mild morning sickness but no other symptoms. She has no known medical problems and takes only a prenatal multivitamin. She has unprotected sexual intercourse with 3 male partners. Her temperature is 98.6°F (37.0°C), blood pressure is 118/70 mmHg, pulse is 80/min, and respirations are 18/min. Physical exam reveals a well-appearing woman in no acute distress. There are no lesions appreciated on skin exam. Cardiopulmonary exam is unremarkable. An antibody test for human immunodeficiency virus (HIV)-1/2 is positive. The patient is started on an appropriate treatment regimen. Assuming that her HIV viral load is undetectable by the time of delivery, which of the following maternal regimens is she most likely receiving and what neonatal prophylaxis is indicated (Figure A)?
A
B
C
D
C
C
This pregnant patient presents with a positive HIV antibody test in the setting of unprotected sexual intercourse. An appropriate maternal HIV treatment regimen is abacavir, lamivudine, and raltegravir; prophylaxis in the infant consists of zidovudine alone if maternal HIV viral load is suppressed to < 50 copies/mL. All pregnant women should be screened for HIV as early as possible during pregnancy. Maternal treatment for HIV should be initiated as early as possible (before pregnancy if possible) with highly-active anti-retroviral therapy (HAART) consisting of 2 nucleoside reverse transcriptase inhibitors (NRTIs) such as abacavir/lamivudine, plus an integrase inhibitor (e.g., raltegravir) or a protease inhibitor (e.g., ritonavir/atazanavir). Regimens for prophylaxis in infants of HIV-positive mothers depends on the HIV viral load at the time of delivery. If the viral load is < 50 copies/mL, infant prophylaxis with zidovudine alone for 4 weeks is sufficient. If the viral load is ≥ 50 copies/mL or the mother did not receive HAART during pregnancy, infant prophylaxis should consist of the 3-drug regimen of: zidovudine, lamivudine, and either nevirapine or raltegravir for 6 weeks. Harris et al. reviewed the treatment of HIV infection in pregnancy. They found that although anti-retroviral therapy during pregnancy significantly reduces mother-to-child transmission of HIV, it is still associated with certain perinatal complications such as low birthweight and preterm delivery. Despite these risks, they recommend anti-retroviral therapy during pregnancy and during breastfeeding to prevent transmission. Figure A shows the answer choices. Incorrect Answers: Answer A: Maternal treatment with abacavir/lamivudine/raltegravir and no neonatal treatment is inappropriate because all infants of HIV-positive mothers should receive zidovudine (if maternal viral load < 50 copies/mL at delivery). Lamivudine and raltegravir would be added if maternal viral load is ≥ 50 copies/mL at delivery. Answer B: Maternal treatment with abacavir/lamivudine/raltegravir and neonatal prophylaxis with zidovudine/lamivudine/raltegravir would be appropriate if the maternal viral load were ≥ 50 copies/mL at delivery. The HIV viral load in this patient was assumed to be undetectable at delivery. Answer D: Maternal treatment with raltegravir/ritonavir/atazanavir and neonatal prophylaxis with zidovudine/lamivudine/raltegravir is inappropriate because raltegravir is an integrase inhibitor and ritonavir/atazanavir are protease inhibitors. HAART must include 2 NRTIs. Atazanavir is often co-administered with ritonavir to increase the former’s bioavailability. Additionally, zidovudine/lamivudine/raltegravir is the neonatal regimen for infants whose mothers have a viral load ≥ 50 copies/mL at delivery. Answer E: Maternal treatment with raltegravir/ritonavir/atazanavir and neonatal prophylaxis with zidovudine only is inappropriate because HAART for the mother must include 2 NRTIs. Bullet Summary: Maternal HIV infection should consist of highly-active anti-retroviral therapy consisting of 2 nucleoside reverse transcriptase inhibitors plus either an integrase inhibitor or protease inhibito, while neonatal prophylaxis consists of zidovudine only for infants of mothers with viral load < 50 copies/mL at delivery.
E
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A 59-year-old woman presents to her primary care physician for trouble sleeping. When she goes to bed at night, she has an urge to get up out of bed and walk around. The patient often wakes her husband when she does this which irritates him. There is a perpetual uneasiness and feeling of a need to move at night which is relieved by getting up and walking around. The patient denies symptoms during the day. She works as a mail carrier and is nearing retirement. She has a medical history of anxiety, depression, irritable bowel syndrome, and dysmenorrhea. She is not currently taking any medications. Her temperature is 99.5°F (37.5°C), blood pressure is 157/98 mmHg, pulse is 80/min, respirations are 17/min, and oxygen saturation is 98% on room air. Physical exam reveals 5/5 strength in the upper and lower extremities, 2+ reflexes in the upper and lower extremities, a stable gait pattern, and normal sensation. Cardiopulmonary and abdominal exams are within normal limits. Which of the following is the most appropriate initial step in management?
Alprazolam
Iron studies
Pramipexole
Supportive therapy and an exercise routine
B
Iron studies
This patient is presenting with the need to get up and walk around at night, which is suggestive of restless leg syndrome. The most appropriate initial step in management is collecting iron studies to assess for iron deficiency. Restless leg syndrome presents with a sustained sense of restlessness and a feeling of a need to move one's legs. Symptoms typically occur at night and are relieved by activities such as walking. Iron deficiency can be a precipitating factor in restless leg syndrome as iron is a cofactor in the production of dopamine. Prior to administering iron, it is important to first confirm or rule out the diagnosis of iron deficiency with laboratory values which should include a CBC and iron studies. If iron deficiency exists, iron should be administered. If iron deficiency is not present, then dopamine agonists such as pramipexole or ropinirole can be administered. Gossard et al. review the evidence regarding the diagnosis and treatment of restless leg syndrome. They discuss how iron deficiency is associated with the development of this disorder. They recommend iron repletion in deficient patients. Incorrect Answers: Answer A: Alprazolam could be an appropriate step in management if the patient suffers from episodic panic attacks/anxiety. It is not an optimal long-term therapy for anxiety or depression. Benzodiazepines are not first-line in treating restless leg syndrome. Answer B: Ferrous sulfate would not be indicated until iron deficiency is confirmed. Iron deficiency is associated with restless leg syndrome as iron is a cofactor in the production of dopamine. Answer D: Pramipexole is a dopamine agonist that is an effective therapy for restless leg syndrome. However, it is more important to treat any underlying causes for restless leg syndrome such as iron deficiency prior to starting a dopamine agonist. Answer E: Supportive therapy and an exercise routine ignore the potential underlying pathology and do not offer any specific treatment for this patient's restless leg syndrome. Exercise can improve symptoms of restless leg syndrome but iron deficiency must still be ruled out. Bullet Summary: Patients with restless leg syndrome should be assessed for iron deficiency and treated if they are iron deficient.
nan
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A 45-year-old morbidly obese woman with a history of asthma and type 2 diabetes mellitus presents to her primary care physician for advice on weight loss. She thinks that her husband is having sex with other women because they have not had sex over the past year. She feels that her co-workers also disrespect her for her weight and constant sweat stains around her armpits and chest. She has noticed that the sweat stains get itchy and induce a burning sensation unless she showers or changes her shirt. She has been compliant with her medications, which include albuterol, metformin, glyburide, and atorvastatin. Her temperature is 98.6°F (37°C), blood pressure is 128/85 mmHg, pulse is 91/min, and respirations are 11/min. On physical exam, the patient has a flat affect, with moist oral mucosa and nasal polyps. She denies sinus tenderness. Her neck is thick with a posterior cervical fat pad. During cardiac auscultation, the finding in Figure A is noted below her breasts. What is the most appropriate next step in management for this finding?
Fluconazole
Prednisone
Topical clobetasol
Topical imiquimod
E
Topical nystatin
This patient has a rash consistent with candidal intertrigo given the location and history of a damp, pruritic rash. The most appropriate next step in management is topical nystatin powder. Intertrigo is caused by the Candida species and is characterized by pruritic, painful, and erythematous superficial patches surrounded by satellite lesions. They are commonly seen in intertriginous areas such as the breasts, groin, axilla, or abdominal pannus. Obesity and diabetes are significant risk factors. Initial management involves maintaining skin dryness along with medical treatment using topical nystatin, clotrimazole, or miconazole. Persistent lesions may require oral antifungal treatment. Nobles and Miller review the evidence regarding the diagnosis and treatment of intertrigo. They discuss how these lesions often become infected with Candida. They recommend the use of antifungal medications. Figure/Illustration A shows candidal intertrigo in the breast fold. Note the satellite lesions that surround the erythematous patch. Incorrect Answers: Answer A: Fluconazole is indicated for moderate to severe disease or if the rash is refractory to topical antifungals. Topical antifungal agents should be used before progressing to oral medications. Answer B: Prednisone is not indicated and would potentially worsen the rash through immunosuppression and exacerbation of the patient's diabetes mellitus. Answer C: Topical clobetasol is a high-potency corticosteroid. This would not be used because it can promote infection and lead to skin atrophy. Low-potency corticosteroids such as triamcinolone or hydrocortisone can be used as adjuncts for symptom control only. Answer D: Topical imiquimod is indicated for autoimmune skin diseases such as alopecia areata not for candidal intertrigo. This disease would present with segmental regions of hair loss Bullet Summary: The treatment of candidal intertrigo involves topical antifungals including nystatin, clotrimazole, and miconazole.
Topical nystatin
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A 67-year-old man is seen on the surgical floor after a transplant procedure. The previous day, the patient had a renal transplant from a matched donor. He is currently recovering and doing well. The patient has a medical history of IV drug use, diabetes mellitus, oral cold sores, hypertension, renal failure, and dyslipidemia. The patient's current medications include lisinopril, atorvastain, insulin, and aspirin. Prior to the procedure, he was also on dialysis. The patient is started on cyclosporine. The patient successfully recovers over the next few days. His temperature is 98.5°F (36.9°C), blood pressure is 121/76 mmHg, pulse is 70/min, and respirations are 13/min. Which of the following medications should be started in this patient?
Valacyclovir
Low dose acyclovir
TMP-SMX
Azithromycin
C
TMP-SMX
This patient is presenting status post renal transplant and is currently on immunosuppressive therapy. This patient should be started on TMP-SMX as prophylaxis against Pneumocystis jiroveci pneumonia (PJP). After a transplant procedure, patients are started on immunosuppressive therapy in order to avoid rejection of the transplanted organ. The most important interventions for a patient after a transplant procedure are to receive all of their vaccinations and to receive TMP-SMX as prophylaxis against PJP. Other appropriate but less dire measures include giving ganciclovir as prophylaxis against cytomegalovirus (CMV). Patients should be closely monitored to avoid transplant rejection as well as infection. Weyant et al. review the evidence regarding the treatment of patients with PJP. They discuss how TMP-SMX has been the classic prophylaxis and treatment for this disease. They recommend considering echinocandins for patients who experience significant side effects. Incorrect Answers: Answer A: Azithromycin is appropriate prophylaxis for patients with HIV with CD4 counts < 50. Patients on immunosuppressive drugs should receive some of the same prophylactic medications but they do not require azithromycin upon initiation of immunosuppressive medications. Answer B: Low-dose acyclovir would be an inappropriate intervention in this patient after a transplant procedure. Ganciclovir and high-dose acyclovir are the appropriate options for prophylaxis of CMV. Answer C: Penicillin can be used as prophylaxis for patients with rheumatic heart disease or asplenia; however, it would not be the most appropriate initial measure in a transplant patient. Answer E: Valacyclovir is the treatment for herpes simplex virus (HSV). It is not routinely indicated as prophylaxis in transplant patients. HSV will present with vesicular rashes developing on the affected areas. Bullet Summary: Patients on immunosuppressive therapy after a transplant should be vaccinated (pneumovax, hepatitis A and B, influenza, and tetanus-diphtheria) and started on TMP-SMX.
nan
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A 22-year-old man presents to the emergency department with difficulty breathing. He has experienced similar symptoms before. Today, he was hit in the face with a football, followed by significant swelling of his lips and tongue. The patient speaks with a muffled voice. His temperature is 98.1°F (36.7°C), blood pressure is 120/83 mmHg, pulse is 80/min, respirations are 16/min, and oxygen saturation is 98% on room air. Physical exam reveals swollen lips and a protruding, enlarged tongue. His breath sounds are clear. He demonstrates no rash and his abdomen is soft and nontender. Which of the following is the most effective therapy for this patient?
Diphenhydramine
Dexamethasone
Fresh frozen plasma
Epinephrine
C
Fresh frozen plasma
This patient is presenting with recurrent episodes of significant swelling of his lips and tongue with minor trauma, which are concerning for hereditary angioedema. Fresh frozen plasma is the most effective therapy for hereditary angioedema of the options listed. Angioedema is a potentially life-threatening condition that presents with severe edema of the gastrointestinal and respiratory tracts. It can be caused by antigens, medications, and trauma or may occur spontaneously. It often presents with notable edema of the lips and tongue and may threaten the airway. There are 3 subtypes. Hereditary and acquired angioedema occur secondary to a deficiency of C1-esterase inhibitor. As a result, the most effective therapy is to administer fresh frozen plasma which has C1-esterase inhibitor. On the other hand, drug-induced angioedema (often from angiotensin-converting enzyme inhibitors and angiotensin receptor blockers) does not respond well to C1-esterase inhibitor replacement and supportive therapy; discontinuing the offending agent is the mainstay of therapy. Other effective but costly treatments that can be used in hereditary angioedema include: icatibant, ecallantide, and C1 inhibitor. Paige and Anderson note the management of hereditary angioedema. They review the different treatments and discuss C1-esterase inhibitor replacement. They recommend this treatment for acute episodes. Incorrect Answers: Answer A: Dexamethasone and other steroids can be given in acute allergic reactions and may reduce the recurrence of symptoms but are unlikely to be effective in angioedema, nor are they the most effective therapy. Steroids can take up to 4 hours to begin having any effect. Answer B: Diphenhydramine is an antihistamine that could be given for anaphylaxis or urticaria and works immediately; however, it is not the mainstay of therapy for angioedema nor is it the most effective therapy. It is the mainstay of therapy for urticaria. It can also be given in anaphylaxis. Answer C: Epinephrine is the treatment of choice for anaphylaxis which presents with urticaria, upper airway swelling, hypotension, nausea/vomiting, and wheezing. It is often given in angioedema but has limited efficacy when compared to fresh frozen plasma. Answer E: Tranexamic acid is effective in treating angioedema and current evidence supports its use; however, the mainstay of therapy is still fresh frozen plasma. Tranexamic acid is also used in hemorrhagic shock to stop fibrinolysis and reduce bleeding. Bullet Summary: A possible treatment in hereditary or acquired angioedema is the administration of fresh frozen plasma to replace C1-esterase inhibitor.
nan
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A 55-year-old man presents to the emergency department after feeling lightheaded then fainting while he was carrying boxes upstairs. The patient works on a farm and does not see a doctor often. He has a history of hypertension but does not take medications routinely. He has not had any chest pain, dyspnea, or weakness during this time frame and currently feels at his baseline. He smokes cigarettes occasionally. His temperature is 98.1°F (36.7°C), blood pressure is 152/93 mmHg, pulse is 86/min, respirations are 15/min, and oxygen saturation is 98% on room air. Physical exam is unremarkable and the patient walks with a stable gait and no focal weakness. An ECG is performed as seen in Figure A. Laboratory studies are ordered as seen below. Hemoglobin: 12 g/dL Hematocrit: 36% Leukocyte count: 6,500/mm^3 with normal differential Platelet count: 197,000/mm^3 Serum: Na+: 140 mEq/L Cl-: 102 mEq/L K+: 4.3 mEq/L HCO3-: 24 mEq/L BUN: 20 mg/dL Glucose: 139 mg/dL Creatinine: 1.1 mg/dL Troponin: < 0.01 ng/mL Which of the following is the most likely diagnosis?
Left ventricular hypertrophy
Non-ST elevation myocardial infarction
ST elevation myocardial infarction
Torsades des pointes
A
Left ventricular hypertrophy
This patient with chronic untreated hypertension is presenting with syncope and an ECG demonstrating large voltages in leads V1-V6 and a left ventricular strain pattern (ST depressions in V5/V6 and aVL), which are concerning for left ventricular hypertrophy. Left ventricular hypertrophy occurs secondary to chronic hypertension or conditions that increase afterload such as aortic stenosis. This leads to an increase in the mass of the left ventricle. There are many possible criteria on ECG for left ventricular hypertrophy including left axis deviation, large R waves, a large S wave in aVR, or ST segment depression and T wave inversion in the left heart leads (referred to as left ventricular strain). A more simple method of recalling left ventricular hypertrophy is very large voltages in the ECG leads that serve the left ventricle. Left ventricular hypertrophy should be considered in presentations such as syncope. Note that while ECG findings suggest left ventricular hypertrophy, the diagnosis must be supported with echocardiography. Management involves treating the underlying cause (most commonly, this is hypertension). Artham et al. discuss left ventricular hypertrophy. They note that it is a risk factor for future cardiac events and increased mortality. They recommend early diagnosis and management to improve outcomes. Figure/Illustration A is an ECG with very large voltages (red arrows) and ST segment depressions/T wave inversions (blue arrows, a more classic left ventricular strain pattern), concerning for left ventricular hypertrophy. Incorrect Answers: Answer B: Non-ST elevation myocardial infarction would present with nonspecific ST segment and T wave changes with an elevated troponin. While this patient does demonstrate ST depressions in the lateral leads, the troponin level is normal. Answer C: ST elevation myocardial infarction would present with ST elevation in a vascular distribution on ECG in the setting of chest pain, diaphoresis, and an elevated troponin level. Note that this patient’s lack of symptoms (pain) and normal troponin in the setting of the ST elevation in leads V1-V3 make this unlikely to be ST elevation myocardial infarction and more likely to be left ventricular hypertrophy. Answer D: Torsades des pointes occurs in patients with a prolonged QT interval that evolves into twisting of the QRS complexes around an isoelectric baseline. It may cause syncope or cardiac arrest. Note there is no QT prolongation on this patient’s ECG that would predispose him to this condition. Answer E: Wolff Parkinson White syndrome presents with a delta wave on ECG (a slurred upstroke into the QRS complex) and may undulate with supraventricular or ventricular tachycardia. There is no delta wave in this patient’s ECG supporting this diagnosis. Ablation is the most effective long-term treatment. Bullet Summary: Left ventricular hypertrophy can be associated with syncope and presents with large voltages in the leads that serve the left ventricle and a left ventricular strain pattern.
Wolff Parkinson White syndrome