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A 37-year-old man presents to the emergency department (ED) for a severe sudden "tearing" chest pain that radiates to his back. The patient is tall, has abnormal laxity of his joints, and long extremities. A medical student suggests that this patient has Marfan syndrome, whereas the resident in the ED contends that long extremities and joint laxity may be features of other inherited conditions. Which of the following mechanism's explains the resident’s argument? +
A 2-year-old girl is brought to the pediatrician by her mother for excessive drinking and urination. The patient’s mother recalls an uneventful pregnancy and delivery. The patient had a normal neonatal growth course and has successfully met previous developmental milestones. The patient’s vital signs are a temperature of 37.2 C (99.0F), pulse of 140 bpm, and blood pressure of 110/80 mmHg. On physical examination, the patient’s skin turgor shows delayed recoil and her extremities are mottled and cyanotic. Physical examination is otherwise unremarkable. Laboratory testing is significant for metabolic alkalosis, hypokalemia, elevated plasma renin and aldosterone. Urinalysis reveals markedly elevated urinary calcium and potassium with no evidence of casts, hematuria, or leukocyturia. What is the most likely diagnosis? +
A 46-year-old male Chinese patient presents to the physician for uncontrolled hypertension. The patient is completely asymptomatic and has detected high blood pressures incidentally on several occasions that prompted his medical visit. Patient has an unremarkable past medical history. He does not take any medication. Before the diagnosis of essential hypertension can be made, which of the following questions might be helpful to rule out secondary causes of hypertension? +
Two medical teams are conducting identical studies that aim to detect an association between vitamin K deficiency and osteoporosis in France and Russia. Both teams are using the same test kit that is designed to measure vitamin K concentration in the blood. The sensitivity of the test is 90% and its specificity is 85%. According to previous studies, the prevalence of vitamin K deficiency is higher in Russia than it is in France. Based on the previous information, which of the following statements is correct? +
A 23 year old male patient presents to his primary care physician for two-month-history of abdominal pain. The pain is intermittent in nature and is not associated with a particular type of food. The patient reports several episodes of bloody diarrhea and generalized fatigue. The patient underwent several blood and stool tests and was referred to a GI specialist for endoscopic evaluation. The endoscopy reveals deep ulcerations in a patchy distribution in the small intestine and colon. Which of the following findings is consistent with the diagnosis? +
A 35-year-old woman presents to her ophthalmologist for impaired dark adaptation. Over the past month she has experienced increasing difficulty distinguishing objects, especially when driving home from work at night. Her past medical history is significant for severe Crohn's disease. She denies any family history of ocular disease. She reports her family history is only significant for essential hypertension in her father and dyslipidemia in her mother. On physical examination, the physician notes white patches on the oral mucosa. What is the most likely diagnosis? +
A 1-year-old boy is brought by his parents for failure to thrive. The parents report that their child appears listless and disengaged. He has failed to begin sitting on his own and responds poorly to verbal cues. On physical examination, the pediatrician notes that the patient is very pale. A complete blood count demonstrates Hb=7.2 g/dL and MCV=71 fLa. Hemoglobin electrophoresis demonstrates absent HbA, elevated HbA2 and HbF, and negative HbS. Chronic treatment for this child’s condition is likely to cause symptoms most similar to the genetic disease caused by mutations in which of the following? +
A 54-year-old homeless man presents to the emergency department with back pain, fever, and dry cough. He reports that these symptoms have approximately been present for the past 2 months. The patient was evaluated for similar symptoms 1 month earlier but a definite diagnosis was not made at the time. He reports he has been smoking 1 pack of cigarettes daily for the past 30 years and drinks 7-8 beers each day. The patient denies intravenous drug use. Upon review of systems, the patient complains of a 6-month history of anorexia, weight loss, and night sweats. Work-up in the ED is remarkable for a hilar opacity in the lung and presence of a mass with lytic destruction of the anterior portion of the T6-T9 vertebral bodies on imaging. CT-guided fine needle aspiration (FNA) of the vertebral mass confirms the diagnosis. Which of the following organisms is most likely responsible for this patient's symptoms? +
A 36-year-old gravida-2 para-2 woman, with a past medical history of hypertension, obesity, and diabetes mellitus, brings her son to the emergency department for increasing irritability, fever, and a rash. The mother explains she gave birth to her infant 3 weeks ago in a foreign country. She reports that she had painful genital lesions during delivery but were left untreated. Shortly after admission, the child develops a seizure. An MRI of the brain reveals a right temporal lobe infiltrate. Which of the following is most likely responsible for this patient's condition? +
A 41-year-old man with Down syndrome is brought to the family's physician for cognitive decline. The family reports that the patient’s memory has been deteriorating for the past year. He cannot recall the names of his brothers and sisters and often has difficulty finding words. He is unable to find his way back home and gets lost when a member of his family does not assist him. Which of the following neuropathologic changes is most likely present in this patient? +
A 43-year-old woman presents to her primary care physician for severe joint pain and fatigue over the past three months. She complains of joint stiffness in the morning, which improves throughout the day. She describes her pain as a persistent, dull pain that “hurts all over”. On physical examination, the physician notes mild joint swelling around the knee and ulnar deviation of the fingers. Laboratory testing reveals a positive ELISA for anti-IgG antibodies. The patient is started on a first-line disease-modifying drug, but 2 years later is found to be critically leukopenic. Following appropriate work-up, the physician suspects the patient's leukopenia is a an adverse event associated with the drug. Which of the following medications can be used to quickly reverse the effects of the administered drug? +
A 45-year-old man presents to his primary care physician with painless jaundice. Abdominal CT shows a mass at the head of the pancreas. Biopsy confirms the diagnosis of pancreatic adenocarcinoma. The patient is treated with an analogue of uracil. Which of the following describes the mechanism of action of this drug? +
A 76-year-old woman, with a past medical history significant for gout on allopurinol, presents to her primary care physician for fatigue and weight loss. Complete blood count and peripheral smear reveal increased number of lymphoblasts. Bone marrow biopsy confirms the diagnosis of Acute Lymphoblastic Leukemia (ALL). Remission is induced with combination chemotherapy consisting of dexamethasone, vincristine, asparaginase, and daunorubicin. Repeat bone marrow biopsy later reveals residual disease that requires intensification therapy. Which of the following drugs should be avoided in this patient? +
A scientist is studying the effects of a chemotherapeutic agent on a lymphoma cell line. He treats the cells with the chemotherapeutic agent and then stains them with propidium iodide, a molecule that binds to DNA and fluoresces in proportion to the amount of DNA. He then measures the propidium iodide staining of the cells with flow cytometry and observes the following plot (shown below). Which of the following chemotherapeutic agents was most likely used by this investigator?
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<img src="http://static.wikidoc.org/7/7b/WBR0159.svg" style="width:600px"> +
A 7-year-old boy is brought by his mother to the physician's office for pain in his left thigh. Following history-taking and physical examination, X-ray shows a destructive mass at the metadiaphysis of the left femur. Biopsy demonstrates anaplastic, small, blue, CD99 positive cells. Karyotype of the tumor demonstrates a t(11;22) translocation consistent with Ewing’s sarcoma. The patient is placed on a chemotherapy regimen which includes an agent that inhibits RNA polymerase by inserting into the minor groove of the DNA double-helix between adjacent cytosine and guanine base pairs. Which of the following agents is most likely administered in this patient? +
A 75-year-old man presents to his physician for excessive pruritus that is exacerbated by taking showers. On physical exam, the spleen is palpable 9 cm below the costal margin. Laboratory testing is notable for a hematocrit of 70%. Further testing confirms the diagnosis with the presence of a ''JAK2'' mutation. Therapy consisting of regular phlebotomy and an agent that inhibits ribonucleotide reductase is initiated. For which of the following conditions may this drug also be used? +
A 74-year-old woman with chronic lymphocytic leukemia (CLL) presents to her oncologist for worsening fever and weight loss over the past month. The oncologist initiates therapy with fludarabine, cyclophosphamide, and rituximab. Three weeks later, the patient experiences bloody urine with suprapubic tenderness. Which of the following drugs could have prevented this condition? +
A 45-year-old man presents to his primary care physician for fatigue and weight loss of 10 pounds over the past month. On physical examination, the spleen is palpable 7 cm below the costal margin, and several bruises are observed across the body. Laboratory testing is significant for an elevated white blood cell count and reduced platelet count. Bone marrow biopsy demonstrates hyperplastic granulocytes which are positive for a t(9;22) translocation. The patient is started on an inhibitor of the fusion gene produced by this translocation. The administered drug is most likely also effective to treat which of the following conditions? +
A 3-year-old boy is brought to the pediatrician by his parents after they noticed blood in the child’s urine. Physical examination is remarkable for a palpable left flank mass. Biopsy of the mass reveals a mixed population of tubules, small round blue cells, and stroma. The child undergoes left nephrectomy and is placed on a course of adjuvant chemotherapy. Two weeks after the administration of chemotherapy, the child complains of dizziness, has trouble walking, and displays difficulty in dorsiflexion of both feet. What is the mechanism of action of the drugs most likely responsible for this patient's symptoms? +
A 62-year-old woman presents to her physician for vague abdominal pain, bloating, and unintentional 10-pound weight loss over the past two months. Pelvic examination is remarkable for an adnexal mass. A transvaginal ultrasound shows a pelvic mass. Further work-up reveals markedly elevated serum CA-125 level. When the therapeutic regimen is explained to the patient, the patient recognizes the name of one drug saying, “That drug was on the stent my husband received after his myocardial infarction”. What is the mechanism of action of the most likely chemotherapeutic agent? +