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Practice sample: USMLE Step 1 exam

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Practice sample: USMLE Step 1 exam

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Question 1 of 5

Question 1.A 6-month-old girl has a markedly protuberant abdomen, doll-like facies, and stunted growth. After a brief fast she becomes hypoglycemic and tachypneic. Labs show glucose 38 mg/dL, lactate 7.5 mmol/L, triglycerides 850 mg/dL, and uric acid 9.5 mg/dL. Liver biopsy shows hepatocytes massively distended with glycogen and lipid, with NO increase in glycogen-branching abnormalities. The defect impairs the terminal dephosphorylation shared by both glycogenolysis and gluconeogenesis. Which enzyme deficiency, and why is uric acid elevated?

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USMLE Step 1 Exam Prep is built as exam practice, not a generic sales page. This uses the same timed runner enrolled students use for practice.

  • Available bank: 2,112 questions
  • Full student attempts: up to 40 questions per attempt
  • Shuffled choices and explanations after submission
  • Original questions; no real or recalled exam items
Topics in this sample
  • Glycolysis — von Gierke disease (glucose-6-phosphatase deficiency)
  • Pharmacodynamics: efficacy vs potency
  • Neuromuscular junction: presynaptic toxin mechanism
  • Congenital heart defects: maternal infection and patent ductus arteriosus organism
  • Cerebrovascular pathology: hypertensive intracerebral hemorrhage

National Course Portal is an independent provider. This sample uses original educational prep questions and does not include real, recalled, or leaked questions from any official exam.