USMLE Step 1 Practice Questions - United States Medical Licensing Examination Step 1 Exam

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A 6-month-old infant presents with fasting hypoglycemia, hepatomegaly, and lactic acidosis. A liver biopsy shows glycogen accumulation. Which enzyme deficiency is most likely?

Glucose-6-phosphatase Glycogen phosphorylase Branching enzyme Debranching enzyme

Correct Answer:

Glucose-6-phosphatase

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