A 6-year-old has hypoglycemia on fasting, hepatomegaly and lactic acidosis. Which enzyme is deficient in this glycogen storage disease?
Answer and explanation
Correct answer: B. Glucose-6-phosphatase
- Step 1: Von Gierke disease prevents the liver from releasing free glucose. Answer: Glucose-6-phosphatase. Why not the others: Pompe disease affects the heart and muscle, McArdle affects muscle only and debranching deficiency has milder hypoglycemia.
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