KROK 1 2023 – Question 71 · KROK (Medicine) · Biological Chemistry
A child with von Gierke disease presents with slow growth and enlarged liver and kidneys. Reduced glucose levels and increased levels of fats and uric acid are detected in the child’s blood. What enzyme is absent in this case, causing this type of glycogenosis?
- A. Phosphofructokinase
- B. Glucose-6-phosphatase
- C. Glycogen synthase
- D. Amylo-1,6-glucosidase
- E. Hepatic phosphorylase
Hint
Fasting hypoglycemia with an enlarged liver and raised lactate, lipids and uric acid in a glycogen storage disease means the last step of glucose release from the liver is blocked.
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