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Biochemistry · Carbohydrate metabolism: fructose
Sample itemA child develops vomiting, hypoglycemia, and jaundice after introduction of fruit juice. Which enzyme deficiency is most likely?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
D. Aldolase B deficiency
Hereditary fructose intolerance is caused by aldolase B deficiency, causing accumulation of fructose-1-phosphate and impaired gluconeogenesis. Fructose should be avoided once recognized.
Option by option
A. Galactokinase deficiency
Galactokinase deficiency affects galactose metabolism.
B. Fructokinase deficiency
Fructokinase deficiency causes benign essential fructosuria.
C. Lactase deficiency
Lactase deficiency causes osmotic diarrhea after lactose ingestion.
D. Aldolase B deficiency (correct)
Correct. Aldolase B deficiency causes toxic fructose-1-phosphate accumulation.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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