KROK 1 2009 – Question 8 · KROK (Medicine) · Biological Chemistry
A baby refuses the breast, he is anxious, presents with arrhythmic respiration. The urine smells of "brewer’s yeast" or "maple syrup". This pathology was caused by the inherited defect of the following enzyme:
- A. Glycerol kinase
- B. Glucose 6-phosphate dehydrogenase
- C. UDP-glucuronyl transferase
- D. Aspartate aminotransferase
- E. Dehydrogenase of branched-chain alpha-keto acids
Hint
Think about which inherited enzyme defect in amino acid catabolism produces a characteristic urinary odour and neurological signs in a newborn.
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