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Exam questionKROK 1Medicine ยท 2009

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:

Answer options

  1. Aspartate aminotransferase
  2. UDP-glucuronil transferase
  3. Glucose 6-phosphate dehydrogenase
  4. Dehydrogenase of branched-chain alpha-keto acids
  5. Glycerol kinase
Correct answer

Dehydrogenase of branched-chain alpha-keto acids

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