KROK 1 2023 – Question 137 · KROK (Medicine) · Biological Chemistry

The patient's body fluids, especially urine, have a specific sweet smell, caused by disturbed metabolism of certain amino acids, such as leucine, isoleucine, and valine. What disease can be characterized by these pathological changes?

  1. A. Fructosuria
  2. B. Alkaptonuria
  3. C. Galactosemia
  4. D. Maple syrup urine disease
  5. E. Phenylketonuria
Hint

Think about the enzyme complex that decarboxylates the keto acids derived from branched-chain amino acids, and what happens to body odour when it is deficient.

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