Pediatrics Deficiency of enzyme a-keto acid decarboxylase leading to a block in the metabolism of branch chain amino acids is observed in A Maple syrup urine disease B Hanup's disease C Alkaptonuria D Phenylketonuria 💡 Explanation Deficiency of enzyme a-keto acid decarboxylase leading to a block in the metabolism of branch chain amino acids is observed in ✓ Correct Answer: A. Maple syrup urine disease