Pediatrics Deficiency of enzyme α–keto acid decarboxylase leading to a block in the metabolism of branch chain amino acids is observed in – A Maple syrup urine disease B Hartnup's disease C Alkaptonuria D Phenylketonuria 💡 Explanation Deficiency of enzyme α–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