Analysis of diagnosis and treatment in twelve patients with maple syrup urine disease

Twelve patients with maple syrup urine disease were treated with restricted natural protein intake,special nutrition powder,and vitmin B1.The manifestations of these patients were greatly improved after treatment.The blood levels ofleucine and valine were significantly decreased after treatment(P＜0.01).Although the level of branched-chain α-ketoacids in urine was reduced,but not significantly. Key words: Maple syrup urine disease;  Diagnosis;  Treatment