枫糖尿病患者12例诊治分析  

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

作　　者：韩连书[1] 杨楠[1] 叶军[1] 邱文娟[1] 张惠文[1] 高晓岚[1] 王瑜[1] 李筱燕[1] 许浩[1] 顾学范[1] 

机构地区：[1]上海交通大学医学院附属新华医院,上海币儿科医学研究所,200092

出　　处：《中华内分泌代谢杂志》2012年第11期914-915,共2页Chinese Journal of Endocrinology and Metabolism

基　　金：国家863高技术发展计划（2007AA022447）;上海市卫生局重大课题（2008ZD001）;上海市卫生局科研课题（2009210）;上海市科委重大课题（11dz1950300）

摘　　要：枫糖尿病患者12例，经给予限制天然蛋白质摄入、特殊营养粉及维生素B，治疗后，临床症状明显改善，血亮氨酸和缬氨酸水平较治疗前明显下降（P〈0．01），尿支链仅一酮酸较治疗前有所下降，但未达显著程度。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 of leucine and valine were significantly decreased after treatment（ P〈0. O1 ）. Although the level of branched-chain a-ketoacids in urine was reduced, but not significantly.

关 键 词：枫糖尿病 诊断 治疗 

分 类 号：R725.8[医药卫生—儿科]