低苯丙氨酸饮食治疗苯丙酮尿症的临床研究  

Clinical study on low - phenylalanine dietary treatment in phenylketonuria.

作　　者：李晓雯[1] 喻唯民[2] 王琳[2] 贺春[2] 

机构地区：[1]中日友好医院营养部,北京100029 [2]中日友好医院临床医学研究所

出　　处：《中国优生与遗传杂志》2006年第2期112-113,116,共3页Chinese Journal of Birth Health & Heredity

摘　　要：目的总结20年对652例苯丙酮尿症患儿进行低苯丙氨酸饮食治疗经验,探讨有效的饮食控制方案。方法由营养师计算制定食谱,对治疗患儿进行严格饮食控制,并定期测定血中苯丙氨酸浓度,根据血浓度调整饮食,使其维持在理想水平。结果严格随诊早治疗的60例患儿,其体重、身高、头围均在正常儿童相应标准值的±2s之间。<3个月开始治疗的56例智商可达96±10,晚治疗的83例,治疗前为61±23,治疗后为72±26。治疗前后智商比较有显著性差异( P<0．05)。结论合理安排食谱,正确添加辅食是保证患儿正常生长、发育、维持血苯丙氨酸浓度在理想范围的关键,而且一定要在营养师指导下注意患儿的全方位营养。Objective： To explore effective dietary regimens in treating phenylketonuria （PKU） after summing up 20 years practice in 652 patients. Methods ： Patients＇ daily diet were instructed by the dietation who optimize their diets according to their plasma phenylalanine levels. In order to keep their favorite levels, patients were detected regularly. Results ： Of 60 patients who were under early treatment and follow up, their body weight, height and head circumferenee were within normal range （average ＋2s）. For 56 patients who were treated within 3 months of age, their intellectual quotient （1Q） were 96 ± 10, while the 83 late - treated patients only got IQ 72 ± 26, Significant difference were found in two groups （ P 〈 0. 05）. Conclusion： Strictly diet regimen with proper supplementation are the key to keep ideal phenylalanine levels which will end in normal or nearly normal development. In this point, the dietation play an important role in keep ideal phenylalanine levels and full nutrition.

关 键 词：苯丙酮尿症 饮食治疗 智商 体格发育 

分 类 号：R725.9[医药卫生—儿科]