Abstract: Objective To research on phenylketonuria children in low-phenylalanine milk treatment,comparison of breast milk and formula milk on blood phenylalanine values. Methods Children patients who had been diagnosed of phenylketonuria by Neonatal Disease Screening Center of Northwest Women and Children Hospital from January to December 2012 were retrospectively analyzed.The patient were divided into breast feeding group(n=68)and formula milk feeding group(n=20)according to the feeding pattern,and the blood phenylalanine values and the condition of growth and development regulary were observed. Results The mean blood phenylalanine values of 60 patients ( 88.24%) in breast feeding groups were in normal range,however only 13 patients (65.00%)in formula milk feeding groups,the rate was statistically different(χ²=4.372,P＜0.05).The T value of height,weight and intelligence development of two groups were 0.17,2.42 and 2.7,there was no significantly difference(P＞0.05). Conclusion There are different effects between breast feeding group and formula milk feeding group in treatment of patients with phenylketonuria,the breast feeding may contribute to control the blood phenylalanine values of patients with phenylketonuria.   

Key words: phenylketonuria, breast feeding, dietary therapy, phenylalanine

摘要： 目的 研究母乳对苯丙酮尿症患儿血苯丙氨酸值的影响,了解母乳喂养对苯丙酮尿症患儿治疗的影响。方法 选取2012年1-12月经西北妇女儿童医院新生儿疾病筛查中心确诊为苯丙酮尿症的患儿为研究对象,除立即采用低苯丙氨酸饮食治疗外,根据是否用母乳喂养分为母乳喂养组(n=68)和配方奶喂养组(n=20),定期检测两组患儿血苯丙氨酸值及生长发育。结果 母乳喂养组68例患儿中60例(88.24%)患儿平均血Phe值在正常范围,人工喂养组患儿平均血phe值正常的有13例(65.00%),两组正常率比较差异有统计学意义(χ²=4.372,P＜0.05)。两组患儿6月龄时的身高、体重比较差异无统计学意义(t=0.17和2.42,P＞0.05)。两组患儿智力发育比较差异无统计学意义(t=2.70,P＞0.05)。结论 两种喂养方法对苯丙酮尿症患儿血苯丙氨酸的影响无明显差异,母乳喂养更利于苯丙酮尿症患儿血phe值的控制。

关键词: 苯丙酮尿症, 母乳喂养, 饮食治疗, 苯丙氨酸