【摘要】 目的对宁夏地区低苯丙氨酸饮食控制治疗的106例苯丙酮尿症患儿疗效进行评估,为苯丙酮尿症患儿临床治疗提供依据。方法选取坚持低苯丙氨酸饮食控制治疗的106例苯丙酮尿症患儿作为观察组,定期根据观察组患儿血苯丙氨酸浓度调整饮食,另外随机选取本院儿童保健中心常规体检的106例健康儿童作为对照组,定期对两组儿童生长发育和智力发育进行监测评估。结果观察组患儿治疗前、后血苯丙氨酸浓度大幅度下降,从(1222.6±130.5)μmol·L-1下降至(22.6±13.5)μmol·L-1(t=13.279,P=0.000);观察组与对照组儿童身高、体重及智力发育上比较差异均无统计学意义(P>0.05)。结论长期坚持低苯丙氨酸饮食控制治疗,适量补充微量元素,注意均衡膳食,可有效避免苯丙酮尿症患儿智力和生长发育出现异常。更多还原

【Abstract】 Objective To evaluate the therapeutic effect of low phenylalanine diet in 106 cases with phenylketonuria in Ningxia,and to provide evidence for clinical treatment of children with phenylketonuria.Methods 106 children with phenylketonuria were selected as low phenylalanine diet therapy group and their diet was adjusted regularly according to the blood phenylalanine concentration changes while 106 healthy children were selected as control group. Children’s growth development and intellectual development were determined and evaluated in two groups.Results 1)Blood pheconcentration in children in the treatment group was significantly decreased from before treat to after treat(t=13.279,P=0.000). 2)The physical development showed no significant difference between the two grou ps(P>0.05). 3)The intellectual development showed no significant difference between the two groups(P>0.05). Conclusion Low phenylalanine diet control,supplement of trace elements and balanced diet can improve the intellectual and physical development in children with phenylketonuria.更多还原