苯丙酮尿症患儿居家饮食治疗护理现状调查

Investigation and status of dietary therapy and nursing at home of phenylketonurics children

目的调查苯丙酮尿症患儿居家饮食治疗护理的情况及所面临的问题。方法采用自设问卷,对确诊为经典型苯丙酮尿症的47例患儿进行调查。结果不足2岁的患儿与2岁以上的患儿比较,在新生儿筛查确诊、开始治疗时间<3个月、复查血苯丙氨酸浓度时间<1个月、喂养困难、智力情况正常方面差异具有统计学意义(P﹤0.01或P﹤0.05);经济负担重是饮食治疗中最大的困难。结论苯丙酮尿症患儿居家饮食治疗面临着较多问题,护理人员应参与到家庭饮食治疗的干预和指导工作中。

Objective To investigate the status and problems of dietary therapy and nursing at home of phenylketonurics （PKU） children. Method Forty - seven children with phenylketonuria were investigated by a self - designed questionnaire. Results Comparisons of neonatal screening diagnose, starting time of dietary therapy 〈 3 months, feeding difficulties, review blood phenylalanine eoncenration time 〈 1 month, normal intelligence between children less than 2 years old and over 2 years old were found to have statistical differences （ P 〈 0.01 or P 〈 0.05 ）. The biggest difficulty found in dietary therapy was the heary economic burden. Conclusion Many problems exist in dietary therapy for children with phenyketonuria. It is suggested that nursing staffs should participate in the job of interfering and guiding of dietary therapy at home.