停止低苯丙氨酸饮食对苯丙酮尿症患儿智力发育的影响。
Effect of stopping low-phenylalanine diet on intellectual progress of children with phenylketonuria.
作者信息
Smith I, Lobascher M E, Stevenson J E, Wolff O H, Schmidt H, Grubel-Kaiser S, Bickel H
出版信息
Br Med J. 1978 Sep 9;2(6139):723-6. doi: 10.1136/bmj.2.6139.723.
Forty-seven patients at the Hospital for Sick Children, London, who had phenylketonuria and were on a low-phenylalanine diet (21 early-treated--that is, treatment started before the age of 4 months--and 26 late-treated) were placed on a normal diet between the ages of 5 and 15 years. They showed significant falls in mean IQ of about six points after the diet was withdrawn. Twenty-two similar patients (five early-treated and 17 late-treated) at the Universitäts-Kinderklinik, Heidelberg, who were placed on a relaxed low-phenylalanine rather than a normal diet, showed smaller and non-significant falls in mean IQ. During the period of strict diet none of the patients in London or Heidelberg showed any consistent falls in IQ. These results suggest that complete withdrawal of the low-phenylalanine diet during childhood leads to a fall in intellectual progress in many patients.
伦敦大奥蒙德街儿童医院的47名苯丙酮尿症患儿接受低苯丙氨酸饮食治疗（21名早期接受治疗，即4个月前开始治疗；26名晚期接受治疗），在5至15岁期间改为正常饮食。停止饮食后，他们的平均智商显著下降了约6分。海德堡大学儿童医院的22名类似患儿（5名早期接受治疗，17名晚期接受治疗），接受的是宽松低苯丙氨酸饮食而非正常饮食，其平均智商下降幅度较小且无统计学意义。在严格饮食期间，伦敦和海德堡的患儿智商均未出现持续下降。这些结果表明，儿童期完全停止低苯丙氨酸饮食会导致许多患儿智力发育倒退。
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