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苯丙酮尿症患儿最佳产科及产后条件的降低及其对治疗效果的影响

[Reduced optimal obstetric and postnatal conditions in children with phenylketonuria and their effect on the success of treatment].

作者信息

Wässer S, Theile H, Scheer A, Graustein I

出版信息

Padiatr Padol. 1986;21(4):351-6.

PMID:3562045
Abstract

We found a total of 433 reduced obstetric an postnatal conditions in 115 newborns with pku with a range of 0 (n = 4) to -11 (n = 1) per child. The 50th percentile of reduced optimal conditions was at -3, the 90th at -6. Early treated patients with excellent or good dietary control have significantly reduced developmental quotients (DQ) at the age of 3 years when the optimal conditions were reduced above the 90th percentile. Reduced optimal obstetric and postnatal conditions could be found in about 25 per cent of the patients with excellent or good dietary control and a DQ below 100, and in about 50 per cent with a DQ below 85. The effect of treatment is better in patients with excellent and good dietary control and a high value of reduced optimal conditions compared with patients with poor dietary control and an only low value of reduced optimal condition. The quality of dietary control therefore is the most important point to reach an optimal effect in early treated patients.

摘要

我们在115例苯丙酮尿症新生儿中总共发现了433种产科及产后状况不佳的情况,每个孩子的状况不佳范围为0(n = 4)至-11(n = 1)。最佳状况不佳的第50百分位数为-3,第90百分位数为-6。当最佳状况不佳超过第90百分位数时,早期接受治疗且饮食控制良好或优秀的患者在3岁时发育商(DQ)显著降低。在饮食控制良好或优秀且DQ低于100的患者中,约25%可发现产科及产后最佳状况不佳;在DQ低于85的患者中,这一比例约为50%。与饮食控制不佳且最佳状况不佳程度仅为低水平的患者相比,饮食控制良好或优秀且最佳状况不佳程度高的患者治疗效果更好。因此,对于早期接受治疗的患者而言,饮食控制质量是达到最佳治疗效果的最重要因素。

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