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苯丙酮尿症导致智力发育迟缓发病率反弹的预测。

Projections of a rebound in frequency of mental retardation from phenylketonuria.

作者信息

Kirkman H N

出版信息

Appl Res Ment Retard. 1982;3(3):319-28. doi: 10.1016/0270-3092(82)90023-6.

Abstract

The present decade marks the end of a unique generation--when the public benefited from the prevention of mental retardation phenylketonuria (PKU) without having to deal fully with the problems which can be anticipated from the reproduction of successfully treated, phenylketonuric individuals. The dysgenic effect (increase in prevalence of the PKU gene) from this reproduction will have negligible influence on the frequency of mental retardation from PKU over the next few centuries. In contrast, a dysgenic effect from maternal PKU will cause a rebound in frequency of mental retardation within this decade. Failure of the PKU programs as a result of maternal PKU, could affect the public attitude toward programs for the study and prevention of mental retardation and genetic diseases. Minimizing and explaining the rebound in frequency of mental retardation will be a difficult but necessary task for workers in the field of mental retardation.

摘要

当前十年标志着独特一代人的终结——这一代人见证了公众受益于苯丙酮尿症(PKU)所致智力迟钝的预防,却无需完全应对那些成功治疗后的苯丙酮尿症患者生育可能引发的问题。这种生育带来的不良遗传效应(PKU基因患病率上升)在未来几个世纪对PKU所致智力迟钝的发生率影响可忽略不计。相比之下,母体苯丙酮尿症带来的不良遗传效应将在本十年内导致智力迟钝发生率反弹。由于母体苯丙酮尿症导致PKU项目失败,可能会影响公众对智力迟钝及遗传疾病研究与预防项目的态度。将智力迟钝发生率的反弹降至最低并作出解释,对于智力迟钝领域的工作者而言将是一项艰巨但必要的任务。

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