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短肢畸形

Phocomelia

作者信息

Davis Donald D., Kane Steven M.

机构信息

Wellstar Atlanta Medical Center

Abstract

Phocomelia is a rare congenital defect defined by the absence of intermediate segments of the extremity. Children with phocomelia present with their hands or feet directly attached to the trunk. Phocomelia is famously a teratogenic side effect of the drug "thalidomide," a drug first marketed to treat anxiety and morning sickness. Despite claims that the drug was safe during pregnancy, it was removed from the market in the 1960s when clinicians noted an association with phocomelia. Forty percent of patients affected by the teratogenic effects of thalidomide died near the time of birth. These congenital disabilities had a profound social and political impact on drug regulation and proof of safety.

摘要

海豹肢症是一种罕见的先天性缺陷,其特征是四肢中间段缺失。患有海豹肢症的儿童的手或脚直接与躯干相连。海豹肢症是药物“沙利度胺”著名的致畸副作用,该药物最初上市用于治疗焦虑和孕吐。尽管声称该药物在孕期是安全的,但在20世纪60年代临床医生注意到其与海豹肢症有关联后,它就被撤市了。受沙利度胺致畸作用影响的患者中有40%在出生时或出生前后死亡。这些先天性残疾对药物监管和安全性证明产生了深远的社会和政治影响。

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