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[EEC综合征。病例报告及其发病机制的一些建议(作者译)]

[The EEC-syndrome. Case report and some suggestions about its pathogenesis (author's transl)].

作者信息

Aldenhoff P, von Mühlendahl K E, Waldenmaier C

出版信息

Monatsschr Kinderheilkd (1902). 1978 Sep;126(9):575-8.

PMID:692527
Abstract

The clinical data of a boy with ectrodactyly, ectodermal dysplasia, and cleft lip and palate (EEC-syndrome) are presented. Until now about 80 case histories with this syndrome have been published, 30 of them having all 3 symptoms. The combination of defects in rather different organs of ectodermal as well as mesodermal origin is difficult to understand. Changes in ectodermal tissues may well be the key factor in explaining the pathogenesis of this syndrome. This is supported by evidence gained by ontogenetical research with animals.

摘要

本文介绍了一名患有缺指(趾)畸形、外胚层发育不良和唇腭裂(EEC综合征)男孩的临床资料。到目前为止,已经发表了约80例该综合征的病例史,其中30例具有全部三种症状。外胚层和中胚层来源的相当不同器官中的缺陷组合难以理解。外胚层组织的变化很可能是解释该综合征发病机制的关键因素。动物个体发育研究获得的证据支持了这一点。

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