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[波兰综合征]

[Poland's syndrome].

作者信息

Slezak R, Sasiadek M

机构信息

Zakładu Genetyki AM we Wrocławiu.

出版信息

Pol Merkur Lekarski. 2000 Aug;9(50):568-71.

Abstract

Poland's syndrome consists of the variable clinical features, but always includes unilateral aplasia of the chest wall muscles and ipsilateral anomalies of upper extremity. The incidence of Poland's syndrome, reported by different authors ranges from 1:10,000 to 1:100,000 and is observed more frequently in males than in females with the right side of the body affected more often than the left. The etiology of this syndrome is still discussed. However most of described cases were sporadic, rare familial incidence of Poland's syndrome were also presented. Therefore different etiologic factors of the Poland's syndrome are taken into account: genetic, vascular compromise during early stages of embriogenesis but also teratogenic effect of environmental xenobiotics (e.g. cigarette smoking by pregnant women). The authors present also the case of 20-years old man with inherited bilateral syndactyly with the right side aplasia of major pectoralis muscle and face asymmetry. The familial history was negative in respect to the features, associated with Poland's syndrome.

摘要

波兰综合征具有多种临床特征,但始终包括胸壁肌肉的单侧发育不全和同侧上肢异常。不同作者报道的波兰综合征发病率在1:10000至1:100000之间,男性比女性更常观察到,身体右侧比左侧更常受累。该综合征的病因仍在讨论中。然而,大多数描述的病例是散发性的,也有波兰综合征罕见的家族发病率报道。因此,考虑到波兰综合征的不同病因因素:遗传因素、胚胎发育早期的血管受损以及环境异生物(如孕妇吸烟)的致畸作用。作者还介绍了一名20岁男性的病例,该患者患有遗传性双侧并指畸形,右侧胸大肌发育不全和面部不对称。家族史中没有与波兰综合征相关的特征。

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