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[乙状结肠穿孔所致埃勒斯-当洛综合征]

[Ehlers-Danlos syndrome revealed by sigmoid perforation].

作者信息

Bedda S, Radovanovic A, Fajardy A, Bataille N, Montariol T

机构信息

Service de chirurgie digestive, CHI Poissy, 20, rue Armagis, 78100 Saint-Germain-en-Laye, France.

出版信息

Ann Chir. 2005 Jan;130(1):44-6. doi: 10.1016/j.anchir.2004.09.011.

Abstract

We report a case of colonic perforation revealing Ehlers -Danlos syndrome type IV in a male adult. This syndrome is a heritable disorder of collagen synthesis. Its prognosis is severe resulting in vascular rupture or bowel perforation. In his surgical strategy, the surgeon has to cope with the recurrent feature of the colonic perforation. In order to prevent other perforations, a therapeutic scheme has been set up according to literature.

摘要

我们报告一例成年男性因结肠穿孔而被诊断为IV型埃勒斯-当洛综合征的病例。该综合征是一种遗传性胶原合成障碍疾病。其预后严重,可导致血管破裂或肠穿孔。在手术策略上,外科医生必须应对结肠穿孔的复发性。为预防其他穿孔,已根据文献制定了一种治疗方案。

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