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埃勒斯-当洛综合征中的大量自发性膈肌破裂

Massive spontaneous diaphragmatic rupture in Ehlers-Danlos syndrome.

作者信息

Hamaoui K, Riaz A, Hay A, Botha A

机构信息

Department of Renal and Transplant Medicine, Hammersmith Campus, Imperial College London, London, UK.

出版信息

Ann R Coll Surg Engl. 2012 Jan;94(1):e5-7. doi: 10.1308/003588412X13171221499306.

DOI:10.1308/003588412X13171221499306
PMID:22524904
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3954211/
Abstract

Ehlers-Danlos syndrome (EDS) is a rare disorder caused by abnormalities in the synthesis and structure of collagen. The resultant tissue fragility and weakness can lead to multiple surgical conditions. In this report we present the very rare and life threatening case of a massive spontaneous diaphragmatic rupture in a 35-year-old man with EDS and reflect on the literature, highlighting points to consider when managing such complex patients.

摘要

埃勒斯-当洛综合征(EDS)是一种由胶原蛋白合成和结构异常引起的罕见疾病。由此导致的组织脆弱和虚弱会引发多种外科疾病。在本报告中,我们介绍了一名35岁患有EDS的男性发生巨大自发性膈肌破裂这一极其罕见且危及生命的病例,并对相关文献进行思考,强调在处理此类复杂患者时需要考虑的要点。

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Massive spontaneous diaphragmatic rupture in Ehlers-Danlos syndrome.埃勒斯-当洛综合征中的大量自发性膈肌破裂
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本文引用的文献

1
Acute diaphragmatic rupture in a patient with Ehlers-Danlos syndrome.一名患有埃勒斯-丹洛斯综合征的患者发生急性膈肌破裂。
J Emerg Med. 2011 Oct;41(4):366-8. doi: 10.1016/j.jemermed.2008.03.004. Epub 2008 Apr 24.
2
Recurrent congenital diaphragmatic hernia in Ehlers-Danlos syndrome.埃勒斯-当洛综合征中的复发性先天性膈疝
Cardiovasc Intervent Radiol. 2006 Sep-Oct;29(5):920-3. doi: 10.1007/s00270-005-0154-5.
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Spontaneous rupture of the diaphragm.膈肌自发性破裂
Arch Dis Child. 1999 Oct;81(4):341-2. doi: 10.1136/adc.81.4.341.
4
Diaphragmatic hernia in an 8-year-old with Ehlers-Danlos syndrome.一名患有埃勒斯-当洛综合征的8岁儿童的膈疝。
Pediatr Surg Int. 1998 Oct;13(8):553-5. doi: 10.1007/s003830050401.
5
Multiple surgical problems in two patients with Ehlers-Danlos syndrome.
Surgery. 1980 Mar;87(3):319-24.