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与C1抑制剂缺乏相关的获得性头颈部血管性水肿：一例病例报告并文献复习

Acquired form of angioedema of the head and neck related to a deficiency in c1-inhibitor: a case report with a review of the literature.

作者信息

Hallak Bassel, Konu Propser, Lang Florian, Simon Christian, Monnier Philippe

机构信息

Department of Otorhinolaryngology, University Hospital CHUV, Rue du Bugnon 46, 1011 Lausanne, Switzerland.

出版信息

Case Rep Otolaryngol. 2012;2012:405824. doi: 10.1155/2012/405824. Epub 2012 Dec 10.

DOI:10.1155/2012/405824
PMID:23304594
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3523597/
Abstract

Angioedema related to a deficiency in the C1-inhibitor protein is characterized by its lack of response to therapies including antihistamine, steroids, and epinephrine. In the case of laryngeal edema, mortality rate is approximately 30 percent. The first case of the acquired form of angioedema related to a deficiency in C1-inhibitor was published in 1972. In our paper, we present a case of an acquired form of angioedema of the oropharyngeal region secondary to the simultaneous occurrence of two causative factors: neutralization of C1-inhibitor by an autoantibody and the use of an angiotensin convertin enzyme inhibitor.

摘要

与C1抑制蛋白缺乏相关的血管性水肿的特点是对包括抗组胺药、类固醇和肾上腺素在内的治疗无反应。对于喉水肿，死亡率约为30%。首例与C1抑制蛋白缺乏相关的获得性血管性水肿病例于1972年发表。在我们的论文中，我们报告了一例口咽区域获得性血管性水肿病例，其继发于两个致病因素同时出现：自身抗体对C1抑制蛋白的中和作用以及血管紧张素转换酶抑制剂的使用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d9f/3523597/0c2ad1105bfc/CRIM.OTOLARYNGOLOGY2012-405824.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d9f/3523597/7b45a923f185/CRIM.OTOLARYNGOLOGY2012-405824.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d9f/3523597/0c2ad1105bfc/CRIM.OTOLARYNGOLOGY2012-405824.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d9f/3523597/7b45a923f185/CRIM.OTOLARYNGOLOGY2012-405824.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d9f/3523597/0c2ad1105bfc/CRIM.OTOLARYNGOLOGY2012-405824.002.jpg

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2010 International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema.2010 遗传性血管性水肿诊断、治疗和管理国际共识算法。
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