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遗传性血管性水肿(HAE)与克罗恩病的巧合。

Coincidence of hereditary angioedema (HAE) with Crohn's disease.

作者信息

Farkas H, Gyeney L, Nemesánszky E, Káldi G, Kukán F, Masszi I, Soós J, Bély M, Farkas E, Füst G, Varga L

机构信息

Department of Oto-Rhino-Laryngology, Semmelweis Medical University, Budapest, Hungary.

出版信息

Immunol Invest. 1999 Jan;28(1):43-53. doi: 10.3109/08820139909022722.

Abstract

A patient with two diseases, based presumably on different immunopathological mechanisms, hereditary angioedema (HAE) and Crohn's disease, was followed for 8 years. For more than three years of this observation period, detailed laboratory data were also available and could be analyzed. Both diseases had severe courses requiring chronic treatment with danazol and sulfasalazine, respectively. During exacerbation of Crohn's disease, the levels of C4 was found to be significantly lower than during the periods free of symptoms of both diseases. This drop was probably due to an impaired C1-inhibitor activity. HAE attacks and acute exacerbation of Crohn's disease never occurred simultaneously. This finding may be a mere chance but may also indicate that the different immunopathological processes underlying HAE and Crohn's disease influence each other.

摘要

一名患有两种可能基于不同免疫病理机制的疾病——遗传性血管性水肿(HAE)和克罗恩病的患者,被随访了8年。在这一观察期的三年多时间里,还获得了详细的实验室数据并进行了分析。两种疾病都病程严重,分别需要用达那唑和柳氮磺胺吡啶进行长期治疗。在克罗恩病发作期间,发现C4水平明显低于两种疾病无症状期间。这种下降可能是由于C1抑制物活性受损所致。HAE发作和克罗恩病急性发作从未同时发生。这一发现可能纯属偶然,但也可能表明HAE和克罗恩病潜在的不同免疫病理过程相互影响。

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