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[遗传性血管性水肿的诊断与排除:实践中的标准化方法]

[Diagnostics and exclusion of hereditary angioedema : a standarized approach for the practice].

作者信息

Magerl M, Brasch J, Förster U, Hauswald B, Mohr E B, Prässler J, Treudler R, Vetter R, Wahn V, Zampeli V, Ziemer M, Maurer M

机构信息

Klinik für Dermatologie, Venerologie und Allergologie, Allergie-Centrum, Charité - Universitätsmedizin Berlin, Deutschland.

出版信息

Hautarzt. 2012 Jul;63(7):567-72. doi: 10.1007/s00105-012-2388-x.

Abstract

The differentiation between mast cell mediator-mediated and bradykinin-mediated forms of angioedema can be difficult. Bradykinin-mediated hereditary angioedema is a rare autosomal dominant hereditary disease which is characterized by recurrent edema attacks of varying magnitude. The edema occurs in the skin and mucous membranes and can be temporarily disfiguring, very painful and life-threatening by attacks in the laryngeal region. Because of the multitude of differential diagnoses, a final diagnosis is only achieved after an average duration of more than 10 years. The anamnestic and laboratory diagnostic algorithm presented here is designed to assist a simpler differentiation of the various forms of angioedema and to reach the correct diagnosis more quickly.

摘要

肥大细胞介质介导型血管性水肿和缓激肽介导型血管性水肿之间的鉴别可能存在困难。缓激肽介导的遗传性血管性水肿是一种罕见的常染色体显性遗传病,其特征为反复发作的程度各异的水肿发作。水肿出现在皮肤和黏膜,发作时可能会暂时毁容、疼痛剧烈,若发生在喉部区域甚至会危及生命。由于鉴别诊断众多,平均超过10年才能做出最终诊断。此处给出的既往史及实验室诊断算法旨在帮助更简便地区分各种类型的血管性水肿,并更快做出正确诊断。

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