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[遗传性血管性水肿。临床特点、发病机制与治疗]

[Hereditary angioneurotic edema. Clinical aspects, pathogenesis and therapy].

作者信息

Schindera F, Rother U, Hufnagel C

出版信息

Helv Paediatr Acta. 1978 Aug;33(3):259-66.

PMID:711490
Abstract

A family with hereditary angioedema is presented. In 4 members of the family the diagnosis was confirmed by decreased C1 esterase inhibitor levels. Clinical course and pathogenesis are described briefly. New ways of treatment are discussed, especially injection of purified C1 esterase inhibitor and stimulation of C1 esterase inhibitor synthesis by oral therapy with attenuated androgens.

摘要

本文介绍了一个患有遗传性血管性水肿的家族。该家族中有4名成员经C1酯酶抑制剂水平降低确诊。简要描述了其临床病程和发病机制。讨论了新的治疗方法,尤其是注射纯化的C1酯酶抑制剂以及通过口服减毒雄激素刺激C1酯酶抑制剂的合成。

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