Estrogen-dependent inherited angioedema
Estrogen-dependent inherited angioedema
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DOI：
10.1016/j.transci.2003.08.002
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发表时间：
2003-12-01
影响因子：
1.9
通讯作者：
Davis, AE
中科院分区：
文献类型：
--
作者：
Binkley, KE;Davis, AE
遗传性血管性水肿的典型形式在临床上表现为反复发作的血管性水肿，生物化学上表现为C1抑制剂水平和/或功能降低，遗传学上表现为C1抑制剂基因的异质组突变，具有常染色体显性传播模式。雄激素和雌激素在遗传性血管性水肿患者中具有显著的临床作用，且往往对Cl抑制剂蛋白水平具有拮抗作用。雄激素可提高C1抑制蛋白水平，减少血管性水肿的发作，是治疗患者的重要药物。性类固醇激素达到这些效果的机制尚不清楚。最近对一种新的雌激素依赖性血管性水肿的认识可能为性激素发挥作用的机制以及雌激素依赖性和经典遗传性血管性水肿的发病机制和治疗提供重要的见解。(C) 2003 Elsevier Ltd.版权所有。
Classic forms of hereditary angioedema are characterized clinically by recurrent episodes of angioedema, biochemically by reduced C1 inhibitor level and/or function, and genetically by a heterogeneous group of mutations in the C1 inhibitor gene that have an autosomal dominant mode of transmission. Androgens and estrogens have significant clinical effects in patients with hereditary angioedema, and tend to have antagonist effects of the levels of Cl inhibitor protein. Androgens increase the levels of C1 inhibitor protein, reduce attacks of angioedema, and thus are an important therapy for patients. The mechanisms by which the sex steroid hormones achieve these effects are not understood. The recent recognition of a novel estrogen-dependent form of angioedema may offer important insights into the mechanisms by which the sex hormones exert their effects, and the pathogenesis and treatment of both estrogen-dependent and classic forms of hereditary angioedema. (C) 2003 Elsevier Ltd. All rights reserved.