Section of Clinical Immunology and Allergy, Department of Medicine, University of California, Los Angeles-David Geffen School of Medicine, Los Angeles, CA 90095, USA.
Allergy Asthma Proc. 2013 Jan-Feb;34(1):13-8. doi: 10.2500/aap.2013.34.3635.
This review on hereditary angioedema (HAE) focuses on special topics regarding HAE in female patients. HAE is a bradykinin-mediated disorder, and the role of hormonal regulation of disease expression will be discussed focusing on the effect of estrogen on disease mechanism. The impact of exogenous estrogen on symptom exacerbation leads to special consideration regarding choice of contraceptives and safety of hormone replacement therapy. The effects of pregnancy and childbirth will be examined on the course of disease control. Unique considerations regarding therapeutic management for female HAE patients will be addressed, including the role of C1 inhibitor (C1-INH), ecallantide, and icatibant. Finally, this review will provide an overview of the more recently characterized HAE with normal C1-INH (HAE type III) that predominantly affects women and is in some cases associated with factor XII gene mutations.
本文主要探讨女性遗传性血管性水肿（HAE）的特殊问题。HAE 是一种缓激肽介导的疾病，本文将重点讨论雌激素对疾病机制的调控作用。由于外源性雌激素会加重症状，因此在选择避孕药具和激素替代疗法的安全性方面需要特别考虑。妊娠和分娩对疾病控制过程的影响也将进行研究。本文还将探讨女性 HAE 患者治疗管理方面的特殊考虑因素，包括 C1 抑制剂（C1-INH）、艾卡替班和依替巴肽的作用。最后，本文将概述最近发现的 C1-INH 正常的 HAE （HAE 型 III），这种疾病主要影响女性，在某些情况下与 XII 因子基因突变有关。
