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遗传性血管性水肿:儿童、育龄妇女及老年人的特殊考量

Hereditary angioedema: special consideration in children, women of childbearing age, and the elderly.

作者信息

Kuhlen James L, Banerji Aleena

机构信息

Division of Rheumatology, Allergy, and Immunology, Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts and Harvard Medical School, Boston, Massachusetts, USA.

出版信息

Allergy Asthma Proc. 2015 Nov-Dec;36(6):425-32. doi: 10.2500/aap.2015.36.3911.

Abstract

This review on hereditary angioedema (HAE) focused on special topics regarding HAE in children, women of childbearing age, and the elderly. HAE is a rare autosomal dominant bradykinin-mediated disorder characterized by recurrent attacks of subcutaneous or submucosal swelling that usually affects the face, upper airway, extremities, gastrointestinal tract, or genitalia. These recurrent attacks cause significant morbidity and can be life threatening, especially when the swelling affects the airway. Our objective was to summarize the published data available on the disease epidemiology, pathophysiology, clinical presentation, on demand and prophylactic therapy, and focus on management considerations for these special patient populations. Unique aspects of HAE in women with regard to contraception, hormone replacement therapy, pregnancy, lactation, and menopause were also reviewed.

摘要

本关于遗传性血管性水肿(HAE)的综述聚焦于儿童、育龄妇女和老年人HAE的特殊主题。HAE是一种罕见的常染色体显性缓激肽介导的疾病,其特征为皮下或黏膜下肿胀反复发作,通常累及面部、上呼吸道、四肢、胃肠道或生殖器。这些反复发作会导致严重发病,甚至可能危及生命,尤其是当肿胀影响气道时。我们的目的是总结已发表的关于该疾病流行病学、病理生理学、临床表现、按需治疗和预防性治疗的数据,并关注这些特殊患者群体的管理考量。还综述了HAE在女性中关于避孕、激素替代疗法、妊娠、哺乳和绝经的独特方面。

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