遗传性血管性水肿的治疗  

Treatment for Hereditary Angioedema

作　　者：汤蕊[1] 张宏誉[1] 

机构地区：[1]中国医学科学院 北京协和医学院 北京协和医院变态反应科,北京100730

出　　处：《中华临床免疫和变态反应杂志》2012年第4期313-317,共5页Chinese Journal of Allergy & Clinical Immunology

摘　　要：遗传性血管性水肿(HAE)是一种罕见的常染色体显性遗传病,主要表现为皮肤黏膜水肿,严重者可出现喉水肿,危及生命。HAE的治疗可分为发作期急诊治疗和缓解期的短期及长期预防性治疗。除传统的弱雄性激素、抗纤溶制剂及补体第一成分抑制物(C1INH)浓缩剂外,近年来一些新药的研发,如重组C1INH、激肽释放酶抑制剂和缓激肽受体拮抗剂为HAE患者带来更多选择和希望。了解HAE的治疗方法对避免患者因延误治疗而造成的伤害、挽救患者生命有重要意义。Hereditary angioedema is a rare dominant autosomal inherited disease, characterized by episodic subcutaneous and mucous edema. It could be life-threatening when the edema occurred in larynxes. The aim of management of HAE is targeted to either the treatment of acute attacks or short- and long-term prophylaxis. Besides the current treatments such as androgens, anti-fibrinolytics and CIINH concentrate, the recent development of some new therapeutic methods such as recombinant CIINH, kallikrein inhibitor and bradykinin [32 receptor antagonist provide further options for HAE patients. It is very important to grasp the managements of HAE to avoid the damage to the HAE patients due to delayed treatment, and to save their lives.

关 键 词：遗传性血管性水肿 治疗 

分 类 号：R596[医药卫生—内科学]