Aygören-Pürsün Emel, Bork Konrad
Angioödem-Ambulanz und Comprehensive Care Center für hereditäres Angioödem, Klinik für Kinder- und Jugendmedizin, Universitätsklinikum Frankfurt, Goethe-Universität, Theodor-Stern-Kai 7, 60590, Frankfurt am Main, Deutschland.
Hautklinik, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland.
Internist (Berl). 2019 Sep;60(9):987-995. doi: 10.1007/s00108-019-0644-1.
Hereditary angioedema (HAE) encompasses a heterogeneous group of diseases with similar phenotypes but different underlying genotypes. Specific clinical signs may point to HAE as opposed to histaminergic angioedema: the typical prolonged development of angioedema over time, positive family history, a lack of response to antihistamines and steroids and response to bradykinin antagonists are typical signs of HAE. The different types of HAE may be associated with a severe clinical course. They are life-long conditions and are still potentially life-threatening. The quality of life of patients with HAE may be considerably impaired. Management plans should be individualized, which is facilitated by the variety of specific medicastions available.
遗传性血管性水肿（HAE）包括一组具有相似表型但潜在基因型不同的异质性疾病。与组胺能性血管性水肿不同，特定的临床体征可能提示HAE：血管性水肿随时间典型的长期发展、阳性家族史、对抗组胺药和类固醇无反应以及对缓激肽拮抗剂有反应是HAE的典型体征。不同类型的HAE可能与严重的临床病程相关。它们是终身疾病，仍然有潜在的生命威胁。HAE患者的生活质量可能会受到相当大的损害。管理计划应个体化，现有多种特定药物有助于实现这一点。
