Aabom Anne Louise Nørmølle, Palarasah Yaseelan, Bygum Anette
Hudafdeling I, Odense Universitetshospital, Sdr. Boulevard 29, 5000 Odense C, Denmark.
Ugeskr Laeger. 2012 Aug 20;174(34):1894-8.
Hereditary angio-oedema (HAE) is a rare genetic disease caused by deficiency of complement C1 inhibitor. It is characterised by recurrent episodes of subcutaneous or submucosal oedema typically involving the extremities, bowel, face or larynx. Within the latest years it has become evident that the active mediator of HAE attacks is an increased level of bradykinin and various new treatment modalities have been developed. The aim of this paper is to give an update from the Danish HAE Comprehensive Care Centre on current treatment possibilities and address some of the challenges when diagnosing HAE.
遗传性血管性水肿(HAE)是一种由补体C1抑制剂缺乏引起的罕见遗传病。其特征是皮下或粘膜下水肿反复发作,通常累及四肢、肠道、面部或喉部。近年来,很明显HAE发作的活性介质是缓激肽水平升高,并且已经开发出各种新的治疗方法。本文的目的是提供丹麦HAE综合护理中心关于当前治疗可能性的最新情况,并探讨诊断HAE时的一些挑战。