遗传性血管性水肿是一种常染色体显性遗传病,基本缺陷是患者血清中缺乏活化C_1和激肽酶(kallikreir)的抑制剂——神经氨糖蛋白(neuroamino glycoprotein),即C_1酯酶抑制剂,或是由于其无活性。本病急性发作时常引起喉头水肿、窒息而死亡,死亡率可达30％以上。本病治疗困难,用抗组织胺类药物和皮质类固醇激素等治疗常无效。今将近十几年来的一些治疗进展简要综述如下。 Hereditary angioedema is an autosomal dominant genetic disease, the basic flaw is the lack of patients with serum activated c_1 and kallikreir inhibitor (neuroamino glycoprotein), that C_l esterase inhibition Agent, or because of its inactivity. The disease often causes acute laryngeal edema, suffocation and death, the mortality rate of up to 30%. The disease is difficult to treat, with antihistamines and corticosteroids and other treatment often ineffective. A brief review of some of the advances in the past decade or so has been given below.