遗传性血管神经性水肿是由于补体C_1的灭活因子(inactivator简称C_1INA)先天缺乏所引起的常染色体显性遗传病。症状为急性反复发作的限局性一过性血管神经性水肿。主要侵犯四肢和颜面等皮肤及消化道和吸呼道粘膜。水肿的病理改变为皮下组织和粘膜下组织的水肿。发病机制还不完全清楚。大致如下:由于抗原抗体反应或外伤使Hageman因子激活和通过凝固系统、激活系统和纤溶系统等使C_1激活。激活的C_1和显 Hereditary angioedema is due to autosomal dominant genetic disease caused by the congenital absence of complement C_1 inactivator (C_1INA). Symptoms of acute recurrent seizure of transient neurovascular edema. Mainly affects the limbs and face and other skin and gastrointestinal and respiratory tract mucosa. Pathological changes of edema subcutaneous tissue and submucosal tissue edema. The pathogenesis is not fully understood. Roughly as follows: activation of Hageman factor by antigen-antibody reaction or trauma and activation of C-1 through the coagulation system, activation system and fibrinolytic system. Activation of C_1 and significant