Qian X
ENT Institute, Shanghai Medical University.
Zhonghua Er Bi Yan Hou Ke Za Zhi. 1990 Jun;25(3):176-8, 191.
Hereditary angioedema is a rare familial disease caused by the defect of complement C1esterase inhibitor (C1-INH). It is characterized by recurrent acute edema of the extremities, the face, the respiratory tract and the gastrointestinal tract. Acute laryngeal edema usually produces laryngeal obstruction. Two cases have been treated since 1986, one of them had been admitted for forty-five times because of recurrent acute laryngeal edema. Investigations showed two families with a high incidence of this disease. Laboratory examination showed a remarkable decrease of C1-INH and C4. Tracheotomy is indicated in patients with laryngeal edema. Great success was achieved in two patients treated with danazol.
遗传性血管性水肿是一种由补体C1酯酶抑制剂（C1-INH）缺陷引起的罕见家族性疾病。其特征为四肢、面部、呼吸道和胃肠道反复出现急性水肿。急性喉水肿通常会导致喉梗阻。自1986年以来共治疗了2例患者，其中1例因反复急性喉水肿入院45次。调查显示有两个家族该病发病率很高。实验室检查显示C1-INH和C4显著降低。喉水肿患者需行气管切开术。用达那唑治疗的2例患者取得了巨大成功。
