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遗传性血管神经性水肿(hereditary angioneuro-tic edema,HAE)是补体第1成份抑制因素(C_1-INH)缺陷,属常染色体显性遗传病。本文研究31个家系,患病个体150人,家族成员发病率56％,男女之比0.85。150例中,经血清 C_1抗原定量和功能测定而确诊者104例。初次发病的年龄 <12岁者占56％,13～20岁25％,21～30岁14％,30岁后发病者仅占5％。水肿见于皮下组织者86％、肠粘膜75％,上呼吸道78％。
Hereditary angioneurotic edema (HAE) is a deficiency of the first component of complement (C 1 -INH) and is an autosomal dominant disease. In this study, 31 pedigrees, 150 sick individuals, 56% of family members, and a ratio of male to female of 0.85.150, 104 cases were diagnosed by quantitative and functional measurement of serum C_1 antigen. The initial onset of age <12 years of age accounted for 56%, 13 ~ 20 years old 25%, 21 ~ 30 years old 14%, 30 years after onset, only 5%. Edema found in subcutaneous tissue of 86%, 75% of intestinal mucosa, upper respiratory tract 78%.