遗传性血管性水肿是C_1抑制物的遗传缺陷,是一种常染色体的显性遗传病,发病率约为1/50万。约有30％发生喉水肿窒息而死亡。女患,59岁,医师。曾于20a(年)前因蚊虫叮咬面部后,引起前额部、面部水肿,数日后自行消退。20多年来,经常在四肢,尤其在头面部及前臂、手背等裸露部位发生水肿,每次发作均无一定规律,并能自行消退。于1988—04—02水肿再发,开始在上肢、手部、颜面,发展迅速,自感病情危笃,于午后3时求医。体检:患者颜面、颈部及全身均见严重水肿,头部 Hereditary angioedema is a genetic defect in the C_1 inhibitor and is an autosomal dominant genetic disease with a prevalence of about 1 in 500,000. About 30% of laryngeal edema asphyxiation and death. Female, 59 years old, physician. Had 20a (years ago) due to mosquito bites the face, causing forehead, facial edema, a few days later subsided. More than 20 years, often in the limbs, especially in the head and face and forearm, back of the hand and other parts of the edema, each attack is not a certain law, and to subside on their own. In 1988-04-02 edema recurrence, beginning in the upper extremities, hands, face, rapid development, self-esteem dangerous, seeking medical treatment at 3:00 pm. Physical examination: patients face, neck and body were severe edema, head