Davis P J, Davis F B, Charache P
Birth Defects Orig Artic Ser. 1976;12(6):283-7.
HAE is an autosomal dominant trait of decreased levels or function of circulating and tissue C'1 esterase inhibitor. The clinical illness is characterized by disabling episodes of peripheral, oropharyngeal and gut-wall edema. Long-term fluoxymesterone treatment of 5 affected males (393 patient-months) and oxymetholone treatment of 6 affected females (204 patient-months) has significantly decreased the frequency of attacks of edema without substantive side effects.
遗传性血管性水肿是一种常染色体显性性状,表现为循环和组织中C1酯酶抑制剂水平降低或功能异常。临床症状的特征是外周、口咽和肠壁水肿反复发作,导致功能障碍。对5名受影响男性进行长期氟甲睾酮治疗(393个患者月),对6名受影响女性进行羟甲烯龙治疗(204个患者月),显著降低了水肿发作频率,且无明显副作用。