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本病是遗传性疾病,由于C_1酯酶抑制物(C_1-esterase inhibitor,C_1-INH)缺乏或功能缺陷所致。其特征为反复发作的自发性皮下和粘膜下肿胀,可持续24～48小时。当水肿侵及胃肠道时,可发生恶心、呕吐、腹泻和腹痛;如果肿胀累及上呼吸道,患者可因喉水肿而窒息死亡。遗传性血管性水肿可用抗纤维蛋白溶剂或雄激素衍生物进行预防。作者等治疗120例的经验证明止血环酸(tranexamic acid)可使70%患者的发作减轻,即使长期应用也不产生严重的副作用。用17α-烷基化雄激素衍生物如danazol和羟甲雄烷吡唑(stanozolol)治疗的效果更好,它们可使血清C_1INH活性增高4～5倍,从而使临
The disease is a genetic disease, due to lack of C 1 -Esterase inhibitor (C 1-INH) or functional defects. It is characterized by recurrent spontaneous subcutaneous and submucosal swelling, sustainable 24 to 48 hours. Nausea, vomiting, diarrhea, and abdominal pain can occur when edema invades the gastrointestinal tract; if swelling involves the upper respiratory tract, the patient may succumb to suffocation of laryngeal edema. Hereditary angioedema can be prevented with antifibrinolytic or androgen derivatives. The authors’ experience of treating 120 patients demonstrated that tranexamic acid reduced seizures in 70% of patients and did not cause serious side effects even in long-term use. Treatment with 17α-alkylated androgen derivatives such as danazol and stanozolol performed better and they increased serum C 1 HH activity by 4 to 5 times,