Löhle E, Mann W
HNO. 1980;28(9):305-7.
Hereditary angioneurotic edema is a rare autosomally-inherited disease, which is caused by a deficiency or functional inactivation of C-1 inactivator in the complement system. The poor prognosis of the disease mandates that an early diagnosis be made and drug therapy begun to prevent a lethal outcome. The pathogenesis, clinical course and therapies available are detailed.
遗传性血管性水肿是一种罕见的常染色体显性遗传病，由补体系统中C1酯酶抑制物缺乏或功能失活引起。该疾病预后较差，因此必须尽早诊断并开始药物治疗以防止致命后果。本文详细阐述了其发病机制、临床病程及现有治疗方法。
