Chng H H, Boey M L
Department of Medicine IV, Tan Tock Seng Hospital, Singapore.
Singapore Med J. 1990 Apr;31(2):177-9.
Hereditary angioedema is a rare autosomal dominant disorder due to the deficiency of functionally active C1-inhibitor. It is characterised by recurrent episodes of subcutaneous and mucosal edema. We report a case of hereditary angioedema presenting with the classic features of recurrent swelling of the extremities, abdominal pain and laryngeal edema. Serum complement C3 level was normal but C4 was low. She responded well to danazol and had no further attacks of angioedema.
遗传性血管性水肿是一种罕见的常染色体显性疾病，由功能活性C1抑制因子缺乏所致。其特征为皮下和黏膜水肿反复发作。我们报告一例遗传性血管性水肿病例，具有四肢反复肿胀、腹痛和喉水肿的典型特征。血清补体C3水平正常，但C4水平较低。她对达那唑反应良好，未再发生血管性水肿发作。
