Plaza J, Malasit P, Kerr D N
Postgrad Med J. 1977 Oct;53(624):627-30. doi: 10.1136/pgmj.53.624.627.
A patient with hereditary angio-oedema (HAO) developed mesangiocapillary glomerulonephritis (MCGN) under observation. HAO is characterized by an inherited defect of complement-deficiency of C1 esterase. MCGN is often associated with another complement abnormality which leads to depression of serum C3 and there is some evidence that the complement abnormality precedes the nephritis. The coincidence of these two rare diseases in the present patient, and in one previously described, suggests that other complement abnormalities may predispose to the development of MCGN.
一名遗传性血管性水肿(HAO)患者在观察期间出现了系膜毛细血管性肾小球肾炎(MCGN)。HAO的特征是C1酯酶补体缺乏的遗传性缺陷。MCGN常与另一种补体异常相关,这种异常会导致血清C3降低,并且有一些证据表明补体异常先于肾炎出现。这两种罕见疾病在该患者以及之前报道的一例患者中同时出现,提示其他补体异常可能易引发MCGN的发生。