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遗传性奥尔波特肾病患者同种异体肾移植后抗基底膜肾小球肾炎

[Anti-basal membrane glomerulonephritis after homologous kidney transplantation in hereditary Alport's nephropathy].

作者信息

Bach D, Peters A, Röwemeier H, Degenhardt S, Helmchen U, Grabensee B

机构信息

Abteilung für Nephrologie, Universität Düsseldorf.

出版信息

Dtsch Med Wochenschr. 1991 Nov 15;116(46):1752-6. doi: 10.1055/s-2008-1063814.

Abstract

A patient, born in 1968, was found at the age of 9 years to have Alport's syndrome. In his 20th year, when in terminal renal failure, his father's kidney was transplanted into him. 14 months later there was a sudden worsening of his renal function after a varicella infection; for the first time antibodies against the glomerular basal membrane (GBM) were detected. Despite an increase in prednisolone dosage and 6 plasmaphereses chronic haemodialysis again became necessary. Renal biopsy revealed necrotizing intra- and extracapillary glomerulonephritis. Simultaneously there was a raised anti-NC-1-antibody titre (1:80) in the serum. After removal of the transplant the titre fell to normal. The NC-1 antigen, a component of the GBM in healthy persons, may be absent in Alport's syndrome. An analysis of the few cases of anti-GBM glomerulonephritis in renal transplants of patients with Alport's syndrome may make it possible to recognize factors which precipitate or favour this form of glomerulonephritis.

摘要

一名患者出生于1968年,9岁时被诊断患有阿尔波特综合征。20岁时,他处于终末期肾衰竭,接受了父亲的肾脏移植。14个月后,在感染水痘后,他的肾功能突然恶化;首次检测到抗肾小球基底膜(GBM)抗体。尽管泼尼松龙剂量增加且进行了6次血浆置换,慢性血液透析再次成为必要。肾活检显示坏死性毛细血管内和毛细血管外肾小球肾炎。同时,血清中抗NC-1抗体滴度升高(1:80)。切除移植肾后,滴度降至正常。NC-1抗原是健康人GBM的一个组成部分,在阿尔波特综合征中可能缺失。对阿尔波特综合征患者肾移植中少数抗GBM肾小球肾炎病例的分析,可能有助于识别促使或有利于这种形式肾小球肾炎发生的因素。

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