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1例抗肾小球基底膜肾炎(新月体性肾小球肾炎)合并膜性肾病

[A case of anti-GBM nephritis (crescentic glomerulonephritis) associated with membranous nephropathy].

作者信息

Hayano K, Miura H, Fukui H, Otsuka Y, Hattori S

机构信息

Department of Internal Medicine, Kumamoto Chuo Hospital.

出版信息

Nihon Jinzo Gakkai Shi. 1992 Jul;34(7):821-6.

PMID:1479722
Abstract

We report a case of endstage renal disease due to simultaneous occurrence of membranous nephropathy and crescentic glomerulonephritis associated with anti-GBM antibodies. The patient was a 60-year-old male and was hospitalized for prolonged anorexia and general malaise. On admission, his body temperature was 38.5 degrees C. Urinalysis revealed 3+ proteinuria and the sediment contained abundant erythrocytes. The urea nitrogen was 142.4 mg/dl, the creatinine 19.5 mg/dl, the potassium 6.47 mEq/dl and CRP 10.1 mg/dl. Anti-GBM antibodies were 1000EU/ml. Immediately after initiating hemodialysis, pulse steroid therapy, plasma exchange and continuous heparinization were performed. However, renal function had been impaired and maintenance hemodialysis was required. Histological examination of the renal specimen revealed marked epithelial crescent formation, whereas thickening of basement membrane and mesangial proliferation were not observed. By immunofluorescent staining, both bright linear and fine granular fixation of IgG and fine granular fixations of C3 along the glomerular capillary walls were observed. Electron microscopy showed subepithelial electron lucent deposits and thickening of the glomerular basement membrane, diagnostic of the advanced membranous nephropathy (stage IV).

摘要

我们报告一例终末期肾病,病因是膜性肾病与抗肾小球基底膜(GBM)抗体相关的新月体性肾小球肾炎同时发生。患者为60岁男性,因长期厌食和全身乏力入院。入院时,他的体温为38.5摄氏度。尿液分析显示蛋白尿3+,尿沉渣中有大量红细胞。尿素氮为142.4mg/dl,肌酐为19.5mg/dl,钾为6.47mEq/dl,CRP为10.1mg/dl。抗GBM抗体为1000EU/ml。开始血液透析后,立即进行了脉冲类固醇治疗、血浆置换和持续肝素化。然而,肾功能已经受损,需要维持性血液透析。肾脏标本的组织学检查显示有明显的上皮新月体形成,而未观察到基底膜增厚和系膜增生。通过免疫荧光染色,观察到沿肾小球毛细血管壁有IgG的明亮线性和细颗粒状沉积以及C3的细颗粒状沉积。电子显微镜显示上皮下电子透亮沉积物和肾小球基底膜增厚,诊断为晚期膜性肾病(IV期)。

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