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肺出血肾炎综合征——病例报告

Goodpasture's syndrome--a case report.

作者信息

Yang C W, Huang C C, Hsueh S

机构信息

Department of Internal Medicine, Chang Gung Memorial Hospital, Taipei, Taiwan, R.O.C.

出版信息

Changgeng Yi Xue Za Zhi. 1990 Jun 20;13(2):143-51.

PMID:2224607
Abstract

Goodpasture's syndrome is a disease characterized by diffuse pulmonary hemorrhage and glomerulonephritis with circulating anti-glomerular basement membrane antibody (anti-GBM antibody). Although it is reported in the western world, it is rarely diagnosed in Taiwan. We report a 26-year-old male admitted due to pulmonary hemorrhage and rapid progressive glomerulonephritis. Renal biopsy showed diffuse glomerular proliferation and 90% crescent formation, and immunofluorescence stain showed diffuse linear staining of IgG, C3, IgA, IgM along glomerular basement membranes and tubular basement membranes. He was treated with immunosuppressive agents. Pulmonary hemorrhage improved rapidly, but his renal function was severely impaired and he remained dialysis dependent.

摘要

肺出血肾炎综合征是一种以弥漫性肺出血、肾小球肾炎伴循环抗肾小球基底膜抗体(抗GBM抗体)为特征的疾病。尽管在西方世界有相关报道,但在台湾地区很少被诊断出来。我们报告了一名26岁男性,因肺出血和快速进展性肾小球肾炎入院。肾活检显示弥漫性肾小球增生和90%的新月体形成,免疫荧光染色显示IgG、C3、IgA、IgM沿肾小球基底膜和肾小管基底膜呈弥漫线性染色。他接受了免疫抑制剂治疗。肺出血迅速改善,但他的肾功能严重受损,仍依赖透析。

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