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首页> 外文期刊>Clinical kidney journal. >Fibronectin glomerulopathy
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Fibronectin glomerulopathy

机译：纤连蛋白肾小球病

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Fibronectin glomerulopathy occurs between the second and fifth decades of life in most patients, and it is known to be slowly progressive with mild proteinuria leading to kidney failure. The case of a 78-year-old woman with a rapid course of nephrotic syndrome due to fibronectin glomerulopathy is reported. She had proteinuria that rapidly increased to 6.8 g/day in a month and microscopic haematuria. A renal biopsy specimen showed lobular glomerulopathy and membranoproliferative glomerulonephritis-like lesions on light microscopy. There was scanty staining for immunoglobulins and complement. Electron microscopy revealed granular deposits with fibril formation. Immunohistochemistry of the fibronectin showed intense staining in the mesangium and peripheral loop. Therefore, this case was diagnosed as fibronectin glomerulopathy. The kidney function was rapidly decreasing, necessitating haemodialysis 2 months after renal biopsy. It is important to consider fibronectin glomerulopathy in the differential diagnosis of nephrotic syndrome in older people.
机译：纤连蛋白肾小球病发生在大多数患者的生命的第二个到第五个十年之间，并且已知它是缓慢进展的，伴有轻度蛋白尿，导致肾衰竭。据报道，一名78岁的妇女由于纤连蛋白肾小球病而患有肾病综合征的快速病程。她的蛋白尿在一个月内迅速增加至6.8 g /天，并伴有镜下血尿。肾活检标本在光学显微镜下显示小叶性肾小球病和膜增生性肾小球肾炎样病变。免疫球蛋白和补体染色很少。电镜观察发现有原纤维形成的颗粒状沉积物。纤连蛋白的免疫组织化学显示在系膜和周围环中强烈染色。因此，该病例被诊断为纤连蛋白肾小球病。肾脏功能迅速下降，需要在肾脏活检后2个月进行血液透析。在老年人肾病综合征的鉴别诊断中，考虑纤维连接蛋白肾小球病很重要。

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