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伴有纤连蛋白沉积的肾小球病(GFND)的临床病理分析:一例散发性、老年起病的GFND伴IgA、C1q和纤维蛋白原共沉积。

Clinicopathological analysis of glomerulopathy with fibronectin deposits (GFND): a case of sporadic, elderly-onset GFND with codeposition of IgA, C1q, and fibrinogen.

作者信息

Yoshino Masabumi, Miura Naoto, Ohnishi Takahiro, Suzuki Keisuke, Kitagawa Wataru, Nishikawa Kazuhiro, Imai Hirokazu

机构信息

Division of Nephrology and Rheumatology, Department of Internal Medicine, Aichi Medical University School of Medicine, Japan.

出版信息

Intern Med. 2013;52(15):1715-20. doi: 10.2169/internalmedicine.52.0046. Epub 2012 Mar 1.

Abstract

A 67-year-old Japanese man with recurrent nephrotic syndrome and impaired kidney function had a sporadic, elderly-onset case of glomerulopathy with fibronectin deposits. The daily urinary protein, serum albumin, blood urea nitrogen, and creatinine levels were 3.6 g/day, 2.7 g/dL, 19.5 mg/dL, and 1.70 mg/dL, respectively. Kidney biopsy samples were evaluated using electron microscopy and demonstrated membranoproliferative glomerulonephritis-like lesions with massive subendothelial depositions and no fibrillary structures. Immunofluorescent studies showed 1+ staining for IgA, C1q, and fibrinogen. Anti-fibronectin immunostaining demonstrated that the subendothelial deposits were positive for fibronectin. The administration of prednisolone and mizoribine improved the nephrotic syndrome.

摘要

一名67岁的日本男性,患有复发性肾病综合征且肾功能受损,患有一例散发性、老年发病的伴有纤连蛋白沉积的肾小球病。每日尿蛋白、血清白蛋白、血尿素氮和肌酐水平分别为3.6g/天、2.7g/dL、19.5mg/dL和1.70mg/dL。使用电子显微镜对肾活检样本进行评估,结果显示为膜增生性肾小球肾炎样病变,伴有大量内皮下沉积且无纤维状结构。免疫荧光研究显示IgA、C1q和纤维蛋白原呈1+染色。抗纤连蛋白免疫染色显示内皮下沉积物纤连蛋白呈阳性。泼尼松龙和咪唑立宾的治疗改善了肾病综合征。

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