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一名肾病综合征患者出现结节性系膜病变、显著的系膜溶解以及来源不明的指纹状沉积物:肾小球病变的独特组合。

Nodular mesangial lesions, marked mesangiolysis, and fingerprint deposits of unknown origin in a patient with nephrotic syndrome: a unique combination of glomerular lesions.

作者信息

Ohtani Hiroshi, Wakui Hideki, Komatsuda Atsushi, Okuyama Shin, Masai Rie, Maki Nobuki, Kigawa Akihiko, Sawada Ken-Ichi

机构信息

Third Department of Internal Medicine, Akita University School of Medicine, 1-1-1 Hondo, Akita 010-8543, Japan.

出版信息

Clin Exp Nephrol. 2006 Jun;10(2):140-5. doi: 10.1007/s10157-006-0412-0.

Abstract

A 46-year-old woman developed nephrotic syndrome at the age of 16 in 1973. On the basis of the histological findings of the first renal biopsy, she was diagnosed as having minimal change nephrotic syndrome. Initial treatment with steroid was effective, but she had several relapses during tapering of the daily dose of steroid. The second renal biopsy, performed in 1997, disclosed glomerular lobulation, mesangial proliferation, nodular mesangial lesions, and mesangiolysis. From 2001, the degree of proteinuria increased, with urinary protein being 5 g/day in January 2003, when a third renal biopsy was performed. On light microscopy, the glomerular lesions were similar to those observed in 1997. Immunofluorescence microscopy revealed coarse granular stainings for IgG, IgA, IgM, kappa, lambda, and C3 in the mesangial area and along the capillary walls. On electron microscopy, fingerprint structures were observed in the mesangial and subendothelial deposits. There were no characteristic fibers in the nodular lesions. On the basis of clinical and laboratory findings in this patient, we excluded disease entities in which nodular mesangial lesions, mesangiolysis, and fingerprint deposits had been reported. To our knowledge, such a unique combination of glomerular lesions has not been described previously in the literature.

摘要

一名46岁女性于1973年16岁时患上肾病综合征。根据首次肾活检的组织学结果,她被诊断为微小病变型肾病综合征。最初使用类固醇治疗有效,但在逐渐减少类固醇日剂量期间她多次复发。1997年进行的第二次肾活检显示肾小球分叶、系膜增生、结节性系膜病变和系膜溶解。从2001年起,蛋白尿程度增加,2003年1月尿蛋白为5g/天,此时进行了第三次肾活检。光镜下,肾小球病变与1997年观察到的相似。免疫荧光显微镜检查显示在系膜区和沿毛细血管壁有IgG、IgA、IgM、κ、λ和C3的粗颗粒染色。电镜下,在系膜和内皮下沉积物中观察到指纹结构。结节性病变中没有特征性纤维。根据该患者的临床和实验室检查结果,我们排除了已报道有结节性系膜病变、系膜溶解和指纹状沉积物的疾病实体。据我们所知,这种独特的肾小球病变组合在以前的文献中尚未有描述。

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