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伴有各种结晶沉积物的肾小球肾炎。

Glomerulonephritis with various crystalline deposits.

作者信息

Nishi S, Ueno M, Suzuki S, Karasawa R, Hayashi H, In H, Saitou T, Kon K, Miura Y, Arakawa M

机构信息

Second Department of Medicine, Niigata University School of Medicine, Japan.

出版信息

Am J Nephrol. 1993;13(4):271-4. doi: 10.1159/000168632.

Abstract

A 60-year-old Japanese male was admitted with nephrotic syndrome complaining of facial and pedal edema. Renal biopsy revealed mesangial and endocapillary proliferation with massive mesangial and subendothelial deposits. Deposits of IgA, IgG, IgM, C3 and fibrinogen were identified by immunofluorescence technique in both mesangial and subendothelial areas. Deposits of kappa and lambda light chains were also recognized in similar patterns. Electron microscopy revealed crystalline structures of various appearance in these deposits. A large deposit consisted of various small triangular, quadrilateral and polygonal shapes of deposits with crystalline structures. These crystalline structures exhibited lattice or parallel lamellar lines with different periodic distances. Although a disease producing paraproteinemia was suspected, no immunoglobulin disorder or light chain abnormality could be detected on clinical and laboratory examinations. On the second biopsy, a faint reaction for cryoglobulin was recognized. However, the crystalline deposits observed in this case were absolutely different from the tubular structures seen in cryoglobulinemic nephropathy. This was a very rare and unclassified case.

摘要

一名60岁的日本男性因肾病综合征入院,伴有面部和足部水肿。肾活检显示系膜和毛细血管内增生,伴有大量系膜和内皮下沉积物。通过免疫荧光技术在系膜和内皮下区域均鉴定出IgA、IgG、IgM、C3和纤维蛋白原的沉积物。κ和λ轻链的沉积物也呈相似模式被识别。电子显微镜检查显示这些沉积物中有各种外观的晶体结构。一个大的沉积物由各种带有晶体结构的小三角形、四边形和多边形沉积物组成。这些晶体结构呈现出具有不同周期距离的晶格或平行层状线。尽管怀疑存在产生副蛋白血症的疾病,但临床和实验室检查未发现免疫球蛋白紊乱或轻链异常。在第二次活检时,发现了微弱的冷球蛋白反应。然而,该病例中观察到的晶体沉积物与冷球蛋白血症性肾病中所见的管状结构完全不同。这是一个非常罕见且未分类的病例。

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