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与新月体性肾小球肾炎相关的肾淀粉样变性

Renal amyloidosis associated with crescentic glomerulonephritis.

作者信息

Harada A, Tomita Y, Yamamoto H, Onoyama K, Omae T, Oh Y, Kimura T

出版信息

Am J Nephrol. 1984;4(1):52-5. doi: 10.1159/000166774.

Abstract

A case of renal amyloidosis coincidentally associated with crescentic glomerulonephritis is described. A 59-year-old female with 30 years' history of rheumatoid arthritis developed nephrotic syndrome followed by rapid deterioration of renal function. Among 28 glomeruli in a kidney biopsy specimen, glomerular amyloid deposition was present in 17 and crescent formation in 20. Only one glomerulus was free from amyloid deposition or crescent. The amyloid deposition was clearly demonstrated by congo red, thioflavin-T staining as well as electron-microscopic examination. Glomerular deposition of immunoglobulins and/or complements were not noted. While cyclophosphamide, dipyridamole, and heparin followed by pulse methylprednisolone were given, the renal function deteriorated progressively and dialysis was initiated. Renal function, however, recovered partially without medication after peritoneal dialysis of 3 months' duration. The second renal biopsy revealed the progression of glomerular amyloid deposition and the increase in fibrous component of crescent.

摘要

本文描述了一例巧合合并新月体性肾小球肾炎的肾淀粉样变性病例。一名患有30年类风湿关节炎病史的59岁女性出现肾病综合征,随后肾功能迅速恶化。在一份肾活检标本的28个肾小球中,17个存在肾小球淀粉样沉积,20个有新月体形成。仅1个肾小球无淀粉样沉积或新月体。刚果红、硫黄素-T染色以及电子显微镜检查均清晰显示了淀粉样沉积。未发现免疫球蛋白和/或补体的肾小球沉积。给予环磷酰胺、双嘧达莫和肝素,随后进行甲泼尼龙冲击治疗,但肾功能仍逐渐恶化,遂开始透析。然而,在持续3个月的腹膜透析后,肾功能在未用药的情况下部分恢复。第二次肾活检显示肾小球淀粉样沉积进展以及新月体纤维成分增加。

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