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青少年类风湿关节炎中的肾淀粉样变性

Renal amyloidosis in juvenile rheumatoid arthritis.

作者信息

Akhtar Naureen, Kiran Sadia, Hussain Abid, Suleman Bilquis A, Jaleel Shahila

机构信息

Department of Pediatric Nephrology, The Children's Hospital and Institute of Child Health, Lahore.

出版信息

J Coll Physicians Surg Pak. 2009 Feb;19(2):130-2.

Abstract

We report a case of a rare disorder of renal amyloidosis occurring as a complication of juvenile rheumatoid arthritis in a 16-year-old adolescent male. He presented with generalized edema and hypertension. The laboratory work-up showed nephrotic-range proteinuria and hypoalbuminemia and normal renal function despite bilateral shrunken kidneys revealed by the abdominal ultrasound. His renal biopsy showed deposition of amyloid fibrils in the form of homogenous eosinophilic material within the glomeruli demonstrating the pathognomonic apple-green birefringence by polarized light microscopy.

摘要

我们报告一例罕见的肾淀粉样变性疾病,该疾病发生于一名16岁青少年男性,是幼年型类风湿关节炎的并发症。他表现为全身水肿和高血压。实验室检查显示为肾病范围的蛋白尿和低白蛋白血症,尽管腹部超声显示双侧肾脏萎缩,但肾功能正常。他的肾活检显示淀粉样纤维以均匀嗜酸性物质的形式沉积在肾小球内,偏振光显微镜检查显示出特征性的苹果绿双折射。

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