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膜周型肾淀粉样变性:一种特殊形式的轻链淀粉样变性。

Perimembranous-type renal amyloidosis: a peculiar form of AL amyloidosis.

作者信息

Shiiki H, Shimokama T, Yoshikawa Y, Onoyama K, Morimatsu M, Watanabe T

机构信息

Department of Pathology, Saga Medical School, Japan.

出版信息

Nephron. 1989;53(1):27-32. doi: 10.1159/000185697.

Abstract

Six cases of perimembranous-type renal amyloidosis were reported. This type of renal amyloidosis was characterized by amyloid deposition predominantly involving the epithelial aspect of the glomerular capillary wall. Florid spicular arrangement was another representative feature. This type of amyloid deposition was found in 8% of autopsy cases of systemic amyloidosis. All cases were categorized as AL amyloidosis and developed the nephrotic syndrome irrespective of the amount of amyloid within the glomeruli. The results obtained suggest that perimembranous-type renal amyloidosis is a peculiar form of AL amyloidosis both in morphology and clinical manifestations. autopsy cases of systemic amyloidosis. All cases were categorized as AL amyloidosis and developed the nephrotic syndrome irrespective of the amount of amyloid within the glomeruli. The results obtained suggest that perimembranous-type renal amyloidosis is a peculiar form of AL amyloidosis both in morphology and clinical manifestations. autopsy cases of systemic amyloidosis. All cases were categorized as AL amyloidosis and developed the nephrotic syndrome irrespective of the amount of amyloid within the glomeruli. The results obtained suggest that perimembranous-type renal amyloidosis is a peculiar form of AL amyloidosis both in morphology and clinical manifestations.

摘要

报告了6例膜周型肾淀粉样变性病例。这种类型的肾淀粉样变性的特征是淀粉样蛋白沉积主要累及肾小球毛细血管壁的上皮侧。典型的针状排列是另一个代表性特征。这种类型的淀粉样蛋白沉积在系统性淀粉样变性尸检病例中占8%。所有病例均归类为AL型淀粉样变性,且均出现肾病综合征,无论肾小球内淀粉样蛋白的量多少。所得结果表明,膜周型肾淀粉样变性在形态学和临床表现上都是AL型淀粉样变性的一种特殊形式。系统性淀粉样变性尸检病例。所有病例均归类为AL型淀粉样变性,且均出现肾病综合征,无论肾小球内淀粉样蛋白的量多少。所得结果表明,膜周型肾淀粉样变性在形态学和临床表现上都是AL型淀粉样变性的一种特殊形式。系统性淀粉样变性尸检病例。所有病例均归类为AL型淀粉样变性,且均出现肾病综合征,无论肾小球内淀粉样蛋白的量多少。所得结果表明,膜周型肾淀粉样变性在形态学和临床表现上都是AL型淀粉样变性的一种特殊形式。

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