Silicon Nephropathy Mimicking Fabry’s Disease
作者:
Daniel E. Banks,
Jovan Milutinovic,
Robert J. Desnick,
Gregory A. Grabowski,
N. LeRoy Lapp,
Brian A. Boehlecke,
期刊:
American Journal of Nephrology
(Karger Available online 1983)
卷期:
Volume 3,
issue 5
页码: 279-284
ISSN:0250-8095
年代: 1983
DOI:10.1159/000166730
出版商: S. Karger AG
关键词: Silicosis;Nephropathy;Fabry’s disease
数据来源: Karger
摘要:
Percutaneous renal biopsy was performed in a surface coal miner with radiographic and histopathologic pulmonary changes consistent with acute silicolipoproteinosis who developed proteinuria and hematuria. Electron microscopic evaluation of the renal tissue specimen revealed a diffusely thickened glomerular basement membrane, foot process effacement, and dense lamellar inclusions in swollen glomerular epithelial cells, similar to those seen in Fabry’s disease. However, normal levels of plasma α-galactosidase A, normal urinary sediment glycosphingolipids and the absence of the clinical characteristics of Fabry’s disease excluded this diagnosis. This case illustrates that electron-dense lamellar inclusions, similar to those seen in Fabry’s disease, may be seen in other entities such as nephropathy associated with sil
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