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Congenital Nephrotic Syndrome with Focal Segmental Glomerular Sclerosis, Renal Insufficiency and Tubular Dysfunction

 

作者: Takashi Igarashi,   Toru Shibata,   Tsutomu Iwata,   Shigehiko Kamoshita,  

 

期刊: Pediatrics International  （WILEY Available online 1987）
卷期: Volume 29, issue 4  

页码: 614-618

 

ISSN:1328-8067

 

年代: 1987

 

DOI:10.1111/j.1442-200X.1987.tb02249.x

 

出版商: Blackwell Publishing Ltd

 

关键词: Congenital nephrotic syndrome;Glomerular sclerosis;Proximal tubular dysfunction;Lamina densa;Lamina rara

 

数据来源: WILEY

 

摘要:

AbstractA patient with congenital nephrotic syndrome with focal segmental glomerular sclerosis is reported. There was moderate renal insufficiency, proximal renal tubular acidosis, hematuria and glycosuria. The glomerular basement membrane showed irregular thickening, splitting of the lamina densa and reticulation of the lamina rara interna. In addition to the features of the nephrotic syndrome and renal insufficiency, tubular dysfunction is one of the early manifestations of this disease.

 

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