A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy
Adult-onset nephrotic syndrome (NS) is commonly caused by minimal change disease, focal segmental glomerulosclerosis, andmembranous nephropathy. Rare causes of NS include amyloidosis, immunoglobulin deposition disease, fibronectin glomerulopathy, and Collagenofibrotic glomerulopathy (CG). CG is caus...
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Wolters Kluwer Medknow Publications
2021-01-01
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Series: | Saudi Journal of Kidney Diseases and Transplantation |
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doaj-d65deeec4bfd4b1eaba28f375ac1fa082021-07-07T14:41:50ZengWolters Kluwer Medknow PublicationsSaudi Journal of Kidney Diseases and Transplantation1319-24422021-01-0132122322610.4103/1319-2442.318528A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic GlomerulopathyPradeep ShenoyJoshi DivyaAdult-onset nephrotic syndrome (NS) is commonly caused by minimal change disease, focal segmental glomerulosclerosis, andmembranous nephropathy. Rare causes of NS include amyloidosis, immunoglobulin deposition disease, fibronectin glomerulopathy, and Collagenofibrotic glomerulopathy (CG). CG is caused by deposition of Type 3 collagen in the mesangium and subendothelial area. It usually presents as asymptomatic proteinuria, NS, hypertension, and renal failure. Histologically, it can present as Congo red-negative nodular glomerulosclerosis and requires electron microscopy for confirmation of diagnosis. Electron microscopy shows characteristic fibers which are curved, frayed and have a transverse band with periodicity of 43–65 nm. There is no specific treatment, and it can recur after kidney transplantation.http://www.sjkdt.org/article.asp?issn=1319-2442;year=2021;volume=32;issue=1;spage=223;epage=226;aulast=Shenoy |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Pradeep Shenoy Joshi Divya |
spellingShingle |
Pradeep Shenoy Joshi Divya A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy Saudi Journal of Kidney Diseases and Transplantation |
author_facet |
Pradeep Shenoy Joshi Divya |
author_sort |
Pradeep Shenoy |
title |
A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy |
title_short |
A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy |
title_full |
A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy |
title_fullStr |
A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy |
title_full_unstemmed |
A Rare Cause of Nephrotic Syndrome in Adults – Collagenofibrotic Glomerulopathy |
title_sort |
rare cause of nephrotic syndrome in adults – collagenofibrotic glomerulopathy |
publisher |
Wolters Kluwer Medknow Publications |
series |
Saudi Journal of Kidney Diseases and Transplantation |
issn |
1319-2442 |
publishDate |
2021-01-01 |
description |
Adult-onset nephrotic syndrome (NS) is commonly caused by minimal change disease, focal segmental glomerulosclerosis, andmembranous nephropathy. Rare causes of NS include amyloidosis, immunoglobulin deposition disease, fibronectin glomerulopathy, and Collagenofibrotic glomerulopathy (CG). CG is caused by deposition of Type 3 collagen in the mesangium and subendothelial area. It usually presents as asymptomatic proteinuria, NS, hypertension, and renal failure. Histologically, it can present as Congo red-negative nodular glomerulosclerosis and requires electron microscopy for confirmation of diagnosis. Electron microscopy shows characteristic fibers which are curved, frayed and have a transverse band with periodicity of 43–65 nm. There is no specific treatment, and it can recur after kidney transplantation. |
url |
http://www.sjkdt.org/article.asp?issn=1319-2442;year=2021;volume=32;issue=1;spage=223;epage=226;aulast=Shenoy |
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