Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity

Focal segmental glomerulosclerosis refers to a set of particular histopathologic lesions in which steroid-resistant podocyte injury leads to patchy adhesions between the glomerular tuft and Bowman's capsule, followed by progressive glomerulosclerosis and proteinuric renal failure. Because of th...

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Main Authors: Elena Torban, Martin Bitzan, Paul Goodyer
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:International Journal of Nephrology
Online Access:http://dx.doi.org/10.1155/2012/246128
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author Elena Torban
Martin Bitzan
Paul Goodyer
author_facet Elena Torban
Martin Bitzan
Paul Goodyer
author_sort Elena Torban
collection DOAJ
description Focal segmental glomerulosclerosis refers to a set of particular histopathologic lesions in which steroid-resistant podocyte injury leads to patchy adhesions between the glomerular tuft and Bowman's capsule, followed by progressive glomerulosclerosis and proteinuric renal failure. Because of the nonspecific nature of this lesion, it has been difficult to classify the various forms of primary nephrotic syndrome in children. However, with the recognition of hereditary FSGS caused by mutations podocyte slit diaphragm genes, it is increasingly clear that the steroid-resistant form of FSGS that recurs in the renal allografts (R-FSGS) constitutes a distinct clinical entity. Capitalizing on recent studies in which patients have been screened for slit diaphragm gene mutations, this review focuses on the natural history and pathogenesis of R-FSGS.
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series International Journal of Nephrology
spelling doaj-art-398af2cf5adb4bf6a9d8267278cc6cc12025-02-03T01:31:42ZengWileyInternational Journal of Nephrology2090-214X2090-21582012-01-01201210.1155/2012/246128246128Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical EntityElena Torban0Martin Bitzan1Paul Goodyer2Division of Nephrology, Department of Medicine, McGill University, Montreal, QC, H3A 1A1, CanadaDivision of Pediatric Nephrology, Department of Pediatrics, McGill University, Montreal, QC, H3H 1P3, CanadaDivision of Pediatric Nephrology, Department of Pediatrics, McGill University, Montreal, QC, H3H 1P3, CanadaFocal segmental glomerulosclerosis refers to a set of particular histopathologic lesions in which steroid-resistant podocyte injury leads to patchy adhesions between the glomerular tuft and Bowman's capsule, followed by progressive glomerulosclerosis and proteinuric renal failure. Because of the nonspecific nature of this lesion, it has been difficult to classify the various forms of primary nephrotic syndrome in children. However, with the recognition of hereditary FSGS caused by mutations podocyte slit diaphragm genes, it is increasingly clear that the steroid-resistant form of FSGS that recurs in the renal allografts (R-FSGS) constitutes a distinct clinical entity. Capitalizing on recent studies in which patients have been screened for slit diaphragm gene mutations, this review focuses on the natural history and pathogenesis of R-FSGS.http://dx.doi.org/10.1155/2012/246128
spellingShingle Elena Torban
Martin Bitzan
Paul Goodyer
Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
International Journal of Nephrology
title Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
title_full Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
title_fullStr Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
title_full_unstemmed Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
title_short Recurrent Focal Segmental Glomerulosclerosis: A Discrete Clinical Entity
title_sort recurrent focal segmental glomerulosclerosis a discrete clinical entity
url http://dx.doi.org/10.1155/2012/246128
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