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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Format: | Article |
Language: | English |
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Wiley
2012-01-01
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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. |
format | Article |
id | doaj-art-398af2cf5adb4bf6a9d8267278cc6cc1 |
institution | Kabale University |
issn | 2090-214X 2090-2158 |
language | English |
publishDate | 2012-01-01 |
publisher | Wiley |
record_format | Article |
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 |
work_keys_str_mv | AT elenatorban recurrentfocalsegmentalglomerulosclerosisadiscreteclinicalentity AT martinbitzan recurrentfocalsegmentalglomerulosclerosisadiscreteclinicalentity AT paulgoodyer recurrentfocalsegmentalglomerulosclerosisadiscreteclinicalentity |