Pathogenesis and therapy of focal segmental glomerulosclerosis: an update

Focal and segmental glomerulosclerosis (FSGS) is an important cause of steroid-resistant nephrotic syndrome in adults and children. It is responsible for 5–20% of all cases of end-stage kidney disease (ESKD) in the United States. The pathogenesis of FSGS has not been fully elucidated; however, data...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2011-07, Vol.26 (7), p.1001-1015
Main Authors: Gbadegesin, Rasheed, Lavin, Peter, Foreman, John, Winn, Michelle
Format: Article
Language:English
Subjects:
Care and treatment
Chronic kidney failure
Classification
Development and progression
Etiology
Genetic Predisposition to Disease
Glomerulonephritis
Glomerulosclerosis, Focal Segmental - classification
Glomerulosclerosis, Focal Segmental - etiology
Glomerulosclerosis, Focal Segmental - pathology
Glomerulosclerosis, Focal Segmental - therapy
Health aspects
Heparan sulfate
Heroin
Humans
Immunosuppressive Agents - therapeutic use
Kidney - pathology
Kidney diseases
Medicine & Public Health
Molecular genetics
Morphology
Nephrology
Pathogenesis
Pediatric research
Pediatrics
Podocytes - pathology
Remission (Medicine)
Review
Risk Factors
Stem Cell Transplantation
Steroids
Treatment Outcome
Urology
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Summary:Focal and segmental glomerulosclerosis (FSGS) is an important cause of steroid-resistant nephrotic syndrome in adults and children. It is responsible for 5–20% of all cases of end-stage kidney disease (ESKD) in the United States. The pathogenesis of FSGS has not been fully elucidated; however, data from molecular studies of familial cases in the last two decades suggest that FSGS is a defect of the podocyte. The therapeutic agents available for treatment of FSGS are not very effective and only a small percentage of affected individuals will achieve complete remission. Recent data from molecular biology and molecular genetics has provided insight into the mechanisms of action of old agents and also identification of other novel therapeutic targets. This review focuses on recent advances in the molecular pathogenesis of FSGS and currently available therapeutic agents as well as potential novel therapies.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-010-1692-x