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Pathogenesis and therapy of focal segmental glomerulosclerosis: an update

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Abstract

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.

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Acknowledgements

Funding

NIH K08DK082495-01 and grants from the Nephcure foundation to RG. RG is a recipient of a Doris Duke Clinical Scientist Development Award.

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  1. Department of Pediatrics, Duke University Medical Center, Durham, NC, 27710, USA

    Rasheed Gbadegesin & John Foreman

  2. Department of Medicine, Duke University Medical Center, Durham, NC, 27710, USA

    Peter Lavin & Michelle Winn

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  1. Rasheed Gbadegesin
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  2. Peter Lavin
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Correspondence to Rasheed Gbadegesin.

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Gbadegesin, R., Lavin, P., Foreman, J. et al. Pathogenesis and therapy of focal segmental glomerulosclerosis: an update. Pediatr Nephrol 26, 1001–1015 (2011). https://doi.org/10.1007/s00467-010-1692-x

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