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Sickle cell nephropathy: challenging the conventional wisdom

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Abstract

This review explores the current model of sickle cell nephropathy and the limitations of the model. Renal abnormalities are common complications of sickle cell disease (SCD). Beginning in childhood, patients with SCD develop a urinary concentrating defect resulting in polyuria and a predisposition to nocturnal enuresis and dehydration. The current model of sickle cell nephropathy suggests that destruction of the renal medulla induces production of renal vasodilating substances that feedback to the glomerulus causing hyperfiltration. Hyperfiltration leads to glomerulosclerosis and proteinuria, with eventual reduction in kidney function. The crucial steps of vasodilating substance production and hyperfiltration in children with SCD have not been proven. Treatment of sickle cell nephropathy is aimed at the reduction of proteinuria with angiotensin converting enzyme inhibitors or angiotensin receptor blockers. Hydroxyurea and chronic transfusion therapy may also alter the progression of sickle cell nephropathy in children. Further studies are needed to identify an accurate model and effective treatments for sickle cell nephropathy.

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Acknowledgements

I would like to thank Dr. George Buchanan for his mentorship. This work was supported in part by the CTSA NIH Grant UL1-RR024982 and the Sickle Cell Scholar Award from NHLBI Grant U54-HL70588.

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  1. Department of Pediatrics, University of Texas Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX, 75390-9063, USA

    Amy M. Becker

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Becker, A.M. Sickle cell nephropathy: challenging the conventional wisdom. Pediatr Nephrol 26, 2099–2109 (2011). https://doi.org/10.1007/s00467-010-1736-2

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