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Nephrol Ther. 2014 Feb;10(1):10-6. doi: 10.1016/j.nephro.2013.07.366. Epub 2013 Oct 7.

[Spectrum of renal manifestations in sickle cell disease].

[Article in French]

Author information

  • 1Inserm U970, Paris cardiovascular centre (PARCC), université Paris Descartes, Sorbonne Paris cité, 75015 Paris, France.
  • 2Service de pédiatrie générale, hôpital Robert-Debré, université Paris Diderot, AP-HP, 75019 Paris, France.
  • 3Service d'anatomopathologie, hôpital européen Georges-Pompidou, université Paris Descartes, AP-HP, 75015 Paris, France.
  • 4Inserm U970, Paris cardiovascular centre (PARCC), université Paris Descartes, Sorbonne Paris cité, 75015 Paris, France; Service de néphrologie, hôpital européen Georges-Pompidou, université Paris Descartes, AP-HP, 75015 Paris, France.
  • 5Inserm U 955, service de néphrologie et de transplantation, institut francilien de recherche en néphrologie et transplantation (IFRNT), hôpital Henri-Mondor, université Paris Est Créteil, AP-HP, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil, France. Electronic address: vincent.audard@hmn.aphp.fr.

Abstract

Sickle cell disease (SCD), the most common hemoglobinopathy, is an increasing cause of chronic kidney disease. In the last decade, we have witnessed a better understanding in the characterization of clinical manifestations and pathogenesis of sickle cell nephropathy. The spectrum of renal diseases during SCD includes various renal manifestations such as impairment of urinary concentrating ability, defect in urine acidification, renal papillary necrosis and proteinuria related to glomerular injury leading to progressive end-stage renal disease. Endothelial dysfunction related to chronic hemolysis and the relative renal hypoxia caused by vaso-occlusive sickle red blood cells are probably two key factors for SCN development. Optimal therapeutic management (including the use of blockers of the renin-angiotensin system) of patients with proteinuria remains to be determined. Renal replacement therapy with dialysis is required in SCD patients with end-stage renal disease but these patients should probably undergo kidney transplantation that requires careful management.

Copyright © 2013 Association Société de néphrologie. Published by Elsevier SAS. All rights reserved.

KEYWORDS:

Drépanocytose; Falciformation; Glomerulopathy; Glomérulopathie; Insuffisance rénale; Nécrose papillaire; Papillary necrosis; Proteinuria; Protéinurie; Red cell sickling; Renal failure; Sickle cell disease

PMID:
24113202
[PubMed - in process]
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