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[Kidney and hemoglobinopathy].

Philippe Rémy1, Vincent Audard1, Frédéric Galactéros1

  • 1Service de néphrologie-dialyse-transplantation, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France.

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|March 8, 2016
PubMed
Summary
This summary is machine-generated.

Sickle-cell disease (SCD) significantly increases the risk of chronic kidney disease due to hemoglobin S polymerization. This review explores the causes and varied kidney problems in SCD patients.

Keywords:
DrépanocytoseHemoglobinopathyHémoglobinopathiesInsuffisance rénaleNephropathyNéphropathieRenal failureSickle cell diseaseThalassémie

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Area of Science:

  • Nephrology
  • Hematology
  • Genetics

Background:

  • Sickle-cell disease (SCD) is a common, severe monogenic disorder globally.
  • It stems from a mutation in the beta-globin gene, producing hemoglobin S (HbS).
  • HbS polymerization causes red blood cell rigidity and vaso-occlusive episodes, central to SCD pathogenesis.

Purpose of the Study:

  • To review the pathogenesis of sickle cell nephropathy.
  • To outline the spectrum of renal manifestations in SCD patients.

Main Methods:

  • Literature review focusing on SCD and renal involvement.
  • Analysis of pathophysiological mechanisms underlying sickle cell nephropathy.

Main Results:

  • SCD is linked to a high frequency of chronic kidney disease.
  • Renal manifestations in SCD are diverse and age-dependent.
  • Endothelial dysfunction from chronic hemolysis is a key factor in kidney damage.

Conclusions:

  • Understanding SCD pathogenesis is crucial for managing kidney complications.
  • The broad spectrum of renal diseases in SCD requires comprehensive clinical attention.