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Renal abnormalities in sickle cell disease.

K I Ataga1, E P Orringer

  • 1Division of Hematology/Oncology, Department of Medicine, UNC Comprehensive Sickle Cell Program & General Clinical Research Center, University of North Carolina, Chapel Hill, North Carolina, USA. kataga@med.unc.edu

American Journal of Hematology
|March 8, 2000
PubMed
Summary

Sickle cell disease (SCD) causes various kidney problems, including impaired function and increased risk of end-stage renal disease (ESRD). Managing blood pressure and proteinuria may slow progression, but a cure for SCD is needed for prevention.

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Area of Science:

  • Nephrology
  • Hematology
  • Genetics

Background:

  • Sickle cell anemia and hemoglobinopathies present a wide range of renal abnormalities.
  • Patients exhibit impaired urinary concentration, acidification, and potassium excretion, alongside enhanced proximal tubular function.

Purpose of the Study:

  • To review the spectrum of renal abnormalities in sickle cell disease (SCD).
  • To discuss the pathophysiology, clinical manifestations, and management of sickle cell nephropathy.

Main Methods:

  • Literature review of renal complications in sickle cell disease.
  • Analysis of physiological and pathological changes in the kidneys of SCD patients.

Main Results:

  • Young SCD patients show supranormal renal hemodynamics (ERPF, GFR) that decline with age and prostaglandin inhibitor use.

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  • Proteinuria, hypertension, and anemia predict end-stage renal disease (ESRD).
  • Hematuria is common, but an association with renal medullary carcinoma exists, necessitating thorough evaluation.

    • Conclusions:

    • Sickle cell nephropathy encompasses diverse renal dysfunctions, including impaired concentrating ability, tubular defects, and hemodynamic changes.
    • Progression to ESRD can be slowed by managing hypertension and proteinuria, but a definitive cure for SCD is required for prevention.
    • Renal complications in SCD are significant, impacting patient morbidity and mortality, highlighting the need for targeted therapies and potential cures.