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Published on: June 22, 2012
Renal abnormalities in sickle cell disease
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
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.
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.
- 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.
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