Sickle Cell Nephropathy

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Abstract
The presence of renal failure in sickle cell disease (SCD) ranges from 5 to 18% of the total population of SCD patients (1). Powars et al. (2), in a prospective, case-control study of patients with SCD compared with sickle cell hemoglobin C patients, documental 31 (4.2%) patients affected by renal failure. The median age at the time of renal failure was 23.1 yr. Survival time was 4 yr with a median age of death of 27 yr after the diagnosis of end-stage renal disease (ESRD) in spite of dialysis treatment. Proteinuria, hypertension, severe anemia, and hematuria were reliable predictors of chronic renal failure (2). In this series, the presence of the inherited Central African Republic βs-gene cluster haplotype in a patient with SCD increased significantly the risk of chronic renal failure (2). Recently, in a prospective survival analysis of 964 patients with sickle cell anemia in adults, Platt et al. (3) observed an 18% overall mortality in adult SCD patients with 40% of these (7.6% of the total) manifesting overt renal failure. None received a kidney transplant. By multivariate regression analysis, renal failure was identified as the major risk factor for early mortality in adult patients with SCD.

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