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Renal abnormalities in sickle cell disease.

M Allon1

  • 1Department of Medicine, University of Oklahoma Health Sciences Center, Oklahoma City.

Archives of Internal Medicine
|March 1, 1990
PubMed
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Sickle cell disease causes kidney problems, including impaired urine concentration and altered filtration. Early detection and management are key to preserving kidney function in affected individuals.

Area of Science:

  • Nephrology
  • Hematology
  • Pediatrics

Background:

  • Sickle cell disease (SCD) is linked to numerous renal abnormalities.
  • Patients exhibit impaired urinary concentrating ability but intact diluting capacity.
  • Defects in urinary acidification and potassium excretion are common, though severe acidosis and hyperkalemia are infrequent.

Purpose of the Study:

  • To summarize the spectrum of renal structural and functional abnormalities in sickle cell disease.
  • To highlight the impact of these abnormalities on kidney function and disease progression.
  • To discuss management strategies for renal complications in SCD.

Main Methods:

  • Review of existing literature on renal manifestations of sickle cell disease.
  • Analysis of functional tests assessing urinary concentrating/diluting capacity, acidification, and potassium excretion.

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  • Evaluation of markers for glomerular filtration rate (GFR) and renal plasma flow.
  • Assessment of proteinuria, renal lesions, and outcomes of end-stage renal disease management.

    • Main Results:

    • Proximal tubular function is supranormal, leading to hyperphosphatemia and overestimation of GFR by creatinine clearance.
    • GFR and renal plasma flow are elevated in young patients, decreasing with age and affected by prostaglandin inhibitors.
    • Proteinuria, focal glomerular sclerosis, and nephrotic syndrome are frequent, with hyperfiltration implicated in pathogenesis.
    • Hemodialysis and kidney transplantation are successful for end-stage renal disease; recurrent hematuria and papillary necrosis also occur.

    Conclusions:

    • Sickle cell disease significantly impacts renal structure and function, necessitating close monitoring.
    • Glomerular hyperfiltration and specific renal lesions contribute to progressive kidney damage.
    • While end-stage renal disease can be managed, other complications like hematuria and papillary necrosis require attention.