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Sickle hemoglobinopathy and the kidney

T Manis, E A Friedman

    Contributions to Nephrology
    |January 1, 1977
    PubMed
    Summary
    This summary is machine-generated.

    Sickle cell anemia causes kidney problems, including impaired urine concentration and potential kidney failure. Treatments like dialysis and transplantation can manage advanced kidney disease in these patients.

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    Area of Science:

    • Nephrology
    • Hematology
    • Pathophysiology

    Background:

    • Sickle cell anemia (SCA) is associated with diverse renal functional and anatomical abnormalities.
    • The renal medulla is consistently affected, leading to reduced urinary concentrating and acidifying capacities.

    Purpose of the Study:

    • To elucidate the mechanisms underlying renal aberrations in sickle cell anemia.
    • To discuss potential therapeutic strategies for renal complications in SCA.

    Main Methods:

    • Review of existing literature on renal manifestations in sickle cell anemia.
    • Analysis of pathophysiological mechanisms, including sickling in the renal medulla and potential immune complex formation.

    Main Results:

    • Impaired renal medulla function, including the loop of Henle, is a common finding, potentially due to sickling.

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  • Hematuria, papillary necrosis, nephrotic syndrome, and glomerulonephritis can occur.
  • Evidence suggests immune complex glomerulonephritis secondary to tubular injury is a possible cause of progressive renal failure.

    • Conclusions:

    • Renal dysfunction in sickle cell anemia is multifactorial, involving vascular and immune-mediated processes.
    • Hemodialysis and renal transplantation are viable options for managing end-stage renal disease in SCA patients.