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Sickle Cell Nephropathy in Children

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Pediatric Nephrology

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Abstract

Sickle cell disease (SCD) is one of the most common genetic disorders in the United States, affecting over 100,000 Americans and millions of people worldwide, primarily of African or Mediterranean descent. SCD is a group of hemoglobinopathies inherited in an autosomal recessive fashion. Nearly all organ systems are affected by SCD, including the kidneys. The renal manifestations of SCD are known as sickle cell nephropathy (SCN). Multiple pathogenic processes contribute to development of SCN, notably renal ischemia and hemolysis, which result in both glomerular and tubulointerstitial disease. Clinically significant complications of SCD include hyperfiltration, albuminuria, hyposthenuria, hematuria, hypertension, acute kidney injury (AKI), and chronic kidney disease (CKD). Despite these complications, occurrence of end-stage kidney disease (ESKD) is relatively uncommon, particularly in children. Of those who progress to ESKD, both dialysis and transplantation are viable treatments. While transplantation in SCN is associated with increased complications compared to other causes of ESKD, it is preferred over dialysis due to more favorable outcomes.

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Authors and Affiliations

  1. Rainbow Babies and Children’s Hospital, Cleveland, OH, USA

    Thomas Graf

  2. Pediatric Hematology, Carle Foundation Hospital, Urbana, IL, USA

    Connie Piccone

  3. Center for Pediatric Nephrology, Cleveland Clinic Children’s, Cleveland, OH, USA

    Katherine MacRae Dell

  4. Department of Pediatrics, Case Western Reserve University, Cleveland, OH, USA

    Katherine MacRae Dell

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  1. Thomas Graf
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  2. Connie Piccone
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  3. Katherine MacRae Dell
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Correspondence to Katherine MacRae Dell .

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Editors and Affiliations

  1. Division of Nephrology, Department of Pediatric Specialties, Bambino Gesù Children’s Hospital – IRCCS, Rome, Italy

    Francesco Emma

  2. Division of Nephrology and Hypertension, The Heart Institute, Cincinnati Children’s Hospital Medical Center, College of Medicine, Cincinnati, OH, USA

    Stuart L. Goldstein

  3. Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India

    Arvind Bagga

  4. Division of Pediatric Nephrology, UPMC Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA

    Carlton M. Bates

  5. Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, NHS Foundation Trust and University College London Institute of Child Health, London, UK

    Rukshana Shroff

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Graf, T., Piccone, C., Dell, K.M. (2022). Sickle Cell Nephropathy in Children. In: Emma, F., Goldstein, S.L., Bagga, A., Bates, C.M., Shroff, R. (eds) Pediatric Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-52719-8_44

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  • DOI: https://doi.org/10.1007/978-3-030-52719-8_44

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-52718-1

  • Online ISBN: 978-3-030-52719-8

  • eBook Packages: MedicineReference Module Medicine

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