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Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study

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Abstract

Background

Renal damage is a progressive complication of sickle cell disease (SCD). Microalbuminuria is common in children with SCD, while a smaller number of children have more severe renal manifestations necessitating kidney biopsy. There is limited information on renal biopsy findings in children with SCD and subsequent management and outcome.

Methods

This is a multicenter retrospective analysis of renal biopsy findings and clinical outcomes in children and adolescents with SCD. We included children and adolescents (age ≤ 20 years) with SCD who had a kidney biopsy performed at a pediatric nephrology unit. The clinical indication for biopsy, biopsy findings, subsequent treatments, and outcomes were analyzed.

Results

Thirty-six SCD patients (ages 4–19 years) were identified from 14 centers with a median follow-up of 2.6 years (0.4–10.4 years). The indications for biopsy were proteinuria (92%) and elevated creatinine (30%). All biopsies had abnormal findings, including mesangial hypercellularity (75%), focal segmental glomerulosclerosis (30%), membranoproliferative glomerulonephritis (16%), and thrombotic microangiopathy (2%). There was increased use of hydroxyurea, angiotensin-converting-enzyme inhibitors, and angiotensin receptor blockers following renal biopsy. At last follow-up, 3 patients were deceased, 2 developed insulin-dependent diabetes mellitus, 6 initiated chronic hemodialysis, 1 received a bone marrow transplant, and 1 received a kidney transplant.

Conclusions

Renal biopsies, while not commonly performed in children with SCD, were universally abnormal. Outcomes were poor in this cohort of patients despite a variety of post-biopsy interventions. Effective early intervention to prevent chronic kidney disease (CKD) is needed to reduce morbidity and mortality in children with SCD.

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Author information

Authors and Affiliations

  1. Department of Pediatrics, Division Nephrology and Hypertension, The University of Tennessee and Le Bonheur Children’s Hospital, 49 N. Dunlap, Memphis, TN, 38105, USA

    Rima S. Zahr

  2. Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Division of Hematology/Oncology, Emory University School of Medicine, Atlanta, GA, USA

    Marianne E. Yee

  3. Levine Children’s Hospital, Charlotte, NC, USA

    Jack Weaver

  4. Medical University of South Carolina, Charleston, SC, USA

    Katherine Twombley

  5. Cleveland Clinic, Cleveland, OH, USA

    Raed Bou Matar

  6. Division of Pediatric Nephrology, LSU Health Sciences Center and Children’s Hospital New Orleans, New Orleans, LA, USA

    Diego Aviles

  7. Medical College of Wisconsin, Milwaukee, WI, USA

    Rajasree Sreedharan

  8. University of Minnesota, Minneapolis, MN, USA

    Michelle N. Rheault

  9. Baylor School of Medicine, Houston, TX, USA

    Rossana Malatesta-Muncher

  10. Childrens’s Mercy Hospital, Kansas City, MO, USA

    Hillarey Stone & Tarak Srivastava

  11. Children’s Hospital of Michigan, Wayne State University, Detroit, MI, USA

    Gaurav Kapur & Poornima Baddi

  12. Cincinnati Children’s Hospital, Cincinnati, OH, USA

    Oded Volovelsky

  13. Duke University, Durham, NC, USA

    Jonathan Pelletier & Rasheed Gbadegesin

  14. University of Miami Miller School of Medicine, Miami, FL, USA

    Wacharee Seeherunvong

  15. Nationwide Children’s Hospital, Columbus, OH, USA

    Hiren P. Patel

  16. Department of Pediatrics, Division of Nephrology, Emory University School of Medicine and Children’s Healthcare of Atlanta, Atlanta, GA, USA

    Larry A. Greenbaum

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  1. Rima S. Zahr
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  2. Marianne E. Yee
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  18. Hiren P. Patel
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  19. Larry A. Greenbaum
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Corresponding author

Correspondence to Rima S. Zahr.

Ethics declarations

Each of the 14 pediatric nephrology centers received approval from their local Institutional Review Boards for this retrospective study.

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The authors declare that they have no conflict of interest.

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Zahr, R.S., Yee, M.E., Weaver, J. et al. Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study. Pediatr Nephrol 34, 1435–1445 (2019). https://doi.org/10.1007/s00467-019-04237-3

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  • DOI: https://doi.org/10.1007/s00467-019-04237-3

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