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

  • Rima S. ZahrEmail author
  • Marianne E. Yee
  • Jack Weaver
  • Katherine Twombley
  • Raed Bou Matar
  • Diego Aviles
  • Rajasree Sreedharan
  • Michelle N. Rheault
  • Rossana Malatesta-Muncher
  • Hillarey Stone
  • Tarak Srivastava
  • Gaurav Kapur
  • Poornima Baddi
  • Oded Volovelsky
  • Jonathan Pelletier
  • Rasheed Gbadegesin
  • Wacharee Seeherunvong
  • Hiren P. Patel
  • Larry A. Greenbaum
Original Article

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.

Keywords

Children Proteinuria Glomerulopathy Glomerular hyperfiltration Chronic kidney disease Sickle cell disease 

Notes

Compliance with ethical standards

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

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© IPNA 2019

Authors and Affiliations

  • Rima S. Zahr
    • 1
    Email author
  • Marianne E. Yee
    • 2
  • Jack Weaver
    • 3
  • Katherine Twombley
    • 4
  • Raed Bou Matar
    • 5
  • Diego Aviles
    • 6
  • Rajasree Sreedharan
    • 7
  • Michelle N. Rheault
    • 8
  • Rossana Malatesta-Muncher
    • 9
  • Hillarey Stone
    • 10
  • Tarak Srivastava
    • 10
  • Gaurav Kapur
    • 11
  • Poornima Baddi
    • 11
  • Oded Volovelsky
    • 12
  • Jonathan Pelletier
    • 13
  • Rasheed Gbadegesin
    • 13
  • Wacharee Seeherunvong
    • 14
  • Hiren P. Patel
    • 15
  • Larry A. Greenbaum
    • 16
  1. 1.Department of Pediatrics, Division Nephrology and HypertensionThe University of Tennessee and Le Bonheur Children’s HospitalMemphisUSA
  2. 2.Aflac Cancer and Blood Disorders Center, Children’s Healthcare of Atlanta, Department of Pediatrics, Division of Hematology/OncologyEmory University School of MedicineAtlantaUSA
  3. 3.Levine Children’s HospitalCharlotteUSA
  4. 4.Medical University of South CarolinaCharlestonUSA
  5. 5.Cleveland ClinicClevelandUSA
  6. 6.Division of Pediatric NephrologyLSU Health Sciences Center and Children’s Hospital New OrleansNew OrleansUSA
  7. 7.Medical College of WisconsinMilwaukeeUSA
  8. 8.University of MinnesotaMinneapolisUSA
  9. 9.Baylor School of MedicineHoustonUSA
  10. 10.Childrens’s Mercy HospitalKansas CityUSA
  11. 11.Children’s Hospital of MichiganWayne State UniversityDetroitUSA
  12. 12.Cincinnati Children’s HospitalCincinnatiUSA
  13. 13.Duke UniversityDurhamUSA
  14. 14.University of Miami Miller School of MedicineMiamiUSA
  15. 15.Nationwide Children’s HospitalColumbusUSA
  16. 16.Department of Pediatrics, Division of NephrologyEmory University School of Medicine and Children’s Healthcare of AtlantaAtlantaUSA

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