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

Rima S. Zahr, 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

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalPediatric Nephrology
DOIs
StatePublished - Jan 1 2019

Keywords

  • Children
  • Chronic kidney disease
  • Glomerular hyperfiltration
  • Glomerulopathy
  • Proteinuria
  • Sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

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    Zahr, R. S., Yee, M. E., Weaver, J., Twombley, K., Matar, R. B., Aviles, D., Sreedharan, R., Rheault, M. N., Malatesta-Muncher, R., Stone, H., Srivastava, T., Kapur, G., Baddi, P., Volovelsky, O., Pelletier, J., Gbadegesin, R., Seeherunvong, W., Patel, H. P., & Greenbaum, L. A. (2019). Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study. Pediatric Nephrology. https://doi.org/10.1007/s00467-019-04237-3