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 journalArticlepeer-review

4 Scopus citations

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)
Number of pages11
JournalPediatric Nephrology
Volume34
Issue number8
DOIs
StatePublished - Aug 2019

Keywords

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

PubMed: MeSH publication types

  • Journal Article
  • Multicenter Study

Fingerprint Dive into the research topics of 'Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study'. Together they form a unique fingerprint.

Cite this