Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.
Document Type
Article
Publication Date
8-1-2019
Identifier
DOI: 10.1007/s00467-019-04237-3
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.
Journal Title
Pediatric nephrology (Berlin, Germany)
Volume
34
Issue
8
First Page
1435
Last Page
1445
MeSH Keywords
Anemia, Sickle Cell; Kidney Diseases; Biopsy; Child, Preschool; Child; Adolescent
Keywords
Children; Chronic kidney disease; Glomerular hyperfiltration; Glomerulopathy; Proteinuria; Sickle cell disease
Recommended Citation
Zahr RS, Yee ME, Weaver J, et al. Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study. Pediatr Nephrol. 2019;34(8):1435-1445. doi:10.1007/s00467-019-04237-3
COinS
