Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.
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.
Pediatric nephrology (Berlin, Germany)
Anemia, Sickle Cell; Kidney Diseases; Biopsy; Child, Preschool; Child; Adolescent
Children; Chronic kidney disease; Glomerular hyperfiltration; Glomerulopathy; Proteinuria; Sickle cell disease
Zahr, R. S., Yee, M. E., Weaver, J., Twombley, K., Matar, R., 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. Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study. Pediatric nephrology (Berlin, Germany) 34, 1435-1445 (2019).