Format

Send to

Choose Destination
Pediatr Nephrol. 2019 Aug;34(8):1435-1445. doi: 10.1007/s00467-019-04237-3. Epub 2019 Apr 3.

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

Author information

1
Department of Pediatrics, Division Nephrology and Hypertension, The University of Tennessee and Le Bonheur Children's Hospital, 49 N. Dunlap, Memphis, TN, 38105, USA. rzahr@uthsc.edu.
2
Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta, Department of Pediatrics, Division of Hematology/Oncology, Emory University School of Medicine, Atlanta, GA, USA.
3
Levine Children's Hospital, Charlotte, NC, USA.
4
Medical University of South Carolina, Charleston, SC, USA.
5
Cleveland Clinic, Cleveland, OH, USA.
6
Division of Pediatric Nephrology, LSU Health Sciences Center and Children's Hospital New Orleans, New Orleans, LA, USA.
7
Medical College of Wisconsin, Milwaukee, WI, USA.
8
University of Minnesota, Minneapolis, MN, USA.
9
Baylor School of Medicine, Houston, TX, USA.
10
Childrens's Mercy Hospital, Kansas City, MO, USA.
11
Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
12
Cincinnati Children's Hospital, Cincinnati, OH, USA.
13
Duke University, Durham, NC, USA.
14
University of Miami Miller School of Medicine, Miami, FL, USA.
15
Nationwide Children's Hospital, Columbus, OH, USA.
16
Department of Pediatrics, Division of Nephrology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.

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; Chronic kidney disease; Glomerular hyperfiltration; Glomerulopathy; Proteinuria; Sickle cell disease

PMID:
30945006
DOI:
10.1007/s00467-019-04237-3

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center