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


Journal

Pediatric nephrology (Berlin, Germany)
ISSN: 1432-198X
Titre abrégé: Pediatr Nephrol
Pays: Germany
ID NLM: 8708728

Informations de publication

Date de publication:
08 2019
Historique:
received: 11 09 2018
accepted: 11 03 2019
revised: 09 03 2019
pubmed: 5 4 2019
medline: 6 6 2020
entrez: 5 4 2019
Statut: ppublish

Résumé

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. 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. 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. 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.

Sections du résumé

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.

Identifiants

pubmed: 30945006
doi: 10.1007/s00467-019-04237-3
pii: 10.1007/s00467-019-04237-3
doi:

Types de publication

Journal Article Multicenter Study

Langues

eng

Sous-ensembles de citation

IM

Pagination

1435-1445

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Auteurs

Rima S Zahr (RS)

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.

Marianne E Yee (ME)

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.

Jack Weaver (J)

Levine Children's Hospital, Charlotte, NC, USA.

Katherine Twombley (K)

Medical University of South Carolina, Charleston, SC, USA.

Raed Bou Matar (RB)

Cleveland Clinic, Cleveland, OH, USA.

Diego Aviles (D)

Division of Pediatric Nephrology, LSU Health Sciences Center and Children's Hospital New Orleans, New Orleans, LA, USA.

Rajasree Sreedharan (R)

Medical College of Wisconsin, Milwaukee, WI, USA.

Michelle N Rheault (MN)

University of Minnesota, Minneapolis, MN, USA.

Rossana Malatesta-Muncher (R)

Baylor School of Medicine, Houston, TX, USA.

Hillarey Stone (H)

Childrens's Mercy Hospital, Kansas City, MO, USA.

Tarak Srivastava (T)

Childrens's Mercy Hospital, Kansas City, MO, USA.

Gaurav Kapur (G)

Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.

Poornima Baddi (P)

Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.

Oded Volovelsky (O)

Cincinnati Children's Hospital, Cincinnati, OH, USA.

Jonathan Pelletier (J)

Duke University, Durham, NC, USA.

Rasheed Gbadegesin (R)

Duke University, Durham, NC, USA.

Wacharee Seeherunvong (W)

University of Miami Miller School of Medicine, Miami, FL, USA.

Hiren P Patel (HP)

Nationwide Children's Hospital, Columbus, OH, USA.

Larry A Greenbaum (LA)

Department of Pediatrics, Division of Nephrology, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA, USA.

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