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01-08-2019 | Chronic Kidney Disease | Original Article

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

Authors: 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

Published in: Pediatric Nephrology | Issue 8/2019

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.

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Title: Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study
Authors: 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
Publication date: 01-08-2019
Publisher: Springer Berlin Heidelberg
Keyword: Chronic Kidney Disease
Published in: Pediatric Nephrology / Issue 8/2019
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-019-04237-3

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

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

Abstract

Background

Methods

Results

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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