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

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

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalPediatric Nephrology
DOIs
StatePublished - Jan 1 2019

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Nephrology
Sickle Cell Anemia
Pediatrics
Kidney
Biopsy
Thrombotic Microangiopathies
Membranoproliferative Glomerulonephritis
Transplants
Focal Segmental Glomerulosclerosis
Child Mortality
Hydroxyurea
Angiotensin Receptor Antagonists
Disease Management
Chronic Renal Insufficiency
Proteinuria
Angiotensin-Converting Enzyme Inhibitors
Renal Dialysis
Creatinine
Bone Marrow
Morbidity

Keywords

  • Children
  • Chronic kidney disease
  • Glomerular hyperfiltration
  • Glomerulopathy
  • Proteinuria
  • Sickle cell disease

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology

Cite this

Kidney biopsy findings in children with sickle cell disease : a Midwest Pediatric Nephrology Consortium study. / Zahr, Rima S.; Yee, Marianne E.; Weaver, Jack; Twombley, Katherine; Matar, Raed Bou; Aviles, Diego; Sreedharan, Rajasree; Rheault, Michelle N.; Malatesta-Muncher, Rossana; Stone, Hillarey; Srivastava, Tarak; Kapur, Gaurav; Baddi, Poornima; Volovelsky, Oded; Pelletier, Jonathan; Gbadegesin, Rasheed; Seeherunvong, Wacharee; Patel, Hiren P.; Greenbaum, Larry A.

In: Pediatric Nephrology, 01.01.2019.

Research output: Contribution to journalArticle

Zahr, RS, Yee, ME, Weaver, J, Twombley, K, Matar, RB, Aviles, D, Sreedharan, R, Rheault, MN, Malatesta-Muncher, R, Stone, H, Srivastava, T, Kapur, G, Baddi, P, Volovelsky, O, Pelletier, J, Gbadegesin, R, Seeherunvong, W, Patel, HP & Greenbaum, LA 2019, 'Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study', Pediatric Nephrology. https://doi.org/10.1007/s00467-019-04237-3
Zahr, Rima S. ; Yee, Marianne E. ; Weaver, Jack ; Twombley, Katherine ; Matar, Raed Bou ; Aviles, Diego ; Sreedharan, Rajasree ; Rheault, Michelle N. ; Malatesta-Muncher, Rossana ; Stone, Hillarey ; Srivastava, Tarak ; Kapur, Gaurav ; Baddi, Poornima ; Volovelsky, Oded ; Pelletier, Jonathan ; Gbadegesin, Rasheed ; Seeherunvong, Wacharee ; Patel, Hiren P. ; Greenbaum, Larry A. / Kidney biopsy findings in children with sickle cell disease : a Midwest Pediatric Nephrology Consortium study. In: Pediatric Nephrology. 2019.
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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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AU - Zahr, Rima S.

AU - Yee, Marianne E.

AU - Weaver, Jack

AU - Twombley, Katherine

AU - Matar, Raed Bou

AU - Aviles, Diego

AU - Sreedharan, Rajasree

AU - Rheault, Michelle N.

AU - Malatesta-Muncher, Rossana

AU - Stone, Hillarey

AU - Srivastava, Tarak

AU - Kapur, Gaurav

AU - Baddi, Poornima

AU - Volovelsky, Oded

AU - Pelletier, Jonathan

AU - Gbadegesin, Rasheed

AU - Seeherunvong, Wacharee

AU - Patel, Hiren P.

AU - Greenbaum, Larry A.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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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