학술논문
Kidney biopsy findings in children with sickle cell disease: a Midwest Pediatric Nephrology Consortium study.
Document Type
Article
Author
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.
Source
Subject
*ACE inhibitors
*Kidney disease diagnosis
*Proteinuria diagnosis
*Sickle cell anemia diagnosis
*Sickle cell anemia treatment
*Thrombocytopenia
*Glomerulonephritis
*Hydroxyurea
*Angiotensin receptors
*Biopsy
*Bone marrow transplantation
*Creatinine
*Hemodialysis
*Type 1 diabetes
*Kidney transplantation
*Medical cooperation
*Pediatrics
*Research
*Urinary organ disease diagnosis
*Treatment effectiveness
*Retrospective studies
*Focal segmental glomerulosclerosis
*Diagnosis
*Therapeutics
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Language
ISSN
0931-041X
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. [ABSTRACT FROM AUTHOR]