End Stage Kidney Disease Outcomes in Children and Young Adults with Sickle Cell Disease in the United States Renal Data System
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Published on: April 4, 2025
Rima S Zahr1, Kenneth I Ataga2, Jeffrey D Lebensburger3
1University of Tennessee Health Science Center College of Medicine Memphis.
Insights
Children and young adults with sickle cell disease (SCD) experience end-stage kidney disease (ESKD) earlier and have worse outcomes. SCD patients face higher mortality and longer transplant wait times compared to matched controls.
Area of Science:
- Nephrology
- Pediatric Hematology
Background:
- Sickle cell disease (SCD) frequently leads to early-onset kidney disease in children and young adults.
- End-stage kidney disease (ESKD) prevalence and outcomes in pediatric SCD patients are not well-defined.
Approach:
- Retrospective analysis of the United States Renal Data System (USRDS) from 1998-2019.
- Examined ESKD outcomes in pediatric and young adult SCD patients.
- Matched SCD patients with non-SCD ESKD controls.
Key Points:
- Identified 97 pediatric/young adult SCD patients with ESKD and 96 matched controls.
- SCD patients had significantly shorter survival (7.0 vs. 12.4 years).
- SCD patients experienced longer transplant wait times (10.3 vs. 5.6 years).
Conclusions:
- Pediatric and young adult SCD patients with ESKD exhibit significantly higher mortality rates.
- SCD patients face prolonged waiting periods for kidney transplantation.
- These findings highlight the critical impact of SCD on kidney health and transplant outcomes.
Abstract:
Background: Children and young adults with sickle cell disease (SCD) develop kidney disease early in childhood with some patients progressing to require dialysis and kidney transplantation. The prevalence and outcomes of children with end stage kidney disease (ESKD) due to SCD is not well described. This study aimed to assess the burden and outcomes of ESKD in children and young adults with SCD in a large national database. Methods: Utilizing the United States Renal Data System (USRDS) we retrospectively examined ESKD outcomes in children and young adults with SCD from 1998 - 2019. Results: We identified 97 patients with SCD that developed ESKD and identified 96 matched controls with median age of 19 years (IQR 17, 21) at time of ESKD diagnosis. SCD patients had significantly shorter survival (7.0 years vs. 12.4 years, p < 0.001) and had a longer waiting time to their first transplant when compared to matched non-SCD-ESKD patients (10.3 years vs. 5.6 years, p < 0.001). Conclusions: Children and young adults with SCD-ESKD have a significantly higher mortality when matched to non-SCD-ESKD children and experience a longer mean time to kidney transplant.
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Acute Kidney Injury I: Introduction
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