Kidney failure outcomes in children and young adults with sickle cell disease in the United States Renal Data System

Pediatr Nephrol. 2024 Feb;39(2):619-623. doi: 10.1007/s00467-023-06136-0. Epub 2023 Sep 1.

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 kidney failure (chronic kidney disease stage 5) due to SCD are not well described. This study aimed to assess the outcome of children and young adults with SCD with kidney failure compared to matched children and young adults without SCD with kidney failure in a large national database.

Methods: Utilizing the United States Renal Data System (USRDS), we retrospectively examined kidney failure outcomes in children and young adults with SCD from 1998 to 2019.

Results: We identified 97 patients with SCD who developed kidney failure and identified 96 matched controls with a median age of 19 years (IQR 17, 21) at the time of kidney failure diagnosis. SCD patients had significantly shorter survival (8.4 years vs. 14.0 years, p < 0.001) and had a longer waiting time for their first transplant when compared to matched non-SCD kidney failure patients (12.1 years vs. 7.3 years, p < 0.001).

Conclusions: Children and young adults with SCD kidney failure have significantly higher mortality when matched to non-SCD kidney failure children and experience a longer mean time to kidney transplant.

Keywords: Dialysis; Outcomes; Sickle cell disease; Transplant.

MeSH terms

  • Anemia, Sickle Cell* / complications
  • Anemia, Sickle Cell* / epidemiology
  • Anemia, Sickle Cell* / therapy
  • Child
  • Humans
  • Kidney
  • Kidney Failure, Chronic* / epidemiology
  • Kidney Failure, Chronic* / etiology
  • Kidney Failure, Chronic* / therapy
  • Renal Dialysis
  • Retrospective Studies
  • United States / epidemiology
  • Young Adult