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Kidney Week

Abstract: TH-OR048

Children in CKiD with Congenital, Non-Glomerular Form of Progressive CKD Will Experience Renal Replacement Therapy by 40 Years of Age

Session Information

Category: Pediatric Nephrology

  • 1600 Pediatric Nephrology

Authors

  • Ng, Derek, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, United States
  • Matheson, Matthew, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, United States
  • Warady, Bradley A., Children's Mercy Kansas City , Kansas City, Missouri, United States
  • Furth, Susan L., The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States
  • Munoz, Alvaro, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, United States
Background

The Chronic Kidney Disease in Children (CKiD) study provides a unique opportunity to prospectively describe a life course characterization of progressive non-glomerular disease prior to renal replacement therapy (RRT). Our aim was to maximize the heterogeneous disease duration at entry in this cohort to estimate the age by which all children with congenital, non-glomerular disease will require RRT.

Methods

The times to RRT in CKiD children were characterized by parametric survival models (generalized gamma (GG) distribution). The time scale was years from NG diagnosis (age) and accounted for late entries due to heterogeneous disease duration at enrollment. Models were fit overall, and stratified by race and gender.

Results

Among 626 children, the median duration of disease at entry was 9 years (range 2 to 18) and median duration at end of follow-up was 15 years (maximum= 27). Three diagnosis groups represented 67%: obstructive uropathy (24%), hypo/dysplasia (24%) and reflux nephropathy (19%). Over approximately 25 years (capitalizing on staggered entries), 186 initiated RRT. The GG fit was excellent and demonstrated that 50% received RRT by age 18.5 and estimated that 99.6% will receive RRT by age 40 (Figure). The times to RRT did not differ significantly by race (p=0.25) or gender (p=0.40), with similar 40 year estimates of RRT for nonblacks (99.9%), blacks (98.8%), males (99.9%) and females (97.9%).

Conclusion

Practically all CKiD participants with progressive non-glomerular disease will receive RRT by 40 years old, much earlier than reports from European registries. This is likely due to a combination of access to care, organ availability and disease severity. Methodologically, these data enable handling right censored observations as interval censored, thus enriching parametric survival methods.

Percent of children with non-glomerular CKD initiating RRT by age

Funding

  • NIDDK Support