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Abstract: TH-PO631

Membranous Nephropathy Outcomes Among Children and Adults: Cure Glomerulonephropathy Study (CureGN)

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Villegas, Leonela A., Connecticut Children's Medical Center, Hartford, Connecticut, United States
  • Helmuth, Margaret, Arbor Research Collaborative for Health, Ann Arbor, Michigan, United States
  • Derebail, Vimal K., University of North Carolina Wilmington, Wilmington, North Carolina, United States
  • Ayoub, Isabelle, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Almaani, Salem, The Ohio State University Wexner Medical Center, Columbus, Ohio, United States
  • Robinson, Bruce M., University of Michigan, Ann Arbor, Michigan, United States
  • Laurin, Louis-Philippe, Hopital Maisonneuve-Rosemont, Montreal, Quebec, Canada
  • Waldman, Meryl, National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, Maryland, United States
Background

Membranous nephropathy (MN) is a rare entity in children, whereas it’s one of the most common causes of primary nephrotic syndrome in adults. There are limited data on disease progression in children, as well as uncertainty about differences between childhood vs adult-onset MN.

Methods

CureGN is a multi-center prospective, observational cohort of individuals with biopsy-proven glomerular diseases. For this analysis, baseline clinical data and proportion of time on immunosuppression (IS) across study follow up were reported. Cox proportional hazard models were used to estimate time to kidney outcomes, and linear mixed models to assess estimated glomerular filtration rate (eGFR) trajectories. Models were adjusted for sex, age at biopsy, IS prior to biopsy, urine protein to creatinine ratio (UPCR), eGFR, and time from biopsy to enrollment.

Results

Among 608 MN participants (580 with ≥1 follow-up visit), 54 (9%) had childhood-onset MN (age <18 at time of biopsy) with characteristics including: 48% female, median age 14 years (IQR, 12-17), and 15% self-identify as Black. Overall, childhood-onset MN had an increased risk of end stage kidney disease (HR, 5.1; 95% CI, 1.45-18) in comparison to adults. The eGFR decline was steeper in children (-3.75 (±0.87) ml/min/1.73m2 per year) than adults (-0.14 (±0.78) ml/min/1.73m2 per year) (p<0.0001).

Conclusion

Despite potential for remission, children with MN had steeper eGFR decline and faster time to kidney failure than adults. Additional data are needed to extend our understanding of determinants of disease and outcomes to guide therapies and inform practice guidelines.

 Children (n=54)Adults (n=554)
Enrollment  
Median eGFR (ml/min/1.73m2, IQR)*107 (91-122)77 (50-98)
Median UPCR (mg/mg, IQR)1.5 (0.4-5.3)2.9 (0.9-6.6)
   
Follow-up  
Follow-up time (years, IQR)4.7 (3.0-6.4)4.4 (2.4-6.0)
Proportion of time off IS (%)5774

*CKiD-U25 if age <25 year, CKD-EPI-2021 if age ≥ 25 years

Funding

  • NIDDK Support