Abstract: FR-PO0699
Challenging Clinical Practice Guideline Definitions in Childhood Nephrotic Syndrome
Session Information
- Pediatric Nephrology: CKD, ESKD, and Glomerular Diseases
November 07, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1900 Pediatric Nephrology
Authors
- Robinson, Cal, The Hospital for Sick Children, Toronto, Ontario, Canada
- Aman, Nowrin F., The Hospital for Sick Children, Toronto, Ontario, Canada
- Banh, Tonny Hue Minh, The Hospital for Sick Children, Toronto, Ontario, Canada
- Brooke, Josefina A., The Hospital for Sick Children, Toronto, Ontario, Canada
- Dhillon, Vaneet, The Hospital for Sick Children, Toronto, Ontario, Canada
- Licht, Christoph, The Hospital for Sick Children, Toronto, Ontario, Canada
- McKay, Ashlene Maree, The Hospital for Sick Children, Toronto, Ontario, Canada
- Pearl, Rachel Jane, The Hospital for Sick Children, Toronto, Ontario, Canada
- Radhakrishnan, Seetha, The Hospital for Sick Children, Toronto, Ontario, Canada
- Selvathesan, Nithiakishna, The Hospital for Sick Children, Toronto, Ontario, Canada
- Teoh, Chia Wei, The Hospital for Sick Children, Toronto, Ontario, Canada
- Vasilevska-Ristovska, Jovanka, The Hospital for Sick Children, Toronto, Ontario, Canada
- Parekh, Rulan S., The Hospital for Sick Children, Toronto, Ontario, Canada
Background
In childhood nephrotic syndrome, definitions of immunosuppression response, frequent relapses (FRNS), and long-term remission are conflicting and not evidence based. Our goal was to define treatment response, FRNS, and long-term remission based on associated disease outcomes.
Methods
We included children (6mo-18yr) diagnosed with nephrotic syndrome between 1993-2023 in combined Canadian cohorts. We evaluated different definitions of 1) immunosuppression response, 2) FRNS, including KDIGO and IPNA criteria, and 3) long-term remission. Outcomes were time-to-chronic kidney disease (CKD), relapse count throughout follow-up, and time-to-relapse, analyzed by Cox proportional hazards and negative binomial regression.
Results
We included 1114 children with nephrotic syndrome (median 3.8-years at diagnosis, 63% male, median 4.7-year follow-up). Of these, 1054 (95%) were steroid-sensitive, 60 (5%) were steroid-resistant (SRNS), and 73% with SRNS achieved complete remission with steroid-sparing immunosuppression. No child with treatment-responsive SRNS developed CKD. Within 1-year of diagnosis, 281 steroid-sensitive children (27%) were classified with FRNS by KDIGO and 383 (36%) by IPNA criteria. Children with FRNS by IPNA criteria (vs. KDIGO) had a similar number of relapses (adjusted rate ratio 0.95, 95%CI 0.81-1.12) and CKD risk (2% each) but received less steroid-sparing immunosuppression (hazard ratio 0.42, 95%CI 0.32-0.56).
Conclusion
Children with SRNS that achieve remission have a similar CKD risk as steroid-sensitive children. Children with FRNS by IPNA and KDIGO criteria experience similar rates of relapse and CKD. This supports defining treatment resistance by response to any immunosuppression and implementation of the IPNA FRNS criteria.
Figure. Number of relapses throughout follow up (median 4.6-years), stratified by the number of relapses by a) 6-months, b) 1-year, and c) nephrotic syndrome classification criteria by 1-year after diagnosis