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Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
Published on: August 19, 2020
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C3 glomerulopathy in children: a European longitudinal study evaluating outcome
Andrea Cappoli1, Tanja Kersnik-Levart2, Valeria Silecchia3
1Division of Nephrology, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.
Pediatric Nephrology (Berlin, Germany)
|November 26, 2024
Summary
Pediatric C3 glomerulopathy (C3G) shows a better prognosis in children than adults. Lower proteinuria and endocapillary proliferation at onset predict complete remission in children with C3G.
Area of Science:
- Nephrology
- Immunology
- Pediatric Medicine
Background:
- C3 glomerulopathy (C3G) is a rare kidney disease involving complement dysregulation.
- Limited data exist on the natural history of C3G in pediatric patients.
Purpose of the Study:
- To define the natural history and identify prognostic factors for C3G in children.
- To compare pediatric C3G outcomes with adult data.
Main Methods:
- Retrospective analysis of 108 pediatric patients diagnosed between 2011-2020 across 12 European centers.
- Data collected at baseline, 6, 12 months, and last follow-up.
- Complete remission defined by urinary protein creatinine ratio (UPCR) < 0.3 mg/mg and normal estimated glomerular filtration rate (eGFR).
Main Results:
- 65.7% of patients achieved complete remission (CR).
- Probabilities of CR were 50% at 1.8 years and 78% at 7 years.
- Predictors of CR included lower UPCR and endocapillary proliferation at presentation.
Conclusions:
- Pediatric C3G demonstrates a more favorable outcome compared to adults.
- Endocapillary proliferation and initial proteinuria levels are key prognostic indicators for pediatric C3G.
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