Related Experiment Video
Updated: Jun 7, 2026
Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
Published on: August 19, 2020
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.
Insights
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.
Background:
C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce.
Methods:
Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units. Data were collected from baseline, 6 months, 12 months and at the last follow-up. Complete remission (CR) was defined as a urinary protein creatinine ratio (UPCR) < 0.3 mg/mg with normal estimated glomerular filtration rate (eGFR). Partial remission was defined as a decrease in UPCR to 0.3 and 3 mg/mg with normal eGFR. Lack of remission was defined as non-response.
Results:
A total of 108 pediatric patients were included. Complete remission was achieved in 71/108 patients (65.7%), with probability of CR of 50% at 1.8 years and of 78% at 7 years. At presentation by univariate analysis the predictive factors at presentation associated with CR included eGFR (p = 0.028), UPCR (p = 0.004), serum C3 levels (p = 0.018), elevated plasma sC5b9 levels, defined as > 400 ng/ml, (p = 0.037), the presence of endocapillary proliferation (p = 0.017), and the absence of dense deposits on electron microscopy (p = 0.032). By multivariate analysis a low UPCR at presentation (p < 0.001) and the presence of endocapillary proliferation (p < 0.01) remained positively associated with CR.
Conclusions:
Our data confirm that C3G has a more benign outcome in children compared to previous reports in adults, and suggest that endocapillary proliferation and the degree of proteinuria at onset are the most relevant prognostic factors.
Related Concept Videos
01:26Pharmacokinetics in Pediatric Patients: Drug Excretion
01:26Nephrotic Syndrome II : Assessment and Medical Management
01:29Acute Kidney Injury III: Clinical Manifestations
01:30Acute Kidney Injury IV: Diagnostic Studies and Prevention
01:24Chronic Kidney Disease II: Clinical Manifestations
01:28Chronic Kidney Disease III: Interprofessional Care