ArticlePediatric nephrology (Berlin, Germany)2018
Outcome of membranoproliferative glomerulonephritis and C3-glomerulopathy in children and adolescents.
Article in Pediatric nephrology (Berlin, Germany), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 23 citations in OpenAlex.
- Immunofluorescence-based reclassification of idiopathic membranoproliferative glomerulonephritis: Insights from a single-center cohort.World journal of nephrology · 2026Article
- Complement diagnostics and therapeutics for the pediatric population: early successes and opportunities for further advancement.Frontiers in pharmacology · 2026Review
- Assessing C3 glomerulopathy outcomes in children: how concerned should we be?Pediatric nephrology (Berlin, Germany) · 2025Article
- C3 glomerulopathy in children: a European longitudinal study evaluating outcome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Clinical characteristics and short term outcomes of childhood immune complex membranoproliferative glomerulonephritis and C3 glomerulopathy: a single centre retrospective study.BMC nephrology · 2025Article
- Short-term renal and patient outcomes of primary immunoglobulin-associated mesangiocapillary glomerulonephritis: Insights from a developing country.World journal of nephrology · 2024Article
- Clinicopathological characteristics and outcomes of adult patients with idiopathic membranoproliferative glomerulonephritis according to an immunofluorescence-based classification.Journal of nephrology · 2024Article
- Iptacopan in Idiopathic Immune Complex-Mediated Membranoproliferative Glomerulonephritis: Protocol of the APPARENT Multicenter, Randomized Phase 3 Study.Kidney international reports · 2024Article
- Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial.Kidney international reports · 2022Article
- Second and Third Generational Advances in Therapies of the Immune-Mediated Kidney Diseases in Children and Adolescents.Children (Basel, Switzerland) · 2022Review
- Long-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.Pediatric nephrology (Berlin, Germany) · 2022Article
- Characteristics and outcomes of glomerulonephritis with membranoproliferative pattern in children.Translational pediatrics · 2021Article
- Predictors of poor kidney outcome in children with C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2021Article
- Pediatric C3 glomerulopathy: a 12-year single-center experience.Pediatric nephrology (Berlin, Germany) · 2021Observational
- Therapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis.Pediatric nephrology (Berlin, Germany) · 2021Article
- Article
- Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children.Kidney international reports · 2020Article
- Value of electron microscopy in the pathological diagnosis of native kidney biopsies in children.Pediatric nephrology (Berlin, Germany) · 2020Article
- The C18:3n6/C22:4n6 ratio is a good lipid marker of chronic kidney disease (CKD) progression.Lipids in health and disease · 2020Article
- Eculizumab for pediatric dense deposit disease: A case report and literature review.Clinical nephrology. Case studies · 2020Article
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Authors and funding
7 authors at 1 institution in 1 country.
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Abstract
backgroundMembranoproliferative glomerulonephritis (MPGN) is a rare cause of glomerulopathy in children. Recently, a new classification based on immunohistological features has been established. Infections and anomalies in complement-regulating genes, leading to alternative complement pathway activation, are suspected to trigger the disease. Nevertheless, little is known about optimal treatment and outcome in children with immune-complex-MPGN (IC-MPGN) and C3-glomerulopathy (C3G).
methodsThe method used is retrospective analysis of clinical, histological, and genetic characteristics of 14 pediatric patients with MPGN in two medical centers.
resultsMean age of the patients was 10.6 ± 4.5 years. Patients were grouped into C3G (n = 6) and IC-MPGN (n = 8). One patient showed a likely pathogenic variant in the CFHR5 gene. All 10 patients had risk polymorphisms in complement-regulating genes. Most patients were treated with ACE inhibition, steroids, and mycophenolate mofetil. Three patients with C3G received eculizumab. Median follow-up was 2.3 years. After 1 year of disease, three patients (two C3G, one IC-MPGN) reached complete, five patients partial (three IC-MPGN, two C3G), and five patients no remission (four IC-MPGN, one C3G). One patient progressed to end-stage renal disease (ESRD) 6 years after disease onset.
conclusionsIC-MPGN and C3G are rare disorders in children. Most patients have signs of complement activation associated with risk polymorphisms or likely pathogenic variants in complement-regulating genes. Steroids and mycophenolate mofetil seem to be effective and for some patients, eculizumab might be a treatment option. Outcome is heterogeneous and precise differentiation between IC-MPGN and C3G is still pending.
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