Observational studyPediatric nephrology (Berlin, Germany)2021
Pediatric C3 glomerulopathy: a 12-year single-center experience.
Observational study in Pediatric nephrology (Berlin, Germany), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 13 citations in OpenAlex.
- Response to: "Age-Related Differences in Anti-Glomerular Basement Membrane Disease: Distinct Biology or Distinct Timing of Presentation?"Kidney international reports · 2026Article
- Pegcetacoplan in idiopathic and familial pediatric C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2026Observational
- C3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.Kidney medicine · 2026Review
- A Narrative Review of C3 Glomerulopathy: From Pathogenesis to Targeted Therapy.Canadian journal of kidney health and disease · 2026Review
- C3 glomerulopathy in children: a European longitudinal study evaluating outcome.Pediatric nephrology (Berlin, Germany) · 2025Article
- C3 glomerulopathy in children: experience at a resource-limited center.Clinical and experimental pediatrics · 2025Article
- Apical tubular complement activation and the loss of kidney function in proteinuric kidney diseases.Clinical kidney journal · 2024Review
- Complement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?Pediatric nephrology (Berlin, Germany) · 2024Article
- Efficacy and Safety of Iptacopan in Patients With C3 Glomerulopathy.Kidney international reports · 2023Article
- C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease.American journal of medical genetics. Part C, Seminars in medical genetics · 2022Review
- Treatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review.Medical sciences (Basel, Switzerland) · 2020Article
- Treatment and Disease Burden in Patients with Complement 3 Glomerulopathy: Multinational Real-World Study Results.Glomerular diseasesArticle
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundComplement component 3 glomerulopathy (C3G) is a disease with limited data in children. We aimed to compare childhood C3G cases with adults. We also studied subgroups of pediatric C3G and predictors of poor outcome.
methodsThis is a 12-year retrospective, single-center cohort, observational study. All cases of C3G were defined based on the 2013 consensus guidelines.
resultsC3G was diagnosed in 162 patients (119 adults, 43 pediatric) predominantly affecting males. With varied light microscopic patterns, pediatric C3G cases were categorized as follows: 23 C3 glomerulonephritis (C3GN) and 11 dense deposit disease (DDD) on electron microscopy. The pediatric DDD patients were relatively younger with more severe disease at presentation (more crescents in biopsy) but with lesser chronicity in biopsy compared with pediatric C3GN patients; however, both had a similar outcome. On comparing pediatric and adult C3G cases, adults had lower median eGFR and a higher degree of chronicity in the biopsy. The prognosis of C3G was better in pediatric patients. Predictors of kidney failure in pediatric C3G were low eGFR (HR = 0.82, p = 0.05) and severe interstitial fibrosis/tubular atrophy (HR = 1.05, p = 0.02).
conclusionsElectron microscopy-based subgroups of pediatric C3G differ in clinical presentation and course of the disease but have similar prognosis and long-term outcomes. Pediatric C3G differs from adult C3G with respect to presentation, laboratory results, biopsy features, treatment, and outcome, and as such, it should be considered as a separate entity rather than a smaller version of adult C3G.
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